46 results on '"Hsiang-Hung Shih"'
Search Results
2. Iron‐deficiency anemia caused by duodenal paraganglioma in an adolescent
- Author
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Pei‐Chin Lin, Hsiang‐Hung Shih, Yi‐Ting Chen, and Yu‐Tang Chang
- Subjects
Medicine (General) ,R5-920 - Published
- 2022
- Full Text
- View/download PDF
3. Unusual paediatric sigmoid perivascular epithelioid cell tumour with regional lymph node metastasis treated using gemcitabine and docetaxel: a case report and literature review
- Author
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Hsiu-Chung Cheng, Chia-Yu Kuo, Ching-Wen Huang, Hsiang-Hung Shih, Chih-Hung Lin, and Jaw-Yuan Wang
- Subjects
Medicine (General) ,R5-920 - Abstract
Perivascular epithelioid cell tumour (PEComa) is an extremely rare neoplasm with distinctive morphology and specific expression of immunohistochemical markers. The lesion is typically diagnosed in middle-aged women, with few reports of paediatric cases, and there is no standardized treatment for the tumour type. Here, the case of a 17-year-old female, who presented with painless haematochezia for 2 days and was diagnosed with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis after serial examination, is presented. She was treated by surgical resection of the tumour and cytotoxic chemotherapy comprising 900 mg/m 2 gemcitabine and 100 mg/m 2 docetaxel every 3 weeks for six cycles. Haematochezia did not recur, and complete response was achieved, with progression-free survival at the 24-month follow-up examination. Surgical resection with adjuvant conventional cytotoxic chemotherapy may be considered as an option for treating gastrointestinal PEComa.
- Published
- 2021
- Full Text
- View/download PDF
4. Gastric MALT lymphoma presented with primary perforation in an adolescent: a case report
- Author
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Yu-Tang Chang, Ming-Yii Huang, Hsiang-Hung Shih, Chun-Chieh Wu, Tzu-Ying Lu, and Pei-Chin Lin
- Subjects
Gastric MALT lymphoma ,Laparoscopy ,Perforation ,Iron deficiency ,Adolescent ,Pediatrics ,RJ1-570 - Abstract
Abstract Background Primary lymphomas of the gastrointestinal tract are rare, accounting for only 1 to 4% of malignancies arising in the stomach, small intestine, or colon. The stomach is the most common extranodal site of lymphoma and gastric mucosa-associated lymphoid tissue (MALT) lymphoma accounts for 40% of primary gastric lymphoma. Gastric MALT lymphoma reaches its peak incidence between 50 to 60 years of age, therefore, it is rarely encountered in pediatric population. The presenting symptoms of gastric MALT lymphoma are usually nonspecific and primary perforation of gastric MALT lymphoma is uncommon. Case presentation A 12 year-old female presented with iron deficient anemia developed gastric perforation. Emergency laparoscopic repair of the perforation was performed and tissue pathology showed gastric MALT lymphoma infiltration. Helicobacter pylori eradication and radiotherapy were sequentially performed. Complete remission was achieved at two months after radiotherapy. To our best knowledge, she is the youngest patient with gastric MALT lymphoma reported in the literature. Conclusion Iron deficient anemia is a common presenting manifestation of malignancies in adulthood. In pediatric population, iron deficient anemia is usually caused by nutritional deficient or blood loss. In this case report, we present a teenaged female without previous gastric ulcer history who presented with a rare gastric tumor and an uncommon primary perforation. Even if there is an uncertainty about the exact diagnosis prior to the surgery, the strategy of stomach-preserving therapy by laparoscopy for primary perforation was successful and provided a good quality of life.
- Published
- 2019
- Full Text
- View/download PDF
5. Chronic Nocturnal Abdominal Pain as the Presentation of Inverted Meckel Diverticulum: A Case Report
- Author
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Ting-Yu Wang, Yu-Tsun Su, Po-Jui Ko, Yea-Ling Chen, Hsiang-Hung Shih, and Ching-Chung Tsai
- Subjects
inverted Meckel’s diverticulum ,intussusception ,nocturnal abdominal pain ,Pediatrics ,RJ1-570 - Abstract
The common clinical manifestations of Meckel’s diverticulum include painless lower gastrointestinal bleeding and intestinal obstruction due to intussusception. Intussusception induced by inverted Meckel’s diverticulum has rarely been reported; furthermore, there is no report thus far of chronic nocturnal abdominal pain as a presenting symptom in children with Meckel’s diverticulum. A 4-year-and-10-month-old girl with no significant history of previous illness presented with the sole complaint of chronic nocturnal abdominal pain for 3 months. The patient was reported to be asymptomatic during the day. A provisional diagnosis of chronic ileoileal intussusception was already under consideration in her previous hospital visits elsewhere. Physical examination revealed a soft, non-distended abdomen without tenderness. Imaging studies revealed ileoileal intussusception. Exploratory laparotomy showed ileoileal intussusception induced by an inverted Meckel’s diverticulum with ulceration. The patient underwent successful surgery and made a full recovery. We report this case to remind physicians that Meckel’s diverticulum should be considered in differential diagnosis of children presenting with the isolated symptom of chronic nocturnal abdominal pain.
- Published
- 2022
- Full Text
- View/download PDF
6. Toll-like receptor 7 agonist induces hypoplasia of the biliary system in a neonatal mouse model
- Author
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Ying-Hsien Huang, Hsiang-Hung Shih, Mao-Meng Tiao, Chao-Cheng Huang, Kuang-Che Kuo, Fu-Chen Huang, Ya-Ling Yang, and Jiin-Haur Chuang
- Subjects
Microbiology ,QR1-502 - Abstract
Background/Purpose: Viral infections and innate immunity signaling, especially Toll-like receptor 7 (TLR7) have been implicated in the pathogenesis of biliary atresia (BA). Administration of rhesus rotavirus-type A to newborn Balb/c mice produces inflammatory obstruction of bile ducts, which resembles human BA. However, whether activation of TLR7 signaling plays a role in neonatal hepatobiliary injury remains to be investigated. Methods: TLR7 agonist, imiquimod (R837), was intraperitoneally administered to Balb/c mice within 24 hours of birth and then every other day. Morphological and histological injuries of liver and gallbladder were examined at 2 weeks. Hepatic messenger RNA expression of TLR7 signaling was studied. Terminal deoxynucleotidyl transferase 2′-deoxyuridine 5′-triphosphate nick end labeling staining was used to delineate hepatobiliary apoptosis upon TLR7 stimulation. Results: TLR7 agonist, imiquimod, induced hypoplasia of the biliary system of neonatal Balb/c mice both in atrophic gallbladder and in paucity of intrahepatic bile ducts. There was significantly higher hepatic expression of TLR7 and downstream innate immunity-mediated interferon regulatory factor 7, interferon-α, and tumor necrosis factor-α. In addition, terminal deoxynucleotidyl transferase 2′-deoxyuridine 5′-triphosphate nick end labeling-positive cells in the liver were increased after injections of TLR7 agonist. Conclusion: The results demonstrate that TLR7 activation may trigger innate immunity pathways and induce apoptosis and hypoplasia of neonatal biliary trees in Balb/c mice. The novel findings give an implication of pathogenesis of infantile cholestasis, such as BA. Keywords: animal model, Balb/c mice, biliary atresia, infantile cholestasis, innate immunity, toll-like receptor 7
- Published
- 2018
- Full Text
- View/download PDF
7. Hiatal hernia: A rare cause of iron-deficiency anemia in children
- Author
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Tai-Chuan Shih, Hsiang-Hung Shih, Yu-Tang Chang, Zen-Kong Dai, and I-Chen Chen
- Subjects
Pediatrics ,RJ1-570 - Published
- 2017
- Full Text
- View/download PDF
8. Factors Contributing to Prolonged Hospitalization of Patients With Infantile Hypertrophic Pyloric Stenosis
- Author
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Kuo-Shu Tang, I-Fei Huang, Hsiang-Hung Shih, Ying-Hsien Huang, Chi-Hung Wu, Chung-Ching Lu, Fu-Chen Huang, Mao-Meng Tiao, and Chi-Di Liang
- Subjects
barium enema ,IHPS ,ultrasonography ,vomiting ,Pediatrics ,RJ1-570 - Abstract
To study the influence of clinical audit on diagnosis, complications, and factors contributing to hospitalization of patients with infantile hypertrophic pyloric stenosis. Study Design: Retrospective cohort study. Method: There were 214 patients from 1991 to 2004 from three medical centers in Kaohsiung. Data were analyzed with respect to diagnostic methods, complications, and factors requiring patient hospitalization. Results: The ratio of male to female was 4.8:1 (177 males and 37 females). The diagnoses before admission were as follows: 22% had milk intolerance and 14.5% had esophageal reflux. There was a significant increase in the use of sonogram diagnostic test (p=0.005) and a decrease in the incidence of diagnosis by olive mass palpation but not by barium meal test. Surgery time of 48 hours after admission was significant with barium meal examination and related to longer hospital stay (p
- Published
- 2011
- Full Text
- View/download PDF
9. Edema and Cirrhosis Caused by Wilson's Disease
- Author
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Jui-Yen Huang and Hsiang-Hung Shih
- Subjects
Pediatrics ,RJ1-570 - Published
- 2015
- Full Text
- View/download PDF
10. Sex, Gender, and Disease Susceptibility: A Translational Perspective
- Author
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Hsiang-Hung Shih
- Subjects
Pediatrics ,RJ1-570 - Published
- 2013
- Full Text
- View/download PDF
11. Unusual paediatric sigmoid perivascular epithelioid cell tumour with regional lymph node metastasis treated using gemcitabine and docetaxel: a case report and literature review
- Author
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Hsiang-Hung Shih, Chih-Hung Lin, Chia-Yu Kuo, Hsiu-Chung Cheng, Ching-Wen Huang, and Jaw-Yuan Wang
- Subjects
Pathology ,medicine.medical_specialty ,Medicine (General) ,Adolescent ,Perivascular Epithelioid Cell Neoplasms ,Perivascular epithelioid cell tumour ,PEComa–not otherwise specified ,Docetaxel ,Case Reports ,paediatric sigmoid PEComa ,Biochemistry ,Deoxycytidine ,Lesion ,R5-920 ,Colon, Sigmoid ,Regional lymph node metastasis ,medicine ,Neoplasm ,Humans ,case report ,PEComa ,early-stage gastrointestinal PEComa ,business.industry ,Biochemistry (medical) ,Cell Biology ,General Medicine ,medicine.disease ,Gemcitabine ,Lymphatic Metastasis ,Immunohistochemistry ,Female ,medicine.symptom ,Neoplasm Recurrence, Local ,business ,gastrointestinal PEComa ,medicine.drug - Abstract
Perivascular epithelioid cell tumour (PEComa) is an extremely rare neoplasm with distinctive morphology and specific expression of immunohistochemical markers. The lesion is typically diagnosed in middle-aged women, with few reports of paediatric cases, and there is no standardized treatment for the tumour type. Here, the case of a 17-year-old female, who presented with painless haematochezia for 2 days and was diagnosed with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis after serial examination, is presented. She was treated by surgical resection of the tumour and cytotoxic chemotherapy comprising 900 mg/m2 gemcitabine and 100 mg/m2 docetaxel every 3 weeks for six cycles. Haematochezia did not recur, and complete response was achieved, with progression-free survival at the 24-month follow-up examination. Surgical resection with adjuvant conventional cytotoxic chemotherapy may be considered as an option for treating gastrointestinal PEComa.
- Published
- 2021
12. Unusual Pediatric Sigmoid Perivascular Epithelioid Cell Tumor with Regional Lymph Node Metastasis Treated Using Gemcitabine and Docetaxel: A Case Report and Literature Review
- Author
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Hsiu-Chung Cheng, Chia-Yu Kuo, Ching-Wen Huang, Hsiang-Hung Shih, Chih-Hung Lin, and Jaw Yuan Wang
- Abstract
Background Perivascular epithelioid cell tumor (PEComa) is an extremely rare neoplasm with distinctive morphology and specific expression of immunohistochemical markers. The lesion is typically diagnosed in middle-aged women, and few pediatric cases have been reported. However, standardized treatment for the tumor type remains unestablished. Herein, we report a case of a pediatric patient with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis. The patient was successfully treated with complete tumor resection and gemcitabine/docetaxel combination chemotherapy without local or distant recurrence after 12 months postsurgery.Case presentation A 17-year-old female adolescent presented with gastrointestinal PEComa of the sigmoid colon with regional lymph node metastasis. She was treated with surgical resection of the tumor and cytotoxic chemotherapy with gemcitabine (900 mg/m2) and docetaxel (100 mg/m2) every 3 weeks. There was no recurrence of hematochezia stool, and complete response was achieved, with a progression-free survival of 12 months.Conclusion Surgical resection with adjuvant conventional cytotoxic chemotherapy can be considered as the first-line treatment for early-stage gastrointestinal PEComa.
- Published
- 2020
13. Intrapleural nasogastric tube placement: An unintentional complication indicating the cause of tension pneumothorax during esophageal balloon dilation
- Author
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Yuh-Shyan Wu, Hsiang-Hung Shih, Yung-Ho Hsu, and Yu-Tang Chang
- Subjects
Esophageal balloon ,business.industry ,Perforation (oil well) ,Nasogastric tube placement ,Pneumothorax ,Endoscopy ,Dilatation ,Tension pneumothorax ,Catheterization ,Dilation (metric space) ,Anesthesiology and Pain Medicine ,Anesthesia ,Humans ,Medicine ,business ,Complication ,Intubation, Gastrointestinal - Published
- 2021
14. Additional file 1: of Gastric MALT lymphoma presented with primary perforation in an adolescent: a case report
- Author
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Yu-Tang Chang, Ming-Yii Huang, Hsiang-Hung Shih, Chun-Chieh Wu, Lu, Tzu-Ying, and Pei-Chin Lin
- Subjects
InformationSystems_INFORMATIONINTERFACESANDPRESENTATION(e.g.,HCI) ,ComputingMilieux_PERSONALCOMPUTING ,ComputingMethodologies_DOCUMENTANDTEXTPROCESSING ,InformationSystems_MISCELLANEOUS - Abstract
Timeline. (PDF 650 kb)
- Published
- 2019
- Full Text
- View/download PDF
15. Gastric MALT lymphoma presented with primary perforation in an adolescent: a case report
- Author
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Yu-Tang Chang, Hsiang-Hung Shih, Chun-Chieh Wu, Tzu-Ying Lu, Pei-Chin Lin, and Ming-Yii Huang
- Subjects
medicine.medical_specialty ,Adolescent ,Anemia ,medicine.medical_treatment ,Gastric MALT lymphoma ,Stomach Diseases ,Case Report ,Gastroenterology ,Helicobacter Infections ,03 medical and health sciences ,0302 clinical medicine ,immune system diseases ,Stomach Neoplasms ,030225 pediatrics ,Internal medicine ,hemic and lymphatic diseases ,medicine ,Humans ,030212 general & internal medicine ,Laparoscopy ,Child ,Gastrointestinal tract ,Perforation ,medicine.diagnostic_test ,biology ,Anemia, Iron-Deficiency ,Helicobacter pylori ,business.industry ,Stomach ,Iron deficiency ,digestive, oral, and skin physiology ,lcsh:RJ1-570 ,lcsh:Pediatrics ,Lymphoma, B-Cell, Marginal Zone ,medicine.disease ,biology.organism_classification ,digestive system diseases ,Lymphoma ,Anti-Bacterial Agents ,Radiation therapy ,Lymphatic system ,medicine.anatomical_structure ,Pediatrics, Perinatology and Child Health ,Spontaneous Perforation ,Female ,business - Abstract
Background Primary lymphomas of the gastrointestinal tract are rare, accounting for only 1 to 4% of malignancies arising in the stomach, small intestine, or colon. The stomach is the most common extranodal site of lymphoma and gastric mucosa-associated lymphoid tissue (MALT) lymphoma accounts for 40% of primary gastric lymphoma. Gastric MALT lymphoma reaches its peak incidence between 50 to 60 years of age, therefore, it is rarely encountered in pediatric population. The presenting symptoms of gastric MALT lymphoma are usually nonspecific and primary perforation of gastric MALT lymphoma is uncommon. Case presentation A 12 year-old female presented with iron deficient anemia developed gastric perforation. Emergency laparoscopic repair of the perforation was performed and tissue pathology showed gastric MALT lymphoma infiltration. Helicobacter pylori eradication and radiotherapy were sequentially performed. Complete remission was achieved at two months after radiotherapy. To our best knowledge, she is the youngest patient with gastric MALT lymphoma reported in the literature. Conclusion Iron deficient anemia is a common presenting manifestation of malignancies in adulthood. In pediatric population, iron deficient anemia is usually caused by nutritional deficient or blood loss. In this case report, we present a teenaged female without previous gastric ulcer history who presented with a rare gastric tumor and an uncommon primary perforation. Even if there is an uncertainty about the exact diagnosis prior to the surgery, the strategy of stomach-preserving therapy by laparoscopy for primary perforation was successful and provided a good quality of life. Electronic supplementary material The online version of this article (10.1186/s12887-019-1431-9) contains supplementary material, which is available to authorized users.
- Published
- 2018
16. Hiatal hernia: a rare cause of iron-deficiency anemia in children
- Author
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I-Chen Chen, Yu-Tang Chang, Hsiang-Hung Shih, Tai-Chuan Shih, and Zen-Kong Dai
- Subjects
medicine.medical_specialty ,business.industry ,lcsh:RJ1-570 ,MEDLINE ,lcsh:Pediatrics ,medicine.disease ,Gastroenterology ,Hiatal hernia ,03 medical and health sciences ,0302 clinical medicine ,Iron-deficiency anemia ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,030211 gastroenterology & hepatology ,Pediatrics, Perinatology, and Child Health ,business ,030217 neurology & neurosurgery - Published
- 2016
- Full Text
- View/download PDF
17. Toll-like receptor 7 agonist induces hypoplasia of the biliary system in a neonatal mouse model
- Author
-
Hsiang-Hung Shih, Ying-Hsien Huang, Ya-Ling Yang, Jiin-Haur Chuang, Kuang-Che Kuo, Fu-Chen Huang, Mao-Meng Tiao, and Chao-Cheng Huang
- Subjects
Rotavirus ,0301 basic medicine ,Interferon Regulatory Factor-7 ,toll-like receptor 7 ,lcsh:QR1-502 ,Apoptosis ,lcsh:Microbiology ,Balb/c mice ,Mice ,0302 clinical medicine ,Immunology and Allergy ,Biliary Tract ,innate immunity ,Mice, Inbred BALB C ,Toll-like receptor ,Cholestasis ,Imiquimod ,Membrane Glycoproteins ,infantile cholestasis ,Gallbladder ,virus diseases ,General Medicine ,medicine.anatomical_structure ,Infectious Diseases ,Aminoquinolines ,030211 gastroenterology & hepatology ,Chemical and Drug Induced Liver Injury ,Agonist ,Microbiology (medical) ,medicine.medical_specialty ,medicine.drug_class ,Intrahepatic bile ducts ,biliary atresia ,Biology ,Rotavirus Infections ,03 medical and health sciences ,DNA Nucleotidylexotransferase ,Biliary atresia ,Internal medicine ,Immunology and Microbiology(all) ,medicine ,Animals ,RNA, Messenger ,General Immunology and Microbiology ,Tumor Necrosis Factor-alpha ,animal model ,Interferon-alpha ,TLR7 ,medicine.disease ,Disease Models, Animal ,030104 developmental biology ,Endocrinology ,Terminal deoxynucleotidyl transferase - Abstract
Background/Purpose: Viral infections and innate immunity signaling, especially Toll-like receptor 7 (TLR7) have been implicated in the pathogenesis of biliary atresia (BA). Administration of rhesus rotavirus-type A to newborn Balb/c mice produces inflammatory obstruction of bile ducts, which resembles human BA. However, whether activation of TLR7 signaling plays a role in neonatal hepatobiliary injury remains to be investigated. Methods: TLR7 agonist, imiquimod (R837), was intraperitoneally administered to Balb/c mice within 24 hours of birth and then every other day. Morphological and histological injuries of liver and gallbladder were examined at 2 weeks. Hepatic messenger RNA expression of TLR7 signaling was studied. Terminal deoxynucleotidyl transferase 2′-deoxyuridine 5′-triphosphate nick end labeling staining was used to delineate hepatobiliary apoptosis upon TLR7 stimulation. Results: TLR7 agonist, imiquimod, induced hypoplasia of the biliary system of neonatal Balb/c mice both in atrophic gallbladder and in paucity of intrahepatic bile ducts. There was significantly higher hepatic expression of TLR7 and downstream innate immunity-mediated interferon regulatory factor 7, interferon-α, and tumor necrosis factor-α. In addition, terminal deoxynucleotidyl transferase 2′-deoxyuridine 5′-triphosphate nick end labeling-positive cells in the liver were increased after injections of TLR7 agonist. Conclusion: The results demonstrate that TLR7 activation may trigger innate immunity pathways and induce apoptosis and hypoplasia of neonatal biliary trees in Balb/c mice. The novel findings give an implication of pathogenesis of infantile cholestasis, such as BA. Keywords: animal model, Balb/c mice, biliary atresia, infantile cholestasis, innate immunity, toll-like receptor 7
- Published
- 2016
- Full Text
- View/download PDF
18. Decreasing Rate of Biliary Atresia in Taiwan: A Survey, 2004–2009
- Author
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Hsiang-Hung Shih, Tzee-Chung Wu, An-Chyi Chen, I-Hsien Lee, Shu Ti Chiou, Yi-Chun Lin, Yen-Hsuan Ni, Wen-Chung Lee, Huey-Ling Chen, Yao Jong Yang, Beng-Huat Lau, Mei-Hwei Chang, Jia-Feng Wu, Ming-Wei Lai, Chieh-Chung Lin, Shu-Fen Liao, Mao-Meng Tiao, Hong-Yuan Hsu, Hung-Chang Lee, and Tong-Chi Tsai
- Subjects
Male ,medicine.medical_specialty ,Pediatrics ,Gross Domestic Product ,Taiwan ,medicine.disease_cause ,Rotavirus vaccination ,Biliary Atresia ,Biliary atresia ,Rotavirus ,Epidemiology ,medicine ,Humans ,business.industry ,Incidence ,Incidence (epidemiology) ,Infant, Newborn ,Rotavirus Vaccines ,medicine.disease ,Rotavirus vaccine ,Social Class ,Pediatrics, Perinatology and Child Health ,Female ,National registry ,Live birth ,business ,Cholangiography ,Demography - Abstract
OBJECTIVES: The pathogenesis of biliary atresia (BA) is unclear, but epidemiological studies may help to elucidate possible causes. The goals of this study were to identify BA incidence changes in Taiwan in 2004–2009 and to survey the factors that might influence incidence changes to elucidate the possible causes of BA. METHODS: A Taiwan national registry system for BA has been established since 2004. By using data from the national registry system for BA, we identified BA incidence changes in 2004–2009. We also evaluated the correlations between BA incidences and estimated rotavirus vaccine coverage rates and between BA incidences and the gross domestic product. RESULTS: A total of 185 patients with BA were identified in 2004–2009 in Taiwan, whereas the number of live births was 1 221 189. Compared with the incidence of BA in 2004–2006 (1.79 cases per 10 000 live births), the incidence of BA in 2007–2009 (1.23 cases per 10 000 live births) was decreased significantly (P = .01). BA incidences were negatively correlated with the gross domestic product (P = .02) and marginally negatively correlated with rotavirus vaccine coverage rates (P = .07). CONCLUSIONS: A significant decrease in BA incidence in Taiwan since 2007 has been noted and may be related to improvements in the general socioeconomic status and the popularity of rotavirus vaccination. Although more evidence is needed to establish a direct correlation, this phenomenon may shed light on possible causes of and preventive interventions for BA.
- Published
- 2011
19. Factors Contributing to Prolonged Hospitalization of Patients With Infantile Hypertrophic Pyloric Stenosis
- Author
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I-Fei Huang, Hsiang-Hung Shih, Chung-Ching Lu, Mao-Meng Tiao, Ying-Hsien Huang, Fu-Chen Huang, Chi-Hung Wu, Kuo-Shu Tang, and Chi-Di Liang
- Subjects
Male ,Clinical audit ,medicine.medical_specialty ,vomiting ,Palpation ,Pyloric Stenosis ,Cohort Studies ,Internal medicine ,medicine ,Humans ,Pediatrics, Perinatology, and Child Health ,Hypertrophic Pyloric Stenosis ,Retrospective Studies ,Barium enema ,IHPS ,medicine.diagnostic_test ,business.industry ,Incidence (epidemiology) ,Infant, Newborn ,lcsh:RJ1-570 ,Infant ,Retrospective cohort study ,lcsh:Pediatrics ,Hypertrophy ,ultrasonography ,Length of Stay ,Surgery ,Barium meal ,Pediatrics, Perinatology and Child Health ,Vomiting ,barium enema ,Female ,medicine.symptom ,business - Abstract
To study the influence of clinical audit on diagnosis, complications, and factors contributing to hospitalization of patients with infantile hypertrophic pyloric stenosis. Study Design: Retrospective cohort study. Method: There were 214 patients from 1991 to 2004 from three medical centers in Kaohsiung. Data were analyzed with respect to diagnostic methods, complications, and factors requiring patient hospitalization. Results: The ratio of male to female was 4.8:1 (177 males and 37 females). The diagnoses before admission were as follows: 22% had milk intolerance and 14.5% had esophageal reflux. There was a significant increase in the use of sonogram diagnostic test (p=0.005) and a decrease in the incidence of diagnosis by olive mass palpation but not by barium meal test. Surgery time of 48 hours after admission was significant with barium meal examination and related to longer hospital stay (p
- Published
- 2011
20. Infantile hypertrophic pyloric stenosis before 3 weeks of age in infants and preterm babies
- Author
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I-Fei Huang, Hsiang-Hung Shih, Hong-Hsiang Hu, Javier Perez Ruiz, Mao-Meng Tiao, and Christine C. Chiou
- Subjects
Pediatrics ,medicine.medical_specialty ,business.industry ,Southern taiwan ,Clinical course ,Postoperative complication ,Jaundice ,Barium meal ,Lower body ,Pediatrics, Perinatology and Child Health ,Medicine ,Symptom onset ,medicine.symptom ,business ,Hypertrophic Pyloric Stenosis - Abstract
Background: Most infantile hypertrophic pyloric stenosis (IHPS) cases are diagnosed between 3 and 12 weeks after birth. Few data exist regarding Asian infants with IHPS who are younger than 3 weeks or are preterm. The goal of this study is to identify unusual clinical manifestations, clinical course, duration of hospital stay, and complications of Asian infants with IHPS who are preterm or younger than 3 weeks of age. Methbods: From 1991 to 2004, all IHPS patients admitted to three tertiary centers in southern Taiwan were enrolled. The clinical manifestations, duration of hospital stay and complications were further compared between the IHPS patients diagnosed before and after 3 weeks; preterm and term infants. Results: A total of 214 patients were enrolled into the study; the mean age of diagnosis was 40 days of age; the average duration of hospital stay was 6.27 days. Eighteen (8.41%) patients were diagnosed before 3 weeks of age. A significantly shorter timeframe of diagnosis, a higher rate of jaundice, a lower daily body weight gain and longer duration of hospital stay were noted in the IHPS group prior to 3 weeks compared with those in IHPS group after 3 weeks. Eighteen were preterm infants. A significantly older age of symptom onset, a lower body weight at admission, more cases diagnosed by barium meal study and higher postoperative complication rates were noted in the preterm group versus full-term infants with IHPS. Conclusions: The IHPS cases diagnosed before 3 weeks of age had longer duration of hospital stay. Preterm infants with IHPS had more postoperative complications.
- Published
- 2011
21. Laparoscopy in Children With Acute Intestinal Obstruction by Aberrant Congenital Bands
- Author
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Bai-Hsiun Chen, Yu-Mei Hsin, Chi-Shu Chiou, Yu-Tang Chang, and Hsiang-Hung Shih
- Subjects
Male ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Infant, Newborn ,Surgery ,Child, Preschool ,medicine ,Humans ,Female ,Laparoscopy ,Amniotic Band Syndrome ,business ,Intestinal Obstruction - Abstract
Aberrant congenital bands are a rare cause of acute intestinal obstruction and usually present a diagnostic challenge. In 2008, the authors encountered 2 children with acute terminal ileal herniation. In the first case, it was caused by a mesodiverticular band, and numerous freely hanging filmy membranes attached to the antimesenteric side of the small intestine were found concurrently; whereas, an anomalous band from the distal ileum to the cecum was the leading cause in the second case. The vascularity of both herniated intestines was not compromised, and laparoscopy was successfully carried out 84 and 93 hours after the onset of the symptoms, respectively. Instead of cohesive adhesions, both of the causes related to a single vascular band, and laparoscopy was an effective and safe tool in diagnosis and subsequent treatment. The case with a mesodiverticular band and filmy membranes is the first case report with incomplete regression of both the vitelline circulation and the ventral mesentery.
- Published
- 2010
22. Use of Lactobacillus casei rhamnosus to Prevent Cholangitis in Biliary Atresia After Kasai Operation
- Author
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Ling-Nan Bu, Mei-Hwei Chang, Yen-Hsuan Ni, An-Chyi Chen, Hsiang-Hung Shih, I-Hsien Lee, Ming-Wei Lai, Tien-Hau Lien, Huey-Ling Chen, Hong-Yuan Hsu, and Jia-Feng Wu
- Subjects
Male ,medicine.medical_specialty ,Lactobacillus casei ,Cirrhosis ,Cholangitis ,Pilot Projects ,Portoenterostomy, Hepatic ,Body weight ,Disease-Free Survival ,Feces ,fluids and secretions ,Lactobacillus rhamnosus ,Biliary atresia ,Biliary Atresia ,Recurrence ,Lactobacillus ,medicine ,Escherichia coli ,Humans ,Recurrent cholangitis ,biology ,business.industry ,Lacticaseibacillus rhamnosus ,Probiotics ,Body Weight ,Gastroenterology ,Infant, Newborn ,food and beverages ,Infant ,Neomycin ,biology.organism_classification ,medicine.disease ,Infant newborn ,Surgery ,Anti-Bacterial Agents ,Lacticaseibacillus casei ,Pediatrics, Perinatology and Child Health ,Female ,business - Abstract
Recurrent cholangitis may aggravate cholestatic liver cirrhosis in biliary atresia (BA) after the Kasai operation. This pilot study aimed to investigate whether Lactobacillus casei rhamnosus has the prophylactic efficacy for recurrent cholangitis in comparison with the conventional neomycin prophylaxis.Twenty jaundice-free patients with BA ages 0 to 3 years who underwent a Kasai operation were enrolled and randomized into 2 groups with 10 patients each: neomycin (25 mg · kg · day for 4 days/wk) and L casei rhamnosus (8 × 10 colony-forming unit per day) groups. The treatment duration was 6 months. Bacterial stool cultures were performed before treatment and 1, 3, and 6 months after starting treatment. In addition, 10 patients with BA with similar status but without prophylaxis served as the historical control group.In the Lactobacillus group, 2 patients (20%, mean 0.03 ± 0.07 episodes per month) developed cholangitis during the study period, with the same frequency as in the neomycin group and significantly lower than that in the control group (80%, P = 0.005, mean 0.22 ± 0.16 episodes per month). The mean change in body weight z score during the 6 months in the Lactobacillus group was 0.97 ± 0.59, which was significantly better than that in the control group (-0.01 ± 0.79, P = 0.006). In bacterial stool cultures, the Lactobacillus and Escherichia coli populations significantly increased and decreased, respectively, in the Lactobacillus group.The use of L casei rhamnosus was as effective as neomycin in preventing cholangitis in patients with BA who underwent Kasai operation, and therefore could be considered as a potential alternative prophylactic regimen.
- Published
- 2014
23. Promoter Polymorphism of the CD14 Endotoxin Receptor Gene Is Associated With Biliary Atresia and Idiopathic Neonatal Cholestasis
- Author
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Suh-Hang Hank Juo, Hock-Liew Eng, Hsiang-Hung Shih, Tsun-Mei Lin, Fu-Chen Huang, Jiin-Haur Chuang, Chao-Long Chen, and Huey-Ling Chen
- Subjects
Adult ,Liver Cirrhosis ,Male ,medicine.medical_specialty ,Genotype ,Lipopolysaccharide Receptors ,Single-nucleotide polymorphism ,Polymorphism, Single Nucleotide ,Gene Frequency ,Cholestasis ,Biliary Atresia ,Biliary atresia ,Internal medicine ,Humans ,Medicine ,Neonatal cholestasis ,Receptors, Immunologic ,Allele ,Child ,Promoter Regions, Genetic ,Allele frequency ,Hepatitis ,Tumor Necrosis Factor-alpha ,business.industry ,Homozygote ,Infant, Newborn ,medicine.disease ,Endocrinology ,Pediatrics, Perinatology and Child Health ,Immunology ,Female ,business - Abstract
Objective.To investigate whether single-nucleotide polymorphisms in the promoter regions of endotoxin-responsive genes CD14 and tumor necrosis factor-α (TNF-α) are associated with biliary atresia (BA) and idiopathic neonatal cholestasis (INC).Methods.We obtained genomic DNA from 90 patients with established diagnosis of BA and 28 patients with INC. Forty-two adult patients with hepatitis B–related cirrhosis and 143 healthy children served as control populations. The genotypes of CD14/C(−159)T and TNF-α/G(−308)A (G allele, TNF*1; A allele, TNF*2) were determined by using a restriction enzyme–based assay. Plasma soluble CD14 levels were determined in different disease stages and genotypes of BA.Results.The frequencies of T allele and T/T homozygosity of the CD14/−159 promoter polymorphism were significantly higher in patients with BA (T allele: 61.7%; T/T genotype: 42.2%) and in patients with INC (T allele: 67.9%; T/T genotype: 53.6%) but not in control populations. Decrease of plasma soluble CD14 from the early stage of BA when the patients received a Kasai operation to the late stage of liver cirrhosis was observed in carriers of the T/T and T/C genotypes but not in carriers of the C/C genotype. The TNF-α/−308 promoter polymorphisms (TNF*1 and TNF*2) were not associated with BA.Conclusion.These findings show that the single-nucleotide polymorphism at CD14/−159 is associated with the development of BA and INC. Endotoxin susceptibility may play a role in the pathogenesis of infantile cholestasis.
- Published
- 2005
24. Reduced Apoptosis in Newborn Compared to Adult Rat Intestine after Ischemia-Reperfusion Injury
- Author
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Chih Cheng Luo, Wei Chun Ma, Hsiang Hung Shih, Cheng Hsun Chiu, and Hui Ying Chung
- Subjects
Aging ,Programmed cell death ,Pathology ,medicine.medical_specialty ,Ischemia ,Apoptosis ,Vascular occlusion ,Rats, Sprague-Dawley ,Andrology ,Intestine, Small ,In Situ Nick-End Labeling ,Animals ,Medicine ,TUNEL assay ,business.industry ,medicine.disease ,Small intestine ,Rats ,medicine.anatomical_structure ,Animals, Newborn ,Reperfusion Injury ,Pediatrics, Perinatology and Child Health ,Necrotizing enterocolitis ,medicine.symptom ,business ,Reperfusion injury ,Developmental Biology - Abstract
The pathogenesis of necrotizing enterocolitis (NEC) is unknown. Ischemia and reperfusion (I/R) injury has been considered to be a major contributing factor. More recent reports have noted that apoptosis is a significant and perhaps the principal contributor to cell death after I/R. Some reports revealed that infants with NEC and perforated bowel can completely recover with drainage alone. This study aims to assess the ability of newborn rat intestine to resist apoptosis after I/R injury compared with adult rat intestine. Intestines from 10 groups of rats (n = 6 for each study group) were studied: (1) normal control group; (2) ischemia group, receiving vascular occlusion for 60 min; (3) I/R groups receiving vascular occlusion for 60 min and reperfusion for 15, 30, and 60 min, respectively. Apoptosis was quantified by TUNEL methods. Statistical analysis was performed using ANOVA with Dunn’s test. TUNEL-positive cells per 10 crypts were significantly increased in the ischemia and I/R groups compared to the control group. The peak number of positive cells by TUNEL was recognized 30 min after reperfusion in adult and newborn rats and then reduced gradually. The newborn rats had significantly less TUNEL-positive cells per 10 crypts than adult rats subjected to I/R injury (p < 0.05). We demonstrated that the activation of apoptosis occurred after intestinal I/R injury, especially during the reperfusion phase. The newborn intestine was more resistant to I/R injury and thus may have significant clinical application.
- Published
- 2004
25. Bacterial Translocation of Coagulase-Negative Staphylococci in Rats following Intestinal Ischemia-Reperfusion Injury
- Author
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Cheng Hsun Chiu, Chih Cheng Luo, Hsiang Hung Shih, and Jer N. Lin
- Subjects
Pathology ,medicine.medical_specialty ,biology ,Ischemia ,Chromosomal translocation ,biology.organism_classification ,medicine.disease ,medicine.disease_cause ,Small intestine ,Microbiology ,Sepsis ,medicine.anatomical_structure ,Bacteremia ,Pediatrics, Perinatology and Child Health ,medicine ,Reperfusion injury ,Staphylococcus ,Bacteria ,Developmental Biology - Abstract
Many patients with sepsis have bacteremia for which no septic focus is identified either clinically or by autopsy. This study was designed to determine the relationship between the ischemia-reperfusion injury (IRI) and bacterial translocation that might be involved in the pathogenesis of necrotizing enterocolitis. In the first experiment, a total of 32 Sprague-Dawley rats weighing 150–200 g were divided into four groups. The mesentery to isolated loop was occluded for 30, 60, and 90 min following 30-min reperfusion in the three groups of experimental animals with a micro-bulldog clamp. A control group involved the same technique and exposure, without occlusion of the mesentery. Two sets of blood culture were taken through a catheter in the portal vein immediately and 15 min after the reperfusion, respectively. In another experiment, bacteria isolated were fed in different doses to control rats and those after 30- or 60-min ischemia and 30-min reperfusion. Two sets of blood culture were taken following the procedure. Invasion and transcytosis of the bacteria through epithelial cells were studied in vitro using a Madin-Derby canine kidney (MDCK) cell monolayer model. PCR for delta toxin gene was performed on all bacteria isolated, using Staphylococcus epidermidis as the control. Coagulase-negative staphylococci (CoNS) were invariably isolated from mice with prolonged ischemia (90 min) and reperfusion. When bacteria were fed into mice with only 30-min ischemia, an inoculum as low as 5 × 105 CFU/ml could induce bacteremia. No bacterial translocation was found in control mice even fed with a higher dose of bacteria (5 × 108 CFU/ml). In vitro experiments showed that CoNS failed to transcytose MDCK monolayer. These isolates were not cytotoxic to MDCK cells and contained no delta toxin gene. Bacterial translocation of CoNS occurred following severe bowel ischemia and reperfusion injury. Intact mucosa integrity readily prevented bacterial translocation; however, bacterial translocation could occur in rats following mild IRI in the presence of a higher number of CoNS in the gut.
- Published
- 2004
26. Role of hepatitis B immunoglobulin in infants born to hepatitis B e antigen-negative carrier mothers in Taiwan
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Ching Chuan Liu, Mei-Hwei Chang, Meng Feng Lee, Yao Jong Yang, Sheng Hsien Chen, Hsiang Hung Shih, and Te Jen Chen
- Subjects
Male ,Microbiology (medical) ,medicine.medical_specialty ,HBsAg ,Hepatitis B vaccine ,medicine.medical_treatment ,Taiwan ,Immunoglobulins ,Passive immunity ,medicine.disease_cause ,Risk Assessment ,Gastroenterology ,Drug Administration Schedule ,Statistics, Nonparametric ,Group B ,Orthohepadnavirus ,Pregnancy ,Internal medicine ,medicine ,Humans ,Hepatitis B Vaccines ,Serologic Tests ,Hepatitis B e Antigens ,Prospective Studies ,Pregnancy Complications, Infectious ,Probability ,Hepatitis B virus ,Dose-Response Relationship, Drug ,biology ,business.industry ,Infant, Newborn ,Pregnancy Outcome ,virus diseases ,Hepatitis B ,biology.organism_classification ,digestive system diseases ,Treatment Outcome ,Infectious Diseases ,Hepadnaviridae ,HBeAg ,Case-Control Studies ,Carrier State ,Pediatrics, Perinatology and Child Health ,Immunology ,Female ,business ,Follow-Up Studies - Abstract
The efficacy of hepatitis B immunoglobulin (HBIG) in infants of hepatitis B e antigen (HBeAg)-negative hepatitis B surface antigen (HBsAg) carrier mothers in Taiwan is not clear.To describe the responses of infants born to HBeAg-negative carrier mothers receiving HBIG combined with hepatitis B vaccine.Term babies born to HBeAg-negative carrier mothers were assigned based on chart number to 1 of the 2 treatment groups. Group A infants (n = 94) received 0.5 ml (145 IU) of HBIG within 24 h of birth and 3 subsequent doses of recombinant hepatitis B virus (HBV) vaccine at 3 to 5 days, 1 month and 6 months of age. Group B infants (n = 122) received 3 doses of vaccines only. Infants (n = 19) born to HBeAg-positive carrier mothers were treated like those in Group A and are referred to as Group C. Sera obtained from infants at 2 and 7 months of age were tested for hepatitis B virus (HBV) markers.There were 2 (1%; one in Group A and one in Group B) subclinical breakthrough hepatitis B infections among studied infants. One (5%) child of Group C had asymptomatic HBV infection at the age of 7 months and became a chronic carrier. The rate of protective anti-hepatitis B surface antibody (anti-HBs) titers achieved (10 mIU/ml) by 2 months of age was significantly higher in Group A than that in Group B (98% vs. 57%, P0.001). However, it was not different by 7 months of age. Infants (Group A) immunized with HBIG and vaccine had a significantly higher geometric mean titer (GMT, milli-International Units/ml) of anti-HBs than those (Group B) with vaccines only at 2 months of age (P0.001). Conversely at 7 months of age, the GMT of anti-HBs was significantly higher in infants who received vaccine only (P = 0.001).A protective level of antibodies was achieved earlier in those infants receiving both passive and active immunizations. However, infants receiving active immunizations alone achieved a higher GMT at 7 months of age. There was no clear benefit of passive-active vs.active immunization alone for chronic HBV infection in infants of HBsAg-positive, HBeAg-negative mothers.
- Published
- 2003
27. Edema and Cirrhosis Caused by Wilson's Disease
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Hsiang-Hung Shih and Jui-Yen Huang
- Subjects
Liver Cirrhosis ,Male ,medicine.medical_specialty ,Cirrhosis ,Bilirubin ,Slit Lamp Microscopy ,Gastroenterology ,Excretion ,chemistry.chemical_compound ,Hepatolenticular Degeneration ,Edema ,Internal medicine ,Ascites ,medicine ,Humans ,Hypoalbuminemia ,Pediatrics, Perinatology, and Child Health ,Child ,business.industry ,Penicillamine ,lcsh:RJ1-570 ,lcsh:Pediatrics ,medicine.disease ,eye diseases ,Wilson's disease ,chemistry ,Pediatrics, Perinatology and Child Health ,sense organs ,medicine.symptom ,Tomography, X-Ray Computed ,business ,medicine.drug - Abstract
Figure 1 Kayser-Fleischer ring, copper deposition in Descemet’s membrane, was revealed as a crescent at the top of the cornea (arrow) by ocular slit-lamp examination. A previously healthy 11-year-old boy had puzzling pitting edema of his lower legs for 5 weeks prior to presentation, as well as progressive weight gain (46e52 kg), puffy eyelids, and scrotal edema. Laboratory tests showed severe hypoalbuminemia (1.67 g/dL), absence of urinary protein, and hypertransaminasemia (AST 311 IU/L, ALT 72 IU/L, bilirubin 0.97/0.26 mg/dL). Ocular slit-lamp examination revealed yellowish cloudy crescent Kayser-Fleischer rings at the upper cornea (Figure 1). An axial computed tomography scan of the abdomen demonstrated cirrhotic liver with encapsulated and intraperitoneal ascites (Figure 2). Wilson’s disease with Child’s class C cirrhosis was confirmed by low serum ceruloplasmin (8.65 mg/dL; normal range, 20e35 mg/dL) and high urinary copper excretion (353.8 mg in 24 hours; normal
- Published
- 2015
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28. Long term immune response of universal hepatitis B vaccination in infancy: a community-based study in Taiwan
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Hong-Yuan Hsu, Ding-Shinn Chen, Ping-Ing Lee, Yen-Hsuan Ni, Mei-Hwei Chang, and Hsiang-Hung Shih
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Male ,Microbiology (medical) ,Immunization, Secondary ,Taiwan ,Immune system ,medicine ,Humans ,Hepatitis B Vaccines ,Hepatitis B Antibodies ,Child ,Hepatitis B Surface Antigens ,Booster (rocketry) ,biology ,Immunization Programs ,business.industry ,Infant, Newborn ,Infant ,Hepatitis B ,medicine.disease ,Vaccination ,Infectious Diseases ,Immunization ,Hepatitis b vaccination ,Pediatrics, Perinatology and Child Health ,Immunology ,biology.protein ,Female ,Viral disease ,Antibody ,business ,Immunologic Memory - Abstract
To evaluate the long term immunity provided by a universal hepatitis B vaccination program in infancy and the booster effect on school age children who had no protective antibody titers to hepatitis B surface antigen.We conducted a community-based seroepidemiologic study of 1337 healthy 7-year-old children in Taiwan one decade after the implementation of a mass hepatitis B vaccination program. A booster vaccination was suggested for noncarrier children who did not have protective titers of surface antibody. Serologic responses and infection rates were compared with those of the nonboostered children. In a nonselected group of 39 volunteer noncarrier vaccinees, quantitative serologic response was determined before, 1 month after a booster vaccination and 1 year later.A total of 572 children (42.8%) had low concentrations of surface antibody, and 9 were hepatitis B surface antigen carriers (0.7%). Eighty-two percent of "nonprotected" vaccinees showed immunologic memory to a booster dose and developed protective antibody titers 1 month later; 60.6% maintained protective titers 1 year later. The frequency of new hepatitis B virus infection was similar for those who received a booster and those who did not as investigated by the core antibody seroconversion during 1-year follow-up. However, the risk was low, with annual incidences of1% in both groups, and none became chronic carriers.According to these data a universal vaccination program in infancy provides adequate protection against hepatitis B virus infection for school age children and a booster vaccination is not recommended.
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- 1999
29. Infantile facioscapulohumeral muscular dystrophy revisited: Expansion of clinical phenotypes in patients with a very short EcoRI fragment
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Chin Wen Lin, Yuh-Jyh Jong, Kanako Goto, Ichizo Nishino, Pei Lun Lee, Tai-Heng Chen, Yukiko K. Hayashi, Chao Ching Huang, Wen Fu Wang, Hsiang Hung Shih, Wen-Chen Liang, Jong Hau Hsu, and Yu Hung Lai
- Subjects
Adult ,Male ,medicine.medical_specialty ,Weakness ,Pediatrics ,Adolescent ,Hearing loss ,Hearing Loss, Sensorineural ,Severity of Illness Index ,Deoxyribonuclease EcoRI ,Cohort Studies ,Epilepsy ,Young Adult ,Intellectual Disability ,Severity of illness ,medicine ,Facioscapulohumeral muscular dystrophy ,Humans ,Muscular dystrophy ,Child ,Genetics (clinical) ,Genetic Association Studies ,Retrospective Studies ,business.industry ,medicine.disease ,Muscular Dystrophy, Facioscapulohumeral ,Surgery ,Blotting, Southern ,Heart Block ,Neurology ,Pediatrics, Perinatology and Child Health ,Sensorineural hearing loss ,Female ,Neurology (clinical) ,medicine.symptom ,Age of onset ,Chromosomes, Human, Pair 4 ,business ,Gene Deletion - Abstract
Contrary to the classical form, infantile facioscapulohumeral muscular dystrophy (FSHD) usually denotes a severe phenotype and is frequently associated with extramuscular involvements. To elucidate the genotype-phenotype correlation in this severe subgroup, we identified a cohort of nine patients with infantile FSHD who also carried a very short (10-13kb) EcoRI fragment. Their current age ranged from 8 to 33 years and age of onset ranged from 0.4 to 5 years. One patient even manifested his first FSHD-related symptoms at as early as 5 months of age, including inability to smile, poor response to call, and infantile spasms. To date, four patients were wheelchair-bound and six patients had asymmetric weakness. Sensorineural hearing loss and abnormal fundoscopic findings were observed in eight and all of patients respectively. Three with the smallest EcoRI fragments (10-11kb, with normal length being 50-300kb) had mental retardation. Two of these had epilepsy. Cardiac arrhythmias were found in five patients. Restrictive ventilatory defects were observed in seven patients, with one progressing to chronic respiratory failure. Two had swallowing difficulties; one of these required gastrostomy. We identified several rarely reported phenotypes in infantile FSHD, including cardiac arrhythmia, respiratory insufficiency, and swallowing difficulties. There seems to be a correlation between the severity of phenotype and the very short EcoRI fragment in the chromosome 4q35 region. We conclude that the high frequency of multi-organ involvements in this severe FSHD variant suggests the need for an early and multidisciplinary intervention.
- Published
- 2012
30. Infantile hypertrophic pyloric stenosis before 3 weeks of age in infants and preterm babies
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I-Fei, Huang, Mao-Meng, Tiao, Christine C, Chiou, Hsiang-Hung, Shih, Hong-Hsiang, Hu, and Javier Perez, Ruiz
- Subjects
Male ,Postoperative Complications ,Age Factors ,Infant, Newborn ,Taiwan ,Humans ,Female ,Pyloric Stenosis, Hypertrophic ,Infant, Premature, Diseases ,Length of Stay ,Retrospective Studies - Abstract
Most infantile hypertrophic pyloric stenosis (IHPS) cases are diagnosed between 3 and 12 weeks after birth. Few data exist regarding Asian infants with IHPS who are younger than 3 weeks or are preterm. The goal of this study is to identify unusual clinical manifestations, clinical course, duration of hospital stay, and complications of Asian infants with IHPS who are preterm or younger than 3 weeks of age.From 1991 to 2004, all IHPS patients admitted to three tertiary centers in southern Taiwan were enrolled. The clinical manifestations, duration of hospital stay and complications were further compared between the IHPS patients diagnosed before and after 3 weeks; preterm and term infants.A total of 214 patients were enrolled into the study; the mean age of diagnosis was 40 days of age; the average duration of hospital stay was 6.27 days. Eighteen (8.41%) patients were diagnosed before 3 weeks of age. A significantly shorter timeframe of diagnosis, a higher rate of jaundice, a lower daily body weight gain and longer duration of hospital stay were noted in the IHPS group prior to 3 weeks compared with those in IHPS group after 3 weeks. Eighteen were preterm infants. A significantly older age of symptom onset, a lower body weight at admission, more cases diagnosed by barium meal study and higher postoperative complication rates were noted in the preterm group versus full-term infants with IHPS.The IHPS cases diagnosed before 3 weeks of age had longer duration of hospital stay. Preterm infants with IHPS had more postoperative complications.
- Published
- 2010
31. HLA typing associated with hepatitis B E antigen seroconversion in children with chronic hepatitis B virus infection: a long-term prospective sibling cohort study in Taiwan
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Chi-Rong Li, Yen-Hsuan Ni, Jia-Feng Wu, Wen-Yi Shau, Hsiang-Hung Shih, Huey-Ling Chen, Hong-Yuan Hsu, Chen-Hsin Chen, Chih-Yao Chiang, Mei-Hwei Chang, Rhong-Phong Hsieh, and Yi-Hau Chen
- Subjects
Male ,HBsAg ,Adolescent ,Taiwan ,Human leukocyte antigen ,medicine.disease_cause ,Cohort Studies ,Hepatitis B, Chronic ,Antigen ,HLA-DQ Antigens ,Medicine ,Humans ,Hepatitis B e Antigens ,Seroconversion ,Prospective cohort study ,Child ,Hepatitis B virus ,Polymorphism, Genetic ,business.industry ,Histocompatibility Testing ,Siblings ,Histocompatibility Antigens Class I ,Infant, Newborn ,virus diseases ,Infant ,HLA-DR Antigens ,Hepatitis B ,medicine.disease ,digestive system diseases ,HBeAg ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Immunology ,Female ,business - Abstract
Objective To conduct a prospective cohort study to clarify the relationship between human leukocyte antigen (HLA) polymorphisms and the seroconversion of hepatitis B e antigen (HBeAg). Study design In the prospective cohort study, 81 HBeAg-positive children with chronic hepatitis B virus (HBV) infection from 40 unrelated families were recruited and followed-up regularly for a mean period of 17.70 ± 3.23 years. The association between HLA antigen and the age at HBeAg seroconversion was analyzed using Cox regression model with shared frailties under left truncation and right censorship. Results HLA-B61 and HLA-DQB1*0503 antigens predicted a higher HBeAg seroconversion rate (relative incidence = 6.17 and 3.22, P = .024 and .017, respectively). Within-family frailty in our sibling cohort study demonstrated a negligible or a low degree of within-family correlation with spontaneous HBeAg seroconversion in each HLA antigen. Conclusions HLA class I antigen B61 and class II antigen DQB1*0503 are associated with earlier HBeAg seroconversion in Taiwanese children with chronic HBV infection.
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- 2005
32. Fecal elastase 1, serum amylase and lipase levels in children with cholestasis
- Author
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Wan-Hsin Wen, Mei-Hwei Chang, Yen-Hsuan Ni, Wen-Ming Hsu, Hong-Shiee Lai, Huey-Ling Chen, and Hsiang-Hung Shih
- Subjects
Male ,medicine.medical_specialty ,Fecal elastase ,Endocrinology, Diabetes and Metabolism ,Hepatobiliary Disorder ,Cholestasis, Intrahepatic ,Gastroenterology ,Feces ,Cholestasis ,Biliary atresia ,Biliary Atresia ,Internal medicine ,Alagille syndrome ,medicine ,Humans ,Amylase ,Lipase ,Child ,Hepatology ,biology ,Pancreatic Elastase ,business.industry ,Progressive familial intrahepatic cholestasis ,Infant ,medicine.disease ,Alagille Syndrome ,Child, Preschool ,Choledochal Cyst ,Amylases ,biology.protein ,Female ,business - Abstract
The pancreatic functions of children with cholestatic liver diseases were unclear. Due to anatomic vicinity and common ontogenic origin, hepatobiliary disorders of infancy may also affect pancreatic function. The aim of the study was to evaluate the exocrine pancreatic function and common pancreatic function tests in children with cholestatic disorders.In 40 children with cholestasis, fecal elastase 1 (FE1) concentrations were measured. Serum amylase and lipase values were tested. The diagnoses included 32 patients with extrahepatic cholestasis (biliary atresia (BA) and choledochal cyst), and 8 patients with intrahepatic cholestasis (progressive familial intrahepatic cholestasis and Alagille syndrome). None had renal insufficiency or clinical symptoms/signs of acute pancreatitis.All the patients had normal FE1 (200 microg/g). Nineteen percent (7/37) had elevated serum amylase levels (100 U/l). Thirty-two percent (12/37) had elevated serum lipase levels above the normal (120 U/l). Seventy-three percent (8/11) of BA patients with bilirubin2 mg/dl had elevated serum lipase levels compared to 18% (3/17) with bilirubinor = 2 mg/dl (p = 0.0036). None had detectable pancreatic abnormality on ultrasonography and magnetic resonance images.None of the cholestatic children in this study had exocrine pancreatic insufficiency as detected by FE1. Hyperamylasemia and/or hyperlipasemia were frequently found. In children with BA, those with impaired biliary excretion tended to have elevated serum pancreatic enzymes as compared with those who had no jaundice. A decreased hepatic metabolism may be the cause.
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- 2004
33. Congenital chloride diarrhea in a child
- Author
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Wen-Chieh, Li, Hsiang-Hung, Shih, Kun-Lin, Wu, and Chien-Chen, Chou
- Subjects
Diagnosis, Differential ,Diarrhea ,Male ,Feces ,Chlorides ,Child, Preschool ,Humans - Abstract
Congenital chloride diarrhea (CLD) is a rare autosomal recessive disease characterized by persistent, lifelong, watery diarrhea with high fecal chloride concentration. It results from a defect of the bicarbonate/chloride exchange system in the distal ileum and colon. Polyhydramnios, premature birth, ileus without meconium passage, hypochloremia, and hyponatremia are typical features of CLD in the neonate, followed by chronic metabolic alkalosis, hypokalemia, hypochloremia, retarded growth, and renal impairment in older children and adults if the disease is not adequately treated. The diagnosis of CLD is highly dependent on the alertness of physicians. Prompt recognition and adequate replacement of fecal loss of chloride, sodium, potassium, and water are mandatory for satisfactory disease outcome. We report a case of CLD complicated with recurrent episodes of ileus, metabolic alkalosis, and hypokalemia causing frequent hospitalization in a 4-year-old boy. Normalized electrolytes and gasometric parameters, decreased abdominal circumference, and growth catch-up were achieved after education of the parents about daily care, and provision of adequate fluid and electrolyte supplementation.
- Published
- 2003
34. Translocation of coagulase-negative bacterial staphylococci in rats following intestinal ischemia-reperfusion injury
- Author
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Chih-Cheng, Luo, Hsiang-Hung, Shih, Cheng-Hsun, Chiu, and Jer-Nan, Lin
- Subjects
Coagulase ,L-Lactate Dehydrogenase ,Staphylococcus ,Colony Count, Microbial ,Staphylococcal Infections ,Rats ,Intestines ,Rats, Sprague-Dawley ,Intestinal Diseases ,Mice ,Dogs ,Bacterial Translocation ,Reperfusion Injury ,Sepsis ,Animals ,Intestinal Mucosa - Abstract
Many patients with sepsis have bacteremia for which no septic focus is identified either clinically or by autopsy. This study was designed to determine the relationship between the ischemia-reperfusion injury (IRI) and bacterial translocation that might be involved in the pathogenesis of necrotizing enterocolitis. In the first experiment, a total of 32 Sprague-Dawley rats weighing 150-200 g were divided into four groups. The mesentery to isolated loop was occluded for 30, 60, and 90 min following 30-min reperfusion in the three groups of experimental animals with a micro-bulldog clamp. A control group involved the same technique and exposure, without occlusion of the mesentery. Two sets of blood culture were taken through a catheter in the portal vein immediately and 15 min after the reperfusion, respectively. In another experiment, bacteria isolated were fed in different doses to control rats and those after 30- or 60-min ischemia and 30-min reperfusion. Two sets of blood culture were taken following the procedure. Invasion and transcytosis of the bacteria through epithelial cells were studied in vitro using a Madin-Derby canine kidney (MDCK) cell monolayer model. PCR for delta toxin gene was performed on all bacteria isolated, using Staphylococcus epidermidis as the control. Coagulase-negative staphylococci (CoNS) were invariably isolated from mice with prolonged ischemia (90 min) and reperfusion. When bacteria were fed into mice with only 30-min ischemia, an inoculum as low as 5 x 10(5) CFU/ml could induce bacteremia. No bacterial translocation was found in control mice even fed with a higher dose of bacteria (5 x 10(8) CFU/ml). In vitro experiments showed that CoNS failed to transcytose MDCK monolayer. These isolates were not cytotoxic to MDCK cells and contained no delta toxin gene. Bacterial translocation of CoNS occurred following severe bowel ischemia and reperfusion injury. Intact mucosa integrity readily prevented bacterial translocation; however, bacterial translocation could occur in rats following mild IRI in the presence of a higher number of CoNS in the gut.
- Published
- 2002
35. Adenocarcinoma of the colon in children presenting as abdominal pain: report of two cases
- Author
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Hsiang-Hung, Shih, Chung-Ching, Lu, Mao-Meng, Tiao, Sheung-Fat, Ko, and Jiin-Haur, Chuang
- Subjects
Male ,Adolescent ,Colonic Neoplasms ,Humans ,Adenocarcinoma ,Abdominal Pain - Abstract
Adenocarcinoma of the colon is an unusual disease in patients under 30 years of age, and generally presents as advanced disease because of a lack of awareness of its occurrence, especially in the pediatric age group. The authors report on 2 cases of colon cancer in children less than 17 years old, whose initial presentations were abdominal pain of unclear etiology and non-specific abdominal complaints. No other abnormal laboratory results were found except that 1 patient had anemia. Barium studies revealed the typical colon lesions in both patients, and colonoscopic pathologic examination disclosed mucinous adenocarcinoma. The first patient was rather unusual in that 2 separate tumors were found simultaneously in the large intestine. The therapeutic approach included surgery and adjuvant chemotherapy, but both patients ultimately died due to poor response and early recurrence. Survival obviously depends on the extent of the disease at diagnosis; the earlier the diagnosis is, the better the prognosis will be. Clinical characteristics, diagnosis, and treatment of colon cancer in pediatric patients are discussed.
- Published
- 2002
36. Prevalence and Risk Factors for Feeding and Swallowing Difficulties in Spinal Muscular Atrophy Types II and III
- Author
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Chang-Hung Kuo, Yuh-Jyh Jong, Tai-Heng Chen, Hsiang-Hung Shih, and Yen-Shan Chen
- Subjects
Adult ,Male ,medicine.medical_specialty ,Pediatrics ,Adolescent ,Spinal Muscular Atrophies of Childhood ,Aspiration pneumonia ,Pneumonia, Aspiration ,Sitting ,Eating ,Young Adult ,Swallowing ,Risk Factors ,medicine ,Humans ,Risk factor ,Child ,business.industry ,digestive, oral, and skin physiology ,Spinal muscular atrophy ,Middle Aged ,medicine.disease ,SMA ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Physical therapy ,Female ,Underweight ,medicine.symptom ,Deglutition Disorders ,Choking ,business - Abstract
Objective To identify the prevalence and risk factors of feeding and swallowing problems in patients with type II and type III spinal muscular atrophy (SMA). Study design Cross-sectional data from 108 genetically confirmed patients with SMA (age range, 3-45 years; 60 with type II and 48 with type III) were analyzed. The questionnaire survey included demographic data, current motor function and respiratory status, feeding and swallowing difficulties, and consequences. The risk factors were analyzed via logistic regression. Results The 3 most common feeding and swallowing difficulties in patients with type II and III SMA were choking (30.6%), difficulty conveying food to the mouth (20.4%), and difficulty chewing (20.4%). Current motor function status was an independent risk factor for feeding and swallowing difficulties (sitters vs walkers: OR, 7.59; 95% CI, 1.22-47.46). All 4 nonsitters (ie, patients with type II SMA who had lost their sitting ability) had feeding and swallowing difficulties. Patients with feeding and swallowing difficulties had significantly higher rates of underweight and aspiration pneumonia than those without these problems. Conclusion Patients with type II and III SMA have a high prevalence of risk factors for feeding and swallowing difficulties, suggesting that an individualized treatment plan should depend on current motor function status.
- Published
- 2012
37. Congenital muscular dystrophy with fatty liver and infantile-onset cataract caused by TRAPPC11 mutations: broadening of the phenotype.
- Author
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Wen-Chen Liang, Wenhua Zhu, Satomi Mitsuhashi, Satoru Noguchi, Sacher, Michael, Megumu Ogawa, Hsiang-Hung Shih, Yuh-Jyh Jong, and Ichizo Nishino
- Subjects
MUSCULAR dystrophy diagnosis ,FATTY liver ,CATARACT diagnosis ,GENETIC mutation ,PHENOTYPES ,HEPATOMEGALY ,DIAGNOSIS - Abstract
Background: Transport protein particle (TRAPP) is a multiprotein complex involved in endoplasmic reticulum-to-Golgi trafficking. Zebrafish with a mutation in the TRAPPC11 orthologue showed hepatomegaly with steatosis and defects in visual system development. In humans, TRAPPC11 mutations have been reported in only three families showing limb-girdle muscular dystrophy (LGMD) or myopathy with movement disorders and intellectual disability. Methods: We screened muscular dystrophy genes using next-generation sequencing and performed associated molecular and biochemical analyses in a patient with fatty liver and cataract in addition to infantile-onset muscle weakness. Results: We identified the first Asian patient with TRAPPC11 mutations. Muscle pathology demonstrated typical dystrophic changes and liver biopsy revealed steatosis. The patient carried compound heterozygous mutations of a previously reported missense and a novel splice-site mutation. The splice-site change produced two aberrantly-spliced transcripts that were both predicted to result in translational frameshift and truncated proteins. Full-length TRAPPC11 protein was undetectable on immunoblotting. Conclusion: This report widens the phenotype of TRAPPC11-opathy as the patient showed the following: (1) congenital muscular dystrophy phenotype rather than LGMD; (2) steatosis and infantile-onset cataract, both not observed in previously reported patients; but (3) no ataxia or abnormal movement, clearly indicating that TRAPPC11 plays a physiological role in multiple tissues in human. [ABSTRACT FROM AUTHOR]
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- 2015
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38. Use of Lactobacillus casei rhamnosus to Prevent Cholangitis in Biliary Atresia After Kasai Operation.
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Tien-Hau Lien, Ling-Nan Bu, Jia-Feng Wu, Huey-Ling Chen, An-Chyi Chen, Ming-Wei Lai, Hsiang-Hung Shih, I-Hsien Lee, Hong-Yuan Hsu, Yen-Hsuan Ni, and Mei-Hwei Chang
- Published
- 2015
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39. P0381 ENDOGENOUS NITRIC OXIDE IN EXHALED AIR FROM CHILDREN WITH LIVER CIRRHOSIS
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Chyong-Hsin Hsu, Cheng-Yi Wang, C. Huang, Mao-Meng Tiao, and Hsiang-Hung Shih
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medicine.medical_specialty ,Cirrhosis ,business.industry ,Internal medicine ,Pediatrics, Perinatology and Child Health ,Gastroenterology ,medicine ,Endogenous nitric oxide ,medicine.disease ,business ,Exhaled air - Published
- 2004
40. P0192 PROMOTER POLYMORPHISM OF THE CD14 ENDOTOXIN RECEPTOR GENE IS ASSOCIATED WITH BILIARY ATRESIA
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Jiin-Haur Chuang, S. Juo, Hsiang-Hung Shih, T. Lin, and Fu-Chen Huang
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business.industry ,Biliary atresia ,CD14 ,Pediatrics, Perinatology and Child Health ,Gastroenterology ,Promoter polymorphism ,Medicine ,Receptor ,business ,medicine.disease ,Gene ,Molecular biology - Published
- 2004
41. EPC Society News
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Paola Capelli, Kenzo Kaneko, Christina Manti, Thomas Kirchner, Masahiko Kawamoto, Ahmet Gülçubuk, R. McMahon, Özlem Güzel, Kenji Shirono, Christoph Herold, Giulia Zamboni, Changbin Shi, Sven-Börje Ewers, Ken Shiozawa, Hsiang-Hung Shih, I. Schimke, Yohei Mizuta, Detlef Ockert, Hafize Uzun, S.H. Rahman, Shinitiro Makimoto, Yutaka Nagasaki, A.K. Siriwardena, M.J. McMahon, Michael Safioleas, Bhanu P. Jena, Alberto Fusco, C. Shields, Giancarlo Mansueto, Mei-Hwei Chang, Aydın Gürel, Philippe Bulois, C.A. Jacobi, H. Guski, Patrick Hastier, Tohru Nakagoe, Kengo Shirahane, Kazuhiro Kikuta, Eike Staub, Kenji Tanaka, Ralf Jesenofsky, Masao Tanaka, Alexandre Pariente, Masahiro Satoh, Volker Keim, Konstantinos G. Moulakakis, Fumiaki Nozawa, Akira Hayashibe, Louis Buscail, Kıvılcım Sönmez, Parviz M. Pour, Xia Zhao, Arnaud Boruchowicz, Koji Yamaguchi, Seval Aydin, Gwen Lomberk, Kenichi Souda, Martin E. Fernandez-Zapico, Ingemar Ihse, Daniel Neureiter, E.J. Balthazar, Susumu Higuchi, Matthias Löhr, Roberto Malago, Atsushi Masamune, A. Neumann, Dag Dittert, Matthias Ocker, Huey-Ling Chen, Ingo Alldinger, Katsuya Maruyama, Marc Barthet, Hajime Isomoto, Matthew Mulvahill, Ravindra S Date, Matthias Peiper, Masamichi Nagasawa, Hans-Detlev Saeger, Gustavo B. Baretton, Yance Lele Dapawole, Jean Louis Gineston, Nezahat Gürler, D. Schofield, Michael Sachs, F.A. Wenger, C.S. Verbeke, Takeshi Nakamoto, Masafumi Nakamura, Neil V. McFerran, Henri Licht, M.K. Walz, Hong-Shiee Lai, Philippe Ruszniewski, Steffen Zopf, Kazuki Sakamoto, Kiyoshi Kume, Mark A. Taylor, Masahiro Matsushita, Akira Horii, Ken Ohnita, Eckhart G. Hahn, Xiangdong Wang, François Mauvais, Alkiviadis Kostakis, Aleksandar Jeremic, Roland Andersson, Rania Abu-Hamdah, Marie L. Kelly, Kazuo Ohba, Shigeru Kohno, Yoshimasa Kobayashi, Masaya Shinbo, I. Heukamp, Karl-Göran Tranberg, Hiroyuki Konomi, Kenji Kimura, Alain Courrier, Wen-Ming Hsu, Massimo Falconi, Hiroshi Ohara, Alina-Elena Ilie, Christian Pilarsky, H.P. Redmond, Arno Dimmler, Fuminao Takeshima, R. Albazaz, Dermot O'Toole, J.I. Gregor, Wan-Hsin Wen, Yen-Hsuan Ni, Mirko D'Onofrio, T Diamond, Sang-Joon Cho, M. Kilian, Yuko Akazawa, Thierry Thevenot, Tooru Shimosegawa, Simone Vasori, Dongming Su, Kennichi Satoh, Lütfiye Öksüz, Yoshihiko Sato, Anders Ask, Philippe Lévy, Masayukii Ohta, J. Hardman, Ajith K. Siriwardena, Gennaro Chiappetta, Jean Claude Duchmann, Yuji Nakamura, Damian J. Mole, Jean-Louis Frossard, Katsuhisa Omagari, Gert Lindell, Tohru Yasutake, Robert Grützmann, Hiroya Mizutamari, Sebastian Stintzing, Noriaki Suzuki, and Kemal Altunatmaz
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Hepatology ,business.industry ,Endocrinology, Diabetes and Metabolism ,Gastroenterology ,Media studies ,Medicine ,business - Published
- 2002
42. LONG-TERM IMMUNE RESPONSE OF UNIVERSAL HEPATITIS B VACCINATION IN INFANCY: A COMMUNITY-BASED STUDY IN TAIWAN
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Yen-Hsuan Ni, Mei-Hwei Chang, Hsiang-Hung Shih, Ping-Ing Lee, and Hong-Yuan Hsu
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Immune system ,Hepatitis b vaccination ,business.industry ,Pediatrics, Perinatology and Child Health ,Immunology ,Gastroenterology ,Medicine ,business ,Community based study ,Term (time) - Published
- 1999
43. Promoter Polymorphism of the CD14 Endotoxin Receptor Gene Is Associated With Biliary Atresia and Idiopathic Neonatal Cholestasis.
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Hsiang-Hung Shih, Tsun-Mei Lin, Jiin-Haur Chuang, Hock-Liew Eng, Suh-Hang Hank Juo, Fu-Chen Huang, Chao-Long Chen, and Huey-Ling Chen
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ENDOTOXINS , *BILIARY atresia , *CHOLESTASIS in newborn infant , *DNA restriction enzymes , *BILE duct abnormalities - Abstract
Objective. To investigate whether single-nucleotide polymorphisms in the promoter regions of endotoxin-responsive genes CD14 and tumor necrosis factor-α (TNF-α) are associated with biliary atresia (BA) and idiopathic neonatal cholestasis (INC). Methods. We obtained genomic DNA from 90 patients with established diagnosis of BA and 28 patients with INC. Forty-two adult patients with hepatitis B-related cirrhosis and 143 healthy children served as control populations. The genotypes of CD14/C(-159)T and TNF-α/G(-308)A (G allele, TNF*1; A allele, TNF*2) were determined by using a restriction enzyme-based assay. Plasma soluble CD14 levels were determined in different disease stages and genotypes of BA. Results. The frequencies of T allele and T/T homozygosity of the CD14/-159 promoter polymorphism were significantly higher in patients with BA (T allele: 61.7%; T/T genotype: 42.2%) and in patients with INC (T allele: 67.9%; T/T genotype: 53.6%) but not in control populations. Decrease of plasma soluble CD14 from the early stage of BA when the patients received a Kasai operation to the late stage of liver cirrhosis was observed in carriers of the T/T and T/C genotypes but not in carriers of the C/C genotype. The TNF-α/-308 promoter polymorphisms (TNF*1 and TNF*2) were not associated with BA. Conclusion. These findings show that the single-nucleotide polymorphism at CD14/-159 is associated with the development of BA and INC. Endotoxin susceptibility may play a role in the pathogenesis of infantile cholestasis. [ABSTRACT FROM AUTHOR]
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- 2005
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44. Fecal Elastase 1, Serum Amylase and Lipase Levels in Children with Cholestasis.
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Wan-Hsin Wen, Huey-Ling Chen, Mei-Hwei Chang, Yen-Hsuan Ni, Hsiang-Hung Shih, Hong-Shiee Lai, and Wen-Ming Hsu
- Abstract
Background/Aim: The pancreatic functions of children with cholestatic liver diseases were unclear. Due to anatomic vicinity and common ontogenic origin, hepatobiliary disorders of infancy may also affect pancreatic function. The aim of the study was to evaluate the exocrine pancreatic function and common pancreatic function tests in children with cholestatic disorders. Methods: In 40 children with cholestasis, fecal elastase 1 (FE1) concentrations were measured. Serum amylase and lipase values were tested. The diagnoses included 32 patients with extrahepatic cholestasis (biliary atresia (BA) and choledochal cyst), and 8 patients with intrahepatic cholestasis (progressive familial intrahepatic cholestasis and Alagille syndrome). None had renal insufficiency or clinical symptoms/signs of acute pancreatitis. Results: All the patients had normal FE1 (>200 μg/g). Nineteen percent (7/37) had elevated serum amylase levels (>100 U/l). Thirty-two percent (12/37) had elevated serum lipase levels above the normal (>120 U/l). Seventy-three percent (8/11) of BA patients with bilirubin >2 mg/dl had elevated serum lipase levels compared to 18% (3/17) with bilirubin ≤2 mg/dl (p = 0.0036). None had detectable pancreatic abnormality on ultrasonography and magnetic resonance images. Conclusions: None of the cholestatic children in this study had exocrine pancreatic insufficiency as detected by FE1. Hyperamylasemia and/or hyperlipasemia were frequently found. In children with BA, those with impaired biliary excretion tended to have elevated serum pancreatic enzymes as compared with those who had no jaundice. A decreased hepatic metabolism may be the cause. Copyright © 2005 S. Karger AG, Basel and IAP [ABSTRACT FROM AUTHOR]
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- 2005
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45. Role of hepatitis B immunoglobulin in infants born to hepatitis B e antigen-negative carrier mothers in Taiwan.
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YAO-JONG YANG, CHING-CHUAN LIU, TE-JEN CHEN, MENG-FENG LEE, SHENG-HSIEN CHEN, HSIANG-HUNG SHIH, and MEI-HWEI CHANG
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- 2003
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46. Congenital muscular dystrophy with fatty liver and infantile-onset cataract caused by TRAPPC11 mutations: broadening of the phenotype
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Satomi Mitsuhashi, Ichizo Nishino, Yuh-Jyh Jong, Hsiang-Hung Shih, Michael Sacher, Wenhua Zhu, Satoru Noguchi, Megumu Ogawa, and Wen-Chen Liang
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Pathology ,medicine.medical_specialty ,Transport protein particle (TRAPP) ,Steatosis ,Congenital muscular dystrophy ,Fatty liver ,Muscle weakness ,Case Report ,Cell Biology ,Biology ,Bioinformatics ,medicine.disease ,Compound heterozygosity ,Cataract ,3. Good health ,medicine ,Missense mutation ,Orthopedics and Sports Medicine ,Muscular dystrophy ,medicine.symptom ,Myopathy ,Molecular Biology ,Endoplasmic reticulum-to-Golgi trafficking - Abstract
Background Transport protein particle (TRAPP) is a multiprotein complex involved in endoplasmic reticulum-to-Golgi trafficking. Zebrafish with a mutation in the TRAPPC11 orthologue showed hepatomegaly with steatosis and defects in visual system development. In humans, TRAPPC11 mutations have been reported in only three families showing limb-girdle muscular dystrophy (LGMD) or myopathy with movement disorders and intellectual disability. Methods We screened muscular dystrophy genes using next-generation sequencing and performed associated molecular and biochemical analyses in a patient with fatty liver and cataract in addition to infantile-onset muscle weakness. Results We identified the first Asian patient with TRAPPC11 mutations. Muscle pathology demonstrated typical dystrophic changes and liver biopsy revealed steatosis. The patient carried compound heterozygous mutations of a previously reported missense and a novel splice-site mutation. The splice-site change produced two aberrantly-spliced transcripts that were both predicted to result in translational frameshift and truncated proteins. Full-length TRAPPC11 protein was undetectable on immunoblotting. Conclusion This report widens the phenotype of TRAPPC11-opathy as the patient showed the following: (1) congenital muscular dystrophy phenotype rather than LGMD; (2) steatosis and infantile-onset cataract, both not observed in previously reported patients; but (3) no ataxia or abnormal movement, clearly indicating that TRAPPC11 plays a physiological role in multiple tissues in human.
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