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Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a group of systemic small vasculitis characterized by ANCA positive in serum. Three diseases are included in this group of diseases: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). In China, standardized diagnosis and treatment of AAV is still lacking. Based on the evidence and guidelines from China and abroad, the Chinese Rheumatology Association formulated the standardization of diagnosis and treatment of ANCA associated vasculitis. The purpose is to standardize the diagnosis of AAV and disease activity assessment, and recommend the treatment strategies.抗中性粒细胞胞质抗体（ANCA）相关血管炎（AAV）是一组以血清中能检测到ANCA为最突出特点的系统性小血管炎。经典AAV包括肉芽肿性多血管炎、显微镜下多血管炎和嗜酸性肉芽肿性多血管炎。我国AAV的规范化诊断与治疗依然欠缺，中华医学会风湿病学分会在借鉴国内外诊治经验和指南的基础上，制定了本规范，旨在规范AAV的诊断与疾病活动度评估，对患者的治疗策略予以建议。.

In recent years, the clinical experts consensuses or guidelines of ankylosing spondylitis (AS)/spondyloarthritis (SpA) have been constantly updated, but to better understand and practice, patient self-participation management is one of the key points to improve the level of diagnosis and treatment. Through questionnaire survey of these patients, we screened out the most concerned issues, and established the AS/SpA patient practice guideline working group with multidisciplinary physicians and patients. Fifteen opinions, as the AS/SpA patient practice guidelines, are proposed in accordance with the relevant principles of the "WHO guidelines development manual" , and with the international normative process.近年来，强直性脊柱炎（AS）/脊柱关节炎（SpA）临床专家共识或指南不断更新，但如何更好地理解与执行，患者自我参与管理是提高诊疗水平的关键环节之一。通过成立多学科专家和患者共同参与的AS/SpA患者实践指南工作组，开展AS/SpA病友会患者与专家共同参与的AS/SpA患者关注问题的调查，筛选出问题，采用《世界卫生组织指南制订手册》相关原则，按照国际规范流程，提出AS/SpA患者实践指南意见15条。.

Hyperuricemia/gout is a common metabolic disease in China, which is a serious threat to people's health. In clinical practice, the standardization of prevention and diagnosis and the rate of treat-to-target need to be improved. There is still a lack of education for the patients about the understanding of clinical guidelines, the disease knowledge and the importance of cooperating with doctors to carry out diagnosis and treatment. From the most concerned issues of the patients, we established the hyperuricemia/gout patient practice guideline working group with multidisciplinary physicians and patients. Seventeen opinions, as the hyperuricemia/gout patient practice guidelines, are proposed in accordance with the relevant principles of the "WHO guidelines development manual" , and with the international normative process, aiming to improve the patients compliance, improve the level of health management of the disease.高尿酸血症/痛风是我国日趋递增的常见代谢性疾病，严重威胁人民健康。临床上，该病诊断的规范性及临床治疗达标率均有待提高，其中还欠缺针对患者对该病专家临床指南的理解、疾病知识普及和配合医生执行规范诊疗的重要性的教育。为此，从患者最关切的问题出发，成立多学科专家和患者共同参与的患者实践指南工作组，采用《世界卫生组织指南制订手册》相关原则，按照国际规范流程，提出高尿酸血症/痛风患者实践指南的意见17条，旨在提高患者诊治的依从性，提高该病健康管理水平。.

A plasmonic two-wire transmission-line is experimentally exploited for the demonstration of second-harmonic generation (SHG) despite of material and geometric symmetries. Interestingly, regardless of the two potential modes excited at the fundamental frequency, the resulting SHGs are only coupled into the asymmetric mode. This characteristic is explained through surface nonlinear polarization calculations.

We demonstrate a novel scheme for photonic generation of chirped millimeter-wave (MMW) pulse with ultrahigh time-bandwidth product (TBP). By using a fast wavelength-sweeping laser with a narrow instantaneous linewidth, wideband/high-power photonic transmitter-mixers, and heterodyne-beating technique, continuously tunable chirped MMW waveforms at the W-band are generated and detected through wireless transmission. Compared with the reported optical grating-based wavelength-to-time mapping techniques for chirped pulse generation, our approach eliminates the problem in limited frequency resolution of grating, which seriously limits the continuity, tunability, and TBP of the generated waveform. Furthermore, by changing the alternating current (AC) waveform of the driving signal to the sweeping laser, linearly or nonlinearly continuously chirped MMW pulse can be easily generated and switched. Using our scheme, linearly and nonlinearly chirped pulses with record-high TBPs (89-103 GHz/ 50 μs/7 × 105) are experimentally achieved.

Unrepaired DNA double-strand breaks can lead to apoptosis or tumorigenesis. In mammals double-strand breaks are repaired mainly by nonhomologous end-joining mediated by the DNA–PK complex. The core protein of this complex, DNA–PKcs, is a DNA-dependent serine/threonine kinase that phosphorylates protein targets as well as itself. Although the (auto)phosphorylation activity has been shown to be essential for repair of both random double-strand breaks and induced breaks at the immunoglobulin locus, the corresponding phosphatase has been elusive. In fact, to date, none of the putative phosphatases in DNA double-strand break repair has been identified. Here we show that protein phosphatase 5 interacts with DNA–PKcs and dephosphorylates with surprising specificity at least two functional sites. Cells with either hypo- or hyperphosphorylation of DNA–PKcs at these sites show increased radiation sensitivity.

The present authors report the first case of Beare-Stevenson syndrome in Taiwan. The patient shares several clinical characteristics of Beare-Stevenson syndrome such as cutis gyrata, cloverleaf skull, prominent eyes, cleft palate, ear defects and a protruding umbilical stump. Molecular genetic analysis of the FGFR2 gene in this patient's DNA revealed a missense A --> G mutation on nucleotide 1303 of the FGFR2 cDNA. This mutation leads to a Tyr --> Cys substitution at residue 375 located at the N-terminal end of the transmembrane domain of FGFR2. The present results are in accordance with other previously published reports and strengthen the importance of the FGFR2 gene in the pathogenesis of Beare-Stevenson syndrome.

Lactoferrin (LF), traditionally known as an iron-binding protein present in high concentrations in milk and various secretions, has emerged as a multifunctional protein involved in many aspects of the host defense against infection. Recently, LF has been shown to inhibit the growth of solid tumors and reduce experimental metastasis in mice, suggesting that LF also may play a role in the defense against tumorigenesis. Here we provide the sequence of the cDNA and promoter region, the chromosome assignment, and tissue expression pattern of a novel form of LF mRNA (delta LF). The sequence of delta LF mRNA is nearly identical to that of LF mRNA; however, at the 5' end, we find a novel sequence that replaces the N-terminal signal peptide sequence of LF mRNA. We map the delta LF mRNA to human chromosome 3 and find that both delta LF and LF sequences colocalize to the same cloned 90- to 150-kb genomic DNA fragment. We further show that the delta LF mRNA is the product of alternative splicing of the LF gene and likely is specified by use of an alternative promoter. Although we find delta LF mRNA at various levels in 20 of 20 adult and fetal human tissues, we do not find delta LF mRNA in any of 14 diverse tumor-derived cell lines.

To investigate the underlying molecular mechanisms of renal cell carcinoma (RCC) by using the microarray expression profiles of normal kidney and RCC tissue for early diagnosis and treatment of RCC.The gene expression profile of GES781 was downloaded from Gene Expression Omnibus database, including including nine tissue samples of RCC tissues removed from nine patients and eight adjacent normal renal tissue samples. We identified the differentially expressed genes (DEGs) by Multtest package in R software. The screened DEGs were further analyzed by bioinformatics methods. Firstly, the comparison of the DEGs expression degree was performed by cluster analysis. Secondly, DAVID was used to perform functional analysis of up- and down- regulated genes and the protein-protein interaction (PPI) networks were constructed by prePPI. Finally, the pathways of genes in PPI networks were discovered by WebGestalt.Compared with the control, we screened 648 down-regulated and 681 up-regulated DEGs. And the down- and up-regulated DEGs with maximum expression degree were UMOD (uromodulin) and FABP7 (fatty acid binding protein 7), respectively. There was significant difference in the gene expression between the normal kidney and RCC tissue. The up-regulated DEGs in RCC tissue were significantly related to the immune responses and the down-regulated DEGs were significantly related to the oxidation reduction. The most significant pathway in the PPI network of UMOD was cytokine-cytokine receptor interaction.The screened DEGs have the potential to become candidate target molecules to monitor, diagnose and treat the RCC, and might be beneficial for the early diagnosis and medication control of RCC.

A size exclusion liquid chromography (HPSEC) method using refractive index (RI) and photodiode array (PDA) detectors and a BASIC calculation program was developed for high molecular weight contaminant determination in the C15 linear paraffin-based cold rolling oil. Eight laboratory standards were analyzed to determine the new HPSEC/RI calibration factor for the C15 linear paraffin rolling oil. Three contaminants and thirteen standard solutions were evaluated to establish calibration curves. The HPSEC/RI/PDA method will quantitatively determine the concentrations of polyisobutylene-based hydrodynamic backup bearing lubricant, mist-applied extreme pressure gear oil for the workroll bearing and antiwear hydraulic oil in the cold rolling oil. Deviations for backup bearing lubricant, workroll bearing lubricant and hydraulic oil are approximately 3%, 4% and 7% of the contaminant concentration, respectively. The BASIC calculation program can assist operators in reducing calculation error and improving s...

We demonstrate a 10-Gb/s optical-carrier-distributed network with wireless communication system. The 10-Gb/s W-band wireless-signal was generated by a near-ballistic-uni-traveling-carrier-photodiode (NBUTD-PD) based transmitter optically excited by 0.1-THz optical pulses. 20-km optical fiber upstream transmission is achieved.

By using optimized shaped optical pulses, we demonstrate a factor-of-∼25 enhancement in the spectral power density of sub-THz wave at 93 GHz compared with that for quasi-sinusoidal optical modulation.

In order to lower costs and improve functionality in a quick turn-around time, the module-in-systems, such as the multi-chip module (MCM) and 3-D packages, have been applied in the integrated circuits (ICs) industry. The wire bonding technology, providing versatile and reliable chip-connection method, is usually adopted for MCM and 3-D packages. Wire sweep, denotes visible wire deformation, typically a lateral movement in the direction of the compound flow through the cavity, is usually a main concern in the semiconductor packaging industry. Wire sweep can cause device shorting and/or current leakage leading to IC failure. Thus the influence of wire sweep becomes critical on semiconductor package. Wire sweep has been proved to be closely related to bond span and bond height of a wire bond. However, die thickness and bond height vary from chip to chip within MCM and 3-D packages. In the paper, an attempt is made to investigate the effects of die thickness and bond height of a wire bond on their wire sweep. The concept of sweep stiffness defined by the slope of the load-transverse displacement curves is employed in representing the sweep resistance of a wire bond. At the MCM level, four die thicknesses, 160, 200, 250 and 550mum, are tested and their sweep stiffness are then compared with numerical results.

A simple processing technology for oxide confined vertical-cavity surface-emitting lasers fabrication is reported in this article. Results of the burn-in test up to the present-day show that the high temperature lifetime of the fabricated devices exceeded 3300 hours. The eye diagrams show that the devices can be modulated up to 10 Gbps.

We report the results of our very large aperture, oxide-confined vertical-cavity surface-emitting lasers (OC-VCSELs). The apertures of the laser-emitting windows are 300 to 500 /spl mu/M in diameter. P-type metal grid lines were also formed within the aperture for better current spreading. A maximum cw optical power of 42.7 mW was measured for a 500 /spl mu/m aperture device.

Summary form only given. In recent years there have been great advances on the fabrication techniques of fiber gratings and fiber lasers. The combination of the two technologies has enabled the emergence of new types of fiber lasers. We report the performance of an active harmonic modelocked. Er-fiber laser stabilized by a reflective fiber grating Fabry-Perot resonator. As is well known, typical harmonic modelocked fiber lasers are not be stable if no additional stabilization scheme is used. This is because there are many supermodes which compete with each other for maximum gain and thus make the laser unstable. In the literature several methods have been developed to stabilize a harmonic modelocked fiber laser. Among these methods, using an intracavity Fabry-Perot resonant filter which has a longitudinal mode separation that matches the Nth harmonic repetition frequency of the cavity is the most direct and efficient method since it forces the laser to lase at a single supermode. We adopt this approach and use a reflective fiber grating Fabry-Perot resonator as the stabilization filter. Compared to the reported works in the literature where a bulk transmission Fabry-Perot resonator is used, our approach has the advantages of being all fiber-integrated and being able to simultaneously achieve wavelength selection, laser stabilization, and dispersion compensation without requiring additional optical components in the cavity. These advantages should be valuable if a compact and stable harmonic modelocked fiber laser is to be built.

The present authors report the first case of Beare-Stevenson syndrome in Taiwan. The patient shares several clinical characteristics of Beare-Stevenson syndrome such as cutis gyrata, cloverleaf skull, prominent eyes, cleft palate, ear defects and a protruding umbilical stump. Molecular genetic analysis of the FGFR2 gene in this patient's DNA revealed a missense A --G mutation on nucleotide 1303 of the FGFR2 cDNA. This mutation leads to a Tyr --Cys substitution at residue 375 located at the N-terminal end of the transmembrane domain of FGFR2. The present results are in accordance with other previously published reports and strengthen the importance of the FGFR2 gene in the pathogenesis of Beare-Stevenson syndrome.

Non-Hodgkin's lymphoma usually involves the central nervous system by metastatic disease. Primary spinal epidural non-Hodgkin's lymphoma (PSENL) is a relatively rare cause of spinal cord compression. It mainly occurs in adults past the 4th or 5th decades. This entity is even rarer in children. The proper treatment modalities are controversial in adults with PSENL. Radiotherapy is the main strategy after surgery; the role of chemotherapy is uncertain. Therapeutic experience in childhood PSENL is extremely limited. We report a 10-year-old boy presenting with backache and bilateral lower leg weakness after minor trauma. Small non-cleaved cell non-Hodgkin's lymphoma of the epidural space was proven after subtotal tumor removal. Other investigations including computed tomography of the chest and abdomen, bone scan, gallium scan, bone marrow aspiration, and cerebrospinal fluid study were all negative for occult disease. The patient received combined therapy with irradiation and chemotherapy after surgery. Esophageal stricture resulting from radiotherapy developed during treatment and colon interposition was performed. He has remained disease free 42 months after the diagnosis with normal functional status.

To understand the functions of a protein, it often requires cloning of the other cellular proteins that interact with it. Several approaches have been used by biologists to clone new genes based on protein-protein interactions, including co-immunoprecipitation, affinity purification, and the yeast two-hybrid system. The yeast two-hybrid cDNA cloning technology is a powerful in vivo protein-protein interaction assay first introduced in 1989 by Fields et al. (Fields and Song 1989; Chien et al. 1991; Chevray and Nathans 1992; Durfee et al. 1993; Zervos et al. 1993; Mendelsohn and Brent 1994). It is based on co-expression of two proteins, X and Y, fused to GAL4 DNA binding domain (GAL4B) and GAL4 transcription activation domain (GAL4A) respectively (Fig. 1 A). If the protein X interacts with the protein Y, the GAL4 transcription activation domain will be brought to the promoter containing the GAL4 DNA binding sites and will activate the transcription of reporter gene HIS3 or lacZ. The two-hybrid system can be used to clone cDNA encoding a novel protein that interacts with a known protein (bait) in yeast. It can also be used to study protein-protein interactions between two known proteins.

To investigate if there are microsatellite loci in the long arm of chromosome 6 that have close relationship with non-small cell lung cancer, Multiple PCR approach was used to analyze the 36 loci in the long arm of chromosome 6. The PCR products were analyzed in PAGE and then the electrophoresis maps were analyzed with Genescan and Genotyper. There is different LOH frequency in different loci. The total frequency of LOH in 41 lung cancers was 78%(32/41), with the highest frequency of LOH was detected on the locus D6S302(75%). There are 14 loci which have LOH frequency more than 20% and the loci are mainly located in 2 regions: 6q16.3-q21 [6 loci D6S458 (21.43%), D6S1694 (26.92%), D6S1717 (35.71%), D6S1565 (40%), D6S302 (75%), D6S1706 (36.36%) and 6q26-q27 (5 loci D6S1550 (38.46%), D6S264 (20%), D6S1585 (25%), D6S446 (33.33%), D6S281 (30.77%)], There may be tumor suppressor genes located in the two regions, which have a close relationship with non-small cell lung cancer.

Neonatal ascites is an uncommon problem that may be caused by a number of etiologies including diseases of genitourinary system and gastrointestinal system, cardiac disease, hepatic disease, systemic infection such as TORCH or parvovirus, chylous, ovarian cause, inborn error of metabolism and idiopathic. We reported two cases of neonatal ascites, one was caused by cytomegalovirus and no obvious causes could be detected in the second one. The ascites were diagnosed by prenatal ultrasound at the gestational age of 25 weeks and 37 weeks respectively and were resolved spontaneously after birth. One-year follow-up of both cases revealed normal growth and development. No recurrent ascites could be detected by abdominal sonography except for evidence of mild hepatomegaly that was noted in case 1. Thus, isolated fetal and neonatal ascites without other concomitant abnormalities were diagnosed, a separate entity from non-immune hydrops fetalis with excellent prognosis.

A 10-year-old girl was admitted for abdominal pain for 1 week. Morning vomiting with 5 Ascaris and diarrhea with Ascaris were found. Radiograph of the abdomen disclosed no significant abnormality. Abdominal sonogram revealed a normal biliary tree; but mildly enlarged pancreatic thickness, and thickened gallbladder wall. Within the thickened gallbladder wall a linear echogenic structure with worm-like movement suspected of being Ascaris was found. We report this case because pediatric pancreatitis and a gallbladder wall thickened with worm-movement have rarely been reported. Urgent treatment and surgery are required for the very ill child with a tensely distended abdomen or signs of peritoneal irritation. Early diagnosis is very important to prevent further complications. We emphasize the role of sonography in the diagnosis of this case and the prevention of progressive deterioration.

Acute appendicitis is the most common surgically amenable cause of acute abdominal pain in children. We analyzed our past experience of appendectomies in children and present the clinical characteristics of appendicitis in children younger than 3 years of age. A better understanding of appendicitis in early childhood would allow us to achieve an earlier diagnosis.A group of 475 children from 4 months to 15 years of age who underwent appendectomy for appendicitis was studied over a 5-year period from July 1994 to June 1999. Excluding cases with negative pathological findings (n = 34), they were divided into 2 age groups: group I (or = 3 years old) and group II (3 years old). Medical records were reviewed and comparisons between clinical findings, laboratory data, pathology findings, and complications were made.Of 441 cases enrolled in our study, 24 (5.4%) were 3 years of age or younger. Of all children older than 3 years of age, 32 (7.1%) had negative pathological findings compared to children younger than 3 years of age (7.7%). The duration of symptoms prior to diagnosis in group I was 3.6 days compared to group II at 2.0 days. Children from group I frequently showed a higher incidence of fever (90% vs. 53.4%), abdominal distention (50% vs. 9.8%), perforation (50% vs. 40.1%), and missed first impression (29% vs. 2.4%) than those from group II. Children of group I also had a higher complication rate (41.7% vs. 11.5%).In early childhood the symptoms and signs of appendicitis usually are nonspecific. There is a longer duration before diagnosis, more instances of fever and abdominal distention, less right lower quadrant pain, less local tenderness and rebounding pain, and no obturator sign. Close observation and on-going evaluation of patients are essential.

To localize cytochrome P450 enzymes 4A and 2C in central nervous cells of normal male rats.Eight drug/alcohol untreated normal male rats (150-200 g of body weight) were treated by the optimized perfusion technique, then brain tissues were postfixed, paraffin-embedded and cut into series sections, which were labeled by the improved strept-avidin-biotin complex DAB-nickel enhancer (SABC-DAB-Ni) immunohistochemistry and hematoxylineosin (HE) stain techniques.The immunohistochemical results indicated that P450 2C-11 enzyme was localized in diverse numbers of neurons as well as some neuroglial cells, with focal or defuse distribution in many brain regions such as cerebrum, thalamus, olfactory bulb, hypothalamus, brain-stem, hippocampus, cerebellum, interpositus nucleus, caudate-putamen, and globus pallidus. In contrast, no positive findings of P450 4A-2, 3 and 8 enzymes were obtained in the same animals. With high magnification, 2C-11 protein was able to be roughly observed on the endoplasmic reticulum of the rat neurons.P450 2C-11 protein, rather than P450 4A-2, 3 and 8, may be a candidate of brain P450 enzymes in the normal male rats.

We reviewed the histories of four children (2 boys and 2 girls, aged 6 mo to 10 yr) with surgically proven bezoars, treated from 1991 to 1998. Two had gastric bezoars and two had intestinal bezoars. All four patients presented with gastrointestinal symptoms and none had undergone previous surgery. Three had a history of abnormal ingestion, including one who ate a pickled fruit from the Boraginaceae (locally known as Po-pu-tsu) plant for 1 year. Plain radiographs revealed intestinal obstruction in all four patients. Abdominal sonography disclosed a hyperechoic band-like lesion and acoustic shadow in both cases of gastric bezoar, and these bezoars were subsequently confirmed by gastric endoscopy. Lower gastrointestinal studies showed obstruction considered most likely to be due to bezoar in one case, while computed tomography (CT) revealed a characteristic small bowel mottled gas pattern in another. Surgical treatment relieved obstruction in all four patients. Bezoar should be suspected in patients with intestinal obstruction who have a history of ingestion of unusual substances. The findings of this report suggest that sonography and endoscopy are useful in the diagnosis of gastric bezoar, while CT is useful in the detection of intestinal bezoar.

Hypertrophic pyloric stenosis is commonly seen in infants 2 to 4 weeks old. We report a case of pyloric stenosis diagnosed in a boy 5 months and 11 days old suffering from the sudden onset of vomiting. Gastric volvulus was initially diagnosed at another hospital. Abdominal ultrasonography at first using an Acuson 5-MHz transducer revealed a negative diagnosis. However, a tubular pyloric mass measuring 5.5 mm in thickness, 15 mm in the transverse diameter, and 2.0 cm in length was detected by a 7-MHz transducer immediately after the infant vomited. On physical examination, no abdominal mass was palpable. This suggested that this might have been a case of hypertrophic pyloric stenosis which was missed until the infant was older than 5 months. We believe this is the oldest reported case of infantile hypertrophic pyloric stenosis in Taiwan.

Airway intubation injuries occur frequently but are often neglected because of spontaneous regression. Although most airway injuries that result from intubation resolve without sequelae, severe complications can develop even when initial symptoms of upper airway obstruction are absent. This retrospective study assessed the clinical features, flexible bronchoscopic findings, and clinical outcomes in children with airway intubation injuries.From February 1998 to February 1999, 90 children underwent flexible bronchoscopic examination in our hospital. Fifteen of these patients (6 girls, 9 boys; age range, 1 mo to 5 yr; mean, 21.1 +/- 24.4 mo) were noted to have intubation injuries.The average time for symptoms to emerge after extubation was 1.8 days (range, 0-14 days). The airway intubation injuries diagnosed by flexible bronchoscopy were subglottic stenosis in seven patients, vocal cord granulation in four, supraglottic granulation in two, subglottic web in two, bronchial granulation in two, glottic stenosis in one, and tracheal stenosis in one; two cases were discovered incidentally during postoperative follow-up. Four patients had undergone repeated intubation and eventually required tracheostomy.The results of this study indicate that flexible bronchoscopy is a simple, safe, and useful technique for the diagnosis and follow-up of airway intubation injury. It should be performed on all patients who have symptoms of upper airway obstruction after extubation as well as those who have undergone long-term or emergency intubation.

Vitamin K prophylaxis for all neonates has been recommended to prevent hemorrhagic disease of the newborn (HDN), but it is still an uncommon practice in most developing countries throughout the world. In the United States and Canada, where vitamin K injections continue to be recommended in the newborn period, HDN is not a major concern. The risk factors for HDN include inadequate vitamin K prophylaxis, exclusively breast-fed infants, diarrhea, and alternative causes of vitamin K deficiency, such as liver disease and cystic fibrosis. We present an exclusively breast-fed 3-week-old infant with diarrhea for 2 days who died from intracranial hemorrhage related to HDN despite having received a single intramuscular injection of 0.2 mg of vitamin K at birth. Hemorrhage in the infant from vitamin K deficiency should be a concern for pediatricians and obstetricians. We emphasize the importance of administering an adequate dosage of vitamin K for prevention of HDN, particularly in an exclusively breast-fed infant.

Toxic megacolon is a complication that usually presents in inflammatory bowel disease such as ulcerative colitis or Crohn's disease. It also occurs in typhoid fever, acute bacillary dysentery, amebic colitis, ischemic colitis, pseudomembranous colitis and severe salmonellosis. This report describes the occurrence of toxic megacolon in two young children with Salmonella colitis manifesting as abdominal distension, diarrhea and fever. The clinical course of these two cases suggests that toxic megacolon is a severe complication of salmonellosis in children, irrespective of the use of antibiotics. In the light of its paucity in children and severity, accurate diagnosis and appropriate intensive medical-surgical management is essential.

We identify and discuss risk factors related to prolonged hospitalization, as compared with the average hospital stay (5.1 days) of normal delivery neonates throughout the last 10 years, associated with babies delivered unexpectedly in southern Taiwan.This is a retrospective case series study; those babies delivered unexpectedly, including both pre-term and term infants, were pooled out from normal-birth babies, and their laboratory data and clinical histories were reviewed and discussed.There was a total of 18 babies delivered unexpectedly during the past 10 years. Out of these 18 babies, there was only 1 mortality. The mean gestational age was 38 weeks; the mean birth body weight was 3097 g, and the average hospital stay was 6.2 days. Possible clinical factors related to prolonged hospitalization include the following: hypothermia, hypoglycemia, hyperkalemia, polycythemia, and a relatively high ratio of positive findings of chest X-ray study.The best way to reduce prolonged hospitalization caused by unexpected delivery is by prevention, or if this is not possible, by increasing the knowledge and effectiveness of handling intrapartum accidents. Hypothermia, hypoglycemia, hyperkalemia, polycythemia, and positive chest radiological results were the most common reasons for prolonged hospital stays in unexpectedly delivered neonates. However, due to the limited cases in our study, a larger group study is still needed in the future for more precise statistical information.

In view of cytokine's effects in promoting or inhibiting inflammation, the objective of this study was to explore the characteristics of the proinflammatory cytokine, interleukin-8 (IL-8), and the inhibitory cytokine, interleukin-10 (IL-10), in the bronchoalveolar lavage (BAL) fluid of premature infants suffering from respiratory distress disease. Eighteen premature neonates with respiratory distress disease with gestational age (GA) ranging from 24 to 37 weeks were recruited for study. BAL fluids were collected following endotracheal intubation during an episode of hypoxemia or dyspnea. A series of BAL samples were obtained on day 1, 2, 4 and 7 after intubation for measuring IL-8 and IL-10 levels. The results indicate that premature infants with GA ranging from 24 to 32 weeks had a higher level of IL-8 (p = 0.029), but not level of IL-10 (p = 0.109), in the BAL obtained during the first intubation compared to premature infants with GA ranging from 33 to 37 weeks. The administration of exogenous surfactant did not influence the profiles of IL-8 and IL-10, as compared to those in-patients without treatment. Levels of IL-8 were correlated with IL-10 levels (r = 0.613, p = 0.007) in BAL fluid samples obtained on the day of intubation. The level of IL-8, but not IL-10, was significantly correlated with the duration of intubation. IL-8 and IL-10 levels in BAL fluid samples collected on the day of intubation were correlated with the development of chronic lung disease (CLD). The results suggest that extreme prematurity tends to have increased IL-8 and IL-10 levels in BAL fluid compared to premature infants with older GA, and that these increased levels are associated with the development of CLD.

Vascular rings are a diagnostic and therapeutic challenge for pediatricians. Many diagnostic modalities contribute to the detection of these rare congenital vascular anomalies. The role of flexible bronchoscopy is still being debated. We present 3 cases to emphasize the usefulness of pediatric flexible bronchoscopy (PFB) in the early diagnosis and postoperative evaluation of vascular rings. In patient 1, PFB was performed before conventional techniques were available. A right aortic arch with a retroesophageal aortic diverticulum and mirror-image branching was later confirmed. In patients 2 and 3, pulmonary artery slings could not be detected clearly by imaging studies before PFB was performed. PFB was arranged again postoperatively for these 2 patients, because of difficulty weaning patient 2 from ventilator support and persistent respiratory distress in patient 3. In conclusion, we expect that more vascular rings will be diagnosed using PFB. This instrument is also useful in making a decision for surgery, and for detecting associated tracheobronchial anomalies preoperatively, assuring appropriate correction intraoperatively, and monitoring the condition of vascular rings postoperatively.

A cystic hygroma was found in a full-term (39 week-old) male newborn delivered by cesarian section. Polyhydramnios was diagnosed by sonography at 21 weeks of gestation. Clinical manifestations at birth, such as respiratory distress, tachypnea and chylothorax were successfully relieved by the insertion of chest tube and medical therapy. Other conditions, which included mild hydronephrosis of left kidney, thickening of aortic and pulmonary valves with mild mitral and tricuspid valves regurgitation, subsided within six months. The cystic hygroma regressed to a webbed neck. In general, the features and clinical manifestations of the present case resembled the criteria of Noonan syndrome including various congenital heart defects; webbed neck; chest deformity; a characteristic facial appearance comprising of a broad forehead, ocular hypertelorism, antimongoloid slant of palpebral fissures, low set ears; and bilateral undescended testis etc., suggested by Noonan and other subsequent authors. The outcome of this infant was satisfactory following medical and surgical therapy (chest tube, orchiopexy) and a special dietary regimen.

A case of bilateral cystic neuroblastoma with liver metastases in a newborn is reported. CT showed a 10 cm right suprarenal multicystic mass and numerous hepatic cystic masses with intracystic fluid-fluid levels. Multiple smaller cystic lesions were also present in the left adrenal gland. To our knowledge, the CT findings of neonatal bilateral cystic neuroblastoma with liver metastasis and massive acute intracystic haemorrhage has not been previously documented.

Spleen rupture has been a rare but feared complication in patients with infectious mononucleosis. There have been many cases reported during the last decade. However, it has rarely been mentioned among the young infant group. Here, we present a case of an infant, two months of age, who suffered from spleen rupture due to infectious mononucleosis, and was latter cured by himself under conservative treatment.

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital disorder characterized by a massively enlarged urinary bladder without mechanical outlet obstruction and microcolon, as well as a hypoperistaltic bowel with normal ganglion cell distribution. We report one such case to discuss the findings of antenatal ultrasound and the radiologic and pathologic features of this condition.

We report a very rare case of systemic sclerosis in a 6-year-old girl. She presented with diffuse scleroderma, Raynaud's phenomenon, pulmonary interstitial fibrosis, pulmonary hypertension, and right ventricular failure. The diagnosis was confirmed by skin manifestations, high resolution computed tomography, cardiac catheterization, and anti-nuclear antibodies. Nifedipine, prednisolone, digoxin, and furosemide were given. There was remission of the right ventricular failure and dyspnea, and the skin showed partial improvement. The patient remained asymptomatic for a year. The symptoms of respiratory and right heart failure developed again after an episode of lower respiratory tract infection and she eventually died. We discuss the clinical manifestations, treatment, and outcome.

Recognition of rejection and other hepatic complications by needle biopsy plays a significant role in the management of liver allograft recipients. In this report, 22 pediatric patients (below 18 years old) were selected from the 37 liver transplants. Seven of the 22 cases have an uneventful posttransplant course. The most common cause of allograft injury in these patients appeared to be acute cellular rejection. It occurred in 7 (31.8%) of the 22 cases and was the primary process in 8 of the 25 episodes of liver dysfunction. Other etiologies, such as opportunistic viral infection (3 cases), biliary obstruction (2 cases), preservation injury (1 case), and vascular obstruction (1 case) were less common. Acute graft rejection causing liver dysfunction was associated with a mixed portal inflammation, destruction of the interlobular bile ducts, and varied degree of venous endotheliitis, followed by centrilobular hepatocyte necrosis. Chronic rejection was not seen in our pediatric cases. Cold ischemic injury causing transient graft dysfunction as seen in one of our patients demonstrated focal, limited areas of hepatocyte necrosis, mild centrilobular hepatocyte ballooning, and cholestasis without evidence of bile duct damage. More severe ischemic injury resulted from vascular complication causing diffuse hepatocyte necrosis was seen in one patient with hepatic vein thrombosis. The histologic patterns observed were not pathognomic; however, liver biopsies were helpful in suggesting the probable cause of liver dysfunction and in predicting subsequent allograft recovery when used in conjunction with clinical information, radiologic, and other laboratory tests.

A 2-day-old newborn was treated with tolazoline for persistent pulmonary hypertension due to meconium aspiration syndrome. Hypoxemia improved during continuous tolazoline infusion, but gastrointestinal bleeding occurred. After treatment with cimetidine, there was a rapid deterioration with decreased oxygen saturation and arterial PO2 values. Tolazoline induces a dilatation of the pulmonary vascular system by stimulating H2 receptors. Cimetidine, an H2 blocker, may partially abolish the effect of tolazoline. However, given the clinically significant result to the interaction between tolazoline and cimetidine we report, the use of cimetidine in tolazoline induced upper gastrointestinal hemorrhage should deserve more attention.

A new and highly effective method, termed suppression subtractive hybridization (SSH), has been developed for the generation of subtracted cDNA libraries. It is based primarily on a recently described technique called suppression PCR and combines normalization and subtraction in a single procedure. The normalization step equalizes the abundance of cDNAs within the target population and the subtraction step excludes the common sequences between the target and driver populations. In a model system, the SSH technique enriched for rare sequences over 1,000-fold in one round of subtractive hybridization. We demonstrate its usefulness by generating a testis-specific cDNA library and by using the subtracted cDNA mixture as a hybridization probe to identify homologous sequences in a human Y chromosome cosmid library. The human DNA inserts in the isolated cosmids were further confirmed to be expressed in a testis-specific manner. These results suggest that the SSH technique is applicable to many molecular genetic and positional cloning studies for the identification of disease, developmental, tissue-specific, or other differentially expressed genes.