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Introduction: Neonatal jaundice and prematurity pose significant barriers to breastfeeding in the first days of life. There is limited literature exploring the relationship between prolonged jaundice in breastfed infants and Gilbert's (Meulengraght) syndrome. This case study describes the diagnostic and therapeutic challenges associated with Gilbert's syndrome in a late preterm breastfed infant born in Germany., Main Issue: In this case report, an infant born to a primipara woman presented at 3 weeks postpartum to an International Board Certified Lactation Consultant. The initial assessment revealed a late preterm infant with inadequate weight gain and jaundice. The dyad received breastfeeding support and eventually achieved adequate weight gain; however, the infant's jaundice persisted., Management: The consulting midwife suggested that the persistent jaundice was "breastmilk jaundice" and recommended temporarily interrupting breastfeeding. Due to a suspected family history of Gilbert's Syndrome, the dyad was referred, instead, to a pediatric gastroenterologist. Pathologic liver disease was excluded, and genetic testing confirmed Gilbert's Syndrome. At 6 months of age, the dyad was successfully breastfeeding and beginning complementary feeding., Conclusion: Genetic testing for Gilbert's Syndrome should be considered for infants with prolonged jaundice and positive family history. Interruption or cessation of breastfeeding are not evidence-based recommendations, and current guidelines do not support these practices. Lactation professionals play a critical role in the management of breastfeeding for preterm infants with prolonged jaundice and should refer to specialists to rule out pathologic etiologies., Competing Interests: Disclosures and Conflicts of InterestThe authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: MT has previously received consultancy fees from the United Nations Children’s Fund (UNICEF), the World Health Organization (WHO), the Ludwig-Maximilians-University (LMU) Munich, the German Society for Paediatric and Adolescent Medicine (Deutsche Gesellschaft für Kinder- und Jugendmedizin e.V. (DGKJ). She has received travel fees from the Austrian Society of Paediatric and Adolescent Medicine (Österreichische Gesellschaft für Kinder und Jugendheilkunde).

Background/aim: Prolonged jaundice is one of the most common problems during neonatal period. The aim of this study was to evaluate the efficiency of ursodeoxycholic acid (UDCA) treatment in newborn infants with prolonged unconjugated hyperbilirubinemia., Materials and Methods: The present study included 27 patients who were fed by breast milk and followed up in the outpatient clinic due to prolonged jaundice without any underlying etiological factor; 10 mg/kg/day UDCA was administrated in two doses for 7 days. Furthermore, 20 newborns diagnosed with prolonged jaundice with same characteristics were enrolled as the control group. The control group was also given a placebo; demographic characteristics, liver functions tests before and after the treatment, bilirubin decrease rates, and hemogram parameters of groups were compared., Results: Total bilirubin levels in the study and control groups before the treatment were 16.02 ± 1.41 mg/dL and 15.93 ± 1.66 mg/dL, respectively (P = 0.84). Total bilirubin levels in the study and control groups at day 7 after UDCA treatment were detected 8.18 ± 2.31 mg/dL and 13.92 ± 2.66 mg/dL, respectively (P < 0.001), and at day 14 after the treatment were 5.45 ± 2.59 mg/dL and 11.91 ± 2.83 mg/dL, respectively (P < 0.001). Furthermore, serum aspartate aminotransferase (AST) was detected <21 U/L in the ROC analysis after UDCA treatment (P = 0.04)., Conclusion: The study outcomes indicated that an efficient reduction in total bilirubin levels may be achieved, and outpatient clinic follow-up period may be reduced in patients whom UDCA was administrated. Moreover, it may be speculated that AST can be used to evaluate the efficacy after treatment. However, studies with larger sample sizes are needed for the routine use of UDCA in the treatment of prolonged jaundice., Competing Interests: None

Neonatal liver disease encompasses many diagnoses, including structural and genetic aetiologies. Many have significant health implications requiring long-term specialist treatment including liver transplantation. Jaundice is a common presenting feature. The ability of health-care professionals to differentiate neonatal liver disease from benign diagnoses such as physiological jaundice is very important. Persistent (more than 2 weeks) of conjugated jaundice always warrants investigation. Severe unconjugated jaundice (requiring prolonged phototherapy) should also be promptly investigated. Recent advances in genomics have enabled previously elusive, precise diagnoses in some patients with neonatal liver disease. This review paper discusses the commoner causes, with a focus on early detection and need for referral to paediatric liver services., (© 2020 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Introduction: An extremely variable and high prevalence of urinary tract infection (UTI) in infants with prolonged jaundice has been reported in Iran. However, there is no research from the area that has attempted to estimate pooled prevalence of UTI from considerably diverse evidence. Therefore, this systematic review and meta-analysis study aimed to estimate the prevalence of UTI in infants with prolonged jaundice who were admitted into clinics or hospitals in Iran., Methods: A systematic review and meta-analysis was conducted of published articles on UTI prevalence in infants with prolonged jaundice in Iran. Electronic databases were searched, including Web of Sciences, PubMed/Medline, Scopus, Iranian Scientific Information Database (SID) and Iranmedex, for both English and Persian language articles published between January, 2000 and March, 2017. All possible combinations of the following keywords were used: jaundice, icterus, hyperbilirubinemia during infancy, infection and neonatal. Nine studies that reported prevalence of UTI in infants with prolonged jaundice were included. The overall prevalence of UTI was estimated using random-effects meta-analysis models., Results: A total of 1750 infants were pooled to estimate the overall prevalence of UTI in infants with prolonged jaundice. The prevalence reported by the studies included in this literature review was extremely variable and ranged 0.6-53.9%. The overall prevalence was 11% (95% Confidence Interval (CI): 5.0-18.0), and Escherichia coli was found to be the main cause of UTI., Conclusion: The overall prevalence of UTI was 11%, and E. coli was the main cause of UTI in infants with prolonged jaundice. Screening of UTI should be considered for infants with prolonged jaundice., (Copyright © 2018 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Introduction: Prolonged jaundice is very common and can affect about 2-40% of all neonates. The condition warrants close monitoring to avoid missing serious pathology but not to over investigate a normal neonate. Transcutaneous bilirubin (TcB) measurement has been recommended by the AAP. However, there is no published data on the usage of Drager Jaundice Meter JM-105 as a screening modality for prolonged jaundice in term and late preterm neonates in Malaysia. We aimed to evaluate the accuracy of transcutaneous bilirubinometer in monitoring neonates with prolonged jaundice. Materials and methods: A prospective cross-sectional study was conducted in a university hospital in east coast Malaysia, involving 50 neonates with prolonged jaundice. Drager JM-105 device was used to measure TcB while the TSB sample was obtained simultaneously. Fifty pair measurements of TcB and TSB were compared. Results: The mean TSB was 201.96µmol/L (SD 80.98) where the mean TcB was 184.28 µmol/L (SD 73.37). This study demonstrated the mean difference of 17.68 µmol/L in total bilirubin measured by total serum bilirubin (TSB) and transcutaneous bilirubin (TcB) measured by Drager JM-105. This study also showed excellent correlation between TSB values and TcB with correlation coefficient r = 0.963 and r2 = 0.927 (p<0.05). Conclusion: This study showed excellent correlation between TSB values and TcB values. [ABSTRACT FROM AUTHOR]

Introduction: Jaundice is one of the most common problems at neonatal period and seen in 60-70% of all newborns at first days of life. Prolonged jaundice is defined as hyperbilirubinemia persisting at the end of week 2 in term newborns. In this study, we reviewed term newborns diagnosed with prolonged jaundice. It was aimed to demonstrate delaying laboratory evaluations for a week in infants with favorable clinical presentation can prevent unnecessary tests in majority of newborns. Materials and Methods: The study included full-term newborns who presented after day 14 of life and diagnosed with prolonged jaundice at neonatology outpatient clinic of Health Sciences University, Keçiören Teaching and Research Hospital in 2016. Overall, 336 infants with prolonged jaundice were screened. The infants with congenital anomaly, those with findings of sepsis or severe infection, those with history of intrauterine infection, those with history of acholic stool and those with no available data were excluded. In 180 patients included, clinical evaluations, bilirubin levels and advanced test results were assessed at baseline and weekly follow-up. Findings: Of the infants included, 51.7% were boys while 48.3% were girls. The most common blood type was A Rh (+). There was ABO incompatibility alone in 24 infants ('4.2%), Rh incompatibility alone in 6 infants (3.5%) and ABO plus Rh incompatibility in 3 infants ('.2%). In '30 infants, total bilirubin was studied on both week 2 and 3. At week 3, total bilirubin value was >10 mg/dl in only 36 infants (27.7%) while it was decreased below '0 mg/dL in 94 infants (72.3%). Urinary tract infection (UTI) was detected in 7 of 38 infants with available tests at week 2. Two of 6 infants with UTI had other clinical signs of UTI. Mean total bilirubin value was '7.9 mg/dL in 5 infants. A significant correlation was found between UTI and vomiting, breastfeeding and feeding pattern (p<0.05). Congenital hypothyroidism was detected in 6 of 38 infants with available tests at week 2. It was seen that 2 infants had suspected congenital hypothyroidism in neonatal heel prick test and underwent further evaluations while mean total bilirubin value was '7.5 mg/dL at week 2 in remaining 4 infants. Given the vast majority of cases are breast milk jaundice, a novel cut-off value was defined for total bilirubin measurement at week 2 to distinguish the cases in which total bilirubin decreases below '0 mg/dL at week 3. Conclusion: Although several disorders that may lead prolonged jaundice at neonatal period have been identified, it is well-known that prolonged jaundice can be seen without any pathological condition in majority of infants fed by breast milk. Delaying laboratory evaluations recommended at week for prolonged jaundice is an approach that may prevent unnecessary testing in most infants. [ABSTRACT FROM AUTHOR]

Biliary atresia is one of the commonest causes of prolonged jaundice in neonate, whereas cystic biliary atresia is a rare form of biliary atresia with similar presentation, but a slightly different sonographic finding. Being able to differentiate them radiologically is important as the surgical management is different for these two diagnoses. An ultrasound examination was done for a 2-month-old baby girl with prolonged jaundice with the finding of a cyst along the portal triad, which raised the suspicion for cystic biliary atresia. The diagnosis was confirmed with intraoperative cholangiography (IOC) and Kasai procedure was done. The patient was well after the surgery with resolution of jaundice. [ABSTRACT FROM AUTHOR]

Congenital Adrenal Hyperplasia (CAH) is a genetic disorder that leads to cortisol deficiency. However, prolonged neonatal jaundice is a rare presentation of CAH. The pathophysiology of hyperbilirubinemia in CAH is still ill-defined. Plausible causes are related to the synthesis of bile, maturity of the liver and adrenal function. This case reported a neonate who presented with severe prolonged jaundice that lasted for more than a month. A short Synacthen test confirmed diagnosis of CAH. He was started on steroid replacement. He had regular follow-up under paediatric endocrinologist and primary care physician for long-term monitoring and overall health care. This case demonstrates the importance of recognizing the clinical and biochemical features of CAH for early detection and referral. Long-term follow-up and monitoring is necessary due to the risk of complications and side effects of medications. This is the first case of CAH presented with persistent hyperbilirubinemia to be reported from Malaysia. The case describes the difficult workup that has been encountered in the patient's care and management. [ABSTRACT FROM AUTHOR]

Objective: This study aims to evaluate the demographic information, clinical and laboratory findings of patients with glucose 6 phosphate dehydrogenase deficiency. Material and Methods: We collected data by reviewing files and electronic records of 65 patients with glucose 6 phosphate dehydrogenase deficiency under the age of 18 years who were followed up in our clinic between 2007 and 2019. Demographic, clinical, and laboratory features, family history, complications of the disease, and history of splenectomy and cholecystectomy were evaluated. Mean, standard deviation, and median values were used when descriptive analyses were presented. Results: The age of diagnosis ranged between 1-192 months and the median age of diagnosis was two months. Fifty-nine patients (90.7%) were boys and six (9.2%) were girls. The mean value of glucose 6 phosphate dehydrogenase enzyme on admission was 1,9±1,4 U/g of hemoglobin (Hb). Family history was present in 40% of patients in whom information was available. The most common presentation was prolonged jaundice and the most common physical finding was jaundice. Splenomegaly was detected in none of the patients. Cholelithiasis was present in one of 21 patients who were evaluated with ultrasonography. None of the patients required splenectomy, cholecystectomy, and regular erythrocyte transfusion during follow-up. Conclusion: As G6PD variants with chronic hemolysis are not usually seen in Turkey, patients who required splenectomy, cholecystectomy, and regular erythrocyte transfusion were not detected. Although glucose 6 phosphate dehydrogenase deficiency is more common in males, it can also be seen in girls. In Turkey, glucose 6 phosphate dehydrogenase deficiency should be considered in patients presenting with prolonged jaundice. [ABSTRACT FROM AUTHOR]

Urinary tract infection (UTI) is a common disease in childhood. The incidence of bacterial infections in the neonatal period is 5-6%.Prolonged jaundice is one of the common problems of the neonatal period, and urinary tract infection is also included in the etiology. The aim of this study is to determine the incidence of urinary tract infection, which is one of the important causes in newborns with prolonged jaundice, and to determine accompanying etiological factors. In addition, to review clinical, laboratory and radiological findings and to compare cases with positive radiological findings, to prevent complications secondary to urinary tract infection with early diagnosis. [ABSTRACT FROM AUTHOR]

Background: There were only few publications related to pancreaticoduodenal resection (PDR)/Whipple procedure in Indonesia in the past decade. Method: Retrospectively report of the characteristics and outcomes of PDR performed by Cipto Mangunkusumo Hospital surgical team from 1993 to 2017 were collected. Results: PDR were performed in 213 patients, with a mean age of 50.6 years and 54.4% patients were females. Predominant preoperative clinical findings were jaundice (68.9%) and mild hypoalbuminemia (69.9%). Biliary decompression was performed in 112 (52.6%) subjects. Average surgical waiting time was 3.5 months. While PDR were performed in 84 (39.5%) subjects, pyloric preserving pancreaticoduodenal technique was predominated in 128 (59.8) and predominated, especially during the latter years. Fifteen (9.0%) cases were benign. Thirtyone (14.6%) subjects underwent relaparotomy, 16 (51.6%) of whom died post-operatively. Overall operative mortality decreased from 16.9% to 5.5% in 2016, while resection rate generally increased over time, ranging from 2 - 21/year. Less than 10% of subjects survived for > 5 years, while < 20% survived for < 24 months. Overall morbidity was 65.1% in 177 survivors, with surgical site infection in 52.5%, pancreatic fistula in 24.2%, and post-pancreatectomy haemorrhage (PPH) as a fatal postoperative complication in 19 (8.9%) cases. Patients who died within 30 days postoperatively had significantly more relaparotomies and PPH (p < 0.001). Conclusion: Prolonged jaundice and mild hypoalbuminemia are dominant characteristics in our Indonesian PDR subjects. Cipto Mangunkusumo Hospital is a high-volume PDR centre and world class hospital. Mortality rates decreased with the increasing resection rates. Relaparotomy and PPH are predictors of poor outcome. [ABSTRACT FROM AUTHOR]

Background: Hemizygous mutations in the immunoglobulin superfamily member 1 ( IGSF1) gene have been demonstrated to cause congenital central hypothyroidism in males. This study reports a family with a novel mutation in the IGSF1 gene located on the long arm of the X chromosome. Patient findings: A two-month-old boy was diagnosed with central hypothyroidism because of prolonged jaundice. A thyrotropin-releasing hormone (TRH) stimulation test indicated dysfunction in both the hypothalamus and the pituitary gland, and prompted the IGSF1 gene to be analyzed. The patient had a novel nonsense variant, c.2713C>T (p.Q905X), in exon 14 of the IGSF1 gene. Studies of the family revealed that the patient's sister and mother were heterozygous carriers of the IGSF1 mutation. The patient's maternal uncle carried the same mutation as the proband but had no overt symptoms. The mother and uncle started levothyroxine supplementation because of subclinical hypothyroidism. Summary: A novel mutation (c.2713C>T, p.Q905X) of the IGSF1 gene was identified that causes congenital central hypothyroidism in a Japanese family. The findings further expand the clinical heterogeneity of this entity. [ABSTRACT FROM AUTHOR]

Background: Jaundice is the most common clinical problem among the newborns. Prolonged jaundice in the neonate is defined as a jaundice that lasts longer than 14 days and has multiple possible causes. This study aimed to evaluate the causes of prolonged jaundice in newborns referred to Shahid-Beheshti hospital, Kashan, Iran. Materials and Methods: This cross-sectional study was conducted on 237 neonates with prolonged jaundice referred to Shahid-Beheshti hospital in Kashan. Clinical prolonged jaundice was defined as the yellow discoloration of the skin and mucous membranes, and persisting beyond 14 days of age. Blood tests were performed to determine the causes of jaundice. Results: None of the infants had a direct hyperbilirubinemia. Breast milk jaundice (59.1%) was the most common cause of newborn jaundice and other possible causes were urinary tract infection (12.2%), Glucose-6-phosphate dehydrogenase deficiency (9.7%) and hypothyroidism (6.3%). The exact etiology was unknown in 12.7% of the cases. Moreover, while there was a relation between the incidence of neonatal jaundice and sex, no relation was seen between the incidence and age, weight and the initial bilirubin level. Conclusion: Although the most common cause of prolonged jaundice is breast milk jaundice with no serious complication, a detailed investigation is recommended to determine the other important causes of the disease. [ABSTRACT FROM AUTHOR]