Predictors of Short-Term Outcome of Kasai Portoenterostomy for Biliary Atresia in Infants: a Single-Center Study / 대한소아소화기영양학회지

Purpose@#The outcome predictors of Kasai portoenterostomy (KPE) for biliary atresia (BA) are controversial. This study aimed to identify possible short-term outcome predictors of KPE for BA in infants. @*Methods@#This retrospective study included infants with BA who underwent KPE between January 2015 and December 2017 and were followed up for at least 6 months after surgery at the Pediatric Hepatology Unit, Cairo University Pediatric Hospital, Egypt. The short-term outcome was jaundice clearance within 6 months following surgery. All data were compared between the jaundice free group and those with persistent jaundice to identify the predictors of jaundice clearance. @*Results@#The study included 75 infants. The mean age at the time of surgery was 82.43±22.77 days (range, 37–150 days), and 28 (37.3%) infants cleared their jaundice within 6 months postoperative. Age at surgery did not significantly affect the outcome (p=0.518). Infants with persistent jaundice had significantly higher pre-operative levels of aspartate aminotransferase (AST) than those who were jaundice free (p=0.041). Receiver operating characteristic curve analysis showed that preoperative AST ≤180 IU/L was predictive of a successful KPE, with sensitivity 74.5% and specificity 60.7%. Infants with bile plugs in liver biopsy had a 6-fold higher risk of persistent jaundice than those without bile plugs (95% confidence interval: 1.59–20.75, p=0.008). @*Conclusion@#Jaundice clearance after KPE for BA can be predicted using preoperative AST and presence of bile plugs in liver biopsy.

Ocular findings in patients with cholestatic disorders of infancy: a single-centre experience

Background and study aims: Neonatal cholestasis can be associated with ocular findings that might aid in its diagnosis, e.g., Alagille syndrome [AGS] and Niemann Pick disease [NPD]. We aimed to investigate the frequency of ocular manifestations in infants with cholestasis

Patients and methods: This cross-sectional study included cholestatic infants presenting to the Paediatric Hepatology Unit, Cairo University Paediatric Hospital, Cairo, Egypt. All infants underwent examination of lid, ocular motility, anterior and posterior segments and measurement of intraocular pressure, cycloplegic refraction, ocular ultrasonography and vision

Results: The study included 112 infants with various cholestasis; 73 [65.2%] were males. The median age was 2 months. Diagnosis was reached in 39 cases: 14 had AGS, 14 had biliary atresia [BA], 4 had NPD, 4 had post-haemolytic cholestasis, 2 had cytomegalovirus neonatal hepatitis, and one case had hepatorenal tyrosinaemia. Thirteen cases were probably having progressive familiar intrahepatic cholestasis [PFIC] type 1 or 2 considering their persistent cholestasis in the presence of normal gamma-glutamyl transpeptidase; 28 were left with a diagnosis of "idiopathic neonatal hepatitis" [INH], and 32 [28.6%] had no definite diagnosis. Ophthalmologic abnormalities were found in 39 cases [34.8%]. The commonest finding was unilateral/bilateral optic nerve drusen in 12 [10.7%], followed by posterior embryotoxon in 11 [9.8%]. Ocular findings were observed in 64.3% patients with AGS, 50% patients with NPD, 30.8% cases with suspected PFIC type 1or 2, 28.6% infants with INH, and 14.3% patients with BA

Conclusion: Ophthalmologic findings are not uncommon among cholestatic infants. Ophthalmologic examination should be routinely performed, including assessment of anterior segment, fundus examination, and ocular ultrasound

Blind percutaneous liver biopsy in infants and children: comparison of safety and efficacy of percussion technique and ultrasound assisted technique

Background and study aims: Liver biopsy remains the most reliable method to diagnose various hepatic disorders in children. We aimed to assess the technical success and complication rate of ultrasound [US] assisted percutaneous liver biopsy versus transthoracic percussion guided technique in paediatrics

Patients and methods: This randomized controlled study included all cases performing liver biopsy at Paediatric Hepatology Unit, Cairo University Paediatric Hospital over 12 months

Results: Patients were 102 cases; 62 were males, with age range 18 days to 12 years. Fifty seven procedures were done using the percussion guided technique and 45 cases were US assisted. The total number of complicated biopsies was 14 [13.7%], with more serious complications occurring in the percussion group. Complications were more frequent with younger age, lower platelet count, number of passes and occurrence of hypotension

Conclusion: US assisted percutaneous liver biopsy, although more costly, but may be safer to perform particularly in younger age

Haemaphagocytic lymphohistiocytosis presenting as neonatal liver failure: a case series

Background and study aim: Haemophagocytic lymphohistiocytosis [HLH] is a life-threatening clinical syndrome with liver involvement varying from mild dysfunction to severe fulminant failure. The aim of this study was to present a case series of four HLH patients presenting with acuw liver failure [ALF] in the neonatal period

Patients and methods: All four patients were neonates at the onset of symptoms. They presented to Cairo University Pediatric Hospital with ALF; they underwent prompt investigations including determination of ferritin, fibrinogen, and higlyceride levels as part of our ALF workup. Further investigations were tailored according to the associated clinical features and the results of preliminary investigations

Results: HLH was diagnosed according to HLH-2004 criteria. Three patients fulfilled at least five out of eight criteria. Fever, splenomegaly, elevated ferktin levels, and low fibrinogen levels were present in all patients. The fourth patient had a serum ferritin level >10,000 nglml, favouring the diagnosis of HLH, despite fullilling only four out of eight criteria. For three patients, positive consanguinity and previous sibling death were reported, suggesting a genetic aetiology of HLH

Conclusion: ALF can be the presenting feature of HLH; thus, a high index of suspicion is necessary. Fever is a hallmark, especially in neonates. Diagnosis is important for this potentially treatable condition

Chanarin-Dorfman syndrome: a case report and review of the literature

Chanarin-Dorfman syndrome, a "neutral lipid storage disease with ichthyosis," is a multisystem inherited metabolic disorder associated with congenital ichthyosis and accumulation of lipid droplets in various types of cells. Case report: A 3-year-old male presented to the Pediatric Hepatology Unit, Cairo University Children's Hospital, Cairo, Egypt, with accidentally discovered hepatomegaly. He had generalised ichthyosis with dark skin pigmentation and bilateral ectropion. Abdominal examination revealed generalised abdominal distention with firm nontender hepatomegaly. His liver functions were deranged. Blood film showed many vacuolated neutrophils. Serum triglyceride and creatine kinase levels were elevated. Abdominal ultrasound showed a moderately enlarged liver with a bright echo pattern. Liver biopsy revealed marked diffuse macrovesicular fatty changes. The diagnosis of Chanarin-Dorfman Syndrome was made based on the dermatological, haematological, and liver biopsy findings

Glycogen storage disease type III in Egyptian children: a single centre clinico-laboratory study

Glycogen storage disease type III [GSD III] is an autosomal recessive disorder caused by deficiency of glycogen debrancher enzyme and is characterised by clinical variability. We herein describe the clinical and laboratory findings in 31 Egyptian patients with GSD III presenting to the Paediatric Hepatology Unit, Cairo University, Egypt. Eighteen patients [58%] were males. Their ages ranged between 6 months to 12 years. The main presenting complaint was progressive abdominal distention in 55%. Twelve patients [38.7%] had a history of recurrent attacks of convulsions; four had an erroneous diagnosis of hypocalcaemia and epilepsy. Dolllike facies was noted in 90%. Abdominal examination of all cases revealed abdominal distention and soft hepatomegaly which had bright echogenicity by ultrasound. Hypertriglyceridaemia was present in 93.6%, hyperlactacidaemia in 51.6% and hyperuricaemia in 19.4%. Liver biopsy showed markedly distended hepatocytes with well distinct cytoplasmic boundaries and 32% had macrovesicular fatty changes. Serum creatine kinase was elevated in 64.6% of patients and correlated positively and significantly with age [r = 0.7 and P = <0.001], while serum triglycerides correlated negatively with age [r = -0.4 and P = 0.05]. Blood glucose assessment and search for hepatomegaly in an infant with recurrent seizures may prevent delay in the diagnosis. A huge soft liver reaching the left midclavicular line that appears echogenic on ultrasonography is characteristic of GSD III. A distended hepatocyte with rarified cytoplasm is pathognomonic but not diagnostic. Hypertriglyceridaemia correlates negatively with age, in contrary to CK level

Acute pancreatitis complicating acute hepatitis a virus infection

Acute pancreatitis complicating acute hepatitis A is very rare especially in children. We report here an 11 year old female patient with picture of acute hepatitis proved to be caused by hepatitis A. One week later patient's condition worsened, she was jaundiced, with persistent vomiting and looked acutely ill and uncomfortable with severe steady abdominal pain mainly in the epigastrium and upper quadrants. Acute pancreatitis was suspected and proved by a clinical picture associated with elevated serum amylase and serum lipase and by MRCP. The patient was managed conservatively with gradual clinical and laboratory improvement, and she was discharged after one week in a good clinical condition

clinical study of Wilson's disease: the experience of a single Egyptian paediatric hepatology unit

Most paediatric patients with Wilson's disease [WD] present with hepatic manifestations, but some may have neurologic or psychiatric features. Our aim was to define the clinical, biochemical features and the outcome of therapy of a group of Egyptian children diagnosed with WD. The study was carried out at the Paediatric Hepatology Unit at Cairo University Children's Hospital, Egypt; 54 patients were diagnosed with WD from 1996 to 2009. The diagnosis was based on low serum ceruloplasmin levels, increased urinary copper concentrations before or after D-penicillamine challenge and/or the presence of Kayser-Fleischer [K-F] rings. The clinical presentation was as follows: hepatic presentation in 33 patients [61%], hepato-neurologic 3 [5.5%], neurologic 5 [9.3%] and presymptomatic 13 [24%]. Twelve couples had more than one affected sib. Increased urinary copper concentrations before or after D-penicillamine challenge was found in all patients, low serum ceruloplasmin in 97% and K-F rings in 31.5%. All patients were treated with penicillamine and zinc sulphate except one presymptomatic case who was treated with zinc sulphate only. Three patients underwent liver transplantation and eight patients died after a median duration of treatment of 6 months [1-36]. The hepatic symptoms improved with treatment but the neurological symptoms remained stationary. Clinical and biochemical assays remain the standard for diagnosis of WD. Penicillamine and zinc therapy can effectively treat WD with hepatic symptoms. Liver transplantation remains life saving for those with fulminant and end stage WD. Screening for presymptomatic sibs is of utmost importance

Factors associated with fibrosis progression in HCV infected children in Egypt

This study aimed at identification of factors that could be associated with development of hepatic fibrosis in children with HCV infection. The study was carried out at the Pediatric Hepatology Unit, Cairo University Children's Hospital, Egypt. Liver biopsies were obtained from 43 children with HCV infection after having informed consent from their parents in the period "1995-2002". Their mean age at liver biopsy was and 8.67 +/- 4.3 years. Boys: girls ratio was 1.3:1. The results proved that, by examining the 43 patients' biopsies, 12 were having no fibrosis, 20 were having mild fibrosis and 11 were having moderate to severe fibrosis. The median time for development of fibrosis was estimated to be 5.5 years. Developing fibrosis was significantly associated with shorter duration from first detected ALT elevation to biopsy [P =0.015] and having higher levels of direct serum bilirubin [P Value=0.048]. Unexpectedly, development of fibrosis was slower in the group with co- morbid conditions compared to the group with no co-morbid conditions [P =0.04]. The development of hepatic fibrosis in children with HCV infection was associated with shorter duration of first detected ALT elevation to biopsy and higher direct serum bilirubin levels and it was progressing more slowly in the group having co-morbid conditions

Safety and efficacy of rifampicin in children with cholestatic pruritus

Objective: The present study aimed at verifying the safety and efficacy of rifampicin in ameliorating pruritus in pediatric patients suffering from persistent cholestasis

Methods: Twenty-three patients attending the Pediatric Hepatology Unit at Cairo University Children's Hospital, Egypt, were included in the present study. They were suffering from intractable pruritus secondary to persistent cholestasis from various etiologies. They were 14 males [60.87%] and 9 females [39.13%]. The mean duration of itch was 19 +/- 27.5 months. Rifampicin was started at a dose of 10 mg/kg/day in two divided doses. Liver function tests were followed up weekly to detect any deterioration that may be attributed to the drug

Results: Seventeen patients [74%] showed improvement of pruritus with rifampicin. Fourteen out of the seventeen [61%] improved at a dose of 10 mg/kg/day in 2 divided doses. The remaining 3 patients [13%] needed gradual dose increase by increments of 2 mg/kg/day every 2 weeks [maximum dose 20 mg/kg/day] until clinical improvement was observed. None of the patients showed any deterioration in liver functions, even though, a significant improvement in total serum bilirubin, ALT and AST was noticed following therapy

Conclusions: Rifampicin in a dose of 10-20 mg/kg/day is safe and effective in ameliorating uncontrollable pruritus in pediatric patients suffering from persistent cholestasis. No hepatoxicity was noted on close follow up in the studied children