ABSTRACT
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
ABSTRACT
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