Is hepatovenocaval syndrome a different entity from budd-chiari syndrome in children?

Objective: To differentiate between clinical and demographic spectrum, and outcome in hepatovenocaval syndrome [HVCS] and Budd-Chiari syndrome [BCS]. Study Design: Descriptive study. Place and Duration of Study: Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Children Hospital, Lahore, from January 2014 to January 2017

Methodology: All children less than 18 years of age, presenting with ascites and visible veins over abdomen, flanks and back were enrolled in the study. Real time Doppler Ultrasonogram was performed in all children for documentation of intrahepatic part of IVC obstruction along with or without hepatic venous obstruction. Children meeting inclusion criteria underwent liver profile, coagulation profile, diagnostic paracentesis for SAAG gradient, and Gadolinium enhanced multiphasic MR scan. Liver biopsy and venography was performed in selected patients

Results: A total of 92 children presented with ascites, among them 58 children met our inclusion criteria. Intrahepatic IVC obliteration, i.e. HVCS, found in 67% [n=39] and hepatic venous outflow obstruction, i.e. BCS was found in 33% [n=19] children. Children with BCS were older than HVCS with mean age of 9.5 +/-2.58 versus 4.12 +/-0.977 years. HVCS group had 14 boys and 24 girls with a ratio of 1:1.8, while BCS had a ratio of 1:0.9 with 10 boys and 9 girls. No etiological factor was found for HVCS, while most of patients with BCS had a procoagulant disorder. Caudate lobe hypertrophy was a consistent feature in BCS, while IVC obstruction was found in HVCS persistently. Orthotopic liver transplant was needed in three cases [7.6%] of HVCS and four [20.96%] of BCS cases. Antibiotic therapy has a good role in HVCS, while anticoagulation and diuretics had good result in BCS

Conclusion: Hepatovenocaval syndrome [HVCS] mostly affected younger children, especially girls. BCS usually affected older age groups with pro-coagulant disorders who responded to anticoagulation and diuretic. Further studies are needed to compare both conditions

Berardinelli-seip congenital generalised lipodystrophy

Berardinelli-Seip congenital lipodystrophy [BSCL] syndrome is a rare genetic disorder caused by dysregulation of glycemic and lipid metabolism. We report five BSCL cases with typical clinical pictures and complications. These, to the best of our knowledge, represent the first case series from Pakistan. BSCL is characterized by marked atrophy of adipose tissue, acromegaly, acanthosis nigricans and tall stature. We could not perform genetics studies in any patient owing to non-availability of genetic laboratory in Pakistan. All the cases presented hypertriglyceridemia. One case developed hyperinsulinism controlled with metformin. There is no curative treatment and the current approach is low-fat diet and management of insulin resistance and diabetes. Recently published studies showed that leptin-replacement therapy is promising in the metabolic correction of complications of BSCL. This highlights the importance of further research in BSCL treatment

Etiology and pattern of presentation of gastritis in children, our experience at a tertiary care hospital

Objective: This study aimed to look in to the frequency of different causes and pattern of presentation of gastritis in children less than 16 years of age. Study Design: Prospective cross sectional study. Place and Duration of Study: This study was performed in the Children Hospital and the Institute of Child health, Lahore, from Jan 2016 to Jan 2017

Material and Methods: This study was performed in the department of Pediatric Gastroenterology, Hepatology and Nutrition at Children Hospital and the Institute of Child health, Lahore, from Jan 2016 to Jan 2017. One hundred and sixty children fulfilling the inclusion criteria were enrolled in this study. All patients went through endoscopy. Macroscopic findings were noted and gastritis was confirmed by histological analysis of gastric mucosa. Biopsies were taken from gastric antrum, the body and pylorus

Results: The mean age of children was 8.73 +/- 3.70 years [range 2-16years], and majority were in the age range of 6-10 years. Males were affected slightly more than females with a ratio of 1.3:1. Drug induced gastritis was the major contributor in 58 [36.2%] patients followed by food related gastritis in 55 [34.3%]. H.Pylori positive gastritis was seen in 38 [23.7%] patients. No cause of gastritis was found in 5.6% of patients

Conclusion: Our study concluded that drug induced gastritis and food related gastritis was more common than H. Pylori positive gastritis in children in our setting

Chanarin-Dorfman syndrome

Chanarin-Dorfman syndrome is a rare, genetically determined autosomal recessive disorder, characterised by the presence of lipid droplets in the cytoplasm of multiple tissues of the body, particularly in the blood leukocytes and congenital non-bullous icthyosiform erythroderma. In this paper, we report one-year child who presented with skin lesions since birth and hepatomegaly. Liver biopsy showed steatohepatitis; and peripheral blood smear confirmed Jordan`s anomaly, which is a permanent feature of Chanarin-Dorfman syndrome

Age, pattern of presentation and risk factor for enteric fever in 2-15 years old children

Objective: To find out age related common pattern of presentation of enteric fever in children

Study design: A cross sectional study

Place and duration: Pediatric Department Al-Nafees Medical College and Hospital Islamabad over a period of 6 months from 1[st] July 2013- 31[st] Dec 2013

Methodology: Patients irrespective of gender between 2-15 years, with fever of more than 4 days without focus were included in the study. Complete blood count and typhi dot test performed on all patients for diagnosis. Performa containing bio data, symptoms and clinical signs was filled of patients with positive serology

Results: A total of 60 patients studied and among them 92 %[ n= 55] and 90% [n=54] patient presented with poor appetite and fever respectively. Among them 90% [n=54] had white coated tongue and 66% [n=40] had low grade fever [100[degree]F -102[degree]F] while 33% [n=20] were having high grade fever [?102[degree]F]. Hepatomegaly was found in 73% [n=44] patients while Hepatosplenomegaly in 20% [n=12] patients. Leukocytosis was more common in children than leucopenia

Conclusion: Common symptom of enteric fever is poor appetite and low grade fever, and white coated tongue with hepatomegaly is common findings on clinical examination. Thrombocytopenia is consistent laboratory finding