B. R. Thapa

Natural history and risk factors in fulminant hepatic failure

Background: The natural history of fulminant hepatic failure (FHF) without liver transplantation is not well known. Aims: To study the natural history and prognostic factors, especially the presence of ascites and spontaneous bacterial peritonitis (SBP), in children with FHF. Methods: FHF was defined by the onset of encephalopathy within 12 weeks of onset of jaundice. From August 1997 to December 2000, 67 children (≤12 years) were diagnosed with FHF. Their clinical features, investigations and outcome were noted. Viral markers A to E (IgM, anti-HAV; IgM, anti-HEV, HBsAg, and anti-HCV) were determined by ELISA. SBP was defined by the presence of ≥250 neutrophils with or without a positive culture in ascitic fluid. Results: Mean age of the children was 5.8 years with an almost equal sex distribution. Viral markers were positive in 63 (94%) cases: hepatitis A in 34 (54%), E in 17 (27%), A+E in seven (11%), and B in five (8%). Thirty one children presented with grade I or II encephalopathy and all recovered, whereas 17 of 36 children who had grade III or IV encephalopathy died. Ascites was detected (both clinically and ultrasonically) in 34 (51%) cases, nine (26%) of which had SBP. Overall mortality was 25%. Mortality was higher in those who had ascites than in those who did not (32% v 18%); among those with ascites it was maximum in those who had SBP (78% v 16%). Total serum bilirubin and grade of encephalopathy were significantly higher, serum albumin was significantly lower, and prothrombin time was significantly prolonged in those who died than in those who recovered. Conclusion: The natural history of FHF in Indian children depends on age, grade of encephalopathy, ascites, and SBP. SBP depicts worse outcome. In all cases of FHF with ascites, the presence of SBP should be investigated.

Health factors in colostrum

Colostrum is a breast milk produced after the birth of the newborn and lasts for 2–4 days. Colostrum is very important part of breast milk and lays down the immune system and confers growth factors and other protective factors for the young ones in mammals. This is the source of passive immunity achieved by the mother and is transferred to the baby. This is the major source of secretory IgA and gives protection against gastrointestinal infections. In view of so many health factory through colostrum, the use of colostrum has been extended to so many health problems of mankind. Human and bovine colostrums have many similarities barring that bovine colostrum can be obtained in large quantity, so bovine colostrum has been used in various disorders in human beings. This is the nature’s gift that is for the young ones to grow as well as for the treatment of many health problems in older age group

Gastric augmentation in isolated congenital microgastria

A 10-month-old boy with isolated congenital microgastria is reported. This is an extremely rare condition with only 2 previous similar case reports. The patient was treated successfully with early gastric augmentation. Although the embryologic origin of this anomaly was widely believed to be in the fourth week of gestation, the anatomic features in the current case point to the arrest of development at least after the eighth week of fetal life.

Role of Colostrum in Gastrointestinal Infections

Colostrum is breast milk produced after the birth of the newborn and lasts for 2–4 days. Colostrum is very important part of breast milk and lays down the immune system and confers growth factors and other protective factors for the young ones in mammals. This is the source of passive immunity transferred to the baby from the mother. The biological value of bovine colostrum in present day medical practice is documented in clinical trials and large databases containing case reports and anecdotal findings. The main actions include an antibacterial effect and modulation of immune response with the ability to neutralize lipopolysaccharides arising from gram negative bacterial pathogens. It has been found to be effective in infantile hemorrhagic diarrheas, other diarrheas and reduces the likelihood of disease progressing to hemolytic uremic syndrome. It has also been tested in H pylori infection and diarrhea in immunodeficiency. Side effects of clinical relevance are limited to possible intolerance due to lactose and sensitivity to milk proteins.

Segmental dilatation of ileum: an unusual cause of severe malnutrition.

Segmental dilatation of the ileum causing blind loop syndrome was seen in a 6-year-old boy presenting with nonspecific symptoms and severe malnutrition. Barium meal was characteristic. Resection of the affected segment and an end-to-end anastomosis was curative and restored normal bowel function. Clinicians’ awareness of this entity would help in appropriately managing such cases.

Juvenile polyposis in a tropical country

The clinical profile, malignant potential, and management of 17 children with juvenile polyposis (more than five juvenile polyps) were evaluated clinically and endoscopically. Colonoscopy and polypectomy were done three weekly until colonic clearance was achieved, and thereafter two yearly. All polyps were subjected to histological examination. Mean age was 7.7 years, with a male preponderance (3:1). Presentation was with rectal bleeding (94%), pallor (65%), stunted growth (53%), and oedema (47%), and the mean (SD) duration of symptoms was 33 (27) months. None had a positive family history or any congenital anomaly. Two children had six polyps up to the transverse colon; the rest had numerous polyps all over the colon. All children had juvenile polyps on histology and 10 (59%) had adenomatous changes (dysplasia). Total colectomy was done in six for intractable symptoms. Colon clearance was achieved in eight after an average 3.4 polypectomy sessions, and three were still on the polypectomy programme. In conclusion, juvenile polyposis is commonly associated with low grade dysplasia. Serial colonoscopic polypectomy is effective but colectomy is required for intractable symptoms and when clearance of the colon is not possible.

Mitochondrial neuro-gastrointestinal encephalopathy syndrome

Mitochondrial neurogastrointestinal encephalomyopathy is a rare disorder affecting the pediatric age group with a heterogenous multisystem involvement. We happen to manage a young child with symptoms of constipation since infancy alongwith cachexia, seizures and peripheral neuropathy. The child later went into encephalopathy preterminally. This clinical syndrome fitted very well with mitochondrial neurogastrointestinal encephalomyopathy. The child had elevated lactate levels and electron microscopy of the rectal biopsy was suggestive of a mitochondrial disorder To the best of our knowledge there is no case report of this syndrome from India and since this presents with diagnostic difficulties so is being reported.

PEUTZ-JEGHERS POLYPOSIS WITH ENTERITIS CYSTICA PROFUNDA--A DIAGNOSTIC PITFALL

A10-year-old female child presentedwith history of o¡-and-on abdominal pain present for 2 years. The pain had become more frequent and colicky in the last 15 days. It was present in the hypogastrium and periumbilical area.There was history of constipation and an occasional episode of fresh blood with stools. She was the secondof twins and the other twin sister was asymptomatic. On examination,melanosis was seen on the lips, oral mucosa, and tips of ¢ngers and toes since 1 year of age. Her mother had similar symptoms at 15 years of age for which she underwent an abdominal surgery. A part of the small bowel was resected and it was said to be studded with a few polyps. No written document or histopathology report is available. The mother has been asymptomatic ever since.The mother and the younger brotheralsohadoralmelanosis.Thebrother, however, hasbeenasymptomatic so far. On examination of the index case there was amass present in the periumbilical area measuring 5 3 cm. Reducible intussusception was present. Resection of the small intestine at two places was done with end-to-end anastomosis.Two segments (18 cm each) of the small intestine were resected, 9 inches from the duodenojejunal junction and at the jejunoilial junction. Both segments of the small intestine contained two polyps each.The largest polyp (5 cm in diameter) was seen 9 cm distal to the duodenojejunal junction, was pedunculated, and there were small cysts with mucoid material present at all levels of the wall including the serosa (Figure 1). The other three polyps were sessile and ranged from 2 to 4 cm in diameter. They too showed small cystic spaces with mucoid material. Microscopically the polyps revealed a classic morphology. Normal smallintestinal glands rested on a branching smooth muscle framework (Figure 2). There was no evidence of dysplasia. Transmural herniation of the mucosal glands admixed with smooth muscle ¢bers and lamina propria was seen until the serosa (Figure 3). Apart from this there were cystically dilated spaces (0.2^1 cm diameter)

A molecular marker of disease activity in autoimmune liver diseases with histopathological correlation; FoXp3/RORγt ratio

Autoimmune liver diseases (AILDs) encompass a group of diseases with variable clinicopathological manifestations. Th17 and Treg cells have roles in the pathogenesis of AILDs with a balance shifted towards a relative increase in activity of the Th17 cells. In this study, the balance between the transcription factors of Treg and Th17 cells (FoXp3 and RORγt) was sought as a molecular marker of disease activity and to highlight the pathogenesis. The peripheral blood samples of 46 treatment‐naive patients were collected and RNA was extracted. Real time PCR was performed and the ratio of gene expression was calculated. Histopathology of 18 patients was obtained and the activity score of these biopsies were also corroborated with their respective molecular (FoXp3/RORγt) (FRGT=FoXp3‐ROR Gamma T) ratio. The FRGT ratio in healthy individuals was close to 1 and in disease the ratio changed significantly. This ratio (FRGT) was not significantly different in different varieties of AILD or in adult or paediatric form of the disease. However, the ratio remained consistently below 1 (mean 0.3) in acute disease and high (mean 224.7) in chronic or asymptomatic form of the disease (p < 0.001). The histopathological activity score also significantly correlated with the ratio. This signified the relative excess of Th17 (RORγt) in active disease as compared to Treg (FoXp3) and the reverse in chronic form. This ratio can be an important peripheral molecular marker to assess the disease activity without the necessity of performing a liver biopsy.

Henoch Schönlein purpura presenting as duodenal ulcer and gastric outlet obstruction.

Henoch-Schönlein purpura (HSP) is an acute small vessel leucocytoclastic vasculitis. It is the commonest vasculitis in children, with an incidence of about 10 cases per 100, 000 a year. Gastrointestinal manifestations are commonly encountered, however hematemesis and gastric outlet obstruction are rarely reported. The authors present the case of a 5-y-old boy having hematemesis, gastric outlet obstruction and multiple duodenal ulcers. He improved with steroids and conservative management.