Investigative approach and medical management or portal hypertension.
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Biomedical subjects
Publications and source records attributed to B R Thapa.
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A 2 year old female child with Niemann-Pick disease was referred as case of chronic liver disease; clinically, she had neurological involvement. Liver biopsy showed Niemann-Pick cells. Hepatic manifestations of Niemann-Pick disease as seen in nine cases reported from India are discussed.
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Eighty colonoscopies were performed in 72 children suffering from various colonic disorders. The indications for colonoscopic examination were bleeding per rectum (83.3%), prolonged colitis (14.0%) and colonic obstruction (2.7%). Total bowel wash with normal saline was very good for colonic preparation in majority of the cases. Pediatric colonoscope (PCF-Olympus) was used and the procedure performed under sedation with intravenous diazepam and pentazocine. Juvenile polyps were the commonest lesions (69.4%). Diagnosis of idiopathic ulcerative colitis, acute colitis, tuberculous colitis, amebic colitis and allergic colitis was made in 5.5, 4.2, 2.7, 1.3 and 1.3% cases, respectively. Tuberculous hypertrophic lesion and tuberculous stricture was seen in one case each. The commonest site of juvenile polyps was rectosigmoid region. Ninety per cent of them were single, 8% had polyps ranging from 2-4 in number and one (2%) child had juvenile polyposis coli. Successful colonoscopic removal of polyps with polypectomy snare was possible in 96% cases. No complications were observed. Colonoscopy is a safe and useful mode of investigation of colonic disorders in expert hands. It has a great therapeutic value in childhood.
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Thalassaemia intermedia with distressing complications is reported from India. An 11 year old boy presented with recurrent fractures since 6 years of age. Investigations revealed that he had multiple healed fractures of long bones, hypersplenism, iron overload and a red blood cell half life (t1/2) of 17 1/2 days. Splenectomy was planned.
Kayser-Fleischer ring is considered an important diagnostic sign of Wilson's disease. We report a 9 year old boy with autoimmune chronic active hepatitis who exhibited Kayser-Fleischer like ring.
Thirty children, aged 7 months to 13 years, with bleeding esophageal varices were managed by endoscopic sclerotherapy (EST). Of these children, 73.3% had extrahepatic portal vein obstruction (EHPVO), 16.6% had cirrhosis of the liver, and 10% had noncirrhotic portal fibrosis. EST was done with fiberoptic pediatric upper gastrointestinal endoscopes and a flexible sclerotherapy needle under sedation with intravenous diazepam and pentazocine. The sclerosants used were ethoxysclerol 1% and absolute alcohol. Ten injections were given to control active variceal bleeding, and 145 injections were given on planned basis at 2-3-week interval. An average of five injections was required to obliterate the esophageal varices. In 90% of cases, an avariceal state was achieved; 10% had decreased size and number of varices. Emergency EST was effective to control bleeding in all sessions. Complications, including retrosternal discomfort, esophageal ulceration, stricture formation, and aspiration pneumonia, occurred in 60, 20, 20, and 6.6% of cases, respectively; complications were managed conservatively. Strictures were dilated with Eder-Puestow's olive dilators. Recurrence of esophageal varices, gastric varices, and rebleeding was seen in 13.3, 13.3, and 10% of cases, respectively. Shunt surgery was performed in 13.3% cases, who had developed gastric varices and were having EHPVO.
Gastrointestinal disturbances like anorexia, nausea, vomiting, abdominal discomfort and diarrhoea are known adverse effects of rifampicin. We report an upper gastrointestinal bleeding due to haemorrhagic gastric erosions after ingestion of rifampicin for pulmonary tuberculosis. The cause and effect relationship between development of haemorrhagic gastric erosions and rifampicin administration was confirmed by rechallenge with rifampicin. To our knowledge no such adverse effect of rifampicin has been reported previously.
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Blood sugar (BS), serum immunoreactive insulin (IRI) and growth hormone (GH) were measured in 5 normal children and 15 with varying grades of protein energy malnutrition (PEM) during a standard oral glucose tolerance test (OGTT). Impaired glucose tolerance was noted in grade III and IV PEM but not in grade II. Serum insulin responses were low but appropriate to blood glucose in grade II and grade III PEM and were lower (p less than 0.001) in Grade IV PEM subjects. Higher basal GH was observed in most cases of grade III and IV PEM (p less than 0.001) and the elevated GH was not suppressed following the oral glucose load. Further, a paradoxical rise in GH during OGTT was seen in 3 of the 5 subjects with grade-IV PEM.