ABSTRACT: Sixteen children underwent 17 orthotopic liver transplantations between September 1993 to November 1998. Living-related liver transplant (n=12) outnumbered reduced-size liver transplant (n=5). The age ranged from 7 months to 11 years (mean 11 months) and their body weight ranged from 6 to 25kg (mean 6.5 Kg). United Network for Organ Sharing (UNOS) status was as follows: 2 patients belonged to status 2,4 to status 3 and none to status 4. Despite a range of early and late complications which required surgical intervention, the overall results were good. At follow-up ranging from 11 to 72 months, patient survival was 94 percent (15/16) and graft survival was also 94 percent (16/17). All donors are well and were pursuing all pretransplant activities. Pediatric liver transplantation is extremely taxing, but with complete dedication and good cooperation between all involved specialities, a favourable outcome can be expected.

ABSTRACT: The etiology of neonatal necrotising enterocolitis (Nec) remains elusive although most investigators agree that it results from the injury to the newborn's highly vulnerable intestinal mucosa. Mucosal ischemia leading to gut injury is accepted as the most plausible cause for this event. This can result from many stressful situations experienced by premature infants. Hypoxia is known to be one of the commonest physiological stress to which the neonates are exposed. It has been associated with NEC in clinical and experimental situations. Hypoxia results in decreased intestinal perfusion, which may lead to bowel necrosis. If the period of ischemia or hypoxia is not long enough to injure the tissue irreversibly, most of it can be salvaged by reperfusing the tissue and therefore reintroducing oxygen and other nutrients. Reintroduction of O2 to an ischemic or hypoxic tissue could cause and additonal damage to the tissue which is mediated by free oxygen radicals. If the hypoxia or ischemia is partial, it is the reoxygenation/reperfusion injury to the tissue which will determine the ultimate damage to the tissue. An endogenous antioxidant mechanism already exists to counteract the effect of free radicals in the tissues. This defence system can be overwhelmed either due to the excess of free radical generation or inadequate stores of endogenous antixidants, as in sick premature infant. There may be a possible window opportunity to prevent the aggravation of injury to the gut and other body tissues. This may be possible by providing the antioxidant supplementation before the onset of free radical damage to the tissue. Recent research have shown promising possibilities with the use of simple antioxidants. We are currently assessing the role of selenium, vitamin C and vitamin E in a rat model of NEC.

ABSTRACT: The encouraging result of OK-432 in Lymphangioma has led to the formation of Group Study for OK-432 for Lymphangiomas. The effect of intralesional injection of OK-432 in lymphangioma has been studied in this article. OK-432 is a lyophilized biological preparation containing the cells of Streptococcus pyogenes Sustrain treated with benzylpenicillin. OK-432 has lost its streptolysin s-producing ability and losts its toxic activities but produces various immunopharmacological activities and causes shrinkage of lymphangioma. The OK-432 is dissolved in physiological saline and is injected into the lymphatic cyst after aspiration of lymphatic fluid to a maximum doze 0.1mg at each injection. This has been tried in 26 cystic lymphangiomas and 18 cavernous lesion. 1 to 6 injections were given depending on the result of shrinkage of cysts. 92 percent of cystic lymphangioma and 42 percent of cavernous lymphangioma showed favourable result. The OK-432 has also been tried in post-opeative cases with limited success. The adverse reactions of OK-432 injection are fever which subsided with antipiretic drugs, local reaction and hypersensitivity reaction of skin. Other sides effects are general malaise, anorexia and nausea. The author also has described the mechanism action of OK-432.

ABSTRACT: Aim:Lymphangiomas are characterized by a proliferation of small lymphatics and vessels with intervening fibrous tissue and this is a common condition seen in children. Surgical excision of these leaves behind tell tale signs which could aesthetically be relevant to the child. OK-432 is a promising alternative for this condition. This study was designed to evaluate the effectiveness of OK-432 in the treatment of lymphangiomas in children. Patients and Methods:Twenty-four patients with clinical diagnosis of lymphangioma underwent the treatment, between the period 1996-1998, with OK-432 and formed the study group of this non-randomized clinical trial. Upon investigation 12 were cystic and the remaining were diffuse. Results: Seven out of the 12 cystic lymphangiomas responded very well. The remaining showed a partial response. All the 12 cases of diffuse variety showed poor response. conclusions: Intrta-lesional OK-432 is best suitable for the cystic variety of lymphangioma. this therapy has minimal side effects and should be the first choice for the treatment of cystic lymphangiomas.

ABSTRACT: Operated undescended abdominal testes were analysed with reference to the different type of operations to detect their present status and to detect the fertility status of the individuals. Retrospective analysis of hospital records from 1971 to 1995 was followed by examination of testes and seminal fluid analysis in suitable cases. One hundred and seventy-four (14.24 percent of total) patients had 196 abdominal testes. Their mean age of operation was 5.8 year (range=1-1/2-12years). One hundred and nine testes underwent transinguinal orchiopexy, 33 underwent transabdominal orchiopexy and 24 underwent transabdominal Fowler-stephen's orchiopexy. Twenty four testes were excised and six were absent (vanishing testes). Eighty six testes in 82 patients were evaluated. The mean follow-up period was 5.98 years (range=1-1/2-15 years). Forty nine percent of testes were rated as good and 22 percent as fair. Testis salvage was possible in a significant number. In patients who were operated within 5 years of age, the long-term result was better than in others. On seminal fluid analysis (n=6), in 2 unilateral undescended testes cases, seminal fluid rating was poor, indicating that unilateral undescended testis may cause impaired fertility.

ABSTRACT: Early diagnosis and surgery should promptly be instituted to prevent neurological sequelae of hypoglycemia in nesidioblastosis. We report a case of diffuse of nesidioblastosis in a 5-1/2 year boy who presented with hypoglycemic coma preceded by early morning convulsions. During this 3 year follow up after a near total pancreatectomy the boy showed no clinical or biochemical evidence of hypoglycemia.

ABSTRACT: The association between Hirschsprung's disease (HD) and colonic atresia (CA) is very rare. CA per se could lead to HD by failing to provide the pathway for the migration of the enteric neuroblasts in utero. Alternatively to this, HD could lead to in-utero volvulus and may result in CA as a result of vascular compromise. HD was diagnosed in the child after definitive treatment for the CA. High degree of suspicion of HD should be kept in mind while treating CA and multiple colonic seromuscular biopsies are recommended to make sure to exclude HD. The literature on this association is briefly reviewed and the possible mechanisms discussed.

ABSTRACT: Spondylo Costal type of Jarcho Levin syndrome is inherited as an autosomal dominant disorder. Neural tube defects in this subtype are uncommon. This report highlights the features and associated complications with this anomaly.

ABSTRACT: Short bowel syndrome is a term used for the anatomical, physiological and nutritional distrubances that occur when an extensive portion of the bowel has to be sacrificed for gangrene. We report here a case of a three and a half month old child who required extensive resection of the small bowel for gangrene secondary to malrotation and volvulus and the treatment modalities that were tried for the severe diarrhea which followed, and the use of tincture opium for the same.