Extramedullary haematopoietic tumor producing small intestinal intussusception in a beta-thalassemia/hemoglobin E Thai boy: a case report.

The authors report a case of beta-thalassemia/hemoglobin E disease with extramedullary haematopoietic tumor which developed at the small intestine and caused intussusception. A 7 year-old boy with homozygous beta-thalassemia/hemoglobin E presented with recurrent abdominal pain. The abdominal ultrasonography showed ileo-ileal intussusception with a solid mass as the leading point. Resection of the ileal segment was performed. Pathological examination revealed an extramedullary haematopoietic tumor forming an intraluminal polypoid mass, being the leading point of the intussusception. Extramedullary haematopoiesis in the intestinal tract is rare. To our knowledge, this is the first case of extramedullary haematopoietic tumor that produced intussusception of the small intestine in a beta-thalassemia/hemoglobin E patient.

Recombinant activated factor VII in children with acute bleeding resulting from liver failure and disseminated intravascular coagulation.

Recombinant activated factor VII (rFVIIa) was given to three children with acute bleeding resulting from liver failure and disseminated intravascular coagulation. Cases I and II (girls aged 3 years and 6 years, respectively) were diagnosed with Dengue hemorrhagic fever and prolonged shock. Case III, a boy aged 9 months, underwent left lobe hepatectomy for a hepatoblastoma, during which 60% of his liver was removed. This case was complicated by myoglobinuria, liver and renal impairment and early disseminated intravascular coagulation. All three patients exhibited active bleeding. Cases I and II received rFVIIa combined with other blood component replacement, while Case III received rFVIIa as the only hemostatic agent. A bolus of 40-180 microg/kg b.w. was administered followed by 16.5-33 microg/kg b.w. per h continuous infusion. As a result, bleeding was controlled, the prothrombin time was shortened and FVII clotting activity was significantly increased. In conclusion, rFVIIa has shown some efficacy in controlling acute bleeding in children with liver failure and disseminated intravascular coagulation.

Persistent hyperinsulinemic hypoglycemia of infancy: long-term outcome following subtotal pancreatectomy.

BACKGROUND: Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is the most common cause of persistent hypoglycemia in infants. The current standard treatment is subtotal pancreatectomy (Px). However, the long-term outcome following surgery needs further attention. METHODS: We analyzed 10 children (7 M, 3 F) with PHHI who underwent partial (65-80%) and subtotal (81-95%) Px. Follow-up ranged from 2 to 9.4 yr (mean = 4.2 yr). We divided them into 2 groups based upon the age at onset of hypoglycemia: early (< 1 mo) and late (> or = 1 mo). RESULTS: The seven patients in the early-onset group underwent 85-95% Px between ages of 18 d and 3 mo. Three of them initially treated by 85-90% Px had persistent hypoglycemia postoperatively. Two out of three required a 2nd operation with 95% Px for controlling hypoglycemia, though both still had persistent hypoglycemia and required medication to control blood glucose. The remaining four had 95% Px and had maintained euglycemia postoperatively. One patient developed diabetes 6 yr after surgery. Six of seven patients had delayed development and subnormal IQ. Three patients of the late-onset group (3 mo, 6 mo and 4 yr) underwent partial Px (80%, 65% and 65%, respectively) and maintained euglycemia postoperatively. Despite 65% Px, one developed diabetes 3 yr after surgery. CONCLUSIONS: These results suggest that children with early-onset hypoglycemia have more severe hyperinsulinism than those with late-onset hypoglycemia. The former require 95% Px for maintaining euglycemia, but long-term complications with diabetes may be common. In contrast, the latter require lower percentage Px which may reduce the incidence of diabetes in the future.

Subcutaneous portacath utilization in pediatric oncology patients: Ramathibodi Hospital experience.

Subcutaneous portacaths (SQP) placement in 19 pediatric oncology patients were studied. Complications of SQP were evaluated. Two patients had premature SQP removal due to fungal infection and breakage, 1 for each. Two patients had catheter-related bacteremia which was resolved by antibiotic administration. Only 1 patient had occasional difficult blood drawing episodes, because the tip of catheter was inserted through external jugular vein instead of subclavian vein. There were no other serious complications except that some of them had clotted formations, which were resolved easily by urokinase administration. Long-term SQP utilization was possible in 17 of 19 patients, with the average time of 7.5 months. Few complications occurred in the group of patients studied. SQP improved quality of medical care and significantly lessened the anxiety of patients who need long-term chemotherapy treatment. Therefore, placement of the intravenous access device is feasible for pediatric oncology patients in Thailand. The patients are no longer suffering from repeated venipunctures. Although it is expensive, it is convenient and useful for some patients with relatively high socioeconomic status. It should be considered for every pediatric cancer patient who needs prolonged chemotherapy and who has affordable means.