Subtotal pancreatectomy for hypoglycemia due to congenital hyperinsulinism: long-term follow-up of neurodevelopmental and pancreatic function.

OBJECTIVE: To evaluate neurodevelopmental status as well as endocrine and exocrine pancreatic function in children who have undergone subtotal pancreatectomy for hypoglycemia due to congenital hyperinsulinism. PATIENTS AND METHODS: Out of 15 identified patients, eight children (mean age 12.7 +/- 0.8 yr) participated in detailed psychometric testing and studies assessing glucose homeostasis, secretion of proinsulin, insulin, glucagon and C-peptide during a test meal. Additionally, a 24-h fast, glucagon challenge test, 72-h stool collection, and ultrasonography of the pancreatic remnant were performed. RESULTS: Five of the 15 initially identified children had seizure disorders, including two with mental retardation. Diabetes developed in two of 15 children. All eight children investigated in the present study had evidence for attentional control impairment and 50% had subnormal intellectual functioning. Two had symptomatic hypoglycemia during the 24-h fast, while one had an elevated fasting glucose concentration. Four children, including the latter patient, had proinsulin/insulin ratios resembling patients with type 2 diabetes. Exocrine pancreatic function was normal in all eight children. No correlation was found between pancreatic endocrine function and pancreatic remnant size, nor between multiple pre- and postoperative factors (i.e., age at diagnosis and surgery) and neurodevelopmental outcome. CONCLUSION: While severe mental retardation or diabetes occurred infrequently in our patient population compared with previous reports, all of the studied children had subtle anomalies in their cognitive performance tests and the majority had endocrine test results indicative of abnormal insulin secretion and stressed pancreatic beta cells. Although partial pancreatectomy remains the treatment of choice after medical therapy fails, improved therapeutic means are necessary to achieve better clinical outcome.

Retrograde filling of the renal vein on computerized tomography for blunt renal trauma: an indicator of renal artery injury.

Assessment of the traumatically injured pediatric patient with computerized tomography has become standard medical practice. We report the unique finding of retrograde flow of intravenous contrast material into the renal vein as a diagnostic indicator of traumatic renal artery injury. With the increasing use of high-speed computerized tomography as the initial study to evaluate and stage blunt abdominal trauma, this finding may assist the physician in the early diagnosis of severe renovascular injury.

Vancouver hybrid: preliminary experience in the treatment of Hodgkin's disease in childhood and adolescence.

OBJECTIVE: To describe our preliminary experience with 19 young patients with newly diagnosed Hodgkin's disease who received the Vancouver hybrid chemotherapeutic regimen. DESIGN: We summarized the characteristics of our 19 study patients, the treatment administered (between June 1988 and June 1992), and the outcome. RESULTS: The Vancouver hybrid, which consists of mechlorethamine, vincristine sulfate (Oncovin), procarbazine hydrochloride, prednisone, doxorubicin hydrochloride (Adriamycin), bleomycin, and vinblastine sulfate (MOPP/ABV), was based on the hypothesis of preventing drug resistance by early introduction and alternation of all active agents and was aimed at decreasing the severity and frequency of treatment-related complications. Of our 19 patients with Hodgkin's disease (age range, 6 to 20 years) treated with this regimen, 2 had clinical stage I disease, 10 had stage II, 6 had stage III, and 1 had stage IV. Only two patients had systemic symptoms, and nodular sclerosis was the most common histologic feature. Patients were given four to eight cycles of chemotherapy, depending on the clinical stage of disease. In addition, 10 patients received irradiation, including 6 of 9 patients with bulky disease. In all patients, complete remission was achieved. After a median follow-up of 3.3 years, only two patients had had a relapse; both underwent autologous bone marrow transplantation and were alive and well with no evidence of disease at last follow-up. The treatment was well tolerated, and delivery of treatment was excellent. The only severe toxicity was myelosuppression; 8 patients experienced a total of 15 episodes of fever and neutropenia that necessitated hospitalization and antibiotic therapy, but no systemic infections were confirmed during 104 cycles of therapy. CONCLUSION: The MOPP/ABV hybrid is an effective and well-tolerated therapy in most young patients with Hodgkin's disease. Long-term monitoring is needed to evaluate late effects.