Maynika V Rastogi1, Ninad Desai, J B Quintos. 1. King's County Hospital Center and Children's Hospital at Downstate, SUNY Downstate Medical Center, Brooklyn, NY, USA.
Abstract
BACKGROUND: Non-islet-cell tumor hypoglycemia (NICTH) is a rare cause of hypoglycemia associated with tumors of mesenchymal, epithelial, or hematopoietic origin. Lactic acidosis is likewise an uncommon complication of hematological malignancy associated mainly with leukemia and lymphoma. Most cases of NICTH and lactic acidosis have been described in the adult population. We report a child with congenital HIV and AIDS who developed Burkitt's lymphoma, lactic acidosis and NICTH. PATIENT: An 11 year-old boy with AIDS, cerebral palsy and seizure disorder presented with intractable hypoglycemia 12 days after diagnosis of Burkitt's lymphoma. He had persistent hypoglycemia (serum glucose 20-40 mg/dl; 1.1-2.2 mmo/l) despite glucose infusion rate of 6 mg/kg/minute and trial of diazoxide treatment. Critical sample obtained at time of hypoglycemia showed insulin at 1.78 microU/ml (normal <5 microU/ml), pro-insulin 5.6 pmol/l (<18.8 pmol/l), IGF-I <25 ng/ml (80-723 ng/ml), IGF-II 422 ng/ml (610-1,217 ng/ml), lactate 15.6 mmol/l (normal: 0.5-2.2 mmol/l), cortisol 21 microg/dl (580 nmol/l; normal >10 microg/dl; 276 nmol/l), and negative insulin antibodies. He remained alert and seizure free despite profound hypoglycemia. A 1 mg glucagon stimulation test showed a rise in serum glucose of 29 mg/dl (>1.6 mmol/l). Continuous glucagon infusion at 0.15-0.3 mg/h maintained euglycemia until the time of his demise (1 month after admission) due to complications of his underlying illness. CONCLUSION: We present a case of lactic acidosis and NICTH in an 11 year-old boy with AIDS and Burkitts's lymphoma. We review the mechanism of hyperlacticacidemia in supporting cerebral function during profound hypoglycemia. NICTH and lactic acidosis in association with malignancy carries a poor prognosis. In this patient, continuous glucagon infusion was a successful alternative to corticosteroid treatment in maintaining euglycemia.
BACKGROUND: Non-islet-cell tumor hypoglycemia (NICTH) is a rare cause of hypoglycemia associated with tumors of mesenchymal, epithelial, or hematopoietic origin. Lactic acidosis is likewise an uncommon complication of hematological malignancy associated mainly with leukemia and lymphoma. Most cases of NICTH and lactic acidosis have been described in the adult population. We report a child with congenital HIV and AIDS who developed Burkitt's lymphoma, lactic acidosis and NICTH. PATIENT: An 11 year-old boy with AIDS, cerebral palsy and seizure disorder presented with intractable hypoglycemia 12 days after diagnosis of Burkitt's lymphoma. He had persistent hypoglycemia (serum glucose 20-40 mg/dl; 1.1-2.2 mmo/l) despite glucose infusion rate of 6 mg/kg/minute and trial of diazoxide treatment. Critical sample obtained at time of hypoglycemia showed insulin at 1.78 microU/ml (normal <5 microU/ml), pro-insulin 5.6 pmol/l (<18.8 pmol/l), IGF-I <25 ng/ml (80-723 ng/ml), IGF-II 422 ng/ml (610-1,217 ng/ml), lactate 15.6 mmol/l (normal: 0.5-2.2 mmol/l), cortisol 21 microg/dl (580 nmol/l; normal >10 microg/dl; 276 nmol/l), and negative insulin antibodies. He remained alert and seizure free despite profound hypoglycemia. A 1 mg glucagon stimulation test showed a rise in serum glucose of 29 mg/dl (>1.6 mmol/l). Continuous glucagon infusion at 0.15-0.3 mg/h maintained euglycemia until the time of his demise (1 month after admission) due to complications of his underlying illness. CONCLUSION: We present a case of lactic acidosis and NICTH in an 11 year-old boy with AIDS and Burkitts's lymphoma. We review the mechanism of hyperlacticacidemia in supporting cerebral function during profound hypoglycemia. NICTH and lactic acidosis in association with malignancy carries a poor prognosis. In this patient, continuous glucagon infusion was a successful alternative to corticosteroid treatment in maintaining euglycemia.