Cerebral edema causing death in children with maple syrup urine disease
| Autor: | Angelo M. DiGeorge, Iraj Rezvani, Catherine M. Foley, James J. Riviello |
|---|---|
| Rok vydání: | 1991 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities Pediatrics medicine.medical_specialty medicine.medical_treatment Brain Edema Autopsy Cerebral edema Peritoneal dialysis Maple Syrup Urine Disease Edema medicine Humans Dialysis Acidosis Dehydration business.industry Maple syrup urine disease nutritional and metabolic diseases Prognosis medicine.disease Surgery Child Preschool Acute Disease Pediatrics Perinatology and Child Health Female medicine.symptom business Complication |
| Zdroj: | The Journal of Pediatrics. 119:42-45 |
| ISSN: | 0022-3476 |
| DOI: | 10.1016/s0022-3476(05)81036-4 |
| Popis: | Four children with the classic form of maple syrup urine disease (MSUD) died of cerebral edema during an intercurrent infection that caused severe dehydration and acidosis. The diagnosis of MSUD had been established during the neonatal period in all four patients, on day 1 of life in three of them. All were in satisfactory control before the intercurrent illness. Two patients underwent peritoneal dialysis. Signs of brain-stem compression occurred after treatment, when biochemical abnormalities were improving. Computed tomography of the head, which was done in two patients, revealed cerebral edema; one of these patients also had subarachnoid hemorrhage. Autopsy in one case revealed cerebral edema with herniation. Our experience documents that cerebral edema may occur in the older child with MSUD as well as in the neonate. The pathogenesis of cerebral edema in MSUD remains unclear. Early treatment of dehydration and acidosis may prevent the catastrophic consequences that we have observed. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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