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Washington University Experience | METABOLIC | Neuronal Ceroid Lipofuscinosis (NCL) | 7A0 Case 7 History
Case 7 History) ---- The patient was a 21 year old woman with a history of disseminated intravascular coagulation, left lower lobe pneumonia, neuronal ceroid lipofuscinosis, secondary blindness, seizure disorder, muscle wasting and atrophy, and contractures. The patient had severe mental retardation and had been cared for in a nursing home for several years. Because of her decreasing ability to swallow and handle secretions, the patient had a percutaneous gastrostomy tube placed in April 1986. She tolerated the procedure well and had very little difficulty after the procedure. The tube was not used in the nursing home, but two days after placement of the tube the patient was admitted to an OSH with sepsis, rapid heart rate, cyanosis, hypotension and tachypnea. The patient was treated with oxygen and IV fluids with a chest Xray showing a faint infiltrate in the left lower lobe. She was begun on broad spectrum antibiotics. Over the next two days she improved markedly. Her initial fever spikes of 103° subsequently abated on antibiotics. An NG tube was placed and had relatively high outputs until the patient began having spontaneous bowel movements. On 4/9/87 she had a fever of 99°F. The NG tube had very little output and the gastrostomy tube was in good working order. Tube feedings were begun later that day. It was noted that she had some blood tinged fluid in her Foley catheter and was bleeding from an old IV site at least one week old. Blood studies at that time showed prolongation of the PT, PTT and elevated fibrin split products; however, the patient had no dramatic evidence of blood loss. She became progressively hypotensive. She was supported with IV fluids, but was not transfused. The patient had a cardiovascular collapse, did not respond from her state of shock and expired in April 1987. The exact cause of her DIC was not known. The patient was clinically not septic with no obvious white blood cell elevation.
