Necrotizing enterocolitis following exchange transfusion.
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Biomedical subjects
Publications and source records attributed to G Karayalcin.
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An 11-year-old black boy with sickle cell anemia developed profound pancytopenia during the course of his disease, but fully recovered therefrom. The patient was receiving anticonvulsant drugs for a seizure disorder secondary to a "stroke," and, therefore, a drug-related marrow aplasia cannot be ruled out.
A group of 150 healthy American black children were found to have neutrophil counts significantly lower than those of a similar group of healthy American white children. Absolute lymphocyte counts were similar in the black and white children. The lower neutrophil counts in black children may represent a genetic or hitherto unidentified acquired differences.
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The clinical and hematological features of 100 patients with sickle cell anemia are reviewed. The heart was enlarged and a murmur was heard in nearly 80 percent of patients. Pneumonia and pulmonary infarction occurred in 43 percent and 12 percent of patients, respectively. Musculoskeletal involvement included the hand-foot syndrome (15 percent), leg ulcers (55 percent), aseptic necrosis ofbone (11 percent), and osteomyelitis (4 percent). Symptoms and signs related to the gastrointestinal system included jaundice (55 percent), hepatomegaly (50 percent), splenomegaly (23 percent), hepatitis (11 percent) and gallstones (9 percent). Three patients underwent cholecystectomy and three patients had their spleens removed. Pyelonephritis occurred in 17 patients, priapism in five and hematuria in seven. Nineteen women had 39 pregnancies, of which 35 resulted in the birth of healthy infants. At least 328 painful crises occurred in 73 patients. There were also 13 hemolytic crises, eight sequestration crises, and five aplastic crises. A trail of alkali therapy in 33 crises in children failed to produce beneficial effects greater than hydration and analgesics alone as used in the control group. Laboratory findings in the 100 patients were comparable to those previously reported in the literature. The renal concentrating defect in most patients was confirmed. There were six deaths: hepatic coma secondary to post-transfusion hepatitis, thrombosis of inferior vena cava, congestive heart failure, exsanguination from erosion of the pancreaticoduodenal artery, extensive bronchopneumonia, and pulmonary infarction.
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Lymphomatoid papulosis (LyP) is a benign, self-healing, papular eruption that can wax and wane over the course of time. Transformation to T-cell lymphoma has been well documented in 10% to 20% of adults with LyP, but there are have been no cases reported in patients younger than age 26 years. We describe the first pediatric patient, a 16-year-old girl, who had clinical features of LyP and concurrently was found to have a lesion diagnosed as Ki-1+ anaplastic large cell lymphoma. After treatment with chemotherapy, she has been in continuous remission for 16 months.
The most significant clinical manifestations of sickle cell disease (SCD) are those associated with CNS involvement. This disease is systemic in character, with the tissues of the CNS being the most sensitive to even temporary anoxia. For this reason, the incidence of peripheral and central auditory dysfunction in patients with SCD may be greater than in the "normal" population. We examined the peripheral auditory function of 54 patients with homozygous sickle cell disease and the central auditory function of 28 of these patients. Results were compared with a control population. Peripheral hearing loss was found in 11% (6) of the SCD population. Central auditory testing indicated that 46% (13) of the sickle cell population had mild central auditory dysfunction not involving Heschl's gyrus. This study suggests a higher than usual incidence of peripheral and central auditory impairment in sickle cell disease.