A Case of Neurofibromatosis without Cafe-au-lait Macule

Almost always patients with multiple neurofibromatosis show cafe-au-lait macules on their skin. At times the skin macules are seen even before the appearance of the neurofibromata in childhood. The author experienced one case of neurofibromatosis in a 12-year-old boy who has no cafe-au-lait macules. Besides the clinical peculiarity of having no skin pigmentation, the histopathology of the case showed well-formed double-palisading structure, which is not common among the micro-scopical findings of neurofibromata.

Follow-up Study of 104 Cases of Blood Exchange Transfusion for Hyperbilirubinemia

A study was made of 104cases of neonatal hyperbiliruinemia receiving blood exchange transfusion at Kwangju Christian Hospital from January 1969 to December 1975, with the following results: 1. 52 patients(50%) returned for follow-up, 9 patients(8.7%) had died, and 43 patients(41.3%) did not return. 2. Of the 52 patients returning, developmental status was studied by D.D.S.T. 43 patients(82.7%) had normal development, 8(15.4%) were retarded, and 1(1.9%) was questionable. No patients had a failing score. Most of the retarded patients had cerebral palsy, due to kernicterus. 3. There was no retardation among patients exchange-transfused at age of less than 5days, or over 8 days(except 1 pts.) , and with less than 30mg% of Pre-B.E.T. serum bilirubin level. 4. No significant difference of sex incidence was noted. 5. ABO incompatibility (57%) was predominant as the etiology and main cause of retarded development (19.4%), but the idiopathic type of hyperbilirubinemia(41.3%) was interestingly high among these B.E.T. patients. 6. Mean value and standard error of pre-B.E.T. serum bilirubin levels are as follows: Normal development group:30.80+/-1.60 Retarded development group:38.83+/-2.35 The difference of men values between both groups is very significant according to the t-test. 7. Motor disturbance was the predominant handicap in retarded patients at ages less than 3 years, and after this there was a tendency toward recovery, accompanied by mild speech disturbance. Hearing loss, especially of high pitched tone, was noted in 1 patient and confirmed by audiometry.