A Case of Chronic Granulomatous Disease (Autopsy case)

A 3 year and 3 months old boy with recurrent infections since his age of 5 months was presented with clinical data and autopsy findings. He was the 4th product of healthy parents. His elder brother died of recurrent perianal abscess and sepsis at his age of 3 years. His 2nd elder sister died on the 14th day of life probably from the complication of BCG vaccination. Beginnig with perianal abscesses at his age of 5 months, he has been continuously suffering from recurrent infections such as arthritis, ostomyelitis, pneumonia, epididymitis, subcutaneous abscesses and perianal abscesses. In spite of meticulous supportive and aggressive antibiotic therapy persistent positive cultures for staph. Aureus, klebsiella, E. Coli, Enterococcus and coliform bacilli from different sited were noted. Erythrocyte sedimentation rate of 25 to 40 were constant. White cell count varied frem 15500 to 33400 with polymorphonucleocytes predominance. NBT test showed persistent low scoring of 2% throught the course. He finally died of pneumonia and empyema. At postmortem examination, multiple abscesses and grnulomas of right lung and multipe granulomas in the liver, spleen, lymph node, bone, marrow, adrenal gland, kidney and intestinal wass were noted. At microscopic examination histiocytic granulomas with lipid containing histiocyte infiltrations were noted in every organs described including brain.

A Clinical Observation on the Premature & Low Birth Weight Infants

A clinical & statistical analysis was made on 325 cases of premature & low birth weight infants, admitted to the department of Pediatrics, National Medical Center, during the period. Of September, 1968 to august, 1976. The results obtained were as follows. 1. Sex ratio was 1.1 :1 between male & female. 2. Monthly distribution showed higher incidence in cold season than in warm season. 3. In regard to the place of delivery, there showed increasing tendency of hospital delivery. 4. Important predisposing factors disclosed toxemia of pregnancy, inappropriate pelvic condition, acute infection & twin delivery in order of frequency. Among them toxemia of pregnancy was the most important. 5. Overall incidence of premature delivery was 5.0% in 1968 and 3.1% in 1975 and revealed. Decreasing tendency. 6. Concerning the order of pregnancy, the highest incidence was among the 1st pregnancy. (33.2%). 7. In low birth weight infants, Apgar score was higher weight group. 8. The mortality rate was higher in males and in the group of out side delivery. The highest mortality rate was noted in the birth weight group of 1001-1250 gm. The larger the gestational period the lower the mortality rate was also noted. 9. Most of the deaths occurred during the first 48 hours and sharp decrease after the 3rd day of life. Main causes of death were idiopathic respiratory distress syndrome and pneumonia. 10. Initial weight loss was marked between the 7 th and 11 th day of life and about 10% of birth weight. The lower weight group showed the greater loss. 11. Regain as well as true weight gain during the 1 st month of life was slower in low birth weight group.