Pneumocystis pneumonia associated with agammaglobulinaemia.

C.O., a female infant, had a birth weight of 6 lb. (2,720 g.). The parents were English and had one other normal child. She was admitted to hospital at the age of 3 days because of abdominal distension and vomiting. This settled without specific treatment. She went home but was readmitted two weeks later with similar symptoms together with loose stools. Intravenous fluids were required and E. coli 0 26 was isolated from the stool. 'She was treated with neomycin, recovered and went home for two weeks before being readmitted again, dehydrated with diarrhoea and vomiting, and with abdominal distension. Following a course of tetracycline, she made satisfactory progress, gaining 21 lb. (1,100 g.) in a month, reaching 9j lb. (4,100 g.), but then had another attack of loose stools with gross abdominal distension. She was now aged 3 months and respiratory distress was noticed for the first time and was thought to be caused by the abdominal distension. A radiographic examination using a barium meal showed a distended colon, a rectum of normal calibre and coning in the rectosigmoid area. A diagnosis was made of Hirschsprung's disease, associated with enteritis. She failed to thrive. She was transferred to the Royal Manchester Children's Hospital at the age of 5i months. She then still weighed 91 lb. There was gross abdominal distension with an empty rectum and she had a respiratory rate of 90 per minute associated with a bronchopneumonia. She was having loose offensive stools and vomiting. E. coli 0 26 was again isolated from the stool. Tetracycline was given and she slowly improved. The E. coli 0 26 disappeared and 10 days after admission a laparotomy was carried out. The colon was dilated and appeared to cone to normal size at the pelvi-rectal junction. Biopsies were taken above and below this site and a colostomy carried out proximal to it. However, post-operatively the distension persisted; and as normal nerve plexuses were found in the biopsies, the diagnosis of Hirschsprung's disease was not confirmed. She had continuous loose stools and vomiting, and required frequent intravenous fluids, including blood and plasma. An antibiotic resistant Proteus morgani was continually found in the faeces. From this time until her death the respiratory rate was rarely below 50 per minute and for several periods when she had signs of a bronchopneumonia was around 70 per minute. In between these exacerbations in the lungs there were persistent scattered crepitations, mainly at the bases. Radiographs taken at this time between attacks of bronchopneumonia were reported as normal. In retrospect, however, the lung fields do appear a little hazy. Because of the diarrhoea, large doses of potassium were given to maintain blood levels at a satisfactory value. She developed a bilateral otitis media, which did not respond to courses of erythromycin, novobiocin or streptomycin. The white cell count reached 12,000 per c.mm. with a polymorphic preponderance. The eosinophils were often absent and never rose above 130 per c.mm. At this time, at the age of 7 months, the plasma proteins were examined by paper electrophoresis. It was found that the gamma globulin was only just detectable, although she had received blood and plasma before the estimation. The isohaemagglutinins were also reduced. The parents had a normal plasma electrophoretic pattern. In view of her critical condition, it was decided to institute gamma globulin therapy without delay. She was given 0-5 g. of gamma globulin i.m. and 0-25 g. bi-weekly together with further blood transfusions. It was calculated that this would produce a level near the normal. This therapy had no apparent effect on her continuing diarrhoea and otitis media. Two weeks later a course of cortisone 5 mg. t.d.s. was given for three weeks, with novobiocin and streptomycin. Again the response was disappointing. She was given a short course of A.C.T.H. A week later she died at the age of 8 months. The immediate cause of death was a recrudescence of bronchopneumonia.