The number of well-documented reports of the findings in primary pulmonary hypertension is increasing rapidly, but the ctiology remains obscure. A review of the published series reveals two possible clues. First, most of them show a significant female preponderance and, secondly, some suggest that primary pulmonary hypertension can be familial. Thus Dresdale et al. (1954) described three cases, mother and son and the mother's sister, all of whom died of right heart failure. Cardiac catheterization in the mother and son demonstrated severe pulmonary hypertension and showed no congenital cardiac defects, and in the case of the sister the diagnosis was based on clinical, electrocardiographic, and radiological findings: a brother of the mother died of undiagnosed heart disease at an early age. Clarke et al. (1927) in a paper on lesions of the pulmonary arteries described two sisters: one of them died in heart failure at the age of 52 years, and necropsy showed a dilated and hypertrophied right heart with no septal defects and marked atheroma of the pulmonary arteries; the other sister, aged 7 years, was cyanosed and dyspncic and showed clinical evidence of right ventricular hypertrophy. Lange (1948) described a family of 186 members of whom 82 were said to be cyanosed: of 42 members personally examined, 30 were cyanosed and 10 had an accentuated pulmonary second sound; 13 out of 21 examined radiologically had an enlarged pulmonary conus, and some had cardiographic evidence of right ventricular preponderance: none of these cases came to necropsy. Because of lack of post-mortem confirmation, this evidence, though suggesting that primary pulmonary hypertension can be familial, remains inconclusive. For this reason we report the findings in a brother and two sisters who died of primary pulmonary hypertension confirmed in each case by necropsy.Case Reports Case 1. The patient, a 22-year-old housewife, was admitted to hospital under the care of Mr. J. S. Hovell in the 36th week of her third pregnancy, because she had developed a cough and dyspncea. She had had diphtheria at the age of 6 years, but otherwise her health had been good and her previous pregnancies uneventful. On admission moist sounds were heard at both lung bases. Labour, four days later proceeded normally, lasting two hours, but immediately afterwards her condition deteriorated, and she was seen by one of us (A. W. B. E.) four hours later. She was then breathless and cyanosed; there was no aedema and the jugular venous pressure was not increased. The heart rate was 100 a minute with regular rhythm and the blood pressure was 120/90. On auscultation the lungs were clear; a presystolic murmur was heard at the apex and an early diastolic murmur below the pulmonary area. She was thought to have mitral stenosis and aortic incompetence with impending pulmonary cedema. In spite of treatment, she collapsed suddenly and died, early on the following day.Necropsy. The body was that of a well-built young woman. There were 300 ml. of fluid in each pleural cavity and a mod...
MethodsWet and stained films of centrifuged deposits from catheter specimens of the urines were examined and the urines cultured on MacConkey's medium and blood agar. Any coliform bacilli isolated were identified by biochemical tests, and their sensitivity to sulphanilamide, penicillin, and streptomycin was determined. The fermentation reactions using glucose, marnitol, lactose, sucrose, and salicin were employed. Other tests used were: the formation of indole, gas production at 44' C., utilization of citrate, Vosges Proskauer test (Barritt's modification), and the methyl red reaction. The coliform bacilli were identified by the criteria described in Topley and Wilson's Principles of Bacteriology and Immunity (third edition).Of the eighteen strains of Bact. aerogenes tested, six produced gas at 44o C. after 48 hours' incubation though not after 24 hours'. They were, however, classified as Bact. aerogenes. P. vulgaris was distinguished from P. morgani by the ability to swarm on blood agar. The paracolon bacilli included three anaerogenic strains, two of which were late lactose fermenters.Sulphanilamide sensitivity tests were carried out by the method of Harper and Cawston (1945). Penicillin and streptomycin sensitivities were deternined by inoculating one drop of an overnight broth culture of the organism under test into broth containing the drug in the appropriate concentration. These cultures were incubated overnight and examined for growtb next morning.
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