The mauriac syndrome

An insulin-dependent diabetic was diagnosed at the age of 7 years. After two years of satisfactory control she began to have several bouts of hospitalization with hyperglycaemic ketoacidosis, and developed tender hepatomegaly, which persisted to age 11 years. With restabilisation of her diabetes, the liver regressed and she continued to maintain good health for another 1« years when she died suddenly while asleep. Post-mortem examination by the coroner revealed ascites in the abdomen, hepatomegaly and fatty metamorphosis of the liver. Her diabetes control required up to 2.3 i.u. insulin per kg body weight per day plus a 1,900 calorie diet. Her growth was well below the tenth percentile, weight for height (Harvard charts). This clinical picture of high insulin dosage, hepatomegaly, unstable diabetes and growth failure approximates to the Mauriac syndrome (AU)

Pulmonary stenosis with intact ventricular septum, 1968 - 1978

Twelve cases of pulmonary stenosis with intact ventricular septum had correction of their defects using cardiopulmonary bypass. One patient of acute cardiac failure in the early postoperative period. There were no late deaths. The 11 late survivors are well. The majority (10 patients) were in NYHA grade 1 and there has been a significant reduction in right ventricular size as determined electrocardiographically (AU)

The development of cardiac catheterization at the University Hospital

Cardiac catheterisation at the University Hospital from 1967 to 1978 has been described, with an outline of its development over the years, the techniques used, the results obtained and the complications encountered. Six hundred and six patients were subjected to cardiac catheterisation. Morbidity was low and there was one death due to malignant hyperthermia complicating general anaesthesia (AU)

Acute rheumatic fever in Jamaican children - abstract

This presentation summarizes experience with rheumatic fever as seen at the Children's Hospital over a 4-year period. There were 115 admissions for rheumatic fever accounting for 1 percent of medical admissions over this period. All cases except a small minority were from the lower socio-economic group of the population. There was a wide age ditribution but 56 percent of the first attacks were in the age group 7-10 years. The most frequent presenting features were polyarthritis in 56 percent and carditis 51 percent. Chorea was present in 9.2 percent of the attacks. There was laboratory evidence of previous streptococcal infection in 90 percent of cases with carditis there was mitral valve involvement always present with heart failure in 50 percent of cases. The aortic valve was involved in 27 percent of attacks. Up to the time of follow-up, 6 percent had died from heart failure and there was evidence of residual valve lesions in 27 percent. There was a 19 percent recurrence rate which was usually associated with faulty prophyaxis. These figures illustrate that rheumatic fever is a serious medical problems in Jamaica with considerable mortality and morbidity (AU)

Palliative surgery for congenital heart disease in small children - abstract

Ten small children with severe congenital heart disease have had palliative surgical procedures performed with good clinical results and without mortality at the University Hospital of the West Indies. Seven of these were cases of Tetralogy of Fallot and 3 were cases of large ventricular septal defects. It is planned to do a curative surgical operation for all these patients when they are old enough to permit the safe use of cardiopulmonary bypass. The 7 cases of Tetralogy of Fallot were aged from 11 months to 5 years. A Blalock-Taussig shunt was performed in the first 3 cases, and a shunt between the ascending aorta and right pulmonary atery was carried out in the last 4 cases. The indications for surgery were: (a) severe cyanosis with gross exercise intolerance (5 cases), and (b) severe paroxysmal cyanotic attacks not responding to "conservative" measures (2 ases). One patient, aged 4 years, had not been able to stand or walk. Another had a history of hypoxic convulsions. There were two post-operative complications - post-intubation laryngeal swelling, and left iliac vein thrombosis secondary to a previous saphenous vein exposure in the groin for intravenous infusion. The follow-up period ranged from 3 months to 31/2 years. All the patients did well. Their effort tolerance was markedly improved and cyanosis was absent or nearly so. The 3 cases of venticular septal defects were aged 3 months, 11 months, and 1 year. They had banding of the pulmonary artery performed for severe congestive cardiac failure which did not respond to intensive conservative therapy with diuretics and digoxin. In one case the diagnosis was confirmed pre-operatively by cardiac catherization which demonstrated a large left to right shunt with a pulmonary: systemic flow ratio of 7:1. In the other 2 cases the diagnosis was made on clinical radiological and electrocardiographic evidence. At surgery, a patient ductus arteriosus was excluded and the presence of a typical thrill over the anterior surface of the right ventricle and a large pulmonary artery were demonstrated. There were no post-operative complications directly attributable to the surgery. All 3 patients are still attending regularly for follow-up which ranged from 3 to 7 months in January, 1972. Two have shown marked improvement but the third is only moderately improved (AU)

Pre and post-operative findings in 18 cases of congenital heart disease treated by open heart surgery and cardiopulmonary bypass - abstract

The main purpose of this paper is to review the results of open heart surgery at the University Hospital of the West Indies in the past three years. 18 patients with congenital heart disease have had corrective surgery performed. The mean age at operation was 14.8 years ranging from 6.8 to 28 years. Seven of the patients were under 12 years and all but two 18 years or under at the same time of surgery: Five patients have had total correction of the Tetralogy of Fallot. Four of them, including a girls of 12 1/2 years who had pulmonary embolus one month after operation, are well at follow-up. One patient, a girl of 11 years, who was operated on in June, 1970, had evidence of right ventricular failure for six months, but when last seen was well although signs of tricuspid incompetence persist. Four patients with atrial septal defect, including one with an ostium primum defect and mitral incompetence, are well at follow-up with no evidence of heart disease. Four patients with ventricular septal defect have had their defect repaired. Two of these had significant pulmonary hypertension pre-operatively and one of these developed a leak post-operatively and has required a second operation. Four cases of pulmonary stenosis (two valvular, one infundibular and one both valvular and infundibular) have been corrected and all these patients are now well and active. A young woman of 27 who was thought on cardiac catheterization to have pulmonary stenosis, was found at operation to have a double outlet right ventricle and infundibular stenosis. Right and left outflow tract reconstruction carried out and although she had a stormy post-operative course was well when last seen. Pre- and post-operative electrocardiographic and radiological data will also be presented (AU)

Acute veno-occlusive disease of the liver: fine structure in Jamaican children

Electron microscopic observations in humans veno-occlusive disease (VOD) of the liver are presented for the first time . Marked endothelial damage was seen in the sinusoids and subterminal and terminal hepatic veins, accompanied by extravasation of erthrocytes into Disse's space and naked hepatocytes showing a reduction in microvilli. Endothelial damage was seen in all zones of the liver. At the level of terminal hepatic veins, the closed structure of these vessels, absence of fenestrations, existence of a basement membrane, and the presence of collagen and connective tissue cells in the wall provide resistance to erthrocytes and cellular debris tracking up Disse's space. These factors tend to result in narrowing of the lumen where the sinusoid enters the vein. Parenchymal cell alterations, at the time of the biopsies, included dilation of bile canaliculi, abundant glycogen, and nuclear invaginations.(AU)

The 1964 poliomyelitis outbreak in Jamaica

The cases were almost entirely in the Kingston Metropolitan area. Of 51 cases seen at the Children's Hospital, Kingston, 90 percent were below 4 years of age, 78 percent had no immunization, 84.3 percent were paralytic cases, respiratory involvement was present in 13.7 percent, 3.9 percent were of the bulbar type, poliovirus type I was isolated in 37.2 percent of cases (AU)