Paediatric acquired immunodeficiency syndrome (PAIDS) in Trinidad and Tobago

The first twenty-one cases of Paediatric Acquired Immunodeficiency Syndrome (PAIDS) in Trinidad and Tobago were studied. An overwhelming majority of patients were of African descent. Most of the children presented within the first year of life, the average time between presentation and death was one and a half months, and the majority presented with either diarrhoea or pneumonia or failure to thrive, common conditions in the West Indies. Fever lasting longer than two weeks as well as hepatomegaly were clues which led to a definite diagnosis (AU)

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)

Neonatal manifestation of congenital rubella following an outbreak in Trinidad

An outbreak of rubella occurred in Trinidad during 1982 and continued into 1983 when 992 cases were reported in a 17-month period. Twenty infants were diagnosed as suffering from the congenital rubella syndrome based on clinical and laboratory findings. There was a positive history of maternal rubella in 17 (85 per cent), the majority of whom were under 25 years of age. There was equal sex distribution in the affected infants; half of whom showed growth retardation. Hepatosplenomegaly was the commonest finding (90 and 80 per cent). Other findings were thrombocytopenia (55 per cent) abnormal central nervous system signs (60 per cent), congenital heart disease (55 per cent), eye defects (50 per cent), X-ray changes in the lung (35 per cent) and bones (35 per cent), purpura (25 per cent) and anaemia (40 per cent). Two of the twenty neonates died (10 per cent mortality). Positive rubella antibody titre (HAI) ranged from 1:20 to 1:320. Rubella-specific IgM was positive in seven of 15 babies (46 per cent) (AU)

Two-year follow-up of hycanthone-treated schistosomiasis mansoni patients in St. Lucia

Of 433 schistosomiasis mansoni patients in St. Lucia who were treated with hycanthone ( 3mg/kg of body weight), 190 were seen 2 years after treatment and 143 of these had attended all follow-up examinations at 6 weeks, 6 months, 1 year, and 2 years. Viable eggs were not detected in 86 percent at 1 year nor in 76 percent at 2 years. The reinfection rate, as judged by a significant increase in egg excretion, was 15 percent and was related to the geographic area to which the patient returned. Extremely high total reduction in egg excreation (98 percent) was achieved through 1 year, and even with reinfections this fell only to 87 percent at 2 years. Liver and spleen enlargement was related to intensity of infection and responded to treatment in 92 percent and 83 percent of instances, respectively. Among patients with hepatosplenomegaly, those 15 years or older showed less clinical response than younger patients but were too few for statistical comparison. (AU)

A controlled study of morbidity of Schistosomiasis mansoni in St. Lucian children, based on quantitative egg excretion

A study of the morbidity of schistosomiasis mansoni was made in 138 schoolchildren (age range, 7-16 years) in St. Lucia. The study was based on quantitative egg excretion data collected over a 4-year period before the study and included a comparable uninfected control group. All examinations were carried out "blind". Infected subjects were divided into three levels of intensity of infection: heavy, 400 or more eggs/ml of feces; moderate, 100 to 300 eggs/ml; and light, 10 to 75 eggs/ml. The children were hospitalized for medical histories and physical and laboratory examinations. Gastrointestinal symptoms were present at relatively high levels in all groups, and were more frequent in the Schistosoma mansoni-infected subjects than in the controls. Anthropometric measurements showed no differences between the groups. Hepatomegaly and splenomegaly were significantly more frequent in the heavy-moderate infection group, and extension of the liver below the coastal margin was found to increase with intensity of infection; serum albumin level was inversely related. It was clear that in this St. Lucian study, infection with S. mansoni did not result in disease in most of the school-age subjects investigated; assessment of possible long-term(>4 years) effects was not made. This study provides a method for assessing morbidity of schistosomiasis based on quantitative egg excretion and demonstrates the relation of objective morbidity to intensity of infection.(AU)

The clinical features of haemoglobin SC disease in Jamaica

The clinical and haematological features of 90 Jamaican patients with haemoglobin SC disease are reviewed. Mean haemoglobin levels indicated mild anaemia although individual haemoglobin levels were often within the normal range. The clinical features were qualitatively similar to those of homozygous sickle cell disease(SS disease) although they were generlly less frequent and of lesser severity. Ocular pathology was an exception, occuring more frequently in SS disease even in age-sex-matched groups. There is some evidence that the higher haemoglobin level in SC disease may be aetiologically related to retinal vascular disease. (AU)

Immunological studies in Schistosoma mansoni infection in St. Lucia

An investigation was made of the immunological status of matched groups of patients with Schictosoma mansoni infection in St. Lucia, one group with hepatosplenic disease and the other with only intestinal disease. No impairment of humoral or cellular immunity was detected in either group. IgG and IgM levels were above normal range and were higher in the patients with intestinal disease, but only the difference between groups in IgG level was statistically significant. In their reaction to specific schistisome antigens and ability to develop delayed hypersensitivity, the groups were about equal. (AU)

Clinical features of homozygous SS disease in Jamaican children

The clinical features in the first 12 years of life of 100 patients with homozygous SS anaemia are described. The majority of patients presented before age 2 years. The earliest diagnosis was made at 3 months of age. The hand foot syndrome, painful crisis and megaloblastic change were early manifestations of disease, whereas leg ulceration did not occur until beyond the age of 6 years. No examples of haemolytic crises were seen. Growth did not appear to be retarded by Jamaican standards. Death occurred in 2 cases (AU)

Clinical trial of Hycanthone in Schistomiasis mansoni in St. Lucia

A clinical trial of a new antischistosomal drug, hycanthone methanesulfonate, was undertaken in 103 patients with schistosomiasis mansoni hospitalized in St. Lucia, West Indies. The drug was administered in a single intramuscular dose of 3mg per kg of body weight. The commonest side effect, vomiting, occurred in 48 patients (47 percent); 33 other patients complained of nausea, anorexia, or abdominal pain. In general, these symptoms ceased within 24 hours after treatment. Quantitative examination for Schistosoma mansoni eggs was carried out on three fecal specimens before treatment and at 6 weeks, 4 months, and 6 months after treatment. At 6 months after treatment, 26 of 94 patients (28 percent) were no longer excreting eggs, and 76 patients (81 percent) had sustained a greater than 90 percent reduction in egg-excretion level; for these 94 as a group, the total number of eggs excreted had been reduced by 97 percent. The efficacy of the drug did not appear to vary with severity of infection as measured by number of eggs excreted or by enlargement of the liver or spleen, or both. In this group of patients, two-thirds of whom were under 20 years of age, enlargement of the liver or spleen was frequently found to regress after treatment.(AU)

Symptoms and clinical and laboratory findings in 123 cases of Schistosomiasis mansoni in St. Lucia

The presenting symptomatology and clinical and laboratory findings in 123 patients, referred to a clinic for the treatment of schistosomiasis mansoni, are reported and discussed. Thirty-three patients (26.8 per cent) had no complaint when seen initially. The complaints of the others, in order of frequency, were abdominal pain, weakness, diarrhoea (with or without blood in faeces), giddiness, anorexia, dyspnoea and fever. The mean faecal egg load of patients with bloody diarrhoea was not significantly higher than that of patients with diarrhoea alone. Eighteen patients had clinical enlargement of the liver (14 or 11.4 percent) and of the spleen (4 or 3.2 percent) which could be ascribed to schistosomiasis. Hepatosplenomegaly in three other patients as ascribed to other conditions. The mean faecal egg concentration was significantly higher in patients with hepatosplenomegaly than in those with hepatomegaly alone. Ranges and mean values of haemoglobin and eosinophilia are given by age group. Alteration in plasma proteins was found in patients who had spleen and/or liver enlargement, the latter group having a reversed albumen/globulin ratio which, in the two instances in which electroporesis could be carried out, was found to be the result of a tremendous increase in the gamma globulin fraction.(Summary)

Effect of malnutrition on activity of two enzymes concerned with aminoacid metabolism in human liver

Aminoacid-activating enzymes and argininosuccinase were measured in liver-biopsy samples from twenty-two malnourished and recovering Jamaican infants. Aminoacid-activating-enzyme levels were increased initially and fell on recovery; argininosuccinase activity was low initially and rose on recovery. It is suggested that these changes are part of the adaptation which takes place in aminoacid metabolism in the malnourished state. (AU)

Sickle-cell disease in pregnancy

17 patients with sickle-cell disease were followed through pregnancy. 9 of them had sickle-cell anaemia, 7 had sickle-cell/haemoglobin-C disease, and one had an unusual form of sickle-cell thalassaemia. The maternal and foetal hazards are discussed, and seem to be considerably less serious than in most other reports. Complications were more frequent in the puerperium. 5 out of 7 cases of sickle-cell/haemoglobin-C disease had no complications at any time. The birth-weight of the infants in this series was very much below average. The incidence of sickle-cell disease amongst pregnant women in Jamaica seems to be very similar to the incidence in the general population (Summary)

Some disease associated with protein malnutrition, as seen in Jamaica

Three diseases which occur in Jamaica, akee poisoning, veno-occlusive disease, and kwashiorkor are described. It is thought that previous protein malnutrition is necessary for these disease to appear (AU)

Epidemic nephritis in South Trinidad

Over a period of 6 months a remarkable increase was seen in the incidence of acute nephritis among children in South Trinidad, and 263 cases were admitted to a general hospital. The patients were found to differ in their age, sex, and geographic distribution. The disease was associated with respiratory infection rather than skin infection, the form usually seen here, and the appearance of desquamation in many of the children suggested that the underlying organism was one capable of producing scarlet fever. The disease was mild. Only five children (1.9 percent) died, all from cardiac failure. Recovery appears so far to be complete in all but one of the survivors. (Summary)

Further clinical and investigative uses of liver biopsy: an analysis of five hundred and twenty-seven biopsies

Five hundred twenty-seven biopsies have been performed on 330 patients. The conditions studied included kwashiorkor, marasmus, veno-occlusive disease of the liver, vomiting sickness, diabetes mellitus, various neuropathies, nephrosis, sickle-cell anemia, and unexplained hepatomegaly, splenomegaly, jaundice, pyrexia, and anemia. A clinical outline has been given of veno-occlusive disease, kwashiorkor, marasmus, and the vomiting sickness of Jamaica. The tissue obtained at biopsy was examined histologically in all cases. In a number of cases chemical examination of the liver tissue was also performed. The chemical studies included nucleic acids and protein, water and fat content, and glycogen estimations. In them malnutrition group it is shown (a) that the degree of fatty infiltration is no indication of the severity or probable outcome of the disease, (b) that hepatic protein depletion is severe in these infants but that its degree cannot be quantitatively correlated with the clinical picture or prognosis, (c) that fatty infiltration does not apparently interfere with the ability of the liver to store glycogen. Occlusion of the smaller and medium-sized branches of the hepatic veins is responsible for the hepatomegaly and ascites found clinically in veno-occlusive disease. The prognosis of veno-occlusive disease is linked with the severity of the associated hepatocellular damage. Serial biopsies have made it possible to define more accurately the clinical natural history of this disease. In cases that recover the histological appearances of the liver return to normal. When the disease advances to the chronic stage, a progressive nonportal cirrhosis is found to develop in biopsy specimens. The histological changes in liver in diabetes are minimal and cannot be correlated with the response to treatment, clinical hepatomegaly, or alteration of liver-function tests. The hypoglycemia of vomiting sickness is associated with severe depletion of hepatic glycogen, which is rapidly restored by successful glucose therapy. There is a high incidence of fibrosis of the liver in patients with sickle-cell anemia, and a possible etiological relationship is suggested. Because of the frequently mixed nature of hepatic cirrhosis in the tropics and the variability of the clinical syndromes presented, liver biopsy is a useful and often essential tool for establishing a correct diagnosis. Its uses in unexplained hepatomegaly, jaundice, and splenomegaly have also been demonstrated. It is also pointed out that liver biopsy often makes it necessary to alter an apparently firmly established clinical diagnosis. The advantages of simultaneous histopathological and chemical examination of portions of the tissue obtained at biopsy have been stressed (SUMMARY)

Veno-occlusive disease of the liver and Indian childhood cirrhosis

Cirrhosis of the liver is common in children in parts of India, especially in West Bengal and Madras, and in the West Indian island of Jamaica, where it usually occurs as a late result of 'veno-occlusive disease of the liver'. The literature concerning veno-occlusive disease is reviewed, with especial reference to its possible relation to the ingestion of plant toxins of certain 'bush teas', particularly species of Crotalaria and Senecio. The clinical and pathological features of 15 cases of Indian childhood cirrhosis (I.C.C) seen in Calcutta, West Bengal, are presented, analysed and compared with accounts in the literature. A two-stage clinical classification is suggested: (1) stage of hapatomegaly, (2) stage of clinical cirrhosis. The socio-economic background of the present cases is compared with reports by previous workers. The clinical picture and morbid anatomy of veno-occlusive disease and I.C.C., as judged by the literature and the present cases, are compared and contrasted. While some cases are clinically similar, the absence in I.C.C. of the acute episode that occurs in some children with veno-occlusive disease is stressed. Hepatic venous occlusion was found in all six of the 15 children who were liver biopsied or who came to necropsy. This histological feature has been noted by some other investigators, although more workers have stressed the presence of parenchymal damage. It is noted that, in both experimental animals and in veterinary medicine, it has been shown that certain plant toxins may sometimes act on liver cells and sometimes on the hepatic veins. The possibility of a similarly varied response in children is suggested (Summary)