The painful crises of sickle cell disease - is Jamaican experience relevant?

Bone marrow necrosis, is the most common cause of the painful crises in the sickle cell disease, is a recurrent cause of morbidity. In Jamaica, these events increase in incidence in late teenage, are most common in young adults and decrease progressively in frequency and severity after the age of 30 years. Precipitating events include cold exposure, exertion, infections, and stress but skin cooling is the commonest factor in Jamaica. Risk factors include pregnancy, especially the last trimester and immediate post partum period and a high haemoglobin level. The MRC Laboratories [Jamaica] undertakes the management of over 5000 patients islandwide and treats 4-12 painful crises daily. Crises admitted to the day care centre are deemed of sufficient severity to require narcotic analgesia and patients are given rehydration and their response to analgesia monitored during the day. After assessment at 4pm. they are given the option of hospital admission or discharge home with similar analgesia in oral form. Over 90 percent elect to return home. It is estimated that the South East London Health Authority spent 1.9 million pounds on the inpatient management of the painful crises last year, four times the entire budget of the MRC Laboratories [Jamaica] which supervises the outpatient clinical care and research programmes among a much larger population. It is proposed that the Jamaican model of outpatient care for the painful crises may provide a more acceptable and less socially disruptive therapeutic approach and would free resources which could be used to improve other aspects of patient care in sickle cell disease. Its applicability should be explored in the United Kingdom.[AU]

Necrotizing haemorrhagic colitis caused by resistant shigella flexneri. Report of a case

A case of fatal infection with shigella flexneri is reported. The 19-year-old male patient who presented with fulminating haemorrhagic colitis died nine days after the onset of symptoms. The infecting strain of shigella flexneri was resistant to multiple antimicrobial agents, including amoxycillin, co-trimoxazole and chloramphenicol (AU)

The clinical features of sickle cell disease

Evidence from structural studies of DNA suggest that the sickle cell mutation has arisen on at least three separate occasions in Africa and as a fourth independent mutation in the Eastern Province of Saudi Arabia or India. The pathophysiology of sickle cell disease is essentially similar in these different areas although the frequency and severity of complications may vary between areas. Generally, the chronic haemolysis and resulting anaemia is well tolerated, although serious morbidity and occasionally mortality may be associated with the aplastic crisis or cholelithiasis. Exacerbation of anaemia below steady state levels occurs with chronic glomerular damage and renal failure, especially in older patients. Most of the morbidity of the disease arises from bone marrow necrosis in the painful crisis or from vaso-occlusive manifestation. Changes in the splenic circulation results in life-threatening episodes of acute splenic sequestration, the chronic morbidity of hypersplenism, and splenic dysfunction renders children prone to pneumococcal septicaemia. Chronic organ damage contributes to chronic leg ulceration in adolescence and progressive renal,pulmonary,and occasionally cardiovascular impairment in later life. The clinical spectrum of homozygous sickle cell disease varies widely between patients. Factors contributing to this variability include alpha-thalassaemia,persistence of high HbF levels, haematology, social circumstances, and geographical and climatic variation. Many of the causes of mortality may be prevented or more effectively treated,leading to increased survival and an increased quality of life in affected subjects (AU)

Leg ulceration in sickle cell anaemia

In Jamaica, leg ulcers are a major manifestation of sickle cell disease. The chronicity of the lesions is such that education and consequent work potential are seriously affected. Although these lesions rarely cause mortality, they are a major determinant of the morbidity of the disease. (AU)

Haemoglobin SC disease in Jamaica

A skeletal survey was carried out on 84 cases of haemoglobin SC disease. The radiological features noted were increased translucency of bone, abnormal trabecular pattern, medullary expansion, wide vascular channels in phalanges, and radiological evidence of infarction, both medullary and cortical. The frequency of bone lesions thus demonstrated increased with age. The most characteristic lesions in this region were those of vascular necrosis; contributing factors such as the high haemoglobin level characteristic of the condition are discussed. An interesting feature was the lack of correlation betweeen clinical severity and degree of radiological change. (AU)

Sickle cell beta thalassaemia in Jamaica

An analysis of the radiological appearances in a skeletal survey of 48 patients in Jamaica with sickle cell beta thalassaemia is presented. The appearances are similar to those in homozygous sickle cell disease and SC disease and a confident distinction between the conditions on radiological grounds alone is likely. (AU)

Central rupture of the liver with massive liver-cell necrosis: A case report and review of the literature

A case of massive central rupture of the liver in which the patient became jaundiced is described. Autopsy revealed, in addition to the intrahepatic haematoma, large areas of infarction in the liver, some of which had become infected and had given rise to septic embolization to various organs. There was deranged liver function which is ascribed to the structural changes found in the liver. The occurrence of hepatocellular jaundice with this type of liver injury has not, to our knowledge, been previously recorded. It is suggested that immediate investigation and treatment are essential if the patient is to survive.(AU)

Microscopic and enzyme histochemical studies on the liver and kidney of rats treated with fulvine, the toxic alkaloidal constituent of crotalaria fulva

Histochemical changes in the liver of rats, treated with the pyrrolizidine alkaloidal constituent of Crotalaria fulva, indicated primarily damage of the hepatic parenchymal cells. The observed differences in activity of enzymes suggested functional inpairment of the cell membrane, mitochondria, endoplasmatic reticulum, and lysosomes in the liver cells. In large doses, fulvine produced a hyaline droplet degeneration in the epithelium of the proximal convoluted tubules of the kidney. Stability of the mitochondrial and changes in activity of the lysosomal enzymes would imply a functional adaptation, rather than primary damage of the epithelium.(AU)

Subclavian artery aneurysm and idiopathic cystic medionecrosis

The clinical, radiological, and pathological findings in case of localized aneurysm of the left subclavian artery due to cystic medionecrosis are presented. The patient was a 22-year-old Negress with no stigmata of Marfan's syndrome. The aneurysm was treated successfully by resection and insertion of a Dacron graft. The pathogenesis of the aneurysm is briefly discussed. A search of the published material has failed to reveal any such case previously reported (AU)