Wiskott-Aldrich Syndrome in a Jamaican

A 3-week-old boy presented with severe thrombocytopenia and recurrent infections. He was subsequently found to have Wiskott-Aldrich Syndrome, a rare X-linked disorder. Splenectomy was performed and he died of post-splenectomy sepsis despite prophylatic antibiotic therapy. This is the first recorded case in the English-speaking Caribbean.

Wiskott-Aldrich Syndrome in a Jamaican

A 3-week-old boy presented with severe thrombocytopenia and recurrent infections. He was subsequently found to have Wiskott-Aldrich Syndrome, a rare X-linked disorder. Splenectomy was performed and he died of post-splenectomy sepsis despite prophylatic antibiotic therapy. This is the first recorded case in the English-speaking Caribbean. (AU)

Role of splenectomy in homozygous sickle cell disease in childhood

60 Jamaican children with homozygous sickle cell (SS) disease underwent splenectomy, 14 for prophylaxis against recurrent acute splenic sequestration and 46 for treatment of sustained hypersplenism. Age at operation varied from 9 months to 27 years (median 6 years), with a total of 369 years of patient-observation. None of the 3 patients who died, at ages 2«, 6«, and 21 years, had received prophylaxis against infection. Overwhelming sepsis was possible but not confirmed in the first two deaths which occurred 11 months and 2« years after operation; the third died from chronic renal failure 11 years after splenectomy. After operation, there were no confirmed cases of pneumococcal septicaemia or meningitis, and the commonest clinical event was the acute chest syndrome. (Summary)

Heart size and chest shape in homozygous sickle cell disease

The hoop-shaped chest is a well-recognised but unexplained feature of the abnormal anthropometry of SS disease, developing in children by the age of 4-6 years. The hypothesis that cardiomegaly may influence abnormal chest growth has been tested by examining the relationship between cardiac volume and the antero-posterior/lateral chest diameter ratio in children aged 6-8 years. The finding of a significant positive correlation supports this hypothesis (AU)

The role of splenectomy in homozygous sickle cell disease in childhood - abstract

The persistence of splenomegaly in children with homozygous sickle cell (SS) disease can pose difficult management problems for the clinician. In the young child the prognosis of acute spenic sequestration (ASS) is worse if superimposed on hypersplenism and in the older child the development of chronic hypersplenism may have a wide range of detrimental effects on the child's health. Elective splenectomy abolishes the risk of further acute, or chronic, sequestration but the increased risk of fulminant sepsis following splenectomy and the known propensity to spontaneous atrophy counsels for conservative mangement. To understand better the role of splenectomy in SS disease the outcome of elective splenectomy in 60 Jamaican children is reviewed. The age at time of operation ranged from 9 months to 17 years, with a mean of 6 years and the period of follow up ranged from 1 month to 19 years. The principal indications were recurrent ASS under the age of 2 years and persistent hypersplenism in those over this age. There was no mortality or significant morbidity during the operative period. Two deaths occurred, one from pneumonia 11 months post operatively and one from unknown causes 30 months post operatively. The haematological response to splenectomy was striking, with significant sustained increases in haemoglobin, platelets and red cell survival times, and fall in reticulocyte count. In the last five years prophylaxis with penicillin and/or pneumococcal and Haemophilus influenzae vaccine has been given to most patients and may have improved the prognosis, but in 54 patients splenectomy precede this period. Elective splenectomy with modern surgical and anaesthetic techniques carries minimal morbidity and with careful patient selection (two episodes of ASS, or sustained hypersplenism) the procedure may confer considerable benefit on selected children with SS disease (AU)