Peripheral retinal vasculature in normal Jamaican children

A prospective study of the peripheral retinal vasculature in a Jamaican cohort of subjects with sickle cell disease has been in progress over a period of 12 years using flourescein angiograpphy. Various vascular patterns were identified but their significance was unclear since no comparable records were available in subjects of a similar age with normal (AA) haemoglobin genotype. Fluorescein retinal angioscopy and angiography have been performed in 76 heamoglobin AA controls participating in the cohort study. The peripheral retinal capillary bed could be seen and photographed in a limited portion of the temporal peripheral fundus in a majority of this group, and there was considerable variation in the vascular pattern which could be characterised. These observations allow deviations from normal to be identified in the retinal vasculature in subjects with sickle cell disease (AU)

Electron microscopy of herpes simplex hepatitis with hepatocyte pulmonary embolization in kwashiorkor

We report a case of herpes simplex hepatitis in a child with edematous malnutrition. Electron microscopy showed virus in parenchymal cells. With pulmonary embolization of necrotic, infected hepatic cell fragments. Systemic dissemination of herpes simplex may be related both to the profound immunoincompetence associated with kwashiorkor and to a reduction in the circulating and fixed polyanions that normally inhibit viral attachment to cells.(AU)

Macular vasculature, visual acuity, and irreversibly sickled cells in homozygous sickle cell disease

Observations of visual acuity and the conjunctival, macular, and perimacular vascularity have been assessed in patients with homozygous sickle cell (SS) disease. There were 17 matched pairs, each consisting of one patient with a high count (>15 percent) and one with a low count (<5 percent) of irreversibly sickled cell (ISCs). The macular vascular bed was assessed by measurements of the foveal avascular zone (FAZ), perimacular zones, and counts of perimacular vascular abnormalities (perimacular counts). Small foveal avascular zones and high perimacular counts were commoner in younger than older patients and there was a significant inverse correlation between size of the FAZ and the perimacular count. These observations were compatible with the hypothesis that perimacular vessel anomalies represent the early vaso-occlusive phase which progresses to ischaemia and the formation and enlargement of avascular areas. Visual acuity was assessed by Snell's test type and by measuring contrast sensitvity. There was no obvious relationship between acuity measured by the 2 methods and no relationship between acuity and observations of mascular vascularity. High ISC counts were significantly related to abnormalities of conjunctival vasculature, but no relationship was noted with abnormalities of the macular vasculature or with visual acuity (AU)

The conjunctival sign in sickle cell anaemia: a relationship with irreversibly sickled cells

Patients with sickle cell disease were divided into 5 groups according to the extent of abnormal vessels in the conjunctival vasculature. There was a significant relationship between the degree of abnormality and the number of irreversibly sickled cells, suggesting that these cells must be related to the genesis of these vessel anomalies. Evidence is accumulating that irreversibly sickled cells are related to several pathological processes in sickle cell anaemia, and we believe the sign may be useful not only in suggesting the diagnosis but also in gauging the severity of the condition (AU)