Long-term growth and development in children after home parental nutrition.

Growth and development after the cessation of prolonged parenteral nutrition (PN) has not been fully evaluated. Growth, body composition, and nutritional and developmental status were documented in nine children (five boys, four girls) 2 to 6 years old (mean 4.9 +/- 1.0 years) who had previously received long-term PN (mean 14.6 +/- 11.4 months). PN had been discontinued in all subjects for at least 6 months (mean 3.4 +/- 1.4 years); they were receiving oral feedings only. One subject had a significantly low height-for-age, and another had a low percent ideal body weight; five subjects had low total body fat. Serum vitamin A was low in six subjects. Seventy-two-hour fecal fat analysis was abnormal in two of eight subjects. Abnormal bone mineral density was present in four of nine subjects. Psychomotor development was normal in all nine subjects. Two had functional difficulties in swallowing. One or more abnormalities were present in all nine subjects. These findings suggest that children who require prolonged PN in early life are at risk for abnormalities in growth and nutritional status in later childhood; they require long-term dietary, growth, and nutritional monitoring.

New classification of peripheral retinal vascular changes in sickle cell disease.

The systemic complications of homozygous sickle cell disease (SS) are more severe than in sickle cell haemoglobin C (SC) disease, and yet visual loss due to proliferative retinopathy is more common in the latter. This anomaly is unexplained. It is believed that proliferative disease occurs in response to closure of the peripheral retinal vasculature, yet a systematic longitudinal survey of the peripheral retinal vascular bed has not been undertaken. In the Jamaica Sickle Cohort study all subjects are scheduled to receive annual ocular examination and fluorescein angiography. The results have now been analysed in subjects with SS and SC disease using a new classification system based on a comparison of the peripheral retinal vascular bed with that recorded in the cohort with normal haemoglobin (AA) genotype. The vascular patterns could be classified as qualitatively normal (type I) or abnormal (type II). An abnormal vascular pattern was identified more commonly with age, in a significantly larger proportion of subjects with SC than SS disease, and was associated with the development of proliferative disease. In order to establish the dynamics of change, the angiograms were analysed in the 18 subjects (24 eyes) who developed proliferative disease. It is shown that a qualitatively normal vascular pattern may be retained despite loss of capillary bed and posterior displacement of the vascular border. A border which is qualitatively abnormal does not revert to normal, and once abnormal, continuous evolution may occur before development of proliferative lesions. The peripheral border of the retinal vasculature was too peripheral to photographed in 13 of the 24 eyes before it becoming qualitatively abnormal. It is concluded that a normal border, if posterior, results from gradual modification of the capillary bed and indicates low risk of proliferative disease. A qualitatively abnormal vascular border occurs as a radical alteration of retinal perfusion in subjects in whom little modification of the vascular bed occurred before the event, and signals risk of proliferative disease. This classification system is useful in identifying the likelihood of threat to vision in young Jamaicans with sickle cell disease, and the higher frequency of proliferative retinopathy in SC can be explained by the higher prevalence of a qualitatively abnormal peripheral retinal vasculature.

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 fluorescein angiography. 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 haemoglobin 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.