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Biomedical subjects

S Appan

Publications and source records attributed to S Appan.

3 recordsLinked to original sources

Monitoring treatment in congenital adrenal hyperplasia.

We report results of monitoring treatment in 41 patients with congenital adrenal hyperplasia controlled over 0.3-13.1 years using standard auxological techniques alone. Doses of glucocorticoid (15-25 mg/m2/day) and mineralocorticoid (0.15 mg/m2/day) replacement were determined initially using biochemical indices and thereafter adjusted according to surface area. Monitoring was solely directed at maintaining a 50th centile height velocity for chronological age. Of 41 patients, 32 were referred after the newborn period. Nearly half of these patients were either overtreated or undertreated before their referral. Of the nine treated from birth, all but one were in good control and only two have had a second hospital admission. Present height standard deviation scores (SDS) for chronological age range from -1.60 to -0.26. Height SDS for bone age were compared with midparental heights in 33 patients: 15 treated with early emphasis on growth had a height prognosis exceeding midparental values; patients who had experienced appreciable prior overtreatment or undertreatment fared less well. In the long term management of congenital adrenal hyperplasia correction of salt loss is of primary importance. Doses of glucocorticoid required in addition to mineralcorticoid replacement should be continuously assessed and adjusted to maintain a normal growth velocity. This is most conveniently achieved by standardising replacement doses on surface area.

Adrenal Hyperplasia, Congenital↗

Multiple thromboses in systemic lupus erythematosus.

A 5 year old girl with systemic lupus erythematosus developed extensive thromboses at multiple sites. Coagulation screen confirmed presence of the lupus anticoagulant. She recovered satisfactorily on high dose steroids without the use of anticoagulant treatment.

Anemia, Hemolytic, Autoimmune↗

Growth and growth hormone therapy in hypochondroplasia.

The growth of 84 patients with hypochondroplasia (56 male, 28 female) was studied. A wide spectrum of severity was found from quite severe short limbed dwarfism to short apparently normal prepubertal children who manifested disproportion only at puberty when growth failed. The onset of puberty was at the normal time but the pubertal growth spurt appeared not to materialize and it is this lack which resulted in severely compromised adult heights of 145-165 cm in boys and 133-151 cm in girls. Twenty (12 M, 8 F) hypochondroplastic children aged between 4.3 and 12.8 years were recruited to a study of the effects of biosynthetic growth hormone. All had normal growth hormone responses (greater than 15 mU/l) to a pharmacological test of growth hormone secretion. Biosynthetic growth hormone in doses between 12-32 mu/m2/week produced a significant acceleration in height velocity standard deviation score (SDS) for chronological age (CA) from a pretreatment mean of -1.66 (SD 1.36) to +1.62 (SD 1.52) (p less than 0.001). Significant increases were also observed in the height SDS for bone age (BA), sitting height (SH) SDS and subischial leg length (SILL) SDS. A longer period will be required to assess the effect of treatment on adult height prognosis.

Adolescent↗