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Biomedical subjects

G M Komrower

Publications and source records attributed to G M Komrower.

6 recordsLinked to original sources
PubMed1979

The Manchester regional screening programme: a 10-year exercise in patient and family care.

Up to the end of 1978 the Willink Biochemical Genetics Unit had screened 506821 babies for metabolic abnormalities over 10 years--98-99% of the children born in the region. Sixty-nine cases of phenylketonuria (PKU), 42 cases of histidinaemia, and six cases of homocystinuria were detected. As well as treating affected children, the staff of the unit have concentrated on providing full support for their families and maintaining good communications with parents, general practitioners, health visitors, and midwives. A clinic liaison sister has provided valuable support for health visitors and an important link between the unit and community services. A study of the costs of screening and treating cases of PKU for the year 1978 showed that this was cheaper, by pound 569000, than the costs of looking after patients with untreated PKU.

Child↗
PubMed1979

Management of maternal phenylketonuria: an emerging clinical problem.

The children of untreated phenylketonuric women have a significantly higher mortality and morbidity than average. The success of neonatal screening for phenylketonuria (PKU) means that an increasing number of healthy intelligent women with PKU are reaching childbearing age and will create a new therapeutic problem for physicians and obstetricians in the next decade. This unit managed six mothers with PKU and treated three of them with diets throughout four pregnancies. The treatment and outcome was assessed in relation to those in other reported cases. Although treatment was beneficial in the four pregnancies studied directly, in all the reported cases the outcome of treatment varied considerably and was not clearly related to any one factor such as onset of treatment or the type of PKU. It is therefore not possible to give confident advice about the management of such cases. Since the number of women at risk will reach a peak in 10 years' time there is an immediate need for a well-planned prospective study of this problem.

Cephalometry↗
PubMed1978

Management of congenital adrenal hyperplasia. Urinary steroid estimations--review of their value.

A retrospective study was made of 16 children with 21-hydroxylase-deficient congenital adrenal hyperplasia of the salt-losing variety, who were treated with fludrocortisone and prednisone and were in good health during the period under review. The height velocity of the children was subnormal, height achievement was poor, and their bone ages retarded. Urinary 17-oxosteroid and pregnanetriol excretion were used to monitor the therapy of the children and these data have been related to growth velocities. In spite of urinary steroid figures in excess of those published as desirable for monitoring therapy, the children failed to grow properly, probably as a result of glucocorticoid overdosage. Published urinary steroid criteria are considered too strict and in order to achieve them one would need to give unnecessarily high doses of steroid. Regular measurement of height velocity and skeletal maturation rate are better indicators of therapeutic control and should lead to more satisfactory growth and ultimate height.

17-Ketosteroids↗