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Biomedical subjects

L S Taitz

Publications and source records attributed to L S Taitz.

13 recordsLinked to original sources
PubMed1992

Height and body proportions in child abuse.

Abused children are said to retain 'infantile body proportions'. The presenting height, sitting height, and leg length standard deviation scores of 91 victims have been calculated from data derived from a study of local inner city schoolchildren. In the study population 31/91 (34%) were significantly short and all but two of these had relatively shorter legs than backs, this limb disproportion being significant in 17. In 25 patients of the original group followed up after various social interventions there was a significant recovery of leg length. Measurement of height and proportionality, especially when compared with appropriate modern or local standards, is an important means of detecting and following up victims of child abuse.

Adolescent↗
PubMed1990

Medium-chain acyl-CoA dehydrogenase deficiency: a useful diagnosis five years after death.

We report a family in whom a fatal case of medium-chain acyl-CoA dehydrogenase (MCAD; EC 1.3.99.3) deficiency was diagnosed by enzymatic analysis of heart tissue that had been stored for five years. Three healthy siblings underwent subsequent investigation with the 3-phenylpropionic acid loading test. All siblings had been asymptomatic; however, one (age 2.5 years) excreted large amounts of 3-phenylpropionylglycine in response to the load and exhibited an organic aciduria consistent with the diagnosis of MCAD deficiency. The other two siblings did not demonstrate 3-phenylpropionylglycinuria after the loading test. This case underlines the importance of considering family history and using appropriate diagnostic tests in the recognition of hereditary metabolic disorders.

Acyl-CoA Dehydrogenase↗
PubMed1979

Biotin-responsive alopecia and developmental regression.

A 10-month-old boy presented with dermatitis and alopecia and became severely hypotonic. Screening for urinary organic acids revealed a large quantity of 3-hydroxyisovaleric acid and raised levels of beta-methylcrotonylglycine and 3-hydroxypropionate. Activities of propionyl CoA carboxylase, beta-methylcrotonyl CoA carboxylase, and pyruvate carboxylase in cultured fibroblasts were normal. Treatment with oral biotin resulted in a dramatic clinical improvement, which might therefore suggest a defect in biotin absorption or transport.

Alopecia↗
PubMed1977

Sodium intake and health in infancy.

Although the healthy infant can tolerate the range of sodium intakes normally present in the variety of diets offered in Britain without developing hypernatraemia or evidence of serious disease, there is enough evidence to suggest that care is required in the amount of sodium given to babies. The consequence of excessive sodium intake may be short term or long term. The most common short term complication is hypertonic dehydration. In the long term, the fear is that high intakes of sodium in early infancy may predispose to hypertension in later life, though this is as yet unproven.

Animals↗
PubMed1976

Cerebro-hepato-renal syndrome with parental consanguinity.

A case of cerebro-hepato-renal syndrome with some unusual features is reported. The neuropathological findings are described in detail. Electronmicroscopy showed astrocytes in the demyelinated areas of the brain to contain granules composed of laminated osmiophilic material. These structures could be abnormal mitochondria. The parental consanguinity in this case would further support an autosomal recessive mode of inheritance.

Astrocytes↗
PubMed1976

Triglyceride storage disease. A report of two affected children associated with neurological abnormalities.

Two children are described with congenital abnormalities (microcephaly, nystagmus, deafness, hepatomegaly) and the anomalous feature of triglyceride deposits in peripheral adipose tissue associated with severe malnutrition. Peripheral adipose tissue of one of these children displayed: (a) reduced sensitivity of adenyl cyclase to stimulation by noradrenaline (b) no response in tissue levels of cyclic AMP when stimulated by isoprenaline and (c) impaired release of glycerol following stimulation with isoprenaline. The other child, with similar clinical features, showed abnormal deposits of glycogen in the liver. It is postulated that a primary metabolic defect occurs in peripheral adipose tissue (and possibly at other sites such as the liver) that interferes with triglyceride (and glycogen) mobilization during prolonged malnutrition.

Abnormalities, Multiple↗