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Biomedical subjects

R J West

Publications and source records attributed to R J West.

At least 73 records · Page 4Linked to original sources
PubMed1980

Long-term follow-up of children with familial hypercholesterolaemia treated with cholestyramine.

35 children with the heterozygous form of familial hypercholesterolaemia (FH) who were followed for up to 8 years after starting on cholestyramine treatment showed a progressive decrease in compliance with therapy with time, so that only 55% remained on treatment after 6 years and only 48% after 8 years. Long-term compliance was significantly better in those starting treatment before age 10. Plasma-cholesterol was lowered in all children taking cholestyramine: for the group the mean reduction in plasma-cholesterol ranged from 26 to 44% during the years of the study, on a mean cholestyramine dose of around 0.4 g/kg/day. Cholestyramine thus effectively lowers plasma-cholesterol in FH, but for long-term use compliance is a problem in many children.

Adolescent↗
PubMed1980

Radiographic studies of the ventricles in syringomyelia.

Radiographic investigations of 171 patients with "communicating' syringomyelia have been reviewed. Hydrocephalus was found in one-third of the cases and has occasionally progressed after operation on the posterior fossa, sometimes with accompanying clinical deterioration. The outlets of the fourth ventricle were usually abnormal; tonsillar descent, arachnoiditis and both together were seen. Arachnoiditis correlated strongly with a history of difficult birth. The foramen of Magendie was sometimes patent and sometimes blocked. There was no consistent level of occlusion corresponding to a persistent roof of the fourth ventricle. The cisterna magna was usually small or obliterated but some examples of large cisterns or subarachnoid pouches were found. Radiological demonstration of a communication from the fourth ventricle to the syrinx occurred in only seven patients by positive contrast material and not by air. It is suggested that a sizable communication is rare at the time when patients seek treatment.

Adult↗
PubMed1980

Familial insulin resistance with pineal hyperplasia: metabolic studies and effect of hypophysectomy.

Mendenhall's syndrome comprises insulin-resistant diabetes, pineal hyperplasia, and various somatic anomalies. A boy with this condition now aged 12 years is reported; a similarly affected sibling died aged 7.8 years. Hypophysectomy has been of short-term benefit, but the problems of insulin resistance persist. On the basis of monocyte-binding studies it seems likely that in this condition there is an inherited deficiency of insulin receptors.

Child↗
PubMed1979

Malnutrition in infants receiving cult diets: a form of child abuse.

Severe nutritional disorders, including kwashiorkor, marasmus, and rickets, were seen in four children and were due to parental food faddism, which should perhaps be regarded as a form of child abuse. All disorders were corrected with more normal diets and vitamin supplements. In view of the potentially serious consequences of restricted diets being fed to children, families at risk should be identified and acceptable nutritional advice given. When children are found to be suffering from undernutrition due to parental food faddism a court order will normally be a necessary step in providing adequate treatment and supervision.

Child Abuse↗
PubMed1978

The reasons for failure of endoscopic retrograde cholangio-pancreatography in patients with jaundice.

The results of 100 attempts at endoscopic retrograde cholangio-pancreatography (ERCP) performed in patients with jaundice have been reviewed. The examination provided a diagnosis in 75% of cases. The reason for failure to cannulate the correct duct was obstruction at the lower end of the common bile duct in 15 patients and distortion of the duodenum in seven. There was no cause for the failure in only two patients. Even with a perfect technique it will not always be possible to obtain an endoscopic cholangiogram. However, as the commonest cause of failure is obstruction, transhepatic cholangiography should usually succeed when ERCP fails.

Adult↗
PubMed1976

The complement abnormalities of lipodystrophy.

Investigation of the serum complement system in 25 patients with various forms of lipodystrophy showed no abnormality in three patients with total lipodystrophy; a single patient with limb lipodystrophy had evidence of activation of the classical complement pathway. However, of the 21 patients with partial lipodystrophy, 17 had low serum C3, with normal C4 and C2, concentrations, accompanied in 14 by a serum C3 splitting factor indistinguishable from nephritic factor, suggesting activation of the alternative pathway. These abnormalities occurred in 10 patients without clinically overt renal disease. Seven patients had overt nephritis; renal biopsies obtained in six showed mesangiocapillary (membranoproliferative) nephritis in all. Thus, the majority of patients with partial lipodystrophy have hypocomplementemia. Although nephritis may not invariably develop, the high rate of mesangiocapillary nephritis in these patients suggests that complement activation via the alternative pathway predisposes to the development of this form of glomerular disease.

Adolescent↗