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Biomedical subjects

J P Shield

Publications and source records attributed to J P Shield.

42 records · Page 3Linked to original sources
PubMed1992

Immunodeficiency presenting as hypergammaglobulinaemia with IgG2 subclass deficiency.

Recurrent bacterial infections, lymphadenopathy, and failure to thrive are unlikely to be attributed to immune deficiency if they occur in the presence of hypergammaglobulinaemia, and other explanations will usually be sought. We describe eight patients who presented with all these features in infancy or early childhood. Deficiencies of immunoglobulin and antibody production were initially discounted, and the children were referred for investigation of possible lymphoma, autoimmune disease, or chronic viral infection. The patients were later referred to us for more detailed immunological investigation, which revealed low levels of IgG2 and poor specific antibody production to common pathogens. Treatment with intravenous immunoglobulin resulted in resolution of signs and symptoms in all patients. Thus we have shown that hypergammaglobulinaemia does not preclude the presence of immunoglobulin/antibody deficiency. We suggest that investigation of children with high levels of IgG and features of immunodeficiency should include IgG subclass analysis.

Adolescent↗
PubMed1992

Lethal congenital erythroderma: a newly recognised genetic disorder.

We report 4 patients and their extended families comprising 17 cases, all of whom had congenital exfoliative erythroderma resistant to treatment, associated with failure to thrive and hypoalbuminaemia. All died in the first year of life. This condition appears to be inherited in an autosomal recessive manner and the underlying defect remains unknown.

Consanguinity↗
PubMed1991

Erythroderma, palmoplantar keratoderma and profound failure to thrive in an infant.

The case is reported of a female infant, who at the age of 3 months developed severe erythroderma, marked hyperkeratosis of the palms and soles and subsequently extreme growth failure and intermittent diarrhoea. Her course was complicated by life-threatening infections but detailed investigation revealed no recognized underlying metabolic or immune abnormality.

Dermatitis, Exfoliative↗
PubMedDate not supplied

Microalbuminuria in diabetic adolescents and children--feasibility phase of a national cross-sectional study. MIDAC Research Group.

Our objective was to assess the feasibility of examining the prevalence of microalbuminuria nationally and to validate the effectiveness of the collection and central analysis of biological specimens. In a centrally coordinated, cross-sectional, multicenter pilot study in pediatric, diabetes outpatients clinics in UK and the Republic of Ireland, blood and urine samples were collected from patients aged between 10 and 20 years, with a duration of insulin-dependent diabetes greater than 1 year attending diabetes outpatient clinics, between April 1996 and February 1997. These samples were analyzed at a central reference laboratory for HbA1C using high performance liquid chromatography (HPLC) and for urinary albumin and creatinine concentrations using spectrophotometry. Clinical data were collected locally and sent to the coordinator for analysis. A total of 419 patients were recruited to the study from ten centers of whom 308 (74%) correctly provided three timed overnight urine samples; 13 patients (4.2%) were microalbuminuric (AER > or =20 microg min(-1) in at least two of three urine samples), 1 (0.3%) was proteinuric (AER > or = 200 microg min(-1) in at least two of three urine samples). All patients found to have microalbuminuria and proteinuria were pubertal or post-pubertal. This study demonstrates that it is possible to coordinate a network of centers in the United Kingdom and the Republic of Ireland for the purposes of diabetes research in young adults and children. The level of abnormal albumin excretion of 4.5% is less than previously described in such age groups in other countries.

Adolescent↗