Giant Congenital Juvenile Xanthogranuloma

Abstract: Congenital giant juvenile xanthogranuloma is an uncommon lesion that apparently has benign biologic behavior in most reported patients. In one of our three patients it was necessary to perform histochemical reactions with CD 1 (-), MAC 387, and electron microscopy to rule out Langerhans cell histiocytosis. The other patients had a typical histologlc picture, with foamy histiocytes and Touton cells in association wtth lymphocytes and eosinophils. Comments about treatment are also made.     

Panniculitis with pseudomonas septicaemia in AIDS

A 27 year old female with acquired immunodeficiency syndrome (AIDS) developed tender erythematous nodules on her thighs. A skin biopsy of the lesions showed septal panniculitis and venulitis and Pseudomonas species was cultured from both the urine and the skin biopsy tissue. The lesions resolved on antibiotic therapy. Panniculitic nodules may represent an early stage of ecthyma gangrenosum which is a well recognised cutaneous complication of pseudomonas septicaemia.     

Neurobehaviour rounds and interdisciplinary education in neurology and psychiatry

Increasing emphasis on interdisciplinary medical treatment and education suggests that something valuable has arisen from medical specialization beyond the further development of specialty knowledge: an integration of specialty knowledge that compliments and extends the integrating aspects of the primary care approach to medicine. Several educational models have been described which serve this function. In this paper the authors describe interdisciplinary clinical teaching, and research team linking neurology, neuroradiology, psychiatry and neuropsychology. The team provides neurobehavioural evaluations and sponsors monthly Neurobehaviour Rounds, an interdisciplinary patient conference that is the main formal teaching vehicle for the programme. After the model had been in place for 1 year, eight of nine neurology residents had Residency In-Service Training Examination scores in behavioural neurology that exceeded their overall average scores. This suggests that encouraging neurology residents to see patients through the eyes of different specialists may have contributed to improvement in their performance on a test of interdisciplinary knowledge. A neurobehavioural programme anchored to a formal neurobehaviour conference may encourage interdisciplinary learning within the related disciplines of neurology, neuropsychology and psychiatry.     

Influence of contractile agonists on the mechanism of endothelium-dependent relaxation in rat isolated mesenteric artery.

This study demonstrates directly that the relative contribution of nitric oxide (NO) and an NO synthase-independent repolarization to acetylcholine-evoked relaxation in rat isolated mesenteric resistance arteries is determined by the processes which mediate pre-contraction. Noradrenaline-induced contractions were reversed by acetylcholine via both NO and NO synthase-independent smooth muscle repolarization. In contrast, reversal of contractions to the thromboxane-mimetic, U46619, by acetylcholine was entirely mediated by the actions of NO, independently of a change in membrane potential.     

Change in Cellular Phenotype from Lymphoid to Erythroid in a Case of ALL

Acute leukaemia was diagnosed in a 3-month-old boy. Initial blood leucocyte count was 97 × 10⁹/1, 92% of the cells were morphologically small lymphoblasts which according to surface marker analysis were of the common ALL (non-T non-B) type. Remission was achieved with initial treatment. During relapse 5 months later a morphologically different leukaemic cell appeared in the bone marrow, blood and liquor. This cell type which dominated during the terminal stage of the disease was larger and had an abundant bosophilic cytoplasm which contained PAS-positive granulae. Sudan, myeloperoxidase and benzidine staining gave negative results. Neither T nor B lymphocyte markers were seen but a strong surface expression of glycophorin A was found by indirect immunofluorescence with specific rabbit anti-glycophorin A antiserum. The cells showed a unique surface glycoprotein pattern. Bone marrow karyotype analysis gave in about 80%: 47,XY, + 8,t(4:11) indicating that this blast cell represented a malignant clone. As glycophorin A is expressed exclusively on erythroid cells and their precursors, this finding indicates a change from a ‘lymphoid’ to an erythroid phenotype of the leukaemic cells during the course of the disease.     

Reliability and clinical validity of the children''s fear survey schedule

This paper traces the early development of the Children's Fear Survey Schedule (CFSS) and reports initial reliability and validity studies. The CFSS is a simple self-report instrument to be used for children between the ages of 4 and 12 years. It has been designed to identify specific fear stimuli and to measure the intensity of each stimulus. Applications are twofold: clinical and research. Three hypotheses were tested: test-retest reliability, clinical validity, sex differences on total fear scores. Test-retest reliability of 0.85 was obtained. Clinical validity was established. Females were found to report higher fear scores than males. The authors call for more research on the CFSS.