Malignant fibrous histiocytoma of sternum: an unusual cause of pyrexia of undetermined origin

Malignant fibrous histiocytoma of the bone is a very rare tumour mainly affecting long bones, and the most common presenting symptom is local pain. A case of malignant fibrous histiocytoma presenting with pyrexia of undetermined origin and arising in the sternum, a localization not previously described, is reported. The patient died with septic shock after multi-drug chemotherapy including high dose methotrexate treatment with citrovorum factor rescue.     

Bone mineral measurement in the newborn infant

In order to achieve parameters to evaluate mineralization of premature infants, bone mineral content at the midshaft of the radius was measured in 173 normal newborns. Data were correlated with the following factors: gestational age, postnatal age, sex and weight at birth. In spite of the wide range of variation of individual values, there was a statistically significant correlation between gestational age, sex and BMC.     

Unusual complication of soft tissue calcifications in chronic renal disease: The articular erosions

A rare complication, articular bone erosions adjacent to peri-articular calcifications, was observed in patients undergoing chronic haemodialysis. Three cases are described and the discussion describes the pathogenesis of the lesion, which, as far as is known by the authors, has not yet been reported in the literature.     

Renal function during calcitriol therapy in chronic renal failure

Some authors have reported that vitamin D treatment worsens renal function. We studied 10 patients with stable renal function, who were given calcitriol (0.5 micrograms/day) for a 4-month period. Creatinine and inulin clearance were performed at the beginning and at the end of the treatment. Although serum creatinine increased and creatinine clearance decreased, inulin clearance did not show significant variations. Furthermore, serum creatinine fell to the baseline value within 60 days after discontinuation of vitamin D therapy. The increased serum creatinine may be explained by an augmented release from muscular tissue, probably due to the improvement of uremic myopathy induced by calcitriol.     

Inappropriate growth-hormone (GH) response to thyrotropin-releasing hormone (TRH) occurs infrequently in well-regulated diabetes mellitus

Summary We randomly administered thyrotropin-releasing hormone (200 μg, as an i.v. bolus) or control saline (in isovolumic amount) to 30 male diabetic subjects (23 IDDM, 7 NIDDM) in fair metabolic control (HbA₁ 9.7±0.3%, ) and to 12 healthy male controls on two different mornings. While GH in the basal state was similar in IDDM, NIDDM and normal subjects, TRH administration evoked a significant GH release only in a single IDDM individual. The only GH-responder to TRH was a newly-diagnosed (two weeks) IDDM patient, still with a high glycated hemoglobin level (HbA₁ 11.1%), despite normal plasma glucose levels. Saline infusion did not affect GH concentrations either in normals or in diabetics. Exaggerated GH responses to TRH are uncommon in diabetic patients in good metabolic conditions. This study was performed in the context of theRicerca Finalizzata della Regione Toscana, and supported in part by a grant (87.00381.56) from the Italian National Research Council (CNR) and by a grant from theMinistero della Pubblica Istruzione (Ricerca Scientifica 1987).     

A new locus for postaxial polydactyly type A/B on chromosome 7q21-q34

Postaxial polydactyly (PAP) is the occurrence of one or more extra ulnar or fibular digits or parts of it. In PAP-A, the extra digit is fully developed and articulates with the fifth or an additional metacarpal/metatarsal, while it is rudimentary in PAP-B. Isolated PAP usually segregates as an autosomal dominant trait, with variable expression. Three loci are known for PAP in humans. PAPA1 (including PAP-A/B in one patient) on 7p13 caused by mutations in the GLI3 gene, PAPA2 on 13q21-q32 in a Turkish kindred with PAP-A only, and a third one (PAPA3) in a Chinese family with PAP-A/B on 19p13.1-13.2. We identified a fourth locus in a large Dutch six-generation family with 31 individuals including 11 affecteds. Their phenotype varied from either PAP-A, or PAP-B to PAP-A/B with or without the co-occurence of partial cutaneous syndactyly. We performed a whole-genome search and found linkage between PAP and markers on chromosome 7q. The highest LOD score was 3.34 obtained at D7S1799 and D7S500 with multipoint analysis.