Unusual movements of axial structures.
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Biomedical subjects
Publications and source records attributed to M Nathanson.
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Two cases, in children of distinct families, of a particular form of "dry syndrome", are described. This syndrom, which associates xerophthalmy, xerostomy and cutaneous dryness, is congenital and familial. He looks different from previously described diseases or syndroms which include one or several of these three components.
A case of chronic neurogenic hypernatremia with arhinencephaly has been studied. The clinical and biological picture of neurogenic hypernatremia is reviewed. Hormonal studies have been performed, to try to clear up the physiopathology of this syndrom.
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No immunodeficiency could be found in a case of generalized BCG infection. The therapeutic trials were unsuccessful, and the child died at the age of 19 months.
The authors report 4 cases in 2 different families of a syndrome characterized by nephrotic syndrome of early onset (during the first 2 years of life) and microcephaly. Such an association was previously reported in 5 cases. In 4 it was familial. The study of families suggests an autosomal recessive transmission. Microcephaly was associated with psychomotor retardation, sometimes dysmorphic facies and various neurologic abnormalities. The nephrotic syndrome was characterized by its early onset and prognostic severity. However, the renal histologic lesions were heterogeneous: either minimal glomerular changes with focal and segmental hyalinosis or mesangial sclerosis, or, so-called "microcystic dysplasia". This heterogeneity does not suggest a single genetically determined disorder.
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Care of patients with subarachnoid haemorrhage caused by ruptured cerebral artery aneurysm requires careful assessment of neurological function and prevention of rebleeding and ischaemia throughout the perioperative period. An understanding of the cerebral protection techniques used during periods of ischaemia or circulatory arrest will assist the provision of optimal conditions for successful surgical treatment of the aneurysm.
In a three and a half old girl, we have noticed the apparition: first, of on idiopathic pulmonary hemosiderosis; then, five months later, of a rheumatoid arthritis with LE cells and antinuclear antibodies of very difficult detection. The meaning of this articular involvement is discussed: rheumatoid arthritis with LE cells, or rheumatoid arthritis marking the beginning of a systemic lupud erythematosus. The literature concerning the association of an hemosiderosis and a connectivite is reviewed.