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PubMed · 334651

[Progress in flexor-tendon surgery].

E Scharizer
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Abstract

The treatment of fresh injuries with the modified technique of primary suture followed by a dressing in the relaxed position of the suture together with the parallel progress in transplantation have brought a new stimulus to flexor tendon surgery. The possibilities of selecting the best approach in every case have become greater and have been extended to include the field of micro-surgery. Also, technical progress has become more cautious. Each intensive specialization, which must necessarily be limited in its application to only a few centres, obliges all surgeons however, to master and apply the theoretical principles of these advances.

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ContractureFinger InjuriesHumansPostoperative ComplicationsSuture TechniquesTendon InjuriesThumbTime FactorsEnglish AbstractJournal Article

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E Scharizer. 1977-09-29. [Progress in flexor-tendon surgery].. https://pubmed.ncbi.nlm.nih.gov/334651/

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Restrictive dermopathy. Report of two affected siblings and a review of the literature.

BACKGROUND: Restrictive dermopathy is a lethal genetic disorder consisting of abnormally tight skin, generalized joint contractures, distinctive facies, and pulmonary hypoplasia. Autosomal recessive inheritance has been suggested based on multiply affected siblings and some reports of parental consanguinity. This article describes two siblings with the restrictive dermopathy syndrome and reviews previously reported cases. OBSERVATIONS: Eight other cases have been reported in the literature as restrictive dermopathy. These cases have shared striking similarities in their clinical histories and phenotypes. The skin in these infants has been described as rigid and tense, with skin biopsy specimens showing a thick epidermis, thin dermis, abnormally arranged collagen bundles, and poorly developed appendages. Other prominent features are flexion contractures and craniofacial and pulmonary abnormalities. The genetic and/or developmental defects leading to the restrictive dermopathy syndrome are presently not known. CONCLUSIONS: The restrictive dermopathy syndrome is distinct and is easily differentiated from other congenital diseases such as the icthyoses and also from the clinical conditions of sclerema neonatorum and subcutaneous fat necrosis of the newborn. Recognition of this syndrome is important for determining the prognosis of affected infants and for recommending genetic counseling to affected families.

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