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1.
Orphanet J Rare Dis ; 16(1): 382, 2021 09 09.
Artículo en Inglés | MEDLINE | ID: mdl-34503540

RESUMEN

BACKGROUND: Mucopolysaccharidosis consists of a group of diseases caused by the deficiency of lysosomal enzymes, which may lead to the compression of the median nerve in the carpal tunnel due to the accumulation of glycosaminoglycan, resulting in the hand disability. The study purpose is to present functional results of carpal tunnel release in mucopolysaccharidosis patients. Patients were selected from an enzyme replacement group in the Department of Pediatric Neurology. The legal guardians of the patients were informed about the likely functional change of the hands induced by compression of the median nerve. Clinical evaluation was performed in those patients who received their legal guardians' consent to participate and was included inspection, assessment of functional level, wrinkle test and the digital pinch function to manipulate small and large objects. Ultrasound and electromyography were performed to confirm the clinical median nerve compression. Bilateral extended opening technique was performed to access the carpal tunnel and analyze the anatomic findings of the median nerve and the flexed tendons of the fingers. After the surgical release of the carpal tunnel, the clinical evaluation was repeated. Subjective observations of the legal guardians were also considered. RESULTS: Seven patients underwent bilateral surgical opening of the carpal tunnel; six boys, mean age of 9.5 (5 to 13), five of them presenting Type II mucopolysaccharidosis, 1 Type I and 1 Type VI. The average follow-up was 12 months (10-13 months). The functional results observed included the improvement in the handling of small and large objects in all children who underwent decompression of the median nerve. The comparison between the pre-operative and post-operative functional levels revealed that 2 patients evolved from Level II to IV, 3 from Level III to IV, 1 from Level IV to V and 1 patient remained in Level III. Tenosynovitis around the flexor tendons and severe compression of the median nerve in the fourteen carpal tunnels were observed during the surgical procedure. In 6 wrists, partial tenosynovitis was performed. CONCLUSIONS: Despite the improvement in the overall function of the children' hands, we cannot conclude that only surgery was responsible for the benefit. Better designed studies are required.


Asunto(s)
Síndrome del Túnel Carpiano , Mucopolisacaridosis , Síndrome del Túnel Carpiano/cirugía , Niño , Humanos , Masculino , Nervio Mediano/diagnóstico por imagen , Nervio Mediano/cirugía , Mucopolisacaridosis/cirugía , Tendones , Ultrasonografía
2.
Rev. bras. ortop ; 43(4): 103-107, abr. 2008. tab
Artículo en Portugués | LILACS | ID: lil-484525

RESUMEN

Há grande variação com relação à ortografia de termos científicos em artigos e textos médicos. Visando padronizar esses termos, vigora atualmente a Terminologia Anatômica, publicada pelo Federative Committee on Anatomical Terminology e traduzida pela Comissão de Terminologia Anatômica da Sociedade Brasileira de Anatomia. Este trabalho correlaciona alguns termos usados na linguagem cotidiana com aqueles preconizados pela Nomina Anatomica. Também, discute a necessidade e a possibilidade de essa lista vir a tornar-se referência para escrita de assuntos ortopédicos.


The spelling of scientific words in medical papers and textbooks varies a lot. In order to standardize such spelling, we now have the Anatomical Terminology, published by the Federative Committee on Anatomical Terminology, which has been translated into Portuguese by the Terminology Committee of the Brazilian Anatomy Society. This paper correlates some of the words used in daily language to some of the words suggested by the Nomina Anatomica. The paper also discusses the need and the prospects of having such list become the reference of the spelling in orthopedic matters.


Asunto(s)
Anatomía , Ortopedia , Terminología
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