Clinical Case: Infant Arthrogryposis

Popova, L.Yu.; Voropaev, I.V.; Ukolova, E.S.; Likhogray, L.I.; Drobinina, A.V.

doi:10.31550/1727-2378-2019-160-5-30-32

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“…Артрогрипоз это тяжелое врожденное заболевание опорно-двигательного аппарата, характеризующееся врожденными контрактурами двух и более суставов несмежных областей в сочетании с мышечной гипотрофией или атрофией [1]. Типичными клиническими признаками дистального артрогрипоза являются выраженные деформации верхних и нижних конечностей, / 2020 / ТОМ 19 / № 4 CURRENT PEDIATRICS / 2020 / V. 19 / № 4 а также аномалии лицевого скелета.…”

Section: обоснованиеunclassified

“…Однако, как было отмечено выше, возможны различные комбинации указанных признаков или их отсутствие [3,4]. В отечественной [1,4] и зарубежной литературе [5,6] За время нахождения ребенка в отделении патологии новорожденных и недоношенных детей в течение 21 сут проводилась лечебная гимнастика с наложением этапных ладонных гипсовых лонгет от кончиков пальцев до верхней трети предплечий в положении максимально возможного разгибания II-V пальцев и отведения и разгибания I пальца. На фоне гипсования отмечена небольшая положительная динамика с увеличением амплитуды разгибания пальцев кисти (рис.…”

Section: обоснованиеunclassified

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Distal Arthrogryposis in Newborn: Clinical Case

Гаврилюк

Евсеева²,

Черевко³

et al. 2020

Vopr. sovr. pediatr.

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Background. Arthrogryposis is severe congenital musculoskeletal disease with contractures of two or more joints of the lower and/or upper limbs and usually in combination with muscular hypo- or atrophy.Clinical Case Description. Child F. was hospitalized in the department of newborns and premature babies’ pathology on 6th day of life in critical condition. Spontaneous motor activity was severely decreased. He responds to the examination with little increase in motor activity and weak painful scream. Muscle tone is dystonic: it is diffusely reduced in the proximal parts of the upper and lower limbs, in the distal parts of the upper limbs it is significantly increased, there are flexion contractures of the fingers on both hands (mostly on the left one). Passive fingers contractures reversal is difficult. There were no feet deformities or craniofacial anomalies. Hereditary history is burdened: child’s father (1984 year of birth), great-grandfather and cousin uncle (on the paternal line) have finger deformities on both hands. Father and relatives have not been diagnosed before. The clinical diagnosis was established after consultation of geneticist and orthopedist: «Distal arthrogryposis, type 2A, autosomal dominant type of inheritance with incomplete penetrance». The molecular genetic testing was not performed due to the refusal of the child's parents. Therapeutic gymnastics, stage plaster correction of finger contractures were performed in the department during the child hospitalization (21 days). Positive dynamics was noted: finger extension amplitude has increased.Conclusion. Early conservative treatment of infants with arthrogryposis allows to correct musculoskeletal deformities. Early initiation of treatment is expected to increase the amplitude of both passive and active movements in the hand joints, whereas, it will improve the function of hand grasping and self-care capacities of patients.

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Section: обоснованиеunclassified