International journal of dermatology · 2004
Transverse nail ridging (Beau's lines) and rough nails after bone marrow transplantation.
Клинический случайПисьмо в редакциюНазвание и аннотация: Перевод пока не подготовлен
Обработка статьи · этапы, ошибки и управление
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Ошибки и ограничения
История · было → стало
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Liu Yiguo, Cheng Nuo, Wang Zhehan, Zhao Chengdong, Zhang Xuyan, Li Dong, Mao Zixiang, Jiang Zijian, Wang Bowen, Luo Yan, Zhu Chao, Jiang Weixiang, Liu Yichen, Tao Li
Аннотация
BACKGROUND: Tibial hemimelia is a rare malformation with a wide clinical spectrum of presentation. The severity of this condition can be typed using different classification systems. It can exist as an independent entity or can be associated with upper limb or visceral malformations. The aims of our study are therefore, a. to report the incidence of upper limb deformities in relation to the severity of tibial hemimelia classified by the Jones classification, b. incidence of tibial hemimelia as a part of a syndrome c. to report the overall incidence of the associated upper limb and visceral deformities.
METHODS: A retrospective study was done using radiographs and clinical notes. The severity of the tibia deformity was assessed using the Jones classification. The clinical notes were reviewed to report the additional findings in the upper limbs and the visceral organs.
RESULTS: The study included 69 patients with tibial hemimelia aged from 10 months to 34 years. Twenty of them (28.9%) had bilateral involvement. Additional malformations were observed in 56 patients (81%) involving the upper and lower limb and visceral organs. In 11 patients (16%), tibial hemimelia occurred as part of a syndrome, most often being Gollop-Wolfgang complex. The incidence of malformations of the upper extremities was 15 (21.7%), four of which (26.6%) involved bilateral upper extremity malformation. The cleft hand was the most frequent malformation of the upper extremities, followed by hypoplasia or aplasia of the thumb and fingers.
CONCLUSION: Jones type I tibial hemimelia is often associated with visceral and upper limb malformations. visceral anomalies are associated with syndromal forms of Tibiail hemimelia. Several forms of upper limb malformations with varying severity were associated with the disorder. Therefore, a holistic approach to the patient should be initiated soon after birth involving a paediatric, hand and visceral surgeon, to provide the best possible care.
LEVEL OF EVIDENCE: Level IV study, retrospective review of 69 patients with tibial hemimelia.
METHODS: A retrospective study was done using radiographs and clinical notes. The severity of the tibia deformity was assessed using the Jones classification. The clinical notes were reviewed to report the additional findings in the upper limbs and the visceral organs.
RESULTS: The study included 69 patients with tibial hemimelia aged from 10 months to 34 years. Twenty of them (28.9%) had bilateral involvement. Additional malformations were observed in 56 patients (81%) involving the upper and lower limb and visceral organs. In 11 patients (16%), tibial hemimelia occurred as part of a syndrome, most often being Gollop-Wolfgang complex. The incidence of malformations of the upper extremities was 15 (21.7%), four of which (26.6%) involved bilateral upper extremity malformation. The cleft hand was the most frequent malformation of the upper extremities, followed by hypoplasia or aplasia of the thumb and fingers.
CONCLUSION: Jones type I tibial hemimelia is often associated with visceral and upper limb malformations. visceral anomalies are associated with syndromal forms of Tibiail hemimelia. Several forms of upper limb malformations with varying severity were associated with the disorder. Therefore, a holistic approach to the patient should be initiated soon after birth involving a paediatric, hand and visceral surgeon, to provide the best possible care.
LEVEL OF EVIDENCE: Level IV study, retrospective review of 69 patients with tibial hemimelia.