Loós T, Kocsmárszki Z, Mártha I
Monatsschr Kinderheilkd (1902). 1976 Nov;124(11):733-40.
Between 1972--1974 26 children (15 boys and 11 girls) aged 11--14, with sarcoidosis were observed in county Szabolcs-Szatmár, Hungary. The number of cases varied between 1--6 per year, so the incidence of sarcoidosis is about 2--11/100000 for the age group 10--14 years of this county. Sarcoidosis was detected because of complaints in 4 cases, and by mass radiographic surveys in 22 cases. In each of the patients the thoracic form of sarcoidosis was in the foreground (in 6 cases mild bilateral hilar adenopathy, in 2 cases only unilateral hilar adenopathy, in 18 cases typical BHL-syndrome sometimes with "tumorous" adenopathy and in 7 cases out of the 18, hilar adenopathy and mottling in the lungs). The laboratory data were an unsatisfactory basis for the diagnosis. The tuberculin test was negative in 65 per cent. There was no proof for infection with atypical mycobacteria by intradermal tests. The most important basis of diagnosis was the biopsy (mediastinoscopy), especially in cases of simultaneous infection with tuberculosis. Prednisone treatment was carried out in the first place in cases of adenopathy and mottling, and in the cases of elevated serum calcium. Prednisone resistance was observed in two cases; in one of the patients it was secondary since it manifested itself only at the relapse of the BHL-syndrome; in the other patient the drug resistance was primary.
1972年至1974年间,在匈牙利的萨博尔奇-萨特马尔县观察到26名年龄在11至14岁之间的结节病患儿(15名男孩和11名女孩)。每年的病例数在1至6例之间,因此该县10至14岁年龄组的结节病发病率约为2至11/100000。4例因出现症状而被检测出结节病,22例通过大规模X线检查被发现。每位患者均以结节病的胸型为主(6例为轻度双侧肺门淋巴结肿大,2例仅为单侧肺门淋巴结肿大,18例为典型的双侧肺门淋巴结肿大综合征,有时伴有“肿瘤样”淋巴结肿大,在这18例中的7例中,除肺门淋巴结肿大外,肺部还有斑点状阴影)。实验室数据作为诊断依据并不理想。结核菌素试验65%呈阴性。皮内试验未证实非典型分枝杆菌感染。诊断的最重要依据是活检(纵隔镜检查),尤其是在合并结核感染的病例中。对于出现淋巴结肿大和斑点状阴影以及血清钙升高的病例,首先进行泼尼松治疗。观察到2例泼尼松耐药;其中1例为继发性耐药,因为仅在双侧肺门淋巴结肿大综合征复发时才出现;另一例患者的耐药为原发性。