Schwenk H U, Windorfer A, Sitzmann F C
Klin Padiatr. 1975 May;187(3):257-63.
In a 3-year-old girl, who was admitted to our hospital with marked splenomegaly, a swollen abdomen and progressive loss of weight, the difficulty in establishing the correct diagnosis, namely a very rare generalized infection with Mycobacterium avium (serum type III), and the lengthy course of the disease are demonstrated. Norcardiosis, sarcoidosis and BCG-granulomatosis were excluded. From the spleen, lymphatic nodes, gastric juice, stools and urine of the child, who had not been vaccinated with BCG vaccine, masses of acid-fast bacilli were isolated. The generalisation of the disease was promoted by an immunological deficiency. At autopsy an atrophic thymus was discovered. The extensive clinical and immunological investigations pointed to a partial deficiency of the cell-mediated immune systeme. The patient died 8 months after admission.
一名3岁女童因脾肿大、腹部膨隆及体重进行性减轻入住我院。该病例体现了确诊的困难，即一种非常罕见的鸟分枝杆菌（血清III型）全身感染，以及病程的漫长。已排除诺卡菌病、结节病和卡介苗肉芽肿。从未接种过卡介苗的该患儿的脾脏、淋巴结、胃液、粪便和尿液中分离出大量抗酸杆菌。免疫缺陷促使疾病全身播散。尸检发现胸腺萎缩。广泛的临床和免疫学检查表明存在细胞介导免疫系统的部分缺陷。患者入院8个月后死亡。
