Willgeroth C, Zugehör M, Kraus W
Z Gesamte Inn Med. 1982 Dec;37(23):813-6.
It is reported on a 16-year-old patient with Goodpasture's syndrome in whom in a period of two years recurrent respiratory infection with haemoptyses appeared. Shortly after admission to hospital he died of respiratory insufficiency. Immunohistologically post mortem on pulmonary and renal tissue could be proved diffuse linear deposits of IgG and C3 on the basement membrane of the renal glomeruli and the alveoli. Histologically prevailed pulmonary haemorrhages and pulmonary haemosiderosis, whereas on the kidneys only a focal segmental mesangioproliferative glomerulonephritis was present. References are given to the possibility of early diagnosis by means of immunohistological examinations on the lungs and/or biopsies of the kidney and the determination of circulating glomerular antibodies of the basement membrane, since despite general knowledge also in adequate anamnesis with relapsing pulmonary haemorrhages at juvenile age it is not always thought of this fact and hopeful courses under early therapy with corticoids, immunosuppressive drugs and perhaps plasmapheresis are described in literature.
据报道，一名16岁的古德帕斯彻综合征患者在两年内反复出现呼吸道感染并伴有咯血。入院后不久，他死于呼吸功能不全。尸检时，通过免疫组织化学方法证实，在肺和肾组织中，IgG和C3在肾小球和肺泡的基底膜上呈弥漫性线性沉积。组织学上，以肺出血和肺含铁血黄素沉着症为主，而在肾脏仅存在局灶节段性系膜增生性肾小球肾炎。文中提到通过对肺进行免疫组织学检查和/或肾活检以及测定循环中的基底膜肾小球抗体来进行早期诊断的可能性，因为尽管大家都了解这一情况，但在青少年出现反复肺出血的充分病史中，这一事实并不总是被想到，并且文献中描述了在早期使用皮质类固醇、免疫抑制药物以及可能的血浆置换疗法下有望取得的病程。
