Fontana D, Leli R, Della Beffa V
Divisione di Chirurgia B, Ospedale G. Bosco, Torino.
Minerva Chir. 1996 Mar;51(3):167-9.
A case of a woman affected by an unusual association of rare diseases, is presented. The patient was referred to our department for acute anaemia. Preoperative investigations revealed that the patient was affected by fibrous polyostotic dysplasia, so called Jaffè-Lichtenstein syndrome. The presence of skin brown spot and endocrine disorders requiring pill administration, allows to classify the patient as carrier of Albright syndrome. Moreover, the angiography pointed out a celiac trunc stenosis (Dunbar syndrome). The long-standing administration of the pill could be the cause of bleeding adenoma. The patient underwent hepatic resection. We did not treat the Dunbar syndrome because of poor symptoms. From literature, we review some opinions on the fibrous dysplasia of the bone.
本文报告了一例患有罕见疾病异常组合的女性病例。该患者因急性贫血被转诊至我科。术前检查发现患者患有纤维性骨发育异常，即所谓的贾菲-利希滕斯坦综合征。皮肤褐色斑以及需要服药治疗的内分泌紊乱症状的存在，使该患者被归类为奥尔布赖特综合征携带者。此外，血管造影显示存在腹腔干狭窄（邓巴综合征）。长期服用避孕药可能是出血性腺瘤的病因。患者接受了肝切除术。由于症状较轻，我们未对邓巴综合征进行治疗。我们从文献中回顾了一些关于骨纤维发育异常的观点。
