Gocmen A, Peters H A, Cripps D J, Bryan G T, Morris C R
Department of Pediatrics, School of Medicine, University of Hacettepe, Ankara, Turkey.
Biomed Environ Sci. 1989 Mar;2(1):36-43.
During the period 1955-1959, approximately 4000 people in southeast Anatolia developed porphyria due to the ingestion of hexachlorobenzene (HCB), a fungicide added to wheat seedlings. These HCB exposures subsequently led to the development of bullae on sun-exposed areas, hyperpigmentation, hypertrichosis, and porphyrinuria. The condition was called kara yara or "black sore." Many of the breast-fed children under the age of 2 years whose mothers had ingested HCB-treated grain died from a disease known as pembe yara or "pink sore." In this follow-up study of 252 patients, 20-30 years postexposure, there were 162 males and 90 females, with an average current age of 35.7 years, an average of onset of 7.6 years, and a duration of 2.2 years. Many patients had dermatologic, neurologic, and orthopedic symptoms and signs. The observed clinical findings include scarring of the face and hands (83.7%), hyperpigmentation (65%), hypertrichosis (44.8%), pinched facies (40.1%), painless arthritis (70.2%), small hands (66.6%), sensory shading (60.6%), myotonia (37.9%), cogwheeling (41.9%), enlarged thyroid (34.9%), and enlarged liver (4.8%). Urine and stool porphyrin levels were determined in all patients, and 17 have at least one of the porphyrins elevated. A total of 56 specimens of human milk obtained from mothers with porphyria were analyzed for HCB. The average value was 0.51 ppm in HCB-exposed patients compared to 0.07 ppm in unexposed controls. Offspring of mothers with three decades of HCB-induced porphyria appear normal.
1955年至1959年期间,安纳托利亚东南部约4000人因摄入添加到小麦幼苗中的杀菌剂六氯苯(HCB)而患上卟啉症。这些六氯苯暴露随后导致暴露于阳光下的部位出现大疱、色素沉着、多毛症和卟啉尿。这种病症被称为卡拉亚拉或“黑疮”。许多母亲摄入了经六氯苯处理谷物的2岁以下母乳喂养儿童死于一种名为彭贝亚拉或“粉疮”的疾病。在这项对252名患者进行的随访研究中,暴露后20至30年,男性162名,女性90名,当前平均年龄为35.7岁,平均发病年龄为7.6岁,病程为2.2年。许多患者有皮肤、神经和骨科症状及体征。观察到的临床发现包括面部和手部瘢痕形成(83.7%)、色素沉着(65%)、多毛症(44.8%)、面容消瘦(40.1%)、无痛性关节炎(70.2%)、小手(66.6%)、感觉减退(60.6%)、肌强直(37.9%)、齿轮样强直(41.9%)、甲状腺肿大(34.9%)和肝脏肿大(4.8%)。对所有患者的尿液和粪便卟啉水平进行了测定,17名患者至少有一种卟啉升高。对从患卟啉症母亲处获得的56份人乳样本进行了六氯苯分析。六氯苯暴露患者的平均值为0.51 ppm,未暴露对照组为0.07 ppm。患六氯苯诱发卟啉症三十年的母亲的后代看起来正常。