Singh Ankur, Krishnan Rajeshwari, Bhattacharya Malobika, Pradhan Gaurav, Salzer Ulrich, Kapoor Seema
Division of Genetics, Department of Pediatrics, Maulana Azad Medical College and Associated Lok Nayak Hospital, Bahadur Shah Zafar Marg, New Delhi, 110 002, India.
Indian J Gastroenterol. 2013 Nov;32(6):409-12. doi: 10.1007/s12664-013-0358-6. Epub 2013 Aug 17.
Cartilage hair hypoplasia is a genetic disease, characterized by generalized metaphyseal dysplasia mainly affecting the knee joints. Variable extraskeletal features like anemia, malabsorption, impaired spermatogenesis, impaired immunity, and malignancies have been reported. The work up of malabsorption and short stature led to the diagnosis of celiac disease in three previous cases of cartilage hair hypoplasia. Here, we report an Indian girl with a novel genotype, diagnosed as celiac disease with cartilage hair hypoplasia, and review the previous three cases.
软骨毛发发育不全是一种遗传性疾病,其特征为主要影响膝关节的全身性干骺端发育异常。曾有报道称存在如贫血、吸收不良、精子发生受损、免疫功能受损及恶性肿瘤等多种骨骼外特征。在之前三例软骨毛发发育不全病例中,针对吸收不良和身材矮小的检查诊断出了乳糜泻。在此,我们报告一名具有新基因型的印度女孩,其被诊断为患有乳糜泻合并软骨毛发发育不全,并回顾之前的三例病例。