Literature DB >> 3350959

Thick lips, bumpy tongue, and slipped capital femoral epiphysis--a deadly combination.

C L Saltzman1, J E Herzenberg, W A Phillips, R N Hensinger, N J Hopwood.   

Abstract

Multiple endocrine neoplasia type 2b (MEN 2b) is a rare genetic disorder. Affected individuals have malignant thyroid tumors, pheochromocytoma, and ganglioneuromatosis. The musculoskeletal manifestations of MEN 2b include a Marfanoid habitus, pes cavus, scoliosis, slipped capital femoral epiphysis, joint laxity, poor muscle development, and delayed maturation. The initial clinical presentation of MEN 2b frequently involves the musculoskeletal system. The characteristics ganglioneuromatosis of the lips and tongue, however, should alert the orthopedic surgeon to the underlying disorder. Effective treatment of the malignant neoplasms hinges on early diagnosis. The risk of perioperative hypertensive crisis is significant; it may be prevented by appropriate treatment of the pheochromocytoma.

Entities:  

Mesh:

Year:  1988        PMID: 3350959

Source DB:  PubMed          Journal:  J Pediatr Orthop        ISSN: 0271-6798            Impact factor:   2.324


  3 in total

Review 1.  Management of medullary thyroid carcinoma and MEN2 syndromes in childhood.

Authors:  Steven G Waguespack; Thereasa A Rich; Nancy D Perrier; Camilo Jimenez; Gilbert J Cote
Journal:  Nat Rev Endocrinol       Date:  2011-08-23       Impact factor: 43.330

Review 2.  Multiple endocrine neoplasia type 2B (mucosal neuroma syndrome, Wagenmann-Froboese syndrome).

Authors:  P J Morrison; N C Nevin
Journal:  J Med Genet       Date:  1996-09       Impact factor: 6.318

3.  Painful Hip Leading to the Diagnosis of MEN 2B Syndrome.

Authors:  Mehtab Ahmad; Imran Rizvi; Amit Jain; Noorin Zaidi
Journal:  Case Rep Endocrinol       Date:  2012-11-26
  3 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.