Syndrome of extreme insulin resistance (Rabson-Mendenhall phenotype) with atrial septal defect: Clinical presentation and treatment outcomes

JCRPE Journal of Clinical Research in Pediatric Endocrinology

Volumn 5, Issue 1, 2013, Pages 58-61

  Dutta, Deep ^a   Maisnam, Indira ^a   Ghosh, Sujoy ^a   Mukhopadhyay, Satinath ^a   Chowdhury, Subhankar ^a  

^a INSTITUTE OF POST GRADUATE MEDICAL EDUCATION AND RESEARCH   (India)

Author keywords

Atrial septal defect; Insulin resistance; Lipoatrophy; Rabson Mendenhall syndrome; Short stature

Indexed keywords

PIOGLITAZONE; TESTOSTERONE;

ACANTHOSIS; ADOLESCENT; ARTICLE; BODY FAT; CASE REPORT; CLITORAL ABSCESS; DENTITION; DUAL ENERGY X RAY ABSORPTIOMETRY; ECHOCARDIOGRAPHY; ECHOGRAPHY; FACIES; FEMALE; HEART ATRIUM SEPTUM DEFECT; HUMAN; HYPERGLYCEMIA; HYPERINSULINEMIA; HYPERPIGMENTATION; KARYOTYPE 46,XX; METABOLIC SYNDROME X; PRIMARY AMENORRHEA; SEX DIFFERENTIATION DISORDER; WEIGHT GAIN;

ADOLESCENT; DONOHUE SYNDROME; FEMALE; HEART SEPTAL DEFECTS, ATRIAL; HUMANS; HYPOGLYCEMIC AGENTS; INSULIN RESISTANCE; THIAZOLIDINEDIONES; TREATMENT OUTCOME;

EID: 84875752852     PISSN: 13085727     EISSN: 13085735     Source Type: Journal    
DOI: 10.4274/Jcrpe.857     Document Type: Article

References (9)

1. Goldfine AB, Moses AC. Syndromes of extreme insulin resistance. In: Kahn CR, Weir GC, et al. Joslin's Diabetes Mellitus, 14th ed. 2005;28: 493-498.
2. Longo N, Wang Y, Pasquali M. Progressive decline in insulin levels in Rabson-Mendenhall syndrome. J Clin Endocrinol Metab 1999;84:2623-2629.
3. Nakae J, Kato M, Murashita M, Shinohara N, Tajima T, Fujieda K. Long-term effect of recombinant human insulin-like growth factor I on metabolic and growth control in a patient with leprechaunism. J Clin Endocrinol Metab 1998;83:542-549.
4. Musso C, Cochran E, Moran SA, Skarulis MC, Oral EA, Taylor S, Gorden P. Clinical course of genetic diseases of the insulin receptor (type A and Rabson-Mendenhall syndromes): a 30-year prospective. Medicine (Baltimore) 2004;83:209-222.
5. Cochran E, Young JR, Sebring N, DePaoli A, Oral EA, Gorden P. Efficacy of recombinant methionyl human leptin therapy for the extreme insulin resistance of the Rabson-Mendenhall syndrome. J Clin Endocrinol Metab 2004;89:1548-1554.
6. D'Ercole AJ, Underwood LE, Groelke J, Plet A. Leprechaunism: studies of the relationship among hyperinsulinism, insulin resistance, and growth retardation. J Clin Endocrinol Metab 1979;48:495-502.
7. Kobayashi M, Takata Y, Sasaoka T, Shigeta Y, Goji K. Fluctuation of insulin resistance in a leprechaun with a primary defect in insulin binding. J Clin Endocrinol Metab 1988;66:1084-1088.
8. West RJ, Leonard JV. Familial insulin resistance with pineal hyperplasia: metabolic studies and effect of hypophysectomy. Arch Dis Child 1980;55:619-621.
9. Longo N, Singh R, Griffin LD, Langley SD, Parks JS, Elsas LJ. Impaired growth in Rabson-Mendenhall syndrome: lack of effect of growth hormone and insulin-like growth factor-I. J Clin Endocrinol Metab 1994;79:799-805.