Intractable early childhood obesity as the initial sign of insulin resistant hyperinsulinism and precursor of polycystic ovary syndrome.
OBJECTIVE
We report that intractable early childhood obesity may be associated with severe insulin resistance syndromes (pseudo-Cushing's syndrome and pseudo-acromegaly) and precede polycystic ovary syndrome (PCOS).


RESULTS
Patient 1 had prepubertal obesity followed by early puberty and was diagnosed with pseudo-Cushing's syndrome and insulin resistance at 10.3 years. Oligomenorrhea, androgen excess, and type 2 diabetes mellitus (DM2) emerged at 13.5 years. Patient 2 developed intractable prepubertal obesity followed by atypical true sexual precocity and pseudo-Cushing's syndrome in early childhood. By 11.3 years, oligomenorrhea, androgen excess, and DM2 had appeared. Patient 3 had prepubertal overgrowth in weight and height and was diagnosed with pseudo-acromegaly, menstrual irregularity, androgen excess, and impaired glucose tolerance at 14.3 years of age. Patient 4 had prepubertal overgrowth that evolved into pseudo-acromegaly, insulin resistance, secondary amenorrhea, and androgen excess at 15.6 years.


CONCLUSIONS
Intractable prepubertal obesity was recognized to culminate in early childhood pseudo-Cushing's syndrome or pseudo-acromegaly, which are manifestations of insulin-resistant hyperinsulinism, and to herald adolescent PCOS.
Littlejohn
Elizabeth E
EE
University of Chicago, Pritzker School of Medicine, Department of Pediatrics, Section of Pediatric Endocrinology, 5841 S. Maryland Avenue, IL 60637, USA. ebaumann@peds.bsd.uchicago.edu
Weiss
Roy E
RE
Deplewski
Dianne
D
Edidin
Deborah V
DV
Rosenfield
Robert
R
D000293:Adolescent; D002648:Child; D003480:Cushing Syndrome / Q000473:pathology; D005260:Female; D006801:Humans; D006946:Hyperinsulinism / Q000175:diagnosis / Q000209:etiology*; D007333:Insulin Resistance; D009765:Obesity / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology; D011085:Polycystic Ovary Syndrome / Q000175:diagnosis / Q000209:etiology*; D011629:Puberty, Precocious / Q000473:pathology; D013997:Time Factors