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Cohen's Syndrome with Diabetes Mellitus
Author(s) -
Nambu Mitsuhiko,
Oshima Yusei,
Kakiuchi Toshitaka,
Hayakawa Yasushi,
Ito Tadashi,
Hasegawa Tomoko,
Fujita Hiroko
Publication year - 1988
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1988.tb02502.x
Subject(s) - medicine , short stature , diabetes mellitus , diabetic ketoacidosis , hypoplasia , urinary system , endocrinology , pediatrics
We report a case of 12‐year‐old girl with Cohen's syndrome (CS) associated with diabetes mellitus (DM). She had the characteristic facies, with a short philtrum, an open‐mouth appearance, prominent maxillary central incisors, high palate, mild maxillary hypoplasia and micrognathia. Other features included truncal obesity, short stature, mental deficiency, small hands and feet, and accentuated thoracic kyphosis. She had been hypotonic during the neonatal and early infantile periods. Laboratory examinations showed hyperglycemia and glucosuria. HbA l and HbA 1 c levels were raised. A 75‐g oral glucose tolerance test (OGTT) showed glucose intolerance consistent with DM, but there was a normal level of urinary C‐peptide. There were no islet cell antibodies (ICAs) and no history of ketoacidosis. This is the first reported case of CS with non‐insulin‐dependent diabetes mellitus (NIDDM).