Turner syndrome: An unusual case of secondary diabetes

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Date
2013-11
Authors
Fasanmade, O.O
Iwuala, S.O
Odeniyi, I.A
Fasanmade, O.A
Ohwovoriole, A.E
Journal Title
Journal ISSN
Volume Title
Publisher
American Association of Clinical Endocrinologists, Nigeria Chapter
Abstract
Background: Turner syndrome is named after Henry Turner, the endocrinologist who first described it in 1938.Turner syndrome 45,X, encompasses several conditions in human females, of which monosomy X is most common. It is a chromosomal abnormality in which all or part of one of the sex chromosomes is absent or has other abnormalities. In some cases, the chromosome is missing in some cells but not others, a condition referred to as mosaicism. Objective: The purpose of this report is to bring to the fore the metabolic/endocrine conditions seen in patients with Turner and to increase awareness so as to actively screen them at intervals for these conditions. Method: The case note of a patient with secondary diabetes on a background of Turner Syndrome and the literature was reviewed. Results: Patient is a 29 year old lady, known patient with Turner Syndrome diagnosed 2010 by a standard 30-cell karyotype analysis when she was being evaluated by the gynaecologist on account of primary amenorrhoea. She was referred to our clinic in 2011 on account of elevated fasting blood sugar of 332mg/dl and 2hr post prandial glucose of 389mg/dl which was detected during a routine pre-employment screening . She was asymptomatic but had 3+ glycosuria, ketone 1+ in urine. She was also noted to have elevated total cholesterol of 307mg/dl. HbA1c of 12.2%. She was commenced on Insulin, Metformin and Rosuvastatin. Metabolic control has improved but she is still desirous of normal menstruation and in future child bearing. Discussion: Patients with Turner syndrome are at increased risk of a range of autoimmune conditions including hypothyroidism, hyperthyroidism. Abnormalities of glucose metabolism, including overt diabetes mellitus, are more common in them. Short stature and ovarian failure are risk factors for osteoporosis. Conclusion: Turner syndrome patients are at increased risk of metabolic/endocrine disorders, hence they should be screened regularly for these disorders. As routine health maintenance, patients with Turner syndrome should have blood urea nitrogen, creatinine, fasting blood sugar (FBS) or hemoglobin A1C, fasting lipids, T4, and TSH levels measured annually after childhood. Since osteoporosis risk is increased, care should be taken to ensure adequate daily intake of calcium and vitamin D. Bone mineral density should also be monitored in them. They should avoid obesity, which increases the already high risks of hypertension and insulin resistance. Key words: Turner Syndrome, Diabetes Mellitus, Hypothyroidism, Hyperthyroidism, Osteoporosis, Insulin Resistance
Description
Staff Publications
Keywords
Turner syndrome , Endocrinologist , Patients , Secondary diabetes mellitus , Research Subject Categories::MEDICINE
Citation
Fasanmade OA, Iwuala SO Odeniyi IA, Fasanmade OA, Ohwovoriole AE. Turner syndrome: An unusual case of secondary diabetes. Abstract from 5th Congress of AACE Nigeria chapter