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The subtle signs of Wolfram (DIDMOAD) syndrome: not all juvenile diabetes is type 1 diabetes
-
Claudia Boettcher
,
Burkhard Brosig
Veröffentlicht/Copyright:
16. März 2011
Abstract
Wolfram syndrome (also known as DIDMOAD=diabetes insipidus, diabetes mellitus, optic atrophy, deafness) is an autosomal recessive disorder characterized by the association of childhood non-immune insulin-dependent diabetes mellitus (DM) with progressive bilateral optic atrophy. Additional symptoms including signs of severe neurodegeneration and psychiatric illness are likely to evolve over time resulting in premature death. We report on two siblings of Turkish origin from our diabetes clinic who were diagnosed with Wolfram syndrome after 6 years and 2 years duration of DM, respectively. Subtle symptoms such as attitude changes, growing reading difficulties in the history of children or adolescents with antibody negative and ketone negative DM should alert the treating physician and lead to re-evaluation of the diagnosis, keeping in mind that not all juvenile DM is type 1 DM.
Published Online: 2011-03-16
Published in Print: 2011-03-01
©2011 by Walter de Gruyter Berlin New York
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Artikel in diesem Heft
- Publisher's Note
- Publisher's Note
- Editorial
- Sex steroid priming for growth hormone (GH) provocative tests: an endless debate with insufficient solution
- Not ready for prime time
- Commentary
- Sex hormone priming
- Pros of priming in the diagnosis of growth hormone deficiency
- Images in Pediatric Endokrinology
- Images in pediatric endocrinology: vitamin D deficiency rickets and other nutritional deficiencies in a 12-month-old infant
- Original Contributions
- The influence of an educational program on the HbA1c-level of adolescents with type 1 diabetes mellitus: a retrospective study
- Contribution of clinical, metabolic, and genetic factors on hypertension in obese children and adolescents
- Hyperphosphatemic tumoral calcinosis: a 10-year follow-up
- Vitamin D deficiency and insulin resistance in obese African- American adolescents
- Evaluation of children with nutritional rickets
- The effects of delivery route and anesthesia type on early postnatal weight loss in newborns: the role of vasoactive hormones
- Abdominal obesity is associated with cardiovascular risk in Japanese children and adolescents
- PPAR-γ2 Pro12Ala polymorphism is associated with post-challenge abnormalities of glucose homeostasis in children and adolescents with obesity
- The relationship between pubertal gynecomastia, prostate specific antigen, free androgen index, SHBG and sex steroids
- Severe hypercalcemia associated with Williams syndrome successfully treated with pamidronate infusion therapy
- The subtle signs of Wolfram (DIDMOAD) syndrome: not all juvenile diabetes is type 1 diabetes
- The frequency of Hashimoto thyroiditis in children and the relationship between urinary iodine level and Hashimoto thyroiditis
- The relationship between serum phosphate levels with childhood obesity and insulin resistance
- The relationship of salivary and cord blood cortisol inpreterm infants
- A comparison of referral patterns to the pediatric endocrine clinic before and after FDA approval of growth hormonefor idiopathic short stature
- Benign transient hyperphosphatasemia in infants and children: a prospective cohort
- Carotid arterial stiffness in overweight and obese Indian children
- Association study in Taiwanese girls with precocious puberty
- Patient Report Letters
- A homozygous recurring mutation in WISP3 causing progressive pseudorheumatoid arthropathy
- Two pediatric patients with Von Hippel-Lindau disease type 2b: from patient to screening, from screening to patient
- Hot Topic
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- Meetings
- Meetings Calendar
