Complete Wolfram’s syndrome and successful pregnancy

Rugolo, Salvatore; Mirabella, Daniela; Palumbo, Marco; Chiantello, Rosalba; Fiore, Gaetano

doi:10.1016/s0301-2115(02)00150-1

Cited by 4 publications

(2 citation statements)

References 5 publications

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“…8 The diabetes insipidus seemed to have mounted a stiff challenge to efficient management of the antenatal and postpartum periods in previously reported cases. 1 Our patient has been on regular desmopressin since the age of 25 years, and her diabetes insipidus remained stable during the antenatal period, ensured by close follow-up of her electrolyte status and osmolality. For better control of her blood glucose levels, she was initially put on multiple daily injections of insulin.…”

Section: Successful Use Of Insulin Pump In Didmoadmentioning

confidence: 72%

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An Asian Indian Woman with Wolfram Syndrome on Insulin Pump: Successful Pregnancy and Beyond

Kesavadev

Kumar

Shankar

et al. 2011

Diabetes Technology & Therapeutics

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Wolfram syndrome (WS), or DIDMOAD (diabetes insipidus, diabetes mellitus, optic atrophy, and deafness), is a rare autosomal recessive neurodegenerative disorder with a median life expectancy of 30 years and occurs in one in 770,000 live births. To date only five successful pregnancies have been reported among WS subjects worldwide. Here we describe the sixth report of successful pregnancy in a WS patient and the first from India. The subject is still on an insulin pump, now 31 years old and doing well. She developed diabetes at 5 years of age, optic atrophy at 14 years, and diabetes insipidus at 25 years and had a successful delivery in 2007 while on an insulin pump. Sequencing of exonic regions of the WFS1 gene showed five changes, two of which were pathogenic (exon 8). Magnetic resonance imaging of brain showed generalized neurodegenerative changes. The benefits of continuous subcutaneous insulin infusion and that of tight metabolic control in prevention of abortions and fetal malformations in diabetes associated with pregnancy are well documented. The impression of probable pleiotropic action of insulin pumps over and above that of glycemic reduction is gaining momentum. Recent evidence supports use of insulin pumps in alleviating neuropathic pain in diabetes, probably by virtue of its action in minimizing mean amplitude of glycemic excursions not possible with conventional insulin shots. WS is a progressive neurodegenerative disorder, which will probably help us in understanding the positive impact of continuous subcutaneous insulin infusion in prolonging the life span and retarding neuronal damage in WS.

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Section: Successful Use Of Insulin Pump In Didmoadmentioning

confidence: 72%

“…Previously only five cases of successful pregnancy in WS have been reported worldwide. 1 Here we report the sixth case (and the first from India) of successful pregnancy in such a patient. She was started on an insulin pump, considering that her diabetes was poorly controlled, and that she had long-term plans to marry and conceive.…”

mentioning

confidence: 84%

An Asian Indian Woman with Wolfram Syndrome on Insulin Pump: Successful Pregnancy and Beyond

Kesavadev

Kumar

Shankar

et al. 2011

Diabetes Technology & Therapeutics

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Current Awareness in prenatal diagnosis

2003

Prenatal Diagnosis

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In order to keep subscribers up‐to‐date with the latest developments in their field, John Wiley & Sons are providing a current awareness service in each issue of the journal. The bibliography contains newly published material in the field of prenatal diagnosis. Each bibliography is divided into 17 sections: 1 Books, Reviews & Symposia; 2 General Interest; 3 Normal Fetal Development; 4 Gametogenesis and Pre‐implantation Diagnosis; 5 First Trimester Diagnosis; 6 Second Trimester Diagnosis; 7 Fetal Diagnosis by Ultrasound and Other Imaging; 8 Maternal Screening; 9 Screening for Carriers of Genetic Abnormality; 10 Technological Developments; 11 Confined Placental Mosaicism and Uniparental Disomy; 12 Molecular Cytogenetics; 13 Fetal Cells in Maternal Circulation; 14 Fetal Therapy; 15 Psychosocial Aspects; 16 Epidemiology and Environmental Factors; 17 Developmental Pathology. Within each section, articles are listed in alphabetical order with respect to author. If, in the preceding period, no publications are located relevant to any one of these headings, that section will be omitted

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