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mody in de schijnwerpers

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Tijdschrift voor kindergeneeskunde

Summary

Maturity Onset Diabetes of the Young (mody) is an inheritable form of diabetes mellitus, that often presents itself in childhood. It is characterized by an autosomal dominant pattern of inheritance and can be divided in at least six subtypes. The severity of the diabetic symptoms varies considerably, which complicates the diagnostic process. Although approximately 5% of the total diabetic population suffers from mody, only in a small subset of patients the diagnosis is made.

Samenvatting

Maturity Onset Diabetes of the Young (mody) is een vorm van diabetes die zich vaak in de jongere leeftijdscategorie presenteert. Het wordt veroorzaakt door een genetisch defect in de pancreatische ß-celfunctie met een autosomaal dominant overervingspatroon. Hierdoor ontstaat een inadequate insulineafgifte in relatie tot de serumglucosespiegel. De ernst van de diabetische symptomatologie is zeer variabel, zowel tussen als binnen de verschillende mody-families, waardoor herkenning van dit ziektebeeld bemoeilijkt wordt. Hoewel naar schatting 5% van de totale populatie diabetespatiënten aan deze aandoening lijdt, wordt de diagnose vaak gemist.

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Authors and Affiliations

  1. www.kindergeneeskunde.bsl.nl

    K. Marcus & P. Stouthart

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  1. K. Marcus
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  2. P. Stouthart
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Drs. K. Marcus, Máxima Medisch Centrum, Veldhoven. Drs. P. Stouthart, Maasland ziekenhuis, Sittard.

Correspondentieadres: Drs. K. Marcus, Máxima Medisch Centrum, Postbus 7777, 5500 MB Veldhoven, tel: 040-8888000, e-mail: k.marcus@mmc.nl.

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Marcus, K., Stouthart, P. mody in de schijnwerpers. KIND 74, 208–213 (2006). https://doi.org/10.1007/BF03061634

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