How Do You Spell DENYS DRASH SYNDROME?

Pronunciation: [dɪnˈa͡ɪz dɹˈaʃ sˈɪndɹə͡ʊm] (IPA)

Denys Drash Syndrome is a rare disorder that affects the kidneys, genitalia, and the development of the gonads. The spelling of the term is den-eez drahsh sin-drohm, using the International Phonetic Alphabet (IPA) phonetic transcription system. The first syllable of Denys is pronounced as "den" with a long "e" sound, while the second syllable is pronounced like the word "knees" but with a "z" instead of "k". Drash is pronounced like "drahsh" with an elongated "a" sound. The syndrome is named after the Canadian physician Dr. A.M. Denys and the American pediatric pathologist Dr. Robert Drash.

DENYS DRASH SYNDROME Meaning and Definition

  1. Denys-Drash Syndrome is a rare genetic disorder characterized by a combination of kidney disease, genital abnormalities, and an increased risk of developing a type of kidney cancer called Wilms tumor. It is named after the two doctors who first identified and described the syndrome in 1967 - Jean-Pierre Denys and Robert Drash.

    Individuals with Denys-Drash Syndrome typically present with early-onset renal disease, leading to progressive renal failure. This includes abnormalities in the structure and function of the kidneys, such as glomerular sclerosis. Additionally, these individuals may experience abnormal genital development, such as ambiguous or underdeveloped genitalia. In males, this can manifest as severe abnormalities of the testes, while in females it may involve malformed or absent reproductive organs.

    Furthermore, individuals with Denys-Drash Syndrome have an increased risk of developing Wilms tumor, a type of kidney cancer that primarily affects children. This tumor often presents in the first few years of life.

    The underlying cause of Denys-Drash Syndrome is mutations in the Wilms tumor suppressor gene (WT1) located on chromosome 11. These genetic mutations disrupt the development and functioning of the kidneys and other related structures, leading to the characteristic features of the syndrome.

    Due to the serious nature of the associated kidney disease and the risk of developing cancer, early diagnosis and management of Denys-Drash Syndrome are crucial. Treatment may involve medications and surgical interventions to manage kidney function and address any genital abnormalities. Regular monitoring for the potential development of Wilms tumor is also necessary.

Common Misspellings for DENYS DRASH SYNDROME

  • senys drash syndrome
  • xenys drash syndrome
  • cenys drash syndrome
  • fenys drash syndrome
  • renys drash syndrome
  • eenys drash syndrome
  • dwnys drash syndrome
  • dsnys drash syndrome
  • ddnys drash syndrome
  • drnys drash syndrome
  • d4nys drash syndrome
  • d3nys drash syndrome
  • debys drash syndrome
  • demys drash syndrome
  • dejys drash syndrome
  • dehys drash syndrome
  • dents drash syndrome
  • dengs drash syndrome
  • denhs drash syndrome
  • denus drash syndrome

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