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Multiple congenital anomalies-hypotonia-seizures syndrome

Multiple congenital anomalies-hypotonia-seizures syndrome (MCAHS) is a rare multi-systemic genetic disorder which is characterized by developmental delay, seizures, hypotonia and heart, urinary, and gastrointestinal abnormalities.

Presentation

People with this disorder often show the following symptoms:

General

Heart

Genito-urinary

  • Hydrocele
  • Renal collection system dilatation
  • Hydroureter
  • Hydronephrosis
  • Trabecular urinary bladder hypertrophy

Gastrointestinal

Facial

Auricular

  • Auricle abnormalities

Causes

It is caused by an autosomal recessive mutation in PIGN (gene), on chromosome 18. It is caused by a C to A nucleotide substitution.

Epidemiology

Only 15 cases of this syndrome have been reported in medical literature.

References