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Resolution of Hydronephrosis in a Patient With Mucopolysaccharidosis Type II With Enzyme Replacement Therapy

Kei Nishiyama1, Takashi Imai1, Kazuhiro Ohkubo1

  • 1Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Higashi-ku, Fukuoka, Japan.

Urology
|December 25, 2016
PubMed

Insights

Mucopolysaccharidosis type II (MPS II), a genetic disorder, can affect the kidneys. Enzyme replacement therapy improved hydronephrosis in a patient with MPS II, suggesting a link between the condition and kidney issues.

Area of Science:

  • Biochemistry
  • Genetics
  • Pediatrics

Background:

  • Mucopolysaccharidosis type II (MPS II) results from iduronate-2-sulfatase deficiency, leading to glycosaminoglycan accumulation and multisystem disease.
  • Kidney and urinary tract complications are considered less common in MPS II patients.

Observation:

  • A pediatric patient with diagnosed MPS II presented with left hydronephrosis.
  • The patient's hydronephrosis showed improvement following the initiation of enzyme replacement therapy (ERT).

Findings:

  • This case suggests a potential association between MPS II and the development of hydronephrosis.
  • ERT may be beneficial in managing renal complications like hydronephrosis in MPS II patients.

Implications:

  • Further research is warranted to elucidate the precise mechanisms linking MPS II to hydronephrosis.
  • This finding may inform clinical monitoring and treatment strategies for renal manifestations in MPS II.

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