Alström syndrome with acute pancreatitis: a case report

Kaohsiung J Med Sci. 2003 Jul;19(7):358-61. doi: 10.1016/S1607-551X(09)70438-3.


We report the case of a 21-year-old female with Alström syndrome who also suffered from acute pancreatitis of obscure manifestation. The patient had underlying cone-rod dystrophy of the retinas, nystagmus, obesity, progressive sensorineural hearing impairment, diabetes mellitus, and hypertriglyceridemia, compatible with the clinical diagnosis of Alström syndrome. Serial examinations showed liver dysfunction and pancreatitis. In treating a patient with poor communication (i.e. cone-rod dystrophy and hearing impairment) suffering from acute illness, understanding the underlying disease and the potential for pancreatitis with hypertriglyceridemia is necessary. It is also a challenge to treat a patient with multiple system involvement. In conclusion, Alström syndrome is a disease of systemic multi-organ involvement, and hepatic disease and pancreatitis, possibly due to dyslipidemia, appear to be manifestations of Alström syndrome.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple
  • Acute Disease
  • Adult
  • Deafness
  • Diabetes Mellitus, Type 2 / complications*
  • Female
  • Hearing Loss, Sensorineural / complications*
  • Humans
  • Liver Diseases / complications
  • Nystagmus, Pathologic / complications*
  • Obesity / complications
  • Pancreatitis / diagnosis
  • Pancreatitis / etiology*
  • Retinitis Pigmentosa / complications*
  • Syndrome