title: Menorrhagia in patients with type I glycogen storage disease.
abstract: OBJECTIVE
  To evaluate menorrhagia in a cohort of women with glycogen storage disease type I because it appears to be an under-recognized problem in females of reproductive age.
  
  
  METHODS
  A retrospective chart review was performed on 13 menstruating patients with glycogen storage disease type I (age 23-48 years) for a diagnosis of menorrhagia.
  
  
  RESULTS
  Nine (69%) (confidence interval 0.39-0.91) women had development of menorrhagia. Median hemoglobin values in these patients were generally low (range 9.5-12.85 g/dL) but not different from those of the nonmenorrhagia group (hemoglobin range 9.55-11.0 g/dL) with glycogen storage disease type I. Four patients with menorrhagia required hospitalization or emergency department visits for treatment of menorrhagia. Two of the four patients hospitalized required blood transfusion, with an additional patient requiring a transfusion during pregnancy. Eight patients (89%) either were recommended to have or required medical or surgical treatment of their menorrhagia.
  
  
  CONCLUSIONS
  Glycogen storage disease type I is associated with menorrhagia. The evaluation should include assessment of coagulation functions and referral to a gynecologist, hematologist, or both, because bleeding diathesis and polycystic ovary syndrome are common in patients with glycogen storage disease type I.
authors.affiliation: Departments of Pediatrics, Divisions of Medical Genetics, Duke University Medical Center, Durham, North Carolina, and University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania; and the Department of Obstetrics and Gynecology, University of Virginia School of Medicine, Charlottesville, Virginia.
authors.forename: Stephanie L
authors.initials: SL
authors.lastname: Austin
authors.forename: Areeg H
authors.initials: AH
authors.lastname: El-Gharbawy
authors.forename: Vellore G
authors.initials: VG
authors.lastname: Kasturi
authors.forename: Andra
authors.initials: A
authors.lastname: James
authors.forename: Priya S
authors.initials: PS
authors.lastname: Kishnani
mesh_terms: D000236:Adenoma / Q000150:complications*; D000328:Adult; D000740:Anemia / Q000097:blood / Q000209:etiology; D001786:Blood Glucose / Q000378:metabolism; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000097:blood* / Q000150:complications*; D006454:Hemoglobins / Q000378:metabolism; D006728:Hormones / Q000627:therapeutic use; D006801:Humans; D006949:Hyperlipidemias / Q000097:blood / Q000150:complications; D019344:Lactic Acid / Q000097:blood; D008113:Liver Neoplasms / Q000150:complications*; D008595:Menorrhagia / Q000097:blood* / Q000150:complications* / Q000628:therapy; D008875:Middle Aged; D010974:Platelet Aggregation; D012189:Retrospective Studies; D055815:Young Adult; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D001786:Blood Glucose; D006454:Hemoglobins; D006728:Hormones; D014841:von Willebrand Factor; D019344:Lactic Acid