title: Bariatric surgery is not contraindicated in obese patients suffering from glycogen storage disease type IXa. A case report with follow-up at three years.
abstract: BACKGROUND
  Glucose storage disease type IXa (GSD IXa) is an uncommon condition presenting with childhood onset hepatomegaly, growth retardation, and often, fasting ketosis and hypoglycemia. Despite its benign course, the lack of dietary counseling may favor uncontrolled weight gain. We investigated the efficacy of bariatric surgery in one 17 years old female suffering from GSD IXa and morbid obesity.
  
  
  METHODS
  The diagnosis was GSD type IXa in a patient with a body mass index (BMI) of 45.5kg/m(2). Onset of hypoglycemia was reported twice each month. She was treated her implanting an adjustable gastric banding through laparoscopy. Three years after surgery the patient presents a BMI of 30.1kg/m(2) and an excess of weight loss (EWL) of 71.1%. Only once, following surgery, she had to deflate her band to allow a faster transit of food through her stomach, thus reaching a prompt euglycemic condition, due to an incoming hypoglycemic crisis.
  
  
  CONCLUSIONS
  Laparoscopic adjustable gastric banding (LAGB) is one of the most used approaches to treat morbid obesity. It is a restrictive procedure unable to affect the absorption of any nutrient, presenting a very low intra and perioperative complication rate. In our GSD IXa patient, it offered a prompt modification of food intake restriction whenever requested, thus avoiding hypoglycemia.
  
  
  CONCLUSIONS
  LAGB is effective in determining weight loss without inducing significant side effects or worsening hypoglycemia, in this morbid obese patient, suffering from GSD type IXa.
authors.affiliation: Advanced Biomedical Sciences Department - General Surgery, "Federico II" University, Naples, Italy. Electronic address: mario.musella@unina.it.
authors.forename: Mario
authors.initials: M
authors.lastname: Musella
authors.affiliation: Advanced Biomedical Sciences Department - General Surgery, "Federico II" University, Naples, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Milone
authors.affiliation: Advanced Biomedical Sciences Department - General Surgery, "Federico II" University, Naples, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Maietta
authors.affiliation: Advanced Biomedical Sciences Department - General Surgery, "Federico II" University, Naples, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Bianco
authors.affiliation: Advanced Biomedical Sciences Department - General Surgery, "Federico II" University, Naples, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Pisapia
authors.affiliation: Advanced Biomedical Sciences Department - General Surgery, "Federico II" University, Naples, Italy.
authors.forename: Dario
authors.initials: D
authors.lastname: Gaudioso
authors.affiliation: Advanced Biomedical Sciences Department - General Surgery, "Federico II" University, Naples, Italy.
authors.forename: Rubina
authors.initials: R
authors.lastname: Palumbo
keywords: Bariatric surgery; GSD IXa; Glucose storage disease type IXa; Laparoscopic adjustable gastric banding (LAGB); Morbid obesity