Biomaterial Database

Failure of biliopancreatic diversion in Prader-Willi syndrome. 2000

G Grugni, and G Guzzaloni, and F Morabito

Department of Auxology, IRCCS S. Giuseppe Hospital, Istituto Auxologico Italiano Foundation, Verbania, 28921 Italy. gragru@jumpy.it

BACKGROUND Prader-Willi syndrome (PWS) is the most common genetic obesity. Excessive weight gain follows failure-to-thrive in early infancy; in adolescents and young adults, excess body weight can exceed 100%. The hyperphagia associated with PWS is responsible for the early mortality. Dietary restriction, alone or combined with anorexic drugs, are ineffective to induce a permanent weight loss. Thus, surgical treatment of morbid obesity in PWS has been attempted, but gastric restrictive operations are unable to produce stable weight loss. In a small number of patients, favorable results have been reported with biliopancreatic diversion (BPD). METHODS A 24-year-old woman with PWS, Pickwickian, at age 21 weighed 80 kg (BMI= 50) and underwent BPD. RESULTS 3 years after the BPD she regained 21 of the 26 kg lost; somnolence and respiratory difficulties were the same as before surgery. The patient now presents severe reduction of bone mass density, hypochromic anemia, hypoproteinemia, and diarrhea associated with eating. CONCLUSIONS The regain of weight following BPD suggests that this procedure alone is not adequate for long-term control of obesity in PWS.

UI	MeSH Term	Description	Entries
D007413	Intestinal Mucosa	Lining of the INTESTINES, consisting of an inner EPITHELIUM, a middle LAMINA PROPRIA, and an outer MUSCULARIS MUCOSAE. In the SMALL INTESTINE, the mucosa is characterized by a series of folds and abundance of absorptive cells (ENTEROCYTES) with MICROVILLI.	Intestinal Epithelium,Intestinal Glands,Epithelium, Intestinal,Gland, Intestinal,Glands, Intestinal,Intestinal Gland,Mucosa, Intestinal
D009767	Obesity, Morbid	The condition of weighing two, three, or more times the ideal weight, so called because it is associated with many serious and life-threatening disorders. In the BODY MASS INDEX, morbid obesity is defined as having a BMI greater than 40.0 kg/m2.	Morbid Obesity,Obesity, Severe,Morbid Obesities,Obesities, Morbid,Obesities, Severe,Severe Obesities,Severe Obesity
D011218	Prader-Willi Syndrome	An autosomal dominant disorder caused by deletion of the proximal long arm of the paternal chromosome 15 (15q11-q13) or by inheritance of both of the pair of chromosomes 15 from the mother (UNIPARENTAL DISOMY) which are imprinted (GENETIC IMPRINTING) and hence silenced. Clinical manifestations include MENTAL RETARDATION; MUSCULAR HYPOTONIA; HYPERPHAGIA; OBESITY; short stature; HYPOGONADISM; STRABISMUS; and HYPERSOMNOLENCE. (Menkes, Textbook of Child Neurology, 5th ed, p229)	Labhart-Willi Syndrome,Royer Syndrome,Labhart-Willi-Prader-Fanconi Syndrome,Prader Labhart Willi Syndrome,Prader-Labhart-Willi Syndrome,Royer's Syndrome,Willi-Prader Syndrome,Labhart Willi Prader Fanconi Syndrome,Labhart Willi Syndrome,Prader Willi Syndrome,Royers Syndrome,Syndrome, Labhart-Willi,Syndrome, Labhart-Willi-Prader-Fanconi,Syndrome, Prader-Labhart-Willi,Syndrome, Prader-Willi,Syndrome, Royer,Syndrome, Royer's,Syndrome, Willi-Prader,Willi Prader Syndrome
D004734	Energy Metabolism	The chemical reactions involved in the production and utilization of various forms of energy in cells.	Bioenergetics,Energy Expenditure,Bioenergetic,Energy Expenditures,Energy Metabolisms,Expenditure, Energy,Expenditures, Energy,Metabolism, Energy,Metabolisms, Energy
D005260	Female		Females
D005500	Follow-Up Studies	Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease.	Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D015904	Biliopancreatic Diversion	A surgical procedure which diverts pancreatobiliary secretions via the duodenum and the jejunum into the colon, the remaining small intestine being anastomosed to the stomach after antrectomy. The procedure produces less diarrhea than does jejunoileal bypass.	Biliopancreatic Bypass,Bilio Pancreatic Bypasses,Bilio Pancreatic Diversions,Bilio-Pancreatic Bypass,Bilio-Pancreatic Diversion,Bilio Pancreatic Bypass,Bilio Pancreatic Diversion,Bilio-Pancreatic Bypasses,Bilio-Pancreatic Diversions,Biliopancreatic Bypasses,Biliopancreatic Diversions,Bypass, Bilio-Pancreatic,Bypass, Biliopancreatic,Bypasses, Bilio Pancreatic,Bypasses, Bilio-Pancreatic,Bypasses, Biliopancreatic,Diversion, Bilio Pancreatic,Diversion, Bilio-Pancreatic,Diversions, Bilio Pancreatic,Diversions, Bilio-Pancreatic,Diversions, Biliopancreatic,Pancreatic Bypasses, Bilio,Pancreatic Diversion, Bilio,Pancreatic Diversions, Bilio
D017211	Treatment Failure	A measure of the quality of health care by assessment of unsuccessful results of management and procedures used in combating disease, in individual cases or series.	Failure, Treatment,Failures, Treatment,Treatment Failures