Two years of growth hormone treatment in adults with Prader-Willi syndrome do not improve the low BMD.

BACKGROUND: Bone mineral density (BMD) in adult patients with Prader-Willi syndrome (PWS) might be low due to high bone turnover. OBJECTIVES: The objective of the study was to investigate bone mass in a group of adult PWS subjects and study the effects of GH treatment on BMD and markers of bone turnover. DESIGN: Forty-six adults with genetically verified PWS were randomized to GH or placebo for 12 months, followed by open prospective GH for 24 additional months. BMD at the lumbar spine (LS) L1-4, the total hip, and the total body was assessed by dual-energy x-ray absorptiometry at baseline and every 12th month thereafter. Markers of bone turnover were measured at baseline and at the end of the controlled study. RESULTS: In this cohort of adult subjects with PWS, baseline BMD was reduced in all compartments compared with the reference (Z-scores). Men had lower Z-scores BMD than women in LS and total body (P < .05). With 12 months of GH, LS-BMD was significantly reduced compared with placebo. No changes in BMD were observed with continuous GH treatment for 24 months. The bone formation markers increased with GH therapy compared with placebo, whereas the resorption marker did not change. CONCLUSIONS: Adult PWS subjects, especially the men, have low bone mass that was not improved with GH treatment for 2 years. Because PWS subjects are short, BMD might be underestimated and should be adjusted for. Further studies, with adequate GH and sex hormone replacement throughout puberty and early adult life, are needed to better characterize PWS.

[Duplication of the small intestine. Unusual cause of abdominal pain]. / Duplikasjon av tynntarm. Uvanlig årsak til magesmerter.

Duplications of the alimentary tract are rare. Since 1982 only three patients with such anomalies have been treated in the Department of Paediatric Surgery at the University Hospital in Trondheim, Norway. We describe the last patient treated, where duplication of the ileum was found upon laparotomy, performed because of exaggerated recurrent abdominal pain. Duplications may be found anywhere along the alimentary tract and diagnosis can be difficult. The duplications can be found upon laparotomy for suspected appendicitis or bowel obstruction. The condition may also be suspected upon antenatal ultrasonography and in the event of abdominal symptoms after birth.