Normal bone density but altered geometry in girls with Turner syndrome.

OBJECTIVES: Data on bone density and stability in Turner syndrome (TS) are contradictory. A confounding factor for interpretation is short stature. The aim was to measure bone density, geometry and stability in girls with TS compared to idiopathic short stature (ISS). METHODS: From 1999 to 2008, 59 girls with TS (35 prepubertal) were evaluated by pQCT. Mean age was 8.9 in prepubertal and 17.3 years in adolescent girls. Mean height was -3.1 and -1.8 SDS in prepubertal treatment-free and in adolescent, formerly rhGH-treated girls. For comparison, 18 prepubertal ISS girls were studied (age 7.7 years; height -3.3 SDS). Examination of radius with pQCT (XCT 2000). Cortical (CD) and trabecular density (TD), total bone area (TBA), cortical area (CA), cortical thickness, muscle area and strength strain index (SSI) were determined and compared with height related references. RESULTS: In prepubertal girls with TS, TD and CD were normal (0.55 and 0.90 SDS) and comparable to ISS (0.95 and 1.53 SDS). TBA was greater in girls with TS than in ISS (0.87 vs. -0.33 SDS) whereas CA was similar (1.48 vs. 1.43 SDS). The SSI was comparable (1.61 vs. 1.56 SDS). Adolescent girls with TS showed similar results with a TD of 0.48 SDS, a CD of -0.32, TBA of 1.99, a CA of -0.05 and an SSI of 0.88 SDS. CONCLUSIONS: The observations are consistent with normal bone density and stability but altered bone geometry in prepubertal and substituted adolescent girls with TS. This peculiarity may reflect SHOX deficiency. We therefore think that timely and adequate estrogen substitution could prevent bone loss in TS.

Dysphagia after arteria lusoria dextra surgery: Anatomical considerations before redo-surgery.

Aberrant right subclavian artery (arteria lusoria) is the most common congenital root anomaly, remaining asymptomatic in most cases. Nevertheless, some of the 20%-40% of those affected present tracheo-esophageal symptoms. We report on a 6-year-old previously healthy girl presenting with progressive dysphagia over 4 wk. Diagnostics including barium swallow, echocardiography and magnetic resonance angiography (MRA) revealed a retro-esophageal compression by an aberrant right subclavian artery. Despite the successful, uneventful transposition of this arteria lusoria to the right common carotid via right-sided thoracotomy, the girl was suffering from persisting dysphagia. Another barium swallow showed the persistent compression of the esophagus on the level where the arteria lusoria had originated. As MRA showed no evidence of a significant re-obstruction by the transected vascular stump, we suspected a persisting ligamentum arteriosum. After a second surgical intervention via left-sided thoracotomy consisting of transecting the obviously persisting ligamentum and shortening the remaining arterial stump of the aberrant right subclavian artery, the patient recovered fully. In this case report we discuss the potential relevance of a persisting ligamentum arteriosum for patients with left aortic arch suffering from dysphagia lusoria and rational means of diagnosing, as well as the surgical options to prevent re-do surgery.

Expression and localization of growth hormone receptor in the oviduct of cyclic and pregnant pigs and mid-implantation conceptuses.

Previously it was shown that growth hormone (GH) and its receptor (GH-R) are involved in growth-promoting events during early embryonic development. However, it is still unknown if GH-induced GH-R signalling may support other functions within the oviduct. The purpose of our study was to analyse GH-R expression and localization in the porcine oviduct during different stages of the oestrus cycle and pregnancy (days 2-3 post inseminationem to days 65-71). As shown by reverse transcription polymerase chain reaction (RT-PCR), GH-R is expressed in the porcine oviduct during all stages of the oestrus cycle and pregnancy, respectively. Additionally, GH-R mRNA was detected in porcine conceptuses collected at day 18 of pregnancy. Using immunohistochemistry, GH-R was exclusively localized to the epithelium of the porcine oviduct throughout all segments examined. Localization of GH-R was mainly observed in the cytoplasm of ciliated epithelial cells. Generally, the number of GH-R-positive cells was elevated in the periovulatory phase of the oestrus cycle. Except for the isthmic epithelium, staining intensity of GH-R-positive cells was highest at oestrus and markedly reduced at met- and dioestrous stages. In infundibular and ampullar segments, percentage of GH-R-positive cells was significantly higher at days 2-3 post inseminationem compared to days 65-71 of pregnancy. Furthermore, in porcine conceptuses on day 18 of pregnancy GH-R protein expression was almost exclusively localized to trophectoderm. Our data suggest that GH-R mRNA and protein expression in the porcine oviduct throughout the oestrus cycle and pregnancy may suggest other activities of GH not described previously. Specifically, autocrine or paracrine GH-induced GH-R signalling may be linked to ciliated cell homeostasis of the porcine oviduct. Additionally, our results indicate that GH-R expression in the pig trophectoderm may be responsible for trophoblastic elongation.