Hypothalamic-pituitary-gonadal function in two infants with Smith-Lemli-Opitz syndrome

We report on the hypothalamic-pituitary-gonadal function in 2 male infants with the Smith-Lemli-Opitz (SLO or RSH) syndrome. Both infants had abnormal external genitalia. Basal and LHRH stimulated plasma gonadotropins were normal for age (1 month). Plasma testosterone, androstenedione, and dehydroepiandrosterone sulfate were normal for age and sex. Some forms of congenital adrenal hyperplasia (17,20-desmolase deficiency, 17α-hydroxylase deficiency, and 3β-hydroxysteroid dehydrogenase deficiency) were ruled out by hormonal studies. The endocrinological findings indicate a normal hypothalamic-pituitary-gonadal function and a normal adrenal steroid biosynthesis in these 2 patients. A partial androgen receptor defect causing the genital malformations seems possible in one patient. Whether 5α-reductase deficiency is the cause of the male pseudohermaphroditism in SLO syndrome remains the subject of future studies. © 1992 Wiley-Liss, Inc.     

Function of the supraspinatus muscle: Abduction of the humerus studied in cadavers

We evaluated the function of the supraspinatus tendon with a dynamic shoulder model. Active glenohumeral joint motion was simulated in 10 cadaveric shoulder specimens with hydrodynamic cylinder forces at the deltoid muscle and at the rotator cuff. Computerized regulation initiated standardized cycles of glenohumeral joint motion, where the isolated effect of the supraspinatus muscle could be studied. The efficacy of the supraspinatus muscle on elevation of the glenohumeral joint was measured with an ultrasonic sensor system. Pressures underneath the coracoa-cromial vault were recorded with capacitive sensors, as an indicator of the impingement at the shoulder. Elimination of force of the supraspinatus muscle led to a 6 percent decrease in elevation of the glenohumeral joint. The deltoid muscle was able to reverse this loss of elevation by a force increase of one third of the lost supraspinatus force. If no force was applied to the supraspinatus muscle, average pressures underneath the coracoacromial vault decreased 8 percent. It was concluded that the supraspinatus produces less torque and more glenohumeral joint compression than the deltoid. However, the supraspinatus has no effect on depression of the humeral head during elevation.

The clinical consequence of our observations is that operative closure of supraspinatus tendon defects is not mandatory.     

Über die Einwirkung von Säuredämpfen in westfälischen Metallbeizereien,Akkumulatoren- und Säurefabriken auf die Zähne der Arbeiter

Ohne ZusammenfassungDie einzelnen Befunde wurden auf Schwert-Karte DIN A 5 Nr. 24 988 festgelegt.Am Schlusse der Arbeit crlaube ich mir, dem Herrn Reichsarbeitsminister für die Bereitstellung der erforderlichen Mittel ergebenst zu danken.     

Musculoskeletal Ultrasound in Pediatric Trauma

Abstract Background and Purpose: The radiologic evaluation of musculoskeletal injuries in young children is difficult due to the absent ossification of several secondary ossification centers, which therefore cannot be seen directly on plain radiographs. When relying on conventional radiographs, even severe cartilaginous or soft tissue injuries cannot be visualized, as there is no possibility to differentiate them from bruises or sprains. It is the aim of this review to present clinically relevant applications of ultrasound imaging in pediatric musculoskeletal trauma. Methods: Currently used ultrasound techniques for evaluation of typical pediatric fractures or joint dislocations, techniques for differentiation between stable and unstable intraarticular fractures, the use of sonography for monitoring reduced bone length in long bones after fracture, sonographic guidelines for evaluation of tendon injuries or lesions, and sonographic imaging techniques in arthritis and osteomyelitis are described. Conclusion: Musculoskeletal sonography—despite its inability to view through intact cortical bone and its accuracy being dependent on skill and experience of the investigator—provides important information about soft tissue, bony or cartilaginous injuries in children.     

Inhalation of the Phosphodiesterase-3 Inhibitor Milrinone Attenuates Pulmonary Hypertension in a Rat Model of Congestive Heart Failure

Background: Most patients with congestive heart failure (CHF) develop pulmonary venous hypertension, but right ventricular afterload is frequently further elevated by increased pulmonary vascular resistance. To investigate whether inhalation of a vasodilatory phosphodiesterase-3 inhibitor may reverse this potentially detrimental process, the authors studied the effects of inhaled or intravenous milrinone on pulmonary and systemic hemodynamics in a rat model of CHF.

Methods: In male Sprague-Dawley rats, CHF was induced by supracoronary aortic banding, whereas sham-operated rats served as controls. Milrinone was administered as an intravenous infusion (0.2-1 [mu]g [middle dot] kg body weight-1 [middle dot] min-1) or by inhalation (0.2-5 mg/ml), and effects on pulmonary and systemic hemodynamics and lung water content were measured.

Results: In CHF rats, intravenous infusion of milrinone reduced both pulmonary and systemic arterial blood pressure. In contrast, inhalation of milrinone predominantly dilated pulmonary blood vessels, resulting in a reduced pulmonary-to-systemic vascular resistance ratio. Repeated milrinone inhalations in 20-min intervals caused a stable reduction of pulmonary artery pressure. No hemodynamic effects were detected when 0.9% NaCl was administered instead of milrinone or when milrinone was inhaled in sham-operated rats. No indications of potentially adverse effects of milrinone inhalation in CHF, such as left ventricular volume overload, were detected. Moreover, lung edema was significantly reduced by repeated milrinone inhalation.     

Liver resection without hilus preparation and with selective intrahepatic hilus stapling for benign tumors and liver metastasis

Background Nowadays, liver resection is a routine operative procedure in surgical centers, and strategies must be aimed at avoiding additional risk factors. Extrahepatic isolation of portal vein, hepatic artery and hepatic duct, as well as lymphadenectomy of the liver hilum are generally accepted steps of liver resection, even for metastatic and benign indications. Our primary aim was to analyze the feasibility, blood loss, blood transfusion requirements, incidence of complications, and outcome using the approach for intrahepatic devascularization leaving the extrahepatic hilus untouched. Materials and methods Thirty-eight consecutive patients with resection for metastases and benign liver tumors were selected. After hilar examination, the extrahepatic structures remain intact, and during parenchyma dissection, the whole right or left or the appropriate bi-segmental pedicle is isolated intrahepatically and then transected using a stapler device. Results The used technique was feasible in all cases, and no intra- or postoperative surgical complications were observed. To date, no tumor recurrence was found in the hilum during the follow-up period. Conclusion The intrahepatic pedicle stapling technique appears to be feasible and safe in liver resection. Hilar dissection can, thus, be avoided in liver metastasis and benign liver tumors.     

Anti-phospholipid and anti-DNA antibodies are not associated with the elevated release of circulatory fetal DNA in pregnancies affected by preeclampsia.

OBJECTIVES: We have previously shown that the levels of circulatory fetal DNA are elevated in preeclampsia and that these increases correspond to disease severity. Several reports have indicated that increased levels of antiphospholipid (anti-PL) and anti-DNA antibodies may be associated with preeclampsia, in particular with the severe forms of the disorder. Since the release of cell-free DNA by the placenta is attributed to some form of cell death or damage and as anti-PL and anti-double-stranded DNA (dsDNA) antibodies have been proposed to lead to placental damage, we have studied the relationship between these parameters in preeclampsia. METHODS: Circulating fetal DNA levels in samples taken from pregnant women with mild (n = 12) or severe (n = 12) preeclampsia and from normal pregnant controls (n = 35) were quantified using a Taqman real-time Polymerase Chain Reaction (PCR) assay. The Anti-PL antibodies (IgG and IgM) were assayed by anticardiolipin ELISA and by commercial anti-beta2-Glycoprotein I (GPI) ELISA kits. Anti-dsDNA antibodies (IgG and IgM) were analyzed by a commercially available anti-dsDNA ELISA kit. RESULTS: No correlation could be drawn with the quantity of circulatory fetal DNA in the samples analyzed and corresponding anti-PL or anti-dsDNA antibody levels. Furthermore, no significant difference existed between the levels of these antibodies in the two study groups and the control cohort. CONCLUSION: Our data suggest that the mechanism leading to the increased release of cell-free circulatory DNA from the placenta does not involve trophoblast damage mediated by these agents. Our analysis also questions the reported involvement of anti-PL and anti-DNA antibodies in preeclampsia.