Pelvis-shoulder dysplasia

Pelvis-shoulder dysplasia is a rare focal skeletal dysostosis. We present the long-term follow-up of a patient with this condition. This patient has severe pelvic dysplasia but no involvement of the scapulae or clavicles. Despite the severity of the pelvic dysplasia, this man is able to function well. This is the fifth case of pelvis-shoulder dysplasia reported, but the only one documenting follow-up into adulthood. Received: 16 July 1997 Accepted: 4 February 1998     

Synthesis and Structure-Activity Relationships of Vasicine Analogues as Bronchodilatory Agents

The series of vasicine (1) analogues, an alkaloid from Adhatoda vasica Nees., were synthesized with changes in A, B or C rings. Compounds 13-19 were evaluated for in vitro bronchodilatory activity using isolated guinea pig tracheal chain. Compounds 3-8 were also synthesized in good yields using microwave-mediated synthesis under solvent free conditions. Compounds 5 and 8 with seven-member C ring were more active than etofylline and caused 100% relaxation of both the histamine and acetycholine pre-contracted guinea pig tracheal chain. The structure-activity relationship studies showed that the quinazoline and oxo functionalities were essential for activity. The compounds without C ring and instead having aliphatic and phenyl substitutions in B ring showed relaxation against histamine pre-contracted tracheal chain only, 2-methyl substituted analogues, 12 and 13, being most active with 100% relaxation effect.     

Effect of Zr and Ti Addition and Aging Treatment on the Microstructure and Tensile Properties of Al-2%Cu-Based Alloys

The present work investigated the effect of aging treatment on the microstructure and tensile properties of an Al-2%Cu base alloy containing various additions of Zr and other alloying elements. Aging was carried out at temperatures of 180–300 °C for different aging times at each temperature. The tensile properties indicated that Zr additions improved the strength of the base alloy, especially at high Zr levels at 180 °C. At the 220 °C aging temperature, however, while Zr addition did not have a beneficial effect on the alloy strength, the ductility was found to improve. Zr-Ti combined additions had a significant effect on the microstructure of the base alloy, as the morphology of the α-Al grains transformed into a non-dendritic morphology, and the grain size decreased sharply. These effects were at their maximum at 180 °C and 0.5 wt% Zr addition. Moreover, the Zr-containing alloys aged at higher temperatures, such as 220 °C and 240 °C, maintained a noticeably higher level of strength over the base alloy aged at the same temperatures. Quality index charts based on the tensile test data also reflected an improvement in alloy quality and strength with Zr-Ti combined additions.     

IL-24 intrinsically regulates Th17 cell pathogenicity in mice

In certain instances, Th17 responses are associated with severe immunopathology. T cell–intrinsic mechanisms that restrict pathogenic effector functions have been described for type 1 and 2 responses but are less well studied for Th17 cells. Here, we report a cell-intrinsic feedback mechanism that controls the pathogenicity of Th17 cells. Th17 cells produce IL-24, which prompts them to secrete IL-10. The IL-10–inducing function of IL-24 is independent of the cell surface receptor of IL-24 on Th17 cells. Rather, IL-24 is recruited to the inner mitochondrial membrane, where it interacts with the NADH dehydrogenase (ubiquinone) 1 α subcomplex subunit 13 (also known as Grim19), a constituent of complex I of the respiratory chain. Together, Grim19 and IL-24 promote the accumulation of STAT3 in the mitochondrial compartment. We propose that IL-24–guided mitochondrial STAT3 constitutes a rheostat to blunt extensive STAT3 deflections in the nucleus, which might then contribute to a robust IL-10 response in Th17 cells and a restriction of immunopathology in experimental autoimmune encephalomyelitis.     

Evaluation of Dietary Intake and Anthropometric Status in 1–9-Year-Old Children Living in Serbia: National Food Consumption Survey according to the EU Menu Methodology

The Serbian Food Consumption Survey among 1–9-year-old-children was conceptualized and conducted in compliance with the principles, established protocols, and guidelines of the EU Menu project between 2017 and 2021. Valid data were collected for 576 individuals (290 1–3-year-old toddlers and 276 3–9-year-old children). Regardless of age and gender category, the majority (68.80%) of children had normal weights according to the Body Mass Index-for-age classification system. The median daily energy intake was 1406.71 kcal with no differences between the settlement types. The overall median contributions of carbohydrates, protein, and fat to the total energy intake were 47.54%, 14.06%, and 37.88%, respectively. The proportions of the macronutrient intake deviated from the dietary reference values with compliance to the recommendations being particularly poor for fat and fiber. The consumption of energy-dense food groups such as meat and meat products, fat and oil, sugar, and confections was more pronounced among older children. The survey results provide a valuable insight into the nutritional status and dietary habits of toddlers and children 1–9 years old living in Serbia. They may serve as an evidence platform for public health programs, a valuable asset for decision-makers, and a reliable reference to guide nutritional policies, diet monitoring, and interventions targeting this population group in the future.     

Concentration Dependent Effect of (−)‐Epicatechin on Na+/K+‐ATPase and Ca2+‐ATPase Inhibition Induced by Free Radicals in Hypertensive Patients: Comparison with L‐ascorbic Acid

Although the antioxidant properties of flavonoids are well documented, it is still unclear whether these effects are dependent on radical scavenging or iron chelating activities. Oxidative stress, a state of excessive reactive oxygen species (ROS) activity, is associated with vascular disease conditions such as hypertension. Both the anti‐ and pro‐oxidant effects of tea catechins have been implicated in the alterations of cellular functions that determine their chemoprotective and therapeutic potentials in health and diseases. The present study examined the concentration dependent (10^?7 to 10^?4 m ) effects of (?)‐epicatechin and L‐ascorbic acid on Na⁺/K⁺‐ATPase and Ca²⁺‐ATPase activity in hypertensive patients and normal subjects. L‐ascorbic acid has been used as a positive control to compare the effect of (?)‐epicatechin. A significant (p < 0.0001) decrease in the activities of Na⁺/K⁺‐ATPase and Ca²⁺‐ATPase was observed in hypertensive patients compared with normal subjects. We report that (?)‐epicatechin shows a significant (p < 0.001) dose‐dependent protective effect against oxidative stress induced by tert‐butyl hydroperoxide (t‐BHP), which is manisfested as a decrease in the activity of erythrocyte Na⁺/K⁺‐ATPase and Ca²⁺‐ATPase, in hypertensive patients as well as normal subjects. The effect of L‐ascorbic acid was also significant (p < 0.001) and was comparable with that of (?)‐epicatechin. Copyright © 2012 John Wiley & Sons, Ltd.     

Experiences in the perinatal management of congenital diaphragmatic hernia during the last 15 years in a tertiary referral institute

OBJECTIVE: Review of cases of perinatally diagnosed congenital diaphragmatic hernias with special regard to time of diagnosis, organs herniated into the thorax, associated malformations, and outcomes. METHODS: We analyzed the data of 106 cases between July 1, 1990, and June 30, 2005. The observation period was analyzed in two parts. RESULTS: Prenatal ultrasound was performed in 89.6% (95/106) of the cases. 51.7% (46/89) of the pre- and postnatally verified congenital diaphragmatic hernia cases were diagnosed before the 24th week of gestation. 11% (11/100) of the hernias were identified postnatally. 71% (71/100) of the cases were associated with other malformations. The hernia was on the left side in 86% (86/100) and bilateral in 5% (5/100) of the cases. Between 1990 and 1997, the proportion of right-sided hernias was 4.3% (2/46), while during the second period it was 13% (7/54). 27.5% (14/51) of the newborns survived the perinatal period. The survival rate of the newborns delivered by caesarean section was three times higher than that of infants delivered vaginally. In the latter group, the rate of premature deliveries was considerably higher. The pregnancy was terminated in 45% (40/89) of the cases. 15% (6/40) of the terminated cases were isolated. Intrauterine or intrapartum deaths occurred in 5.6% (5/89) of the prenatally diagnosed cases. Simultaneous thoracic herniation of liver, spleen, or stomach decreased the survival rate. In these cases, the discharge rate was between 6 and 22%. CONCLUSIONS: According to the analysis of 15-year data, the herniated organs, multiple malformations, as well as the mode of delivery influence the survival in a large number of cases. Early diagnosis predicts a large hernia, a higher malformation rate, and a less favourable prognosis.     

Decision-making after ultrasound diagnosis of fetal abnormality

During the last few decades, the use of ultrasonography for the detection of fetal abnormalities has become widespread in many industrialised countries. This resulted in a shift in timing of the diagnosis of congenital abnormalities in infants from the neonatal period to the prenatal period. This has major implications for both clinicians and the couples involved. In case of ultrasound diagnosis of fetal anomaly, there are several options for the obstetric management, ranging from standard care to non-aggressive care and termination of pregnancy. This essay explores the context of both clinical and parental decision making after ultrasound diagnosis of fetal abnormality, with emphasis on the Dutch situation. While normal findings at ultrasound examination have strong beneficial psychological effects on the pregnant woman and her partner, the couple is often ill prepared for bad news about the health of their unborn child in the case of abnormal findings. This is, in particular, true in settings where ultrasonography for the detection of fetal abnormalities is offered as an integral part of antenatal care without appropriate counselling. An important question is to what extent the couple should be supported in decision making when a fetal abnormality is diagnosed. In this context, the parental perception of having a choice varies markedly. When parents consider end-of-life decisions, they experience both ambivalent and emotional feelings. On the one hand, they are committed to their pregnancy, while on the other hand, they want to protect their child, themselves and the family from the burden of severe disability. These complex parental reactions have implications for the counselling strategy.

Letrozole versus testosterone. a single-center pilot study of HIV-infected men who have sex with men on highly active anti-retroviral therapy (HAART) with hypoactive sexual desire disorder and raised estradiol levels

IntroductionSince the advent of Highly Active Anti-Retroviral Therapy (HAART), men with HIV experience good quality of life and expect to have normal sexual function. However, it appears that men infected with HIV commonly complain of sexual problems. There is evidence that men on HAART develop low sexual desire that is associated with raised estradiol levels. It has been postulated that abnormal metabolism seen in this group of men increases the aromatization of testosterone to estradiol. We hypothesized that letrozole, an aromatase inhibitor that inhibits the conversion of testosterone to estradiol, would be beneficial in these men.AimThe aim of this study was to compare the effects of testosterone vs. an aromatase inhibitor, letrazole, in HIV-infected men with raised estradiol and low sexual desire.MethodsThirteen men who have sex with men on HAART with low sexual desire as well as raised estradiol levels (>120 pmol/L) were randomly allocated to receive either parenteral testosterone (Sustanon 250 intramuscular injection) (N = 6) or letrozole 2.5 mg orally daily (N = 7) for 6 weeks.Main Outcome MeasuresSex steroid hormone assays, sex hormone-binding globulin, virological, hematological, and biochemical parameters were measured before and after treatment. Each subject was given the Spector Sexual Desire Inventory and the Depression/Anxiety Stress Scale before and immediately after treatment. Subjects were also asked to estimate the number of actual sexual acts before and after treatment.ResultsInventory data showed a rise in dyadic desire in both treatment arms. Mean actual sexual acts rose from 0.33 to 1.5 in the testosterone group and from 0.43 to 1.29 for the letrozole group. Luteinizing hormone increased in seven of seven men on letrozole. Serum testosterone increased in seven of seven men on letrozole. There were no adverse events from either medication.ConclusionLetrozole may be useful in the management of men on HAART who have low sexual desire. Richardson D, Goldmeier D, Frize G, Lamba H, De Souza C, Kocsis A, and Scullard G. Letrozole versus testosterone. A single-center pilot study of HIV-infected men who have sex with men on Highly Active Anti-Retroviral Therapy (HAART) with hypoactive sexual desire disorder and raised estradiol levels.