Anthropometrics of Polish children with osteogenesis imperfecta: a single-centre retrospective cohort study.

BACKGROUND: Osteogenesis imperfecta (OI) causes a number of abnormalities in somatic development. The predominant symptoms are reduced bone mass and an increased risk of fractures as well as bone deformities and short stature. Due to the lack of causal treatment options, bisphosphonates are considered the gold standard of therapy. The aim of our study is to present selected anthropometric parameters (body weight, height, BMI) in children with type I and III of OI. METHODS: We performed a retrospective analysis of medical records of patients with osteogenesis imperfecta type I and III confirmed by genetic testing. The study group included individuals admitted to the Department in 2020. We analysed the anthropometric parameters of 108 children (receiving and not receiving bisphosphonates treatment). RESULTS: In the group of children with OI type I admitted for follow-up (group 1), the median weight percentile was 37, while in the group 2 it was 17. In the patients with OI type III (group 3), the median weight percentile was 0.1. The median height percentile in group 1 was 21, in group 2 it was 5, whereas in group 3 = 0.1. The differences in anthropometric measurements of the patients with OI type I and OI type III were statistically significant (p < 0.001). Among the analysed patients, an abnormal BMI was found in 41.67% of whom 37.78% were underweight, 48.89% were overweight and 13.33% were obese. CONCLUSION: Considering prevalence of the disease, it is not only low stature but also abnormal BMI, and especially excessive body weight, that play an important role in the somatic development disorder.

Adsorptive removal of fluoride using ionic liquid-functionalized chitosan - Equilibrium and mechanism studies.

In this study, novel biosorbents, based on chitosan and imidazolium ionic liquid, were prepared for the removal of fluoride from aqueous solutions. The adsorbents were characterized by FTIR, SEM-EDS and low-temperature nitrogen adsorption-desorption. To investigate the adsorption mechanism and behavior of chitosan adsorbents, batch experiments were conducted under different adsorbent dosages (2, 4, 10 g/L), pH (4, 7, 9) and initial concentration (0.5-25.0 mg/L). The influence of the method of synthesis of ionic liquid on the adsorption performance were also studied. Experimental data were evaluated by Freundlich, Langmuir and Sips models. The introduction of ionic liquid significantly improved the uptake of fluoride compared to pure chitosan. The adsorption was influenced by the experimental conditions, as well as the method of ionic liquid synthesis. The highest fluoride removal was observed at pH 4 and found to decrease with increasing pH. The removal efficiency and adsorption capacity values indicated that the dose of 4 g/L was the optimum adsorbent dosage. The equilibrium data fitted best with the Sips isotherm and the maximum adsorption capacity reached 8.068 mg/g for modified chitosan beads. The mechanism of fluoride adsorption onto ionic liquid-modified chitosan involves electrostatic attraction, ion exchange and ion pair interaction.

Effects of individual versus group housing system during the weaning-to-estrus interval on reproductive performance of sows.

Selection of appropriate housing conditions for sows is critical for their physical health and long-term reproductive success. The present objective was to evaluate the influences of housing system postweaning (i.e., individual stalls (IS) or group pens (GP)), season and parity on piglet productivity of sows in a commercial setting. This study utilized 3 053 Polish Large White×Polish Landrace sows that were weaned at a rate of 20-30 animals per week at the median age of 4 weeks; 1 474 sows were moved into GP of seven to eight animals each, while 1 579 were placed in IS after weaning. Starting 2 days postweaning all animals were checked for estrus with a teaser boar and then artificially inseminated using 3×109 spermatozoa per dose of an inseminate at the onset of heat and 24 h later. The proportion of sows showing the signs of standing heat at or before 6 days postweaning was greater (P < 0.05) for sows moved to GP compared with IS; this difference manifested mainly in second parity sows weaned in the summer and fall. Conception and farrowing rates were significantly higher (P < 0.01) and the weaning-to-estrus interval shorter in GP compared with IS sows in every season but autumn. Mean litter size was lower (P < 0.05) in IS groups in summer, autumn, and winter, and the number of live-born piglets/sow was lower (P < 0.05) for IS sows in the summer and fall. Beneficial effects of group housing on piglet productivity manifested up until the seventh consecutive farrowing and then began to wane. In summary, there was a significantly greater proportion of sows going estrus "on time" (i.e., <7 days) in group housing compared to single stalls but this effect was confined to the second parity sows during the summer and fall months; these results suggest the existence of a seasonal and age-related aspect to sow fertility worthy of further investigation. While both housing systems have their pros and cons, our present results indicate that, in commercial settings, group housing postweaning improved nearly all reproductive parameters of sows.

The influence of azaperone treatment at weaning on reproductive function in sows: ovarian activity and endocrine profiles during the weaning-to-ovulation interval.

Azaperone can reduce stress caused by weaning and relocation of breeding sows, but its effects on reproductive processes are still poorly understood. The primary aim of this study was to describe and compare the endocrine and ovarian activity in ultrasonographically monitored second parity sows, with or without azaperone treatment at weaning (2 mg/kg BW i.m.). The intervals from weaning to the onset of estrus and ovulation were both greater (P<0.05) in azaperone-treated (n=12) than in control sows (n=12) by ~12 h. Mean daily growth rates of identified antral follicles were less (P<0.05) in azaperone-treated than in control sows (1.08±0.17 v.1.23±0.18 mm/day; mean±SD) and treated animals exceeded (P<0.05) controls in the mean ovulation rate (13.7±1.3 v. 12.6±1.2). A transient suppression of cortisol release was observed in the treatment group (at 10 and 30 min after azaperone injections) but circulating cortisol concentrations were variable in both groups of sows for the remainder of the study. The preovulatory rise in LH and estradiol secretion was delayed (P<0.05), and the duration of the LH surge was greater (P<0.001) in azaperone-treated sows compared with their control counterparts. The amplitude of episodic fluctuations in serum cortisol concentrations was correlated with the number of stillborn piglets in control sows (r=0.63, P=0.04). The amplitude and concentration of the preovulatory rise in estradiol secretion were negatively correlated with ovulatory response and litter size (r=-0.63 to -0.82, P<0.05), whereas the time at which the LH surge ended was directly related to the number of live-born piglets (r=0.82, P=0.002) in azaperone-treated animals. The present results indicate that administration of azaperone at weaning had a profound effect on preovulatory LH secretion as well as growth kinetics and estrogenicity of ovarian antral follicles. However, the causative associations among various characteristics of the preovulatory LH discharge, ovarian and adrenal steroid secretion post-weaning, and reproductive variables in sows remain equivocal.

The influence of azaperone treatment at weaning on reproductive performance of sows: altering effects of season and parity.

Azaperone treatment can control aggression and decrease stress due to weaning, re-grouping and hierarchical fighting of gilts and sows. However, the effects of this butyrophenone neuroleptic and sedative administered at weaning on pig reproductive function are poorly characterized. In this year-long study, a total of 619 cross-bred sows (Polish Large White×Polish Landrace) kept on a commercial farm received an i.m. injection of azaperone (Stresnil®; 2 mg/kg BW) just before weaning and were artificially inseminated during the ensuing estrus with 3×109 spermatozoa per dose of an inseminate; 1180 sows served as untreated controls. Immediately after weaning, the sows were moved to four pens of seven to nine animals each. A teaser boar was used twice daily to check for estrus and sows were bred at heat detection. Subsequently, all sows stayed in individual stalls until pregnancy testing on day 30 post-artificial insemination and were then re-grouped until farrowing. The proportion of pigs that were in estrus within 6 days post-weaning was significantly lower in azaperone-treated groups of animals than in controls (71.4% v. 84.2%). Overall, the azaperone-treated sows had a significantly longer weaning-to-estrus interval (WEI; 8.7±10.1 v. 6.3±8.1 days; mean±SD) and a significantly larger litter size (LS: 11.8±3.0 v.11.3±3.2; azaperone-treated v. control sows). Treatment of the winter-farrowing sows was associated with increased LS (12.8±2.6 and 11.3±3.1 piglets/sow, respectively; P<0.05) and longer (P<0.05) weaning-to-effective-service intervals (11.7±19.3 and 8.4±12.3 days, respectively) as well as farrowing intervals (155.7±19.7 and 152.2±16.1 days, respectively) compared with untreated controls. In the summer months, significantly longer WEIs (12.1±21.0 v. 8.4±16.9 days) were accompanied by a significant decline in LS only in azaperone-treated sows that were inseminated within 6 days post-weaning (10.8±2.9 v. 11.5±3.3 piglets/sow; azaperone-treated v. controls). Azaperone-treated second parity sows had greater LS (P<0.001) along with prolonged WEIs (P<0.05) in comparison to their respective controls, regardless of the timing of estrus. An application of azaperone at weaning increased the annual piglet productivity of winter-farrowing animals and of second parity sows but depressed it significantly in summer. The extra cost and labor due to delayed onset of estrus may cancel out any reproductive benefits of azaperone treatment.

Penetrating neck injuries: a guide to evaluation and management.

Introduction Penetrating neck injury is a relatively uncommon trauma presentation with the potential for significant morbidity and possible mortality. There are no international consensus guidelines on penetrating neck injury management and published reviews tend to focus on traditional zonal approaches. Recent improvements in imaging modalities have altered the way in which penetrating neck injuries are now best approached with a more conservative stance. A literature review was completed to provide clinicians with a current practice guideline for evaluation and management of penetrating neck injuries. Methods A comprehensive MEDLINE (PubMed) literature search was conducted using the search terms 'penetrating neck injury', 'penetrating neck trauma', 'management', 'guidelines' and approach. All articles in English were considered. Articles with only limited relevance to the review were subsequently discarded. All other articles which had clear relevance concerning the epidemiology, clinical features and surgical management of penetrating neck injuries were included. Results After initial resuscitation with Advanced Trauma Life Support principles, penetrating neck injury management depends on whether the patient is stable or unstable on clinical evaluation. Patients whose condition is unstable should undergo immediate operative exploration. Patients whose condition is stable who lack hard signs should undergo multidetector helical computed tomography with angiography for evaluation of the injury, regardless of the zone of injury. Conclusions The 'no zonal approach' to penetrating neck trauma is a selective approach with superior patient outcomes in comparison with traditional management principles. We present an evidence-based, algorithmic and practical guide for clinicians to use when assessing and managing penetrating neck injury.

Influence of coral cover and structural complexity on the accuracy of visual surveys of coral-reef fish communities.

Using manipulated patch reefs with combinations of varying live-coral cover (low, medium and high) and structural complexity (low and high), common community metrics (abundance, diversity, richness and community composition) collected through standard underwater visual census techniques were compared with exhaustive collections using a fish anaesthetic (clove oil). This study showed that reef condition did not influence underwater visual census estimates at a community level, but reef condition can influence the detectability of some small and cryptic species and this may be exacerbated if surveys are conducted on a larger scale.

The impact of implantation technique on frontal and sagittal alignment in total lumbar disc replacement: a comparison of anterior versus oblique implantation.

The concept of total lumbar disc replacement (TDR) is gaining acceptance due to good clinical short-term outcome. Standard implantation is strict anterior, which poses especially above the segment L5/S1 sometimes difficulties due to the vessel configuration. Therefore, oblique implantable TDR have been invented. In oblique implantation the anterior longitudinal ligament (ALL) is only partially resected, with additional partial resection of lateral annulus fibers. This could have an impact on biomechanical properties, which has not been evaluated until now. We therefore compared the standing ap and lateral X-rays pre- and postoperative after anterior and oblique implantation of TDR in segment L4/5. Significant differences between the groups were not found. In both the anterior and oblique group, segmental lordosis showed a significant increase, whereas total lordosis as well as ap balance were unchanged. The absolute segmental lordosis increase was nearly double in the anterior group. In conclusion, both anterior and oblique implanted TDR significantly increase segmental lordosis while retaining total lordosis and ap balance. The segmental increase is lower in the oblique implanted group which is probably due to the remaining ALL. Further studies should evaluate whether this finding has any implication for the long-term outcome.

Effect of parenteral cladribine on relapse rates in patients with relapsing forms of multiple sclerosis: results of a 2-year, double-blind, placebo-controlled, crossover study.

OBJECTIVE: This randomized, 2-year, double-blind, placebo-controlled, crossover study evaluated cladribine for relapsing forms of multiple sclerosis. PATIENTS: (n = 84) received seven 5-day courses of subcutaneous cladribine at 5 mg/day (group A) or placebo (group B) in year 1; treatment was reversed in year 2. RESULTS: Cladribine was well tolerated and associated with a favorable safety profile. Mean Expanded Disability Status Scale scores remained stable. In group A, mean relapse rates were 0.15 in year 1 (cladribine) and 0.42 in year 2. In group B, relapse rates were 0.61 in year 1 and 0.50 in year 2 (cladribine). PATIENTS required fewer steroid courses during cladribine periods. The therapeutic efficacy of cladribine was associated with a sustained reduction in lymphocyte count.

A new view of patterning domains in the vertebrate mesoderm.

The musculoskeletal system of vertebrates is derived from the embryonic mesoderm. Its structures are categorized as epaxial or hypaxial based on their adult position and innervation. The epaxial/hypaxial terminology is also used to describe regions of the embryonic somites based on fate mapping of somitic derivatives. However, the adult, functional distinctions are not fully consistent with the changing embryonic environments of mesodermal populations during morphogenesis, and the traditional terminology loses accuracy when used to describe certain mutant phenotypes. Here we describe a new terminology naming two mesodermal environments defined by the lineage of the included cells. We discuss how mutant phenotypes may be better explained by consideration of the embryonic context in which genes take their effect and argue that the recognition of these embryonic territories clarifies description and discussion of the morphogenesis and patterning of the musculoskeletal system.

[Disorders of the bone density in the chest x-ray examination in the course of myelosclerosis]. / Zaburzenia utkania kostnego w obrazie radiologicznym klatki piersiowej w przebiegu zwlóknienia szpiku.

The authors report a rare case of myelosclerosis diagnosed during hospital stay in internal Department on account of hepatosplenomegaly and anaemia. The case of patient described by authors was diagnosed on the ground of examination and executed additional investigations: morphology, peripheral blood picture, chest radiography, scintiscan of skeleton.

Assembly of trunk and limb blood vessels involves extensive migration and vasculogenesis of somite-derived angioblasts.

Vascular development requires the assembly of precursor cells into blood vessels, but how embryonic vessels are assembled is not well understood. To determine how vascular cells migrate and assemble into vessels of the trunk and limb, marked somite-derived angioblasts were followed in developing embryos. Injection of avian somites with the cell-tracker DiI showed that somite-derived angioblasts in unperturbed embryos migrated extensively and contributed to trunk and limb vessels. Mouse-avian chimeras with mouse presomitic mesoderm grafts had graft-derived endothelial cells in blood vessels at significant distances from the graft, indicating that mouse angioblasts migrated extensively in avian hosts. Mouse graft-derived endothelial cells were consistently found in trunk vessels, such as the perineural vascular plexus, the cardinal vein, and presumptive intersomitic vessels, as well as in vessels of the limb and kidney rudiment. This reproducible pattern of graft colonization suggests that avian vascular patterning cues for trunk and limb vessels are recognized by mammalian somitic angioblasts. Mouse-quail chimeras stained with both the quail vascular marker QH1 and the mouse vascular marker PECAM-1 had finely chimeric vessels, with graft-derived mouse cells interdigitated with quail vascular cells in most vascular beds colonized by graft cells. Thus, diverse trunk and limb blood vessels have endothelial cells that developed from migratory somitic angioblasts, and assembly of these vessels is likely to have a large vasculogenic component.

[Foreign body in the right frontal lobe of the brain as a result of right eyeball and right eye-socket trauma--case report]. / Cialo obce w prawym placie czolowym mózgu w nastepstwie urazu prawej galki ocznej i oczodolu prawego--opis przypadku.

We report a case of a 44 year old man, who was admitted to our hospital because of right eyeball and right eye-socket due to a piece of diamond circular saw. Right eyeball was sutured. CT--examination showed a metallic foreign body in the right frontal lobe of brain. Metallic foreign body, which damaged the right eyeball, caused upper restriction of right eye-socket ceiling and stuck in a frontal lobe of the brain was removed.

Hox genes and morphological identity: axial versus lateral patterning in the vertebrate mesoderm.

The successful organization of the vertebrate body requires that local information in the embryo be translated into a functional, global pattern. Somite cells form the bulk of the musculoskeletal system. Heterotopic transplants of segmental plate along the axis from quail to chick were performed to test the correlation between autonomous morphological patterning and Hox gene expression in somite subpopulations. The data presented strengthen the correlation of Hox gene expression with axial specification and focus on the significance of Hox genes in specific derivatives of the somites. We have defined two anatomical compartments of the body based on the embryonic origin of the cells making up contributing structures: the dorsal compartment, formed from purely somitic cell populations; and the ventral compartment comprising cells from somites and lateral plate. The boundary between these anatomical compartments is termed the somitic frontier. Somitic tissue transplanted between axial levels retains both original Hox expression and morphological identity in the dorsal compartment. In contrast, migrating lateral somitic cells crossing the somitic frontier do not maintain donor Hox expression but apparently adopt the Hox expression of the lateral plate and participate in the morphology appropriate to the host level. Dorsal and ventral compartments, as defined here, have relevance for experimental manipulations that influence somite cell behavior. The correlation of Hox expression profiles and patterning behavior of cells in these two compartments supports the hypothesis of independent Hox codes in paraxial and lateral plate mesoderm.

[Thoracic aortic dissecting aneurysm with bleeding into the pleural cavity]. / Tetniak rozwarstwiajacy aorty piersiowej z krwawieniem do jamy oplucnej.

A rare case of dissecting aneurysm of the aortic arch with left haemothorax was presented. The authors describe modern methods of visualising diagnostics used in case of suspected dissecting aortic aneurysm.

[Distal transfer of the greater trochanter among other surgical procedures for the treatment of hip deformity]. / Obnizenie kretarza wiekszego wsród innych metod leczenia operacyjnego deformacji biodra.

Distal greater trochanteric transfer is a rarely used procedure for treatment of hip deformity. Surgical technique is described. Potential for changing morphology of proximal end of the femur is underlined and pitfalls indicated. The main advantage of the procedure is reconstruction of the femoral neck. It is biomechanically more sound than valgus osteotomy done frequently in similar cases. Advantages of trochanteric transfer are confirmed by other authors who after this procedure achieved clinical and radiological improvement in patients with degenerative disease of the hip.

The value of distal greater trochanteric transfer in the treatment of deformity of the proximal femur owing to avascular necrosis.

A series of 49 patients (55 hips) who had distal greater trochanteric transfer at the mean age of 13.9 years because of the deformity of the proximal femur after treatment for developmental dislocation of the hip was reviewed to verify the value of the procedure. Mean follow-up was 8.8 years. Clinical and radiologic assessment was supplemented with strain-gauging evaluation of the muscles involved. Good results were achieved in patients who had good range of movement or isolated restriction of abduction before the operation. After surgery, a 22% increase of abductors torque was found, the Trendelenburg sign disappeared in 30 individuals, and 15 patients regained normal gait. No complications were observed. Our study confirmed the value of distal transfer of the greater trochanter in treatment for proximal femur deformity owing to avascular necrosis after management of developmental dislocation of the hip and clarified indications for this procedure.

Up-regulation of genes involved in cellular stress and apoptosis in a rat model of hippocampal degeneration.

Changes in gene expression within the hippocampus induced by denervation after electrolytic fimbria-fornix lesion in rat were compared to morphological and biochemical alterations. Fimbria-fornix lesion results in degeneration of hippocampal cholinergic terminals as evidenced by a sustained (2 days to 1 month) decrease in cholineacetyltransferase (ChAT) activity (50%). These changes were accompanied by a decrease in growth associated protein 43 (GAP-43) immunoreactivity in all hippocampal layers 4 days after lesion followed by a subsequent increase and return to normal levels by 20 days postinjury. This increase in GAP-43 expression in the hippocampus between 7 to 20 days after lesion may reflect heterotypic sprouting. TUNEL-positive cells were revealed by in situ assay within the hippocampus at 10 days, but not at 3 days, after lesion. Two subtracted cDNA libraries from the dorsal hippocampus of control and injured rats (at 3 and 10 days postlesion) were constructed in order to search for new genes potentially implicated in degeneration/regeneration phenomena. We analysed 1,536 clones from each library by differential screening and found a total of 46 up-regulated genes. Among the 15 known genes, 6 coded for proteins involved in signal transduction pathways. The upregulation of growth arrest DNA damage induced gene (GADD153), brain-specific RING finger protein, JNK interacting protein (JIP-1), protein kinase A (PKA), and Na+K+ ATPase was studied by quantitative polymerase chain reaction (PCR). Two of these genes, GADD153 and JIP-1, have been previously shown to participate in cell modifications induced by stress and apoptosis.