Davenportreed8387

The outcomes of animal experiments can be influenced by a variety of factors. Thus, precise reporting is necessary to provide reliable and reproducible data. Initiatives such as the ARRIVE guidelines have been enrolled during the last decade to provide a road map for sufficient reporting. To understand the sophisticated process of bone regeneration and to develop new therapeutic strategies, small rodents, especially mice, are frequently used in bone healing research. Since many factors might influence the results from those studies, we performed a systematic literature search from 2010 to 2019 to identify studies involving mouse femoral fracture models (stable fixation) and evaluated the reporting of general and model-specific experimental details. 254 pre-selected publications were systematically analyzed, showing a high reporting accuracy for the used mouse strain, the age or developmental stage and sex of mice as well as model-specific information on fixation methods and fracturing procedures. However, reporting was more often insufficient in terms of mouse substrains and genetic backgrounds of genetically modified mice, body weight, hygiene monitoring/immune status of the animal, anesthesia, and analgesia. Consistent and reliable reporting of experimental variables in mouse fracture surgeries will improve scientific quality, enhance animal welfare, and foster translation into the clinic.

Hypophosphatasia, a genetic disease impeding development of teeth and bones, is associated with premature exfoliation of primary teeth. Hypophosphatasia is caused by mutations in the ALPL gene, which encodes the tissue non-specific form of alkaline phosphatase. S63845 order Asfotase alfa (Strensiq®) is a human recombinant bone-targeted alkaline phosphatase.

To review development and exfoliation patterns of primary/permanent teeth in a cohort of patients with hypophosphatasia enrolled in an open-label clinical trial of enzyme replacement therapy (ERT) with asfotase alfa.

Data were collected from existing study files of a cohort of patients ≤5years of age with infantile hypophosphatasia. Children were recruited at the Winnipeg site of a global clinical trial and were treated with ERT. Dental information, including the exfoliation/eruption patterns, were recorded at each visit.

Eleven children (7 females, 4 males) participated. Participants enrolled as infants (5 infants; mean age 3.0±2.3months) prematurely lost significantly fewer teeth to hypophosphatasia than patients recruited as preschoolers (6 preschoolers; mean age 52.5±11.3months), who started on asfotase alfa at a later age. Conclusion The oral health of children with early onset infantile hypophosphatasia may be improved with early and continued administration of ERT, compared to institution of therapy later in childhood.

Eleven children (7 females, 4 males) participated. Participants enrolled as infants (5 infants; mean age 3.0 ± 2.3 months) prematurely lost significantly fewer teeth to hypophosphatasia than patients recruited as preschoolers (6 preschoolers; mean age 52.5 ± 11.3 months), who started on asfotase alfa at a later age. Conclusion The oral health of children with early onset infantile hypophosphatasia may be improved with early and continued administration of ERT, compared to institution of therapy later in childhood.Physical activity enhances proximal femur bone mass, but it remains unclear whether the benefits translate into an enhanced ability to resist fracture related loading. We recently used baseball pitchers as a within-subject controlled model to demonstrate physical activity induced proximal femur adaptation in regions associated with weight bearing and increased strength under single-leg stance loading. However, there was no measurable benefit to resisting common injurious loading (e.g. a fall onto the greater trochanter). A lack of power and a small physical activity effect size may have contributed to the latter null finding. Softball pitchers represent an alternative within-subject controlled model to explore adaptation of the proximal femur to physical activity, exhibiting greater dominant-to-nondominant (D-to-ND) leg differences than baseball pitchers. The current study used quantitative computed tomography, statistical parametric mapping, and subject-specific finite element (FE) modeling to explore adaptafits, albeit smaller, on resisting loads associated with a sideways fall onto the greater trochanter.Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome due to a phosphaturic tumor, which overproduces Fibroblast Growth Factor 23 (FGF-23), causing hyperphosphaturia, hypophosphatemia, low 1,25(OH)2D and osteomalacia. Tumor localization is critical, diagnostic delay ranges from 2.5 to 28 years and to date surgical removal is considered effective treatment. We retrospectively evaluated patients with definite diagnosis of TIO referred to a tertiary Rheumatology Center between September 2000 and May 2020, investigating clinical management and disease outcome. We included 17 patients 10 (58.8%) were females, mean age at diagnosis was 55.3 ± 13.9 years (mean ± standard deviation), with a diagnostic delay from symptoms onset to tumor detection of 6.6 ± 6.25 years. Biochemical data were serum phosphorus 1.3 ± 0.4 mg/dL (Reference Range 2.5-4.6), serum 1,25(OH)2D 31.8 ± 22.9 ng/mL (RR 25-86), intact FGF-23, 358.9 ± 677 pg/mL (RR 25-45); 24 h-Urine Phosphorus was increased in only 2 patients, while tubulapproaches have been successful in curing TIO. Active surveillance on possible recurrence is always needed. Burosumab appears a promising therapy.

Patients with idiopathic anaphylaxis (IA) may fail to respond to a combination of high-dose H

and H

antihistamines and mast cell stabilizers. Treatment options for these patients are currently limited.

To describe the clinical experience of omalizumab use for the treatment of patients with IA with no evidence of underlying clonal mast cell disorders.

We performed a retrospective review at 2 separate institutions of medical records of patients with a diagnosis of IA without evidence of mast cell clonality who had received treatment with omalizumab. We searched PubMed for studies describing omalizumab use in similar patients. Information on symptoms and omalizumab therapy was compiled, and response pattern of anaphylaxis was determined.

A total of 35 patients with IA and no evidence of mast cell clonality who received omalizumab were identified. The median age was 36 years at the start of omalizumab (range, 11-54 years; n=29). The frequency of anaphylaxis episodes before omalizumab treatment varied from 2 total episodes to several episodes per month.