Systematic Review: Efficacy of Medical Therapy on Outcomes Important to Pediatric Patients with X-Linked Hypophosphatemia.

Objective: To examine the evidence addressing the management of X-linked hypophosphatemia (XLH) in children to inform treatment recommendations.

Methods: We searched Embase, MEDLINE, Web of Science, and Cochrane Central up to May 2023. Eligible studies included RCTs and observational studies of individuals less than 18yrs with clinically or genetically confirmed XLH.

COL9A1-related disorder with pectus carinatum, without epiphyseal dysplasia: case report and review of literature.

COL9A1 encodes the alpha-1 chain of type IX collagen heterotrimer, which is a vital component of collagen fibrils in hyaline cartilage. There are preliminary lines of evidence suggesting that COL9A1 mutations may be associated with autosomal dominant multiple epiphyseal dysplasia (MED), a disorder affecting the epiphysis of long bones. With only 2 reported cases (both from the same family) of MED in autosomal dominant COL9A1-related disorders (MIM 614135) in the clinical scientific literature hitherto, the phenotype is poorly understood at present.

Hypophosphatasia diagnosis: current state of the art and proposed diagnostic criteria for children and adults.

Background: This manuscript provides a summary of the current evidence to support the criteria for diagnosing a child or adult with hypophosphatasia (HPP). The diagnosis of HPP is made on the basis of integrating clinical features, laboratory profile, radiographic features of the condition, and DNA analysis identifying the presence of a pathogenic variant of the tissue nonspecific alkaline phosphatase gene (ALPL). Often, the diagnosis of HPP is significantly delayed in both adults and children, and updated diagnostic criteria are required to keep pace with our evolving understanding regarding the relationship between ALPL genotype and associated HPP clinical features.

Proposed diagnostic criteria for the diagnosis of hypophosphatasia in children and adolescents: results from the HPP International Working Group.

Hypophosphatasia (HPP) is a rare inborn error of metabolism that presents variably in both age of onset and severity. HPP is caused by pathogenic variants in the ALPL gene, resulting in low activity of tissue nonspecific alkaline phosphatase (TNSALP). Patients with HPP tend have a similar pattern of elevation of natural substrates that can be used to aid in diagnosis.

Effect of low dose denosumab on bone mineral density in postmenopausal women with osteoporosis after a transition from 60 mg dose: a prospective observational study.

Introduction: Denosumab is an effective antiresorptive molecule and reduces the risk of fracture in postmenopausal osteoporosis. Cessation of denosumab therapy however is associated with rapid declines in bone mineral density (BMD), rises in bone remodeling, and an increased risk of fracture. We evaluated the effect of low dose denosumab (30 mg every 6 months) on the prevention of bone loss following a switch from standard dose (60 mg of denosumab every 6 months) in a prospective observational study.

Post hip fracture orthogeriatric care-a Canadian position paper addressing challenges in care and strategies to meet quality indicators.

Introduction: Osteoporosis is a major disease state associated with significant morbidity, mortality, and health care costs. Less than half of the individuals sustaining a low energy hip fracture are diagnosed and treated for the underlying osteoporosis.

Objective: A multidisciplinary Canadian hip fracture working group has developed practical recommendations to meet Canadian quality indicators in post hip fracture care.

Health Economics of Selective Early Rescue Surfactant Administration and Standard Surfactant Administration for Newborns With Respiratory Distress Syndrome.

Objective: To quantify the effect of early rescue surfactant administration techniques for preterm infants with respiratory distress syndrome (RDS) from a health care delivery system perspective.

Methods: A cost-consequence model was developed based on previously published literature to compare the health economic impact of implementing early surfactant administration strategies vs standard surfactant administration via endotracheal intubation and mechanical ventilation (MV).

Results: Early rescue surfactant treatment strategies are associated with a decrease in the number of patients requiring MV, cumulative MV days, and rate of neonatal complications.

Parathyroid Hormone Therapy for Managing Chronic Hypoparathyroidism: A Systematic Review and Meta-Analysis.

The efficacy and safety of parathyroid hormone (PTH) therapy for managing long-term hypoparathyroidism is being evaluated in ongoing clinical trials. We undertook a systematic review and meta-analysis of currently available randomized controlled trials to investigate the benefits and harms of PTH therapy and conventional therapy in the management of patients with chronic hypoparathyroidism. To identify eligible studies, published in English, we searched Embase, PubMed, and Cochrane CENTRAL from inception to May 2022.

Complications, Symptoms, Presurgical Predictors in Patients With Chronic Hypoparathyroidism: A Systematic Review.

The complications and symptoms of hypoparathyroidism remain incompletely defined. Measuring serum parathyroid hormone (PTH) and calcium levels early after total thyroidectomy may predict the development of chronic hypoparathyroidism. The study aimed (i) to identify symptoms and complications associated with chronic hypoparathyroidism and determine the prevalence of those symptoms and complications (Part I), and (ii) to examine the utility of early postoperative measurements of PTH and calcium in predicting chronic hypoparathyroidism (Part II).

Management of Hypoparathyroidism.

Hypoparathyroidism (HypoPT) is a rare disorder characterized by hypocalcemia in the presence of a low or inappropriately normal parathyroid hormone level. HypoPT is most commonly seen after neck surgery, which accounts for approximately 75% of cases, whereas approximately 25% have HypoPT due to nonsurgical causes. In both groups of patients, conventional therapy includes calcium and active vitamin D analogue therapy aiming to maintain serum calcium concentration in the low normal or just below the normal reference range and normalize serum phosphorus, magnesium concentrations, and urine calcium levels.

Initial Assessment and Monitoring of Patients with Chronic Hypoparathyroidism: A Systematic Current Practice Survey.

Chronic hypoparathyroidism (HypoPT) is associated with significant morbidity and impaired quality of life (QoL). The goals of management for chronic HypoPT include improvement in QoL and the prevention of both hypo- and hypercalcemia symptoms and long-term complications. Several groups have provided consensus statements and guidelines on the management of HypoPT; however, due to limited evidence, these recommendations have largely been based on literature reviews, expert opinion, and consensus statements.

Directive clinique n 422g : Ménopause et ostéoporose.

Objectif: Proposer des stratégies pour améliorer les soins aux femmes en périménopause ou ménopausées d'après les plus récentes données probantes publiées.

Population Cible: Femmes en périménopause ou ménopausées. BéNéFICES, RISQUES ET COûTS: La population cible bénéficiera des plus récentes données scientifiques publiées que leur communiqueront les fournisseurs de soins de santé.

Guideline No. 422g: Menopause and Osteoporosis.

Objective: Provide strategies for improving the care of perimenopausal and postmenopausal women based on the most recent published evidence.

Target Population: Perimenopausal and postmenopausal women.

Benefits, Harms, And Costs: Target population will benefit from the most recent published scientific evidence provided via the information from their health care provider.

Hypercalcemia in Pregnancy.

Hypercalcemic disorders are rare in pregnant women and are usually due to primary hyperparathyroidism. Clinical manifestations of hypercalcemia are nonspecific and can be masked by the physiologic changes of pregnancy. Furthermore, routine antenatal screening does not include serum calcium measurement and a hypercalcemia diagnosis may therefore be delayed until term or even after delivery.

Primary Hyperparathyroidism in Pregnancy: Literature Review of the Diagnosis and Management.

Background: Parathyroid disease is uncommon in pregnancy. During pregnancy, multiple changes occur in the calcium regulating hormones which may make the diagnosis of primary hyperparathyroidism more challenging. Close monitoring of serum calcium during pregnancy is necessary in order to optimize maternal and fetal outcomes.

Primary Hyperparathyroidism: A Narrative Review of Diagnosis and Medical Management.

Primary hyperparathyroidism (PHPT) is the most common cause of hypercalcemia in the outpatient setting. Symptomatic presentation includes non-specific signs and symptoms of hypercalcemia, skeletal fragility, nephrolithiasis and nephrocalcinosis. The majority of individuals present at an asymptomatic stage following routine biochemical screening, without any signs or symptoms of calcium or parathyroid hormone (PTH) excess or target organ damage.

Hypoparathyroidism in Pregnancy and Lactation: Current Approach to Diagnosis and Management.

Background: Hypoparathyroidism is an uncommon endocrine disorder. During pregnancy, multiple changes occur in the calcium-regulating hormones, which may affect the requirements of calcium and active vitamin D during pregnancy in patients with hypoparathyroidism. Close monitoring of serum calcium during pregnancy and lactation is ideal in order to optimize maternal and fetal outcomes.

Providing nighttime pharmaceutical services through telepharmacy.

A nighttime telepharmacy service serving a community hospital is described. At a 340-bed acute care community hospital, the level of nighttime activity related to medication use did not support the establishment of a full night pharmacy shift. After-hours access to medications was mostly the responsibility of nursing and medical staff using a separate night closet, automated dispensing machines, and limited floor stock.

Drug-associated Guillain-Barré syndrome: a literature review.

Objective: To present an overview of reported drug-associated Guillain-Barré syndrome (GBS) and current management of the disease.

Data Sources: Case reports and reviews of drug-associated GBS published in the English-language literature from 1982 through 1994 were retrieved from MEDLINE. An additional search was done through the Iowa Drug Information System for the same period.

Stability of dopamine hydrochloride exposed to blue-light phototherapy.

The stability of dopamine hydrochloride in 5% dextrose injection when exposed to blue-light phototherapy was assessed. Solutions of dopamine hydrochloride (1000 micrograms/mL) were prepared, and samples were subjected to one of three light sources (fluorescent, dark, phototherapy) and two flow rates (2 mL/hr and no flow) at ambient room temperature. Irradiation levels were maintained between 5.