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Cureus Feb 2024Albright's hereditary osteodystrophy is a rare hereditary disease due to a mutation of the complex guanine nucleotide-binding protein, alpha-stimulating activity...
Albright's hereditary osteodystrophy is a rare hereditary disease due to a mutation of the complex guanine nucleotide-binding protein, alpha-stimulating activity polypeptide. This condition is commonly associated with type 1A and 1C pseudohypoparathyroidism and pseudo-pseudohypoparathyroidism due to resistance of parathyroid hormone. Patients present with specific characteristics such as brachydactyly, short stature, round facies, subcutaneous ossifications, developmental delay, and obesity, associated with hypocalcemia and hyperphosphatemia. This case presents a 55-year-old woman with short stature and neurocognitive impairment, who was admitted to the emergency department with acute decompensated heart and respiratory failure. On admission, hypocalcemia and hyperphosphatemia were noted, which in combination with the patient's clinical history led to an etiological investigation. This case stresses the importance of not only treating the acute disease but also looking at the patient and their clinical and analytical features to diagnose this disease and prevent its complications.
PubMed: 38558694
DOI: 10.7759/cureus.55200 -
BMC Nephrology Sep 2023This study aimed to investigate the effect of a family-centered empowerment program on hyperphosphatemia management. (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
This study aimed to investigate the effect of a family-centered empowerment program on hyperphosphatemia management.
METHOD
This experimental study was performed on 80 randomly selected eligible patients with hyperphosphatemia undergoing hemodialysis. Patients were assigned randomly to two groups of family-centered empowerment program (FCEPG) and control group (CG) by coin toss (40 people per group). Data collection tools were the researcher-made Phosphate Control Knowledge Scale, the researcher-made Adherence to Dietary Restriction of Phosphorus Intake Scale, the eight-item Morisky Medication Adherence Scale, and serum phosphorus measurements. Data were collected before the intervention, one month, and three months after the intervention. Patients in FCEPG participated in a family-centered empowerment program. The statistical significance level was considered to be 0.05.
RESULTS
Inter-group comparisons showed no significant difference between FCEPG and CG in terms of the mean score of knowledge of phosphate control, adherence to dietary restriction of phosphorus intake, adherence to medication, and the mean serum phosphorus level before the empowerment program, but showed significant differences between them in these respects at one month after the program and three months after the program (p < 0.05). Intra-group comparisons showed a significant difference in FCEPG between the mean and standard deviation of all four variables before the empowerment program and the corresponding values one month and three months after the program (P < 0.05).
CONCLUSION
The findings of this study can be used in various fields of healthcare in the hospital and community.
Topics: Humans; Phosphates; Hyperphosphatemia; Phosphorus, Dietary; Renal Dialysis; Phosphorus
PubMed: 37661281
DOI: 10.1186/s12882-023-03311-1 -
Clinical Medicine Insights. Cardiology 2023Preventing hypertension by restricting dietary salt intake, sodium chloride, is well established in public health policy, but a pathophysiological mechanism has yet to...
Preventing hypertension by restricting dietary salt intake, sodium chloride, is well established in public health policy, but a pathophysiological mechanism has yet to explain the controversial clinical finding that some individuals have a greater risk of hypertension from exposure to salt intake, termed salt-sensitive hypertension. The present perspective paper synthesizes interdisciplinary findings from the research literature and offers novel insights proposing that the pathogenesis of salt-sensitive hypertension is mediated by interaction of salt-induced hypervolemia and phosphate-induced vascular calcification. Arterial stiffness and blood pressure increase as calcification in the vascular media layer reduces arterial elasticity, preventing arteries from expanding to accommodate extracellular fluid overload in hypervolemia related to salt intake. Furthermore, phosphate has been found to be a direct inducer of vascular calcification. Reduction of dietary phosphate may help reduce salt-sensitive hypertension by lowering the prevalence and progression of vascular calcification. Further research should investigate the correlation of vascular calcification with salt-sensitive hypertension, and public health recommendations to prevent hypertension should encourage reductions of both sodium-induced hypervolemia and phosphate-induced vascular calcification.
PubMed: 37434790
DOI: 10.1177/11795468231158206 -
Cureus Mar 2024Thyroidectomy technique and extent are related to parathyroid injury and hypoparathyroidism. Total thyroidectomy is one of the most commonly performed endocrine...
INTRODUCTION
Thyroidectomy technique and extent are related to parathyroid injury and hypoparathyroidism. Total thyroidectomy is one of the most commonly performed endocrine surgeries, and the majority of patients recover completely without any complications. However, persistent hypoparathyroidism is the most prevalent long-term consequence following total thyroidectomy. While it is seldom deadly, it can cause severe morbidity for the patient and raise healthcare expenses.
METHODS
This retrospective cohort study was conducted at King Abdulaziz Medical City, Jeddah, Saudi Arabia. We included all confirmed thyroid cancer cases that underwent thyroidectomy with or without neck dissection between July 2016 and August 2022. The data was collected from a chart review of the electronic medical record system (BEST-care), and a data collection sheet was utilized. SPSS version 26 was used to analyze the data.
RESULTS
A total of 192 patients undergoing thyroid surgery were enrolled. One hundred forty-three (74.5%) were females and the mean age of participants was 45.29 ± 16.88 years. Most patients, 170 (88.5%), had a papillary histological type, and total thyroidectomy was performed in 150 (78.1%). A significant association was found between the type of surgery and postoperative hypoparathyroidism (p=<0.05*). In addition, hypocalcemia was seen in 147 (76.6%) of the patients. Postoperative hypoparathyroidism was significantly higher among patients who had asymptomatic postoperative hypocalcemia and those who received IV calcium gluconate (p=<0.05*). Moreover, postoperative hypocalcemia, hypomagnesemia, and hyperphosphatemia were significantly associated with postoperative hypoparathyroidism (p=<0.05*).
CONCLUSION
The incidence of postoperative hypoparathyroidism is significantly higher among patients who underwent total thyroidectomy and had a normal level of preoperative parathyroid hormone (PTH) and magnesium (Mg) levels. Identifying these factors is a crucial step to minimize the occurrence of such complications.
PubMed: 38646308
DOI: 10.7759/cureus.56585 -
Biomedicines Feb 2024Vitamin D deficiency and insufficiency are highly prevalent in CKD, affecting over 80% of hemodialysis (HD) patients and requiring therapeutic intervention....
Vitamin D deficiency and insufficiency are highly prevalent in CKD, affecting over 80% of hemodialysis (HD) patients and requiring therapeutic intervention. Nephrological societies suggest the administration of cholecalciferol according to the guidelines for the general population. The aim of the observational study was to evaluate the efficacy and safety of the therapy with a high dose of cholecalciferol in HD patients with 25(OH)D deficiency and insufficiency to reach the target serum 25(OH)D level > 30 ng/mL. A total of 22 patients (16 M), with an average age of 72.5 ± 13.03 years and 25(OH)D concentration of 13.05 (9.00-17.90) ng/mL, were administered cholecalciferol at a therapeutic dose of 70,000 IU/week (20,000 IU + 20,000 IU + 30,000 IU, immediately after each dialysis session). All patients achieved the target value > 30 ng/mL, with a mean time of 2.86 ± 1.87 weeks. In the first week, the target level of 25(OH)D (100%) was reached by 2 patients (9.09%), in the second week by 15 patients (68.18%), in the fourth week by 18 patients (81.18%), and in the ninth week by all 22 patients (100%). A significant increase in 1,25(OH)D levels was observed during the study. However, only 2 patients (9.09%) achieved a concentration of 1,25(OH)D above 25 ng/mL-the lower limit of the reference range. The intact PTH concentrations remained unchanged during the observation period. No episodes of hypercalcemia were detected, and one new episode of hyperphosphatemia was observed. In conclusion, our study showed that the administration of a high-therapeutic dose of cholecalciferol allowed for a quick, effective, and safe leveling of 25(OH)D concentration in HD patients.
PubMed: 38397979
DOI: 10.3390/biomedicines12020377 -
Jornal Brasileiro de Nefrologia 2023Brazil has a vast territory divided into five geographic regions with important differences in sociodemographic indices. We aimed to present and compare...
INTRODUCTION
Brazil has a vast territory divided into five geographic regions with important differences in sociodemographic indices. We aimed to present and compare socio-demographic characteristics, biochemical results, and drug prescription of patients on chronic hemodialysis (HD) treatment in the five geographic regions.
METHODS
We evaluated data from the Brazilian Dialysis Registry of all adult patients undergoing chronic HD in 2021. Variables included sociodemographic characteristics, serum levels of phosphate, calcium, and albumin, hemoglobin, urea reduction rate, and prescription of phosphate binders, erythropoietin, and intravenous iron. Data from the North and Northeast regions were combined into one group.
RESULTS
A total of 13,792 patients (57.9 ± 16.0 years old, 58.5% male, median HD vintage of 31 (11-66) months) from 73 dialysis centers were analyzed. Regional distribution was 59.5% in the Southeast; 21.7% in the South; 5.9% in the Midwest; and 12.9% in the North/Northeast. Sociodemographic features, biochemical results, and medication prescriptions differed across regions. The prevalence of elderly patients was lower in the Midwest and North/Northeast. The South region had the highest prevalence of hyperphosphatemia (41.2%) and urea reduction rate <65% (24.8%), while anemia and hypoalbuminemia were more prevalent in the Southeast, 32.7% and 11.6%, respectively.
CONCLUSION
We found differences in socio-demographics, clinical features, and drug prescriptions across Brazilian geographic regions. Some findings reflect the socio-demographic diversity of the country, while others deserve further elucidation.
Topics: Adult; Humans; Male; Aged; Middle Aged; Female; Renal Dialysis; Kidney Failure, Chronic; Brazil; Drug Prescriptions; Phosphates; Urea; Demography
PubMed: 37395543
DOI: 10.1590/2175-8239-JBN-2022-0169en -
JCEM Case Reports Jul 2023PTH resistance is characterized by hypocalcemia and hyperphosphatemia in the presence of elevated PTH concentrations, resulting in pseudohypoparathyroidism, which is...
PTH resistance is characterized by hypocalcemia and hyperphosphatemia in the presence of elevated PTH concentrations, resulting in pseudohypoparathyroidism, which is subdivided into different types according to its different pathogenesis and phenotype. PTH receptor is the alpha subunit of stimulatory G protein (Gα)-coupled receptor. Pathogenic variants of GNAS gene, encoding for Gα, lead to reduced Gα function and PTH resistance. We report a patient with PHP type 1a, with no documented evidence of hypocalcemia, presenting with AHO phenotype and multihormone resistance to PTH, TSH, and GnRH. Her genetic testing showed a novel heterozygous pathogenic variants, a c.934T > G change in exon 11 in adenylate cyclase stimulatory G protein that has not been reported in the literature so far.
PubMed: 37908988
DOI: 10.1210/jcemcr/luad088 -
Clinical Kidney Journal Oct 2023Hyperphosphatemia is associated with increased mortality and cardiovascular morbidity of end-stage kidney failure (ESKF) patients. Managing serum phosphate in ESKF...
BACKGROUND
Hyperphosphatemia is associated with increased mortality and cardiovascular morbidity of end-stage kidney failure (ESKF) patients. Managing serum phosphate in ESKF patients is challenging and mostly based on limiting intestinal phosphate absorption with low phosphate diets and phosphate binders (PB). In a multi-centric, double-blinded, placebo-controlled study cohort of maintenance hemodialysis patients with hyperphosphatemia, we demonstrated the efficacy of nicotinamide modified release (NAMR) formulation treatment in addition to standard PB therapy in decreasing serum phosphate. Here we aimed to assess the relationship between phosphate, FGF23, inflammation and iron metabolism in this cohort.
METHODS
We measured the plasma concentrations of intact fibroblast growth factor 23 (iFGF23) and selected proinflammatory cytokines at baseline and Week 12 after initiating treatment.
RESULTS
We observed a strong correlation between iFGF23 and cFGF23 (C-terminal fragment plus iFGF23). We identified iFGF23 as a better predictor of changes in serum phosphate induced by NAMR and PB treatment compared with cFGF23. Recursive partitioning revealed at baseline and Week 12, that iFGF23 and cFGF23 together with T50 propensity were the most important predictors of serum phosphate, whereas intact parathyroid hormone (iPTH) played a minor role in this model. Furthermore, we found serum phosphate and iPTH as the best predictors of iFGF23 and cFGF23. Sex, age, body mass index, and markers of inflammation and iron metabolism had only a minor impact in predicting FGF23.
CONCLUSION
Lowering serum phosphate in ESKF patients may depend highly on iFGF23 which is correlated to cFGF23 levels. Serum phosphate was the most important predictor of plasma FGF23 in this ESKF cohort.
PubMed: 37779856
DOI: 10.1093/ckj/sfad040 -
Irish Veterinary Journal Sep 2023Anaemia is a common condition in alpacas and attributable to a variety of causes. Severe anaemia with a packed cell volume (PCV) less than 10% is frequently diagnosed,...
BACKGROUND
Anaemia is a common condition in alpacas and attributable to a variety of causes. Severe anaemia with a packed cell volume (PCV) less than 10% is frequently diagnosed, usually due to blood loss resulting from haemonchosis. Many South American camelids (SACs) also suffer from gastric ulcers, which are often associated with anaemia in other species. However, in alpacas and llamas, gastric ulcers usually do not lead to anaemia due to blood loss according to the current literature. There are no detailed clinical and laboratory data on this condition in the scientific literature so far.
CASE PRESENTATION
We report on the case of a nine-year-old male alpaca that was presented to the clinic with suspected forestomach acidosis. The animal showed clinical signs of colic, hypothermia, tachypnea, tachycardia, pale mucous membranes, and died shortly after admission to the clinic. Laboratory diagnosis revealed a markedly decreased haematocrit (0.13 l/l), leucopaenia with band neutrophils, azotaemia, hypocalcaemia, hyperphosphataemia and vitamin D deficiency. Post-mortem examination revealed multiple ulcers in the first and third compartment with perforation of one ulcer in the first compartment, resulting in intraluminal blood loss and purulent peritonitis.
CONCLUSIONS
To the authors' knowledge, this is the first detailed description of clinical and laboratory data of severe anaemia due to a perforated gastric ulcer in a SAC. Although the current literature suggests that severe blood loss due to gastric ulcers does not occur in SACs, this condition should be considered as a possible differential diagnosis in anaemic animals. Clinical indicators can be colic and pale mucous membranes.
PubMed: 37689785
DOI: 10.1186/s13620-023-00251-y -
International Journal of Nephrology and... 2024Hyperphosphataemia represents a significant challenge in the management of chronic kidney disease, exerting a pronounced influence on the pathogenesis of cardiovascular... (Review)
Review
Hyperphosphataemia represents a significant challenge in the management of chronic kidney disease, exerting a pronounced influence on the pathogenesis of cardiovascular complications and mineral bone disorders. Traditional approaches to address hyperphosphataemia involve implementing dietary phosphate restrictions, administering phosphate binders, and, in cases of end-stage renal disease, resorting to dialysis. Unfortunately, these interventions frequently prove inadequate in maintaining phosphate levels within recommended ranges. Additionally, commonly employed pharmacological agents are not immune to eliciting adverse events, thereby limiting their prescription and therapeutic adherence. There is a growing focus on exploring novel therapeutic strategies in this context. The current discussion centres on tenapanor, a pharmacological agent predominantly acting as a selective inhibitor of sodium/hydrogen exchanger isoform 3 (NHE3). Its mechanism of action involves modulating tight junctions, resulting in reduced sodium absorption and intestinal paracellular permeability to phosphate. Furthermore, tenapanor downregulates sodium-dependent phosphate 2b transport protein (NaPi2b) expression, thereby impeding active transcellular phosphate transport. Clinical trials have elucidated the efficacy and safety profile of tenapanor. This evidence hints at a potential paradigm shift in the management of hyperphosphataemia. However, the burgeoning optimism surrounding tenapanor warrants tempered enthusiasm, as further research remains indispensable. The imperative lies in meticulously delineating its efficacy and safety contours within the crucible of clinical practice. In this review, we synthesize the intricate interplay between hyperphosphataemia and Chronic Kidney Disease-Mineral Bone Disorder, and we discuss the existing pharmacological interventions for hyperphosphataemia and explore emerging treatment paradigms that offer novel perspectives in managing elevated phosphate levels in CKD patients.
PubMed: 38831770
DOI: 10.2147/IJNRD.S385826