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1.
Swiss Med Wkly ; 154: 3771, 2024 Jul 24.
Artículo en Inglés | MEDLINE | ID: mdl-39137372

RESUMEN

AIMS: Bariatric surgery induces several micronutrient deficiencies that require supplementation. For iron, parenteral infusions are usually preferred over oral supplementation. Ferric carboxymaltose infusion has been associated with hypophosphataemia, mostly transient and asymptomatic. However, in some cases, ferric carboxymaltose-induced hypophosphataemia may persist for weeks to months and may induce muscle weakness, osteomalacia and bone fractures. The aim of this study was to identify possible predictors of a clinically relevant decrease in serum phosphate after ferric carboxymaltose infusion in patients with previous Roux-en-Y gastric bypass. METHODS: Patients with previous Roux-en-Y gastric bypass who received ferric carboxymaltose infusions between January 2018 and September 2019 and had recorded phosphataemia before and after ferric carboxymaltose infusion at the Lausanne University Hospital, Lausanne, Switzerland, were studied retrospectively. A multiple linear regression model was built with delta phosphataemia as the outcome to investigate the factors related to magnitude of serum phosphate lowering. RESULTS: Seventy-seven patients (70 females and 7 males) with previous Roux-en-Y gastric bypass were studied. Mean age (SD) was 43.2 (10.7) years and median BMI was 30.9 kg/m2 (IQR 27.9-36.4). Sixty-eight patients (88.3%) received an infusion of 500 mg ferric carboxymaltose and 9 patients (11.7%) received 250 mg ferric carboxymaltose. Forty-nine patients (63.6%) developed hypophosphataemia (<0.8 mmol/l) after ferric carboxymaltose infusion. Median plasma phosphate significantly decreased by 0.33 mmol/l (IQR 0.14-0.49) (p<0.0001). Multiple linear regression identified the ferric carboxymaltose dose as the only risk factor significantly associated with the magnitude of serum phosphate lowering, with an additional mean loss of 0.26 mmol/l with a 500 mg infusion compared to a 250 mg infusion (p = 0.020). CONCLUSION: Ferric carboxymaltose infusions substantially decreased plasma phosphate levels in patients with previous Roux-en-Y gastric bypass. Compared to a dose of 250 mg, infusion of a dose of 500 mg ferric carboxymaltose decreased the plasma phosphate further in this population.


Asunto(s)
Compuestos Férricos , Derivación Gástrica , Hipofosfatemia , Maltosa , Fosfatos , Humanos , Femenino , Masculino , Derivación Gástrica/efectos adversos , Maltosa/análogos & derivados , Maltosa/administración & dosificación , Maltosa/uso terapéutico , Compuestos Férricos/administración & dosificación , Compuestos Férricos/uso terapéutico , Estudios Retrospectivos , Adulto , Fosfatos/sangre , Persona de Mediana Edad , Infusiones Intravenosas , Suiza
2.
Nutrients ; 16(15)2024 Aug 03.
Artículo en Inglés | MEDLINE | ID: mdl-39125435

RESUMEN

This study aimed to evaluate the incidence and risk factors associated with refeeding syndrome (RFS) in preterm infants (≤32 weeks gestational age) during their first week of life. Infants (gestational age ≤ 32 weeks; birth weight < 1500 g) who were admitted to the neonatal intensive care unit (NICU), level III, and received parenteral nutrition between January 2015 and April 2024 were retrospectively evaluated. Modified log-Poisson regression with generalized linear models and a robust variance estimator was applied to adjust the relative risk of risk factors. Of the 760 infants identified, 289 (38%) developed RFS. In the multivariable regression analysis, male, intraventricular hemorrhage (IVH), and sodium phosphate significantly affected RFS. Male infants had significantly increased RFS risk (aRR1.31; 95% CI 1.08-1.59). The RFS risk was significantly higher in infants with IVH (aRR 1.71; 95% CI 1.27-2.13). However, infants who received higher sodium phosphate in their first week of life had significantly lower RFS risk (aRR 0.67; 95% 0.47-0.98). This study revealed a notable incidence of RFS among preterm infants aged ≤32 gestational weeks, with sex, IVH, and low sodium phosphate as significant risk factors. Refined RFS diagnostic criteria and targeted interventions are needed for optimal management.


Asunto(s)
Recien Nacido Prematuro , Nutrición Parenteral , Fosfatos , Síndrome de Realimentación , Humanos , Factores de Riesgo , Masculino , Recién Nacido , Incidencia , Síndrome de Realimentación/epidemiología , Síndrome de Realimentación/etiología , Femenino , Estudios Retrospectivos , Fosfatos/sangre , Nutrición Parenteral/efectos adversos , Edad Gestacional , Unidades de Cuidado Intensivo Neonatal/estadística & datos numéricos , Enfermedades del Prematuro/epidemiología , Enfermedades del Prematuro/etiología
3.
Nutr J ; 23(1): 81, 2024 Jul 18.
Artículo en Inglés | MEDLINE | ID: mdl-39026252

RESUMEN

BACKGROUND: Data is limited on the prevalence of hypophosphatemia in general hospitalized patients, and its association with length of hospital stay (LOS) and mortality remained unclear. We aimed to investigate the prevalence of admission phosphate abnormality and the association between serum phosphate level and length of hospital stay and all-cause mortality in adult patients. METHODS: This was a multi-center retrospective study based on real-world data. Participants were classified into five groups according to serum phosphate level (inorganic phosphorus, iP) within 48 h after admission: G1, iP < 0.64 mmol/L; G2, iP 0.64-0.8 mmol/L; G3, iP 0.8-1.16 mmol/L; G4, iP 1.16-1.45 mmol/L; and G5, iP ≥ 1.45 mmol/L, respectively. Both LOS and in-hospital mortality were considered as outcomes. Clinical information, including age, sex, primary diagnosis, co-morbidity, and phosphate-metabolism related parameters, were also abstracted from medical records. RESULTS: A total number of 23,479 adult patients (14,073 males and 9,406 females, aged 57.7 ± 16.8 y) were included in the study. The prevalence of hypophosphatemia was 4.74%. An "L-shaped" non-linear association was determined between serum phosphate level and LOS and the inflection point was 1.16 mmol/L in serum phosphate level. Compared with patients in G4, patients in G1, G2 or G3 were significantly associated with longer LOS after full adjustment of covariates. Each 0.1 mmol/L decrease in serum phosphate level to the left side of the inflection point led to 0.64 days increase in LOS [95% confidence interval (CI): 0.46, 0.81; p for trend < 0.001]. But there was no association between serum phosphate and LOS where serum levels of phosphate ≥ 1.16 mmol/L. Multivariable logistic regression analysis showed that adjusted all-cause in-hospital mortality was 3.08-fold greater in patients in G1 than those in G4 (95% CI: 1.52, 6.25; p for trend = 0.001). Similarly, no significant association with either LOS or mortality were found in patients in G5, comparing with G4. CONCLUSIONS: Hypophosphatemia, but not hyperphosphatemia, was associated with LOS and all-cause mortality in adult inpatients. It is meaningful to monitor serum levels of phosphate to facilitate early diagnosis and intervention.


Asunto(s)
Mortalidad Hospitalaria , Hipofosfatemia , Tiempo de Internación , Fosfatos , Humanos , Masculino , Femenino , Persona de Mediana Edad , Estudios Retrospectivos , Fosfatos/sangre , Estudios Transversales , Tiempo de Internación/estadística & datos numéricos , Hipofosfatemia/mortalidad , Hipofosfatemia/sangre , Hipofosfatemia/epidemiología , Anciano , Adulto , Prevalencia
4.
J Vet Intern Med ; 38(4): 2180-2195, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38952053

RESUMEN

BACKGROUND: Plasma total magnesium concentration (tMg) is a prognostic indicator in cats with chronic kidney disease (CKD), shorter survival time being associated with hypomagnesemia. Whether this risk factor is modifiable with dietary magnesium supplementation remains unexplored. OBJECTIVES: Evaluate effects of a magnesium-enriched phosphate-restricted diet (PRD) on CKD-mineral bone disorder (CKD-MBD) variables. ANIMALS: Sixty euthyroid client-owned cats with azotemic CKD, with 27 and 33 allocated to magnesium-enriched PRD or control PRD, respectively. METHODS: Prospective double-blind, parallel-group randomized trial. Cats with CKD, stabilized on a PRD, without hypermagnesemia (tMg >2.43 mg/dL) or hypercalcemia (plasma ionized calcium concentration, (iCa) >6 mg/dL), were recruited. Both intention-to-treat and per-protocol (eating ≥50% of study diet) analyses were performed; effects of dietary magnesium supplementation on clinicopathological variables were evaluated using linear mixed effects models. RESULTS: In the per-protocol analysis, tMg increased in cats consuming a magnesium-enriched PRD (ß, 0.25 ± .07 mg/dL/month; P < .001). Five magnesium supplemented cats had tMg >2.92 mg/dL, but none experienced adverse effects. Rate of change in iCa differed between groups (P = .01), with decreasing and increasing trends observed in cats fed magnesium-enriched PRD and control PRD, respectively. Four control cats developed ionized hypercalcemia versus none in the magnesium supplemented group. Log-transformed plasma fibroblast growth factor-23 concentration (FGF23) increased significantly in controls (ß, 0.14 ± .05 pg/mL/month; P = .01), but remained stable in the magnesium supplemented group (ß, 0.05±.06 pg/mL/month; P =.37). CONCLUSIONS AND CLINICAL IMPORTANCE: Magnesium-enriched PRD is a novel therapeutic strategy for managing feline CKD-MBD in cats, further stabilizing plasma FGF23 and preventing hypercalcemia.


Asunto(s)
Enfermedades de los Gatos , Suplementos Dietéticos , Magnesio , Insuficiencia Renal Crónica , Animales , Gatos , Magnesio/sangre , Magnesio/administración & dosificación , Magnesio/uso terapéutico , Enfermedades de los Gatos/dietoterapia , Enfermedades de los Gatos/tratamiento farmacológico , Insuficiencia Renal Crónica/veterinaria , Insuficiencia Renal Crónica/dietoterapia , Método Doble Ciego , Femenino , Masculino , Estudios Prospectivos , Dieta/veterinaria , Factor-23 de Crecimiento de Fibroblastos , Fosfatos/sangre , Calcio/sangre
5.
Zhongguo Dang Dai Er Ke Za Zhi ; 26(7): 677-682, 2024 Jul 15.
Artículo en Chino | MEDLINE | ID: mdl-39014942

RESUMEN

OBJECTIVES: To study the diagnosis, treatment, and complications of hypophosphatemic rickets (HR) in children, explore effectiveness evaluation indicators for the disease, and understand the pattern in height growth among these patients. METHODS: A retrospective analysis of the initial clinical data and five-year follow-up data of 85 children with HR treated at Children's Hospital of Nanjing Medical University from January 2008 to December 2022. RESULTS: Among the 85 children with HR, there were 46 males (54%) and 39 females (46%). The age at initial diagnosis ranged from 6 months to 13 years and 9 months, with a median age of 2.75 years. The average height standard deviation score was -2.0±1.1. At initial diagnosis, children exhibited reduced blood phosphate levels and elevated alkaline phosphatase (ALP), with 99% (84/85) presenting with lower limb deformities. The positive rate for PHEX gene mutations was 93% (55/59). One year post-treatment, there was a significant reduction in ALP levels and the gap between the lower limbs (P<0.05). The fastest height growth occurred in the first year after treatment, at 8.23 cm/year, with a peak height velocity (PHV) phase lasting about two years during puberty. The height increased by 9-20 cm in male children during the PHV stage and 10-15 cm in female children. Major complications included nephrocalcinosis and hyperparathyroidism. The incidence rate of nephrocalcinosis in the first year after treatment was 55% (22/40), which increased with the duration of the disease (P<0.001); an increased urinary phosphate/creatinine ratio was positively associated with a higher risk of nephrocalcinosis (OR=1.740, P<0.001). The incidence of hyperparathyroidism in the first year after treatment was 64% (27/42). CONCLUSIONS: For children presenting with lower limb deformities, short stature, and slow growth, early testing for blood levels of phosphate, calcium, and ALP, along with imaging examinations of the lower limbs, can aid in the early diagnosis of HR. Genetic testing may be utilized for definitive confirmation when necessary. ALP combined with improvements in skeletal deformities and annual height growth can serve as indicators of therapeutic effectiveness for HR. Compared to normal children, children with HR demonstrate a lower height increase during the PHV phase, necessitating close follow-up and timely adjustment of treatment plans Citation:Chinese Journal of Contemporary Pediatrics, 2024, 26(7): 677-682.


Asunto(s)
Raquitismo Hipofosfatémico , Humanos , Masculino , Femenino , Niño , Estudios Retrospectivos , Preescolar , Lactante , Adolescente , Estudios de Seguimiento , Raquitismo Hipofosfatémico/genética , Raquitismo Hipofosfatémico/etiología , Fosfatasa Alcalina/sangre , Estatura , Endopeptidasa Neutra Reguladora de Fosfato PHEX/genética , Fosfatos/sangre , Mutación
6.
Clin Lab ; 70(7)2024 Jul 01.
Artículo en Inglés | MEDLINE | ID: mdl-38965943

RESUMEN

BACKGROUND: Clinical laboratory tests are being evaluated with reference intervals (RI). Therefore, it is important that each laboratory determines and classifies its own reliable RI for each test to ensure an accurate and effective interpretation. The proposed method for determining RI is the "direct" approach, but it is a difficult, troublesome, time-consuming, and expensive method. An alternative approach is the "indirect" approach. In this study, we aimed to compare the RI values determined by the indirect method from the Calcium (Ca), Magnesium (Mg), Phosphate (P), 25-Hydroxy Vitamin D (25(OH)D), and Parathyroid hormone (PTH) test results with the RI provided by the manufacturer. METHODS: A total of 1,520,314 Ca, Mg, P, 25(OH)D, and PTH test results, which were studied in our laboratory between January and November 2022, were included in the study. Data cleaning was done for individuals between the ages of 18 - 89, and only one record was allowed. The Tukey method was used to determine and exclude extreme values. Ca and Mg tests were divided into age groups (18 - 59 and 60 - 89 years), P, 25(OH)D, and PTH tests were divided into female - male groups. RI was calculated by using the Bhattacharya and Hoffmann methods. CLIA 19 acceptable limits were used to evaluate the compliance with the manufacturer's RI. RESULTS: The RI results obtained by applying the Bhattacharya and Hoffmann methods were found to be significantly consistent and compatible with each other. According to the manufacturer's RI, Ca and Mg were compatible with RI in both methods, P was considered compatible with PTH and 25(OH)D upper reference limit in the Bhattacharya method, P was considered compatible with 25(OH)D lower reference limit and PTH upper reference limit in the Hoffmann method, while 25(OH)D lower reference limit was found to be different in the Bhattacharya method, and 25(OH)D upper reference limit and PTH lower reference limit were found to be different in the P male group in the Hoffmann method. CONCLUSIONS: We believe that it is of great importance for each laboratory to determine the RI specific for the population they serve and to choose the analytical method they use according to age and gender while periodically updating them to interpret the test results correctly.


Asunto(s)
Calcio , Magnesio , Hormona Paratiroidea , Vitamina D , Humanos , Valores de Referencia , Persona de Mediana Edad , Femenino , Masculino , Adulto , Anciano , Adulto Joven , Hormona Paratiroidea/sangre , Anciano de 80 o más Años , Adolescente , Calcio/sangre , Magnesio/sangre , Vitamina D/sangre , Vitamina D/análogos & derivados , Fosfatos/sangre
7.
Calcif Tissue Int ; 115(3): 229-241, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-39008126

RESUMEN

To assess the efficacy and safety of burosumab in children and adults with X-linked hypophosphatemia based on real-world evidence. MEDLINE (via PubMed) and Cochrane Library were searched until 18 October 2023 for single-arm (before-after) studies. Registries including Clinicaltrials.gov, EU Clinical Trials, WHO International Clinical Trials Registry Platform, and conference abstracts. The outcomes were a change in serum phosphorus concentrations and change in RSS, a change in serum ALP, bone-specific ALP, a change in the ratio of Tubular maximum reabsorption of Phosphate to Glomerular Filtrate rate, a change in serum 1,25(OH)2D and 25(OH)2D concentrations, change in height Z-score, McMaster Universities Osteoarthritis Index (WOMAC) and safety outcomes. An inverse variance random-effects meta-analysis was applied for data synthesis. Fifteen studies (289 participants) were included. Burosumab treatment improved serum phosphate concentrations [mean difference 0.88 mg/dl, 95% confidence interval 0.70 to 1.07, I2 = 92%), Rickets Severity score (mean difference - 1.86, 95% confidence interval - 2.5 to - 1.21, I2 = 71%), serum alkaline phosphate concentrations (mean difference - 1.86, 95% confidence interval - 2.5 to - 1.21, I2 = 71%), serum 1,25(OH)2D concentrations (mean difference 18.91 pg/ml, 95% confidence interval 6.39 to 31.43, I2 = 96%) and renal phosphate reabsorption (mean difference 1.22 mg/dl, 95% confidence interval 0.70 to 1.74, I2 93%). Burosumab treatment improved overall clinical and laboratory findings in patients with X-linked hypophosphatemia.


Asunto(s)
Anticuerpos Monoclonales Humanizados , Raquitismo Hipofosfatémico Familiar , Humanos , Raquitismo Hipofosfatémico Familiar/tratamiento farmacológico , Anticuerpos Monoclonales Humanizados/uso terapéutico , Anticuerpos Monoclonales Humanizados/efectos adversos , Resultado del Tratamiento , Fosfatos/sangre
8.
Curr Med Res Opin ; 40(8): 1345-1356, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-39041778

RESUMEN

What is this summary about?This is a summary of an article that was published in the medical journal Kidney360 describing results from the NORMALIZE study. The NORMALIZE study looked at how well tenapanor tablets reduced higher-than-normal levels of phosphate in the blood of persons with kidney disease who are on maintenance dialysis. These persons are unable to keep their blood phosphate levels in a normal range, and high levels of phosphate can contribute to several serious health consequences.Tenapanor is approved as an add-on treatment for high levels of phosphate in the blood of adults with chronic kidney disease who are on maintenance dialysis and whose disease does not respond adequately to treatment with phosphate binders or who are not able to take phosphate binders. In earlier clinical studies, tenapanor was studied alone or studied together with phosphate binders. In a 1-year clinical study called PHREEDOM, researchers learned that when tenapanor was used alone, it lowered blood phosphate levels, and treated patients experienced acceptable safety and tolerability as determined by the doctors running the study. In the NORMALIZE study, adult patients took a 30-mg tenapanor tablet twice a day, either alone or with sevelamer, for up to 18 months after they completed the PHREEDOM study.What were the main conclusions reported by the researchers?The researchers found that one-third of patients taking tenapanor, either alone or with sevelamer, achieved normal blood phosphate levels. This is an improvement from the current standard of care with sevelamer alone to reduce blood phosphate levels. As seen in the earlier studies of tenapanor, the most common adverse event experienced by patients was softer or loose stools. No new safety concerns were reported in the NORMALIZE study.What are the key takeaways?The researchers concluded that tenapanor, used alone or combined with sevelamer, can be used long-term by adult patients receiving maintenance dialysis to reduce the phosphate levels in their blood to within the normal range. Patients who take tenapanor may experience softer or loose stools.This summary was developed by the authors to help adult patients with chronic kidney disease receiving dialysis, and their family members and/or caregivers, better understand the effects of taking tenapanor.[Box: see text]Link to original article here.


Asunto(s)
Fosfatos , Diálisis Renal , Humanos , Fosfatos/sangre , Sulfonamidas/uso terapéutico , Hiperfosfatemia/sangre , Hiperfosfatemia/etiología , Hiperfosfatemia/tratamiento farmacológico , Femenino , Masculino
12.
Calcif Tissue Int ; 115(3): 215-228, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38951179

RESUMEN

This systematic review was performed to understand better the myriad presentations, various therapeutic options, response to therapy, and its clinical outcomes in hyperphosphatemic tumoral calcinosis (HTC). Full texts were selected according to strict inclusion criteria. All case reports of HTC wherein baseline phosphate was measured, treatment offered was mentioned, and information on follow-up and response to therapy that were available were included. A total of 43 of 188 eligible studies (N = 63 patients) met the inclusion criteria. A list of desired data was extracted and graded for methodological quality. A total of 63 individuals (Males = 33) were included from the 43 eligible case studies. The median age of the patients was 18 (IQR 8-32) years. The most frequently involved sites were the hip/gluteal region (34/63; 53.9%) followed by the elbow/forearm (26/63; 41.2%), and the shoulder (18/63; 28.5%). Three patients had conjunctival calcific deposits. The mean (SD) phosphate was 6.9 (1.1) mg/dL. Among the subjects, 36/63 (57.1%) underwent surgical excision with some form of medical therapy. Two patients underwent only surgical excision (2.1%). One patient was maintained on follow-up (1.6%) and 24/63 (38.1%) patients were treated with medical measures. The median (IQR) follow-up duration was 3 (1-9) years. Regression or reduction in lesion size was reported in 19/63 (30.2%) subjects; 20/63 (31.7%) showed progression, 24/63 (38.1%) had features of stable disease, and mortality was reported in 3 patients (4.7%). We report for the first time a detailed description of the clinical and therapeutic response of HTC. A combination of medical measures aimed at lowering serum phosphate appears to be the cornerstone of treatment, although clinical responses may vary.


Asunto(s)
Calcinosis , Hiperfosfatemia , Humanos , Calcinosis/terapia , Femenino , Adulto , Resultado del Tratamiento , Masculino , Adulto Joven , Adolescente , Fosfatos/sangre , Niño
13.
Int J Chron Obstruct Pulmon Dis ; 19: 1681-1693, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39055391

RESUMEN

Purpose: COPD patients frequently have abnormal serum phosphorus levels. The objective of this study was to examine the correlation between serum phosphorus levels with hospital and 90-day mortality in critically ill patients with COPD. Patients and Methods: The MIMIC IV database was used for this retrospective cohort analysis. We extracted demographics, vital signs, laboratory tests, comorbidity, antibiotic usage, ventilation and scoring systems within the first 24 hours of ICU admission. Restricted cubic splines and multivariate cox regression analysis models were used to evaluate the connection between serum phosphorus with hospital and 90-day mortality. We assessed and classified various factors including gender, age, renal disease, severe liver disease, the utilization of antibiotics and congestive heart failure. Results: We included a total of 3611 patients with COPD, with a median age of 70.7 years. After adjusting for all other factors, we observed a significant positive association between serum phosphate levels with both hospital mortality (HR 1.19, 95% CI: 1.07-1.31, p<0.001) and 90-day mortality (HR 1.15, 95% CI: 1.06-1.24, p<0.001). Compared to the medium group (Q2 ≥3.15, <4.0), the adjusted hazard ratios for hospital mortality were 1.47 (95% CI: 1.08-2, p=0.013), and 1.31 (95% CI: 1.06-1.61, p=0.013) for 90-day mortality in the high group (Q3≥4.0). Hospital mortality decreased at serum phosphate levels below 3.8 mg/dl (HR 0.664, 95% CI: 0.468-0.943, p=0.022), but increased for both hospital (HR 1.312, 95% CI: 1.141-1.509, p<0.001) and 90-day mortality (HR 1.236, 95% CI: 1.102-1.386, p<0.001) when levels were above 3.8 mg/dl. Subgroup and sensitivity analyses yielded consistent results. Conclusion: In critical ill COPD patients, this study demonstrated a non-linear association between serum phosphate levels and both hospital and 90-day mortality. Notably, there was an inflection point at 3.8 mg/dl, indicating a significant shift in outcomes. Future prospective research is necessary to validate this correlation.


Asunto(s)
Biomarcadores , Enfermedad Crítica , Mortalidad Hospitalaria , Fosfatos , Enfermedad Pulmonar Obstructiva Crónica , Humanos , Estudios Retrospectivos , Masculino , Femenino , Anciano , Enfermedad Crítica/mortalidad , Enfermedad Pulmonar Obstructiva Crónica/mortalidad , Enfermedad Pulmonar Obstructiva Crónica/sangre , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Persona de Mediana Edad , Factores de Tiempo , Factores de Riesgo , Biomarcadores/sangre , Fosfatos/sangre , Medición de Riesgo , Bases de Datos Factuales , Pronóstico , Anciano de 80 o más Años , Hiperfosfatemia/sangre , Hiperfosfatemia/mortalidad , Hiperfosfatemia/diagnóstico
14.
Eur J Med Genet ; 70: 104958, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38950880

RESUMEN

BACKGROUND AND OBJECTIVE: X-linked hypophosphatemic rickets (XLH) is due to loss-of-function mutations in the phosphate-regulating endopeptidase homologue on the X chromosome (PHEX) that lead to increased fibroblast growth factor 23 (FGF23) production. FGF23 excess causes renal phosphate wasting and insufficient 1,25-dihydroxyvitamin D (1,25(OH)2D) synthesis with reduced intestinal phosphate absorption, ultimately resulting in chronic hypophosphatemia. Children with XLH show typical skeletal lesions of rickets, deformities of the lower limbs, stunted growth with disproportionate short stature, bone pain, and physical dysfunctions. Burosumab, a fully human IgG1 monoclonal antibody that binds to FGF23 to inhibit its activity, is more effective to improve the biochemical and clinical signs of XLH than conventional treatment with phosphate supplements and vitamin D active metabolites. Data on adolescents with XLH during the transition period to young adulthood are few. In this prospective case series, we aimed to assess safety and efficacy of burosumab in adolescents with XLH who discontinued long-term conventional therapy. METHODS: Five Caucasian adolescents (4 males, 1 female; mean age 15.4 ± 1.5 years) with XLH were recruited and switched from conventional treatment to burosumab (0.8-1.2 mg/kg, s. c. QW2). Burosumab was continued for 12-48 months and, once discontinued, patients were followed-up for 6-12 months. In all patients, serum calcium, phosphate, alkaline phosphatase (ALP), parathyroid hormone (PTH), and 1,25(OH)2D levels, and renal tubular reabsorption of phosphate (TmP/GFR) values were assessed at entry and during burosumab. Intact FGF23 plasma levels were measured at entry. Patient-reported outcomes (PROs) were assessed at entry and every 3-6 months to evaluate the impact of low extremity pain, stiffness, and difficulties performing daily activities. RESULTS: At entry, all patients showed hypophosphatemia, increased intact FGF23 levels, reduced TmP/GFR, insufficient 1,25(OH)2D levels, and in four out of five increased ALP levels. Two patients had radiological signs of rickets. During burosumab, all patients showed a significant increase in serum phosphate and 1,25(OH)2D levels, and in TmP/GFR values (P < 0.05 - P < 0.0001). Serum ALP levels significantly declined (P < 0.05) to normal values. No changes of serum calcium and PTH levels (PNS) were found during burosumab. PROs significantly improved (P < 0.02 - P < 0.0001) in all patients. Four patients discontinued burosumab when they turned 18 or 19, whereas one continued the treatment since he was still younger than 18 during the study period. Four patients who suspended burosumab showed a rapid decline in serum phosphate and 1,25(OH)2D levels and in TmP/GFR values; serum ALP levels increased, and PROs progressively worsened with a significant reduction in quality of life. These consequences were not observed in the patient who continued burosumab treatment. DISCUSSION: Our data showed that conventional treatment improved only in part the signs and symptoms of XLH. Burosumab was well tolerated and was effective in improving phosphate metabolism, bone health, and PROs. All the benefits of burosumab were lost after its discontinuation. These results suggested that continuing burosumab is required to achieve and maintain the clinical benefits of the treatment during the transition to young adulthood in patients with XLH.


Asunto(s)
Anticuerpos Monoclonales Humanizados , Raquitismo Hipofosfatémico Familiar , Factor-23 de Crecimiento de Fibroblastos , Fosfatos , Calidad de Vida , Humanos , Raquitismo Hipofosfatémico Familiar/tratamiento farmacológico , Raquitismo Hipofosfatémico Familiar/genética , Raquitismo Hipofosfatémico Familiar/patología , Adolescente , Masculino , Fosfatos/sangre , Anticuerpos Monoclonales Humanizados/uso terapéutico , Femenino , Niño , Factores de Crecimiento de Fibroblastos/genética , Factores de Crecimiento de Fibroblastos/sangre
15.
J Nutr Sci Vitaminol (Tokyo) ; 70(3): 237-247, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38945889

RESUMEN

Fibroblast growth factor-23 (FGF23) is a phosphaturic hormone secreted by osteocytes in response to dietary phosphate intake. An increase in FGF23 level is an indicator of excess phosphate intake relative to the residual nephron number. Therefore, avoiding excessive phosphate intake and inhibiting the elevation of serum FGF23 levels are important to preserve the number of functional nephrons. This randomized crossover trial aimed to determine the potential differences in the impacts on serum FGF23 levels between plant protein and animal protein-based meals in individuals with normal renal function. Nine young men were administered plant (no animal protein) or animal protein-based meals (70% of their protein was from animal sources) with the same phosphate content. The test meals consisted of breakfast, lunch, and dinner. Blood samples were collected in the morning, after overnight fasting, and before and after eating the test meals (for two consecutive days at the same hour each day). Furthermore, a 24-h urine sample was obtained on the day the test meal was consumed. No significant interactions were found among serum phosphate, calcium, and 1,25-dihydroxyvitamin D levels. However, after eating plant protein-based meals, serum FGF23 levels decreased and serum intact parathyroid hormone levels increased (interaction, p<0.05). Additionally, urine 24-h phosphate excretion tended to be lower in individuals consuming plant protein-based meals than in those consuming animal protein-based meals (p=0.06). In individuals with normal renal function, plant protein-based meals may prevent an increase in serum FGF23 levels and kidney damage caused by phosphate loading.


Asunto(s)
Estudios Cruzados , Factor-23 de Crecimiento de Fibroblastos , Factores de Crecimiento de Fibroblastos , Comidas , Hormona Paratiroidea , Fosfatos , Humanos , Masculino , Factores de Crecimiento de Fibroblastos/sangre , Adulto Joven , Hormona Paratiroidea/sangre , Fosfatos/sangre , Adulto , Proteínas en la Dieta/administración & dosificación , Proteínas en la Dieta/farmacología , Calcio/sangre , Calcio/orina , Vitamina D/sangre , Vitamina D/administración & dosificación , Vitamina D/análogos & derivados
16.
Nutrients ; 16(11)2024 May 24.
Artículo en Inglés | MEDLINE | ID: mdl-38892532

RESUMEN

(1) Background: Cardiovascular diseases (CVDs) are the leading cause of mortality worldwide. The aim of the study was to examine the existing published results of the association between elevated serum phosphate concentrations and cardiovascular mortality, along with the CVD incidence and subclinical coronary atherosclerosis, in primary prevention among non-selected samples of the general population. (2) Methods: A systematic review and meta-analysis were carried out using literature obtained from PubMed, SCOPUS, and the Web Of Science until March 2024 and following the PRISMA guidelines. Relevant information was extracted and presented. Random and fixed effects models were used to estimate the pooled odds ratio (OR) and hazard ratio (HR) with their 95% coefficient interval (CI), and I2 was used to assess heterogeneity. (3) Results: Twenty-five studies met our inclusion criteria and were included in the meta-analysis (11 cross-sectional and 14 cohort studies). For cardiovascular mortality, which included 7 cohort studies and 41,764 adults, the pooled HR was 1.44 (95% CIs 1.28, 1.61; I2 0%) when the highest versus the reference level of serum phosphate concentrations were compared. For CVDs, which included 8 cohort studies and 61,723 adults, the pooled HR was 1.12 (95% CIs 0.99, 1.27; I2 51%). For subclinical coronary atherosclerosis, which included 11 cross-sectional studies and 24,820 adults, the pooled OR was 1.44 (95% CIs 1.15, 1.79; I2 88%). (4) Conclusions: The highest serum phosphate concentrations were positively associated with a 44% increased risk of cardiovascular mortality and subclinical coronary atherosclerosis.


Asunto(s)
Enfermedades Cardiovasculares , Enfermedad de la Arteria Coronaria , Fosfatos , Humanos , Enfermedad de la Arteria Coronaria/sangre , Enfermedad de la Arteria Coronaria/mortalidad , Enfermedad de la Arteria Coronaria/epidemiología , Fosfatos/sangre , Enfermedades Cardiovasculares/mortalidad , Enfermedades Cardiovasculares/sangre , Factores de Riesgo , Femenino , Masculino , Incidencia , Persona de Mediana Edad , Adulto
17.
Folia Med (Plovdiv) ; 66(2): 203-212, 2024 Apr 30.
Artículo en Inglés | MEDLINE | ID: mdl-38690815

RESUMEN

AIM: This study aimed to analyze the oral health conditions of pregnant women. The analysis involves evaluating two key indices: the decayed, missing, and filled teeth (DMFT) index and the basic erosive wear examination (BEWE) index. Furthermore, this study investigated potential correlations between calcium (Ca) and phosphate (P) levels within specific time intervals and the aforementioned oral health indices.


Asunto(s)
Calcio , Salud Bucal , Fosfatos , Humanos , Femenino , Embarazo , Fosfatos/sangre , Fosfatos/análisis , Calcio/sangre , Adulto , Adulto Joven , Índice CPO , Complicaciones del Embarazo/sangre , Complicaciones del Embarazo/epidemiología
18.
BMJ Case Rep ; 17(5)2024 May 02.
Artículo en Inglés | MEDLINE | ID: mdl-38697682

RESUMEN

Tumour-induced osteomalacia is caused by tumorous production of fibroblast growth factor 23 (FGF23) leading to urinary phosphate wasting, hypophosphataemia and decreased vitamin D activation. The resulting osteomalacia presents with muscle weakness and bone pain but progresses to multiple pathological fractures. Patients often remain undiagnosed for years with severe physical, psychological and economic ramifications. A young woman presented with multiple spontaneous fractures including bilateral femoral fractures. Laboratory tests revealed severe hypophosphataemia, elevated bone turnover markers and low to normal calcium and 25-hydroxy-vitamin D levels. Treatment with phosphate, alfalcalcidol, calcium and magnesium was initiated. 68Gallium-DOTATOC positron emission tomography imaging revealed a mass in the right foot and venous sampling of FGF23 from all extremities confirmed this tumour as the culprit. Biopsy and histology were consistent with a phosphaturic mesenchymal tumour, which was surgically resected. Phosphate levels quickly normalised postoperatively but a long convalescence with hungry bone syndrome, fracture healing and physical therapy followed.


Asunto(s)
Factor-23 de Crecimiento de Fibroblastos , Neoplasias de Tejido Conjuntivo , Osteomalacia , Humanos , Osteomalacia/etiología , Femenino , Neoplasias de Tejido Conjuntivo/diagnóstico , Neoplasias de Tejido Conjuntivo/cirugía , Adulto , Síndromes Paraneoplásicos/diagnóstico , Hipofosfatemia/etiología , Factores de Crecimiento de Fibroblastos/sangre , Fracturas del Fémur/cirugía , Fracturas del Fémur/diagnóstico por imagen , Fracturas Espontáneas/etiología , Fracturas Espontáneas/cirugía , Fracturas Espontáneas/diagnóstico por imagen , Fosfatos/sangre
19.
Pancreas ; 53(7): e611-e616, 2024 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-38696363

RESUMEN

OBJECTIVES: Acute pancreatitis (AP) is a complex disease representing a significant portion of gastrointestinal-related hospitalizations in the U.S. Understanding risk factors of AP might provide attractive therapeutic targets. We evaluated hypophosphatemia a prognostic marker in AP. METHODS: We performed a retrospective review of electronic health records of patients with AP from 01/ 01/2012-12/31/2021 at Cedars-Sinai Medical Center with serum phosphate measured within 48 hours of admission. Multivariable logistic regression modeling was used to evaluate associations with ICU admission and AP severity. Multivariable log-linear modeling was employed to examine associations with length of stay (LOS). RESULTS: Of 1526 patients admitted for AP, 33% (499) had a serum phosphate level measured within 48 hours. Patients with hypophosphatemia were more likely to have ICU admission (adjusted odds ratio (AOR) = 4.57; 95% confidence interval (CI): 2.75-7.62; P < 0.001), have a longer hospital stay (log-LOS = 0.34; SE; 0.09; 95% CI: 0.17-0.52; P < 0.001), and have moderate or severe AP (AOR = 1.80; 95% CI: 1.16-2.80; P < 0.001) compared with those without hypophosphatemia. CONCLUSION: Serum phosphate is infrequently measured in patients with AP and shows promise as an early prognostic marker for outcomes of AP.


Asunto(s)
Biomarcadores , Hipofosfatemia , Tiempo de Internación , Pancreatitis , Humanos , Hipofosfatemia/sangre , Hipofosfatemia/diagnóstico , Femenino , Masculino , Estudios Retrospectivos , Pancreatitis/sangre , Pancreatitis/diagnóstico , Persona de Mediana Edad , Pronóstico , Tiempo de Internación/estadística & datos numéricos , Biomarcadores/sangre , Adulto , Anciano , Enfermedad Aguda , Índice de Severidad de la Enfermedad , Fosfatos/sangre , Factores de Riesgo , Unidades de Cuidados Intensivos/estadística & datos numéricos , Modelos Logísticos
20.
Nutrients ; 16(9)2024 Apr 29.
Artículo en Inglés | MEDLINE | ID: mdl-38732596

RESUMEN

Chronic diseases may affect the nutritional status of children and adolescents. Calcium (Ca), phosphorus (P), and vitamin D (Vit-D) are crucial nutrients for their growth and development. Proper diagnosis and treatment are critical components of personalized and precision medicine. Hence, we conducted a cross-sectional and comparative study to evaluate Ca, P, and Vit-D levels in their non-skeletal functions and their association with health and nutritional biomarkers in children and adolescents with diverse chronic conditions. We performed anthropometric, body composition, clinical evaluation, biochemical analysis, and dietary survey methods. A total of 78 patients (1-19 years, 43 females, 42 children) took part in this study. Overall, 24, 30, and 24 participants were obese, undernourished, and eutrophic, respectively. Results found that 74% and 35% of individuals had deficient Vit-D and Ca intake, respectively. Most cases were normocalcemic. Results also found that 47% of the subjects had Vit-D deficiency (VDD), 37% were insufficient, and 37% had hypophosphatemia. Of the 46% and 31% of patients with VDD and insufficient levels, 19% and 11% were hypophosphatemic, respectively. Calcium, P, and Vit-D levels were associated with anthropometric parameters, body mass index, body composition, physical activity, diet, growth hormones, and the immune, liver, and kidney systems. These results show the coincident risk of altered Ca, P, and Vit-D metabolism in children and adolescents with chronic diseases.


Asunto(s)
Calcio , Estado Nutricional , Fosfatos , Deficiencia de Vitamina D , Vitamina D , Humanos , Femenino , Adolescente , Estudios Transversales , Niño , Masculino , Vitamina D/sangre , Enfermedad Crónica , Calcio/sangre , Preescolar , Fosfatos/sangre , Deficiencia de Vitamina D/sangre , Deficiencia de Vitamina D/epidemiología , Lactante , Adulto Joven , Fósforo/sangre , Composición Corporal , Biomarcadores/sangre , Índice de Masa Corporal
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