BACKGROUND: Secondary hyperparathyroidism (SHPT) is a common complication of chronic kidney disease (CKD). Cinacalcet use is controversial in non-dialysis patients. METHODS: This retrospective observational study recruited patients receiving cinacalcet (off-label use) in 2010 and 2011. Patients were followed for three years from the beginning of treatment using an intention-to-treat approach. RESULTS: Forty-one patients were studied: 14 CKD stage 3 (34.1%), 21 CKD stage 4 (51.2%), and 6 CKD stage 5 (14.6%). Median baseline parathyroid hormone (PTH) was 396 (101–1,300) pg/mL. Upon cinacalcet treatment (22 ± 12 months), PTH levels decreased by ≥ 30% in 73.2% of patients (P < 0.001; 95% confidence interval [CI], 59–87%), with a mean time for response of 18.7 months (95% CI, 15.4–22.1). Sixteen patients were followed for 36 months and treated for 32 ± 9 months. Mean reduction in their PTH levels was 50.1% (P < 0.001; 95% CI, 33.8–66.4%) at 36 months, with 62.5% of patients (P < 0.001; 95% CI, 35.9–89.1%) presenting reductions of ≥ 30%. Serum calcium levels decreased from 9.95 ± 0.62 mg/dL to 9.21 ± 0.83 and 9.12 ± 0.78 mg/dL at 12 and 36 months, respectively (P < 0.001). Serum phosphorus levels increased from 3.59 ± 0.43 to 3.82 ± 0.84 at 12 months (P = 0.180), remaining so at 36 months (P = 0.324). At 12 and 36 months, 2 (12.5%) patients experienced hypocalcemia. Meanwhile, 1 (6.3%) and 4 (25.0%) patients reported hyperphosphatemia at 12 and 36 months, respectively. CONCLUSION: Cinacalcet remained effective for at least 36 months in non-dialysis patients with SHPT. Electrolytic disturbances were managed with concurrent use of vitamin D and its analogs or phosphate binders.
Calcium ; Cinacalcet Hydrochloride ; Humans ; Hyperparathyroidism, Secondary ; Hyperphosphatemia ; Hypocalcemia ; Observational Study ; Parathyroid Hormone ; Phosphorus ; Renal Insufficiency, Chronic ; Retrospective Studies ; Vitamin D

BACKGROUND: Chronic kidney disease (CKD)-mineral and bone disorder (MBD) and fracture risk are both closely related to declining renal function. Controlling hyperphosphatemia with phosphate binders is a basic principle of CKD-MBD treatment. The aim of this study was to identify differences in fracture risk between pre-dialysis CKD patients and end-stage renal disease (ESRD) on dialysis, and to evaluate the effects of phosphate binders on fracture risk in ESRD patients. METHODS: Data from a total of 89,533 CKD patients comprising CKD diagnosis, dialysis, fracture history, and phosphate binder prescription history from 2012 to 2016 were retrieved from the Health Insurance Review and Assessment Service Database. Multivariate Cox regression analyses were performed to identify whether dialysis or phosphate binders were associated with an increased fracture risk. RESULTS: Overall, the rate of fractures in pre-dialysis CKD patients was 74 per 1,000 patient-years, while that in dialysis patients was 84 per 1,000 patient-years. The risk of fracture in ESRD patients was higher than pre-dialysis CKD patients (hazard ratio, 1.16; 95% confidence interval, 1.12–1.21; P < 0.001) after adjusting for confounding variables. In addition, the fracture risk in patients who were not taking phosphate binders was 20.0% higher compared to ESRD patients taking phosphate binders. CONCLUSION: Fractures were more prevalent in ESRD patients on dialysis than pre-dialysis CKD patients. Use of phosphate binders was associated with a lower fracture risk in ESRD patients.
Cohort Studies ; Confounding Factors (Epidemiology) ; Diagnosis ; Dialysis ; Humans ; Hyperphosphatemia ; Insurance, Health ; Kidney Failure, Chronic ; Prescriptions ; Renal Insufficiency, Chronic

We report a case of transient pseudohypoparathyroidism in a full-term newborn that presented at 20 hours of life with hypocalcemic seizures, hyperphosphatemia and raised parathormone levels. The diagnosis of pseudohypoparathyroidism was made according to biochemical investigations. The infant was treated with calcium supplementation and vitamin D analog therapy, and he remained stable and symptom-free with normal serum biochemistries during follow-up. We suggest that transient pseudohypoparathyroidism of the newborn (ntPHP) might be included among inactivating parathyroid hormone (PTH)/PTH-related protein signaling disorders as defined by the classification schema recently proposed by the European Pseudohypoparathyroidism Network. To the best of our knowledge, this is the first report in which the new classification has been applied to a case of ntPHP.
Calcium ; Classification ; Diagnosis ; Follow-Up Studies ; Humans ; Hyperphosphatemia ; Infant ; Infant, Newborn ; Parathyroid Hormone ; Precision Medicine ; Pseudohypoparathyroidism ; Seizures ; Vitamin D

BACKGROUND: For phosphate control, patient education is essential due to the limited clearance of phosphate by dialysis. However, well-designed randomized controlled trials about dietary and phosphate binder education have been scarce. METHODS: We enrolled maintenance hemodialysis patients and randomized them into an education group (n = 48) or a control group (n = 22). We assessed the patients’ drug compliance and their knowledge about the phosphate binder using a questionnaire. RESULTS: The primary goal was to increase the number of patients who reached a calcium-phosphorus product of lower than 55. In the education group, 36 (75.0%) patients achieved the primary goal, as compared with 16 (72.7%) in the control group (P = 0.430). The education increased the proportion of patients who properly took the phosphate binder (22.9% vs. 3.5%, P = 0.087), but not to statistical significance. Education did not affect the amount of dietary phosphate intake per body weight (education vs. control: −1.18 ± 3.54 vs. −0.88 ± 2.04 mg/kg, P = 0.851). However, the dietary phosphate-to-protein ratio tended to be lower in the education group (−0.64 ± 2.04 vs. 0.65 ± 3.55, P = 0.193). The education on phosphate restriction affected neither the Patient-Generated Subjective Global Assessment score (0.17 ± 4.58 vs. −0.86 ± 3.86, P = 0.363) nor the level of dietary protein intake (−0.03 ± 0.33 vs. −0.09 ± 0.18, P = 0.569). CONCLUSION: Education did not affect the calcium-phosphate product. Education on the proper timing of phosphate binder intake and the dietary phosphate-to-protein ratio showed marginal efficacy.
Body Weight ; Compliance ; Dialysis ; Diet* ; Dietary Proteins ; Education* ; Humans ; Hyperphosphatemia ; Patient Education as Topic ; Phosphates ; Renal Dialysis*

No abstract available.
Humans ; Hyperphosphatemia* ; Patient Education as Topic*

No abstract available.
Humans ; Hyperphosphatemia* ; Mortality* ; Renal Dialysis*

No abstract available.
Humans ; Hyperphosphatemia* ; Mortality* ; Renal Dialysis*

Introduction: This is a prospective, post marketing surveillance study that aims to determine the efficacy and safety of sevelamer carbonate in hyperphosphatemic chronic kidney disease (CKD) patients in the Philippines. Methods: Adult CKD patients with serum phosphorous levels >1.78 mmol/L and whose physician had decided to treat with sevelamer carbonate 800 mg were enrolled in the study and followed-up for a minimum of three visits from baseline within a six-month period. The primary endpoint was the change in serum phosphorous levels from baseline to the sixth month. Adverse events were noted and recorded during the treatment period. Results: There were 233 patients included in the study from five centers in Metro Manila from 2010 to 2013. Of the 233 patients, 199 were on chronic dialysis, 33 were not on dialysis, and 1 had no data. There was a statistically significant (P-value <0.0001) reduction in serum phosphorous levels from baseline after treatment with sevelamer carbonate. There were 16 patients reported to have adverse drug reactions, 13 of whom had serious adverse events (SAE) and three were non-serious. Of the 13 patients with SAEs, only one was possibly/probably related to sevelamer carbonate and all three non-SAEs were possibly/definitely related to sevelamer carbonate. Conclusion The results showed sevelamer carbonate to be effective in lowering serum phosphorous levels and the most common adverse events were related to the gastrointestinal tract (1.4%). There were sixteen patients with adverse events, three of which were non-serious, while 13 were reported to be serious adverse events. Only one was probably related to the drug.
Renal Insufficiency, Chronic ; Hyperphosphatemia ; Sevelamer

Pseudohypoparathyroidism type 1b (PHP 1b) is the result of end organ resistance to parathyroid hormone (PTH) in the absence of any features of Albright's hereditary osteodystrophy. There are two subtypes of PHP 1b with different genetic mechanisms. One subtype is related to a maternally derived 3kb microdeletion involving STX 16 gene, and is inherited in an autosomal dominant mode. Familial autosomal dominant inheritance of PHP 1b is relatively rare. The other subtype is associated with more extensive loss of imprinting at the GNAS locus that affects at least one additional differential methylated (hypermethylation at neuroendocrine secretory protein and hypomethylation at antisense transcript and or extra-large stimulatory G protein region) without microdeletion of the STX 16 or AS gene. It can be sporadic due to an imprinting defect in the GNAS gene. In our case, an 8-year-old girl was referred for suspected PHP with no feature of Albright hereditary osteodystrophy. Blood test results revealed hypocalcemia and hyperphosphatemia. Elevated PTH was also checked. There was no family history of endocrine or developmental problem. Her intelligence was normal, but she had inferior sociability at that time. Based on above, we diagnosed a rare case of paternal uniparental disomy of the long arm of chromosome 20 as the cause of PHP 1b by microsatellite marker test of chromosome 20.
Arm ; Child ; Chromosomes, Human, Pair 20 ; Female ; GTP-Binding Proteins ; Hematologic Tests ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Intelligence ; Microsatellite Repeats ; Parathyroid Hormone ; Pseudohypoparathyroidism* ; Uniparental Disomy* ; Wills

Pseudohypoparathyroidism (PHP) is a rare disorder caused by genetic and epigenetic aberrations in the GNAS complex locus resulting in impaired expression of stimulatory G protein (Gsα). PHP type Ib (PHP-Ib) is characterized by hypocalcemia and hyperphosphatemia due to renal resistance to the parathyroid hormone, and is distinguished from PHP-Ia by the absence of osteodystrophic features. An 11-yr-old boy presented with poor oral intake and cramping lower limb pain after physical activity. Laboratory studies revealed hypocalcemia, hyperphosphatemia, and increased parathyroid hormone levels. The GNAS complex locus was evaluated using the methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) assay. Gain of methylation in the NESP55 domain and loss of methylation in the antisense (AS) transcript, XL, and A/B domains in the maternal allele were observed. Consequently, we present a case of PHP-Ib diagnosed using MS-MLPA.
Alleles ; Epigenomics ; GTP-Binding Proteins ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Lower Extremity ; Male ; Methylation* ; Motor Activity ; Multiplex Polymerase Chain Reaction ; Muscle Cramp ; Parathyroid Hormone ; Pseudohypoparathyroidism*