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Bone Reports Jun 2022Hereditary Hypophosphatemic Rickets with Hypercalciuria (HHRH) ( gene, OMIM 241530) is an autosomal recessive disorder that results in a loss of function of the...
BACKGROUND
Hereditary Hypophosphatemic Rickets with Hypercalciuria (HHRH) ( gene, OMIM 241530) is an autosomal recessive disorder that results in a loss of function of the sodium-phosphate NPT2c channel at the proximal tubule. Phosphate supplementation rarely improves serum phosphate, hypercalciuria, nephrocalcinosis, 1,25(OH) vitamin D (1,25(OH)D) levels or short stature.
METHODS
We describe Na MRI and the successful use of recombinant human growth hormone (rhGH) and Fluconazole to improve growth (possibly confounded by puberty) and hypercalciuria in a now 12-year-old male with HHRH (novel homozygous mutation, c.835_846 + 10del.T).
RESULTS
The patient had chronic bone pain, hypophosphatemia (0.65 mmol/L[reference interval 1.1-1.9]), pathological fractures and medullary nephrocalcinosis/hypercalciuria (urinary calcium/creatinine ratio 1.66 mol/mmol[<0.6]). TmP/GFR was 0.65 mmol/L[0.97-1.64]; 1,25(OH)D was >480 pmol/L[60-208]. Rickets Severity Score was 4. Treatment with 65 mg/kg/day of sodium phosphate and potassium citrate 10 mmol TID failed to correct the abnormalities.Adding rhGH at 0.35 mg/kg/week to the phosphate therapy, improved bone pain, height z-score from -2.09 to -1.42 over 6 months, without a sustained effect on TmP/GFR. Fluconazole was titrated to 100 mg once daily, resulting for the first time in a reduction of the 1,25(OH)D to 462 and 426 pmol/L; serum phosphate 0.87 mmol/L, and calcium/creatinine ratio of 0.73.Na MRI showed normal skin (z-score + 0.68) and triceps surae muscle (z-score + 1.5) Na levels; despite a defect in a sodium transporter, hence providing a rationale for a low sodium diet to improve hypercalciuria.
CONCLUSIONS
The addition of rhGH, Fluconazole and salt restriction to phosphate/potassium supplementation improved the conventional therapy. Larger studies are needed to confirm our findings.
PubMed: 35663378
DOI: 10.1016/j.bonr.2022.101591 -
Drugs in R&D Mar 2024Potassium bismuth citrate is a gastric mucosal protector and a key drug for treating peptic ulcers.
BACKGROUND
Potassium bismuth citrate is a gastric mucosal protector and a key drug for treating peptic ulcers.
OBJECTIVE
To evaluate the pharmacokinetic characteristics and safety of 120-mg bismuth potassium citrate formulations administered orally under fasting conditions in healthy Chinese subjects.
METHOD
A single-center open two-cycle trial was conducted on 12 healthy subjects who received a single oral dose of 120 mg of bismuth potassium citrate. The plasma concentration of bismuth was determined using a validated inductively coupled plasma mass spectrometry (ICP‒MS) method. The pharmacokinetic parameters, including maximum serum concentration (C) and area under the curve concentration-time curve (AUC and AUC), and safety were evaluated via noncompartment analysis.
RESULTS
The ratios of the least square geometric mean ratio between the test (T) and reference (R) formulations for C, AUC, and AUC were 44.8%, 55.5%, and 64.4%, respectively; the bilateral 95% confidence intervals (Cis) for these parameters were 20.2-99.6%, 24.1-127.5%, and 23.7-175.0%, respectively, and the non-inferior limits for these parameters were 169.4%, 198.8%, and 200.5%, respectively. The upper limits of the one-sided 97.5% confidence interval for the least squares geometric mean ratio (T/R) were lower than the non-inferior limits. No serious adverse reactions or adverse reactions leading to detachment were observed among the subjects.
CONCLUSION
The concentration of bismuth in the blood of healthy subjects in the T formulation was not greater than that in the R formulation. Similarly, the safety of oral administration of 120 mg of bismuth potassium citrate formulations to healthy subjects was good. The trial registration number (TRN) was [2018] 013, 6 December 2018.
PubMed: 38345697
DOI: 10.1007/s40268-024-00455-9 -
American Journal of Clinical and... 2022To evaluate the correlation between the pH readings in 24-h urine and the random fasting specimen in patients with urolithiasis using 2 methods.
PURPOSE
To evaluate the correlation between the pH readings in 24-h urine and the random fasting specimen in patients with urolithiasis using 2 methods.
METHODS
A total of 114 patients with urinary lithiasis using potassium citrate were prospectively analyzed. All patients collected 24-h urine and an additional sample, after nocturnal fasting, collected on the day they brought the 24-h sample at the lab. Two different methods (test strip and digital meter) were used to determine pH values.
RESULTS
The pH analysis using strips in the 24-h urine presented a mean value similar to the one obtained in the fasting sample (6.07 ± 0.74 vs. 6.02 ± 0.82, respectively; P > 0.05). The same behavior was seen considering the readings with a digital pH meter (5.8 ± 0.78 vs. 5.75 ± 0.83; P > 0.05). However, readings conducted in the same specimen with pH meter and test strip were dissonant (P < 0.05), suggesting that the colorimetric method is not reliable in the assessment of urinary pH in this population.
CONCLUSION
pH assessment in a random urinary specimen proved as efficient as the 24-h urine standard method to monitor patients with kidney stones in the use of potassium citrate. Classical test strip analysis is not sensitive enough to evaluate the urine pH in this population and digital pH meter reading is preferred.
PubMed: 35874289
DOI: No ID Found -
Kidney International May 2021To study human idiopathic hypercalciuria we developed an animal model, genetic hypercalciuric stone-forming rats, whose pathophysiology parallels that of human...
To study human idiopathic hypercalciuria we developed an animal model, genetic hypercalciuric stone-forming rats, whose pathophysiology parallels that of human idiopathic hypercalciuria. Fed the oxalate precursor, hydroxyproline, every rat in this model develops calcium oxalate stones. Using this rat model, we tested whether chlorthalidone and potassium citrate combined would reduce calcium oxalate stone formation and improve bone quality more than either agent alone. These rats (113 generation) were fed a normal calcium and phosphorus diet with hydroxyproline and divided into four groups: diets plus potassium chloride as control, potassium citrate, chlorthalidone plus potassium chloride, or potassium citrate plus chlorthalidone. Urine was collected at six, 12, and 18 weeks and kidney stone formation and bone parameters were determined. Compared to potassium chloride, potassium citrate reduced urinary calcium, chlorthalidone reduced it further and potassium citrate plus chlorthalidone even further. Potassium citrate plus chlorthalidone decreased urine oxalate compared to all other groups. There were no significant differences in calcium oxalate supersaturation in any group. Neither potassium citrate nor chlorthalidone altered stone formation. However, potassium citrate plus chlorthalidone significantly reduced stone formation. Vertebral trabecular bone increased with chlorthalidone and potassium citrate plus chlorthalidone. Cortical bone area increased with chlorthalidone but not potassium citrate or potassium citrate plus chlorthalidone. Mechanical properties of trabecular bone improved with chlorthalidone, but not with potassium citrate plus chlorthalidone. Thus in genetic hypercalciuric stone-forming rats fed a diet resulting in calcium oxalate stone formation, potassium citrate plus chlorthalidone prevented stone formation better than either agent alone. Chlorthalidone alone improved bone quality, but adding potassium citrate provided no additional benefit.
Topics: Animals; Calcium; Calcium Oxalate; Chlorthalidone; Hypercalciuria; Kidney Calculi; Potassium Citrate; Rats
PubMed: 33417997
DOI: 10.1016/j.kint.2020.12.023 -
Neuropediatrics Oct 2023Although it is a valuable option for children with drug-resistant epilepsy, ketogenic diet (KD) therapy is associated with several side effects. The frequency of kidney...
BACKGROUND
Although it is a valuable option for children with drug-resistant epilepsy, ketogenic diet (KD) therapy is associated with several side effects. The frequency of kidney stones and risk factors for their development in epileptic children receiving KD is unclear. The aim of this study was to determine the frequency and risk factors for the development of renal stones in children receiving KD therapy.
METHODS
A total of 95 patients receiving KD were identified. Of these, seven patients were excluded from the study due to the duration of KD being less than 12 months. The remaining 88 children were enrolled in the study.
RESULTS
Renal stones were detected in 15 patients (17%), of which 12 (73.3%) received potassium citrate treatment. Two (13.3%) patients needed lithotripsy despite receiving potassium citrate treatment, and one of these, who received potassium citrate treatment for 5 months, developed acute vesicourethral reflux and underwent surgery. No patient discontinued KD due to renal stone development. The serum uric acid concentrations and urine calcium/creatinine ratio did not change significantly over the 24-month follow-up period. Age, gender, etiology, age at seizure onset, duration of KD, mobility status, use of topiramate or zonisamide, and the number of antiepileptic drugs used were not significantly different between patients with and without kidney stones.
CONCLUSION
Renal stone appears to be a common adverse effect of KD therapy. Although adequate hydration and potassium citrate treatment are effective in most patients, lithotripsy and surgery may be required in a minority of patients.
Topics: Child; Humans; Diet, Ketogenic; Potassium Citrate; Uric Acid; Kidney Calculi; Risk Factors; Treatment Outcome
PubMed: 37257495
DOI: 10.1055/s-0043-1768987 -
The Journal of Pediatrics Feb 2023The objective of this study was to explore associations between day 10 postpartum (D10) secretory activation biomarkers and the breastfeeding outcome measures.
OBJECTIVE
The objective of this study was to explore associations between day 10 postpartum (D10) secretory activation biomarkers and the breastfeeding outcome measures.
STUDY DESIGN
This prospective longitudinal descriptive study collected antepartum, D10, and day 60 postpartum (D60) questionnaire data and D10 milk samples. Protein, lactose, and citrate were analyzed with enzymatic spectrophotometric assays. Sodium and potassium were analyzed with inductively coupled plasma optical emission spectrophotometry. Group comparison data were analyzed using χ, Fisher exact, and independent sample t tests, as appropriate, using SPSS for Mac (version 28).
RESULTS
Participants (n = 92) provided a D10 breastmilk sample and completed D10 questionnaires, and 83 completed D60 questionnaires. Participants with D10 impaired secretory activation sodium (>23.0 mM) were more likely to report D10 perceived insufficient milk supply, χ = 7.002, P < .05; and less D10 feeding/pumping frequency a day, P < .05; and partial breastfeeding at D60, P < .05. Additionally, participants with D10 impaired secretory activation sodium-to-potassium ratio (sodium: potassium) > 0.8 were more likely to partially breastfeed at D60, P < .05.
CONCLUSION
Elevated milk sodium and sodium: potassium are biomarkers related to variables indicative of low milk supply. Therefore, immediate milk testing can be useful in identifying lactation compromise and improving breastfeeding duration. Because breastfeeding affords maternal and infant health benefits, clinicians should identify ways to measure lactation compromise in conjunction with an examination and clinical history to provide early interventions to increase breastfeeding duration and exclusivity.
Topics: Infant; Female; Humans; Breast Feeding; Milk, Human; Prospective Studies; Lactation; Biomarkers; Potassium; Sodium; Mothers
PubMed: 36208666
DOI: 10.1016/j.jpeds.2022.09.055 -
BMC Urology Aug 2023To assess the routine serum and 24-hour urine tests proficiency in diagnosing the baseline metabolic abnormality of kidney stone formers.
OBJECTIVE
To assess the routine serum and 24-hour urine tests proficiency in diagnosing the baseline metabolic abnormality of kidney stone formers.
METHODS
This study analyzes the routine serum and 24-hour urine tests proficiency in diagnosing the baseline metabolic abnormality of kidney stone formers. The sensitivity and specificity, false positive, and negative results of the tests are extracted from diagnostic kits used in the laboratories of the target community. To accurately infer the results, a simulation based on 1000 people was used through 22 standard laboratory tests (Additional File 2), including calcium, oxalate, phosphate, uric acid, sulfate, potassium, sodium, citrate, and magnesium in 24-hour urine; and calcium, creatinine, Vit D, uric acid, and intact parathyroid hormone (PTH) in serum. The incremental cost-effectiveness ratio (ICER) was calculated and compared for each diagnostic test versus other diagnostic tests according to the incremental cost required for correct diagnoses of stone causes.
RESULTS
Urinary uric acid, citrate, and serum potassium constitute the cost-effectiveness boundary curve in this study. This means that other diagnostic tests are not cost-effective compared to these three tests in terms of indexing at least one item of cost and effectiveness. The ICER index for each correct diagnosis with the urinary uric acid test was $ 1.25 per diagnosis, the most cost-effective test compared to serum potassium and urinary citrate.
CONCLUSION
The simplified blood and 24-hour urine metabolic evaluation, including urinary uric acid, citrate, and serum potassium, constitute the cost-effectiveness boundary curve. The most cost-effective test was urinary uric acid measurement.
Topics: Humans; Calcium; Cost-Benefit Analysis; Uric Acid; Kidney Calculi; Citrates; Citric Acid
PubMed: 37635222
DOI: 10.1186/s12894-023-01310-w -
International Urology and Nephrology Sep 2021To evaluate the effect of potassium citrate administration on the composition of encrusted material on the ureteral stent after Double-J insertion. (Randomized Controlled Trial)
Randomized Controlled Trial
PURPOSE
To evaluate the effect of potassium citrate administration on the composition of encrusted material on the ureteral stent after Double-J insertion.
METHODS
We designed a randomized clinical trial for our study; 65 patients that underwent transurethral lithotripsy and Double-J stent insertion were included in the study after informed consent and divided into two groups. In the first group (33 patients) potassium citrate was prescribed after surgery till stent removal and the second group (32 patients) followed without prescribing this medication. After stent removal, encrusted materials on removed stents were analyzed then the type and composition of encrusted material compared with the primary stone that was removed.
RESULTS
Our results revealed that the type and composition of primary stone and encrusted stone were similar in patients that do not receive potassium citrate (p-value of 0.073, 0.251 and 0.944 for calcium oxalate, uric acid, and calcium phosphate respectively). In patients that taking potassium citrate rate of calcium oxalate (p-value < 0.001) and uric acid (p-value < 0.001) material on encrusted stent significantly decreased compared with the non-intervention group.
CONCLUSION
Results of this study revealed that taking of potassium citrate after ureteral stent insertion significantly decreases the formation of calcium oxalate and uric acid encrusted material on Double-J stent so it could be recommended for prevention of stent encrustation in patients that primary stone analysis are calcium oxalate and uric acid stone.
Topics: Adult; Female; Humans; Male; Middle Aged; Potassium Citrate; Prosthesis Design; Prosthesis Failure; Stents; Ureter; Ureteral Calculi
PubMed: 34050877
DOI: 10.1007/s11255-021-02891-x -
Annals of Translational Medicine Sep 2019
PubMed: 31656798
DOI: 10.21037/atm.2019.08.95 -
Alternative Therapies in Health and... Nov 2023To investigate the clinical impact of dietary intervention in combination with bismuth potassium citrate in the management of chronic atrophic gastritis (CAG) caused by... (Randomized Controlled Trial)
Randomized Controlled Trial
OBJECTIVE
To investigate the clinical impact of dietary intervention in combination with bismuth potassium citrate in the management of chronic atrophic gastritis (CAG) caused by Helicobacter pylori.
METHODS
From April 2019 to October 2022, 160 patients with newly identified Helicobacter pylori-related CAG were treated at our facility. They were split into two groups at random: the bismuth potassium citrate medication group (n = 80) and the diet intervention + bismuth potassium citrate experimental groups (n = 80). The bismuth potassium citrate treatment group was given bismuth potassium citrate capsule treatment only, and the diet intervention + bismuth potassium citrate treatment group was given diet intervention based on bismuth potassium citrate capsule. The diet intervention score, symptom score, and pathological score of the two groups were observed at baseline and after treatment, and the relationship between dietary intervention and symptoms and pathology of Helicobacter pylori-related CAG was analyzed.
RESULTS
During the baseline period, there was no discernible difference in the diet intervention score, symptom score, or pathology score between the two groups (P > .05); after the diet intervention combination treatment, the diet intervention score, diet intervention + bismuth potassium citrate experimental groups symptom score, and pathology score were considerably lower than those in the bismuth potassium citrate treated group (P < .05).
CONCLUSIONS
Dietary intervention combined with bismuth potassium citrate exhibited more effective treatment than bismuth potassium citrate-only treatment in Helicobacter pylori-related CAG, which hinted us proper diet has a positive impact on improving the therapeutic efficacy of bismuth potassium citrate.
Topics: Humans; Amoxicillin; Anti-Bacterial Agents; Bismuth; Drug Therapy, Combination; Gastritis, Atrophic; Helicobacter Infections; Helicobacter pylori; Potassium; Potassium Citrate; Treatment Outcome
PubMed: 37856797
DOI: No ID Found