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RSC Advances Oct 2023Metal organic frameworks (MOFs), with structural tunability, high metal content and large surface area have recently attracted the attention of researchers in the field...
Metal organic frameworks (MOFs), with structural tunability, high metal content and large surface area have recently attracted the attention of researchers in the field of electrochemistry. In this work, an unprecedented use of multi-walled carbon nanotubes (MWCNTs)/copper-based metal-organic framework (Cu-BTC MOF) composite as an ion-to-electron transducer in a potentiometric sensor is proposed for the determination of orphenadrine citrate. A comparative study was conducted between three proposed glassy carbon electrodes, Cu-MOF, (MWCNTs) and MWCNTs/Cu-MOF composite based sensors, where Cu-MOF, MWCNTs and their composite were utilized as the ion-to-electron transducers. The sensors were developed for accurate and precise determination of orphenadrine citrate in pharmaceutical dosage form, spiked real human plasma and artificial cerebrospinal fluid (ACSF). The sensors employed β-cyclodextrin as a recognition element with the aid of potassium tetrakis(4-chlorophenyl)borate (KTpCIPB) as a lipophilic ion exchanger. The sensors that were assessed based on the guidelines recommended by IUPAC and demonstrated a linear response within the concentration range of 10 M to 10 M, 10 M to 10 M and 10 M to 10 M for Cu-MOF, MWCNTs and MWCNTs/Cu-MOF composite based sensors, respectively. MWCNTs/Cu-MOF composite based sensor showed superior performance over other sensors regarding lower limit of detection (LOD), wider linearity range and faster response. The sensors demonstrated their potential as effective options for the analysis of orphenadrine citrate in quality control laboratories and in different healthcare activities.
PubMed: 37876650
DOI: 10.1039/d3ra06710f -
Children (Basel, Switzerland) Jul 2023Löwe syndrome (the oculocerebrorenal syndrome of Löwe, OCRL, OMIM #309000, ORPHA: 534) is a very rare multisystem X-linked disorder characterized by ocular, kidney and...
OBJECTIVES
Löwe syndrome (the oculocerebrorenal syndrome of Löwe, OCRL, OMIM #309000, ORPHA: 534) is a very rare multisystem X-linked disorder characterized by ocular, kidney and nervous system anomalies.
CASE PRESENTATION
We present the first Bulgarian genetically confirmed patient with OCRL. The patient had facial dysmorphism, cryptorchidism, congenital cataracts, nystagmus, delayed physical and mental development, and poor nutritional status. He had severe rickets, metabolic acidosis, hypokalaemia, hypophosphataemia, and low IGF-1 levels at the age of three, in addition to his developmental delay. The molecular-genetic analysis reported a pathogenic variant c.1124A>G, p.H375R in the gene. This variant was inherited from the mother, who was a carrier. Following the diagnosis of OCRL, treatment with potassium citrate, phosphate, and calcitriol was initiated, along with an increase in caloric intake. Following general physical and biochemical improvement, therapy with rhGH started 4 years ago, and current results are presented.
CONCLUSIONS
The patient with Löwe syndrome who was presented with a 6-year follow-up demonstrates the complexity of rare disease cases and the value of multidisciplinary care together with growth hormone treatment for better results in these patients.
PubMed: 37508663
DOI: 10.3390/children10071166 -
Insects Apr 2024In this study, the changes in the conventional nutrient and mineral compositions as well as the metabolomics characteristics of the red palm weevil (RPW) Olivier...
Combined Analysis of Metabolomics and Biochemical Changes Reveals the Nutritional and Functional Characteristics of Red Palm Weevil (Coleoptera: Curculionidae) Larvae at Different Developmental Stages.
In this study, the changes in the conventional nutrient and mineral compositions as well as the metabolomics characteristics of the red palm weevil (RPW) Olivier (Curculionidae: Coleoptera) larvae at early (EL), middle (ML) and old (OL) developmental stages were investigated. Results showed that the EL and ML had the highest content of protein (53.87 g/100 g dw) and fat (67.95 g/100 g), respectively, and three kinds of RPW larvae were all found to be rich in unsaturated fatty acids (52.17-53.12%), potassium (5707.12-15,865.04 mg/kg) and phosphorus (2123.87-7728.31 mg/kg). In addition, their protein contained 17 amino acids with the largest proportion of glutamate. A total of 424 metabolites mainly including lipids and lipid-like molecules, organic acids and their derivatives, organic heterocycle compounds, alkaloids and their derivatives, etc. were identified in the RPW larvae. There was a significant enrichment in the ABC transport, citrate cycle (TCA cycle), aminoacyl-tRNA biosynthesis, and mTOR signaling pathways as the larvae grow according to the analysis results of the metabolic pathways of differential metabolites. The water extract of EL exhibited relatively higher hydroxyl, 2,2-diphenyl-1-pyrroline hydrochloride (DPPH) and 2,2'-azobis (3-ethylbenzothiazoline-6-sulfonic acid) (ABTS) radical-scavenging ability with the EC values of 1.12 mg/mL, 11.23 mg/mL, and 2.52 mg/mL, respectively. These results contribute to a better understanding of the compositional changes of the RPW larvae during its life cycle and provide a theoretical grounding for its deep processing and high-value utilization.
PubMed: 38667424
DOI: 10.3390/insects15040294 -
Materials (Basel, Switzerland) Jul 2023Composites synthesized from iron(III) citrate and carbon spheres, and activated with potassium compounds were prepared and then characterized using XRD, SEM, and...
Composites synthesized from iron(III) citrate and carbon spheres, and activated with potassium compounds were prepared and then characterized using XRD, SEM, and low-temperature nitrogen adsorption methods. The adsorption properties of the composites toward carbon dioxide were assessed using CO uptake measurement, as well as by measuring their selectivity toward carbon dioxide, given their further application as photocatalysts for the reduction of this gas. The effect of changing preparation conditions on the structural and adsorption properties of the material was assessed. The potential strength of such material is a synergistic effect between the high adsorption capacity related to the microporosity of carbon spheres combined with the catalytic properties of iron particles.
PubMed: 37569932
DOI: 10.3390/ma16155227 -
Kidney360 Feb 2024Thiazide treatment successfully lowered urine calcium and both calcium oxalate and calcium phosphate supersaturations in both types of stone formers (SFs). Alkali...
KEY POINTS
Thiazide treatment successfully lowered urine calcium and both calcium oxalate and calcium phosphate supersaturations in both types of stone formers (SFs). Alkali therapy may not confer the same benefits on calcium phosphate SFs as it does on calcium oxalate SFs.
BACKGROUND
Randomized controlled trials have shown that both thiazide diuretics and potassium citrate (K-Cit) can prevent calcium stone recurrence, but most participants formed calcium oxalate (CaOx) stones. While thiazides are expected to lower risk of calcium phosphate (CaP) stone formation, the effect of K-Cit on risk of CaP stone formation is unclear.
METHODS
To study the effect of common calcium stone treatments, we analyzed the 24-hour urines of CaOx and CaP stone formers (SFs) by four treatment types: Lifestyle, K-Cit, Thiazide, or Both medications.
RESULTS
Patients treated with thiazides reduced urine calcium in both CaOx (=−74.4, =94.6 mg/d) and CaP (=−102, =99.7 mg/d) SFs while those on K-Cit had no change in urine calcium. Among CaOx SFs, urine citrate rose in patients administered K-Cit with or without thiazide, but citrate did not rise significantly in CaP SFs. Urine pH rose in all CaOx SFs, but among CaP SFs, only rose in patients receiving K-Cit. CaOx supersaturation (SS) decreased in all patients who received Thiazide, and decreased among CaOx SFs treated with K-Cit. CaP SS decreased in both CaOx SFs (=−0.46, =0.86) and CaP SFs (=−0.76, =0.85) treated with Thiazide, except CaOx SFs who received Both. Patients treated with K-Cit alone increased CaP SS in CaOx SFs (=0.25, =0.79).
CONCLUSIONS
Patients treated with Thiazide lowered urine calcium and SS in both stone groups. Patients treated with K-Cit had no significant changes in urine calcium and had a decrease in CaOx SS in CaOx SFs. The study raises questions about the best preventive treatment for patients with CaP stones and suggests that K-Cit may not confer the same benefits on CaP SFs as it does on CaOx SFs.
Topics: Humans; Calcium Oxalate; Phosphates; Calcium Phosphates; Kidney Calculi
PubMed: 38251931
DOI: 10.34067/KID.0000000000000362 -
Urology Case Reports May 2024Uric acid is one of the few kidney stone minerals that can dissolve using oral alkalinization therapies such as potassium citrate. We report an obese female whose...
Uric acid is one of the few kidney stone minerals that can dissolve using oral alkalinization therapies such as potassium citrate. We report an obese female whose recalcitrant uric acid stones were eliminated using the weight loss medication phentermine/topiramate (Qsymia), a metabolic stimulant and carbonic anhydrase inhibitor. Pre- and post-dissolution 24-h urine studies and computed tomography images are included with a proposed mechanism of action of this medication. This is the first description of a non-alkaline oral therapy used alone for uric acid stone dissolution. Additional investigation of this medication in obese or diabetic uric acid stone formers is warranted.
PubMed: 38756527
DOI: 10.1016/j.eucr.2024.102748 -
Heliyon Feb 2024A denitrifying bacteria, which we named DNB-15, was screened and isolated from the Shengli Oilfield polymer-containing wastewater. The strain was characterized by 16S...
A denitrifying bacteria, which we named DNB-15, was screened and isolated from the Shengli Oilfield polymer-containing wastewater. The strain was characterized by 16S rDNA analysis and the effects of nutrient substrate type, pH, mineralization and temperature on the activity of the strain were also investigated. The strain was identified as , of which the most efficient carbon source is sodium citrate, the most suitable nitrogen source is potassium nitrate, the suitable temperature for growth is 35~45 °C, the suitable pH range for growth is 7.0~9.0, and the maximal tolerable mineralization is 4 × 10 mg/L. The desulfurization experiment showed that DNB-15 has desulfurization ability to some extent. When the initial sulfide concentration is lower than 50 mg/L, DNB-15 grows rapidly, and the sulfides are removed quickly within 24 h, displaying a sulfur removal rate higher than 99 %; When the initial sulfide concentration is higher than 150 mg/L, the growth of DNB-15 is severely restricted, the desulfurization effect is not obvious, and the desulfurization rate is almost stagnant.
PubMed: 38333781
DOI: 10.1016/j.heliyon.2024.e25135 -
CEN Case Reports Apr 2024A 11-year-old girl was referred to the pediatric nephrology services of our hospital for evaluation of vitamin-D-refractory rickets. She was born to second-degree...
A 11-year-old girl was referred to the pediatric nephrology services of our hospital for evaluation of vitamin-D-refractory rickets. She was born to second-degree consanguineous parents. On examination, she had wrist widening and bilateral genu varum. She had normal anion gap metabolic acidosis, hypokalemia, and hyperchloremia. The fractional excretion of bicarbonate was 3% and the urine anion gap was positive. She also had hypercalciuria, but no phosphaturia, glucosuria or aminoaciduria. In view of a family history of an elder sister having rigidity with cognitive and speech impairment, an ophthalmic evaluation by slit lamp examination was performed in the index case that revealed bilateral Kayser-Fleischer rings. Serum ceruloplasmin was low and 24-h urine copper was elevated in the index case. Whole exome sequencing unveiled a novel pathogenic variant in exon 2 of the ATP7B gene (chr13: c.470del; Depth: 142x) (homozygous) that resulted in a frameshift and premature truncation of the protein, 15 amino acids downstream to codon 157 (p. Cys157LeufsTer15; NM_000053.4) confirming Wilson disease. There were no mutations in the ATP6V0A4, ATP6V1B1, SLC4A1, FOXI1, WDR72 genes or other genes that are known to cause distal RTA. Therapy with D-penicillamine and zinc supplements was initiated. A low dose of 2.5 mEq/kg/day of potassium citrate supplementation normalized the serum bicarbonate levels. This case was notable for the absence of hepatic or neurological involvement at admission. Wilson disease is well known to cause proximal renal tubular acidosis and Fanconi syndrome, with relatively lesser involvement of the distal renal tubules in the literature. However, isolated distal renal tubular involvement as presenting manifestation of Wilson disease (without hepatic or neurological involvement) is rare and can lead to diagnostic confusion.
Topics: Aged; Child; Female; Humans; Acidosis, Renal Tubular; Bicarbonates; Forkhead Transcription Factors; Hepatolenticular Degeneration; Mutation; Potassium Citrate; Vacuolar Proton-Translocating ATPases
PubMed: 37415038
DOI: 10.1007/s13730-023-00806-6