Dietary phosphate supplement does not rescue skeletal phenotype in a mouse model for craniometaphyseal dysplasia

Liu, Yaling; Dutra, Eliane H; Reichenberger, Ernst; Chen, I‐Ping

doi:10.1186/s12952-016-0061-0

Cited by 6 publications

(7 citation statements)

References 36 publications

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“…Hypoproteinemia is treated with exogenous human albumin infusions for symptomatic support, according to previous results; nevertheless, albumin is costly (17). There are also certain infusion issues that result in albumin loss, despite the fact that the overall time spent maintaining plasma protein after infusion is minimal (18). As a result, it is unable to effectively treat hypoproteinemia and malnutrition in the body.…”

Section: Discussionmentioning

confidence: 99%

Effect of Steroid Therapy on Growth hormone in Children with Nephrotic Syndrome

Elhameed

Elgendy

El-gawaad

et al. 2021

Benha Journal of Applied Sciences

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Background: Nephrotic syndrome is the most frequent kidney-related condition in the United States today, providing a severe health risk. The cornerstone of therapy is still corticosteroids. Protein deficiency was a major cause of linear growth retardation in nephrotic individuals before steroids were administered. The goal of our research was to see how corticosteroids affected growth hormone in children with nephrotic syndrome. Methodologies: From February to December 2020, a cross-sectional research was conducted on children with nephrotic syndrome who visited the Benha University Nephrology clinic. After receiving written agreement from the patients' parents, the research included 36 patients with nephritic syndrome of both sexes, divided into two groups: corticosteroid response group and corticosteroid plus other immunosuppressive medications group. The following conditions were applied to all of the instances in the study: Growth hormone level in blood was measured after a thorough history, full clinical examination, and laboratory testing. Results: The majority of our NS patients (66.7%) were on corticosteroid medication, while 33.3 percent were on corticosteroid plus immunosuppressive medication, according to our research. Complete remission was 1 (2.8%), steroid dependent was 20 (55.5%), rare recurrence was 3 (8.4%), and corticosteroid resistant was 12 in our research (33.3). According to the findings of the present research, small stature accounted for 15 percent of the cases analysed (41.7 percent ). The G.H distribution in the examined cases was found to vary between 0.04 and 2.80ng/ml, with a mean of 0.76 0.86. The distribution of elicited growth hormone in the examined patients varied from 1.17 to 14.60 (ng/ml), with a mean of 5.28 2.81. According to this research, the distribution of activated growth hormone categories among the analysed patients was as follows: borderline (10.78%), normal (6.17%), and subnormal (20%). (55.6 percent ). There was a statistically significant negative connection between cumulative steroid dosage and mortality in the present trial (growth hormone and provocated Growth hormone). Conclusion: The majority of children with nephrotic syndrome exhibited a height disadvantage. Children with nephrotic syndrome may have growth retardation as a result of chronic steroid therapy. In most instances of NS, growth hormone levels are below normal. Corticosteroid cumulative doses have a negative impact on linear growth.

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Section: Discussionmentioning

confidence: 99%

Effect of Steroid Therapy on Growth hormone in Children with Nephrotic Syndrome

Elhameed

Elgendy

El-gawaad

et al. 2021

Benha Journal of Applied Sciences

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“…Previous findings have shown that hypoproteinemia is treated by the infusion of exogenous human albumin for symptomatic support treatment; however, albumin is expensive ( 10 ). Additionally, there are certain infusion complications, leading to loss of albumin while the total time of maintaining plasma protein after infusion is short ( 11 ). Therefore, it cannot fundamentally correct the hypoproteinemia and malnutrition of the body.…”

Section: Discussionmentioning

confidence: 99%

Effects of recombinant human growth hormone on protein malnutrition and IGF-1 and IL-2 gene expression levels in chronic nephrotic syndrome

2018

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The aim of the study was to investigate the effects of recombinant human growth hormone on protein malnutrition and insulin-like growth factor-1 (IGF-1) and interleukin-2 (IL-2) gene expressions in chronic nephrotic syndrome. Eighty patients with chronic nephrotic syndrome were admitted to our hospital. The patients were included in the study period from January 2015 to December 2016 and were divided into two groups (40 cases in each group) according to the random number method. All the patients enrolled received symptomatic and supportive treatment. The observation group was injected subcutaneously with recombinant human growth hormone, while the control group was treated with Shenyankangfu tablets. The recovery time of the clinical symptoms, change in serum protein, caloric intake and protein metabolism after intervention were compared between the two groups. Changes in serum cystatin C, IGF-1 and IL-2 before intervention, and at 1 week, 1 month and 3 months after intervention were detected, and the adverse reactions in the two groups were observed during the treatment. After intervention, the improvement time of proteinuria, hypoproteinemia, edema and hyperlipidemia in the observation group was significantly shorter than that in the control group (P<0.05). The expression of transferrin, pre-albumin, albumin and total protein in the observation group was significantly superior increased compared to those in the observation group prior to intervention and the control group after intervention (P<0.05). In addition the caloric intake, protein intake and urea nitrogen survival rate in the observation group were significantly superior to those in the observation group prior to intervention and the control group after intervention (P<0.05). At 1 week, 1 month and 3 months after intervention, the levels of serum cystatin C, IGF-1 and IL-2 in the observation group were markedly obviously lower than those in the control group during the same period (P<0.05). The total proportion of allergy, systemic pruritus, nausea and vomiting, abdominal distension and abdominal pain in the observation group was obviously lower than that in the control group (P<0.05). Compared with the traditional Chinese medicine Shenyankangfu tablets applied in the control group, the recombinant human growth hormone used for patients with chronic nephrotic syndrome can improve the clinical symptoms more quickly, regulate the protein metabolism and reduce the inflammatory response in the body, which also has fewer adverse reactions and higher safety.

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“…( 5 ) Although Ank KI/KI mice showed a tendency of decreased serum Pi, the difference was not statistically significant in comparison to Ank +/+ mice. ( 10 ) Other Pi regulating hormones such as PTH, 25‐OHD, and intact and cleaved FGF23, were unexpectedly normal in the CMD mouse model as well as in patients with CMD. ( 11 ) Ank KI/KI mice have reduced osteoblastogenesis.…”

Section: Introductionmentioning

confidence: 98%

Restriction of Dietary Phosphate Ameliorates Skeletal Abnormalities in a Mouse Model for Craniometaphyseal Dysplasia

Fujii

Kozák

Dutra

et al. 2020

Journal of Bone and Mineral Research

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Craniometaphyseal dysplasia (CMD), a rare genetic bone disorder, is characterized by lifelong progressive thickening of craniofacial bones and metaphyseal flaring of long bones. The autosomal dominant form of CMD is caused by mutations in the progressive ankylosis gene ANKH (mouse ortholog Ank), encoding a pyrophosphate (PPi) transporter. We previously reported reduced formation and function of osteoblasts and osteoclasts in a knockin (KI) mouse model for CMD (Ank KI/KI) and in CMD patients. We also showed rapid protein degradation of mutant ANK/ANKH. Mutant ANK protein displays reduced PPi transport, which may alter the inorganic phosphate (Pi) and PPi ratio, an important regulatory mechanism for bone mineralization. Here we investigate whether reducing dietary Pi intake can ameliorate the CMD-like skeletal phenotype by comparing male and female Ank +/+ and Ank KI/KI mice exposed to a low (0.3%) and normal (0.7%) Pi diet for 13 weeks from birth. Serum Pi and calcium (Ca) levels were not significantly changed by diet, whereas PTH and 25-hydroxy vitamin D (25-OHD) were decreased by low Pi diet but only in male Ank +/+ mice. Importantly, the 0.3% Pi diet significantly ameliorated mandibular hyperostosis in both sexes of Ank KI/KI mice. A tendency of decreased femoral trabeculation was observed in male and female Ank +/+ mice as well as in male Ank KI/KI mice fed with the 0.3% Pi diet. In contrast, in female Ank KI/KI mice the 0.3% Pi diet resulted in increased metaphyseal trabeculation. This was also the only group that showed increased bone formation rate. Low Pi diet led to increased osteoclast numbers and increased bone resorption in all mice. We conclude that lowering but not depleting dietary Pi delays the development of craniofacial hyperostosis in CMD mice without severely compromising serum levels of Pi, Ca, PTH, and 25-OHD. These findings may have implications for better clinical care of patients with CMD.

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Dietary phosphate supplement does not rescue skeletal phenotype in a mouse model for craniometaphyseal dysplasia

Cited by 6 publications

References 36 publications

Effect of Steroid Therapy on Growth hormone in Children with Nephrotic Syndrome

Effect of Steroid Therapy on Growth hormone in Children with Nephrotic Syndrome

Effects of recombinant human growth hormone on protein malnutrition and IGF-1 and IL-2 gene expression levels in chronic nephrotic syndrome

Restriction of Dietary Phosphate Ameliorates Skeletal Abnormalities in a Mouse Model for Craniometaphyseal Dysplasia

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