Grip Strength in Adults and Children with Cystinosis

Iyob-Tessema, Helina; Wang, Chia-shi; Kennedy, Sabina; Reyes, Loretta; Shin, Stella; Greenbaum, Larry A.; Hogan, Julien

doi:10.1016/j.ekir.2020.11.017

Cited by 4 publications

(5 citation statements)

References 28 publications

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“…Additionally, in another study, according to the degree of CKD, there were differences in the HGS z-score, which found those with stage 2-5 CKD had a lower HGS z-score than those with stage 1 CKD [59]. HGS is a reliable bedside tool for nutritional assessments in children with CKD [55,59,60].…”

Section: Feasibility and Accuracy Of Hgs In Detection Mwd Among Hospi...mentioning

confidence: 94%

“…HGS z-scores are used in children with CKD to assess muscle strength and detect MWD. For example, a cohort study assessing muscle strength found that HGS z-scores in children suffering from CKD with cystinosis were lower than those in children with CKD without cystinosis [55]. Additionally, in another study, according to the degree of CKD, there were differences in the HGS z-score, which found those with stage 2-5 CKD had a lower HGS z-score than those with stage 1 CKD [59].…”

Section: Feasibility and Accuracy Of Hgs In Detection Mwd Among Hospi...mentioning

confidence: 98%

“…MWD is common among patients with chronic diseases such as cystic fibrosis (CF) [53], Duchenne muscular dystrophy (DMD) [54], chronic kidney disease (CKD) [55], cancer [12], and hematopoietic cell transplantation (HCT) [56]. Muscle assessment was also considered a major part of overall nutritional assessments in many studies that used the HGS z-score as one of the tools to assess muscle strength [23,[50][51][52].…”

Section: Feasibility and Accuracy Of Hgs In Detection Mwd Among Hospi...mentioning

confidence: 99%

“…The study found significantly lower HGS z-scores in the undernutrition group than in the normal and overnutrition groups [23,56,61] A summary of the findings of all the above-mentioned studies is presented in Table 3, which shows that HGS has been frequently used in field research conducted on hospitalized children to assess the overall nutritional status and muscle strength in particular. However, almost all these studies recruited children with a single chronic disease and supported the use of HGS as a clinically accurate and feasible tool in hospital settings among children with certain chronic diseases [12,[53][54][55][56]. We cannot generalize these results to all hospitalized children due to a lack of cutoff points to determine MWD, as well as the need to extract data relevant to age, gender, ethnicity, pubertal phases, and clinical population.…”

Section: Feasibility and Accuracy Of Hgs In Detection Mwd Among Hospi...mentioning

confidence: 99%

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Muscle Wasting among Hospitalized Children: A Narrative Review of the Feasibility and Accuracy of Diagnostic Methods

AlQahtani

Aldisi

2023

Children

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Muscle wasting is associated with a worse quality of life and increased morbidity and mortality among hospitalized children, especially those with chronic diseases. This review was conducted to summarize the data available on the most feasible and accurate diagnostic methods for detecting muscle wasting among hospitalized children, especially to review the evidence of the accuracy and feasibility of conducting assessments using handgrip strength (HGS). Many diagnostic methods are used in hospital settings to assess muscle wasting, by evaluating either muscle mass or strength, with evidentiary support for assessing muscle mass provided using ultrasonography, magnetic resonance imaging, computed tomography, dual-energy X-ray, bioelectrical impedance analysis, and anthropometry measurements. Currently, the most common diagnostic method used to detect muscle strength loss is the handheld dynamometer. Studies support using HGS among healthy and hospitalized children to assess the overall nutritional status and especially muscle function. However, almost all of these studies have been conducted in hospital settings and recruited children with different chronic diseases using a small sample size. More longitudinal cohort studies with large sample sizes are needed to assess the accuracy and feasibility of using HGS among hospitalized children.

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Section: Feasibility and Accuracy Of Hgs In Detection Mwd Among Hospi...mentioning

confidence: 94%

Section: Feasibility and Accuracy Of Hgs In Detection Mwd Among Hospi...mentioning

confidence: 98%

Section: Feasibility and Accuracy Of Hgs In Detection Mwd Among Hospi...mentioning

confidence: 99%

Section: Feasibility and Accuracy Of Hgs In Detection Mwd Among Hospi...mentioning

confidence: 99%

See 2 more Smart Citations

Muscle Wasting among Hospitalized Children: A Narrative Review of the Feasibility and Accuracy of Diagnostic Methods

AlQahtani

Aldisi

2023

Children

View full text Add to dashboard Cite

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“…Later, patients show pronounced distal weakness, more so than proximal weakness and contractures [ 35 ]. A recent study in 76 pediatric and adult INC patients showed a mean grip strength SD-score of −2.1, which was significantly lower compared to CKD patients with other underlying kidney diseases [ 39 ]. Reduced grip strength was associated with the male gender, delayed cysteamine therapy, and low levels of physical activity.…”

Section: Cystinosis Metabolic Bone Diseasementioning

confidence: 99%

Muscle and Bone Impairment in Infantile Nephropathic Cystinosis: New Concepts

Haffner

Leifheit-Nestler

Alioli

et al. 2022

Cells

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Cystinosis Metabolic Bone Disease (CMBD) has emerged during the last decade as a well-recognized, long-term complication in patients suffering from infantile nephropathic cystinosis (INC), resulting in significant morbidity and impaired quality of life in teenagers and adults with INC. Its underlying pathophysiology is complex and multifactorial, associating complementary, albeit distinct entities, in addition to ordinary mineral and bone disorders observed in other types of chronic kidney disease. Amongst these long-term consequences are renal Fanconi syndrome, hypophosphatemic rickets, malnutrition, hormonal abnormalities, muscular impairment, and intrinsic cellular bone defects in bone cells, due to CTNS mutations. Recent research data in the field have demonstrated abnormal mineral regulation, intrinsic bone defects, cysteamine toxicity, muscle wasting and, likely interleukin-1-driven inflammation in the setting of CMBD. Here we summarize these new pathophysiological deregulations and discuss the crucial interplay between bone and muscle in INC. In future, vitamin D and/or biotherapies targeting the IL1β pathway may improve muscle wasting and subsequently CMBD, but this remains to be proven.

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Leptin signalling altered in infantile nephropathic cystinosis‐related bone disorder

Cheung,

Zhou,

Zheng

et al. 2024

J cachexia sarcopenia muscle

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BackgroundThe CTNS gene mutation causes infantile nephropathic cystinosis (INC). Patients with INC develop Fanconi syndrome and chronic kidney disease (CKD) with significant bone deformations. C57BL/6 Ctns−/− mice are an animal model for studying INC. Hyperleptinaemia results from the kidney's inability to eliminate the hormone leptin in CKD. Ctns−/− mice have elevated serum leptin concentrations. Leptin regulates bone metabolism through its receptor that signals further via the hypothalamic melanocortin 4 receptor (MC4R). Leptin signalling may affect bone health in Ctns−/− mice.MethodsWe first defined the time course of bone abnormalities in Ctns−/− mice between 1 and 12 months of age. We used both genetic and pharmacological approaches to investigate leptin signalling in Ctns−/− mice. We generated Ctns−/−Mc4r−/− double knockout mice. Bone phenotype of Ctns−/−Mc4r−/− mice, Ctns−/− mice and wild type (WT) mice at 1, 4, and 9 months of age were compared. We then treated 12‐month‐old Ctns−/− mice and WT mice with a pegylated leptin receptor antagonist (PLA) (7 mg/kg/day, IP), a MC4R antagonist agouti‐related peptide (AgRP) (2 nmol, intracranial infusion on days 0, 3, 6, 9, 12, 15, 18, 21, 24, and 27), or vehicle (normal saline), respectively, for 28 days. Whole‐body (BMC/BMD, bone area) and femoral bone phenotype (BMC/BMD, bone area, length and failure load) of mice were measured by DXA and femoral shaft biochemical test. We also measured lean mass content by EchoMRI and muscle function (grip strength and rotarod activity) in mice. Femur protein content of JAK2 and STAT3 was measured by ELISA kits, respectively.ResultsBone defects are present in Ctns−/− mice throughout its first year of life. The deletion of the Mc4r gene attenuated bone disorder in Ctns−/− mice. Femoral BMD, bone area, length, and strength (failure load) were significantly increased in 9‐month‐old Ctns−/−Mc4r−/− mice than in age‐matched Ctns−/− mice. PLA and AgRP treatment significantly increased femoral bone density (BMC/BMD) and mechanical strength in 12‐month‐old Ctns−/− mice. We adopted the pair‐feeding approach for this study to show that the protective effects of PLA or AgRP on bone phenotype are independent of their potent orexigenic effect. Furthermore, an increase in lean mass and in vivo muscle function (grip strength and rotarod activity) are associated with improvements in bone phenotype (femoral BMC/BMD and mechanical strength) in Ctns−/− mice, suggesting a muscle‐bone interplay. Decreased femur protein content of JAK2 and STAT3 was evident in Ctns−/− mice. PLA or AgRP treatment attenuated femur STAT3 content in Ctns−/− mice.ConclusionsOur findings suggest a significant role for dysregulated leptin signalling in INC‐related bone disorder, either directly or potentially involving a muscle‐bone interplay. Leptin signalling blockade may represent a novel approach to treating bone disease as well as muscle wasting in INC.

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Grip Strength in Adults and Children with Cystinosis

Cited by 4 publications

References 28 publications

Muscle Wasting among Hospitalized Children: A Narrative Review of the Feasibility and Accuracy of Diagnostic Methods

Muscle Wasting among Hospitalized Children: A Narrative Review of the Feasibility and Accuracy of Diagnostic Methods

Muscle and Bone Impairment in Infantile Nephropathic Cystinosis: New Concepts

Leptin signalling altered in infantile nephropathic cystinosis‐related bone disorder

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