scholarly journals The Significance of Serum Phosphate Level on Healing Index and Its Relative Effects in Skeletally Immature and Mature Patients with Hypophosphatemic Rickets

2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Sang-Heon Song ◽  
Hanna Lee ◽  
Ji-Min Jeong ◽  
Woo-In Cho ◽  
Sung Eun Kim ◽  
...  
Keyword(s):  
Skeletal Maturity ◽  
Deformity Correction ◽  
Serum Phosphate ◽  
Hypophosphatemic Rickets ◽  
Serum Phosphate Level ◽  
Skeletally Immature ◽  
Laboratory Findings ◽  
Healing Index ◽  
Preoperative Serum ◽  
Immature Group

The aim of this study was to find out the ideal cut-off level of phosphate for safe healing when deformity correction and concomitant lengthening are indicated in the two different skeletal maturity groups of patients with rickets. Thirty-nine hypophosphatemic rickets patients were selected for the study and were divided into two groups: 27 skeletally immature (group IM) and 12 skeletally mature (group M). The outcomes were evaluated with respect to the healing index (HI), laboratory findings, and complications with the mean follow-up of 5.1 years (range, 3.1–7.9). The healing index (HI) of group IM was 1.44 month/cm and HI of group M was 1.68 month/cm. The negative correlation between the level of serum phosphate and HI in group M (coefficient = −0.94) was evaluated to be less than the correlation in group IM (coefficient = −0.50), indicating that the HI is more likely to be affected by serum phosphate in group M than in group IM. Preoperative serum phosphate levels of 2.3 mg/dL and 2.6 mg/dL were analyzed to be the cut-off values of group IM and group M, respectively, in which the cut-off points divided the series into two groups having the most significantly different HI.

scholarly journals Genotype and Phenotype Analysis in X-Linked Hypophosphatemia

2021 ◽  
Vol 9 ◽  
Author(s):  
Peong Gang Park ◽  
Seon Hee Lim ◽  
HyunKyung Lee ◽  
Yo Han Ahn ◽  
Hae Il Cheong ◽  
...  
Keyword(s):  
Serum Phosphate ◽  
Hypophosphatemic Rickets ◽  
Phosphate Level ◽  
Serum Phosphate Level ◽  
Onset Age ◽  
Presenting Symptoms ◽  
Truncating Mutation ◽  
Mutation Group

Background: X-linked hypophosphatemia (XLH) is the most frequent form of hypophosphatemic rickets and is caused by mutations in the PHEX gene. We analyzed genotype-phenotype correlations in XLH patients with proven PHEX mutations.Methods:PHEX mutations were detected in 55 out of 81 patients who clinically presented with hypophosphatemic rickets. The patients were grouped into nontruncating (n = 9) and truncating (n = 46) mutation groups; their initial presentation as well as long-term clinical findings were evaluated according to these groups.Results: Initial findings, including presenting symptoms, onset age, height standard deviation scores (SDS), and laboratory tests, including serum phosphate level and tubular resorption of phosphate, were not significantly different between the two groups (onset age: nontruncating mutation group, 2.0 years, truncating mutation group, 2.2 years; height SDS: nontruncating mutation group, −1.9, truncating mutation group, −1.7; serum phosphate: nontruncating mutation group, 2.5 mg/dL, truncating mutation group, 2.6 mg/dL). However, at their last follow-up, the serum phosphate level was significantly lower in patients with truncating mutations (nontruncating mutation group: 3.2 mg/dl, truncating mutation group: 2.3 mg/dl; P = 0.006). Additionally, 62.5% of patients with truncating mutations developed nephrocalcinosis at their last follow-up, while none of the patients with nontruncating mutations developed nephrocalcinosis (P = 0.015). Orthopedic surgery due to bony deformations was performed significantly more often in patients with truncating mutations (52.3 vs. 10.0%, P = 0.019).Conclusion: Although considerable inconsistency exists regarding the correlation of truncating mutations and their disease phenotype in several other studies, we cautiously suggest that there would be genotype-phenotype correlation in some aspects of disease manifestation after long-term follow-up. This information can be used when consulting patients with confirmed XLH regarding their disease prognosis.

scholarly journals Vertebral growth modulation by posterior dynamic deformity correction device in skeletally immature patients with moderate adolescent idiopathic scoliosis

2020 ◽  
Vol 9 (1) ◽  
pp. 149-153 ◽  
Author(s):  
Yizhar Floman ◽  
Ron El-Hawary ◽  
Baron S. Lonner ◽  
Randal R. Betz ◽  
Uri Arnin
Keyword(s):  
Vertebral Body ◽  
Skeletal Maturity ◽  
Deformity Correction ◽  
Skeletally Immature ◽  
Growth Modulation ◽  
Apical Vertebra ◽  
Curve Magnitude ◽  
Post Hoc ◽  
One Way Anova

Abstract Study design Retrospective, comparative, multicenter. Introduction Growth modulating spinal implants are used in the management of scoliosis such as anterior vertebral body tethering. A motion-sparing posterior device (PDDC) was recently approved for the treatment of moderate AIS. The purpose of this study was to determine if the PDDC can modulate growth in skeletally immature patients with AIS. Methods From a database of patients treated with the PDDC over 4 years, we identified those who had a minimum of 2 years follow-up. Pre-operative and post-operative Cobb angles and coronal plane wedging of the apical vertebra were evaluated on standing full length radiographs. Independent sample t test and one-way ANOVA with post-hoc Tukey HSD analysis was used to compare three groups in varying skeletal maturity: Risser 0–1, Risser 2–3, and Risser 4–5. Results 45 patients (14.2-years old, 11–17) were evaluated with a mean pre-op curve of 46° (35°-66°). The average preoperative major curve magnitude, of either Lenke 1 or 5 curve type, was similar among the three groups 47.6°, 46° and 41.5°. Deformity correction was similar in the three groups, with reduction to 26.4°, 20.4° and 26.2°, respectively, at final follow-up [p < 0.05]. Pre-op wedging 7.4° (3.8°–15°) was reduced after surgery to 5.7° (1°–15°) (p < 0.05). Of those patients, Risser 0–1 (n = 16) had preoperative wedging of 9.5° (6°–14.5°) that was reduced to 5.4° (1°–8°) postoperatively (p < 0.05); Risser 2–3 (n = 15) had pre-op 7.7° (4°–15°) vs. post-op 7.0° (3°–15°); Risser 4–5 (n = 14) had pre-op 4.8° (3.8°–6.5°) vs. post-op 4.7° (3.7°–6.5°). Delta Wedging in Risser 0–1 stage was significantly different than for Risser 2–3 and for Risser 4–5. Conclusion The posterior dynamic deformity correction device was able to modulate vertebral body wedging in skeletally immature patients with AIS. This was most evident in patients who were Risser 0–1. In contrast, curve correction was similar among the three groups. This finding lends support to the device’s ability to modulate growth.

scholarly journals Pathogenic role of Fgf23 in Dmp1-null mice

2008 ◽  
Vol 295 (2) ◽  
pp. E254-E261 ◽  
Author(s):  
Shiguang Liu ◽  
Jianping Zhou ◽  
Wen Tang ◽  
Rochelle Menard ◽  
Jian Q. Feng ◽  
...  
Keyword(s):  
Growth Retardation ◽  
Matrix Protein ◽  
Serum Levels ◽  
Serum Phosphate ◽  
Hypophosphatemic Rickets ◽  
Null Mouse ◽  
Matrix Mineralization ◽  
Severe Growth ◽  
Inactivating Mutations

Autosomal recessive hypophosphatemic rickets (ARHR), which is characterized by renal phosphate wasting, aberrant regulation of 1α-hydroxylase activity, and rickets/osteomalacia, is caused by inactivating mutations of dentin matrix protein 1 ( DMP1). ARHR resembles autosomal dominant hypophosphatemic rickets (ADHR) and X-linked hypophosphatemia (XLH), hereditary disorders respectively caused by cleavage-resistant mutations of the phosphaturic factor FGF23 and inactivating mutations of PHEX that lead to increased production of FGF23 by osteocytes in bone. Circulating levels of FGF23 are increased in ARHR and its Dmp1-null mouse homologue. To determine the causal role of FGF23 in ARHR, we transferred Fgf23 deficient/enhanced green fluorescent protein (eGFP) reporter mice onto Dmp1-null mice to create mice lacking both Fgf23 and Dmp1. Dmp1−/− mice displayed decreased serum phosphate concentrations, inappropriately normal 1,25(OH)2D levels, severe rickets, and a diffuse form of osteomalacia in association with elevated Fgf23 serum levels and expression in osteocytes. In contrast, Fgf23−/− mice had undetectable serum Fgf23 and elevated serum phosphate and 1,25(OH)2D levels along with severe growth retardation and focal form of osteomalacia. In combined Dmp1−/−/Fgf23−/−, circulating Fgf23 levels were also undetectable, and the serum levels of phosphate and 1,25(OH)2D levels were identical to Fgf23−/− mice. Rickets and diffuse osteomalacia in Dmp1-null mice were transformed to severe growth retardation and focal osteomalacia characteristic of Fgf23-null mice. These data suggest that the regulation of extracellular matrix mineralization by DMP1 is coupled to renal phosphate handling and vitamin D metabolism through a DMP1-dependent regulation of FGF23 production by osteocytes.

Deformity correction for vitamin D-resistant hypophosphatemic rickets of adults

2007 ◽  
Vol 128 (10) ◽  
pp. 1137-1143 ◽  
Author(s):  
Hidenori Matsubara ◽  
Hiroyuki Tsuchiya ◽  
Tamon Kabata ◽  
Keisuke Sakurakichi ◽  
Koji Watanabe ◽  
...  
Keyword(s):  
Vitamin D ◽  
Deformity Correction ◽  
Hypophosphatemic Rickets

Tissue level mechanical properties of cortical bone in skeletally immature and mature dogs

2009 ◽  
Vol 22 (03) ◽  
pp. 210-215 ◽  
Author(s):  
C.A. Phillips ◽  
S.A. Fernandez ◽  
Y. Li ◽  
S.S. Huja
Keyword(s):  
Mechanical Properties ◽  
Cortical Bone ◽  
Tissue Level ◽  
Mechanical Tests ◽  
Indentation Modulus ◽  
Skeletally Immature ◽  
Beagle Dogs ◽  
Cross Sectional ◽  
Specimen Holder ◽  
Immature Group

Summary Objectives: The purpose of this study was to quantify the tissue level mechanical properties of cortical bone of skeletally immature (~five-month-old) Beagle dogs and compare them to data from mature dogs measured in a previous study. Methods: Eight femoral cross sectional specimens (two bone sections / dog) were obtained from four skeletally immature dogs. A pair of calcein bone labels were administered intravenously to the dogs to mark sites of active mineralization prior to euthanasia. Prepared bone specimens were placed in a nanoindenter specimen holder and the previously identified calcein labelled osteons were located. Labelled (n = 128) and neighbouring unlabelled (n = 127) osteons in skeletally immature femurs were examined by instrumented indentation testing. Indents were made to a depth of 500 nm at a loading rate of 10 nm/s. Indentation modulus (IM) and hardness (H) were obtained. Results: The overall IM of the cortical bone in the skeletally mature groups was significantly greater than in the immature group (p = 0.0011), however overall H was not significantly different. The differences between the groups in IM were significant for the unlabelled osteons (p = 0.001), but not for the labelled osteons (p = 0.56). Conclusion: There are differences in the IM of unlabelled osteons in skeletally immature and mature groups of Beagle dogs. In contrast to whole bone mechanical tests, where there are obvious differences between growing and mature bones, there are only small differences in the micro-mechanical properties.

scholarly journals Bilateral Tibial Proximal Fractures Caused by Secondary Osteomalacia due to Autoimmune Polyendocrine Syndrome Type 2: A Case Report

2021 ◽  
Vol 11 (4) ◽  
Author(s):  
Daisuke Nakagawa ◽  
Keisuke Oe ◽  
Tomoaki Fukui ◽  
Ryosuke Kuroda ◽  
Takahiro Niikura
Keyword(s):  
Case Report ◽  
Pharmacological Treatment ◽  
Plate Osteosynthesis ◽  
Deformity Correction ◽  
Serum Phosphate ◽  
Tibial Osteotomy ◽  
Syndrome Type ◽  
Radiographic Findings ◽  
Autoimmune Polyendocrine Syndrome

Introduction: Hypophosphatemic osteomalacia can be overlooked or confused with other musculoskeletal disorders due to the variety of associated clinical, laboratory, and radiographic findings. If osteomalacia is diagnosed early and the fractures are not displaced, they often heal with nutritional supplements, but, if they progress to displaced fractures, they may require surgical intervention. Case Report: We present a case of secondary osteomalacia due to autoimmune polyendocrine syndrome Type 2 due to this condition, the patient developed bilateral tibial proximal fractures and her varus deformity had progressed. No clear indication of the timing for surgery for adults with osteomalacia has been reported. However, medical treatment improves the symptoms of osteomalacia and it is reported that in children, appropriate level of the serum phosphate (P) should be attained and maintained for the successful bone healing after osteotomy. Therefore, we prioritized pharmacological treatment and prescribed surgery after confirming that the value of serum phosphate P had been improved to recommended levels (2.5-3.5 mg/dl). We performed high tibial osteotomy for the right side and gradual correction by an external fixation for the left tibia, because of more severe deformation, and converted to an internal fixation to shorten the treatment period. During conversion, we performed the operation with a locking plate by the minimal invasive plate osteosynthesis method (MIPO). Conclusion: We conclude that the use of different deformity correction methods, depending on the degree of deformity, and the pharmacological treatment of osteomalacia may lead to favorable results. Keywords: Osteomalacia, autoimmune polyendocrine syndrome type 2, deformity correction method.

scholarly journals Risk Estimation of Aortic Stiffness in Patients with End Stage Renal Disease on Maintenance Haemodialysis

2019 ◽  
Vol 9 (1) ◽  
pp. 59-62
Author(s):  
Muhammad Abdur Razzak ◽  
Debasish Kumar Saha ◽  
Muhammad Ehsan Jalil ◽  
Mohammad Omar Faruque Miah ◽  
Abu Noim Md Abdul Hai ◽  
...  
Keyword(s):  
Renal Disease ◽  
Serum Calcium ◽  
End Stage Renal Disease ◽  
Aortic Stiffness ◽  
Serum Phosphate ◽  
Serum Phosphate Level ◽  
Maintenance Haemodialysis ◽  
Stage Renal Disease ◽  
End Stage ◽  
Esrd Patients

Background: The stiffness of the large elastic arteries increase the morbidity and mortality. The purpose of the present study was to estimate the risk of aortic stiffness among end stage renal disease patients on maintenance haemodialysis. Methods: This cross-sectional study was carried out in the Department of Nephrology at National Institute of Kidney Diseases and Urology (NIKDU), Dhaka and National Institute of Cardiovascular Disease and Hospital (NICVD), Dhaka, Bangladesh from January 2013 to December 2014 for a period of two years. Chronic kidney disease in stage 5 [CKD-5(D)] patients older than 18 years on maintenance haemodialysis (MHD) for more than 3 months were designated as case group and age and sex matched non CKD patients were considered as control group. Serum calcium, serum albumin, serum phosphate and iPTH were estimated by semi-automated biochemistry analyzer from the Department of Biochemistry of NIKDU, Dhaka and NICVD, Dhaka. Plain Xray abdomen in lateral view was performed for all patients. Result: A total number of 100 patients were enrolled for this study of which 50 patients were in end stage renal disease (ESRD) group and the rest 50 patients were in non-CKD group. Mean (±SD) aortic stiffness index was significantly higher (P<0.001) among ESRD population (3.27±1.70) compared to non CKD group of population (2.00±0.73). Mean (±SD) serum calcium (corrected) level was significantly high (P<0.001) in ESRD patients (9.79±0.87) compared to non CKD group of population (9.13±0.70). Mean (±SD) serum phosphate level was significantly higher (P<0.001) in ESRD patients (5.71±0.96) compared to non CKD group of population (4.20±0.59). However, mean (±SD) iPTH level showed no significant difference between ESRD (25.33±51.98) and non CKD group of population (38.53±19.52). Conclusion: In conclusion, aortic stiffness is significantly higher among ESRD subjects. Birdem Med J 2019; 9(1): 59-62

scholarly journals Serum Phosphate Predicts Early Mortality among Underweight Adults Starting ART in Zambia: A Novel Context for Refeeding Syndrome?

2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
John R. Koethe ◽  
Meridith Blevins ◽  
Christopher K. Nyirenda ◽  
Edmond K. Kabagambe ◽  
Janelle M. Chiasera ◽  
...  
Keyword(s):  
Odds Ratio ◽  
Cellular Metabolism ◽  
Serum Phosphate ◽  
Early Mortality ◽  
Serum Phosphate Level ◽  
Similar Relationship ◽  
Refeeding Syndrome ◽  
Art Initiation ◽  
Low Body Mass Index ◽  
Pretreatment Serum

Background. Low body mass index (BMI) at antiretroviral therapy (ART) initiation is associated with early mortality, but the etiology is not well understood. We hypothesized that low pretreatment serum phosphate, a critical cellular metabolism intermediate primarily stored in skeletal muscle, may predict mortality within the first 12 weeks of ART.Methods. We prospectively studied 352 HIV-infected adults initiating ART in Lusaka, Zambia to estimate the odds of death for each 0.1 mmol/L decrease in baseline phosphate after adjusting for established predictors of mortality.Results. The distribution of phosphate values was similar across BMI categories (median value 1.2 mmol/L). Among the 145 participants with BMI<18.5 kg/m2, 28 (19%) died within 12 weeks. Lower pretreatment serum phosphate was associated with increased mortality (odds ratio (OR) 1.24 per 0.1 mmol/L decrement, 95% CI: 1.05 to 1.47;P=0.01) after adjusting for sex, age, and CD4+lymphocyte count. A similar relationship was not observed among participants with BMI ≥18.5 kg/m2(OR 0.96, 95% CI: 0.76 to 1.21;P=0.74).Conclusions. The association of low pretreatment serum phosphate level and early ART mortality among undernourished individuals may represent a variant of the refeeding syndrome. Further studies of cellular metabolism in this population are needed.

scholarly journals An Alternative Approach to Treatment of Hypophosphatemia in Nonsurgical Critically Ill Patients in Countries With Limited Resources

Dose-Response ◽  
2019 ◽  
Vol 17 (2) ◽  
pp. 155932581985042
Author(s):  
Tijana Kovačević ◽  
Peđja Kovačević ◽  
Boris Tomić ◽  
Saša Dragić ◽  
Danica Momčičević
Keyword(s):  
Critically Ill ◽  
Critically Ill Patients ◽  
Phosphate Buffer ◽  
Dose Range ◽  
Clinical Status ◽  
Serum Phosphate ◽  
Serum Phosphate Level ◽  
Alternative Approach ◽  
Medical Intensive Care ◽  
A Prospective Study

Background: Hypophosphatemia can complicate and prolong the treatment of critically ill patients, and it is even thought to be related to mortality rate. Objectives: The aim of this study is to determine whether using extemporary prepared phosphate buffer in pharmacy would help correct serum phosphate in critically ill patients. Methods: A prospective study was conducted at the medical intensive care unit over a period of 1 year and included 50 patients who were diagnosed with hypophosphatemia. Phosphate buffer was prepared at the pharmacy, and the dose range was recommended by a clinical pharmacist. Results: Patients were administered phosphate buffer via the nasogastric tube, and the doses chosen by the physicians depended on serum phosphate level and the severity of the patients’ clinical status. Serum phosphate levels were successfully corrected in all treated patients. The most frequently used dose was 60 mmoL/d, and in most patients 1-day therapy was sufficient. No adverse effects were observed. Conclusion: The phosphate buffer is an adequate alternative for the treatment of hypophosphatemia of nonsurgically critically ill patients. One-day therapy with the 60 mmoL phosphate dose divided into 3 single doses resulted in normalization of serum phosphate values in most patients.

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