Primary biliary cholangitis(PBC) is an autoimmune cholestatic liver disease with multiple debilitating complications. Osteoporosis is a common complication of PBC resulting in frequent fractures and leading to signifi...Primary biliary cholangitis(PBC) is an autoimmune cholestatic liver disease with multiple debilitating complications. Osteoporosis is a common complication of PBC resulting in frequent fractures and leading to significant morbidity in this population, yet evidence for effective therapy is lacking. We sought to summarize our current understanding of the pathophysiology of osteoporosis in PBC, as well as current and emerging therapies in order to guide future research directions. A complete search with a comprehensive literature review was performed with studies from Pub Med, EMBASE, Web of Science, Cochrane database, and the Countway Library. Osteoporosis in PBC is driven primarily by decreased bone formation, which differs from the increased bone resorption seen in postmenopausal osteoporosis. Despite this fundamental difference, current treatment recommendations are based primarily on experience with postmenopausal osteoporosis. Trials specific to PBC-related osteoporosis are small and have not consistently demonstrated a benefit in this population. As it stands, prevention of osteoporosis in PBC relies on the mitigation of risk factors such as smoking and alcohol use, as well as encouraging a healthy diet and weight-bearing exercise. The primary medical intervention for the treatment of osteoporosis in PBC remains bisphosphonates though a benefit in terms of fracture reduction has never been shown. This review outlines what is known regarding the pathogenesis of bone disease in PBC and summarizes current and emerging therapies.展开更多
AIM： To estimate the prevalence and identify the risk factors for metabolic bone disease in patients with cirrhosis. METHODS： The study was performed on 72 Indian patients with cirrhosis （63 male, 9 female; aged 〈...AIM： To estimate the prevalence and identify the risk factors for metabolic bone disease in patients with cirrhosis. METHODS： The study was performed on 72 Indian patients with cirrhosis （63 male, 9 female; aged 〈 50 years）. Etiology of cirrhosis was alcoholism （n = 37）, hepatitis B （n = 25） and hepatitis C （n = 10）. Twenty-three patients belonged to Child class A, while 39 were in class B and 10 in class C. Secondary causes for metabolic bone disease and osteoporosis were ruled out. Sunlight exposure, physical activity and dietary constituents were calculated. Complete metabolic profiles were derived, and bone mineral density （BMD） was measured using dual energy X ray absorptiometry. Low BMD was defined as a Z score below -2. RESULTS： Low BMD was found in 68% of patients. Lumbar spine was the most frequently and severely affected site. Risk factors for low BMD included low physical activity, decreased sunlight exposure, and low lean body mass. Calcium intake was adequate, with unfavorable calcium： protein ratio and calcium： phosphorus ratio. Vitamin D deficiency was highly prevalent （92%）. There was a high incidence of hypogonadism （41%）. Serum estradiol level was elevated significantly in patients with normal BMD. Insulin-like growth factor （IGF） 1 and IGF binding protein 3 levels were below the age-related normal range in both groups. IGF-1 was significantly lower in patients with low BMD. Serum osteocalcin level was low （68%） and urinary deoxypyridinoline to creatinine ratio was high （79%）, which demonstrated low bone formation with high resorption. CONCLUSION： Patients with cirrhosis have low BMD. Contributory factors are reduced physical activity, low lean body mass, vitamin D deficiency and hypogonadism and low IGF-1 level.展开更多
AIM: To investigate the correlation between hepatic osteodystrophy and osteoporosis in patients with liver cirrhosis. METHODS: Bone mineral density of the patients (n = 55) and that of the control group (n = 30) were ...AIM: To investigate the correlation between hepatic osteodystrophy and osteoporosis in patients with liver cirrhosis. METHODS: Bone mineral density of the patients (n = 55) and that of the control group (n = 30) were measured by dual-energy X-ray absorptiometry. All the women in the study were premenopausal. Deoxypyridinoline, pyridinoline and urinary Ca 2+ were measured as bone destruction markers, while alkaline phosphatase (ALP), osteocalcin and insulin-like growth factor-1 (IGF-1) were measured as bone formation markers. Furthermore, interleukin-1 (IL-1), IL-6, tumor necrosis factor α (TNF-α), vitamin D3, direct bilirubin, albumin, cortisol and parathyroid hormone (PTH) levels were measured. The independent Student t test and χ 2 test were employed in comparing both groups, and the Pearson correlation test was used to determine associations. RESULTS: Comparing cirrhosis and control groups, lumbar total T-score (-1.6 ± 1.2 g/cm 2 vs -0.25 ± 1.3 g/cm 2 , P < 0.001), lumbar total Z-score (-1.2 ± 1.23 g/cm 2 vs -0.6 ± 1.3 g/cm 2 , P < 0.001), total femur T-score (-0.05 ± 1 g/cm 2 vs -0.6 ± 0.9 g/cm 2 , P = 0.003) and total femur Z-score (-0.08 ± 1.5 g/cm 2 vs 0.7 ± 0.9 g/cm 2 , P =0.003) showed significantly lower values in the cirrhosis group. Blood ALP level (109.2 ± 57 U/Lvs 62.6 ± 32.5 U/L, P < 0.001), IL-6 level (27.9 ± 51.6 pg/mL vs 3.3 ± 3.1 pg/mL, P = 0.01), TNF-α level (42.6 ± 33.2 pg/mL vs 25.3 ± 12.3 pg/mL, P = 0.007) and direct bilirubin level (0.9 ± 0.7 mg/dL vs 0.3 ± 0.2 mg/dL, P < 0.001) were significantly higher in the cirrhosis group. IGF-1 level (47.7 ± 26.2 ng/mL vs 143.4 ± 53.2 ng/mL, P < 0.001), osteocalcin level (1.05 ± 2.5 ng/mL vs 7.0 ± 13 ng/mL, P = 0.002) and 24 h urinary Ca 2+ (169.6 ± 227.2 mg/dL vs 287 ± 168.6 mg/dL, P = 0.003) were significantly lower in the cirrhosis group. Urinary deoxypyridinoline/creatinine (9.4 ± 9.9 pmol/μmol vs 8.1 ± 5.3 pmol/μmol, P = 0.51), urinary pyridinoline/creatinine (51.3 ± 66.6 pmol/μmol vs 29 ± 25.8 pmol/μmol, P = 0.展开更多
Hepatic osteodystrophy (HO) is the generic term defining the group of alterations in bone mineral metabolism found in patients with chronic liver disease. This paper is a global review of HO and its main pathophysiol... Hepatic osteodystrophy (HO) is the generic term defining the group of alterations in bone mineral metabolism found in patients with chronic liver disease. This paper is a global review of HO and its main pathophysiological, epidemiological and therapeutic aspects. Studies examining the most relevant information concerning the prevalence, etiological factors, diagnostic and therapeutic aspects involved in HO were identified by a systematic literature search of the PubMed database. HO generically defines overall alterations in bone mineral density (BMD) (osteoporosis or osteopenia) which appear as a possible complication of chronic liver disease. The origin of HO is multifactorial and its etiology and severity vary in accordance with the underlying liver disease. Its exact prevalence is unknown, but different studies estimate that it could affect from 20% to 50% of patients. The reported mean prevalence of osteoporosis ranges from 13%-60% in chronic cholestasis to 20% in chronic viral hepatitis and 55% in viral cirrhosis. Alcoholic liver disease is not always related to osteo-penia. HO has been commonly studied in chronic cholestatic disease (primary biliary cirrhosis and primary sclerosing cholangitis). Several risk factors and pathogenic mechanisms have been associated with the loss of BMD in patients with chronic liver disease. However, little information has been discovered in relationship to most of these mechanisms. Screening for osteopenia and osteoporosis is recommended in advanced chronic liver disease. There is a lack of randomized studies assessing specific management for HO.展开更多
AIM:To determine the contributions of insulin-like growth factor 1 (IGF-1),cytokines and liver disease severity to bone mineral density in patients pre-transplantation.METHODS:Serum IGF-1,tumor necrosis factor-α (TNF...AIM:To determine the contributions of insulin-like growth factor 1 (IGF-1),cytokines and liver disease severity to bone mineral density in patients pre-transplantation.METHODS:Serum IGF-1,tumor necrosis factor-α (TNFα) and interleukin 6 (IL-6) were measured and the Model for End-Stage Liver Disease (MELD) score calculated in 121 adult patients referred to a single centre for liver transplantation.Bone mineral density (BMD) of the lumbar spine and femoral neck were assessed via dual energy X-ray absorptiometry.Demographics,liver disease etiology,medication use and relevant biochemistry were recorded.RESULTS:A total of 117 subjects were included,with low BMD seen in 68.6%,irrespective of disease etiol-ogy.In multivariable analysis,low body mass index (BMI),increased bone turnover and low IGF-1 were independent predictors of low spinal bone density.At the hip,BMI,IGF-1 and vitamin D status were predictive.Despite prevalent elevations of TNFα and IL-6,levels did not correlate with degree of bone loss.The MELD score failed to predict low BMD in this pre-transplant population.CONCLUSION:Osteopenia/osteoporosis is common in advanced liver disease.Low serum IGF-1 is weakly predictive but serum cytokine and MELD score fail to predict the severity of bone disease.展开更多
Background and Aims:Skeletal manifestation in liver dis-eases represents the minimally scrutinized part of the disease spectrum.Vitamin D deficiency has a central role in developing hepatic osteodystrophy in patients ...Background and Aims:Skeletal manifestation in liver dis-eases represents the minimally scrutinized part of the disease spectrum.Vitamin D deficiency has a central role in developing hepatic osteodystrophy in patients with chronic liver disease.This study aimed to investigate vitamin D levels and their relationship with disease advancement in these patients.Methods:Vitamin D levels were checked in 125 chronic liver disease patients.The patients were classified in three stages according to Child-Pugh score:A,B and C.The relationship of vitamin D levels with Child-Pugh score and other variables in the study was assessed by the contingency coefficient.Correlation and logistic regression analyses were also carried out to find additional predictors of low vitamin D levels.Results:Among the patients,88%had either insufficient or deficient stores of vitamin D,while only 12%had sufficient vitamin D levels(p>0.05).Vitamin D levels were notably related to Child-Pugh class(contingency coefficient=0.5,p<0.05).On univariate and multinomial regression analyses,age,female sex,MELD and Child-Pugh class were predictors of low vitamin D levels.Age,model of end-stage liver disease score and Child-Pugh score were negatively correlated to vitamin D levels(p<0.05).Conclusions:Vitamin D deficiency is notably re-lated to age,female sex and model of end-stage liver disease score,in addition to Child-Pugh class of liver cirrhosis.Vita-min D levels should be routinely checked in patients with ad-vanced liver cirrhosis(Child-Pugh class B and C)and this deficiency must be addressed in a timely manner to improve general well-being of cirrhotic patients.展开更多
Primary hyperoxaluria （PH） is a rare autosoma recessive disorder of glyoxylate metabolism in which specific hepatic enzyme deficiencies result in overproduction of oxalate. Because oxalate is excreted exclusively by...Primary hyperoxaluria （PH） is a rare autosoma recessive disorder of glyoxylate metabolism in which specific hepatic enzyme deficiencies result in overproduction of oxalate. Because oxalate is excreted exclusively by the kidney, hyperoxaluria leads to calcium oxalate nephrolithiasis, nephrocalcinosis, and renal failure. PH are considered rare with a prevalence of 0.1-0.2 per 106 population.2 PH was misdiagnosed in some cases initially and unfortunately a case may not be detected until the post-transplant period by allograft biopsy.展开更多
AIM:To determine the relationship between non-alcoholic steatohepatitis(NASH)and bone mineral density(BMD).METHODS:A total of 38 patients(25 males)with a diagnosis of histologically proven NASH and 42 healthy control... AIM:To determine the relationship between non-alcoholic steatohepatitis(NASH)and bone mineral density(BMD).METHODS:A total of 38 patients(25 males)with a diagnosis of histologically proven NASH and 42 healthy controls(24 males)were enrolled in the study.Demographic features,clinical findings,complete blood count and routine biochemical analysis,as well as adrenal,thyroid and gonadal functions,were recorded.Additionally,intact parathormone,25-OH-vitamin-D3,tumor necrosis factor-α,interleukin-6,interleukin-1,in-sulin-like growth factor-1 and insulin-like growth factor binding protein-3 levels were measured in both groups.Furthermore,lumbar spine and femoral neck BMD of both groups were measured by the dual-energy X-ray absorptiometry(DXA)method.RESULTS:The mean age was 41±12 years in the NASH group and 43±11 years in the control group.Among demographic features,waist circumference was significantly larger in the NASH group compared to the control group(P【0.019).Among laboratory parameters,serum triglyceride(P【0.008),alanine transaminase(P【0.0001),aspartate transaminase(P【0.001),alkaline phosphatase(P【0.016),gamma glutamyl transferase(P【0.0001),ferritin(P【0.001)and 25-OH-vitamin-D3levels(P【0.0001)were significantly higher in the NASH group compared to the control group.Lumbar BMD was significantly higher in the NASH group compared to the control group(1.057±0.119 g/cm2vs 0.941±0.133 g/cm2;P【0.001,respectively).In the NASH group,there was no significant relationship between BMD and fibrosis stage in liver biopsy.CONCLUSION:NASH increases BMD and may be related to an elevated serum 25-OH-vitamin D3 level.展开更多
Biliary atresia(BA) is one of the major hepatobiliary abnormalities in infants and one of the causes of hepatic osteodystrophy.Bone disease may be caused by the malabsorption of calcium and magnesium by vitamin D in h...Biliary atresia(BA) is one of the major hepatobiliary abnormalities in infants and one of the causes of hepatic osteodystrophy.Bone disease may be caused by the malabsorption of calcium and magnesium by vitamin D in hepatobiliary diseases in which bile flow into the intestines is deficient or absent.Bone fracture before Kasai hepatic portoenterostomy or within one month after the procedure in an infant with BA is very rare.We herein report two infants:one infant with BA who initially presented with a bone fracture before Kasai hepatic portoenterostomy,and the other at 4 wk after Kasai hepatic portoenterostomy,and also provide a review of the literature.Moreover,we conclude that clinicians should consider BA in infants with bone fracture during early infancy.展开更多
Given that the liver is involved in many metabolic mechanisms,it is not surprising that chronic liver disease(CLD)could have numerous complications.Secondary osteoporosis and increased bone fragility are frequently ov...Given that the liver is involved in many metabolic mechanisms,it is not surprising that chronic liver disease(CLD)could have numerous complications.Secondary osteoporosis and increased bone fragility are frequently overlooked complications in CLD patients.Previous studies implied that up to one-third of these individuals meet diagnostic criteria for osteopenia or osteoporosis.Recent publications indicated that CLD-induced bone fragility depends on the etiology,duration,and stage of liver disease.Therefore,the increased fracture risk in CLD patients puts a severe socioeconomic burden on the health system and urgently requires more effective prevention,diagnosis,and treatment measures.The pathogenesis of CLD-induced bone loss is multifactorial and still insufficiently understood,especially considering the relative impact of increased bone resorption and reduced bone formation in these individuals.It is essential to note that inconsistent findings regarding bone mineral density measurement were previously reported in these individuals.Bone mineral density is widely used as the“golden standard”in the clinical assessment of bone fragility although it is not adequate to predict individual fracture risk.Therefore,microscale bone alterations(bone microstructure,mechanical properties,and cellular indices)were analyzed in CLD individuals.These studies further support the thesis that bone strength could be compromised in CLD individuals,implying that an individualized approach to fracture risk assessment and subsequent therapy is necessary for CLD patients.However,more well-designed studies are required to solve the bone fragility puzzle in CLD patients.展开更多
BACKGROUND This case report contributes to the medical literature by highlighting the successful management of a neglected femoral neck fracture in a patient with renal osteodystrophy and secondary hyperparathyroidism...BACKGROUND This case report contributes to the medical literature by highlighting the successful management of a neglected femoral neck fracture in a patient with renal osteodystrophy and secondary hyperparathyroidism(SHPTH)who was on dialysis due to end-stage renal disease(ESRD).It underscores the efficacy of parathyroidectomy(PTX)in restoring bone mineral density(BMD)and promoting fracture healing,addressing a significant complication in ESRD patients.CASE SUMMARY A 36-year-old female with renal osteodystrophy and on dialysis due to ESRD presented with a history of left patellar tendon rupture and later,a right achilles tendon avulsion fracture.Persistent right hip pain led to the discovery of a neglected right femoral neck fracture,which was initially overlooked due to the patient’s complex medical history.Two months post-achilles tendon repair,the patient underwent PTX to manage the refractory SHPTH.The postoperative course included rehabilitation and weight-bearing exercises.Remarkably,2 years after osteosynthesis,radiographic assessments indicated a solid union of the periprothesis fracture and significant improvement in BMD,showcasing the efficacy of the treatment approach.CONCLUSION PTX,combined with appropriate rehabilitation,is crucial for improving BMD and fracture healing in ESRD patients with SHPTH.展开更多
The persistence and severity of hyperparathyroidism(HPT) post-renal transplantation is relatively frequent and primarily associated with the timing and its magnitude in the pre-transplant period and with the presence ...The persistence and severity of hyperparathyroidism(HPT) post-renal transplantation is relatively frequent and primarily associated with the timing and its magnitude in the pre-transplant period and with the presence of parathyroid adenomas. HPT after renal transplantation is clinically manifested with hypercalcemia, hypophosphatemia, bone pain, fractures, and in more serious cases with cardiovascular calcifications that affect the survival. The primary clinical objective for patients with secondary HPT after renal transplantation is to obtain a level of parathyroid hormone(PTH) adequate to the renal transplanted function and to normalize levels of calcium, phosphorus and vitamin D. In many cases during this period, the development of hypercalcemiaand/or hypophosphatemia makes it necessary to take different therapeutic measures. The use of vitamin D or its analogues has been extrapolated from the management of pre-transplant HPT obtaining variable outcomes, although its use is limited by its capacity to produce hypercalcemia. Calcimimetics are drugs that have proven be effective in reducing PTH levels in patients with HPT on dialysis and has been effective in reducing up to 50% PTH levels in moderate to severe HPT in post-renal transplantation.When HPT persists after renal transplantation and does not respond to medical treatment, invasive management by percutaneous ethanol injection therapy of parathyroid glands or parathyroidectomy should be considered. The emergence of new methods for the management of HPT expands the availability of therapeutic tools for transplant patients.展开更多
Bilateral, coincident, atraumatic hip fracture is extremely uncommon and usually occurs secondary to seizures, trauma and metabolic disease including renal osteodystrophy. One of the major types<span>,</span&...Bilateral, coincident, atraumatic hip fracture is extremely uncommon and usually occurs secondary to seizures, trauma and metabolic disease including renal osteodystrophy. One of the major types<span>,</span><span> secondary hyperparathyroidism </span><span>is </span><span>associated with high bone turnover due to excess parathyroid hormone and usually seen in a chronic dialysis patient. We reported a 20-year-old woman with end stage renal failure and renal osteodystrophy. She sustained atraumatic right subtrochanteric fracture and left neck of femur fracture (Garden 1), then underwent bilateral long proximal femoral nail. Renal osteodystrophy causes pathological fracture by affecting calcium metabolism that stimulates bone resorptions and lead</span><span>s</span><span> to osteoporotic bone. The aim of this case report is to discuss the approach and management done to the patient presented to our center. Bilateral long proximal femoral nail (PFN) was chosen, taking into account the patient’s premorbid, age, fracture pattern and potential complications. Careful multidisciplinary team approach led by the orthopaedic surgeon, nephrologist and physical therapist </span><span>is</span><span> vital for </span><span>the </span><span>patient to achieve good outcome postoperatively, thus reducing morbidity and mortality.</span>展开更多
基金Supported by NIH T32 training grant,No.4T32GM103702-04 to Trivedi HD
文摘Primary biliary cholangitis(PBC) is an autoimmune cholestatic liver disease with multiple debilitating complications. Osteoporosis is a common complication of PBC resulting in frequent fractures and leading to significant morbidity in this population, yet evidence for effective therapy is lacking. We sought to summarize our current understanding of the pathophysiology of osteoporosis in PBC, as well as current and emerging therapies in order to guide future research directions. A complete search with a comprehensive literature review was performed with studies from Pub Med, EMBASE, Web of Science, Cochrane database, and the Countway Library. Osteoporosis in PBC is driven primarily by decreased bone formation, which differs from the increased bone resorption seen in postmenopausal osteoporosis. Despite this fundamental difference, current treatment recommendations are based primarily on experience with postmenopausal osteoporosis. Trials specific to PBC-related osteoporosis are small and have not consistently demonstrated a benefit in this population. As it stands, prevention of osteoporosis in PBC relies on the mitigation of risk factors such as smoking and alcohol use, as well as encouraging a healthy diet and weight-bearing exercise. The primary medical intervention for the treatment of osteoporosis in PBC remains bisphosphonates though a benefit in terms of fracture reduction has never been shown. This review outlines what is known regarding the pathogenesis of bone disease in PBC and summarizes current and emerging therapies.
基金Supported by Corpus generated by Department of Endocrinology, KEM Hospital, Mumbai, India
文摘AIM： To estimate the prevalence and identify the risk factors for metabolic bone disease in patients with cirrhosis. METHODS： The study was performed on 72 Indian patients with cirrhosis （63 male, 9 female; aged 〈 50 years）. Etiology of cirrhosis was alcoholism （n = 37）, hepatitis B （n = 25） and hepatitis C （n = 10）. Twenty-three patients belonged to Child class A, while 39 were in class B and 10 in class C. Secondary causes for metabolic bone disease and osteoporosis were ruled out. Sunlight exposure, physical activity and dietary constituents were calculated. Complete metabolic profiles were derived, and bone mineral density （BMD） was measured using dual energy X ray absorptiometry. Low BMD was defined as a Z score below -2. RESULTS： Low BMD was found in 68% of patients. Lumbar spine was the most frequently and severely affected site. Risk factors for low BMD included low physical activity, decreased sunlight exposure, and low lean body mass. Calcium intake was adequate, with unfavorable calcium： protein ratio and calcium： phosphorus ratio. Vitamin D deficiency was highly prevalent （92%）. There was a high incidence of hypogonadism （41%）. Serum estradiol level was elevated significantly in patients with normal BMD. Insulin-like growth factor （IGF） 1 and IGF binding protein 3 levels were below the age-related normal range in both groups. IGF-1 was significantly lower in patients with low BMD. Serum osteocalcin level was low （68%） and urinary deoxypyridinoline to creatinine ratio was high （79%）, which demonstrated low bone formation with high resorption. CONCLUSION： Patients with cirrhosis have low BMD. Contributory factors are reduced physical activity, low lean body mass, vitamin D deficiency and hypogonadism and low IGF-1 level.
文摘AIM: To investigate the correlation between hepatic osteodystrophy and osteoporosis in patients with liver cirrhosis. METHODS: Bone mineral density of the patients (n = 55) and that of the control group (n = 30) were measured by dual-energy X-ray absorptiometry. All the women in the study were premenopausal. Deoxypyridinoline, pyridinoline and urinary Ca 2+ were measured as bone destruction markers, while alkaline phosphatase (ALP), osteocalcin and insulin-like growth factor-1 (IGF-1) were measured as bone formation markers. Furthermore, interleukin-1 (IL-1), IL-6, tumor necrosis factor α (TNF-α), vitamin D3, direct bilirubin, albumin, cortisol and parathyroid hormone (PTH) levels were measured. The independent Student t test and χ 2 test were employed in comparing both groups, and the Pearson correlation test was used to determine associations. RESULTS: Comparing cirrhosis and control groups, lumbar total T-score (-1.6 ± 1.2 g/cm 2 vs -0.25 ± 1.3 g/cm 2 , P < 0.001), lumbar total Z-score (-1.2 ± 1.23 g/cm 2 vs -0.6 ± 1.3 g/cm 2 , P < 0.001), total femur T-score (-0.05 ± 1 g/cm 2 vs -0.6 ± 0.9 g/cm 2 , P = 0.003) and total femur Z-score (-0.08 ± 1.5 g/cm 2 vs 0.7 ± 0.9 g/cm 2 , P =0.003) showed significantly lower values in the cirrhosis group. Blood ALP level (109.2 ± 57 U/Lvs 62.6 ± 32.5 U/L, P < 0.001), IL-6 level (27.9 ± 51.6 pg/mL vs 3.3 ± 3.1 pg/mL, P = 0.01), TNF-α level (42.6 ± 33.2 pg/mL vs 25.3 ± 12.3 pg/mL, P = 0.007) and direct bilirubin level (0.9 ± 0.7 mg/dL vs 0.3 ± 0.2 mg/dL, P < 0.001) were significantly higher in the cirrhosis group. IGF-1 level (47.7 ± 26.2 ng/mL vs 143.4 ± 53.2 ng/mL, P < 0.001), osteocalcin level (1.05 ± 2.5 ng/mL vs 7.0 ± 13 ng/mL, P = 0.002) and 24 h urinary Ca 2+ (169.6 ± 227.2 mg/dL vs 287 ± 168.6 mg/dL, P = 0.003) were significantly lower in the cirrhosis group. Urinary deoxypyridinoline/creatinine (9.4 ± 9.9 pmol/μmol vs 8.1 ± 5.3 pmol/μmol, P = 0.51), urinary pyridinoline/creatinine (51.3 ± 66.6 pmol/μmol vs 29 ± 25.8 pmol/μmol, P = 0.
文摘 Hepatic osteodystrophy (HO) is the generic term defining the group of alterations in bone mineral metabolism found in patients with chronic liver disease. This paper is a global review of HO and its main pathophysiological, epidemiological and therapeutic aspects. Studies examining the most relevant information concerning the prevalence, etiological factors, diagnostic and therapeutic aspects involved in HO were identified by a systematic literature search of the PubMed database. HO generically defines overall alterations in bone mineral density (BMD) (osteoporosis or osteopenia) which appear as a possible complication of chronic liver disease. The origin of HO is multifactorial and its etiology and severity vary in accordance with the underlying liver disease. Its exact prevalence is unknown, but different studies estimate that it could affect from 20% to 50% of patients. The reported mean prevalence of osteoporosis ranges from 13%-60% in chronic cholestasis to 20% in chronic viral hepatitis and 55% in viral cirrhosis. Alcoholic liver disease is not always related to osteo-penia. HO has been commonly studied in chronic cholestatic disease (primary biliary cirrhosis and primary sclerosing cholangitis). Several risk factors and pathogenic mechanisms have been associated with the loss of BMD in patients with chronic liver disease. However, little information has been discovered in relationship to most of these mechanisms. Screening for osteopenia and osteoporosis is recommended in advanced chronic liver disease. There is a lack of randomized studies assessing specific management for HO.
基金Supported by the Transplant Program of the University of Alberta Hospital
文摘AIM:To determine the contributions of insulin-like growth factor 1 (IGF-1),cytokines and liver disease severity to bone mineral density in patients pre-transplantation.METHODS:Serum IGF-1,tumor necrosis factor-α (TNFα) and interleukin 6 (IL-6) were measured and the Model for End-Stage Liver Disease (MELD) score calculated in 121 adult patients referred to a single centre for liver transplantation.Bone mineral density (BMD) of the lumbar spine and femoral neck were assessed via dual energy X-ray absorptiometry.Demographics,liver disease etiology,medication use and relevant biochemistry were recorded.RESULTS:A total of 117 subjects were included,with low BMD seen in 68.6%,irrespective of disease etiol-ogy.In multivariable analysis,low body mass index (BMI),increased bone turnover and low IGF-1 were independent predictors of low spinal bone density.At the hip,BMI,IGF-1 and vitamin D status were predictive.Despite prevalent elevations of TNFα and IL-6,levels did not correlate with degree of bone loss.The MELD score failed to predict low BMD in this pre-transplant population.CONCLUSION:Osteopenia/osteoporosis is common in advanced liver disease.Low serum IGF-1 is weakly predictive but serum cytokine and MELD score fail to predict the severity of bone disease.
文摘Background and Aims:Skeletal manifestation in liver dis-eases represents the minimally scrutinized part of the disease spectrum.Vitamin D deficiency has a central role in developing hepatic osteodystrophy in patients with chronic liver disease.This study aimed to investigate vitamin D levels and their relationship with disease advancement in these patients.Methods:Vitamin D levels were checked in 125 chronic liver disease patients.The patients were classified in three stages according to Child-Pugh score:A,B and C.The relationship of vitamin D levels with Child-Pugh score and other variables in the study was assessed by the contingency coefficient.Correlation and logistic regression analyses were also carried out to find additional predictors of low vitamin D levels.Results:Among the patients,88%had either insufficient or deficient stores of vitamin D,while only 12%had sufficient vitamin D levels(p>0.05).Vitamin D levels were notably related to Child-Pugh class(contingency coefficient=0.5,p<0.05).On univariate and multinomial regression analyses,age,female sex,MELD and Child-Pugh class were predictors of low vitamin D levels.Age,model of end-stage liver disease score and Child-Pugh score were negatively correlated to vitamin D levels(p<0.05).Conclusions:Vitamin D deficiency is notably re-lated to age,female sex and model of end-stage liver disease score,in addition to Child-Pugh class of liver cirrhosis.Vita-min D levels should be routinely checked in patients with ad-vanced liver cirrhosis(Child-Pugh class B and C)and this deficiency must be addressed in a timely manner to improve general well-being of cirrhotic patients.
文摘Primary hyperoxaluria （PH） is a rare autosoma recessive disorder of glyoxylate metabolism in which specific hepatic enzyme deficiencies result in overproduction of oxalate. Because oxalate is excreted exclusively by the kidney, hyperoxaluria leads to calcium oxalate nephrolithiasis, nephrocalcinosis, and renal failure. PH are considered rare with a prevalence of 0.1-0.2 per 106 population.2 PH was misdiagnosed in some cases initially and unfortunately a case may not be detected until the post-transplant period by allograft biopsy.
文摘 AIM:To determine the relationship between non-alcoholic steatohepatitis(NASH)and bone mineral density(BMD).METHODS:A total of 38 patients(25 males)with a diagnosis of histologically proven NASH and 42 healthy controls(24 males)were enrolled in the study.Demographic features,clinical findings,complete blood count and routine biochemical analysis,as well as adrenal,thyroid and gonadal functions,were recorded.Additionally,intact parathormone,25-OH-vitamin-D3,tumor necrosis factor-α,interleukin-6,interleukin-1,in-sulin-like growth factor-1 and insulin-like growth factor binding protein-3 levels were measured in both groups.Furthermore,lumbar spine and femoral neck BMD of both groups were measured by the dual-energy X-ray absorptiometry(DXA)method.RESULTS:The mean age was 41±12 years in the NASH group and 43±11 years in the control group.Among demographic features,waist circumference was significantly larger in the NASH group compared to the control group(P【0.019).Among laboratory parameters,serum triglyceride(P【0.008),alanine transaminase(P【0.0001),aspartate transaminase(P【0.001),alkaline phosphatase(P【0.016),gamma glutamyl transferase(P【0.0001),ferritin(P【0.001)and 25-OH-vitamin-D3levels(P【0.0001)were significantly higher in the NASH group compared to the control group.Lumbar BMD was significantly higher in the NASH group compared to the control group(1.057±0.119 g/cm2vs 0.941±0.133 g/cm2;P【0.001,respectively).In the NASH group,there was no significant relationship between BMD and fibrosis stage in liver biopsy.CONCLUSION:NASH increases BMD and may be related to an elevated serum 25-OH-vitamin D3 level.
文摘Biliary atresia(BA) is one of the major hepatobiliary abnormalities in infants and one of the causes of hepatic osteodystrophy.Bone disease may be caused by the malabsorption of calcium and magnesium by vitamin D in hepatobiliary diseases in which bile flow into the intestines is deficient or absent.Bone fracture before Kasai hepatic portoenterostomy or within one month after the procedure in an infant with BA is very rare.We herein report two infants:one infant with BA who initially presented with a bone fracture before Kasai hepatic portoenterostomy,and the other at 4 wk after Kasai hepatic portoenterostomy,and also provide a review of the literature.Moreover,we conclude that clinicians should consider BA in infants with bone fracture during early infancy.
文摘Given that the liver is involved in many metabolic mechanisms,it is not surprising that chronic liver disease(CLD)could have numerous complications.Secondary osteoporosis and increased bone fragility are frequently overlooked complications in CLD patients.Previous studies implied that up to one-third of these individuals meet diagnostic criteria for osteopenia or osteoporosis.Recent publications indicated that CLD-induced bone fragility depends on the etiology,duration,and stage of liver disease.Therefore,the increased fracture risk in CLD patients puts a severe socioeconomic burden on the health system and urgently requires more effective prevention,diagnosis,and treatment measures.The pathogenesis of CLD-induced bone loss is multifactorial and still insufficiently understood,especially considering the relative impact of increased bone resorption and reduced bone formation in these individuals.It is essential to note that inconsistent findings regarding bone mineral density measurement were previously reported in these individuals.Bone mineral density is widely used as the“golden standard”in the clinical assessment of bone fragility although it is not adequate to predict individual fracture risk.Therefore,microscale bone alterations(bone microstructure,mechanical properties,and cellular indices)were analyzed in CLD individuals.These studies further support the thesis that bone strength could be compromised in CLD individuals,implying that an individualized approach to fracture risk assessment and subsequent therapy is necessary for CLD patients.However,more well-designed studies are required to solve the bone fragility puzzle in CLD patients.
文摘BACKGROUND This case report contributes to the medical literature by highlighting the successful management of a neglected femoral neck fracture in a patient with renal osteodystrophy and secondary hyperparathyroidism(SHPTH)who was on dialysis due to end-stage renal disease(ESRD).It underscores the efficacy of parathyroidectomy(PTX)in restoring bone mineral density(BMD)and promoting fracture healing,addressing a significant complication in ESRD patients.CASE SUMMARY A 36-year-old female with renal osteodystrophy and on dialysis due to ESRD presented with a history of left patellar tendon rupture and later,a right achilles tendon avulsion fracture.Persistent right hip pain led to the discovery of a neglected right femoral neck fracture,which was initially overlooked due to the patient’s complex medical history.Two months post-achilles tendon repair,the patient underwent PTX to manage the refractory SHPTH.The postoperative course included rehabilitation and weight-bearing exercises.Remarkably,2 years after osteosynthesis,radiographic assessments indicated a solid union of the periprothesis fracture and significant improvement in BMD,showcasing the efficacy of the treatment approach.CONCLUSION PTX,combined with appropriate rehabilitation,is crucial for improving BMD and fracture healing in ESRD patients with SHPTH.
文摘The persistence and severity of hyperparathyroidism(HPT) post-renal transplantation is relatively frequent and primarily associated with the timing and its magnitude in the pre-transplant period and with the presence of parathyroid adenomas. HPT after renal transplantation is clinically manifested with hypercalcemia, hypophosphatemia, bone pain, fractures, and in more serious cases with cardiovascular calcifications that affect the survival. The primary clinical objective for patients with secondary HPT after renal transplantation is to obtain a level of parathyroid hormone(PTH) adequate to the renal transplanted function and to normalize levels of calcium, phosphorus and vitamin D. In many cases during this period, the development of hypercalcemiaand/or hypophosphatemia makes it necessary to take different therapeutic measures. The use of vitamin D or its analogues has been extrapolated from the management of pre-transplant HPT obtaining variable outcomes, although its use is limited by its capacity to produce hypercalcemia. Calcimimetics are drugs that have proven be effective in reducing PTH levels in patients with HPT on dialysis and has been effective in reducing up to 50% PTH levels in moderate to severe HPT in post-renal transplantation.When HPT persists after renal transplantation and does not respond to medical treatment, invasive management by percutaneous ethanol injection therapy of parathyroid glands or parathyroidectomy should be considered. The emergence of new methods for the management of HPT expands the availability of therapeutic tools for transplant patients.
文摘Bilateral, coincident, atraumatic hip fracture is extremely uncommon and usually occurs secondary to seizures, trauma and metabolic disease including renal osteodystrophy. One of the major types<span>,</span><span> secondary hyperparathyroidism </span><span>is </span><span>associated with high bone turnover due to excess parathyroid hormone and usually seen in a chronic dialysis patient. We reported a 20-year-old woman with end stage renal failure and renal osteodystrophy. She sustained atraumatic right subtrochanteric fracture and left neck of femur fracture (Garden 1), then underwent bilateral long proximal femoral nail. Renal osteodystrophy causes pathological fracture by affecting calcium metabolism that stimulates bone resorptions and lead</span><span>s</span><span> to osteoporotic bone. The aim of this case report is to discuss the approach and management done to the patient presented to our center. Bilateral long proximal femoral nail (PFN) was chosen, taking into account the patient’s premorbid, age, fracture pattern and potential complications. Careful multidisciplinary team approach led by the orthopaedic surgeon, nephrologist and physical therapist </span><span>is</span><span> vital for </span><span>the </span><span>patient to achieve good outcome postoperatively, thus reducing morbidity and mortality.</span>
