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Abstract Introduction: Renal osteodystrophy (ROD) refers to a group of bone morphological patterns that derive from distinct pathophysiological mechanisms. Whether the ROD subtypes influence long-term outcomes is unknown. Our objective was to explore the relationship between ROD and clinical outcomes. Methods: This study is a subanalysis of the Brazilian Registry of Bone Biopsies (REBRABO). Samples from individual patients were classified as having osteitis fibrosa (OF), mixed uremic osteodystrophy (MUO), adynamic bone disease (ABD), osteomalacia (OM), normal/minor alterations, and according to turnover/mineralization/volume (TMV) system. Patients were followed for 3.4 yrs. Clinical outcomes were: bone fractures, hospitalization, major adverse cardiovascular events (MACE), and death. Results: We enrolled 275 participants, of which 248 (90%) were on dialysis. At follow-up, 28 bone fractures, 97 hospitalizations, 44 MACE, and 70 deaths were recorded. ROD subtypes were not related to outcomes. Conclusion: The incidence of clinical outcomes did not differ between the types of ROD.
Resumo Introdução: Osteodistrofia renal (OR) refere-se a um grupo de padrões morfológicos ósseos que decorrem de mecanismos fisiopatológicos distintos. É desconhecido se os subtipos de OR influenciam desfechos em longo prazo. Nosso objetivo foi explorar as relações entre OR e desfechos. Métodos: Este estudo é uma subanálise do Registro Brasileiro de Biópsias Ósseas (REBRABO). As amostras de cada paciente foram classificadas em osteíte fibrosa (OF), osteodistrofia urêmica mista (MUO), doença óssea adinâmica (ABD), osteomalácia (OM), alterações normais/menores, e pelo sistema Remodelação / Mineralização / Volume (RMV). Os pacientes foram acompanhados por 3,4 anos. Os eventos clínicos foram: fraturas ósseas, hospitalizações, eventos cardiovasculares adversos maiores (MACE), e óbito. Resultados: Analisamos 275 indivíduos, 248 (90%) deles estavam em diálise. No acompanhamento, 28 fraturas ósseas, 97 hospitalizações, 44 MACE e 70 óbitos foram registrados. Os subtipos de OR não foram relacionados aos desfechos clínicos. Conclusão: A incidência de desfechos clínicos não diferiu entre os tipos de OR.
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O complexo de desordens hiperostóticas é uma condição rara e autolimitante, que tem as mesmas características histopatológicas, que cursa com proliferação óssea de caráter não neoplásico. Acomete cães jovens de raças distintas, com variabilidade quanto ao tipo de proliferação óssea e quanto aos ossos acometidos. O complexo é composto pela osteopatia craniomandibular, hiperostose da calota craniana e osteodistrofia hipertrófica. Podendo estar presente nos ossos da calota craniana, mandíbulas, coluna cervical e esqueleto apendicular. O presente relato, descreveu o quadro de uma cadela, da raça American Bully, não castrada, três meses de idade, que foi atendida com queixa de aumento de volume doloroso das mandíbulas, hiporexia e sialorreia há 15 dias, apresentando ao exame físico, amplitude de movimento diminuída e sensibilidade dolorosa da articulação temporomandibular, espessamento firme bilateral do crânio em região de fossa temporal, espessamento palpável de consistência firme das mandíbulas e crepitação respiratória. Após avaliação clínica e realização de exames complementares, chegou-se ao diagnóstico presuntivo, de complexo de desordens hiperostóticas. Foi instituído como conduta terapêutica o suporte analgésico, sendo eficaz para a manutenção das necessidades fisiológicas até a paciente alcançar a fase adulta. O prognóstico para esta paciente foi considerado bom, uma vez que não havia indícios de anquilose da articulação temporomandibular e/ou manifestações neurológicas.
The complex of hyperostotic disorders is a rare and self-limiting condition, which has the same histophatological characteristics, which courses with non-neoplastic bone proliferations. It affects young dogs of different breeds, with variability the bones affected. The complex is composed of craniomandibular osteopathy, calvarial hyperostotic syndrome and hypertrophic osteodystrophy. It may be present in the bones of the skullcap, jaws, cervical spine and appendicular skeleton. The present report describes the condition of a female dog, American Bully breed, entire, three months old, with a complaint of painful swelling of the jaws, hyporexia and drooling for 15 days, presenting on physical examination, reduced amplitude and pain of the temporomandibular joint, bilateral firm thickening of the skull in the temporal fossa region, palpable firm-consistent thickening of the mandibles and respiratory crackle. After clinical evaluation and complementary tests, a presumptive diagnosis of hyperostotic disorders complex was reached. It was instituted pain management as a treatment, being effective for the maintenance of physiological needs until the patient reaches the adulthood. The prognosis for this patient was considered good, since there was no evidence of temporomandibular joint ankylosis and/or neurological manifestations.
Subject(s)
Animals , Dogs , Temporomandibular Joint/abnormalities , Bone Development , Hyperostosis/veterinary , Craniomandibular Disorders/veterinary , Dogs/abnormalities , Facial Bones/pathology , Analgesics/therapeutic useABSTRACT
Objective:To investigate the effect and safety of calcitriol on high turnover osteodystrophy in the treatment of patients with maintenance hemodialysis renal failure.Methods:Eighty patients with maintenance hemodialysis renal failure from April 2018 to June 2020 were selected as study objects and divided into the control group (40 cases) and the observation group (40 cases) by the random number table method. The control group was treated with oral calcium carbonate and other oral calcium preparations. The observation group was additively treated with calcitriol on the basis of the control group for 3 months. The therapeutic efficacy and the incidence of adverse reactions during the treatment were compared between the two groups; the levels of bone metabolism related index, kidney function index, hematocrit (Hct) and hemoglobin (Hb) and other index were compared between the two groups before and after treatment.Results:The total effective rate of the observation group was higher than that of the control group: 95.0%(38/40) vs. 80.0%(32/40), the difference was statistically significant ( χ2 = 4.11, P<0.05). After treatment, the levels of immune reactivity parathyroid hormone (iPTH), serum phosphorus, bone alkaline phosphatase (BALP) in the observation group were lower than those in the control group: (391.74 ± 28.69) ng/L vs. (468.50 ± 30.52), (1.02 ± 0.16) mmol/L vs. (1.63 ± 0.21) mmol/L, (70.59 ± 4.15) U/L vs.(73.64 ± 4.09) U/L, and the level of serum calcium was higher than that in the control group: [(2.05 ± 0.13) mmol/L vs. (1.93 ± 0.11) mmol/L, the differences were statistically significant ( P<0.05). After the treatment, the levels of serum creatinine (SCr), blood urea nitrogen (BUN), serum albumin (ALB) and the incidence of adverse reactions between the two groups had no significant differences ( P>0.05). Conclusions:The administration of calcitriol in the treatment of patients with maintenance hemodialysis renal failure can improve the status of high turnover osteodystrophy and anemia, and has a high safety.
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La principal causa de mortalidad en enfermedad renal crónica (ERC), en el 80% de pacientes se da por enfermedad cardiovascular asociada, los parámetros bioquímicos clásicos del metabolismo óseomineral aún no logran explicar la progresión patológica por tanto, se ha empezado a estudiar nuevos marcadores con relación al daño cardiovascular, donde se ha encontrado al marcador FGF-23 y su correceptor Klotho, implicados en la génesis del daño cardiovascular y enfermedad óseomineral asociada al fosforo, que en conjunto causan remodelamiento cardiovascular, lo que ha empezado aclarecer aún más esta dinámica fisiopatológica. Esta revision busca conocer la implicación de los marcadores FGF-23 y Klotho en ERC y el riesgo cardiovascular asociado y para ello realizó una revision sistemática de literatura, indagando en bases biomédicas como COCHRANE, Embase, LILACS, Scielo, Pub-Med, EBSCO, Hinari, Sociedades médicas, así como tesauros MeSH propios de esta investigación.
The main cause of mortality in chronic kidney disease (CKD), in 80% of patients, is due to associated cardiovascular disease, the classic biochemical parameters of bone-mineral metabolism will not yet be able to explain the pathological progression, therefore, new markers have begun to be studied in relation to cardiovascular damage, where the marker FGF-23 and its co-receptor Klotho have been found, involved in the genesis of cardiovascular damage and bone-mineral disease associated with phosphorus, which together cause cardiovascular remodeling , which has begun to further clarify this pathophysiological dynamic.This review seeks to know the implication of the FGF-23 and Klotho markers in CKD and the associated cardiovascular risk and for this purpose, a systematic review of the literature was carried out, investigating biomedical bases such as COCHRANE, Embase, LILACS, Scielo, Pub-Med, EBSCO, Hinari, Medical Societies, as well as MeSH thesauri specific to this research.
Subject(s)
Cardiovascular Diseases , Medical Subject HeadingsABSTRACT
Leontiasis ossea is an uncommon complication of advanced chronic kidney disease that alters the facial bone and the airway, making its perioperative management more complex. We present a clinical case of a female with Leontiasis ossea presenting a difficult airway which requires parathyroidectomy. Assessment, planning and management of the airway by awake intubation is described.
La leontiasis ossea es una complicación infrecuente de la enfermedad renal crónica avanzada que altera el macizo facial óseo y la vía aérea, complejizando su manejo perioperatorio. Presentamos caso clínico de mujer portadora de leontiasis ossea con vía aérea difícil requiriendo paratiroidectomía. Se describe valoración, planificación y manejo de vía aérea mediante intubación vigil.
Subject(s)
Humans , Female , Adult , Hyperostosis Frontalis Interna/complications , Parathyroidectomy/methods , Airway Management/methods , Anesthetics/administration & dosage , Chronic Kidney Disease-Mineral and Bone Disorder/complications , Hyperostosis Frontalis Interna/etiologyABSTRACT
Renal osteodystrophy (ROD) is a skeletal complication resulting from pathologic alterations in calcium, phosphate, and bone metabolism. The potential link between bone turnover and bone quality is an important question meriting study because of the relatively high incidence of fractures reported. In this case presents a pathological fracture on a routinely hemodialyzed woman. She complained pain on her left subtrochanteric area after low energy trauma accident. A thickening of the shaft femoral bone cortex was also found, reflecting the osteosclerosis event due to imbalance of proliferation and differentiation of osteoblast with increase bone formation. She also had a blastic lesion on her contralateral shaft femoral during the bone survey, but this finding remains asymptomatic. Decreased osteoclastic activity may contribute to cortical thickening, resulting in overall bone mass increase, which may lead to decreased elasticity of the bone or impaired repair capabilities, therefore increasing the risk of fracture. The blastic lesion that occurred on contralateral side may indicated effect of ROD or primary bone lesion. Careful assessment and holistic management of patients with kidney disease is necessary to achieve optimal outcome. The prevention of falls is also an important strategy to prevent pathological fractures. End stage renal disease (ESRD) have reduced bone mineral density, a risk factor for fracture incidence. Careful anamnesis and clinical examination are needed for diagnosis and management.
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Hepatic osteodystrophy is frequent complication in patients with chronic liver disease, particularly with chronic cholestasis. We report a male infant with congenital hepatoblastoma, who had osteodystrophy complicated by multiple bone fractures despite adequate supplementation of fat-soluble vitamins including vitamin D. He was born by Caesarean section because of a 7 cm–sized abdominal mass detected by prenatal ultrasonography. The pathologic diagnosis was hepatoblastoma, PRETEXT staging III or IV. Whole body bone scan at the time of diagnosis showed no abnormal uptake. Oral vitamin D3 of 2,000 IU/day was administered with other fat-soluble vitamins. Serum direct bilirubin level gradually increased up to 28.9 mg/dL at postnatal 6 days and was above 5 mg/dL until 110 days of age. Bony changes consistent with rickets became apparent in left proximal humerus since 48 days of age, and multiple bone fractures developed thereafter. With resolving cholestasis by chemotherapy, his bony lesions improved gradually after add-on treatment of bisphosphonate and parenteral administration of vitamin D with calcium. High level of suspicion and prevention of osteodystrophy is needed in patients with hepatoblastoma, especially when cholestasis persists.
Subject(s)
Female , Humans , Infant , Male , Pregnancy , Bilirubin , Calcium , Cesarean Section , Cholecalciferol , Cholestasis , Diagnosis , Drug Therapy , Fractures, Bone , Hepatoblastoma , Humerus , Liver Diseases , Rickets , Ultrasonography, Prenatal , Vitamin D , VitaminsABSTRACT
@#We describe a 29-year-old Para 1 post-Emergency Lower Segment Caesarean Section (EMLSCS) for fetal distress and Preterm Rupture of the Membrane (PROM) referred by the Obstetric team for persistent bradycardia. She had the typical features of Albright’s Hereditary Osteodystrophy (AHO). The laboratory investigation revealed hypocalcemia, hyperphosphatemia with a high Parathyroid hormone (PTH) level and low free Thyroxine 4 (fT4) with high Thyroid Stimulating Hormone (TSH). The patient was diagnosed with Pseudohypoparathyroidism (PHP) Type 1A associated with TSH resistance based on the somatic features of AHO present as well as biochemical and radiological abnormalities.
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Resumen Introducción: el hiperparatiroidismo terciario es la consecuencia final de las alteraciones del metabolismo calcio-fósforo en pacientes con enfermedad renal crónica. Aquellos pacientes que no logran controlarlo con el tratamiento médico, requieren paratiroidectomía. Objetivo: describir la evolución clínica y paraclínica en pacientes con enfermedad renal crónica y diagnóstico de hiperparatiroidismo terciario, que fueron llevados a paratiroidectomía. Metodología: estudio prospectivo, que incluyó pacientes con hiperparatiroidismo terciario llevados a paratiroidectomía entre los años 2006 y 2015. Se realizaron estudios bioquímicos pre y post quirúrgicos y se evaluó la presencia y progresión de síntomas. Resultados: se incluyeron 32 pacientes, 68.8% mujeres, con media de edad de 46.2 años. Se identificaron diferencias estadísticamente significativas en el cambio en los valores de calcio, fósforo y PTH pre y post quirúrgicos. El 81.5% de los pacientes reportaron presencia de síntomas. El 34.6, 26.9, y 23% presentaron mejoría total de dolor óseo, dolor articular y prurito, respectivamente. El 28% presentaron hipocalcemia sintomática post operatoria. No hubo casos de muerte durante el seguimiento. Conclusiones: la paratiroidectomía se constituye en un alternativa segura y confiable para los pacientes con hiperparatiroidismo terciario, mejorando significativamente los síntomas y las alteraciones en el metabolismo óseo y mineral. Vigilar los niveles de calcio en el post operatorio es importante para evitar las complicaciones del síndrome de hueso hambriento.
Abstract Introduction: tertiary hyperparathyroidism is the final consequence of alterations in calcium-phosphorus metabolism in patients with chronic kidney disease. Those patients who fail to control it with medical treatment require parathyroidectomy. Objective: go describe the clinical and paraclinical evolution in patients with chronic kidney disease and diagnosis of tertiary hyperparathyroidism, who underwent parathyroidectomy. Methodology: prospective study, which included patients with tertiary hyperparathyroidism taken to parathyroidectomy between 2006 and 2015. Pre and post-surgical biochemical studies were performed and the presence and progression of symptoms was evaluated. Results: 32 patients were included. 68.8 (%) were women with an average age of 46.2 years. Statistically significant differences were identified in the change in calcium, phosphorus and PTH values before and after surgery. 81.5% of patients reported the presence of symptoms. 34.6, 26.9, and 23% presented total improvement of bone pain, joint pain and pruritus, respectively. 28% presented postoperative symptomatic hypocalcemia. There were no deaths during follow-up. Conclusions: parathyroidectomy is a safe and reliable alternative for patients with tertiary hyper-parathyroidism, significantly improving symptoms and alterations in bone and mineral metabolism. Monitoring calcium levels in the postoperative period is important to avoid the complications of the hungry bone syndrome.
Subject(s)
Humans , Female , Middle Aged , Renal Insufficiency, Chronic , Chronic Kidney Disease-Mineral and Bone Disorder , Parathyroidectomy , Kidney Transplantation , Dialysis , HyperparathyroidismABSTRACT
Objective To investigate the characteristic changes of biochemical markers of mineral metabolism, vascular calcification, and renal osteodystrophy in an adenine-induced rat model of chronic kidney disease (CKD). Methods A total of 20 male Sprague Dawley rats (SD rats) were randomly divided into two groups: the normal group fed with a diet without adenine, and the CKD group fed with an adenine-containing diet (7. 5 g/kg) for the first 4 weeks and then a diet without adenine for the following 2 weeks. At the end of the 2nd week, serum biochemical markers were detected. At the end of the 6th week, the SD rats were sacrificed and serum biochemical markers were detected once again. The aortas were collected for pathological examination and detection of vascular calcium and phosphorus contents. Femurs and the fifth lumbar vertebrae were taken for bone mineral density (BMD) measurement and bone histomorphometric analysis. Results At the end of the 2nd and 6th weeks, compared with the normal control group, the levels of serum creatinine, urea nitrogen, phosphorus and parathyroid hormone (PTH) in the CKD group were significantly increased (P＜0. 05 or P＜ 0. 01), and the level of serum calcium was significantly decreased (P＜ 0. 05 or P＜ 0. 01). Medial layer vascular calcification of the aorta occurred in 50％ of the rats in the CKD group, but was not observed in the normal control group. Vascular calcium and phosphorus contents were significantly higher in the CKD group compared with the normal control group (P＜ 0. 05). The BMD of total femur, cortical and trabecular bone tissues of the femur, and the fifth lumbar vertebra was significantly decreased in the CKD group (P＜ 0. 05 or P＜ 0. 01). The histomorphometric analysis showed that both bone resorption and bone formation of the trabecular bone in the CKD group were increased, indicating a high bone turnover status. The volumes of both trabecular and cortical bones of rats in the CKD group were significantly lower than that of the normal control group (P ＜ 0. 05 or P ＜ 0. 01). However, the trabecular bone mineralization was not significantly different between the two groups. Conclusions The adenine-induced rat model of chronic kidney disease (CKD) established in this study shows reduced serum calcium and increased serum phosphorus and PTH, and medial layer vascular calcification of the aorta. With respect to renal osteodystrophy, this model shows a high trabecular bone turnover, normal trabecular bone mineralization, and low bone volume of cortical and trabecular bone, which meets the characteristics of osteitis fibrosa. This model may become a useful tool for future study of chronic kidney disease-mineral and bone disorder (CKD-MBD).
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OBJECTIVES: The present study was designed to evaluate the bone phenotypes and mechanisms involved in bone disorders associated with hepatic osteodystrophy. Hepatocellular disease was induced by carbon tetrachloride (CCl4). In addition, the effects of disodium pamidronate on bone tissue were evaluated. METHODS: The study included 4 groups of 15 mice: a) C = mice subjected to vehicle injections; b) C+P = mice subjected to vehicle and pamidronate injections; c) CCl4+V = mice subjected to CCl4 and vehicle injections; and d) CCl4+P = mice subjected to CCl4 and pamidronate injections. CCl4 or vehicle was administered for 8 weeks, while pamidronate or vehicle was injected at the end of the fourth week. Bone histomorphometry and biomechanical analysis were performed in tibiae, while femora were used for micro-computed tomography and gene expression. RESULTS: CCl4 mice exhibited decreased bone volume/trabecular volume and trabecular numbers, as well as increased trabecular separation, as determined by bone histomorphometry and micro-computed tomography, but these changes were not detected in the group treated with pamidronate. CCl4 mice showed increased numbers of osteoclasts and resorption surface. High serum levels of receptor activator of nuclear factor-κB ligand and the increased expression of tartrate-resistant acid phosphatase in the bones of CCl4 mice supported the enhancement of bone resorption in these mice. CONCLUSION: Taken together, these results suggest that bone resorption is the main mechanism of bone loss in chronic hepatocellular disease in mice.
Subject(s)
Animals , Male , Bone Diseases, Metabolic/etiology , Bone Diseases, Metabolic/drug therapy , Bone Remodeling/drug effects , Diphosphonates/pharmacology , Bone Density Conservation Agents/pharmacology , Liver Diseases/complications , Phosphorus/administration & dosage , Bone and Bones/drug effects , Bone and Bones/metabolism , Bone and Bones/diagnostic imaging , Bone Diseases, Metabolic/metabolism , Bone Resorption/metabolism , Carbon Tetrachloride , Disease Models, Animal , Core Binding Factor Alpha 1 Subunit/genetics , RANK Ligand/genetics , Osteoprotegerin/genetics , X-Ray Microtomography , Tartrate-Resistant Acid Phosphatase/genetics , Liver Cirrhosis/chemically induced , Liver Cirrhosis/metabolism , Liver Diseases/metabolism , Mice, Inbred C57BLABSTRACT
Objective To explore the risk factors of bone density disorder and vascular calcification in non-dialysis chronic kidney disease (CKD) patients.Methods Clinical data of nondialysis CKD patients who were admitted to the First Affiliated Hospital of Fujian Medical University between January 2013 and June 2014 were retrospectively analyzed.Using dual energy X-ray absorptiometry to evaluate their bone mineral density (BMD) and T value.Patients were divided into normal BMD group (T≥-1),osteopenia group (-2.5 ＜ T ＜-1) and osteoporosis group (T≤-2.5).The vascular calcification was evaluated by pectoral computed tomography.Multi-factor stepwise logistic regression analysis was used to assess the risk factors for low bone density and vascular calcification in non-dialysis CKD patients.Results A total of 337 non-dialysis CKD patients were enrolled.There were 110 (32.6％) patients with normal BMD,and 146(43.3％) patients with osteopenia,and 81(24.0％) patients with osteoporosis.Gender,history of hypertension,25-hydroxy vitamin D and N-terminal osteocalcin shown statistical differences among three groups (all P ＜ 0.05).The incidence rate of 25-hydroxy vitamin D deficiency shown statistical difference among three groups (P=0.012).Further,the rates were increased with the decreased bone mass (x2=7.100,P=0.008).The other mineral bone disorders,such as hypocalcemia,hyperphosphatemia,low intact parathyroid hormone (iPTH) and high iPTH had no statistical difference among three groups (all P ＞ 0.05).Multi-factor stepwise logistic regression analysis revealed that increased iPTH (OR=1.938),and low bone density (OR=1.724) were independent risk factors for CKD patients with vascular calcification (all P ＜ 0.05),while women (OR=3.312) and vascular calcification (OR=1.742) were independent risk factors for CKD patients with low bone density (all P ＜ 0.05).Conclusion Increased iPTH and low bone density are independent risk factors for non-dialysis CKD patients with vascular calcification,while women and vascular calcification are independent risk factors for non-dialysis CKD patients with low bone density.
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Chromosome 2q37 deletion syndrome is a rare chromosomal disorder characterized by mild to moderate developmental delay, brachydactyly of the third to fifth digits or toes, short stature, obesity, hypotonia, a characteristic facial appearance, and autism spectrum disorder. Here, we report on a patient with 2q37 deletion presenting with dilated cardiomyopathy (DCMP). Congenital heart malformations have been noted in up to 20% of patients with 2q37 deletions. However, DCMP has not been reported in 2q37 deletion patients previously. The patient exhibited the characteristic facial appearance (a flat nasal bridge, deep-set eyes, arched eyebrows, and a thin upper lip), developmental delay, mild mental retardation, peripheral nerve palsy, and Albright hereditary osteodystrophy (AHO)-like phenotypes (short stature and brachydactyly). Conventional chromosomal analysis results were normal; however, microarray-based comparative genomic hybridization revealed terminal deletion at 2q37.1q37.3. In addition, the patient was confirmed to have partial growth hormone (GH) deficiency and had shown a significant increase in growth rate after substitutive GH therapy. Chromosome 2q37 deletion syndrome should be considered in the differential diagnosis of patients presenting with AHO features, especially in the presence of facial dysmorphism. When patients are suspected of having a 2q37 deletion, high-resolution cytogenetic analysis is recommended.
Subject(s)
Humans , Autism Spectrum Disorder , Brachydactyly , Cardiomyopathy, Dilated , Chromosome Disorders , Comparative Genomic Hybridization , Cytogenetic Analysis , Cytogenetics , Deoxycytidine Monophosphate , Diagnosis, Differential , Eyebrows , Growth Hormone , Heart , Intellectual Disability , Muscle Hypotonia , Obesity , Paralysis , Peripheral Nerves , Phenotype , ToesABSTRACT
@#<p style="text-align: justify;">The association of Pseudohypoparathyroidism (PHP) with Turner syndrome is very rare and only a single case has been reported so far. Both manifest with short stature and lack of secondary sexual characteristics along with other stigmata similar to each other, creating a diagnostic dilemma.<br />We describe a case of a 15-year-old Asian Indian female who presented with short stature and delayed puberty with overlapping phenotype of PHP and Turner syndrome. The diagnosis of Turner syndrome was made easily on the basis of typical history, clinical features and karyotype but the diagnosis of PHP was suspected only after radiological and biochemical investigations. The association of Turner syndrome with PHP can be easily missed due to similar phenotypes and subtle manifestations.</p>
Subject(s)
Humans , Female , Adolescent , Dwarfism , Karyotype , Karyotyping , Phenotype , Pseudohypoparathyroidism , Puberty , Puberty, Delayed , Turner Syndrome , BrachydactylyABSTRACT
Abstract. Chronic kidney disease is a public health problem worldwide, with disorders of bone mineralisation and metabolism being common problems associated with this disease, causing significant morbidity and impaired quality of life. The expression of the findings in the chronic kidney disease can be categorised based on the classification proposed by the international conference Kidney Disease: Improving Global Outcomes, that divides them into metabolic disorders of calcium and phosphorus (without findings in radiology), bone structure and composition disorders, and extra-skeletal calcifications. These conditions give characteristic radiographic patterns, such as bone resorption and sclerosis, brown tumours, osteomalacia-rickets, osteopenia, and extra-skeletal calcifications, in addition to treatment related disorders of chronic kidney failure. In this article, concepts related to metabolism disorders and bone mineralisation associated with chronic renal disease and renal osteodystrophy will be categorised and updated, showing their various manifestations in radiology.
La enfermedad renal crónica es un problema de salud pública a nivel mundial, siendo los trastornos de la mineralización y el metabolismo óseo problemas comunes asociados a esta enfermedad, que causan una importante morbilidad y un deterioro de la calidad de vida. La expresión de los hallazgos en la enfermedad renal crónica puede sistematizarse con base en la clasificación propuesta por la conferencia internacional Kidney Disease: Improving Global Outcomes, que las divide en trastornos del metabolismo del calcio y el fósforo (sin hallazgos en imágenes), alteración de la estructura y la composición del hueso, y calcificaciones extraesqueléticas. Estos trastornos otorgan patrones radiológicos característicos, como son la resorción y esclerosis ósea, tumores pardos, osteomalacia-raquitismo, osteopenia y calcificaciones extraesqueléticas, además de los trastornos asociados al tratamiento de la falla renal crónica. En el presente artículo se sistematizarán y actualizarán los conceptos relacionados con los trastornos del metabolismo y la mineralización ósea, asociados a la enfermedad renal crónica y la osteodistrofia renal, mostrando sus diversas manifestaciones en radiología.
Subject(s)
Humans , Chronic Kidney Disease-Mineral and Bone Disorder/metabolism , Chronic Kidney Disease-Mineral and Bone Disorder/diagnostic imaging , Chronic Kidney Disease-Mineral and Bone Disorder/physiopathology , Sclerosis/diagnostic imaging , Bone Diseases, Metabolic , Bone Resorption/diagnostic imaging , Calcinosis , Renal Insufficiency, Chronic/complicationsABSTRACT
Objective To understand the clinical characteristics of elderly patients aged 60 years and over in Anhui Province who receive maintenance hemodialysis (MHD).Methods Clinical data of MHD patients in hemodialysis centers of 26 hospitals in Anhui Province from March 31, 2014 to January 1, 2014 were collected.With non-elderly MHD patients (aged over 18 old and under 60) serving as controls, similarities and differences in the primary disease, vascular access, nutritional status, mineral and bone disorders (MBD), and other related aspects in MHD patients were compared.Results The top three original diseases for elderly patients were chronic glomerulonephritis (CGN) (32.8 ％), hypertensive nephrosclerosis (HNS) (27.7 ％) and diabetic nephropathy (DN) (26.2％).The proportion of patients with DN and HNS in the elderly was higher than in the control group (P ＜ 0.001).Vascular access types in elderly MHD patients were arteriovenous fistula (AVF) (89.4 ％), tunneled cuffed catheter (TCC) (8.9 ％), and temporary venous catheter (1.3％).The proportion of patients with AVF was lower (P＜0.001) and the proportion of patients with TCC was higher (P＜0.001) in the elderly group than in the control group.The incidence of anemia was higher in the elderly patients than in the control group (P＜0.05);the proportion of elderly patients with hemoglobin (Hb) levels was 30.3％, in which had no significant difference as compared with the control group (P ＞ 0.05).The incidence of hypoalbuminemia was 21.2％ in elderly patients, which was higher than in the control group.The proportions of elderly MHD patients with normal levels of adjusted serum calcium, serum phosphorus, and intact parathyroid hormone (iPTH) were 52.0％, 27.9％ (higher than in the control group, P＜0.001), and 47.9％, respectively, while there was no difference in adjusted serum calcium and iPTH between the two groups (P＞0.05).The treatment rates for low serum calcium,hyperphosphatemia and secondary hyperparathyroidism (SHPT) in elderly patients were 50.6％,51.6％ and 71.5％, respectively, which had no significant difference as compared with those in the control group (P＞0.05).Conclusions Elderly patients with MHD show distinct characteristics in primary diseases, vascular access types, and nutrition and MBD status, including higher proportions of HNS and DN as their primary diseases.The type of vascular access is still dominated by AVF, and the proportion of TCC is higher.The nutritional status is poor, with a lower incidence of high phosphorus and a higher incidence of low iPTH.Efforts should be made to promote compliance in elderly patients to better control MBD, improve nutritional status and increase safety and effectiveness of treatment.
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Objective To investigate the relationship between the biochemical markers of bone turnover and renal osteodystrophy (ROD) in patients with primary nephrotic syndrome (PNS).Methods A total of 30 patients with PNS and 50 healthy subjects (controls) were included in the study.The BMD of lumbar vertebrae and femur was measured by dual-energy X-ray absorptiometry.The levels of total procollagen type Ⅰ amino-terminal propeptide (TP Ⅰ NP),β-isomerized carboxyterminal propeptide (β-CTx),intact PTH (iPTH),serum calcium,serum phosphorus,ALP,25-OH-Vitamin D3 (25-OH-VD3),β2-micro-globulin(β2-MG),and ratio of urinary to creatinine (UA/Cr) were measured and calculated.The risk factors related to ROD in PNS patients were analyzed.Two-sample t test,multiple linear regression and partial correlation analysis were used to analyze data.Results The BMD values of lumbar vertebrae and femur in the PNS group were significantly decreased compared with those in controls (t =6.162,5.583,3.891 (＜40 years),5.923,5.324,3.129 (≥40 years),all P＜0.05) and the serum levels of TPⅠNP,β-CTx,iPTH,ALP,β2-MG and UA/Cr in the PNS group were significantly higher than those in controls (t:2.738-10.129(＜40 years),3.226-12.581 (≥40 years),all P＜0.05),and the levels of serum calcium and 25-OH-VD3 in the PNS group were significantly lower than those in controls (t =3.624,7.223 (＜40 years),2.011,2.564 (≥40 years),all P＜0.05).But there was no significant difference for serum phosphorus between the 2 groups (t=0.811,0.513,both P＞0.05).TP Ⅰ NP was positively correlated with β-CTx,iPTH,ALP,UA/Cr,β2-MG(r:0.512-0.682,all P＜0.01),and TP Ⅰ NP was negatively correlated with serum calcium and 25-OH-VD3(r=-0.314,-0.562,both P＜0.01)in the PNS group.β-CTx was positively correlated with iPTH,ALP,UA./Cr,β2-MG(r:0.459-0.618,all P＜0.01),and negatively correlated with serum calcium and 25-OH-VD3(r=-0.212,-0.589,both P＜0.01).The iPTH was positively correlated with ALP,UA/Cr and β2-MG (r =0.410,0.606,0.508,all P＜0.05),and negatively correlated with serum calcium and 25-OH-VD3(r=-0.315,-0.516,both P＜0.05).Conclusions The BMD in PNS patients is lower than that in healthy subjects.Combined measurement of TP Ⅰ NP,β-CTx and the BMD is helpful for the diagnosis of ROD in PNS patients.
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Objective To explore the clinical value and the correlation of parathyroid hormone and osteocalcin in hemodialysis patients.Methods According to the dialysis duration of hemodialysis patients,they are divided into the four groups,Ⅰ (dialysis time ＜2 years),Ⅱ (dialysis time 2-5 years),Ⅲ(dialysis time ＞5-10 years),Ⅳ (dialysis time ＞ 10 years),50 cases were randomly selected from each group,50 cases of healthy people were taken as a control group,to detect the contents of PTH and N-MID in serum and analyze the results.Results The content of PTH in hemodialysis patients of Ⅰ,Ⅱ,Ⅲ,Ⅳ group were (170.9 ± 45.9) ng/L,(405.4 ± 65.3) ng/L,(717.1 ± 199.6) rng/L,(1 555.6 ± 417.8) ng/L,the content of N-MID were (79.23 ± 31.62) μg/L,(140.22 ± 50.98) μg/L,(496.11 ± 159.49)μg/L,(617.9 ± 386.18)μg/L,the content of PTH and N-MID in control group were (6.85 ± 3.74) ng/L,(16.6 ± 8.57) μg/L,PTH and N-MID levels of the four hemodialysis patients groups were significantly higher than those of normal control group,the differences had statistically significance(PTH levels of the four groups compared with the control group:group Ⅰ compared with the control group,t =25.199,P ＜0.01 ;group Ⅱ compared with the control group,t =43.091,P ＜0.01 ;group Ⅲ compared with the control group,t =25.402,P ＜0.01 ;group Ⅳ compared with the control group,t =26.211,P ＜0.01 ;N-MID levels of the four groups compared with the control group:group Ⅰ compared with the control group,t =13.518,P ＜ 0.01 ; group Ⅱ compared with the control group,t =16.909,P ＜ 0.01 ; group Ⅲ compared with the control group,t =21.229,P ＜ 0.01 ; group Ⅳ compared with the control group,t =11.007,P ＜ 0.01),the content of PTH and N-MID in Ⅰ,Ⅱ,Ⅲ,Ⅳ groups had statistically significance (four groups of PTH levels compare to each other:group Ⅰ compared with group Ⅱ,t =20.774,P ＜0.01 ;group Ⅰ compared with group Ⅲ,t =18.858,P＜0.01;group Ⅰ compared with group Ⅳ,t =23.295,P＜0.01;groupⅡ compared with group Ⅲ,t =10.495,P ＜ 0.01 ; group Ⅱ compared with group Ⅳ,t =19.233,P ＜ 0.01 ; group Ⅲ compared with group Ⅳ,t =12.805,P ＜ 0.01 ; Four groups of N-MID levels compare to each other:group Ⅰ compared with groupⅡ,t=7.189,P ＜0.01;group Ⅰ compared with group Ⅲ,t =18.130,P ＜0.01 ;group Ⅰ compared with group Ⅳ,t =9.830,P ＜ 0.01 ;group Ⅱ compared with group Ⅲ,t =15.029,P ＜ 0.01 ;group Ⅱ compared with group Ⅳ,t =8.671,P ＜ 0.01 ; group Ⅲ compared with group Ⅳ,t =2.061,P ＜ 0.05).Conclusion PTH levels were positively correlated with N-MID content in hemodialysis patients,high PTH hemodialysis patients should prevent osteoporosis early and to take appropriate treatment.
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Objective To investigate the present state of mineral metabolism and its related factors in elderly patients with maintenance hemodialysis (MHD),in order to improve the quality of life and survival rate in elderly patients with MHD in Xinxiang.Methods Clinical data of 466 patients with treatment of MHD for over 3 months in 4 hospitals in Xinxiang district were enrolled from January 2012 to August 2013 in this study,and the patients were divided into the elderly group (n=159,aged ≥ 60 years) and the non-elderly group (n =307,aged ＜ 60 years).The evaluation indexes for mineral metabolism were determinated,such as serum levels of calcium,phosphorus,intact parathyroid hormone (iPTH) and alkaline phosphatase (ALP).The present state of mineral metabolism and its relationships with anemia,dialysis ages,adequacy of dialysis,malnutrition and inflammatory factors were analyzed in elderly patients with MHD.Results Among 466 patients,the serum levels of calcium,phosphorus and iPTH in MHD patients were (1.95 ± 0.34) mmol/L,(2.54±1.38) mmol/L and (409.5±345.6) ng/L respectively,and their control rates were 34.3％,20.4％ and 25.5％ respectively.And in patients with hemodialysis three times a week,the control rates were 50.8％,31.4％ and 32.2％ respectively.The serum levels of phosphorus,iPTH,ALP,albumin and transferrin saturation were lower in elderly group than in non-elderly group (all P＜0.01),and the serum levels of pre-albumin,hemoglobin and feritin were lower in elderly group than in non-elderly group (all P＜0.05).But the serum levels of calcium and C-reactive protein (CRP)were higher in elderly group than in non-elderly group (all P＜0.05).Conclusions The elderly patients with MHD exhibit unique clinical manifestations of abnormal mineral metabolism,and low bone transformation osteopathy is the main manifestation.The micro-inflammation,malnutrition and anemia are more severe in elderly patients than in non elderly patients.
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Chronic kidney disease-mineral and bone disorder (CKD-MBD) refers to a series of clinical symptoms and biochemical and imaging abnormalities caused by minerals and calcium phosphorus metabolic disorder,which is associated with chronic kidney disease (CKD).Since 2006 ,the concept of CKD-MBD was put forward for the first time,doctors are increasingly paying more attention to it.This review introduces the epidemiological characteristics, clinical manifestation, examination methods, diagnostic criteria and the research progress of treatment and prevention of CKD-MBD.