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OBJECTIVES: To compare the efficacy and safety of larotrectinib with those of infigratinib in adult glioma patients with tyrosine kinase alterations. METHODS: Patients received oral infigratinib 125 mg (IN cohort, n = 125) or oral larotrectinib (LB cohort, n = 105) until unacceptable toxicity or disease progression. RESULTS: Duration of treatment was longer in the LB cohort than in the IN cohort (8 [9.5-6.25] months vs. 5.5 [6-5.25] months, p < 0.0001). Patients with partial responses (p = 0.0424) and overall survival (p = 0.03) were higher in the IN cohort than those in the LB cohort. The number of patients with disease progression was higher in the LB cohort (p = 0.0015). All the patients reported diarrhea, fatigue, vomiting, constipation, and decreased appetite. Patients in the IN cohort reported hyperphosphatemia, hyperlipasemia, stomatitis, dry skin, alopecia, dyspepsia, onycholysis, palmar-plantar erythrodysesthesia, nail disorders, and dry eyes. Patients in the LB cohort reported upper respiratory tract infections, pyrexia, cough, anemia, bacterial/viral infections, conjunctivitis, urinary tract infections, headaches, ataxia, dizziness, and muscle tremors. A total of 30 (24 %) and 40 (38 %) patients from the IN and the LB cohorts died at the follow-up of 18 months (p = 0.03). Patients who received bevacizumab initial therapy had higher overall survival (p = 0.048). CONCLUSIONS: Infigratinib has higher efficacy and overall survival than larotrectinib but has higher adverse effects in the management of both glioma and tyrosine kinase alterations after failure of initial therapies. Initial bevacizumab therapy is associated with a higher overall survival.
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Glioma , Compostos de Fenilureia , Proteínas Tirosina Quinases , Pirazóis , Pirimidinas , Adulto , Humanos , Bevacizumab , Glioma/tratamento farmacológico , Progressão da DoençaRESUMO
Abstract Objectives To compare the efficacy and safety of larotrectinib with those of infigratinib in adult glioma patients with tyrosine kinase alterations. Methods Patients received oral infigratinib 125 mg (IN cohort, n = 125) or oral larotrectinib (LB cohort, n = 105) until unacceptable toxicity or disease progression. Results Duration of treatment was longer in the LB cohort than in the IN cohort (8 [9.5-6.25] months vs. 5.5 [6-5.25] months, p < 0.0001). Patients with partial responses (p = 0.0424) and overall survival (p = 0.03) were higher in the IN cohort than those in the LB cohort. The number of patients with disease progression was higher in the LB cohort (p = 0.0015). All the patients reported diarrhea, fatigue, vomiting, constipation, and decreased appetite. Patients in the IN cohort reported hyperphosphatemia, hyperlipasemia, stomatitis, dry skin, alopecia, dyspepsia, onycholysis, palmar-plantar erythrodysesthesia, nail disorders, and dry eyes. Patients in the LB cohort reported upper respiratory tract infections, pyrexia, cough, anemia, bacterial/viral infections, conjunctivitis, urinary tract infections, headaches, ataxia, dizziness, and muscle tremors. A total of 30 (24 %) and 40 (38 %) patients from the IN and the LB cohorts died at the follow-up of 18 months (p = 0.03). Patients who received bevacizumab initial therapy had higher overall survival (p = 0.048). Conclusions Infigratinib has higher efficacy and overall survival than larotrectinib but has higher adverse effects in the management of both glioma and tyrosine kinase alterations after failure of initial therapies. Initial bevacizumab therapy is associated with a higher overall survival.
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RESUMEN El manejo de la hiperfosfatemia de los pacientes con insuficiencia renal crónica en diálisis permanece como un desafío. A pesar de utilizar un enfoque multifacético que incluye la restricción dietética, la remoción de fósforo por la diálisis y el uso de quelantes de fósforo, esta estrategia múltiple no logra reducir los niveles de fósforo en más de 2 mg/dl. El control de fósforo de los pacientes en diálisis es fundamental en razón de la relación monotónica entre los niveles séricos de fosfato y el incremento del riesgo cardiovascular. Por lo tanto, hay una necesidad de explorar nuevas estrategias para reducir los niveles séricos de fosfato a niveles normales. Recientes avances en nuestra compresión de los mecanismos que subyacen a la homeostasis del fósforo sugieren que el transporte gastrointestinal del fósforo podría ser un objetivo. Recientemente se han desarrollado inhibidores de los cotransportadores sodio fosfato del intestino y se ha revalorizado el uso de la nicotinamida, en su formulación de liberación prolongada, que también actuaria por ese mecanismo. También se han drogas como el tenapanor, que inhibiendo el intercambiador sodio/hidrogeno isoforma 3 del enterocito, disminuyen la absorción paracelular de fósforo.
ABSTRACT Management of hyperphosphatemia in patients with chronic renal failure on dialysis remains challenging. Despite using a multifaceted approach that includes dietary restriction, phosphorus removal by dialysis, and phosphate binders, these multiple strategies fail to reduce phosphorus levels by more than 2 mg/dL. Phosphorus control in dialysis patients is essential due to the monotonic relationship between serum phosphate levels and increased cardiovascular risk. Therefore, there is a need to explore new strategies to reduce serum phosphate levels to normal levels. Recent advances in understanding the mechanisms underlying phosphorus homeostasis suggest that the gastrointestinal transport of phosphorus could be a target. Inhibitors of intestinal sodium phosphate cotransporters recently developed, and using of nicotinamide, in its prolonged release formulation, which would also act by this mechanism, has been revalued. There have also been drugs such as tenapanor, which, by inhibiting the isoform three sodium/hydrogen exchanger of the enterocyte, decreases the paracellular absorption of phosphorus.
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Introducción: La osteodistrofia renal es una osteopatía metabólica difusa, relacionada con la insuficiencia renal crónica, que incluye diversas patologías en el sistema musculoesquelético. Se produce en respuesta a trastornos metabólicos generados por cambios electrolíticos, la inflamación crónica y la alteración hormonal. Estas variaciones modifican el proceso de remodelación ósea. Las manifestaciones clínicas incluyen alteración en el parénquima y estroma óseo, y van desde lesiones expansivas, produciendo deformidad, hasta fracturas patológicas del hueso. Objetivo: Relacionar las manifestaciones clínicas, imagenológicas e histológicas en el componente óseo cráneo facial para el diagnóstico de la osteodistrofia renal. Presentación de caso: Se presentan dos casos clínicos de pacientes con enfermedad renal crónica e hiperparatiroidismo secundario de base, con múltiples masas en maxilares que causan asimetría facial y alteración funcional. Las imágenes tomográficas revelan alteración en la morfología ósea cortical y trabecular. Ambos individuos evidenciaron alteraciones en niveles de hormona paratiroidea, fosfatasa alcalina, fósforo y calcio sérico. La histopatología comprobó tejido fibroóseo con hueso neoformado y gran vascularización, con células multinucleadas tipo osteoclastos sin presencia de hemosiderina. Pacientes manejados de forma interdisciplinaria entre medicina interna, endocrinología y cirugía maxilofacial. Conclusiones: Los pacientes con enfermedad renal crónica avanzada presentan alteración de la estructura y del metabolismo óseo y mineral. Tal situación puede comprometer el complejo óseo craneofacial. Los casos graves de osteodistrofia renal se caracterizan por una marcada expansión de los maxilares, que genera asimetría y rasgos de leontiasis. Las imágenes tomográficas asociadas a osteodistrofia renal presentan óseos trabeculares con expansión de cortical, que evidencia el recambio óseo inmaduro presente. La histopatología no es específica y puede ser similar a los casos de displasia ósea craneofacial. Ante lo anteriormente planteado es fundamental relacionar estos hallazgos con la clínica para definir un diagnóstico adecuado(AU)
(AU)Introduction: Renal osteodystrophy is a diffuse metabolic osteopathy, related to chronic renal failure, which includes various pathologies in the musculoskeletal system. It occurs in response to metabolic disorders generated by electrolyte changes, chronic inflammation and hormonal alteration. These variations modify the process of bone remodeling. Clinical manifestations include alteration in the parenchyma and bone stroma, and range from expansive lesions, producing deformity, to pathological fractures of the bone. Objective: Relate the clinical, imaging and histological manifestations in the skull-facial bone component for the diagnosis of renal osteodystrophy. Case Presentation: Two clinical cases of patients with chronic kidney disease and secondary underlying hyperparathyroidism are presented, with multiple masses in the jaws that cause facial asymmetry and functional alteration. Tomographic images reveal alteration in cortical and trabecular bone morphology. Both individuals showed alterations in levels of parathyroid hormone, alkaline phosphatase, phosphorus and serum calcium. Histopathology verified fibro-bone tissue with neoformed bone and great vascularization, with multinucleated osteoclast-like cells without the presence of hemosiderin. Patients were attended in an interdisciplinary way between internal medicine, endocrinology and maxillofacial surgery. Conclusions: Patients with advanced chronic kidney disease present alteration of the structure and bone and mineral metabolism. Such a situation can compromise the craniofacial bone complex. Severe cases of renal osteodystrophy are characterized by a marked expansion of the jaws, which generates asymmetry and traits of leonthiasis. The tomographic images associated with renal osteodystrophy present trabecular bones with cortical expansion, which evidences the immature bone turnover present. Histopathology is not specific and may be similar to cases of craniofacial bone dysplasia. Given the above, it is essential to relate these findings to the clinic to define an adequate diagnosis(AU)
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Humanos , Masculino , Feminino , Distúrbio Mineral e Ósseo na Doença Renal Crônica/diagnósticoRESUMO
Phosphate chelators are frequently used in patients with chronic kidney disease (CKD). New iron-based chelators remain understudied and offer a promising therapeutic option for the control of bone and mineral disorders of chronic kidney disease (BMD-CKD). We assessed the effect of the phosphorus chelator, chitosan-iron III (CH-FeCl), compared to calcium carbonate (CaCO3) in BMD-CKD and the potential iron overload in uremic rats. Thirty-two animals were divided into four groups, namely the control, CKD, CKD/CH-FeCl, and CKD/CaCO3 groups. CKD was induced by adding 0.75% (4 weeks) and 0.1% (3 weeks) adenine to the diet. The chelators were administered from week 3 through week 7. The renal function, BMD-CKD markers, and histomorphometry of the femur were assessed at week 7. The CKD group showed a significant increase in creatinine (83.9 ± 18.6 vs. 41.5 ± 22.1 µmol/L; P = 0.001), phosphate (3.5 ± 0.8 vs. 2.2 ± 0.2 mmol/L; P = 0.001), fractional excretion of phosphorus (FEP) (0.71 ± 0.2 vs. 0.2 ± 0.17; P = 0.0001), and FGF23 (81.36 ± 37.16 pg/mL vs. 7.42 ± 1.96; P = 0.011) compared to the control group. There was no accumulation of serum or bone iron after the use of CH-FeCl. The use of chelators reduced the FEP (control: 0.71 ± 0.20; CKD/CH-FeCl: 0.40 ± 0.16; CKD/CaCO3 0.34 ± 0.15; P = 0.001), without changes in the serum FGF23 and parathyroid hormone levels. Histomorphometry revealed the presence of bone disease with high remodeling in the uremic animals without changes with the use of chelators. The CH-FeCl chelator was efficient in reducing the FEP without iron accumulation, thereby paving the way for the use of this class of chelators in clinical settings in the future.
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Osso e Ossos , Quelantes , Fósforo , Insuficiência Renal Crônica , Animais , Osso e Ossos/metabolismo , Quelantes/farmacologia , Fatores de Crescimento de Fibroblastos , Ferro/metabolismo , Hormônio Paratireóideo , Fosfatos/metabolismo , Fósforo/metabolismo , Ratos , Insuficiência Renal Crônica/tratamento farmacológico , Insuficiência Renal Crônica/metabolismoRESUMO
Renal hyperparathyroidism stands out among the complications of kidney disease in dogs due to phosphorus retention with a predisposition to hypocalcemia, parathyroid hormone stimulation with mobilization of calcium from the bones, characterizing fibrous osteodystrophy, unusual in the elderly. The objective was to report it in 12-year-old Labrador with polyuria, polydipsia, and emesis for five months due to maxillary and mandibular volume increase, followed by loosely fixed teeth, and facial deformity. Blood tests showed anemia, thrombocytosis, azotemia, hypoalbuminemia and hyperphosphatemia and urinalysis showed low density, glycosuria, proteinuria, and moderate caudate and transitional epithelial cells. Oral x-rays showed loss of dental bone support and decreased bone radiopacity. Chest radiographs showed decreased density in the ribs and costochondral junction; on the other hand, organs of the cardiorespiratory system showed no changes. The electrocardiogram and echocardiogram did not show impairment. Abdominal ultrasound revealed kidneys with asymmetry, increased echogenicity of the cortical and poorly preserved cortico-medullary definition. Oral histopathology showed intense fibroplasia associated with bone reabsorption. Support therapy was instituted, but the patient died ten days after consultation. Thus, although uncommon in the elderly, fibrous osteodystrophy should be investigated in dogs with advanced-stage chronic kidney disease and, even with conservative therapies, the prognosis is unfavorable.
O hiperparatireoidismo renal destaca-se entre as complicações da doença renal em cães, pela retenção de fósforo com predisposição à hipocalcemia, estimulação de paratormônio com mobilização do cálcio dos ossos, caracterizando a osteodistrofia fibrosa, incomum em idosos. O objetivo foi relatá-la em Labrador de 12 anos com poliúria, polidipsia e vômitos há cinco meses, além de aumento de volume maxilar e mandibular seguido de dentes frouxamente fixados e deformidade facial. Os exames sanguíneos denotaram anemia, trombocitose, azotemia, hipoalbuminemia, hiperfosfatemia, urinálise, baixa densidade, glicosúria, proteinúria e moderadas células caudadas e epiteliais de transição. Pelos raios X orais, houve perda da sustentação óssea dentária e diminuição da radiopacidade óssea. As radiografias de tórax demonstraram diminuição da densidade óssea na região dos arcos costais e junção costocondral; em contrapartida, órgãos do sistema cardiorrespiratório se mostraram sem alterações aparentes. O eletrocardiograma e o ecocardiograma não incidiram comprometimento. O ultrassom abdominal revelou rins com assimetria, aumento da ecogenicidade cortical e definição corticomedular pouco preservada, e a histopatologia oral apontou intensa fibroplasia associada à reabsorção óssea. Foi instituída terapia suporte, mas o paciente veio a óbito 10 dias após a consulta. Assim, mesmo que incomum em idosos, a osteodistrofia fibrosa deve ser investigada em cães com doença renal crônica em estágio avançado, mesmo com as terapias conservadoras, o prognóstico é desfavorável.
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Animais , Masculino , Cães , Distúrbio Mineral e Ósseo na Doença Renal Crônica/veterinária , Hiperfosfatemia/veterinária , Hipocalcemia/veterinária , Mandíbula/patologia , Ultrassonografia/veterinária , Insuficiência Renal Crônica/complicaçõesRESUMO
RESUMEN Los niños con deficiencia de vitamina D pueden tener fósforo normal o alto a pesar de tener una prueba de hormona paratiroidea (PTH) elevada. El pseudohipoparatiroidismo (PHP) se caracteriza por cursar con hiperfosfatemia. La similitud que puede ocurrir entre la deficiencia de vitamina D asociada a hiperfosfatemia y el PHP hace importante revisar reportes de casos de deficiencia de vitamina D asociada a hiperfosfatemia para entender por qué puede ocurrir esta asociación y cuál es la relevancia de estudiar el nivel de vitamina D en niños con sospecha de PHP. El objetivo de esta revisión fue identificar reportes de niños con deficiencia de vitamina D asociada a hiperfosfatemia y discutir los mecanismos de esta asociación. Se identificaron reportes de 7 casos en niños. La deficiencia de vitamina D reduciría la respuesta fosfatúrica ante una PTH elevada. Se concluye que es importante descartar deficiencia de vitamina D en todo niño con sospecha de PHP.
ABSTRACT Children with vitamin D deficiency can have normal or high phosphorus despite having a high parathyroid hormone test (PTH). Pseudohypoparathyroidism (PHP) is characterized by hyperphosphatemia. The similarity that can occur between vitamin D deficiency associated with hyperphosphatemia and PHP makes it important to review case reports of vitamin D deficiency associated with hyperphosphatemia to understand why this association may occur and what is the relevance of studying the vitamin D level in children with suspected PHP. The aim of this review was to identify reports of children with vitamin D deficiency associated with hyperphosphatemia and to discuss the mechanisms of this association. Reports of 7 children cases were identified. Vitamin D deficiency could reduce the phosphaturic response to elevated PTH. It is concluded that it is important to rule out vitamin D deficiency in all children with suspected PHP.
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CONTEXT: Hypoparathyroidism is a rare disease and, as such, its natural history, long-term complications, and correct clinical management remain unclear. OBJECTIVE: To describe the natural history and clinical characteristics of the disease. DESIGN AND SETTING: To present a retrospective observational analysis from 7 specialized centers in Buenos Aires, Argentina. PATIENTS: Chronic hypoparathyroid patients followed-up between 1985 and December 2018. MAIN OUTCOME MEASURES: Data on demographics, etiology, clinical complications, biochemical parameters, dual-energy x-ray absorptiometry (DXA) values, and treatment doses were collected. RESULTS: A total of 322 subjects with chronic hypoparathyroidism were included; 85.7% were female, the mean age was 55.2â ±â 16.8 years, and the mean age at diagnosis was 43.8â ±â 16.8 years. Prevalence of surgical hypoparathyroidism was 90.7%, with the most common causes being thyroid carcinoma and benign thyroid disease. A history of hypocalcemia requiring hospitalization was present in 25.7% of the whole group and in 4.3% of patients who had a history of seizures. Overall, 40.9% of our patients had reported at least 1 neuromuscular symptom. Renal insufficiency was present in 22.4% of our patients and was significantly associated with age (Pâ <â 0.0001). Hyperphosphatemia was present in 42% of patients. A history of severe hypocalcemia, paresthesias, tetany, ganglia calcifications, seizures, and cataracts was significantly higher in nonsurgical patients. CONCLUSION: Although these patients were followed-up by experienced physicians, clinical management was heterogeneous and probably insufficient to assess all the potential complications of this chronic disease. Almost 70% of the study's group of patients met the experts' indications for considering the use of rhPTH 1-84. Being aware of this fact is the 1st step in improving our medical management of this disease in the future.
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Chronic kidney disease (CKD) represents a serious concern for the Mexican population since the main predisposing diseases (diabetes, hypertension, etc.) have a high prevalence in the country. The development of frequent comorbidities during CKD such as anemia, metabolic disorders, and hyperphosphatemia increases the costs, symptoms, and death risks of the patients. Hyperphosphatemia is likely the only CKD comorbidity in which pharmaceutical options are restricted to phosphate binders and where nutritional management seems to play an important role for the improvement of biochemical and clinical parameters. Nutritional interventions aiming to control serum phosphate levels need to be based on food tables, which should be specifically elaborated for the cultural context of each population. Until now, there are no available food charts compiling a high amount of Mexican foods and describing phosphorus content as well as the phosphate to protein ratio for nutritional management of hyperphosphatemia in CKD. In this work, we elaborate a highly complete food chart as a reference for Mexican clinicians and include charts of additives and drug phosphate contents to consider extra sources of inorganic phosphate intake. We aim to provide an easy guideline to contribute to the implementation of more nutritional interventions focusing on this population in the country.
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Hiperfosfatemia/dietoterapia , Política Nutricional , Insuficiência Renal Crônica/complicações , Dieta , Aditivos Alimentares/administração & dosagem , Humanos , Hiperfosfatemia/etiologia , México , Fósforo na Dieta/administração & dosagem , Fósforo na Dieta/farmacocinética , Insuficiência Renal Crônica/dietoterapia , Insuficiência Renal Crônica/tratamento farmacológicoRESUMO
ABSTRACT Objective The aim of the present study was to evaluate whether arterial stiffness is affected in the patients with hypoparathyroidism through pulse wave analysis (PWA). Subjects and methods Sixty-three patients diagnosed with hypoparathyroidism and sixty volunteers were evaluated for the study. When 21 patients were excluded in the hypoparathyroidism group due to exclusion criteria, the research continued with 42 patients and 60 volunteers who are similar to the patients in terms of age, gender and body mass index (BMI). Fasting plasma glucose after 10 hours of fasting, creatinine, thyroid stimulating hormone (TSH), free thyroxine (fT4), albumin, calcium, phosphorus, magnesium, 25-OH vitamin D, parathormone (PTH) and urine calcium results in 24-hour urine for the patients in the hypoparathyroidism group were recorded. Evaluation of arterial stiffness was performed by Mobil-O-Graph 24h PWA device. Results Systolic blood pressure (SBP) (p = 0.01), diastolic blood pressure (DBP) (p = 0.005), mean blood pressure (p = 0.009), central SBP (p = 0.004), central DBP (p = 0.01) and pulse wave velocity (PWV) (p = 0.02) were found higher in the hypoparathyroidism group. A positive correlation was detected between phosphorus level and SBP [(p = 0.03. r = 0.327)], central SBP [(p = 0.04, r = 0.324)] and PWV [(p = 0.003, r = 0.449)]. We detected that age and serum phosphorus levels were independent predictor variables for PWV (B = 0.014, p < 0.001 and B = 0.035, p < 0.001, respectively). Conclusion We detected that hypoparathyroidism causes an increase in blood pressure and arterial stiffness. The most significant determinant factors were detected as advanced age and hyperphosphatemia. The patients diagnosed with hypoparathyroidism should be closely monitored and treatment planning should include to prevent the patients from hyperphosphatemia.
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Humanos , Rigidez Vascular , Hipoparatireoidismo , Pressão Sanguínea , Índice de Massa Corporal , Análise de Onda de PulsoRESUMO
ABSTRACT Objective Hypoparathyroidism is a rare condition, whose most common etiology is complications of neck surgery. The aim of the study was to identify the clinical and biochemical profile of the patients with diagnosis of hypoparathyroidism, including the frequency of symptoms, clinical signs, long-term complications and disease control. Additionally, the study sought to know what the medication profile was, and the doses required by the patients. Subjects and method A retrospective cohort study was conducted wherein all patients with ICD-10 codes associated with hypoparathyroidism between 2011 and 2018 at the Hospital Universitario San Vicente Fundación were included. We investigated the etiology of the disease; biochemical profile including lowest serum calcium, highest serum phosphorus, 25OHD levels, calciuria and calcium/phosphorus product; medication doses, disease control, and presence of complications, especially renal and neurologic complications were also evaluated. Results The cohort included 108 patients (99 women/9 men) with a mean age of 51.6 ± 15.6 years. The main etiology was postoperative (93.5%), the dose of elemental calcium received was relatively low (mean 1,164 mg/day), and in only 9.2% of cases more than 2,500 mg/day of elemental calcium was necessary. We were able to evaluate the follow-up in 89 patients, and found that only 57.3% met the criteria for controlled disease. Conclusion The clinical profile of patients with hypoparathyroidism in our cohort is similar to that described in other international studies, with predominantly postoperative etiology. With standard therapy, only adequate control is achieved in a little more than half of patients. Arch Endocrinol Metab. 2020;64(3):282-9
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Humanos , Masculino , Feminino , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Adulto Jovem , Hormônio Paratireóideo/sangue , Hipoparatireoidismo/complicações , Biomarcadores/sangue , Estudos Retrospectivos , Colômbia , Hipoparatireoidismo/sangue , Pessoa de Meia-IdadeRESUMO
Resumen Introducción: Las calcificaciones vasculares forman parte del trastorno mineral óseo en la enfermedad renal crónica y constituye una de las principales causas de mortalidad. Existe plausibilidad y asociación experimental entre el trastorno bioquímico con la calcificación vascular, sin embargo, no existe evidencia suficiente de su asociación clínica. Objetivo: Determinar la asociación de las alteraciones bioquímicas del trastorno mineral óseo (calcio >10 mg/dl, fósforo >5 mg/dl, paratohormona >300 pg/ml) con las calcificaciones vasculares valoradas de acuerdo al score de Kauppila. Material y métodos: Estudio observacional, transversal y analítico. Se incluyeron 97 pacientes con ERC estadio V, en terapia de hemodiálisis, 69% prevalente (establecido en >6 meses) con un tiempo promedio de 5,3 años. Se estableció asociación estadística según test Chi2 de Pearson y regresión logística. Resultados: El 60,8% presentó algún grado de calcificación vascular con un score de Kauppila >1 y el 43,3% presentó un score ≥3, que fue predominante en la población prevalente en hemodiálisis (78,6%). Sin embargo, no se encontró asociación estadística con el trastorno bioquímico mineral óseo en el análisis bivariado por Chi2 ni por regresión logística. Conclusiones: Una valoración transversal de la alteración bioquímica del trastorno mineral óseo no permite establecer su asociación con las calcificaciones vasculares. Es necesario establecer previamente el balance positivo prospectivo de calcio y de fósforo para demostrar esta asociación.
Abstract Introduction: Vascular calcifications are part of the mineral bone disorder in chronic kidney disease and they are one of the main causes of mortality. There is plausibility and experimental association between metabolic disorder and vascular calcification; however, there is no enough evidence for their clinical connection. Objective: To determine the association of biochemical alterations of mineral bone disorder (calcium: >10 mg/dL; phosphorus: >5 mg/dL; paratohormone: >300 pg/mL) with vascular calcifications evaluated according to the Kauppila score. Methods: An observational, cross-sectional, analytical study was performed. 97 stage V CKD patients undergoing hemodialysis were included; 69% were prevalent cases (diagnosed within >6 months) with an average time of 5.3 years. A statistical association was established according to Pearson's Chi2 test and logistic regression. Results: A level of vascular calcification was found with a Kauppila score of >1 in 60.8% of patients and of ≥3 in 43.3% of them, being predominant in the prevalent dialysis population (78.6%). However, no statistical association was found with mineral bone disorder in the chi-squared bivariate analysis or the logistic regression. Conclusions: A cross-sectional test of the biochemical alteration in mineral bone disorder does not allow to find an association with vascular calcifications. It is necessary to establish a prospective calcium-phosphorus positive balance first to prove this association.
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Vascular calcification (VC) is common in chronic kidney disease, increases in prevalence as patients progress to end-stage renal disease, and is significantly associated with mortality. VC is a complex and highly regulated process similar to bone formation whereby hydroxyapatite crystals deposit in the intimal or medial layer of arteries. Mineral bone abnormalities are common in chronic kidney disease; reduction in glomerular filtration rate and changes in vitamin D, parathyroid hormone, and fibroblast growth factor 23 result in the dysregulation of phosphorus and calcium metabolism. Cell culture studies, animal models, and observational and clinical studies all suggest this abnormal mineral metabolism plays a role in the initiation and progression of VC in kidney disease. This review will focus on these mineral bone abnormalities and how they may contribute to mechanisms that induce VC in kidney disease.
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Insuficiência Renal Crônica/metabolismo , Calcificação Vascular/metabolismo , Animais , Cálcio/metabolismo , Fator de Crescimento de Fibroblastos 23 , Fatores de Crescimento de Fibroblastos/metabolismo , Glucuronidase/metabolismo , Humanos , Proteínas Klotho , Hormônio Paratireóideo/metabolismo , Fósforo/metabolismo , Insuficiência Renal Crônica/complicações , Calcificação Vascular/etiologia , Vitamina D/metabolismoRESUMO
Abstract Introduction: It is unclear whether residual renal function (RRF) in dialysis patients can attenuate the metabolic impact of the long 68-hour interdialytic interval, in which water, acid, and electrolyte accumulation occurs. Objective: to evaluate serum electrolyte levels, water balance, and acid-base status in dialytic patients with and without RRF over the long interdialytic interval (LII). Methodology: this was a single-center, cross-sectional, and analytical study that compared patients with and without RRF, defined by diuresis above 200 mL in 24 hours. Patients were weighed and serum samples were collected for biochemical and gasometric analysis at the beginning and at the end of the LII. Results: 27 and 24 patients with and without RRF were evaluated, respectively. Patients without RRF had a higher increase in serum potassium during the LII (2.67 x 1.14 mEq/L, p < 0.001), reaching higher values at the end of the study (6.8 x 5.72 mEq/L, p < 0.001) and lower pH value at the beginning of the interval (7.40 x 7.43, p = 0.018). More patients with serum bicarbonate < 18 mEq/L (50 x 14.8%, p = 0.007) and mixed acid-base disorder (57.7 x 29.2%, p = 0.042), as well as greater interdialytic weight gain (14.67 x 8.87 mL/kg/h, p < 0.001) and lower natremia (137 x 139 mEq/L, p = 0.02) at the end of the interval. Calcemia and phosphatemia were not different between the groups. Conclusion: Patients with RRF had better control of serum potassium, sodium, acid-base status, and volemia throughout the LII.
Resumo Introdução: Não se sabe ao certo se a função renal residual (FRR) de pacientes dialíticos pode atenuar o impacto metabólico do maior intervalo interdialítico (MII) de 68 horas, no qual ocorre acúmulo de volume, ácidos e eletrólitos. Objetivo: Avaliar os níveis séricos de eletrólitos, balanço hídrico e status ácido-básico de pacientes dialíticos com e sem FRR ao longo do MII. Metodologia: Tratou-se de estudo unicêntrico, transversal e analítico, que comparou pacientes com e sem FRR, definida como diurese acima de 200 mL em 24 horas. Para tal, os pacientes foram pesados e submetidos à coleta de amostras séricas para análise bioquímica e gasométrica no início e fim do MII. Resultados: Foram avaliados 27 e 24 pacientes com e sem FRR, respectivamente. Pacientes sem FRR apresentaram maior aumento de potássio sérico durante o MII (2,67 x 1,14 mEq/L, p < 0,001) atingindo valores mais elevados no fim (6,8 x 5,72 mEq/L, p < 0,001); menor valor de pH no início do intervalo (7,40 x 7,43, p = 0,018), maior proporção de pacientes com bicarbonato sérico < 18 mEq/L (50 x 14,8 %, p = 0,007) e distúrbio ácido-básico misto (70,8 x 42,3 %, p = 0,042), além de maior ganho de peso interdialítico (14,67 x 8,87 mL/kg/h, p < 0,001) e menor natremia (137 x 139 mEq/L, p = 0,02) no fim do intervalo. A calcemia e fosfatemia não foram diferentes entre os grupos. Conclusão: Pacientes com FRR apresentaram melhor controle dos níveis séricos de potássio, sódio, status ácido-básico e da volemia ao longo do MII.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Equilíbrio Hidroeletrolítico/fisiologia , Diálise Renal/efeitos adversos , Insuficiência Renal/sangue , Rim/fisiopatologia , Fosfatos/sangue , Potássio/sangue , Sódio/sangue , Desequilíbrio Ácido-Base/fisiopatologia , Bicarbonatos/sangue , Aumento de Peso , Cálcio/sangue , Estudos Transversais , Progressão da Doença , Insuficiência Renal/fisiopatologia , Insuficiência Renal/urina , Insuficiência Renal/terapia , Rim/metabolismo , Rim/química , Testes de Função Renal/métodosRESUMO
Hyperphosphatemia is a common complication in patients with chronic kidney disease (CKD), particularly in those requiring renal replacement therapy. The importance of controlling serum phosphate has long been recognized based on observational epidemiological studies that linked increased phosphate levels to adverse outcomes and higher mortality risk. Experimental data further supported the role of phosphate in the development of bone and cardiovascular diseases. Recent advances in our understanding of the mechanisms involved in phosphate homeostasis have made it clear that the serum phosphate concentration depends on a complex interplay among the kidneys, intestinal tract, and bone, and is tightly regulated by a complex endocrine system. Moreover, the source of dietary phosphate and the use of phosphate-based additives in industrialized foods are additional factors that are of particular importance in CKD. Not surprisingly, the management of hyperphosphatemia is difficult, and, despite a multifaceted approach, it remains unsuccessful in many patients. An additional issue is the fact that the supposedly beneficial effect of phosphate lowering on hard clinical outcomes in interventional trials is a matter of ongoing debate. In this review, we discuss currently available treatment approaches for controlling hyperphosphatemia, including dietary phosphate restriction, reduction of intestinal phosphate absorption, phosphate removal by dialysis, and management of renal osteodystrophy, with particular focus on practical challenges and limitations, and on potential benefits and harms.
RESUMO
Resumen Introducción: la hiperfosfatemia es una complicación común de la enfermedad renal crónica (ERC) y empeora progresivamente a medida que disminuye la función renal. Actualmente disponemos de diversas moléculas farmacéuticas para su tratamiento. Dentro de ellas, existen quelantes que contienen hierro, como es el caso del oxihidróxido sucroférrico. Su uso se ha extendido fundamentalmente entre pacientes en hemodiálisis, en sustitución de otros quelantes. Objetivo: describir la tolerabilidad, la aparición de efectos secundarios, la adherencia terapéutica y las cifras de fósforo sérico en pacientes en tratamiento con oxihidróxido sucroférrico en nuestro centro. Materiales y métodos: se analizaron 5 pacientes de la unidad de hemodiálisis del Servicio de Nefrologia del Hospital Universitario de Burgos, España, en el periodo comprendido entre enero de 2017 a mayo de 2018, todos ellos en tratamiento con oxihidróxido sucroférrico. Se evaluaron las concentraciones plasmáticas de fósforo, calcio y hormona paratiroidea durante el tratamiento con oxihidróxido sucroférrico, además de los efectos secundarios y las causas de abandono. El análisis de los datos se realizó mediante el software estadístico IBM SPSS 22 con un intervalo de confianza del 95 %. Se evaluaron las posibles diferencias con el análisis de la t-Student. Resultados: se evidenció una reducción media del 12,27 % de la hiperfosforemia y una reducción en el número de comprimidos diarios del 15,79 %, con buena tolerancia del fármaco en todos los casos. No se evidenció reducción estadísticamente significativa en los niveles plasmáticos de calcio, ni de hormona paratiroidea (PTH). Conclusiones: el oxihidróxido sucroférrico es un fármaco bien tolerado, que generó una disminución de los niveles séricos de fósforo en la población estudiada. Sin embargo, dado el bajo número de casos analizados, no es posible recomendar el uso terapéutico de este fármaco como primera línea de tratamiento de la hiperfosforemia.
Abstract Introduction: Hyperphosphatemia is a common complication of CKD and progressively worsens as renal function decreases. Currently we have several pharmaceutical molecules for its treatment. Among them, there are chelators that contain iron, as is the case of sucroferric oxyhydroxide. Its use has been extended mainly among those on hemodialysis, replacing other chelators. Objective: Describe the tolerability, the appearance of side effects, therapeutic adherence and serum phosphorus levels in patients undergoing treatment with sucroferric oxyhydroxide in our center. Materials and methods: Five patients were analyzed from the hemodialysis unit of the Nephrology Service of the University Hospital of Burgos, from January 2017 to May 2018, all of them under treatment with sucroferric oxyhydroxide. Plasma concentrations of phosphorus, calcium and parathyroid hormone were evaluated during treatment with sucroferric oxyhydroxide, in addition to side effects and causes of abandonment. For the analysis of the data, they were processed using the IBM SPSS 22 statistical software with a confidence interval of 95%. Possible differences were evaluated with the t-Student analysis. Results: There was an average reduction of 12.27% in hyperphosphataemia and a reduction in the number of daily tablets of 15.79%, with good tolerance of the drug in all cases. There was no statistically significant reduction in plasma levels of calcium or parathyroid hormone (PTH). Conclusions: Sucroferric oxyhydroxide is a well-tolerated drug, which generated a decrease in serum phosphorus levels in the population studied. However, given the low number of cases analyzed, it is not possible to recommend the therapeutic use of this drug as the first line of treatment for hyperphosphatemia.
Assuntos
Humanos , Masculino , Feminino , Distúrbio Mineral e Ósseo na Doença Renal Crônica , Evolução Clínica , Diálise Renal , Espanha , Terapêutica , QuelantesRESUMO
Abstract Introduction: Hyperphosphatemia is a serious consequence of chronic kidney disease and has been associated with an increased risk for cardiovascular disease. Controlling serum phosphorus levels in patients on dialysis is a challenge for the clinicians and implies, in most cases, the use of phosphate binders (PB). Part of the reason for this challenge is poor adherence to treatment because of the high pill burden in this patient group. Objective: To assess the real-world effectiveness of sucroferric oxyhydroxide (SO) in controlling serum phosphorus levels and determine the associated pill burden. Methods: A multicenter, quantitative, retrospective, before-after study was conducted with patients receiving online hemodiafiltration. Patients who switched to SO as a part of routine care were included in the study. PB treatment, number of pills, serum phosphorus levels, and intravenous iron medication and dosage were collected monthly during the six months of treatment with either PB or SO. Results: A total of 42 patients were included in the study. After switching from a PB to SO, the prescribed pills/day was reduced 67% from 6 pills/day to 2 pills/day (p < 0.001) and the frequency of pill intake was lowered from 3 times/day to 2 times/day (p < 0.001). During the treatment with SO, the proportion of patients with serum phosphorus ≤ 5.5 mg/dL increased from 33.3% at baseline to 45% after six months of treatment. Conclusion: During the six-month follow-up with SO, serum phosphorus levels were controlled with one third of the pills/day compared to other PB.
Resumo Introdução: A hiperfosfatemia é uma grave consequência da doença renal crônica associada a risco aumentado de doença cardiovascular. O controle dos níveis séricos de fósforo dos pacientes em diálise é um desafio que requer, na maioria dos casos, o uso de quelantes de fosfato (QF). Parte da dificuldade se deve à baixa adesão ao tratamento oriunda do grande número de medicamentos receitados para esse grupo de pacientes. Objetivo: Avaliar a real eficácia do oxihidróxido sucroférrico (OHS) no controle dos níveis séricos de fósforo e determinar a carga de comprimidos associada. Métodos: Estudo multicêntrico, quantitativo, retrospectivo, antes e depois conduzido com pacientes em hemodiafiltração on-line. Pacientes remanejados para OHS como parte dos cuidados de rotina foram incluídos no estudo. Tratamento com QF, número de comprimidos, níveis séricos de fósforo, reposição férrica endovenosa e dosagens foram registrados mensalmente durante seis meses de tratamento com QF ou OHS. Resultados: Foram incluídos 42 pacientes no estudo. Após a mudança de QF para OHS, o número de comprimidos prescritos por dia caiu em 67%, de seis para duas unidades diárias (p < 0,001). A frequência de ingestão de comprimidos caiu de três para duas vezes ao dia (p < 0,001). Durante o tratamento com OHS, o percentual de pacientes com fósforo sérico ≤ 5,5 mg/dL aumentou de 33,3% no início para 45% após seis meses de tratamento. Conclusão: Durante os seis meses de seguimento com OHS, os níveis séricos de fósforo foram controlados com um terço dos comprimidos por dia em relação aos tratamentos com outros QF.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Sacarose/uso terapêutico , Compostos Férricos/uso terapêutico , Hemodiafiltração , Hiperfosfatemia/tratamento farmacológico , Fósforo/sangue , Estudos Retrospectivos , Seguimentos , Resultado do Tratamento , Combinação de Medicamentos , Insuficiência Renal Crônica/complicações , Hiperfosfatemia/etiologia , Adesão à Medicação , Sevelamer/efeitos adversos , Sevelamer/uso terapêuticoRESUMO
ABSTRACT Objective: to evaluate the health-related quality of life of hyperphosphatemic chronic renal patients on hemodialysis before and after a nursing educational intervention. Method: a quasi-experimental before-and-after study, conduted with 63 hyperphosphatemic chronic renal patients from a renal unit of the state of Rio Grande do Sul (Brazil). A sociodemographic and clinical characterization form was used, pruritus intensity record, Kidney Disease Quality of Life - Short Form and Nursing Educational Intervention. This included individual guidance, clarification of doubts and a printed manual. Laboratory data and pruritus evaluated before, 30 and 60 days after intervention, and quality of life, before and after two months. Analysis from descriptive and analytical measures. Results: after the intervention, there was a statistical difference between the mean scores of the following dimensions: Physical function, Emotional function, Energy/Fatigue, List of problems/symptoms and Cognitive function. Significant correlation was found in the following dimensions: Physical functioning, Physical function, Pain, General health, Emotional wellbeing, List of problems/symptoms, Effects of kidney disease, Kidney disease overload, Sleep and stimulation by the dialysis team. With the intervention there was a reduction in phosphorus, creatinine, calcium and parathyroid hormone levels. Itching also decreased. Regarding the evaluation of their health, 31.7% considered it "good" and 58.8% "regular" before the intervention; afterwards, 50.8% and 36.5% rated it as "good" and "fair" respectively. Conclusion: nursing educational intervention improves health-related quality of life and health perception of hyperphosphatemic chronic renal patients on hemodialysis.
RESUMEN Objetivo: evaluar la calidad de vida relacionada con la salud de pacientes renales crónicos hiperfosfatémicos en hemodiálisis antes y después de una intervención educativa de enfermería. Método: estudio cuasi experimental del tipo antes y después, realizado con 63 pacientes renales crónicos hiperfosfatémicos de una unidad renal del estado de Rio Grande do Sul (Brasil). Se utilizó un formulario de caracterización sociodemográfica y clínica, registro de intensidad de prurito, Kidney Disease Quality of Life - Short Form y la Intervención Educativa de Enfermería. Esta intervención incluyó orientación individual, esclarecimiento de dudas y un manual impreso. Los datos de laboratorio y de prurito se evaluaron antes de la intervención, y 30 y 60 días después de la misma; y la calidad de vida se evaluó previamente y después de dos meses. El análisis se realizó a partir de medidas descriptivas y analíticas. Resultados: después de la intervención se evidenció una diferencia estadística entre las medias de los puntajes de las siguientes dimensiones: Función física, Función emocional, Energía/Fatiga, Lista de problemas/síntomas y Función cognitiva. Se verificó una correlación significativa entre las siguientes dimensiones: Funcionamiento físico, Función física, Dolor, Salud general, Bienestar emocional, Lista de problemas/síntomas, Efectos de la enfermedad renal, Sobrecarga de la enfermedad renal, y Sueño y Estímulo por parte del equipo de diálisis. Con la intervención se registró una reducción en los niveles de fósforo y creatinina, y un aumento del calcio y de la hormona paratiroidea. El prurito también disminuyó. En relación con la evaluación de su salud, el 31,7% de los participantes la consideraron "buena" y el 58,8% "regular" antes de la intervención; luego de la misma, el 50,8% y el 36,5% la evaluaron como "buena" y "regular", respectivamente. Conclusión: la intervención educativa de enfermería mejora la calidad de vida relacionada con la salud y la percepción de la salud de pacientes renales crónicos hiperfosfatémicos en hemodiálisis.
RESUMO Objetivo: avaliar a qualidade de vida relacionada à saúde de pacientes renais crônicos hiperfosfatêmicos em hemodiálise antes e após uma intervenção educacional de enfermagem. Método: estudo quase experimental do tipo antes e depois, realizado com 63 pacientes renais crônicos hiperfosfatêmicos de uma unidade renal estado do Rio Grande do Sul (Brasil). Utilizado formulário de caracterização sociodemográfica e clínica, registro de intensidade de prurido, Kidney Disease Quality of Life - Short Form e Intervenção Educacional de Enfermagem. Esta compreendeu orientação individual, esclarecimento de dúvida e manual impresso. Dados laboratoriais e prurido avaliados antes, 30 e 60 dias após intervenção, e qualidade de vida, previamente e após dois meses. Análise a partir de medidas descritivas e analíticas. Resultados: após a intervenção evidenciou-se diferença estatística entre as médias dos escores das dimensões Função física, Função emocional, Energia/fadiga, Lista de problemas/sintomas e Função cognitiva. Verificou-se correlação significativa nas dimensões: Funcionamento físico, Função física, Dor, Saúde geral, Bem-estar emocional, Lista de problemas/sintomas, Efeitos da doença renal, Sobrecarga da doença renal, Sono e Estímulo por parte da equipe de diálise. Com a intervenção houve redução nos níveis de fósforo, creatinina, elevação de cálcio e paratormônio. O prurido também diminuiu. Em relação à avaliação de sua saúde, 31,7% a consideraram "boa" e 58,8% "regular" anteriormente à intervenção; após, 50,8% e 36,5% a avaliaram com "boa" e "regular" respectivamente. Conclusão: intervenção educacional de enfermagem melhora a qualidade de vida relacionada à saúde e à percepção de saúde de pacientes renais crônicos hiperfosfatêmicos em hemodiálise.
Assuntos
Humanos , Adulto , Qualidade de Vida , Educação em Saúde , Diálise Renal , Insuficiência Renal Crônica , HiperfosfatemiaRESUMO
Introdução: O controle da hiperfosfatemia é um desafio no tratamento de pacientes hemodialíticos, sendo um dos principais objetivos a serem alcançados. O acompanhamento nutricional e a prática de atividades educativas contribuem para o sucesso na adesão ao tratamento. Objetivo: Descrever, em indivíduos em hemodiálise, o conhecimento do tratamento da hiperfosfatemia, bem como avaliar o possível impacto das orientações nutricionais nos níveis séricos de fósforo após intervenção nutricional em participantes do programa de HD no Hospital Universitário na cidade de Juiz de Fora, MG. Material e Métodos: Estudo quase-experimental, do tipo antes e depois com 35 pacientes em tratamento hemodialítico, com 18 anos ou mais, de ambos os sexos, em diálise há, pelo menos, dois meses e que haviam passado por, pelo menos, uma consulta com a nutricionista do setor. Foram coletados do prontuário dados sociodemográficos e clínicos e aplicados questionário de frequência alimentar adaptado e recordatório 24h para avaliação do consumo alimentar, além de questões fechadas sobre o conhecimento das causas da hiperfosfatemia, alimentos ricos em fósforo e uso de quelantes. A intervenção foi realizada mediante a utilização de cartilha educativa. Após a intervenção foram verificados os níveis séricos de fósforo, considerando hiperfosfatemia valores >5,5 mg/dL. Resultados: Na amostra, 57,1% (n=20) eram do sexo masculino, com média de idade de 61,8 ± 14,3 anos e tempo médio em diálise de 61,1 ± 68,9 meses. A prevalência de hiperfosfatemia antes da intervenção era de 60% (n=21) e ao final da intervenção houve redução para 25,7% (n=9). Houve diferença na redução dos níveis séricos de fósforo quando comparadas as médias antes e depois da intervenção [(5,9 ± 1,3 mg/dL; 4,9 ± 1,7 mg/dL; p<0,001)]. Conclusão: Os resultados mostraram que a intervenção educativa possivelmente contribuiu para melhorar a compreensão e adesão ao tratamento, tendo um papel complementar no manejo da hiperfosfatemia de pacientes dialíticos.
Introduction: Hyperphosphatemia control is a challenge in the treatment of patients in hemodialysis, which is one of the main objectives to be reached. Nutritional accompaniment and practice of educational activities contribute to the success in the adhesion to the treatment. Objective: Describe, in individuals in hemodialysis, the knowledge of the treatment of hyperphosphatemia, as well as to evaluate the possible impact of nutritional orientations on phosphorus levels after nutritional intervention in participants of the program HD at the University Hospital in the city of Juiz de Fora, MG. Material e Methods: Quasi-experimental study, of the before and after kind with 35 patients in hemodialysis treatment, with 18 years or more, of both sexes, in dialysis for, at least, two months and who have had, at least, one appointment with the nutritionist in this division. Sociodemographic and clinical data was collected from patient records and adapted reminder questionnaires of food frequency in the last 24 hours were applied for assessment of food intake, as well as multiple-choice questions about the knowledge of the causes of hyperphosphatemia, phosphorus-rich food and use of binders. The intervention was made through the use of an educational leaflet. After the intervention, phosphorus serum levels were verified, taking into consideration hyperphosphatemia values >5,5 mg/dL. Results: In the sample, 57.1% (n=20) were male, with mean age of 61.8 ± 14.3 years and mean time in dialysis of 61.1 ± 68.9 months. The prevalence of hyperphosphatemia before the intervention had been of 60% (n=21) and at the end of the intervention there was a reduction to 25.7% (n=9). There was difference in the reduction of phosphorus serum levels when compared to the medians before and after the intervention [(5.9 ± 1.3 mg/dL; 4.9 ± 1.7 mg/dL; p<0,001)]. Conclusion: The results showed that the educational intervention possibly contributed to a better understanding and treatment adherence, having a complementary role in the management of hyperphosphatemia in dialysis patients.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Educação Alimentar e Nutricional , Diálise Renal , Insuficiência Renal Crônica , Hiperfosfatemia , NutricionistasRESUMO
Abstract Introduction: Pseudohypoparathyroidism (PHP) is a rare hereditary disease, characterized by hypocalcemia/hyperphosphatemia secondary to peripheral resistance to parathyroid hormone (PTH). PHP diagnosis is usually precluded since hypocalcemia is considered as the primary diagnosis, thus delaying further diagnostic studies and preventing an adequate management of this clinical condition. Materials and methods: Retrospective review of the databases of the Endocrinology departments of two tertiary care centers of Medellin, Colombia from January 2012 to December 2016. Patients diagnosed with PHP based on clinical presentation and confirmatory laboratory values were included. Results: Four patients met the inclusion criteria. All PHP cases were diagnosed in adulthood despite strong early clinical and laboratory evidence of the disease. Three patients were diagnosed with Fahr's syndrome and two with Albright's hereditary osteodystrophy. The mean values obtained were PTH of 376.8 pg/mL, calcium of 6.17 mg/dL and phosphorus of 6.55 mg/dL. Conclusions: PHP is a rare disorder. This paper describes four PHP cases diagnosed during adulthood. Emphasis should be placed on the judicious approach to the patient with hypocalcemia and hyperphosphatemia with increased PTH and normal renal function, since these symptoms strongly suggest a diagnosis of PHP.
Resumen Introducción. El pseudohipoparatiroidismo (PHP) es una condición rara caracterizada por hipocalcemia e hiperfosfatemia secundarias a resistencia periférica a la hormona paratiroidea (PTH). Es frecuente que la hipocalcemia sea establecida de forma equivocada como diagnóstico primario y que el diagnóstico definitivo de PHP sea tardío, difiriendo los estudios y el manejo específico que exigen estos pacientes. Materiales y métodos. Se revisaron de forma retrospectiva las bases de datos de endocrinología de dos centros terciarios de Medellín, Colombia, desde enero de 2012 a diciembre de 2016. Se incluyeron pacientes con diagnóstico de PHP por presentación clínica y valores confirmatorios de laboratorio. Resultados. Cuatro pacientes cumplieron los criterios de inclusión. Todos los casos fueron diagnosticados en la adultez a pesar de tener evidencia temprana, clínica y bioquímica de la enfermedad. Tres pacientes tenían síndrome de Fahr y dos tenían osteodistrofia hereditaria de Albright. Los valores medios registrados fueron PTH de 376.8 pg/mL, calcio de 6.17 mg/dL y fósforo de 6.55 mg/dL. Conclusiones. El PHP es un trastorno raro; se describen cuatro casos diagnosticados de forma tardía en la adultez. Se enfatiza en el enfoque juicioso del paciente con hipocalcemia, la cual, en presencia de hiperfosfatemia con PTH elevada y función renal normal, debe hacer sospechar el diagnóstico de PHP.