RESUMO
Vizcarra-Vizcarra, Cristhian A., Eduardo Chávez-Velázquez, Carmen Asato-Higa, and Abdías Hurtado-Aréstegui. Treatment of focal and segmental glomerulosclerosis secondary to high altitude polycythemia with acetazolamide. High Alt Med Biol. 23:286-290, 2022.-Focal segmental glomerulosclerosis (FSGS) is a morphological pattern, caused by glomerular injury and is the leading cause of nephrotic syndrome in adults. We present the case of a 59-year-old female patient, resident of a high-altitude city (3,824 m), who had polycythemia and nephrotic syndrome. A renal biopsy was performed, and the findings were compatible with FSGS. The patient received phlebotomy 500 ml three times, which reduced, partially, the hemoglobin concentration. However, she had refractory proteinuria, despite the use of enalapril and spironolactone. We observed that proteinuria worsened with the increase in hemoglobin levels. So, she was treated with acetazolamide 250 mg bid for 4 months, which reduced proteinuria and hemoglobin. During the coronavirus disease 2019 (COVID-19) pandemic, the patient did not take acetazolamide and again, she had an increase in hemoglobin and proteinuria levels. We conclude that acetazolamide may be an effective treatment in FSGS due to high altitude polycythemia.
Assuntos
Doença da Altitude , COVID-19 , Glomerulosclerose Segmentar e Focal , Síndrome Nefrótica , Policitemia , Acetazolamida/uso terapêutico , Adulto , Altitude , Doença da Altitude/complicações , Doença da Altitude/tratamento farmacológico , Feminino , Glomerulosclerose Segmentar e Focal/complicações , Glomerulosclerose Segmentar e Focal/etiologia , Humanos , Pessoa de Meia-Idade , Síndrome Nefrótica/complicações , Síndrome Nefrótica/patologia , Policitemia/complicações , Policitemia/etiologia , Proteinúria/etiologiaRESUMO
INTRODUCTION: Considering the evolving diagnostic criteria of polycythemia vera (PV), we analyzed the utility of serum erythropoietin (EPO) as a predictive marker for differentiating polycythemia vera (PV) from other etiologies of erythrocytosis. PATIENTS AND METHODS: We conducted a retrospective study after a review of electronical medical records from January 2005 to December 2016 with diagnosis of erythrocytosis using International Classification of Disease-specific codes. To evaluate the diagnostic performance of EPO levels and JAK2-V617F mutation, we constructed a receiver-operated characteristic curve of sensitivity versus 1-specificity for serum EPO levels and JAK2-V617F mutation as predictive markers for differentiating PV from other causes of erythrocytosis. RESULTS: We surveyed 577 patients with erythrocytosis. Median patient age was 59.2 years, 57.72% (n = 329) were male, 86.3% (n = 491) were white, and only 3.3% (n = 19) were African American. A total of 80.88% (n = 351) of those diagnosed with PV had a JAK2-V617F mutation compared to only 1.47% (n = 2) whose primary diagnosis was secondary polycythemia. When comparing JAK2-V617 mutation to the EPO level, the area under the curve of JAK2-V617 (0.8970) was statistically larger than that of EPO test (0.6765). Therefore, the PV diagnostic methodology using JAK2-V617 is better than the EPO test. An EPO level of < 2 mIU/mL was > 99% specific to predict PV but was only 12% sensitive. CONCLUSION: In the appropriate clinical setting, cytogenetic and molecular studies such as JAK2 mutation status prevail as the most useful tools for PV case identification. The use of isolated EPO to screen patients with erythrocytosis is not a good diagnostic approach.
Assuntos
Eritropoetina/sangue , Janus Quinase 2/genética , Policitemia Vera/diagnóstico , Policitemia/etiologia , Biomarcadores Tumorais/sangue , Biomarcadores Tumorais/genética , Institutos de Câncer , Feminino , Testes Genéticos , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Policitemia Vera/sangue , Policitemia Vera/complicações , Policitemia Vera/genética , Valor Preditivo dos Testes , Curva ROC , Estudos RetrospectivosRESUMO
INTRODUCTION: Renal transplantation presents multiple complications after its completion, some of them related to the behavior of hemoglobin levels. The objective of this study is to determine the behavior and prevalence of anemia and erythrocytosis in the first year after renal transplantation. MATERIAL AND METHODS: A retrospective, observational study was conducted of a cohort of patients of the 21st Century National Medical Center in Mexico of transplants performed from January 1, 2013 to December 31, 2017. A total of 649 met the inclusion criteria. Pre-transplant hemoglobin (Hb) levels were determined, as well as levels 1 month, 3, 6, 9, and 12 months after transplantation, and the prevalence of anemia and erythrocytosis was determined in each month. Descriptive analysis was performed with measures of central tendency and measures of dispersion. The statistical program SPSS version 25 was used. RESULTS: The mean pre-transplant Hb was 10.69 g/dL (standard deviation [SD] 2.04). One year after the renal transplant, Hb averaged 14.45 g/dL (SD 2.30), which meant an increase over the first year after renal transplantation of 3.76 g/dL. Pre-transplant anemia occurred in 73.1% of patients, and erythrocytosis in 0.1%; 12.9% of patients and 5.9% in erythrocytosis continued with anemia for a year. CONCLUSIONS: Renal transplantation allows Hb levels to recover in a multifactorial way; however, the persistence of anemia and erythrocytes creates a study challenge in any transplant unit, due to their prevalence of 12.9 and 5.9% respectively.
Assuntos
Anemia/epidemiologia , Transplante de Rim/efeitos adversos , Policitemia/epidemiologia , Adulto , Anemia/etiologia , Estudos de Coortes , Feminino , Hemoglobinas/análise , Humanos , Masculino , México , Pessoa de Meia-Idade , Policitemia/etiologia , Prevalência , Estudos Retrospectivos , Adulto JovemRESUMO
La policitemia primaria es producida por una mutación adquirida o heredada en las células progenitoras de los glóbulos rojos, mientras que la poliglobulia secundaria está relacionada con un aumento de la eritropoyetina sérica como respuesta a la hipoxia tisular o a la producción autónoma tumoral. Hace más de medio siglo que se conoce que la hidronefrosis puede actuar como una rara causa de eritrocitosis debido al aumento de producción de eritropoyetina por un riñón que censa una disminución de oxígeno, mecanismo también observado en la estenosis de la arteria renal y en los quistes renales. Se describe a continuación el caso de un paciente de 38 años con poliglobulia atendido en el Hospital Italiano de San Justo (Argentina), que presenta como hallazgo una hidronefrosis unilateral severa y cuya resolución quirúrgica a través de una nefrectomía revierte el cuadro hematológico de base. (AU)
Primary polycythemia is produced by an acquired or inherited mutation in progenitor cells of red blood cells, while secondary polyglobulia is related to an increase in serum erythropoietin in response to tissue hypoxia or autonomous tumor production. Since the middle of the twentieth century, the hydronephrosis is known to be a rare etiology of secondary polycythemia, with increased erythropoietin production caused by diminished oxygen sensing by the kidney, also seen in renal artery stenosis and kidney cysts. We describe a case of a 38 year old patient with polycythemia studied in the "Hospital Italiano de San Justo" (Argentina) that presented an incidental severe unilateral hydronephrosis, and nephrectomy was carried out as a final resolution of the hematological disorder. (AU)
Assuntos
Humanos , Animais , Masculino , Adulto , Pessoa de Meia-Idade , Policitemia/diagnóstico , Pielonefrite/diagnóstico , Infecções Urinárias/complicações , Eritropoetina/sangue , Hidronefrose/diagnóstico , Nefrectomia/tendências , Policitemia/complicações , Policitemia/etiologia , Pielonefrite/sangue , Obstrução da Artéria Renal/patologia , Dor Lombar , Hipóxia-Isquemia Encefálica/patologia , Eritrócitos/fisiologia , Doenças Renais Císticas/patologia , Disuria , Febre , Hidronefrose/cirurgia , Hidronefrose/complicações , Anemia , Nefrectomia/métodosAssuntos
Adaptação Fisiológica , Altitude , Encefalopatias/etiologia , Hipóxia/fisiopatologia , Pneumopatias/etiologia , Encéfalo/fisiopatologia , Encefalopatias/fisiopatologia , Hematócrito , Hemoglobinas/metabolismo , Humanos , Hipóxia/sangue , Hipóxia/complicações , Hipóxia/terapia , Pulmão/fisiopatologia , Pneumopatias/fisiopatologia , Policitemia/etiologiaRESUMO
INTRODUCCIÓN: Entre 15-20% de los embarazos gemelares monocoriales biamnióticos se complican con el síndrome de transfusión feto/fetal el cual se asocia con mortalidad superior a 90% y morbilidad significativa en el 50% del gemelo sobreviviente. La técnica láser que coagula la superficie de la placa coriónica entre los principales canales a lo largo del ecuador (técnica de Solomon), se ha sugerido para disminuir la recurrencia, prevenir complicaciones secundarias sin incrementar resultados adversos. Métodos: REVISIÓN de la literatura existente en las bases de datos MEDLINE, EBSCO, OVID, PROQUEST, COCHRANE, Lilacs, SciELO, desde el año 2000 al 2015. Se incluyeron los artículos de revisión e investigaciones originales que compararon la técnica estándar de fotocoagulación secuencial con láser y la técnica de Solomon, el resultado primario fue la reducción de la incidencia Secuencia Anemia Policitemia, recurrencia del síndrome de transfusión feto/fetal, mortalidad perinatal y morbilidad neonatal severa. RESULTADOS: Se encontraron 200 artículos, se seleccionaron seis: 1 ensayo clínico y su análisis secundario, 2 estudios de cohorte retrospectivos, 1 revisión sistemática y un estudio que compara los resultados del neurodesarrollo. Los estudios sugieren una mejoría en la sobrevida de algunos de los fetos con la técnica Solomon, menor recurrencia del síndrome de transfusión feto/fetal y Secuencia Anemia Policitemia, sin la presencia de eventos adversos. CONCLUSIÓN: La técnica de Solomon mejora la sobrevida de algunos gemelos, sin embargo no puede concluirse que haya mejoría en la mortalidad pues los estudios no tienen el suficiente poder para determinarlo.
INTRODUCTION: Between 15 to 20% of monochorionic diamniotic twin pregnancies are complicated by the twin-twin transfusion syndrome. It has a mortality greater than 90% and a significant morbidity, 50% in the surviving twin. The Solomon technique (laser photocoagulation of the main vascular channels of the chorio-nic plate surface along the entire vascular equator) has been suggested to reduce the recurrence, and pre-vent secondary complications without increasing adverse results. METHODS: Systematic review of electronic searches of the literature from 2000 to 2015 (MEDLINE, EBSCO, OVID, PROQUEST, COCHRANE, Lilacs, and SciELO). We included review articles and original investigations comparing the standard photocoagulation technique with laser ablation against the Solomon technique. The primary results were reduction of Anemia Polycythemia Sequence incidence, twin-twin transfusion syndrome recurrence, perinatal mortality and severe neonatal morbidity. RESULTS: Of 200 articles, we selected six: one clinical essay and its secondary analysis, two retrospective cohort studies, one systematic review and a study comparing neurodeve-lopmental outcomes. The studies suggested a survival improvement in some fetuses using the Solomon technique, less twin-twin transfusion syndrome recurrence and Anemia Polycythemia Sequence without the presence of adverse effects. CONCLUSION: Solomon technique improves the survival of some twins, although we cannot conclude there is mortality improvement, because the studies do not have enough power to determine that.
Assuntos
Humanos , Feminino , Gravidez , Recém-Nascido , Fotocoagulação a Laser/métodos , Transfusão Feto-Fetal/cirurgia , Policitemia/etiologia , Síndrome , Gêmeos , Resultado da Gravidez , Resultado do Tratamento , Transfusão Feto-Fetal/complicações , Fetoscopia , Anemia/etiologia , FotocoagulaçãoRESUMO
PTE is defined as hematocrit >51% or hemoglobin >17 g/dL after renal transplantation. Risk factors include native kidneys with adequate erythropoiesis pretransplant, smoking, renal artery stenosis, and cyclosporine treatment. We report the case of a 14-yr-old female kidney transplant patient, with triple therapy immunosuppression and stable graft function who developed PTE at 12 months post-transplant with hemoglobin 17.3 g/dL, hematocrit 54.2%, stable graft function, and normotensive with normal cardiac echocardiogram and erythropoietin levels. The only risk factor found was tobacco use. As she had no spontaneous improvement, enalapril treatment was started at 19 months post-transplant with a hemoglobin level of 17.5 g/dL and hematocrit 53%; by 23 months post-transplant, hemoglobin lowered to 15 g/dL and hematocrit to 44.5% and continued to be in normal range thereafter. PTE is a rare condition in childhood and can be successfully treated with enalapril.
Assuntos
Transplante de Rim/efeitos adversos , Policitemia/etiologia , Adolescente , Feminino , HumanosRESUMO
Populations living at high altitudes (HAs), particularly in the Peruvian Andes, are characterized by a mixture of subjects with erythrocytosis (16 g dl(-1)