Orthostatic proteinuria: an overestimated phenomenon?

Arslan, Zumrut; Koyun, Mustafa; Erengin, Hakan; Akbaş, Halide; Aksoy, Gülşah Kaya; Çomak, Elif; Akman, Sema

doi:10.1007/s00467-020-04586-4

Cited by 11 publications

(5 citation statements)

References 17 publications

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“…La proteinuria ortostática tiene una menor prevalencia, la cual es del 1-5 % y se presenta más frecuentemente en la población pediátrica no obesa [7]. La proteinuria ortostática o postural, es aquella en la que hay presencia de proteinuria fuera de rangos normales cuando el paciente está en bipedestación y que retorna a valores normales en decúbito [5].…”

Section: Discusión Proteinuria En La Edad Pediátricaunclassified

“…La proteinuria ortostática o postural, es aquella en la que hay presencia de proteinuria fuera de rangos normales cuando el paciente está en bipedestación y que retorna a valores normales en decúbito [5]. Se define como aquella en la cual los valores de proteína /creatinina en la primera orina de la mañana son menores a 0.2 mg/mg y pueden ser mayores a 0.2 mg/mg durante tomas realizadas en el transcurso del día [7].…”

Section: Discusión Proteinuria En La Edad Pediátricaunclassified

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Proteinuria en la infancia: a propósito de un caso de enfermedad de Dent

Murillo

Paz-Córdoba

Forero-Delgadillo

et al. 2023

Rev. Colomb. Nefrol.

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Introducción: la proteinuria en la edad pediátrica es una entidad relativamente frecuente, la cual puede ser fisiológica o patológica. La segunda, por una alteración a nivel glomerular con pérdida de proteínas de gran tamaño o a nivel tubular, caracterizada por pérdida de proteínas de bajo peso molecular y alteraciones en la excreción de iones. Entre las enfermedades hereditarias que cursan con proteinuria tubular, se ha descrito la enfermedad de Dent, una patología ligada al cromosoma X. Esta enfermedad se manifiesta principalmente en varones, pero las mujeres pueden ser portadoras y tener manifestaciones clínicas leves de la enfermedad. La primera descripción de esta enfermedad fue hecha por Dent y Friedman en 1964. La mayoría de los casos recientemente reportados han sido en China y Alemania. Objetivo: realizar una revisión general de la enfermedad de Dent y del enfoque diagnóstico de la proteinuria en la infancia con base en nuestro caso, para así, sospechar de esta enfermedad. Descripción del caso: se presenta el caso de un paciente masculino sin antecedentes prenatales ni personales de importancia, quien presenta proteinuria persistente desde los primeros meses de vida y a quien, a los 7 años de edad, se le documenta la presencia de una variante ya conocida en el gen CLCN5, causante de la enfermedad de Dent tipo 1. Discusión: la proteinuria persistente patológica en la infancia debe ser estudiada debido a su posible relación con patologías que pueden afectar la función renal. Además de la diferenciación de la proteinuria persistente, de origen glomerular y tubular, la evaluación de alteraciones en la excreción de electrolitos, puede guiarnos hacia la realización de estudios genéticos y, por ende, al diagnóstico de patologías infrecuentes como la enfermedad de Dent. Conclusión: el enfoque diagnóstico de causas poco frecuentes de proteinuria tubular en la infancia, como la enfermedad de Dent, requiere de la valoración conjunta entre nefrología pediátrica y genética clínica.

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Section: Discusión Proteinuria En La Edad Pediátricaunclassified

Proteinuria en la infancia: a propósito de un caso de enfermedad de Dent

Murillo

Paz-Córdoba

Forero-Delgadillo

et al. 2023

Rev. Colomb. Nefrol.

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“…OP was defined as normal protein excretion in the supine posture but increased protein excretion after the patient was in the upright position for at least four to six hours (9,10). The persistent presence of more than 3 red cells per high power field (HPF) in centrifuged urine was defined as hematuria.…”

Section: Relevant Definitionsmentioning

confidence: 99%

Value of magnetic resonance imaging indices of left renal vein entrapment in the diagnosis of nutcracker syndrome in children

et al. 2021

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Background: Nutcracker syndrome (NCS) is also known as left renal vein (LRV) compression syndrome.Magnetic resonance imaging (MRI) can better depict the anatomical structure of soft tissues in the area of compression. Diagnosis of NCS using MRI in adults is not uncommon. However, there are few studies on the diagnosis of NCS using MRI in children. Therefore, we conducted this study to evaluate the clinical value of the MRI indices of the LRV in the diagnosis of NCS in children.Methods: This study was a single-center retrospective analysis. One hundred seventy-four patients with suspected NCS were enrolled from January 2017 to July 2020. The inclusion criteria for suspected NCS were mainly based on clinical symptoms or signs, laboratory examinations and imaging reports. Other diseases that may cause hematuria and/or proteinuria were excluded. We grouped the patients based on the diagnostic criteria for NCS into a nutcracker group and a control group. The receiver operating characteristic (ROC) curve was constructed for evaluating the value of MRI indices in the diagnosis of NCS.Results: The majority of NCS patients presented with orthostatic proteinuria (OP) (67.2%), followed by hematuria (55.2%), abdominal pain (19.0%), and left flank pain (15.5%). The areas under the curve (AUCs) for the superior mesenteric artery (SMA) angle, beak sign, and compression ratio (CR) in the diagnosis of NCS were 0.870, 0.895, and 0.878, respectively, and the best cutoff values of the SMA angle and CR were 36.8 and 3.99, respectively. The specificities of SMA angle <36.8°, beak sign, CR >3.99, SMA angle <36.8° and beak sign, SMA angle <36.8° and CR >3.99, and beak sign and CR >3.99 were 82.5%, 93.8%, 93.8%, 97.9%, 95.9% and 97.9%, respectively.Conclusions: Children with SMA angles less than 36.8°, beak signs and CR greater than 3.99 should be highly suspected of having NCS. Among these parameters, "beak sign" showed the highest diagnostic accuracy by MRI, and the combination of any two of the above parameters achieved a higher specificity than the single parameters.

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“…Orthostatic proteinuria is characterised by elevated protein excretion in the ambulatory position and normal protein excretion in the recumbent position, first morning excretion, or 24-hour urine. Nutcracker phenomenon, often referring to left renal vein (LRV) entrapment, which is prevalent in underweight children and adolescent, is one of the most common causes of orthostatic proteinuria [ 1 – 3 ]. The mechanisms of orthostatic proteinuria were supposed to be related to exaggerated hemodynamic abnormality leading to altered glomerular permeability, on the basis subtle glomerular injury [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Orthostatic proteinuria due to inferior vena cava interruption without nutcracker phenomenon in an old obese female: a case report and literature review

et al. 2023

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Background Nutcracker syndrome (NCS) caused by left renal vein (LRV) entrapment, is one of the most common causes of orthostatic proteinuria. In stereotype, orthostatic proteinuria is often accompanied by left renal vein obstruction and is found in young and underweight individuals. Here, we report a rare case with orthostatic proteinuria in an old obese female caused by a rare type of congenital inferior vena cava (IVC) interruption. Case presentation A 65-year-old obese woman, who suffered from fluctuated proteinuria, had been misdiagnosed as chronic glomerulitis for 30 years. Instead of having any sign of NCS, she had a unique type of IVC interruption. Most venous blood from infrarenal IVC and right kidney drained into her LRV, and then through the expanded communicating vessel, drained into the left ascending lumbar vein which extended as hemiazygos vein. To the best of our knowledge, this is one of the first cases reported of orthostatic proteinuria attributed to the subsequent hemodynamic irregularity caused by IVC interruption without nutcracker phenomenon. Conclusion Adult-onset orthostatic proteinuria is relatively rare, hard to be recognized and could be misdiagnosed as chronic glomerulonephritis. The case provided a novel differential diagnostic condition for those who suffered from fluctuated proteinuria of unknown causes.

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Orthostatic proteinuria: an overestimated phenomenon?

Cited by 11 publications

References 17 publications

Proteinuria en la infancia: a propósito de un caso de enfermedad de Dent

Proteinuria en la infancia: a propósito de un caso de enfermedad de Dent

Value of magnetic resonance imaging indices of left renal vein entrapment in the diagnosis of nutcracker syndrome in children

Orthostatic proteinuria due to inferior vena cava interruption without nutcracker phenomenon in an old obese female: a case report and literature review

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