Risk factors for lower renal compensation after nephrectomy: an analysis of living kidney donors in an Amazonian cohort / Fatores de risco para menor compensação renal pós-nefrectomia: análise de doadores de rim vivo em uma coorte amazônica

Abstract Introduction: Living donor kidney transplantation is considered the ideal renal replacement therapy because it has a lower complication rate and allows an efficient response to the high demand for grafts in the healthcare system. Careful selection and adequate monitoring of donors is a key element in transplantation. Individuals at greater risk of developing kidney dysfunction after nephrectomy must be identified. Objective: To identify risk factors associated with a renal compensation rate (CR) below 70% 12 months after nephrectomy. Methods: This observational retrospective longitudinal study included living kidney donors followed up at the Lower Amazon Regional Hospital between 2016 and 2022. Data related to sociodemographic variables, comorbid conditions and kidney function parameters were collected. Results: The study enrolled 32 patients. Fourteen (43.75%) had a CR < 70% 12 months after kidney donation. Logistic regression found obesity (Odds Ratio [95%CI]: 10.6 [1.7-65.2]), albuminuria (Odds Ratio [95%CI]: 2.41 [1.2-4.84]) and proteinuria (Odds Ratio [95%CI]: 1.14 [1.03-1.25]) as risk factors. Glomerular filtration rate was a protective factor (Odds Ratio [95% CI]: 0.92 [0.85-0.99]). Conclusion: Obesity, albuminuria and proteinuria adversely affected short-term renal compensation rate. Further studies are needed to uncover the prognostic implications tied to these risk factors. Our findings also supported the need for careful individualized assessment of potential donors and closer monitoring of individuals at higher risk.

Resumo Introdução: O transplante de rim de doador vivo é considerado a terapia renal substitutiva ideal por oferecer menor taxa de complicações e possibilitar uma resposta eficiente à grande demanda por enxertos no sistema de saúde. A seleção criteriosa e o acompanhamento adequado dos doadores constituem um pilar fundamental dessa modalidade terapêutica, sendo essencial a identificação dos indivíduos em maior risco de disfunção renal pós-nefrectomia. Objetivo: Identificar fatores de risco para uma Taxa de Compensação (TC) da função renal inferior a 70% 12 meses após a nefrectomia. Métodos: Estudo observacional, retrospectivo e longitudinal conduzido com doadores de rim vivo acompanhados no Hospital Regional do Baixo Amazonas entre 2016 e 2022. Foram coletados dados correspondentes a variáveis sociodemográficas, comorbidades e parâmetros de função renal. Resultados: Foram incluídos 32 pacientes na amostra final. Destes, 14 (43,75%) obtiveram TC < 70% 12 meses após a doação. A regressão logística identificou a obesidade (Odds Ratio [IC95%]: 10.6 [1.7-65.2]), albuminúria (Odds Ratio [IC95%]: 2.41 [1.2-4.84]) e proteinúria (Odds Ratio [IC95%]: 1.14 [1.03-1.25]) como fatores de risco. A taxa de filtração glomerular atuou como fator de proteção (Odds Ratio [IC95%]: 0.92 [0.85-0.99]). Conclusão: Obesidade, albuminúria e proteinúria demonstraram impacto negativo na taxa de compensação renal em curto prazo, o que reitera a necessidade de estudos acerca das implicações prognósticas desses fatores. Além disso, reforça-se a necessidade de avaliação cuidadosa e individualizada dos possíveis doadores, com acompanhamento rigoroso, especialmente para indivíduos de maior risco.

Nefropatía por IgA con microangiopatía trombótica / IgA nephropathy with thrombotic microangiopathy

INTRODUCTION: IgA nephropathy is the most common glomerulopathy in the world, it has a wide clinical expression, from asymptomatic to rapidly progressive glomerulonephritis. The definitive diagnosis is renal biopsy, within which the IgA pattern can be identified, including thrombotic microangiopathy. CLINICAL CASE: 28-year-old female patient, with a history of preeclampsia in the last pregnancy, presents high blood pressure, hematuria and proteinuria. Study begins with initially negative results. Renal biopsy confirms IgA nephropathy with thrombotic microangiopathy. DISCUSSION: Vascular damage is underestimated in IgA nephropathy. Thrombotic microangiopathy can be associated with various clinical manifestations, however when it is associated with IgA Nephropathy it is usually associated with proteinuria, arterial hypertension and elevation of creatinine. In the presence of microangiopathy, secondary causes must be ruled out. In general, there is no pathognomonic serological marker. Eventually patients could benefit from the use of eculizumab. CONCLUSION: IgA nephropathy is the most common glomerulopathy worldwide; there is a wide range of clinical presentations, among which thrombotic microangiopathy can be found. This presentation is associated with a higher risk of progression to end-stage renal disease.

INTRODUCCIÓN: La nefropatía por IgA es la glomerulopatía más frecuente en el mundo, tiene una amplia expresión clínica, desde asintomática hasta glomerulonefritis rápidamente progresivas. El diagnóstico definitivo es la biopsia renal, dentro de las cuales se puede identificar el patrón de la IgA, dentro de los cuales está la microangiopatía trombótica. CASO CLÍNICO: Paciente femenina 28 años, con antecedentes de preeclampsia en último embarazo, presenta hipertensión arterial, hematuria y proteinuria. Se inicia estudio con resultados inicialmente negativos. Biopsia renal confirma nefropatía por IgA con microangiopatía trombótica. DISCUSIÓN: En la nefropatía por IgA se subestima el daño vascular. La microangiopatía trombótica se puede asociar con varias manifestaciones clínicas, sin embargo, cuando está asociada a NIgA suele estar asociado con proteinuria, hipertensión arterial y elevación y creatinina. Ante la presencia de microangiopatía, se deben descartar causas secundarias de la misma. En general no existe un marcador serológico patognomónico. Eventualmente los pacientes se podrían beneficiar del uso de eculizumab. CONCLUSIÓN: La nefropatía por IgA es la glomerulopatía más frecuente a nivel mundial, existe una gran gama de presentaciones clínicas, dentro de las cuales se puede encontrar microangiopatía trombótica. Esta última presentación se asocia con mayor riesgo de progresión a enfermedad renal en etapa terminal.

The association of serum ferritin with preeclampsia and its severity

Background: Preeclampsia, a pregnancy-related condition with heightened blood pressure and organ damage after 20 weeks, prompts concern. Serum ferritin, an iron-storing protein, gauged by blood tests, mirrors iron levels. Investigating link before conception between serum ferritin and preeclampsia could impact how we identify, manage, and treat this condition during pregnancy. Study aimed to assess the association of serum ferritin with preeclampsia and its severity.Methods: This case-control study was conducted in Bangabandhu Sheikh Mujib medical university hospital and Dhaka medical college hospital, Dhaka, Bangladesh from July 2011 to June 2012. A total of 100 pregnant women, comprising 50 cases (Preeclamptic) and 50 controls (Normal pregnant women), were purposively included as study subjects. Data analysis was performed using SPSS version 23.0.Results: In the case group, 64% were with mild and 36% with severe preeclampsia. Mean serum ferritin was significantly higher in cases than in controls (p<0.001); 76% of cases had elevated serum ferritin, compared to 44% in controls (p=0.001). Severe preeclampsia group had a mean serum ferritin of 192.8, mild preeclampsia group had 86.1, and normal pregnant women had 21.7 ng/ml, indicating higher serum ferritin with preeclampsia severity (p<0.001).Conclusions: Preeclamptic cases exhibit significantly elevated serum ferritin levels, with a fourfold increased likelihood compared to normal pregnancies. Furthermore, the severity of preeclampsia is associated with higher serum ferritin concentrations in comparison to uncomplicated pregnancies.

Improving efficiency in preeclampsia diagnosis: spot urinary protein/creatinine ratio

Background: Preeclampsia poses significant challenges to maternal healthcare due to its potential complications. Timely and accurate diagnosis is crucial for maternal and fetal well-being. Traditional methods like the 24-hour urine collection for assessing proteinuria have limitations. The spot urinary protein/creatinine ratio offers a quicker alternative, but its clinical significance remains underexplored.Methods: This cross-sectional study, conducted from July 1, 2018, to June 30, 2019, aimed to compare the spot urinary protein/creatinine ratio with the conventional 24-hour urine protein collection method in pregnant women with preeclampsia. A total of 90 inpatients were included, meeting specific inclusion and exclusion criteria.Results: In our study, 6.66% of subjects exhibited abnormal fundus examination findings, lower than a similar study (13%). No subjects had papilloedema, and conservatively managed abnormalities were comparable between studies. The mean urine protein creatinine ratio in our study was 1.75±2.32.Conclusions: This study highlights the potential of the spot urinary protein/creatinine ratio as an efficient diagnostic tool for preeclampsia at Kamla Nehru State Hospital for Mother and Child. Swift identification of significant proteinuria can streamline patient care, benefiting maternal and fetal outcomes in resource-constrained healthcare settings.

Proteinuria assessment in preeclampsia: a comparative study

Background: Preeclampsia, a severe pregnancy-related hypertensive disorder, presents substantial maternal and fetal health risks. Accurate proteinuria assessment is crucial but traditional methods are cumbersome and error-prone. This study compares the spot urinary protein/creatinine ratio with 24-hour urine collection for proteinuria estimation in preeclampsia at Kamla Nehru State Hospital for Mother and Child.Methods: A cross-sectional study with 90 eligible pregnant women collected comprehensive medical data. Both spot urinary protein/creatinine ratios and 24-hour urine collections were analyzed. Strong correlation (r=0.942, p<0.0001) was observed.Results: Spot urinary protein/creatinine ratio demonstrated moderate diagnostic accuracy (AUC=0.3). Sensitivity was 100%, specificity 87.9%, with PPV and NPV at 90.4% and 92%, confirming its clinical utility for proteinuria diagnosis.Conclusions: This study validates the spot urinary protein/creatinine ratio as an efficient method for proteinuria assessment in preeclampsia, with a strong correlation and high diagnostic value. Widespread adoption has the potential to expedite diagnosis, enhance outpatient care, and improve outcomes for preeclampsia patients, addressing a crucial healthcare challenge in maternal and fetal health.

Eclampsia atípica tardía: compromiso inusual con proteinuria, pero sin hipertensión / Late atypical eclampsia: unusual involvement with proteinuria, but without hypertension

RESUMEN Paciente primigesta de 27 años, sin antecedentes médicos de importancia y con un apropiado control prenatal, quien recibió atención por parto vaginal a las 39 semanas de gestación con anestesia epidural. Una hora después del parto, presentó cefalea holocraneana a predominio derecho, tratada con antiinflamatorios y relajantes muscular por indicación del servicio de neurología. Horas después de su alta, al tercer día posparto, presentó convulsiones tónico-clónicas bilaterales. Por un examen de orina con proteinuria (+) en tira reactiva y elevaciones discretas de la presión arterial, se solicitó un conteo de proteínas en 24 horas, con valores en 1094,5 mg (valor normal: 0-140). La resonancia magnética con contraste solicitada durante su admisión fue normal, recibiendo tratamiento con fenitoína y sulfato de magnesio durante su hospitalización. Fue dada de alta al quinto día, con controles posteriores por consultorio externo, sin cefalea, proteinuria y/o hipertensión.

ABSTRACT A 27-year-old primigravida patient without a relevant medical history and appropriate prenatal control received attention for vaginal delivery at 39 weeks of gestation. One -hour later, she experienced holocranial headache with right predominance, treated with anti-inflammatories and muscle relaxants by the indication of a neurologist. Hours after her discharge, on the third day post-partum, she developed bilateral tonic-clonic seizures.. Following a urine test in the emergency room with proteinuria (+) in a dipstick, we tested 24-hour protein count in 1094 mg (normal values 0-140). Magnetic resonance with contrast at admission was normal. She received Phenytoin and Magnesium Sulfate during her hospitalization. The evolution was favorable, and he was discharged at five days with ambulatory controls in the medical office without headache, proteinuria, and/or hypertension.

Undiagnosed hypertension and proteinuria in an outpatient population in Northern Ghana

Background: There is an upsurge in chronic kidney disease incidence worldwide. Late presentation characterises chronic kidney disease in sub-Saharan Africa. Hypertension and proteinuria are independent risk factors for worsening kidney function, irrespective of the cause of the kidney disease. We assessed the prevalence and predictors of hypertension and proteinuria in an outpatient population in Northern Ghana. Methods: We retrospectively reviewed screening data among adults ?18 years of age in two of Ghana抯 Northern regions. The data retrieved included socio-demographic information, blood pressure recordings, urine dipsticks and fingerpick blood glucose levels. The data were analysed for the prevalence of hypertension and proteinuria in the participants. Binary logistic regression analysis was employed to identify the predictors of significant proteinuria in these participants. A p-value <0.05 was considered statistically significant. Results: Total 1018 participants were included in the study, comprising 50.5% males. The prevalence of uncontrolled hypertension was 28.1%, using a blood pressure cut-off value of ? 140/90 mmHg. Significant proteinuria (? 1+ or 30 mg/dl) was present in 10.7% of the participants. Hypertension (AOR 2.433, 95% CI 1.582-3.742, p<0.001) and hyperglycaemia (AOR 2.226, 95% CI 1.159-4.275, p=0.016) were independent predictors of the presence of significant proteinuria. Conclusions: Uncontrolled hypertension and proteinuria were common in this outpatient population in Northern Ghana. The cost-effectiveness of community-based screening for chronic kidney disease and its risk factors in low-resource settings like Ghana, with the aim to treat to improve outcomes, needs to be explored.

Analysis on clinicopathology and prognosis of primary IgA nephropathy in children with massive proteinuria / 中华肾脏病杂志

Objective:To investigate the clinicopathological features and the prognosis of IgA nephropathy (IgAN) in children with massive proteinuria.Methods:It was a retrospective cohort study. Clinical data of IgAN children with massive proteinuria admitted to the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2008 to December 2021 were retrospectively analyzed. Patients were divided into effective group and ineffective group according to whether urine protein turned negative after 6 months of initial treatment. The follow-up endpoint event was defined as a reduction in proteinuria of less than 50% or end-stage renal disease (ESRD) achievement. MedCalc software was used to perform Kaplan-Meier survival analysis, and Log-rank test was used to compare the difference of renal survival between the two groups.Results:A total of 127 patients were diagnosed as primary IgAN by renal biopsy, of whom 57 patients with IgAN showed massive proteinuria. These 57 IgAN patients with macroproteinuria accounted for 44.9% of the total IgAN patients and were enrolled in the study. Among the 57 cases, 33 cases (57.9%) were Lee's grade Ⅲ, 11 cases (19.3%) were below Lee's grade Ⅲ, and 13 cases (22.8%) were above Lee's grade Ⅲ. The follow-up time was 4.0 (3.0,5.8) years. In the initial treatment, among 57 patients, 46 (80.7%) were effective (effective group) and 11 (19.3%) were ineffective (ineffective group). Compared with the effective group, the ineffective group had a higher proportion of concurrent AKI at the onset of disease and longer recovery time of renal function, with significant difference (7/11 vs. 13/46, χ2=4.878, P=0.027). Compared with the effective group, the proportion of Lee grade Ⅲ or above was higher in the ineffective group, and the difference was statistically significant (5/11 vs. 8/46, χ2=3.971, P=0.046). There were significant differences in endocapillary hypercellularity (E1), segmental glomerulosclerosis or adhesion (S1) and cellular/fibrocellular crescents (C2) of Oxford classification between IgAN children with Lee grade Ⅲ or below and those over Lee grade Ⅲ (11/13 vs. 20/44, χ2=6.204, P=0.013; 12/13 vs. 17/44, χ2=11.566, P=0.001; 9/13 vs. 7/44, χ2=14.131, P=0.001). Among 57 patients, endpoint events occurred in 2 patients who both were urinary protein unmitigated, and none of the children progressed to ESRD. There was no significant difference in cumulative renal survival between the two groups by Kaplan-Meier survival analysis and Log-rank test ( χ2=0.537, P=0.460) after addition of calcineurin inhibitors (CNIs) to the initial treatment ineffective group. Conclusions:Macroproteinuria is the prominent manifestation of IgAN in children. The pathological type is mainly Lee grade Ⅲ. Children with macroproteinuria have a good prognosis in the short and medium term after active treatment. For IgAN with macroproteinuria that does not respond well to initial treatment, AKI is more common at onset, and renal function recovery time is longer. The application of CNIs may have a certain effect on improving the renal outcome of IgAN with massive proteinuria.

A Case of Dent Disease in Children Presenting with Massive Proteinuria / 罕见病研究

This article reported the diagnosis and treatment of a boy with Dent disease presenting with massive proteinuria.He was 3 years old and found to have massive proteinuria during routine physical examination without hypoalbuminemia, urine protein electrophoresis indicated mainly low molecular weight proteins, with hypercalciuria, and metabolic acidosis, no diabetes, no amino acid urine, and renal ultrasound showed no renal calcium deposition, He had no mental and physical developmental delay and no abnormal family history. Gene detection revealed one missense mutation in exon 15 of the OCRL1 gene, c.1477C > T (p.Arg493Trp). After the diagnosis was confirmed, restrictions in dietary intake of calcium, sodium, and oxalate was restricted and oral potassium citrate and hydrochlorothiazide was prescribed. During two months of follow-up, we observed a decrease in urinary calcium levels and normal renal function. This article aims to improve the understanding of this disease among physicians and provide reference for the diagnosis and treatment of this disease through typical case report and review of previous literatures.

A Case of Childhood-Onset Bartter Syndrome Type 1 with Renal Dysfunction / 罕见病研究

Bartter syndrome (BS, OMIM #601678) is a rare inherited salt-losing tubulopathy characterized by hypokalemia metabolic alkalosis with secondary renin-angiotensin-aldosterone system activation. As reported, BS type 1 is generally presented prenatal and neonatal period, and symptoms usually appear before and after birth or in infancy, accompanied by severe salt loss, whilst kidney function remains mostly normal. In this study, we report a case of BS type 1 with childhood onset and proteinuria and renal impairment. The child was born preterm due to hyperamniotic fluid, but there were no apparent symptoms after birth until the age of 3 when the child began to present with polydipsia, polyuria and increased nocturnal uria. At the age of 5, she had elevated serum creatinine level and proteinuria. After admission, she was diagnosed with chronic tubulointerstitial disease and stage 2 chronic kidney disease(CKD). According to the chloride clearance test, the abnormal function of medullary thick ascending limb Henle′s loop, was confirmed and BS type 1 was diagnosed by gene sequencing. After active management of complications, kidney function of the child improved. In the long-term follow-up, the urinary protein amount of the child still increased, eGFR slowly decreased, and the child was currently in the CKD2 stage. Children with prenatal BS may not present typical clinical manifestations immediately after birth until the onset of relevant clinical symptoms in childhood. BS type 1 patients may have renal impairment, which needs to be identified in time. Clinical differentiation diagnosis between BS and Gitelman syndrome can be made by chloride clearance tests. Early diagnosis and treatment are critical to improve prognosis.

Research Progress of Monogenic Mutation in the Pathogenesis of Steroid-Resistant Nephrotic Syndrome / 罕见病研究

Steroid-resistant nephrotic syndrome (SRNS) is the second cause of chronic kidney disease in children. The SRNS has high risk of rapid progression to end-stage renal disease. With the advancement of high-throughput sequencing technology, more than 70 monogenic mutation having the Mendelian inheritance patterns are identified to be associated with SRNS. Most of these genes are involved in podocyte function. Accurate diagnosis of monogenic mutation in SRNS patients helps with guiding clinical treatment protocols and genetic counseling, avoiding the excessive use of steroids/immunosuppressive therapy, and opening up possibilities for targeted therapies in SRNS patients. In this article, our research team summarizes and generalizes the molecular mechanisms, genetic testing, and specific treatment for the major types of monogenic mutations associated with SRNS.

A Case of Dent Disease in Children Presenting with Massive Proteinuria / 罕见病研究

This article reported the diagnosis and treatment of a boy with Dent disease presenting with massive proteinuria.He was 3 years old and found to have massive proteinuria during routine physical examination without hypoalbuminemia, urine protein electrophoresis indicated mainly low molecular weight proteins, with hypercalciuria, and metabolic acidosis, no diabetes, no amino acid urine, and renal ultrasound showed no renal calcium deposition, He had no mental and physical developmental delay and no abnormal family history. Gene detection revealed one missense mutation in exon 15 of the OCRL1 gene, c.1477C > T (p.Arg493Trp). After the diagnosis was confirmed, restrictions in dietary intake of calcium, sodium, and oxalate was restricted and oral potassium citrate and hydrochlorothiazide was prescribed. During two months of follow-up, we observed a decrease in urinary calcium levels and normal renal function. This article aims to improve the understanding of this disease among physicians and provide reference for the diagnosis and treatment of this disease through typical case report and review of previous literatures.

A Case of Childhood-Onset Bartter Syndrome Type 1 with Renal Dysfunction / 罕见病研究

Bartter syndrome (BS, OMIM #601678) is a rare inherited salt-losing tubulopathy characterized by hypokalemia metabolic alkalosis with secondary renin-angiotensin-aldosterone system activation. As reported, BS type 1 is generally presented prenatal and neonatal period, and symptoms usually appear before and after birth or in infancy, accompanied by severe salt loss, whilst kidney function remains mostly normal. In this study, we report a case of BS type 1 with childhood onset and proteinuria and renal impairment. The child was born preterm due to hyperamniotic fluid, but there were no apparent symptoms after birth until the age of 3 when the child began to present with polydipsia, polyuria and increased nocturnal uria. At the age of 5, she had elevated serum creatinine level and proteinuria. After admission, she was diagnosed with chronic tubulointerstitial disease and stage 2 chronic kidney disease(CKD). According to the chloride clearance test, the abnormal function of medullary thick ascending limb Henle′s loop, was confirmed and BS type 1 was diagnosed by gene sequencing. After active management of complications, kidney function of the child improved. In the long-term follow-up, the urinary protein amount of the child still increased, eGFR slowly decreased, and the child was currently in the CKD2 stage. Children with prenatal BS may not present typical clinical manifestations immediately after birth until the onset of relevant clinical symptoms in childhood. BS type 1 patients may have renal impairment, which needs to be identified in time. Clinical differentiation diagnosis between BS and Gitelman syndrome can be made by chloride clearance tests. Early diagnosis and treatment are critical to improve prognosis.

Research Progress of Monogenic Mutation in the Pathogenesis of Steroid-Resistant Nephrotic Syndrome / 罕见病研究

Steroid-resistant nephrotic syndrome (SRNS) is the second cause of chronic kidney disease in children. The SRNS has high risk of rapid progression to end-stage renal disease. With the advancement of high-throughput sequencing technology, more than 70 monogenic mutation having the Mendelian inheritance patterns are identified to be associated with SRNS. Most of these genes are involved in podocyte function. Accurate diagnosis of monogenic mutation in SRNS patients helps with guiding clinical treatment protocols and genetic counseling, avoiding the excessive use of steroids/immunosuppressive therapy, and opening up possibilities for targeted therapies in SRNS patients. In this article, our research team summarizes and generalizes the molecular mechanisms, genetic testing, and specific treatment for the major types of monogenic mutations associated with SRNS.

TANG Shui-Fu's Experience in Treating Renal Proteinuria by the Method of Replenishing and Elevating Qi / 广州中医药大学学报

Professor TANG Shui-Fu believes that the development of renal proteinuria in chronic kidney disease is due to the deficiency of spleen and kidney,which leads to the malfunction in lifting lucid yang and lowering turbid yin of the body and the internal retention of turbid-toxin.The early stage of renal proteinuria is mainly induced by qi deficiency of spleen and kidney,and the middle and late stage of renal proteinuria results from the long-lasting spleen and kidney deficiency together with damp-toxin accumulation and is characterized by the mixture of healthy-qi deficiency and pathogenic-qi excess.Aimed at the key pathogenesis of the dysfunction of ascending and descending of qi movement,Professor TANG Shui-Fu treated the renal proteinuria with the principal method of strengthening the spleen and tonifying the kidney,combined with method of eliminating dampness,draining turbidity and dissolving stasis targeted at the pathogenic-qi excess of damp-turbidity and stasis-toxin diffusing in the triple energizer.Meanwhile,the use of the method of replenishing and elevating Qi for regulating the qi movement of the spleen and kidney was stressed.In the early stage of renal proteinuria,the Chinese medicines with the actions of promoting the lifting of lucid yang such as Cimicifugae Rhizoma and Bupleuri Radix were added on the basis of herbs for strengthening the spleen and benefiting the kidney,so that the essence can be consolidated and proteinuria can be eliminated.In the middle and late stage of renal proteinuria,the course of the disease lasted a long time,and the internal attack of the pathogens caused the reversal and chaos of the lucid yang and turbid yin.In this case,treatment should follow the principal method of lowering turbid yin and by the assistance of the herbs for replenishing and elevating qi such as Astragali Radix,Atractylodis Macrocephalae Rhizoma,Poria,Dioscoreae Rhizoma,Cimicifugae Rhizoma,and Bupleuri Radix,so that the ascending and descending of qi movement can be coordinated,the lucid yang is raised and turbid yin is directed downward,the renal function is protected and the progression of chronic kidney disease is slowed down.

Research progress of cardiac structure and function in patients with diabetic nephropathy / 中国基层医药

Diabetic nephropathy (DN) is a common microvascular complication of diabetes mellitus. Cardiovascular disease often occurs in patients with DN. Patients with DN often experience changes in cardiac structure and function as proteinuria increases, glomerular filtration rate decreases, and blood creatinine levels increase, leading to the occurrence of cardiovascular disease. Additionally, inflammatory factors play a crucial role in cardiac structure and function. Understanding the pathological and physiological effects of inflammation on diabetic nephropathy-related cardiovascular disease and clarifying the relationship between cardiac structure and function in patients with DN are crucial for effective prevention and treatment of DN.

An unusual coalition of medullary nephrocalcinosis with a novel genotypic variant of Alport syndrome type-1

Alport’s syndrome is a type of inherited disorder of the basement membrane characterized by a spectrum of phenotypes ranging from progressive renal injury to varied extrarenal manifestations comprising auditory and ocular abnormalities. Here in, we present a 3-year-old child born out of nonconsanguineous marriage who presented with fever, intermittent microscopic haematuria, and recurrent gross haematuria, proteinuria with normal auditory brainstem response and ocular slit lamp examination findings. Renal biopsy yielded normal light microscopy and immunofluorescence study whereas minimal changes in the glomerular basement membrane (GBM) collagen were detected on electron microscopy, suggesting possibilities of Alport’s syndrome. Ultrasonographic renal imaging yielded the presence of bilateral medullary nephrocalcinosis. Angiotensin converting enzyme inhibitors along with angiotensin receptor blockers were used to curb the disease progression. A final clinical exome sequencing corroborated the phenotype with a diagnosis of Alport’s syndrome type-1 linked to a novel pathogenic variant c.1892dup (p.Gly632ArgfsTer2) showing hemizygous single base pair insertion/duplication in COL4A5 gene. To the best of our knowledge, this unusual association of Alport’s syndrome with medullary nephrocalcinosis has not been reported worldwide in any previous medical literature making this report a primi one.

Desempeño del analizador de orinas Laura XL-Erba Mannheim en comparación con el iRICELL-Beckman Coulter en una población general / Performance of the Laura XL urine analyzer-Erba Mannheim in comparison with IRISCell-Beckman Coulter in a general population / Desempenho do analisador de urina Laura XL-Erba Mannheim em comparação com iRICELL-Beckman Coulter em uma população geral

Resumen Los objetivos del trabajo fueron evaluar el desempeño del analizador de orinas Laura XL® Erba Mannhein, por comparación con el analizador iRICELL® Beckman Coulter y valorar la determinación de proteínas urinarias semicuantitativas como tamizaje en su estudio, con el establecimiento de un punto de corte para su cuantificación. Se emplearon 225 muestras de orina procesadas en simultáneo. Se evaluó pH, densidad, turbidez, color, hemoglobina, glucosa, cetonas, nitritos, proteínas, número de tiras reactivas positivas; células epiteliales, leucocitos, hematíes y criterio de normalidad del sedimento por microscopía. Se cuantificaron las proteínas urinarias positivas por tiras en un Modular COBAS® 8000 (Hitachi-Roche). Se empleó el coeficiente de concordancia Kappa de Cohen (k) y el índice de correlación de Spearman. Se obtuvo escasa concordancia en turbidez (k=0,334), buena concordancia en color (k=0,681), hemoglobina (k=0,620), glucosa (k=0,677), cetonas (k=0,768), nitritos (k=0,827), tiras reactivas positivas (k=0,620), células epiteliales (k=0,783), leucocitos (k=0,745) y hematíes (k=0,609); muy buena concordancia en proteínas (k=0,842) y criterio de normalidad del sedimento (k=1,000). Correlación estadísticamente significativa en pH (r=0,8064; p<0,0001), densidad (r=1,000; p<0,0001) y proteína urinaria (rs=0,9157; p<0,0001) comparada con COBAS® 8000. Se concluyó un desempeño satisfactorio de Laura XL®; se muestra un rendimiento acorde a las necesidades y normativas de este laboratorio y se avala su utilidad como test de tamizaje para la valoración de proteínas urinarias. Se estableció, además, la cuantificación de orinas que presentaron 1+ o más por tira en Laura XL®. Se consideró realizar mejoras en el software.

Abstract The objectives of this work were to evaluate the performance of the Laura XL® Erba Mannheim urine analyzer, in comparison with the iRICELL® Beckman Coulter analyzer, and to assess the determination of semiquantitative urinary protein as screening in its study, establishing a cut-off point for its quantification. A total of 225 urine samples were simultaneously processed. pH, density, turbidity, colour, hemoglobin, glucose, ketones, nitrites, proteins, and number of positive reactive strips were evaluated; and epithelial cells, leukocytes, red blood cells and criteria for normality of the sediment were evaluated by microscopy. Positive urinary proteins per strip were quantified on a Modular COBAS® 8000 (Hitachi-Roche). Cohen's Kappa concordance coefficient (k) and Spearman's correlation index were used. Little agreement was obtained in turbidity (k=0.334), good agreement in colour (k=0.681), hemoglobin (k=0.620), glucose (k=0.677), ketones (k=0.768), nitrites (k=0.827), positive test strips (k=0.620), epithelial cells (k=0.783), leukocytes (k=0.745) and red blood cells (k=0.609); very good agreement for proteins (k=0.842) and sediment normality criteria (k=1,000). Statistically significant correlation in pH (r=0.8064; p<0.0001), density (r=1.000; p<0.0001), urinary protein (rs=0.9157; p<0.0001) compared with COBAS® 8000. A satisfactory performance of Laura XL® was concluded, showing a performance consistent with the needs and regulations of this institution, and its usefulness is endorsed as a screening test for the assessment of urinary proteins, establishing the quantification of urines that present 1+ or more per strip in Laura XL®. Software improvements are considered.

Resumo Os objetivos do trabalho foram avaliar o desempenho do analisador de urina Laura XL® Erba Mannheim, em comparação com o analisador iRICELL® Beckman Coulter, e avaliar a determinação de proteínas urinárias semiquantitativa como triagem em seu estudo, estabelecendo um ponto de corte para sua quantificação. Foram utilizadas 225 amostras de urina processadas simultaneamente. Foram avaliados pH, densidade, turbidez, cor, hemoglobina, glicose, cetonas, nitritos, proteínas, número de testes reativos positivos; células epiteliais, leucócitos, hemácias e critérios de normalidade do sedimento à microscopia. As proteínas urinárias positivas por teste foram quantificadas em um Modular COBAS® 8000 (Hitachi-Roche). Foram utilizados o coeficiente de concordância Kappa de Cohen (k) e o índice de correlação de Spearman. Escassa concordância foi obtida em turbidez (k=0,334), boa concordância em cor (k=0,681), hemoglobina (k=0,620), glicose (k=0,677), cetonas (k=0,768), nitritos (k=0,827), testes reativos positivos (k=0,620), células epiteliais (k=0,783), leucócitos (k=0,745) e hemácias (k=0,609); concordância muito boa para proteínas (k=0,842) e critérios de normalidade do sedimento (k=1,000). Correlação estatisticamente significativa em pH (r=0,8064 p<0,0001), densidade (r=1,000 p<0,0001), proteína urinária (rs=0,9157 p<0,0001) em comparação com COBAS® 8000. A conclusão é um desempenho satisfatório de Laura XL®, mostrando um rendimento consistente com as necessidades e normas deste laboratorio e sua utilidade é endossada como teste de triagem para avaliação de proteínas urinárias, estabelecendo também a quantificação de urinas que apresentaram 1+ ou mais por teste em Laura XL®. Melhorias no software são consideradas.

A successful outcome of pregnancy with Berger disease

With the increasing prevalence of chronic kidney disease (CKD) in the general population, female patients of fertile age with impaired kidney function are becoming more common. The presence of CKD in pregnant patients has been associated with poorer pregnancy outcomes. IgA nephropathy is the most common glomerulonephritis worldwide. The outcome of pregnancy in patients with CKD is related to impaired glomerular filtration rate and the degree of proteinuria. In non-aggressive IgA nephropathy, there is traditionally a slow progression to chronic kidney failure in 25�% of cases during a period of 20 years. Women with immunoglobulin g A nephropathy (IgAN) are at higher risk of hypertension, preeclampsia, and fetal loss; the prognosis is worse for those who have advanced chronic kidney disease and proteinuria. Here we present two case reports who successfully delivered having aggressive IgA nephropathy and chronic hypertension in pregnancy.

Imerslund-Gräsbeck Syndrome: Clinical Case

Introduction: Imerslund-Gräsbeck syndrome (IGS) is a rare congenital disorder characterized by decreased vitamin B12, megaloblastic anemia, and proteinuria. Clinical case: A 58-year-old woman with four episodes of generalized tonic movements whose paraclinical findings showed cyanocobalamin deficiency. The presence of gait disturbances and constitutional syndrome was reported upon questioning, which required further investigation. The extension tests confirmed type 1 IGS, so it was decided to continue the cyanocobalamin management and nutrition evaluation, with which an adequate evolution was achieved. The patient was eventually discharged. Conclusion: This pathology is low prevalence and mainly affects the first decade of life. It prefers the female sex and is characterized by a decrease in vitamin B12, which can predispose to other disorders such as ataxia and growth retardation.

Introducción: el síndrome de Imerslund-Gräsbeck es un trastorno congénito infrecuente caracterizado por disminución de la vitamina B12, anemia megaloblástica y proteinuria. Caso clínico: mujer de 58 años de edad con cuatro episodios de movimientos tónicos generalizados cuyos paraclínicos mostraban deficiencia de cianocobalamina, por lo que en el interrogatorio se reportaba la presencia de alteraciones en la marcha y síndrome constitucional que requería ampliar los estudios. Los exámenes de extensión confirmaron el síndrome de Imerslund-Gräsbeck tipo 1, de modo que se decidió continuar el manejo con cianocobalamina y valoración con nutrición, con lo que se obtuvo una adecuada evolución y se decidió dar egreso a la paciente. Conclusión: esta patología tiene una baja prevalencia y afecta principalmente a la primera década de la vida, tiene predilección por el sexo femenino y se caracteriza por una disminución de la vitamina B12, que puede predisponer a otras alteraciones como ataxia y retraso en el crecimiento.