Síndrome nefrótico como manifestación de microangiopatía trombótica secundaria al uso crónico de sunitinib / Nephrotic syndrome as a manifestation of thrombotic microangiopathy due to long-term use of sunitinib

El síndrome nefrótico en los pacientes con cáncer se puede asociar a su enfermedad de base o al tratamiento quimioterapéutico. El cáncer de órganos sólidos puede producir una glomerulonefritis membranosa que se manifiesta con síndrome nefrótico; otras presentaciones histológicas menos frecuentes son la glomeruloesclerosis focal y segmentaria y la enfermedad de cambios mínimos. Adicionalmente, los tratamientos quimioterapéuticos pueden causar toxicidad renal por afección de los pequeños vasos sanguíneos, los glomérulos, los túbulos y el intersticio. Los inhibidores de la tirosina quinasa como el sunitinib pueden causar daño endotelial y podocitario, produciendo una microangiopatía trombótica limitada a los riñones, que se manifiesta con proteinuria e hipertensión. Se presenta el caso de un hombre anciano con tumor del estroma gastrointestinal (GIST, por sus siglas en inglés) que fue tratado con sunitinib y como complicación presentó una microangiopatía trombótica manifestada con síndrome nefrótico e hipertensión de difícil control, que se controló al suspender este medicamento pero con desenlace fatal por su neoplasia maligna. (AU)

Nephrotic syndrome in patients with cancer may be related to the primary malignancy or chemotherapeutic therapy. Solid organ cancers may cause membranous glomerulonephritis manifesting with nephrotic syndrome; other less common histologic presentations include focal and segmental glomerulosclerosis and minimal change disease. In addition, chemotherapy agents can cause renal toxicity by affecting the small blood vessels, glomeruli, tubules, and interstitium. Tyrosine kinase inhibitors such as sunitinib may cause endothelial and podocyte damage leading to renal limited thrombotic microangiopathy, manifested by proteinuria and hypertension. We report a case of an elderly man with gastrointestinal stromal tumor (GIST) on treatment with sunitinib who had as a complication of a thrombotic microangiopathy manifested with nephrotic syndrome and difficult-to-control hypertension, which was controlled by stopping this drug but with a fatal outcome due to its malignant neoplasm. (AU)

Ziehl-Neelsen stain in the pathology laboaratory: Performance and diagnostic aid for mycobacteria in bronchoalveolar lavage / Coloración de Ziehl-Neelsen en el laboratorio de patología: rendimiento y contribución al diagnóstico de micobacterias en el lavado broncoalveolar.

Introduction: With more than 100 years of use, the Ziehl-Neelsen stain is still currently used worldwide. Objective: To compare the performance of diagnostic tests used to determine mycobacteria in the clinic and pathology laboratory in bronchoalveolar lavage samples. Materials and methods: We retrospectively reviewed 737 bronchoalveolar lavage samples from 2019 to 2020 in the San Vicente Fundación hospital (Medellín, Colombia) comparing the performance of three tests done in parallel: mycobacteria and resistance PCR, culture, and Ziehl-Neelsen stain. Results: In total, 93/737 patients were classified as sick due to a positive result in any of the three tests. The culture, PCR, and Ziehl-Neelsen stain had a sensibility of 0.80, 0.76 y 0.51, respectively. However, only 5/75 (6.5%) of the positive cultures had results within the first four weeks and the rest in eight weeks. The PCR test combined with the Ziehl-Neelsen stain improved the sensibility of the PCR test alone from 0.76 a 0.88, a change that was statistically significant (p = 0.022). Conclusion: At least in bronchoalveolar lavage samples, culture is still the test with better sensibility. The use in parallel of the PCR test and the Ziehl-Neelsen stain improved in a statistically significant manner the performance of the PCR test alone, regardless of the higher turnaround time of the Ziehl-Neelsen stain.

Introducción. La coloración de Ziehl-Neelsen, con más de 100 años de uso, continúa vigente mundialmente. Objetivo. Comparar el rendimiento de las pruebas diagnósticas utilizadas para la determinación de micobacterias en el laboratorio clínico de patología en muestras de lavado broncoalveolar. Materiales y métodos. Se revisaron retrospectivamente 737 muestras de lavado broncoalveolar procesadas en el 2019 y el 2020 en el Hospital San Vicente Fundación (Medellín, Colombia) y se compararon las características de tres pruebas diagnósticas realizadas en paralelo: la reacción en cadena de la polimerasa (PCR) para micobacterias con detección de resistencia, el cultivo, y la coloración de Ziehl-Neelsen. Resultados. Se catalogaron como enfermos a 93 de los 737 pacientes a partir de los resultados positivos en alguna de las tres pruebas. El cultivo tuvo una sensibilidad de 0,80, la PCR una de 0,76 y la coloración de Ziehl-Neelsen una de 0,51. Sin embargo, solo 5 de 75 (6,5 %) cultivos fueron positivos a las cuatro semanas y el resto lo fue a las ocho semanas. La PCR combinada con la coloración de Ziehl-Neelsen mejoró la sensibilidad de la PCR por sí sola, de 0,76 a 0,88, diferencia que fue estadísticamente significativa (p=0,022). Conclusión. En las muestras de lavado broncoalveolar, el cultivo sigue siendo la prueba con mejor sensibilidad. El uso conjunto de la prueba de PCR y la coloración de Ziehl-Neelsen mejora significativamente la sensibilidad de la primera, lo que compensa la demora relativa en la entrega de los resultados debida al tiempo requerido para la tinción de Ziehl-Neelsen.

Coloración de Ziehl-Neelsen en el laboratorio de patología: rendimiento y contribución al diagnóstico de micobacterias en el lavado broncoalveolar / Ziehl-Neelsen stain in the pathology laboaratory: Performance and diagnostic aid for mycobacteria in bronchoalveolar lavage

Introducción. La coloración de Ziehl-Neelsen, con más de 100 años de uso, continúa vigente mundialmente. Objetivo. Comparar el rendimiento de las pruebas diagnósticas utilizadas para la determinación de micobacterias en el laboratorio clínico de patología en muestras de lavado broncoalveolar. Materiales y métodos. Se revisaron retrospectivamente 737 muestras de lavado broncoalveolar procesadas en el 2019 y el 2020 en el Hospital San Vicente Fundación (Medellín, Colombia) y se compararon las características de tres pruebas diagnósticas realizadas en paralelo: la reacción en cadena de la polimerasa (PCR) para micobacterias con detección de resistencia, el cultivo, y la coloración de Ziehl-Neelsen. Resultados. Se catalogaron como enfermos a 93 de los 737 pacientes a partir de los resultados positivos en alguna de las tres pruebas. El cultivo tuvo una sensibilidad de 0,80, la PCR una de 0,76 y la coloración de Ziehl-Neelsen una de 0,51. Sin embargo, solo 5 de 75 (6,5 %) cultivos fueron positivos a las cuatro semanas y el resto lo fue a las ocho semanas. La PCR combinada con la coloración de Ziehl-Neelsen mejoró la sensibilidad de la PCR por sí sola, de 0,76 a 0,88, diferencia que fue estadísticamente signifcativa (p=0,022). Conclusión. En las muestras de lavado broncoalveolar, el cultivo sigue siendo la prueba con mejor sensibilidad. El uso conjunto de la prueba de PCR y la coloración de ZiehlNeelsen mejora signifcativamente la sensibilidad de la primera, lo que compensa la demora relativa en la entrega de los resultados debida al tiempo requerido para la tinción de Ziehl-Neelsen.

Introduction: With more than 100 years of use, the Ziehl-Neelsen stain is still currently used worldwide. Objective: To compare the performance of diagnostic tests used to determine mycobacteria in the clinic and pathology laboratory in bronchoalveolar lavage samples. Materials and methods: We retrospectively reviewed 737 bronchoalveolar lavage samples from 2019 to 2020 in the San Vicente Fundación hospital (Medellín, Colombia) comparing the performance of three tests done in parallel: mycobacteria and resistance PCR, culture, and Ziehl-Neelsen stain. Results: In total, 93/737 patients were classifed as sick due to a positive result in any of the three tests. The culture, PCR, and Ziehl-Neelsen stain had a sensibility of 0.80, 0.76 y 0.51, respectively. However, only 5/75 (6.5%) of the positive cultures had results within the frst four weeks and the rest in eight weeks. The PCR test combined with the Ziehl-Neelsen stain improved the sensibility of the PCR test alone from 0.76 a 0.88, a change that was statistically signifcant (p = 0.022). Conclusion: At least in bronchoalveolar lavage samples, culture is still the test with better sensibility. The use in parallel of the PCR test and the Ziehl-Neelsen stain improved in a statistically signifcant manner the performance of the PCR test alone, regardless of the higher turnaround time of the Ziehl-Neelsen stain.

Giant Parathyroid Adenoma-Associated Fracture, Not All Lytic Bone Lesions are Cancer: A Case-Based Review.

INTRODUCTION: Due to the early diagnosis of primary hyperparathyroidism the musculoskeletal manifestations of this disease are becoming less frequent. When this disease manifests secondary to a giant adenoma, it presents with more aggressive symptoms and can have important repercussions such as the hungry bone syndrome after parathyroidectomy. There are few reported cases of hyperparathyroidism secondary to a giant adenoma in the literature, as the presence of a brown tumor is often misinterpreted as a metastatic lesion from an unknown primary tumor. METHODS: We describe a case and performed a literature review to identify all case reports. A literature search was carried out on PubMed/MEDLINE and EMBASE bibliographic databases. All available studies from May 2009 to May 2021 were included. Data were tabulated, and outcomes were cumulatively analyzed. RESULTS: Twenty-four cases of primary hyperparathyroidism due to giant adenoma have been described; the majority were women, with a mean age of 52 years. They presented with heterogeneous symptoms such as palpable nodules (45%), bone pain (33%), brown tumor (12.5%), asymptomatic (12.5%), metabolic profile with a mean calcemia of 13.8 mg/dL, PTH 1109 ng/L, and mean tumor weight of 47.24 g. CONCLUSION: Primary hyperparathyroidism due to giant adenoma increases the risk of developing potentially serious postoperative complications such as hungry bone syndrome. This implies the need of implementing preventive measures comprising administration of intravenous zoledronic acid and early supplementation of oral calcium to prevent complications after resection.

Nephrotic syndrome as a manifestation of thrombotic microangiopathy due to long-term use of sunitinib.

Nephrotic syndrome in patients with cancer may be related to the primary malignancy or chemotherapeutic therapy. Solid organ cancers may cause membranous glomerulonephritis which is manifested by nephrotic syndrome; other less common histologic presentations include focal and segmental glomerulosclerosis and minimal change disease. In addition, chemotherapy agents may cause renal toxicity by affecting the small blood vessels, glomeruli, tubules, and interstitium. Tyrosine kinase inhibitors such as sunitinib may cause endothelial and podocyte damage leading to thrombotic microangiopathy affecting only the kidney and manifested by proteinuria and hypertension. We report a case of an elderly man with gastrointestinal stromal tumor (GIST) on treatment with sunitinib who had as a complication a thrombotic microangiopathy manifested with nephrotic syndrome and a hypertension of difficult control, which was finally controlled by stopping this drug but had a fatal outcome due to its malignancy.

Nephrotic syndrome as a manifestation of thrombotic microangiopathy due to long-term use of sunitinib. / Síndrome nefrótico como manifestación de microangiopatía trombótica secundaria al uso crónico de sunitinib.

Nephrotic syndrome in patients with cancer may be related to the primary malignancy or chemotherapeutic therapy. Solid organ cancers may cause membranous glomerulonephritis manifesting with nephrotic syndrome; other less common histologic presentations include focal and segmental glomerulosclerosis and minimal change disease. In addition, chemotherapy agents can cause renal toxicity by affecting the small blood vessels, glomeruli, tubules, and interstitium. Tyrosine kinase inhibitors such as sunitinib may cause endothelial and podocyte damage leading to renal limited thrombotic microangiopathy, manifested by proteinuria and hypertension. We report a case of an elderly man with gastrointestinal stromal tumor (GIST) on treatment with sunitinib who had as a complication of a thrombotic microangiopathy manifested with nephrotic syndrome and difficult-to-control hypertension, which was controlled by stopping this drug but with a fatal outcome due to its malignant neoplasm.

Urinary levels of ceruloplasmin and monocyte chemoattractant protein-1 correlate with extra-capillary proliferation and chronic damage in patients with lupus nephritis.

BACKGROUND AND AIM: There are few studies of urinary biomarkers and histopathologic features in lupus nephritis (LN). The aim was to analyze the correlation between a wide panel of urinary biomarkers and serum concentrations of anti C1q antibodies with histological items of activity and chronicity on kidney biopsy in LN patients. METHODS: Patients with systemic lupus erythematosus (SLE) according to American College of Rheumatology (ACR) criteria were included. LN diagnosis was based on ACR criteria. Histologic features of activity and chronicity indices were analyzed according to the Austin classification. Serum Anti C1q levels were determined by commercial ELISA. Urinary levels of transferrin, ceruloplasmin (CP), VCAM-1, TWEAK, monocyte chemoattractant protein-1 (MCP-1), neutrophil gelatinase-associated lipocalin (NGAL), and alpha-1-acid glycoprotein were measured by commercial ELISA. RESULTS: We included 120 SLE patients (81% female, mean age 33.1 ± 9.3 years, 59.4% Mestizo, 37.8% Afro-Latin American): 64% had LN. Kidney biopsy was performed in 55 patients, but only 37 were made in our center. Anti C1q antibodies were associated with endocapillary proliferation. In patients with cellular crescents, urinary concentrations of CP were significantly higher. In patients with a chronicity index (CI) ≥ 4, fibrous crescents, tubular atrophy, and interstitial fibrosis, urinary MCP-1 levels were higher. CONCLUSIONS: In SLE patients, serum anti C1q antibodies and urinary CP were associated with activity on kidney biopsy and MCP-1 with chronic damage. This panel of biomarkers could be validated in larger, multi-ethnic population as a complementary tool for better stratification of LN patients. Key Points • Urinary biomarkers are complementary useful tools for the assessment of SLE patients. • Urinary levels of CP correlated with activity findings on kidney biopsy in LN patients. • Urinary levels of MCP-1 correlated with chronic damage, especially with fibrous crescents, tubular atrophy, and interstitial fibrosis.

Glomerulonefritis rápidamente progresiva mediada por inmunocomplejos (tipo II). Revisión de la literatura / Immune complex-mediated rapidly progressive glomerulonephritis (type II). Literature review

La glomerulonefritis rápidamente progresiva mediada por complejos inmunes (GMNRP II) es un síndrome clínico caracterizado por el rápido deterioro de la función renal asociado a hematuria, edemas y oliguria. Histológicamente se manifiesta como una glomerulonefritis crescéntica, con la presencia de depósitos granulares en la inmunofluorescencia. Aunque es una enfermedad rara, es grave y puede evolucionar a una enfermedad renal crónica, por lo cual es fundamental su identificación temprana. A continuación, se presenta una revisión sobre este tipo de glomerulonefritis, con énfasis en su etiología y en las opciones terapéuticas existentes en la actualidad

Rapidly progressive immune complex-mediated glomerulonephritis (RPGNMN II) is a clinical syndrome characterized by severe deterioration of renal function associated with hematuria, edema, and oliguria. It is histologically characterized as a crescentic glomerulonephritis, with the presence of granular deposits on immunofluorescence. Although it is a rare condition, it is a potentially serious disease that may progress to chronic renal disease, therefore its early identification is essential. Here we present a review of this form of glomerulonephritis, with emphasis on its etiology and the currently available therapeutic options

Immunoglobulin A Nephropathy With Extracapillary Proliferation and Positive Anti-Neutrophil Cytoplasmic Antibodies: A Rare Cause of Dual Glomerulonephritis.

The coexistence of immunoglobulin A (IgA) nephropathy and associated pauci-immune anti-neutrophil cytoplasmic antibody (ANCA) glomerulonephritis represents a rare concurrence of two common forms of glomerulonephritis; the pathogenesis, treatment, and prognosis of this dual glomerulopathy are not well described. This illustrative case can present this association in an HIV-positive patient and how despite the coexistence of these two entities, the patient had control of his kidney disease with low doses of steroids, contrary to the different reports of cases in the literature in which the treatment is often more aggressive. In this case report, we review the literature on this dual glomerulonephritis and confront clinical and treatment aspects regarding the different clinical cases reported in the databases.

Adrenal paracoccidioidomycosis / [Paracoccidiodomicosis suprarrenal].

Primary adrenal insufficiency is a defect in glucocorticoid, mineralocorticoid and sexual androgens production. Patients with this disorder have low cortisol levels and aldosterone deficiency with concomitant hyponatremia and hyperkalemia. The most common etiology of this disease is the production of antibodies against the enzyme 21 hydroxylase. Another common cause, particularly in low income countries, are infectious diseases. Several micro-organisms have been reported as a causal agent in adrenal insufficiency including Mycobacterium tuberculosis, Mycobacterium avium complex, Neisseria meningitidis, Pseudomonas aeruginosa, Haemophilus influenzae, cytomegalovirus, Pneumocystis jirovecii, Histoplasma capsulatum, Blastomyces dermatiditis, Cryptococcus neoformans, Cocciodiodes immitis, Nocardia spp. and Paracoccidioides brasiliensis. In this article, we present the computerized tomography and the adrenal biopsy of a patient with adrenal insufficiency. The final diagnosis was paracoccidioidomycosis.

La insuficiencia suprarrenal primaria es un defecto en la producción de glucocorticoides, mineralocorticoides y andrógenos sexuales. Los pacientes afectados por esta condición se caracterizan por concentraciones bajas de cortisol y deficiencia de aldosterona con hiponatremia e hiperpotasemia concomitantes. La etiología más común es el desarrollo de anticuerpos contra la enzima 21 hidroxilasa. Otra causa importante de la insuficiencia suprarrenal primaria son las enfermedades infecciosas, en especial en los países de bajos ingresos. Entre las causas infecciosas que se han descrito se encuentran: Mycobacterium tuberculosis, el complejo de Mycobacterium avium, Neisseria meningitidis, Pseudomonas aeruginosa, Haemophilus influenzae, citomegalovirus, Pneumocystis jirovecii, Histoplasma capsulatum, Blastomyces dermatiditis, Cryptococcus neoformans, Cocciodiodes immitis, Nocardia spp. y Paracoccidioides brasiliensis. En este artículo se presenta la imagen de la tomografía de un paciente que presentó falla suprarrenal, con masas en las glándulas suprarrenales, cuya biopsia permitió establecer el diagnóstico final de paracoccidioidomicosis.

Paracoccidiodomicosis suprarrenal / Adrenal paracoccidioidomycosis

La insuficiencia suprarrenal primaria es un defecto en la producción de glucocorticoides, mineralocorticoides y andrógenos sexuales. Los pacientes afectados por esta condición se caracterizan por concentraciones bajas de cortisol y deficiencia de aldosterona con hiponatremia e hiperpotasemia concomitantes. La etiología más común es el desarrollo de anticuerpos contra la enzima 21 hidroxilasa. Otra causa importante de la insuficiencia suprarrenal primaria son las enfermedades infecciosas, en especial en los países de bajos ingresos. Entre las causas infecciosas que se han descrito se encuentran: Mycobacterium tuberculosis, el complejo de Mycobacterium avium, Neisseria meningitidis, Pseudomonas aeruginosa, Haemophilus influenzae, citomegalovirus, Pneumocystis jirovecii, Histoplasma capsulatum, Blastomyces dermatiditis, Cryptococcus neoformans, Cocciodiodes immitis, Nocardia spp. y Paracoccidioides brasiliensis. En este artículo se presenta la imagen de la tomografía de un paciente que presentó falla suprarrenal, con masas en las glándulas suprarrenales, cuya biopsia permitió establecer el diagnóstico final de paracoccidioidomicosis.

Primary adrenal insufficiency is a defect in glucocorticoid, mineralocorticoid and sexual androgens production. Patients with this disorder have low cortisol levels and aldosterone deficiency with concomitant hyponatremia and hyperkalemia. The most common etiology of this disease is the production of antibodies against the enzyme 21 hydroxylase. Another common cause, particularly in low income countries, are infectious diseases. Several micro-organisms have been reported as a causal agent in adrenal insufficiency including Mycobacterium tuberculosis, Mycobacterium avium complex, Neisseria meningitidis, Pseudomonas aeruginosa, Haemophilus influenzae, cytomegalovirus, Pneumocystis jirovecii, Histoplasma capsulatum, Blastomyces dermatiditis, Cryptococcus neoformans, Cocciodiodes immitis, Nocardia spp. and Paracoccidioides brasiliensis. In this article, we present the computerized tomography and the adrenal biopsy of a patient with adrenal insufficiency. The final diagnosis was paracoccidioidomycosis.

Influence of Aging on Microvascular Supply of the Gluteus Medius Tendon: A Cadaveric and Histologic Study.

PURPOSE: The purpose of this cadaveric study was to assess the relation between age and microvascular supply of 3 areas of the gluteus medius tendon using a previously validated CD31 immunohistochemistry staining technique. METHODS: Twenty-four fresh-frozen gluteus medius specimens were obtained through a posterolateral approach to the hip. Specimens aged 18 years or older, of either sex, and of any race were considered for this study. The average age of donors was 47.3 years (range, 18-68 years). Each sample was divided into 3 portions: musculotendinous, tendinous, and tendon-bone junction. H&E staining was used for qualitative structural analysis, and then all samples underwent staining with CD31 immunohistochemistry for quantitative assessment of vessels per square millimeter. A comparison of the microvessel density between zones according to age was performed by an analysis of variance. To evaluate the relation between microvessel supply and age, a regression model with curvilinear estimation was used. The data were fitted to a quadratic model. RESULTS: Vascular supply in transversal and longitudinal cuts regardless of the zone was, on average, 53.9 ± 32.1 vessels/mm2 and 51.1 ± 19.3 vessels/mm2, respectively. All the areas of the tendon showed a strength of relation (R) ranging from 0.41 to 0.76 between age and vascular supply. In addition, the proportion of vascular supply change explained by age (R2) was significant in most cases (ranging from 0.17 to 0.56, with P < .05). CONCLUSIONS: There is a chronological relation between aging and microvascular supply of the gluteus medius tendon, in which an initial increase occurs from 18 years of age to 30 to 40 years of age, with a progressive decrease after 50 years of age. CLINICAL RELEVANCE: The findings of our study may have implications for increased vulnerability of the gluteus medius tendon and decreased healing potential.

Infiltrating CD16+ Are Associated with a Reduction in Peripheral CD14+CD16++ Monocytes and Severe Forms of Lupus Nephritis.

Our aim was to characterize glomerular monocytes (Mo) infiltration and to correlate them with peripheral circulating Mo subsets and severity of lupus nephritis (LN). Methods. We evaluated 48 LN biopsy samples from a referral hospital. Recognition of Mo cells was done using microscopic view and immunohistochemistry stain with CD14 and CD16. Based on the number of cells, we classified LN samples as low degree of diffuse infiltration (<5 cells) and high degree of diffuse infiltration (≥5 cells). Immunophenotyping of peripheral Mo subsets was done using flow cytometry. Results. Mean age was 34.0 ± 11.7 years and the mean SLEDAI was 17.5 ± 6.9. The most common SLE manifestations were proteinuria (91%) and hypocomplementemia (75%). Severe LN was found in 70% of patients (Class III, 27%; Class IV, 43%). Severe LN patients and patients with higher grade of CD16+ infiltration had lower levels of nonclassical (CD14+CD16++) Mo in peripheral blood. Conclusions. Our results might suggest that those patients with more severe forms of LN had a higher grade of CD14+CD16+ infiltration and lower peripheral levels of nonclassical (CD14+CD16++) Mo and might reflect a recruitment process in renal tissues. However, given the small sample, our results must be interpreted carefully.

The regional microvascular density of the gluteus medius tendon determined by immunohistochemistry with CD31 staining: a cadaveric study.

BACKGROUND AND PURPOSE: There are no studies to date about the vascularisation into the gluteus medius tendon. The purpose of this study was to define the microvessel density of the gluteus medius in 3 zones through a special staining with CD31 and to identify regional differences in microvascular density that may have implications for the healing. METHODS: We obtained 12 complete gluteus medius tendons from cadavers who had been an average age of 30.3 years old (range 18 to 55). All the donors were males with no known history of hip abnormalities.Following a rigorous protocol, each gluteus medius tendon was divided in 3 portions. Each gluteus medius tendon was divided in 3 portions (I: musculotendinous, II: Tendon, III: Tendon-Bone junction). RESULTS: There were regional differences between all anatomic zones in both the transverse section (p<0.001) and the longitudinal section (p = 0.007). Furthermore, a significant difference was found between zones II and I (mean difference -23.45 IC95% -38.77 to -8.13, p<0.001) and between zones II and III (mean difference -26.08 IC95% -41.39 to -10.76, p<0.001) in transverse section. In longitudinal sections, this difference was found as well between zones II and I (mean difference -29.48 IC95% -51.54 to -7.43, p = 0.01), but not between zones II and III (mean difference -10.87 IC95% -32.93 to 11.18, p = 0.67). CONCLUSIONS: The microvessel density was significantly lower in the length of the tendon (central portion) compared to the other 2 regions.

Response to the comment on 'IgM nephropathy in children: clinicopathologic analysis'

No disponible

Nefropatía IgM en niños: análisis clinicopatológico / IgM nephropathy in children: clinicopathologic analysis

INTRODUCCIÓN: La nefropatía IgM (NIgM) es una glomerulonefritis caracterizada por depósitos mesangiales difusos de inmunoglobulina M (IgM), que suele manifestarse con proteinuria en rango nefrótico y que, según algunos trabajos previos, se presenta más frecuentemente en pacientes que son resistentes o dependientes del tratamiento con esteroides. OBJETIVO: Realizar una descripción clínica, histológica e inmunopatológica, y evaluar la respuesta al tratamiento esteroideo de los pacientes pediátricos con diagnóstico de síndrome nefrótico y depósitos mesangiales y difusos de IgM. MÉTODOS: Estudio descriptivo, retrospectivo, realizado en dos centros hospitalarios, donde se analizaron los registros clínicos de pacientes pediátricos con diagnóstico de NIgM y se revaluaron los cortes histológicos. RESULTADOS: Trece niños fueron incluidos en el presente estudio. La NIgM correspondió al 5,17 % de todas las biopsias renales pediátricas. La edad de los pacientes estuvo entre 1 y 12 años (mediana: 2 años); el 46,7 % fueron mujeres. El hallazgo morfológico más frecuente fue hipercelularidad mesangial difusa (46,1 %), seguido de glomeruloesclerosis focal y segmentaria (30,8 %) y cambios glomerulares mínimos (23,1 %). Todos los pacientes recibieron esteroides, en 4 de ellos (30,7 %) como el único medicamento inmunosupresor; 3 (23,1 %) recibieron además ciclofosfamida, 5 (38,4 %) micofenolato y 1 (7,7 %) ciclosporina. Siete pacientes (53,8 %) presentaron recaídas frecuentes, 5 (38,5 %) fueron corticorresistentes y 1 (7,7 %) corticodependiente. Dos pacientes (15,38 %) presentaron alteración crónica de la función renal. CONCLUSIÓN: La presencia de IgM mesangial difusa en pacientes pediátricos con síndrome nefrótico no es un hallazgo muy infrecuente, su presentación clínica se ha asociado con menor respuesta a esteroides. Sin embargo, su pronóstico a largo plazo aún no se conoce

INTRODUCTION: IgM nephropathy (IgMN) is a glomerulonephritis characterized by diffuse mesangial deposits of immunoglobulin M (IgM), which usually presents with nephrotic range proteinuria and, according to some previous studies, occurs most often in patients who are resistant or dependent of steroid treatment. AIM: To perform a clinical, histological and inmunopathological description, and assess response to steroid treatment of pediatric patients diagnosed with nephrotic syndrome and diffuse mesangial deposits of IgM. METHODS: This is a descriptive, retrospective study done in two hospitals. Clinical data were taken from medical records. All the slides with the histologic sections were re-evaluated. RESULTS: thirteen children were included in this study. IgMN corresponded to 5.17% of all pediatric renal biopsies. The age of patients ranged from 1 year to 12 years (median: 2 years), 46,15% were women. The most common morphological finding was diffuse mesangial hypercellularity (46,1%), followed by focal segmental glomerulosclerosis (30,8%) and minimal glomerular changes (23,1%). All patients received steroids, in 4 cases (30,7%) as the only immunosuppressant medication, 3 (23,1%) also received cyclophosphamide, 5 (38,4%) mycophenolate, and 1 (7.7%) cyclosporine. Seven patients (53,8%) had frequent relapses, 5 (38.5%) were cortico-resistant, and 1 (7.7%) corticodependent. Two patients (15,38%) had chronic impairment of renal function. CONCLUSION: The presence of diffuse mesangial IgM in pediatric patients with nephrotic syndrome is not a too uncommon finding, the clinical presentation has been associated with lower response to steroids, but their longterm prognosis is still unknown

Histologic variants of primary focal segmental glomerulosclerosis: presentation and outcome.

INTRODUCTION: The clinical significance of histologic variants of primary focal segmental glomerulosclerosis (FSGS) remains unclear. With the aim to determine presentation and outcome of the variants of FSGS in a hispanic population, we studied our cases of this glomerulopathy. METHODS: In this retrospective study, all renal biopsies with FSGS (1998-2009), were classified according to the Columbia's classification. We analyzed histological, clinical and follow-up data and compared among variants. RESULTS: Among 291 cases, 224 (77.0%) corresponded to NOS variant, 40 cases (13.7%) to tip variant (TIP), 14 cases (4.8%) to perihilar (PH), 10 cases (3.4%) to collapsing (COLL) and three cases (1.0%) to cellular variant (CELL). Median age: 26 years (range 1 to 79); 74 patients (25.4%) were < 15 years of age. Hypertension and renal dysfunction were more frequent in PH and COLL cases. PH presented frequently as nonnephrotic proteinuria. There were fewer histologic chronic lesions in TIP cases. There was remission in 23.5% of patients with NOS, 57.7% of patients with TIP, 22.2% of patients with COLL and 0 patients with PH (p < 0.01). Chronic kidney disease (CKD) was less frequent in TIP than in the other variants (p = 0.03). There were not statistical differences for end-stage renal disease among variants. CONCLUSIONS: Glomerular histological appearance is not a good indicator of outcome. COLL is a disease with many differences to the other variants and bad prognosis; PH is a variant mainly of adults, with frequent evolution to CKD. TIP appears as a less aggressive, although not benign, variant.