Maria Fernanda Soares

Intradermal spindle cell/pleomorphic lipoma of the vulva: case report and review of the literature

Background: Spindle cell/pleomorphic lipoma (SC/PL) is a benign adipose tissue tumor that usually affects the subcutaneous tissues of shoulders, backs, and neck region of middle‐aged male patients. Histologically, it is characterized by the presence of primitive CD34‐positive spindle cells arranged in short fascicles, bizarre floret‐like multinucleated giant cells, mature adipocytes, and a small number of lipoblasts. Recently, an intradermal subset has been described, which mainly affects female patients and presents a wider antomical distribution when compared to the classical variant of SC/PL.

Podocyte Wnt/ß-catenin pathway is activated by integrin-linked kinase in clinical and experimental focal segmental glomerulosclerosis.

BACKGROUND Changes in podocyte phenotype and function are characteristic of proteinuric glomerular diseases. Integrin-linked kinase (ILK) functions as a common downstream effector in proteinuric diseases. In addition, ILK was shown to interact with the Wnt signaling pathway. Here, we investigated ILK expression as well as its involvement with the Wnt signaling pathway in renal biopsies of patients with primary focal segmental glomerulosclerosis (FSGS), and in a correspondent in vivo model of podocyte lesion. METHODS Biopsies from 37 patients with primary FSGS were evaluated by immunohistochemistry for ILK, phosphorylated GSK-3ß (pGSK-3ß) and ß-catenin expression. As experimental model, male Wistar rats received 5 injections of puromycin aminonucleoside (PAN) at 2-week intervals, and their kidneys were evaluated for ILK, P-cadherin and pAkt expression as well as ß-catenin and LEF-1 colocalization. RESULTS Patients presented de novo ILK expression and pGSK-3ß in podocytes. In animals, there was an increase in gene and protein expression of ILK, mainly detected in the podocytes, as well as increased protein expression of pAkt compared with controls. ß-Catenin translocated to the nuclei of podocytes in animals and patients. ß-Catenin colocalized with LEF-1 in the nuclei of podocytes of animals. Gene expression of ß-catenin and P-cadherin in PAN rats was lower compared with controls. CONCLUSIONS Our findings suggest that activation of ILK activated the Wnt signaling pathway in damaged podocytes. This phenomenon could have an important role in development and/or progression of clinical and experimental FSGS.

Electroacupuncture and Moxibustion Decrease Renal Sympathetic Nerve Activity and Retard Progression of Renal Disease in Rats

Background/Aim: Chronic kidney disease (CKD) is an increasing major public health problem worldwide. The sympathetic nervous system and nitric oxide play an important role in the pathogenesis of CKD. Traditional Chinese medicine has accumulated thousands of years of therapeutic experiences. Electroacupuncture (EA) and moxibustion (MO) are two such therapeutic strategies. The aim of this study was to investigate the renal and hemodynamic effects of EA-MO in an experimental model of a CKD. Methods: Male Wistar rats submitted to 5/6th nephrectomy (5/6 NX) were studied for 8 weeks. There were four groups: (1) control, normal rats; (2) NX, 5/6 NX only; (3) NX-AS, 5/6 NX and EA-MO session using sham points, and (4) NX-AM, 5/6 NX and EA-MO session using real acupoints. Biochemical and blood pressure studies, renal sympathetic nerve activity measurements, nitric oxide levels and the histopathological indices were assessed. Results: The EA- and MO-treated group presented significant improvement in all measured functional and histopathological parameters. Conclusion: These findings suggest that EA-MO had beneficial effects on CKD. This effect was probably achieved by the modulation of the renal sympathetic nerve activity and nitric oxide levels, leading to decreased blood pressure, which is associated with less proteinuria.

Electroacupuncture and Moxibustion Attenuate the Progression of Renal Disease in 5/6 Nephrectomized Rats

Background/Aim: Chronic kidney disease is a worldwide public health problem and the prevention of its progression is still a major challenge in nephrology. Specific therapies that inhibit or attenuate this process are neither available nor satisfactory. Traditional Chinese medicine has been increasingly recognized as an effective therapeutic approach in several fields of medicine. The aim of this study was to investigate the effects of electroacupuncture (EA) and moxibustion (MO) in an experimental model of progressive renal disease in rats. Methods: Twenty-one male Wistar rats were submitted to 5/6th nephrectomy (NX) and assessed 8 weeks later and were divided into three groups: NX = only 5/6 NX, NX-AS = 5/6 NX and a 20-min EA-MO session in sham points, and NX-AM = 5/6 NX and a 20-min EA-MO session in three real acupuncture points. The treatment consisted of 16 sessions twice a week. Renal function, urine volume, serum creatinine, 24-hour proteinuria, direct and indirect blood pressure, glomerulosclerosis and tubulointerstitial fibrosis indices were assessed. Results: The NX-AM group showed a significant decrease in all investigated parameters when compared to the control groups. Conclusion: Our results suggest that EA and MO attenuated the progression of renal disease in the experimental model of 5/6 NX.

Post-transplant soluble CD30 levels are associated with early subclinical rejection in kidney transplantation.

Several studies have shown association of high pre- or post-transplant levels of soluble CD30 (sCD30) with acute rejection and poor late kidney transplant outcome. Our goal was to investigate whether sCD30 levels at month-3 post-transplant are associated with subclinical rejection, presence of CD30(+) cells within the graft, and expression of immune response genes in peripheral blood mononuclear cells. The study comprised 118 adult first kidney graft recipients, transplanted at a single center, receiving tacrolimus in low concentration. All were submitted to a protocol biopsy at month-3. Subclinical rejection was identified in 10 biopsies and sCD30 levels ≥ 61.88 ng/mL (P = 0.004), younger recipient age (P = 0.030) and non-Caucasian ethnicity (P = 0.011) were independently associated with this outcome. Rare CD30(+) cells were present in only two biopsies. There was a correlation between sCD30 levels and CD30 gene expression in peripheral blood mononuclear cells (r = 0.385, P = 0.043). These results show that high sCD30 levels are independent predictors of graft dysfunction and may contribute to patient selection protocols by indicating those who could benefit from a more thorough evaluation.

Acute kidney injury in patients infected by H1N1 - clinical histological correlation in a series of cases

INTRODUCTION Influenza A (H1N1) virus was first reported on April 2009 and, since then, several studies have reported the characteristics concerning the clinical presentation and pulmonary involvement. However, accurate information about the acute kidney injury (AKI) and kidney histopathological findings in these patients remain scarce. OBJECTIVE To describe the kidney histopathological findings of 6 patients with H1N1 who developed AKI and underwent kidney biopsy, correlating them with clinical features. METHODS We studied six patients admitted to Hospital de Clínicas UFPR with a PCR-confirmed diagnosis of H1N1who developed ARF and underwent kidney biopsy. We reviewed their medical file and the microscopy findings of the biopsy. RESULTS Clinical and/or laboratory evidence of AKI was present in all cases, and only one did not present oliguria. Kidney tissues revealed glomerular lesions in two patients: one patient, with systemic lupus erythematosus, showed changes consistent with lupus nephritis class III A-C according to the ISN/RPS 2003 and focal thrombotic microangiopathy; the other one had intercapillary nodular glomerulosclerosis, but without clinical or laboratory evidence of diabetes. Vacuolar degenerative tubular changes were present in all cases, with focus of oxalosis in two cases. Mild to moderate atherosclerosis was found in two patients. CONCLUSION In this study, varying degrees of vacuolar degenerative tubular changes were present in all patients, but there were no signs of acute tubular necrosis. It seems that in the present study a prerenal cause of acute renal failure was the main involved mechanim to explain the cause of renal failure in these patients.

Early presentation of squamous cell carcinoma after bone marrow transplantation in a boy with Fanconi anemia

Os autores relatam o caso de um menino de 11 anos de idade com anemia de Fanconi que foi submetido a transplante de medula ossea. Com 763 dias de evolucao, foi detectada uma lesao ulcerada na lingua, compativel com carcinoma de celulas escamosas. Poucos casos semelhantes foram reportados na literatura, geralmente envolvendo as membranas mucosas em pacientes adultos. Os fatores relacionados ao desenvolvimento de neoplasias secundarias em transplante de medula ossea sao discutidos e a idade precoce de apresentacao do paciente enfatiza a necessidade de um alto indice de suspeicao, mesmo em pacientes muito jovens, para que se faca o diagnostico da lesao em fase inicial.

An update on pathology of IgA nephropathy

IgA Nephropathy (IgAN) is the commonest of the glomerular diseases in the world. Its progression rate of 30-40% of the cases em 20-30 years makes IgAN an important healthcare issue in Nephrology. Diagnosis of IgAN depends on biopsy findings, particularly at immunofluorescence microscopy. The frequence of IgAN diagnosis is variable in different populations and depends on screening and biopsy indication policies. IgAN pathogenesis is considered multifactorial; its primordial defect is the production of galactosis-deficient IgA molecules. This review paper discusses the most uptodate aspects of the pathogenesis, pathological classification and clinical implications of IgAN.

Influence of immunosuppressive drugs on the CD30 molecule in kidney transplanted patients

BACKGROUND Soluble CD30 (sCD30) is a suggested marker for kidney transplantation outcomes. We investigated whether sCD30 serum levels are influenced by immunosuppression and whether they correlate with findings in protocol biopsies and with CD30 gene expression in peripheral blood mononuclear cells (PBMC). METHODS We studied 118 kidney transplant recipients that initially received tacrolimus (TAC) and, at month-3, were converted or not to sirolimus (SRL). RESULTS sCD30 serum levels gradually declined after transplantation, being the decline more pronounced in the SRL group. CD30 gene expression in PBMC was higher in the SRL group than in the TAC group. Patients with IF/TA ≥ I in the month-24 protocol biopsy had higher sCD30 levels than patients without IF/TA, in the SRL group (P = .03) and in the TAC group (P = .07). CD30+ cells were observed in three out of 10 biopsies with inflammatory infiltrate from the SRL group. In mixed lymphocyte cultures, SRL and TAC diminished the number of CD30+ T cells and the sCD30 levels in the supernatant, but the effect of SRL was stronger. CONCLUSIONS Overall, sCD30 levels are lower in SRL-treated patients, but the association between increased sCD30 levels and IF/TA at month-24 post-transplantation is stronger in SRL than in TAC-treated patients.

A case of renal recovery in atypical hemolytic uremic syndrome treated with eculizumab

Atypical Hemolytic Uremic Syndrome (aHUS) is a rare, life-threatening disease that can occur at any age and be sporadic or familial. aHUS is caused by an uncontrolled activation of the complement system. Plasma Exchange (PE) has been the standard treatment for years with poor results, leading approximately 40% of patients to end-stage renal disease (ESRD) or death during the first clinical manifestation. Eculizumab, an humanized monoclonal antibody directed against complement component C5, has emerged in the last few years as a new therapheutic aproach with promising results. Our goal is to present a case of an adult patient where eculizumab was sucessfully used as upfront therapy avoiding the potential significant morbidity of PE.