The accuracy of 99mTc-DTPA scintigraphy in the evaluation of acute renal graft complications

PURPOSE: Renal scintigraphy has been used for many years in the evaluation of renal transplants and can help in the diagnosis of graft complications, leading to prompt clinical management and preventing further deterioration of renal function. The purpose of this study was to evaluate the overall accuracy of renal scintigraphy with 99mTc-DTPA in the diagnosis of acute renal graft complications. MATERIALS AND METHODS: Seventy-six scintigraphic studies performed in 55 patients (ages ranging from 6 to 65 years), were reviewed. Scintigraphy results were compared to biopsies performed within 5 days of imaging. 99mTc-DTPA study was performed within a mean time of 19 days after kidney transplants. Dynamic images were performed in the anterior position of the abdomen and pelvis every 2 seconds for 80 seconds (flow phase) and every 15 seconds for 30 minutes (functional phase), after an intravenous injection of 370 MBq (10 mCi) of 99mTc-DTPA. RESULTS: The scintigraphic results were concordant with the biopsies in 86% of the cases studied. The sensitivities of renal scintigraphy for detection of acute tubular necrosis (ATN), acute rejection (AR) and cortical necrosis (CN) were 98%, 87% and 100%, respectively. Specificities and accuracies for detection of ATN, AR and CN were 89%, 86% and 100%, and 95%, 87% and 100%, respectively. CONCLUSION: Renal scintigraphy with 99mTc-DTPA showed a good overall accuracy in the detection of acute renal graft complications. It can be used as a reliable tool in the routine evaluation of these patients50751

Downregulation Of 14q32 Micrornas In Primary Human Desmoplastic Medulloblastoma.

Medulloblastoma (MB) is one of the most common pediatric cancers, likely originating from abnormal development of cerebellar progenitor neurons. MicroRNA (miRNA) has been shown to play an important role in the development of the central nervous system. Microarray analysis was used to investigate miRNA expression in desmoplastic MB from patients diagnosed at a young age (1 or 2 years old). Normal fetal or newborn cerebellum was used as control. A total of 84 differentially expressed miRNAs (64 downregulated and 20 upregulated) were found. Most downregulated miRNAs (32/64) were found to belong to the cluster of miRNAs at the 14q32 locus, suggesting that this miRNA locus is regulated as a module in MB. Possible mechanisms of 14q32 miRNAs downregulation were investigated by the analysis of publicly available gene expression data sets. First, expression of estrogen-related receptor-γ (ESRRG), a reported positive transcriptional regulator of some 14q32 miRNAs, was found downregulated in desmoplastic MB. Second, expression of the parentally imprinted gene MEG3 was lower in MB in comparison to normal cerebellum, suggesting a possible epigenetic silencing of the 14q32 locus. miR-129-5p (11p11.2/7q32.1), miR-206 (6p12.2), and miR-323-3p (14q32.2), were chosen for functional studies in DAOY cells. Overexpression of miR-129-5p using mimics decreased DAOY proliferation. No effect was found with miR-206 or miR-323 mimics.325

Clinical manifestations of head and neck cancer in pediatric patients, an analysis of 253 cases in a single Brazilian center

Pediatric head and neck cancer (PHNC) is rare and its nonspecific clinical manifestations may often lead to delayed diagnosis. We aimed to describe the signs, symptoms, and clinicopathological characteristics of PHNC. Medical records were retrospectively reviewed for all PHNC cases diagnosed from 1986 to 2016 affecting patients aged 19-years and younger from a tertiary referral center in Brazil. Demographic variables, anatomical site of primary tumors, histopathological diagnoses, signs and symptoms, and patterns of misdiagnosis were collected and interpreted by statistical and descriptive analysis. A total of 253 PHNC cases were included. The mean age was 9.3 years and male patients were more frequently affected (60.9%). Burkitt lymphoma (23.7%), nasopharyngeal carcinoma (15.8%), and rhabdomyosarcoma (15.4%) were the most common cancer types. The nasopharynx (28.9%), cervical/lymph node region (25.3%), and craniofacial bones (8.3%) were the predominant anatomical sites. Tumor/swelling (68.4%), was the clinical finding often presented. The univariable analysis showed association between tumor histology and clinical variables such as sex (p=0.022), age (p<0.0001), anatomical location (p<0.0001) tumor/swelling (p=0.034), pain (p=0.031), systemic/general manifestations (p=0.004), nasal/breathing alterations (p=0.012), orbital/ocular alterations (p<0.0001). Misdiagnosis such as tonsillitis, otitis, and abscess were frequent. Although the clinical findings of PHNC are often unspecific, this study provided signs and symptoms with significant correlations between tumor histology. The suspicion of malignancy should be considered when the main signs and symptoms reported here appear and persist, in order to conduct a timely diagnosis

The Accuracy Of (99m)tc-dtpa Scintigraphy In The Evaluation Of Acute Renal Graft Complications.

Renal scintigraphy has been used for many years in the evaluation of renal transplants and can help in the diagnosis of graft complications, leading to prompt clinical management and preventing further deterioration of renal function. The purpose of this study was to evaluate the overall accuracy of renal scintigraphy with (99m)Tc-DTPA in the diagnosis of acute renal graft complications. Seventy-six scintigraphic studies performed in 55 patients (ages ranging from 6 to 65 years), were reviewed. Scintigraphy results were compared to biopsies performed within 5 days of imaging. (99m)Tc-DTPA study was performed within a mean time of 19 days after kidney transplants. Dynamic images were performed in the anterior position of the abdomen and pelvis every 2 seconds for 80 seconds (flow phase) and every 15 seconds for 30 minutes (functional phase), after an intravenous injection of 370 MBq (10 mCi) of (99m)Tc-DTPA. The scintigraphic results were concordant with the biopsies in 86% of the cases studied. The sensitivities of renal scintigraphy for detection of acute tubular necrosis (ATN), acute rejection (AR) and cortical necrosis (CN) were 98%, 87% and 100%, respectively. Specificities and accuracies for detection of ATN, AR and CN were 89%, 86% and 100%, and 95%, 87% and 100%, respectively. Renal scintigraphy with (99m)Tc-DTPA showed a good overall accuracy in the detection of acute renal graft complications. It can be used as a reliable tool in the routine evaluation of these patients.29507-1

Classificação de glomerulonefrites : proposta para uma nova abordagem baseada em estudo de 700 biopsias renais em microscopia comum, imunofluorescente e eletronica

Orientador: Athanase BillisTese (doutorado) - Universidade Estadual de Campinas, Faculdade de Ciencias MedicasResumo: Passados 80 anos desde a classificação de VOLHARD & FAlIR (1914), que pôs certa ordem no caos então existente, ainda hoje a classificação de glomerulonerntes (GN) desafia nefrologistas e anatomopatologistas. A última tentativa neste propósito, apresentada pela Organização Mundial de Saúde (OMS), ainda não solucionou satisfatoriamente o problema. Três fatores podem ser citados que dificultam uma classificação adequada: 1) uma determinada lesão anatômica glomerular pode corresponder a várias doenças; 2) uma única doença pode ter várias lesões anatômicas. No 1úpus eritematoso, em particular, há variações de lesões no decorrer da evolução da moléstia; 3) muitas doenças glomerulares recebem o nome da lesão anatômica. O presente trabalho tem por objetivo apresentar uma nova abordagem para a classificação de glomerulonerntes. A proposta é dissociar o diagnóstico anatômico (DA) do diagnóstico nosológico (DN), sendo ambos consignados no laudo anatomopatológico. O diagnóstico anatômico é elaborado através da microscopia óptica comum. A elaboração do dia~óstico nosológico é baseada nos dados clínicos, laboratoriais e de microscopia óptica comum, imunotluorescente e eletrônica. A lesão anatômica mais freqüente, verificada no presente estudo, foi a GN proliferativa mesangial (168/700, 24,0%), que correspondeu a um número grande e variado de moléstias, como: doença de Berger; síndrome de Alport; GN pós-infecciosa; doença de membranas finas; lúpus eritematoso sistêmico (LES); púrpura de Henoch¬Shõnlein. A segunda lesão anatômica mais freqüente correspondeu às lesões combinadas (16,8%), seguida da esclerose focal segmentar (11,4 %). Nos 700 casos estudados, o sexo masculino correspondeu a 54,go;ó dos casos e a cor branca, a 84,3%. A idade média dos pacientes foi de 28,23 :!: 16,4 anos. O complexo LM/GNPM!EF, que engloba a doença de lesão mínima idiopática (LM), a GN proliferativa mesangial idiopática, com proteinúria e deposição de IgM (GNPM), e a esclerose focal idiopática (EF), foi o diagnóstico nosológico mais freqüente. Este correspondeu a 21 % do total de 700 casos, seguido pelo LES (11 %), GN membranosa idiopática (8,0%), doença de Berger (7,3%), GN pós-infecciosa (6,1%) e GN membranoproliferativa idiopática tipo I (5,6%). o complexo LM/GNPM/EF é motivo de controvérsia na literatura. Alguns autores acreditam que, possivelmente, trata-se de uma única doença, tendo, de um lado, o pólo mais benigno, representado pela doença de lesão núnima idiopática, e, de outro, a esclerose focal idiopática, de evolução clínica mais agressiva. A GN proliferativa mesangial idiopática, com proteinúria e deposição de IgM, representaria uma fase intermediária entre estas duas entidades. Apoiando esta interpretação, em um total de 16 pacientes com biópsias seriadas, verificamos que 12,5% deles apresentavam lesão núnima na primeira biópsia e esclerose focal na segunda; 31,2%, GN proliferativa mesangial na primeira e esclerose focal na segunda; 6,3%, lesão mínima na primeira e GN proliferativa mesangial na segunda. Estes achados sugerem que, possivelmente, o complexo LM/GNPM/EF represente fases evolutivas de uma única doença. A imunofluorescência (IF) e a microscopia eletrônica (ME) contribuíram significativamente para a determinação do diagnóstico nosológico. À imunofluorescência, a forte positividade para o Clq foi um importante marcador para o LES. Este achado esteve presente em 98,2% das biópsias de pacien~es com lúpus eritematoso sistêmico. Observamos que não existe uma lesão única, em microscopia óptica comum, imunofluorescente e eletrônica, que, isoladamente, seja patognomônica de uma determinada doença, à exceção, talvez, dos corpos hematoxilínicos, encontrados exclusivamente no lúpus eritematoso sistêmico. Nós enfatizamos a necessidade da utilização da microscopia óptica comum, imunofluorescente e eletrônica, juntamente com os dados clínicos e laboratoriais, para determinação do diagnóstico nosológico. Nós acreditamos que esta nova abordagem para a classificação de glomerulonemtes é simples, fácil, didática e está de acordo com o fenômeno biológico, isto é, leva em conta, para uma mesma doença, as variabilidades de lesão glomerular de paciente para paciente e, num mesmo paciente, as diferentes fases evolutivas da moléstiaAbstract: Past eighty years since Volhard and Fahr put some order in the existent chaos, classification of glomerulonephritis is still a challenge for pathologists and nephrologists. Even the last proposal for classification by the World Health Organization (WHO) is not entirely satisfactory. Three factors contribute for making classification of glomerulonephritis diflicult: 1. A particular glomerular lesion may correspond to several diseases; 2. A single disease may present several glomerular lesions, for example, systemic lupus erythematosus may present different glomerular lesions in the evolution of the disease; 3. Some primary glomerular diseases are named according to the anatomical lesion they present. The present work has the aim to show a new approach to the classification of glomerulonephritis. The proposal is to dissociate the anatomic diagnosis (AD) from the nosologic diagnosis (ND). Both AD and ND should be included in the pathology reporto The anatomic diagnosis is based on light microscopy and the nosologic diagnosis after combining the light, imunofluorescent and electronic microscopy with the clinical and laboratory findings. The most fTequent anatomic "lesion was mesangial proliferative glomerulonephritis (1681700,24%) and corresponded to a large number of diseases, such'as Berger's disease, Alport's syndrome, postinfectious glomerulonephritis, thin membrane disease, lupus nephritis, Henoch-Schõnlein purpura and many others. The second most fTequent anatomic lesion corresponded to combined lesions (16.8%) followed by focal segmental glomerulosclerosis (11.4%). In the 700 cases studied, males corresponded to 54.9% of the cases and whites to 84.3%. At the time of the biopsy the mean age of the patients was 28.23 1: 16.4 years. The most fTequent nosologic diagnosis was the complex ML/MPGN/FS corresponding to idiopathic minimallesion disease (ML), idiopathic mesangial proliferative GN with deposition ofIgM (MPGN) and idiopathic focal sclerosis (EF). It corresponded to 21 % of the total of 700 cases followed by lupus nephritis (11 %), idiopathic membranous glomerulonephritis (8%), Berger's disease (7.3%), postinfectious GN (6.1%) and type 1 idiopathic membranoproliferative GN (5.6%). There is much controversy in the literature concerning the complex ML/MPGN/FS. For some authors, this complex is possibly a single disease which has at one side the most benign pole represented by idiopathic minimallesion disease and at the other extreme the idiopathic focal sclerosis which has a more aggressive course. The idiopathic mesangial proliferative GN with deposition of IgM may represent an intermediate phase between these two entities. bur study favors this interpretation. In a total of 16 patients with serial biopsies we found that 12.5% ofthe patients had minimallesion in the first biopsy and focal sclerosis in the second; 31.2% had mesangial proliferative GN in the first and focal sclerosis in the second; and, 6.3% had minimallesion in the first and mesangial proliferative GN in the second. These findings suggest that possibly the complex ML/MPGN/FS represent evolution phases of a single disease. Immunotluorescent and electron microscopy high1y contribute for the determination ofthe nosologic diagnosis. Intense staining for c1q is particuiarly useful ÍR the diagnosis for lupus nephritis. This finding was present in 98.2% of the biopsies of patients with systemic lupus erythematosus. We observed that there is not a single lesion in light, immunotluorescent and electron microscopy that is pathognomic for a. disease except for hematoxylinic bodies thàt were found exclusively in lupus nephritis. We emphasize the need for using light, immunotluorescent and electron microscopy combined with clínical and laboratory findings for establishing the nosologic diagnosis. We believe that this new approach to the classification of glomerulonephritis is simple, easy, didactic and in accord with the biologic phenomenon, that is, it considers the variability of glomeruiar lesions from patient to patient and, in a single patient, the several evolution phases of the diseaseDoutoradoAnatomia PatologicaDoutor em Ciências Médica

Gonadal dysgenesis and morphometric histologic analysis

Partial gonadal dysgenesis (PGD) is a sexual differentiation disorder characterized by bilateral dysgenetic testes, persistent Müllerian ducts and cryptorchidism in intersex patients with a 46,XY karyotype. Although the abnormalities observed in dysgenetic testes are well defined, they are not routinely evaluated by the pathologist. This work reviews in detail the gonadal histologic structure evolution during the lifespan and the histologic data necessary to evaluate the cases with gonadal dysgenesis suspected. We also show the morphometric and histologic features of 22 gonads from 13 children with clinical and laboratory diagnosis of PGD, but without histologic confirmation in the first evaluation. These morphometric and histologic findings confirmed the diagnosis of PGD in every patient in the present series. However, gonadal histology was variable and a careful morphometric evaluation may be necessary to establish the diagnosis.A disgenesia gonadal parcial (DGP) é um distúrbio da diferenciação sexual caracterizado por testículos disgenéticos bilaterais, derivados dos ductos de Müller e criptorquidismo em pacientes com ambigüidade genital e cariótipo 46,XY. Entretanto, os critérios histológicos diagnósticos de disgenesia gonadal, apesar de existentes, na prática são pouco utilizados. Este artigo apresenta uma detalhada revisão da evolução das modificações da estrutura histológica gonadal durante a vida e os dados histológicos necessários na avaliação de casos com suspeita de disgenesia gonadal. Mostra-se também os achados morfométricos gonadais de 13 crianças com diagnóstico clínico e laboratorial de DGP, porém sem a confirmação histológica numa avaliação inicial. Após estudo morfométrico histológico das gônadas destas crianças, o diagnóstico de DGP foi confirmado em todos os casos. Portanto, devido à variabilidade da análise histológica gonadal, um estudo morfométrico cuidadoso torna-se necessário para o estabelecimento do diagnóstico de DGP.12813

Lymphoblastic transformation of myelodysplastic syndrome

Mielodysplastic syndromes (MDS) are clonal disorders of the hemopoietic stem cell. About one third of the cases terminate in an acute leukemia, usually acute myeloblastic leukemia. However, few cases of transformation into acute lymphoblastic leukemia (ALL) have been described. We present a case of refractory anemia that transformed into ALL two months after diagnosis and was successfully treated with conventional chemotherapy. Two years later a hyperfibrotic form of MDS was detected in the patient, that soon after terminated in acute megakaryoblastic leukemia. The course of MDS in the present case provides evidence that MDS can involve a pluripotent stem cell, presenting clonal evolution, documented by successive changes in its clinical and hematological features