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O presente estudo tece investigações sobre os aspectos epidemiológicos de malformações do aparelho urinário de uma população de natimortos, neomortos, lactentes, pré-escolares e escolares de uma amostra representativa da população de Curitiba e região metropolitana - análise de 40 anos de necropsias na Unidade de Patologia Pediátrica e Perinatal (UPPP) do Serviço de Anatomia Patológica do Hospital de Clínicas de Curitiba da Universidade Federal do Paraná (UFPR). Variáveis como sexo, idade, faixa etária e causas de morte são correlacionadas, estratificando a amostra em grupos específicos de indivíduos, apontando anormalidades epidemiológicas, doenças raras e a relação das doenças com o processo de morte. Foram encontrados 182 casos com malformações do aparelho urinário, correspondendo a 2,9% do número de necropsias avaliadas. Não foram observadas diferenças entre os sexos, e houve maior prevalência de recém-natos com tais anormalidades. A classe mais prevalente foi a de malformações renais e de trato urinário superior, com 150 casos.

INTRODUÇÃO: A cisplatina é utilizada como droga de escolha para o tratamento do câncer de ovário, antes ou depois do procedimento cirúrgico, no qual há freqüentemente a confecção de uma anastomose intestinal. OBJETIVO: Avaliação histológica do efeito da cisplatina sobre a cicatrização das anatomoses colônicas. MATERIAL E MÉTODOS: Foram utilizados 80 ratos Wistar divididos em três grupos: um grupo controle, que foi submetido ao procedimento cirúrgico e recebeu solução salina nos cinco dias subseqüentes, incluindo o dia da operação; um grupo que recebeu cisplatina intraperitoneal na dose de 0,35mg/kg por cinco dias antes do procedimento cirúrgico; e um grupo que recebeu cisplatina intraperitoneal na mesma dose por cinco dias depois do procedimento cirúrgico. O procedimento cirúrgico constou de secção da alça colônica esquerda, 5cm acima da reflexão peritoneal, e anastomose em plano único. Metade dos ratos foi sacrificada no quinto dia de pós-operatório e a outra metade, no oitavo. Foram avaliados parâmetros histológicos de reação inflamatória com a elaboração de índices e escores para comparação entre os grupos do estudo. RESULTADOS: A média do índice infiltrado neutrofílico foi significativamente maior no grupo que recebeu cisplatina depois do procedimento cirúrgico em relação ao grupo controle e ao grupo que recebeu droga antes do procedimento cirúrgico. A média do índice intensidade do edema foi significativamente maior nos grupos que receberam cisplatina em relação ao grupo controle. A análise do escore histológico final permitiu a classificação do processo inflamatório como subagudo nos grupos que receberam cisplatina e como crônico no grupo controle. DISCUSSÃO E CONCLUSÃO: A cisplatina interfere no processo de cicatrização das anastomoses colônicas, produzindo uma lentificação do mesmo.BACKGROUND: Cisplatin is the first line drug in the treatment of the ovarian cancer. This drug is often applied before or after a surgical procedure to whom a large bowel anastomosis is done. OBJECTIVE: Histological evaluation of the effect of the cisplatin on the healing of the colonic anastomoses. METHODS: Eighty male Wistar rats were divided in three groups: the control group, the group that received intraperitoneal cisplatin injection for five days before the surgical procedure and the group that received the cisplatin for five days after the surgical procedure. The cisplatin was used in the dose of 0,35mg/kg. The large bowel was transected 5cm above peritoneal reflection and it was anastomosed in one plan suture. Half of the animals were sacrificed on the fifth day and the other half on the eighth day after the surgical procedure. Histological parameters of inflammatory reaction were evaluated with the elaboration of indexes and scores to compare the groups of this study. RESULTS: The average of neutrophilic infiltrating index was significatively bigger in the group that received cisplatin after the surgical procedure when compared with the control group and the group that received cisplatin before the surgery. The average of intensity of the edema index was significatively bigger in the groups that received cisplatin when compared with the control group. The analysis of the final histological score allowed the classification of the inflammatory process as subacute in the groups that received cisplatin and chronic in the control group. DISCUSSION AND CONCLUSION: Cisplatin produces an impairment on the healing of the colonic anastomoses.

ABSTRACT Introduction: Breast cancer is the second leading cause of cancer death among women worldwide, and epidemiological studies may help understanding its mechanisms. Objective: To carry out a survey of the number of breast cancer cases diagnosed in a period of six years. Methods: The profile of breast cancers diagnosed in a tertiary hospital in Curitiba was compared with the literature, using a retrospective analysis of ductal/special types and lobular breast carcinoma reports issued between 2008 and 2013. Results: Three hundred twenty-seven (91.6%) cases of ductal/special types carcinoma and 30 (8.4%) cases of lobular carcinoma were diagnosed, totaling 357 samples. From these cases, 27 (7.5%) were carcinoma in situ (20 ductal and seven lobular) and 330 (92.4%) were invasive carcinoma (307 invasive ductal/special types and 23 lobular). The prevalence of breast cancer among women was 991% and the majority of patients were older than 50 years of age (67.2%). Regarding the União Internacional de Controle do Câncer/American Joint Committee on Cancer (UICC/AJCC) staging, 49.2% of the ductal/special types tumors were diagnosed in Stages I or II, while 56.6% of lobular carcinomas were diagnosed in Stages II or III/IV. Regarding the Nottingham score, most cases were intermediate grade (43.9%). A total of 61% of the tumors were estrogen receptor positive (ER+) and 54% were progesterone receptor positive (PR+). Moreover, 36.1% presented positive human epidermal growth factor receptor 2 (HER2+), a rate higher than that indicated by the literature. Conclusion: The breast carcinomas evaluated in this study presented a profile similar to that reported in the literature, with some peculiarities inherent to the local pathology service. Nevertheless, the low frequency of in situ cases indicates failure in early diagnosis.

ABSTRACT INTRODUCTION: According to the cancer stem-cell theory, tumors originate from a small population of cancer stem cells, which lose the mechanism of self-regulation and begin to differentiate and proliferate indefinitely. The CD44+/CD24- phenotype may be considered a stem-cell marker in breast cancer. OBJECTIVE: To evaluate the correlation between CD44+/CD24- phenotype and different molecular subtypes of breast cancer in invasive ductal carcinoma samples. METHODS: The expression of CD44, CD44v6, and CD24 markers was investigated in 133 cases of invasive mammary carcinoma with immunohistochemistry. CD44+/CD24- phenotype was identified and correlated with the molecular subtypes and classical prognostic factors such as age, histological grade, tumor size, and lymph node status. RESULTS: Eighteen (14%) cases were positive for CD44+/CD24- (CD44+/CD24- or CD44v6+/CD24-) phenotype; among these, 11.1%, 27.8%, 38.9%, and 22.2% were luminal, luminal B-human epidermal growth factor receptor 2 (HER2), HER2, and triple-negative subtype, respectively. CD44+/ CD24- phenotype was more common in HER2 subgroup (p = 0.0197). CONCLUSION: CD44+/CD24- phenotype was correlated with molecular subtypes of breast cancer. The highest expression of CD44+/CD24- phenotype was reported in patients with HER2+ disease, a molecular subtype associated with more aggressive behavior and worse prognosis.

Introduction: Medulloblastoma is a malignant embryonal tumor of the cerebellum with poor prognosis. The treatment is based only on clinical criteria, such as risk group that only considers age, extent of tumor resection, recurrence, and metastasis. Objective: To evaluate a possible relationship between the immunoexpression of biomarkers (Ki67, receptor neutrophin-3 [TRKC], epidermal growth factor receptor [EGFR], B-cell lymphoma 2 [Bcl-2], and cyclin-D1), and the classical clinical prognostic factors of medulloblastoma. Material and method: thirty-five samples of pediatric medulloblastoma free of neoadjuvant chemotherapy were separated and reviewed for their histopathological classification; two areas representative of tumor were used in the construction of tissue microarrays. The following clinical data from 29 patients were used for comparison with the biomarkers expression: patient's age, presence or absence of complete tumor resection, staging patient's risk group, presence or absence of metastases, presence or absence of postoperative chemotherapy, and presence or absence of recurrence. Clinical follow-up of the study ranged from two to thirteen years, and cases with fatal outcome were also analyzed. Results: Patients with upper age showed higher expression of TRKC (p = 0.033). There was inversely proportional and statistically significant correlation between TRKC and Ki67 (p = 0.027). There was no statistical significance in the analysis of EGFR, Bcl2, and cyclin-D1. Conclusion: The immunoexpression of TRKC might be considered a biomarker related to tumors with better prognosis in patients with medulloblastoma, contributing to better risk groups' stratification.

INTRODUCTION: Nephroblastoma or Wilms' tumor is the most frequent renal cancer in children. Although its prognosis is favorable for most patients, it may relapse or have a fatal outcome. The characterization of risk groups by applying immunohistochemical biomarkers aims to adapt the treatment to its corresponding group as well as to reduce relapses and fatal outcome. p53, B-cell lymphoma 2 (BCL-2), BCL-2 associated protein X (BAX) and vascular endothelial growth factor receptor 1 (VEGFR1) are among the most widely studied biomarkers, which are related to the apoptotic pathway, DNA repair and neovascularization. OBJECTIVE: The objective of this study is to assess the immunohistochemical expression of p53, BCL-2, BAX and VEGFR1 in samples of human nephroblastoma and to correlate them with clinicopathological prognostic factors. MATERIAL AND METHODS: Twenty-nine surgical specimens of nephroblastoma diagnosed from 1994 to 2007 were selected from the Anatomopathological Service of two hospitals in Curitiba. The immunohistochemical analysis of tissue microarrays was performed through immunoperoxidase staining and the yielded results were compared with clinicopathological prognostic factors. RESULTS: The major immunohistochemical expression of VEGFR1 in blastema and epithelium presented positive association with the risk group. Hence this may be related to higher vascular neoplastic invasion apparently caused by the endothelial growth factor, which maximizes the chances of metastasis and ultimately changes tumor staging, risk group and clinical evolution. CONCLUSIONS: The immunohistochemical expression of VEGFR1 substantiated a directly proportional association with the nephroblastoma risk group.INTRODUÇÃO: O nefroblastoma, ou tumor de Wilms, é a neoplasia renal mais frequente na infância. Embora o prognóstico seja favorável para a maioria dos pacientes, muitos evoluem para recidiva ou óbito. A caracterização de grupos de risco por meio de marcadores imuno-histoquímicos tem por objetivo adequar o tratamento ao grupo de risco e reduzir recidivas e óbitos. Entre os marcadores mais estudados estão p53, B-cell lymphoma 2 (BCL-2), BCL-2 associated protein X (BAX) e fator de crescimento vascular endotelial e seu receptor 1 (VEGFR1), relacionados com a via apoptótica, o reparo do ácido desoxirribonucleico (DNA) e a neovascularização. OBJETIVO: O objetivo deste estudo é avaliar a expressão imuno-histoquímica de p53, BCL-2, BAX e VEGFR1 em amostras de nefroblastoma humano e correlacioná-las com fatores prognósticos clínico-patológicos. MATERIAL E MÉTODO: Foram utilizadas 29 amostras de nefroblastomas retiradas dos Serviços de Anatomia Patológica de dois hospitais de Curitiba, com diagnósticos entre 1994 e 2007. Por meio da técnica de imunoperoxidase, com as amostras em arranjo tecidual em matriz, realizou-se análise da imuno-histoquímica desses marcadores e sua comparação com fatores prognósticos clínico-patológicos. RESULTADOS: A maior imunoexpressão de VEGFR1 nos componentes blastematoso e epitelial mostrou associação positiva ao grupo de risco, sendo que isso poderia estar relacionado com a maior capacidade de invasão vascular neoplásica que pode ser conferida por esse fator de crescimento endotelial, aumentando, assim, as chances de metástases e alterando o estadimento, o grupo de risco e a evolução clínica. CONCLUSÃO: A expressão imuno-histoquímica aumentada de VEGFR1 mostrou associação diretamente proporcional ao grupo de risco dos pacientes com nefroblastoma.

INTRODUCTION: It is widely known that the expression levels of molecules involved in apoptosis regulation and cell proliferation have prognostic value in patients with neuroblastomas. OBJECTIVE: To determine the expression of Ki67, B-cell lymphoma 2 (BCL-2), phosphatase and tensin homolog (PTEN), BCL-2 associated protein X (BAX) and caspase-8 proteins in neuroblastomas and to propose new prognostic biomarkers that could enable a better classification of risk groups. MATERIAL AND METHODS: Formalin fixed paraffin embedded neuroblastoma samples (n = 23) were arranged into tissue microarray blocks and analyzed by immunohistochemistry. The patients were classified according to clinical and pathological prognostic factors (age, site, presence or absence of bone-marrow infiltration, poorly or well differentiated ganglioneuroblastoma, Schwannian stroma rich or poor, favorable or unfavorable Shimada histology, and presence or absence of MYCN oncogene amplification) and clinical course (with or without fatal outcome, with or without relapses/residual lesion). RESULTS: Twelve patients were female; nine children were over 18 months old; nine had extra-abdominal tumors; nine had tumors with unfavorable histology. Fifteen patients underwent bone-marrow biopsy and four were positive for metastasis. Nine patients progressed to fatal outcome. CONCLUSION: Ki67 immunoexpression was lower in cases of Schwannian-stroma rich neuroblastomas (p = 0.018) and higher in poorly differentiated cases (p = 0.013). PTEN was less positive in stroma rich neuroblastomas (p = 0.024). Caspase-8 was more immunopositive in cases of negative bone marrow infiltration (p = 0.035). Therefore, these biomarkers could be applied to discriminate groups with poor prognosis.INTRODUÇÃO: Sabe-se que os níveis de expressão de moléculas envolvidas na regulação da apoptose e da proliferação celular apresentam valor prognóstico em pacientes com neuroblastomas. OBJETIVO: Avaliar a imunoexpressão das proteínas Ki67, B-cell lymphoma 2 (BCL-2), phosphatase and tensin homolog (PTEN), caspase-8 e BCL-2 associated protein X (BAX) em neuroblastomas na tentativa de propor novos biomarcadores prognósticos que poderiam auxiliar na melhor discriminação dos grupos de risco. MATERIAL E MÉTODOS: Amostras de neuroblastoma (n = 23) foram submetidas à técnica tissue microarray e analisadas com imuno-histoquímica. Os pacientes foram classificados de acordo com os fatores prognósticos clínico-patológicos (idade, localização, medula óssea infiltrada ou não, pouco diferenciado ou em diferenciação/ganglioneuroblastoma, rico ou pobre em estroma com células de Schwann, histologia favorável ou desfavorável segundo Shimada, presença ou não da amplificação do MYCN) e com o curso clínico (se em óbito ou vivo, com ou sem lesão residual/recidiva). RESULTADOS: Doze casos eram do sexo feminino; nove tinham idade acima de 18 meses; nove apresentavam tumores extra-abdominais; e nove cursavam com histologia desfavorável. Quinze pacientes foram submetidos à biópsia de medula óssea, sendo quatro com apresentação de metástase. Nove pacientes evoluíram ao óbito. CONCLUSÃO: A imunoexpressão do Ki67 foi mais baixa nos casos ricos em estroma (p = 0,018) e elevada nos casos pouco diferenciados (p = 0,013). O PTEN apresentou-se menos positivo em neuroblastomas ricos em estroma (p = 0,024). A caspase-8 foi mais imunopositiva em casos com medula óssea negativa (p = 0,035). Esses biomarcadores poderiam ser utilizados para auxiliar a discriminar grupos de pacientes de pior prognóstico.

INTRODUCTION AND OBJECTIVE: The aim of the present study is the immunohistochemical assessment of tumor progression markers (E-cadherin, β-catenin, CEACAM-1 and PTEN) in primary cutaneous melanomas and their correlation with prognostic factors. METHOD: Primary lesions in patients with cutaneous melanoma were recorded as to clinical data (age, gender, location and metastases) and anatomopathologic data (Breslow, Clark level, margins, histological type, mitosis, ulceration, regression, satellitosis and TIL type). It was made a comparison between immunohistochemical expression of the markers and prognostic and anatomopathologic factors. RESULTS: Breslow thickness was > 1 mm (thick) in 21 patients (48.83%) and < 1 mm (thin) in 22 (51.16%). There was a higher CEACAM-1 positive expression in thick melanomas than in thin ones (p = 0.002). There was a more frequent E-cadherin (p = 0.008), b-catenin (p = 0.001) and PTEN (p = 0.005) positive expression in thin melanomas than in thick ones. There was a more frequent CEACAM-1 positive expression in superficial (p = 0.003) and deep (p = 0.002) samples of thick melanomas than in thin ones. No statistically significant differences between clinical and histopathological data were found when comparing patients with (n = 6) and without metastasis (n = 15). DISCUSSION AND CONCLUSION: There was a higher positivity for E-cadherin, b-catenin and PTEN in thin melanomas, whereas there was a higher positivity for CEACAM-1 in thick melanomas.INTRODUÇÃO E OBJETIVO: O objetivo do presente estudo está na avaliação imuno-histoquímica de marcadores de progressão tumoral (E-caderina, β-catenina, CEACAM-1 e PTEN) em melanomas cutâneos primários e sua correlação com fatores prognósticos. MÉTODO: Lesões primárias de pacientes portadores de melanoma cutâneo foram tabuladas quanto a dados clínicos (idade, sexo, localização e metástases) e anatomopatológicos (Breslow, nível de Clark, margens, tipo histológico, mitoses, ulceração, regressão, satelitose e tipo de TIL). Foi realizada comparação da expressão imuno-histoquímica dos marcadores em estudo com fatores prognósticos clínicos e anatomopatológicos. RESULTADOS: Breslow foi > 1 mm (espesso) em 21 pacientes (48,83%) e ≤ 1 mm (fino) em 22 (51,16%). CEACAM-1 foi mais positivo em melanomas grossos que em finos (p = 0,002). E-caderina (p = 0,008), β-catenina (p = 0,001) e PTEN (p = 0,005) foram mais frequentemente positivos em melanomas finos que em grossos. CEACAM-1 foi mais frequentemente positivo nas porções superficiais (p = 0,003) e profundas (p = 0,002) dos melanomas grossos que nas dos finos. Não houve diferenças estatisticamente significativas entre dados clínicos e histopatológicos quando comparamos os pacientes com (n = 6) e sem (n = 15) metástases. DISCUSSÃO E CONCLUSÃO: Observou-se maior tendência de positividade para E-caderina, b-catenina e PTEN em melanomas finos. Por sua vez, CEACAM-1 demonstrou maior frequência de positividade nos melanomas grossos.

INTRODUÇÃO: A displasia broncopulmonar (DBP) continua sendo a principal complicação nos recém-nascidos (RN) prematuros. Com o uso de surfactante exógeno e da prevenção de doenças respiratórias no período neonatal a incidência de DBP clássica vem diminuindo, porém uma nova forma de DBP tem surgido, mais branda e associada aos desenvolvimentos pulmonar alveolar e vascular incompletos. Do ponto de vista anatomopatológico a DBP clássica é caracterizada por processos de lesão e reparação, e os achados da "nova" DBP são de hipoplasia alveolar com nenhuma fibrose. OBJETIVOS: Demonstrar as alterações histopatológicas e morfométricas em pulmões de prematuros que foram a óbito, com quadro clínico compatível com "nova" DBP, comparando-as com um grupo controle (sem DBP) e com a forma clássica da doença, além de correlacionar os três grupos com o tempo de uso de oxigênio entre outros fatores de risco da DBP. MATERIAL E MÉTODOS: A população foi composta por 59 amostras de pulmões de prematuros com idade gestacional (IG) menor que 34 semanas e submetidos à oxigenioterapia. Fatores de risco para DBP foram coletados por meio da revisão de prontuários. Amostras pulmonares foram separadas em dois grupos, o com DBP clássica e o sem DBP clássica. O segundo grupo foi então submetido à análise morfométrica para contagem do número de alvéolos, medidas as áreas e os perímetros dos alvéolos. Após esta análise a população estudada ficou dividida em grupo com DBP clássica; com "nova" DBP (casos com mais de sete dias de oxigenioterapia); e grupo controle ou sem DBP clássica ou "nova" (casos com menos de sete dias de oxigenioterapia). RESULTADOS: o primeiro grupo apresentava inflamação e fibrose septal evidentes. Já os segundo e terceiro grupos apresentavam alterações histopatológicas mínimas, sendo então necessária a análise morfométrica para separá-los. O grupo com "nova" DBP apresentou número de alvéolos, sua área e perímetro diminuídos (p < 0,005) quando comparados ao grupo controle. Não houve diferença estatisticamente significativa entre os três grupos com relação aos fatores de risco para desenvolvimento de DBP. CONCLUSÃO: As formas de DBP clássica e "nova" são de etiologia multifatorial, porém distintas entre si. Para diagnóstico anatomopatológico da "nova" DBP pode ser necessária a análise morfométrica. As alterações histopatológias e morfométricas deste estudo antecederam a necessidade de oxigenioterapia por mais de 28 dias. Portanto, instituir terapêutica para DBP antes do 28º dia de vida em RN prematuro em oxigenioterapia estaria justificado pelos nossos achados histopatológicos e morfométricos.INTRODUCTION: The Bronchopulmonary dysplasia (BPD) remains as a major complication in premature infants. The incidence of classic BPD has decreased due to the use of exogenous surfactant and prevention of respiratory diseases in the neonatal period. However, a new and milder form of BPD has appeared, which is associated with incomplete vascular and pulmonary alveolar development. Anatomopathologically, classic BPD is characterized by lesion and repair processeses and "new" BPD findings are alveolar hypoplasia with no fibrosis. OBJECTIVES: To demonstrate the morphometric and histopathological alterations in the lungs of deceased premature infants with clinical course consistent with the new BPD by comparing these changes with a control group (without BPD) and with its classic form. Furthermore, to correlate the three groups with the duration of oxygen therapy and other risk factors. METHODS: The population comprised 59 lungs samples from premature infants of gestational age lower than 34 weeks and that had undergone oxygen therapy. The risk factors for BPD were collected from the review of clinical records. The lungs samples were separated into 2 groups: 1 - with classic BPD and 2 - without classic BPD. Group 2 underwent morphometric analysis for alveoli counting and measurement of alveolar area and perimeter. Subsequently, the studied population was divided into: 1 - with classic BPD, 2 - with new BPD (cases with more than 7 days of oxygen therapy) and 3 - control group or without classic or new BPD (cases with less than 7 days of oxygen therapy). RESULTS: Group 1 (classic BPD) had inflammation and evident septal fibrosis. Groups 2 and 3 (new BPD and control) showed minimal histopathological alterations requiring morphometric analysis to separate them. Group 2 (new BPD) showed reduced number of alveoli, area and perimeter when compared with group 3 (control), p < 0,005. There was no statistically significant difference among the 3 groups regarding the risk factors for the development of BPD. CONCLUSION: Both forms of classic and new BPD have a multifactorial etiology, but they are different among themselves. The anatomopathological diagnosis of new BPD may require morphometric analysis. Histopathological and morphometric alterations in this study preceded the need for oxygen for more than 28 days. Accordingly, our histopathological and morphometric findings would justify the establishment of treatment for BPD before the 28th day of life in premature neonates.

Uma paciente de 4 meses de idade foi encaminhada ao hospital por aumento de volume abdominal, cianose e febre há dois meses. Ao exame, apresentou hepatoesplenomegalia. Com os resultados laboratoriais constatou-se anemia hipocrômica microcítica, leucocitose, plaquetopenia e provas de função hepática alteradas. Levantou-se a hipótese de erros inatos do metabolismo. A paciente evoluiu desfavoravelmente e foi a óbito. À necropsia o fígado apresentou-se cirrótico e com grande depósito de ferro no parênquima. O diagnóstico anatomopatológico foi hemocromatose hereditária, no entanto os resultados laboratoriais confirmaram tirosinemia tipo 1. Tanto a hemocromatose primária quanto a tirosinemia evoluem com cirrose hepática, sendo que a segunda pode ocasionalmente levar ao depósito de ferro do tipo hemocromatose secundária, o que dificulta muito a diferenciação entre elas com bases puramente anatomopatológicas, acarretando, assim, um diagnóstico errôneo de hemocromatose hereditária. Esse diagnóstico diferencial muitas vezes só é possível com os achados de rastreamento de erros inatos do metabolismo.A 4-month-old female patient was taken to hospital due to increase in abdominal volume, cyanosis and fever for two months. At examination, she presented hepatosplenomegaly. Laboratory tests revealed hypochromic anemia (due to iron deficiency), leucocytosis, low platelet count and altered hepatic functions. Innate metabolic error was suspected. Follow-up developed unfavorably and she died. At necropsy, the liver was cirrhotic, with large iron deposits in the parenchyma. Necropsy diagnosis was haemochromatosis, and laboratorial results confirmed type 1 tyrosinemia. Either primary haemochromatosis or tyrosinemia could cause hepatic cirrhosis. Tyrosinemia may also cause iron deposits in the liver parenchyma, similar to those observed in secondary haemochromatosis. Such facts can make differential diagnosis difficult between these and other conditions such as hereditary haemochromatosis. In fact, differential diagnosis is only achievable by using laboratorial tests for innate metabolic errors.

INTRODUÇÃO: Os efeitos da desnutrição intra-uterina dependem da fase de desenvolvimento em que está o feto ou o órgão, sendo tanto mais intensos e permanentes quanto mais precocemente ocorrer e mais tarde se iniciar a recuperação nutricional. OBJETIVOS: Avaliar alterações no intestino delgado diante da desnutrição intra-uterina e após a lactação em filhotes de ratas Wistar submetidas à restrição dietética na gestação e comparar com o grupo controle. MATERIAL E MÉTODOS: As ratas prenhes foram divididas em dois grupos; o grupo controle recebeu dieta ad libitum enquanto o grupo com restrição alimentar recebeu 50% dessa dieta. Ao nascimento as ninhadas foram avaliadas quanto ao número de filhotes e ao peso. Os filhotes foram sacrificados em até 24 horas após o nascimento e avaliados individualmente. Na morfometria do intestino delgado estudou-se: altura total da mucosa, altura da vilosidade, espessura da vilosidade e altura do enterócito. Após a lactação as mesmas medidas foram realizadas e comparadas entre os grupos. RESULTADOS: Ao nascimento, o peso individual, o comprimento do corpo e o do intestino foram menores no grupo com restrição alimentar. Tais resultados mantiveram-se após a lactação, exceto o comprimento do corpo, que se mostrou semelhante nos dois grupos. À morfometria observou-se, após o nascimento, que todas as variáveis estudadas foram significativamente menores no grupo de filhotes de mães com restrição alimentar. Após a lactação houve recuperação da espessura da vilosidade, porém as demais variáveis permaneceram menores. DISCUSSÃO E CONCLUSÕES: A restrição de 50% da dieta materna durante a gestação leva a baixo ganho de peso e produz conceptos menores, com menor comprimento do intestino em relação a grupo controle e morfometria microscópica revelando medidas menores estatisticamente significativas. A maioria dessas alterações se mantém após a lactação e apresenta uma correlação positiva com o peso dos animais.BACKGROUND: The effects of in-uterus malnutrition depend on the developmental stage of the fetus or the organ, being more intense and permanent the earlier it happens and the later the nutritional recovery occurs. OBJECTIVES: Evaluate the changes in the small bowel regarding in-uterus malnutrition and after lactation in Wistar rats' pups that underwent pregnancy in dietary restriction and compare them to a control group. MATERIAL AND METHOD: The pregnant dams were divided into two groups; the control group received ad libitum diet whereas the food deprivation group had 50% of that intake. At birth, the litters were evaluated on the number of pups and their weight. Twenty-four hours after delivery, part of them was sacrificed and evaluated one at a time. In the intestine, the total height of the mucosa, the height of the villi, the height of the enterocyte and the thickness of the villi were studied by means of microscopic morphometry. The same measures were applied after lactation and the results were compared between groups. RESULTS: After delivery, individual weight, body length and intestine length were lower in the pups whose mothers were fed with a restricted diet during pregnancy. Such results were sustained after lactation, except the body length, that was similar for both groups. All the varieties studied in the microscopic morphometry were significantly lower in the group of malnutrition mothers. After lactation, the thickness of the villi was recovered, although the other varieties remained low. DISCUSSION AND CONCLUSION: This study indicates that a maternal diet restriction to 50% ad libitum induces lower maternal weight and produces smaller pups. The small intestinal length and the microscopic morphometry of the malnutrition group are diminished and the majority of the differences are maintained after the lactation period with positive correlation to the weight of the pups.

INTRODUÇÃO: A fibroelastose endocárdica (FEE) é definida como espessamento difuso do endocárdio ventricular em função da proliferação de fibras colágenas e elásticas. Pode ser classificada como primária ou secundária. A FEE primária apresenta-se na forma dilatada, mais comum, ou na forma restritiva. Geralmente manifesta-se como insuficiência cardíaca inexplicada, em crianças com menos de 12 meses de idade. OBJEIVOS: Avaliar aspectos epidemiológicos, clínicos e anatomopatológicos da FEE primária. MATERIAL E MÉTODO: Foram pesquisadas, entre 1960 e 2000, 6.211 necropsias perinatais e pediátricas do Hospital de Clínicas da Universidade Federal do Paraná (UFPR), tendo sido verificados 11 casos de FEE. RESULTADOS: Em nove casos (81,8%), o surgimento dos sintomas ocorreu antes de 1 ano de idade. Em oito (72,7%), o óbito ocorreu antes de 1 ano de idade. Dentre os sinais clínicos, destacaram-se taquipnéia (54,5%), hepatomegalia (45,5%), tosse (36,4%), cianose (36,4%) e febre (36,4%). Todas as radiografias (n = 6) apresentavam cardiomegalia. O espessamento endocárdico predominou no coração esquerdo (90,9%). A causa de óbito foi insuficiência respiratória aguda em cinco casos (45,5%) e choque cardiogênico em quatro (36,4%). DISCUSSÃO E CONCLUSÕES: Os resultados mostram acometimento predominante de crianças com menos de 1 ano de idade e rápida progressão para óbito. Sinais clínicos freqüentes, como taquipnéia, tosse e febre, sugeriram infecção respiratória, que foi confirmada por necropsia em três casos (27,3%). Os exames complementares mostraram apenas achados sugestivos de insuficiência cardíaca. Os dados analisados neste estudo mostram que, em nosso meio, a FEE primária não difere significativamente do que é apresentado na literatura internacional em relação a características epidemiológicas, clínicas e anatomopatológicas.BACKGROUND: Endocardial fibroelastosis (EFE) is a diffuse thickening of the ventricular endocardium resulting from proliferation of fibrous and elastic tissue. Primary and secondary forms have been described. Primary EFE occurs in the dilated and contracted forms. EFE usually manifests as unexplained congestive heart failure in infants younger than 1 year old. OBJECTIVES: To analyze the epidemiologic, clinical and anatomopathological aspects of primary EFE. METHODS: Eleven cases of histologically confirmed EFE were found in 6211 necropsies performed between 1960 and 2000 in the Hospital of the University of Paraná. Results: In nine cases (81.8%) the initial clinical presentation occurred before the age of 1 year. In eight cases (72.7%) patients died before the age of 1 year. The most common clinical findings were tachypnea (54.5%), hepatomegaly (45.5%), cough (36.4%) and fever (36.4%). Cardiomegaly was the main radiographic finding, present in all cases (n = 6). The endocardial thickening was more frequent at the left heart (90.9%). The death cause was acute respiratory failure in five cases (45.5%) and cardiogenic chock in four cases (36.4%). DISCUSSION AND CONCLUSIONS: The results showed that EFE affects usually infants younger than 1 year old that rapidly progress to death. Some common clinical findings like tachypnea, cough and fever suggested respiratory infection which was confirmed by necropsy in three cases (27.3%). The complementary tests showed only non-specific findings of congestive heart failure. The information analyzed here demonstrated no significant difference of the epidemiological, clinical and anatomopathological characteristics of primary EFE between Brazil and the international literature.

O uso do laser neodymium-yttrium-aluminium-garnet (Nd-YAG) como instrumento auxiliar para o rejuvenescimento propiciou um refinamento da técnica tradicional. Tal procedimento mostra resultados satisfatórios com relação ao aprimoramento técnico e à recuperação pós-operatória. OBJETIVO: Evidenciar as alterações histológicas e morfométricas encontradas na pálpebra de humanos após aplicação do laser Nd-YAG e comparar com a pele normal. MATERIAL E MÉTODO: Nove pacientes do sexo feminino foram submetidas à aplicação do laser Nd-YAG em região palpebral direita. A pálpebra esquerda correspondeu à área controle, livre de qualquer tipo de procedimento. Foram realizadas várias aplicações do laser Nd-YAG, sendo que, no último dia, foi realizada biópsia palpebral bilateralmente. Os critérios morfométricos avaliados foram: medida da espessura da epiderme, da área subepidérmica livre de lesão, da zona de degeneração do colágeno e da espessura total da derme. RESULTADOS: Nas áreas submetidas à aplicação do laser Nd-YAG, a espessura média da epiderme foi de 33,53 micrômetros (µm) e a da pele normal foi de 29,61µm (p = 0,1099). A média da medida da área subepidérmica livre de lesão, no lado direito, foi de 40,93µm, e de 36,27µm (p = 0,1373) no esquerdo. A espessura da região de degeneração do colágeno na pálpebra que recebeu tratamento com laser teve média de 293,54µm, enquanto que na pálpebra normal ela foi de 292,22µm (p = 0,4835). No lado onde se aplicou o laser Nd-YAG, a espessura total da derme teve uma média de 681,48µm e, no lado livre de procedimento, 664,14µm (p = 0,3492). DISCUSSÃO E CONCLUSÕES: Apesar da diferença clínica observada após tratamento de lesões de envelhecimento em pálpebra de humanos com o uso do laser Nd-YAG, nota-se que não houve diferença histológica ou morfométrica após tal procedimento.The use of laser Neodymium-Yttrium-Aluminium-Garnet (Nd-YAG) as an auxiliary tool on rejuvenation provided a refining of the traditional technique. This laser procedure show satisfactory results with technical improvement and postoperatory recovery. OBJECTIVES: The aim of this study is to show the histological and morphometrical changes found in human eyelid tissue after the use of laser Nd-YAG and its comparision to the normal structure. MATERIAL AND METHODS: Nine female patients underwent laser Nd-YAG application on their right eyelid. The left eyelid was taken by control-tissue, free of any kind of procedure. It was made many applications of the laser and on the last day, it was made a bilateral biopsy. The morphometrical criteria appraised were: epidermal denseness measure, subepidermal area free of injury, colagen degeneration and total dermal density. RESULTS: In those areas whose underwent laser the epidermal measure was of 33,53 micrometers (µm) and on the normal tissue, was of 29.61µm (p = 0.1099). The subepidermal measure was, on average, of 40.93µm on right side and of 36.27µm on left side (p = 0.1373). The colagen degeneration measure on the right side was of 293.54µm and on the left side was of 292.22µm (p = 0.4835). On the procedure site, the total dermal measure was of 681,48µm and on the free site, it was of 664.14µm (p = 0.3492). DISCUSSION AND CONCLUSIONS: Despite the clinical changes observed after laser Nd-YAG therapy, there were neither histological nor morphometrical significative changes.

Este trabalho analisa o efeito da reparação cutânea do laser erbium:yttrium aluminunn garnet (YAG) (Er:YAG) em pele de ratos através de estudo histológico e morfométrico. Tatuaram-se duas figuras no dorso de cada animal (n = 35), denominadas controle e experimento. Na figura experimento, aplicaram-se três passadas de laser Er:YAG com parâmetros padronizados. Cinco animais foram eutanasiados nos dias 0, 7, 14, 28, 56, 84 e 112 de pós-operatório. O estudo histológico demonstrou no dia 0 dano de ablação médio de 215,24 micrômetros (mm), sendo homogêneo com vaporização da epiderme e porções superficiais da derme. O dano térmico residual foi pequeno (média de 41,45mm), observando-se áreas onde estava praticamente ausente. A fibroplasia cicatricial já estava presente no dia 7 (média de 55,58mm), aumentando rapidamente até o dia 28 (média de 209,49mm), quando atingiu um platô e se manteve até o dia 112. Não houve sinais de processo inflamatório agudo no dia 7 e a reepitelização já estava completa. Concluiu-se que a aplicação de laser na pele de ratos induz reparação cutânea ao longo de 112 dias nas condições do experimento.This study analyses the skin contracture effect of the erbium:YAG (Er:YAG) laser on the skin of rats by light microscopic histological and morphometrical evaluations. Two squares of back skin were precision tattooed on each of the animals (n = 35), that were termed control and experimental skin. The experimental skin was treated with three passes of the Er:YAG laser with standardized parameters. Five animals were killed and skin specimens were analyzed at 00, 07, 14, 28, 56, 84 and 112 days. Histologic examination demonstrated a mean of 215.24 micrometers ablative damage that was homogeneous with complete vaporization of the epidermis and superficial dermis. The residual thermal damage was small (mean of 41.45 micrometers) observing areas where it was absent. The cicatritial fibroplasy was present at day 07 (mean 55.58 micrometers) rising quickly until day 28 (mean of 209.49 micrometers) when it reaches a plateau and maintaining it until day 112. There are no signs of acute inflammatory process at day 07 and the reepithealization was complete at this day. This study revealed that the laser had induced shortening of the treated areas which persisted beyond the 112 days.

O presente estudo tem como objetivo analisar, do ponto de vista anatomopatológico, os efeitos térmicos encontrados na pele de ratos wistar após a aplicação do laser de CO2 e do laser erbium. Utilizaram-se oito ratos submetidos a tricotomia em toda a região toracodorsal. Selecionaram-se duas áreas separadas, as quais receberam a aplicação do laser. Na primeira foram realizadas duas passadas do laser de CO2 e na segunda, duas passadas do laser erbium. A área-controle correspondeu àquela imediatamente adjacente à área submetida ao laser. A análise microscópica da lesão causada pelo laser de CO2 revela lesão em forma de U, com ablação completa da epiderme em toda a sua extensão. A derme superficial apresenta degeneração do colágeno, correspondendo ao dano térmico residual, e a transição deste para a derme normal é bem demarcada. Na pele lesada com laser erbium observa-se também extensa área de pele lesada em forma de platô, com algumas pequenas áreas de pele não-lesada. Pode-se observar, ainda, dano do colágeno na derme superficial, porém mais discreto que aquele causado pelo CO2.

Os autores relatam um caso de linfoma T subcutâneo do tipo paniculite em uma paciente feminina de 3 anos, apresentando havia um mês múltiplos nódulos subcutâneos, indolores, disseminados no abdome, região peitoral e cervical. Ao exame histopatológico, evidenciou-se um linfoma T infiltrando tecido adiposo subcutâneo. Os linfomas T subcutâneos representam uma entidade clinicopatológica distinta, sendo raro o acometimento pediátrico.We report a case of lypotrophic lymphoma affecting a 3-year-old child with 1-month history of nontender subcutaneous nodules disseminated throughout the abdomen, chest and cervical region. Histological examination showed a T-cell lymphoma affecting the subcutaneous tissue. Subcutaneous panniculitis-like T-cell lymphoma is an unusual cutaneous T-cell lymphoma that rarely affects pediatric patients.

Linfangite granulomatosa de genitália não-infecciosa é causa rara de linfedema genital autolimitado e idiopático em crianças(4, 6, 9). A maioria dos casos de linfedema com granuloma não-infeccioso de genitália em pacientes jovens ocorre em associação à doença de Crohn subseqüente ou concomitante(1, 3, 4, 9). O caso relatado é de um menino de 14 anos que apresentava história de linfedema genital principalmente em região dorsal do corpo do pênis, poupando prepúcio, parte distal do pênis e saco escrotal. Estudos laboratoriais não revelavam doenças sistêmicas. O exame anatomopatológico da peça revelou tratar-se de linfangite granulomatosa não-infecciosa.Non-infectious granulomatous lymphangitis of genitalia is a rare cause of self-limited and idiopathic genital lymphoedema in children(4, 6, 9). Most cases of lymphoedema with non-infectious genital granulomas in young patients occur subsequent to or concomitant with Crohn's disease(1, 3, 4, 9). The case described is of a 14-year-old boy with history of genital lymphoedema mainly on the dorsal region of the body of the penis, sparing the prepuce, the distal part of the penis and the scrotum. Laboratory studies did not reveal systemic diseases. The histopathological analysis showed it was non-infectious granulomatous lymphangitis.

ABSTRACT Introduction: The analysis of deaths occurred in the neonatal period and the association of these data to necropsy data are crucial to reduce infant mortality rate worldwide. Objective: To analyze the preventable causes of death and the factors associated with a higher risk of early newborn death. Methods: A cross-sectional and descriptive study was performed with data about newborns that died during the neonatal period at a university hospital located in Curitiba; 314 cases of pediatric necropsies were selected, and preventable causes of death, survival time, sex, weight, gestational age, first- and fifth-minute Apgar score, cyanosis, acidosis, meconium aspiration, the need for oxygen resuscitation, cause of death and baseline disease were analyzed. Results: When considering only the cause of death, 300 cases (95.54%) would have preventable causes, but when analyzing the underlying disease, the number of cases decreased to 209 (66.56%). The most frequent cause of death was hypoxia (85%), and the main baseline disease was diffuse alveolar damage (52.9%). There was a positive association between these variables with survival time: cyanosis (p = 0.02), gestational age (p = 0.012), cause of death (p < 0.001), Apgar score < 6 (p < 0.001) and pH value (p < 0.001). Conclusion: The incidence of preventable causes of death is probably lower when analyzed concurrently with the underlying disease. Cyanosis, gestational age, cause of death, Apgar < 6 and arterial blood pH are associated with survival time of newborns.

ABSTRACT Introduction: Despite the importance of cardiovascular diseases to population health, there is limited knowledge about how neonatal hypoxia, related or not with prematurity, may cause cell injury to cardiomyocytes in the neonatal period and what the consequences to adult life are. Objective: To analyze the tissue immunostaining of biomarkers involved in the process of cell death and growth in the myocardium of hypoxic newborns. Method: Human myocardium samples (left ventricle) from necropsies of hypoxic newborns were organized in multi-sample blocks and submitted to immunohistochemical reactions by the immunoperoxidase technique. The primary antibodies used were anti-B-cell lymphoma 2 (Bcl2)-associated X protein (BAX), anti-mitofusin-2 (Mfn2), anti-tumor necrosis factor receptor-associated protein 1 (TRAP1), anti-Bcl2, anti-angiotensin II, anti-serine/threonine (Akt) 1, anti-Akt2 and anti-Akt3. Tissue immunostaining data were correlated with clinical data (gender, weight, gestational age, first-minute and fifth-minute Apgar score, arterial blood pH and survival time) and pathological data (death cause and primary disease). Results: The average tissue immunostaining of BAX was 25.61%; TRAP1 was 7.86%, angiotensin II was 1.24%, Akt2 was 16.35% and Akt3 was 20.61%. The biomarkers Akt1, Mfn2 and Bcl2 presented very low or absent tissue immunostaining in most cases of this study. There was no correlation between the average biomarker tissue immunostaining and the survival time or any other clinical or pathological factor studied. Conclusion: These data seem to strengthen the coordinated action of the pro-apoptotic, anti-apoptotic and cell growth biomarkers in the myocardium of hypoxic newborns in order to determine the degree of lesion or cell death and the tissue recovery capacity.

ABSTRACT Introduction: The pathophisiology of chronic lung disease (CLD), clinically known as bronchopulmonary dysplasia is not clear. It is believed that protective mechanisms, such as the release of inflammatory mediators and the activation of apoptotic and/or proliferative processes are activated in the lung tissue of premature infants in an attempt to repair tissue injury caused by exposure to oxygen and mechanical ventilation. Objective: Assess the presence of apoptosis and cell proliferation in the lungs of premature infants with CLD, exposed to oxygen and/or mechanical ventilation, by analyzing the proteins expression: proliferating cell nuclear antigen (PCNA), phosphatase and tensin homolog (PTEN), B-cell lymphoma 2 (Bcl-2), tumor necrosis factor receptor family member (Fas), fas-associated protein with death domain (FADD), tumor necrosis factor receptor type 1-associated death domain protein (TRADD), Caspase 3 and Caspase 8. Material and methods: We analyzed 32 infants autopsies at gestational age of less than 34 weeks exposed to oxygen therapy. The study was divided into three groups: "classic" CLD, "new" CLD and "without" CLD. Immunohistochemical analysis was performed. Results and discussion: A higher proliferation rate was observed in infants with CLD suggesting that longer exposure to mechanical ventilation may stimulates cell proliferation. The PTEN and Caspase 8 expressions were higher in the "new" CLD group, compared to the "without" CLD group, indicating that the "new" CLD form is more susceptible to apoptosis. Conclusion: Apoptosis and cell proliferation are involved in the pathophisiology of CLD. The "new" CLD form is more susceptible to apoptosis, while cell proliferation is more evident in the groups with CLD.

ABSTRACT INTRODUCTION: Chronic lung disease (CLD), clinically known as bronchopulmonary dysplasia (BPD), is a major cause of morbidity in premature newborn and were submitted to oxygen therapy. OBJECTIVE: Immunohistochemical identification of inflammatory molecules in the lung tissue of premature neonates that died with CLD. METHODS: Immunohistochemical analysis of 51 samples of premature newborn lungs – grouped in: without CLD,"classic" CLD and"new" CLD. RESULTS: Neutrophil influx and the number of CD4+ and CD45RO+ cells were higher in the"classic" CLD group (p < 0.001). CONCLUSION: Our findings suggest that the inflammatory response is mediated by neutrophils and CD45RO+ and CD4+ T lymphocytes in the"classic" CLD.