Hipobetalipoproteinemia primária: relato de caso / Primary Hypobetalipoproteinemia: a case report

Descrevemos o caso de uma paciente de 19 anos diagnosticada com hipobetalipoproteinemia primária. A paciente apresentava sintomas compatíveis com a doença como diarreia desde o primeiro mês de vida, défice de crescimento e retinopatia. A biópsia duodenal evidenciou presença de vacúolos lipídicos intraepiteliais, os quais foram altamente sugestivos para o diagnóstico. Os exames complementares evidenciaram disfunção hepática, baixos níveis séricos de triglicerídeos, e de colesterol total e frações. Após a dosagem de apolipoproteína B abaixo dos valores da normalidade, aliada a clínica e exames complementares, o diagnóstico foi realizado. A relativa escassez de dados na literatura em nosso meio, atrelada à raridade da doença, ilustra a relevância deste relato de caso, somado à importância do diagnóstico precoce

The case of a 19-year-old female patient who was diagnosed with Primary Hypobetalipoproteinemia (HBL) is described.The patient presented symptoms that were consistent with the disease, such as diarrhea from the very first month of life, growth failure and retinopathy. The duodenal biopsy showed the presence of intraepithelial lipid vacuoles that were highly suggestive of the diagnosis. Further tests showed liver dysfunction, low serum levels of triglycerides and total cholesterol and fractions. After the dosage of Apolipoprotein B below normal values, and clinical exam along with laboratory tests, the diagnosis was made. The lack of data in the literature and the rarity of the disease illustrate the importance of this case report,and of an early diagnosis.

Granulomatose linfomatóide-relato de caso / Lymphomatoid granulomatosis -case report

Objetivo: descrever um caso com manifestaçõs clínicas e achados histopatológicos de Granulomatose Linromatóide (GL), uma rara doença linfoproliferativa das células B, angiocêntrica e angiodestrutiva associada ao vírus Epstein-Barr (EBV). A apresentação clínica mais comum é no pulmão, podendo acometer qualquer órgão, sendo os achados extra-pulmonares as principais manifestações nestes casos. Relato do caso: mulher de 65, com lesão cutânea vulvar e que evoluiu com quadro neurológico associado a lesão expansiva envolvendo parênquima cerebelar, sem alterações pulmonares. Considerações Finais: pelo seu prognóstico e capacidade de ser confundida com outras vasculites a GL impõe-se como um importante diagnóstico diferencial dessas doenças.

lntroduction: this report describe a case of Lymphomatoid Granulomatosis (LG,s a rare linfoproliferative disorder of B cell showing angiocentric growth and destructive behaviour caused by Epstain-Barr virus (EBV). The predominant clinical presentations are confined to the pumonary system, however ; extra-pumonary manifestations can sometimes be the main feature of the dieseses. Method: here we report a 65 year old female, with a skin vulvar lesion, and another in cerebelus, without pulmonary disease. Conclusion: the LG can mimics systemic vasculits and is a diagnostic challenge.

Presença extra-intestinal de cistos unizóicos de Isospora belli em paciente com SIDA: relato de caso / Extraintestinal finding of Isospora belli unizoic cysts in a patient with AIDS: case report

Descreve-se a presença de cistos unizóicos de Isospora belli em linfonodos mesentéricos e de gametócitos no epitélio da vesícula biliar de um paciente brasileiro de 26 anos de idade, com a Síndrome da Imunodeficiência Adquirida que recebeu tratamento, por diversas vezes, com sulfametoxazol-trimetoprim. Discute-se a importância dos cistos teciduais de I. belli como possíveis focos de resistência do parasita e a associaçäo destes a episódios de recidivas da infecçäo mesmo após tratamento com medicaçäo anticoccídios

Frequency of Trichomonas vaginalis, Candida sp and Gardnerella vaginalis in cervical-vaginal smears in four different decades

CONTEXT: Vaginitis is one of the principal motives that lead women to seek out an obstetrician or gynecologist. Bacterial vaginosis, candidiasis and trichomoniasis are responsible for 90 percent of the cases of infectious vaginitis. OBJECTIVE: To verify the frequency of the three main causative agents of vaginitis, Trichomonas vaginalis, Candida sp and Gardnerella vaginalis, in four different decades (1960's, 1970's, 1980's and 1990's). DESIGN: Retrospective. PLACE: A tertiary referral center. PARTICIPANTS: Patients attended to as gynecology and obstetrics outpatients at the Faculdade de Medicina do Triângulo Mineiro during the years 1968, 1978, 1988, 1998, taken as samples of each decade. MAIN MEASUREMENTS: Diagnoses of infection by Trichomonas vaginalis, Candida sp and Gardnerella vaginalis were gathered from 20,356 cervical-vaginal cytology tests on patients attended to as gynecology outpatients at Faculdade de Medicina do Triângulo Mineiro during the years 1968, 1978, 1988, 1998, representing the four decades. The results were grouped according to the age group of the patients: under 20, between 20 and 29, between 30 and 39, between 40 and 49, and 50 or over. Statistical analysis was done via the chi-squared (Mantel-Haentzel) test with a significance level of 5 percent. RESULTS: In 1968 infections by Trichomonas vaginalis and Candida sp were diagnosed in 10 percent and 0.5 percent of the cytology tests and in 1978, 5.1 percent and 17.3 percent, respectively (P < 0.0001). Infection by Gardnerella vaginalis could only be evaluated in the latter two decades. In 1988, 19.8 percent of the women had positive tests for Gardnerella vaginalis, which was the most frequent agent in that year, diminishing in the subsequent decade to 15.9 percent (P < 0.0001). Candidiasis was the most frequent infection in 1998, detected in 22.5 percent of the tests (P < 0.0001). In a general manner, all the infections were most frequent among younger patients, especially those aged under 20, in all decades, whereas infections were least frequent among patients aged 50 or over (P < 0.05). CONCLUSION: There was a reduction in the frequency of cervical-vaginal infection by Trichomonas vaginalis and an increase in the frequency of Candida sp over the four decades studied. All the infections were most frequent in patients aged under 20 years

Morphologic and morphometric evaluation of pancreatic islets in chronic Chagas' disease

PURPOSE: Hyperglycemia and abnormal glucose tolerance tests observed in some patients with chronic Chagas' disease suggest the possibility of morphological changes in pancreatic islets and/or denervation. The purpose of this study was to describe the morphology and morphometry of pancreatic islets in chronic Chagas' disease. METHODS: Morphologic and computerized morphometric studies were performed in fragments of the head, body, and tail regions of the pancreas obtained at necropsies of 8 normal controls and 17 patients with chronic Chagas' disease: 8 with the digestive form (Megas) and 9 with the congestive heart failure form. RESULTS: The Megas group had a larger (p < 0.05) pancreatic islet area in the tail of the pancreas (10649.3 + or - 4408.8 æm²) than the normal control (9481.8 + or 3242.4 æm²) and congestive heart failure (9475.1 + or 2104.9 æm²) groups; likewise, the density of the pancreatic islets (PI) was greater (1.2 + or 0.7 vs. 0.9 + or - 0.6 vs. 1.9 + or - 1.0 PI/mm², respectively). In the tail region of the pancreas of patients with the Megas form, there was a significant and positive correlation (r = +0.73) between the area and density of pancreatic islets. Discrete fibrosis and leukocytic infiltrates were found in pancreatic ganglia and pancreatic islets of the patients with Chagas' disease. Trypanosoma cruzi nests were not observed in the examined sections. Individuals with the Megas form of Chagas' disease showed increased area and density of pancreatic islets in the tail of the pancreas. CONCLUSION: The observed morphometric and morphologic alterations are consistent with functional changes in the pancreas, including glycemia and insulin disturbances

Widespread hematogenous metastases and Trousseau's syndrome in gastric adenocarcinoma

A case of widespread hematogenous metastases and Trousseau's syndrome is reported in a 40 year-old white housewife with gastric cancer, presenting subdural hematoma, ecchymoses, epistaxis, stomach and uterine bleeding. After undergoing hematoma drainage, she was unsuccessfully treated with platelets, red blood cells, plasma cryoprecipitate transfusions, and antibiotics. Necropsy disclosed gastric ring-signet adenocarcinoma invading the serous layer, with massive disseminated intravascular coagulation and systemic neoplastic embolism. Multiple old and recent hyaline (rich in fibrin and platelets) microthrombi, and tumor emboli were observed in the bone marrow, meninges, liver, lungs, kidneys, lymph nodes, adrenals, thyroid, heart, pancreas, and ovaries (Krukenberg tumor)

Pulmonary adiaspiromycosis: report of two cases / Adiaspiromicose pulmonar: relato de dois casos

Relatam-se casos de adiaspiromicose pulmonar em homens brancos de 29 e 54 anos, lavradores, com sintomas "gripais" e achados radiológicos sugestivos de doença granulomatosa intersticial. Biópsias transbrônquicas e transtoracoscópicas foram realizadas. A funçäo pulmonar foi avaliada por espirometria. Um paciente usou cetoconazol. Enfatizam-se possibilidades de confusäo diagnóstica na adiaspiromicose pulmonar

Contribuiçäo ao diagnóstico morfológico da adiaspiromicose pulmonar / Contribution to morphologic diagnosis of pulmonary adiaspiromycosis

Adiaspiromicose é usualmente diagnosticada em tecido pulmonar corado por hematoxilina-eosina, ácido periódico Schiff e prata-metenamina. Os autores descrevem a morfologia do fungo corado pelo mucicarmim, picro-sírius e vermelho Congo, inclusive à luz polarizada. Tratando-se de diagnósticos duvidosos, essas técnicas poderiam facilitar na diferenciaçäo entre Emmonsia parva var crescens e outros agentes

Cyto-histological correlation of 219 patients submitted to surgical treatment due to diagnosis of cervical intraepithelial neoplasia

Cervical cytology continues to be the mostappropriate method for investigating cervical neoplasia and its precursors. Greater diagnostic acuity is obtained by combining cytology, colposcopy and guided biopsy methods. Objective: To analyze the diagnostic acuity of cyto-and histopathological exams and causes of diagnostic error. Design: Retrospective study. Setting: A public tertiary referral center. Sample: Reports on 219 patients submitted to cone biopsy and/or hysterectomy due to diagnosis of cervical intraepithelial neoplasia (CIN) in the period between January 1982 and March 1997 were reviewed, comparing. Main Measurements: cytological and histological exams (guided biopsy and surgically-removed tissue). In cases of discordance, the cyto-and histological preparations were reviewed to try to evaluate the causes of errors. Results: In 193 cases (88.1 per cent) there was cyto-histological agreement but none in 26 (11.9 per cent). Review of the discordant cases showed that in 2 (0.9 per cent) there was invasion of the stromata to a depth greater than 3 mm, and in 7 (3.2 per cent) microinvasion, unsuspected via cytology; in 2 (0.9 per cent) microinvasion was suspected via cytology but not confirmed by the final histological exam; and in 15 (6.8 per cent) there was disagreement about the degree of CIN. Conclusion: The principal causes of error in the cytological exam were the lack of reliable morphological criteria for microinvasion, absence of sampling of the squamocolumnar junction, and scarcity of neoplastic cells in the sample. As for the histological exam, the errors were related to inadequate technical processing and underestimation of focal lesions.

Linfoma MALT gástrico em paciente jovem / Gastric lymphoma MALT in an young patient

É descrito um caso incomum de linfoma de tecido linfóide associado à mucosa (MALT) gástrico em paciente de 25 anos, que evoluiu com vários episódios de hematêmese, resultando em choque hipovolêmico, necrose tubular aguda e óbito. Embora o paciente tenha cursadocom gastrite, a pesquisa para Helicobacter pylori foi negativa. A descrição se justifica tanto pela evolução rápida da neoplasia, quanto pela faixa etária do paciente. Além disso, a descrição deste tipo de linfoma colabora no aumento da casuística documentada por exame necroscópico na literatura biomédica.

An uncommon case of the mucosa associated lymphoid tissue (MALT) gastric lymphoma is described in 25 year-old patient, that developed with several hematemesis episodes, resulting in hipovolemic shock, acute tubular necrosis and death. Although the patient had gastritis, the research for Helicobacter pylori in the stomach was negative. These paper is justified by the fast evolution of the neoplasm, and the patient's age group. In addition, the descrition of this lymphoma type collaborates in the increase of the casuistic documented by autopsies in the biomedical literature.

Persistência da infecçäo por papilomavírus humano: análise da idade, sexarca, cor, hábito de fumar e método contraceptivo / Persisting human papillomavirus infection: analyse of age, sexarch, race amd cigarette smoking and contraceptive methods

O objetivo deste estudo foi analisar um grupo de mulheres com alteraçöes citológicas compatíveis com infecçäo por papilomavírus humano (HPV), estudando a evoluçäo desta infecçäo, na tentativa de correlacionar com aspectos epidemiológicos como idade, sexarca, métodos contraceptivos, cor e hábito de fumar. Analisaram-se 507 mulheres entre janeiro a dezembro de 1991 com seguimento de 39 a 183 meses. Os resultados demonstraram que 66 (13 por cento) das mulheres permaneceram com sinais citológicos de infecçäo por HPV ou evoluíram para lesäo de alto grau após um período médio após um período médio de 48 meses. As outras 441 (87 por cento) das mulheres näo apresentaram nenhum sinal de infecçäo por HPV após esse tempo. Nossos dados mostraram que näo houve diferença estatísticamente significante entre o grupo de mulheres que permaneceu com a infecçäo e o grupo no qual os sinais citológicos de HPV näo foram detectados com relaçäo aos parâmetros avaliados

Histiocitose de células de Langerhans no ísquio: relato de caso / Langerhans' cell histiocytose of the left ischium: case report

Os autores descrevem, em criança do sexo masculino de três anos, um caso de histiocitose de células de Langerhans no ísquio esquerdo. Realçam a pouca freqüência da lesäo nesta localidade, discutem o diagnóstico diferencial e a conduta adotada.

Paracoccidioidomicose do estômago: relato de caso e consideraçöes sobre a patogenia desta lesäo / Gastric paracoccidiodomycosis: a case report and considerations on the pathogenesis of the disease

Relata-se um caso incomum de lesäo fúngica gástrica em pacientes com a forma crônica de paracoccidioidomicose (blastomicose sul-americana). Durante um período de 8 meses os sintomas principais do paciente foram dor abdominal e perda de peso. A esofagogastroduodenoscopia mostrou uma gastrectomia parcial feita previamente, uma úlcera grande e irregular no estômago e aspecto granuloso da mucosa duodenal. No exame histopatológico do tecido retirado por biopsia do estômago e do duodeno observou-se processo granulomatoso e o Paracoccidioides brasiliensis. A cicatrizaçäo da úlcera gástrica foi verificada em nova avaliaçäo endoscópica feita após 4 meses de tratamento com sulfadiazina. Sugere-se que o comprometimento de gânglios linfáticos abdominais, a infecçäo duodenal e também alteraçöes anatômicas e tróficas do estômago, sejam fatores predisponentes da paracoccidioidomicose gástrica