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.

Primary small cell carcinoma of the vagina.

The primary small cell carcinoma of the vagina is rare, and it is a highly aggressive malignancy with no consensus regarding the treatment of this tumor. The survival rate for patients treated in the early stages is around two years. We related the case report of a patient of 41 years with a vegetative and necrotic lesion in left vaginal wall, in middle and upper third, and involvement of parametrium in its proximal third and medium third. A biopsy showed a small cell undifferentiated carcinoma composed of epithelial cells with round nuclei, oval or elongated, hyperchromatic nuclei, with little distinct nucleoli, and scarce cytoplasm. Immunohistochemistry showed positivity for AE1/AE3, CD57, and chromogranin A. The patient received 6 cycles of chemotherapy with cisplatin and etoposide and radiotherapy, achieving complete response, with complete regression of the lesion. The patient had no sign of tumor recurrence and locoregional or distant metastases after 5 months of followup.

Cellular fibroma of the ovary with multiloculated macroscopic characteristics: case report.

Ovarian fibroma is the commonest benign tumor of the ovarian stroma. The cellular subtype accounts for around 10% of ovarian fibromatous tumors. The cellular fibroma is a tumor of uncertain malignant potential that may recur or be associated with peritoneal implants. Usually these are solid tumors, sometimes with small areas of cystic degeneration. This case is reported to highlight an unusual feature for an ovarian fibroma: the tumor was predominantly cystic with a small solid part; the multiple cavities contents consisted of viscous liquid that solidified under room temperature. The multiloculated cysts, the mucinous contents, and the solid areas simulated a borderline mucinous ovarian tumor on both CT scan and gross pathologic examination.

Morphologic analysis of fetal stress organsin different causes of perinatal death.

Complications act as stress-inducers during pregnancy so the fetus can develop functional compensatory mechanisms or morphologic changes. The cases analyzed are with congenital malformations or acute stress; chronic included cases with ascending infection (AI) and perinatal hypoxia/anoxia (PHA). The hematoxylin-eosin (H&E) was done to analyze the vacuolization, and the immunohistochemistry to the phagocytosis. The discreet standard of vacuolization was observed in 52.6% of the cases, 22.1% moderate, and 25.3% severe. The number of macrophages was higher in PHA. Changes in these organs are closely related to the cause of death and to the period during which the harmful agent.

Thickness of the cervical epithelium of autopsied patients with acquired immunodeficiency syndrome.

Several general pathologic processes that affect the uterine cervix have been described in Brazil and in the world. The aim of the current study was to evaluate morphometrically the cervical epithelium of autopsied patients with AIDS. This is a cross-sectional study of the cervix of 38 women autopsied from 1993 to 2003. We evaluated the cervix of women aged 20 to 40 years, 20 with AIDS (52.63%) and 18 without (47.37%). The inflammatory infiltrate found in the AIDS patients was more accentuated and predominantly composed of monocytes, which is characteristic of chronic inflammation. In addition, necrosis, fibrosis, and metaplasia were also more frequent in these patients. The cervical epithelium was considerably thinner in the AIDS patients. This hypotrophy occurred because of a significantly accentuated reduction in the number and surface area of cells (P < .01). There was still a significant correlation between the thickness of the epithelium and the nutritional status of AIDS patients. General pathologic processes, which affect the cervices of patients with AIDS, especially hypotrophy, could facilitate the penetration of other infectious agents. We suggest that the nutritional status of these patients and the adherence to antiretroviral therapy be routinely evaluated and the use of condoms stimulated even between HIV-positive partners to prevent possible increases in the viral load and reinfections that contribute to the aggravation of the disease.

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.

Bronchogenic carcinoma of squamous cells in a young pregnant woman.

Lung cancer continues to be the most incident neoplasia in the world and also the most frequent cause of death by cancer, with more than 900000 new cases per year. Women present greater risk of developing bronchogenic carcinoma, possibly because of differences in metabolism of the carcinogens associated with tobacco and also because of hormonal differences because estrogens can promote the appearance of lung cancer, including during pregnancy. We have described the case of a 25-year-old patient who developed squamous cell carcinoma of the lung during her third pregnancy, with a progressive worsening of her general condition and progression until death. We believe that the pregnancy could have contributed to the evolution of the disease, especially because of the increased levels of gestational hormones, particularly the estrogen, because its receptors have an important role in regulating growth and in the differentiation of several tissues facilitating like this, the development of the neoplasia, and complicating its early diagnosis.

The Lanz endotracheal tube decreases tracheal injury in dogs.

PURPOSE: To determine, in dogs anesthetized with nitrous oxide (N2O), (whether the endotracheal tube (ETT) cuffed with a Lanz pressure regulating valve decreases the tracheal consequences of tracheal intubation. METHODS: Sixteen mixed-breed dogs were allocated to two groups according to the ETT used: Control group (n = 8) - Rüsch ETT, and Lanz group (n = 8) - ETT with Lanz pressure regulating valve. The ETT cuffs in both groups were inflated with air to an intracuff pressure of 30 cm H2O. Anesthesia was induced and maintained with pentobarbitone and N2O (1.5 L x min(-1)) and O2 (1 L x min(-1)). ETT cuff pressures were measured before (control) and 60, 120, and 180 min during N2O administration. The dogs were sacrificed, and biopsy specimens from four predetermined areas of the tracheal mucosa in contact with the ETT were collected for light and scanning electron microscopy (SM) examination. RESULTS: Cuff pressures in the Control group were higher than in the Lanz group at all time points studied (P < 0.001), with an increase over time only in the Control group (P < 0.001). Median neutrophilic inflammatory infiltration values of the epithelial surface, and in the subepithelial layer in contact with the cuff, were higher in the Control group as compared to the Lanz group (3.0 vs 1.0 and 3.0 vs 1.5 respectively) (P < 0.05). On SM examination, median histological grades were higher in the Control group compared to Lanz group (2.9 vs 1.9 respectively), (P < 0.05). CONCLUSIONS: The Lanz ETT decreases tracheal mucosal injury in dogs.

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

This report describes the presence of Isospora belli unizoic cysts in mesenteric lymph nodes and of gametocytes in the gallbladder epitelium of a 26 year-old Brazilian male patient with Acquired Immune Deficiency Syndrome. This patient had received treatment for several times with sulfamethoxazole-trimethoprim. It is discussed the significance of I. belli tissue cysts as possible foci of resistance of the parasite and their association with the infection relapse even post-treatment with anticoccidian medication.

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.