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1.
Autops. Case Rep ; 13: e2023423, 2023. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1429896

ABSTRACT

ABSTRACT Introduction Endocarditis is a rare, often fatal complication of rat bite fever caused by Streptobacillus moniliformis. Only 39 cases have been reported (including this case) as of 2022. We describe a case and aim to perform this entit's first systematic literature review. Methods We performed a systematic review in CENTRAL, EMBASE, MEDLINE, SciELO, and LILACS. The terms used were terms used were (but not limited to) rat bite fever, Streptobacillus moniliformis, Spirillum minus, and endocarditis. We included all abstracts and articles with patients with echocardiographic or histologic-proven endocarditis. In case of discordance, a third reviewer was involved. Our protocol was submitted to PROSPERO (CRD42022334092). We also performed searches for studies on the reference list of included articles. Results We retrieved 108 and included 36 abstracts and articles. A total of 39 patients (including our report) were identified. The mean age was 41.27, and 61.5% were males. The most common findings were fever, murmur, arthralgias, fatigue, splenomegaly, and rash. Underlying heart disease was present in 33%. Exposure to rats was noted in 71.8% of patients, with 56.4% recalling a rat bite. Anemia was seen in 57%, leukocytosis in 52%, and elevated inflammatory markers in 58% that had lab work performed. The mitral valve was most affected, followed by the aortic, tricuspid, and pulmonary valves. Surgical intervention was required in 14 (36%) cases. Of those, 10 required valve replacement. Death was reported in 36% of cases. Unfortunately, the literature available is limited to case series and reports. Conclusion Our review allows clinicians to suspect better, diagnose, and manage Streptobacillary endocarditis.

2.
Autops. Case Rep ; 12: e2021382, 2022. graf
Article in English | LILACS-Express | LILACS | ID: biblio-1374487

ABSTRACT

ABSTRACT Pulmonary alveolar proteinosis (PAP) is a rare lung disease with an incidence of 0.2 cases per million. PAP has multiple causes, including autoimmune, hereditary, congenital, or secondary. The latter includes hematologic conditions and exposure to different kinds of dust. Most patients present fever, dyspnea, and cough. The chest computed tomography (CT) may reveal the crazy-paving polygonal shapes with superimposed ground glass opacities delimited by thickened interlobular septa; however, this finding is more prevalent in patients with autoimmune PAP. Bronchoalveolar lavage (BAL) shows a milky-opaque appearance with PAS-positive debris on cytology. Treatment is focused on the underlying disease; however, some patients may require whole lung lavage for symptomatic management. We report a case of a 30-year-old female with a history of familial myelodysplastic syndrome (MDS) with GATA 2 mutation who presented to the outpatient clinic with several months of progressive dyspnea and nonproductive cough. The chest CT revealed bilateral ground-glass opacities prominently in the upper lobes. She underwent a bronchoscopy with lavage and biopsy, which revealed fragments of lung parenchyma with intra-alveolar coarse granular eosinophilic material strongly positive for PAS and d-PAS. The overall clinical presentation and histologic findings were diagnostic of PAP. Her GM-CSF was negative, and due to her history of MDS, secondary PAP (S-PAP) was strongly suspected. She underwent a successful allogeneic bone marrow pluripotent stem cell transplant to treat the myelodysplastic syndrome, with a follow-up chest CT showing clear lung parenchyma. The patient had resolution of symptoms about four months after the bone marrow transplant, confirming the diagnosis of S-PAP.

3.
Autops. Case Rep ; 9(1): e2018069, Jan.-Mar. 2019. ilus
Article in English | LILACS | ID: biblio-987151

ABSTRACT

Skin involvement in systemic lupus erythematosus (SLE) occurs in more than 75% of patients with this condition. Vesicles and blisters in lupus erythematosus (LE) may be present in SLE secondary to interface vacuolar changes in the epidermis, in discoid LE also secondary to vacuolar epidermal changes, and in bullous LE secondary to antibodies anti-collagen VII deposits with neutrophilic aggregates. In addition, blisters can occur due to the association of SLE with other autoimmune blistering diseases (e.g. bullous pemphigoid). BSLE is a rare blistering disease that mainly occurs in females (30­40 years old), and less frequently in children and adolescents. The most common presentation is rapid and widespread development of tense vesicles and bullae over erythematous macules or plaques. Preferential sites are: superior trunk, proximal superior limbs, and face (lips) with symmetrical distribution. Mucosal involvement is common on perioral, pharyngeal, laryngeal, and genital areas. The involvement of sun-exposed areas is not mandatory. The lesions usually progress with no scarring, but hypo or hyperchromia may be present. We report an 18-year-old female patient with blistering lesions at admission, who was diagnosed with BSLE. She was initially treated with systemic prednisone and hydroxychloroquine. Her condition evolved with relapsing lesions, which required the introduction of Dapsone. The authors emphasize the relevance of recognizing BSLE­a rare presentation of SLE­which may evolve with marked clinical presentation


Subject(s)
Humans , Female , Adolescent , Skin Diseases, Vesiculobullous , Lupus Erythematosus, Systemic/diagnosis , Blister , Rare Diseases
4.
ABCD (São Paulo, Impr.) ; 28(2): 113-116, Apr-Jun/2015. tab
Article in English | LILACS | ID: lil-751840

ABSTRACT

BACKGROUND: Association between esophageal achalasia/ gastroesophageal reflux disease (GERD) and cholelithiasis is not clear. Epidemiological data are controversial due to different methodologies applied, the regional differences and the number of patients involved. Results of concomitant cholecistectomy associated to surgical treatment of both diseases regarding safety is poorly understood. AIM: To analyze the prevalence of cholelithiasis in patients with esophageal achalasia and gastroesophageal reflux submitted to cardiomyotomy or fundoplication. Also, to evaluate the safety of concomitant cholecistectomy. METHODS: Retrospective analysis of 1410 patients operated from 2000 to 2013. They were divided into two groups: patients with GERD submitted to laparocopic hiatoplasty plus Nissen fundoplication and patients with esophageal achalasia to laparoscopic cardiomyotomy plus partial fundoplication. It was collected epidemiological data, specific diagnosis and subgroups, the presence or absence of gallstones, surgical procedure, operative and clinical complications and mortality. All groups/subgroups were compared. RESULTS: From 1,229 patients with GERD or esophageal achalasia, submitted to laparoscopic cardiomyotomy or fundoplication, 138 (11.43%) had cholelitiasis, occurring more in females (2.38:1) with mean age of 50,27 years old. In 604 patients with GERD, 79 (13,08%) had cholelitiasis. Lower prevalence occurred in Barrett's esophagus patients 7/105 (6.67%) (p=0.037). In 625 with esophageal achalasia, 59 (9.44%) had cholelitiasis, with no difference between chagasic and idiopathic forms (p=0.677). Complications of patients with or without cholecystectomy were similar in fundoplication and cardiomyotomy (p=0.78 and p=1.00).There was no mortality or complications related to cholecystectomy in this series. CONCLUSIONS: Prevalence of cholelithiasis was higher in patients submitted to fundoplication (GERD). Patients with chagasic or idiopatic ...


RACIONAL: São controversas as relações entre megaesôfago e doença do refluxo gastroesofágico (DRGE) com colelitíase, especialmente a forma mais adequada de conduzir pacientes com ambas. Dados epidemiológicos são díspares devido às diversas metodologias aplicadas, às diferenças regionais e à quantidade de pacientes envolvidos. OBJETIVO: Estudar a prevalência de colelitíase em pacientes submetidos às operações de refluxo gastroesofágico e megaesôfago (chagásicos ou não) e a segurança da colecistectomia estar associada. MÉTODO: Análise retrospectiva de 1410 pacientes operados entre 2000 e 2013. Eles foram divididos em dois grupos: os com DRGE e operados por hiatoplastia/fundoplicatura a Nissen laparoscópicas e os com acalásia por cardiomiotomia e fundoplicatura parcial laparoscópicas. Foram coletados dados epidemiológicos, diagnóstico, a presença ou não de litiase biliar, tratamento cirúrgico efetuado, complicações clínicas ou cirúrgicas e mortalidade. Todos os grupos e subgrupos foram comparados. RESULTADOS: Foram estudados 1229 pacientes portadores de megaesôfago e/ou DRGE, operados por fundoplicatura com hiatoplastia, nos casos de DRGE, e cardiomiectomia com fundoplicatura, nos casos de megaesôfago, no período de 2000 a 2013, verificando-se presença de colelítiase ou colecistectomia prévia. A colelítiase ocorreu mais no sexo feminino (2,38:1) e na faixa etária entre os 50 e 70 anos. A prevalência global foi de 11,43%; 13,08% na DRGE, menor nos portadores de esôfago de Barrett (6,67%) sendo a diferença significativa (p=0,037); e 9,44% no megaesôfago, não havendo diferença significativa entre os chagásicos e os idiopáticos (p=0,677). Não houve mortalidade ou complicações relacionadas à colecistectomia nesta série. CONCLUSÕES: A prevalência de colelitíase é maior nos pacientes com DRGE do que nos com megaesôfago. Não há diferenças na prevalência de colelitíase nos pacientes com megaesôfago chagásico e não chagásico. É mais frequente litíase ...


Subject(s)
Female , Humans , Male , Middle Aged , Cholecystectomy , Esophageal Achalasia/surgery , Gallstones/epidemiology , Gallstones/surgery , Gastroesophageal Reflux/surgery , Laparoscopy , Esophageal Achalasia/complications , Gallstones/complications , Gastroesophageal Reflux/complications , Prevalence , Retrospective Studies
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