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CASE: Background: CD is an inflammatory disease that predominantly affects the gastrointestinal tract and has a progressive course. Immunodeficiencies can occur by altering different components of innate or adaptive immune responses. Such changes put the patient at greater risk for infectious diseases or non-infectious complications. Among the non-infectious complications are cancer, autoimmune diseases and gastrointestinal diseases, which should be suspected in patients with recurrent infections, with clinical, radiological or histological features of the disease are atypical or in the occurrence of an unsatisfactory response to conventional therapy. OBJECTIVE: To describe a case report of a CD patient complicated with enteric alterations related to immunodeficiency. RESULTS: A 31-year-old male patient started presenting abdominal pain, diarrhea, and weight loss in October 2020. He underwent a colonoscopy that showed diffuse involvement of the colon suggestive of CD. He didn`t respond to therapy with prednisone and azathioprine and lost 40kg until January 2021, when he was referred to an IBD center. He had signs of malnutrition (eg, BMI = 15.4, Albumin = 1.8g/dL). The MRI showed diffuse thickening of the walls of the entire length of the ileum, colon and rectum, associated with submucosal edema and more intense enhancement in the inner layer. Findings compatible with nonspecific diffuse ileocolitis. After negative infectious screening, hydrocortisone, azathioprine and infliximab were prescribed, but the patient remained clinical worsening. During the 6 weeks of therapy use, the patient presented oral candidiasis, septic pyoarthritis, DNA testing for Clostridium difficile and PCR for Cytomegalovius were positive. Despite the treatment of infectious diseases, the patient continued to worsen and was considered a primary non-response to anti-TNF, opting for the initiation of Ustekinumab. There was improvement in endoscopic and fecal calprotectin, but continued progression of ileal disease and diarrhea, with no response to enteral or parenteral nutritional therapies. An investigation for immunodeficiencies was carried out and IgM/IgG deficiency was noted, and parenteral immunoglobulin was started. He presented an intestinal subocclusion, requiring a loop ileostomy. In this surgery, it was decided to send a sample of ileal tissue for diagnostic differentiation, where ileitis was observed with intense plasma cell reaction and lymphoid nodular hyperplasia, uncharacteristic of DC, which may correspond to enteropathy secondary to immunodeficiency. Despite the treatment, the patient presented fungal endocarditis, esophageal moniliasis and septic condition of undetermined origin. He remained in hospital for 120 days until complete improvement of the infectious complications. With the slow improvement in nutritional parameters, there was a reduction in the incidence of infections as well as an increase in immunoglobulins. After five months of combined nutrition and anti-interleukin therapy, the patient presented clinical improvement, weight gain and complete normalization of IgG and IgM. Ileal disease is in regression and in the colon, in endoscopic remission. CONCLUSION: We present a case report of the initial presentation of DC complicated with severe malnutrition where there was depletion of part of immunoglobulins, resulting in infectious and non-infectious complications, where ileal involvement intensified the manifestation of colonic DC.
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BACKGROUND/AIMS: Inflammatory bowel disease (IBD) are chronic entities characterized by local and systemic inflammation and may be associated with thrombosis. The aim of this study was to identify the prevalence of thromboembolic events (TEE) in hospitalized IBD patients and identify risk factors for their occurrence. METHODS: This retrospective, single-center study included patients treated at a Brazilian IBD referral unit between 2004 and 2014. Patients hospitalized for more than 48 hours due to active IBD and who did not receive prophylaxis for TEE during hospitalization were included. Patients were allocated to 2 groups: those with TEE up to 30 days or at the time of hospitalization (TEE-group) and patients without TEE (control-group). Clinical and laboratory characteristics were evaluated. RESULTS: Of 53 patients evaluated, 69,8% with Crohn's disease (CD) and 30.2% with ulcerative colitis (UC). The prevalence of TEE 30 days before or during hospitalization was 15.1%, with 10.8% in CD and 25% in UC. In the TEE group, mean serum albumin was 2.06 g/dL versus 3.30 g/dL in the control group. Patients with albumin levels below 2.95 g/dL (43.18%) had a higher risk of developing TEE (relative risk, 1.72; 95% confidence interval, 1.17-2.53) (P<0.001). CONCLUSIONS: Albumin levels were significantly lower in patients with TEE, and hypoalbuminemia was considered a risk factor for the development of TEE in this population.
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INTRODUCTION: Celiac crisis is a life-threatening complication of celiac disease that is rarely described in adults. CASE REPORT: We report the case of a 31-year-old man with celiac crisis as a first manifestation of celiac disease. The patient presented with severe diarrhea, metabolic acidosis, and electrolyte disturbances accompanied by electrocardiographic alterations. A satisfactory clinical response was obtained after the correction of electrolyte abnormalities, hydration, and nutritional support with a gluten-free diet according to recommendations for patients at high risk of refeeding syndrome. CONCLUSION: Celiac crisis generally occurs in patients with no previous diagnosis of celiac disease. The physician should therefore be aware of this diagnosis and consider celiac crisis in cases of unexplained intense secretory diarrhea, metabolic acidosis and severe electrolyte alterations in adults. The risk of refeeding syndrome should be assessed when a gluten-free diet is introduced and treatment of celiac crisis should include prevention and management of this possible complication.
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Doença Celíaca/terapia , Síndrome da Realimentação/prevenção & controle , Adulto , Doença Celíaca/complicações , Doença Celíaca/dietoterapia , Dieta Livre de Glúten , Humanos , MasculinoRESUMO
BACKGROUND: Celiac disease is described in association with several autoimmune diseases, but rarely with myasthenia gravis. CASE REPORT: We describe the case of a 31-year-old white woman with celiac disease who presented manifestations related to a hyperactive immune system, including macroamylasemia, false-positive anti-HCV, positive antinuclear antibody, and Raynaud's phenomenon. The introduction of a gluten-free diet (GFD) resolved these features, but myasthenia gravis (MG) symptoms unexpectedly occurred on that occasion. DISCUSSION: The role of a GFD in the course of autoimmune diseases has been studied and improvement has been reported in many diseases. However, there is no consensus in the literature regarding the course of neurological disorders associated with celiac disease. In the present case, a GFD did not prevent the appearance of symptoms related to myasthenia gravis. There are few reports on the association of celiac disease with myasthenia gravis and therefore little is known about the course and time of onset of myasthenia in celiac patients. The present case increases the knowledge about this unusual autoimmune neurological disease associated with celiac disease.
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Doença Celíaca/complicações , Doenças do Sistema Imunitário/complicações , Miastenia Gravis/complicações , Adulto , Doença Celíaca/sangue , Doença Celíaca/dietoterapia , Dieta Livre de Glúten , Feminino , Humanos , Miastenia Gravis/dietoterapia , Miastenia Gravis/tratamento farmacológicoRESUMO
Norwegian scabies is a highly contagious skin infestation caused by an ectoparasite, Scarcoptes scabiei var. Hominis, which mainly affects immunosuppressed individuals. Clinically, it may simulate various dermatoses such as psoriasis, Darier's disease, seborrheic dermatitis, among others. This is a case report of a 33-year-old woman, immunocompetent, diagnosed with generalized anxiety disorder (cancer phobia), who had erythematous, well-defined plaques, covered with rupioid crusts, on her neck, axillary folds, breast, periumbilical region, groin area, besides upper back and elbows, mimicking an extremely rare variant of psoriasis, denominated rupioid psoriasis.
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Psoríase/diagnóstico , Escabiose/diagnóstico , Adulto , Diagnóstico Diferencial , Feminino , Humanos , Hospedeiro Imunocomprometido , Escabiose/patologia , Índice de Gravidade de DoençaRESUMO
Norwegian scabies is a highly contagious skin infestation caused by an ectoparasite, Scarcoptes scabiei var. Hominis, which mainly affects immunosuppressed individuals. Clinically, it may simulate various dermatoses such as psoriasis, Darier's disease, seborrheic dermatitis, among others. This is a case report of a 33-year-old woman, immunocompetent, diagnosed with generalized anxiety disorder (cancer phobia), who had erythematous, well-defined plaques, covered with rupioid crusts, on her neck, axillary folds, breast, periumbilical region, groin area, besides upper back and elbows, mimicking an extremely rare variant of psoriasis, denominated rupioid psoriasis.
A sarna norueguesa é uma infestação cutânea altamente contagiosa causada pelo ectoparasita Sarcoptes scabiei var. hominis, que atinge principalmente indivíduos imunossuprimidos. Clinicamente, pode simular várias dermatoses, tais como psoríase, doença de Darier, dermatite seborréica, entre outras. O artigo relata o caso de uma mulher de 33 anos, imunocompetente, porém com diagnóstico de transtorno de ansiedade generalizada e cancerofobia, que apresentava placas bem delimitadas, sobre base eritematosa, recobertas por crostas rupioides, nas regiões cervical, axilar, mamária, umbilical, inguinal, além de dorso superior e cotovelos, mimetizando uma variante extremamente rara de psoríase, denominada psoríase rupioide.
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Adulto , Feminino , Humanos , Psoríase/diagnóstico , Escabiose/diagnóstico , Diagnóstico Diferencial , Hospedeiro Imunocomprometido , Índice de Gravidade de Doença , Escabiose/patologiaRESUMO
The case of a 52-year-old woman with a past history of thymoma resection who presented with chronic diarrhea and generalized edema is the focal point of this article. A diagnosis of Giardia lamblia infection was established, which was complicated by protein-losing enteropathy and severely low serum protein level in a patient with no urinary protein loss and normal liver function. After anti-helmintic treatment, there was recovery from hypoalbuminemia, though immunoglobulins persisted at low serum levels leading to the hypothesis of an immune system disorder. Good's syndrome is a rare cause of immunodeficiency characterized by the association of hypogammaglobulinemia and thymoma. This primary immune disorder may be complicated by severe infectious diarrhea secondary to disabled humoral and cellular immune response. This is the first description in the literature of an adult patient with an immunodeficiency syndrome who presented with protein-losing enteropathy secondary to giardiasis.
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Giardíase/complicações , Síndromes de Imunodeficiência/complicações , Enteropatias Perdedoras de Proteínas/complicações , Timoma/complicações , Neoplasias do Timo/complicações , Agamaglobulinemia/complicações , Diarreia/complicações , Feminino , Giardia lamblia/isolamento & purificação , Humanos , Pessoa de Meia-IdadeRESUMO
OBJECTIVE: a strong association has been observed between celiac disease, generally its silent clinical form, and autoimmune disorders. A potential correlation with inflammatory bowel disease has also been suggested. Anti-tissue transglutaminase antibodies have been detected in Crohn´s disease. We investigated the prevalence of celiac disease in patients with autoimmune diabetes and in Crohn´s disease patients and also evaluated the correlation between anti-transglutaminase antibody positivity and the clinical status of these diseases. METHODS: anti-tissue transglutaminase and anti-endomysium antibodies were assessed by enzyme-linked immunosorbent assay and indirect immunofluorescence, respectively. Upper digestive endoscopy and duodenal biopsy were indicated for cases with positive serology. RESULTS: anti-transglutaminase antibodies were detected in five diabetic patients (prevalence of 11.1%), only one serum sample was positive for IgG isotypes. Nine of thirty-three patients with Crohn´s disease had low positive levels for IgA anti-transglutaminase. Antiendomysium antibodies were detected only in celiac patients. Celiac disease was confirmed in all diabetic patients submitted to duodenal biopsies who presented both anti-transglutaminase and anti-endomisyum antibodies positivity. In Crohn´s disease, its clinical status and the diagnosis of celiac disease were not associated with positiveanti-transglutaminase result. CONCLUSIONS: the prevalence of celiac disease was high in diabetic patients. Anti-tissue transglutaminase antibodies were sensitive and specific markers of celiac disease in this diabetic group, while these antibodies were of limited value for celiac disease screening in patients with Crohn´s disease.
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Doença Celíaca/complicações , Doença de Crohn/complicações , Diabetes Mellitus Tipo 1/complicações , Imunoglobulina A/sangue , Imunoglobulina G/sangue , Transglutaminases/imunologia , Adolescente , Adulto , Idoso , Biomarcadores/sangue , Doença Celíaca/imunologia , Doença Celíaca/patologia , Doença de Crohn/imunologia , Doença de Crohn/patologia , Diabetes Mellitus Tipo 1/imunologia , Diabetes Mellitus Tipo 1/patologia , Duodenoscopia , Duodeno/patologia , Ensaio de Imunoadsorção Enzimática , Feminino , Técnica Indireta de Fluorescência para Anticorpo , Humanos , Mucosa Intestinal/patologia , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
BACKGROUND: An increased number of intraepithelial lymphocytes (IELs) can be the only histological feature in early stages of celiac disease (CD). This is also presented in duodenum of patients with Helicobacter pylori-associated gastritis and in autoimmune diseases. Because CD is frequently associated with type 1 diabetes mellitus, we analyzed the density of IELs in the distal duodenum of non-celiac diabetic patients associated or not with H.pylori infection. METHODS: IEL density and the presence of H.pylori were determined in biopsies of the distal duodenum and gastric antrum and body obtained from Brazilian diabetic adolescents who were negative for anti-human tissue transglutaminase and anti-endomysial. The results were compared with the histological findings of gastric and duodenal biopsies obtained from non-diabetic older children and adolescents. RESULTS: H.pylori was detected in 33.3% of diabetic patients and in 56.7% of the control group. No association was observed between the presence of H.pylori and an increased lymphocyte density in the distal duodenum in either group. Diabetic patients presented a duodenal IEL density similar to that of the control group. Lymphocytic gastritis was not identified in any of the biopsies analyzed. CONCLUSIONS: The density of IELs in the distal duodenum of diabetic adolescents did not differ from that observed in older children and adolescents without this autoimmune disease. H.pylori infection, which is frequent among adolescents from developing countries, did not modify lymphocyte density in the distal duodenum in the absence of lymphocytic gastritis.
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Diabetes Mellitus Tipo 1/patologia , Duodeno/patologia , Gastrite/patologia , Infecções por Helicobacter/patologia , Linfocitose/patologia , Adolescente , Brasil , Diabetes Mellitus Tipo 1/imunologia , Diabetes Mellitus Tipo 1/microbiologia , Duodeno/citologia , Feminino , Gastrite/imunologia , Gastrite/microbiologia , Infecções por Helicobacter/imunologia , Infecções por Helicobacter/microbiologia , Helicobacter pylori/crescimento & desenvolvimento , Helicobacter pylori/imunologia , Humanos , Imuno-Histoquímica , Contagem de Linfócitos , Linfocitose/imunologia , Linfocitose/microbiologia , MasculinoRESUMO
Relato de um caso de doença celíaca em asso»ciação com diabetes melito insulino-dependente, sarcoidose e doença auto-imune da tiróide. Exis»tem poucos relatos da associação destas quatro doenças, mas nenhum caso no Brasil. Neste caso, a dieta sem glúten foi seguida pela remissão de um nódulo sarcóide cutâneo e pelo desapareci»mento de auto-anticorpos relacionados a tiróide.
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Humanos , Feminino , Adulto , Doença Celíaca , Glutens , Sarcoidose , Autoanticorpos , Diabetes Mellitus , Glândula TireoideRESUMO
Introdução: Constipação funcional é uma das mais freqüêntes causas de consulta médica em gastroenterologiae apenas 60por cento dos casos respondem ao aumento de fibras na dieta. A lactulose é um açucar semi-sintético, cuja formulação líquida contém outros açucares, aos quais muitos de seus efeitos adversos são atribuídos. Lactulose em cristais, uma forma altamente purificada deste dissacarídeo, poderia diminuir a incidência de tais reações adversas. Objetivos: Avaliar a eficácia, segurança e tolerabilidade da lactulose em cristais como medicamento auxiliar à dieta no tratamento da constipação intestinal funcional. Casuística e métodos: Pacientes com idade de 20 a 60 anos portadores de constipação intestinal funcional foram inicialmente submetidos a dieta laxativa por três dias. Aqueles sem resposta ao tratamento dietético receberam lactulose em cristais 20g/dia por quatro semanas, além de preencher um diário sobre hábito intestinal, sintomas gastrointestinais e a freqüência do uso de suporsitórios e clisteres como tratamento alternativo. Resultados: Oitenta pacientes completaram o tratamento. Houve melhora da constipação intestinal em 74 e, destes, 77por cento referiram normalização do hábito intestinal. A média de tempo necessário para ação da droga foi de 1,5 dia, embora para cinco pacientes esse tempo tenha ultrapassado quatro dias. Reações adversas relacionaram-se ao trato gastrointestinal: 67,8por cento referiram cólicas abdominais; 63,3por cento flatulência; e 89,0por cento, náuseas, que regridiram com o decorrer do tratameno (p<0,001), o qual foi interrompido apenas em dois casos. A aceitação da medicação foi referida com muito boa ou boa por 97,5por cento dos pacientes. Conclusão: lactulose em cristais é eficaz e segura como droga auxiliar à dieta no tratamento da constipação intestinal funcional
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Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Constipação Intestinal , Lactulose , Estudos Multicêntricos como Assunto , Constipação Intestinal , Sacarose Alimentar , Dissacarídeos/administração & dosagemRESUMO
FUNDAMENTOS: A etiopatogenia da retocolite ulcerativa inespecífica (RCUI) e de suas manifestações extra-intestinais permanece em discussão, embora o envolvimento do sistema imune seja enfatizado, e uma possível participação dos neutrófilos é demonstrada pela detecção do anticorpo anticitoplasma de neutrófilo (ANCA) nessa doença inflamatória intestinal. O pioderma gangrenoso (PG) é considerado manifestação cutânea rara da retocolite ulcerativa, e o Anca também tem sido detectado nessa dermatose. OBJETIVOS: Investigar a relação entre o comportamento clínico da RCUI e o aparecimento do PG e sua associação com ANCA. CASUíSTICA E MÉTODOS: Anca foi pesquisado nos soros de oito pacientes com PG, quatro apresentando RCUI, e os outros, PG não associado a doenças sistêmicas. RESULTADOS: Não se detectou o Anca nos soros dos portadores exclusivamente de pioderma gangrenoso. Dois casos de pancolite em atividade inflamatória acompanhada de pioderma e colangite esclerosante primária (CEP) apresentaram positividade para ANCA, enquanto os soros de dois outros pacientes com RCUI e PG tiveram resultados negativos. CONCLUSÕES: A Presença de ANCA nos soros de pacientes com PG associado a RCUI e CEP sugere que a associação com CEP seja responsável pela positividade do ANCA na presente amostra.
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Cardiomiopatias/etiologia , Doença de Whipple/complicações , Anti-Infecciosos/uso terapêutico , Cardiomiopatias/tratamento farmacológico , Cardiomiopatias/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico , Doença de Whipple/tratamento farmacológico , Doença de Whipple/patologiaRESUMO
BACKGROUND/AIMS: Anti-neutrophil cytoplasmic antibody has been observed in the sera of patients with inflammatory bowel disease, but its prevalence depends on the population being studied and the method employed for its detection. METHODOLOGY: We evaluated the prevalence of antineutrophil cytoplasmic antibody by immunofluorescence assay in a series of Brazilian patients with inflammatory bowel disease: 40 patients with ulcerative colitis and 36 with Crohn's disease. We also correlated the presence of this antibody with duration of symptoms, site of the disease and inflammatory activity. Thirty healthy individuals comprised the control group. RESULTS: Anti-neutrophil cytoplasmic antibody was detected in 27.5% of the patients with ulcerative colitis, and in 14.3% of those with Crohn's colitis. Perinuclear staining pattern was the most common, but atypical and nuclear patterns were also observed. There was no correlation between the presence of this antibody and any of the studied clinical variables. No patient of the control group presented positive test. CONCLUSIONS: A positive anti-neutrophil cytoplasmic antibody test would not be helpful in the differential diagnosis between ulcerative colitis and Crohn's colitis, since it has a moderate specificity of 86% for ulcerative colitis patients.
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Anticorpos Anticitoplasma de Neutrófilos/análise , Colite Ulcerativa/imunologia , Doença de Crohn/imunologia , Adulto , Brasil , Colite Ulcerativa/diagnóstico , Colite Ulcerativa/etnologia , Doença de Crohn/diagnóstico , Doença de Crohn/etnologia , Diagnóstico Diferencial , Feminino , Imunofluorescência , Humanos , Masculino , Pessoa de Meia-Idade , Sensibilidade e EspecificidadeRESUMO
This report concerns a patient with articular and cardiac manifestations of Whipple's disease. The disease was diagnosed only when gastrointestinal symptoms had appeared, because all cardiac symptoms were attributed exclusively to myocardial bridging. After 18 months of treatment with trimethoprim-sulfamethoxazole, the patient is fully asymptomatic with a normal echocardiogram
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Humanos , Masculino , Pessoa de Meia-Idade , Cardiopatias , Doença de Whipple , Anti-Infecciosos , Vasos Coronários , Cardiopatias , Combinação Trimetoprima e Sulfametoxazol , Doença de WhippleRESUMO
BACKGROUND: The serum albumin concentration has frequently been regarded as an indicator of nutritional status, although the hypoalbuminemia may reflect an acute phase protein response during inflammation mediated by cytokines. Both hypoalbuminemia and malnutrition are observed in Crohn's disease. OBJECTIVES: To correlate the serum albumin values to disease activity and also to nutritional status in patients with Crohn's disease. PATIENTS/METHODS: Thirty six patients were studied. Nutritional status was assessed by anthropometry measures and inflammatory activity determined by Harvey's simple clinical index and erythrocyte sedimentation rate. RESULTS: No correlation was found between malnutrition and hypoalbuminemia. The serum albumin levels correlated inversely with the disease activity. Hypoalbuminemia was 100% sensitive for detection of disease activity. CONCLUSION: This study suggests that serum albumin concentration is a very sensitive marker of inflammatory activity and not good indicator of the nutritional status in Crohn's disease. It is necessary a suitable laboratorial parameter for routine nutrition assessment in patients with this inflammatory bowel disease.
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Humanos , Masculino , Feminino , Adulto , Doença de Crohn , Albumina Sérica , Biomarcadores , Distribuição de Qui-Quadrado , Inflamação , Avaliação Nutricional , Distúrbios Nutricionais , Sensibilidade e Especificidade , Índice de Gravidade de Doença , Estatísticas não ParamétricasRESUMO
Os autores estudaram 1 024 recém-nascidos na cidade do Recife com a finalidade de observar a associaçao entre o peso ao nascer e o perímetro braquial. Observaram uma forte correlaçao entre peso e perímetro braquial (R= +0,78). A sensibilidade e especificidade deste parãmetro para detectar baixo peso também foi elevada. Concluem que o perímetro braquial possa ser utilizado como medida antropométrica alternativa para seleccionar pacientes em risco nos lugares onde não se possa medir corretamente o peso no nascimiento
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Recém-Nascido de Baixo Peso , Circunferência Braquial , Cuidado Pré-Natal , Saúde Materno-Infantil , Fatores Socioeconômicos , BrasilRESUMO
A sample of 1,024 newborns in Recife, Brazil, was studied to help determine the precision with which arm circumference measurements could be used to indicate low birth weight. The results support the view that when birth weight data are unobtainable, arm circumference measurements may be of value in screening for newborns needing special care
Published in Portuguese in Bol. Oficina Sanit. Panam 111(3):215-217, 1991