Síndrome de Wilkie: relato de caso / Wilkie's syndrome: case report

Objetivo: Apresentar um raro caso de Síndrome de Wilkie, devido a sua dificuldade diagnostica. Relato do caso: Paciente masculino, 18 anos, em tratamento no INCA para Osteossarcoma de fêmur esquerdo, retorna ao hospital no pós-operatório, com queixa de dores epigástricas de forte intensidade e vômitos incoercíveis. Tomografia Computadorizada (TC) de abdome total evidenciou distensão gástrica até a terceira porção do duodeno, onde há redução abrupta de calibre por compressão da artéria mesentérica superior. Diagnóstico estabelecido, através dos exames de imagem, como Síndrome da Artéria Mesentérica Superior (Síndrome de Wilkie), por complicação pós-operatória da doença de base. O tratamento consistiu em uma anastomose duodenojejunal, sem intercorrências. Discussão: uma das teorias mais aceitas como fator desencadeante desta síndrome é a diminuição ou perda de gordura mesentérica, que serve como conexão entre a aorta e a artéria mesentérica superior. As causas congênitas incluem um ligamento de Treitz curto. A TC deve seguir alguns critérios para o diagnóstico, como: obstrução abrupta da terceira porção do duodeno, ângulo aorto-mesentérico e distância da aorta para artéria mesentérica reduzidos, fixação alta do duodeno pelo ligamento de Treitz (causa congênita). Conclusão: Apesar da grande quantidade de relatos de caso publicados, a Síndrome de Wilkie continua sendo de difícil diagnóstico. Ela deve ser considerada um diagnóstico diferencial em pacientes com sintomas de trato gastrointestinal alto. Os estudos de imagem constituem uma ferramenta fundamental para identificar precocemente os casos

Objective: Report a rare case of Wilkie's Syndrome, due to its diagnostic difficulty. Case Report: Male patient, 18 years old, under treatment at INCA for Osteosarcoma of the left femur, returns to the hospital postoperatively, complaining of severe epigastric pain and incoercible vomiting. Computed tomography (CT) of the abdomen revealed gastric distension up to the third portion of the duodenum, where there was an abrupt reduction in caliber due to compression of the superior mesenteric artery. Diagnosis was established, through imaging exams, as Superior Mesenteric Artery Syndrome (Wilkie's Syndrome), by postoperative complication of the underlying disease. Treatment consisted of an uneventful duodenojejunal anastomosis. Discussion: One of the most accepted theories as a triggering factor of this syndrome is the decrease or loss of mesenteric fat, which serves as a connection between the aorta and the superior mesenteric artery. Congenital causes include a short Treitz ligament. CT should follow some criteria for diagnosis, such as: abrupt obstruction of the third portion of the duodenum, reduced aorto-mesenteric angle and distance from aorta to mesenteric artery, high fixation of the duodenum by the ligament of Treitz (congenital cause). Conclusion: Despite the large number of published case reports, Wilkie's Syndrome remains difficult to diagnose. It should be considered a differential diagnosis in patients with upper GI tract symptoms. Imaging studies are a fundamental tool to identify cases early

Flucloxacillin-Induced Hepatotoxicity - Association with HLA-B*5701.

Drug-induced liver injury (DILI) to flucloxacillin is rare and is classified as idiosyncratic, as it is dependent on individual susceptibility, unpredictable, and dose-independent. The authors present the case of a 74 - year - old man with a history of monoclonal gammopathy under investigation and alcoholic habits of 24 g/day, with asthenia, anorexia, nausea, abdominal discomfort, and fever with three days of evolution. He was treated with two courses of antibiotic therapy with flucloxacillin to erysipelas previously (3 months and 2 weeks before admission). Lab tests showed serum AST levels of 349 U/L, ALT 646 U/L, alkaline phosphatase 302 U/L, GGT 652 U/L, total bilirubin 3.3 mg/dL and direct bilirubin 2.72 mg/dL. Infectious, autoimmune, and metabolic causes were ruled out. Magnetic resonance cholangiopancreatography showed normal results. Liver biopsy showed mild multifocal (predominantly microvesicular) steatosis; marked changes in the centrilobular areas (sinusoidal dilatation, marked congestion, hemorrhage, and multifocal hepatocyte collapse); expansion of the portal areas with the formation of bridges; proliferated bile ducts and inflammatory infiltrate of variable density, predominantly mononuclear type. The HLA-B*5701 screening test was positive. Hepatic biochemical tests remain abnormal with a significative increase in total bilirubin, which reached levels of 24.1 mg/dL, with the development of jaundice, pruritus, and choluria. DILI was assumed, and the patient was treated with ursodeoxycholic acid. There was favorable evolution, without evidence of blood coagulation dysfunction or encephalopathy. The analytic normalization was, however, slow, with evolution to chronicity. The authors present this case to remind the possibility of moderate/severe drug-induced liver injury to flucloxacillin, an antibiotic commonly used in clinical practice and association with the HLA-B * 5701 allele reported in the literature.

Flucloxacillin-Induced Hepatotoxicity - Association with HLA-B*5701

SUMMARY Drug-induced liver injury (DILI) to flucloxacillin is rare and is classified as idiosyncratic, as it is dependent on individual susceptibility, unpredictable, and dose-independent. The authors present the case of a 74 - year - old man with a history of monoclonal gammopathy under investigation and alcoholic habits of 24 g/day, with asthenia, anorexia, nausea, abdominal discomfort, and fever with three days of evolution. He was treated with two courses of antibiotic therapy with flucloxacillin to erysipelas previously (3 months and 2 weeks before admission). Lab tests showed serum AST levels of 349 U/L, ALT 646 U/L, alkaline phosphatase 302 U/L, GGT 652 U/L, total bilirubin 3.3 mg/dL and direct bilirubin 2.72 mg/dL. Infectious, autoimmune, and metabolic causes were ruled out. Magnetic resonance cholangiopancreatography showed normal results. Liver biopsy showed mild multifocal (predominantly microvesicular) steatosis; marked changes in the centrilobular areas (sinusoidal dilatation, marked congestion, hemorrhage, and multifocal hepatocyte collapse); expansion of the portal areas with the formation of bridges; proliferated bile ducts and inflammatory infiltrate of variable density, predominantly mononuclear type. The HLA-B*5701 screening test was positive. Hepatic biochemical tests remain abnormal with a significative increase in total bilirubin, which reached levels of 24.1 mg/dL, with the development of jaundice, pruritus, and choluria. DILI was assumed, and the patient was treated with ursodeoxycholic acid. There was favorable evolution, without evidence of blood coagulation dysfunction or encephalopathy. The analytic normalization was, however, slow, with evolution to chronicity. The authors present this case to remind the possibility of moderate/severe drug-induced liver injury to flucloxacillin, an antibiotic commonly used in clinical practice and association with the HLA-B * 5701 allele reported in the literature.

RESUMO A hepatotoxicidade à flucloxacilina é rara e classifica-se como idiossincrática, uma vez que é dependente da suscetibilidade individual, não expectável e independente da dose. Apresentamos o caso de um homem, 74 anos, antecedentes de gamapatia monoclonal e hábitos alcoólicos de 24 g/dia, com quadro de astenia, anorexia, náuseas, desconforto abdominal e febrícula com três dias de evolução. Referência a dois ciclos de antibioterapia com flucloxacilina por erisipela (três meses e duas semanas antes da admissão). Analiticamente com AST 349 U/L, ALT 646 U/L, FA 302 U/L, GGT 652 U/L, bilirrubina total 3,3 mg/dL, bilirrubina direta 2,72 mg/dL. Excluídas etiologias infecciosa, autoimune, metabólica, bem como patologia das vias biliares por colangio-RM. Biópsia hepática mostrou esteatose multifocal ligeira (predominantemente microvesicular); alterações acentuadas nas áreas centrolobulares (dilatação sinusoidal, congestão acentuada, hemorragia e colapso multifocal de hepatócitos); expansão das áreas portais com constituição de pontes; ductos biliares proliferados e infiltrado inflamatório de densidade variável, predominantemente de tipo mononucleado. Tipagem de HLA-B*5701 positiva. Agravamento analítico atingindo bilirrubina total 24,1 mg/dL, com desenvolvimento de icterícia, prurido e colúria. Admitida a hepatotoxicidade, iniciou terapêutica com ácido ursodesoxicólico. Verificou-se evolução favorável, sem evidência de coagulopatia ou encefalopatia. A normalização analítica foi, no entanto, lenta, com evolução para cronicidade. Os autores apresentam este caso para alertar para a possibilidade de hepatotoxicidade moderada a grave à flucloxacilina, antibiótico de uso comum na prática clínica e associação com o alelo HLA-B*5701 relatada na literatura.

Essential thrombocythemia - a predisponent factor for stroke.

The essential thrombocythemia is one of the seven described forms of myeloproliferative neoplasms. It is characterized by megakaryocytic hyperplasia with consequent thrombocytosis maintained in the peripheral blood, favoring the occurrence of thrombo-hemorrhagic phenomena. We present the case of an 81-year-old woman with a history of ischemic stroke in the context of a sustained thrombocytosis, which led to a spinal study and a search for the V617F mutation in the JAK2 gene, which was positive. The patient started cytoreductive therapy with hydroxyurea with favorable current evolution.

Essential thrombocythemia - a predisponent factor for stroke

SUMMARY The essential thrombocythemia is one of the seven described forms of myeloproliferative neoplasms. It is characterized by megakaryocytic hyperplasia with consequent thrombocytosis maintained in the peripheral blood, favoring the occurrence of thrombo-hemorrhagic phenomena. We present the case of an 81-year-old woman with a history of ischemic stroke in the context of a sustained thrombocytosis, which led to a spinal study and a search for the V617F mutation in the JAK2 gene, which was positive. The patient started cytoreductive therapy with hydroxyurea with favorable current evolution.

Olmesartan-Associated Enteropathy: An Unexpected Cause of Chronic Diarrhoea.

Olmesartan-associated enteropathy is a rare cause of severe enteropathy that should be considered in the differential diagnosis of patients with unexplained chronic diarrhoea. It may be difficult to recognise because of its clinical and histologic similarities to other clinical entities. The authors present the case of a 72-year-old woman with a 6-month clinical history of non-bloody diarrhoea and weight loss. Discontinuation of olmesartan resulted in clinical and histologic recovery, and therefore, physicians need to be aware of olmesartan-associated enteropathy in order to avoid unnecessary testing. Although rare, it is considered an emerging and underdiagnosed enteropathy. LEARNING POINTS: Olmesartan-associated enteropathy is characterised by chronic diarrhoea (often severe) and weight loss that is unresponsive to a gluten-free diet.When a patient presents with unexplained chronic diarrhoea, a detailed medication review is needed. If duodenal biopsies reveal villous atrophy and coeliac disease is excluded, drug-induced enteropathy is likely.Clinical response and histologic improvement are expected after olmesartan is withdrawn.

Morpho and Cytological Differentiation of Calli of Eucalyptus grandis x Eucalyptus urophylla During Somatic Embryogenesis

ABSTRACT The aim of this study was to induce and analyze embryogenic calli from two types of explants (leaves and meristems) of the hybrid Eucalyptus grandis x Eucalyptus urophylla. Leaves and meristems of plants kept in a nursery were disinfected and inoculated in Petri dishes containing MS culture medium supplemented with different concentrations of the growth regulator dicamba (1.13, 4.52, and 9.04 µM) and without it. At 60 days of culturing, the calli were analyzed by scanning electron microscopy and at 90 days were evaluated by light microscopy in regard to the embryogenic characteristics of the cells. Different type of calli were induced in leaf explants, designated as Type I with light yellow coloring, Type II with dark yellow coloring, and Type III of brown coloring; however, only Type I had embryogenic characteristics. In the meristematic explants, only one type of callus was induced, and it had embryogenic characteristics. At 90 days of culturing, the formation of somatic embryos in the different embryogenic stages was observed and the formation of procambium, protoderm, and ground meristem tissues. At 150 days of culturing, the concentration of 1.13 µM of dicamba was prominent in the formation of somatic embryos in the different embryogenic stages.