Double-Positive Anti-GBM and ANCA-MPO Vasculitis Presenting With Crescentic Glomerulonephritis.

We report the case of a 61-year-old man with rapidly progressing glomerulonephritis (RPGN) due to double-positive anti-neutrophil cytoplasmic antibodies (ANCA) and anti-glomerular basement membrane antibodies (GBM) vasculitis. The past medical history included stable untreated psoriatic arthritis and arterial hypertension. He presented with asthenia, anorexia, and rapidly deteriorating renal function with metabolic acidosis and hyperkalemia evolving with the need for hemodialysis. No nephrotoxic drugs were identified. Urinalysis showed proteinuria, erythrocyturia, and mild leukocyturia with no pathological casts and renal ultrasound excluded obstruction as the cause of the acute kidney injury. The subsequent study established the diagnosis of double-positive ANCA and anti-GBM vasculitis with renal biopsy confirming the presence of crescentic glomerulonephritis. The patient was started on corticosteroids, cyclophosphamide, and plasmapheresis with the improvement of symptoms and decrease of antibody titers. The renal function recovery was not obtained and referral for transplantation is ongoing.

Pheochromocytoma: a retrospective study from a single center.

Objectives. Pheochromocytoma (PCC) is a neuroendocrine tumor derived from chromaffin tissue more frequently found in the adrenal medulla. Many discoveries over the last decade have significantly improved our understanding of PCC.Methods. We retrospectively reviewed all patients with a histological diagnosis of PCC at the Centro Hospitalar Universitario de Sao Joao, a tertiary and university hospital in Oporto, Portugal, between January 2009 and December 2017.Results. The study group included 33 patients. In most cases the diagnosis was suspected with more than half of patients presenting with hypertension and the third diagnosed during the work-up of an adrenal incidentaloma. About half of the patients was referred for genetic testing and 6 patients had a positive inherited susceptibility genetic pathogenic variant associated with classic cancer predisposition syndromes and also associated with newly described genes. In the incidentaloma group, genetic testing was performed in 3 (9%) patients with only 1 positive result. In the suspected group, 15 (45%) genetic tests were performed.Conclusions. In contrast to other studies, where only a minority of patients with PCC were referred for genetic counselling, in our study 54% of patients was referred for genetic testing. This study suggests that clinicians were correctly recognizing the need to refer young patients and patients with positive family history. However, opportunities for genetic testing are frequently missed due to low referral rates in patients with apparently sporadic PCC, particularly older than 30 years old. It is imperative that all the providers involved in the multidisciplinary care of patients with pheochromocytomas are aware of the genetic disorders associated with these unique tumors.

Severe neutropenia and thrombocytopenia in a young adult: a case-report

We present the case of a 20-year-old male hospitalized with severe neutropenia and thrombocytopenia. After exclusion of other etiologies, it was concluded to be of autoimmune etiology. The prompt and sustained response of both cell lines to corticosteroids, reinforced this diagnosis. This case illustrates a very rare association of severe primary neutropenia and thrombocytopenia. The lack of serological marker makes this diagnosis delayed and resource-consuming

No disponible

Schizophyllum commune sphenoidal sinusitis as presentation of a non-Hodgkin Lymphoma.

Schizophyllum commune is a basidiomycetes worldwide distributed that has emerged as cause of invasive infections in immunosuppressed patients. We present a case of a man who was simultaneously diagnosed with a small cell non-Hodgkin lymphoma and a sphenoid sinusitis by S. commune. Intraoperative observation and histology description were crucial to consider an alternative diagnosis to mucormycosis suggested by the MRI. The diagnosis was made based on PCR identification and sequencing.

Encephalopathy in a kidney transplant recipient.

A 60-year-old man presented several times to the emergency department due to confusion and behavioral changes. He was a kidney transplant recipient dependent on hemodialysis due, presumably, to chronic nephropathy of the transplanted kidney, and was not under any immunosuppressive therapy. He was admitted to the hospital ward due to elevation of C reactive protein and severe proteinuria, leukocyturia and erythrocyturia. The alterations found in the spot urine examination were suggestive of nephritic syndrome, consistent with chronic nephropathy of the transplanted kidney. The neurologic deterioration, however, remained unexplained. CT of the brain and cerebrospinal fluid examination were unremarkable. Infection, auto-immune disease and malignancy were excluded. Corticoid therapy was started for rejection nephropathy. The patient improved dramatically and ultimately the transplanted kidney was removed. Chronic nephropathy of the transplanted kidney was confirmed histologically and the patient remained clinically asymptomatic, without corticoid therapy.

Acute esophageal necrosis in association with acute cholecystitis

No disponible

Acute esophageal necrosis in association with acute cholecystitis.

We report a unique case of acute esophageal necrosis in association with perforated acute cholecystitis and secondary Klebsiella pneumoniae bacteremia. An 83-year-old male with history of diabetes mellitus, dyslipidemia, ischemic cardiomyopathy and recent right hemicolectomy for colon adenocarcinoma presented to emergency department with acute epigastric pain and hematemesis. The patient appeared cachectic and dehydrated. He was afebrile and hemodynamically stable. Laboratory studies revealed anemia, leukocytosis, hyponatremia and hyperlactatemia. Esophagogastroduodenoscopy displayed characteristic features of acute esophageal necrosis. Abdominal computerized tomography revealed acute cholecystitis with perforation contained by the liver. Percutaneous cholecystostomy was performed. Fluid therapy, intravenous pantoprazole and bowel rest were started. Klebsiella pneumoniae was cultured in blood and bile and broad-spectrum antibiotic therapy was administered. The patient improved clinically and, three weeks later, esophagogastroduodenoscopy demonstrated nearly complete healing of esophageal mucosa. To our knowledge, this is the first case of acute esophageal necrosis in association with acute cholecystitis.