Heart Transplantation in Arrhythmogenic Right Ventricular Dysplasia: Case Reports

Objective. Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial disease of familiar, origin where the myocardium is replaced by fibrofatty tissue predominantly in the right ventricle. Herein we have presented the clinical courses of 4 patients with ARVD who underwent orthotopic heart transplantation. Patients and Methods. Among 358 adult patients undergoing heart transplantation, 4 (1.1%) displayed ARVD. The main indication for transplantation was the progression to heart failure associated with arrhythmias. All 4 patients displayed rapid, severe courses leading to heart failure with left ventricular involvement and uncontrolled arrhythmias. Results. In all cases the transplantation was performed using a bicaval technique with prophylactic tricuspid valve annuloplasty. One patient developed hyperacute rejection and infection, leading to death on the 7th day after surgery. The other 3 cases showed a good evolution with clinical remission of the symptoms. Pathological study of the explanted hearts confirmed the presence of the disease. Conclusions. ARVD is a serious cardiomyopathy that can develop malignant arrhythmias, severe ventricular dysfunction with right ventricular predominance, and sudden cardiac death. Orthotopic heart transplantation must always be considered in advanced cases of ARVD with malignant arrhythmias or refractory congestive heart failure with or without uncontrolled arrhythmias, because it is the only way to remit the symptoms and the disease.

Orofacial granulomatosis: A diagnostic problem for the unwary and a management dilemma. Case reports

Orofacial granulomatosis is a condition that, may be difficult to diagnose for those unfamiliar with the entity. This paper describes two cases and addresses the presentation, pathogenesis and treatment. The clinical recognition of his condition is important as is the subsequent investigation by an appropriate specialist. Management of patients needs to take into account the results of further investigations, the patient's expectations, and the severity of the condition.

The Versatile Adani`s Flap for Fingertip Coverage Indications and Case Reports

Heterodigital pedicle flaps have 2 possible patters of vascularization: anterograde flow (Littler) or reverse flow (Adani). The Adani`s flap uses the Y-V pedicle lengthening principle. The flap is raised from the adjacent uninjured finger based on the digital artery. The common digital artery is ligated and a long pedicle is formed from the 2 converging digital arteries to supply a reverse flow flap. Four patients with severe fingertips injuries were submitted to surgical treatment with the Adani`s flap. All flaps integrated and provided skin coverage. The Adani`s flap has a long vascular pedicle that allows a wide arch of transposition. An easier vascular dissection in a site distant from the trauma which evolves a higher diameter digital artery and proximal interphalangeal arterial system promotes a high survival rate and good functional results. Adani`s flap is a reliable technique for severe fingertip injuries.

Bilateral lung transplantation in asymmetric thorax: Case reports

Suppurative lung diseases, such as cystic fibrosis and bronchiectasis, when diffuse and associated with important functional loss, can be treated with bilateral lung transplantation with good results. These diseases are frequently associated with previous lung resections presenting an asymmetric thorax, thus making lung extraction difficult and generating disproportion between the graft and the pleural cavity. To treat this condition, pneumonectomy and single lung transplantation is a feasible option; however, there are associated comorbidities and an invariable need for extracorporeal circulation. Described herein are 2 patients with an asymmetric thorax, treated with bilateral transplantation and lung volume reduction with lobectomy.

Hemorrhagic Cystitis Secondary to Adenovirus or Herpes Simplex Virus Infection Following Renal Transplantation: Four Case Reports

Viral infections are common complications following renal transplantation. However, there have been few reported cases of viral cystitis secondary to herpes simplex virus or adenovirus infection. Herein, we have reported four cases of hemorrhagic cystitis secondary to infections with herpes simplex virus and adenovirus following renal transplantation. The etiology was adenovirus in three cases and herpes simplex virus in the remaining case. In all four cases, the primary cause of the renal dysfunction was diabetic nephropathy. All four patients presented with a clinical profile characterized by dysuria, pollakiuria, macroscopic hematuria, and graft dysfunction. Three of the four patients developed these symptoms within the first 3 months after renal transplantation. In all four cases, there was an increase, albeit slight, in creatinine levels, which returned to normal or near-normal values upon resolution of the symptoms. Acute cellular rejection was observed in only one case. Although rare, hemorrhagic cystitis secondary to infection, which typically occurs early in the posttransplant period, causes pronounced symptoms. The infection appears to be self-limiting, resolving completely within 4 weeks.

Genital Manifestation of Graft-vs.-Host Disease: A Series of Case Reports

Introduction. After hematopoietic stem cell transplantation (HSCT), many patients present genital graft-vs.-host disease (GVHD) that can culminate with sexual problems, which are poorly dimensioned. Aim. We hope to draw attention to the need to perform genital biopsy to diagnose genital GVHD, and thus to call attention to the need to incorporate careful attention to sexual health in the treatment of these patients. Methods. Five allogeneic stem cell transplant recipients complaining of coital pain after HSCT were clinically diagnosed for genital GVHD. Genital biopsies were given for histological analysis, and microphotographs of the corresponding marked field in the slide were taken. Specimens were evaluated by the site pathologist and then sent to a reference pathologist, each blinded to the histological findings. A literature search was performed in PubMed/MEDLINE (1966-2009) for cross-sectional and cohort studies or trials related to genital GVHD. Expert opinions peer reviews and case reports were also considered. Main Outcome Measures. HSCT, genital GVHD, genital biopsy. Results. The biopsy showed evidence of dilated apoptotic cells in the basal layer and detachment of the epithelial lining of the mucosa, hyalinization and thickening of collagen fibers, capillary ectasia, and mononuclear inflammatory infiltrate of the submucosa. Three patients presented vulval lesion such as leucoplasia and ulcer on the large lip. Histological analyses showed evidence of epithelial hyperplasia and influx of inflammatory cells to the epithelial surface, intercellular edema and spongiosis, apoptotic bodies on the basal layer of the epithelium, spongiosis, and nuclear vacuolization. A common treatment based on corticotherapy resulted in complete remission of coetaneous or mucous genital lesions in all five patients. Conclusion. Genital biopsy is important to differentially diagnose GVHD and secondary symptoms due to hypoestrogenism. Prevention is the most important step in controlling the evolution GVHD in the vagina to prevent vaginal obstruction and sexual dysfunction. da Silva Lara LA, de Andrade JM, Mauad LMQ, Ferrarese SR, Marana HRC, Tiezzi DG, and de Sa Rosa e Silva ACJ. Genital manifestation of graft-vs.-host disease: A series of case reports. J Sex Med 2010;7:3216-3225.

Syndromes with salivary dysfunction predispose to tooth wear: case reports of congenital dysfunction of major salivary glands, Prader-Willi, congenital rubella, and Sjogren's syndromes.

Four cases-of congenital dysfunction of the major salivary glands as well as of Prader-Willi, congenital rubella, and Sjogren's syndromes-were identified in a series of 500 patients referred for excessive tooth wear. Although there was evidence of consumption of highly acidic drinks, some occlusal parafunction, and unacceptable toothbrushing habits, salivary dysfunction was the salient factor predisposing a patient to tooth wear in these syndromal cases. The 500 subjects have been characterized either as having medical conditions and medications that predispose them to xerostomia or lifestyles in which workplace- and sports-related dehydration lead to reduced salivary flow. Normal salivation, by buffering capacity, clearance by swallowing, pellicle formation, and capacity for remineralization of demineralized enamel, protects the teeth from extrinsic and intrinsic acids that initiate dental erosion. Thus, the syndromes, unrelated in many respects, underline the importance of normal salivation in the protection of teeth against tooth wear by erosion, attrition, and abrasion.

Lethal lesions and amputation caused by plastic debris and fishing gear on the loggerhead turtle Caretta caretta (Linnaeus, 1758). Three case reports from Terceira Island, Azores (NE Atlantic)

Copyright © 2014 Elsevier Science Ltd.

Orbital tuberculosis diagnosed by immunohistochemistry: case reports

PURPOSE: To describe three cases of orbital tuberculosis, with their clinical characteristics and respective diagnostic procedures, showing the importance of histopathology for the correct diagnosis. PATIENTS AND METHODS: Three patients diagnosed and treated for orbital tuberculosis, in the "Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo" during the years of 1999 and 2001. RESULTS: All of the patients were female and referred a chronic evolution of swelling of one eyelid associated or not with other symptoms. They had not HIV infection. Two of them had positive epidemiology for tuberculosis. However, the search for systemic evidence of the disease and staining for bacilli (Ziehl-Neelsen method) were negative in all three patients. In all the cases, only the biopsy with immunohistochemical analysis could confirm the diagnosis. COMMENTS: Orbital involvement is a rare ocular manifestation of tuberculosis. Even in a tertiary hospital, few cases, if any, are diagnosed per year. However, the global incidence and prevalence of M. tuberculosis infections is not under control yet, and it has been a serious public health problem. Thus, one must be aware of the possibility of tuberculosis among the orbital diseases.

Early diagnosis of relapse in borderline leprosy: two case reports

Two cases of relapse in borderline leprosy were reported. Despite the late-reversal, reaction-like feature, the suspicion of relapse in both was based on persistent and slow-developing skin lesions and an absence of acute neuritis or reaction during one year of follow-up. The authors have considered this possible occurrence in lepromatous borderline-treated patients after their immune cellular restoration and defend that not all Type 1 reactions would be an inflammatory answer to persistent Mycobacterium leprae, but that they could be. Therefore, a relapse diagnosis could be applied and it is more advisable, as one year of Multi-Drug Therapy (MDT) is less dangerous and more efficient for these cases than one year of corticosteroids.

Calciphylaxis: a Literature Review Based in Two Case Reports

Calciphylaxis is a rare and devastating obliterative vasculopathy, leading to ischemia and subcutaneous necrosis. In most cases it affects patients with renal disease and is associated with high morbidity and mortality. We present two case reports followed recently in our department, and a literature review on this topic. Case one refers to an 80 -year -old Caucasian woman with chronic kidney disease stage 5 and primary hyperparathyroidism with secondary brown tumour and calciphylaxis. Case two refers to a 59 -year -old Caucasian woman admitted with severe nephrotic syndrome associated with amyloidosis, that developed a catastrophic picture of calciphylaxis, ending in the patient’s death. There is a critical need to understand the pathogenesis of calciphylaxis. Its comprehension is the only way to improve the survival of these patients, and may help to elucidate the pathophysiology of vascular calcification in general. Educating physicians in the prevention and early detection of calciphylaxis is crucial. Only by increasing the knowledge about risk factors, pathophysiology, response to treatment and outcome, will we be able to improve prophylaxis and therapy of patients with calciphylaxis, decreasing the high mortality of this entity.

Severe dengue in the early postoperative period after kidney transplantation: two case reports from Hospital Geral de Fortaleza

Abstract: Dengue is an arbovirosis that ranges from an asymptomatic presentation to a more severe disease, which is characterized by a vascular leakage syndrome where abdominal pain is a major symptom. Transplant recipients are immunosuppressed and are less likely to develop a severe form of the disease because of a reduction in immune-mediated responses that trigger plasma extravasation events. Herein, we report two cases of severe dengue in the early postoperative period of two kidney transplant recipients. Considering the severity of the cases, we emphasize the importance of dengue screening immediately before transplantation in areas endemic for the disease.

Electroconvulsive therapy in treatment-resistant mania: case reports

Electroconvulsive therapy is known to be effective in the treatment of mood disorders, more specifically for depression and mania. Although a large body of evidence confirms the efficacy of electroconvulsive therapy in the treatment of mania, few prospective studies have been done to assess its effectiveness in treatment-resistant manic episodes. These case reports describe the initial results of a study that is being conducted to evaluate the efficacy of Electroconvulsive therapy among treatment-resistant bipolar patients. METHODS: Three manic patients (according to DSM-IV criteria) who were considered treatment-resistant underwent a series of 12 bilateral Electroconvulsive therapy sessions. Before the treatment and then weekly, they were evaluated with the following rating scales: Young Mania Rating Scale, Hamilton Rating Scale for Depression, Brief Psychiatric Rating Scale, and Clinical Global Impressions-Bipolar Version. RESULTS: The 3 patients showed a satisfactory response to Electroconvulsive therapy, although some differences in the course of response were observed. CONCLUSION: These case reports suggest that Electroconvulsive therapy needs further evaluation for the treatment of resistant bipolar patients.

Secondary amyloidosis: a severe complication of ankylosing spondylitis. Two case-reports.

STUDY OBJECTIVE: To report two cases of amyloidosis secondary to ankylosing spondylitis. PATIENTS AND RESULTS: Of the 47 ankylosing spondylitis patients who have received follow-up at our department over the last few years, two have developed AA amyloidosis. Both have extremely severe, long-standing joint disease, with virtually complete spinal ankylosis and destructive peripheral arthritis of the hips and wrists; one also has tarsal joint destruction. Renal dysfunction was the first manifestation of amyloidosis in both cases. One patient required chronic hemodialysis and developed peritonitis due to colonic perforation, probably at a site of amyloid deposition. CONCLUSIONS: Secondary amyloidosis is a rare complication of ankylosing spondylitis that can cause severe renal and gastrointestinal complications. No treatment capable of clearing established amyloid deposits is available to date.

Usefulness of postmortem biochemistry in forensic pathology: illustrative case reports.

The aim of this work is to present some practical, postmortem biochemistry applications to illustrate the usefulness of this discipline and reassert the importance of carrying out biochemical investigations as an integral part of the autopsy process. Five case reports are presented pertaining to diabetic ketoacidosis in an adult who was not known to suffer from diabetes and in presence of multiple psychotropic substances; fatal flecainide intoxication in a poor metabolizer also presenting an impaired renal function; diabetic ketoacidosis showing severe postmortem changes; primary aldosteronism presented with intracranial hemorrhage and hypothermia showing severe postmortem changes. The cases herein presented can be considered representative examples of the importance of postmortem biochemistry investigations, which may provide significant information useful in determining the cause of death in routine forensic casework or contribute to understanding the pathophysiological mechanisms involved in the death process.