Renal and extrarenal manifestations of autosomal dominant polycystic kidney disease

The objective of the present study was to determine the frequency of the most common clinical features in patients with autosomal dominant polycystic kidney disease in a sample of the Brazilian population. The medical records of 92 patients with autosomal dominant polycystic kidney disease attended during the period from 1985 to 2003 were reviewed. The following data were recorded: age at diagnosis, gender, associated clinical manifestations, occurrence of stroke, age at loss of renal function (beginning of dialysis), and presence of a family history. The involvement of abdominal viscera was investigated by ultrasonography. Intracranial alterations were prospectively investigated by magnetic resonance angiography in 42 asymptomatic patients, and complemented with digital subtraction arteriography when indicated. Mean age at diagnosis was 35.1 ± 14.9 years, and mean serum creatinine at referral was 2.4 ± 2.8 mg/dL. The most frequent clinical manifestations during the disease were arterial hypertension (63.3%), lumbar pain (55.4%), an abdominal mass (47.8%), and urinary infection (35.8%). Loss of renal function occurred in 27 patients (mean age: 45.4 ± 9.5 years). The liver was the second organ most frequently affected (39.1%). Stroke occurred in 7.6% of the patients. Asymptomatic intracranial aneurysm was detected in 3 patients and arachnoid cysts in 3 other patients. In conclusion, the most common clinical features were lumbar pain, arterial hypertension, abdominal mass, and urinary infection, and the most serious complications were chronic renal failure and stroke. Both intracranial aneurysms and arachnoid cysts occurred in asymptomatic patients at a frequency of 7.14%.

HEART ANEURYSM IN CHAGAS' DISEASE

This prospective study on 41 autopsy collected human hearts concerns the "apical" lesion in Chagas' disease. Previous report did not show a correlation between lesion frequency and heart weight then discarding a vascular factor in its pathogenesis. The present paper involves other variables besides the heart weight to evaluate the relative coronary insufficiency. Distinct colored gel (green and red) injected through the capillary beds of both coronary arteries defined the extent of both vessels before separating the atria and removing the sub-epicardium fat. The Right Ventricle (RV) and Left Ventricle (LV) free walls furnished the RV/LV mass ratio. The myocardium mass colored green (right coronary artery - RC) and the whole Ventricular Weight (VW) determined the RC/VW mass ratio. The heart weight plus these mass ratios, graded and added, composed a score inversely proportional to the myocardium irrigation condition. It intended to be a more sensitive morphologic evaluation of the relative ischaemia to correlate to the apical lesion. This study showed a right deviation for the relative accumulated frequency of lesions plotted as a score function and a significant difference for higher scores in hearts with aneurysm. It suggests a ischaemic factor intervening in the apical lesion pathogenesis in Chagas' cardiopathy.

Treatment by splenectomy of a portal vein aneurysm in hepatosplenic schistosomiasis

Portal vein aneurysm is a rare medical entity that can be caused by chronic hepatic diseases with portal hypertension. We describe a 45-year-old man with variceal bleeding from hepatosplenic schistosomiasis and an incidentally found intrahepatic aneurysm. Diagnosis was confirmed with non-invasive imaging exams, arteriography and liver biopsy. Following splenectomy, the aneurysm diameter decreased substantially.

Ring enhancing intracranial lesion responding to antituberculous treatment in an HIV-infected patient

Cerebral tuberculomas constitute a major differential diagnosis of cerebral toxoplasmosis in human immunodeficiency virus (HIV)-infected patients in developing countries. We report the case of a 34-year old woman co-infected with HIV and possible disseminated tuberculosis (hepatitis, lymphadenopathy, and pleural effusion) who presented a large and solitary intracranial mass lesion. Despite extensive diagnostic efforts, including brain, ganglionar, and liver biopsies, no definitive diagnosis was reached. However, a trial with first-line antituberculous drugs led to a significant clinical and radiological improvement. Atypical presentations of cerebral tuberculomas should always be considered in the differential diagnosis of intracranial mass lesions in HIV-infected patients and a trial with antituberculous drugs is a valuable strategy to infer the diagnosis in a subset of patients.

Mycotic aneurysm of the tibioperoneal trunk: a first manifestation of an infected endocarditis

Infrapopliteal mycotic aneurysm resulting from endocarditis is rare, with only a few reported cases. We describe the case of a 28-year-old male patient who was suffering with pain and edema in the right leg. The ultrasound revealed an aneurysm of the right tibioperoneal trunk and a deep vein thrombosis (DVT). The patient was admitted and developed acute congestive heart failure, being diagnosed with possible endocarditis. A pseudo-aneurysm was revealed by arteriography. Aggressive antibiotic treatment was initiated, and open surgery confirmed a mycotic pseudo-aneurysm of the tibioperoneal trunk. To our knowledge, this is the 8th case reported of an infected aneurysm in this particular location.

Ventriculoperitoneal shunts for treating increased intracranial pressure in cryptococcal meningitis with or without ventriculomegaly

Introduction Cryptococcosis is an opportunistic mycosis, especially in patients that are human immunodeficiency virus (HIV)-positive, and frequently involves the central nervous system. Methods We assessed the potential of ventriculoperitoneal shunting (VPS) in preventing mortality due to uncontrollable intracranial hypertension (ICH) in 15 patients with acquired immunodeficiency syndrome (AIDS)-related cryptococcal meningitis. Results After 2 weeks of antifungal therapy consisting of amphotericin B deoxycholate with or without fluconazole, patients with persistent ICH underwent VPS, despite having persistent Cryptococcus neoformans infection. In 12 patients, the uncontrollable ICH was resolved by VPS. Conclusions Patients with cryptococcal meningoencephalitis who have ICH must be considered for VPS even with positive cerebrospinal fluid cultures.

Apical aneurysm and left ventricular hypertrophy

A 59-year-old woman presented with an embolic transient ischemic attack and a history of controlled hypertension for 16 years. Both echocardiogram and MRI showed severe biventricular hypertrophy and an apical aneurysm with a thrombus. The occurrence of an apical aneurysm in the presence of cardiac hypertrophy is a rare finding and has been described in patients with hypertrophic cardiomyopathy. However, it has not been reported in patients with systemic arterial hypertension. In this patient the lack of a relationship between the severity of the hypertrophy and the levels of blood pressure, together with the presence of histologic disorganization of myocardial cardiac muscle cells by endomyocardial biopsy suggested the diagnosis of hypertrophic cardiomyopathy.

Aneurysm of the left main coronary artery

Aneurysm of the left main coronary artery is a rare angiographic finding, with few cases described in the international literature. We report the case of a 42-year-old male with a previous history of acute myocardial infarction, whose coronariography indicated triple vessel coronary disease and an aneurysm of the left main coronary artery. A review of the etiology, clinical aspects, and surgical management of coronary arterial aneurysm is presented.

Submitral left ventricular aneurysm. Case report and review of published Brazilian cases

Submitral left ventricular aneurysm is a cardiac pathology widely recognized, but relatively unknown, occurred almost exclusively in African black patients. Although still this idea of racial prevalence exists, cases have been described in patients of all the races. Ten Brazilian cases were reported. One of them was presented inside an Italian paper that refers the surgical treatment of a Brazilian patient of black race. We reported one more submitral left ventricular aneurysm case in a brown female patient, with antecedents of peripheral thromboembolism initially not identified as consequence of the cardiac pathology.

Aneurysm of the Right Atrial Appendage

Atrial aneurysms involving the free wall or atrial appendage are rare entities in cardiology practice and may be associated with atrial arrhythmias or embolic phenomena. We review the literature and report a case of aneurysm of the right atrial appendage in a young adult, whose diagnosis was established with echocardiography after an episode of paroxysmal atrial flutter.

Repair of an Atherosclerotic Coronary Artery Aneurysm by Implantation of a Coronary Covered Stent

An atherosclerotic aneurysm of the right coronary artery complicated by a recent myocardial infarction was successfully treated with coronary artery stenting, using a device consisting of 2 stents with a layer of expandable polytetrafluorethylene (PTFE) placed between them. A follow-up angiograph 5 months after the procedure showed sustained initial results.

Silent Patent Ductus Arteriosus Aneurysm

Ductus arteriosus aneurysm, a rare and potentially fatal condition, has been reported as a complication after surgical ductus arteriosus closure. Its spontaneous appearance as a septic complication, which was common in the presurgical and preantibiotic era, has been rarely reported in the contemporary literature. Persistence of silent ductus arteriosus in healthy children and adults is a frequent condition that currently has an increasing diagnostic possibility due to the availability of more accurate investigative methods, especially echocardiography. We report the case of a 1-year-old child, in whom no previous heart disease was known, who developed a giant aneurysm of the ductus arteriosus during a staphylococcal infection. This complication appeared after craniotomy for emptying an accidental subdural hematoma. This report associates the persistence of ductus arteriosus with a complication considered rare, which has a rapidly fatal evolution.

Aneurysmal dilation of the reimplant segment of the visceral vessels after thoracoabdominal aneurysm correction

We present a case of aneurysmal dilation of the aortic residual segment, involving abdominal vessels in corrective surgeries for thoracoabdominal aortic aneurysm, through the identification of risk groups for recurrent dilation, aiming at using a specific operative technique with a branched graft, to prevent aneurysm relapse.