Clinical Dilemma in the Treatment of a Patient with Microangiopathic Haemolytic Anaemia, Thrombocytopaenia and Severe Hypertension

While haemolytic uraemic syndrome in children is predominantly associated with Shiga toxin -producing Escherichia coli (typically 0157:H7), some cases occur without associated diarrhoea, or as the manifestation of an underlying disorder other than infection. Haemolytic uraemic syndrome is characterised by microangiopathic anaemia, thrombocytopaenia and renal failure, on occasion accompanied by severe hypertension. Malignant hypertension is a syndrome that sometimes exhibits the same laboratory abnormalities as haemolytic uraemic syndrome as it may share the same pathological findings: thrombotic microangiopathy. As clinical features of both entities overlap, the distinction between them can be very difficult. However, differentiation is essential for the treatment decision, since early plasma exchange dramatically reduces mortality in haemolytic uraemic syndrome not associated with diarrhoea. An increasing number of genetic causes of this pathology have been described and may be very useful in differentiating it from thrombotic microangiopathy due to other aetiologies. Despite advances in the understanding of the pathophysiology of haemolytic uraemic syndrome not associated with diarrhoea, the management often remains empirical. We describe a patient with simultaneous microangiopathic haemolytic anaemia, thrombocytopaenia and severe hypertension managed in the acute period of illness with plasma exchange.

De Novo Hypertension and Decline of Renal Function in a Young Woman with Systemic Lupus Erythematosus and Antiphospholipid Syndrome

Antiphospholipid syndrome nephropathy and lupus nephritis have similar clinical and laboratory manifestations and achieving the accuracy of diagnosis required for correct treatment frequently necessitates a kidney biopsy. We report the case of a 29-year-old woman referred to the nephrology service for de novo hypertension, decline of renal function and proteinuria. She had had systemic lupus erythematosus and antiphospholipid syndrome since the age of 21 and was taking oral anticoagulation. Two weeks later, after treatment of hypertension and achievement of adequate coagulation parameters, a percutaneous renal biopsy was performed. The biopsy revealed chronic lesions of focal cortical atrophy, arterial fibrous intimal hyperplasia and arterial thromboses, which are typical features of antiphospholipid syndrome nephropathy. We describe the clinical manifestations and histopathology of antiphospholipid syndrome nephropathy and review the literature on renal biopsy in patients receiving anticoagulation.

Focal Malignant Liver Lesions: Diagnosis by Dynamic Incremental CT, Early Phase

The purpose of our study was to evaluate the accuracy of dynamic incremental bolus-enhanced conventional CT (DICT) with intravenous contrast administration, early phase, in the diagnosis of malignancy of focal liver lesions. A total of 122 lesions were selected in 74 patients considering the following criteria: lesion diameter 10 mm or more, number of lesions less than six per study, except in multiple angiomatosis and the existence of a valid criteria of definitive diagnosis. Lesions were categorized into seven levels of diagnostic confidence of malignancy compared with the definitive diagnosis for acquisition of a receiver-operator-characteristic (ROC) curve analysis and to determine the sensitivity and specificity of the technique. Forty-six and 70 lesions were correctly diagnosed as malignant and benign, respectively; there were 2 false-positive and 4 false-negative diagnoses of malignancy and the sensitivity and specificity obtained were 92 and 97%. The DICT early phase was confirmed as a highly accurate method in the characterization and diagnosis of malignancy of focal liver lesions, requiring an optimal technical performance and judicious analysis of existing semiological data.

Primary Malignant Peritoneal Mesothelioma Associated with Renal Cell Carcinoma. A Concise Review Based on a Clinical Case Baseada num Caso Clínico

O Mesotelioma peritoneal maligno é um tumor maligno relacionado frequentemente com exposição prolongada a fibras de amianto, de mau prognóstico, de diagnóstico geralmente tardio, face à pouca expressão clínica na fase inicial da doença. Como o mesotelioma evolui geralmente só na cavidade peritoneal, doentes seleccionados poderão ter maior sobrevivência se for possível a peritonectomia extensa e quimioterapia hipertérmica intraperitoneal intraoperatória. Os autores referem a sincronicidade ainda não descrita, de mesotelioma peritoneal maligno primário e carcinoma de Grawitz. São revistos concisamente: a clínica destes tumores, síndromes paraneoplásicos (disfunção bioquímica hepática, emagrecimento extremo); etiopatogenia da acção cancerígena das fibras de amianto; mecanismos de disseminação intraperitoneal; avaliação tomodensitométrica; importância da imunohistoquímica no diagnóstico histopatológico; estadiamento; importância do tratamento multidisciplinar destes tumores.

Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry

Introduction. Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers; therefore, the availability of epidemiological national data is critical. Methods. We conducted a prospective, observational, and multicenter registry with a joint collaboration from five centers from Portugal and included adult incident patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH). Results. Of the 79 patients enrolled in this study, 46 (58.2%) were classified as PAH and 33 patients (41.8%) as CTEPH. PAH patients had a mean age of 43.4 ± 16.4 years. Idiopathic PAH was the most common etiology (37%). At presentation, PAH patients had elevated right atrial pressure (RAP) (7.7 ± 5.9mmHg) and mean pulmonary vascular resistance (11.4 ± 6.5 Wood units), with a low cardiac index (2.7 ± 1.1 L⋅min−1 m−2); no patient was under selective pulmonary vasodilators; however, at follow-up, most patients were on single (50%), double (28%), or triple (9%) combination vasodilator therapy. One-year survival was 93.5%, similar to CTEPH patients (93.9%), that were older (60.0 ± 12.5 years) and had higher RAP (11.0 ± 5.2mmHg,

Case Report of Malignant Endometrial Polyps

The aims of this retrospective review were to determine the frequency of malignant endometrial polyps diagnosed with ambulatory hysteroscopy in the Obstetrics/Gynaecology Department of HDE, Lisbon, between January 2001 and December 2005 and to characterize these cases according to risk factors, sonographic and endoscopic findings tumoral histology, and tumor stage.We found seven cases of malignant endometrial polyps in a total of 1333 polyps initially diagnosed: an incidence rate of 0. 53%. These seven patients had a mean age of 68 years (55–82 years), and all were postmenopausal, with five having one risk factor each for endometrial cancer. Metrorrhagia was present in six of the seven patients(85.7%). Ultrasonography was abnormal in all seven patients, with a mean endometrial thickness of 26 mm(range: 12–44 mm). The hysteroscopy images suggested malignancy in all cases. All except one patient had a single polyp. The polyps had volumes between 1.5 and 3 cm; two were removed completely and five were biopsed. The histological subtype was: mixed endometrioid/serous papillary or clear cell (2), adenocarcinoma with squamous differentiation (2), carcinosarcoma (2), and clear cell carcinoma (1). Malignancy inside polyps is rare, but diagnostic hysteroscopy with visual guided biopsies can identify these cases in the earlier stages. The risk factors are not different from those of other endometrial carcinomas, but the histological subtype seems to point to more aggressive cancers.

Health-Related Quality of Life Impact of a Triple Combination of Olmesartan Medoxomil, Amlodipine Besylate and Hydrochlorotiazide in Subjects with Hypertension

BACKGROUND: A post-hoc analysis was performed on the data from a 54 weeks phase III study (ClinicalTrials.gov identifier: NCT00923091) to measure changes in the health-related quality of life (HRQoL) of 2,690 patients aged ≥18 with moderate-to-severe hypertension who received one of six doses of olmesartan/amlodipine/hydrochlorothiazide (OLM/AML/HCTZ), using the MINICHAL and EQ-5D instruments. METHODS: Descriptive statistics were used to assess blood pressure and HRQoL scores over the study period. Analysis of covariance (ANCOVA) was used to identify those factors that could possibly have influenced HRQoL. Linear regression was used to assess the relationship between changes in blood pressure and HRQoL scores. RESULTS: Patients' baseline MINICHAL mood and somatic domains scores were 5.5 and 2.6. Over the study period HRQoL improved as both MINICHAL scores decreased by 31-33%. Patients' baseline EQ-5D index and VAS scores were 0.9 and 73.4 respectively, increasing by 6% and 12% over the study period. Patients' QALY gain over the 54 weeks study period was estimated to be 0.029 QALYs. The ANCOVA showed that changes in patients' HRQoL was likely to have been influenced by patients' achievement of blood pressure control, the amount of concomitant medication and patients' last used dosage strength of antihypertensive. Linear regression showed that blood pressure improvement may have been associated with improved HRQoL. CONCLUSIONS: This study showed that OLM/AML/HCTZ reduced blood pressure and significantly increased blood pressure control whilst improving patients' HRQoL. Achieving blood pressure control, amount of concomitant medication and dosage strength of antihypertensive impacted on patients' HRQoL.