Angiotensin II receptor agonist antibodies are associated with microvascular damage in lupus nephritis.

Angiotensin II type 1 receptor agonist antibodies (AT1R-AAs) have been associated with hypertension, atherosclerosis and vascular inflammation in human diseases. The aim of the study was to evaluate the prevalence of AT1R-AAs in active lupus nephritis (LN) patients and their association with vascular damage. One hundred and seven active LN patients underwent a complete clinical examination, measurement of AT1R-AAs, ambulatory blood pressure monitoring, carotid intima-media thickness measurement and morphometric analysis of subintimal fibrosis and medial hyperplasia of the vessels in the kidney tissue. Plasma AT1R-AAs were positive in 58 (54.2%) patients. The Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score, complement C3 and C4 levels and titers of anti-dsDNA antibodies were higher in the group with positive AT1R-AAs compared with those with negative AT1R-AAs. The AT1R-AA titers correlated with anti-dsDNA antibody titers and with complement C3 and C4 serum levels. In the kidney biopsy, the percentage of subintimal fibrosis and the area of medial hyperplasia were greater in the AT1R-AA-positive patients. No differences in arterial pressure, carotid intima-media thickness and response to therapy were detected. In conclusion, AT1R-AAs are prevalent in active LN patients and are associated with histologic features of microvascular damage.

[Foundations of the diagnosis of clinical syndromes]. / Bases para el diagnóstico de los síndromes clínicos.

AIMS: The aim of this paper is to offer a reflection on the principles used to define a syndromic entity and for the evaluation of its manifestations and the aetiopathogenesis. METHOD: First, the paper looks at several of the different definitions of syndrome available and examines which are the essential features for this term to be employed in a clinical entity. The topographical and semiological perspectives are then dealt with, so as to be able to sort clinical syndromes and charts are used to offer representative examples. The main deficiencies in the use of terms to describe syndromes and the possible causes of such situations are also investigated. Following that, the chief semiological aspects in the diagnosis of a syndrome are assessed. Emphasis is placed on the factors that influence the variability of clinical behaviour, on the factors that come to bear on the recognition of the manifestations using the research methods available to us, on the rule of iterative confirmation of the symptom and on the characterisation and coordination of the symptom with other related phenomena. Lastly, the aetiopathological foundations of clinical syndromes are evaluated. We highlight the importance of determining the underlying anatomofunctional disorder, the classical distinctions concerning this, and the relation between syndrome and disease. The diagnostic criteria from numerous neurological syndromes are used as examples. CONCLUSIONS: The concept of syndrome has been defined in an attempt to illustrate the presence of inappropriate, incorrect terms with adverse repercussions in the theoretical and practical areas. We also propose ways of resolving the faults found.

[Non-infectious thrombosis of the cerebral venous sinuses and veins in adults. A report of five cases]. / Trombosis no infecciosa de los senos y venas cerebrales en adultos. Presentación de cinco casos.

INTRODUCTION: Thrombosis of the veins and venous sinuses is an uncommon condition. In 1947 it was described in adults by Garcin and Pestel. It is a potentially serious disorder, in which there is severe intracranial hypertension which may be fatal or cause serious neurological sequelae. Diagnosis is made, during life, on the clinical condition and neuro-imaging features. We consider that treatment of the underlying condition is essential to improve prognosis, although this is controversial. In Cuba there are no publications reporting series of patients with this condition. CLINICAL CASES: We present five patients, four of whom were women, with a neurological disorder involving focal neurological deficits, headache and severe intracranial hypertension, of subacute onset and with convulsions in 60%. The results of imaging studies were compatible with thrombosis of veins and/or venous sinuses. The diagnosis was conformed at necropsy in two patients. The commonest risk factor was the puerperium, occurring in four cases. The patients were treated symptomatically. Two patients died. The other patients made good progress. CONCLUSIONS: The puerperium is the commonest risk factor in thrombosis of the venous sinuses and the veins draining into them. Headache, neurological focal signs and level of consciousness are the main symptoms and signs. Coma, haemorrhagic infarct and convulsions are of bad prognosis and followed by unsatisfactory progress of the disorder.

Estenosis aortica supravalvular. / Supravalvular aortic stenosis

Se describen los datos caracteristicos de 15 casos de estenosis aortica supravalvular estudiados en 12 anos de trabajo del Servicio de Cardiologia del Instituto Nacional de Pediatria. Aunque la designacion clasica es referida a la aorta, es muy frecuente que haya estenosis de las ramas pulmonares de numero y magnitud variable.De hecho, en esta serie se observaron ademas casos con estenosis valvular de la arteria pulmonar. Estos problemas se presentan generalmente en ninos con retraso mental e integran el llamado sindrome de Williams