Hantavirus pulmonary syndrome in Brazil: clinical aspects of three new cases

Hantavirus pulmonary syndrome (HPS) has been recognized recently in Brazil, where 28 cases have been reported as of September 1999. We report here the clinical and laboratory findings of three cases whose diagnoses were confirmed serologically. All the patients were adults who presented a febrile illness with respiratory symptoms that progressed to respiratory failure that required artificial ventilation in two of them. Laboratory findings were most of the time consistent with those reported in the United States in patients infected with the Sin Nombre virus, and included elevated hematocrit and thrombocytopenia; presence of atypical lymphocytes was observed in one patient. The chest radiological findings observed in all the patients were bilateral, diffuse, reticulonodular infiltrates. Two patients died. Histopathological examination of the lungs of these patients revealed interstitial and alveolar edema, alveolar hemorrhage, and mild interstitial pneumonia characterized by infiltrate of immunoblasts and mononuclear cells. In the epidemiologic investigation of one of the cases, serologic (ELISA) tests were positive in 3 (25%) out of 12 individuals who shared the same environmental exposure. HPS should be included in the differential diagnosis of interstitial pneumonia progressing to acute respiratory failure.

Bilateral paracoccidioidomycotic iliopsoas abscess associated with ileo-colonic lesion

This case report shows the clinical development of a patient with systemic paracoccidioidomycosis presenting with lymphatic-intestinalmanifestation. The patient initially had a substantial clinical improvement but had a recrudescence after six months of sulfamethoxazoletrimethoprim oral treatment, with the emergence of feverish syndrome, lumbar pain, and intermittent claudication, characterizing a bilateral iliopsoas muscle abscess, necessitating clinicosurgical therapeutics.

Hydronephrosis in Schinzel-Giedion syndrome: an important clue for the diagnosis

Schinzel-Giedion syndrome is a rare autosomal recessive disorder characterized by coarse facies, midface retraction, hypertrichosis, multiple skeletal anomalies, and cardiac and renal malformations. Craniofacial abnormalities of this syndrome sometimes resemble a storage or metabolic disease. The pathogenesis of the disease remains unknown. The objective of this report was to emphasize the importance of congenital bilateral hydronephrosis for the diagnosis of Schinzel-Giedion syndrome. We describe the first Brazilian case of a newborn with typical facies, generalized hypertrichosis, cardiac and skeletal anomalies, and bilateral hydronephrosis detected during pregnancy and confirmed later by abdominal ultrasonography. Chromosomal constitution was normal. Of the 35 cases already reported in the literature, 31 presented hydronephrosis, which is considered an important clue in diagnosis. If Schinzel-Giedion syndrome were indexed as a cause of congenital hydronephrosis, its identification would be greatly facilitated, since the majority of the other findings in Schinzel-Giedion syndrome are nonspecific and common to many genetic syndromes.

Renal and urinary findings in 20 patients with Williams-Beuren syndrome diagnosed by fluorescence in situ hybridization (FISH)

PURPOSE: Williams-Beuren syndrome is a rare multiple anomalies/mental retardation syndrome caused by deletion of contiguous genes at chromosome region 7q11.23. The aim of this work was to determine the frequency and the types of renal and urinary tract anomalies in 20 patients with Williams-Beuren syndrome. METHODS: The fluorescence in situ hybridization test using a LSI Williams syndrome region DNA probe was performed for all 20 patients to confirm the diagnosis of Williams-Beuren syndrome. A prospective study was performed in order to investigate renal and urinary aspects using laboratory assays to check renal function, ultrasonography of the kidneys and urinary tract, voiding cystourethrogram and urodynamics. RESULTS: Deletion of the elastin gene (positive fluorescence in situ hybridization test) was found in 17 out of 20 patients. Renal alterations were diagnosed in 5 of 17 (29%) the patients with the deletion and in 1 of 3 patients without the deletion. Fourteen patients with the deletion presented dysfunctional voiding. Arterial hypertension was diagnosed in 3 patients with deletions and 1 of these presented bilateral stenosis of the renal arteries. CONCLUSIONS: Due to the high incidence of renal and urinary abnormalities in Williams-Beuren syndrome, performing a systematic laboratory and sonographic evaluation of the patients is recommended.

Hantavirus pulmonary syndrome in Uberaba, Minas Gerais, Brazil

This report describes the epidemiological and clinical-evolutive characteristics of eight patients with hantavirus pulmonary syndrome (HPS) in Uberaba, Minas Gerais, Brazil. A positive history of contact with rodents was present in 100% of the cases. The time between the onset of symptoms and hospital care was, on average, 3.6 days. All patients showed clinical and laboratory findings suggestive of HPS. Elevated urea and creatinine levels were observed in 6 (75%) cases, PO2 was < 60 mmHg in 100% of the cases, and a chest X-ray demonstrated a bilateral interstitial-alveolar infiltrate. The diagnosis was confirmed by the detection of IgM antibodies against Sin Nombre virus by ELISA. Three patients died as a direct consequence of HPS.

Bilateral breast swelling secondary to superior vena cava obstruction and subclavian vein thrombosis

Superior vena cava syndrome is defined by a set of signs and symptoms secondary to superior vena cava obstruction caused principally by malignant diseases. The present report describes the case of an unusual clinical manifestation of this syndrome with bilateral breast swelling, and emphasizes the relevance of knowledge on mammographic signs of systemic diseases.

Facial paralysis and vestibular syndrome in feedlot cattle in Argentina

This paper reports 6 outbreaks of neurological disease associated with paralysis of the facial and vestibulocochlear nerves caused by intracranial space occupying lesions in feedlot cattle. The clinical signs observed were characterized by head tilt, uni or bilateral drooping and paralysis of the ears, eyelid ptosis, keratoconjunctivitis, and different degrees of ataxia. Morbidity and mortality rates ranged from 1.1 to 50% and 0 to 1%, respectively. Gross lesions observed included yellow, thickened leptomeninges, and marked enlargement of the roots of cranial nerves VII (facial) and VIII (vestibulocochlear). Histopathologically, there was severe, chronic, granulomatous meningitis and, in one case, chronic, granulomatous neuritis of the VII and VIII cranial nerves. Attempts to identify bacterial, viral, or parasitic agents were unsuccessful. Based on the morphologic lesions, the clinical condition was diagnosed as facial paralysis and vestibular syndrome associated with space occupying lesions in the meninges and the cranial nerves VII and VIII. Feedlot is a practice of growing diffusion in our country and this is a first report of outbreaks of facial paralysis and vestibular disease associated with space occupying lesions in Argentina.

Delleman syndrome in a Brazilian boy

We report on a Brazilian boy, born to normal and nonconsanguineous parents and presenting facial asymmetry, hypotonia, cerebral anomalies, bilateral anophthalmia, supraorbital cysts, skin tags, cleft lip and palate, micrognatia, glossoptosis, cryptorchidism, and genital hypoplasia.

The diversity of abnormal hormone receptors in adrenal Cushing's syndrome allows novel pharmacological therapies

Recent studies from several groups have indicated that abnormal or ectopic expression and function of adrenal receptors for various hormones may regulate cortisol production in ACTH-independent hypercortisolism. Gastric inhibitory polypeptide (GIP)-dependent Cushing's syndrome has been described in patients with either unilateral adenoma or bilateral macronodular adrenal hyperplasia; this syndrome results from the large adrenal overexpression of the GIP receptor without any activating mutation. We have conducted a systematic in vivo evaluation of patients with adrenal Cushing's syndrome in order to identify the presence of abnormal hormone receptors. In macronodular adrenal hyperplasia, we have identified, in addition to GIP-dependent Cushing's syndrome, other patients in whom cortisol production was regulated abnormally by vasopressin, ß-adrenergic receptor agonists, hCG/LH, or serotonin 5HT-4 receptor agonists. In patients with unilateral adrenal adenoma, the abnormal expression or function of GIP or vasopressin receptor has been found, but the presence of ectopic or abnormal hormone receptors appears to be less prevalent than in macronodular adrenal hyperplasia. The identification of the presence of an abnormal adrenal receptor offers the possibility of a new pharmacological approach to control hypercortisolism by suppressing the endogenous ligands or by using specific antagonists for the abnormal receptors.

Arreflexia pós-calórica bilateral: aplicabilidade clínica da reabilitação vestibular

A perda bilateral da função vestibular é rara em pacientes com vertigem e desequilíbrio, porém os sintomas muitas vezes são incapacitantes e seu tratamento é tema controverso na literatura. OBJETIVO: Nosso objetivo foi avaliar e descrever a resposta clínica de pacientes com distúrbio do equilíbrio corporal secundário à arreflexia vestibular pós-calórica bilateral, documentada pela eletronistagmografia, submetidos à Reabilitação Vestibular. FORMA DE ESTUDO: Retrospectivo, inclui um desenho de descrição de casos. MÉTODO: Foram avaliadas as respostas de 8 pacientes portadores de arreflexia pós-calórica bilateral submetidos à Reabilitação Vestibular, observando-se a relação entre os resultados de exame e sintomas pré e pós-tratamento. A avaliação da resposta clínica foi feita por meio de escala analógico-visual. RESULTADOS: Após a Reabilitação Vestibular, 7 (87,5%) dos 8 pacientes submetidos à terapia apresentaram melhora clínica. CONCLUSÃO: embora não seja esperada melhora completa do equilíbrio corporal, a Reabilitação Vestibular é uma terapia eficaz na recuperação desses pacientes.

Paralisia facial periférica bilateral na leucemia linfóide aguda: relato de caso

A mímica facial é fundamental para a expressão e comunicação humana, que são possíveis apenas através da integridade do nervo facial. Sendo assim, a paralisia facial periférica (PFP) pode deixar seqüelas estéticas, funcionais e psicológicas. A causa mais comum é a paralisia de Bell (50 a 80%), onde a maioria dos pacientes apresenta manifestação unilateral. O acometimento bilateral simultâneo é raro, sendo a leucemia a neoplasia que com maior freqüência pode resultar nesse tipo de manifestação. A seguir, relatamos o caso de um paciente de dezoito anos de idade apresentando leucemia linfóide aguda (LLA) e PFP simultânea, ambas refratárias ao tratamento quimioterápico, culminando com o óbito cinco meses após o início da PFP. Realizou-se considerações importantes sobre a fisiopatologia da PFP na LLA, além de uma revisão da literatura.

Paraganglioma carotídeo bilateral

Os paragangliomas do corpo carotídeo são lesões incomuns, embora representem a maioria dos paragangliomas da cabeça e pescoço. A presença de lesão bilateral é rara. Os autores apresentam um caso de paraganglioma bilateral, dando ênfase aos aspectos clínicos, diagnósticos e tratamento.

Evolução de 31 crianças submetidas à ressecção bilateral das glândulas submandibulares e ligadura dos ductos parotídeos para controlar a sialorréia

OBJETIVOS: Avaliar a segurança da ressecção bilateral das glândulas submandibulares e ligadura dos ductos parotídeos em crianças para reduzir a saliva, a eficácia e as complicações em médio e longo prazo no tratamento da sialorréia. FORMA DE ESTUDO: Coorte longitudinal. MATERIAL E MÉTODO: Trinta e uma crianças, com idades entre 6 e 13 anos (média de 7,6 anos), com múltiplas deficiências de etiologia neurológica foram submetidas à ressecção bilateral das glândulas submandibulares e ligadura dos ductos parotídeos, para controle da sialorréia, entre dezembro de 1999 a dezembro de 2005, e seguimento médio de 36 meses. RESULTADOS: O critério de sucesso foi o estabelecido por Wilkie, e 87% das crianças tiveram resultados excelentes e bons, sendo a morbidade insignificante e a principal complicação foi o edema temporário na região parotídea. CONCLUSÃO: A ressecção bilateral das glândulas submandibulares e a ligadura dos ductos parotídeos constituem técnica segura e eficaz para ser realizada em crianças, com 87% de sucesso no controle da sialorréia.