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Syndrome Presenting (syndrome + presenting)

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Medical Sciences	100%

Selected Abstracts

Hypereosinophilic Syndrome Presenting with Biventricular Cardiac Thrombi

ECHOCARDIOGRAPHY, Issue 6 2010
Ankur Lodha M.D.
Hypereosinophilic syndrome is a rare condition characterized by idiopathic eosinophilia with organ system involvement. Cardiac involvement portends a less favorable prognosis as it can be complicated by development of heart failure, valvular dysfunction, and restrictive cardiomyopathy. We present a rare case of hypereosinophilic syndrome with FIP1L1/PDGFRA fusion in a 50-year-old male associated with thrombus in left and right ventricle. (Echocardiography 2010;27:E57-E59) [source]

A Case of Brugada Syndrome Presenting With Incessant Polymorphic Ventricular Tachycardia

CLINICAL CARDIOLOGY, Issue 3 2010
Harn-Cherng Shiue MD
Brugada syndrome, an inherited arrhythmogenic cardiac disease, manifests with ST-segment changes in the right precordial leads, right bundle block pattern, and susceptibility to ventricular tachyarrhythmias and sudden death. The only established therapy for this disease is prevention of sudden death by implantation of a defibrillator. Herein we describe a case of a patient who presented with incessant ventricular tachycardia (VT) and syncope and who had a type 1 Brugada pattern on ECG. The patient was successfully treated with quinidine, after which the classically described type 2 and 3 patterns emerged. Copyright © 2009 Wiley Periodicals, Inc. [source]

Paraquat-induced Fanconi syndrome

NEPHROLOGY, Issue 5 2005
HYO W GIL
SUMMARY: The ingestion of paraquat, a non-selective herbicide, can be fatal in humans. Paraquat is toxic to multiple organs, including the kidney, heart, gastrointestinal tract and central nervous system. Although paraquat has been established as one cause of acute tubular necrosis, Fanconi syndrome presenting as severe hypophosphataemia after paraquat intoxication has not been reported. Here, we report the case of a 44-year-old Korean woman who presented with generalized proximal tubular dysfunction including aminoaciduria, phosphaturia and glycosuria after paraquat intoxication. We found that severe hypophosphataemia induces deep drowsiness. Renal biopsy findings indicated the presence of acute tubular necrosis that may be reversible. [source]

Hypereosinophilic syndrome presenting as cutaneous necrotizing eosinophilic vasculitis and Raynaud's phenomenon complicated by digital gangrene

BRITISH JOURNAL OF DERMATOLOGY, Issue 3 2000
K-A. Jang
Cutaneous necrotizing eosinophilic vasculitis is a recently identified type of vasculitis that is characterized by an eosinophil-predominant necrotizing vasculitis affecting small dermal vessels. Clinically, it presents with pruritic erythematous and purpuric papules and plaques, peripheral eosinophilia and a good response to systemic steroid therapy. This vasculitis can be idiopathic or associated with connective tissue diseases. Although the pathogenic roles of eosinophil-derived granule proteins and interleukins have been documented in diseases associated with eosinophilia, a role of CD40 (a glycoprotein of the tumour necrosis factor receptor superfamily) has rarely been described. We describe two patients with idiopathic hypereosinophilic syndrome (HES) presenting with multiple erythematous patches and plaques on the lower extremities and Raynaud's phenomenon. They satisfied the criteria for the diagnosis of HES by clinical and laboratory investigations. Histopathology of the cutaneous lesions revealed prominent eosinophilic infiltration with local fibrinoid change in vessel walls in the dermis and subcutis. Immunohistochemical detection of CD3, CD4, CD8 and CD40 was performed. Infiltrating eosinophils were strongly stained by anti-CD40 monoclonal antibody. One patient improved with prednisolone, pentoxifylline and nifedipine, without recurrence. The other patient initially improved with steroids, but after self-withdrawal of steroid developed digital ischaemia that evolved to severe necrosis and required amputation. Cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may develop as cutaneous manifestations of HES. CD40 may play a part in the pathogenesis of eosinophilic vasculitis in HES. [source]

Blepharophimosis: a recommendation for early surgery in patients with severe ptosis

CLINICAL & EXPERIMENTAL OPHTHALMOLOGY, Issue 2 2003
Peter S Beckingsale MB BS
Abstract Purpose:,To determine the optimal age for surgical correction of blepharophimosis. Associated features and their effects on incidence of amblyopia were also investigated. Methods:,The study was a retrospective case series of 28 patients with blepharophimosis, ptosis and epicanthus inversus syndrome presenting to a tertiary referral eyelid, lacrimal and orbital clinic. Results:,Amblyopia was present in 39% of patients. Patients with coexistent strabismus had a 64% incidence of amblyopia compared to 24% for those without strabismus. Hypermetropia was present in 43% of patients and 7% were myopic. Significant astigmatism was found in 40% of patients, but these factors did not increase the risk of amblyopia. Patients with severe ptosis had lower rates of amblyopia than those with moderate ptosis but had their ptosis corrected at a median age of 2 years compared to 5 years for those with moderate ptosis. There was an 18% incidence of nasolacrimal drainage problems. A good to excellent cosmetic outcome was achieved in 86% of patients. A positive family history was noted in 75% of patients, usually with paternal inheritance. Conclusions:,Patients with blepharophimosis have a high rate of amblyopia. Co-existent strabismus doubles the risk of amblyopia. Ptosis alone causes mild to moderate amblyopia only. Patients with severe ptosis should have their ptosis corrected before 3 years of age, and all other patients should undergo surgery before 5 years of age. [source]