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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rapidly progressive heart failure, in part related to severe pulmonary hypertension, developed in a patient with biopsy-proved Whipple's disease. The patient's pulmonary hypertension progressed despite antibiotic therapy and histologic remission of his intestinal disease. The combination of oral nifedipine and low-flow continuous oxygen led to both short- and long-term increases of at least 2 liters per minute in cardiac output and reductions of more than 10 mm Hg in mean pulmonary artery pressure. Accompanying these hemodynamic changes was an improvement of more than 10 percent in right ventricular ejection fraction. The relationship between this patient's pulmonary hypertension and his Whipple's disease is not known.
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PMID:Severe pulmonary hypertension in a patient with Whipple's disease. 241 Nov 33

A patient presenting with congestive cardiac failure and anaemia underwent investigation which led to the diagnosis of Whipple's disease, associated with dilated cardiomyopathy. Conventional antibiotic therapy for Whipple's disease resulted in resolution of the traditional features of Whipple's disease and a marked improvement in the patient's heart failure.
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PMID:Symptomatic cardiomyopathy as a presentation in Whipple's disease. 754 Mar 1

Whipple's disease (WD) is an uncommonly diagnosed infection caused by the recently characterized bacillus, Tropheryma whippelii. The association of WD with pericarditis and endocarditis is widely recognized, although less attention has been paid to the myocardium as a site of disease. Although the disease was uniformly fatal before antibiotic therapy, current treatment usually results in cure. We report two patients whose deaths were directly related to cardiac involvement by WD and whose underlying disease escaped diagnosis for years. The first, a 60-year-old white woman, suffered a cardiovascular collapse, and lymphocytic myocarditis was demonstrated at autopsy. The second, a 48-year-old black man, had a lengthy history of progressive cardiac failure that terminated in arrhythmia. Extensive myocardial fibrosis, with lymphocytic and granulomatous inflammation, was demonstrated at autopsy. The presence of T. whippelii was confirmed by electron microscopic examination in both cases and by polymerase chain reaction in one. Patients with WD might harbor an undiagnosed lymphocytic or granulomatous myocarditis, and this diagnosis should be considered in the evaluation of cardiac failure.
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PMID:Myocarditis in Whipple's disease: an unsuspected cause of symptoms and sudden death. 919 67

A 14-year-old boy presented with a 3-year history of a skin rash typical of juvenile dermatomyositis, and a 2-month history of mild proximal weakness, myalgia, and weight loss. A quadriceps biopsy showed perifascicular fibre atrophy, focal necrosis and regeneration, immunohistochemical labelling for HLA-1 on the surface of the fibres, and focal C5-9 deposition in capillaries. Macrophages with diastase-resistant, PAS-positive cytoplasm were present. Ultrastructural studies showed electron dense and membranous debris. The patient's symptoms responded to intravenous immunoglobulin and oral prednisolone. Four months after discontinuing prednisolone, the patient developed cardiac failure, ventricular tachycardia, and a recurrence of his rash. The 16S ribosomal RNA specific for Tropheryma whippelii was identified by polymerase chain reaction (PCR) analysis in skeletal and cardiac muscle. The myalgia and skin rash responded to prednisolone and oral co-trimoxazole, and the tachycardia is controlled by oral verapamil. This patient appears to have a novel association of juvenile dermatomyositis and Whipple's disease.
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PMID:Dermatomyositis and Whipple's disease. 1067 63

Whipple's disease (WD) is a rare chronic infectious disorder caused by the rod- shaped bacterium Tropheryma whipplei. The disorder is characterized clinically by arthralgia, abdominal pain, diarrhea, malabsorbtion and progressive weight loss. Other important sites of infection include the heart (resulting in the clinical picture of endocarditis and heart failure) and the central nervous system (CNS) (manifestations include confusion, memory loss, focal cranial nerve signs, nystagmus and ophtalmoplegia). The bacterium is presumed to be ubiquitously present. A defect in cellular immune response may predispose patients for an infection with T. whipplei and this might explain the rarity of the disorder despite the ubiquitous bacterial presence. The presumed immunological defect is likely to be quite specific for T. whipplei, since patients are not generally affected by other infections. Decreased production of Interleukin(IL)-12, IL-2 and Interferon (IFN)-g accompanied by an increased secretion of IL-4 are the main features of this defective immunological response. The finding of periodic acid-Schiff (PAS)-positive macrophages in the lamina propria of tissue samples obtained by duodenal biopsy usually establishes the diagnosis. The PAS-positive inclusions represent the remnants of the bacteria. Attempts to isolate the causative agent were unsuccessful for nearby 100 years after the first recognition of the disease. In the year 2000, the bacterium was finally successfully grown on a human fibroblast cell line. Untreated WD patients suffer from a chronic progressive disorder which possibly leads to death. Most patients show a fast clinical improvement to antibiotic therapy, but clinical relapses are described frequently. There is a number of patients, unable to eradicate the bacterium even after several antibiotic treatments and patients with CNS disease, in both of whom alternative therapy strategies are necessary.
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PMID:Current concepts of immunopathogenesis, diagnosis and therapy in Whipple's disease. 1707 38

Whipple's disease or intestinal lipodystrophy is an infection induced by Tropheryma whipplei. It is rare with an estimated incidence of 0.4 per million. Symptoms are arthropathy, weight loss, and diarrhoea, but other organs notably the central nervous system may be affected. We demonstrate a case of cardiac complications in Whipple's disease. The patient presented with endocardial infiltrations on TEE examinations and heart failure and improved after antibiotic treatment.
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PMID:Cardiac complications in Whipple's disease. 1849 Mar 46

Whipple's disease, caused by the bacterium Tropheryma whipplei, is a rare chronic multi-system illness commonly affecting the gastrointestinal (GI) tract and presenting with a triad of diarrhoea, weight loss and malabsorption. While 20-55% of patients with a diagnosis of Whipple's disease have clinically evident cardiac manifestations, the initial presentation with isolated valvular disease, without any GI symptoms, is rare. Whereas cardiac involvement usually involves a single valve, cases of double-valve involvement are extremely rare. We report the case of a patient with T. whipplei native aortic and mitral valvular endocarditis, without GI involvement, who presented with the new-onset cardiac failure and ventricular arrhythmias, which required urgent double-valve replacement. This case report is accompanied by a review of the relevant literature.
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PMID:Tropheryma whipplei endocarditis without gastrointestinal involvement. 2249 4

Tropheryma whipplei culture and genome sequencing have, thanks to new tools, rationalized both diagnosis and treatment of Whipple's disease. Whipple's disease involves mainly Caucasian male, approximately 50-years-old, suffering from arthralgia, weight loss and diarrhea. A worsening of clinical manifestations after immunosuppressive therapy is frequently observed while antibiotics prescribed for another infection improves the clinical status. Clinical manifestations may more rarely suggest lymphoma or sarcoidosis. Positive T. whipplei performed on both saliva and stool samples are strongly suggestive of Whipple's disease. The diagnosis is confirmed by positive periodic acid Schiff staining or immunohistochemistry performed on small-bowel biopsies. Localized chronic infections are defined by the absence of histological duodenal involvement. Endocarditis mainly occurs in 60-years-old men with arthralgia, cardiac failure or embolic events. Encephalitis causes mainly cognitive and psychiatric involvement, and sometimes dementia, ataxia and weight gain. Uveitis and arthritis evolve chronically, and are frequently resistant to immunosuppressive treatment. PCR and culture (particularly for cerebrospinal fluid) performed on various tissues and fluids allow the localized infections diagnosis. In classic Whipple's disease, the treatment with doxycycline (200mg/day) and hydroxychloroquine (600 mg/day) for a length of 12 months followed by a lifetime treatment by doxycycline (200 mg/day) should be recommended to avoid reinfection. In localized infections, a treatment with doxycycline (200mg/day) and hydroxychloroquine (600 mg/day) is proposed for 12 to 18 months followed by a lifetime follow-up.
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PMID:[Whipple's disease and Tropheryma whipplei infections in internal medicine. When to think about it? How to treat?]. 2493 90

Whipple's disease is a rare infective condition, classically presenting with gastrointestinal manifestations. It is increasingly recognized as an important cause of culture-negative endocarditis. We present a case of Whipple's endocarditis presenting with heart failure. A literature review identified 44 publications documenting 169 patients with Whipple's endocarditis. The average age was 57.1 years. There is a clear sex predominance, with 85% of cases being male. Presenting symptoms were primarily articular involvement (52%) and heart failure (41%). In the majority of cases, the diagnosis was made on examination of valvular tissue. Preexisting valvular abnormalities were reported in 21%. The aortic valve was most commonly involved, and multiple valves were involved in 64% and 23% of cases, respectively. Antibiotic therapy was widely varied and included a ceftriaxone, trimethoprim, and sulfamethoxazole combination. The average follow-up was 20 months, and mortality was approximately 24%. Physician awareness is paramount in the diagnosis and management of this rare condition.
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PMID:Tropheryma whipplei Endocarditis: Case Presentation and Review of the Literature. 3064 25