Helical form of hypertrophic cardiomyopathy: a new entity?

A 73-year-old woman was admitted to the hospital because ofrecent onset of progressive dyspnea, fatigue, and malaise. Onphysical examination, an ejective systolic murmur (grade 2-3/6)split from S1     

An autopsy case of isolated eosinophilic coronary periarteritis: a limited form of Churg-Strauss syndrome or a new entity?

A 52-year-old man without a history of asthma or allergic diseases died of ventricular fibrillation early in the morning. His autopsy revealed no significant findings, except for a mild mural-thickening localized at the proximal region of the right coronary artery. Microscopic examination showed periarteritis with infiltration of numerous eosinophils in the adventitia. No significant vasculitis was found in any other organs. Based on the findings this seems to be the second reported case of isolated eosinophilic coronary periarteritis.     

Isolated inflammatory coxitis associated with protrusio acetabuli: a new form of juvenile idiopathic arthritis?

BACKGROUND: Isolated hip disease in the context of chronic childhood inflammatory arthritis is uncommon. This paper reports 14 children who presented to the rheumatology and orthopaedic departments of our hospitals with severe hip symptoms, and who continued to have primarily hip disease throughout their clinical course. Our aim was to characterize and present the relevant demographic, clinical, investigational, treatment and outcome data from the above cohort. METHODS: All paediatric cases with the diagnosis of protrusio acetabuli, Otto pelvis or idiopathic chondrolysis who were seen in the past 15 yr at Great Ormond Hospital and Middlesex Hospital in London were identified and their case notes were searched retrospectively for relevant information. RESULTS: In 11 cases, the disease progressed to involve no joints other than the contralateral hip. None were considered to have a specific subtype of juvenile idiopathic arthritis (JIA) and all tested were negative for HLA-B27. Elevation of serum inflammatory markers was variable. Protrusio acetabuli was the predominant radiological feature. There were definite inflammatory changes on the gadolinium-enhanced magnetic resonance imaging study in all patients who had this procedure performed (seven cases). Microbiological investigations were all consistently negative. Severe hip disease resulted in considerable ongoing symptoms and disability. Six cases were treated with disease-modifying anti-rheumatic drugs. Total hip replacement has been required in four patients to date, with major functional improvement. CONCLUSIONS: These cases represent severe and disabling primary hip disease with considerable clinical and investigational inflammatory features. Such a mode of presentation has not been described previously in the context of childhood chronic inflammatory arthritides, and may represent a separate oligoarthritis subtype of JIA.     

Cholera: a new homeland in Africa?

Cholera was largely eliminated from industrialized countries by water and sewage treatment over a century ago. Today it remains a significant cause of morbidity and mortality in developing countries, where it is a marker for inadequate drinking water and sanitation infrastructure. Death from cholera can be prevented through simple treatment-oral, or in severe cases, intravenous rehydration. The cholera case-fatality rate therefore reflects access to basic health care. We reviewed World Health Organization (WHO) data on cholera cases and deaths reported between 1960 and 2005. In the 1960s, at the beginning of the seventh and current cholera pandemic, cholera had an exclusively Asian focus. In 1970, the pandemic reached sub-Saharan Africa, where it has remained entrenched. In 1991, the seventh pandemic reached Latin America, resulting in nearly 1 million reported cases from the region within 3 years. In contrast to the persisting situation in Africa, cholera was largely eliminated from Latin America within a decade. In 2005, 31 (78%) of the 40 countries that reported indigenous cases of cholera to WHO were in sub-Saharan Africa. The reported incidence of indigenous cholera in sub-Saharan Africa in 2005 (166 cases/million population) was 95 times higher than the reported incidence in Asia (1.74 cases/million population) and 16,600 times higher than the reported incidence in Latin America (0.01 cases/million population). In that same year, the cholera case fatality rate in sub-Saharan Africa (1.8%) was 3 times higher than that in Asia (0.6%); no cholera deaths were reported in Latin America. The persistence or control of cholera in Africa will be a key indicator of global efforts to reach the Millennium Development Goals and of recent commitments by leaders of the G-8 countries to increase development aid to the region.     

Mycobacterium interjectum: a new pathogen in humans?

Molecular genetic techniques have increased the number of species recognized within the genus Mycobacterium. The clinical significance of these is uncertain and their pathogenic potential has still to be proven. We describe here a case of mycobacterial lymphadenitis in a Swedish boy, which supports the role of M. interjectum as a human pathogen.     

Role of recombinant human TSH in the management of large euthyroid multinodular goitre: a new therapeutic option? Pros and cons

Conventional 131I treatment has been used in the last 20 years for large nodular goitres when patients present high surgical risk or simply refuse surgery. 131I therapy causes a mean goitre volume reduction of about 40% after one year. However, the individual response is variable and for low radioiodine uptake and very large goitres, high 131I activities are needed in order to have a adequate 131I accumulation in the thyroid. rhTSH is approved for thyroid cancer management and has been tested off label in large goitres, in whom increases 131I uptake, thus reducing the 131I amount to be administered. The use of lower 131I activities allows to reduce the radiation burden to body and the time of social life restriction. Moreover, depending on the radiation regulations of the different countries, the 131I therapy could be carried out either as outpatients or in a shorter hospitalization period, implying a decrease of costs. The effects of rhTSH on goitre may be due not only to the 131I uptake increase, but also to a more homogeneous distribution of 131I in the gland, and to the thyroid cell activation that makes them more radiosensitive. Acute adverse effects are due to the surge of thyroid hormone in blood and to the goitre volume increase, that cause cardiac symptoms and tracheal compression, respectively. These effects are probably dose dependent and are negligible for rhTSH lower doses.     

Pentoxifylline therapy: a new adjunct in the treatment of pulmonary hypertension?

Patients with markedly elevated pulmonary vascular resistance, whether caused by primary pulmonary hypertension or by congenital heart disease, have a grave prognosis, regardless of the type of therapy they undergo. This brief report presents our experience in treating 6 patients (4 women and 2 men) having pulmonary vascular obstructive disease, by administrating pentoxifylline (Trental), a drug that has been used in patients with chronic occlusive systemic arterial disease. Our patients underwent treadmill testing before the study and again 1 to 3 months after initiation of the study. Duration of exercise was short in all patients; however, it increased significantly while patients were taking pentoxifylline. These preliminary results are encouraging; however, we were unable to confirm other physiologic improvement with noninvasive study, and placebo effect was not ruled out. Therefore, we believe that use of pentoxifylline in patients with pulmonary vascular obstructive disease warrants further investigation by means of detailed direct hemodynamic measurement.