mulibrey nanism is a rare autosomal recessive syndrome caused by a mutation in the trim37 gene with severe growth retardation and multiple organ involvement. early diagnosis is important because 50% of the patients develop congestive heart failure owing to constrictive pericarditis, and this condition plays a critical role in the final prognosis. a 37-year-old female patient presented with symp...

Constrictive pericarditis is caused by fibrosis and calcification of the pericardium, processes that inhibit diastolic filling of the heart. This condition has posed a diagnostic dilemma since it was first recognised clinically. Although many diagnostic approaches have become available subsequently, the diagnostic challenge remains. Because surgical intervention can provide complete relief of s...

Clinical and hemodynamic observations are reported in 13 patients who demonstrated a distinct pathophysiologic form of compressive pericardial disease characterized by effusion into a free pericardial space associated with constriction of the heart by the visceral pericardium. The pericardial disease was idiopathic (nine patients) or subsequent to radiotherapy (four patients), with a maximal du...

Ikram, H., Banim, S. O., and Makey, A. R. (1974). Thorax, 29, 204-208. Clinical features of non-tuberculous constrictive pericarditis. This paper describes the clinical features of five cases of constrictive pericarditis of non-tuberculous aetiology. The findings in this syndrome are compared with those in tuberculous constrictive pericarditis. The non-tuberculous variety had a short history, a...

BACKGROUND The diagnosis of constrictive pericarditis continues to be a clinical challenge. Magnetic resonance imaging provides excellent visualization of the pericardium. The aim of our study is to clarify the contribution of this non invasive exploration in the diagnosis of constrictive pericarditis in our center. METHODS we conducted a prospective study over a period of two years, since 20...

Massive ascites of unknown origin is an uncommon condition, which represent a diagnostic challenge. Patients with delayed diagnosis and treatment may have a poor prognosis. A 22-year-old female was referred to this hospital due to a 4-year progressive abdominal distension with massive ascites of unknown origin. By thorough investigations, she was eventually diagnosed as chronic calcified constr...

Between 1955 and 1972, eight children receiving mediastinal irradiation for intrathoracic malignancy developed constrictive pericarditis. Six of these followed irradiation for Hodgkin's disease and represent 7% of 86 children receiving irradiation for that disease. In patients with Hodgkin's disease, the development of constrictive pericarditis was related to the dose of radiation and the use o...

BACKGROUND Constrictive pericarditis represents a serious hemodynamic syndrome that may lead to heart failure. Studies of its pathophysiological mechanisms have been impeded by the lack of an animal model. METHODS AND RESULTS Cardiac myosin-induced experimental autoimmune myocarditis in interferon (IFN)-gamma-knockout (KO) mice results in increased cardiac inflammation and development of seve...

Constrictive pericarditis is a rare disease, usually the result of long-standing pericardial inflammation leading to pericardial scarring with thickening, fibrosis, and calcification. The most frequent causes are mediastinal radiation, chronic idiopathic pericarditis, cardiac surgery, and tuberculous pericarditis. Nearly 9% of patients with acute pericarditis for any reason go on to develop con...

SIXTY-EIGHT patients with constrictive pericarditis were studied by this laboratory in a 30-year period. During this time 14 patients were encountered whose findings suggested constrictive pericarditis but who showed an abnormal myocardium at operation or autopsy. Two of these patients had both constrictive pericarditis and myocardial fibrosis, each of the other 12 had a no: mal pericardium. Th...