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The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
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Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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AssessmentA comprehensive assessment is essential in managing a patient with rheumatic heart disease (RHD). Begin with obtaining a detailed medical history, including recent streptococcal infections, a history of rheumatic fever, or previously diagnosed rheumatic heart disease. Assess the patient for symptoms such as fever, chest pain, widespread joint pain (arthralgia), tachycardia, pericardial friction rub, muffled heart sounds, heart murmurs, peripheral edema, subcutaneous nodules, and...
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Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
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Ribosome profiling or ribo-sequencing is a deep sequencing technique that produces a snapshot of active translation in a cell. It selectively sequences the mRNAs protected by ribosomes to get an insight into a cell’s translation landscape at any given point in time.
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Ribbing disease: a systematic review.

Bart G Pijls1,2, Koen Steentjes2, Jan W Schoones3

  • 11 Department of Orthopaedics, Leiden University Medical Center, Leiden, The Netherlands.

Acta Radiologica (Stockholm, Sweden : 1987)
|July 11, 2017
PubMed
Summary

Ribbing disease (multiple diaphyseal sclerosis) primarily affects the tibial diaphysis. Surgical interventions like intramedullary reaming effectively reduce pain, though evidence on long-term outcomes remains limited.

Keywords:
Ribbing diseasehereditary multiple diaphyseal sclerosismultiple diaphyseal sclerosissystematic review

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Area of Science:

  • Orthopedics
  • Radiology
  • Genetics

Background:

  • Ribbing disease, also known as multiple diaphyseal sclerosis, is a rare, benign bone dysplasia.
  • Characterized by sclerosis of the bone diaphysis, it presents a diagnostic challenge due to its rarity.

Purpose of the Study:

  • To systematically review existing literature on Ribbing disease.
  • To elucidate the clinical and radiological presentation of affected patients.
  • To evaluate the efficacy of various treatment modalities.

Main Methods:

  • Comprehensive literature search across multiple databases.
  • Independent review of 23 studies encompassing 40 patients with Ribbing disease.
  • Descriptive and mixed-model statistical analyses of pooled patient data.

Main Results:

  • The tibial diaphysis was the most frequently affected bone (35/36 patients).
  • Mean age at diagnosis was 35 years, with symptoms (primarily pain) appearing 1-16 years post-diagnosis.
  • Non-surgical treatments (NSAIDs, prednisone, bisphosphonates) showed mixed results; surgical treatment (reaming, fenestration) was highly effective for pain relief.

Conclusions:

  • Clinical and imaging features of Ribbing disease are increasingly recognized.
  • Further research is needed to understand the natural disease progression and definitively assess treatment effectiveness.