Health supervision for children with Marfan syndrome

Pediatrics
|October 2, 2013
PubMed

Insights

Marfan syndrome is a rare genetic disorder affecting connective tissue. This report guides pediatricians in diagnosing and managing Marfan syndrome, emphasizing a multidisciplinary care approach for affected individuals.

Area of Science:

  • Genetics and наследственное заболевание
  • Pediatrics
  • Connective tissue disorders

Background:

  • Marfan syndrome is a systemic, heritable connective tissue disorder.
  • It impacts multiple organ systems, necessitating comprehensive management.
  • Early recognition and intervention are crucial for patient outcomes.

Purpose of the Study:

  • To provide pediatricians with guidance on recognizing Marfan syndrome features.
  • To support pediatricians in the care of individuals with Marfan syndrome.
  • To promote a multidisciplinary approach to Marfan syndrome management.

Main Methods:

  • This report synthesizes current knowledge and clinical best practices.
  • It focuses on clinical recognition of key diagnostic features.
  • It outlines a framework for multidisciplinary care coordination.

Main Results:

  • The report details the diverse clinical manifestations of Marfan syndrome across organ systems.
  • It emphasizes the importance of a systematic diagnostic approach.
  • It highlights the benefits of a coordinated, multidisciplinary care team.

Conclusions:

  • Pediatricians play a vital role in the early identification of Marfan syndrome.
  • A multidisciplinary approach is essential for optimal management and improved patient outcomes.
  • This guidance aims to enhance the care provided to children and adolescents with Marfan syndrome.

Related Concept Videos

Mitral Stenosis III: Medical Management01:26

Mitral Stenosis III: Medical Management

Mitral stenosis, a condition marked by the narrowing of the mitral valve, necessitates an integrated approach for effective management. This approach includes preventative measures, medical therapy, and surgical interventions to reduce symptoms and prevent complications.PreventionPrevention of mitral stenosis primarily focuses on reducing the incidence of bacterial infections, particularly streptococcal infections, which can lead to rheumatic fever and subsequent valvular damage. Timely...
Mitral Stenosis IV: Nursing Management01:27

Mitral Stenosis IV: Nursing Management

A comprehensive nursing assessment is essential for patients with valvular heart disease, which involves any dysfunction of the heart valves that could impact blood flow and overall heart function.Subjective Data Collection:Chief Complaint and Present Illness: Start with the patient's primary concerns, focusing on the onset, duration, and progression of cardiac symptoms such as dyspnea, fatigue, chest pain, and palpitations.Past Medical History: Collect detailed information on any previous...
Mitral Valve Prolapse III: Nursing Management01:19

Mitral Valve Prolapse III: Nursing Management

The nursing management of Mitral Valve Prolapse, or MVP, centers around patient education, symptom monitoring, and lifestyle modifications.Patient Education on MVP Diagnosis and Heredity: Nurses should provide comprehensive education about MVP, a condition where the mitral valve does not close appropriately during heartbeats. This education often includes the condition's pathophysiology, symptoms, and potential complications, like arrhythmias or mitral regurgitation. Though not fully...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Mitral Valve Prolapse II: Assessment and Management01:22

Mitral Valve Prolapse II: Assessment and Management

IntroductionA range of clinical features characterizes Mitral Valve Prolapse (MVP), but it is important to note that many individuals with MVP are asymptomatic and may remain so throughout their lives. For those who do exhibit symptoms, the following are the key clinical features:Palpitations: This is a common symptom where individuals feel an irregular or rapid heartbeat. Palpitations in MVP are often due to arrhythmias such as premature ventricular contractions or supraventricular tachycardia.
Rheumatic Heart Disease III: Medical Management01:21

Rheumatic Heart Disease III: Medical Management

Rheumatic heart disease (RHD) management can be divided into two main strategies: prevention and long-term management.Primary PreventionPrimary prevention focuses on timely diagnosis and management of group A streptococcal pharyngitis to prevent acute rheumatic fever. The most widely used antibiotic for treating this condition is intramuscular benzathine penicillin G.Acute Rheumatic Fever TreatmentThe primary treatment goal for a patient diagnosed with acute rheumatic fever is to suppress the...