Aug 192012
 

Cyanosis refers to the bluish skin color attributable most often to the presence of desaturated hemoglobin ( 5g/dl ). Primary etiologies include respiratory, cardiac, circulatory and nervous system disorders, as well as abnormal hemoglobin.

Respiratory Diseases

1. Lung disease

Newborn: Lung hypoplasia , Diaphragmatic hernia, respiratory distress syndrome, transient tachypnea of the newborn, Bronchopulmonary dysplasia, Pulmonary interstitial emphysema, Congenital adematoid malformation, Meconium aspiration.

Infectious: Pneumonia, Pneumonitis, Bronchiolitis.

Asthma

Cystic fibrosis

Infiltrative disease: Pulmonary hemosiderosis, sarcoidosis

2. Airway abnormalities or Obstruction

Congenital: choanal atresia, macroglossia, micrognathia, laryngeal web, tracheal stenosis, vascular ring, tracheoesophageal fistula.

Infectious: Acute epiglottitis, croup, retropharyngeal abscess, laryngospasm.

Traumatic: Vocal cord injury, pneumothorax, pneumomediastinum

Others: lymphoma, cystic hygroma, goiter, laryngeal hemangioma or neoplasm, foreign body, obesity.

3. Trauma

Pneumothorax, pneumomediastinum, vocal cord injury.

Pulmonary Vascular Diseases

1. Primary pulmonary hypertension

Cyanosis requires a right to left shunt at the atrial or ductal level unless lung disease is present.

2. Pulmonary arteriovenous malformation

Idiopathic or associated with congenital heart, hepatic or portal disease.

Cardiac Diseases

1. Cyanotic congenital heart diseases Continue reading »

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Aug 092012
 

Rheumatic fever is an autoimmune inflammatory disease that may develop after an infection with Streptococcus bacteria (such as strep throat or scarlet fever). The disease can affect the heart, joints, skin, and brain.

Acute rheumatic fever commonly appears in children between the ages of 6 and 15.

The illness is so named because of its similarity in presentation to rheumatism.

Rheumatic fever is common worldwide and is responsible for many cases of damaged heart valves.

Jones Criteria

Jones criteria are guidelines decided on by the American Heart Association to help doctors clinically diagnose rheumatic fever. Two major criteria or one major and two minor plus a history of a streptococcal throat infection are required to make the diagnosis of rheumatic fever.

The major criteria for diagnosis include

  • arthritis in several joints (polyarthritis),
  • heart inflammation (carditis),
  • nodules under the skin (subcutaneous nodules or Aschoff bodies),
  • rapid, jerky movements (Sydenham’s chorea), and
  • skin rash (erythema marginatum).

The minor criteria include

  • fever,
  • high ESR (erythrocyte sedimentation rate, an laboratory sign of inflammation),
  • joint pain (arthralgia),
  • EKG changes, and
  • other laboratory findings (elevated C-reactive protein, elevated or rising streptococcal antigen test).

Pathophysiology

Acute rheumatic fever is a sequela of a previous group A streptococcal infection, usually of the upper respiratory tract. This is an autoimmune response secondary to molecular mimicry following group A streptococcal pharyngitis.

The disease involves the heart, joints, central nervous system (CNS), skin, and subcutaneous tissues. It is characterized by an exudative and proliferative inflammatory lesion of the connective tissue, especially that of the heart, joints, blood vessels, and subcutaneous tissue. Continue reading »

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Aug 072012
 

Transposition of the Great Arteries is one of the cyanotic congenital heart disorders in which the two main arteries leaving the heart are reversed (transposed).

Transposition of the great vessels is a cyanotic heart defect. This means there is decreased oxygen in the blood that is pumped from the heart to the rest of the body.

In normal hearts, blood that returns from the body goes through the right side of the heart and pulmonary artery to the lungs to get oxygen. The blood then comes back to the left side of the heart and travels out the aorta to the body.

In transposition of the great vessels, the blood goes to the lungs, picks up oxygen, returns to the heart, and then flows right back to the lungs without ever going to the body. Blood from the body returns to the heart and goes back to the body without ever picking up oxygen in the lungs.

Etiology

Etiology for transposition of the great arteries is unknown and is presumed to be multifactorial. Preexisting diabetes mellitus of an expectant mother is a risk factor that has been described for the fetus having TGV.

Clinical Presentation

Infants with transposition of the great arteries (TGA) are usually born at term, with cyanosis apparent within hours of birth.

The clinical course and manifestations depend on the extent of intercirculatory mixing and the presence of associated anatomic lesions.

The common findings include:

  • Blueness of the skin
  • Clubbing of the fingers or toes
  • Poor feeding
  • Shortness of breath.

Workup

If a baby is suspected to have this condition the following tests are usually required: Continue reading »

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