VEPTR (Vertical Expandible Prosthetic Titanium Rib) treatment for Jeune syndrome

Oxygen delivery is critical in clinical care, especially for patients with respiratory disorders or those undergoing surgical procedures. Various systems, such as tracheostomy and the T-piece, deliver oxygen to the lungs, ensuring adequate arterial oxygenation.
Tracheostomy
A tracheostomy is a surgically created opening (stoma) in the anterior part of the trachea. It is used to establish a patient airway, bypass an upper airway obstruction, simplify the removal of secretions, permit long-term...

Ribs are curved, flattened bones forming the thoracic cavity wall with the thoracic muscles. There are 12 pairs of thoracic ribs. The posterior ends of all the ribs articulate with the T1–T12 thoracic vertebrae. In contrast,the anterior ends of most ribs attach to the sternum via their costal cartilages.
Parts of a Typical Rib
A typical rib has a head, neck, and body. The posterior end of the rib is called the head, followed by a narrow neck. The head articulates primarily with the costal facet...

Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme (ECE). Of...

A tracheostomy is a surgical procedure that creates an artificial opening into the trachea, typically at the second or third cartilaginous ring level. This opening allows the insertion of a tracheostomy tube, which can replace an endotracheal tube, provide mechanical ventilation, bypass an upper airway obstruction, or remove accumulated tracheobronchial secretions.
Tracheostomy tubes can be made of semiflexible plastic (polyurethane or silicone), rigid plastic, or metal, and they come in...

Prostacyclin receptor agonists are a class of therapeutic agents integral to managing pulmonary arterial hypertension (PAH). These drugs operate by mimicking the action of prostaglandin I2, or PGI2, a naturally occurring compound in the body.
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...

Oxygen therapy has emerged as a significant tool in enhancing the quality of life for patients suffering from pulmonary arterial hypertension (PAH). While this therapy has principally been studied on patients with significant hypoxemia, this therapeutic approach helps prevent potential organ damage and can be administered in the comfort of one's home.
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue, improving...