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Medical condition
Pulmonary agenesis
Chest radiograph of a woman with unilateral pulmonary agenesis
Specialty
Pulmonology
Pulmonary agenesis is an inborn lung underdevelopment that is rare and potentially lethal.[1] The disorder is caused by a complete developmental arrest of the primitive lung during embryonic life, and it is often associated with other developmental defects.[2] Bilateral and unilateral pulmonary agenesis are classified, depending on whether one side of the lung or both sides are affected. Bilateral pulmonary agenesis is lethal, while the mortality rate of unilateral pulmonary agenesis is higher than 50%.[3] Depending on the severity, the symptom ranges from none to various respiratory complaints.[4] It is detectable prenatally, however, its nonspecific clinical features act as the obstacle for diagnosing.[2] The exact cause of pulmonary agenesis is still obscure. However, theories have been raised regarding the vascular, iatrogenic, viral and genetic causes of pulmonary agenesis in an attempt to explain the pathogenesis of the disorder.[5] In most cases of pulmonary agenesis, surgical resection is performed to remove the malformed lobe or the entire defected lung of the patient depending on the severity of the respiratory impairment.[6]
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^ abCite error: The named reference e was invoked but never defined (see the help page).
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^Dordea Leonte, Laura (2013). "Fetology. Diagnosis and Management of the Fetal Patient". Acta Endocrinologica. 9 (4): 657. doi:10.4183/aeb.2013.657. ISSN 1841-0987.
^Holder, Tom; Ashcraft, Keith (2010), "Dedication", Ashcraft's Pediatric Surgery, Elsevier, pp. v, doi:10.1016/b978-1-4160-6127-4.00084-7, ISBN 978-1-4160-6127-4
and 24 Related for: Pulmonary agenesis information
Pulmonaryagenesis is an inborn lung underdevelopment that is rare and potentially lethal. The disorder is caused by a complete developmental arrest of...
Pulmonary artery agenesis refers to a rare congenital absence of pulmonary artery due to a malformation in the sixth aortic arch. It can occur bilaterally...
development. For example, pulmonary hypoplasia is the underdevelopment of a lung due to various etiologies. These include agenesis due to gene mutation, fetal...
bilateral renal agenesis in 21.25% of cases; cystic dysplasia in 47.5%; obstructive uropathy in 25%; and others in 5.25%. Bilateral renal agenesis has been estimated...
distinct from pulmonary agenesis, which, while similar, has a short-blind ending bronchus in aplasia. Because bilateral pulmonary aplasia is not feasible...
types of tracheal agenesis, designated Types I, II and III. In 2013, a South Korean child, Hannah Warren, born with tracheal agenesis, was successfully...
presence of pulmonary hypoplasia was taken as evidence that diaphragmatic hernias in babies were congenital, not acquired. Pulmonaryagenesis Potter sequence...
body and tail, which are both absent in agenesis of the dorsal pancreas. Patients with dorsal pancreatic agenesis may result in a defect in the metabolism...
nine months old infant. A family with acute intermittent porphyria. Pulmonaryagenesis—vascular airway compression and gastroesophageal reflux influence...
renal agenesis occurs in 1 in 1000 live births, in contrast bilateral renal agenesis occurs in 1 in 3000 to 4000 pregnancies. Unilateral renal agenesis has...
"Homozygous Null TBX4 Mutations Lead to Posterior Amelia with Pelvic and Pulmonary Hypoplasia". American Journal of Human Genetics. 105 (6): 1294–1301. doi:10...
dysostosis Acyl-CoA oxidase deficiency Adenylosuccinate lyase deficiency Agenesis of the corpus callosum with peripheral neuropathy ALDH18A1-related de Barsy...
1542/peds.6.5.721. PMID 14797335. S2CID 26235702. Obrinsky W (1949). "Agenesis of abdominal muscles with associated malformation of the genitourinary...
defined by the following characteristics: dwarfism; osteopetrosis; partial agenesis of the terminal digits of the hands and feet; cranial anomalies, such as...
oligohydramnios. Affected fetuses develop pulmonary hypoplasia, limb deformities, and characteristic facies. Bilateral agenesis of the fetal kidneys is the most...
the presence of severely hypoplastic main pulmonary arteries and branch pulmonary arteries, alongside agenesis of the patent ductus arteriosus. Blood flow...
can encompass various kidney-related issues, including bilateral renal agenesis, also known as Potter syndrome, which is the most prevalent cause of anhydramnios...
of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood; an analysis of the heart malformations in the splenic agenesis syndrome...
genitourinary system are also common. These include underdevelopment or agenesis of the pancreas, adrenal glands, thymus, gallbladder, and thyroid; Hirschsprung's...
septum. Mitral valve agenesis is very rare, defined as an absence or minimal presence of both mitral valve leaflets (complete agenesis) or one of the leaflets...
embolism, and thrombosis. He coined biological terms such as "neuroglia", "agenesis", "parenchyma", "osteoid", "amyloid degeneration", and "spina bifida";...
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