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Congenital Unilateral Pulmonary Hypoplasia

Familial Primary Pulmonary Hypoplasia


Presentation

  • Cases are presented in a form resembling teaching files, and the images are accompanied by concise informative text.[books.google.com]
  • Abstract Authors report twelve patients who presented unilateral diminished lung volume on chest x-ray and analyze ECG, bronchography, lung scan and angiography performed in this patients.[ncbi.nlm.nih.gov]
  • The age of presentation is variable and depends on the type of lesion present.[jpma.org.pk]
  • Patients with right-sided involvement and isolated UAPA present have a late presentation and may survive to adult age. The majority of the adults are asymptomatic.[jpgmonline.com]
  • Pulmonary hypoplasia or agenesis presenting in adults is extremely rare, and the initial chest radiographs may present significant interpretation challenges.[wajradiology.org]
Sepsis
  • Pulmonary Circulation; The Developing Microbiome of the Fetus and Newborn; Hereditary Contribution to Neonatal Hyperbilirubinemia; Mechanistic Aspects of Phototherapy for Neonatal Hyperbilirubinemia; Cerebellar Development; Pathophysiology of Neonatal Sepsis[books.google.com]
  • Therefore, a complete sepsis evaluation was preformed and antibiotic treatment was added.[austinpublishinggroup.com]
  • Vanessa van der Linden, Natacha Calheiros Petribu, Marcelo Moraes Valenca, Paul M Parizel, Roberto Jose Vieira de Mello In the present case series, the cause of death of infants diagnosed with congenital Zika syndrome (CZS) was lung disease (pneumonia and sepsis[readbyqxmd.com]
Stridor
  • Ninety percent of children present during the 1st year of life, often with biphasic stridor.[pubs.rsna.org]
Ectopia Lentis
  • الصفحة 896 - Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the fibrillin genes on chromosomes 15 and 5. N Engl J Med 1992;326:905-909. ‏[books.google.com]
Fetal Disorder
  • There may be features of other fetal disorders - eg, skeletal dysplasia or 'Potter's facies' in oligohydramnios. Other congenital anomalies - eg, cardiac malformations. Diagnosis Suspect if oligohydramnios is present.[patient.info]

Workup

  • He was presented with recurrent pneumothorax and hemoptysis, and on thorough workup, he was diagnosed to have an absence of left pulmonary artery with hypoplasia of the left lung and associated right-sided aortic arch.[jacpjournal.org]

Treatment

  • Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout.[books.google.com]
  • Treatment - Congenital unilateral pulmonary hypoplasia Not supplied. Resources - Congenital unilateral pulmonary hypoplasia Underdeveloped lungs:[checkorphan.org]
  • Authors believe that diagnostic work-up of a patient with unilateral diminished lung volume on chest x-ray should depend on clinical symptoms and possibility of surgical treatment. Therapeutic approach will depend on clinical and anatomic findings.[ncbi.nlm.nih.gov]
  • It maintains a clinical focus while providing state-of-the-art diagnosis and treatment techniques.[books.google.com]
  • Antenatal diagnosis and treatment of fetal goitrous hypothyroidism: case report and review of the literature. Ultrasound Obstet Gynecol 1995; 6:368-71 9. Hadi HA, Strickland RT.[books.google.de]

Prognosis

  • Diagnosis - Congenital unilateral pulmonary hypoplasia * Home Lung Function Tests * Cold & Flu: Home Testing: * Home Fever Tests * Home Ear Infection Tests * Home Flu Tests Prognosis - Congenital unilateral pulmonary hypoplasia Not supplied.[checkorphan.org]
  • Neonatal presentation does not allow for a reliable prognosis, ranging from mild repiratory distress to severe pulmonary failure requiring mechanical ventilation.[content.iospress.com]
  • Lesions causing fetal hydrops have a poor prognosis. Large lesions, by compressing adjacent lung, can produce pulmonary hypoplasia in nonaffected lobes ( Chapter 387.2 ).[clinicalgate.com]
  • The right-sided form carries the poorest prognosis due to severity of co-existent anomalies. Antenatal diagnosis using fetal ultrasound and MRI allows early diagnosis.[patient.info]
  • […] patients are prone to develop repeated respiratory infection and bronchiectasis in the involved lung. 4 The occurrence of bronchiectasis is frequent but not universal; nevertheless, the presence and type of bronchiectasis may influence clinical course and prognosis[ispub.com]

Etiology

  • T-box-genes, Pitx2, FGgf 10 and cell signaling pathways such as sonic hedgehog and Wnt) may be involved in the etiology of pulmonary malformation.[content.iospress.com]
  • Congenital pulmonary malformation in pediatric patients: review and update on etiology, classification and imaging findings. Radiol Clin North Am 2011 ; 49 ( 5 ): 921 - 948. 2. Berrocal T, Madrid C, Novo S, Gutiérrez J, Arjouilla A, Gómez-León N.[rc.rcjournal.com]
  • […] occur at any level embryonic: from conception 5 th week pseudoglandular: from 5 th -17 th week canalicular: from 16 th - 24 th week: this stage may be more affected by oligohydramnios terminal sac or alveolar period: from 24 th week to term and beyond Etiology[radiopaedia.org]
  • […] the ultimate fibrosis of the inter-alveolar septae; resulting in obliteration of the pulmonary capillary bed and reduction in pulmonary artery blood flow. 5 Swyer-James syndrome is usually the consequence of constrictive bronchiolitis that implicate etiological[ispub.com]
  • Congenital pulmonary malformations in pediatric patients: review and update on etiology, classification, and imaging findings. Radiol Clin North Am 2011; 49: 921-948 4 Langston C. New concepts in the pathology of congenital lung malformations.[thieme-connect.com]

Epidemiology

  • Pulmonary agenesis may be compatible with normal life provided co-existent malformations are thoroughly investigated and managed. [ 1 ] Epidemiology Primary isolated bilateral pulmonary hypoplasia is rare and familial occurrence exceptional. [ 2 ] Pulmonary[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • […] of Neonatal White Matter Injury; Pathophysiology of Meningitis; Pathophysiology of Preeclampsia; and Pathophysiology of Chorioamnionitis.[books.google.com]
  • Abnormalities of fetal lung fluid and lung fluid pressure : the underlying pathophysiology which results in abnormal lung fluid pressure and subsequent pulmonary hypoplasia is unclear.[patient.info]
  • Unilateral absence of pulmonary artery: Pathophysiology, symptoms, diagnosis and current treatment. Arch Cardiovasc Dis 2013;106:448-54. [ PUBMED ] 4. Sunam G, Ceran S. Pulmonary artery agenesis and lung hypoplasia. Eur J Gen Med 2009;6:265-7. 5.[jpgmonline.com]
  • Also consistently with the literature, the congenital malformation most frequently associated with PH was diaphragmatic hernia. 10,24,25 This disease is the model that has been used most commonly for the study of early interventions, the pathophysiology[analesdepediatria.org]

Prevention

  • Prevention - Congenital unilateral pulmonary hypoplasia Not supplied.[checkorphan.org]
  • In view of the high incidence of pulmonary hypertension when UAPA is associated with a cardiovascular shunt, it is important to recognize these patients at an early age and correct the associated shunt to prevent or reverse pulmonary hypertension, if[scipers.com]
  • An early diagnosis and treatment can prevent serious complications. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. References 1. Cucci CE, Doyle EF, Lewis EW Jr.[jacpjournal.org]
  • Vaccines like Influenza and Pneumococcal should be instituted to prevent fulminant infections. Case Report A seven month old male infant presented with complaints of low grade fever and mild respiratory distress.[jpma.org.pk]
  • Blockage prevents the bladder from emptying, and the bladder becomes very large and full. The large volume of the full bladder interferes with normal development of other organs, including the lungs.[en.wikipedia.org]

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