Symptoma

Congenital Unilateral Pulmonary Hypoplasia

Familial Primary Pulmonary Hypoplasia

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]

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]

The age of presentation is variable and depends on the type of lesion present. [jpma.org.pk]

On auscultation, normal vesicular breath sounds were present in right hemi-thorax. Broncho-vesicular breath sounds were present in left supra-clavicular, infra-clavicular and supra-scapular regions. [medresearch.in]

Neonatal presentation does not allow for a reliable prognosis, ranging from mild repiratory distress to severe pulmonary failure requiring mechanical ventilation. [content.iospress.com]

  • Pseudotumor

    […] of the gastrointestinal tract in infancy and childhood 508 Benign pneumatosis 509 Reduced intestinal gas 510 Increased distance between the intestinal loops 511 Intraabdominal cysts in children 512 Migratory peritoneal calcifications 514 Peritoneal pseudotumors [books.google.com]

  • Arachnodactyly

    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]

  • Pulmonary Hypoplasia and Dysplasia

    Severe primary pulmonary hypoplasia (“acinar dysplasia”) in sibs: a genetically determined mesodermal defect?. J Med Genet 1998; 35: 964-965 28 Al-Senan KA. Kattan AK. Al-Dayel FH. Congenital acinar dysplasia. [thieme-connect.com]

    Case report A case showing pulmonary hypoplasia, hip dysplasia, hereditary renal adysplasia, and Mayer-Rokitansky-Kuster-Hauser syndrome in adulthood is reported here. [ojrd.biomedcentral.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]

  • 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]

    Signs And Symptoms Displacement (attribute) Displacement (morphologic abnormality) Organ Affected Structure of lens of eye (body structure) Entire lens (body structure) Also Known As Congenital displaced lens Congenital ectopia lentis Familial ectopia [codelay.com]

  • Meningism

    Pulmonary Hypertension of the Newborn; Pathophysiology of Meconium Aspiration Syndrome; Pathophysiology of Ventilator Dependent Infants; Pathophysiology of Hypoxic-Ischemic Brain Injury; Pathophysiology of Neonatal White Matter Injury; Pathophysiology of Meningitis [books.google.com]

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]

  • Bowel Distention

    distention 484 Malabsorption patterns of the small bowel 486 Fixed stiff small bowel loops 487 Destruction of the small bowel mucosa 488 Deformation of the terminal ileum 489 Lesions mimikig regional enteritis 490 Abnormalities of the ileocecal valve [books.google.com]

  • Colonic Stricture

    stricture or narrowing 496 Segmental colonic narrowing in children 497 Toxic megacolon 498 Distention of the small andor large bowelnonobstructive paralytic ileus 499 Distended large bowel 500 Large bowel obstruction 502 Large retrorectal space 503 Large [books.google.com]

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]

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]

Treatment - Congenital unilateral pulmonary hypoplasia Not supplied. Resources - Congenital unilateral pulmonary hypoplasia Underdeveloped lungs: [checkorphan.org]

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]

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]

In general, intralobar sequestration has an excellent prognosis, whereas extralobar sequestration has a poor prognosis because of the high incidence of other defects and hydrops. Copyright 2000 by Pilu, Nicolaides, Ximenes & Jeanty [sonoworld.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]

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]

[…] interstitial and granulonodulo alveolar pattern 324 Interstitialreticular pulmonary pattern 326 Disseminated interstitial pneumonitis 327 The miliary pattern of pneumonia 328 Short linear andor wedgeshaped densities 329 Pulmonary edema in infancy and childhood Etiology [books.google.com]

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]

Etiology Diaphragmatic hernia is usually a sporadic abnormality. [sonoworld.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]

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]

[…] 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]

PubMed Google Scholar Glick PL, Siebert JR, Benjamin DR: Pathophysiology of congenital diaphragmatic hernia: I. [ojrd.biomedcentral.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 - Congenital unilateral pulmonary hypoplasia Not supplied. [checkorphan.org]

Second, it can reverse fetal hydrops, resolve polyhydramnios and thereby reduce the risk of preterm delivery, and may prevent pulmonary hypoplasia. [sonoworld.com]

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]

Some birth defects can be prevented. Taking folic acid can help prevent some birth defects. Talk to your doctor about any medicines you take. Some medicines can cause serious birth defects. [codelay.com]