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Congenital Pulmonary Hypoplasia

Lung Hypoplasia


Presentation

  • We report a full-term male neonate who presented with respiratory failure immediately after birth. Chest X-ray revealed a small lung volume despite advanced ventilator support. Respiratory failure persisted and this baby died at 40.5 hours of age.[ncbi.nlm.nih.gov]
  • It seems logical and timely that the clinical aspects should now be presented.[books.google.com]
  • On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery, are[icd10data.com]
  • Apart from the gas exchange layer, well-documented changes are present in the vascular components consisting of media hyperplasia, peripheral muscularization of pre-acinar vessels and adventitial thickening.[repub.eur.nl]
  • We report on two cases that presented as severe progressive respiratory failure immediately after birth in siblings of a consanguinous marriage, and we postulate that a genetic aetiology may be responsible for the arrest of lung maturation in utero.[ncbi.nlm.nih.gov]
Crying
  • This made me cry. My daughter had two undiagnosed heart defects, and she stopped breathing at 5 days old.[babygaga.com]
Sputum
  • MAC infection was soon confirmed by sputum examination and he was treated with RFP, EB, INH combined with CAM. In spite of the chemotherapy, sputum examination of the patient remained positive.[jstage.jst.go.jp]
Receding Chin
  • For example, with congenital diaphragmatic hernia, P-Hyp will materialize in a boat-shaped abdomen; with olighydramnios, the baby's face will have wide-apart eyes, low-set ears, receding chin, and a flat nose; with lesions in the central nervous system[empowher.com]
Fair Complexion
  • I kept asking about decelerations (drop in heart rate) the week before I had her, her arrhythmia (irregular heart beat), her mostly sleeping, only eating an oz. every 3-4 hours when she would wake up, and her "fair complexion".[babygaga.com]

Workup

Mycobacterium Avium Complex
  • Abstract A case of W-P-W syndrome complicated with pulmonary hypoplasia disclosed by pneumonectomy for pulmonary Mycobacterium avium complex infection associated with intractable pneumothorax was reported.[jstage.jst.go.jp]

Treatment

  • Multidisciplinary contributions on medical treatment, radiation oncology, and surgery and anesthesia are included. Highlights include new material on minimally invasive procedures and thoroughly updated diagnostic and treatment information.[books.google.com]
  • Depending on the size of the fetal lungs and the cause of the pulmonary hypoplasia, different treatment plans can be recommended.[cardinalglennon.com]
  • In contrast, due to the absence of suffi cient lung-protective strategies, most of the newer treatment modalities have replaced mortality for a higher morbidity in these babies.[repub.eur.nl]
  • Babies with birth defects often need special care and treatments. The treatments may include surgery, medicines, assistive devices, and therapies.[icdlist.com]
  • Treatment - Congenital unilateral pulmonary hypoplasia Not supplied. Resources - Congenital unilateral pulmonary hypoplasia Underdeveloped lungs:[checkorphan.org]

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]
  • This is a disease with its own prognosis and its known side effects for the baby before and after birth. (link) Some of these remain present, even if FETO is undertaken prior to birth.[totaltrial.eu]
  • 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]
  • What is the prognosis for patients managed in the recommended ways? Most patients do well after surgical removal of CALA and long-term prognosis is good.[clinicaladvisor.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]

Etiology

  • However, a sound understanding of the etiology and pathogenesis of CDH is necessary in order to prevent the severe morbidity or the anomalies altogether.[repub.eur.nl]
  • 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]
  • Genetic, teratogenic and mechanical factors have been implicated in the etiology of pulmonary agenesis [ 5 , 6 ]. Karyotype was normal in the index case.[omicsonline.org]
  • A thorough history as to the quality and timing of cough and wheezing may help rule out certain etiologies. Physical exam and imaging will also narrow the differential, along with imaging.[clinicaladvisor.com]

Epidemiology

  • Congenital cytomegalovirus infection: review of the epidemiology and outcome. Obstet Gynecol Surv. 2002; 57 :245–256. [ PubMed ] [ Google Scholar ] 8. Fowler KB, Stagno S, Pass RF.[ncbi.nlm.nih.gov]
  • 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

  • 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]
  • 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

  • We recently reported that antenatal treatment with sildenafil or simvastatin prevented pulmonary hypoplasia and PPHN in experimental CDH. Objectives.[erj.ersjournals.com]
  • However, a sound understanding of the etiology and pathogenesis of CDH is necessary in order to prevent the severe morbidity or the anomalies altogether.[repub.eur.nl]
  • Prevention - Congenital unilateral pulmonary hypoplasia Not supplied.[checkorphan.org]
  • Centers for Disease Control and Prevention Intersex (Medical Encyclopedia) [ Read More ] Lung Diseases When you breathe, your lungs take in oxygen from the air and deliver it to the bloodstream. The cells in your body need oxygen to work and grow.[icdlist.com]
  • Most often the pulmonary hypoplasia is a secondary problem due to another diagnosis that is preventing proper development of the lungs such as: Congenital diaphragmatic hernia (CDH) : The lungs are compressed by the abnormal position of the intestines[cardinalglennon.com]

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