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Pulmonary Hypoplasia and Dysplasia

Agenesis Hypoplasia and Dysplasia of Lung Congenital


Presentation

  • CRANIAL BONE VARIATIONS 217 Vertex Birth Molding 219 Vertex Craniotabes 226 Anterior Fontanelle Bone 230 Parietal Foramina 232 Aplasia Cutis Congenita 235 Cephalohematoma 240 Wormian Bones 245 ABNORMAL BIRTH PRESENTATION 249 Breech Presentation Deformation[books.google.com]
  • Three other publications as co-author are presented at the end of the manuscript as "additional contributions".[books.google.com]
  • الصفحة 207 - Pettenati MJ, Haines JL, Higgins RR, Wappner RS, Palmer CG, Weaver DD: Wiedemann-Beckwith syndrome: presentation of clinical and cytogenetic data on 22 cases and review of the literature. ‏[books.google.com]
  • Some CNVs (17q12 and 22q11.21 microdeletions) described in other MRKH patients were not present in this case.[ojrd.biomedcentral.com]
  • For additional information about lung transplants, please click here. back to top Late Presenters and Long-Term Survivors Reports of infants presenting with clinical features of ACDMPV beyond the neonatal period have begun to emerge.[acdassociation.org]
Pathologist
  • Consulting a pathologist with experience in making this diagnosis may be helpful. Pathological features include a paucity of alveolar capillaries, widened alveolar septae, and increased muscularization of pulmonary arterioles.[acdassociation.org]
  • Discussion Renal agenesis and dysplasia (both unilateral and bilateral) are frequently observed in fetuses and newborns, and have been commonly considered by pathologists and pediatricians to be sporadic malformations.[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]
Pelvic Pain
  • The patient reported three years of genital and mammary development as well as cyclic pelvic pain for 4-5 days every month despite the primary amenorrhea. She weighed 42 kg and was 153 cm tall.[ojrd.biomedcentral.com]
Primary Amenorrhea
  • The patient reported three years of genital and mammary development as well as cyclic pelvic pain for 4-5 days every month despite the primary amenorrhea. She weighed 42 kg and was 153 cm tall.[ojrd.biomedcentral.com]
Akinesia
  • ABNORMAL BIRTH PRESENTATION 249 Breech Presentation Deformation 251 Transverse Lie Deformation 263 Face and Brow Presentation Deformation 266 WHOLEBODY DEFORMATION OR DISRUPTION 271 Small Uterine Cavity Deformation 273 Oligohydramnios Sequence 279 Fetal Akinesia[books.google.com]
  • There is probably an overlap of aetiologies. [ 4 ] However, pulmonary hypoplasia may also be idiopathic or related to other syndromes and congenital anomalies - eg, multiple pterygium syndrome/fetal akinesia-hypokinesia sequence (autosomal recessive)[patient.info]
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]

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]
  • It maintains a clinical focus while providing state-of-the-art diagnosis and treatment techniques.[books.google.com]
  • TREATMENT The treatment and management of FRNS is symptomatic such as corrective surgery for hernia repair. Standard supportive treatment is needed for children with cardiac, renal, and pulmonary involvement.[secure.ssa.gov]
  • Early treatment aimed at the underlying disease process may prevent progression. The prognosis is determined by the reversibility of the underlying disease process.[pedsinreview.aappublications.org]
  • Depending on the size of the fetal lungs and the cause of the pulmonary hypoplasia, different treatment plans can be recommended.[cardinalglennon.com]

Prognosis

  • The neonatal prognosis depends on the degree of pulmonary hypoplasia and respiratory distress. 20 – 60 % of cases die within the first weeks of life.[neocyst.de]
  • Fetal echocardiography can accurately measure right atrial enlargement, which is associated with a poor prognosis in the fetus with tricuspid valve disease.[karger.com]
  • PROGNOSIS Prune belly syndrome patients require careful, lifelong follow up with regular assessment of kidney and bladder function and palpation of the testes.[urologyweb.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]
  • Prognosis: The condition is lethal due to severe pulmonary hypoplasia. Recurrence: No increased risk.[fetalmedicine.org]

Etiology

  • […] concerning dermatopathology and pathology Atlas of fetal pathology Congenital malformations Physiological developement of an embryo and fetus, growth and changes of the external shape Congenital malformations and congenital anomalies Basic terminology Etiology[atlases.muni.cz]
  • Background Definition Synonyms Differential Diagnosis Signs and Symptoms Etiology/Epidemiology Diagnosis Treatment Late Presenters and Long-Term Survivors References Background When we breathe, air enters our lungs and fills tiny airsacs called alveoli[acdassociation.org]
  • Etiology/Pathophysiology: A congenital skeletal dysplasia can cause thoracic constriction that leads to limitation of lung development and bilateral pulmonary hypoplasia. Pathology: Not applicable Imaging Findings: Small, hypoplastic lungs.[virtualpediatrichospital.org]
  • Two deletions overlapping a distant FOXF1 enhancer unravel the role of IncRNA LINC01081 in etiology of alveolar capillary dysplasia with misalignment of pulmonary veins. Am J Med Genet A. 2014;164:2013-2019. Sen P, Yang Y, Navarro C, et al.[rarediseases.org]
  • An alternative view to the etiology of this condition is that it is secondary to abnormal mesenchymal development. ANATOMIC FEATURES The abdominal wall defect is variable.[urologyweb.com]

Epidemiology

  • Background Definition Synonyms Differential Diagnosis Signs and Symptoms Etiology/Epidemiology Diagnosis Treatment Late Presenters and Long-Term Survivors References Background When we breathe, air enters our lungs and fills tiny airsacs called alveoli[acdassociation.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]
Sex distribution
Age distribution

Pathophysiology

  • […] of Neonatal White Matter Injury; Pathophysiology of Meningitis; Pathophysiology of Preeclampsia; and Pathophysiology of Chorioamnionitis.[books.google.com]
  • PubMed Google Scholar Glick PL, Siebert JR, Benjamin DR: Pathophysiology of congenital diaphragmatic hernia: I.[ojrd.biomedcentral.com]
  • Etiology/Pathophysiology: A congenital skeletal dysplasia can cause thoracic constriction that leads to limitation of lung development and bilateral pulmonary hypoplasia. Pathology: Not applicable Imaging Findings: Small, hypoplastic lungs.[virtualpediatrichospital.org]
  • The World Health Organization (WHO) classification, initially proposed in 1998 and revised in 2003, categorizes different forms of PH based on similarities in pathophysiology, clinical presentation, and treatment (Table 1).[pedsinreview.aappublications.org]
  • 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]

Prevention

  • Prevention of neural tube defects: results of the Medical Research Council Vitamin Study. ‏ الصفحة 332 - Crane JP, LeFevre ML, Winborn RC, et al.[books.google.com]
  • Treatment is focused on preventing progression to stage 5, end-stage renal disease.[clinicaladvisor.com]
  • Early treatment aimed at the underlying disease process may prevent progression. The prognosis is determined by the reversibility of the underlying disease process.[pedsinreview.aappublications.org]
  • 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]
  • Operative management may be necessary to correct urinary stasis in order to prevent urinary tract infections, which significantly contribute to morbidity, loss of renal function and mortality.[urologyweb.com]

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