| Fetal hydrops and familial pulmonary lymphatic hypoplasia. | |
| | |
MedLine Citation:
|
PMID: 12357424 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Two siblings were born with pleural effusions and hydrops. The first infant was a 26-week-old gestation male and died at 8 hours of life with radiographically small lungs and pulmonary insufficiency. No lung tissue was obtainable. This pregnancy was followed by two normal term infants, a male and female. The fourth pregnancy resulted in a female born at 35 weeks' gestation with pleural effusion and hydrops who died at 32 months of age. This infant was discharged from hospital at 32 days of age with small pleural effusions, but needed supplemental oxygen and daily diuretics to control edema. There were 14 additional admissions until death-all for respiratory distress or infections. An open lung biopsy at 2 months, showed dilated pleural lymphatics, with hypoplasia of the acinar and terminal bronchiolar lymphatics. At postmortem examination there was a markedly thickened pleura, and slit-like hypoplastic lymphatics of the acinar and terminal bronchioles and interlobular septal regions. This is the second family reported with these distinctive pulmonary intra-acinar and peri-acinar hypoplastic lymphatics. This disease is compatible with an autosomal recessive mode of inheritance. |
| | |
Authors:
|
Donald W Thibeault; Philip Black; Eugenio Taboada |
Related Documents
:
|
12434894 - Exhaled and nasal nitric oxide in mechanically ventilated preterm and term newborns. 16781204 - Development of the respiratory system in marsupials. 20629494 - Surfactant administration for neonatal respiratory distress does not improve lung inter... 16918664 - Unusual complication during pediatric thoracoscopy. 19837104 - Processing faces in dyadic and triadic contexts. 7396404 - Assessing birth weight-for-dates on a continuous scale. |
Publication Detail:
|
Type: Case Reports; Journal Article |
Journal Detail:
|
Title: American journal of perinatology Volume: 19 ISSN: 0735-1631 ISO Abbreviation: Am J Perinatol Publication Date: 2002 Aug |
Date Detail:
|
Created Date: 2002-10-01 Completed Date: 2002-12-13 Revised Date: 2004-11-17 |
Medline Journal Info:
|
Nlm Unique ID: 8405212 Medline TA: Am J Perinatol Country: United States |
Other Details:
|
Languages: eng Pagination: 323-31 Citation Subset: IM |
Affiliation:
|
Department of Pediatrics, Children's Mercy Hospital, Kansas City, Missouri 64108, USA. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adult Autopsy Bronchopulmonary Dysplasia / complications*, genetics, pathology Fatal Outcome Female Humans Hydrops Fetalis / complications*, genetics, pathology Infant, Newborn Lymphatic Diseases / complications*, genetics Lymphatic System / abnormalities* Male Pleural Effusion / etiology Pregnancy Pulmonary Edema / etiology Respiratory Distress Syndrome, Newborn / etiology |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Serial protein S-100 serum levels in preterm babies with perinatal asphyxia and periventricular whit...
Next Document: Neonatal and maternal serum levels of soluble ICAM-1 in preeclamptic and normal pregnancies.