Respiratory System - Abnormalities - Revision history

Z8600021 at 04:58, 21 November 2019

2019-11-21T04:58:39Z

← Older revision		Revision as of 14:58, 21 November 2019
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	{{Abnormality Links}}		{{Abnormality Links}}

			{\| class="wikitable mw-collapsible mw-collapsed"
			! System Abnormalities
			\|-
			\| {{Abnormality Links}}
			\|}
	==Some Recent Findings==		==Some Recent Findings==
	{\|		{\|

Z8600021 at 22:53, 16 April 2019

2019-04-16T22:53:29Z

← Older revision		Revision as of 08:53, 17 April 2019
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	==Lobar Emphysema (Overinflated Lung)==		==Lobar Emphysema (Overinflated Lung)==
	[[File:Congenital lobar emphysema.jpg\|~~thumb\|~~Congenital lobar emphysema]]		{\|
			\| [[File:Congenital lobar emphysema.jpg\|200px]]
			Congenital lobar emphysema
			\|
	# There is an overinflated left upper lobe		# There is an overinflated left upper lobe
	# There is a collapsed lower lobe		# There is a collapsed lower lobe
	# The left lung is herniating across the mediastinum		# The left lung is herniating across the mediastinum
			\|}
	==LB00.0 Congenital Diaphragmatic Hernia==		==LB00.0 Congenital Diaphragmatic Hernia==
	[[File:Human_congenital_diaphragmatic_hernia.jpg\|thumb\|Human congenital diaphragmatic hernia{{#pmid:16483386\|PMID16483386}}]]		[[File:Human_congenital_diaphragmatic_hernia.jpg\|thumb\|Human congenital diaphragmatic hernia{{#pmid:16483386\|PMID16483386}}]]
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	\|		\|
	{{ICD11weblink}}1139900219 LB00 Structural developmental anomalies of diaphragm]		{{ICD11weblink}}1139900219 LB00 Structural developmental anomalies of diaphragm]
	* {{ICD11weblink}}1414428936 LB00.0 Congenital diaphragmatic hernia] - ''Congenital diaphragmatic hernia is a posterolateral defect of the diaphragm that allows passage of abdominal viscera into the thorax, leading to respiratory insufficiency and persistent pulmonary hypertension with high mortality.''		* {{ICD11weblink}}1414428936 LB00.0 Congenital diaphragmatic hernia] - ''{{Congenital diaphragmatic hernia}} is a posterolateral defect of the diaphragm that allows passage of abdominal viscera into the thorax, leading to respiratory insufficiency and persistent pulmonary hypertension with high mortality.''
	* {{ICD11weblink}}1952603717 LB00.1 Absence of diaphragm]		* {{ICD11weblink}}1952603717 LB00.1 Absence of diaphragm]
	\|}		\|}

	Really a musculoskeletal abnormality, but included here due to the associated respiratory effects. Failure of the pleuroperitoneal foramen (foramen of Bochdalek) to close allows viscera into thorax, most common (80-85%) on the left side of diaphragm. Intestine, stomach or spleen can enter the pleural cavity, compressing the lung.		Really a musculoskeletal abnormality, but included here due to the associated respiratory effects. Failure of the pleuroperitoneal foramen (foramen of Bochdalek) to close allows viscera into thorax, most common (80-85%) on the left side of diaphragm. Intestine, stomach or spleen can enter the pleural cavity, compressing the lung.
	{\|
	~~\| [[File:Gray0391.jpg\|400px]]~~
	~~\| [[File:Human_congenital_diaphragmatic_hernia.jpg\|400px]]~~
	\|-
	~~\| Normal Adult Diaphragm~~
	~~\| Human congenital diaphragmatic hernia{{#pmid:16483386\|PMID16483386}}~~
	\|}

	{\|		{\|

Z8600021 at 22:49, 16 April 2019

2019-04-16T22:49:48Z

@@ Line 76: / Line 76: @@
 # The left lung is herniating across the mediastinum
-==Congenital Diaphragmatic Hernia==
+==LB00.0 Congenital Diaphragmatic Hernia==
 Really a musculoskeletal abnormality, but included here due to the associated respiratory effects. Failure of the pleuroperitoneal foramen (foramen of Bochdalek) to close allows viscera into thorax, most common (80-85%) on the left side of diaphragm. Intestine, stomach or spleen can enter the pleural cavity, compressing the lung.
 {|

Z8600021 at 02:19, 23 February 2019

2019-02-23T02:19:23Z

← Older revision		Revision as of 12:19, 23 February 2019
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	{{#pmid:28818610}}		{{#pmid:28818610}}

			{{#pmid:25439356}}

	{{#pmid:20727133}}		{{#pmid:20727133}}
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	{{#pmid:15819986}}		{{#pmid:15819986}}


	===Articles===		===Articles===

Z8600021 at 05:16, 22 Ocak 2019

2019-01-22T05:16:04Z

← Older revision		Revision as of 15:16, 22 Ocak 2019
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	\|-bgcolor="F5FAFF"		\|-bgcolor="F5FAFF"
	\|		\|
	* '''Complex Compound Inheritance of Lethal Lung Developmental Disorders due to Disruption of the TBX-FGF Pathway'''{{#pmid:30639323\|PMID30639323}} "Primary defects in lung branching morphogenesis, resulting in neonatal lethal pulmonary hypoplasias, are incompletely understood. To elucidate the pathogenetics of human lung development, we studied a unique collection of samples obtained from deceased individuals with clinically and histopathologically diagnosed interstitial neonatal lung disorders: acinar dysplasia (n = 14), congenital alveolar dysplasia (n = 2), and other lethal lung hypoplasias (n = 10). We identified rare heterozygous copy-number variant deletions or single-nucleotide variants (SNVs) involving ~~TBX4~~ (n = 8 and n = 2, respectively) or FGF10 (n = 2 and n = 2, respectively) in 16/26 (61%) individuals. In addition to ~~TBX4~~, the overlapping ∼2 Mb recurrent and nonrecurrent deletions at 17q23.1q23.2 identified in seven individuals with lung hypoplasia also remove a lung-specific enhancer region. Individuals with coding variants involving either TBX4 or FGF10 also harbored at least one non-coding SNV in the predicted lung-specific enhancer region, which was absent in 13 control individuals with the overlapping deletions but without any structural lung anomalies. The occurrence of rare coding variants involving ~~TBX4~~ or ~~FGF10~~ with the putative hypomorphic non-coding SNVs implies a complex compound inheritance of these pulmonary hypoplasias. Moreover, they support the importance of TBX4-FGF10-FGFR2 epithelial-mesenchymal signaling in human lung organogenesis and help to explain the histopathological continuum observed in these rare lethal developmental disorders of the lung."		* '''Complex Compound Inheritance of Lethal Lung Developmental Disorders due to Disruption of the {{TBX}}-{{FGF}} Pathway'''{{#pmid:30639323\|PMID30639323}} "Primary defects in lung branching morphogenesis, resulting in neonatal lethal pulmonary hypoplasias, are incompletely understood. To elucidate the pathogenetics of human lung development, we studied a unique collection of samples obtained from deceased individuals with clinically and histopathologically diagnosed interstitial neonatal lung disorders: acinar dysplasia (n = 14), congenital alveolar dysplasia (n = 2), and other lethal lung hypoplasias (n = 10). We identified rare heterozygous copy-number variant deletions or single-nucleotide variants (SNVs) involving {{TBX}}4 (n = 8 and n = 2, respectively) or FGF10 (n = 2 and n = 2, respectively) in 16/26 (61%) individuals. In addition to {{TBX}}4, the overlapping ∼2 Mb recurrent and nonrecurrent deletions at 17q23.1q23.2 identified in seven individuals with lung hypoplasia also remove a lung-specific enhancer region. Individuals with coding variants involving either TBX4 or FGF10 also harbored at least one non-coding SNV in the predicted lung-specific enhancer region, which was absent in 13 control individuals with the overlapping deletions but without any structural lung anomalies. The occurrence of rare coding variants involving {{TBX}}4 or {{FGF}}10 with the putative hypomorphic non-coding SNVs implies a complex compound inheritance of these pulmonary hypoplasias. Moreover, they support the importance of TBX4-FGF10-FGFR2 epithelial-mesenchymal signaling in human lung organogenesis and help to explain the histopathological continuum observed in these rare lethal developmental disorders of the lung."

	* '''Predictors of Long-Term Pulmonary Morbidity in Children with Congenital Diaphragmatic Hernia'''{{#pmid:30583297\|PMID30583297}} "The aim is to identify prognostic markers of long-term pulmonary morbidity among congenital diaphragmatic hernia (CDH) survivors. METHODS: A single-institution, retrospective review was performed on all CDH patients from 2000 and 2012 (REB#1000053383). Liver position, patch use, and pulmonary function tests (PFTs) (forced expiratory volume at 1 second [FEV1] and forced vital capacity [FVC] expressed as mean % predicted + SD) were recorded. Data were analyzed using analysis of variance. RESULTS: Patients with acceptable and reproducible PFT (n = 72 for 202 total PFT) with patch repair and liver up (n = 28) had significantly lower FEV1 (72.4 + 17.6) than those with no patch and liver down (n = 98, FEV1= 86.3 + 15.9, p = 0.002). Patients with patch repair and liver down (n = 40) also had significantly lower FEV1 (76.6 + 14.4) than those with liver down and no patch (p = 0.0463). Patients with liver up and patch repair had PFT results consistent with moderate reduction of lung function, while the remainder had mild to no decrease in lung function. All CDH patients older than 14 years had a reduction in FEV1/FVC consistent with obstructive phenotype, with a mean FEV1/FVC = 62.3 for patch repair group and FEV1/FVC = 76.1 in the no patch group. CONCLUSION: Decreased pulmonary function of CDH survivors correlated with the use of patch repair and liver position. CDH lung disease should be monitored in adulthood."		* '''Predictors of Long-Term Pulmonary Morbidity in Children with Congenital Diaphragmatic Hernia'''{{#pmid:30583297\|PMID30583297}} "The aim is to identify prognostic markers of long-term pulmonary morbidity among congenital diaphragmatic hernia (CDH) survivors. METHODS: A single-institution, retrospective review was performed on all CDH patients from 2000 and 2012 (REB#1000053383). Liver position, patch use, and pulmonary function tests (PFTs) (forced expiratory volume at 1 second [FEV1] and forced vital capacity [FVC] expressed as mean % predicted + SD) were recorded. Data were analyzed using analysis of variance. RESULTS: Patients with acceptable and reproducible PFT (n = 72 for 202 total PFT) with patch repair and liver up (n = 28) had significantly lower FEV1 (72.4 + 17.6) than those with no patch and liver down (n = 98, FEV1= 86.3 + 15.9, p = 0.002). Patients with patch repair and liver down (n = 40) also had significantly lower FEV1 (76.6 + 14.4) than those with liver down and no patch (p = 0.0463). Patients with liver up and patch repair had PFT results consistent with moderate reduction of lung function, while the remainder had mild to no decrease in lung function. All CDH patients older than 14 years had a reduction in FEV1/FVC consistent with obstructive phenotype, with a mean FEV1/FVC = 62.3 for patch repair group and FEV1/FVC = 76.1 in the no patch group. CONCLUSION: Decreased pulmonary function of CDH survivors correlated with the use of patch repair and liver position. CDH lung disease should be monitored in adulthood."

Z8600021 at 04:56, 22 Ocak 2019

2019-01-22T04:56:09Z

← Older revision		Revision as of 14:56, 22 Ocak 2019
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	\|		\|
	* '''Complex Compound Inheritance of Lethal Lung Developmental Disorders due to Disruption of the TBX-FGF Pathway'''{{#pmid:30639323\|PMID30639323}} "Primary defects in lung branching morphogenesis, resulting in neonatal lethal pulmonary hypoplasias, are incompletely understood. To elucidate the pathogenetics of human lung development, we studied a unique collection of samples obtained from deceased individuals with clinically and histopathologically diagnosed interstitial neonatal lung disorders: acinar dysplasia (n = 14), congenital alveolar dysplasia (n = 2), and other lethal lung hypoplasias (n = 10). We identified rare heterozygous copy-number variant deletions or single-nucleotide variants (SNVs) involving TBX4 (n = 8 and n = 2, respectively) or FGF10 (n = 2 and n = 2, respectively) in 16/26 (61%) individuals. In addition to TBX4, the overlapping ∼2 Mb recurrent and nonrecurrent deletions at 17q23.1q23.2 identified in seven individuals with lung hypoplasia also remove a lung-specific enhancer region. Individuals with coding variants involving either TBX4 or FGF10 also harbored at least one non-coding SNV in the predicted lung-specific enhancer region, which was absent in 13 control individuals with the overlapping deletions but without any structural lung anomalies. The occurrence of rare coding variants involving TBX4 or FGF10 with the putative hypomorphic non-coding SNVs implies a complex compound inheritance of these pulmonary hypoplasias. Moreover, they support the importance of TBX4-FGF10-FGFR2 epithelial-mesenchymal signaling in human lung organogenesis and help to explain the histopathological continuum observed in these rare lethal developmental disorders of the lung."		* '''Complex Compound Inheritance of Lethal Lung Developmental Disorders due to Disruption of the TBX-FGF Pathway'''{{#pmid:30639323\|PMID30639323}} "Primary defects in lung branching morphogenesis, resulting in neonatal lethal pulmonary hypoplasias, are incompletely understood. To elucidate the pathogenetics of human lung development, we studied a unique collection of samples obtained from deceased individuals with clinically and histopathologically diagnosed interstitial neonatal lung disorders: acinar dysplasia (n = 14), congenital alveolar dysplasia (n = 2), and other lethal lung hypoplasias (n = 10). We identified rare heterozygous copy-number variant deletions or single-nucleotide variants (SNVs) involving TBX4 (n = 8 and n = 2, respectively) or FGF10 (n = 2 and n = 2, respectively) in 16/26 (61%) individuals. In addition to TBX4, the overlapping ∼2 Mb recurrent and nonrecurrent deletions at 17q23.1q23.2 identified in seven individuals with lung hypoplasia also remove a lung-specific enhancer region. Individuals with coding variants involving either TBX4 or FGF10 also harbored at least one non-coding SNV in the predicted lung-specific enhancer region, which was absent in 13 control individuals with the overlapping deletions but without any structural lung anomalies. The occurrence of rare coding variants involving TBX4 or FGF10 with the putative hypomorphic non-coding SNVs implies a complex compound inheritance of these pulmonary hypoplasias. Moreover, they support the importance of TBX4-FGF10-FGFR2 epithelial-mesenchymal signaling in human lung organogenesis and help to explain the histopathological continuum observed in these rare lethal developmental disorders of the lung."

			* '''Predictors of Long-Term Pulmonary Morbidity in Children with Congenital Diaphragmatic Hernia'''{{#pmid:30583297\|PMID30583297}} "The aim is to identify prognostic markers of long-term pulmonary morbidity among congenital diaphragmatic hernia (CDH) survivors. METHODS: A single-institution, retrospective review was performed on all CDH patients from 2000 and 2012 (REB#1000053383). Liver position, patch use, and pulmonary function tests (PFTs) (forced expiratory volume at 1 second [FEV1] and forced vital capacity [FVC] expressed as mean % predicted + SD) were recorded. Data were analyzed using analysis of variance. RESULTS: Patients with acceptable and reproducible PFT (n = 72 for 202 total PFT) with patch repair and liver up (n = 28) had significantly lower FEV1 (72.4 + 17.6) than those with no patch and liver down (n = 98, FEV1= 86.3 + 15.9, p = 0.002). Patients with patch repair and liver down (n = 40) also had significantly lower FEV1 (76.6 + 14.4) than those with liver down and no patch (p = 0.0463). Patients with liver up and patch repair had PFT results consistent with moderate reduction of lung function, while the remainder had mild to no decrease in lung function. All CDH patients older than 14 years had a reduction in FEV1/FVC consistent with obstructive phenotype, with a mean FEV1/FVC = 62.3 for patch repair group and FEV1/FVC = 76.1 in the no patch group. CONCLUSION: Decreased pulmonary function of CDH survivors correlated with the use of patch repair and liver position. CDH lung disease should be monitored in adulthood."

	* '''Maternal asthma is associated with increased risk of perinatal mortality'''{{#pmid:29775476\|PMID29775476}} "Asthma is the most common chronic disease during pregnancy and it may have influence on pregnancy outcome. OBJECTIVES: Our goal was to assess the association between maternal asthma and the perinatal risks as well as possible effects of asthma medication. METHODS: The study was based on a nationwide Finnish register-based cohort between the years 1996 and 2012 in the Drug and Pregnancy Database. CONCLUSION: Asthma is associated with increased risks of perinatal mortality, preterm birth, low birth weight, fetal growth restriction (SGA), and asphyxia. Asthma treatment reduces the risk of preterm delivery, but it does not seem to reduce other complications such as perinatal mortality."		* '''Maternal asthma is associated with increased risk of perinatal mortality'''{{#pmid:29775476\|PMID29775476}} "Asthma is the most common chronic disease during pregnancy and it may have influence on pregnancy outcome. OBJECTIVES: Our goal was to assess the association between maternal asthma and the perinatal risks as well as possible effects of asthma medication. METHODS: The study was based on a nationwide Finnish register-based cohort between the years 1996 and 2012 in the Drug and Pregnancy Database. CONCLUSION: Asthma is associated with increased risks of perinatal mortality, preterm birth, low birth weight, fetal growth restriction (SGA), and asphyxia. Asthma treatment reduces the risk of preterm delivery, but it does not seem to reduce other complications such as perinatal mortality."

Z8600021 at 04:53, 22 Ocak 2019

2019-01-22T04:53:40Z

← Older revision		Revision as of 14:53, 22 Ocak 2019
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	(LC, laryngeal cleft) A rare foregut abnormality allowing digestive tract and the airway to communicate causing chronic cough, aspiration and respiratory distress.		(LC, laryngeal cleft) A rare foregut abnormality allowing digestive tract and the airway to communicate causing chronic cough, aspiration and respiratory distress.

	The downward extension of the cleft determines the classification of the abnormality,{{#pmid:~~2729823PMID2729823~~}}{{#pmid:22151899\|PMID22151899}}		The downward extension of the cleft determines the classification of the abnormality,{{#pmid:2729823\|PMID2729823}}{{#pmid:22151899\|PMID22151899}}

	* '''Type 0''' - submucosal cleft		* '''Type 0''' - submucosal cleft

Z8600021 at 04:52, 22 Ocak 2019

2019-01-22T04:52:40Z

← Older revision		Revision as of 14:52, 22 Ocak 2019
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	\| [[File:Mark_Hill.jpg\|90px\|left]] {{Most_Recent_Refs}}		\| [[File:Mark_Hill.jpg\|90px\|left]] {{Most_Recent_Refs}}

	Search term: [http://www.ncbi.nlm.nih.gov/pubmed/?term=Abnormal+Respiratory+Development ''Abnormal Respiratory Development'] \| [http://www.ncbi.nlm.nih.gov/pubmed/?term=Newborn+Respiratory+Distress+Syndrome ''Newborn Respiratory Distress Syndrome'']		Search term: [http://www.ncbi.nlm.nih.gov/pubmed/?term=Abnormal+Respiratory+Development ''Abnormal Respiratory Development''] \| [http://www.ncbi.nlm.nih.gov/pubmed/?term=Newborn+Respiratory+Distress+Syndrome ''Newborn Respiratory Distress Syndrome''] \| [http://www.ncbi.nlm.nih.gov/pubmed/?term=congenital+diaphragmatic+hernia ''congenital diaphragmatic hernia''] \| [http://www.ncbi.nlm.nih.gov/pubmed/?term=Tracheoesophageal+Fistula ''Tracheoesophageal Fistula'']

	\|}		\|}
	{\| class="wikitable mw-collapsible mw-collapsed"		{\| class="wikitable mw-collapsible mw-collapsed"
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	\| [[File:Congenital_diaphragmatic_hernia_01.jpg\|600px]]		\| [[File:Congenital_diaphragmatic_hernia_01.jpg\|600px]]
	\|-		\|-
	\| Left posterolateral diaphragmatic hernia~~<ref name=PMID22214468><pubmed>~~22214468~~</pubmed>~~\| ~~[http://www.ojrd.com/content/7/1/1 Orphanet J Rare Dis.]</ref>~~		\| Left posterolateral diaphragmatic hernia{{#pmid:22214468\|PMID22214468}}

	* '''A''' - Plain X-ray of the thorax of a newborn with CDH. There are bowel loops into the left hemi-thorax, the mediastinum is displaced to the contralateral side and the space occupied by the lung is reduced.		* '''A''' - Plain X-ray of the thorax of a newborn with CDH. There are bowel loops into the left hemi-thorax, the mediastinum is displaced to the contralateral side and the space occupied by the lung is reduced.
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	===Australian Statistics===		===Australian Statistics===

	A recent Western Australian study~~<ref><pubmed>~~16140678~~</pubmed></ref>~~ of congenital diaphragmatic hernia (CDH) outcomes showed:		A recent Western Australian study{{#pmid:16140678\|PMID16140678}} of congenital diaphragmatic hernia (CDH) outcomes showed:

	* 35% of live-born infants died before referral or transport.		* 35% of live-born infants died before referral or transport.
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	==Surfactant Metabolism==		==Surfactant Metabolism==
	(pulmonary surfactant metabolism dysfunctions, surfactant dysfunction disorders) For review of genetic disorders of surfactant dysfunction~~<ref><pubmed>~~19220077~~</pubmed></ref>~~		(pulmonary surfactant metabolism dysfunctions, surfactant dysfunction disorders) For review of genetic disorders of surfactant dysfunction.{{#pmid:19220077\|PMID19220077}}

	Mutations in the genes encoding:		Mutations in the genes encoding:

Z8600021 at 04:48, 22 Ocak 2019

2019-01-22T04:48:51Z

← Older revision		Revision as of 14:48, 22 Ocak 2019
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	\|-bgcolor="F5FAFF"		\|-bgcolor="F5FAFF"
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			* '''Complex Compound Inheritance of Lethal Lung Developmental Disorders due to Disruption of the TBX-FGF Pathway'''{{#pmid:30639323\|PMID30639323}} "Primary defects in lung branching morphogenesis, resulting in neonatal lethal pulmonary hypoplasias, are incompletely understood. To elucidate the pathogenetics of human lung development, we studied a unique collection of samples obtained from deceased individuals with clinically and histopathologically diagnosed interstitial neonatal lung disorders: acinar dysplasia (n = 14), congenital alveolar dysplasia (n = 2), and other lethal lung hypoplasias (n = 10). We identified rare heterozygous copy-number variant deletions or single-nucleotide variants (SNVs) involving TBX4 (n = 8 and n = 2, respectively) or FGF10 (n = 2 and n = 2, respectively) in 16/26 (61%) individuals. In addition to TBX4, the overlapping ∼2 Mb recurrent and nonrecurrent deletions at 17q23.1q23.2 identified in seven individuals with lung hypoplasia also remove a lung-specific enhancer region. Individuals with coding variants involving either TBX4 or FGF10 also harbored at least one non-coding SNV in the predicted lung-specific enhancer region, which was absent in 13 control individuals with the overlapping deletions but without any structural lung anomalies. The occurrence of rare coding variants involving TBX4 or FGF10 with the putative hypomorphic non-coding SNVs implies a complex compound inheritance of these pulmonary hypoplasias. Moreover, they support the importance of TBX4-FGF10-FGFR2 epithelial-mesenchymal signaling in human lung organogenesis and help to explain the histopathological continuum observed in these rare lethal developmental disorders of the lung."

	* '''Maternal asthma is associated with increased risk of perinatal mortality'''{{#pmid:29775476\|PMID29775476}} "Asthma is the most common chronic disease during pregnancy and it may have influence on pregnancy outcome. OBJECTIVES: Our goal was to assess the association between maternal asthma and the perinatal risks as well as possible effects of asthma medication. METHODS: The study was based on a nationwide Finnish register-based cohort between the years 1996 and 2012 in the Drug and Pregnancy Database. CONCLUSION: Asthma is associated with increased risks of perinatal mortality, preterm birth, low birth weight, fetal growth restriction (SGA), and asphyxia. Asthma treatment reduces the risk of preterm delivery, but it does not seem to reduce other complications such as perinatal mortality."		* '''Maternal asthma is associated with increased risk of perinatal mortality'''{{#pmid:29775476\|PMID29775476}} "Asthma is the most common chronic disease during pregnancy and it may have influence on pregnancy outcome. OBJECTIVES: Our goal was to assess the association between maternal asthma and the perinatal risks as well as possible effects of asthma medication. METHODS: The study was based on a nationwide Finnish register-based cohort between the years 1996 and 2012 in the Drug and Pregnancy Database. CONCLUSION: Asthma is associated with increased risks of perinatal mortality, preterm birth, low birth weight, fetal growth restriction (SGA), and asphyxia. Asthma treatment reduces the risk of preterm delivery, but it does not seem to reduce other complications such as perinatal mortality."

	* '''Longitudinal assessment of lung function in extremely prematurely born children'''{{#pmid:29316378\|PMID29316378}} "To assess longitudinally small airway function in children born extremely prematurely and whether there was a correlation between airway function in infancy and at 11-14 years. Thirty-five children with a mean gestational age of 26 weeks had lung function assessed at 1 year corrected and 11-14 years of age. These results demonstrate in those born extremely prematurely there is tracking of airway function during childhood." [[Birth - Preterm]]		* '''Longitudinal assessment of lung function in extremely prematurely born children'''{{#pmid:29316378\|PMID29316378}} "To assess longitudinally small airway function in children born extremely prematurely and whether there was a correlation between airway function in infancy and at 11-14 years. Thirty-five children with a mean gestational age of 26 weeks had lung function assessed at 1 year corrected and 11-14 years of age. These results demonstrate in those born extremely prematurely there is tracking of airway function during childhood." [[Birth - Preterm]]

	* '''Trends in treatment and in-hospital mortality for neonates with congenital diaphragmatic hernia'''{{#pmid:25950919\|PMID25950919}} "We performed a retrospective cohort study in order to examine recent trends in use of post-partum treatments and in-hospital mortality for congenital diaphragmatic hernia (CDH). Survival improved in large subgroups of term or near-term infants with CDH in this 10-year multicenter cohort, temporally associated with increasing use of multiple vasodilators. Use of vasodilators for infants with CDH is increasing despite a lack of evidence supporting efficacy or safety. Prospective research is needed to clarify specific causal effects contributing to improving survival in these infants."

	* '''Surfactant Metabolism Dysfunction and Childhood Interstitial Lung Disease (chILD).'''{{#pmid:19252722\|PMID19252722}} "Surfactant deficiency and the resultant respiratory distress syndrome (RDS) seen in preterm infants is a major cause of respiratory morbidity in this population. Until recently, the contribution of surfactant to respiratory morbidity in infancy was limited to the neonatal period. It is now recognised that inborn errors of surfactant metabolism leading to surfactant dysfunction account for around 10% of childhood interstitial lung disease (chILD)."
	\|}		\|}
	{\| class="wikitable mw-collapsible mw-collapsed"		{\| class="wikitable mw-collapsible mw-collapsed"
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	\| [[File:Mark_Hill.jpg\|90px\|left]] {{Most_Recent_Refs}}		\| [[File:Mark_Hill.jpg\|90px\|left]] {{Most_Recent_Refs}}

	Search term: [http://www.ncbi.nlm.nih.gov/pubmed/?term=Abnormal+Respiratory+Development ''Abnormal Respiratory Development']		Search term: [http://www.ncbi.nlm.nih.gov/pubmed/?term=Abnormal+Respiratory+Development ''Abnormal Respiratory Development'] \| [http://www.ncbi.nlm.nih.gov/pubmed/?term=Newborn+Respiratory+Distress+Syndrome ''Newborn Respiratory Distress Syndrome'']

	~~<pubmed limit~~=~~5>Abnormal Respiratory Development</pubmed>~~		\|}
			{\| class="wikitable mw-collapsible mw-collapsed"
			! Older papers
			\|-
			\| {{Older papers}}
			* '''Trends in treatment and in-hospital mortality for neonates with congenital diaphragmatic hernia'''{{#pmid:25950919\|PMID25950919}} "We performed a retrospective cohort study in order to examine recent trends in use of post-partum treatments and in-hospital mortality for congenital diaphragmatic hernia (CDH). Survival improved in large subgroups of term or near-term infants with CDH in this 10-year multicenter cohort, temporally associated with increasing use of multiple vasodilators. Use of vasodilators for infants with CDH is increasing despite a lack of evidence supporting efficacy or safety. Prospective research is needed to clarify specific causal effects contributing to improving survival in these infants."

	~~Newborn Respiratory Distress Syndrome~~		* '''Surfactant Metabolism Dysfunction and Childhood Interstitial Lung Disease (chILD).'''{{#pmid:19252722\|PMID19252722}} "Surfactant deficiency and the resultant respiratory distress syndrome (RDS) seen in preterm infants is a major cause of respiratory morbidity in this population. Until recently, the contribution of surfactant to respiratory morbidity in infancy was limited to the neonatal period. It is now recognised that inborn errors of surfactant metabolism leading to surfactant dysfunction account for around 10% of childhood interstitial lung disease (chILD)."

	~~<pubmed limit=5>Newborn Respiratory Distress Syndrome</pubmed>~~
	\|}		\|}
	==Premature Birth==		==Premature Birth==

Z8600021 at 11:02, 27 Mayıs 2018

2018-05-27T11:02:22Z

← Older revision		Revision as of 21:02, 27 Mayıs 2018
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	\|-bgcolor="F5FAFF"		\|-bgcolor="F5FAFF"
	\|		\|
			* '''Maternal asthma is associated with increased risk of perinatal mortality'''{{#pmid:29775476\|PMID29775476}} "Asthma is the most common chronic disease during pregnancy and it may have influence on pregnancy outcome. OBJECTIVES: Our goal was to assess the association between maternal asthma and the perinatal risks as well as possible effects of asthma medication. METHODS: The study was based on a nationwide Finnish register-based cohort between the years 1996 and 2012 in the Drug and Pregnancy Database. CONCLUSION: Asthma is associated with increased risks of perinatal mortality, preterm birth, low birth weight, fetal growth restriction (SGA), and asphyxia. Asthma treatment reduces the risk of preterm delivery, but it does not seem to reduce other complications such as perinatal mortality."

	* '''Longitudinal assessment of lung function in extremely prematurely born children'''{{#pmid:29316378\|PMID29316378}} "To assess longitudinally small airway function in children born extremely prematurely and whether there was a correlation between airway function in infancy and at 11-14 years. Thirty-five children with a mean gestational age of 26 weeks had lung function assessed at 1 year corrected and 11-14 years of age. These results demonstrate in those born extremely prematurely there is tracking of airway function during childhood." [[Birth - Preterm]]		* '''Longitudinal assessment of lung function in extremely prematurely born children'''{{#pmid:29316378\|PMID29316378}} "To assess longitudinally small airway function in children born extremely prematurely and whether there was a correlation between airway function in infancy and at 11-14 years. Thirty-five children with a mean gestational age of 26 weeks had lung function assessed at 1 year corrected and 11-14 years of age. These results demonstrate in those born extremely prematurely there is tracking of airway function during childhood." [[Birth - Preterm]]

← Older revision		Revision as of 14:56, 22 Ocak 2019
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	* '''Complex Compound Inheritance of Lethal Lung Developmental Disorders due to Disruption of the TBX-FGF Pathway'''{{#pmid:30639323\|PMID30639323}} "Primary defects in lung branching morphogenesis, resulting in neonatal lethal pulmonary hypoplasias, are incompletely understood. To elucidate the pathogenetics of human lung development, we studied a unique collection of samples obtained from deceased individuals with clinically and histopathologically diagnosed interstitial neonatal lung disorders: acinar dysplasia (n = 14), congenital alveolar dysplasia (n = 2), and other lethal lung hypoplasias (n = 10). We identified rare heterozygous copy-number variant deletions or single-nucleotide variants (SNVs) involving TBX4 (n = 8 and n = 2, respectively) or FGF10 (n = 2 and n = 2, respectively) in 16/26 (61%) individuals. In addition to TBX4, the overlapping ∼2 Mb recurrent and nonrecurrent deletions at 17q23.1q23.2 identified in seven individuals with lung hypoplasia also remove a lung-specific enhancer region. Individuals with coding variants involving either TBX4 or FGF10 also harbored at least one non-coding SNV in the predicted lung-specific enhancer region, which was absent in 13 control individuals with the overlapping deletions but without any structural lung anomalies. The occurrence of rare coding variants involving TBX4 or FGF10 with the putative hypomorphic non-coding SNVs implies a complex compound inheritance of these pulmonary hypoplasias. Moreover, they support the importance of TBX4-FGF10-FGFR2 epithelial-mesenchymal signaling in human lung organogenesis and help to explain the histopathological continuum observed in these rare lethal developmental disorders of the lung."		* '''Complex Compound Inheritance of Lethal Lung Developmental Disorders due to Disruption of the TBX-FGF Pathway'''{{#pmid:30639323\|PMID30639323}} "Primary defects in lung branching morphogenesis, resulting in neonatal lethal pulmonary hypoplasias, are incompletely understood. To elucidate the pathogenetics of human lung development, we studied a unique collection of samples obtained from deceased individuals with clinically and histopathologically diagnosed interstitial neonatal lung disorders: acinar dysplasia (n = 14), congenital alveolar dysplasia (n = 2), and other lethal lung hypoplasias (n = 10). We identified rare heterozygous copy-number variant deletions or single-nucleotide variants (SNVs) involving TBX4 (n = 8 and n = 2, respectively) or FGF10 (n = 2 and n = 2, respectively) in 16/26 (61%) individuals. In addition to TBX4, the overlapping ∼2 Mb recurrent and nonrecurrent deletions at 17q23.1q23.2 identified in seven individuals with lung hypoplasia also remove a lung-specific enhancer region. Individuals with coding variants involving either TBX4 or FGF10 also harbored at least one non-coding SNV in the predicted lung-specific enhancer region, which was absent in 13 control individuals with the overlapping deletions but without any structural lung anomalies. The occurrence of rare coding variants involving TBX4 or FGF10 with the putative hypomorphic non-coding SNVs implies a complex compound inheritance of these pulmonary hypoplasias. Moreover, they support the importance of TBX4-FGF10-FGFR2 epithelial-mesenchymal signaling in human lung organogenesis and help to explain the histopathological continuum observed in these rare lethal developmental disorders of the lung."

			* '''Predictors of Long-Term Pulmonary Morbidity in Children with Congenital Diaphragmatic Hernia'''{{#pmid:30583297\|PMID30583297}} "The aim is to identify prognostic markers of long-term pulmonary morbidity among congenital diaphragmatic hernia (CDH) survivors. METHODS: A single-institution, retrospective review was performed on all CDH patients from 2000 and 2012 (REB#1000053383). Liver position, patch use, and pulmonary function tests (PFTs) (forced expiratory volume at 1 second [FEV1] and forced vital capacity [FVC] expressed as mean % predicted + SD) were recorded. Data were analyzed using analysis of variance. RESULTS: Patients with acceptable and reproducible PFT (n = 72 for 202 total PFT) with patch repair and liver up (n = 28) had significantly lower FEV1 (72.4 + 17.6) than those with no patch and liver down (n = 98, FEV1= 86.3 + 15.9, p = 0.002). Patients with patch repair and liver down (n = 40) also had significantly lower FEV1 (76.6 + 14.4) than those with liver down and no patch (p = 0.0463). Patients with liver up and patch repair had PFT results consistent with moderate reduction of lung function, while the remainder had mild to no decrease in lung function. All CDH patients older than 14 years had a reduction in FEV1/FVC consistent with obstructive phenotype, with a mean FEV1/FVC = 62.3 for patch repair group and FEV1/FVC = 76.1 in the no patch group. CONCLUSION: Decreased pulmonary function of CDH survivors correlated with the use of patch repair and liver position. CDH lung disease should be monitored in adulthood."

	* '''Maternal asthma is associated with increased risk of perinatal mortality'''{{#pmid:29775476\|PMID29775476}} "Asthma is the most common chronic disease during pregnancy and it may have influence on pregnancy outcome. OBJECTIVES: Our goal was to assess the association between maternal asthma and the perinatal risks as well as possible effects of asthma medication. METHODS: The study was based on a nationwide Finnish register-based cohort between the years 1996 and 2012 in the Drug and Pregnancy Database. CONCLUSION: Asthma is associated with increased risks of perinatal mortality, preterm birth, low birth weight, fetal growth restriction (SGA), and asphyxia. Asthma treatment reduces the risk of preterm delivery, but it does not seem to reduce other complications such as perinatal mortality."		* '''Maternal asthma is associated with increased risk of perinatal mortality'''{{#pmid:29775476\|PMID29775476}} "Asthma is the most common chronic disease during pregnancy and it may have influence on pregnancy outcome. OBJECTIVES: Our goal was to assess the association between maternal asthma and the perinatal risks as well as possible effects of asthma medication. METHODS: The study was based on a nationwide Finnish register-based cohort between the years 1996 and 2012 in the Drug and Pregnancy Database. CONCLUSION: Asthma is associated with increased risks of perinatal mortality, preterm birth, low birth weight, fetal growth restriction (SGA), and asphyxia. Asthma treatment reduces the risk of preterm delivery, but it does not seem to reduce other complications such as perinatal mortality."