scholarly journals Dextrocardia with pulmonary hypoplasia: an unusual cause of unilateral lung white-out

2019 ◽  
Vol 12 (5) ◽  
pp. e229413
Author(s):  
Sagnik Biswas ◽  
Animesh Ray
Keyword(s):  
Situs Inversus ◽  
Developmental Disorder ◽  
Pulmonary Hypoplasia ◽  
Respiratory Infections ◽  
Bronchial Tree ◽  
Lung Hypoplasia ◽  
Pulmonary Agenesis ◽  
Type Iii ◽  
Pulmonary Parenchyma ◽  
Lung Agenesis

Pulmonary agenesis is a rare developmental disorder with many syndromic associations. Type III agenesis presents with variable amounts of bronchial tree, pulmonary parenchyma and supporting vasculature. It is also reported to be associated with dextrocardia due to shifting of the heart to occupy the space left by the underdeveloped lung, which occurs as early as the fourth week of gestation. Right lung agenesis is considered rarer than its left-sided counterpart and also has a poorer prognosis due to increased chances of respiratory infections. We report a case of isolated right-sided lung hypoplasia with dextrocardia without situs inversus or heterotaxy.

scholarly journals Esophageal Atresia and Tracheoesophageal Fistula with Unilateral Pulmonary Agenesis - Hypoplasia

2013 ◽  
Vol 2 (2) ◽  
pp. 21
Author(s):  
Shraddha Verma ◽  
Jai Kumar Mahajan ◽  
Katragadda Laxmi Narsimha Rao
Keyword(s):  
Esophageal Atresia ◽  
Tracheoesophageal Fistula ◽  
Pulmonary Hypoplasia ◽  
Full Term ◽  
Lung Hypoplasia ◽  
Pulmonary Agenesis ◽  
Male Baby ◽  
Unilateral Pulmonary Agenesis

Association of unilateral severe pulmonary hypoplasia or agenesis and esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is an exceedingly rare and highly lethal combination. We report a case of full term male baby who had EA with TEF and right lung hypoplasia, managed at our centre. He is alive and doing well at 10 years of age.

scholarly journals IFN-λ4 is associated with increased risk and earlier occurrence of several common infections in African children

2021 ◽  
Author(s):  
Ludmila Prokunina-Olsson ◽  
Robert D. Morrison ◽  
Adeola Obajemu ◽  
Almahamoudou Mahamar ◽  
Sungduk Kim ◽  
...  
Keyword(s):  
Respiratory Infections ◽  
Genomic Region ◽  
First Episode ◽  
Genetic Associations ◽  
Gastrointestinal Infections ◽  
Type Iii ◽  
Functional Variants ◽  
Younger Age ◽  
Increased Risk ◽  
Pediatric Infections

AbstractGenetic polymorphisms within the IFNL3/IFNL4 genomic region, which encodes type III interferons, have been strongly associated with clearance of hepatitis C virus. We hypothesized that type III interferons might be important for the immune response to other pathogens as well. In a cohort of 914 Malian children, we genotyped functional variants IFNL4-rs368234815, IFNL4-rs117648444, and IFNL3-rs4803217 and analyzed episodes of malaria, gastrointestinal, and respiratory infections recorded at 30,626 clinic visits from birth up to 5 years of age. Compared to children with the rs368234815-TT/TT genotype (IFN-λ4-Null), rs368234815-dG allele was most strongly associated with an earlier time-to-first episode of gastrointestinal infections (p = 0.003). The risk of experiencing an infection episode during the follow-up was also significantly increased with rs368234815-dG allele, with OR = 1.53, 95%CI (1.13–2.07), p = 0.005 for gastrointestinal infections and OR = 1.30, 95%CI (1.02–1.65), p = 0.033 for malaria. All the associations for the moderately linked rs4803217 (r2 = 0.78 in this set) were weaker and lost significance after adjusting for rs368234815. We also analyzed all outcomes in relation to IFN-λ4-P70S groups. Our results implicate IFN-λ4 and not IFN-λ3 as the primary functional cause of genetic associations with increased overall risk and younger age at first clinical episodes but not with recurrence or intensity of several common pediatric infections.

scholarly journals Late presentation of unilateral lung agenesis in adulthood

2021 ◽  
Vol 52 (1) ◽  
Author(s):  
Arshed Hussain Parry ◽  
Mujahed Abdulsattar Ibrahim Raheem ◽  
Hussam Hassan Ismail ◽  
Osama Sharaf
Keyword(s):  
Main Bronchus ◽  
Left Lung ◽  
Left Hemithorax ◽  
Late Adulthood ◽  
Pulmonary Agenesis ◽  
Opaque Hemithorax ◽  
Dismal Prognosis ◽  
Mediastinal Shift ◽  
Lung Agenesis ◽  
Unilateral Pulmonary Agenesis

Abstract Background Pulmonary agenesis is a rare congenital anomaly with a reported prevalence of about 1 in 100,000 births. It may be bilateral or unilateral. Among the unilateral form, left lung agenesis is more common (70%); however, it is the right lung agenesis which carries a dismal prognosis due to the frequent association with a gamut of other congenital anomalies and greater degree of mediastinal shift leading to tracheo-bronchial and vascular distortion. The patients of unilateral pulmonary agenesis usually present in infancy or early childhood. Presentation in late adulthood as seen in our patient is rare. We present a case of left pulmonary agenesis that was diagnosed in 4th decade of life. Case presentation A 36-year-old male presented with gradually progressive exertional dyspnea of 1 month duration. Clinical examination revealed tachycardia and tachypnea. Chest radiograph showed opaque left hemithorax with ipsilateral mediastinal shift. Computed tomography clinched the diagnosis by demonstrating absence of left main bronchus, lung and left pulmonary artery with shift of heart, and great mediastinal vessels into left hemithorax. The patient was managed conservatively and discharged with attachment to out-patient department for regular follow-up. Conclusion Presentation of unilateral lung agenesis in late adulthood, as seen in the present case is extremely rare. This case report highlights that, a rare condition like unilateral pulmonary agenesis, should be considered in the list of differentials in an adult presenting with opaque hemithorax with ipsilateral mediastinal shift on radiography.

Abnormalities of the Fetal Chest

2019 ◽  
Author(s):  
Karen M. Davidson
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Airway Obstruction ◽  
Diaphragmatic Hernia ◽  
Pulmonary Hypoplasia ◽  
Abnormal Development ◽  
Neurenteric Cyst ◽  
Congenital Pulmonary Airway Malformation ◽  
Bronchopulmonary Sequestration ◽  
Lung Agenesis ◽  
Pulmonary Airway

The normal and abnormal development of the organs lying within the fetal thorax is discussed.  The abnormalities reviewed include more common findings of pulmonary hypoplasia, congenital diaphragmatic hernia, congenital pulmonary airway malformation, bronchopulmonary sequestration, as well as the rarer conditions of congenital fetal hydrothorax, congenital high airway obstruction syndrome, bronchogenic cysts, neurenteric cysts, and lung agenesis.  With each abnormality, the clinical implications for the fetus, best methods for prenatal diagnosis, as well as possible additional anomalies, syndromes, and aneuploidies are described.  In utero and postnatal treatments are also reviewed.   This review contains 10 figures, and 37 references. Key Words: Pulmonary hypoplasia, lung-head ratio, congenital diaphragmatic hernia, congenital pulmonary airway malformation, congenital fetal hydrothorax, bronchopulmonary sequestration, congenital high airway obstruction syndrome, bronchogenic cyst, neurenteric cyst, lung agenesis

Abnormalities of the Fetal Chest

2019 ◽  
Author(s):  
Karen M. Davidson
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Airway Obstruction ◽  
Diaphragmatic Hernia ◽  
Pulmonary Hypoplasia ◽  
Abnormal Development ◽  
Neurenteric Cyst ◽  
Congenital Pulmonary Airway Malformation ◽  
Bronchopulmonary Sequestration ◽  
Lung Agenesis ◽  
Pulmonary Airway

The normal and abnormal development of the organs lying within the fetal thorax is discussed.  The abnormalities reviewed include more common findings of pulmonary hypoplasia, congenital diaphragmatic hernia, congenital pulmonary airway malformation, bronchopulmonary sequestration, as well as the rarer conditions of congenital fetal hydrothorax, congenital high airway obstruction syndrome, bronchogenic cysts, neurenteric cysts, and lung agenesis.  With each abnormality, the clinical implications for the fetus, best methods for prenatal diagnosis, as well as possible additional anomalies, syndromes, and aneuploidies are described.  In utero and postnatal treatments are also reviewed.   This review contains 10 figures, and 37 references. Key Words: Pulmonary hypoplasia, lung-head ratio, congenital diaphragmatic hernia, congenital pulmonary airway malformation, congenital fetal hydrothorax, bronchopulmonary sequestration, congenital high airway obstruction syndrome, bronchogenic cyst, neurenteric cyst, lung agenesis

scholarly journals Unilateral Pulmonary Agenesis and Gastric Duplication Cyst: A Rare Association

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Amir Halilbasic ◽  
Fahrija Skokic ◽  
Nesad Hotic ◽  
Edin Husaric ◽  
Gordana Radoja ◽  
...  
Keyword(s):  
Duplication Cyst ◽  
Unique Case ◽  
Gastric Duplication ◽  
Gastric Duplication Cyst ◽  
Pulmonary Agenesis ◽  
Bronchial Secretions ◽  
Rare Association ◽  
Lung Agenesis ◽  
Vague Abdominal Pain ◽  
Unilateral Pulmonary Agenesis

Lung agenesis and gastric duplication cysts are both rare congenital anomalies. Gastric duplication cysts can present with nausea, vomiting, hematemesis, or vague abdominal pain. Unilateral pulmonary agenesis can present with respiratory distress which usually occurs due to retention of bronchial secretions and inflammations. We report the unique case of right pulmonary agenesis associated with gastric duplication cyst.

Unilateral Pulmonary Hypoplasia with Abdominal Situs Inversus

2012 ◽  
Vol 79 (10) ◽  
pp. 1360-1362 ◽  
Author(s):  
Sumantra Sarkar ◽  
Rakesh Mondal ◽  
Madhumita Nandi ◽  
Niloy Kumar Das
Keyword(s):  
Situs Inversus ◽  
Pulmonary Hypoplasia

scholarly journals Unilateral lung hypoplasia presenting in seventh decade of life: a rare case report

2018 ◽  
Vol 5 (6) ◽  
pp. 1532
Author(s):  
Baishakhi Chandra ◽  
Bhaskar K. ◽  
Paramjyothi G. K.
Keyword(s):  
Case Report ◽  
Ct Scan ◽  
Lung Volume ◽  
Clinical Condition ◽  
Rare Case ◽  
Pulmonary Hypoplasia ◽  
Lung Hypoplasia ◽  
Late Adolescent ◽  
Left Hemithorax ◽  
Rare Case Report

Pulmonary hypoplasia is a bronchopulmonary foregut anomaly in which gross morphology of the lung is preserved but there is decrease in the number or size of airways, vessels, and alveoli. Unilateral pulmonary hypoplasia is a rare clinical condition and most patients reported in the literature are newborns and infants, but patients may remain asymptomatic until late adolescent or till adulthood 65 years old male with parkinsonism was referred to our department with complaints of recent chest symptoms. Clinical examination revealed loss of lung volume in left hemithorax. CT scan thorax and bronchoscopy confirmed the diagnosis of left sided pulmonary hypoplasia. This case report highlights the possibility of pulmonary hypoplasia as one of the differential diagnoses in adult patients presenting with loss of lung volume and to the best of our knowledge he is the eldest patient so far reported.

scholarly journals Inhalation Formulation & Dosage of PVP Iodine for Respiratory Infections Treatment

2021 ◽  
Author(s):  
Oron Zachar
Keyword(s):  
Respiratory Infections ◽  
Early Stage ◽  
Wound Care ◽  
Ambient Air ◽  
Iodine Concentration ◽  
Molecular Iodine ◽  
Global Scale ◽  
Bronchial Tree ◽  
Viral Respiratory Infections ◽  
Viral Respiratory

The aftermath of the Covid-19 pandemic calls for a rethink of pharmaceutical options for treatment of viral respiratory infections. Respiratory infections start primarily at the surface epithelial cells. Being in permanent direct contact with the ambient air, the respiratory system tissue surface may be amenable to topical treatment approaches. We evaluate and define the method and options of translating effective wound care treatments into the realm of respiratory infections treatment and preventions. The goal is a broad-range, safe and cheap anti-viral respiratory infections medication, which can be made globally available for early-stage home use. In a previous publication we discussed nanoparticles-based formulations. In this article we propose and evaluate a new mode of use of a well-established topical antiseptic – PVP Iodine (PVP-I). In contrast to all previous proposals of PVP-I disinfection nasal sprays or oral gargle, we present a deep and well controlled inhalation protocol to disinfect in one treatment modality both the bronchial tree, upper respiratory nasal and throat tissue simultaneously, using ultrasonic mesh nebulizers. Molecular iodine is quickly absorbed from the lungs and bronchial tree into the blood, within about 10 minutes. Yet, iodine effectively disinfects most viruses within 1 minute, including SARS-CoV-2. Moreover, the slower to absorb PVP-I complex provides a continuously eluting reservoir that maintains the iodine concentration in the airways surface fluids for several hours. These and other factors unique to inhalation are discussed. We provide: (i) Dosage calculation and delivery protocols; (ii) Safety analysis based on guidelines, animal trials and WHO review reports; (iii) Evaluating published pilot clinical trials of SARS-CoV-2 with related nasal spray or oral gargle PVP-I treatments; (iv) Evaluating the potential use and modification of existing off-the-shelf market products to render our proposed treatment immediately available on a global scale. Our recommended formulations comprise of PVP-I at concentrations 0.5% - 5% and pH between pH=6.5 to pH=7.5 (significantly higher than pH~3.5 of present market products). Inhalation dosage of just 0.5 mL may already be effective, which translates to aerosolizing 2 mL with a common nebulizer device. PVP-I is globally available over the counter (OTC). The same is true of ultrasonic nebulizers. In practice, all the ingredients are already globally available to consumers. Hence, we hope health and regulatory authorities worldwide will invest the efforts to establish the validity of the proposed treatment. In the meantime, to prevent confusion or misuse by free-willing users and to facilitate clinical evaluation, we outline a prescription for the proper adaptation use of common commercially available market products. I addition, we propose that the same formulations and protocols can be implemented prophylactically in hospital intensive care units (ICU) for the prevention of ventilator associated pneumonia (VAP).

scholarly journals Neonatal repair of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome: a case report

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Takahiro Ito ◽  
Ikuo Hagino ◽  
Mitsuru Aoki ◽  
Kentaro Umezu ◽  
Tomohiro Saito ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Pulmonary Veins ◽  
Median Sternotomy ◽  
Bronchial Tree ◽  
Pulmonary Venous Obstruction ◽  
Goldenhar Syndrome ◽  
Venous Obstruction ◽  
Vertical Vein ◽  
Lung Agenesis ◽  
Anomalous Pulmonary Venous Connection

Abstract Background Total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare. Case presentation We present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7–0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction. Conclusion We report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.

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