TOPIC: Lung Pathology TYPE: Medical Student/Resident Case Reports INTRODUCTION: Hereditary Hemorrhagic Telangiectasia is a multisystem disease which results in AV Malformations and telangiectasia. It has autosomal dominant inheritance prevalence of 1 5000 to 10,000. Patients with Pulmonary AVMs can present hypoxia, more post exercise sedentary position improvement on standing. presenting hypoxi...

severe congenital neutropenia (scn) is a rare primary immunodeficiency. different genes are found to be associated with scn, including ela2, hax1, was, gfi1, g-csfr. also, recently g6pc3 as a rare gene in scn has been reported. patients with g6pc3 often have cardiac and/or urogenital malformations. two patients with   persistent   severe   neutropenia,   recurrent   infections   and   maturatio...

introduction: congenital anomalies are a common cause of disability and mortality in newborns and their treatments involves high costs for the society. this study aimed to investigate the prevalence of congenital anomalies and their causes. materials and methods: this research was a descriptive-analytical study and the population included all the newborns in hospitals of the city of ilam in 201...

Thoracic congenital malformations may be associated with long-term pulmonary morbidity. Certainly, this is the case for Esophageal Atresia (EA) and Congenital Diaphragmatic Hernia (CDH). These conditions have variable degrees of impaired development of both the airways and the lung vasculature, with a postnatal impact on lung function and bronchial reactivity. Pulmonary complications themselves...

Congenital lung agenesis is an extremely rare condition with an estimated prevalence of 34 in 1,000,000 live births. It is often associated with other congenital malformations of the skeletal, cardiovascular, urogenital, and gastrointestinal systems. We discuss the case of a 5-month-old who presented with increasing stridor over 1 month. Imaging revealed right lung agenesis, complete dextromalp...

Congenital lung anomalies represent a diverse group of developmental malformations of the lung parenchyma, arterial supply, and venous drainage, which may present anywhere from the prenatal period through adulthood. It is imperative for radiologists to be aware of imaging techniques and imaging appearance of these anomalies across the pediatric age range. This review presents the spectrum of th...

Bronchogenic cysts are benign congenital malformations derived from the tracheobronchial tree that correspond to non-functioning lung tissue.1,2 The majority of cases are diagnosed in the pediatric population.3 In adults they are fundamentally found in mediastinum and lung parenchyma.3 However, they can sometimes be located in the thoracic wall, abdomen or cervical region4; in the cervical area...

INTRODUCTION Pulmonary arteriovenous malformations are a common cause of progressive cyanosis in children after cavopulmonary anastomoses. We analyzed the pulmonary histologic characteristics from children in whom pulmonary arteriovenous malformations developed after procedures that resulted in pulmonary arterial blood flow devoid of hepatic venous effluent. METHODS We performed routine histo...