Surfactant protein B deficiency in infants with respiratory failure

MA Wilder - The Journal of perinatal & neonatal nursing, 2004 - journals.lww.com
Abstract Surfactant Protein B (SP-B) deficiency has been recently identified as an
uncommon, autosomal recessive lung disorder in term infants. This inability to produce SP-B …

Growth of lungs after transplantation in infants and in children younger than 3 years of age

AH Cohen, GB MALLORY Jr, K Ross… - American journal of …, 1999 - atsjournals.org
We report serial measurements of lung volume and airflow in small children after lung
transplantation. We expected that immature lungs could grow and develop normal volumes …

Congenital surfactant protein B deficiency–emphasis on imaging

B Newman, JP Kuhn, SS Kramer, JA Carcillo - Pediatric radiology, 2001 - Springer
Background and objectives. Surfactant protein B deficiency has become increasingly
recognized as a cause of severe prolonged respiratory distress. Little has been written about …

Hereditary surfactant protein B deficiency resulting from a novel mutation

M Somaschini, S Wert, G Mangili, A Colombo… - Intensive care …, 2000 - Springer
Hereditary surfactant protein B (SP-B) deficiency is an autosomal recessive disease in which
affected infants are unable to produce normally functional surfactant, resulting in neonatal …

Recurrent pulmonary alveolar proteinosis secondary to agammaglobulinemia

T Patiroglu, B Akyıldız, TE Patiroglu… - Pediatric …, 2008 - Wiley Online Library
Pulmonary alveolar proteinosis (PAP) is characterized by the accumulation of surfactant
derived material in the lung of patients. PAP is rare in children. The patient presented with …

Treatment of interstitial lung disease in children

R Dinwiddie - Paediatric respiratory reviews, 2004 - Elsevier
The treatment of interstitial lung disease in children depends on the nature of the underlying
pathology. In approximately 50% of cases a specific aetiology can be found such as: chronic …

Inherited disorders of neonatal lung diseases

M Yurdakök - The Turkish Journal of Pediatrics, 2004 - turkjpediatr.org
Although genetic factors are assumed to have a role in the etiology of respiratory distress
syndrome (RDS), specific genes underlying this susceptibility are incompletely known. The …

Pediatric lung transplantation

CB Huddleston - Current treatment options in cardiovascular medicine, 2011 - Springer
Opinion statement Lung transplantation in children is an effective treatment for end-stage
pulmonary disease after all medical therapy has failed. It requires a huge investment in …

Surfactant protein B deficiency: insights into inherited disorders of lung cell metabolism

A Hamvas - Current problems in pediatrics, 1997 - Elsevier
Aaron Hamvas, MD ulmonary surfactant is a complex mixture of p phospholipid and protein
that is synthesized--by alveolar type II pneumocytes and is necessary to maintain alveolar …

Clinical biological and genetic heterogeneity of the inborn errors of pulmonary surfactant metabolism

M Tredano, J Blic, M Griese, JC Fournet, J Elion… - 2001 - degruyter.com
Pulmonary surfactant is a multimolecular complex located at the air-water interface within
the alveolus to which a range of physical (surface-active properties) and immune functions …