Background: Pulmonary alveolar proteinosis is an extremely rare lung disease in animals and humans. It is characterized by the deposition of a large amount of phospholipoproteinaceous material in the alveoli. There are several possible etiologies, both congenital and acquired. Alveolar macrophages play an important role in the clearance of surfactant. This is the first report of pulmonary alveolar proteinosis in the feline species. Case presentation: Pulmonary alveolar proteinosis was diagnosed in an 8-month-old cat with chronic tachypnea, failure to thrive and finally respiratory distress. The diagnosis was based on the milky appearance of the bronchoalveolar lavage fluid taken under general anesthesia after bronchoscopy. Because of the worsening respiratory distress and development of anorexia the kitten was euthanized. Histopathology of the lungs showed alveoli and bronchi filled with eosinophilic material. Electron microscopy revealed lamellated intra-alveolar bodies. As the granulocyte-macrophage colony-stimulating factor was elevated in the serum and no autoantibodies against granulocyte-macrophage colony-stimulating factor were detected, a primary hereditary pulmonary alveolar proteinosis was suspected. The underlying cause was thought to be a dysfunction of the receptor of the granulocyte-macrophage colony-stimulating factor, however, a mutation in the genes encoding the alpha and beta chains of this receptor has not been found. Conclusion: This is the first description of pulmonary alveolar protienosis in a cat. This kitten is thought to have a primary hereditary pulmonary alveolar proteinosis with a possible defect in the signalling pathway of the receptor of the granulocyte-macrophage colony-stimulating factor. The imaging and pathologic findings are similar to those of humans.
BackgroundPaediatric heart failure (HF) has an import-ant economic and social impact in public health. Drugs acting on the renin-angiotensin system are regarded as mainstay to lower the burden of HF for patients and families. A safe and efficient use especially in young chil-dren has been debated since several years and remains a challenge for physicians. We aimed to characterise the different therapeutic strategies for the management of paediatric HF that are currently practiced across Europe with special focus on the use of Angiotensin Converting Enzyme Inhibitors (ACE-I).MethodsA Europe-wide web-based survey and a sub-sequent DELPHI questionnaire was developed in the con-text of EU’s Seventh Framework Programme under grant agreement n°6 02 295 using standard recommendations for survey design. The questionnaire consisted of 23 ques-tions addressing different aspects of drug therapy for HF in children. Use patterns of ACE-I i.e. dosage by age group, effectiveness and toxicity assessment according to HF ae-tiology where investigated. Clinicians from 204 different hospitals of 39 European countries were invited via e-mail to participate. The subsequent DELPHI process discussed controversial responses within a selected expert panel in two rounds.ResultsThe response rate of the survey had been 50%. The survey delivered valuable information about the current paediatric heart failure therapy, especially with regard to the pattern of ACE-I use. Enalapril seems to be already the ACE-inhibitor of choice for children and ad-olescents. A suitable formulation and knowledge about dosing as well as adverse events might offer Enalapril also for neonates and infants. Several controversial aspects which were identified within the survey and which are re-lated to paediatric heart failure therapy had been put up for discussion to the DELPHI expert panel. They showed a high degree of consensus in their professional criteria about most of the contents presented for discussion. Pos-sible starting points in the way towards a standardisation of paediatric heart failure therapy were identified. With re-gard to non-consensus statements, DELPHI experts pro-vided a better visibility to some aspects of clinical practice with greater disparity of opinio Diagnostic and therapeu-tic approachesns among physicians.ConclusionThis survey and the subsequent DELPHI questionnaire provided an overview of the clinical treat-ment routine of paediatric HF across Europe. ACE-I seem to be a crucial part of the treatment strategies. Consensus but also still controversial aspects of clinical practice rou-tines for a safe and effective use of heart failure treatment for children in Europe were identified.The research leading to these results has received funding from the EU’s Seventh Framework Programme (FP7/2007-2013) under grant agreement n°6 02 295 (LENA).
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