This study determined the species of 54 staphylococci isolates from canine otitis and their ability to produce biofilm through the Congo red agar method, confirmed by scanning electron microscopy. The most frequently identified species were S. intermedius and S. simulans. Results showed that 30% of the strains were biofilm producers.
Niemann-Pick disease (NPD) is a rare lysosomal storage disorder that is generalized and severe, as well as being characterized by intracellular sphingomyelin accumulation caused by deficiency of sphingomyelinase activity.(1)From a respiratory standpoint, the various types of NPD, including type B, can cause progressive loss of pulmonary function, with suggestive radiological findings. We report the case of a 27-year-old female patient with type B NPD. The patient was initially referred for outpatient treatment in 2003, presenting with a history of hepatosplenomegaly and delayed psychomotor development since childhood. The diagnosis of NPD was confirmed by measuring peripheral leukocyte sphingomyelinase activity, which was found to be decreased (0.009 nmol • h −1• mg −1 of protein; reference value, 0.745of protein). Serology for HIV, hepatitis B, and hepatitis C was negative, as was serology for syphilis (venereal disease research laboratory test). In addition, ANF was negative. Gaucher's disease was ruled out because beta-glucosidase levels were normal. The patient developed ascites and complained of mild dyspnea, being referred to the pulmonology clinic. She underwent a six-minute walk test (six-minute walk distance [6MWD] = 396 m; predicted 6MWD = 724 m), showing intense dyspnea at the end of the test (modified Borg scale score = 9) but no oxygen desaturation (SpO 2 = 97%). Spirometry results were suggestive of restrictive lung disease (FEV 1 = 1.77 L [61% of predicted]; FVC = 2.07 L [57% of predicted]; and FEV 1 /FVC ratio = 85%). A chest X-ray revealed no pulmonary changes. An HRCT scan revealed interlobular septal thickening in the bases and apices, together with right pleural effusion (Figures 1a and 1b). A rare entity, NPD is an autosomal recessive disorder characterized by abnormal accumulation of sphingomyelin in the reticuloendothelial system. Type B NPD has heterogeneous clinical manifestations and is mainly characterized by hepatosplenomegaly and progressive hypersplenism. Neurological involvement is rare; when it does occur, it is mild. The diagnosis is made by careful history taking and physical examination, as well as by determination of sphingomyelinase activity (which is decreased in peripheral leukocytes), together with fibroblast cell culture or analysis of bone marrow biopsy specimens revealing characteristic sea-blue histiocytes. There can be respiratory impairment secondary to macrophage and sphingomyelin accumulation in the distal airways and the alveoli, which leads to gradual deterioration of pulmonary function, reducing exercise tolerance. The clinical manifestations are heterogeneous, ranging from absence of symptoms to respiratory failure, and lung disease can progress to hypoxemia requiring oxygen therapy. (2,3)
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