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Objective To investigate the clinical characteristics of pediatric Mycoplasma pneumoniae (MP)complicated with lung abscess.Methods Data of 4 cases suffering from pulmonary abscess associated with MP from Jan.2000 to Jun.2011,including manifestations,imaging,laboratory examinations and therapies,were collected and analyzed.Related literatures were reviewed.Results Two female and 2 male cases were included in this study.The age ranged from 3 to 6 years old,and all patients had a history of acute onset,severe cough and little sputum.Three cases had remittent fever,and 3 cases had fixed small and medium-sized blisters sound in the lung.The levels of MP antibody M (MP-IgM) in the serum were higher than 1:160 and sputum fluorescence quantitative-mycoplasma pneumonia (FQ-MP DNA) tests were positive in 4 patients.The white blood cells (WBC),polymorphonuclear leukocyte (PMN),C-reactive protein (CRP),erythrocyte sedimentation rate (ESR) in 3 cases were significantly higher than the normal level,and the FQ-MP DNA tests in the bronchoalveolar lavage fluid (BALF) were positive in 3 cases.The FQ-MP DNA in both blood and chest water were positive in 1 case.Cultures of sputum,blood,chest water,BALF and procalcitionin tests were negative,except for 1 case with positive sputum culture of Branhamellacatarrhalis.The imaging examinations on admission of 4 cases strongly suggested that the interstitial and parenchymal lung disease and subsequently appeared the different sized cystic shadow,liquid fiat cystic shadow,thickened wall empty tissue image during the course of 5 to 20 days.All cases were sequentially treated with azithromycin after full erythromycin,3 cases received intravenous glucocorticoid,1 case received intravenous gamma globulin.The bronchial mucosal hyperemia and edema,submucosal scattered small nodules,pathological changes of bronchial sticky phlegm jam were observed in 3 cases by flexible bronchoscopy.After 3 to 5 days of admission,all patients' temperature returned to normal,the cough was reduced,and the characteristics of the lung were gradually improved.After 3 to 4 weeks,the chest radiograph showed that most pneumonia was absorbed.The 6 to 18 month follow-up indicated that absorption was good.The upper left lung limited atelectasis was found in 1 case.Conclusions Pulmonary abscess is a rare complication of MP.The major clinical manifestations are high fever,significantly higher levels of blood WBC,PMN,CRP and ESR,and high density of shadow in chest Xray.The regular dose of erythromycin doesn't work well.However,the prognosis is good after conservative therapy with the sufficient macrolide antibiotics with glucocorticoid and immunoglobulin.
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<p><b>OBJECTIVE</b>To study the clinical characteristics and diagnosis of the Johanson-Blizzard syndrome.</p><p><b>METHOD</b>The clinical characteristics and diagnosing procedure of 1 case with Johanson-Blizzard syndrome were analyzed, and genetic analysis was made in diagnosing procedure, and 28 cases of Johanson-Blizzard syndrome with detailed clinical data were reviewed and analyzed.</p><p><b>RESULT</b>A one year and nine months old girl, who was initially admitted to the hospital because of fatty diarrhea and increased frequency of defecation. Imperforate anus, and aplastic alae nasi was noticed after birth. On physical examination, short stature, mental retardation, tooth abnormalities and scalp defects were observed. Fat globule was found by routine stool test. Serum biochemistry showed an exocrine and endocrine pancreatic insufficiency, CT scan of the abdomen demonstrated fatty replacement of the pancreas, UBR1 gene analysis showed heterozygous for two missense changes. In all 29 cases, exocrine pancreatic insufficiency (72.4%) and hypoplasia of the alae nasi (93%) were the most common clinical manifestations, and sensorineural hearing loss (59%), scalp defects (69%) and hair thinning or upsweep of the hair (44.8%), hypothyroidism (44.8%), absence of permanent teeth (44.8%) and imperforate anus (21%) were also very common, but did not include consanguineous marriage of parents (10.3%).</p><p><b>CONCLUSION</b>Johanson-Blizzard syndrome is a rare autosomal recessive multisystem disorder, it is characterized by the association of congenital exocrine pancreatic insufficiency and hypoplasia or aplasia of the nasal wings, and can be diagnosed by clinical characteristics and UBR1 gene analysis.</p>