ABSTRACT
ObjectiveTo explore the radiological features of hypophosphatasia.MethodsFive cases of hypophosphatasia were definitely diagnosed,which included 3 males and 2 females aged from 5 months to 23 years.The laboratory assays were analyzed,radiological appearances of bone were determined and differential diagnoses were made.ResultsThe alkaline phosphatase in blood serum of five patients decreased,which were 8,20,13,21,and 18 U/L respectively.Phosphoethanolamine increased in blood serum of the five patients,which were 16.5,13.5,21.6,18.7,and 28.9 μmol/L respectively.Phosphoethanolamine also increased in urine,which were 2350,9120,3520,5280,and 1820 μmol/L respectively.Calcium in blood serum increased,which were 4.2,5.6,4.9,6.1,and 3.5 mmol/L respectively.X-ray images displayed that the density of bone decreased in 5 cases,the metaphyses exhibited widening and cupping in 4 cases,the provisional calcification zone of the metaphysis became thinning or disappearing in 4 cases,long bone bended in 4 cases and pathologically fractured in 1 case.Conclusion Hypophosphatasia can be indicated by its relatively special radiological appearance,and it can be diagnosed and differentiated from rickets,osteomalacia and osteogenesis imperfect by the laboratory examination combined with its clinical presentation.
ABSTRACT
Objective Our purpose was to improve the konwlege and diagnosis level of esophageal intramural pseudodiverticulosis(EIPD).Methods All 3 cases were underwent single and double contrast esophagograms.Analyze the findings on esophagography in 3 cases.Results Multiple small tiny cyst like outpouchings with a narrow neck extending outward esophageal wall were found in all of the 3 cases, the length range of outpouchings diameter was 1-4 mm,and the length range of neck was 1-2 mm,the pseudodiverticula had a diffuse distribution in 2 cases,and segmental distribution on the middle and the lower esophagus in the other case.Some necks of pseudodiverticula incline to stomach with 30?-45? in 2 cases;and intramural tracking was found in 1 case,the length range of tracking was 5-10 mm;esophageal stenosis in esophagogastic junction and reflux esophagitis was found only in 1 case;1 case underwent endoscopy and ostiums of pseudodiverticula were found;biopsy showed submucosal chronic inflammation surrounding the neck of pseudodiverticula,squamous metaplasia of the epithelium in both the neck and outpouchings.Conclusion EIPD is a rare benign disorder characterized by dilation of the submucosal glands.The value of esophagography is to distinguish sublimes ulcer caused by esophagitis and esophageal fenestrate from EIPD.The characteristic radiographic appearance is numerous intramural esophageal contrast-filled cystiform outpouchings,and some necks incline in the direction of stomach.When the typical appearance found on barium studies,the diagnosis of EIPD can be made.