Loading...

Archive

    06 April 2013, Volume 28 Issue 4 Previous Issue    Next Issue

    For Selected: Toggle Thumbnails
    Differential diagnosis of extrahepatic biliary atresia from infantile intrahepatic cholestasis disease within one week.
    2013, 28(4): 263-267. 
    Abstract ( )  

    Abstracts:Objective    Different diagnostic methods of biliary atresia (BA) and infantile intrahepatic cholestasis disease were investigated in order to find a simple, fast,practical,economic and non invasive differential diagnostic method. Methods    A total of 584 cases of infantile cholestasis were collected from May 2006 to June 2012 for persistent jaundice,pale yellow or white shit who lived in Department of Pediatric Digestion and Infection, Tongji Hospital, Tongji Medical College, Huazhong University of Science & Technology(HUST). Seven methods including clinical diagnosis,blood biochemical tests, liver and gallbladder ultrasonography, dynamic continuous duodenal liquid bile check, nuclide hepatic imaging, magnetic resonance imaging and histology were applied for differential diagnosis and the results were analyzed. Results    The correctness of clinical diagnosis method was 74.5%, sensitivity 81.6%,specificity 69.9%; liver size: 49.0%,89.0%, and76.9%; stool color:83.2%,96.1% and96.7%;blood total bilirubin:63.0%,93.1%and91.2% ; serumγ-GT:79.7%, 71.1%and78.7% ; dynamic duodenal liquid color check:93.3%,91.7% and92.7%; bile acid of duodenal liquid:97.8%,100.0% and 100.0%;B graphy :89.7%,91.7%and94.3%;porta fibre block check:72.1%,29.4%and 68.7%;nuclide hepatic imaging :60.5%,100.0% and 100.0%;MRCP:88.3%,96.5%and94.4%;liver pathology :97.4%,98.2% and 94.9%. Conclusion    Differential diagnosis in 1 week can help differentiate biliary atresia from infantile intrahepatic cholestasis. B-ultrasonography and dynamic duodenal fluid test are simple, fast practical,economic  and noninvasive as differential diagnostic methods.

    Diagnosis and treatment of primary intestinal lymphangiectasia in children. 
    2013, 28(4): 268-271. 
    Abstract ( )  

    Abstract:Objective    To improve the diagnosis and treatment level of primary intestinal lymphangiectasia (PIL) in children. Methods    Retrospectively analyze the clinical manifestations, laboratory examinations, gastroscopy and imaging features, lymph radionuclide imaging, pathological results, therapy experience in 13 PIL children who were admitted to our hospital from January ,2007 to February, 2012; related literatures were reviewed. Results    The youngest case was a 4-month-old infant , the oldest case was a 11-year-old child, and 8 cases were within 1 year. Edema[13(100%)],infection[12(92.3%)],diarrhea[11(84.6%)],fever[10(76.9%)],and ascites[9(69.2%)]were common manifestations; hematochezia[3(23.1%)], pleural effusion[3(23.1%)],convulsions[ 3(23.1%)]and abdominal mass[ 1(7.7%)]were relatively rare. Infection was predominant manifestation, including respiratory tract,blood,lower limbs fascia and esophagus; pathogens include bacterium,virus,fungus and ectosarc. The laboratory findings in these patients were lymphocytopenia[13(100%)], hypoalbuminemia[13(100%)], hypogammaglobulinemia[13(100%)], the CD4 cell counts[9(90.0%)] and serum IgG levels [10(100%)],which were significantly decreased, while B cell and NK cell counts were reduced in one of ten cases. The gastroscopy revealed nodular lesions in duodenal that appeared white opaque spots[13(100%)]. Pathological examinations indicated dilated lymphatic channel in mucosal and submucosal [9(69.2%)]. Lymph radionuclide imaging discovered abnormalities consistent with PIL in ten out of twelve patients, including four cases that were negative for pathological examinations. The main ways of treatment for PIL were introduction of a low-fat and full-MCT diet, supplement of albumin and globulin,and control of the infection. The temperature returned to normal and edema were alleviated in most patients after internal therapy. However,therapeutic efficacy of diarrhea was not satisfactory,Only three children recovered from hypoalbuminemia and six children recovered from lymphocytopenia. Two cases had a complete remission after surgical therapy without relapse until 2012-12-31. Conclusion    Compared to adult IL,PIL in children has similar manifestations,but infection is the predominant clinical manifestation in these patients;lymph radionuclide imaging is suggested to be used as one of the valuable methods in diagnosing PIL in children;internal therapy is not satisfactory in child PIL;surgical therapy can be used if pathological focus is localized.

    A time-effect analysis of postural orthostatic tachycardia syndrome treated with midodrine hydrochloride in children.
    2013, 28(4): 274-276. 
    Abstract ( )  

    Abstract:Objective    The time-effect relationship in treating postural orthostatic tachycardia syndrome (POTS) of children with alpha receptor agonist midodrine hydrochloride was analyzed to explore the optimum course of treatment for POTS children. Methods    A total of 104 cases of the POTS children were studied from 2005 to 2011 in Peking University First Hospital. A chi-square test was used to evaluate the relationship between effect and time of medication, and the time-effect curve was constructed. Results    According to the 104 cases, the time accumulative total efficacy for 1 month, 2 months, 3 months, 4 months, 5 months and 6 months were 19.23%, 48.08%, 69.23%, 73.08%, 74.04% and 75.96%, respectively. The time accumulative total efficacy for 3 months was higher than that for 1 month or 2 months (P<0.05), but there was no difference in the efficacy among 4 months, 5 months and 6 months treatment and with 3 months treatment. Conclusion    It has the best treatment efficacy when the course of treatment for POTS children with midodrine hydrochloride is 3 months, and prolonging the duration of treatment does not significantly improve the therapeutic effect.

    Investigation of the treatment with intravenous sotalol for incessant tachyarrhythmias in children. 
    2013, 28(4): 277-280. 
    Abstract ( )  

    Abstract: Objective    To investigate the safety and efficacy of intravenous(iv) sotalol in the treatment of pediatric incessant tachyarrhythmias with normal cardiac function. Methods    Twenty-six  children admitted to Pediatric Cardiologic Department of Tsinghua University First Hospital between October 2011 and April 2012  were treated by iv sotalol and the efficacy of solitary iv sotalol and iv sotalol plus iv propafenone were investigated; the duration between start of iv sotalol to the point of reversion to sinus rhythm was also studied and it was investigated whether sinus rhythm could be maintained. Blood pressure, heart rate and rhythm were closely monitored during iv sotalol; QTc and PR interval were measured before and after iv sotalol. Results     Eighteen patients (69.2%) were successfully reversed to sinus rhythm during solitary iv sotalol; duration between start of iv sotalol to reversion of sinus rhythm was (5.3 ± 9.3)(0.05~26)hours. Six patients (23.1%)were successfully reversed to sinus rhythm during iv sotalol plus iv propafenone; duration between start of iv sotalol to reversion of sinus rhythm was (18.0 ± 22.3)(2.0~48.0)hours. Totally 24 patients(92.3%)were successfully reversed to sinus rhythm. Successful rates of iv sotalol for different types of tachycardia were almost the same, while durations between start of iv sotalol to the point of reversion to sinus rhythm were obviously different. The duration between start of iv sotalol to the point of reversion to sinus rhythm for AVRT was significantly shorter than AT, AF and VT(P<0.05).QTc prolongation(from253~398ms to 486~500ms)appeared in 2 patients (8.0%) within 48hours after sinus rhythm were gained; their QTc reversed to normal range respectively 48 hours and 144 hours after withdrawal of iv sotalol and addition of oral sotalol. No drug-associated arrhythmias or other adverse effects were detected during iv sotalol. Conclusion    (1)Intravenous sotalol can be safely and effectively used to terminate pediatric tachycardia with normal cardiac function.(2)No pro arrhythmic effects or intoxication of organs are detected during iv sotalol. (3)Close monitoring of QTc is required during iv sotalol.(4)Addition of iv propafenone can help to improve the efficacy of iv sotalol for tachyarrhythmia.

    Detection of minimal residual disease in childhood acute lymphoblastic leukemia by multiplex PCR and automated fragment analysis. 
    HUANG Li-bin*, KE Zhi-yong, ZHANG Xiao-li, HU Chang-ming, ZHU Xiao-xia, LUO Xue-qun.
    2013, 28(4): 281-285. 
    Abstract ( )  

    Abstract:Objective    To explore the feasibility of monitoring minimal residual disease (MRD) in childhood acute lymphoblastic leukemia (ALL) by detection of cloned IgH and TCRγ gene rearrangements using multiplex polymerase chain-reaction (PCR) and automated fragment analysis. Methods    In this 1∶3 matched case-control study, 4 cases of very early bone marrow relapsed non-high risk ALL (relapsed group) and 12 cases of non-relapsed non-high risk ALL as control (control group) were enrolled in the First Affiliated Hospital of Sun Yat-sen University from Jan. 2009 to Dec.2011. All patients were treated with Guangdong 2008 ALL protocol. Bone marrow samples were collected at four time points: at diagnosis, the end of induction, the beginning of  reinduction and the third month of maintenace treatment. Cloned IgH and TCRγ gene rearrangements were amplified by multiplex PCR. The clonality of PCR production was analyzed by GENEMAPPERID softwares. Detectable clonality of IgH/TCRγ was defined as MRD positive. Results       At diagnosis, the frequency of cloned IgH and TCRγ in all patients was 100% and 56%. The positive rate of MRD was found to have no statistical difference between two groups at the end of induction, while the difference of the MRD positive proportion between the two groups was statistically significant at the beginning of reinduction and the third month of maintenane therapy, which was much more higher in relapse group than that of control group. Conclusion    Detection of monoclonal IgH/TCRγ gene rearrangements by multiplex PCR with automated fragment analysis can be used as a method to monitor MRD during treatment for childhood ALL.

    Effectiveness of ALL-2005 protocol in the treatment of acute lymphoblastic leukemia in infants. 
    SONG Jun-hong*, TANG Jing-yan, XUE Hui-liang, PAN Ci, ZHOU Min, CHEN Jing.
    2013, 28(4): 286-299. 
    Abstract ( )  

    Abstract:Objective    To evaluate the therapeutic effectiveness and prognostic factors of ALL in infants treated with ALL-2005 protoco1. Methods    Eighty-eight patients with ALL under 3 years of age were retrospectively studied ,who were newly diagnosed by morphologic and immunologic genetics and molecular biology (MICM), and were enrolled in the ALL-2005 treatment protocol from May 2005 to April 2009 in Shanghai Children’s Medical Center SCMC. Results    Data were retrospectively collected since 2005 using the pediatric oncology networked database (POND). And altogether 88 children under 3 years of age with ALL received treatment in SCMC in the following four years. Among the 88 cases of ALL, 61 (69.32%) were males and  27 (30.68%) were females. The median age at diagnosis was 2.08 years (0~2.92 years). Eighy-four were B lineage-ALL and 4 were T lineage-ALL. Thirty-six (40.91%) children were classified as low-risk, 50 (56.82%) were standard-risk and 2 (2.27%) were high-risk. Eighty-one(92.05%) children completely remitted in induction while 17 (19.32%) children relapsed. After a median follow-up of 33 months (1.0~68.2 months), the estimated 5-year event free survival (EFS)rate and overall survival rate were 69.51% and 82.95%, respectively. Conclusion    The estimated 4-year EFS of ALL in infants has been close to 70%; the therapeutic effectiveness of the ALL-2005 protocol for ALL in infants is satisfactory.

    Clinical analysis and follow-up studies of primary IgM nephropathy in 49 children.
    GUO Qin,WU Yu-bin,ZHAO Cheng-guang,Du Yue.
    2013, 28(4): 289-291. 
    Abstract ( )  

    Abstract:Objective    To investigate the clinical characters,pathological characters,treatment and prognosis of primary IgM nephropathy in children for reference. Methods    The data of 49 cases of children with primary IgM nephropathy were reviewed from Jun.2001 to Oct.2009 Department of Nephrology,in Shengjing Hospital Affiliated to China Medical University.The clinical presentation,pathological features , curative effects and prognosis were analyzed. Results    (1) clinical  types  include  asymptomatic proteinuria(1/49), gross hematuria with proteinuria(1/49),primary nephrotic syndrome(47/49). (2)IgM was seen as granular deposition in the mesangium under light-immunofluorescence. 7 cases were ball wall thickening or adhesion layer,8 cases were focal tubular atrophy, 1 case had cellular fibrous crescents,6 caess had focal segmental glomerulosclerosis. (3)Other immunosuppressants together with follow-up observation was applied in that conventional treatment unsatisfactly.Among 44 cases of follow-up observation ,35 cases after complete remission had not yet relapsed. Conclusion    Primary IgM nephropathy should be seen as an independent entity,the main clinical type is primary nephrotic syndrome,refractory nephritic disease is the most common, moderate mesangial cell proliferation is more common histological type,new immunosuppressive agents are expected to gain the better results,long-term prognosis needs further follow-up study.