Fifteen pediatric cases of suspected Japanese encephalitis (JE) were reported in Beijing Children's Hospital during the late summer of 2013. The clinical manifestations in most cases included high fever, seizures, ...Fifteen pediatric cases of suspected Japanese encephalitis (JE) were reported in Beijing Children's Hospital during the late summer of 2013. The clinical manifestations in most cases included high fever, seizures, and abnormal magnetic resonance imaging findings. Twelve of 15 cases were laboratory-confirmed as JE cases by pathogen identification. Epidemiological investigations showed that five of the 12 laboratory-confirmed patients had an incomplete JE vaccination history. Follow-up investigations after discharge indicated that seven laboratory-confirmed JE patients without JE vaccinations had relatively poor prognoses, with an average Modified Rankin Scale (MRS) score of 2.6 when compared with the other five laboratory-confirmed, JE-vaccinated patients with an average MRS score of 0.5. The observation of pediatric JE cases among those with a history of JE vaccination warrants further attention.展开更多
BACKGROUND The etiology of Henoch-Schönlein purpura(HSP)with intussusception remains undefined.AIM To investigate the risk factors for intussusception in children with HSP and gastrointestinal(GI)involvement.METH...BACKGROUND The etiology of Henoch-Schönlein purpura(HSP)with intussusception remains undefined.AIM To investigate the risk factors for intussusception in children with HSP and gastrointestinal(GI)involvement.METHODS Sixty children with HSP and concomitant intussusception admitted to the Beijing Children’s Hospital of Capital Medical University between January 2006 and December 2018 were enrolled in this study.One hundred pediatric patients with HSP and GI involvement but without intussusception,admitted to the same hospital during the same period,were randomly selected as a control group.The baseline clinical characteristics of all patients,including sex,age of onset,duration of disease,clinical manifestations,laboratory test results,and treatments provided,were assessed.Univariate and multiple logistic regression analyses were performed to identify possible risk factors.RESULTS The 60 children in the intussusception group comprised 27 girls(45%)and 33 boys(55%)and the 100 children in the non-intussusception group comprised 62 girls(62%)and 38 boys(38%).The median age of all patients were 6 years and 5 mo.Univariate and multiple regression analyses revealed age at onset,not receiving glucocorticoid therapy within 72 h of emergence of GI symptoms,hematochezia,and D-dimer levels as independent risk factors for intussusception in children with HSP(P<0.05).CONCLUSION The four independent risk factors for intussusception in pediatric HSP with GI involvement would be a reference for early prevention and treatment of this potentially fatal disease.展开更多
AIM:To assess the application of multiple planar volume reconstruction(MPVR) and three-dimensional (3D) transparency lung volume rendering(TL-VR) with 64-row multidetector-row computed tomography (MDCT) in neonates wi...AIM:To assess the application of multiple planar volume reconstruction(MPVR) and three-dimensional (3D) transparency lung volume rendering(TL-VR) with 64-row multidetector-row computed tomography (MDCT) in neonates with congenital esophageal atresia (EA) and distal tracheoesophageal fistula(TEF).METHODS:Twenty neonates(17 boys,3 girls) with EA and distal TEF at a mean age of 4.6 d(range 1-16 d) were enrolled in this study.A helical scan of 64-row MDCT was performed at the 64 mm×0.625 mm collimation.EA and TEF were reconstructed with MPVR and TL-VR,respectively.Initial diagnosis of EA was made by chest radiography showing the inserted catheter in the proximal blind-ended esophageal pouch.Manifestations of MDCT images were compared with the findings at surgery.RESULTS:MDCT showed the proximal and distal esophageal pouches in 20 cases.No significant difference was observed in gaps between the proximal and distal esophageal pouches detected by MPVR and TLVR.The lengths of gaps between the proximal and distal esophageal pouches detected by MPVR and TL-VR correlated well with the findings at surgery(R=0.87,P<0.001).The images of MPVR revealed the orifice of TEF in 13 cases,while TL-VR images showed the orifice of TEF in 4 cases.CONCLUSION:EA and distal TEF can be reconstructed using MPVR and TL-VR of 64-row MDCT,which is a noninvasive technique to demonstrate the distal esophageal pouches and inter-pouch distance in neonates with EA and distal TEF.展开更多
Background Prompt diagnosis of Mycobacterium tuberculosis (MTB) infection is an essential step in tuberculosis control and elimination. However, it is often difficult to accurately diagnose pediatric tuberculosis ...Background Prompt diagnosis of Mycobacterium tuberculosis (MTB) infection is an essential step in tuberculosis control and elimination. However, it is often difficult to accurately diagnose pediatric tuberculosis (TB). The tuberculin test (TST) may have a low specificity because of cross-reactivity with antigens present in Mycobacterium bovis bacillus Calmette-Guerin (BCG) and other mycobacteria, especially in China with a predominantly BCG-vaccinated population.Early-secreted antigenic target 6-kDa protein (ESAT-6) and culture filtrate protein 10 (CFP-10), stand out as suitable antigens that induce an interferon-gamma (IFN-γ) secreting, T-cell-mediated immune response to infection. While,considered the higher costs and complexity of the IFN-γ release assay (TSPOT), we aimed to evaluate the TSPOT and TST test in the clinical diagnosis of pediatric tuberculosis and to establish a diagnostic process suitable for China.Methods The sensitivity and specificity of the assay were evaluated in total seventy four children with active tuberculosis and fifty one nontuberculous children with other disease, and then the results were compared with TST.Logistic regression models were used to identify variables that were associated with positive results for each assay. The independent variables included sex, age, birth place, vaccination history, close contract with an active TB patient.Results The sensitivity of TSPOT was higher than TST in active TB children with or without BCG vaccination, as well as in children with culture-confirmed TB. But the difference was not significant statistically. Combining results of the TSPOT and TST improved the sensitivity to 94.6%. Agreement of the TST and TSPOT was low (77.0%, k=0.203) in active TB patients. The difference in specificity between TSPOT and TST test was statistically significant (94.1% vs.70.6%, P=0.006). Specificity of the two tests in patients without prior BCG vaccination history was similar (80.0% vs.60.0%). The concordance between the two tests results in BCG vaccinated subjects was low (71.7%, k=0.063). For TSPOT, none of the included risk factors was significantly associated with positive results. For TST, BCG vaccination (OR:1.78; 95% CI: 1.30-2.00) was significantly associated with positive results.Conclusions Although IFN-γ release assay had relatively high sensitivity and specificity, we also should consider the higher costs and complexity of this test. Therefore, TSPOT could be used as the complementary tool of TST in circumstances when a suspected patient with negative TST results, or to exclude a positive TST result caused by BCG vaccination.展开更多
Background The basic helix-loop-helix transcription factor HAND2 plays an essential role in cardiac morphogenesis. However, the prevalence of HAND2 mutations in congenial heart disease (CHD) and the correlation betw...Background The basic helix-loop-helix transcription factor HAND2 plays an essential role in cardiac morphogenesis. However, the prevalence of HAND2 mutations in congenial heart disease (CHD) and the correlation between the HAND2 genotype and CHD phenotype have not been studied extensively. Methods We amplified the exons and the flanking intron sequences of the HAND2gene in 131 patients diagnosed with congenital defects of the right ventricle, outflow tract, aortic artery or cardiac cushion and confirmed the mutations by sequencing. Results Seven mutations including three missense mutations (P11R, S36N and V83L), one isonymous mutation (H14H) and three mutations in untranslated region (241A〉G, 604C〉T and 3237T〉A) were identified in 12 out of the 131 patients. Both nonisonymous mutations are located in the transcriptional activation domain on the N-terminus. Only one mutation (S36N) was identified in 250 normal healthy controls. The distribution of 3637T〉A is the unique one which was different between the 2 groups. Conclusions HAND2 may be a potential candidate gene of stenosis of the right ventricle, outflow tract. Further study of those with a family history of HAND2 mutations will help convincingly relate their genotype to the pathogenesis of CHD.展开更多
Objective: To evaluate the clinical efficacy and safety of Chinese drugs for the treatment of children's infectious mononucleosis (CIM). Methods: Sixty CIM patients were assigned into the treated group and the co...Objective: To evaluate the clinical efficacy and safety of Chinese drugs for the treatment of children's infectious mononucleosis (CIM). Methods: Sixty CIM patients were assigned into the treated group and the control group, patients in the treated group were administered with Chinese herbal decoction, and those in the control group were treated with intravenous dripping of ganciclovir 10 mg/kg per day, for a treatment course of 14 days. Results: The total effective rate was 96.0% in the treated group and 97.1% in the control group, showing insignificant difference between groups. The efficacy in the treated group was superior to that in the control group on the fever clearance time (3.0 ± 1.5 days vs 4.9 ± 3.9 days ) and the disappearance time of cervical lymph node swelling (0.8 ± 1.0 score vs 1.5 ± 1.2 score), showing statistical significance (all P〈0.05). T-cell subsets were markedly improved in both groups after treatment. Adverse reaction occurred in four cases of the control group. Conclusion: Using Chinese herbs for clearing heat, removing toxin, activating blood circulation, and dissolving stasis is effective and safe for the treatment of CIM. It can effectively improve the clinical symptoms and shows a certain effect on immune regulation.展开更多
Background Streptococcus pneumoniae (S.pneumoniae) is a major causative agent of severe infections,including sepsis,pneumonia,meningitis,and otitis media,and has become a major public health concern.We report the pn...Background Streptococcus pneumoniae (S.pneumoniae) is a major causative agent of severe infections,including sepsis,pneumonia,meningitis,and otitis media,and has become a major public health concern.We report the pneumococcal serotype and sequence type (ST) distribution,and antimicrobial resistance of 39 S.pneumoniae strains from seven hospitals in China.Methods Blood/cerebrospinal fluid (CSF) and sputum isolates from patients were analyzed to determine S.pneumoniae serotypes by polymerase chain reaction (PCR) and the Neufeld Quellung reaction,the multilocus sequence types (MLST) by PCR and sequencing,and susceptibility to antimicrobial agents by the VITEK Gram Positive Susceptibility Card.Results A total of 39 isolates were collected including 21 blood/CSF and 18 sputum isolates.Conventional serotyping by the Quellung reaction required 749 reactions.In contrast,PCR based typing needed only 106 PCR reactions.The most frequent serotypes from the blood/CSF isolates were 14 (38.1%),19A (14.3%),23F (9.5%),and 18C (9.5%).In the sputum isolates the most frequent serotypes were 19F (33.3%),23F (16.7%),19A (11.1%),and 3 (11.1%).The incidence of penicillin resistance in the blood/CSF and sputum isolates was 66.7% and 55.6%,respectively.Statistical analysis showed that patients ≤5 years old had a higher resistance to penicillin when they compared with the patients ≥65 years old (P=0.011).Serotypes 14,19A and 19F were significantly associated with penicillin resistance (P 〈0.001).ST320,ST271,and ST876 isolates showed high resistant rates to several antibiotics including penicillin (P=0.006).All of the isolates of serotype 19A were resistant to both penicillin and erythromycin,and they were all multi-drug resistant (MDR) isolates.Conclusions The specificity and sensitivity of multiplexPCR are good,and this method represents a substantial savings of time and money,and can be widely used in the laboratory and clinical practice.Data from this research showed an extremely high prevalence of penicillin resistance and an increasing prevalence of multi-drug resistant (MDR) rate in S.pneumoniae.A distinctive emergence of serotype 19A was observed which was also associated with the increasing prevalence of antimicrobial resistance.Therefore,nationwide surveillance of pneumococcal resistance and serotypes is strongly warranted.展开更多
Background Acinetobacter baumanii (A. baumanii ) remains an important microbial pathogen resulting in nosocomialacquired infections with significant morbidity and mortality. The mechanism by which nosocomial bacteri...Background Acinetobacter baumanii (A. baumanii ) remains an important microbial pathogen resulting in nosocomialacquired infections with significant morbidity and mortality. The mechanism by which nosocomial bacteria, like A. baumanii, attain multidrug resistance to antibiotics is of considerable interest. The aim in this study was to investigate the spread status of antibiotic resistance genes, such as multiple 13-1actamase genes and aminoglycoside-modifying enzyme genes, from A. baumanii strains isolated from patients with lower respiratory tract infections (LRTIs). Methods Two thousand six hundred and ninety-eight sputum or the bronchoalveolar lavage samples from inpatients with LRTIs were collected in 21 hospitals in the mainland of China from November 2007 to February 2009. All samples were routinely inoculated. The isolated bacterial strains and their susceptibility were analyzed via VITEK-2 expert system. Several kinds of antibiotic resistant genes were further differentiated via polymerase chain reaction and sequencing methods. Results Totally, 39 A. baumanii strains were isolated from 2698 sputum or bronchoalveolar lavage samples. There was not only a high resistant rate of the isolated A. baumanfi strains to ampicillin and first- and second-generation cephalosporins (94.87%, 100% and 97.44%, respectively), but also to the third-generation cephalosporins (ceftriaxone at 92.31%, ceftazidine at 51.28%) and imipenem (43.59%) as well. The lowest antibiotic resistance rate of 20.51% was found to amikacin. The OXA-23 gene was identified in 17 strains of A. baumanii, and the AmpC gene in 23 strains. The TEM-1 gene was carried in 15 strains. PER-1 and SHV-2 genes were detected in two different strains. Aminoglycoside-modifying enzyme gene aac-3-1a was found in 23 strains, and the aac-6"lb gene in 19 strains, aac-3-1a and aac-6"lb genes hibernated in three A. baumanfi strains that showed no drug-resistant phenotype. Conclusions A. baumanii can carry multiple drug-resistant genes at the same time and result in multi-drug resistance. Aminoglycoside-modifying enzyme genes could be hibernating in aminoglycoside sensitive strains without expressing their phenotype.展开更多
Clinical categories of Langerhans cell histiocytosis (LCH) include single and multi-system disease. Pulmonary LCH is rare, which is an unusual interstitial lung disease with the characteristics of monoclonal prolife...Clinical categories of Langerhans cell histiocytosis (LCH) include single and multi-system disease. Pulmonary LCH is rare, which is an unusual interstitial lung disease with the characteristics of monoclonal proliferation and infiltration of Langerhans' cells to organs. We report our experience of a rare LCH case of multiple organs such as pulmonary and liver as the main clinical manifestation. The patient was treated with chemotherapy which included prednisone, vinblastine, methotrexate and 6-mercaptopurine for 52 weeks and follow up all along. The patient has a favorable clinical outcome.展开更多
Background:A specific feature of the autoimmune response in rheumatoid arthritis(RA)is the presence of anti-citrullinated protein antibodies(ACPAs)in patient sera.These antibodies can appear several years before disea...Background:A specific feature of the autoimmune response in rheumatoid arthritis(RA)is the presence of anti-citrullinated protein antibodies(ACPAs)in patient sera.These antibodies can appear several years before disease onset and are involved in the development of RA.Objective:We performed proteomic analysis by mass spectroscopy to identify novel citrullinated antigens and autoantibodies in RA patients.Methods:Polypeptides isolated from the sera of RA patients were identified by Orbitrap high-precision mass spectrometry and then citrulline-containing proteins were selected.The levels of ACPAs against these newly identified citrullinated autoantigens in sera of 100 RA patients and 50 healthy controls were determined by enzyme-linked immunosorbent assays.Results:A total of 135 proteins were identified in RA patients and the protein profile included 11 citrulline-containing antigens.Three of the 11 citrullinated proteins had been reported in previous studies.ACPAs against the novel citrullinated epitopes from these proteins were increased in sera from the RA patients compared with those from healthy controls.Autoantibodies against one of the citrullinated antigens,lipopolysaccharide-binding protein(LBP),was significantly increased in RA patients and associated with disease activities.The titer of anti-citrullinated LBP antibodies(anti-cLBP)was closely related to the infection incidence in RA patients.Conclusion:Serum protein analysis by high-precision proteomic technology is a feasible method to identify novel citrullinated epitopes in RA patients.Anti-cLBP antibodies are associated with disease severity and infection in RA patients.展开更多
To evaluate and compare the outcomes and complications of three different surgical techniques for treating primary proximal hypospadias with ventral curvature(VC)≥30°,we retrospectively reviewed the medical reco...To evaluate and compare the outcomes and complications of three different surgical techniques for treating primary proximal hypospadias with ventral curvature(VC)≥30°,we retrospectively reviewed the medical records of patients who underwent primary repair of proximal hypospadias with vC≥30°after degloving at Beijing Children's Hospital Affiliated to Capital Medical University(Beijing,China)from January 2019 to January 2021.A total of 152 patients were divided into three groups:transverse preputial island flap(TPIF)combined with Duplay,modified Koyanagi,and staged TPIF,which were performed on 55,16,and 81 patients,respectively.A total of 39(25.7%)patients had complications.Complications rates were similar for the TPIF combined with the Duplay group(40.0%)and modified Koyanagi group(50.0%)but lower for the staged TPIF group(11.1%;P<0.01).The incidence of urethrocutaneous fistulas was significantly higher in TPIF combined with Duplay group(21.8%)compared to staged TPIF group(4.9%;P=0.01).In univariate analysis,the length of the urethral defect was the single factor that could predict complications;the cutoff was 4.55 cm.More patients in the long urethral defect group than in the short one had complications(34.1%vs 15.7%,P=O.O1).These results indicate that staged TPIF produced a better outcome,whereas more patients in the TPIF combined with Duplay group presented with two or more complications.展开更多
There is still debate regarding the optimal surgical approach for proximal hypospadias. This retrospective study aims to evaluate the long-term outcomes using transverse preputial island flap urethroplasty. A total of...There is still debate regarding the optimal surgical approach for proximal hypospadias. This retrospective study aims to evaluate the long-term outcomes using transverse preputial island flap urethroplasty. A total of 320 patients were included, with a mean follow-up of 40.2 months (range: 1–156 months). Complications were encountered in 125 patients (39.1%), including fistulas in 53 (16.6%), urethral strictures in 31 (9.7%), and diverticula in 41 (12.8%). The mean timing of presentation with a complication was 15.8 months (median: 1.7, range: 1–145), of which 79.2% were early complications and 20.8% were late complications. In all, 20.8% of the patients with complications presented after ≥1 year, and 12.8% presented after ≥5 years. Univariate analysis revealed that age at the time of surgery, flap length, and location of the urethral meatus were not correlated with complications. A stricture was present in 31.7%(13/41) of those with diverticula (P < 0.001), while late urethral diverticula were accompanied by urethral strictures in 11.1%(1/9) of cases (P = 0.213). These results indicate that transverse preputial island flap urethroplasty still has a high incidence of complications, even when performed by highly experienced physicians. Most complications of hypospadias are diagnosed within 1 year postoperatively, while fistulas and urinary strictures generally occur within 2 months and diverticula tend to be present by 1 year.展开更多
OBJECTIVE:To research the curative effect of Chinese herbs for clearing away heat,promoting diuresis,nourishing the kidney,and consolidating essence in children with Henoch-Schonlein purpura nephritis(HSPN)with intern...OBJECTIVE:To research the curative effect of Chinese herbs for clearing away heat,promoting diuresis,nourishing the kidney,and consolidating essence in children with Henoch-Schonlein purpura nephritis(HSPN)with internal accumulation of damp-toxin using randomized controlled observations on large samples.To seek the mechanism of the therapy and its scope of indications.METHODS:Overall,186 children with HSPN were randomly divided into two groups:treatment group(n=126)treated with Chinese herbs for clearing heat and promoting diuresis and a control group(n=60)treated with Western Medicine.The treatment was carried out for three courses of 4weeks each.We recorded changes in patient urine routines,24 h urinary protein,blood-coagulating series,immunoglobulin and T-cell subgroups,and improvements in main symptoms.We evaluated the alleviation of clinical symptoms and the improvement of proteinuria,hematuria,and other lab-oratory test results.Finally,we analyzed the patient population suitable for this therapy according to the relationship between the grouping of patient body weight and curative effect.RESULTS:Damp-heat syndrome improved in the treatment group,with a significant difference in total effective rate after a 4-week treatment(χ2=13.5220,P=0.0002)and in curative rate after a12-week treatment(χ2=6.3410,P=0.0118),compared to those in the control group.The curative effect in the treatment group was greater than that in the control group but there was no statistical difference between the two groups.The curative effect after a 4-week treatment of patients in the treatment group weighing 30 kg or less based on Traditional Chinese Medicine(TCM)signs and urinary protein was significantly greater than that in the control group.However,there was no statistical difference in the curative effect on urinary red cells and various indexes after a 12-week treatment between the two groups.CONCLUSION:Therapy for clearing away heat,promoting diuresis,nourishing the kidney,and consolidating essence using TCM is effective in children with HSPN from internal accumulation of damp-toxin.The therapy is especially suitable for patients weighing 30 kg or less.The curative effect may be related to the improvement of immune function and blood-coagulation.展开更多
Background Increasing numbers of pertussis cases have been reported in recent years.The reported cases from Shenzhen Children's Hospital were close to one tenth of all cases in China.The epidemiology of antigenic ...Background Increasing numbers of pertussis cases have been reported in recent years.The reported cases from Shenzhen Children's Hospital were close to one tenth of all cases in China.The epidemiology of antigenic genotype and antibiotic resistance of circulating strains in children have been unknown in Shenzhen,southern China.The aim of this study was to describe the clinical features and explore the genotypes and antimicrobial susceptibility of circulating Bordetella pertussis among children in Shenzhen.Methods Data of hospitalized children with pertussis in Shenzhen Children's Hospital from August 2015 to April 2017 were collected.The genetic variability of isolates was investigated and Etest was performed for phenotypic susceptibility to erythromycin,azithromycin,clarithromycin,clindamycin,and trimethoprim/sulfamethoxazole.Results 469 children with pertussis confirmed by real-time quantitative polymerase chain reaction were hospitalized and strains were isolated from 105 patients.White blood cell count≥20×10^9/L and lymphocyte proportion≥60%were observed in 39.29%of infants younger than 3 months.The two predominant profiles of virulence-associated allelic genes were ptxA1/ptxC1/ptxPl/prnl(48.6%)and ptxA1/ptxC2/ptxP3/prn2(44.8%).Among the isolates,48.6%(51/105)were found resistant to macrolides.Conclusions These findings indicate that leukocytosis is not a sensitive indicator of pertussis.Isolates with the gene profile ptxP3/prn2 were highly circulating in Shenzhen and less resistant to macrolides,different from patterns observed in other parts of China.展开更多
Background Intake of melamine can cause renal and ureteral lithiasis in infants and children. The present study aimed to understand the value of ultrasonography in the diagnosis of renal and ureteral lithiasis in infa...Background Intake of melamine can cause renal and ureteral lithiasis in infants and children. The present study aimed to understand the value of ultrasonography in the diagnosis of renal and ureteral lithiasis in infants and young children caused by melamine, and the characteristics of ultrasonograms of melamine-associated calculi. Methods Ultrasonographic examination on the urinary system was performed for 28 332 children who ingested milk powder that was possibly tainted with melamine; 395 of the children were diagnosed by ultrasonography as having urinary calculus, and 231 cases had lump-like calculi and 164 cases had sand gravel-like calculi. The features of the calculi, the sites of obstruction and the status of hydronephrosis and hydroureterosis were analyzed. Ultrasonographic reexamination was performed for 116 patients and the findings were compared with those of the first ultrasonography, and the short-term expulsion of the calculi was evaluated. Results Most of the 395 patients with urinary lithiasis, except for those who developed renal failure, had no symptoms. The whole profile of the calculi could be seen in most of the cases, because the echogenicity of the calculi with no sharp or absent acoustical shadowing, was weaker as compared with those from calcium-containing calculi. Comet tail sign could be seen behind the echogenicity of single gravel calculus. The rate of diagnostic consistency of ureteral lithiasis between the ultrasound and the results of clinical observation (including stones expelled spontaneously or after cystoscopic intervention) in 51 cases for 76 ureters was 100%. Percutaneous renal biopsy was performed for one case, and histopathological examination showed flocculent, fine strip-like, ellipse and circular deeply stained sand gravel-like material in the renal tubules, and the circular calculi were found to be attached to the walls of the tubules. Chemical analysis of the calculi expelled from 12 cases showed that the main contents of the calculi were uric acid and melamine. Short-term ultrasound reexamination in 116 patients showed that gravel-like calculi disappeared in 80.4% of the cases; in 26 non-hospitalized cases who had lump-like calculi without hydronephrosis or hydroureterosis, none of the lump-like calculi were expelled. Conclusions The ultrasonograpic findings of urinary calculi in children caused by ingestion of melamine-tainted milk powder have a certain features as compared to the calculi containing calcium. Careful ultrasound examination can avoid missed diagnoses of ureteral calculi. Most of the gravel-like calculi can be expelled within a short period of term, while lump-like calculi can hardly be expelled. Ultrasonography is an accurate and reliable method of diagnosis of urinary calculus caused by melamine intake in children and it can be used as diagnostic method of choice although abdominal X-ray plain film can also visualize some of larger calculi.展开更多
Williams-Beuren syndrome (WBS) is a hereditary disease involving multiple systems due to microdeletion of chromosome 7q11.23. The deleted region in most patients is approximately 1.55 Mb, including 26-28 genes. The ...Williams-Beuren syndrome (WBS) is a hereditary disease involving multiple systems due to microdeletion of chromosome 7q11.23. The deleted region in most patients is approximately 1.55 Mb, including 26-28 genes. The incidence of WBS in liveborn children is roughly 1/7 500-1/25 000.1展开更多
Background In 2008, a sharp increase of the number of children diagnosed with urinary calculi was observed in China, 9433 children were diagnosed as having melamine-induced urinary calculi at outpatient clinic in Beij...Background In 2008, a sharp increase of the number of children diagnosed with urinary calculi was observed in China, 9433 children were diagnosed as having melamine-induced urinary calculi at outpatient clinic in Beijing Children's Hospital. This study examined the therapeutic efficacy of potassium sodium hydrogen citrate (PSHC) used to treat melamine-induced urinary stones in Chinese children who consumed melamine-containing infant formula. Methods Seventy-two infants and children (average age (18.2±7.7) months) who were diagnosed with urinary calculi were icuidomly divided into three treatment groups using the SAS Plan program. Group 1 was given a low dose (1 g/d) of PSHC, group 2 was given high dose of PSHC (2 g/d) and group 3 was given no PSHC (control group). The dose of drug was adjusted according to the baseline urinary pH. This study analyzed the influence of the dose of PSHC, the age of patients, stone size and position, and urinary pH on the level of efficacy of PSHC (cured, effectively treated or not cured).Results After 1-6 months of therapy, 19 patients from group 1, five patients from group 2 and six patients from group 3 were cured. Five patients from group 1, five patients from group 2 and four patients from group 3 were effectively treated. There were significant differences in therapeutic efficacy between the two treatment doses after 3 and 6 months as measured by the increase in the successful expulsion rate and time of melamine-induced urinary calculi. After 6 months the mean time of expulsion of urinary calculi in groups 1 and 2 was significantly shorter than in the control group. Conclusions PSHC can significantly increase the successful expulsion rate and time of melamine-induced urinary calculi. The therapeutic efficacy is affected by PSHC dose, treatment duration, calculi position, and urinary pH. There is no relationship between the therapeutic efficacy and the stone size or patient age.展开更多
Background Infants in some areas of China developed urinary lithiasis after being fed with powdered milk that was tainted with melamine in 2008 and very small proportion of the infants developed acute renal failure ca...Background Infants in some areas of China developed urinary lithiasis after being fed with powdered milk that was tainted with melamine in 2008 and very small proportion of the infants developed acute renal failure caused by urinary tract calculus obstruction. The aim of this article was to summarize clinical characteristics, diagnosis and treatment of infants with urinary calculus and acute renal failure developed after being fed with melamine tainted formula milk. Methods Data of infant patients with urinary calculus and acute renal failure due to melamine tainted formula milk admitted to the Beijing Children's Hospital Affiliated to the Capital Medical University and the Xuzhou Children's Hospital in 2008 were used to analyze the epidemiological characteristics, clinical manifestations, imaging features as well as effects of 4 types of therapies. Results All the 34 infants with urinary calculus were complicated with acute renal failure, their blood urea nitrogen (BUN) was (24.1±8.2) mmol/L and creatinine (Cr) was (384.2±201.2) umol/L. The chemical analysis on the urinary calculus sampled from 15 of the infants showed that the calculus contained melamine and acidum uricum. The time needed for the four types of therapies for returning Cr to normal was (3.5±1.9) days for cystoscopy group, (2.7±1.1) days for lithotomy group, (3.8±2.3) days for dialysis group, and (2.7±1.6) days for medical treatment group, which had no statistically significant difference (P=-0.508). Renal failure of all the 34 infants was relieved within 1 to 7 days, averaging (3.00±1.78) days. Conclusions Melamine tainted formula milk may cause urinary calculus and obstructive acute renal failure. It is suggested that firstly the patients with urinary calculus complicated with acute renal failure should be treated with dialysis or medication to correct electrolyte disturbance, in particular hyperkalemia, and then relieve the obstruction with available medical and surgical methods as soon as possible. It was observed that the short-term prognosis was satisfactory.展开更多
Asymmetric superior oblique palsy(SOP)is a complicated,non-comitant type of strabismus.Patients usually present with small-angle hypertropia in the affected eyes,accompanied by inferior oblique muscle overaction(IOOA)...Asymmetric superior oblique palsy(SOP)is a complicated,non-comitant type of strabismus.Patients usually present with small-angle hypertropia in the affected eyes,accompanied by inferior oblique muscle overaction(IOOA).[1]Compensatory head posture and asymmetric development of the face are typically observed in congenital SOP,while diplopia is more common in an acquired SOP.[2]Prism glasses can improve diplopia and vertical deviation in the primary position,but they are not so successful at correcting non-comitant paralytic strabismus.In addition,vertical strabismus and compensatory head posture will return after the prism glasses are removed,wearing prism glasses will lower the vision of children prominently.Surgery like an inferior oblique muscle(IO)weakening procedure is often required eventually in those patients.However,conventional procedures for IO weakening may cause overcorrection and/or secondary A pattern in patients who have mild to moderate IOOA together with small-angle asymmetric SOP,posing a risk to its safety.[3]In the study by Caldeira,[4]21%of the patients with mild to moderate IOOA developed A pattern following the surgery.Therefore,it remains a clinical challenge to select the most appropriate type of surgery for the treatment of small-angle asymmetric SOP.Inferior oblique muscle belly transposition(IOMBT),[5]a new IO weakening procedure introduced to our practice lately,has shown positive results in treating small-angle asymmetric SOP as well as a lower risk of complications such as overcorrection and secondary A pattern.In this study,we evaluated the efficacy and safety of the IOMBT procedure for the treatment of small-angle asymmetric SOP.展开更多
Kasabach-Merritt phenomenon(KMP)is a rare disease that is characterized by severe thrombocytopenia and consumptive coagulation dysfunction caused by kaposiform hemangioendothelioma or tufted hemangioma.This condition ...Kasabach-Merritt phenomenon(KMP)is a rare disease that is characterized by severe thrombocytopenia and consumptive coagulation dysfunction caused by kaposiform hemangioendothelioma or tufted hemangioma.This condition primarily occurs in infants and young children,usually with acute onset and rapid progression.This review article introduced standardized recommendations for the pathogenesis,clinical manifestation,diagnostic methods and treatment process of KMP in China,which can be used as a reference for clinical practice.展开更多
基金grants from the National Key Research and Development Program(2016YFD0500401)a clinical technological innovation project supervised by the Beijing Hospital Authority(XMLX201401)+3 种基金a Development Grant of the State Key Laboratory of Infectious Disease Prevention and Control(2015SKLID505,2014SKLID03)Ministry of Science and Technology of the People’s Republic of China(No.2013ZX10004202,No.2013ZX10004101,No.2012ZX10004215)the National Natural Science Foundation of China(81290342 and 81501757)the Special National Project on Research and Development of Key Biosafety Technologies(2016YFC1201900)
文摘Fifteen pediatric cases of suspected Japanese encephalitis (JE) were reported in Beijing Children's Hospital during the late summer of 2013. The clinical manifestations in most cases included high fever, seizures, and abnormal magnetic resonance imaging findings. Twelve of 15 cases were laboratory-confirmed as JE cases by pathogen identification. Epidemiological investigations showed that five of the 12 laboratory-confirmed patients had an incomplete JE vaccination history. Follow-up investigations after discharge indicated that seven laboratory-confirmed JE patients without JE vaccinations had relatively poor prognoses, with an average Modified Rankin Scale (MRS) score of 2.6 when compared with the other five laboratory-confirmed, JE-vaccinated patients with an average MRS score of 0.5. The observation of pediatric JE cases among those with a history of JE vaccination warrants further attention.
基金Supported by the Beijing Traditional Chinese Medicine Science and Technology Development Fund,No.JJ2018-45the Beijing Municipal Natural Science Foundation,No.7172076 and No.7212169.
文摘BACKGROUND The etiology of Henoch-Schönlein purpura(HSP)with intussusception remains undefined.AIM To investigate the risk factors for intussusception in children with HSP and gastrointestinal(GI)involvement.METHODS Sixty children with HSP and concomitant intussusception admitted to the Beijing Children’s Hospital of Capital Medical University between January 2006 and December 2018 were enrolled in this study.One hundred pediatric patients with HSP and GI involvement but without intussusception,admitted to the same hospital during the same period,were randomly selected as a control group.The baseline clinical characteristics of all patients,including sex,age of onset,duration of disease,clinical manifestations,laboratory test results,and treatments provided,were assessed.Univariate and multiple logistic regression analyses were performed to identify possible risk factors.RESULTS The 60 children in the intussusception group comprised 27 girls(45%)and 33 boys(55%)and the 100 children in the non-intussusception group comprised 62 girls(62%)and 38 boys(38%).The median age of all patients were 6 years and 5 mo.Univariate and multiple regression analyses revealed age at onset,not receiving glucocorticoid therapy within 72 h of emergence of GI symptoms,hematochezia,and D-dimer levels as independent risk factors for intussusception in children with HSP(P<0.05).CONCLUSION The four independent risk factors for intussusception in pediatric HSP with GI involvement would be a reference for early prevention and treatment of this potentially fatal disease.
文摘AIM:To assess the application of multiple planar volume reconstruction(MPVR) and three-dimensional (3D) transparency lung volume rendering(TL-VR) with 64-row multidetector-row computed tomography (MDCT) in neonates with congenital esophageal atresia (EA) and distal tracheoesophageal fistula(TEF).METHODS:Twenty neonates(17 boys,3 girls) with EA and distal TEF at a mean age of 4.6 d(range 1-16 d) were enrolled in this study.A helical scan of 64-row MDCT was performed at the 64 mm×0.625 mm collimation.EA and TEF were reconstructed with MPVR and TL-VR,respectively.Initial diagnosis of EA was made by chest radiography showing the inserted catheter in the proximal blind-ended esophageal pouch.Manifestations of MDCT images were compared with the findings at surgery.RESULTS:MDCT showed the proximal and distal esophageal pouches in 20 cases.No significant difference was observed in gaps between the proximal and distal esophageal pouches detected by MPVR and TLVR.The lengths of gaps between the proximal and distal esophageal pouches detected by MPVR and TL-VR correlated well with the findings at surgery(R=0.87,P<0.001).The images of MPVR revealed the orifice of TEF in 13 cases,while TL-VR images showed the orifice of TEF in 4 cases.CONCLUSION:EA and distal TEF can be reconstructed using MPVR and TL-VR of 64-row MDCT,which is a noninvasive technique to demonstrate the distal esophageal pouches and inter-pouch distance in neonates with EA and distal TEF.
基金This study was supported by the grants from the National Natural Science Foundation of China (No. 30872788) and from the Beijing Municipal Science and Technology Commission (No.Z09050700940903).
文摘Background Prompt diagnosis of Mycobacterium tuberculosis (MTB) infection is an essential step in tuberculosis control and elimination. However, it is often difficult to accurately diagnose pediatric tuberculosis (TB). The tuberculin test (TST) may have a low specificity because of cross-reactivity with antigens present in Mycobacterium bovis bacillus Calmette-Guerin (BCG) and other mycobacteria, especially in China with a predominantly BCG-vaccinated population.Early-secreted antigenic target 6-kDa protein (ESAT-6) and culture filtrate protein 10 (CFP-10), stand out as suitable antigens that induce an interferon-gamma (IFN-γ) secreting, T-cell-mediated immune response to infection. While,considered the higher costs and complexity of the IFN-γ release assay (TSPOT), we aimed to evaluate the TSPOT and TST test in the clinical diagnosis of pediatric tuberculosis and to establish a diagnostic process suitable for China.Methods The sensitivity and specificity of the assay were evaluated in total seventy four children with active tuberculosis and fifty one nontuberculous children with other disease, and then the results were compared with TST.Logistic regression models were used to identify variables that were associated with positive results for each assay. The independent variables included sex, age, birth place, vaccination history, close contract with an active TB patient.Results The sensitivity of TSPOT was higher than TST in active TB children with or without BCG vaccination, as well as in children with culture-confirmed TB. But the difference was not significant statistically. Combining results of the TSPOT and TST improved the sensitivity to 94.6%. Agreement of the TST and TSPOT was low (77.0%, k=0.203) in active TB patients. The difference in specificity between TSPOT and TST test was statistically significant (94.1% vs.70.6%, P=0.006). Specificity of the two tests in patients without prior BCG vaccination history was similar (80.0% vs.60.0%). The concordance between the two tests results in BCG vaccinated subjects was low (71.7%, k=0.063). For TSPOT, none of the included risk factors was significantly associated with positive results. For TST, BCG vaccination (OR:1.78; 95% CI: 1.30-2.00) was significantly associated with positive results.Conclusions Although IFN-γ release assay had relatively high sensitivity and specificity, we also should consider the higher costs and complexity of this test. Therefore, TSPOT could be used as the complementary tool of TST in circumstances when a suspected patient with negative TST results, or to exclude a positive TST result caused by BCG vaccination.
基金This work was supported by a grant from the National Natural Science Foundation of China (No. 30672193).Acknowledgements: We are grateful to the patients and their families for the cooperation, to the members of the Paediatric Heart Centre for support and staff of the Central Laboratory of Molecular Biology for the technical assistance. We specially thank Dr. ZHU Xiao-quan for the excellent advice and assistance and Dr. ZHANG Wei-min for helpful patient information.
文摘Background The basic helix-loop-helix transcription factor HAND2 plays an essential role in cardiac morphogenesis. However, the prevalence of HAND2 mutations in congenial heart disease (CHD) and the correlation between the HAND2 genotype and CHD phenotype have not been studied extensively. Methods We amplified the exons and the flanking intron sequences of the HAND2gene in 131 patients diagnosed with congenital defects of the right ventricle, outflow tract, aortic artery or cardiac cushion and confirmed the mutations by sequencing. Results Seven mutations including three missense mutations (P11R, S36N and V83L), one isonymous mutation (H14H) and three mutations in untranslated region (241A〉G, 604C〉T and 3237T〉A) were identified in 12 out of the 131 patients. Both nonisonymous mutations are located in the transcriptional activation domain on the N-terminus. Only one mutation (S36N) was identified in 250 normal healthy controls. The distribution of 3637T〉A is the unique one which was different between the 2 groups. Conclusions HAND2 may be a potential candidate gene of stenosis of the right ventricle, outflow tract. Further study of those with a family history of HAND2 mutations will help convincingly relate their genotype to the pathogenesis of CHD.
基金Supported by the Natural Science Foundation of Beijing Municipal Science and Technology Committee(No. z0005190042511)the Basic-clinical Cooperative Foundation of Capital Medical University(No.2006JL50)
文摘Objective: To evaluate the clinical efficacy and safety of Chinese drugs for the treatment of children's infectious mononucleosis (CIM). Methods: Sixty CIM patients were assigned into the treated group and the control group, patients in the treated group were administered with Chinese herbal decoction, and those in the control group were treated with intravenous dripping of ganciclovir 10 mg/kg per day, for a treatment course of 14 days. Results: The total effective rate was 96.0% in the treated group and 97.1% in the control group, showing insignificant difference between groups. The efficacy in the treated group was superior to that in the control group on the fever clearance time (3.0 ± 1.5 days vs 4.9 ± 3.9 days ) and the disappearance time of cervical lymph node swelling (0.8 ± 1.0 score vs 1.5 ± 1.2 score), showing statistical significance (all P〈0.05). T-cell subsets were markedly improved in both groups after treatment. Adverse reaction occurred in four cases of the control group. Conclusion: Using Chinese herbs for clearing heat, removing toxin, activating blood circulation, and dissolving stasis is effective and safe for the treatment of CIM. It can effectively improve the clinical symptoms and shows a certain effect on immune regulation.
文摘Background Streptococcus pneumoniae (S.pneumoniae) is a major causative agent of severe infections,including sepsis,pneumonia,meningitis,and otitis media,and has become a major public health concern.We report the pneumococcal serotype and sequence type (ST) distribution,and antimicrobial resistance of 39 S.pneumoniae strains from seven hospitals in China.Methods Blood/cerebrospinal fluid (CSF) and sputum isolates from patients were analyzed to determine S.pneumoniae serotypes by polymerase chain reaction (PCR) and the Neufeld Quellung reaction,the multilocus sequence types (MLST) by PCR and sequencing,and susceptibility to antimicrobial agents by the VITEK Gram Positive Susceptibility Card.Results A total of 39 isolates were collected including 21 blood/CSF and 18 sputum isolates.Conventional serotyping by the Quellung reaction required 749 reactions.In contrast,PCR based typing needed only 106 PCR reactions.The most frequent serotypes from the blood/CSF isolates were 14 (38.1%),19A (14.3%),23F (9.5%),and 18C (9.5%).In the sputum isolates the most frequent serotypes were 19F (33.3%),23F (16.7%),19A (11.1%),and 3 (11.1%).The incidence of penicillin resistance in the blood/CSF and sputum isolates was 66.7% and 55.6%,respectively.Statistical analysis showed that patients ≤5 years old had a higher resistance to penicillin when they compared with the patients ≥65 years old (P=0.011).Serotypes 14,19A and 19F were significantly associated with penicillin resistance (P 〈0.001).ST320,ST271,and ST876 isolates showed high resistant rates to several antibiotics including penicillin (P=0.006).All of the isolates of serotype 19A were resistant to both penicillin and erythromycin,and they were all multi-drug resistant (MDR) isolates.Conclusions The specificity and sensitivity of multiplexPCR are good,and this method represents a substantial savings of time and money,and can be widely used in the laboratory and clinical practice.Data from this research showed an extremely high prevalence of penicillin resistance and an increasing prevalence of multi-drug resistant (MDR) rate in S.pneumoniae.A distinctive emergence of serotype 19A was observed which was also associated with the increasing prevalence of antimicrobial resistance.Therefore,nationwide surveillance of pneumococcal resistance and serotypes is strongly warranted.
文摘Background Acinetobacter baumanii (A. baumanii ) remains an important microbial pathogen resulting in nosocomialacquired infections with significant morbidity and mortality. The mechanism by which nosocomial bacteria, like A. baumanii, attain multidrug resistance to antibiotics is of considerable interest. The aim in this study was to investigate the spread status of antibiotic resistance genes, such as multiple 13-1actamase genes and aminoglycoside-modifying enzyme genes, from A. baumanii strains isolated from patients with lower respiratory tract infections (LRTIs). Methods Two thousand six hundred and ninety-eight sputum or the bronchoalveolar lavage samples from inpatients with LRTIs were collected in 21 hospitals in the mainland of China from November 2007 to February 2009. All samples were routinely inoculated. The isolated bacterial strains and their susceptibility were analyzed via VITEK-2 expert system. Several kinds of antibiotic resistant genes were further differentiated via polymerase chain reaction and sequencing methods. Results Totally, 39 A. baumanii strains were isolated from 2698 sputum or bronchoalveolar lavage samples. There was not only a high resistant rate of the isolated A. baumanfi strains to ampicillin and first- and second-generation cephalosporins (94.87%, 100% and 97.44%, respectively), but also to the third-generation cephalosporins (ceftriaxone at 92.31%, ceftazidine at 51.28%) and imipenem (43.59%) as well. The lowest antibiotic resistance rate of 20.51% was found to amikacin. The OXA-23 gene was identified in 17 strains of A. baumanii, and the AmpC gene in 23 strains. The TEM-1 gene was carried in 15 strains. PER-1 and SHV-2 genes were detected in two different strains. Aminoglycoside-modifying enzyme gene aac-3-1a was found in 23 strains, and the aac-6"lb gene in 19 strains, aac-3-1a and aac-6"lb genes hibernated in three A. baumanfi strains that showed no drug-resistant phenotype. Conclusions A. baumanii can carry multiple drug-resistant genes at the same time and result in multi-drug resistance. Aminoglycoside-modifying enzyme genes could be hibernating in aminoglycoside sensitive strains without expressing their phenotype.
文摘Clinical categories of Langerhans cell histiocytosis (LCH) include single and multi-system disease. Pulmonary LCH is rare, which is an unusual interstitial lung disease with the characteristics of monoclonal proliferation and infiltration of Langerhans' cells to organs. We report our experience of a rare LCH case of multiple organs such as pulmonary and liver as the main clinical manifestation. The patient was treated with chemotherapy which included prednisone, vinblastine, methotrexate and 6-mercaptopurine for 52 weeks and follow up all along. The patient has a favorable clinical outcome.
基金Peking University People's Hospital Research and Development Funds,Grant/Award Number:RDX2019-03National Natural Science Foundation of China,Grant/Award Numbers:81971520,U1903210,81671602Beijing Municipal Science and Technology Project,Grant/Award Numbers:Z191100006619112,Z191100006619110。
文摘Background:A specific feature of the autoimmune response in rheumatoid arthritis(RA)is the presence of anti-citrullinated protein antibodies(ACPAs)in patient sera.These antibodies can appear several years before disease onset and are involved in the development of RA.Objective:We performed proteomic analysis by mass spectroscopy to identify novel citrullinated antigens and autoantibodies in RA patients.Methods:Polypeptides isolated from the sera of RA patients were identified by Orbitrap high-precision mass spectrometry and then citrulline-containing proteins were selected.The levels of ACPAs against these newly identified citrullinated autoantigens in sera of 100 RA patients and 50 healthy controls were determined by enzyme-linked immunosorbent assays.Results:A total of 135 proteins were identified in RA patients and the protein profile included 11 citrulline-containing antigens.Three of the 11 citrullinated proteins had been reported in previous studies.ACPAs against the novel citrullinated epitopes from these proteins were increased in sera from the RA patients compared with those from healthy controls.Autoantibodies against one of the citrullinated antigens,lipopolysaccharide-binding protein(LBP),was significantly increased in RA patients and associated with disease activities.The titer of anti-citrullinated LBP antibodies(anti-cLBP)was closely related to the infection incidence in RA patients.Conclusion:Serum protein analysis by high-precision proteomic technology is a feasible method to identify novel citrullinated epitopes in RA patients.Anti-cLBP antibodies are associated with disease severity and infection in RA patients.
文摘To evaluate and compare the outcomes and complications of three different surgical techniques for treating primary proximal hypospadias with ventral curvature(VC)≥30°,we retrospectively reviewed the medical records of patients who underwent primary repair of proximal hypospadias with vC≥30°after degloving at Beijing Children's Hospital Affiliated to Capital Medical University(Beijing,China)from January 2019 to January 2021.A total of 152 patients were divided into three groups:transverse preputial island flap(TPIF)combined with Duplay,modified Koyanagi,and staged TPIF,which were performed on 55,16,and 81 patients,respectively.A total of 39(25.7%)patients had complications.Complications rates were similar for the TPIF combined with the Duplay group(40.0%)and modified Koyanagi group(50.0%)but lower for the staged TPIF group(11.1%;P<0.01).The incidence of urethrocutaneous fistulas was significantly higher in TPIF combined with Duplay group(21.8%)compared to staged TPIF group(4.9%;P=0.01).In univariate analysis,the length of the urethral defect was the single factor that could predict complications;the cutoff was 4.55 cm.More patients in the long urethral defect group than in the short one had complications(34.1%vs 15.7%,P=O.O1).These results indicate that staged TPIF produced a better outcome,whereas more patients in the TPIF combined with Duplay group presented with two or more complications.
文摘There is still debate regarding the optimal surgical approach for proximal hypospadias. This retrospective study aims to evaluate the long-term outcomes using transverse preputial island flap urethroplasty. A total of 320 patients were included, with a mean follow-up of 40.2 months (range: 1–156 months). Complications were encountered in 125 patients (39.1%), including fistulas in 53 (16.6%), urethral strictures in 31 (9.7%), and diverticula in 41 (12.8%). The mean timing of presentation with a complication was 15.8 months (median: 1.7, range: 1–145), of which 79.2% were early complications and 20.8% were late complications. In all, 20.8% of the patients with complications presented after ≥1 year, and 12.8% presented after ≥5 years. Univariate analysis revealed that age at the time of surgery, flap length, and location of the urethral meatus were not correlated with complications. A stricture was present in 31.7%(13/41) of those with diverticula (P < 0.001), while late urethral diverticula were accompanied by urethral strictures in 11.1%(1/9) of cases (P = 0.213). These results indicate that transverse preputial island flap urethroplasty still has a high incidence of complications, even when performed by highly experienced physicians. Most complications of hypospadias are diagnosed within 1 year postoperatively, while fistulas and urinary strictures generally occur within 2 months and diverticula tend to be present by 1 year.
基金Supported by the Special Traditional Chinese Medicine Research Project of State Traditional Chinese Medicine Administration(No.200807017)
文摘OBJECTIVE:To research the curative effect of Chinese herbs for clearing away heat,promoting diuresis,nourishing the kidney,and consolidating essence in children with Henoch-Schonlein purpura nephritis(HSPN)with internal accumulation of damp-toxin using randomized controlled observations on large samples.To seek the mechanism of the therapy and its scope of indications.METHODS:Overall,186 children with HSPN were randomly divided into two groups:treatment group(n=126)treated with Chinese herbs for clearing heat and promoting diuresis and a control group(n=60)treated with Western Medicine.The treatment was carried out for three courses of 4weeks each.We recorded changes in patient urine routines,24 h urinary protein,blood-coagulating series,immunoglobulin and T-cell subgroups,and improvements in main symptoms.We evaluated the alleviation of clinical symptoms and the improvement of proteinuria,hematuria,and other lab-oratory test results.Finally,we analyzed the patient population suitable for this therapy according to the relationship between the grouping of patient body weight and curative effect.RESULTS:Damp-heat syndrome improved in the treatment group,with a significant difference in total effective rate after a 4-week treatment(χ2=13.5220,P=0.0002)and in curative rate after a12-week treatment(χ2=6.3410,P=0.0118),compared to those in the control group.The curative effect in the treatment group was greater than that in the control group but there was no statistical difference between the two groups.The curative effect after a 4-week treatment of patients in the treatment group weighing 30 kg or less based on Traditional Chinese Medicine(TCM)signs and urinary protein was significantly greater than that in the control group.However,there was no statistical difference in the curative effect on urinary red cells and various indexes after a 12-week treatment between the two groups.CONCLUSION:Therapy for clearing away heat,promoting diuresis,nourishing the kidney,and consolidating essence using TCM is effective in children with HSPN from internal accumulation of damp-toxin.The therapy is especially suitable for patients weighing 30 kg or less.The curative effect may be related to the improvement of immune function and blood-coagulation.
基金This research was funded by Sanming Project of Medicine in Shenzhen(SZSM201512030)The funding organization had no involvement in study design,collection of data,the writing of draft,and the decision to submit the article for publication.
文摘Background Increasing numbers of pertussis cases have been reported in recent years.The reported cases from Shenzhen Children's Hospital were close to one tenth of all cases in China.The epidemiology of antigenic genotype and antibiotic resistance of circulating strains in children have been unknown in Shenzhen,southern China.The aim of this study was to describe the clinical features and explore the genotypes and antimicrobial susceptibility of circulating Bordetella pertussis among children in Shenzhen.Methods Data of hospitalized children with pertussis in Shenzhen Children's Hospital from August 2015 to April 2017 were collected.The genetic variability of isolates was investigated and Etest was performed for phenotypic susceptibility to erythromycin,azithromycin,clarithromycin,clindamycin,and trimethoprim/sulfamethoxazole.Results 469 children with pertussis confirmed by real-time quantitative polymerase chain reaction were hospitalized and strains were isolated from 105 patients.White blood cell count≥20×10^9/L and lymphocyte proportion≥60%were observed in 39.29%of infants younger than 3 months.The two predominant profiles of virulence-associated allelic genes were ptxA1/ptxC1/ptxPl/prnl(48.6%)and ptxA1/ptxC2/ptxP3/prn2(44.8%).Among the isolates,48.6%(51/105)were found resistant to macrolides.Conclusions These findings indicate that leukocytosis is not a sensitive indicator of pertussis.Isolates with the gene profile ptxP3/prn2 were highly circulating in Shenzhen and less resistant to macrolides,different from patterns observed in other parts of China.
文摘Background Intake of melamine can cause renal and ureteral lithiasis in infants and children. The present study aimed to understand the value of ultrasonography in the diagnosis of renal and ureteral lithiasis in infants and young children caused by melamine, and the characteristics of ultrasonograms of melamine-associated calculi. Methods Ultrasonographic examination on the urinary system was performed for 28 332 children who ingested milk powder that was possibly tainted with melamine; 395 of the children were diagnosed by ultrasonography as having urinary calculus, and 231 cases had lump-like calculi and 164 cases had sand gravel-like calculi. The features of the calculi, the sites of obstruction and the status of hydronephrosis and hydroureterosis were analyzed. Ultrasonographic reexamination was performed for 116 patients and the findings were compared with those of the first ultrasonography, and the short-term expulsion of the calculi was evaluated. Results Most of the 395 patients with urinary lithiasis, except for those who developed renal failure, had no symptoms. The whole profile of the calculi could be seen in most of the cases, because the echogenicity of the calculi with no sharp or absent acoustical shadowing, was weaker as compared with those from calcium-containing calculi. Comet tail sign could be seen behind the echogenicity of single gravel calculus. The rate of diagnostic consistency of ureteral lithiasis between the ultrasound and the results of clinical observation (including stones expelled spontaneously or after cystoscopic intervention) in 51 cases for 76 ureters was 100%. Percutaneous renal biopsy was performed for one case, and histopathological examination showed flocculent, fine strip-like, ellipse and circular deeply stained sand gravel-like material in the renal tubules, and the circular calculi were found to be attached to the walls of the tubules. Chemical analysis of the calculi expelled from 12 cases showed that the main contents of the calculi were uric acid and melamine. Short-term ultrasound reexamination in 116 patients showed that gravel-like calculi disappeared in 80.4% of the cases; in 26 non-hospitalized cases who had lump-like calculi without hydronephrosis or hydroureterosis, none of the lump-like calculi were expelled. Conclusions The ultrasonograpic findings of urinary calculi in children caused by ingestion of melamine-tainted milk powder have a certain features as compared to the calculi containing calcium. Careful ultrasound examination can avoid missed diagnoses of ureteral calculi. Most of the gravel-like calculi can be expelled within a short period of term, while lump-like calculi can hardly be expelled. Ultrasonography is an accurate and reliable method of diagnosis of urinary calculus caused by melamine intake in children and it can be used as diagnostic method of choice although abdominal X-ray plain film can also visualize some of larger calculi.
文摘Williams-Beuren syndrome (WBS) is a hereditary disease involving multiple systems due to microdeletion of chromosome 7q11.23. The deleted region in most patients is approximately 1.55 Mb, including 26-28 genes. The incidence of WBS in liveborn children is roughly 1/7 500-1/25 000.1
文摘Background In 2008, a sharp increase of the number of children diagnosed with urinary calculi was observed in China, 9433 children were diagnosed as having melamine-induced urinary calculi at outpatient clinic in Beijing Children's Hospital. This study examined the therapeutic efficacy of potassium sodium hydrogen citrate (PSHC) used to treat melamine-induced urinary stones in Chinese children who consumed melamine-containing infant formula. Methods Seventy-two infants and children (average age (18.2±7.7) months) who were diagnosed with urinary calculi were icuidomly divided into three treatment groups using the SAS Plan program. Group 1 was given a low dose (1 g/d) of PSHC, group 2 was given high dose of PSHC (2 g/d) and group 3 was given no PSHC (control group). The dose of drug was adjusted according to the baseline urinary pH. This study analyzed the influence of the dose of PSHC, the age of patients, stone size and position, and urinary pH on the level of efficacy of PSHC (cured, effectively treated or not cured).Results After 1-6 months of therapy, 19 patients from group 1, five patients from group 2 and six patients from group 3 were cured. Five patients from group 1, five patients from group 2 and four patients from group 3 were effectively treated. There were significant differences in therapeutic efficacy between the two treatment doses after 3 and 6 months as measured by the increase in the successful expulsion rate and time of melamine-induced urinary calculi. After 6 months the mean time of expulsion of urinary calculi in groups 1 and 2 was significantly shorter than in the control group. Conclusions PSHC can significantly increase the successful expulsion rate and time of melamine-induced urinary calculi. The therapeutic efficacy is affected by PSHC dose, treatment duration, calculi position, and urinary pH. There is no relationship between the therapeutic efficacy and the stone size or patient age.
文摘Background Infants in some areas of China developed urinary lithiasis after being fed with powdered milk that was tainted with melamine in 2008 and very small proportion of the infants developed acute renal failure caused by urinary tract calculus obstruction. The aim of this article was to summarize clinical characteristics, diagnosis and treatment of infants with urinary calculus and acute renal failure developed after being fed with melamine tainted formula milk. Methods Data of infant patients with urinary calculus and acute renal failure due to melamine tainted formula milk admitted to the Beijing Children's Hospital Affiliated to the Capital Medical University and the Xuzhou Children's Hospital in 2008 were used to analyze the epidemiological characteristics, clinical manifestations, imaging features as well as effects of 4 types of therapies. Results All the 34 infants with urinary calculus were complicated with acute renal failure, their blood urea nitrogen (BUN) was (24.1±8.2) mmol/L and creatinine (Cr) was (384.2±201.2) umol/L. The chemical analysis on the urinary calculus sampled from 15 of the infants showed that the calculus contained melamine and acidum uricum. The time needed for the four types of therapies for returning Cr to normal was (3.5±1.9) days for cystoscopy group, (2.7±1.1) days for lithotomy group, (3.8±2.3) days for dialysis group, and (2.7±1.6) days for medical treatment group, which had no statistically significant difference (P=-0.508). Renal failure of all the 34 infants was relieved within 1 to 7 days, averaging (3.00±1.78) days. Conclusions Melamine tainted formula milk may cause urinary calculus and obstructive acute renal failure. It is suggested that firstly the patients with urinary calculus complicated with acute renal failure should be treated with dialysis or medication to correct electrolyte disturbance, in particular hyperkalemia, and then relieve the obstruction with available medical and surgical methods as soon as possible. It was observed that the short-term prognosis was satisfactory.
文摘Asymmetric superior oblique palsy(SOP)is a complicated,non-comitant type of strabismus.Patients usually present with small-angle hypertropia in the affected eyes,accompanied by inferior oblique muscle overaction(IOOA).[1]Compensatory head posture and asymmetric development of the face are typically observed in congenital SOP,while diplopia is more common in an acquired SOP.[2]Prism glasses can improve diplopia and vertical deviation in the primary position,but they are not so successful at correcting non-comitant paralytic strabismus.In addition,vertical strabismus and compensatory head posture will return after the prism glasses are removed,wearing prism glasses will lower the vision of children prominently.Surgery like an inferior oblique muscle(IO)weakening procedure is often required eventually in those patients.However,conventional procedures for IO weakening may cause overcorrection and/or secondary A pattern in patients who have mild to moderate IOOA together with small-angle asymmetric SOP,posing a risk to its safety.[3]In the study by Caldeira,[4]21%of the patients with mild to moderate IOOA developed A pattern following the surgery.Therefore,it remains a clinical challenge to select the most appropriate type of surgery for the treatment of small-angle asymmetric SOP.Inferior oblique muscle belly transposition(IOMBT),[5]a new IO weakening procedure introduced to our practice lately,has shown positive results in treating small-angle asymmetric SOP as well as a lower risk of complications such as overcorrection and secondary A pattern.In this study,we evaluated the efficacy and safety of the IOMBT procedure for the treatment of small-angle asymmetric SOP.
文摘Kasabach-Merritt phenomenon(KMP)is a rare disease that is characterized by severe thrombocytopenia and consumptive coagulation dysfunction caused by kaposiform hemangioendothelioma or tufted hemangioma.This condition primarily occurs in infants and young children,usually with acute onset and rapid progression.This review article introduced standardized recommendations for the pathogenesis,clinical manifestation,diagnostic methods and treatment process of KMP in China,which can be used as a reference for clinical practice.