OBJECTIVE: To evaluate the efficacy and safety of mycophenolate mofetil (MMF) plus prednisone on refractory nephrotic syndrome (RNS) in children. METHODS: One hundred and forty-two children with RNS from ten clinical trial centers were divided into two groups: MMF (n=87) and control (n=55). The MMF group patients were administered with oral MMF (30-40 mg/kg daily) for at least 6 months. Afterwards the patients who responded to MMF received another 6 months MMF treatment at a dosage of 10-20 mg/kg daily. The controls were treated with pulse intravenous infusion of cyclophosphamide (CTX) (10 mg/kg daily) for 2 days every 2 weeks for 3 months. Then CTX was administered at a dosage of 500 mg/m2 once a month 4, 7 and 10 months after treatment. While the patients received MMF or CTX treatment, they were treated with oral prednisone (0.5-1 mg/kg daily) for 2 to 3 months, and then the dosage of prednisone was gradually reduced. Urinary protein, liver and renal functions, and side effects of drugs were examined at regular intervals for one year. RESULTS: Of the 87 patients, 58 achieved complete remission, 16 achieved partial remission, 9 achieved early remission and 4 had no response to treatment. In the control group, 35 achieved complete remission, 9 achieved partial remission, 1 achieved early remission and 10 had no response to treatment. The total remission rate in the MMF group (95.4%) was significantly higher than that in the control group (81.8%) (P<0.01). After treatment 67 patients (65.4%) in the MMF group had negative proteinuria compared with 36 patients (65.4%) in the control group (P>0.05). MMF was found to be more effective in reducing proteinuria, and improving hypoproteinemia, oliguria, hyperlipemia, and edema than CTX. MMF was better tolerated with lower incidences of adverse reactions than CTX. CONCLUSIONS: The combined therapy of MMF and prednisone is more effective and tolerable than pulse intravenous infusion of CTX for treatment of RNS in children.

OBJECTIVE: To study the distribution and drug resistance of the isolated bacteria from children with acute respiratory infection (ARI) in Dalian. METHODS: Between January 2006 and February 2007, 930 children with ARI were enrolled, including 364 with acute upper respiratory infection (AURI), and 566 with acute lower respiratory infection (ALRI). The AURI children, who did not receive antimicrobial agent treatment or received oral antimicrobial agents 1-2 times, had bacterial cultures of pharyngeal swab. The ALRI children, who received intravenous antibacterial agents more than 3 days, had bacterial cultures of sputum and bronchoalveolar lavage fluid (BALF). Isolated bacteria were identified by the ATB system (BioMerieux, France). Antimicrobial susceptibility testing was carried out by means of Kirby-bauer. RESULTS: A total of 404 isolates (43.4%) were identified. Haemophilus influenzae, Haemophilus parainfluenzae and Streptococcus pneumoniae accounted for 22.5%, 12.1% and 7.4% respectively. In the isolates from AURI, Haemophilus influenzae, Haemophilus parainfluenzae and Streptococcus pneumoniae accounted for 43.9%, 22.0% and 9.1% respectively; Escherichia coli, Klebsiella pneumonia and Nonfermenters accounted for 4.5%, 8.3% and 3.0% respectively. In the isolates from ALRI, Haemophilus influenzae, Haemophilus parainfluenzae and Streptococcus pneumoniae accounted for 12.1%, 7.4% and 6.6% respectively; Escherichia coli, Klebsiella pneumoniae and Nonfermenters accounted for 16.9%, 13.2% and 21.8% respectively. The resistant rates of Haemophilus to ampicillin and TMP-SMZ were 29.3% and 32.9% respectively, and to amoxicillin-clavulanic acid, cefalotin, cefaclor, cefuroxime and cefotaxime were 12.1%, 10.0%, 10.0%, 11.4% and 5.7%, respectively. The resistant rate of Haemophilus to ampicillin, amoxicillin-clavulanic acid, cefaclor, tetracycine and TMP-SMZ in the ALRI group were significantly higher than that in the AURI group (P<0.05 or 0.01). CONCLUSIONS: In Dalian, Haemophilus was the main isolate of children with ARI. The distribution of bacteria was different between ALRI and AURI. In ALRI, Gram-negative bacilli were in a higher proportion, and the resistant rates of Haemophilus influenzae and Haemophilus parainfluenzae to ampicillin, amoxicillin-clavulanic acid and cefaclor were higher.

OBJECTIVE: The event related potential (ERP-P300) is useful to determine cognitive disturbances. This study examined the changes of ERP-P300 following different dosages of topiramate (TPM) treatment in children with epilepsy in order to investigate the effect of different dosages of TPM on cognitive function. METHODS: Thirty cases of benign childhood epilepsy with centrotemporal spikes (BECTS) were first administered with TPM at a dosage of 2 mg/kg?d for 6 months. Afterwards they received another 6 months of TPM treatment at a dosage of 5 mg/kg?d. ERP-P300 was tested before and after different dosages of TPM treatment. RESULTS: There were no significant differences in the latency and amplitude of ERP-P300 before and after 6 months low dosages of TPM treatment. However, the latency was more prolonged and the amplitude was reduced in the ERP-P300 testing after 6 months high dosage of TMP treatment (P<0.01). CONCLUSIONS: The effect of TPM on cognitive function is related to its dosage in children with epilepsy.

OJECTIVE: To evaluate the effect of the early use of recombinant human erythropoietin (rhu-EPO) on neurobehavioral development in preterm infants. METHODS: Forty-four preterm infants (30 males and 14 females) were randomly divided into two groups: Rhu-EPO treatment and untreated control (n=22 each). From postnatal day 7, the Rhu-EPO treatment group received intravenous rhu-EPO (250 IU/kg, 3 times weekly) for 4 weeks. A Neonatal Behavioral Neurological Assessment (NBNA) was performed at 40 weeks of corrected gestational age. A Gesell Development Schedule was used to evaluate neurological development 6 and 12 months after birth. RESULTS: The NBNA score in the rhu-EPO treatment group (36.20±0.75) was significantly higher than that in the control group (34.40±1.05) at 40 weeks of corrected gestational age (P<0.05). The developmental quotient of fine motor in the rhu-EPO treatment group was significantly higher than that in the control group 6 months after birth (P<0.05). By 12 months after birth, the developmental quotient of gross motor, fine motor and language in the rhu-EPO treatment group was significantly higher than that in the control group (P<0.05). CONCLUSIONS: Early use of Rhu-EPO can promote neurobehavioral development in preterm infants.

OBJECTIVE: To investigate the related perinatal factors in the development of anemia of prematurity by a case-control study. METHODS: The medical data of 165 preterm infants admitted to the department of neonatology in Peking University Third Hospital from January 2005 to December 2006 were retrospectively reviewed. The infants were classified into two groups: anemia and non-anemia (control). The factors associated with anemia of prematurity were identified. RESULTS: Sixty-three preterm infants (38.1%) had anemia. The gestational age (32.5±2.0 weeks vs 33.7±1.9 weeks) and birth weight (1 682.7±393.9 g vs 2 041.1±510.1 g) of the anemia group were significantly lower than those of the control group (P<0.01). The blood loss from laboratory testing (12.4±6.5 mL/kg vs 6.6±3.6 mL/kg) and the duration of hospital stay (25.6±14.2 days vs 14.1±8.7 days) of the anemia group were significantly higher than those of the control group (P<0.01). More critically ill patients (33.3%) were noted in the anemia group compared with in the control group (9.8%) (P<0.01). In the anemia group, 44.4% of the mothers had preeclampsia but only 28.4% in the control group (P<0.05). Multiple factor logistic regression analysis revealed that the blood loss from laboratory testing was the only independent high-risk factor for anemia of prematurity. Compared with the preterm infants whose blood loss from laboratory testing was <5 mL/kg, the OR values of the preterm infants whose blood loss was 5-10, 10-15 and ≥15 mL/kg was 1.737 (95% CI: 0.699-4.316, P>0.05), 4.141 (95% CI: 1.573-10.905, P<0.01) and 32.267 (95% CI: 8.053-129.287, P<0.01), respectively. CONCLUSIONS:Anemia of prematurity is associated with gestational age, birth weight, duration of hospital stay, illness severity and maternal preeclampsia. The blood loss from laboratory testing is an independent risk-factor in the development of anemia of prematurity.

OBJECTIVE: To investigate the efficacy and safety of famotidine treatment for stress ulcers in neonates. MEYHODS: Fifty-four neonates with stress ulcers from 2001 to 2006 were enrolled. Seven cases were confirmed with stress ulcers by gastroscopy. Famotidine was administered intravenously at a dosage of 0.5 mg/kg every other 12 hrs. After cessation of hematemesis and vomiting, famotidine was administered once a day for two days. Primary diseases and complications were concurrently treated. Clinical symptoms and gastric pH were assessed before and after famotidine treatment. Possible adverse effects of famotidine treatment-related were observed. RESULTS: After 24 hrs of famotidine treatment, hematemesis and vomiting ceased in 52 patients (96.3%). Clinical symptoms disappeared in all of the 54 patients 48 hrs after famotidine treatment. Gastric pH value increased 6, 12, 24, 36 and 48 hrs after famotidine treatment from 2.07±0.22 (before treatment) to 5.01-5.15 (P﹤0.01). All of the 54 patients were successfully treated. Famotidine treatment did not lead to abnormal respiration, heart rate and blood pressure. Loss of appetite, nausea, vomiting, diarrhea, constipation and rashes were not seen after famotidine treatment. There were significant differences in white cell count, platelet count and hepatic enzyme levels before and after famotidine treatment. An augmented side effect of the other drugs concurrently used due to famotidine treatment was not noted. CONCLUSIONS: Famotidide is effective and safe for the treatment of stress ulcers in neonates.

OBJECTIVE: To report the experience of the surgical treatment of congenital heart diseases (CHD) in pediatric patients with body weight less than 10 kg. METHODS: Between January 2000 and December 2007, 105 children with CHD, aged 2 months to 3 years and weighing between 3.5 to 10 kg, underwent surgical treatment. Of the 105 patients, 56 were concomitant with moderate to severe pulmonary hypertension or repeated pulmonary infections, and 35 with complex cardiac abnormalities. Operations were performed through median sternotomy with moderate hypothermic cardiopulmonary bypass in 88 cases, with normothermic extracorporeal circulation on beating heart in 14 cases, and with deep hypothermic circulatory arrest in 3 cases. RESULTS: There were 5 early deaths (4.8%). During a follow-up of 2 month to 3 year, 97 survivors with corrective procedure had no late mortality or complications with NYHA class I of cardiac function, excepting 2 cases with little residual shunt. Three survivors with palliative procedure enjoyed higher quality of life. CONCLUSIONS: The surgical treatment of CHD in pediatric patients with body weight less than 10 kg seems to be feasible and safe, with satisfactory early and long-term results.

OBJECTIVE: Interventional treatment for childhood combined congenital heart disease (CHD) has developed very quickly and more new types of occluders have emerged in recent years. The aim of this study is to investigate the efficiency and safety of interventional treatment for combined CHD in children. METHODS: Eight children with combined CHD (4 boys and 4 girls), aged 6.1±2.9 years, underwent simultaneous transcatheter therapy. Of the 8 children with CHD, 1 case had atrial septal defect (ASD), ventricular septal defect (VSD) and patent ductus arteriosus (PDA), 1 case had ASD, PDA and pulmonary stenosis (PS), 1 case had ASD and PDA, 1 case had patent foramen ovale (PFO) and PS, and 4 cases had ASD and PS. The methods of transcatheter intervention for these patients were as follows: in patients with ASD,VSD and PDA, the occlusion of VSD was performed first, followed by PDA and ASD occlusions; in patients with ASD, PDA and PS, the occlusion of percutaneous balloon pulmonary valvuloplasty (PBPV) was performed first, followed by PDA and ASD occlusions; in patients with PFO and PS, the occlusion of PBPV was performed first, and PFO occlusion followed; in patients with ASD and PS, the occlusion of PBPV was performed first, and ASD occlusion followed. RESULTS: The intervention operation was successfully performed in all of the 8 patients. No serious adverse events occurred during the operation. No residual shunt was found and all the occlusion devices were in the suitable sites shown by transthoracic echocardiography (TTE) and X-ray right after the operation. In the 6 patients with PS, the systolic pressure across the pulmonary valve decreased from 75.3±15.6 mmHg (before operation) to 14.0±5.6 mmHg after operation (P<0.05).A 3.4±1.2 years follow-up demonstrated that no residual shunt occurred and gradients across valve or coarctation sites were within the limit of satisfactory results. No complications were observed during the follow-up. CONCLUSIONS: Transcatheter interventional therapy for childhood combined CHD can obtain satisfactory results by proper procedures.

OBJECTIVE: To study the status of growth and development and the relationship between growth disorders and iron overload in children with betathalassemia major. METHODS: Fifty children with beta-thalassemia major and who received blood transfusion therapy regularly (age: 9 months-17 years) were enrolled. They were subjected to a thorough history taking, clinical examinations, and laboratory examinations, including complete blood count, alanine transferasa (ALT) and serum ferritin. The physical growth parameters, such as height and weight, were compared with the reference values of Chinese children. RESULTS: Twenty-four patients (48%) were of short stature with height under the 3th percentile. Among them, 15 cases presented with their height and weight both under the 3th percentile. Spontaneous sex development was seen in 7 cases out of 21 over 10-year-old patients. No sex development was found in 4 out of 8 patients who were over 14 years old. The patients with a height under the 10th percentile (n=31) had higher serum ferritin levels (8 239.2±5 865.5 mg/L vs 5 028.1±3 885.7 mg/L; P<0.05) and lower hemoglobin levels (68.2±12.3 g/L vs 79.7±14.5 g/L; P<0.05) as well as hepatomegaly when compared with those patients with a height over the 10th percentile (n=19). Serum ferritin levels in 20 patients with a weight under the 10th percentile were significantly higher than those in 30 patients with a height over the 10th percentile (9 165.5±6 042.5 mg/L vs 5 567.3±4 447.3 mg/L; P<0.05). CONCLUSIONS: Short stature, low weight and sex development delay are common in children with beta-thalassemia major. This may be related to iron overload.

OBJECTIVE: To study polymorphisms of dopamine D4 receptor (DRD4) in children with primary nocturnal enuresis (PNE) and explore the relationship between DRD4 gene polymorphisms and PNE. METHODS: Genomic DNA was isolated from leukocytes in 86 unrelated children with PNE and in 100 healthy unrelated children (controls). Polymorphisms of DRD4-1240L/S, -616C/G and -521C/T were genotyped by allele-specific primer PCR. RESULTS: There were significant differences in allele frequencies (χ2=8.13, P<0.05) and genotypes frequencies (χ2=6.23, P<0.05) of DRD4-616C/G between PNE patients and healthy controls. The frequency of haplotype LCT consisting of 3 function polymorphic sites DRD4-1240L/S, -616C/G and -521C/T in PNE patients was statistically higher than that in healthy controls (χ2=5.88, P<0.05). CONCLUSIONS: The change of C to G of DRD4-616 may affect the induction and transcription of DRD4 gene. The haplotype LCT consisting of 3 function polymorphic sites DRD4-1240L/S, -616C/G and -521C/T may synergistically inhibit the transcription activity of DRD4 gene. This might lead to a reduction of DRD4 protein expression and cause nocturnal enuresis.

OBJECTIVE: To assess the sensory integration function of children with primary nocturnal enuresis (PNE) and explore the role of sensory integration dysfunction in the pathogenesis of PNE. METHODS: Sensory integration function was assessed by the Childhood Sensory Integration Ability Development Checklist in 46 children with PNE and 46 normal children (control). RESULTS: The incidence of sensory integration dysfunction in the PNE group (82.6%) was significantly higher than that in the control group (43.5%)(P<0.01). Seventeen patients (36.9%) presented with severe sensory integration dysfunction in the PNE group but only 1 (2.1%) in the control group (P<0.01). The scores of all nine sensory integration indexes revealed by sensory integration function testing in the PNE group were significantly lower than those in the control group (P<0.01). CONCLUSIONS: Children with PNE have sensory integration dysfunction. Sensory integration dysfunction may be associated with the pathogenesis of PNE.

OBJECTIVE: To investigate the value of Glasgow Coma Scale (GCS) and Glasgow-Pittsburgh Coma Scale (GCS-P) scoring in predicting the prognosis of coma in children. METHODS: Clinical data of 17 comatose children were retrospectively reviewed. The results of GCS and GCS-P scoring, electroencephalogram (EEG) and cranial imaging were analyzed. Dynamic curves of GCS-P score were drawn. RESULTS: Seven patients received EEG examination and four showed low potential. The four patients had poor prognosis. Cranial CT and MRI were performed in 12 patients. Of these three showed cerebral hemorrhage and ischemia and had a poor prognosis. The accuracy rate for predicting the prognosis of GCS and GCS-P scoring was 85.71% and 88.57% respectively. A continuous GCS-P scoring was performed in 13 patients. A dynamic GCS-P curve showed an ascent in seven cases with good prognosis but a flat or declined tendency in six cases with poor prognosis. COCLUSIONS: GCS-P scoring is valuable for predicting prognosis in children with coma. Combined with EEG and cranial imaging examinations, the accuracy for predicting prognosis of GCS-P scoring will increase.

OBJECTIVE: To investigate the clinical features of recurrent Kawasaki disease (KD) and the relationship of recurrent KD with coronary artery lesions. METHODS: The medical data of 20 children with recurrent KD who were admitted to the Children's Hospital from January 1998 to May 2007 were retrospectively studied. Their clinical features were compared with those of children with initial KD. RESULTS: The incidence of recurrent KD was 1.34% (20/1 489). KD relapsed 2 months to 4.6 years (average: 1.2 years) after the first episode in the 20 children. Compared with the initial KD group, the clinical symptoms in the recurrent KD group were incomplete, complicated and less severe. The period of fever, platelet count, C-reactive protein and ESR were remarkably reduced in the recurrent KD group, but the incidence of coronary artery lesions increased significantly compared with the initial KD group (40% vs 25%; P<0.05). CONCLUSIONS: The clinical symptoms of recurrent KD were incomplete in children. Recurrent KD was associated with an increased incidence of coronary artery lesions.［Chin J Contemp Pediatr, 2008, 10 (5):617-619］

OBJECTIVE: To investigate whether WASP/Verprolin homologous protein 1 (WAVE1) plays a role in the pathogenesis of childhood acute lymphoblastic leukemia (ALL). METHODS: WAVE1 mRNA and protein expression in bone marrow mononuclear cells (BMMCs) was measured by RT-PCR and Western blotting respectively in 4 children with ALL relapse, 15 children with ALL in complete remission (CR) and 40 children with newly diagnosed ALL. Ten normal bone marrow samples were used as controls. Jurkat cells were treated with different concentrations of adriamycin (ADM). The cell proliferation was detected with MTT. The apoptosis rate was measured by flow cytometry. WAVE1 mRNA and protein expression of Jurkat cells treated with ADM was detected by RT-PCR and Western blotting respectively. RESULTS: WAVE1 was not expressed or weakly expressed in BMMCs from normal controls and patients with ALL in CR. Higher WAVE1 mRNA and protein expression was found in BMMCs from patients with newly diagnosed ALL and patients with relapse ALL when compared with the controls and the patients in CR (P<0.01). ADM significantly inhibited the proliferation of the Jurkat cells and the inhibitory effect was dose-and time-dependent (P<0.05). After ADM treatment for 24 hrs, the percentage of apoptosis cells increased significantly and WAVE1 mRNA and protein expression of Jurkat cells decreased significantly when compared with the untreated controls (P<0.05). CONCLUSIONS: The WAVE1 expression increased in children with ALL. WAVE1 may be related to the development of ALL and may be severed as a marker for the evaluation of the severity of ALL in children.

A 13-year-old girl presented with a 1-month history of progressive exertional dyspnea (NYHA class Ⅳ) and exophthalmos for 6 months. She had a history of long-standing asthma and the presence of allergy. Hypereosinophilia and increased serum IgE levels (2 472 IU/mL) were observed. Chest radiography and a high resolution CT scan documented a massive interstitial pulmonary infiltration. Echocardiography confirmed mild tricuspid regurgitation, apical obliteration of the right ventricle by fibrocalcific thickening of the endocardium and echogenic material suggestive of thrombosis. Churg-Strauss syndrome with cardiac involvement (endomyocardiopathy) was diagnosed. The patient received anticoagulation and corticosteroid therapy. In view of rapidly progressive severe endomyocardiopathy and stable hematology, the patient was referred for cardiac surgery. Histopathological examination of resected specimens confirmed laminated thrombus but without any trace of eosinophils embedded.

OBJECTIVE: To study the effect of a low level of galacto-oligosaccharides (GOS) on intestinal bifidobacteria and lactobacilli, and fermentation characteristics in term infants by comparing with human milk and a standard infant formula without GOS. METHODS: A total of 371 term infants from four hospitals of China were enrolled. The infants started with breast-feeding. After 1-2 weeks, some of the infants were changed to feeding with formula milk and then were randomly assigned to two formula-feeding groups: with or without GOS supplementation (2.4 g/L). Growth, stool characteristics, and side effects were recorded in a 3-month-follow-up. Faecal samples were collected for analysis of intestinal bacteria (culture technique), acetic acid (gas chromatography) and pH (indicator strip) at postnatal 3 months. RESULTS: Compared with the formula-feeding group without GOS, the contents of bifidobacteria, lactobacilli and acetic acid and stool frequency increased, and faecal pH decreased significantly in the GOS-formula-feeding and the human milk group. There were no significant differences between the GOS-formula-feeding and the human milk groups. Supplementation with GOS did not lead to an increase in the incidence of crying, regurgitation and vomiting. CONCLUSIONS: A supplementation of low levels of GOS in infant formula seemed to improve stool frequency, decrease faecal pH, and stimulate intestinal bifidobacteria and lactobacilli up to levels as found in breast-fed infants.