Table of Content

15 April 2017 Volume 35 Issue 4

  

Variation in genes related to lipid metabolism and the susceptibility of nonalcoholic fatty liver disease in obese children　

　DAI Dongling, ZHOU Shaoming, LIU Guosheng, WEN Feiqiu, ZHOU Jianli

. 2017, 35(4):  241.  doi:10.3969/j.issn.1000-3606.2017.04.001

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　Objective　To explore the association between variation in genes related to lipid metabolism and the susceptibility of nonalcoholic fatty liver disease (NAFLD). Methods　Obese children with fatty liver aged 6~18 years old were included. All of them got ultrasonic testing, routine examination and biochemical detection. In addition, the DNA of peripheral blood was extracted and the 36 target genes related to lipid metabolism were detected by next generation sequencing.  Results　In 368  obese children who met the inclusion criteria, 183 children (49.7%) were detected to have NAFL . 100 children with NAFLD and 100 children without NAFLD were randomly selected from obese children. The levels of body mass, waistline, alanine aminotransferase (ALT),  triacylglycerol (TG), cholesterol, low density lipoprotein (LDL) and apolipoprotein B (ApoB) in NAFLD children were all higher than those in non-NAFLD children, and there were significant differences (P all<0.05). However, the levels of aspartate aminotransferase (AST), alkaline phosphatase (ALP), apolipoprotein A1 (ApoA1), fasting blood glucose, insulin, high density lipoprotein (HDL), free fatty acid (FFA) and insulin resistance index (HOMA-IR) were not significantly different between the two groups (P all>0.05). The levels of bilirubine in the two groups were within normal range. Logistic regression analysis showed that the genes of MTTP rs2306986 (OR=2.70, 95%CI: 1.38~5.27) and MTTP rs3792683 (OR=7.34, 95%CI: 2.04~26.50) that encode microsomal triglyceride transfer protein (MTTP or MTP), and the mutation of rs738409 (OR=2.11, 95%CI:1.31~4.48) in gene PNPLA3 that encode patatin-like phospholipase domain-containing protein 3 are the independent risk factors for the occurrence of the disease. Conclusion　Genovariation of MTTP rs2306986, MTTP rs3792683, and PNPLA3 rs738409 may increase susceptibility to NAFLD in children.

Fecal bacteria transplantation for treatment of severe gastrointestinal disease caused by food allergy in children: a case report and literature review

ZHU Zhongsheng, ZHENG Yuejie, CAI Huabo, BAI Daming, DAI Dongling, ZHOU Jianli, ZHOU Shaoming

. 2017, 35(4):  247.  doi:10.3969/j.issn.1000-3606.2017.04.002

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　Objective　To explore fecal bacteria transplantation for the treatment of severe gastrointestinal disease caused by food allergy. Method　The therapeutic process of fecal bacteria transplantation for treatment of severe food allergy gastrointestinal disease was retrospectively analyzed, and the related literature was reviewed. Results　A 2-year-old boy had onset of intestinal infection and diarrhea was persistent even though he had received adequate anti-infection therapy and supportive treatment. Finally, the patient received the treatment of fecal bacteria transplantation and the symptoms were then improved. No adverse reactions were observed in 2 months of follow-up. In foreign literature, fecal bacteria transplantation in children is mainly applied to clostridium difficile infection (CDI) and inflammatory bowel disease (IBD), with efficiency of 90% - 100% and 55.6% - 100%, respectively. While in the domestic literature, fecal bacteria transplantation in children is mainly used in CDI and antibiotic associated diarrhea, and the effective rate is 100%. No serious adverse reactions were found in all the researches. Conclusion　Fecal transplantation is safe and effective in the treatment of children with severe gastrointestinal disease caused by food allergy, but its application in children is not yet mature and needs more in-depth researches.

Alagille syndrome in a child combined with hepatocellular carcinoma: a case report and literature review

HU Hui, YANG Yongchen, ZHANG Hong, ZHANG Ting, XIAO Yongmei

. 2017, 35(4):  253.  doi:10.3969/j.issn.1000-3606.2017.04.003

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　Objective　To explore the clinical manifestation, imaging examination, treatment and prognosis of Alagille syndrome in a child combined with hepatocellular carcinoma. Method　The clinical manifestation, assistant examination and diagnosis of Alagille syndrome combined with hepatocellular carcinoma were analyzed in the child, and the pertinent literature were reviewed. Results　The 6-year-old girl was admitted to hospital for repeated jaundice, and had a history of cardiac surgery. After admission, the patient was found to have a typical face look such as frontal bossing, sunken eyes, pointed chin and hypertrophy of nasal tip. Blood biochemistry showed intrahepatic cholestasis and increased alpha-fetoprotein. Abdominal ultrasonography revealed diffuse multiple solid lesions in the liver. And magnetic resonance imaging of the liver indicated that the liver was enlarged and multiple solid space occupying masses. Jagged 1 gene detection showed heterozygosis mutation of c.1205delC. Conclusion　Alagllie syndrome complicated with hepatocellular carcinoma in childhood is extremely rare, and early diagnosis and long-term follow-up are of positive significance for its treatment and prognosis.

Analysis of hepatitis associated aplastic anemia in 5 children

　LI Xiang, ZHAO Xiaoming, SHENG Guangyao

. 2017, 35(4):  256.  doi:10.3969/j.issn.1000-3606.2017.04.004

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　Objective　To explore the clinical feature, treatment and prognosis of hepatitis associated aplastic anemia (HAAA) in children. Method　The clinical data of 5 children with HAAA were retrospectively analyzed. Results　There were 5 patients (4 males and 1 female) with median age of 10 years (7~13 years ). The detection of hepatitis A, B, C, and E were all negative in these 5 children during their acute hepatitis period. In 2 children, parvovirus B19 antibody and EB virus were negative and the proportion of CD4+T cells and the ratio of CD4+/CD8+ were decreased, and the proportion of CD8+T cells was increased by T-lymphocyte subsets analysis. Three children were treated with anti-thymocyte globulin combined with cyclosporine, 2 of whom achieved complete remission and 1 died of pulmonary fungal infection. One child was treated with cyclosporine only and achieved partial remission. One child lost follow-up after giving up treatment. Conclusion　HAAA in children can be caused by viral seronegative hepatitis, and usually has T lymphocyte immune disorders.  Immunosuppressive therapy is effective.

Clinical analysis of severe hand-foot-mouth disease complicated with cardiopulmonary failure in 321 children

GU Fen

. 2017, 35(4):  264.  doi:10.3969/j.issn.1000-3606.2017.04.006

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　Objective　To analyze the clinical and epidemiological characteristics of severe hand-foot-mouth disease (HFMD) complicated with heart failure in children, and to explore the risk factors of severe complications. Method　The clinical data of children with HFMD admitted from January 2014 to December 2014 were retrospectively analyzed and their HFMD was at clinical stage 2 or over. Results　There were totally 321 cases of severe HFMD, in which common group (clinical stage 2) had 306 cases and cardiopulmonary failure group (clinical stage 3 or 4) had 15 cases. There was no death in common group, while 7 cases died in cardiopulmonary failure group, and there was statistical difference (P<0.001). The median age in cardiopulmonary failure group was 9 months (6－20 months), which was lower than that in common group (median age of 24-month-old, 3 months to 12 years) and there was statistical difference (P<0.01). The peak temperature and fever duration were (39.44±0.23)℃, (5.01±0.94) d respectively in cardiopulmonary failure group, both of which were higher than peak temperature of (39.12 ±0.20)℃ and fever duration of (3.93 ± 0.47) d in common group, and the differences were significant (P all<0.05). The incidences of vomit, disturbance of consciousness, peripheral circulation, respiratory rhythm irregular and pneumonedema in cardiopulmonary failure group were higher than those in common group, and there were statistically significant differences (P all<0.001). The positive rate of human enterovirus 71 (EV71) in cardiopulmonary failure group was 85.7%, which was higher than that in common group, and there was significant difference (P<0.01). The levels of N terminal brain natriuretic peptide (NT-pro BNP) in cardiopulmonary failure group were all increased (100%), the rate of which was higher than that in common group (35.3%), and there was significant difference (P<0.001). Conclusion For children with HFMD, vomiting, consciousness disorders, circulatory disorders, respiratory rhythm disorders, EV71 positive and elevated levels of NT-pro BNP were risk factors of cardiopulmonary failure, and disease changes should be closely monitored.

Clinical analysis of primary nephrotic syndrome combined with hypercoagulable state in 57 children

　CHEN Han, YIN Mengdi, LYU Xiaohang, ZHANG Gaofu, WANG Mo, YANG Haiping, LI Qiu

. 2017, 35(4):  268.  doi:10.3969/j.issn.1000-3606.2017.04.007

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　Objective　To explore the clinical characteristics and influencing factors of primary nephrotic syndrome (PNS) combined with hypercoagulability in children. Methods　The clinical data of 57 children with primary PNS were analyzed retrospectively. The clinical features and treatment were compared among high coagulation state group, non high coagulation state group and control group (20 children). At the same time, the differences between the simple nephrotic syndrome group (SNS) and nephritic syndrome group (NNS) in hypercoagulable state were analyzed. In addition, the correlation analysis was performed. Results　Among 57 patients, there were 50 patients in high coagulation state group and 7 in non high coagulation state group. There was no significant difference in gender, age and clinical manifestations between two groups (P>0.05). The platelet (PLT) count, platelet aggregation (PCT), albumin (Alb), fibrinogen (Fib), D-dimer (D2) were significantly higher than those in the control group, and there were statistically significant differences (P all<0.01). There were significant differences in the levels of PLT, Fib, D2 and complement C4 between hypercoagulable state group and non hypercoagulable state group (P all<0.05). There were significant differences in HCT, TC, LDL, PT and complement C3 levels between SNS group (n = 32) and NNS group (n = 18) in 50 patients with high coagulation state (P<0.05). There was positive correlation between HCT and complement C3 (r=0.30, P<0.05), while there was no correlation between PLT and other indices (P>0.05). All of the 57 patients were improved and has no thrombosis after the treatment. Conclusion　Children with primary PNS were usually associated with different degrees of hypercoagulable state, and PLT, Fib, D2 could be used as reference indices for the severity of hypercoagulable state, and the activation of complement system might be related to the occurrence and development of hypercoagulable state.

Early predictive value of platelet related indicators in patent ductus arteriosus in extremely low birth weight infants

ZHONG Junjuan, YAO Zhongwei, MO Jing, ZHANG Jing, YANG Jie

. 2017, 35(4):  273.  doi:10.3969/j.issn.1000-3606.2017.04.008

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Objective　To analyze the predictive value of platelet related indicators for patent ductus arteriosus (PDA) in extremely low birth weight infants (ELBW). Methods　The data of 79 ELBW infants born from June 2013 to June 2016 were retrospective analyzed. There were 48 cases without PDA (nPDA group) and 31 cases with PDA (PDA group). Among 31 cases with PDA, there were 17 cases of non-haemodynamically significant PDA (nhsPDA group) and 14 cases of haemodynamically significant PDA (hsPDA group). The clinical feature and platelet related indicators among nPDA group, PDA group, nhsPDA group and hsPDA group were compared. Multivariate logistic regression was used to analyze the effects of various factors on the occurrence of PDA. ROC curve analysis was performed to evaluate the early predictive value of platelet related indicators for PDA. Results　Compared with the nPDA group, the PDA group had a smaller gestational age, a higher proportion of male infants, and a smaller platelet distribution width (PDW), and there were statistically significant differences in all of those (P all<0.05). Multivariate logistic regression analysis indicated that the risk of PDA was increased as the PDW was decreased (OR=1.26, 95%CI: 1.05~1.52). The ROC curve analysis showed that the best diagnostic value of PDW was 13.4 GSD, and the sensitivity of early prediction of PDA was about 67.74%, and the specificity was 68.75%. Compared with nhsPDA group, hsPDA group had a smaller gestation age, lower cesarean section rate, and there were statistically significant differences (P all<0.05). There was no significant difference in platelet related indicators between hsPDA group and nhsPDA group (P>0.05). Conclusion　PDW has certain early predictive value for PDA in ELBW. ELBW infants with PDW<13.4 GSD need to be watched closely for the occurrence of PDA.

Alstrom syndrome in children: a case report and literature review　

　CAI Qingxia, CHANG Guoying, DING Yu, LI Juan, CHENG Qing, LI Xin, WANG Jian, WANG Xiumin, SHEN Yiping

. 2017, 35(4):  278.  doi:10.3969/j.issn.1000-3606.2017.04.009

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　Objective　To analyze the clinical feature, diagnosis and treatment of Alstrom syndrome. Method　The clinical data of a case of Alstrom syndrome and the result of her ALMS1 sequencing by the two generation sequencing were retrospectively reviewed. Results　The 12 year and 10 month old female suffered from dilated cardiomyopathy, obesity, optic nerve diseases, sensorineural hearing loss, high blood glucose and irregular menstruation since one month of birth. Laboratory examination showed she had high testosterone level, hyperglycemia, hyperlipidemia and fatty liver. High-throughput sequencing confirmed there was ALMS1 gene mutation which includes hybrid frameshift mutations of c.5418delC and p.Y1807Tfs*23, and heterozygous nonsense mutation of c.10549C>T and p.Q3517*, and c.5418delC was a new variation reported for the first time. Conclusion　Alstrom syndrome is an autosomal recessive genetic disease, which is characterized by multiple organ dysfunction and metabolic syndrome, and can be diagnosed by gene detection.

Clinical features and treatment of thyroid carcinoma in children

　WANG Xiaomin, MA Shiyin, HAN Yuefeng, ZHANG Mingjie, LI Hui, CHEN Deshang, QIAN jun, TAO Xinquan

. 2017, 35(4):  282.  doi:10.3969/j.issn.1000-3606.2017.04.010

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　Objective　To explore the clinical features and treatment of thyroid carcinoma in children. Method　The clinical data of 19 children under 14 years old with thyroid carcinoma diagnosed and treated from January 2003 to January 2014 were retrospectively analyzed. Results　In 19 cases (12 males and 7 females), there were 18 cases of papillocarcinoma and one case pf follicular carcinoma. Unilateral lobectomy plus isthmectomy was performed in 6 cases, subtotal thyroidectomy in 4 cases and total thyroidectomy in 9 cases. Unilateral cervical lymph node dissection was performed in 5 cases and bilateral in 11 cases. After the operation, multiple lesions were confirmed by pathology in 9 cases, thyroid capsular invasion in 14 cases, lymphatic metastasis in 15 cases and distant metastasis in 5 cases. All the patients were treated with TSH, and 10 cases were treated with 131I after operation. The median follow-up time was 63 months. There was no death in all cases, while local residual tumor recurrence was found in 2 cases and cervical lymph node metastasis in 2 cases and distant metastasis in one case. Conclusion　Thyroid carcinoma in children is mostly well-differentiated, so the overall prognosis is better. However, children who have extracapsular invasion, multiple lesions in bilateral thyroid, cervical lymph node metastasis and distant metastasis are at high risks and should be treated with comprehensive therapy that includes total thyroidectomy.

The clinical feature and gene analysis of Cardio-facio-cutaneous syndrome in children: a report of 2 cases and literature review　

　ZHANG Huanhuan,LI Niu,YU Tingting,XU Yufei,LI Guoqiang,WANG Xiumin,SHEN Yiping,WANG Jian

. 2017, 35(4):  286.  doi:10.3969/j.issn.1000-3606.2017.04.011

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Objective　To explore the clinical phenotype and gene variation features of Cardio-facio-cutaneous syndrome (CFC). Method　DNA were extracted from peripheral blood in 2 children with CFC and their parents, and the mutation gene were detected by high throughput sequencing of target gene and verified by Sanger sequencing. Results　Two patients were Han nationality females and aged 13 months and 7.5years respectively. Both of them showed craniofacial features of epicanthus, flat nose and scant hair, and had history of growth retardation and epileptic seizure. The electrocardiogram in one case showed that there was a change in T wave and P-R interval reached the normal high limit. The electrocardiogram in the other case was normal. Genetic detection revealed that there were two heterozygous missense mutation in the third exon of MAP2K1, c.383G>T, p.Gly128Val and c.389A>G, p.Tyr130Cys, which were new mutations (de novo) and CFC mutation sites reported in the literature. Conclusion　The two cases of CFC were first reported in China and both had MAP2K1 mutations. The children with CFC were reported to be mostly complicated with heart disease, thus these 2 patients should be followed up for cardiac function.

Poorly differentiated endocardial sarcoma: a case report　

ZHU Chenguang, HE Xuehua, YUAN Yonghua, ZHU Yimin, LIU Liping, LIU Zhenyu, ZHU Qianli, ZHOU Wenwu, LI Yanchun

. 2017, 35(4):  290.  doi:10.3969/j.issn.1000-3606.2017.04.012

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　Objective　To explore the diagnosis and treatment of poorly differentiated endocardial sarcoma. Method　The clinical data of a child with poorly differentiated endocardial sarcoma was retrospectively analyzed. Results　One-year-old girl was admitted for diarrhea, polypnea, cyanosis, and cough. Abnormal heart sound was found by auscultation. Leads Ⅱ, Ⅲ, and aVF of ECG showed high peaked P wave. The diagnosis of poorly differentiated endocardial sarcoma was confirmed by echocardiography and pathology after cardiac operation. Three months after discharge from the hospital, the patient suddenly came into coma and died. Conclusion　The diagnosis of poorly differentiated endocardial sarcoma is mainly based on clinical manifestations, echocardiography and pathology. Surgical resection is the first choice and chemotherapy and radiotherapy play a supporting role. However, there is no cure for it currently.

Systemic lupus erythematosus combined with antiphospholipid syndrome and cerebral thrombosis in a child: a case report and literature review

　PAN Wei, LI Chenli, CHEN Hongbing

. 2017, 35(4):  293.  doi:10.3969/j.issn.1000-3606.2017.04.013

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　Objective　To explore the clinical features of systemic lupus erythematosus (SLE) combined with antiphospholipid syndrome (APS) and cerebral thrombosis in a child. Method　The clinical data of SLE combined with APS and cerebral thrombosis in a child was retrospectively analyzed, and the related literature was reviewed. Results　This was a 12-year-old boy. The disease onset with recurrent fever, confusion and rash in cheek. He had anemia and thrombocytopenia, and positive antinuclear antibody (ANA) and anticardiolipin antibody (aCL). Magnetic resonance imaging showed multiple cerebral infarction. The diagnosis of SLE combined with APS and cerebral thrombosis was clearly made. Methylprednisolone, cyclophosphamide, warfarin, meropenem and acyclovir were used for the treatment. At the same time, the patient also received intravenous immunoglobulin. Conclusion　SLE combined with APS and cerebral thrombosis in children was usually in a severe condition, the prognosis of which can be effectively improve by early diagnosis and reasonable treatment. 

The analysis of gene mutation and diagnosis and treatment of 5α-reductase 2 deficiency in a child

LI Ruizhen, LI Shuang, WU Jing, WANG Jun, YAO Hui, HUANG Xiaoli, CHEN Xiaohong, YANG Luhong, QIN Yuan

. 2017, 35(4):  296.  doi:10.3969/j.issn.1000-3606.2017.04.014

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　Objective　To explore the clinical feature and gene mutation in steroid 5α-reductase 2 deficiency (SRD5A2). Method　The clinical data of SRD5A2 in a child with vulva abnormality as the first manifestation was retrospectively analyzed. Results　This was a 29-month-old child, whose social gender was female. The level of her basic luteinizing hormone (LH) was 0.07 mIU/mL, and follicle-stimulating hormone was (FSH) 0.39 mIU/mL. The baseline levels of testosterone (T), dihydrotestosterone (DHT), 17-hydroxyprogesterone (17-OHP) and androstendione (A2) were 0.06 ng/mL, 19.67 pg/mL, 1.20 ng/mL, and 0.07 ng/mL respectively. Those levels were 3.65 ng/mL, 68.25 pg/mL, 51.72 ng/mL, and 14.70 ng/mL respectively after Human chorionic gonadotropin (HCG) stimulation. The levels of her anti-mullerian hormone (AMH) was 22.97 ng/mL, and inhibin B (INH-B) was 274.4 pg/mL. The uterus and ovaries were not detected by Pelvic ultrasound and MRI. The chromosome showed 46, XY. Sex determination (SRY) gene detection showed normal. Androgen receptor (AR) gene detection showed negative. There was pathogenic mutation of 5α-reductase 2 (SRD5A2) gene in peripheral blood of the child and her parents. The penis grows 2 cm after 4 months of treatment with 2.5% DHT gel. Conclusion　SRD5A2 is diagnosed mainly based on the increase of T/DHT after HCG stimulation experiment and it can be confirmed by detection of pathogenic SRD5A2 mutation.

The analysis CYBB gene mutation and prenatal diagnosis in X-linked chronic granulomatous disease　

LI Shujuan, JIANG Liping

. 2017, 35(4):  300.  doi:10.3969/j.issn.1000-3606.2017.04.015

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　Objective　To analyze the clinical feature of X-linked chronic granulomatous disease (X-CGD) and gene mutation of CYBB. Method　The clinical data of X-CGD in one child and the results of CYBB gene detection in his family were reviewed. Results　This boy had onset in the neonatal period and presented with recurrent severe pulmonary infection as his main manifestation. Results of nitroblue tetrazolium test (NBT) in both non-stimulation group and LPS stimulation group in the child were 0, and neutrophil oxidation index (NOI) was 1.15. Gene analysis showed a deletion mutation in exon 6 of CYBB gene in the child (579-582delATTA), which resulted in frameshift mutation started from coding sequence of 189—isoleucine (I) and stop codon occured in advance in the 212th amino acid (I189fsX212). Both the child's mother and grandmother were carriers of the mutated gene. The same deletion mutation was not found in the CYBB gene in the amniocyte from the mother's next child. Conclusion　One case of X-CGD patient with CYBB gene mutation and his families were diagnosed by gene detection. Prenatal diagnosis can avoid the birth of children with X-CGD.

Eosinophilic cystitis in children: a report of 7 cases and literature review

　ZHAO Shaocong, LIU Yufeng, WANG Dao, LI Bai, SU Shufang, WEI Linlin

. 2017, 35(4):  304.  doi:10.3969/j.issn.1000-3606.2017.04.016

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Objective　To explore the clinical and pathological features, diagnosis and treatment of eosinophilic cystitis in children. Method　The clinical data of 7 patients with eosinophilic cystitis admitted from 2012 to 2016 were retrospectively analyzed, and the related literature were reviewed. Results　The median age of the 7 patients was 9 years, and clinical manifestations were urgent urination, frequent micturition, odynuria, hematuria, abdominal pain and nocturnal enuresis. Ultrasonography and CT examination showed thickened bladder wall and space occupying lesions. All the 7 children received bladder biopsy, and pathology was consistent with eosinophilic cystitis. Six of them were cured after 2 months of drug therapy, and the other one was cured by repeated drug treatment for 1 year. All patients were followed up for 3 months to 4 years until the abnormal symptoms of voiding disappeared and the abnormal changes of bladder disappeared by imaging examination. Conclusion　Eosinophilic cystitis in children is a benign lesion, having extremely similar clinical manifestations to bladder tumor. Without biopsy, the diagnosis of eosinophilic cystitis can also be made according to the clinical manifestation, laboratory examination and treatment effect. The treatment for this disease mainly includes hormone, antihistamine and anti-inflammatory drugs.

Liposomal amphotericin B successfully in treatment of Kala-Azar with antimonial resistance in a child: a case report and literature review　

YUAN Chuanjie, ZHU Yu

. 2017, 35(4):  307.  doi:10.3969/j.issn.1000-3606.2017.04.017

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　Objective　To explore the treatment of kala-azar with antimonial resistance in children. Method　The clinical data of antimony resistant Kala Azar in a child was analyzed retrospectively, and the related literature were reviewed. Results　A 2-year- and 5-month-old boy, suffered from fever, pancytopenia and hepatosplenomegaly. He was diagnosed with kala-azar by bone marrow examination, and improved after the treatment of adequate antimonial. Later, he relapsed twice and the treatment of adequate  was effective.. This was the third relapse, and was considered as antimonial resistance. Liposomal amphotericin B (1 mg/kg on day 1, 2 mg/kg on day 2, 3mg/kg on day 3~7 and day 10, with accumulated dose of 21 mg/kg) was used and effective. The child improved and no relapse in one-year follow-up. Conclusion　Liposomal amphotericin B can be used in the treatment of kala-azar with antimonial resistance in children.

Effect of progesterone on the expression of O4 and O1 in the white matter of neonatal rat model with periventricular leukomalacia　

LEI Xianming, YANG Jie, CAO Yuntao

. 2017, 35(4):  311.  doi:10.3969/j.issn.1000-3606.2017.04.018

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　Objective　To explore the effect of progesterone on the expression of O4 and O1 in the white matter of neonatal rat model with periventricular leukomalacia (PVL). Methods　2-day-old neonatal SD rats were randomly divided into model group, experimental group, and sham operation group. Rats' left common carotid artery was ligated and exposed to hypoxia (8%O2+92%N2) for 0.5 h in both the model group and experimental group to build the PVL animal model. The rats in experimental group was injected intraperitoneally with progesterone 10 mg/(kg·d) immediately after cerebral hypoxia ischemia. In sham operation group, rats' left common carotid artery was only isolated without ligation and hypoxia. 1, 4, 7, and 14 days after operation, the pathological changes of brain tissue were compared among three groups. Immunohistochemical staining was used to detect the expression of O1 and O4 in the cerebral cortex of rats in three groups at different time points. Results　There were no abnormal pathological changes in the white matter in the sham operation group at each time point. The left ventricular enlargement and periventricular leukomalacia were found in both model and experimental groups, while the pathological damages of white matter in experimental group were significantly lighter than those in model group at each time point. The integral optical density (IOD) of O1 and O4 positive cells in the cerebral cortex of the three groups was gradually increased at day 1, day 4, and day 7 after operation and reached the peak level at day 7 , then was decreased at day 14 after operation. There was statistically significant difference (P<0.01). At day 1, day 4, day 7, and day 14, the integral optical density (IOD) of O1 and O4 positive cells in the cerebral cortex of sham operation group was highest, followed by experimental group and model group, and there was significant difference (P<0.01). Conclusion　Progesterone can reduce the pathological damage in the cerebral cortex in neonatal rats with PVL, and promote the expression of O1 and O4 in the periventricular white matter, which can promote the differentiation and maturation of oligodendrocytes.

Progress in Rituximab injection for treatment of refractory nephrotic syndrome in children

GAO Xia, SUN Li

. 2017, 35(4):  315.  doi:10.3969/j.issn.1000-3606.2017.04.019

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Glucocorticoid is the first choice for the treatment of nephrotic syndrome in children. But due to various reasons, nephrotic syndrome becomes hormone dependent or resistant, and progresses into refractory nephrotic syndrome in some children, which forces the clinician to constantly look for new immunosuppressants in order to alleviate the condition.,Rituximab injection (RTX) as a monoclonal antibody against CD20 has been widely used in the treatment of a variety of immune diseases in recent years. The use of RTX in refractory nephrotic syndrome in children, its main adverse effects and possible mechanisms was reviewed in this article.