Objective: To describe the growth velocity percentile curves under breast feeding for 3 months for healthy breastfed infants aged 1 to 2 months, which can provide a more comprehensive reference for the evaluation of infant growth and development. Methods: A multi-center randomized clinical trial was performed. 1~2 months old infants (gestational age ≥37 weeks) fed with formula milk only for mothers' own problems presented in child health clinics of six study settings were included in formula powder group (and then randomly assigned to commercially available formula subgroup 1 or 2); In the same study setting, 1~2 months old infants (gestational age ≥37 weeks) with exclusively breast feeding matched by gender and body weight with formula powder group were included in breast feeding group. The primary outcomes were growth velocity of height, body weight and head circumference at 1, 2 and 3 months. The monthly growth velocity was equal to ［(measured value at this actual follow-up time measured value at last actual follow up time) / (this actual follow-up time the last actual follow up time)d］ ×30. The secondary outcomes were ①the stool character and defecation frequency at the enrolment and 1, 2 and 3 months after enrolment. The parents collected fresh stool and snapped photos on their phones and the stool characters were judged according to Bristol stool shape chart. ②Incidence of adverse events and serious adverse events over the trial period. Full analysis set (FAS) for effectiveness analysis and evaluation of defecation frequency and stool character and safety set (SS) for adverse events were performed. The coefficient of skewness median coefficient of variation (LMS) method was applied to calculate smoothed height, body weight and head circumference percentiles (P3、P5、P10、P25、P50、P75、P90、P95、P97) at 1, 2, 3 months by LMS software. Results: From May 6, 2016 to October 30, 2016, 152 infants were included in formula powder group and 72 in breast feeding group respectively (SS). The numbers of drop-out were 24 in formula powder group and 3 in breast feeding group. The numbers of participants included in FAS were 128 for formula powder group and 69 for breast feeding group respectively. There was no significant difference in gender, day age at enrolment, height, body weight, gestational age and delivery mode between two groups (FAS). ①In breast feeding group, the growth velocity of height was 3.1±1.4 cm at 1 month, 3.0±0.8 cm at 2 months and 2.7±0.6 cm at 3 months, which showed a downward trend month by month; the growth velocity of body weight was 951±371 g at 1 month, 824±278 g at 2 months and 749±219 g at 3 months, which showed a downward trend month by month; the growth velocity of head circumference was 1.5±0.7 cm at 1 month, 1.4±0.5 cm at 2 months and 1.3±0.3 cm at 3 months, which showed a slow downward trend month by month. ② No significant difference was found between formula powder group and breast feeding group in the growth velocity of height, body weight and circumference at 1, 2 and 3 months respectively. The growth velocity of height, body weight and circumference in formula powder group also showed a downward trend month by month. ③There was significant difference in both defecation frequency and stool character at enrolment, 1, 2 and 3 months between formula powder group and breast feeding group. ④None of adverse events was considered directly attributable to formula powder or breast feeding in totally 224 infants during the study period. Conclusion: The growth velocity of height, body weight and head circumference under breast feeding for 3 months of Chinese healthy breast feeding infants aged 1~2 months showed showed a downward trend month by month. The downward change month by month of growth velocity in head circumference was slower than that of height and body weight.The growth velocity trend in formula powder feeding infants was similar to that in breast feeding infants.

Objective: To compare the efficacy and adverse effects of CHFU-LCH 2006 protocol (simply called 2006 protocol) to CHFU-LCH 2012 protocol［simply called 2012 protocol, which was updated to simplify groups, remove methotrexate (MTX) and unify treatment time to 12 months］ formulated by Children's Hospital of Fudan University (our hospital) to treat Langerhans cell histiocytosis (LCH). Methods: Newly diagnosed childhood LCH patients, who were treated in our hospital from January 1, 2006 to November 31, 2012, were enrolled in 2006 protocol and patients from December 1, 2012 to December 31, 2015 were enrolled for 2012 protocol. Diagnosis of patients in both groups was made by pathology. Children who did not complete the initial 6-week treatment were excluded. The subjects were further divided into single-system LCH (SS-LCH) and multisystem LCH (MS-LCH) subgroups. All patients were followed up until March 31, 2017. Effective treatment outcomes were categorized as non active disease (NAD) or active disease better (AD Better). The 5-year expected overall survival rate (OS) and event-free survival rate (EFS) were estimated by Kaplan-Meier analysis. Adverse reactions to chemotherapy were classified into grade 0~4 according to WHO criteria for acute and subacute toxicity. Response to chemotherapy between the two groups were compared at 6 and 12 weeks. In addition, we followed and compared disease progression, recurrence and mortality and the 5-year expected OS, EFS, as well as occurrence of adverse reaction between the two groups. Results: There were 96 patients (64 boys and 32 girls, median age 3.4 years, median follow-up duration 6.6 years) enrolled for 2006 protocol. In 2012 group, 86 patients (59 boys and 27 girls, median age 2.9 years, median follow-up duration 3.7 years) were included. There was no significant difference between the two groups in gender, age of diagnosis, clinical classification and risk organ involvement (RO+). ① There was no significant difference in the response to therapy at 6 and 12 weeks and disease recurrence in either MS-LCH or SS-LCH subgroups between 2006 group and 2012 group; ② Among patients in MS-LCH subgroup, there were 4 cases and 5 cases respectively in 2006 group and 2012 group quitting the protocol and were transferred to other rescue protocols. 5 cases and 4 cases died, respectively in the two groups. ③ There were totally 93 children categorized as MS-LCH in our study. The rate of EFS and OS in patients with MS-LCH younger than 2 years were significantly lower than those older than 2 years ［EFS: (41.9±8.1) % vs (62.6±7.5) %; OS: (80.8±6.2) % vs (98.0±2.0) %, respectively］, P<0.05. The rate of EFS and OS among RO+ MS-LCH patients were also significantly lower than RO- MS-LCH patients ［EFS: (37.4±8.0) % vs (66.0±7.3) %; OS: (80.4±6.3)% vs (98.0±2.0)%］, P<0.05. Among RO- MS-LCH patients, the rate of EFS and OS were not significantly different between children younger than 2 years and those older than 2 years. The EFS rate was significantly lower in children who did not respond to the initial 6-week therapy than those responded ［(33.1±7.9) % vs (70.8±7.2) %］, P<0.05. ④ The 5-year expected EFS of SS-LCH subgroup was (84.8±5.3) % and (86.7±5.6) % for 2006 and 2012 groups, the 5-year expected OS was 100% in both treatment groups. The 5-year expected EFS of MS-LCH subgroup was (50.0±7.1) % and (53.2±10.0) %, the 5-year expected OS was (90.0±4.1) % and (90.6±4.5) % for the 2006 and 2012 group and there was no statistical difference identified between the two treatment groups. ⑤ The Grade 3 and 4 chemotherapy related adverse reactions occurred in 50% (25/50) of patients with MS-LCH in the 2006 group, which was significantly higher than 23.3% (10/43) in 2012 group (P=0.0080). Conclusion: The CHFU-LCH 2012 protocol was not inferior to 2006 protocol with less adverse reaction to chemotherapy.

objective: To systematically review the association between birth weight with risk of the chronic kidney disease. Methods: The Cochrane Library, MEDLINE, OVID, Springer, VIP, WangFang Data and CNKI up to June 30, 2016 were searched to retrieve the online observation studies about birth weight and chronic kidney disease and other relative outcomes. The articles were selected if they were published in English or Chinese regarding the association between birth weight and CKD, which included the exact information on OR and 95%CI. Excluded studies: Studies that assessed the final outcome among neonates or children less than 1 year old, the participants of studies who were infected in utero, congenital abnormalities, the mothers who were exposed to toxins, as well as the incompletely information literature. Two reviewers inspected the included studies on the association between birth weight and chronic kidney disease independently. Quality assessment was performed based on the Newcastle Ottawa Scale( case-control study and cohort study) and AHRQ（cross-sectional study）. OR and 95%CI were extracted from the selected articles and then analyzed with Stata 12.0 software by the way of DerSimonian & Laird. Results: Ten observational studies containing four casecontrol studies(three articles in 8 points and one article in 7 points ), four cohort studies(three articles in 7 points and one article in 8 points ) and two crosssectional studies(7 points and 8 points for the two articles ) were selected finally. The overall combination of weighted estimates from the selected 10 studies about low birth weight associated with risk of the chronic kidney disease was 1.80 (1.37-2.35). The risks of albuminuria (OR, 2.58; 95% CI, 1.49-4.46), endstage renal disease (OR, 1.42; 95% CI, 1.22-1.66), or lower estimated glomerular filtration rate (OR, 1.87; 95% CI, 1.19-2.94) were similar with the chronic kidney disease. However,according to the subgroup analysis that the combined OR of high birth weight between endstage renal disease and lower estimated glomerular filtration rate was OR, 1.19; 95% CI, 0.94-1.49 and OR, 0.09; 95% CI, 0.93-1.27,which showed no significant impact. In the subgroup analysis by gender,increased risk was only found in men:1.83 (1.10-3.05),unexpectedly.Finally, the Egger regression method showed no publication bias.Conclusion: Only men with low birth weight had a greater CKD risk in later life. And the high birth weight did not show any significant impact on CKD.

objective: To examine the use of peripherally inserted central catheters（PICCs） in a tertiary pediatric setting. Methods: The recruited patients with PICC line for intermediate- to long- term fluid infusion were collected from the Pulmonology and Gastroenterology Departments of Children's Hospital of Fudan University from Dec. 2015 to Feb. 2017. Data of indications, complications, efficacy of PICC and reasons for catheter removal were collected and analyzed. Results: 60 cases with PICC insertions（1 case was a child with PICC contraindication） were included in the analysis. There were 38 male cases and 22 female cases with age of 34 days to 14 years. The indications for PICC insertion included long-term parenteral nutrition(45/60, 75.0%),expected long-term（＞6 days）intravenous medication infusion（9/60, 15.0%）and insufficiency of peripheral venous access（6/60, 10%）.Catheter indwelling time was （17.2±10.7）d, while total indwelling time was 1 033 catheter days. Complications occurred in 22 cases（22.3/1000 catheter days）, including 7 malposition, 6 catheter blockage, 3 phlebitis, 3 catheter breakage, 2 arterial puncture, 1 vasospasm, 1 case of failing venous reflux. 42 cases（70%） were planned on catheter removal，while 5 cases（8.3%） were unplanned on catheter removal, 13 cases（21.7%）were transferred to the other ward or discharged with the catheter. The average PICC procedure time was (68.2±43.5)min, and catheterrelated expenditure/hospitalization expenditure ratio was 0.07. Conclusion: As an applicable venous access, PICCs provide convenience in patients requiring intermediate- to long- term intravenous infusion. Meticulous post procedure care is important for minimizing unexpected line removal and catheter related complications.

objective: To systematically review the effectiveness of music intervention on the regulation of physiological parameters of preterm infants during hospitalization. Methods: Databases including CNKI, WANFANG，Pubmed, CINAHL, Embase and Cochrane Library were electronically searched for the randomized controlled trials(RCTs) on the effect of music intervention on the physiological parameters of hospitalized preterm infants from inception to April 18, 2017. Participants were stable preterm infants with normal hearing. The experimental group was treated with music intervention (without limitation of music type, intervention time, frequency and duration). During the intervention, premature infants did not receive any invasive stimuli, and were in a calm state. The control group did not receive any music intervention. Outcome indicators included oxygen saturation, heart rate, respiratory frequency and other physiological indicators. The methodological quality of the literatures was evaluated by JBI literature quality evaluation tool and data were analyzed by RevMan 5.3 software. Results: A total of nine studies met the inclusion and exclusion criteria, and five of them with quality A or B (all in English) were analyzed. All the five studies reported oxygen saturation, and four reported heart rate and respiratory rate. Metaanalysis showed that the difference of oxygen saturation, heart rate and respiration rate of preterm infants was not statistically significant between the intervention group and the control group. According to the different types of music and different forms of music implementation, subgroup analysis of lullaby, recorded music and live music were conducted. Results showed that the oxygen saturation of the recorded music group was higher than that of the control group ［MD=0.81, 95% CI: 0.03-1.59), Z=2.05, P=0.04］ and there was no significant difference in oxygen saturation, heart rate and respiratory rate between the other subgroups and the control group. Conclusion: The effect of music intervention on regulating the vital signs of premature infants is still unclear. In view of the absence of RCTs with good quality in this field, it is necessary to carry out more largescale and highquality RCTs to further explore the effectiveness of music intervention on physical parameters of hospitalized preterm infants.

Objective: To compare the mean scores of norm groups in China and US for children aged 1 to 66 months using Ages and Stages Questionnaires, Third Edition (ASQ-3), and examine the differences in development of young children in these two countries, including a discussion of plausible reasons for these differences. Methods: Parents of children in China norm group completed the pencilpaper version of ASQ-3; and parents of US norm group either completed the pencil-paper version or the web version of ASQ-3. The mean scores of the children aged 1 to 66 months in the two norm groups on five developmental domains (i.e., communication, gross Motor, fine Motor, problem solving, and personal social) were compared. Data were analyzed using SAS 9.3 and MS Excel. Results: China norm group included 4,452 young children, and within each age interval,218 to 227 young children. A sample of 18,572 young children was recruited for US norm group, 352 to 2,088 young children in each age interval. The results showed that the mean scores of the two norm groups were significantly different on11 age intervals (55%) in communication domain. US norm group scored higher than China norm group at 2, 4, 6, 8, and 10 months; there were no major differences between the two groups between12-20 months;and China norm group scored higher between 22-54 months. In gross motor domain, significant differences were found in 15 age intervals (75%). US norm group scored higher than China norm group in age intervals before 20 months; the two groups performed at similar level between 22 and 30 months;and China norm group outperformed US norm group in all age intervals after 33 months. In fine motor domain, the scores of the two norm groups were significantly different in 14 age intervals (70%), and within the 14 age intervals, China norm group scored significantly higher than US norm group only at 54-month age interval. The scores of US norm group were higher than those of China norm group in all age intervals before 24 months. Though the two groups scored at a similar level in age intervals after 24 months, the scores of US norm group were slightly higher. In problem solving domain, significant differences were found in 15 age intervals between the two norm groups (75%), and US norm group scored higher than China norm group in all age intervals except 54 months. In personal-social domain, significant differences were found in 17 age intervals (85%). US norm group performed better than China norm group in the age intervals before 14 months. From 16 to 36 months age intervals, US norm group outperformed China norm group in five age intervals, but China norm group scored higher in the age intervals after 42 months. Conclusion: Young children in China norm group scored significantly lower than their peers in US norm group on ASQ-3 in the age intervals younger than 24 months though their scores increased and eventually were similar or higher than the US norm group at older intervals. It is of great importance to pay close attention to early development of young children, since developmental delays at an early age may have a far more significant impact on the child, family, and our society than we could imagine.

Objective: To investigate differences and characteristics of the development of children aged 1 to 66 months in urban and rural areas in China.MethodsData from the national norm group of Ages and Stages Questionnaires (Third Edition, Chinese) (ASQ-3) were used to compare the development of young children in urban and rural areas of China in the five developmental domains: communication, gross motor, fine motor, problem solving, and personal social. Twenty age intervals from 1 to 66 months were used for comparison. Data were analyzed using SAS 9.3 and MS Excel. Results: The urban group included 2,236 young children and the rural group included 2,216 young children, with 108-114 young children in each age interval. In communication domain, the urban group scored higher than the rural group in 19 age intervals, and significant differences were found in 6 age intervals. In gross motor domain, the urban group outperformed the rural group in 18 age intervals, and significant differences were found in 8 age intervals. In fine motor domain, the urban group scored higher than the rural group in 18 age intervals, and significant differences were found in 7 age intervals. The urban group scored higher than the rural group in all age intervals in problem solving domain, and significance were found in 11 age intervals. In personal social domain, the urban group scored higher than the rural group in 14 age intervals, and in 3 of them the differences were significant. When comparing the total score, the urban group outperformed the rural group in all age intervals, especially in age intervals older than 26 months; and 11 of the comparisons were significantly different. Conclusion: There were differences between young children's development in urban and rural areas in China. Young children in rural group scored lower than those in urban group in the same age interval. Using ASQ-3 is simple and convenient for children in rural areas, because it may help promote the use of parent-completed screening tools, and increase the awareness of early development of young children.

objective: To develop a Clinic Readiness to Evidencebased Nursing Assessment（CREBNA）scale and test its reliability and validity. Methods: According to the qualitative interviews, scoping review of promoting action on research implemrntation in health severice (PARIHS) model results obtained primary item pool of CREBNA. After two rounds of experts evaluation，the CREBNA item pool of EBNP readiness was established, the questionnaire was formed based on item pool, the test version of CREBNA was identified by experts, judgment of the relevance between the item and dimensions in the expected structure, and the content validity of questionnaire. Convenience sampling was used in the research, clinical nurses who were participants in training course of Fudan University School of Nursing in 2016 were selected as investigators. The dimensions, reliability and validity of this scale were tested by exploratory factor analysis and reliability analysis. Results: The CREBNA item pool included 3 dimensions,36 items. CREBNA questionnaire of 36 entries was formed based on item pool, the S-CVI of scale was 0.976. 256 questionnaires were included in the final analysis，the correlations between items and the scale total score ranged from 0.482-0.781（P＜0.01），3 factors were extracted by exploratory factor analysis and labeled as Evidence, Context, Facilitation, 62.524% of total variance were explained. The formal scale contained 31 items, The Cronbach α coefficients were 0.959 for the total scale and 0.915-0.940 for the subscales. The testretest reliability was 0.917 and splithalf reliability coefficient was 0.978 for the total questionnaire. Conclusion: It suggested that the CREBNA scale was reliable and valid enough to be applicable to evaluate the evidencebased practice process.

objective: The present study examined the prevalent trends in overweight and obesity among schoolage children and adolescents over the past 29 years(1985-2014) in Shanghai.Methods： Data for this study were obtained from six crosssectional surveys(1985,1995,2000,2005,2010 and 2014) of school children in Shanghai. The sample sizes were 14 677, 7 916, 15 270, 7 492, 14 301, 15 423, respectively. It was adopted that body mass index (BMI) percentile criteria of Working Group on Obesity in China(WGOC) for screening overweight and obesity in Chinese children and adolescents, analysis of the survey data of students' physical and health of 1985-2014 in 7-18 boys and grils in the prevalence of overweight and obesity and the average growth rate. International comparison was based on the US Centers for Disease Control and Prevention(CDC) BMIforage growth charts. Results: General survey of 6 districts of Shanghai city was based on the sample size, in 10 years(1985,1995, 2005 and 2014) for a period of time, with the increase of age, the students(7 to 18 years old) BMI P50 percentile increased year by year, the overall increase was more obvious in boys than girls, the mean of BMI showed an upward trend in different age groups and was higher in boys than girls. The overall trend of Shanghai and the all nation in 2014, BMI P50 of male and female students of Shanghai were higher than the national level, P85 and P95 of male and female students were coincidence with national level before 11 and 12 years old , then higher than the national level. From 1985 to 2014, the prevalence rate of overweight and obesity among male and female students in Shanghai from 7 to 18 years old showed an upward trend, Compared with 1985, the boys prevalence rate of overweight increased 11 times(19.3% vs 1.7%), the prevalence of obesity increased 29 times(11.7% vs 0.4%), the average growth rate was 12.3%; the girls prevalence rate of overweight increased 7 times(11.6% vs 1.5%), the prevalence of obesity increased 31 times(6.4% vs 0.2%), the average growth rate was 12.7%. The boys prevalence rate of overweight was between 14.3% and 26.9%, the prevalence rate of obesity was between 7.3% and 17.0%, among all age groups , and the prevalence rate of overweight and obesity reached the highest at 11 years old. Among all age groups the girls prevalence rate of overweight was between 8.6% and 14.0%, the prevalence rate of obesity was between 2.8%-11.2%, and the prevalence rate of overweight and obesity was the highest at 7 years old. There was gender difference in prevalence rate of overweight and obesity, boys were higher than girls. Conclusion: The prevalence of overweight and obesity among children and adolescents in Shanghai increased rapidly between 1985 and 2014. The prevalence of overweight in boys with low age was more serious, and it was at the international high level. It is recommended to increase the relevant evidence, to develop targeted prevention and control measures.

Abstract objective: To investigate the diagnostic criteria of hypoalbuminemia in nephrotic syndrome in children. Methods: Data from children with idiopathic nephrotic syndrome hospitalized in Peking University First Hospital during January of 1993 to December of 2012 were collected and analyzed retrospectively. Children were divided into 2 groups, including a group with serum albumin 25 to 30 g·L^-1 and another group with serum albumin < 25 g·L^-1at onset. Data on children's general information, renal pathology, clinical presentations, treatment, steroid response, serious complications and prognosis were compared between two groups. Results: Data from 458 children were analyzed including 28 cases in group with serum albumin 25 to 30 g·L^-1 and 430 cases in group with serum albumin less than 25 g·L^-1. No significant differences were found in gender and onset age between two groups. ①Twelve children from group with serum albumin <25 g·L^-1 and 141 children from group with serum albumin 25 to 30 g·L^-1 underwent renal biopsy. There was no significant difference in the renal pathological diagnosis type between two groups. Focal segmental glomerulosclerosis was the most common pathological change in both groups. Minimal change disease or minor change disease accounted for 21.9% in group with serum albumin < 25 g·L^-1 and 8.3% in group with serum albumin 25 to 30 g·L^-1. ② There were no significant differences between two groups in the clinical phenotype, the kinds of immunosuppressant, the occurrence of severe complications and the incidence of steroid related adverse events. There was a significant difference in steroid response between the two groups . The steroid response rate was 68.4%（294/430）in group with serum albumin < 25 g·L^-1 and 50.0%（14/28）in group with serum albumin 25 to 30 g·L^-1. ③The occurrence of serious prognosis in group with serum albumin 25 to 30 g·L^-1 was significantly higher than in group with serum albumin < 25 g·L^-1（14.3% vs 4.4%，P=0.04）. Conclusion: Children with heavy proteinuria and serum albumin 25 to 30 g·L^-1 manifested mainly with non minimal /minor change disease and more serious prognosis. Early renal biopsy should be performed on them.

objective: To investigate the clinical and genetic features of Kabuki syndrome caused by KMT2D mutation and summarize the clinical features in neonate. Methods: Using Whole-Exome Sequencing (WES) and Clinical panel deep sequencing，combined with data analysis pipeline established by molecular diagnostic center of Children's Hospital of Fudan University, the clinical and molecular features of 6 children with KMT2D mutations were summarized. Databases including PubMed，CNKI，WanFang Database and VIP were searched to collect literature of KS, which describe the clinical features of neonatal period from April 2012 to April 2017. Results: Four males and two females were diagnosed as KS. Three cases were diagnosed by WES due to KS related manifestations were present and the families came to order trio-WES. One case was diagnosed by clinical neonatal panel screening. Another two cases were diagnosed by WES. Seven heterozygous mutations were detected in six patients with KS，mutations were located in exon 11, exon 39, exon 51 and exon 53 respectively. The types of mutations were one stop gained, four missenses and two frameshifts. Mutation of c.12697C>T（p.Q4233X）、c.16498C>T（p.R5500W）、c.16273G>A（p.E5425K）were reported as pathogenic mutations and had recorded in Human Gene Mutation Database (HGMD). Mutation of c.12696G>T(p.Q4232H), c.3495delC (p.Pro1165LeufsTer47), c.10881delT （p.Leu3627Argfs Ter31）and c.12560G>A（p.G418E）were novel, which predicted as harmful variants by SIFT, polyphen 2 and MutationTaster software. In a total of 18 literatures，together with the 2 cases in this study, there were 34 neonates were included. The most common clinical features were as follows: feeding problem was in 19cases, cardiac dysplasia in 20 cases, special appearance in 17 cases, skeletal dysplasia in 15 cases, hypoglycemia in 10 cases and hypotonia in 9 cases. Conclusion: The typical clinical features of KS are not shown in the neonatal period. This disease should be considered when the newborn has feeding problem, abnormal cardiac morphololy, special appearance and other clinical features. Genetic test can help to diagnose earlier in clinical. Early diagnosis can provide accurate information to clinic, may help patients to acquire appropriate treatment and family genetic counseling.

Objective: To report the first case of hydroxychloroquine sulfate treatment for surfactant protein C gene (SFTPC) mutation caused infantile interstitial lung disease. Methods: The clinical data,diagnostic process and curative effects of hydroxychloroquine sulfate in the treatment of surfactant protein C gene mutation with the infantile interstitial lung disease and related literatures were reviewed. Results: A 2-months-old female baby, presented with coughing and shortness of breath for 2 months, was admitted to hospital on September 9th, 2015.The case developed respiratory distress in neonatal period and required supplemental oxygen persistently.Imaging showed that there were lung shadows,various etiology examinations were negative, conventional anti infective therapy was ineffective,family history of pulmonary diseases was negative.A heterozygous missense SP-C mutation SFTPC （exon4）:c.T337C，p.Y113H was identified which had not been reported yet. The patient was treated with hydroxychloroquine sulfate from 13 months of age.After 6 months treatment,respiratory distress,growth and chest CT were improved significantly.The retrieval of interstitial lung disease with mutations in the SFTPC gene was made in PubMed,Web of Science,Chinese CNKI,VIP database and Wanfang database.From the establishments of these databases to December 2016,a total of 12 articles were retrieved, all of them were foreign literatures.Including 1 case of this article, a total of 51 patients with SFTPC mutations which were treated by hydroxychloroquine sulfate were reported and followed up for 0.3-15.8 years.There were good effects in all 12 patients(100%),who were treated with hydroxychloroquine sulfate only,and without adverse drug reactions or no mentions.39 cases were treated with systemic corticosteroids combined with hydroxychloroquine sulfate.Among them, 33 cases were improved(84.6%), 2 cases(5.1%) not improved,4 cases(10.3%) deteriorated (1 case died). Conclusion: Early detections and early diagnoses are very important for these SFTPC gene mutations in infants with interstitial lung diseases.Early treatment of hydroxychloroquine sulfate will be beneficial to improve the growth and development,clinical symptoms and signs, and reduce the incidence of end-stage lung.

Objective: To study the clinical features of a girl with noncompaction cardiomyopathy complicated with multiple coronary artery-to-right ventricle fistula and review the literatures. Methods: Clinical symptoms and assistant examinations such as electrocardiogram, echocardiography and cardiac catheter of the case diagnosed as noncompaction cardiomyopathy complicated with multiple coronary artery-to-right ventricle fistula were reviewed. Two cases of noncompaction cardiomyopathy complicated with coronary artery to ventricle fistula and part of patients with cardiomyopathy complicated with coronary artery to ventricle fistula reported since 2003 were also reviewed in the study. Results: (1)A 6 month girl with poor appetite and night sweat was admitted to our hospital. Transthoracic echocardiography revealed a moderately impaired left ventricular contractility (ejection fraction 34% calculated according to Simpson's rule) and hypertrabeculation of the left ventricular latero-apical region with a maximal ratio of noncompacted to compacted myocardium as 2.5, supporting the diagnosis of noncompaction cardiomyopathy. The color Doppler and contrast echocardiogram showed an unusually evident diastolic flow within the compacted layer of the myocardium. The coronary angiography showed the right ventricle was opaque after left and right coronary injections, disclosing multiple coronary right ventricular fistulae. The patient accepted digoxin, furosemidum, spironolactone treatment and the clinical symptom was improved. （2）Retrospective review of the literatures showed two cases of noncompaction cardiomyopathy complicated with coronary artery to ventricle fistula. These patients were adults and showed symptoms as dyspnea and angina pectoris. Doctors made a definite diagnosis through the examination of echocardiography and coronary angiography. One case of cardiomyopathy complicated with coronary artery to ventricle fistula accepted implantable cardioverter defibrillatror because of syncope. Conclusion: Noncompaction cardiomyopathy associated with multiple coronary artery-to-right ventricle fistulas is one of the rare disease. The clinical symptoms mainly focus on congestive heart failure such as poor appetite and night sweat in infant. The echocardiography and coronary angiography is helpful to make a diagnosis. The medicine such as digitalis, diuretic and angiotensin converting enzyme inhibitors can be used to improve symptoms.