OBJECTIVE:To develop an HPLC method for the determination of L-Carnosine in Polaprezine granules.METHODS:The Chromatographic column was Agilent ODS with detection wavelength of 215 nm and flow rate of 1 mL?min-1.The mobile phase consisted of sodium subsulfite buffer solution(pH=3)-acetonetrile(77:23).The column temperature was set at 30 ℃ and injection size was 10 ?L.RESULTS:The good linearity was obtained in the range of 0.1559～3.1584 ?g for L-Carnosine(r=1.0000).The average recovery was 99.65%(RSD=0.078%).CONCLUSION:The method is simple,accurate and sensitive,and it is suitable for content determination of L-Carnosine in Polaprezine granules.

Objective To observe the clinical efficacy of Yishen Huayu Decoction for decreased ovarian reserve (DOR). Methods Eighty cases of DOR were randomly divided into 2 groups, with 40 cases in each group. The treatment group was treated with oral administration of Yishen Huayu Decotion, one dose a day. The control group was given DHEA, a time of 1 tablet, 3 times a day. 2 groups were observed for 3 menstrual cycles. The levels of basic follicle stimulating hormone (bFSH), basal estradiol (bE2), luteinizing hormone (LH) and serum anti-Müllerian hormone (AMH) were measured. The number of basal follicles and the peak systolic velocity (PSV) were monitored by transvaginal sonography. Quality of life assessment and basal body temperature were measured. Results The total effective rate was 85.00% (34/40) in the treatment group and 62.50% (25/40) in the control group, with statistical significance (P<0.05). After treatment, the levels of bE2 and bFSH decreased, and the levels of AMH, ovarian antral follicles and PSV increased, which were significantly different from those before treatment (P<0.05). After treatment, the levels of bE2 and bFSH in the treatment group were lower than those in the control group, and the levels of AMH, ovarian antral follicle and PSV were higher than those in the control group, with statistical significance (P<0.05). The recovery rate of basal body temperature was 77.50%(31/40) in the treatment group and 52.50%(21/40) in the control group, with statistical significance (P<0.01). After treatment, the scores of four dimensions of life quality, daily activities, health status and self-perception were significantly improved (P<0.01), and the treatment group was higher than the control group (P<0.01). Conclution Yishen Huayu Decotion can improve the ovarian reserve function and the life quality of patients with DOR.

Objective To retrospectively analyze the clinical characteristics of 5 cases with recurrence of congenital tracheoesophageal fistula (rTEF) and to further understand the diagnosis and treatment of this disease.Methods We totally diagnosed 5 cases of rTEF from September 2015 to July 2016 in the department of respiration of Shenzhen Children′s Hospital.The clinical materials,characteristics,risk factors of recurrence and diagnostic methods were analyzed.Results Two cases were diagnosed of rTEF at 8 years after the first repair,one case was at one year after the initial repair and two cases were at 1 to 2 months after their operations.Three cases manifested mainly in bucking after feeding,recurrent pneumonia and growth retardation.One case was admitted for vomiting and abdominal distension.One case was asymptomatic.Esophageal anastomotic leaks occurred in three cases and esophageal trictures occurred in four cases after their repairs.Three cases accompanied with gastroesophageal reflux and five cases with tracheomalacia.Four cases were performed esophagography,but only two cases were suspected of recurrence.All of five cases were performed bronchoscopy.One case was diagnosed of rTEF directly.Two cases were found suspected fistulas.And another two cases were only found surgical scars.But these four cases were confirmed by Methylene blue test observed by bronchoscopy.Conclusion Although the leading clinical manifestations of rTEF are respiratory symptoms and feeding difficulties,sometimes rTEF is asymptomatic.Because rTEF is usually complicated with the other diseases with similar symptoms,it is possible to miss the diagnosis.Esophageal anastomotic leaks and trictures are the risk factors of recurrence.Bronchoscopy is the first choice for the diagnosis of rTEF,and Methylene blue test is golden standard.Thoracotomy is recommended for the treatment of rTEF.

Objective:To investigate the etiology, clinical characteristics and outcome of severe pneumonia-associated hemophagocytic lymphohistiocytosis, and to analyze the risk factors for mortality.Methods:Clinical data of patients with severe pneumonia-associated hemophagocytic lymphohistiocytosis admitted to Shenzhen Children′s Hospital from February 2009 to February 2019 were retrospectively analyzed.The data included clinical characteristics, etiology, clinical manifestations, laboratory data, treatment and outcomes of the patients.The clinical characteristics and laboratory data of the survival group and the death group were compared by independent sample t-test. Results:(1) Clinical characteristics: the patients were aged from 3 months to 8 years and 7 months, including 15 males and 15 females.Severe pneumonia-associated hemophagocytic lymphohistiocytosis accounted for 2.74% (30/1 096 cases) of severe pneumonia in the same period.(2) Etiology: Mycoplasma pneumoniae infection was found in 8 cases (8/30 cases, 26.67%), virus infection in 7 cases (7/30 cases, 23.33%, including 5 cases with adenovirus infection, 1 case with EB virus infection, and 1 case with cytomegalovirus infection), Mycoplasma pneumoniae complicated with adenovirus infection in 4 cases (4/30 cases, 13.33%), bacterial infection in 3 cases (3/30 cases, 10%), and fungal infection in 2 cases, Mycobacterium tuberculosis infection in 1 case.The pathogens were not identified in 5 patients.(3) Clinical manifestations: fever and hepatomegaly were present in all patients.Besides, 86.67% (26/30)patients had fever duration more than 10 days, 83.33% (25/30 cases) patients had cough, 76.66% (23/30 cases) patients had splenomegaly, and 33.33% (10/30 cases) patients had nervous system symptoms.Laboratory data showed varying degrees of reduction of binary and ternary systems in 80.00%(24/30 cases) of the patients.Liver function impairment was found in half of the patients, and serum ferritin and lactate dehydrogenase levels were elevated in all patients.(4) The mortality rate was 30.00% (9/30 cases). The differences in age, hypertriglyceridemia and high serum ferritin levels between the survival and death groups were significant (all P<0.05). Conclusions:Severe pneumonia-associated hemophagocytic lymphohistiocytosis is a disease with a high mortality rate.Patients with Mycoplasma pneumoniae and adenovirus pneumonia are more likely to suffer from secondary hemophagocytic lymphohistiocytosis.Younger age, hypertriglyceridemia and high serum ferritin levels are indicative of poor prognosis.

Objective To explore the characteristics of respiratory tract microbiota and its clinical significance in children with protracted bacterial bronchitis (PBB).Methods Twelve children aged from 5 months to 2 years old with PBB (PBB group) and 12 age-matched tracheomalacia(TM) children (TM group) were included in this study,who were admitted into the Respiratory Department of Shenzhen Children's Hospital.Their bronchoalveolar lavage fluid (BALF) samples were collected.Bacterial DNA was extracted from their BALF samples and the 16S rRNA V3-V4 region was sequenced by using Illumina MiSeq TMII system,and the findings were analyzed by bioinformatics methods.Results Principal component analysis revealed the difference in microbiota composition between 2 groups.Compared with TM group,PBB group exhibited lower microbial diversity:the Shannon indices were also 1.683 ± 0.703 and 2.324 ± 0.142 for PBB group and TM group respectively,and the differences were also significant(all P ＜ 0.05),and the Simpson indices were 0.416 ± 0.216 and 0.191 ± 0.025 for PBB group and TM group,respectively,and the differences were also significant (all P ＜ 0.05).The relative abundance of Actinobacteria was significantly lower in PBB group [(0.215 ± 0.228) ％] than that in TM group [(3.028 ± 0.592) ％] (P ＜ 0.01).The proportions of beneficial genera obviously decreased in PBB group,including Lactococcus [(13.464±7.319)％ in PBB group,and (44.784 ± 5.020)％ in TM group,P ＜0.01],Lactobacillus [(0.153 ±0.076)％ in PBB group,and (0.313 ±0.060)％ in TM group,P＜0.01],andArthrobacter [(0.024 ±0.018)％ in PBB group,and (2.970 ±0.584)％ in TM group,P＜0.01].On the other hand,the relative abundances of opportunistic pathogenic genera increased in PBB group significantly,including Haemophilus [(14.319 ± 29.532) ％ in PBB group,and (0.047 ± 0.127) ％ in TM group,P ＜ 0.Ol],Pseudomonas [(10.406 ± 25.439) ％ in PBB group,and (7.228 ± 0.948) ％ in TM group,P ＜ 0.01],and Escherichia [(0.432 ±0.441)％ in PBB group,and (0.055 ±0.035)％ in TM group,P ＜0.01].Conclusion These findings confirmed the existence of respiratory tract microbiotia dysbiosis in PBB,which probably was one of the pathogenetic mechanisms for PBB.

Objective: To summarize the clinical features of immunodeficiency diseases with interstitial lung disease (ILD) as major clinical manifestations and to improve understanding etiology of ILD. Methods: The clinical features and clinical clues for diagnosis of six cases with immunodeficiency presented with ILD in Shenzhen Children′s Hospital from January 2014 to December 2016 were retrospectively analyzed. Results: The patients′ age ranged from 3 months to 5 years and 9 months, 5 cases were male. All cases had cough and tachypnea, 3 cases had lung infection and respiratory failure, 2 cases had chronic hypoxia and one had clubbing. Three cases had skin rashes; 5 cases had failure to thrive. Chest CT scan showed diffuse ground glass opacity in all the 6 cases, and 2 cases had cystic changes and one had "crazy-paving" pattern. Five patients were suspected to have surfactant dysfunction and genetic testing was performed before diagnosis of immunodeficiency, of which the results were negative. With human immunodeficiency virus antibody test or immunologic laboratory testing and/or immune genetic panel, acquired immune deficiency syndrome was confirmed in one case, hyper-IgM syndrome was confirmed in two cases and hyper-IgE syndrome in one case, Wiskott-Aldrich syndrome in one and STAT3 gain of function genetic mutation in another. All cases had clinical clues indicative of underlying immunocompromise. Conclusions The clinical features of immunodeficiency diseases with ILD are cough, tachypnea or hypoxia, respiratory failure with infection, diffuse ground glass opacity in Chest CT imaging. With thorough medical history and immunology screening, there would be clinical clues indicative of underlying immunocompromise. Screening for immunodeficiency disease should be emphasized in the differential diagnosis of ILD, otherwise it may lead to misdiagnosis or unnecessary testing.