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1.
Zhonghua Er Ke Za Zhi ; 59(9): 777-781, 2021 Sep 02.
Artigo em Chinês | MEDLINE | ID: mdl-34645219

RESUMO

Objective: To explore the early clinical clues for diagnosis of chronic granulomatous disease (CGD). Methods: One hundred and thirty-nine children with CGD seen in Beijing Children's Hospital from January 2007 to October 2020 were included in this study. The clinical features including age of onset, first presentations, reason for being hospitalized, etiology, imaging features, clues for early diagnosis of all patients were evaluated retrospectively. According to the time of diagnosis, the patients were divided into two groups, cases diagnosed before 2015 and after 2015 and 2015. The time of diagnosis, the length of stay and the hospital charges were compared between the two groups. T test and χ2 test were used for statistical analyses. Results: One hundred and nineteen of the cases were males and 20 were females. The age of onset was 4 months (8 d to 14 years), and 103 cases (74.1%) had onset before 1 year of age. The age at diagnosis was 1.8 years (21 d to 14.7 years), and the time of diagnosis delay was 1 year (7 d to 13.7 years). One hundred and thirty-five cases (97.1%) had pulmonary infection as the main reason for hospitalization, of whom 76 cases (56.3%) had positive pulmonary etiology. One hundred and thirty-six patients (97.8%) were referred cases, of whom 5 were suspected of CGD before referral, and the misdiagnosis rate was as high as 96.3% (131/136). Eight early clues for diagnosis were found, the frequency from high to low, large bacillus Callmette-Guer scar in 99 cases (70.5%), left axillary lymphadenopathy or calcification in 73 cases (52.5%), skin or other lymph node infections in 58 cases (41.7%), skin scars in 50 cases (36.0%), multiple lung nodules in 42 cases (30.2%), perianal abscess in 35 cases (25.2%), pulmonary Aspergillus infection in 26 cases (18.7%) and pulmonary Burkholderia infection in 15 cases (10.8%). A total of 120 cases of CGD were diagnosed by respiratory burst test during hospitalization, including 55 cases diagnosed before 2015 and 65 cases diagnosed after 2015. After using these 8 early diagnosis clues, the cases diagnosed after 2015 had shorter time of diagnosis and the length of stay and lower hospitalization charge than cases diagnosed before 2014, and the difference was statistically significant ((25±7) vs. (10±5) d, (29±7) vs. (18±6) d, (3.7×104±1.2×104) vs. (3.2×104±1.2×104) Yuan, t=13.763, 9.262, 2.381, all P<0.05). Conclusions: Patients with CGD are younger at onset and the diagnosis is delayed. Pulmonary infections are the most common. Large BCG scar, left axillary lymphadenopathy or calcification, skin or other lymph node infections, skin scars, multiple lung nodules, perianal abscesses, pulmonary Aspergillus infection and Burkholderia infection can help early diagnosis of CGD.

2.
Zhonghua Er Ke Za Zhi ; 59(10): 871-875, 2021 Oct 02.
Artigo em Chinês | MEDLINE | ID: mdl-34587685

RESUMO

Objective: To investigate the clinical characteristics, treatment and prognosis of congenital pulmonary airway malformation (CPAM) complicated with chronic pulmonary aspergillosis (CPA) in children. Methods: The clinical manifestation, laboratory test, radiological feature, treatment and prognosis of 4 pediatric patients diagnosis of CPAM with CPA ascertained between March 2016 and April 2020 at the Department of No.2 Respiratory Medicine and Thoracic Surgery of Beijing Children's Hospital were retrospectively analyzed. Results: The 4 children included 2 males and 2 females, their age ranged from 9.9 to 13.6 years. Cough presented in 3 cases, hemoptysis in 2 cases, whereas in 1 case, pneumothorax was the first manifestation of the condition. Past history revealed multiple hospital admissions for pneumonia in 2 children and 1 with more than 2 episodes of wheezing. Aspergillus IgG was positive in 3 patients. Significantly elevated total IgE and fumigatus-specific IgE levels were noted in 2 children. CT demonstrated multiple cystic lesions, and fungal balls were seen in 2 children. They all underwent lobectomy and the lung tissue from the 4 children all demonstrated CPAM with Aspergillus infection. Aspergillus fumigatus was isolated from the abscess collected during operation in 1 patient. Postoperative, voriconazole was given in 3 children for 8 weeks, recurrence was observed in 2 children with characteristics of allergic bronchopulmonary aspergillosis. Voriconazole was given in one patient for 12 weeks with no recurrence. Conclusions: CPAM might be complicated with CPA,if there are hemoptysis, and fungal ball in chest CT, cautious preoperative examinations for microorganism are necessary. The necessity and duration of postoperative antifungal therapy need to be determined on individual basis.


Assuntos
Aspergilose Broncopulmonar Alérgica , Aspergilose Pulmonar , Adolescente , Aspergilose Broncopulmonar Alérgica/complicações , Aspergilose Broncopulmonar Alérgica/diagnóstico , Aspergillus fumigatus , Criança , Feminino , Humanos , Masculino , Aspergilose Pulmonar/complicações , Estudos Retrospectivos , Voriconazol
3.
Zhonghua Er Ke Za Zhi ; 59(8): 658-664, 2021 Aug 02.
Artigo em Chinês | MEDLINE | ID: mdl-34333918

RESUMO

Objective: To investigate the etiology of necrotizing pneumonia (NP) in children and the clinical characteristics of NP caused by different pathogens in China. Methods: A retrospective, case-control study was performed in children with NP who were admitted to 13 hospitals in China from January 2008 to December 2019. The demographic and clinical information, laboratory data, etiological and radiological findings were analyzed. The data were divided into three groups based on the following years: 2008-2011, 2012-2015 and 2016-2019, and the distribution characteristics of the pathogens in different period were compared. Meanwhile, the pathogens of pediatric NP in the southern and northern China were compared. And the clinical characteristics of the Mycoplasma pneumoniae (MP) NP and the bacterial NP were also compared. T-test or Mann-Whitney nonparametric test was used for comparison of numerical variables, and χ2 test was used for categorical variables. Results: A total of 494 children with NP were enrolled, the median ages were 4.7 (0.1-15.3) years, including 272 boys and 222 girls. Among these patients, pathogens were identified in 347 cases and the pathogen was unclear in the remaining 147 cases. The main pathogens were MP (238 cases), Streptococcus pneumoniae (SP) (61 cases), Staphylococcus aureus (SA) (51 cases), Pseudomonas aeruginosa (13 cases), Haemophilus influenzae (10 cases), adenovirus (10 cases), and influenza virus A (7 cases), respectively. MP was the most common pathogen in all three periods and the proportion increased yearly. The proportion of MP in 2016-2019 was significantly higher than that in 2012-2015 (52.1% (197/378) vs. 36.8% (32/87), χ2=6.654, P=0.010), while there was no significant difference in the proportion of MP in 2012-2015 and that in 2008-2011 (36.8% (32/87) vs. 31.0% (9/29), χ²=0.314, P=0.575).Regarding the regional distribution, 342 cases were in the southern China and 152 in the northern China. Also, MP was the most common pathogen in both regions, but the proportion of MP was higher and the proportion of SP was lower in the north than those in the south (60.5% (92/152) vs. 42.7% (146/342), χ2=13.409, P<0.010; 7.9% (12/152) vs. 14.3% (49/342), χ2=4.023, P=0.045). Comparing the clinical characteristics of different pathogens, we found that fever and cough were the common symptoms in both single MP and single bacterial groups, but chest pain was more common (17.0% (34/200) vs. 6.1% (6/98), χ2=6.697, P=0.010) while shortness of breath and wheezing were less common in MP group (16.0% (32/200) vs. 60.2% (59/98), χ2=60.688, P<0.01; 4.5% (9/200) vs. 21.4% (21/98), χ2=20.819, P<0.01, respectively). The white blood cell count, C-reactive protein and procalcitonin in the bacterial group were significantly higher than those in the MP group (14.7 (1.0-67.1)×109/L vs. 10.5 (2.5-32.2)×109/L, 122.5 (0.5-277.3) mg/L vs. 51.4 (0.5-200.0) g/L, 2.13 (0.05-100.00) µg/L vs. 0.24 (0.01-18.85) µg/L, Z=-3.719, -5.901 and -7.765, all P<0.01). Conclusions: The prevalence of pediatric NP in China shows an increasing trend during the past years. MP, SP and SA are the main pathogens of NP, and the most common clinical symptoms are fever and cough. The WBC count, C-reactive protein and procalcitonin in bacterial NP are significantly higher than those caused by MP.


Assuntos
Pneumonia por Mycoplasma , Pneumonia Necrosante , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , China/epidemiologia , Feminino , Humanos , Lactente , Masculino , Pneumonia por Mycoplasma/epidemiologia , Estudos Retrospectivos
4.
Artigo em Chinês | MEDLINE | ID: mdl-34365771

RESUMO

Objective: To analyze the spirometer data of coal mine workers, explore the impact of coal dust on the lung function of coal mine workers. Methods: From June to December 2018, 5272 male coal mine dust-exposed workers who underwent occupational health examinations at the Xinjiang Uygur Autonomous Region Occupational Disease Prevention and Treatment Hospital were selected as the research subjects. The basic information and spirometer data of the workers were collected and analyzed for different ages, years of service and the degree of lung function injury of workers exposed to dust and its influencing factors. Results: The total detection rate of lung function injury among dust-exposed workers was 33.9% (1785/5272) . The type of injury was mainly restrictive ventilatory dysfunction (66.7%, 1190/1785) , followed by mixed ventilatory dysfunction (31.4%, 561/1785) , obstructive ventilatory dysfunction (1.9%, 34/1785) . The detection rate of mild lung function impairment was 21.0% (1105/5272) , The detected rate of moderate or higher lung injury was 12.9% (680/5272) . The abnormal detection rate of chest radiography was 3.4% (179/5272) . The logistic regression analysis of the factors affecting lung function damage showed that employees aged 40-<50 were more likely to detect overall lung function injury and the moderate or higher lung injury (P<0.05) , and that they had been working for 35 to 45 years and excavators were more likely to detect overall function injury and different degree of lung injury (P<0.05) . Conclusion: The lung function injury of coal mine dust-exposed workers is related to their age, dust-exposed working years and type of work, mainly with mild injury and restrictive ventilation dysfunction.


Assuntos
Minas de Carvão , Mineradores , Doenças Profissionais , Exposição Ocupacional , Carvão Mineral , Poeira/análise , Humanos , Masculino , Exposição Ocupacional/efeitos adversos
5.
Bull Exp Biol Med ; 171(3): 352-356, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34297293

RESUMO

To better explore the pathophysiology of FA and its therapy, we aimed to establish a simple and practicable FA model with Freund's adjuvant and introduce an easy and reliable laboratory evaluation method for assessment of inflammation in intestinal segments at different anatomical locations. BALB/c mice were sensitized with ovalbumin combined with Freund's adjuvant. Complete Freund's adjuvant was chosen for the first sensitization and two weeks later incomplete Freund's adjuvant was used for a second sensitization. Two weeks later, the sensitized mice were challenged with 50 mg ovalbumin every other day. After the 6 challenge, all mice were assessed for systemic anaphylaxis, and then sacrificed for sample collection. All sensitized mice showed anaphylactic symptoms and markedly increased levels of serum ovalbumin-specific IgE and IgG1. The activity of mast cell protease-1 (mMCPT-1) was significantly increased in the serum and interstitial fluid of the duodenum, jejunum, ileum, and colon. A successful FA model was established, of which inflammation occurred in the duodenum, jejunum, ileum, and colon. This model provides a reliable and simple tool for analysis of the mechanism of FA and methods of immunotherapy. Moreover, combined detection of ovalbumin-specific antibody and local mMCPT-1 levels could potentially be used as the major indicator for assessment of food allergy.

6.
Zhonghua Fu Chan Ke Za Zhi ; 56(5): 335-340, 2021 May 25.
Artigo em Chinês | MEDLINE | ID: mdl-34034420

RESUMO

Objective: To investigate the impact of adding human menopausal gonadotropin (hMG) for in vitro fertilization-embryo transfer pregnancy outcomes in a standard population of non-advanced age with normal ovarian reserve function using a long follicular phase protocol. Methods: Clinical data of 489 patients with normal ovarian reserve function, who were admitted from January 2018 to January 2020 in the Affiliated Hospital of Guizhou Medical University and underwent in vitro fertilization for the first time with the long follicular phase protocol in fresh cycles, were retrospectively analyzed. The patients were divided into three groups according to whether or not to add urine-derived hMG and the timing of addition: non-addition group (group A), medium-term hMG group (group B1), whole course hMG group (group B2); the laboratory parameters of each group were observed, and the effect of ovulation induction drugs and pregnancy outcomes were compared. Results: The ages of B1 and B2 groups were significantly higher than that of group A (P=0.019 and P=0.011). The basal FSH level of group B2 was significantly higher than those of group A and group B1 (P<0.01 and P=0.006), and the basal FSH/LH ratio of group B2 was significantly higher than that of group B1 (P=0.009). Antral follicle counts of group A and group B1 were significantly higher than that of group B2 (P=0.007 and P=0.017). The superior embryo rate of group B2 [(47±27)%] was significantly higher than that of group A (P=0.017). The embryo implantation rate of group B1 was significantly lower than those of group A and group B2 (P=0.043 and P<0.01). The clinical pregnancy rate of group B2 [76.7% (155/202)] was significantly higher than those of group A (P=0.039) and group B1 (P<0.01). The live-birth rate of group B2 [67.3% (136/202)] was significantly higher than those of group A (P=0.017) and group B1 (P=0.001). Conclusions: For non-advanced aged patients with normal ovarian reserve function, the long protocol of follicular phase is suitable for those with relatively low ovarian reserve function. Adding hMG in the whole course of ovulation induction after gonadotropin-releasing hormone agonist reduction could improve the pregnancy outcomes by improving the quality of embryos.


Assuntos
Menotropinas , Reserva Ovariana , Idoso , Feminino , Fertilização In Vitro , Hormônio Foliculoestimulante , Fase Folicular , Humanos , Indução da Ovulação , Gravidez , Taxa de Gravidez , Estudos Retrospectivos
7.
Zhonghua Kou Qiang Yi Xue Za Zhi ; 56(2): 145-151, 2021 Feb 09.
Artigo em Chinês | MEDLINE | ID: mdl-33557497

RESUMO

Objective: To compare the success rates of iRoot BP Plus and mineral trioxide aggregate (MTA) used as pulp capping materials respectively in pulpotomies of human primary molars and to evaluate the clinical efficacy of iRoot BP Plus. Methods: Children who had at least one pair of carious primary molars and needed to receive pulpotomy under general anesthesia were selected in the present split-mouth trail from August to December, 2018. Each pair of the molars was randomly divided into iRoot BP Plus (experimental group) and MTA group (control group). Children were recalled after 3, 6 and 12 months of treatment both for clinical and radiographic evaluations. Relevant medical and radiographic records were collected. Success rates of two groups were compared and influence factors were analyzed. Results: Totally 20 children (10 boys and 10 girls), 28 pairs of split-mouth molars (56 molars, 28 in experimental group and 28 in control group) were included in the study. At the end of the study, 23 pairs of molars were received the 12-month follow-up. The success rates of 3, 6 and 12 months were 96% (25/26), 92% (22/24) and 87% (20/23) in the experimental group, and 100% (26/26), 96% (23/24), 96% (22/23) in the control group, respectively. There were no significant differences between each of the follow-ups in two groups (P>0.05). The success rate of iRoot BP Plus was significantly influenced by the time of hemostasis after amputating the coronal pulp (P<0.05). Conclusions: Pulpotomies of human primary molars with either iRoot BP Plus or MTA had favorable treatment outcomes. The iRoot BP Plus could be a promising biomaterial in pulpotomy.


Assuntos
Agentes de Capeamento da Polpa Dentária e Pulpectomia , Pulpotomia , Compostos de Alumínio , Compostos de Cálcio , Criança , Capeamento da Polpa Dentária , Combinação de Medicamentos , Feminino , Humanos , Masculino , Dente Molar , Óxidos , Silicatos , Dente Decíduo , Resultado do Tratamento
8.
Zhonghua Er Ke Za Zhi ; 58(8): 646-652, 2020 Aug 02.
Artigo em Chinês | MEDLINE | ID: mdl-32842385

RESUMO

Objective: To analyze the clinical features of cystic fibrosis (CF) associated allergic bronchopulmonary aspergillosis (ABPA) in children. Methods: A retrospective study was performed in 22 children who were diagnosed with CF associated ABPA in Beijing Children's Hospital affiliated to Capital Medical University from March 2010 to March 2020. The clinical features, imaging characteristics, laboratory results and the prognosis were reviewed. Results: A total of 22 cases met the diagnostic criterion, including 12 males and 10 females. The age of diagnosis was (10.4±2.8) years and the age of onset was (5.5±4.4) years. Clinical manifestations included cough and expectoration (22 cases), recurrent wheezing (15 cases), hemoptysis (7 cases), failure to thrive (12 cases), pancreatitis (10 cases), hepatomegaly (7 cases), splenomegaly (4 cases) and steatorrhea (4 cases). CT scans of all the patients showed pulmonary infiltrates and central bronchiectasis, combined with mucoid impaction in 17 cases and high density mucus plug in 12 cases. Eosinophilia was found in 18 patients. Total IgE and serum levels of A. fumigatus-specific IgE were elevated in all 22 patients. Positive culture of sputum or bronchoalvedar lauage fluid for fungus were in 15 cases, with single Aspergillus infection in 8 cases and mixed Aspergillus infection in 3 cases. The predominant bacteria found in the airways were Pseudomonas aeruginosa (17 cases), followed by staphylococcas. aureus (6 cases) and stenotrophomonas. maltophilia (5 cases). Pulmonary function revealed obstructive ventilation dysfunction in 4 cases, mixed dysfunction in 11 cases, and small airway dysfunction in 4 cases. Regarding the treatment, 3 were treated only with systemic corticosteroid, while the remaining 19 cases also received antifungal agents.The follow up continued for 1-7 years, and 6 maintained remission, 10 had recurrent episodes, 1 died, and 5 lost to follow up. Conclusions: CF associated ABPA is extremely rare in China. The overlapping clinical, radiographic, and immunologic features of these two diseases make the diagnosis challenging. Systemic corticosteroids are considered the first-line therapy for these patients, and adjuvant antifungal agents may be helpful. Recurrence rate in our center is high.


Assuntos
Aspergilose Broncopulmonar Alérgica/complicações , Aspergillus fumigatus/isolamento & purificação , Fibrose Cística/complicações , Escarro/microbiologia , Aspergilose Broncopulmonar Alérgica/tratamento farmacológico , Criança , China , Fibrose Cística/diagnóstico , Fibrose Cística/microbiologia , Feminino , Glucocorticoides/uso terapêutico , Humanos , Masculino , Estudos Retrospectivos , Resultado do Tratamento
9.
Zhonghua Er Ke Za Zhi ; 58(8): 674-678, 2020 Aug 02.
Artigo em Chinês | MEDLINE | ID: mdl-32842389

RESUMO

Objective: To analyze the clinical features of 2 children with hereditary hemorrhagic telangiectasia (HHT) and review relevant literature. Methods: The clinical data of 2 children with HHT who were admitted to Beijing Children's Hospital, Capital Medical University from February 2017 to June 2018 were retrospectively analyzed. Meanwhile, the relevant papers published before December 2019 in Wanfang and Pubmed database were searched with the key words of "hereditary hemorrhagic telangiectasia" in Chinese and English, and reviewed. Results: The first case was an 11-year-old boy, presented with fever and cough for 6 days. His past history revealed 3 times of left pneumonia and mild epistaxis. Ultrasound of the chest soft tissue found hemangioma and vascular malformation. Enhanced CT with vascular reconstruction suggested pulmonary arteriovenous malformation and multiple arteriovenous fistulas in the liver. After being treated with interventional pulmonary arteriovenous fistula embolization, his condition improved. The second case was a 12-year-old girl, presented with iron deficiency anemia during the past 3 years. Endoscopic resection of polyps had been performed before due to multiple polyps in the colon. High-resolution lung CT revealed multiple ground-glass nodules in bilateral lungs. Ultrasound of the digestive tract suggested small intestine polyps and secondary chronic loose intussusception. After admission, small bowel intussusception reduction was performed, and the polyps were ligated. Her whole exon gene testing suggested SMAD4 heterozygous variation. In the large number of reported cases, HHT1 and HHT2 are the main type. Conclusions: Children with HHT may present with anemia and repeated intestinal polyps. It is worth noting that visceral arteriovenous malformations, such as pulmonary arteriovenous malformations, which is easily misdiagnosed as recurrent pneumonia, can also occur in children with HHT. Enhanced CT with vascular reconstruction can help find pulmonary arteriovenous malformation, and timely genetic test can confirm the diagnosis.


Assuntos
Fístula Arteriovenosa/complicações , Malformações Arteriovenosas/diagnóstico , Telangiectasia Hemorrágica Hereditária/complicações , Telangiectasia Hemorrágica Hereditária/diagnóstico , Fístula Arteriovenosa/cirurgia , Malformações Arteriovenosas/diagnóstico por imagem , Malformações Arteriovenosas/etiologia , Criança , Tosse/etiologia , Feminino , Febre/etiologia , Humanos , Fígado/diagnóstico por imagem , Masculino , Veias Pulmonares/anormalidades , Veias Pulmonares/diagnóstico por imagem , Estudos Retrospectivos , Telangiectasia Hemorrágica Hereditária/genética , Tomógrafos Computadorizados
10.
Zhonghua Yi Xue Za Zhi ; 100(26): 2044-2048, 2020 Jul 14.
Artigo em Chinês | MEDLINE | ID: mdl-32654451

RESUMO

Objective: To investigate the changes of maximum urethral pressure (MUP) and maximum urethral closure pressure (MUCP) after artificial urethral sphincter (AUS) implantation and their prognostic value. Methods: The clinical data of patients who had undergone AUS implantation in multiple medical centers between March and July 2019 were retrospectively analyzed. Data of urethral pressure profilometry, pad usage, related scores and complications related to surgery were collected and compared. The primary endpoint was social continence (defined as 0-1 pad/d) 1 month after activation of the pump. Results: A total of five male patients were included in this study. Two underwent transurethral resection of the prostate for benign prostatic hyperplasia, two underwent radical prostatectomy for prostate cancer, and one underwent urethral reunion, urethral stricture dilatation and cystostomy due to trauma from traffic accident. All patients had different degrees of urinary incontinence. The results of preoperative urethral profilometry test showed that the MUP of five patients were 52, 53, 88, 32, and 66 cmH(2)O(1 cmH(2)O=0.098 kPa), respectively, and the MUCP were 17, 52, 62, 27, and 40 cmH(2)O, respectively. AUS implantation was performed. The intraoperative urethral pressure profilometry showed that the MUP were 53, 113, 50, 77, and 89 cmH(2)O in the inactivated state, and the MUCP were 50, 97, 31, 71, and 51 cmH(2)O, respectively. In the activated state, the MUP were 112, 174, 193, 121, and 120 cmH(2)O, and the MUCP were 109, 160, 175, 114, and 92 cmH(2)O, respectively. All patients met the social continence (0-1 pad/d) criterion. No complications were reported during the follow-up. Conclusions: The relationship between the range of intraoperative urethral pressure and the effect of urinary control can be gained by measuring the specific values of MUP and MUCP during AUS implantation and the post-operative effects, which provides as a data basis for standardizing AUS implantation.


Assuntos
Ressecção Transuretral da Próstata , Incontinência Urinária por Estresse/cirurgia , Esfíncter Urinário Artificial , Humanos , Masculino , Prostatectomia , Estudos Retrospectivos , Resultado do Tratamento , Uretra
12.
Zhonghua Er Ke Za Zhi ; 57(7): 548-552, 2019 Jul 02.
Artigo em Chinês | MEDLINE | ID: mdl-31269556

RESUMO

Objective: To assess the diagnostic value of sweat conductivity testing in Chinese children with cystic fibrosis (CF). Methods: This is a retrospective study. Sweat conductivity tests were conducted in 45 CF children (CF group) and 200 non-CF children (non-CF group) diagnosed with other chronic pulmonary diseases at the No. 2 Department of Respiratory Medicine, Beijing Children's Hospital from May 2014 to June 2018. Pearson's chi-square test was used to assess the differences between CF and non-CF groups. A receiver operating characteristic curve was constructed to calculate the best cut-off value to diagnose or rule out CF. The pulmonary function parameters (forced expiratory volume in the first second, forced vital capacity,forced expiratory flows at 75% of exhaled vital capacity) of CF children over 6 years old were analyzed. The relationship between sweat conductivity and pulmonary function was compared between the two groups (80-120mmol/L vs.>120mmol/L). Results: The age of CF group was 9 (7,12) years old, 19 males (42%) and 26 females(58%); the age of non-CF group was 8 (5,11) years old, 106 males (53%) and 94 females(47%). The results of sweat conductivity test showed that sweat conductivity in CF group 108(99, 122) mmol/L was significantly higher than that in non-CF group 43(36, 52) mmol/L (χ(2)=207, P<0.01). A cut-off value of 80 mmol/L for CF diagnosis showed a sensitivity of 93.3% and a specificity of 98.5%. The receiver operating characteristic curve analysis suggested the best conductivity cut-off value for the diagnosis of CF was at 83.5 mmol/L,with a sensitivity of 93.3% and a specificity of 100%,and an area under the curve of 0.993 (95% confidence interval 0.985-1.000). The best conductivity cut-off value to rule out CF diagnosis was at 63.5 mmol/L,with a sensitivity of 97.8% and a specificity of 90.5%. There was no correlation between the level of sweat conductivity and the extent of pulmonary function decline. Conclusions: Sweat conductivity testing can be used for the screening of CF in Chinese children. A diagnosis of CF should be considered if the value is greater than 80 mmol/L.


Assuntos
Cloretos/análise , Fibrose Cística/diagnóstico , Condutividade Elétrica , Suor/química , Suor/fisiologia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos
13.
Zhonghua Er Ke Za Zhi ; 57(8): 620-624, 2019 Aug 02.
Artigo em Chinês | MEDLINE | ID: mdl-31352748

RESUMO

Objective: To improve the awareness of methylmalonic acidemia and hyperhomocysteinemia with diffuse lung disease as an initial or main presentation. Methods: A retrospective analysis of the clinical manifestations, radiological features, laboratory tests, genetic variations, treatments and prognoses was conducted in six children presented with diffuse lung disease and finally diagnosed with methylmalonic acidemia and hyperhomocysteinemia in Ward 2 of Department of Respiratory Diseases, Beijing Children's Hospital, from August 2017 to November 2018. Results: Six children were included in this study. Two children were male and four were female. The average age of onset was 28 months. The mean age at diagnosis was 34 months. The average interval from onset to diagnosis was 6 months. Four children who underwent genetic tests were found to have variants of gene MMACHC and diagnosed with CblC type. All children had respiratory symptoms and signs as initial or main presentation, which were tachypnea (5 cases), exercise intolerance (5 cases), cough (4 cases), cyanosis (4 cases), clubbing (4 cases), dyspnea (3 cases) and retractions (3 cases). Pulmonary arterial hypertension was found in all six children. Pericardial effusion (4 cases), kidney involvement (3 cases), nervous system involvement (3 cases), gastrointestinal system involvement (3 cases) and anemia (2 cases) also coexisted. The high resolution computed tomography (HRCT) features included dilated pulmonary artery (6 cases), ground-glass opacities (4 cases), diffuse poorly defined ground-glass centrilobular nodules (3 cases), pleural effusion (3 cases), thickening of interlobular septum (2 cases), etc. All children had an elevated concentration of methylmalonic acid in urine and homocysteine in plasma. Genetic tests were performed in four patients, and MMACHC genetic mutations were found in all of them. Clinical manifestations, HRCT features and pulmonary arterial hypertension turned better in five children after treatment. One patient who was not regularly followed-up died. Conclusions: Pulmonary involvement including diffuse lung disease and pulmonary arterial hypertension could coexist with methylmalonic acidemia and hyperhomocysteinemia, which may have respiratory symptoms and signs as the initial or main presentation. Characteristic HRCT features were found in some patients. Plasma homocysteine test is a quick method for screening the disease in children with diffuse lung disease and (or) pulmonary arterial hypertension. Both diffuse lung disease and pulmonary arterial hypertension may turn better after treatment.


Assuntos
Erros Inatos do Metabolismo dos Aminoácidos/diagnóstico , Hiper-Homocisteinemia/diagnóstico , Pneumopatias/etiologia , Pulmão/fisiopatologia , Artéria Pulmonar/diagnóstico por imagem , Idade de Início , Erros Inatos do Metabolismo dos Aminoácidos/urina , Proteínas de Transporte , Criança , Pré-Escolar , Feminino , Humanos , Hiper-Homocisteinemia/sangue , Lactente , Masculino , Oxirredutases , Estudos Retrospectivos , Tomografia Computadorizada por Raios X
14.
Phys Rev Lett ; 122(24): 247001, 2019 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-31322397

RESUMO

We developed novel techniques to fabricate atomically thin Bi_{2.1}Sr_{1.9}CaCu_{2.0}O_{8+δ} van der Waals heterostructures down to two unit cells while maintaining a transition temperature T_{c} close to the bulk, and carry out magnetotransport measurements on these van der Waals devices. We find a double sign change of the Hall resistance R_{xy} as in the bulk system, spanning both below and above T_{c}. Further, we observe a drastic enlargement of the region of sign reversal in the temperature-magnetic field phase diagram with decreasing thickness of the device. We obtain quantitative agreement between experimental R_{xy}(T,B) and the predictions of the vortex dynamics-based description of Hall effect in high-temperature superconductors both above and below T_{c}.

15.
Zhonghua Yi Xue Za Zhi ; 99(20): 1553-1557, 2019 May 28.
Artigo em Chinês | MEDLINE | ID: mdl-31154721

RESUMO

Objective: To investigate the expression and prognosis effect of methylation-regulated SLIT3 and SPRCL1 genes in smoking-related lung adenocarcinoma. Methods: The expression levels of SLIT3 and SPARCL1 in cigarette smoke-induced malignant transformed cell (S30) and lung adenocarcinoma (LUAD) cell lines were measured by real-time fluorescence quantitative PCR (qPCR). Datasets of mRNA expression, DNA methylation and patient information data were obtained from The Cancer Genome Altas (TCGA) database. The mRNA expression levels of SLIT3 and SPARCL1 were validated in LUAD tissues. The 10-year survival curve of LUAD patients with different smoking history was plotted, and the correlation between mRNA expression level and DNA methylation level of LUAD patients was further analyzed. S30 cells were treated with 5-azacytidine (5-aza), an inhibitor of DNA methyltransferase, to analyze the methylation regulatory mechanism of SLIT3 and SPRCL1. Results: The qPCR results showed the significant down-regulation of SLIT3 and SPARCL1 in S30 cell and four LUAD cell lines (SLIT3: 0.493±0.134 and 0.041±0.014, 0.161±0.023, 0.277±0.055, 0.035±0.005; SPARCL1: 0.507±0.131 and 0.453±0.045, 0.420±0.040, 0.153±0.035, 0.430±0.050; all P<0.01). Bioinformatics analysis showed that SLIT3 and SPARCL1 were low expressed in LUAD tissue (8.12±1.58 vs 10.84±0.69 and 11.46±1.06 vs 13.57±0.67; both P<0.001) compared with adjacent peritumoral tissues, and expression levels of SLIT3 and SPARCL1 were significantly correlated with smoking history (both P<0.001). Non-smoker with high expression of SLIT3 and SPARCL1 was associated with better prognosis among LUAD patients. There was a significant negative correlation between promoter methylation and mRNA expression level of the two genes (r=-0.208, -0.574; both P<0.001). 5-aza treatment significantly up-regulated the expression levels of SLIT3 and SPARCL1 genes in S30 cells (2.137±0.281, 3.657±0.882; both P<0.01). Conclusion: SLIT3 and SPARCL1 can be regulated by DNA methylation and down-regulated in LUAD tissue, which has important prognostic significance on the smoking-induced LUAD patients.


Assuntos
Adenocarcinoma de Pulmão , Neoplasias Pulmonares , Proteínas de Ligação ao Cálcio , Proteínas da Matriz Extracelular , Humanos , Proteínas de Membrana , Prognóstico , Fumar
16.
Zhonghua Er Ke Za Zhi ; 57(5): 392-396, 2019 May 02.
Artigo em Chinês | MEDLINE | ID: mdl-31060137
18.
Zhongguo Xue Xi Chong Bing Fang Zhi Za Zhi ; 32(2): 213-216, 2019 Oct 15.
Artigo em Chinês | MEDLINE | ID: mdl-32458616

RESUMO

OBJECTIVE: To investigate the diagnosis and surgical treatment of co-infection with hepatic cystic and alveolar echinococcosis, so as to provide insights into the diagnosis and treatment of these patients. METHODS: The clinical data of patients with co-infections of hepatic cystic and alveolar echinococcosis in Qinghai Provincial People's Hospital between 2017 and 2018 were retrospectively analyzed. RESULTS: Three patients were diagnosed with co-infection of hepatic cystic and alveolar echinococcosis. One patient was diagnosed by preoperative CT scan, and confirmed intraoperatively. The other two cases were diagnosed as cystic echinococcosis by preoperative color ultrasonography and imaging examinations, and were definitively diagnosed as co-infection of hepatic cystic and alveolar echinococcosis by intraoperative examination of the lesion morphology and postoperative pathology. Two patients were given radical surgery, and another case was given removal of the internal capsule and subtotal excision of the outer capsule of Echinococcus granulous in the liver following exploration. CONCLUSIONS: Co-infection with hepatic cystic and alveolar echinococcosis is easy for missed diagnosis and misdiagnosis prior to operation, and the definitive diagnosis may be made by means of imaging examinations combined with postoperative pathology. The surgery is relatively complicated and difficult for patients with co-infection of hepatic cystic and alveolar echinococcosis, and individualized surgical treatment regimen should be employed for patients with various types of infections.


Assuntos
Coinfecção , Equinococose Hepática , Equinococose , Equinococose/complicações , Equinococose/diagnóstico por imagem , Equinococose/cirurgia , Equinococose Hepática/complicações , Equinococose Hepática/diagnóstico por imagem , Equinococose Hepática/cirurgia , Humanos , Diagnóstico Ausente , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Ultrassonografia
19.
Zhongguo Xue Xi Chong Bing Fang Zhi Za Zhi ; 31(6): 676-678, 2019 Oct 15.
Artigo em Chinês | MEDLINE | ID: mdl-32064820

RESUMO

Alveolar echinococcosis is a parasitic zoonosis that severely damages human health. Currently, radical surgical resection is the first choice for hepatic alveolar echinococcosis. For the advanced hepatic echinococcosis patients with refractory radical resection, the palliative surgery combined with chemotherapy, liver transplantation, drug therapy, and radiofrequency microwave ablation may provide comprehensive tools. This article reviews the current situation and progress of comprehensive treatments for hepatic alveolar echinococcosis.


Assuntos
Equinococose Hepática , Zoonoses , Animais , Equinococose Hepática/terapia , Hepatectomia , Humanos , Transplante de Fígado , Zoonoses/terapia
20.
Zhonghua Er Ke Za Zhi ; 56(12): 915-922, 2018 Dec 02.
Artigo em Chinês | MEDLINE | ID: mdl-30518005

RESUMO

Objective: To explore the clinical features, the serotype distribution and drug resistance of the isolates in patient with invasive pneumococcal disease (IPD). Methods: By retrieving the laboratory information system in 18 children's hospitals from 2012 to 2017, the children with IPD were enrolled. Streptococcus pneumoniae (Spn) must be isolated from the sterile sites (blood, cerebrospinal fluid, hydrothorax and joint effusion etc.). The clinical characteristics, serotype, drug resistance, treatment and prognosis were reviewed and analyzed. According to the telephone follow up results, the patients were divided into death group and recovered group. The index as an independent risk factor of mortality was demonstrated by multivariate logistic regression analysis. Results: There were 1 138 children with IPD, including 684 male and 454 female. The proportion of male to female was 1.5∶1. The age ranged from one day to 16 years. The median age was 1 year 3 month. The majority was under 5 years of age (89.3%, n= 1 016), especially under 2 years of age (61.9%, n=704). In all cases, 88.2% (n=1 004) were community acquired infection. The infections included meningitis (n=446, 39.2%), pneumonia with bacteremia (n=339, 29.8%), and bacteremia without focus (n=232, 20.4%). Underlying diseases were found in 242 cases (21.3%). Co-infections were determined in 62 cases (5.4%) with mycoplasma, 27 cases (2.4%) with adenovirus and 34 cases with influenza virus (3.0%). The penicillin insensitivity (PNSP) rates in meningitis and non-meningitis isolates were 69.5% (276/397) and 35.9% (221/615), respectively. There were 81 strains serotyped, in which 93.8% (76/81) were covered by 13-valent protein-polysaccharide conjugate vaccine (PCV13). In the 965 patients who were followed up by phone call, 156 cases (16.2%) were confirmed dead. The independent risk factors for the death were under 2 years of age (OR=2.143, 95%CI 1.284-3.577, P=0.004), meningitis (OR=3.066, 95%CI 1.852-5.074, P<0.01), underlying disease (OR=4.801, 95%CI 2.953-7.804, P<0.01), septic shock(OR=3.542, 95%CI 1.829-6.859, P<0.01), disseminated intravascular coagulation (DIC) (OR=4.150, 95%CI 1.468-11.733, P=0.007), multiple organ failure (OR=12.693, 95%CI 6.623-24.325, P<0.01) and complications of central nervous system (OR=1.975, 95%CI 1.144-3.410, P=0.015). Conclusions: Most children with IPD were under 5 years of age, having underlying diseases and acquired the infection in community. The independent risk factors for death were under two years old, meningitis, underlying diseases and multiple organ failure. The problem of drug resistance was severe. The universal immunization of PCV13 would be effective to prevent IPD in Chinese children.


Assuntos
Infecções Pneumocócicas , Vacinas Pneumocócicas , Streptococcus pneumoniae , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Masculino , Infecções Pneumocócicas/complicações , Infecções Pneumocócicas/tratamento farmacológico , Infecções Pneumocócicas/mortalidade , Vacinas Pneumocócicas/administração & dosagem , Fatores de Risco , Sorogrupo , Sorotipagem , Streptococcus pneumoniae/isolamento & purificação , Vacinas Conjugadas
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