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1.
Chinese Journal of Experimental Ophthalmology ; (12): 554-560, 2023.
Article in Chinese | WPRIM | ID: wpr-990881

ABSTRACT

Objective:To evaluate the feasibility of measurement and characteristics of intraocular lens (IOL) tilt using the swept-source optical coherence tomography (SS-OCT) biometer (IOLMaster 700) and to explore its potentially relevant parameters.Methods:A cross-sectional study was conducted.Two hundred and forty-two eyes (119 right eyes, 123 left eyes) of 185 patients after phacoemulsification and IOL implantation were included in Beijing Tongren Hospital from July to September 2018.The IOL position, angle κ, angle α, corneal curvature, anterior chamber depth (ACD), pupil diameter (PD), and axial length (AL) were obtained by IOLMaster 700, and the IOL tilt direction and magnitude were calculated.The within-subject standard deviation and intraclass correlation coefficient were used to evaluate the repeatability of three IOL tilt measurements.Binocular symmetry of IOL tilt and the correlation between IOL tilt and different influencing factors were evaluated by Pearson linear correlation analysis or Spearman rank correlation analysis.Influencing factors for IOL tilt were assessed by multiple linear regression analysis.This study adhered to the Declaration of Helsinki.The study protocol was approved by the Ethics Committee of Beijing Tongren Hospital, Capital Medical University (No.TRECKY2018-049). Patients were informed of the purpose and methods of this study and signed the written informed consent.Results:The repeatability of IOL tilt measurements was excellent with a within-subject standard deviation of 5.16° for IOL tilt direction and 0.13° for IOL tilt magnitude.IOL tilt was mirror symmetric in both eyes.The IOL tilt direction in right eyes ranged from -89.60° to 87.53° with a mean of (18.85±29.65)°, and the tilt magnitude ranged from 1.32° to 7.05° with a mean of (4.16±1.26)°.The IOL tilt direction in left eyes ranged from -84.30° to 89.44° with a mean of (21.17±32.38)°, and the tilt magnitude ranged from 0.58° to 7.40° with a mean of (3.80±1.31)°.There were moderate and weak positive correlations of IOL tilt direction and tilt magnitude between both eyes ( r=0.473, P<0.001; r=0.335, P=0.011). IOL tilt magnitude was weakly positively correlated with angle α and IOL diopter ( rs=0.272, P=0.003; r=0.285, P=0.002), and was weakly negatively correlated with ACD, PD and AL ( r=-0.303, P=0.001; r=-0.233, P=0.011; rs=-0.331, P<0.001). In backward stepwise regression analysis, the regression equation of IOL tilt magnitude, angle α, ACD, PD, AL and IOL diopter in multiple linear regression analysis was as follows: IOL tilt magnitude=10.503+ 1.456×angle α-0.532×ACD-0.196×AL ( R2=0.400; F=8.588, P<0.001). Conclusions:The SS-OCT biometer can be an effective method to assess IOL tilt.IOL tilt is mirror symmetric between the right eyes and left eyes.

2.
Korean Journal of Radiology ; : 1081-1092, 2023.
Article in English | WPRIM | ID: wpr-1002404

ABSTRACT

Objective@#To investigate the incidence of microvascular myocardial ischemia in diabetic patients without obstructive coronary artery disease (CAD) and its relationship with angina. @*Materials and Methods@#Diabetic patients and an intermediate-to-high pretest probability of CAD were prospectively enrolled. Non-diabetic patients but with an intermediate-to-high pretest probability of CAD were retrospectively included as controls. The patients underwent dynamic computed tomography-myocardial perfusion imaging (CT-MPI) and coronary computed tomography angiography (CCTA) to quantify coronary stenosis, myocardial blood flow (MBF), and extracellular volume (ECV). The proportion of patients with microvascular myocardial ischemia, defined as any myocardial segment with a mean MBF ≤ of 100 mL/min/100 mL, in patients without obstructive CAD (Coronary Artery Disease–Reporting and Data System [CAD-RADS] grade 0–2 on CCTA) was determined. Various quantitative parameters of the patients with and without diabetes without obstructive CAD were compared. Multivariable analysis was used to determine the association between microvascular myocardial ischemia and angina symptoms in diabetic patients without obstructive CAD. @*Results@#One hundred and fifty-two diabetic patients (mean age: 59.7 ± 10.7; 77 males) and 266 non-diabetic patients (62.0 ± 12.3; 167 males) were enrolled; CCTA revealed 113 and 155 patients without obstructive CAD, respectively. For patients without obstructive CAD, the mean global MBF was significantly lower for those with diabetes than for those without (152.8 mL/min/100 mL vs. 170.4 mL/min/100 mL, P < 0.001). The mean ECV was significantly higher for diabetic patients (27.2% vs. 25.8%, P = 0.009). Among the patients without obstructive CAD, the incidence of microvascular myocardial ischemia (36.3% [41/113] vs. 10.3% [16/155], P < 0.001) and interstitial fibrosis (69.9% [79/113] vs. 33.3% [8/24], P = 0.001) were significantly higher in diabetic patients than in the controls. The presence of microvascular myocardial ischemia was independently associated with angina symptoms (adjusted odds ratio = 3.439, P = 0.037) in diabetic patients but without obstructive CAD. @*Conclusion@#Dynamic CT-MPI + CCTA revealed a high incidence of microvascular myocardial ischemia in diabetic patients without obstructive CAD. Microvascular myocardial ischemia is strongly associated with angina.

3.
Chinese Journal of Ocular Fundus Diseases ; (6): 191-196, 2022.
Article in Chinese | WPRIM | ID: wpr-934293

ABSTRACT

Objective:To analyze the ultrasonographic features of adenoma of the nonpigmented ciliary epithelium (ANPCE).Methods:A retrospective series of case studies. From January 2014 to October 2021, 31 patients (31 eyes) with ANPCE (ANPCE group) were diagnosed in the eye center of Beijing Tongren Eye Center of Beijing Tongren Hospital, Capital Medical University, and 17 patients (17 eyes) with ciliary body melanoma (control group) diagnosed at the same time were selected as the control group. There was no significant difference in age ( t=-0.564) and sex composition ratio ( χ 2=0.182) between the two groups ( P=0.576, 0.670). All patients underwent ultrasound biomicroscopy to obtain the measurement parameters: tumor height, maximum basal diameter, maximum diameter, ratio of maximum diameter to basal diameter and ratio of maximum diameter to height; tumor location, shape, internal echogenicity intensity, echogenicity uniformity, degree of sound attenuation, invasion of iris, anterior displacement of the iris, lens subluxation were observed. The measurement parameters and observation indexes of the two groups were compared by independent sample t-test and χ 2 test. Receiver operating characteristic (ROC) curve was drawn, area under the ROC curve (AUC) was determined, and parameter indicators with differential diagnosis value were screened. Results:The maximum diameter, height, maximum basal diameter, ratio of the maximum diameter to the maximum basal diameter, and the ratio of the maximum diameter to the height of the tumors in the ANPCE group and the control group were 5.64±0.98 mm, 4.24±0.59 mm, 3.66±0.71 mm, 1.58±0.34, 1.34±0.19 and 7.82±2.03 mm, 4.47±2.44 mm, 7.02±1.96 mm, 1.13±0.16, 2.09±1.06. The maximum diameter, the maximum basal diameter, and the ratio of the maximum diameter to the height of the tumor in the ANPCE group were all smaller than those of the control group, and the ratio of the maximum diameter to the maximum basal diameter was greater than that of the control group, and the differences were statistically significant ( t=-4.159,-6.808, -2.924, 6.257; P<0.05). The tumors in the ANPCE group were mainly spherical (87.1%, 27/31), with no significant acoustic attenuation (77.4%, 24/31), less invading the root iris (77.4%, 24/31), and the tumors were mostly located in the ciliary body coronal (74.2%, 23/31); tumors in the control group were mainly hemispherical (47.1%, 8/17) or spherical (47.1%, 8/17), with significant sound attenuation (76.5%, 13/17), most of the tumors invaded the iris (70.6%, 12/17), and the tumors were mostly located from the pars plana to the coronal (76.5%, 13/17). There were statistically significant differences in the position, shape, sound attenuation degree, and whether it invaded the iris between the two groups of eyes ( χ 2=15.132, 19.767, 13.118, 10.581; P<0.05). The results of ROC curve analysis showed that the ratio of the largest diameter to the largest base diameter, the degree of sound attenuation and the AUC of whether the iris was violated were higher, which were 0.881, 0.769, and 0.740, respectively. Conclusions:Ultrasound biomicroscopy is helpful in the diagnosis and differential diagnosis of ANPCE and ciliary body melanoma. The ratio of maximum diameter to maximum basal diameter, the degree of sound attenuation and whether it invades the root iris are important parameters to distinguish the two tumors.

4.
Clinical Medicine of China ; (12): 178-182, 2022.
Article in Chinese | WPRIM | ID: wpr-932165

ABSTRACT

Objective:To analyze the characteristics of body composition, dietary intake and vitamin D status of school-age wasting children, and to provide evidence for individual dietary intervention and nutrient supplement.Methods:From September to December 2019, we chose wasting children aged 6-10 years old in the nutrition clinic of Beijing Children's Hospital affiliated to Capital Medical University, and a retrospective case-control study was adopted. To detect body composition and vitamin D level, and conduct dietary survey and assessment. The measurement data of normal distribution were compared by independent sample t-test. The relationship between the two variables was analyzed by Pearson correlation analysis. Rank sum test was used to compare rank data.Results:A total of 60 subjects were studied, including 30 boys and 30 girls. The percentage of inorganic salt in boys (6.81±0.37)% is higher than that in girls (6.37±0.59)%, the difference was statistically significant ( t=7.75, P=0.009). Compared with the target values of normal children of the same age and gender, the contents of fat in boys ((2.39±1.25) kg and (4.20±0.95) kg, t=43.05, P<0.001), in girls ((2.66±1.20) kg and (4.91±1.41) kg, t=59.87, P<0.001); muscle in boys ((17.35±3.23) kg and (21.57±4.94) kg, t=16.89, P<0.001), in girls ((17.69±3.03) kg and (21.79±4.36) kg, t=20.97, P<0.001); total water in boys ((13.54±2.52) kg and (16.80±3.86) kg, t=16.51, P<0.001), in girls ((13.82±2.36) kg and (16.96±3.40) kg, t=20.20, P<0.001); protein in boys ((3.58±0.67) kg and (4.51±1.04) kg, t=18.68, P<0.001), in girls ((3.65±0.63) kg and (4.55±0.92) kg, t=24.55, P<0.001); inorganic salt in boys ((1.42±0.25) kg and (1.56±0.36) kg, t=4.28, P=0.036), in girls ((1.37±0.27) kg and (1.56±0.30) kg, t=8.24, P=0.006) were all significantly reduced. The total energy intake of all the children were lower than the recommended intake for children of the same age and gender. Among them, 70.00%(42/60) of children had insufficient energy intake for breakfast, 50.00% (30/60) for lunch and 40.00%(24/60) for dinner. In terms of energy intake of three macronutrients, 53.33%(32/60) of children were deficient in carbohydrate, 50.00%(30/60) in protein and 58.33%(35/60) in fat. The mean content of 25-hydroxyvitamin D was (46.95±14.53) nmol/L in male and (46.41±14.51) nmol/L in female, all of them had Vitamin Dinsufficiency. Conclusion:Wasting children should be tested for body composition and vitamin D levels, to get individualized dietary instruction and nutrient supplementation, and improve nutritional status.

5.
Chinese Journal of Ultrasonography ; (12): 896-900, 2021.
Article in Chinese | WPRIM | ID: wpr-910137

ABSTRACT

Objective:To investigate the ultrasonic features, contrast-enhanced ultrasound findings and long-term follow-up results of melanocytoma of the optic disc(MCOD).Methods:The data of 35 patients (35 eyes, 35 lesions) diagnosed with MCOD in Beijing Tongren Ophthalmic Center, Beijing Tongren Hospital from September 2012 to October 2020 were retrospectively analyzed. The B-mode ultrasonography and color Doppler flow imaging (CDFI) were performed on them. The size, morphology, internal echo and secondary changes of the lesions were analyzed. Contrast-enhanced ultrasound was performed on 13 patients. At the same time, 9 patients were followed up for long-term observation.Results:Six lesions (17.1%) showed hemispherical high echo, and 29 lesions (82.9%) showed limited uplift high echo. The largest base diameter of the lesions was (4.0±0.8)mm, average height was(1.9±0.4)mm. Internal echo characteristics: 8 lesions (22.9%) showed uniform echo and 27 lesions (77.1%) showed uneven echo.Lesion boundary: 35 lesions (100%) showed clear lesion boundary. Fifheen lesions (42.9%) showed secondary vitreous opacity. CDFI: blood flow signals could be detected inside the lesions in 19 lesions(54.3%), and not in 16 lesions(45.7%). Contrast-enhanced ultrasound showed 11 of the 13 lesions (84.6%) were completely filled with contrast agent.Conclusions:MCOD has certain ultrasonographic characteristics, which can provide a reliable basis for clinical diagnosis and differential diagnosis. There is no significant change in tumor size after long-term follow-up.

6.
Chinese Journal of Ultrasonography ; (12): 563-568, 2021.
Article in Chinese | WPRIM | ID: wpr-910092

ABSTRACT

Objective:To analyze the perfusion characteristics of choroidal metastasis using contrast-enhanced ultrasound, and compare with choroidal hemangioma.Methods:This was a retrospective study.From January 2016 to February 2018 in Beijing Tongren Hospital, a total of 21 eyes from 21 patients who were clinically diagnosed as choroidal metastasis were included as the choroidal metastasis group and 46 eyes from 46 patients who were diagnosed as choroidal hemangioma during the same period were included as the choroidal hemangioma group. All patients underwent contrast-enhanced ultrasound examination, and Sonoliver was used to obtain the data on quantitative parameters of the tumor and the adjacent normal orbital tissues, including maximum of intensity (IMAX), rise time (RT), time to peak (TTP), and mean transit time (mTT). The quantitative parameters between choroidal metastasis and normal orbital tissues were compared. And the quantitative parameters between choroidal metastasis and choroidal hemangioma were compared. The receiver operating characteristic (ROC) curve analysis was performed to evaluate the diagnostic performance.Results:The IMAX of choroidal metastasis was significantly higher than that of normal orbital tissues, while RT, TTP and mTT were significantly shorter than these of normal orbital tissues (all P<0.01). The IMAX of choroidal metastasis was lower than that of choroidal hemangioma, and RT, TTP and mTT were shorter than choroidal hemangioma (all P<0.01). The ROC curve analysis showed that area under curves of the IMAX, RT, TTP and mTT were 0.775 (95% CI=0.666-0.884), 0.970 (95% CI=0.896-0.996), 0.729 (95% CI=0.607-0.831) and 0.992 (95% CI=0.931-1.000) respectively. The sensitivities were 71.7%, 95.7%, 76.1% and 95.7%, and the specificities were 85.7%, 90.5%, 66.7% and 95.2%, respectively. Conclusions:Contrast-enhanced ultrasound can reflect the differences in perfusion characteristics between choroidal metastasis and choroidal hemangioma. RT and mTT are useful parameters in differential diagnosis between the two types of tumors.

7.
Chinese Journal of Ultrasonography ; (12): 161-166, 2021.
Article in Chinese | WPRIM | ID: wpr-884305

ABSTRACT

Objective:To discuss the characteristics of ultrasound diagnosis of optic disc capillary hemangioma.Methods:The study analyzed retrospectively 7 cases of optic disc capillary hemangioma diagnosed in the Beijing Tongren Hospital from 2015 to 2018. The size, morphology, internal echo, and secondary changes of the lesion were analyzed during ultrasound examination.Color Doppler flow imaging was used to check the blood flow in the lesion.Results:Pre-optic disc occupying lesions could be detected in the ultrasound images of the 7 cases. Lesion size: average base diameters (5.39±1.90)mm×(4.79±1.28)mm, average height (3.61±1.37)mm. Lesion morphology: 5 cases were round, and 2 cases were irregular. Echo within the lesion: 3 cases had medium echo inside the lesion, and 4 cases had high echo inside the lesion. Internal echo characteristics: 5 cases had uniform echo, and 2 cases had uneven echo. Secondary changes: 6 cases had secondary retinal detachment and vitreous opacity, and 1 cases was accompanied by retinal hemangioma in other parts. In all cases, blood flow signals could be detected inside the space-occupying lesions, which were in the form of branches, stripes or spots, and the blood flow spectrum showed a parallel spectrum of arteries and veins.Conclusions:Ultrasound examination of optic disc capillary hemangioma has certain characteristics, which can provide a valuable follow-up basis for clinical diagnosis.

8.
Chinese Journal of Experimental Ophthalmology ; (12): 962-966, 2020.
Article in Chinese | WPRIM | ID: wpr-865383

ABSTRACT

Objective:To compare the agreement of biometric parameters measured by a new swept-source optical coherence tomography IOLMaster 700 and a new Scheimpflug imaging combined with partial coherence interferometry biometer Pentacam AXL in cataractous eyes.Methods:A cross-sectional study was performed.A total of 146 eyes from 76 cataract patients were enrolled in Beijing Tongren Eye Center from 2 to 11 in January 2018, including 69 eyes in 36 males and 77 eyes in 40 females, the age ranged from 35 to 88 years, the mean age was (64.52±11.36) years.Axial length (AL), mean keratometry (Km), anterior chamber depth (ACD), central corneal thickness (CCT) and white-to-white (WTW) were measured with IOLMaster 700 and Pentacam AXL, respectively.The differences and correlations of the biometry parameters between the two devices were assessed.Bland-Altman method was used to evaluate the agreement of these parameters between the two devices and the 95% limits of agreement was calculated.This study adhered to the tenets of the Declaration of Helsinki and was approved by an Ethics Committee of Beijing Tongren Hospital (TRECKY2018-049).Results:The mean values of the AL measured by IOLMaster 700 and Pentacam AXL were (24.03±1.72)mm and (23.98±1.73)mm, Km were (44.04±1.71)D and (43.88±1.72)D, ACD were (3.24±0.47)mm and (3.28±0.47)mm, WTW were (11.82±0.51)mm and (11.49±0.49)mm, respectively.Compared with the values in Pentacam AXL, the AL, Km and WTW were larger while ACD was smaller in IOLMaster 700 with significant differences ( t=18.06, 8.94, 23.83, -15.36, all at P<0.01). The mean CCT values from the IOLMaster 700 and Pentacam AXL were (534.18±30.12)μm and (533.42±28.71)μm, respectively, with no significant difference ( t=1.04, P=0.30). The AL, Km, ACD, CCT and WTW from IOLMaster 700 were highly correlated with those from Pentacam AXL ( r=0.999, 0.992, 0.998, 0.956, 0.942; all at P<0.01). The Bland Altman analysis showed that the 95% limits of agreement of AL, Km, ACD, CCT and WTW were-0.02-0.11 mm, -0.27-0.59 D, -0.10-0.02 mm, -16.5-18.1 μm and 0.00-0.67 mm. Conclusions:IOLMaster 700 and Pentacam AXL have good agreements and narrow 95% limits of agreement in the measurements of AL, ACD, CCT, which can be clinically interchangeable.The 95% limits of agreement of WTW and Km are out of the clinically acceptable range, so the WTW and Km measured by the two devices are not interchangeable.

9.
Chinese Journal of Endocrinology and Metabolism ; (12): 25-30, 2020.
Article in Chinese | WPRIM | ID: wpr-798592

ABSTRACT

Objective@#To summarize the clinical characteristics of 6 children with Hutchinson-Gilford progeria syndrome, and to analyze the pathogenic genes carried by some patients.@*Methods@#The clinical data of 6 patients were summarized. The pathogenic genes of 4 families were analyzed. Genomic DNA was extracted from 3ml of the subject′s blood with EDTA anticoagulation. The first-generation sequencing technology was used to analyze the sequence of Lamin A/C(LMNA) gene and to identify the pathogenic mutation sites by comparing with normal sequencing results.@*Results@#All the children had typical clinical manifestations of the disease which has been previously reported in the literature, such as severe growth retardation, special skin manifestations, and distinctive craniofacial manifestations.Gene sequencing results revealed that 2 patients carried classical heterozygous mutation of LMNA c. 1824C>T(p.G608G). The other two patients carried atypical mutations of LMNA IVS8-4 C>A and c. 1968+ 2T>C, among which the mutation of IVS8-4 C>A has not been reported.@*Conclusions@#In Chinese children, both classical and non-classical mutations in LMNA gene lead to the occurrence of premature aging. It is easy to make a diagnosis based on clinical manifestations. Finding of the pathogenic gene may further confirm the diagnosis.

10.
Chinese Journal of Ocular Fundus Diseases ; (6): 447-452, 2020.
Article in Chinese | WPRIM | ID: wpr-871769

ABSTRACT

Objective:To investigate the clinical, ophthalmological and pathological features of primary uveal lymphoma.Methods:Retrospective clinical study. From 2012 to 2018 in Beijing Tongren Eye Cener, 4 cases and 4 eyes of patients with primary uveal lymphoma were included in the study. Among them, 3 cases were male and 1 case was female. The average age was (54 ± 13.58) years old. The average time from initial diagnosis to pathological diagnosis was (18.50 ± 9.29) months. 3 cases were enucleated and 1 case was biopsied. Extranodal marginal zone lymphoma (EMZL) of the mucosa associated lymphoid tissue (MALT) was confirmed by pathological examination. BCVA, fundus color photography, color Doppler ultrasound and orbital MRI were performed in all eyes. UBM, OCT, FFA and ICGA were performed in 2 eyes, 3 eyes, 3 eyes and 2 eyes respectively. The clinical, imaging and pathological changes were observed. Following up time was ≥ 6 months.Results:At the initial diagnosis, BCVA was 0.6, 0.02 and 0.01 in 1, 2 and 1 eye respectively. Choroid, ciliary body and iris were involved in 3 eyes, choroid in 1 eye. The fundus of the eyes showed infiltration of choroid in yellow and white color, and the lesions were beyond the vascular arch to the equator and peripheral areas. Color Doppler ultrasonography showed that choroidal diffuse thickening and extrascleral extension (ESE) which was the corresponding hypoechoic areas behind the sclera. Among them, ESE showed crescent thickening in 1 eye and nodular thickening in 3 eyes. UBM showed that the echo of ciliary body was thicken and the internal echo was decreased with the iris involved. OCT showed that RPE was wavy and local retinal neuroepithelial layer detached. FFA showed that the early lesions were mottled with strong and weak fluorescence, and the late fluorescence leakage. The posterior wall of the eyeball was thickened and enhanced in MRI.Conclusion:The clinical manifestations of uveal lymphoma are various, color Doppler ultrasound has characteristic manifestations and ESE of crescent or nodular thickening is valuable in diagnosis.

11.
Chinese Journal of Ocular Fundus Diseases ; (6): 33-37, 2020.
Article in Chinese | WPRIM | ID: wpr-871699

ABSTRACT

Objective To observe the application value with color Doppler flow imaging (CDFI) in the detection ofmacular diseases in senile cataract.Methods A cross-sectional descriptive study.From December 1 to December 30,2017,720 senile cataract patients (1380 eyes) diagnosed in Beijing Tongren Eye Center were included in this study.There were 330 males (629 eyes) and 390 females (751 eyes),with the mean age of 70.35 ± 19.05 years.There were 716 right eyes and 664 left eyes,with the visual acuity 0.05-0.8.The fundus of all the patients can be observed.All patients were examined by OCT using Cirrus HD-Model 5000 produced by German Carl Zeiss company and CDFI using MyLab90 color Doppler ultrasound system produced by ESAOTE S.P.A.Comparing the detection rate of macular lesions between the two methods by using X2 test.Using statistical methods to evaluate diagnostic tests,a crosstabulation was made to compare the consistency of frequency-domain OCT and CDFI in the diagnosis of macular lesions.Logistic regression analysis of correlation between detection of CDFI macular lesions and central foveal thickness (CFT),average thickness (AT),with or without macular anterior membrane,retinal folds,retinal cystoid edema,hard exudation,macular hole or lamellar macular hole,superficial detachment of neuroepithelium,detachment of pigment epithelium,vitreous macular traction.Results Two hundreds and thirty-nine eyes (17.3%) showed macular disease by OCT,and 161 eyes (11.7%) showed the abnormal echo in the macular region by CDFI.The detection rate of the macular disease between two methods were statistically significant (χ2=851.661,P< 0.001),however,the consistency was well (r=0.766,P=0.000).Logistic analysis showed that the detection rate of macular lesions was related to retinal folds,cysts in retina and CFT (r=1.396,1.041,0.12;P<0.01).Conclusion CDFI can effectively detect macular lesions that affect the visual prognosis of senile cataract patients.

12.
Chinese Journal of Endocrinology and Metabolism ; (12): 25-30, 2020.
Article in Chinese | WPRIM | ID: wpr-869990

ABSTRACT

Objective:To summarize the clinical characteristics of 6 children with Hutchinson-Gilford progeria syndrome, and to analyze the pathogenic genes carried by some patients.Methods:The clinical data of 6 patients were summarized. The pathogenic genes of 4 families were analyzed. Genomic DNA was extracted from 3ml of the subject′s blood with EDTA anticoagulation. The first-generation sequencing technology was used to analyze the sequence of Lamin A/C(LMNA) gene and to identify the pathogenic mutation sites by comparing with normal sequencing results.Results:All the children had typical clinical manifestations of the disease which has been previously reported in the literature, such as severe growth retardation, special skin manifestations, and distinctive craniofacial manifestations.Gene sequencing results revealed that 2 patients carried classical heterozygous mutation of LMNA c. 1824C>T(p.G608G). The other two patients carried atypical mutations of LMNA IVS8-4 C>A and c. 1968+ 2T>C, among which the mutation of IVS8-4 C>A has not been reported.Conclusions:In Chinese children, both classical and non-classical mutations in LMNA gene lead to the occurrence of premature aging. It is easy to make a diagnosis based on clinical manifestations. Finding of the pathogenic gene may further confirm the diagnosis.

13.
Clinical Medicine of China ; (12): 494-497, 2019.
Article in Chinese | WPRIM | ID: wpr-791186

ABSTRACT

Objective To evaluate the nutritional status of children with non-Hodgkin lymphoma (NHL) during induction chemotherapy, and to understand the incidence of malnutrition, so as to give nutrition intervention in time. Methods From January 2017 to December 2018,257 children with NHL were selected from the Center for Hematological Oncology,Peking Children′s Hospital Affiliated to Capital Medical University. Nutritional status and growth risk screening tools were used to assess the nutritional risk of the children,and the malnutrition was classified according to the height and body weight. The body mass,height and other anthropometric indexes, hemoglobin, prealbumin, albumin and other laboratory indexes were measured at the end of initial diagnosis and induction chemotherapy. Results There were 223 cases of moderate nutritional risk ( 86. 8%) and 34 cases of high nutritional risk ( 13. 2%) . After treatment, the nutritional status of the children decreased: 13 cases of mild malnutrition,1 case of moderate malnutrition,4 cases of overweight and 3 cases of obesity before treatment; 32 cases of mild malnutrition, 9 cases of moderate malnutrition,5 cases of severe malnutrition, 3 cases of overweight and 2 cases of obesity after treatment. Ninety-six patients received nutritional support ( 37. 4%), including 15 cases of parenteral nutrition and 81 cases of enteral nutrition. The body weight (( 29. 50 ± 15. 13 ) kg before and after chemotherapy (27. 60±14. 30) kg) and body mass index ((17. 2±3. 28) kg/m2 before and (16. 1±3. 13) kg/m2 after chemotherapy) decreased significantly (t=12. 404,13. 949,all P<0. 01) . Laboratory indicators except prealbumin were lower in hemoglobin (( 109. 00 ± 15. 78) g/L),albumin (( 37. 40 ± 4. 38) g/L), total lymphocyte ((1. 58 ± 1. 26)×109/L) than before treatment ( hemoglobin ( 117. 90 ± 21. 06) g/L), (albumin (39. 60±6. 70) g/L),(total lymphocyte (2. 98±2. 87)×109/L)) (t=6. 514,4. 834,7. 420,all P<0. 01). Conclusion There is a high incidence of malnutrition in NHL children after initial treatment. Early screening of nutritional risk and optimizing nutritional treatment are one of the ways to improve the clinical efficacy of NHL children.

14.
Chinese Journal of Ultrasonography ; (12): 251-255, 2019.
Article in Chinese | WPRIM | ID: wpr-745167

ABSTRACT

Objective To describe the ultrasonographic features of intraocular lymphoma . Methods It was a retrospective analysis of 19 patients ( 26 eyes ) with intraocular lymphoma at the department of ophthalmology ,Beijing Tongren Hospital from January 2015 to December 2017 . T he diagnosis was established by tissue biopsy in 4 patients with diagnostic vitrectomy and 8 with enucleation ,and other 7 cases were clinically diagnosed followed by pathological examination of central nervous system lymphoma . T he ultrasonographic features of all patients were analyzed . Results Of the 19 patients ( 26 eyes ) with intraocular lymphoma ,12 cases ( 19 eyes) of vitreoretinal lymphoma accounted for 63 .2% ,of which 7 cases were bilateral eyes ,and 7 cases ( 7 eyes ) of uveal lymphoma accounted for 36 .8% ,all of w hich were monocular . Ultrasonography of vitreoretinal lymphoma included vitreous opacity , posterior vitreous detachment ,partial retinal detachment ,irregular hypoechoic lesions under the retina ,and CDFI detected blood flow signals in the lesion . T here were two different types of ultrasound findings in uveal lymphoma . Five cases ( 5 eyes ) showed diffuse thickening of the uveal membrane with low echo , and irregular hypoechoic mass around the optic nerve and the ball wall ,in w hich CDFI detected abundant blood flow signals . Ultrasound biological microscope examination showed that the iris‐ciliary body thickened and the internal echo decreased . T his ultrasound finding accounted for 71 .4% of uveal lymphoma . T wo cases ( 2 eyes) showed a solitary ciliary‐choroid choroidal hypoechoic mass ,combined with retinal detachment and blood flow signals with CDFI ,accounting for 28 .6% of uveal lymphoma . Conclusions T he ultrasonography of intraocular lymphoma is various . T he ultrasound findings of vitreoretinal lymphoma are not specific , w hile the diffuse thickened uvea and retrobulbar hypoechoic lesions encircled optic nerve and eye wall suggest the possibility of uveal lymphoma .

15.
Clinical Medicine of China ; (12): 268-271, 2019.
Article in Chinese | WPRIM | ID: wpr-744998

ABSTRACT

Objective To explore the related factors affecting the quality of life of children undergoing hematopoietic stem cell transplantation.Methods A total of 42 children (transplantation group) who received hematopoietic stem cell transplantation successfully from February 2010 to January 2017 in Beijing Children's Hospital were investigated with the Chinese version of the Universal Core Questionnaire for Quality of Life for Children 4.0.40 healthy children matched with their age and sex were selected as healthy control group by 1 ∶ 1 matching method.To compare the quality of life between the two groups and to analyze the factors influencing the quality of life after hematopoietic stem cell transplantation.Results The age of transplantation group was (10.32±3.94) years,and the average time after transplantation was (2.76± 1.15) years.The body weight and school attendance of transplantation group were lower than those of healthy group (P<0.05),and the scores of physiology and school function of transplantation group were lower than those of healthy group ((82.72 ± 13.40) points vs.(91.32 ± 5.90) points,(75.12 ± 15.66) points vs.(88.33 ± 10.01) points,t =3.72,6.18,all P<0.05)).The scores of three dimensions of quantity,affective function and social function were similar to those of healthy group,and there was no significant difference (P >0.05).Regression analysis showed that chronic graft-versus-host disease was the only factor related to quality of life (P =0.030).Conclusion The quality of life of transplant survivors is similar to that of healthy children of the same age,and the existence of chronic graft-versus-host disease is related to the overall poor quality of life.

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Chinese Journal of Infection and Chemotherapy ; (6): 42-47, 2019.
Article in Chinese | WPRIM | ID: wpr-744592

ABSTRACT

Objective To understand the etiology and clinical characteristics of candidemia, and the diagnostic value of(1, 3)-β-Dglucan. Methods This analysis included 31 patients with candidemia, 39 patients with Candida deep colonization, and 50 healthy subjects, who were enrolled from Wuyi Chinese Hospital Affiliated to Jinan University during the period from September 2015 to March 2018. Clinical data such as fungal culture, clinical features, and serum(1, 3)-β-D-glucan level were analyzed and compared between groups. Results Overall, 13 strains of Candida albicans and 18 strains of non-albicans Candida were isolated from the 31 patients with candidemia. The incidence of candidemia was associated with old age(>65 years), prior surgery(≤7 days), indwelling catheter(≥2 catheters and lasting ≥5 days), duration of mechanical ventilation(≥5 days)(P<0.05), but not associated with sex, underlying disease, prior use of anmicrobial and antifungal agents, immunosuppressant or glucocorticoid, or hypoproteinemia(P>0.05). Serum level of(1, 3)-β-D-glucan was significantly higher in candidemia patients than in the patients with candida deep colonization and healthy controls(P<0.05). The sensitivity and specificity of(1, 3)-β-D-glucan in diagnosing candidemia were 87.1% and 96.0%, respectively. The level of(1, 3)-β-D-glucan was significantly higher in candidemia with bacterial infection than candidemia without bacterial infection(P<0.05). The level of of(1, 3)-β-D-glucan was also significantly higher in the candidemia caused by Candida albicans alone than that caused by non-albicans Candida alone(P<0.05). Conclusions Non-albicans Candida is more prevalent than Candida albicans in candidiasis, but Candida albicans is still the most important Candida species. The incidence of candidemia is asociated with advanced age(>65 years), prior surgery within 7 days, indwelling catheters(≥2 catheters and lasting ≥5 days), and mechanical ventilation(≥5 days). Serum(1, 3)-β-D-glucan level is valuable for early diagnosis of candidemia.

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Chinese Journal of Experimental Ophthalmology ; (12): 292-296, 2019.
Article in Chinese | WPRIM | ID: wpr-744033

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Objective To compare the agreement of axial length (AL),anterior chamber depth (ACD) and keratometry measured by IOLMaster 500 with IOLMaster 700 in cataract eyes with high myopia.Methods A cross-sectional study was performed.One hundred and fifty-two eyes of 87 cataract patients with high myopia were included from September to October 2017 in Beijing Tongren Hospital.The best corrected visual acuity (BCVA) was 0.05-1.0,and the refraction power was-6.25--21.00 D,with an average power of (-13.625 ± 7.375) D.A L,ACD and keratometry (Kf,Ks) were measured by IOLMaster 700 and IOLMaster 500 respectively for all the eyes.The eyes were assigned to AL>26-≤28 mm group,AL>28-<30 mm group and AL≥30 mm according to the results of IOLMaster 500.The measured differences between two devices were assessed using paired samples t-test,and the measured consistency of the two devices was evaluated using Bland-Altman agreement analysis.This study protocol was approved by Ethics Committee of Beijing Tongren Hospital and complied with Declaration of Helsinki.Results There were no significant differences in the AL and Kf measured by the two devices in all the three groups (all at P>0.05).Both ACD and Ks values measured by IOLMaster 700 were lower than those by IOLMaster 500 (both at P<0.05),which were clinically acceptable.The percentage beyond 95% agreement limit between IOLMaster 700 and IOLMaster 500 were 4.10%,2.17% and 3.03% for AL differences and 4.10%,4.34% 和 3.03% for Kf difference in the three groups.Significant differences were found in ACD and Ks between IOLMaster 700 and IOLMaster 500 at a clinically acceptable level (both at P<0.05),and Bland-Altamn agreement analysis showed that the percentage beyond 95% agreement limit between IOLMaster 700 and IOLMaster 500 were 4.10%,4.34% and 3.03% for both ACD and Ks differences in the three groups.Conclusions There is a good agreement in AL,ACD,Kf and Ks measured by IOLMaster 700 with IOLMaster 500 and is clinically interchangeable in cataract eyes with high myopia.However,IOLMaster 700 is better because it can provide the OCT image of macula.

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Chinese Journal of Health Management ; (6): 212-215, 2018.
Article in Chinese | WPRIM | ID: wpr-709001

ABSTRACT

Objective To validate bioelectrical impedance analysis(BIA) for measuring the area of visceral fat of school-age populations using MRI as a reference method.Methods Sixty healthy children (30 boys and 30 girls) aged 7 to 18 years were enrolled in the study.Both MRI and BIA were completed in seven days.We obtained the indexes of the body composition from BIA and MRI in the morning before the participants had their breakfast.For reference,the navel plane image obtained with MRI was used to analyze the area of adipose tissue.With BIA,the area of visceral adipose tissue could be derived directly.Results Sixty healthy school-age children were enrolled in the study.The average age of the school children was (11.9±4.6) years,and their BMI was (22.8±7.3) kg/m2.The average area of visceral fat obtained with BIA was (85.4±64.2) cm2,which was higher than that obtained with MRI [(49.4±32.4) cm2](t=-6.524,P =0.000).The Pearson correlation coefficient was r=0.806 (P=0.000) and the simple linear regression equation for the areabetween the two was:y=0.41x + 14.78;β (95%CI) was 0.41(0.04-0.81).The Bland-Altman plot was (x-) ±1.96 s:(35.9± 83.7) cm2.Conclusion BIA is comparable to MRI for assessing the area of visceral fat and is a reliable and simple tool to evaluate the visceral fat area in school-age children.

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Chinese Journal of Medical Ultrasound (Electronic Edition) ; (12): 734-737, 2017.
Article in Chinese | WPRIM | ID: wpr-712021

ABSTRACT

Objective To explore the diagnostic value of ultrasonic elastography in choroidal melanoma. Methods A total of 96 cases (97 eyes) of choroidal melanoma were included in present study from June 2016 to June 2017 in Eye Center of Beijing Tongren Hospital, Capital Medical University. The control group consisted of 18 patients with choroidal hemangioma (18 eyes). All eyes were examined by color Doppler ultrasound and ultrasonic elastosonography. The strain ratio of the tumor and surrounding normal tissue was calculated for each case. By comparing the strain ratio between choroidal melanoma and choroidal hemangioma, the diagnostic value of ultrasound elastography in choroidal melanoma was investigated. Results The strain ratios of the two groups were 39.51±15.79 for choroidal melanoma and 13.15±10.40 for choroidal hemangioma. The independent sample t-test was performed between the two sets of data. The difference of the strain ratio between choroidal melanoma and choroidal hemangioma was statistically significant (t=6.802, P<0.001). Conclusions The stiffness of ocular malignant tumor (choroidal melanoma) is significantly higher than that in intraocular benign tumor (choroidal hemangioma). Ultrasonic elastography can be used as an important examination for the diagnosis and differential diagnosis of choroidal melanoma.

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Chinese Journal of Medical Ultrasound (Electronic Edition) ; (12): 730-733, 2017.
Article in Chinese | WPRIM | ID: wpr-712020

ABSTRACT

Objective To explore the value of conventional ultrasound, color Doppler ultrasound and contrast-enhanced ultrasound (CEUS) in the diagnosis of sub-retinal hemorrhage. Methods A total of 26 patients with sub-retinal hemorrhage were examined by conventional ultrasound, color Doppler ultrasound and CEUS. The results of medical history, ultrasound, color Doppler ultrasound and CEUS were recorded and analyzed.And the difference of the detection rate between the three methods was analyzed by χ2test. Results The mean fundus diameter of the sub-retinal hemorrhage was (5.31±2.21) mm, and the mean height was (1.97±0.98) mm. In twenty-two eyes (84.6%) the lesions were located in the posterior pole of the ocular wall, while in two eyes (7.7%) the lesions were located in the equator and peripheral of the ocular wall. Seven eyes (26.9%) had dome-shaped mass, ten eyes (38.5%) had flat-shaped mass, and nine eyes (34.9%) had irregular-shaped mass. The echogenecity of the lesions was homogeneous in twelve eyes (46.2%), and heterogeneous in fourteen eyes (53.8%). The lesions showed clear margin in seventeen eyes (65.4%). Blood flow signals can be detected in twenty-three eyes (88.5%). The surface of the lesions was filled by contrast-medium in all cases. The detection rate of sub-retinal hemorrhage with color Doppler and contrast-enhanced ultrasound was higher than that of conventional ultrasound, and the difference was statistically significant. Conclusion Color Doppler ultrasonography and CEUS can provide a powerful basis for the clinical diagnosis of sub-retinal hemorrhage, while CEUS has higher application value.

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