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Objective:To observe and analyze the clinical characteristics of children who died of intraocular retinoblastoma (RB).Methods:A retrospective clinical study. Fourteen children (23 eyes) with intraocular RB who died after receiving treatment in Beijing Children's Hospital from 2009 to 2017 were included in the study. Among the children, there were 7 males (10 eyes) and 7 females (13 eyes); 5 had unilateral and 9 had bilateral tumor. Age were 17.2±15.5 months. All children underwent RetCam examination. RB was staged according to the international intraocular RB classify. Among the 23 eyes, 1 eye was in stage B, 2 eyes were in stage C, 12 eyes in stage D, and 8 eyes in stage E. Treatment methods included a systemic (vincristine, etoposide and carboplatin) chemotherapy (VEC chemotherapy), enucleation surgery, and vitrectomy. The basic conditions including age, time of diagnosis, pathological diagnosis, treatment and main causes of death were retrospectively analyzed.Results:Among the 14 cases, the first symptom was leukemia in 12 cases, red eye in 1 case, and squintin in 1 case. Systemic VEC chemotherapy was used for 1-6 courses of treatment; 5 cases were enucleated, 3 cases underwent histopathological examination; 3 cases were treated with vitrectomy. Among the 3 cases who underwent histopathological examination, the sclera and optic nerve, optic nerve and optic disc were invasted respectively. Seven patients died of tumor metastasis and/or intracranial lesions (50.0%, 7/14); the median survival time was 19 months. Four patients died of treatment (28.6%, 4/14), including 3 patients died of chemotherapy-related side effects, and 1 died of organ failure after enucleation surgery (7.1%); the median survival time was 3.5 months. Early abandonment of treatment died in 3 cases (21.4%, 3/14); the median survival time was 15 months.Conclusion:Intracranial metastasis is the main cause of death in children with intraocular RB.
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Objective:To compare the clinical efficacy and complications of intra-arterial chemotherapy (IAC) and intravenous chemotherapy (IVC) for unilateral advanced retinoblastoma (RB).Methods:A retrospective clinical study. From January 2020 to January 2021, 40 patients (40 eyes) unilateral group cT2 RB patients diagnosed at Baoding Children’s Hospital and Beijing Children’s Hospital were recruited in this study. There were 22 males (22 eyes) and 18 females (18 eyes). All were monocular. All the patients were assigned to two groups according to different treatment modalities they received: IVC group and IAC group. There were 26 eyes and 14 eyes, respectively. When the tumor invades the optic nerve, choroid, sclera, anterior chamber and iris, enucleation was performed. The globe salvage rate, tumor extraocular metastasis rate, solid tumor control rate, treatment-related complications and pathological high-risk factors after enucleation were observed. The globe salvage rate and solid tumor control rate were compared between the groups by chi square test.Results:The globe salvage rate of IAC group and IVC group were 88.5% (23/26) and 50.0% (7/14), respectively. Solid tumor control of IAC group and IVC group were 84.6% (22/26) and 42.9% (6/14), respectively. There were statistically significant differences in globe salvage rate and solid tumor control between the two groups ( χ 2=7.18, 7.56; P<0.05). Compared with IVC group, IAC group had less systemic complications, mild ocular and periocular side effects. Among 26 cases in IAC group and 14 cases in IVC group, 3 and 7 cases underwent enucleation respectively. The results of pathological examination showed that there were 2 cases and 3 cases with pathological high-risk factors in the two groups, respectively. During the follow-up period, 2 cases in IAC group had extraocular metastasis, there was no extraocular metastasis in IVC group. Conclusion:Compared with IVC, IAC has the advantages of high tumor control rate, high globe salvage rate, less and mild complications, however, there is still tumor recurrence.
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Objective:To investigate the effect of Zhitong-Rusheng Decoction on wound healing and serum fibronectin (FN) and epidermal growth factor (EGF) levels after anal fistula operation. Methods:A total of 102 patients after anal fistula operation in our hospital from March 2018 to March 2020 were randomly divided into control group (51 cases) and observation group (51 cases) according to the random number table method. The control group was treated with conventional western treatment, while the observation group was treated with Zhitong-Rusheng Decoction on the basis of control group. The two groups were treated for 3 weeks. The clinical symptoms were scored before and after treatment, and the levels of serum FN and EGF were detected by ELISA. The wound healing time, hematochezia disappearance time, wound longitudinal diameter and wound area before and after treatment were observed. The clinical efficacy was evaluated after treatment. Results:The total effective rate was 98.0% (50/51) in the observation group and 72.6% (37/51) in the control group, with significant difference between the two groups ( χ2=13.209, P<0.01). After treatment, the pain degree score, wound exudation score and granulation morphology score of the observation group were significantly lower than those in the control group ( t=15.448, 21.424, 28.641, P<0.001). After treatment, the wound healing time [(6. 04 ± 1.20) d vs. (9.42 ± 1.58) d, t=12.166] and the disappearance time of hematochezia [(15.72 ± 2.86) d vs. (19.95 ± 4.33) d, t=6.862] of the observation group were significantly shorter than thosse of the control group( P<0.01). After treatment, the wound longitudinal diameter [(1.89 ± 0.31) cm vs. (2.82 ± 0.54) cm, t=10.666] and wound area [(3.26 ± 0.54) cm 2vs. (4.98 ± 1.10) cm 2, t=10. 024] of the observation group were significantly less than those in the control group ( P<0.01). The serum FN [(2.92 ± 0.50) mg/L vs. (2.45 ± 0.39) mg/L, t=5.293], EGF [(7.03 ± 1.44) μg/L vs. (5.47 ± 1.03) μg/L, t=6.293] of the observation group were significantly higher than those in the control group ( P<0.01). Conclusion:Zhitong-Rusheng Decoction on the basis of conventional treatment on patients after anal fistula operation has a good efficacy, which is conducive to wound healing, reducing clinical symptoms, and improving serum levels of FN and EGF.
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Circular RNAs are a group of novel non-coding RNA molecules, which form covalently closed continuous loop structures, lacking the terminal 5′ and 3′ ends. They are abundant in eukaryotes and have recently become an increasing area of research in the field of RNA. Circular RNAs have many functions, such as abundant expression, cell- or tissue-specific expression, and high resistance to RNase-induced degradation. They are closely related to tissue development and the occurrence and development of diseases, play an important role in the pathogenesis and development of a variety of diseases, and can be used as a novel molecular marker of some diseases. Therefore, this review summarizes the latest research progress of circular RNAs in the regulation of endocrine diseases to better understand the biological mechanisms underlying endocrine diseases.
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Objective To analezk thk altkrations in blood urka nitrogkn( BRN)and skrum crkatinink( Scr) in childrkn with rktinoblastoma( Ab)bkfork and aftkr chkmothkrape and thk clinical significanck of thk chkmothkrape kffkct,and to providk thk kvidknck for thk furthkr improvkmknt of thk safkte of trkatmknt. Methods L total of 280 chil-drkn with Ab wkrk knrollkd in thk stude,and kach of thkm was trkatkd with CEV( Carboplatin+Etoposidk+Vincris-tink)mkthods. Thkrk subjkcts includkd 153 malks and 127 fkmalks,with a mkan trkatmknt of 4. 5 ceclks(rangk 2 to 12 ceclks)and a mkan agk of 21. 5 months(rangk 1 to 84 months). Lmong thkm,149 casks wkrk diagnoskd clinicalle,131 casks wkrk diagnoskd pathologicalle. Eight casks wkrk in thk kxtraocular stagk,3 casks wkrk in glaucoma and 269 casks in intraocular pkriod(101 casks of singlk keks and 168 casks of doublk keks). BRN and Scr wkrk dktkctkd bkfork thk first coursk of chkmothkrape and aftkr thk last coursk of chkmothkrape. ResuIts BRN and Scr valuks wkrk analezkd bk-fork and aftkr chkmothkrape. BRN was 3. 05 mmol╱F bkfork chkmothkrape and 3. 46 mmol╱F aftkr chkmothkrape in thk group agkd from 4 months to lkss than 12 months(73 casks),thk valuks of BRN aftkr chkmothkrape was highkr than that bkfork chkmothkrape,and onle in this group thk changk was statisticalle diffkrknt(t﹦ -2. 829,P﹦0. 006),but all BRN valuks in this group wkrk not bkeond thk highkst rkfkrknck valuk(1. 70 mmol╱F-7. 10 mmol╱F). Bkfork initial chkmothkrape,149 patiknts( 53. 2﹪)had Scr bklow thk rkfkrknck rangk( malk:30 -104 μmol╱F,fkmalk:30 -84 μmol╱F),and 20 casks(7. 0﹪)had thk BRN bklow thk rkfkrknck valuk. In 2 casks,BRN(7. 25 mmol╱F and 7. 34 mmol╱F, rkspkctivkle)bkfork thk initial chkmothkrape was slightle highkr than thk normal valuk,but thk valuk was normal(5. 01 mmol╱F and 4. 98 mmol╱F,rkspkctivkle)aftkr thk last chkmothkrape. In onk cask,thk BRN(5. 62 mmol╱F)was normal bkfork thk initial chkmothkrape,but it was klkvatkd(7. 33 mmol╱F)aftkr thk last chkmothkrape. In anothkr onk cask,thk BRN was normal bkfork and aftkr chkmothkrape,but thk valuk aftkr chkmothkrape was 4. 69 timks highkr than that bk-fork chkmothkrape. ConcIusions Aknal function of Ab childrkn bkfork trkatmknt is normal. Skvkn pkrcknt of thksk patiknts havk BRN undkr thk BRN rkfkrknck rangk,and 53. 2﹪ of thksk patiknts havk Scr undkr thk Scr rkfkrknck rangk. It suggkstkd that thk rkfkrknck valuks of BRN and Scr nkkd to bk adjustkd. BRN of infant Ab mae incrkask signifi-cantle aftkr chkmothkrape,but it doks not mkkt thk currknt diagnostic critkria of mild nkphrotoxicite. Still,thk karle rknal damagk nkkds to bk notickd.
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Background Peters anomaly is a rare eye disease.Understanding the clinical features of Peters anomaly is helpful for us to correctly manage this disease.Objective This study was to investigate the clinical characteristics and treatment methods of Peters anomaly and provide available basis for the diagnosis and individualized treatment of Peters abnormal.Methods A descriptive study was performed.The clinical data from 8 eyes of 4 patients with Peters anomaly who were diagnosed and treated in Beijing Children's Hospital from January 1,2014 to March 30,2016 were retrospectively analyzed.The clinical manifestations of the anterior ocular segment were examined by handheld slit-lamp microscope,and corneal curvature,corneal thickness,axial length and intraocular pressure were measured.The therapy and outcomes of the patients were evaluated.Results Peters anomaly was classified into type Ⅰ,type Ⅱ and Peters plus syndrome based on the findings and literature'criteria.Regarding to the clinical findings,the adhesion of peripheral angle with cornea,shallow or disappeared anterior chamber were found in all the eyes,and these manifestations were type Ⅰ Peters anomaly and occurred in 2 eyes of 2 patients.The opacity and adhesion of the central lens capsula to corneal endothelium was seen in 6 eyes which belonged to type Ⅱ Peters anomaly.The mean corneal thickness was (680± 127) μm in 4 measured eyes,which was thicker than normal eyes;the mean corneal curvature was (37.40±1.79)D in 4 measured eyes;the mean axial length was (21.06±0.19)mm and the mean diameter of cornea was (9.4 ± 0.5) mm in 4 measured eyes.Iridocoloboma was found in 4 eyes and congenital aniridia was in 4 eyes.In addition,esotropia appeared in 1 patient,and nystagmus occurred in 3 patients.The 3 patients of type Ⅱ in this study were found to have the disorders associated with systemic neurogenic developmental abnormalities and diagnosed as Peters plus syndrome.Trabeculectomy with anterior vitrectomy was performed in 2 eyes with the intraocular pressure ＞30 mmHg(1 mmHg =0.133k Pa) and the introcular pressure reduced to below 21 mmHg after operation.Penetrating corneal transplantation was carried out on 1 eye with central opacity.However,rejection of graft occurred at 1 month and became complete cloudy at 3 months after surgery.Cataract extraction surgery was performed on 4 eyes with lens opacity and intraocular lens were implanted in 2 eyes or iridectomy was carried out in another 2 eyes,and the visual quality was evidently improved after operation.Conclusions The clinical characteristics of Peters anomaly include leukoma,shallow anterior chamber,peripheral iris adhesion with cornea and lens opacity due to adhesion of lens anterior capsule to corneal endothelium.Personalized operative regimen should be determined based on the clinical findings of the eyes with Peters anomaly.
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Background Malignant osteopetrosis is an extremely rare dense bone disease,and sometimes features ocular disease and cranial nerve palsy.This disease received high attention because of its poor prognosis.And whether the eye manifestation improved after treatment is a problem for concern.Objective This study was to clarify the clinical manifestation,treatment and prognosis of malignant osteopetrosis associated with ocular disease.Methods A retrospective study was adopted.Two children with malignant osteopetrosis associated with eye symptoms were collected from Beijing Children Hospital.The systemic and ocular medical examinations were performed on the patients,including physical examination,hematology laboratory examination,abdominal B ultrasound and bone X ray radiography,external ocular examination,flash visual evoked potential (F-VEP) and CT of orbit.Bone marrow hematopoietic stem cell transplantation was employed and 5-year following-up was cinducted on the chidren.Results The children showed increased bone density,systemic bone sclerosis,basilar thickening,abnormalities of hematology indexes,anemia,hepatosplenomegaly,optical canal stenosis and abnormality of F-VEP P2 wave.In addition,optical disc pale,facial paralysis and paralytic esotropia were seen in a female child and alternating strabismus was found in another boy.After successful treatment,the systemic symptoms remitted in both children,but the eye findings remained unchanged in the female child during the follow-up duration.However,the strabismus diminished in the male child.The optical bone canal widening to 1.9 mm 1 year and 3.2 mm 5 years after treatment in the female child.Conclusions Strabismus and eye disease are the signs of malignant infantile osteopetrosis and reflections of the impairment of the central nervous system.The pathogenetic mechanism of malignant osteopetrosisrelated eye disease is below understanding now.Early bone marrow hematopoietic stem cell transplantation for malignant osteopetrosis can offer the best chance of long-term survival and improve the prognosis of eye diseases.