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Objective:To identify the method to reduce X-ray exposure during ablation of atrial fibrillation (AF) by comparing the cryoballoon (CRYO) ablation and remote magnetic navigation (RMN) ablation.Methods:A retrospective analysis was conducted on 144 patients undergoing CRYO ablation (CRYO group) and 121 patients undergoing RMN ablation (RMN group) in our hospital. Entrance surface doses at reference points online, exposure time during procedure and outcomes were analyzed for different types of patients.Results:Compared with the RMN group, the procedure time for the CRYO group significantly decreased [(165.0±23.6), (97.8±18.4) min, t=26.05, P<0.001]. However, the entrance surface dose value [(232.3±130.7), (669.0±387.5) mGy, Z=-12.29, P<0.001] and X-ray exposure time [(8.1±3.1), (23.4±6.2) min, t=-24.57, P<0.001] increased significantly for the CRYO group. No significant difference was found between the two groups in the proportion of maintaining sinus rhythm during follow-up of patients (71.9%, 75.7%, P=0.618). Multiple regression analysis showed that obese patients, patients with non-paroxysmal AF and patients with variant pulmonary veins were associated with an increase in entrance surface dose values in the CRYO group ( t=5.47, 2.23, 3.39, P<0.05). The X-ray exposure time for the three types patients above in the CRYO group also increased ( t=2.87, 3.86, 3.25, P<0.05) in the CRYO group. However, only obese patients in the RMN group had an increase in entrance surface dose value ( Z=-4.15, P<0.001) and no increase in exposure time. For the three types of patients above, there was no significant difference in proportion of maintaining sinus rhythm between the CRYO group and the RMN group during follow-up ( P>0.05). Conclusions:Compared with RMN ablation, the radiation exposure of CRYO AF ablation significantly increased, especially in obese patients, patients with non-paroxysmal AF and patients with pulmonary veins variation. The use of RMN for these types of patients may reduce the radiation exposure without affecting the procedure outcomes.
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Objective:To understand the clinical characteristics and prognosis of Langerhans cell histiocytosis (LCH) with skin-limited lesion.Methods:A retrospective analysis was performed on clinical characteristics and prognosis of 16 skin-limited LCH patients, out of 578 LCH patients who were hospitalized in Beijing Children′s Hospital during December 2013 to June 2018.Results:A total of 16 skin-limited LCH cases, accounted for 2.7% of all 578 cases, were included.Among which, sex ratio (male vs.female) was 1.28∶1.00.Median ages of skin eruption occurrence and of diagnosis of the disease were 3.5 months (3 days to 2 years and 5 months) and 6 months (2 months 14 days to 2 years and 8 months) in this group.Among the 16 cases, seborrheic dermatitis-like lesions(11 cases, 68.7%) was the most common, and the trunk was most frequently involved[75.0% (12 cases)]. Serine/threonine protein kinase gene V600E [ BRAF (p.V600E)] mutation was detected in pathological specimens from 10 skin-limi-ted cases, with 9 cases being positive.Plasma samples from 5 positive cases were further detected for BRAF (p.V600E) mutation, and 4 positive results were gained.Of all 16 patients, 11 cases (68.7%) were treated.Remission were achieved in 3-6 months from treatment start in patients treated whether according to the Histiocyte Society′s LCH-2009 protocol for 25 weeks(6 cases, 37.5%), or with topical mometasonefuroate for 3 months (3 cases, 18.8%). Two patients(12.5%) with solitary cutaneous lesions underwent excision biopsy (one face and one prepuce) and were considered to be in remission immediately after surgery.None of these patients suffered from the recurrence of the disease.The remaining 5 patients (31.3%) with skin-limited LCH were just evaluated regularly, and achieved remission in 3-6 months of commencing observation.Among these untreated patients, 1 with consistently positive BRAF (p.V600E) mutation in plasma had bone involvement in the 24 th month of assessment, and was then treated based on the Histiocyte Society′s LCH-2009 Protocol.No clinical or imageological evidence supporting disease progression was found on this patient.Median follow-up period was 32.8 months (2.9-63.9 months). Except one patient, none of the rest cases had active disease till follow-up ended.Two-year event free survival(EFS) of this research was (92.3± 7.4)%.There was no significant difference between EFS of treated group and that of observation group( χ2=1.250, P=0.264). Conclusions:Skin-limited LCH often occurs in infants and newborns, with strong heterogeneity in clinical manifestations, laboratory indicators, and pathogenesis.Seborrheic dermatitis-like lesions were the most common cutaneous type.The prognosis of the patients is excellent despite progressing into multisystem involvement can be seen in a few patients.
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Objective To investigate the clinical characteristics and outcomes of pediatric Langerhans cell histiocytosis (LCH) with craniofacial bone involvement.Methods A retrospective analysis was performed on 145 pediatric LCH patients with craniofacial bone involvement registered at Beijing Children's Hospital Affiliated to Capital Medical University from January 2007 to July 2013.The patients were divided into 2 groups:central nervous system risk craniofacial bone involvement group(CNS-RISK) and non-central nervous system risk craniofacial bone involvement group(non-CNS-RISK).All patients were assessed at 5 weeks,11 weeks,25 weeks and 52 weeks respectively after chemotherapy started,and 3 months,6 months,1 year and 3 years after chemotherapy withdrawal.Statistics and related risk analysis was performed respectively.Results A total of 145 craniofacial bone involved LCH cases were included,which was composed of 62.5％ of 232 LCH cases hospitalized during the same period.The median age of these patients was 29 months,and median follow-up time period was 31 months.The most commonly involved craniofacial bone was parietal bone(78 cases,53.8％),followed by temporal bone(59 cases,40.7％) and frontal bone(57 cases,39.3％).The onset age was significantly different (26 months vs.54 months,Z =-2.777,P ＜ 0.05) between CNS-RISK group (103 cases) and non-CNS-RISK group (42 cases).Moreover,compared with non-CNS-RISK group,CNS-RISK group showed higher ratio of patients classified as multisystem involvement of risk organs (72/103 cases,69.9％) vs.(15/42 cases,35.7％) (x2 =16.908,P ＜ 0.05),and a higher rate of overall relapse rate (45/103 cases,43.7％) vs.(7/42 cases,16.7％) (x2 =9.427,P ＜ 0.05),a lower survival rate of 3-year relapse-free survival rate [(66.9 ± 5.7) ％ vs.(88.2 ± 7.8) ％,Z =2.205,P ＜ 0.05].The incidence of diabetes insipidus was 13.7％ in 232 LCH patients.Compared with patients without craniofacial bone involvement,patients with craniofacial bone involvement demonstrated a higher rate of diabetes insipidus [(27/145 cases,18.6％) vs.(5/87 cases,5.7％),x2 =7.579,P =0.006].But the incidence of diabetes insipidus showed no statistical difference between CNS-RISK group and non-CNS-RISK group (21.3 ％ and 11.9 ％,x2 =1.760,P =0.185).Diabetes insipidus was not found in single system LCH with Single-Bone CNS-RISK lesions.Till the end of follow-up,1 out of 145 patients died.Among 145 patients,5 cases had a single-bone CNS-RISK lesion.They received systemic chemotherapy.One showed reactivation,and none of them died.Multivariate analysis of variance showed that all the independent factors indicating diabetes insipidus included parietal bone,frontal bone,maxilla and mandible involvement (HR =2.697,3.487,5.425,all P ＜ 0.05),while independent factors indicating relapse included temporal bone,maxilla and mandible involvement (HR =3.712,3.380,all P ＜ 0.05).Conclusions Among involved craniofacial bones,the parietal bone is most commonly involved.LCH occurs averagely at an earlier age in CNS-RISK group,along with lower 3-year relapse-free survival rate,high relapse rate,and more patients classified as multisystem LCH involvement of risk organs.The incidence of diabetes insipidus in children with craniofacial bone involvement with single system CNS-RISK is low.Patients with the parietal bone,frontal bone,maxilla and mandible involvement at diagnosis are at a increasing risk a significantly to develop DI during the course of disease.
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Objective@#To investigate the clinical characteristics and outcomes of pediatric Langerhans cell histiocytosis (LCH) with craniofacial bone involvement.@*Methods@#A retrospective analysis was performed on 145 pediatric LCH patients with craniofacial bone involvement registered at Beijing Children′s Hospital Affiliated to Capital Medical University from January 2007 to July 2013.The patients were divided into 2 groups: central nervous system risk craniofacial bone involvement group(CNS-RISK) and non-central nervous system risk craniofacial bone involvement group(non-CNS-RISK). All patients were assessed at 5 weeks, 11 weeks, 25 weeks and 52 weeks respectively after chemotherapy started, and 3 months, 6 months, 1 year and 3 years after chemotherapy withdrawal.Statistics and related risk analysis was performed respectively.@*Results@#A total of 145 craniofacial bone involved LCH cases were included, which was composed of 62.5% of 232 LCH cases hospitalized during the same period.The median age of these patients was 29 months, and median follow-up time period was 31 months.The most commonly involved craniofacial bone was parietal bone(78 cases, 53.8%), followed by temporal bone(59 cases, 40.7%) and frontal bone(57 cases, 39.3%). The onset age was significantly different (26 months vs.54 months, Z=-2.777, P<0.05) between CNS-RISK group (103 cases) and non-CNS-RISK group (42 cases). Moreover, compared with non-CNS-RISK group, CNS-RISK group showed higher ratio of patients classified as multisystem involvement of risk organs (72/103 cases, 69.9%)vs.(15/42 cases, 35.7%)(χ2=16.908, P<0.05), and a higher rate of overall relapse rate (45/103 cases, 43.7%) vs. (7/42 cases, 16.7%) (χ2=9.427, P<0.05), a lower survival rate of 3-year relapse-free survival rate [(66.9±5.7)% vs.(88.2±7.8)%, Z=2.205, P<0.05]. The incidence of diabetes insipidus was 13.7% in 232 LCH patients.Compared with patients without craniofacial bone involvement, patients with craniofacial bone involvement demonstrated a higher rate of diabetes insipidus [(27/145 cases, 18.6%) vs.(5/87 cases, 5.7%), χ2=7.579, P=0.006]. But the incidence of diabetes insipidus showed no statistical difference between CNS-RISK group and non-CNS-RISK group (21.3% and 11.9%, χ2=1.760, P=0.185). Diabetes insipidus was not found in single system LCH with Single-Bone CNS-RISK lesions.Till the end of follow-up, 1 out of 145 patients died.Among 145 patients, 5 cases had a single-bone CNS-RISK lesion.They received systemic chemotherapy.One showed reactivation, and none of them died.Multivariate analysis of variance showed that all the independent factors indicating diabetes insipidus included parietal bone, frontal bone, maxilla and mandible involvement(HR=2.697, 3.487, 5.425, all P<0.05), while independent factors indicating relapse included temporal bone, maxilla and mandible involvement(HR=3.712, 3.380, all P<0.05).@*Conclusions@#Among involved craniofacial bones, the parietal bone is most commonly involved.LCH occurs averagely at an earlier age in CNS-RISK group, along with lower 3-year relapse-free survival rate, high relapse rate, and more patients classified as multisystem LCH involvement of risk organs.The incidence of diabetes insipidus in children with craniofacial bone involvement with single system CNS-RISK is low.Patients with the parietal bone, frontal bone, maxilla and mandible involvement at diagnosis are at a increasing risk a significantly to develop DI during the course of disease.
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Objective To explore the clinical features of Langerhans cell histiocytosis (LCH) involving the thyroid gland in children,in order to improve the diagnosis and treatment.Methods The clinical and imaging manifestations,thyroid function,treatment and prognosis of hospitalized children with LCH involving the thyroid gland in Beijing Children's Hospital,Capital Medical University,from July 2007 to July 2016 were analyzed retrospectively.Results In total 556 cases with LCH were analyzed,among which 8 cases (1.44％) were with LCH involving the thyroid gland.The onset age of children with LCH involving the thyroid gland was significantly older than the others with significant difference (average onset age:7.6 vs.3.4 year old;t =2.748,P =0.006),while the sex distribution showed no significant difference (1.67 ∶ 1.00 vs.1.38 ∶ 1.00;x2 =0.064,P =0.799).All 8 cases were Group Ⅰ,complicated by multiple organ involvement with vital organs included.None of the 8 cases had significant clinical symptoms of hypothyroidism,and thyroid imaging abnormalities were found in all 8 cases,including goiters and low echoes with irregular shapes,while 5 cases had subclinical hypothyroidism.All 8 cases were given the first-line standard chemotherapy for LCH-Group Ⅰ.Three cases without subclinical hypothyroidism showed good treatment effects and were assessed as non-active state and had already quitted medication.Five cases complicated by subclinical hypothyroidism had unsatisfactory treatment effects,among which 1 case abandoned treatment and 4 cases were adjusted for the second-line standard chemotherapy (Prednisone + Vincristine + Cytarabine + Cladribine).Finally,1 out of 4 case was assessed as non-active state after 3 months of medicine withdrawal,the other 3 cases were still in maintenance therapy.Conclusions LCH involving the thyroid gland is extremely rare,with significantly older onset age,and easily complicated by multiple organ involvement with vital organs included.Patients have no significant clinical symptoms of hypothyroidism,while some have subclinical hypothyroidism.The major imaging changes are goiters and low echoes with irregular shapes.Those LCH involving the thyroid gland complicated by subclinical hypothyroidism turn out to have poor prognosis,but Cladribine and Cytarabine are possible to improve the prognosis of such patients.
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Objective To explore the common genetic abnormalities in childhood acute lymphoblastic leukemia(ALL) and their responses to early treatment response.Methods From December of 2010 to December of 2011,169 newly diagnosed ALL patients at the Department of Hematology,Beijing Children's Hospital Capital Medical University,were detected by karyotype analysis,reverse transcription polymerase chain reaction (RT-PCR) and fluorescent in situ hybridization (FISH),and the relationship between early treatment responses and genetic abnormalities was observed.Results Of the 169 cases,bone marrow cell specimens from 162 cases were successfully cultured,with the success rate reached to 95.9％,and 88 cases (52.1％) had chromosomal abnormalities.Fifty-five cases carried 8 types of fusion genes among the 153 patients who received RT-PCR examination,and the abnormal rate was 35.9％.Forty cases applied for the detection of mixed lineage leukemia (MLL) gene rearrangement by FISH,and 6 cases of them were positive.One hundred and five cases had genetic abnormalities and the detection rate reached to 62.1％ by using three combined methods.The genetic abnormalities were classified into 6 groups,they were t(12;21),t(1;19),t(9;22),MLL rearrangement,hyperdiploid and-6/6q-,-7/7q-respectively,and early therapy response in each group was compared,and statistically significant differences were found among 6 groups (x2 =22.954,19.432,14.045,P =0.001,0.001,0.016).Conclusions Conventional cytogenetics combined with RT-PCR and FISH can enhance the detection rate of genetic abnormalities in childhood ALL.Genetic abnormalities combined with early treatment response in ALL can better guide the clinical treatment and prognosis assessment.