Clinical and experimental study of Castleman disease in children.

BACKGROUND: Castleman disease (CD) is a rare lymphoproliferative disease that is often underdiagnosed or misdiagnosed, especially in children. For this reason, we describe the clinical manifestations, diagnosis and treatment of CD in 11 children. PROCEDURE: A retrospective study was performed to analyze the clinical features of 11 children with CD in a single institution from January 2001 to December 2012. All had computed tomography (CT) and lymph node resection for pathology diagnosis. RESULTS: The average age of patients was 9.67 ± 4.26 years (range 1.3-15.5 years) including eight males (72.73%) and three females (27.27%). All but two (18.18%) had multicentric Castleman disease (MCD). Human immunodeficiency virus (HIV) or human herpes virus 8 (HHV8) infected cells were not detected in all patients. All patients were misdiagnosed in outside hospitals without tissue examination. Only in one case, the preoperative CT scan suggested CD. After treatment, 10 out of 11 children with CD in our study were disease free in the follow-up period ranging from 12 to 136 months (average 65.1 ± 10.21 months). CONCLUSION: CD in children is rare, and is frequently misdiagnosed clinically. Our study shows that surgical resection is very effective in the treatment of unicentric Castleman disease (UCD). The rare UCD patient and all MCD patients treated with the modified NHLBFM-90 protocol had good prognosis.

Totally implantable venous access ports: A prospective randomized study comparing subclavian and internal jugular vein punctures in children.

BACKGROUND AND OBJECTIVES: Totally implantable venous access ports (TIVAPs) are essential in children who require long-term intermittent intravenous therapy. METHODS: Patients who needed to undergo TIVAP implantation were randomly assigned to the internal jugular vein group or the subclavian vein group. The medical histories, operative details and major complications from the time of port implantation to 48 h after port removal were collected. During the use of TIVAPs, satisfaction surveys were regularly conducted for the children and guardians and compared in the two groups. RESULTS: A total of 216 patients in the subclavian vein group and 199 patients in the internal jugular vein group were included. TIVAPs were successfully implanted in all children. The incidence of postoperative venous access occlusion in the subclavian vein group and internal jugular vein group was 1.5% and 5%, respectively, and the difference was statistically significant (P < 0.05). The average satisfaction score of the children and guardians in the subclavian vein group was 9.6 ±â€¯0.3, and that in the internal jugular vein group was 8.3 ±â€¯0.8. There was a significant difference between the 2 groups (P < 0.05). CONCLUSIONS: Subclavian vein should be the first choice for TIVAP implantation in children. THE LEVEL OF EVIDENCE RATING: Treatment study level I.

Afferent pathway dysfunction in children with primary nocturnal enuresis.

OBJECTIVES: To investigate afferent pathway dysfunction in children with primary nocturnal enuresis by measuring pudendal somatosensory evoked potential and tibial somatosensory evoked potential. METHODS: Subjects with primary nocturnal enuresis, 36 boys and 18 girls, aged from 5 to 16 years, were enrolled in this study: 24 subjects had complicated primary enuresis (CPE) and 30 subjects had monosymptomatic primary enuresis (MPE). There were no differences in bodyweight or gender between the MPE and CPE groups (P > 0.05). All of the children underwent physical examination, urine analysis, urinary ultrasound and spinal magnetic resonance imaging. Only subjects without urological and neurological abnormalities (with the exception of spina bifida occulta, which was found in some of the patients) were included in this neurophysiological study. RESULTS: There were 20 children who were positively recorded with pudendal somatosensory evoked potential in the CPE group, and all of the children in the MPE group were positively recorded (P < 0.05). Positive records of tibial somatosensory evoked potential were successfully achieved in both groups. Furthermore, the pudendal and tibial conductive velocity were slower as compared to the normal range, especially in children in the CPE group (P < 0.001). CONCLUSIONS: Afferent pathway function may be impaired by some factors, which should be considered by both clinicians and parents.

[Intervention with thumbtack needling on spinal low back pain in Air Force crew: a randomized controlled trial].

OBJECTIVE: To observe the clinical effect on spinal low back pain (SLBP) in Air Force crew treated with novel thumbtack needling therapy and to analyze the relevant factors of the therapeutic effect. METHODS: A total of 120 Air Force crew with SLBP were randomized into a thumbtack needling group (40 cases), an external treatment group (40 cases, 1 case dropped off ) and a combined treatment group (40 cases, 1 case dropped off ). In the thumbtack needling group, the thumbtack needling therapy was adopted. The novel thumbtack needles were inserted at the lower No.6 region of the wrist-ankle acupuncture, Yaotongdian (EX-UE 7), Yaoyangguan (GV 3), etc. Each point was pressed and kneaded for 1 min each time, 3 or 4 times a day. The treatment for 3 days was taken as one course. At the interval of 2 days, 3 courses were required totally. In the external treatment group, shangshi zhitong plaster was compressed on the center of the tender site in the lumbar region, once daily, consecutively for 6 days as one course. At an interval of 1 day, 2 courses were required totally. In the combined treatment group, the treatments in the thumbtack needling group and the external treatment group were used in combination. Before and after treatment, McGill score and the score of Oswestry dysfunction index (ODI) were compared in the patients among the three groups. The average EMG (AEMG) and the mean power frequency (MPF) were analyzed by using JE-TB0810 electromyography (EMG) acquisition system to evaluate the erector spinae tension in the patients before and after treatment. The clinical effect was observed in the patient of each group and the safety was evaluated. Logistic analysis was performed on the relevant factors of therapeutic effect in the patients. RESULTS: Compared with the values before treatment, McGill scores and ODI scores were reduced (P<0.05), and AEMG and MPF increased in the patients of each group after treatment (P<0.05). After treatment, McGill scores and ODI scores in the thumbtack needling group and the combined treatment group were lower than those in the external treatment group (P<0.05), and AEMG and MPF were higher than the external treatment group (P<0.05). The total effective rates were 87.5% (35/40) and 87.2% (34/39) in the thumbtack needling group and the combined treatment group respectively and were higher than 64.1% (25/39) in the external treatment group (P<0.05). The incidence of the adverse reaction in the combined treatment group was higher than the other two groups (P<0.05). The weekly exercise frequency was the independent factor of the therapeutic effect (OR =12.166, P<0.001). CONCLUSION: The thumbtack needling therapy is significantly effective on spinal low back pain in Air Force crew and is of the safety. Hence, this therapy is applicable to be promoted in the primary care army hospital.

[Effect of NaHS on ATP-induced P2X receptor expression in rat microglia].

OBJECTIVE: To observed the effect of sodium hydrosulphide (NaHS), a donor of H2S on the cell viability,the membrane permeability and the expression of P2X7 receptor induced by adenosine triphosphate(ATP) in rat microglia. METHODS: Rat microglia in logarithmic growth phase was randomly divided into 4 groups. In control group, the cells were cultured without ATP treatment. In ATP group, the cells were treatment with ATP after cultured for 24 hours. In NaHS+ATP group, the cells were incubated with NaHS for 30 min before ATP, and NaHS always existed in the reaction system. In KN-62+ATP group, the cells were pretreated with KN-62 for 30 min, the others were as the same as NaHS+ATP group. The cell viability was detected by MTT. Fluorescent dyes YO-PRO-1 was used to observe the membrane permeability. The expression of P2X7 receptor was examined by immunofluorescence staining. RESULTS: ① Compared with control group, the cell viability dropped after treatment with ATP (1、3、5、10 mmol/L) for 3 hours. When pre-incubation with NaHS(200 µmol/L), the cell viability was apparently higher than that of ATP alone group(P<0.01), while 400 µmol/L had no further beneficial.②The YO-PRO-1 fluorescence intensity was obviously elevated by ATP in rat microglia, but this effect was counteracted by NaHS pretreatment (P<0.01). ③ The expression of P2X7 receptor protein was significantly increased after ATP(3 mmol/L) for 3 h. While the expression upregulation of P2X7 receptor protein induced by ATP was significantly counteracted by pretreating with NaHS(200 µmol/L) (P<0.01). CONCLUSIONS: NaHS could reduce the expression of P2X7 receptor, decrease membrane permeability, and increase the cell viability in rat microglia injured by ATP. So the cytoprotection of hydrogen sulfide may be related to the expression and function of P2X7 receptor.

microRNA-221 Enhances MYCN via Targeting Nemo-like Kinase and Functions as an Oncogene Related to Poor Prognosis in Neuroblastoma.

Purpose:MYCN is one of the most well-characterized genetic markers of neuroblastoma. However, the mechanisms as to how MYCN mediate neuroblastoma tumorigenesis are not fully clear. Increasing evidence has confirmed that the dysregulation of miRNAs is involved in MYCN-mediated neuroblastoma tumorigenesis, supporting their potential as therapeutic targets for neuroblastoma. Although miR-221 has been reported as one of the upregulated miRNAs, the interplay between miR-221 and MYCN-mediated neuroblastoma progression remains largely elusive.Experimental Design: The expression of miR-221 in the formalin-fixed, paraffin-embedded tissues from 31 confirmed patients with neuroblastoma was detected by locked nucleic acid-in situ hybridization and qRT-PCR. The correlation between miR-221 expression and clinical features in patients with neuroblastoma was assessed. The mechanisms as to how miR-221 regulate MYCN in neuroblastoma were addressed. The effect of miR-221 on cellular proliferation in neuroblastoma was determined both in vitro and in vivoResults: miR-221 was significantly upregulated in neuroblastoma tumor cells and tissues that overexpress MYCN, and high expression of miR-221 was positively associated with poor survival in patients with neuroblastoma. Nemo-like kinase (NLK) as a direct target of miR-221 in neuroblastoma was verified. In addition, overexpression of miR-221 decreased LEF1 phosphorylation but increased the expression of MYCN via targeting of NLK and further regulated cell cycle, particularly in S-phase, promoting the growth of neuroblastoma cells.Conclusions: This study provides a novel insight for miR-221 in the control of neuroblastoma cell proliferation and tumorigenesis, suggesting potentials of miR-221 as a prognosis marker and therapeutic target for patients with MYCN overexpressing neuroblastoma. Clin Cancer Res; 23(11); 2905-18. ©2016 AACR.