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Immunotherapy combined with targeted therapy can benefit the survival of patients with unresectable hepatocellular carcinoma. Atezolizumab combined with bevacizumab has achieved remarkable efficacy in patients with advanced hepatocellular carcinoma, but the efficacy of conversion therapy in patients with unresectable hepatocellular carcinoma still needs more evidences. The authors report the clinical efficacy of a case of unresectable hepatocellular carcinoma with hepatitis B virus related liver cirrhosis who was treated with immunotherapy plus targeted therapy combined with local treatment. Results show a good effect in patient without tumor recurrence after postoperative 9 months.
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Objective To explore the characterization of thyroid stimulating hormone receptor(TSHR) gene mutational spectrum in children with hyperthyroidism from Guangzhou. Methods Ninety children were diagnosed with hyperthyroidism from July 2009 to July 2014 in our institute. Their median age at diagnosis was(7.5± 3.4) years, and there were 28 males and 62 females. Mutational analysis were performed by performing polymerase chain reaction (PCR) and DNA direct sequencing of exon 10 of TSHR gene. TSHR gene mutations from 50 unrelated healthy children were served as controls. The correlation between TSHR gene and hyperthyroidism in children was explored. Results A total of 3 mutations were identified in ninety children who were diagnosed with hyperthyroidism, one synonymous mutations(p.V614V), and two missense mutations( p. R707W and p. D727E). Mutation of p. V614V do not change amino acid and do not influence the structure and function of TSHR, no pathogenicity. p.R707W is a SNP associated with human cancers. The frequency of C allele of the D727E in children with hyperthyroidism was 86.7%, while 55.0% in the controls, significant different between the children with hyperthyroidism and the controls( P＜0. 01). In this study, a very high association between the D727E SNP and hyperthyroidism ( OR=18. 86, P＜0. 01) was found. Conclusion Three different mutations of TSHR gene exon 10 were identified in 90 children with hyperthyroidism, (c.1842A＞G,p.V614V、c.2119C＞T,p.R707W、c.2181G＞C,p.D727E), there were association between p.D727E and hyperthyroidism, nor p. V614V and p. R707W. Finally, p. D727E may be correlated with hyperthyroidism in children.
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Objective To study the characteristic of ultra-rapid delayed rectifier-potassium channel K current (IKur) in rat isolated atrial myocytes using whole-cell patch clamp technique, and the effect of Ibulitide on rat isolated atrial myocytes. Methods Atrial myocytes of rats on an in vitro Langendorf perfusion system were obtained by double digestion (collagenase typeⅡ) method. Whole-cell patch-clamp recording technique was used to record IKur currents. The current-voltage (I-V) curves of K+currents of atrial myocytes were fitted. With filled Ibulitide (2μg/L, filled 3 minutes), IKur was recorded. Results The Results showed that IKur was activated rapidly, almost no lag,and deactivation slowly. This current was activated at about-20 mV, the peak current was present apparent voltage dependence. The peak current density was (2.01 ± 0.27) pA/pF. After filled with Ibulite, this current was no any changed activation. Ibulite decreased the peak current density from+30 mV to+50 mV,but no statistical significance. Conclusion The IKur of rats can activate rapidly with no delays and inactivate slowly. This current is ultra-rapid delayed rectify outside current,and obvious displays the characteristic of outward rectification of whole action potential duration. The 2 μg/L Ibulitide shows no statistical significance for this current.
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Objective To investigate the clinical efficacy and adverse events of methimazole ( MMI ) treatment for children with hyperthyroidism, and to identify the predictors of remission and relapse. Methods A total of379children(260girlsand119boys)diagnosedwithhyperthyroidismandtreatedbyMMIinGuangzhouWomenand Children's Medical Center from March, 2004 to July, 2014 were retrospectively analyzed. The average age at diagnosiswas(9.3±2.3)years(range2.0~15.9years). Results AftertreatmentwithMMIfor3and6months, the thyroid functions of 96. 3%(365/379) and 98. 9%(375/379) patients returned to normal, respectively. By the end of this study, 256(67. 5%) patients continued to use MMI treatment and 44 patients(11. 6%) dropped out. 79 patients(20. 8%) achieved remission, 35 patients (44. 3%) of whom experienced a later relapse. Children who achieved constant remission had significantly lower FT3 and FT4 levels at diagnosis compared with the relapsed children(P<0. 05 or P<0. 01). It was more likely to remain long-term remission for children turned to be euthyroid within 3 months after initiating MMI treatment(P<0. 05). The relieved patients with family history of thyroid diseases weremorelikelytoberelapsed(P<0.05). Therewerenosignificantdifferencesinage,gender,exophthalmos, initial goiter size, thyroid peroxidase autoantibody, and thyroglobulin antibody levels between the relieved and relapsed patients. The overall incidence of adverse events associated with MMI was 27. 7%, mainly elevated alanine aminotransferase, bilirubin, and neutropenia. Most(66. 7%) of adverse events occurred within the first three months of MMI treatment. Conclusion MMI has a good effect on pediatric hyperthyroidism, with low remission and high relapse rate. The low thyroid hormone concentrations at diagnosis and normalization of thyroid function within three months seem to be useful predictors of remission. Vigilance is needed concerning MMI-associated adverse events throughout the MMI treatment period, especially during the first trimester of MMI initiation.
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Objective To compare the efficacy of cardiac resynchronization therapy (CRT) on chronic heart failure (CHF) patients with different left bundle branch block (LBBB) morphologies. Methods Patients(n=45)who were treated with CRT were enrolled. According to the intrinsic ECG morphologies, patients were divided into 1)genuineLBBB group (n=32) who present negative dominant V1 and V2 lead wave (QS or rS);mid-QRS notching or slurring in at least 2 leads of Vl, V2, V5, V6, I and aVL as well as QRS duration≥140 ms in male or≥130 ms in female and 2)falseLBBB group (n=10) who meet traditional standards but fail to meet“genuine”LBBB diagnostic standard. The QRS duration, echocardiographic indi?ces and New York Heart Association (NYHA) Functional Classification were evaluated at the 12 months follow-up. CRT re?sponder was defined as patient with≥1 decrease in NYHA class and/or with≥15%reduction in left ventricular end-systolic volume (LVESV). CRT super-responder was defined as patient with≥30%reduction in LVESV. Results There was no dif?ference in basic characteristics of patients between groups. At the 12 months follow-up, 20 patients in genuine LBBB group and 6 patients infalseLBBB group were identified as responders (P>0.05). Compared with those infalseLBBB group, the responders ingenuineLBBB group showed better improvement in left ventricular ejection fraction and left ven?tricular end diastolic diameter (LVEDD) (both P<0.05). Conclusion Left bundle branch block morphology is less predic?tive for the efficacy of CRT. However, patients who show response to CRT withgenuineLBBB profile may get more bene?fits from CRT treatment than the patients withfalseLBBB profile.
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Objective To develop a radiology algorithm and test its the accuracy in distinguish pacing in the septum from the other parts. Methods One hundred patients were implanted with double-chamber pacemakers. Sites of the leads were verified by two-dimensional echocardiography, and the patients were divided into 4 groups according to the echocar?diography:septal right ventricular outflow tract group(RVOT), RVOT anterior free wall group, mid septum group, and anteri?or septum group (near to the anterior free wall ). An algorithm was developed according to radiological characteristics in the 45° left anterior oblique (LAO45° ) view and the 30° right anterior oblique (RAO30° ) view. Then its sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) were tested . Results The algorithm has high sensitivi?ty and specificity, which were 90%and 97%respectively. The positive predictive value and negative predictive value were 90% and 97% respectively. Conclusion The radiology algorithm we developed have a high sensitivity and specificity in identifying the site of the leads.
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Objective To investigate the cause and countermeasures of frequent shocks in patients with implantable cardioverter defibrillators (ICD). Methods Eighty ICD patients with heart failure and malignant ventricular arrhythmias were followed up, including sixty-two male and eighteen female patients. There were 35 patients with single-chamber ICD, 23 with dual-chamber ICD and 22 with three-chamber ICD. Patients in this study were followed up for 1-6 years to analyze the reasons for ICD discharge. According to the specific circumstances, patients were treated. Results Twenty-three pa-tients in 80 patients suffered from shock treatment. Ten patients (12.5%) experienced frequent shocks. The causes of fre-quent shock included repeated episodes of ventricular tachycardia, invalid shock due to increased defibrillation threshold (DF) and false identification of the frequent episodes of paroxysmal ventricular tachycardia or arrhythmias. The management included the identification process adjustment of ventricular tachycardia and supraventricular tachycardia, increased num-bers of beats of ventricular tachycardia judgment and increase the basic pacing rate. The anti-arrhythmic drugs should be combinedly used, especially metoprolol and amiodarone. The ICD shock was significantly reduced after parameter optimiza-tion and anti-arrhythmic therapy. Conclusion The ICD shocks were effectively reduced with rational use of anti-arrhyth-mic drugs and valid ICD programming.
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Objective In the context of worldwide shortage of amytal,explore the intracarotidpropofol test for lateralizing language area and assessing hemispheric memory function.Methods Fourteen patients with refractory partial epilepsy who were candidates for surgical intervention were included in the study.With guide under a digital subtraction angiography,propofol was injected in bilateral intracarotidsequentially.Muscle power deceasing to level 0 at the contralateral limb and eyes gazing to contralateral side were used as the mark of hemispheric anesthesia completely.The immediate language alterations were recorded.To evaluate the bilateral language and memory functions,the visual and auditory memory tasks were performed sequentially once patient could concentrate his attention ; and after limb muscle power recovering to normal level,patients were required to perform a free recall test.Any abnormal responses were recorded.Results Language dominant hemisphere was determined in 14 patients.Nine patients were confirmed as left language dominance,2 patients were right language dominance.The remained 3 patients were considered as bilateral language dominance.Meanwhile,the hemispheric memory function was able be evaluated in 13 patients.More than 67％ memory function was sustained in hemisphere contralateral to mesial temporal lesions.Transient responses including eye pain,facial muscle spasms,laughers and involuntary movements were observed.Conclusion Hemispheric language and memory functions can be assessed with direct intracarotidpropofol injection,and propofol could be an alternative drug to amobarbital used in the Wada test.
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Objective To evaluate the utility of short tau inversion recovery (STIR) sequence in the diagnosis of hippocampal sclerosis ( HS).Methods Twenty-one patients with medial temporal lobe epilepsy without neoplasm lesions or injuries by conventional MRI sequence including T1WI,T2WI and FLAIR were included in this study.STIR imaging in axial,coronal and sagittal sequences was performed on these patients. Diagnosis of HS was based on the findings of hippocampal atrophy,alteration signal,disturbed internal structure and enlargement of the inferior horn. The findings shown on conventional MRI were compared with those on STIR sequence. Furthermore,the correlation of radiologic and histological findings was investigated in 6 patients operated for refractory seizures. Results On conventional MRI sequence,14 patients (66.7％) were confirmed with unilateral HS and 4 patients were suspected with unilateral HS. In contrast,all these 18 patients (85.7％) were confirmed with unilateral HS by STIR.Particularly,STIR sequence delineated the internal structure of hippocampus more clearly than conventional MRI sequences did. C shaped contour in subiculum-CA1-CA2 was revealed in normal hippocampus on STIR sequence and disappeared in HS,correlated to the pathology finding of loss of neuron in CA1 in resected tissues in 6 operated patients.The patients with HS also showed areas of hypodensity in CA4 on STIR,in accordance with pathologic findings of gliosis in this area in the 6 operated patients.Conclusion STIR sequence could depict the internal anatomical structure of hippocampus with high resolution superior to conventional MRI sequences,and can be of great value in the diagnosis of HS.
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Objective To investigate the value of the immunohistochemistry and Western blot in the diagnosis of the benign muscular dystrophy with abnormal dystrophin expression.Methods The medical histories and clinical manifestations of 4 patients were collected.In addition to routine histological and histochemical studies,expression of dystrophin in muscle fibets was observed by immunohistochemical reaction(dys-N,dys-R and dys-C)and Western blot to anti-dystrophin antibody.Results Two patients had muscular weakness while another 2 patients had only muscular pain and elevated creatine kinase blood levels without muscular weakness.Histochemical stains showed atrophy,hypertrophy and fiber splitting in 2 patients,while only variation in fiber size was presented in anothor 2 patients.One patient had no reaction for dys-N,but had immunostains for dys-C and dys-R in the sarcolemma of muscle fibers.Western blot confirmed that the band of dys-C and dys-R was partly deficient,and the band of dys-N was absent compared with control.Three patients had no reaction for dys-R,but had immunostains for dys-C and dys-N.Compared with control,Western blot confirmed that the band of dys-R was absent,and the band of dys-C and dys-N were truncated.Conclusion The immunohistochemistry is stained with three anti-dystrophin antibodies to avoid diagnostic errors.Western blot is essential to further determine the type of dystrophin protein.
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Objective To evaluate the efficacy and tolerability of zonisamide (ZNS) as add-on therapy in patients with refractory partial seizures.Methods In this Chinese muiticenter, double-blind, randomized, placebo-contrclled trial, ZNS was compared with placebo add-on therapy in 217 patients (intent-to treat (ITT) population) with uncontrolled partial-onset seizures.All patients entered a 3-month baseline period followed by a 4-week titration interval and a 12-week maintenance period.The starting dose of ZNS group was 100 mg/d, increased by 100 mg/d every week and reached the goal of 400 mg/d.The main outcome was measured by the median of the percentage of decreased seizure frequency.The secondary ouwomes points included the percentage of patients who had seizure attacks decreased by more than 50%,and adverse events.Results The median of the percentage of decreased seizure frequency in ZNS group was 33.33%, and the placebo group was 0.Thirty-eight patients (34.23%) experienced more than 50% reduction in the seizure frequency in ZNS group, compared with 19.81% of patients (21 cases) in the placebo group (χ2 =5.7159,P =0.0168) ; Moreover, 13 (11.71%) patients in ZNS group and 5 patients in placebo group were seizure free, 25 patients in ZNS group and 16 patients in placebo group who had seizure attacks decreased by more than 50%.The availability rate in ZNS group was higher than placebo group (34.23% vs 19.81%, U=2.4701, P=0.0135).The most common adverse events in ZNS group were drowsiness, fatigue, decreased appetite, gastrointestinal complaints, insomnia and constipation.Conclusion Zonisamide treatment was generally well tolerated and was associated with significant reductions in seizure frequency as adjunctive treatment for partial-onset seizures.