The paper reports a case of coenzyme Q10 deficiency nephrotic syndrome associated with coenzyme Q2 gene mutation and reviews the literature on this topic. The patient presented with hematuria, proteinuria, and a diminution of vision as clinical manifestations. But the proteinuria was not relieved after sufficient doses of glucocorticoids for over 2 months. The patient′s birth history was unremarkable, and his parents were both healthy and not consanguineous. Whole exome sequencing revealed that the patient had a mutation of coenzyme Q2 gene at c.973A>G(p.T325A) and c.517C>T(p.R173C). Combined with renal biopsy pathology, the patient was diagnosed with hereditary nephropathy and started the supplements of coenzyme Q10 after stopping glucocorticoid treatments immediately. After 5 weeks of therapy, the patient′s 24-h urine protein quantification decreased from 6.01 g to 1.53 g.

Objective:To improve the understanding of indolent mantle cell lymphoma (MCL).Methods:The data of a patient with indolent leukemic MCL in the Second Affiliated Hospital of Nanjing Medical University in May 2013 were collected. The cell morphology was analyzed by using cell smear, the flow cytometry was used to make immunophenotype analysis, the karyotype analysis was performed by usig cytogenetic technique, and polymerase chain reaction (PCR) was used to make the immunoglobulin gene analysis. At the same time, lymph node pathology and immunohistochemistry were also analyzed. The related articles published were reviewed to sum up the characteristics and the treatment of indolent MCL.Results:The male patient aged 60 years was obviously asymptomatic accompanied with slow disease progression, leukemic manifestation and without lymphadenopathy. He received pathological biopsy because of located lymphadenopathy in 2008. Small cell morphology, Kappa light chain immunophenotype, t(11;14) translocation showed after the cytogenetic examination, clonal immune globulin gene rearrangement and low Ki-67 positive index were identified. In situ MCL was diagnosed by retrospective pathology.Conclusions:Indolent MCL is extremely rare. It is typically asymptomatic with none or minimal nodal involvement, indolent disease course, leukemic phase with mild lymphocytosis, Kappa light chain expression, simple karyotype, classical or small cell morphology of tumor cells and the positive index of Ki-67 <10%. In situ MCL can be seen in pathology examination. IgVH gene mutation positive and SOX11 negative expression are notable in indolent MCL. International prognostic index of MCL is probably not appropriate in the prognostic analysis of leukemic indolent MCL. It is emphasized that initial observation and having therapies only after the disease progression can be suited for indolent MCL.

Objective:To compare the efficacy of different volume of ropivacaine for subomohyiod anterior suprascapular nerve block (aSSNB) in the patients undergoing arthroscopic shoulder surgery with general anesthesia.Methods:One hundred and thirty-five patients of either sex, aged 18-64 yr, with body mass index of 18-30 kg/m 2, of American Society of Anesthesiologists Physical Status classification Ⅰ or Ⅱ, scheduled for elective arthroscopic shoulder surgery, were divided into 3 groups ( n=45 each) using a random number table method: 0.5% ropivacaine 5 ml group (L group), 10 ml group (M group), and 15 ml group (H group). Before induction, aSSNB was performed with 0.5% ropivacaine 5, 10 and 15 ml in L, M and H groups, respectively.Diaphragmatic excursion, occurrence and degree of diaphragmatic paralysis, decrease in SpO 2, dyspnea and Horner syndrome were recorded at 30 min after injection.The intraoperative consumption of remifentanil and cardiovascular events were recorded.The extubation time, length of post-anesthesia care unit stay, and duration of sensory block were recorded.Quality of Recovery-15 scale score and score for patient′s satisfaction with analgesia were recorded.The first pressing time of analgesic pump, effective pressing frequency of analgesic pump, requirement for rescue analgesia, nausea, vomiting and nerve block-related complications within 24 h after surgery were recorded. Results:Compared with group L, the incidence of diaphragmatic paralysis was significantly increased, the degree of diaphragmatic paralysis was aggravated, the first pressing time of analgesic pump and duration of sensory block were prolonged, the effective pressing times of analgesic pump was reduced, and the requirement for rescue analgesia was decreased in M and H groups, and the decrease in SpO 2 was significantly increased, and the introperative consumption of remifentanil was decreased in group H ( P<0.05). Compared with group M, the decrease in SpO 2 and incidence of diaphragmatic paralysis were significantly increased, the degree of diaphragmatic paralysis was aggravated, the first pressing time of analgesic pump and duration of sensory block were prolonged ( P<0.05), and no significant change was found in the introperative consumption of remifentanil, the effective pressing times of analgesic pump or requirement for rescue analgesia in group H ( P>0.05). There was no significant difference in the incidence of cardiovascular events, score for patient′s satisfaction with analgesia, incidence of dyspnea and extubation time, length of post-anesthesia care unit stay, Quality of Recovery-15 sacle score, and the incidence of nausea and vomiting among three groups ( P>0.05). There were no Horner syndrome and nerve block-related complications in the three groups. Conclusions:Subomohyoid aSSNB with 0.5% ropivacaine hydrochloride 10 ml provides optimal efficacy when used for subomohyiod anterior suprascapular nerve block in patients undergoing arthroscopic shoulder surgery with general anesthesia.

Objective:To explore the clinical characteristics and appropriate treatment of occult pneumococcal bacteremia (OPB) in children.Methods:The clinical characteristics, drug sensitivity and antibiotic use of 39 children with OPB who met the inclusion criteria in the Pediatric Internal Medicine Ward of Shenzhen Children′s Hospital from January 2013 to December 2018 were retrospectively analyzed.Results:The median age of onset in OPB in children was 2 years and 4 months, and 74.4% of children(29/39 cases) were between 6 months and 3 years.The average total fever duration was 3.69 days (1-14 days), and the average hospital stay was 6.74 days.Peripheral blood white blood cell count was >15×10 9/L in 89.7% (35/39 cases) of the children, C-reactive protein was increased in 76.9% (30/39 cases) of the children, and procalcitonin > 2 mg/L in 38.9% (14/36 cases) of the children.During hospita-lization, all the children received the treatment of intravenous antibiotics.The antibiotics used initially included Cefuroxime in 11 cases (28.2%), Amoxicillin sulbactam sodium in 10 cases (25.6%), Ceftriaxone sodium in 7 cases (17.9%) and Meloxicillin sulbactam in 6 cases (15.4%). The average fever clearance time after the antibiotic therapy was 1.4 days, and the average intravenous antibiotics treatment time was 6.2 days.The results indicated that the insensitivity rate of Penicillin was 46.2%, the insensitivity rate of Amoxicillin, Ceftriaxone and Cefotaxime were 22.2%, 10.3% and 17.9%, respectively, Erythromycin resistant rate was 100.0%, and no patient was resistant to Vancomycin and Linezolid. Conclusions:OPB occurs most frequently in infants aged from 6 months to 3 years old, with low drug resistance to Amoxicillin, Ceftriaxone and Cefotaxime.Children with complete immunity have shorter fever duration and good prognosis after antibiotic treatment.

Objective To investigate the effect of ultrasound-guided quadratus lumborum block on cognitive function after laparoscopic colorectal surgery.Methods Seventy-six patients, there is no restriction on gender, aged 50-75 years, falling into ASA physical statusⅡorⅢ, who provided informed consent for their participation in this study and underwent colorectal surgery under general anesthesia were divided into two groups (groups Q and C).Induction of anesthesia was induced by intravenous midazolam 0.03 mg/kg, sufentanil 0.5μg/kg, etomidate 0.3 mg/kg and rocuronium 1 mg/kg in the two groups.Remifentanil 0.2-0.3μg·kg-1·min-1 and propofol 0.10-0.15 mg·kg-1·min-1 were maintained intravenously during anesthesia.0.375％ropivacaine 20 ml was injected between the psoas quadratus muscle and psoas major muscle under ultrasound guidance before extubation in group Q, only equal volume saline was given in group C.Neuropsychological tests were performed preoperatively as well as postoperative day 7.The postoperative pain was evaluated by VAS after surgery.The postoperative sleep quality was measured using a BIS-vista monitor.The changes of serum IL-6, IL-1β, TNF-αand CGRP levels were detected by ELISA at immediately after operation (T0), 1 hour (T1), 2 days (T2), 4 days (T3) and 7 days (T4) after operation.Results Seventy-six patients finished cognitive function tests (38 cases in group Q and 38 cases in group C), 12 cases (31.6％) were diagnosed as POCD in group C, 4 cases (10.5％) in group Q.Compared with group C, the incidence of POCD, VAS scores at 24 and 48 hours after operation in group Q were significantly lower (P<0.05), the sleeping time at night was longer, the serum levels of IL-6, IL-1βand TNF-αwere significantly lower at T2 and T3, and the serum levels of CGRP were significantly higher at T2-T4 (P<0.05 or P<0.01).Conclusion QLB treatment repaired the surgery induced early cognitive dysfunction by inhibiting the postoperative pain, excessive inflammatory response and improving the quality of sleep.

Objective: To investigate the levels of lanthanum, cerium, praseodymium, and neodymium in the blood, urine, and hair samples from residents in the rare earth mining area of a city in China, and to provide a scientific basis for the control of rare earth pollution and the protection of population health. Methods: A total of 147 residents who had lived in the rare earth mining area of a city for a long time were selected as the exposure group, and 108 residents in Guyang County of this city who lived 91 km away from the rare earth mining area were selected as the control group. Blood, urine, and hair samples were collected from the residents in both groups. Inductively coupled plasma mass spectrometry was used to determine the content of lanthanum, cerium, praseodymium, and neodymium in blood, urine, and hair samples. Results: In the exposure group, the median levels of lanthanum, cerium, praseodymium, and neodymium were 0.854, 1.724, 0.132, and 0.839 μg/L, respectively, in blood samples, 0.420, 0.920, 0.055, and 0.337 μg/L, respectively, in urine samples, and 0.052, 0.106, 0.012, and 0.045 μg/g, respectively, in hair samples. The exposure group had significantly higher levels of the four rare earth elements in blood, urine, and hair samples than the control group (P<0.01) . Conclusion The residents in the rare earth mining area of this city have higher content of lanthanum, cerium, praseodymium, and neodymium in blood, urine, and hair than those in the non-mining area; the content of cerium is highest, followed by lanthanum, neodymium, and praseodymium.

Objective: To investigate the expression levels of S100A6, Notch1 in multiple myeloma (MM) patients and its clinical significance. Mathods: The expression levels of S100A6, Notch1 in 28 MM cases and 20 healthy controls were determined by real time quantitative PCR (RQ-PCR) , and their relationships with clinical features and outcomes were analyzed. Immunohistochemical was used to analysis the levels of S100A6 and Notch1 in bone marrow biopsy samples and intramedullary metastases soft tissues. RQ-PCR and Western blot were used to test the changes of Notch1 mRNA and Notch1 protein in U266 MM cells after S100A6 silenced by siRNA. Results: ①The expression levels of S100A6, Notch1 in primary MM patients was 2.19±1.25, 2.98±0.64, significantly higher than those in controls (0.71±0.20, 0.58±0.39, P<0.05) and patients in platform status (0.85±0.26, 0.72±0.40, P<0.05) . 8 cases with intramedullary metastasis had significantly higher levels of S100A6 (3.36±1.23) and Notch1 (5.71±3.96) , as compared to those without extra medullary metastases. ②S100A6 expression was positive correlation with Notch1 (r=0.505, P=0.007) . ③S100A6 and Notch1 proteins were positive in plasma cells of bone marrow biopsy samples and intramedullary metastases soft tissues. ④The Notch1 mRNA and Notch1 expression decreased significantly after 48 hours treatment by S100A6 siRNA in U266 cells. Conclusion S100A6 and Notch1 were closely associated with MM progress and intramedullary metastasis. They have significant correlation and might be as two prognostic molecular markers in MM.

Objective: To investigate the expressions of S100A6, annexin A2 (AnxA2) and c-myc in patients with multiple myeloma (MM) and their clinical significance. Methods: The expressions of S100A6, AnxA2 and c-myc mRNAs in bone marrow mononuclear cells from 28 cases of MM before and after chemotherapy and 20 controls (whose peripheral blood white cell count and the platelet count were a little lower than the normal values, but the result of bone marrow aspiration was normal) were detected by real-time fluorescent quantitative PCR. The relationships among the expressions of S100A6, AnxA2 and c-myc mRNAs were analyzed. The expressions of S100A6, AnxA2 and c-myc mRNAs and proteins in MM U266 cells after transfection with S100A6 siRNA were detected by real-time fluorescent quantitative PCR and Western blotting, respectively. Results: The expression levels of S100A6, AnxA2 and c-myc mRNAs in bone marrow mononuclear cells from patients with MM were higher than those from the controls (all P < 0.05). The expression levels of S100A6, AnxA2 and c-myc mRNAs in bone marrow mononuclear cells from patients with MM before chemotherapy were higher than those after chemotherapy (all P < 0.05). The expression levels of S100A6, AnxA2 and c-myc mRNAs in bone marrow mononuclear cells from MM patients with extramedullary metastasis were higher than those from MM patients not having extramedullary metastasis (all P < 0.05). The expression of S100A6 mRNA was positively correlated with the expressions of AnxA2 and c-myc mRNAs (r = 0.585, P = 0.001; r = 0.540, P =0.004). The expression levels of S100A6, AnxA2 and c-myc mRNAs and proteins in MM U266 cells after transfection with S100A6 siRNA were down-regulated (all P < 0.05). Conclusion: The expression level of S100A6 in patients with MM is higher, and it is positively associated with AnxA2 and c-myc. S100A6 may be involved in the development, progression and extramedullary metastasis of MM.

Objective To compare effectiveness,performance,onset time and complications between ultrasound-guided cervical transverse process block and interscalene brachial plexus block in patients undergoing proximal humeral surgeries.Methods Sixty patients,27 males and 33 females, aged 18-70 years,scheduled for proximal humeral surgeries were randomly divided into two groups. They were given either cervical transverse process block (group T,n =30)or ultrasound-guided in-terscalene brachial plexus block (group I,n =30).All patients received a total of 8 ml of 0.5% ropiv-acaine.The performance, anesthetic onset time, the side effects and block success rate were evaluated.Results Block procedure was quicker in group T than in group I [(8.73 ±3.1 7)min vs. (14.40±8.21)min,P <0.01].The severity of diaphragmatic paralysis in group T was significantly lower than in group I (P <0.01).The ultrasound-guided cervical transverse process block was more effective than the interscalene brachial plexus block in patients undergoing proximal humeral surgeries (100% vs.80%,P <0.05).Conclusion The ultrasound-guided cervical transverse process block has a higher success rate and fewer incidence of diaphragmatic paralysis than the interscalene brachial plexus block in patients undergoing proximal humeral surgeries.

Objective: To retrospectively analyze the clinical efficacy and safety of rivaroxaban in the prevention of deep vein thrombosis ( DVT) after bones of lower extremity surgery. Methods:Totally 94 patients with bones of lower extremity surgery were col-lected in our hospital during June 2010 to June 2013. According to the taken medicine, the patients were divided into groupⅠ(rivarox-aban 10 mg,po,qd) and groupⅡ( low molecular weight heparin injection 4 000 IU, ih,qd) . The bleeding after the surgery, the data of blood routine and coagulation, the occurrence of DVT and the adverse reactions in the two groups were compared. Results:After the medicine treatment, the number of hemoglobin and platelet in the two groups was declined significantly (P<0. 05), while reached the normal value after the medicine withdrawal. No significant difference was found in the blood routine and coagulation routine in the two groups. One case of hemorrhea showed in group I, while three cases showed in groupⅡ. GroupⅠhad no DVT, while groupⅡ had 3 cases of DVT, and the difference was significant (P<0. 05). No severe adverse reactions appeared in the two groups. Conclusion:Rivaroxaban has good effect in the prevention of DVT with better efficacy and safety when compared with low molecular weight heparin.