OBJECTIVETo analyze clinical and imaging features and genetic characteristics of Leigh syndrome with emergent pulmonary edema.

METHODThe clinical features and imaging data of 2 cases (1 male, 1 female) seen in Anhui Provincial Children's Hospital from 2012 to 2014 were analyzed and summarized. Venous blood samples were sent to Guangzhou Jinyu Medical Examination Center for genetic analysis. Peripheral blood DNA was extracted and amplified, then sent to a sequencing facility for presence of genetic mutation by comparing with the reference sequence (NC_012920.1).

RESULT(1) The first patient was a 7 months old boy. The second patient was a 7 months and 21 days old girl. They were presented with abnormal respiration and pulmonary hemorrhage required mechanical ventilation. The first patient had a similar attack after 4 months of his birth, whose psychomotor development was normal, and no abnormal neurological findings. The value of blood lactate was 1.58 mmol/L. The value of pyruvic acid was 0.25 mmol/L. The value of cerebrospinal fluid lactate was 6. 4 mmol/L, which was an abnormal increase. The second patient had abnormal nervous system development, which included motor development retardation and hypotonia. The value of blood lactate was 6. 8 mmol/L, pyruvic acid was 0.31 mmol/L. Cerebrospinal fluid lactate was 8.2 mmol/L. (2) Imaging data: chest X-ray revealed double lung effusion. Bilateral caudate nucleus and lentiform nucleus had high signal, and bilateral internal capsule forelimbs were affected in DWI sequence of head MRI. Hemispheres, basal ganglia, cerebral peduncle, cerebellum, pons, and splenium of corpus callosum had multiple abnormal signals in head MRI of the second patient. NAA peak showed significantly reduced lesion area in magnetic resonance blood-flow scanning, and Cho peak increased significantly, which were double lactate-peak. (3) Genetic testing: ATPase6 m.9185 t > C mutation was found in case 1 that was consistent with Leigh syndrome pathogenesis. Hybrid mutations (m. 10191 t > C) in mitochondrial DNA was found in case 2. Two cases with the diagnosis of Leigh syndrome was clear. They were given combined therapy, such as mechanical ventilation, limited fluid to alleviate lung exudation, coenzyme Q10, and L-carnitine. The illness of case 1 relapsed after discharge. But in case 2, there was no improvement. They both died after treatment was given up.

CONCLUSIONNeurological symptoms were common in Leigh syndrome, in which acute lung hemorrhage was rarely reported. Timely ventilator support can temporarily save lives, but fatality rate is high and prognosis is poor.

Brain ; pathology ; Carnitine ; therapeutic use ; DNA, Mitochondrial ; Female ; Genetic Testing ; Hemorrhage ; etiology ; Humans ; Infant ; Lactic Acid ; Leigh Disease ; complications ; genetics ; Lung Diseases ; etiology ; Magnetic Resonance Imaging ; Male ; Mutation ; Pyruvic Acid

Objective To summarize the main points of nursing the patients during small incision minimally invasive cardiac surgery.Methods Two hundred and seventy-one patients underwent small incision minimally invasive cardiac surgery during April 2010 and December 2010.The cooperative nursing was performed.Results The patients went well with the operations,with the average extracorporeal circulation time of(117.3±47.1)min and aorta blocking time of(82.6±32.1) min.All of the patients were discharged,without postoperative complications.(6.8±2.6)d.Perioperative and after discharge with no deaths.Postoperative recovery and no postoperative complications,were recovered and discharged without any complications.The postoperative hospital length of stay was(6.8±2.6)d.Conclusions The minimally invasive cardiac surgery is effective and safe.The preoperative health education and intraoperative nursing cooperation are critical for the operative success.

Objective To evaluate the practical value of 99m Tc-MIBI scintimammography and breast X-ray in the detection of breast cancer. Methods 100 female patients with 106 suspected focuses of breast cancer on clinical examination underwent 99m Tc-MIBI scintimammography and breast molybdenum target X-ray, and the results were compared with the pathological diagnosis. Results Compared with the histopathologic diagnosis , the sensitivity of molybdenum target X-ray for detecting primary breast cancer was 44.6% and the positive predictability was 31.7%. The specificity of 99m Tc-MIBI was 87.8% and the positive predictability was 76.9%. Conclusions In comparison with breast X-ray, 99m Tc-MIBI scintimammography is of greater clinical value in the diagnosis of breast cancer.

Objective To observe the effect of ultrasound-guided iliopsoas plane block(IPB)on the quality of postoperative recovery in patients undergoing hip arthroplasty.Methods Sixty patients who underwent hip arthroplasty were selected,37 males and 23 females,aged 40-79 years,BMI 18-30 kg/m2,ASA physical status Ⅰ-Ⅲ.The patients were divided into two groups by random number table method:the iliopsoas plane block group(group IPB)and the femoral nerve block(FNB)group(group FNB),30 pa-tients in each group.Before anesthesia induction,IPB was performed with 0.5%ropivacaine 10 ml and lat-eral femoral cutaneous nerve block was performed with 0.5%ropivacaine 5 ml in group IPB.And FNB was performed with 0.5%ropivacaine 10 ml and lateral femoral cutaneous nerve block was performed with 0.5%ropivacaine 5 ml in group FNB.The dosages of propofol,remifentanil,and cis-atracurium during operation were recorded.The quality of recovery-15(QoR-15)scale was evaluated preoperatively and postoperatively 1 day,2 and 3 days.The max VAS(VASmax)pain score and manual muscle test(MMT)score of quadri-ceps muscle were recorded 12,24,and 48 hours after surgery.The time of getting out of bed for the first time,opioid dosage,and patient satisfaction were recorded.The incidence of nerve injury,vascular injury,puncture site infection,and local anesthetic poisoning were recorded.The postoperative complications of diz-ziness,nausea and vomiting,deep vein thromboses,and elirium were also recorded.Results There was no significant difference in the dosage of propofol,remifentanil,and cis-atracurium between the two groups.Compared with group FNB,the QoR-15 scale score in group IPB was significantly higher 1 day,2 and 3 days after operation(P＜0.05).Compared with group FNB,the MMT scores of quadriceps muscle was sig-nificantly higher in group IPB 12 and 24 hours after surgery(P＜0.05),and the first time of getting out of bed was shortened in group IPB(P＜0.05).However,there were no significant differences in the VASmax pain score,MMT score of quadriceps muscle 48 hours after surgery,opioid dosage,and patient satisfaction between the two groups.No nerve block related complications were found in both groups.There were no sig-nificant differences in postoperative complications between the two groups.Conclusion The iliopsoas plane block can improve the quality of postoperative recovery and accelerate the recovery of patients with hip re-placement,and the effect is better than that of femoral nerve block.

BACKGROUND AND OBJECTIVES: Over the past decades, transradial approach for percutaneous coronary intervention (PCI) has been increasingly adopted in clinical practice. Women represent a large population who will possibly benefit from PCI, but they are often under-represented in clinical studies. Therefore, the role of TRI in women remains to be further defined. This study sought to compare safety and efficacy for transradial intervention (TRI) and transfemoral intervention (TFI) in women undergoing PCI in China. METHODS: The study population consisted of 5,067 women undergoing PCI at Fuwai Hospital, Beijing, China between 2006 and 2011 (TRI: n=4,105, TFI: n=962). Incidence rates of clinical outcomes during hospitalization and at 1-year follow-up were compared between TRI and TFI. In order to minimize potential biases, a 1:1 propensity score matching (PSM) was performed. A total of 899 pairs were matched. RESULTS: Baseline and procedural characteristics were well-balanced between TRI and TFI groups after controlling for confounders using PSM. TRI was associated with reduced major post-PCI bleeding (odds ratio [OR], 0.64; 95% confidence interval [CI], 0.54–0.76; p < 0.001) and access site complications (OR, 0.67; 95% CI, 0.61–0.74; p < 0.001) after PSM. There was no statistical differences in the incidence rates of major adverse cardiac events (a composite of cardiac death, myocardial infarction, and target vessel revascularization) both during hospitalization and at 1-year follow-up (p > 0.05). CONCLUSIONS: In this propensity score-based analysis of TRI versus TFI in Chinese women, TRI showed advantages of safety and feasibility over TFI. A wider adoption of TRI in women has the potential to improve outcomes in treatment of coronary artery diseases.
Asian Continental Ancestry Group ; Beijing ; Bias (Epidemiology) ; China ; Coronary Artery Disease ; Death ; Female ; Femoral Artery ; Follow-Up Studies ; Hemorrhage ; Hospitalization ; Humans ; Incidence ; Myocardial Infarction ; Percutaneous Coronary Intervention ; Propensity Score ; Radial Artery

BACKGROUND AND OBJECTIVES: Over the past decades, transradial approach for percutaneous coronary intervention (PCI) has been increasingly adopted in clinical practice. Women represent a large population who will possibly benefit from PCI, but they are often under-represented in clinical studies. Therefore, the role of TRI in women remains to be further defined. This study sought to compare safety and efficacy for transradial intervention (TRI) and transfemoral intervention (TFI) in women undergoing PCI in China. METHODS: The study population consisted of 5,067 women undergoing PCI at Fuwai Hospital, Beijing, China between 2006 and 2011 (TRI: n=4,105, TFI: n=962). Incidence rates of clinical outcomes during hospitalization and at 1-year follow-up were compared between TRI and TFI. In order to minimize potential biases, a 1:1 propensity score matching (PSM) was performed. A total of 899 pairs were matched. RESULTS: Baseline and procedural characteristics were well-balanced between TRI and TFI groups after controlling for confounders using PSM. TRI was associated with reduced major post-PCI bleeding (odds ratio [OR], 0.64; 95% confidence interval [CI], 0.54–0.76; p < 0.001) and access site complications (OR, 0.67; 95% CI, 0.61–0.74; p < 0.001) after PSM. There was no statistical differences in the incidence rates of major adverse cardiac events (a composite of cardiac death, myocardial infarction, and target vessel revascularization) both during hospitalization and at 1-year follow-up (p > 0.05). CONCLUSIONS In this propensity score-based analysis of TRI versus TFI in Chinese women, TRI showed advantages of safety and feasibility over TFI. A wider adoption of TRI in women has the potential to improve outcomes in treatment of coronary artery diseases.