bluuebunny/crossref_metadata_2025 huggingface dataset:
Efficacy and safety of compound Kushen injection combined with chemotherapy on postoperative Patients with breast cancer
Authors: Ao Man , Xiao Xu , Li Qingshan
Published: January 2019

Background:

This meta-analysis aimed to assess efficacy and safety of combination of Kushen and chemotherapy or chemotherapy alone among postoperative patients with breast cancer receiving.

Methods:

A systematic literature search was conducted for relevant randomized controlled trials from 2000 to July 2017. Primary outcomes were clinical response rate (CRR) and performance status improvement by Karnofsky performance scale score (KPSS); secondary outcomes were adverse drug reactions (ADRs) rate and tumor marker decrease rate. Quality assessment and data analysis were performed with Review Manager 5.3.

Results:

A total of 16 studies with 1315 participants were included in the analysis. Compared with chemotherapy alone, compound Kushen injection (CKI or KI) combined with chemotherapy did not significant increase CRR. However, performance status improvement rate was significantly higher among patients given Kushen injection combined with chemotherapy (relative risk 1.25, 95% confidence interval 1.09–1.42, P = .001). In the analysis of ADRs, combination of Kushen and chemotherapy was indicated to significantly reduce the rate liver dysfunction, kidney dysfunction, nausea and vomiting, diarrhea, hair loss, platelet decrease, and oral mucositis.

Conclusion:

Using CKI on the basis of chemotherapy might improve performance status and reduce ADRs among postoperative patients with breast cancer.
Asymmetric limb lengthening in the treatment of tibial hemimelia caused by osteomyelitis
Authors: Yu Li , Yu Guorong , Deng Kai , Wang Guorong
Published: January 2019

Rationale:

Tibial hemimelia is known as a rare congenital lower limb deficiency. It has been classified into different types based on Jones classification, and the traditional treatment of tibial hemimelia is amputation. Here we present a variant and unclassified case of tibial hemimelia, which was caused by osteomyelitis. And the lower limb with tibial hemimelia was salvaged by asymmetric limb lengthening.

Patient concerns:

19-year-old girl had the shortened and curved left lower extremity with walking abnormalities.

Diagnosis:

The patient's deformity was caused by osteomyelitis of tibia occurred when she was 18 month old. The tibial shaft was absent, while the proximal and distal tibia was present but was hypoplastic with radiographic analysis. The fibula was hypertrophied and curved like the capital letter C. The leg length discrepancy (LLD), mostly coming from the left lower leg, was 22 cm.

Interventions:

We were able to salvage the limb successfully by 5 operations, including releasing soft tissue, fusing the proximal tibiofibular joint, fibular osteotomy, femur lengthening, and fibular lengthening.

Outcomes:

The whole treatment time for the patient was 3 years and 2 months, and she was followed up for 5 years afterward. The length of femur lengthening and fibula lengthening during the reconstruction were 7.8 cm and 11 cm, respectively. Most of the deformities were corrected, except that the left lower limb was still 2 cm shorter than the contralateral limb, and the 34 mm of mechanical axis deviation (MAD) of left lower limb remained. The reason why the patient's lower limbs were asymmetric was that the femur and fibular lengthening were performed within the affected limb only. Overall, the patient was very satisfied with her asymmetric limbs and its function after surgeries.

Lessons:

The LLD in this case mainly came from tibial hemimelia. However, the fibula was unable to be lengthened to 22 cm during the lower leg distraction process because of blood flow disturbance. We could only lengthen the femur to salvage the limb in this situation. Even though the patient still had a few residual deformities and a pair of asymmetric lower limbs, she was satisfied with the function and appearance of the reconstructed limb. Therefore, the lower limb with tibial hemimelia can be salvaged by asymmetric limb lengthening in special cases.
Serum levels of total antioxidant status, nitric oxide and nitric oxide synthase in minor recurrent aphthous stomatitis patients
Authors: Zhang Zichuan , Zhang Qian , Xue Yi , Chen Guang , Wu Zhongyin , Fang Huiqing
Published: January 2019

Background:

Recurrent aphthous stomatitis (RAS) is one of the most common inflammatory ulcerative conditions of oral cavity with uncertain etiology. Several studies have reported that oxidative stress may be associated with RAS. The aim of this study was to compare the serum levels of total antioxidant status (TAS), nitric oxide (NO) and nitric oxide synthase (NOS) in minor RAS (MiRAS) patients with healthy individuals and determine the possible association of MiRAS with the 3 physiological parameters mentioned above.

Methods:

Ninety patients with idiopathic MiRAS and 90 race-, age- and sex-matched healthy individuals were included in this study. All these subjects were allocated to 3 groups: MiRAS patients in the active stage (Group A); the same MiRAS patients in Group A in the inactive stage (Group B); healthy individuals without MiRAS (Group C). Serum levels of TAS, NO and NOS were determined by the spectrophotometric method. Independent sample t test and paired t test were performed for statistical evaluation.

Results:

Serum TAS level of Group A was significantly decreased than that of Group C, whereas the serum level of NO was significantly higher in Group A as compared to Group C (P < .05). The serum levels of TAS and NO in Group B were no significant differences when compared with those in Group A or Group C. No significant differences in NOS activities were also found between the 3 groups (P > .05).

Conclusions:

MiRAS is associated with decreased TAS and increased NO levels, but NOS may not play an important role in the aetiopathogenesis.
Complete response to sunitinib for more than three years in a patient with a jejunum gastrointestinal stromal tumor
Authors: Nie Yanli , Sun Wenjia , Xiao Zhihua , Ye Shengwei
Published: January 2019

Rationale:

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract and is characterized by KIT mutations. Patientsresistant to 1st-line imatinib therapy are usually given sunitinib assecond-line treatment, which provides a median progression-free survival of 8 to 12 months. We report the 1st case of metastatic jejunum GIST with a KIT exon 11 deletion that showed complete response (CR) to sunitinib for more than 3 years.

Patient concerns:

A 34-year-old man with advanced jejunum GIST was surgically treated upon initial diagnosis, and was histologically found to carry a high recurrence risk. Genetic testing revealed a KIT exon 11 deletion, and adjuvant therapy with imatinib was administered. The imatinib dose was escalated following recurrence in the abdomen, but the mass continued to grow.

Diagnosis:

He was diagnosed with abdominal recurrence of GIST based on his medical history and histopathological results.

Intervention:

Second-line sunitinib therapy was given.

Outcomes:

The mass disappeared, and CR was seen following 7 months of sunitinib therapy; this CR was sustained for more than 45 months.

Lessons:

In cases of metastatic jejunum GIST with a KIT exon 11 deletion, sunitinib as second-line therapy can be used to achieve CR for more than 3 years.
Ovarian mucinous cystic tumor associated with sarcomatous mural nodule and benign Brenner tumor
Authors: Yang Shaolong , Wang Li , Sun Kai
Published: January 2019

Rationale:

Ovarian mucinous tumor with malignant mural nodule is exceedingly rare. We report a case of ovarian mucinous cystic tumor associated with sarcomatous mural nodule and benign Brenner tumor and accompanied by nodular histiocytic aggregates in the greater omentum.

Patient concerns:

A 60-year-old postmenopausal woman was presented with a history of one month of lower abdominal discomfort, abdominal distension, nausea, and vomiting. A physical examination revealed a hard, palpable mass with mild tenderness in her right lower abdomen.

Diagnoses:

The mucinous tumor was solid and cystic and contained benign, borderline, and malignant elements. Within the solid areas, two nodules representing pleomorphic undifferentiated sarcoma and benign Brenner tumor were identified. The diagnosis of malignant mural nodule was based on vascular invasion and marked nuclear atypia, including atypical mitoses and mitotic activity.

Interventions:

Bilateral salpingo-oophorectomy and partial omentectomy were performed. Malignant cells were not found on cytologic examination of the peritoneal washing fluid. The patient underwent three cycles of chemotherapy with 210 mg paclitaxel liposome via an intravenous drip, 20 mg nedaplatin via an intravenous drip, and 80 mg nedaplatin via intraperitoneal perfusion.

Outcomes:

The patient has been followed up for 3 years without evidence of tumor recurrence and metastasis.

Lessons:

Careful classification of a mural nodule is important to triage patients in need of aggressive adjuvant treatment.
The efficacy of local liposomal bupivacaine infiltration on pain and recovery after Total Joint Arthroplasty
Authors: Zhao Baocheng , Ma Xinlong , Zhang Jinli , Ma Jianxiong , Cao Qing
Published: January 2019

Background:

Total Joint Arthroplasty (TJA) is gradually emerging as the treatment of choice for end-stage osteoarthritis. In the past, Perioperative liposomal bupivacaine treatment is still a controversial subject in TJA. Therefore, we write this systematic review and meta-analysis to evaluate the efficacy of liposomal bupivacaine on pain and recovery after TJA.

Materials and methods:

Embase, Pubmed, and Cochrane Library were comprehensively searched. Randomized controlled trials (RCTs), cohort studies were included in our meta-analysis. Twelve studies that compared liposomal bupivacaine groups with placebo groups were included in our meta-analysis. The research was reported according to the preferred reporting items for systematic reviews and meta-analysis (PRISMA) guidelines. RCTs were included in our meta-analysis.

Results:

Our study demonstrated that liposomal bupivacaine group was as effective as the placebo group in term of VAS score at 24 h (P = .09), 48 h (P = .97); Postoperative nausea (P = .72); and LOS (0.27). There was significant difference in terms of total morphine consumption at 24 h (P < .0001), 48 h (P = .0008).

Conclusion:

Our meta-analysis demonstrated that liposomal bupivacaine has similar pain control and functional recovery after TJA which compared with the control group. However, we still need large sample size, high-quality studies to explore the relationship between complications and dose response to give the final conclusion.
Rivaroxaban for the treatment of venous thromboembolism in real life
Authors: Demelo-Rodríguez Pablo , Galeano-Valle Francisco , García-Fernández-Bravo Irene , Piqueras-Ruiz Sandra , Álvarez-Sala-Walther Luis , del Toro-Cervera Jorge
Published: January 2019

The clinical profile, evolution and complications of treatment with rivaroxaban in a cohort of patients presenting with venous thromboembolism (VTE) were analyzed in an observational, non-interventional and prospective study.

A total of 111 patients were included in the study. Clinical data were collected from the medical history of the patients and recorded in a specific database.

Mean age was 63.8 ± 17.4 years, 53.2% of patients were men, 55.9% had at least another concomitant condition, and 40.9% at least 1 VTE risk factor. 54.1% of patients presented with deep venous thrombosis, 32.4% with pulmonary embolism and 13.5% with both conditions simultaneously. The 61% of patients were admitted to hospital and mean hospital length-of-stay was 8.8 ± 9.9 days. After a mean follow-up 530 ± 464 days (median follow-up of 405 days), 3.9% of patients died and VTE recurrence occurred in 2.9% of patients. While receiving rivaroxaban, a first bleeding complication occurred in 8.1%; all events were minor bleeding.

Our study supports the current literature data and confirms the similar results of real-life VTE patients with those enrolled in the rivaroxaban pivotal clinical trials. Rivaroxaban may facilitate outpatient treatment and might be considered as a first-line therapy for the management of VTE patients.
Therapeutic role of melatonin in migraine prophylaxis
Authors: Long Rujin , Zhu Yousheng , Zhou Shusheng
Published: January 2019

Background

Melatonin is the “clock factor” generated from pineal gland dominating regular circadian rhythm in humans. Migraine is one of the most severe and debilitating primary headache disorders. Thus far, many diseases have been found to associate with melatonin, including the migraine. Therefore, melatonin's therapeutic potential for migraine is drawing attention.

Objectives

The aim of this study is to offer a systematic review of extant data of melatonin in migraine prophylaxis and to provide clinical implications and specific recommendations for future studies.

Data sources and study methods

A systematic research was conducted in September 2018 by using PubMed and Google Scholar databases to search for science literature published after 1988.

Results

In all, 7 eligible articles were identified, including 4 randomized controlled studies and 3 observational studies. Due to high heterogeneities and limited number of studies, meta-analysis was not feasible, and only systematic review was performed. The results show that present evidence cannot claim melatonin's effectiveness according to the conflicting outcomes; however, the two negative outcomes of melatonin not different from placebo and melatonin inferior to amitriptyline are possible under-powering because of methodological, pharmacological, and therapeutic shortcomings. Observational studies also support melatonin's efficacy in migraine. As a result, melatonin is very likely to benefit migraine in prophylaxis and may have a similar effectiveness to other main preventive medications. Immediate-release melatonin 3 mg was established as effective, melatonin receptor agonist (Agomelatine) 25 mg and prolonged-release melatonin 4 mg were observed efficacious in observational studies. Melatonin displayed ineffective in the 2-month trial; thus, 3 months or more may be an enough duration for migraine therapy. Despite melatonin being generally safe, emerging literature is illustrating that a few severe adverse effects can be caused by melatonin, for example, liver injuries, reproductive system dysfunctions, and detrimental immunostimulation.

Conclusions

Melatonin is very likely to be a promising alternative for migraine prophylaxis. Current literature examining melatonin's efficacy in migraine prevention is growing, but still limited. Future studies of perfect design in methodology, pharmacology, and therapeutics are needed to achieve a deeper awareness of melatonin's role in migraine as well as more studies to explore the safety issues of melatonin medicine.
Fractures as a suicidal behavior risk factor
Authors: Tsai Chun-Hao , Cheng Wan-Ju , Muo Chih-Hsin , Lin Tsung-Li
Published: January 2019

This study aimed to evaluate the association between fracture history and sequential risk of suicidal behavior.

A total of 82,804 patients with fractures and 82,804 control subjects without fractures were matched. The influence of fractures on the risk of suicidal behavior-related hospital visit was analyzed using a Cox proportional hazards model.

The overall adjusted hazard ratio (aHR) of suicidal behavior-related hospital visit was 2.21 in fracture cohort. The aHR declined from 2.90 at the 1-year follow-up to 2.00 after 5 years or more. In fracture patients, the risk of suicidal behavior-related hospital visit was higher at age <35 years, with comorbidities of stroke and sleep disorder. Patients with multiple fractures had a 1.69-fold risk.

Fractures are an independent risk factor for suicidal behavior. Individuals aged younger than 35 years, with comorbidities of stroke and sleep disorder, and those who have suffered multiple fractures have elevated risk of suicidal behavior among subjects in the fracture group. Furthermore, this increased risk remained even after 5 years had passed since the fracture incident.
A cohort study of immune and hematopoietic functionality changes in severe aplastic anemia patients treated with immunosuppressive therapy
Authors: Guan Jing , Sun Yingying , Fu Rong , Wang Huaquan , Ruan Erbao , Wang Xiaoming , Qu Wen , Wang Guojin , Liu Hong , Wu Yuhong , Song Jia , Xing Limin , Li Lijuan , Liu Hui , Liu Chunyan , Shao Zonghong
Published: January 2019

To investigate if variations in immune and hematopoietic parameters correlated with immunosuppressive therapy (IST) in severe aplastic anemia (SAA) patients.

A total of 115 SAA patients who received IST were included. Their immune and hematopoietic functionality changes had been evaluated at 0, 0.5, 1, 2, and 3-year(s) IST.

For SAA patients with complete remission (CR), the CD4+/CD8+T cell ratio continued to increase after a year of IST. The T helper (Th)1/Th2 ratio continued to decrease after 6 months of IST, as did the activated CD8+ T cell percentage. The myeloid dendritic cell (mDC)/plasmacytoid dendritic cell (pDC) ratio after 3 years of IST was significantly lower compared to that of untreated patients. The mDC/pDC and Th1/Th2 ratios exhibited positive correlation. The activated CD8+ T cell percentage and the number of peripheral blood neutrophils showed inverse correlation. For SAA patients with partial remission (PR), the CD4+T cell percentage increased at 1-year post-IST, but the later changes were not statistically significant. The other immune indexes of patients in partial remission group and nonremission (NR) group showed no obvious recovery. For all SAA patients, the percentage of T regulatory cells in CD4+ lymphocyte was higher in post-IST group compared to the pretreatment group.

For SAA patients responded well to IST, increase in peripheral neutrophils and improvement in bone marrow myeloid cells were first observed followed reduction in the activated CD8+ T cell percentage, Th1/Th2 ratio, CD4+/CD8+T ratio, along with mDC/pDC ratio, all of which negatively correlated with the hematopoietic parameters. This demonstrates that IST prompts improvements of hematopoietic functionalities of the SAA patients by regulating their immune functionalities.
Pleural amyloidosis with recurrent pleural effusion and pulmonary embolism
Authors: Dai Yong , Liu Chaoling , Chen Jiaping , Zeng Qigang , Duan Chenxia
Published: January 2019

Rational:

Clinical and radiologic manifestations of pleural amyloidosis are non-specific. And it can easily be missed or misdiagnosed. Meanwhile, few studies document amyloidosis presenting with pulmonary infarcts at the same time. Hereby, we report a case of immunoglobulin light chain amyloidosis (AL) pleural amyloidosis with pulmonary embolism rarely reported.

Patient concerns:

A 66-year-old male patient who suffered recurrent pleural effusion for more than 6 months and coughed for 2 months was admitted to hospital for clear diagnosis and treatment. He was previously engaged in a job which exposed him to dust and talcum powder for a long time. He underwent right thoracentesis and anti-infective treatment before admission. The patient's cough and shortness of breath were slightly relieved. He still experienced pleural effusion and had symptoms of cough and shortness of breath.

Diagnosis:

Chest X-ray demonstrated bilateral pleural effusion. Chest computed tomography (CT) angiography demonstrated left lower pulmonary embolism. The thorascopy showed hyperaemia and black tissue of the parietal pleura, which were biopsied. The pathological diagnosis was amyloidosis. The final diagnosis of this patient was AL pleural amyloidosis and left lower pulmonary embolism.

Intervention:

During the hospitalization, the patient underwent thoracentesis several times without any conclusive diagnosis. After the diagnosis of pleural amyloidosis, the patient was repeatedly advised to undergo bone marrow biopsy and pleurodesis which the patient refused. For pulmonary embolism, Nadroparin calcium combined Warfarin were administered as anticoagulative therapy.

Outcomes:

The pulmonary embolism resolved 13 days after the anticoagulant therapy. The patient refused treatment for pleural effusion and requested for discharge. At the time of discharge, shortness of breath was relieved, and the pleural effusion had decreased. The patient was lost to follow-up.

Lessons:

Amyloidosis is a rare disease which can be ignored by many clinicians. It needs to be diagnosed promptly since the prognosis of amyloidosis is poor. Clinicians must improve relevant understandings of this kind of disease so as not to delay the diagnosis and treatment. We must be alert to the occurrence of embolic disease among amyloidosis patients. Last but not least, we should also think of the possibility of amyloidosis in patients with pulmonary embolism and recurrent pleural effusion.
Effects of mitochondrial ATP-sensitive potassium channel activation (nicorandil) in patients with angina pectoris undergoing elective percutaneous coronary interventions
Authors: Zhu Houyong , Xu Xiaoqun , Fang Xiaojiang , Zheng Jianwu , Chen Tielong , Huang Jinyu
Published: January 2019

Aims:

Nicorandil, which is a mitochondrial ATP-sensitive potassium channel opener, is believed to improve perioperative myocardial injury (PMI) in patients undergoing percutaneous coronary intervention (PCI), but recent studies have shown that nicorandil treatment did not improve functional and clinical outcomes in patients with angina pectoris who underwent elective PCI. We performed a meta-analysis to investigate the protective effect of nicorandil on perioperative injury in patients with angina pectoris who underwent elective PCI.

Methods:

The Medline, EMBASE, and Cochrane databases were searched for randomized clinical trials examining the effects of nicorandil. Two investigators independently selected suitable trials, extracted data, and assessed trial quality.

Results:

Seven studies of patients undergoing elective PCI, comprising a total of 979 patients, were included in this review. The results showed that nicorandil did not reduce the levels of markers of myocardial injury (standardized mean difference [SMD] 0.31 [95%CI −0.6, 1.22] for creatine kinase-MB [CK-MB] and 1.29 [95%CI −2.18, 4.76] for troponin I [TNI]), perioperative complications (relative risk [RR] 0.91 [95%CI 0.46–1.81]), target vessel revascularization (RR 0.79 [95%CI 0.50–1.25]) or major adverse cardiac events (MACE) (RR 0.83 [95%CI 0.49–1.43]). Nicorandil did reduce the corrected TIMI frame count (SMD-0.30 [95%CI −0.52, −0.09]).

Conclusion:

Although nicorandil did not reduce the overall incidence of perioperative complications and the incidence of major adverse cardiac events (MACE) in patients with angina pectoris who underwent elective PCI, it could still improve no reflow and slow coronary flow.
Relaxed skin tension line-oriented keystone-designed perforator island flaps considering the facial aesthetic unit concept for the coverage of small to moderate facial defects
Authors: Yoon Chi Sun , Kim Hyo Bong , Kim Young Keun , Kim Hoon , Kim Kyu Nam
Published: January 2019

A variety of reconstructive options including skin grafts, loco-regional flaps, and free flaps exist for the coverage of facial defects. Each technique has advantages and disadvantages. In this study, we present our experience with relaxed skin tension line (RSTL)-oriented, keystone-designed perforator island flap (KDPIF) reconstructions, in consideration of the facial aesthetic unit concept for coverage of small to moderate facial defects.

Between May 2016 and February 2018, 17 patients (11 men and 6 women), with an average age of 63.53 years (range: 37–83 years) underwent KDPIF reconstructions to cover facial defects. We performed the KDPIF reconstructions in consideration of the RSTLs and facial aesthetic unit concept. We retrospectively reviewed the defect causes, locations, and sizes, and flap sizes, types, and survivals, as well as the complications. Cosmetic outcomes were evaluated using the Harris 4-stage scale from 3 independent plastic surgeons’ point of view, and postoperative satisfactory surveys from all patients.

All defects were successfully covered with KDPIF. The defect sizes varied from 1.5 × 1.5 cm2 to 3 × 3.5 cm2. The flap sizes varied from 1.5 × 3 cm2 to 3 × 5.5 cm2. All flaps fully survived and there were no postoperative complications. The average subjective patient satisfaction score was 8.29 (range: 7–10) and the objective cosmetic outcomes were favorable (fair, good, or excellent).

Considering its simplicity and safety, the KDPIF is a good reconstruction option with few complications and high reproducibility. The RSTL-oriented KDPIF reconstruction is a good reconstructive option for covering small to moderate facial defects with superior aesthetic outcomes.
The utility of Jerk-locked back averaging technique in diagnosis of generalized myoclonic epilepsy with normal scalp EEG
Authors: Zheng Xiangyu , Wang Zan , Liu Chang , Hu Minghui , Lv Yudan
Published: January 2019

Rationale:

The diagnosis of myoclonic epilepsy and the classification of generalized or partial type may be challenging, especially when the scalp electroencephalogram (EEG) is normal. In such situation, how to apply another electrophysiological technique to begin the diagnosis and classification? The utility of Jerk-locked back averaging technique has been described in our case.

Patient concerns:

A Chinese patient (male, 21 years old) presented with frequent unilateral or bilateral shoulder-jerking. He has an epilepsy history of complex partial seizure (CPS) or secondary tonic-clonic seizure (sGTCS) for 10 years.

Diagnosis:

After admission, scalp EEG was performed with the normal result when the patient showed the jerks. According to the patient's clinical presentation, we suspected myolconic seizure, but there was lack of objective evidence. Then we used Jerk-locked back averaging technique to help begin the diagnosis. A bilateral-symmetrical time-locked, evoked cortical averaging potential that preceded the jerk has been found. So the jerks were considered as cortical origin and generalized myoclonic seizure was confirmed.

Interventions:

So in this situation, we added another antiepileptic drug of Levetiracetam (1500 mg/24 h).

Outcomes:

One week later, the jerk seizure had disappeared, and in the following visit, he had an improved prognosis with decreased seizure frequency.

Lessons:

Generalized polyspike-slow wave in EEG was common to see in myoclonic seizure and can help to make the classification. However, it should not dissuade clinicians from the diagnosis of myoclonic epilepsy with normal scalp EEG. Under this condition, we may apply other electrophysiological technique such as Jerk-locked back averaging technique, to give objective evidence to verify the cortical origin.
Pediatric plexiform fibromyxoma
Authors: Fukazawa Mitsuharu , Koga Hiroshi , Hiroshige Shoji , Matsumoto Toshifumi , Nakazono Yuichi , Yoshikawa Yasuji
Published: January 2019

Background:

Plexiform fibromyxoma (PF) is a rare gastric mesenchymal tumor, with approximately 80 cases reported to date. Gastrointestinal stromal tumor, the most common primary mesenchymal tumor of the stomach, shows different biological and clinical characteristics between adult and pediatric patients.

Objectives:

This systematic literature review was conducted to elucidate the pathological and clinical features of pediatric PF compared to adult PF.

Methods:

MEDLINE (1948 to March 2018) and EMBASE (1947 to March 2018) were searched, and all English articles that reported clinical data on PF patients were identified. Two authors independently reviewed the articles and extracted data to assess immunohistochemistry, sex, chief complaint, tumor size, tumor-related mortality, and tumor recurrence and metastasis.

Results:

A total of 41 reports with 80 PF patients (of whom 70 were adult PF and 10 were pediatric PF patients) confirmed by histological and immunohistochemical findings were included. Of a total of 80 tumors, 62 (78%) were located in the gastric antrum, 42 (65%) presented with ulceration, and 48 (74%) were resected by partial gastrectomy. Median tumor size of the resected specimen was larger in pediatric PF than in adult PF cases (5.3 cm vs 4.0 cm, P = .036). However, there was no difference between pediatric and adult PFs in immunohistochemical expression, sex predominance, chief complaint, tumor-related mortality, and tumor recurrence and metastasis during the follow-up periods.

Conclusion:

Other than increased tumor growth in pediatric PFs, PF is a single disease entity with similar pathological features and benign clinical behavior regardless of onset age.
Ultrasonographic findings of intrahepatic lymphoepithelioma-like cholangiocarcinoma associated with Epstein–Barr virus
Authors: Ling Wenwu , Lu Changli , Huang He , Qiu Tingting , Lu Qiang , Huang Chengwu , Gong Ping , Luo Yan , Chen Shigao
Published: January 2019

Rationale:

Epstein–Barr virus (EBV)-related lymphoepithelioma-like cholangiocarcinoma (LELCC) is an extremely rare primary liver tumor with nonspecific clinical manifestations. The clinicopathological features of EBV-associated LELCC have been reported in a few cases. But reports of the tumor's imaging characteristics, particularly ultrasonographic findings, are very rare.

Patient concerns:

The first patient was a 64-year-old man with left upper quadrant pain and no nausea and dizziness for about 3 months. The second patient was a 40-year-old man, had an incidental finding of a hepatic tumor in a routine health checkup at a local hospital.

Diagnoses:

In the first patient, the abdominal ultrasound demonstrated a slightly heterogeneous hypoechoic nodule in segment 3 of the liver. The nodule was about 2.0 cm × 1.7 cm in size, with a clear margin and regular shape. Color Doppler flow imaging (CDFI) revealed no blood flow signals in this nodule. According to the clinical information and imaging features, it was difficult to determine the diagnosis of the nodule. In the second patient, gray-scale ultrasound revealed a slightly heterogeneous hypoechoic mass measuring 3.5 cm × 2.5 cm with well-defined margin and regular shape at the superior segment of the left hepatic lateral lobe. There was a blurrily hypoechoic halo around the mass. In contrast-enhanced ultrasound (CEUS), the mass was homogeneous hyperenhancement in the arterial phase. In the portal phase and late phase, the center enhancement of the mass washed out gradually, presenting hypoenhancement, Therefore, the tumor was diagnosed as malignancy.

Intervention:

Finally, a laparoscopic left hepatic lateral lobectomy was performed in the first patient. The second patient underwent a left hepatectomy with cholecystectomy.

Outcomes:

The first patient has been alive without recurrence or distant metastases for 11 months since the surgery. The second patient received routine follow-up after surgery. Until now, he has been tumor-free for 32 months.

Lessons:

We mainly focus on the ultrasound characteristics of EBV-associated LELCC, especially its enhancement patterns on CEUS, which may provide valuable information for diagnosis of the LELCC. When a liver tumor with typical CEUS patterns of malignancy is found in middle-aged adults with EBV positive, the possibility of EBV-related LELCC should be considered.
Novel Approaches for Systemic Mastocytosis
Authors: Fletcher Luke , Borate Uma
Published: January 2019

Purpose of review

The purpose of this review is to summarize the pathophysiology of systemic mastocytosis, review the most recent clinical trials and drug development in systemic mastocytosis, with a specific focus on the advanced systemic mastocytosis subtypes.

Recent findings

Systemic mastocytosis is a clonal neoplasm of mast cells that has had a number of successful therapeutic options being developed in the past few years. The first therapeutic agent to be Food and Drug Administration (FDA) approved in decades was midostaurin in 2017 with a 60% response rate % with improvement in both end-organ damage and symptoms. However, complete responses/remissions with midostaurin have been elusive. Additional clinical trials of tyrosine kinase inhibitors that target the proto-oncogene receptor tyrosine kinase (KIT) mutation show great promise. The two drugs with promising early clinical trial data include avapritinib and DCC-2618 with avapritinib showing potential to induce complete remissions.

Therapies for systemic mastocytosis are in a stage of evolution with further elucidation of additional mutations associated with oncogenesis in addition to the most commonly describedKIT(give details), ongoing clinical trials could potentially with lead to further targeted therapy and increased complete responses and durable remissions.
BAP1 Loss is a Useful Adjunct to Distinguish Malignant Mesothelioma Including the Adenomatoid-like Variant From Benign Adenomatoid Tumors
Authors: Erber Ramona , Warth Arne , Muley Thomas , Hartmann Arndt , Herpel Esther , Agaimy Abbas
Published: January 2019

Malignant mesothelioma (MM) can show areas closely mimicking reactive mesothelial proliferations or recapitulating benign adenomatoid tumors (ATs) making distinction on occasion impossible on morphologic ground alone, particularly in limited biopsy material. Recently, loss of BAP1 by immunohistochemistry (IHC) has been suggested as a potential marker for identifying MM, but data is still limited. We studied 264 MM cases (257 using tissue microarrays; 7 on conventional slides) and 42 genital ATs for BAP1 immunohistochemical expression. Loss of BAP1 protein expression was observed in 119/211 of MM cases (56.4%). Taken by histologic type, 64.3% of biphasic, 55.4% of epithelioid, and 41.7% of sarcomatoid MM were BAP1-deficient. In contrast, all 42 ATs showed retained BAP1 immunoreactivity. Notably, all 4 MM cases with variable adenomatoid-like features were BAP1-deficient. Surface components of MM of the pleura showed concordant loss as the invasive tumor suggesting a potential role for BAP1 loss for recognizing so-called early mesothelioma. In conclusion, BAP1 loss demonstrated by IHC is seen in more than half of MM cases but none of ATs. Thus, BAP1 IHC represents a potential adjunct for distinguishing MM from benign mesothelial proliferations including in particular “MM with bland adenomatoid-like pattern versus benign ATs” on biopsy material and early mesothelioma with limited invasion.
Evaluation of Endoscopic Strip Craniectomy and Orthotic Therapy for Bilateral Coronal Craniosynostosis
Authors: Williams Conor T. , Segar David J. , Naidoo Sybill D. , Skolnick Gary B. , Proctor Mark R. , Smyth Matthew D. , Patel Kamlesh B.
Published: January 2019

Background:

Bilateral coronal craniosynostosis is the premature fusion of both coronal sutures. Traditionally, this condition is treated by frontal–orbital advancement (FOA). Endoscopic strip craniectomy with cranial orthotic therapy, which has gained popularity in treating single suture craniosynostosis, has recently been adapted for the treatment of bicoronal synostosis. There have been few studies documenting the outcomes of this treatment. The objective of this study is to compare the morphological outcomes of endoscopic strip craniectomy and FOA in patients with bicoronal synostosis.

Methods:

A retrospective case series was done on 24 patients with bilateral coronal synostosis treated with endoscopic strip craniectomy or FOA at 2 institutions. Patients with preoperative and 1-year postoperative computed tomography scans were included. Multiple measures of cranial shape and size were assessed: cephalic index, turricephaly index, basofrontal angle, circumference Z-score, and cranial vault volume Z-score.

Results:

The 2 groups were similar statistically in terms of morphology at preoperative scan. There were no statistical differences between the FOA group and endoscopic group at postoperative scan in any of the morphologic outcomes measured. Cranial vault volume Z-scores increased postoperatively in both the endoscopic (P < 0.001) and FOA (P = 0.034) groups.

Conclusions:

One year after repair there were no significant morphological difference between patients with bicoronal synostosis treated with the endoscopic approach and those treated by FOA.
Management of Craniopharyngioma
Authors: Kiliç Mustafa , Can Songül Meltem , Özdemir Burak , Tanik Canan
Published: January 2019

Objective:

Craniopharyngiomas are one of the most challenging problems for neurosurgeons because of the high recurrence rates due to their localization and associated endocrinological disorders. This study reports the outcomes of surgeries and recurrence rates of 45 craniopharyngioma cases.

Method:

Patients who were diagnosed with craniopharyngioma in the authors’ clinic between 1998 and 2016 evaluated retrospectively.

Results:

A total of 45 patients (25 males and 20 females; age, 3–56 years) who had previously undergone surgery for craniopharyngioma were enrolled and followed up for 12 to 222 months (mean follow-up duration, 73.5 ± 55.2 months). Among these, 25 were children (55.5%) with a mean age of 9.5 ± 4.6 years and 20 were adults (45.5%) with a mean age of 37.2 ± 12.7 years. A total of 39 (86.6%) patients underwent total tumor resection, while 6 (13.4%) underwent subtotal tumor removal. During 12 to 30 months of follow-up, the progression of residual tumor was recorded in 4 patients, 3 of whom underwent surgery and postoperative radiotherapy. Despite the total resection of tumor in their first surgery, 3 patients showed tumor recurrence during 15 to 34 months of the follow-up.

Conclusion:

Our findings suggest that the total resection of craniopharyngiomas, when possible, results in a favorable quality of life, with acceptable mortality and morbidity rates.
Three-Dimensional Computed Tomography Analysis of Stability Following Two- and Three-Point Fixation With Biodegradable Plates Among Patients With Zygomatic Fracture
Authors: Na Woong Gyu , Lim Hyoseob , Koh Sung Hoon
Published: January 2019

Background:

Open reduction followed by internal fixation has been regarded as the most effective technique for the surgical repair of zygomatic fractures. However, the ideal number and locations of internal fixation points to maintain stable reduction remain controversial. Using 3-dimensional computed tomography, we aimed to compare the stability of 2-point and 3-point fixation among patients who had undergone surgical repair of zygomatic bone fractures.

Methods:

The study included 22 patients (17 men, 5 women) with unilateral zygomatic bone fractures who had undergone open reduction with 2-point or three-point fixation using biodegradable materials (11 patients in each group). The authors measured the lateral projection and height of the zygoma at 2 landmark points (zygomaticofacial foramen and frontozygomatic suture). In each group, bony displacement was analyzed between the preoperative and immediate postoperative phases, and between the preoperative and follow-up phases. Differences in stability between the 2 groups were analyzed by comparing values between the immediate postoperative and follow-up phases.

Results:

The 2-point group exhibited a lower rate of complex fractures at the frontozygomatic suture than the 3-point group (18.2%, 63.6%, respectively). In both groups, the authors observed significant differences in the lateral projection of the zygomaticofacial foramen between the preoperative and immediate postoperative phases, and between the preoperative and follow-up phases. No significant differences in stability were observed between the groups.

Conclusion:

Our findings demonstrated that 2-point fixation of the zygoma with biodegradable materials is as stable as 3-point fixation. It could be initially considered when open reduction of frontozygomatic suture was not essential.
A Biographical Journey Through the History of Ear Reconstruction
Authors: Price James C. , Lloyd Mark S.
Published: January 2019

This historical literature review of ear reconstruction differs from much of the published literature in that it focuses on the biographies of the innovators and evangelists of ear reconstruction rather than specific techniques. A biographical account of ear reconstruction demonstrates the importance of surgeons as artists, scientists, technicians, politicians, and most importantly carers of this special and fascinating group of patients who benefit from this technically challenging form of reconstruction.
Feature Design for Protein Interface hotspots using KFC2 and Rosetta
Authors: Seeger Franziska , Little Anna , Chen Yang , Woolf Tina , Cheng Haiyan , Mitchell Julie C.
Published: January 2019

Protein-protein interactions regulate many essential biological processes and play an important role in health and disease. The process of experimentally charac-terizing protein residues that contribute the most to protein-protein interaction affin-ity and specificity is laborious. Thus, developing models that accurately characterize hotspots at protein-protein interfaces provides important information about how to inhibit therapeutically relevant protein-protein interactions. During the course of the ICERM WiSDM workshop 2017, we combined the KFC2a protein-protein interaction hotspot prediction features with Rosetta scoring function terms and interface filter metrics. A 2-way and 3-way forward selection strategy was employed to train support vector machine classifiers, as was a reverse feature elimination strategy. From these results, we identified subsets of KFC2a and Rosetta combined features that show improved performance over KFC2a features alone.
Evolving super stimuli for real neurons using deep generative networks
Authors: Ponce Carlos R. , Xiao Will , Schade Peter F. , Hartmann Till S. , Kreiman Gabriel , Livingstone Margaret S.
Published: January 2019

Finding the best stimulus for a neuron is challenging because it is impossible to test all possible stimuli. Here we used a vast, unbiased, and diverse hypothesis space encoded by a generative deep neural network model to investigate neuronal selectivity in inferotemporal cortex without making any assumptions about natural features or categories. A genetic algorithm, guided by neuronal responses, searched this space for optimal stimuli. Evolved synthetic images evoked higher firing rates than even the best natural images and revealed diagnostic features, independently of category or feature selection. This approach provides a way to investigate neural selectivity in any modality that can be represented by a neural network and challenges our understanding of neural coding in visual cortex.

Highlights

A generative deep neural network interacted with a genetic algorithm to evolve stimuli that maximized the firing of neurons in alert macaque inferotemporal and primary visual cortex.

The evolved images activated neurons more strongly than did thousands of natural images.

Distance in image space from the evolved images predicted responses of neurons to novel images.
Conserved transcriptomic profile between mouse and human colitis allows temporal dynamic visualization of IBD-risk genes and unsupervised patient stratification
Authors: Czarnewski Paulo , Parigi Sara M. , Sorini Chiara , Diaz Oscar E. , Das Srustidhar , Gagliani Nicola , Villablanca Eduardo J.
Published: January 2019

Abstrasct

Despite the fact that ulcerative colitis (UC) patients show heterogeneous clinical manifestation and diverse response to biological therapies, all UC patients are classified as one group. Therefore, there is a lack of tailored therapies. In order to design these, an unsupervised molecular re-classification of UC patients is evoked. Classical clustering approaches based on tissue transcriptomic data were not able to classify UC patients into subgroups, likely due to associated covariates. In addition, while genome wide association studies (GWAS) have identified potential new target genes, their temporal dynamic revealing the optimal therapeutic window of time remains to be elucidated. To overcome the limitations, we generated time-series transcriptome data from a mouse model of colitis, which was then cross-compared with human datasets. This allowed us to visualize IBD-risk gene expression kinetics and reveal that the expression of the majority of IBD-risk genes peak during the inflammatory phase, and not the recovery phase. Moreover, by restricting the analysis to the most differentially expressed genes shared between mouse and human, we were able to cluster UC patients into two subgroups, termed UC1 and UC2. We found that UC1 patients expressed higher copy of genes involved in neutrophil recruitment, activation and degranulation compared to UC2. Of note, we found that over 87% of UC1 patients failed to respond to two of the most widely-used biological therapies for UC.

This study serves as a proof of concept that cross-species comparison of gene expression profiles enables the temporal annotation of disease-associated gene expression and the stratification of patients as of yet considered molecularly undistinguishable.
Calcium-driven regulation of voltage-sensing domains in BK channels
Authors: Lorenzo-Ceballos Yenisleidy , Carrasquel-Ursulaez Willy , Castillo Karen , Alvarez Osvaldo , Latorre Ramon
Published: January 2019

Allosteric interplays between voltage-sensing domains (VSD), Ca2+-binding sites, and the pore domain govern the Ca2+- and voltage-activated K+ (BK) channel opening. However, the functional relevance of the Ca2+- and voltage-sensing mechanisms crosstalk on BK channel gating is still debated. We examined the energetic interaction between Ca2+ binding and VSD activation measuring and analyzing the effects of internal Ca2+ on BK channels gating currents. Our results indicate that the Ca2+ sensors occupancy has a strong impact on the VSD activation through a coordinated interaction mechanism in which Ca2+ binding to a single α-subunit affects all VSDs equally. Moreover, the two distinct high-affinity Ca2+-binding sites contained in the C-terminus domains, RCK1 and RCK2, appear to contribute equally to decrease the free energy necessary to activate the VSD. We conclude that voltage-dependent gating and pore opening in BK channels is modulated to a great extent by the interaction between Ca2+ sensors and VSDs.
How sample heterogeneity can obscure the signal of microbial interactions
Authors: Armitage David W. , Jones Stuart E.
Published: January 2019

Microbial community data are commonly subjected to computational tools such as correlation networks, null models, and dynamic models, with the goal of identifying the ecological processes structuring microbial communities. Researchers applying these methods assume that the signs and magnitudes of species interactions and vital rates can be reliably parsed from observational data on species’ (relative) abundances. However, we contend that this assumption is violated when sample units contain any underlying spatial structure. Here, we show how three phenomena — Simpson’s paradox, context-dependence, and nonlinear averaging — can lead to erroneous conclusions about population parameters and species interactions when samples contain heterogeneous mixtures of populations or communities. At the root of this issue is the fundamental mismatch between the spatial scales of species interactions (micrometres) and those of typical microbial community samples (millimetres to centimetres). These issues can be overcome by measuring and accounting for spatial heterogeneity at very small scales, which will lead to more reliable inference of the ecological mechanisms structuring natural microbial communities.