bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical Characteristics and Prognostic Factors of Patients with Intrahepatic Cholangiocarcinoma with Fever: A Propensity Score Matching Analysis
Authors: Gong Zi-Jun , Cheng Jian-Wen , Gao Pin-Ting , Huang Ao , Sun Yun-Fan , Zhou Kai-Qian , Hu Bo , Qiu Shuang-Jian , Zhou Jian , Fan Jia , Yang Xin-Rong 
Published: March 2019

Abstract

Background
Patients with intrahepatic cholangiocarcinoma (ICC) rarely present fever as the initial symptom. We aimed to identify clinical characteristics and prognostic factors for these feverish patients.

Subjects, Materials, and Methods
This study retrospectively reviewed 31 patients with ICC with fever (≥38.0°C) treated at our hospital between January 2002 and December 2014. A propensity score was used to match patients with and without fever at a ratio of 1:2.

Results
Patients with ICC with fever had higher serum γ-glutamyl transferase and carcinoembryonic antigen levels, larger tumors, poorer tumor differentiation, and worse prognosis (all p &lt; .05) than those without fever. This was supported by propensity score matching (PSM) analysis. Univariate and multivariate analyses indicated that microvascular invasion, hilar lymph node metastasis, and temperature ≥ 38.6°C were related to prognosis. Patients with ICC with fever had higher levels of leucocytes, neutrophils, neutrophil-to-lymphocyte ratio (NLR), and platelet-to-lymphocyte ratio (PLR) in peripheral blood before and after PSM analysis. Body temperature positively correlated with leucocytes (r = 0.599, p &lt; .001), neutrophils (r = 0.644, p &lt; .001), NLR (r = 0.681, p &lt; .001), and PLR (r = 0.457, p = .010).

Conclusion
Patients with ICC with fever ≥38.0°C and ≥38.6°C had poor and extremely poor prognosis, respectively. Radical surgical treatment may improve the prognosis of patients with ICC with fever &lt;38.6°C. However, systemic therapy (e.g., anti-inflammatory and immune therapy) may be preferable to surgery for these patients with fever ≥38.6°C.
Mutations in NUP160 Are Implicated in Steroid-Resistant Nephrotic Syndrome
Authors: Zhao Feng , Zhu Jun-yi , Richman Adam , Fu Yulong , Huang Wen , Chen Nan , Pan Xiaoxia , Yi Cuili , Ding Xiaohua , Wang Si , Wang Ping , Nie Xiaojing , Huang Jun , Yang Yonghui , Yu Zihua , Han Zhe 
Published: March 2019

Significance Statement
Mutations in >50 genes can lead to monogenic steroid-resistant nephrotic syndrome (SRNS). The authors found that a young patient with familial SRNS and FSGS carried novel compound-heterozygous mutations in NUP160; this gene encodes nucleoporin 160 kD, one of the protein components of the nuclear pore complex. Using an in vivo renal cell assay on the basis of Drosophila nephrocytes (an experimental podocyte model previously used to validate candidate renal disease genes and specific patient-derived mutant alleles), they validated the NUP160 gene variants as factors implicated in kidney pathology. The findings indicate that NUP160 should be included in the SRNS diagnostic gene panel to identify additional patients with SRNS carrying homozygous or compound-heterozygous NUP160 mutations.

Background
Studies have identified mutations in >50 genes that can lead to monogenic steroid-resistant nephrotic syndrome (SRNS). The NUP160 gene, which encodes one of the protein components of the nuclear pore complex nucleoporin 160 kD (Nup160), is expressed in both human and mouse kidney cells. Knockdown of NUP160 impairs mouse podocytes in cell culture. Recently, siblings with SRNS and proteinuria in a nonconsanguineous family were found to carry compound-heterozygous mutations in NUP160.

Methods
We identified NUP160 mutations by whole-exome and Sanger sequencing of genomic DNA from a young girl with familial SRNS and FSGS who did not carry mutations in other genes known to be associated with SRNS. We performed in vivo functional validation studies on the NUP160 mutations using a Drosophila model.

Results
We identified two compound-heterozygous NUP160 mutations, NUP160R1173×
and NUP160E803K
. We showed that silencing of Drosophila NUP160 specifically in nephrocytes (fly renal cells) led to functional abnormalities, reduced cell size and nuclear volume, and disorganized nuclear membrane structure. These defects were completely rescued by expression of the wild-type human NUP160 gene in nephrocytes. By contrast, expression of the NUP160 mutant allele NUP160R1173×
completely failed to rescue nephrocyte phenotypes, and mutant allele NUP160E803K
rescued only nuclear pore complex and nuclear lamin localization defects.

Conclusions
Mutations in NUP160 are implicated in SRNS. Our findings indicate that NUP160 should be included in the SRNS diagnostic gene panel to identify additional patients with SRNS and homozygous or compound-heterozygous NUP160 mutations and further strengthen the evidence that NUP160 mutations can cause SRNS.
Chilaiditi sendromu ve mide kanseri
Authors: BAŞ Onur , MUSTAFAYEV Fuad , VAHABOV Cavanşir 
Published: March 2019

Karın ağrısı, nefes darlığı
nedeniyle acil servise başvuran 83 yaşındaki kadın hastanın rektal tuşesinde
melena saptanması üzerine yapılan endoskopide mide antrumundaki ülsere adeno kanser
tanısı konuldu. Hastaya malignite evrelemesi amacıyla abdomen bilgisayarlı
tomografi çekildi. Mide duvarı kalınlığı dışında, Chilaiditi belirtisi de
saptandı. Chilaiditi belirtisi kolon ve/veya
ince barsağın karaciğer ve diyafragma arasına transpozisyonu olarak
adlandırılır. Toplumda görülme sıklığı %0.1-%1 arasındadır. Erkeklerde ve
yaşlılarda daha sık görülür. Genelde tesadüfen saptanır
ve hastalar çoğunlukla asemptomatiktir. Sağ üst kadran ağrısı, nefes darlığı, kabızlık
gibi semptomlarla birlikteliğinde Chilaiditi sendromu olarak adlandırılır. En
sık bilinen önemi diyafragma altındaki gaz görünümü nedeniyle pnömoperitoneum ile karıştırılarak gereksiz
cerrahi girişimlere neden olabilmesidir. Chilaiditi belirtisinin bazı
hastalıklar ile birlikte olduğu da bildirilmiştir. Literatürde kolon volvulusu,
kronik obstrüktif akciğer hastalığı, siroz, atrofik veya pitotik karaciğer
varlığı, frenik sinir hasarı ve obezitenin Chilaiditi belirtisi ile beraber görüldüğü
vaka sunumları mevcuttur. Chilaiditi belirtisinin kolon kanseri ile beraber
görüldüğü vakalar bildirilmiştir. Bizim hastamızda mide kanseri ile
birliktelik söz konusudur. Karaciğer pitozu, sirozu gibi bazı
patolojilerdeki gibi neden-sonuç ilişkisi kurulamasa da, tümörle birlikte
olabileceği de unutulmamalıdır.
Clinical features, laboratory characteristics and outcome of patients with drug-induced acute liver failure
Authors: Nabi Tauseef , Rafiq Nadeema , Jamil Imran , Arifa Quratul Ain 
Published: March 2019

Background: Acute liver failure (ALF) is a rare medical emergency. Its rapid progression and high mortality demand early diagnosis and expert management. Drug-induced ALF (DI-ALF) remains the uncommon cause of ALF in India. Clinical and etiological profile varies with geographical area and time. A prospective study of DI-ALF was carried with the aim to determine the clinical features, laboratory characteristics, outcome and hospital course.Methods: A total of 15 patients with a diagnosis of DI-ALF were included in the study. The variables evaluated were demographic, signs and symptoms, biochemical parameters [bilirubin, alanine aminotransferase (ALT), aspartate aminotransferase (AST), prothrombin time (PT), internal normalization ratio (INR) etc.], outcome and course during hospitalization.Results: Out of 15 DI-ALF patients, 12 had Anti-tuberculosis therapy (ATT) induced ALF and 3 patients had ayurvedic induced ALF. Majority of the patients were females (73.3%) and middle-aged (42.60±14.30 years). Coma grade at the time of admission showed that majority of patients (66.8%) had grade I and II encephalopathy. Depending on the pattern of liver injury, hepatocellular pattern was most common (53.3%) followed by mixed and cholestatic pattern. 40% of patients died with DI-ALF complications of which ATT induced ALF contributed 41.7%. Mean AST was more increased as compared to ALT. Development of ascites (P = 0.030) and mannitol use (P = 0.025) was significantly more common in non survived group than survived group. Length of hospital stay was significantly more in non survived group than survived group (P = 0.009).Conclusions: ATT was the class of drugs most frequently associated with DI-ALF. DI-ALF disproportionately affected middle-aged women. Most DILI ALF patients had hepatocellular injury pattern. 40% of patients died with DI-ALF complications. Development of ascites, mannitol use and length of hospital stay was significantly more in non survived group than survived group.
Fracture of an Os Hamulus Proprium
Authors: Graham Jack G. , Matzon Jonas L. , Czachor Boleslaw , Lucenti Ludovico , Beredjiklian Pedro K. 
Published: March 2019

Case:
We report the case of a 39-year-old man who sustained an acute fracture of an os hamulus proprium, which was treated with open surgical excision after nonoperative treatment was unsuccessful. At the most recent follow-up, at 6 years after surgery, the patient was asymptomatic and maintained full function of the hand and the wrist.

Conclusion:
An os hamulus proprium is often confused with a fracture of the hook of the hamate, and little information exists in the literature regarding these accessory ossicles. To our knowledge, an os hamulus proprium fracture has not previously been described in the literature.
Antibiotic Susceptibility of Cronobacter spp. Isolated from Clinical Samples
Authors: HOLÝ ONDŘEJ , ALSONOSI ABDLRHMAN , HOCHEL IGOR , RÖDEROVÁ MAGDALÉNA , ZATLOUKALOVÁ SIMONA , MLYNÁRČIK PATRIK , KOLÁŘ MILAN , PETRŽELOVÁ JANA , ALAZRAQ AIYDA , CHMELAŘ DITTMAR , FORSYTHE STEPHEN 
Published: March 2019

Abstract

Cronobacter spp. have been recognized as causative agents of various severe infections in pre-term or full-term infants as well as elderly adults suffering from serious underlying disease or malignancy. A surveillance study was designed to identify antibiotic resistance among clinical Cronobacter spp. strains, which were isolated from patients of two hospitals between May 2007 and August 2013. Altogether, 52 Cronobacter spp. isolates were analyzed. Although MALDI-TOF mass spectrometry recognized all Cronobacter sakazakii and Cronobacter malonaticus strains, it could not identify Cronobacter muytjensii strain. Nevertheless, all strains were identified as Cronobacter spp. using multilocus sequence typing (MLST). Strains were tested against 17 types of antibiotics, using the standard microdilution method according to the 2018 European Committee on Antimicrobial Susceptibility Testing criteria. Three Cronobacter species were identified as C. sakazakii (n = 33), C. malonaticus (n = 18), and C. muytjensii (n = 1); all isolates were susceptible to all tested antibiotics. All strains were PCR-negative for bla
TEM, bla
SHV, and bla
CTX-M β-lactamase genes, as well. Even though the results of this study showed that Cronobacter spp. isolates were pan-susceptible, continued antibiotic resistance surveillance is warranted.
Immunosuppression and Immunotargeted Therapy in Acute Myeloid Leukemia - The Potential Use of Checkpoint Inhibitors in Combination with Other Treatments
Authors: Leufven Eva , Bruserud Øystein 
Published: March 2019

Introduction:
Immunotherapy by using checkpoint inhibitors is now tried in the
treatment of several malignancies, including Acute Myeloid Leukemia (AML). The treatment
is tried both as monotherapy and as a part of combined therapy.

Methods:
Relevant publications were identified through literature searches in the PubMed
database. We searched for (i) original articles describing the results from clinical studies of
checkpoint inhibition; (ii) published articles describing the immunocompromised status of
AML patients; and (iii) published studies of antileukemic immune reactivity and immunotherapy
in AML.

Results:
Studies of monotherapy suggest that checkpoint inhibition has a modest antileukemic
effect and complete hematological remissions are uncommon, whereas combination with conventional
chemotherapy increases the antileukemic efficiency with acceptable toxicity. The
experience with a combination of different checkpoint inhibitors is limited. Thalidomide derivatives
are referred to as immunomodulatory drugs and seem to reverse leukemia-induced
immunosuppression, but in addition, they have direct inhibitory effects on the AML cells. The
combination of checkpoint targeting and thalidomide derivatives thus represents a strategy for
dual immunotargeting together with a direct antileukemic effect.

Conclusion:
Checkpoint inhibitors are now tried in AML. Experimental studies suggest that
these inhibitors should be combined with immunomodulatory agents (i.e. thalidomide derivatives)
and/or new targeted or conventional antileukemic treatment. Such combinations would
allow dual immunotargeting (checkpoint inhibitor, immunomodulatory agents) together with
a double/triple direct targeting of the leukemic cells.
Women’s Beliefs About Medicines and Adherence to Pharmacotherapy in Pregnancy: Opportunities for Community Pharmacists
Authors: Ceulemans Michael , Lupattelli Angela , Nordeng Hedvig , Odalovic Marina , Twigg Michael , Foulon Veerle 
Published: March 2019

Background:During pregnancy, women might weigh the benefits of treatment against potential risks to the unborn child. However, non-adherence to necessary treatment can adversely affect both mother and child. To optimize pregnant women’s beliefs and medication adherence, community pharmacists are ideally positioned to play an important role in primary care.Objective:This narrative review aimed to summarize the evidence on 1) pregnant women’s beliefs, 2) medication adherence in pregnancy and 3) community pharmacists’ counselling during pregnancy.Methods:Three search strategies were used in Medline and Embase to find original studies evaluating women’s beliefs, medication adherence and community pharmacists’ counselling during pregnancy. All original descriptive and analytic epidemiological studies performed in Europe, North America and Australia, written in English and published from 2000 onwards were included.Results:We included 14 studies reporting on women’s beliefs, 11 studies on medication adherence and 9 on community pharmacists’ counselling during pregnancy. Women are more reluctant to use medicines during pregnancy and tend to overestimate the teratogenic risk of medicines. The risk perception varies with the type of medicine, level of health literacy, education level and occupation. Furthermore, low medication adherence during pregnancy is common. Finally, limited evidence showed that the current community pharmacists’ counselling is insufficient. Barriers hindering pharmacists are insufficient knowledge and limited access to reliable information.Conclusion:Concerns about medication use and non-adherence are widespread among pregnant women. Community pharmacists’ counselling during pregnancy is insufficient. Further education, training and research are required to support community pharmacists in fulfilling all the opportunities they have when counselling pregnant women.
Tubular and Glomerular Biomarkers of Acute Kidney Injury in Newborns
Authors: Kamianowska Monika , Szczepański Marek , Wasilewska Anna 
Published: March 2019

Background:Acute Kidney Injury (AKI) is a sudden decrease in kidney function. In the early period, the highest percentage of AKI occurs among newborns hospitalized in the neonatal intensive care units, especially premature neonates. The prognosis of AKI depends on the type and severity of the cause of an injury, the accuracy and the time of diagnosis and treatment. The concentration of serum creatinine is still the main diagnostic test, although it changes in the course of AKI later than glomerular filtration rate GFR. In addition, the reliability of the determination of creatinine level is limited because it depends on many factors. New studies have presented other, more useful laboratory markers of renal function that can be measured in serum and/or in urine.Objective:The aim of the work was to present the latest data about tubular and glomerular biomarkers of acute kidney injury in newborns.Methods:We undertook a structured search of bibliographic databases for peer-reviewed research literature by using focused review topics. According to the conceptual framework, the main idea of research literature has been summarized and presented in this study.Results:The concentrations of some novel biomarkers are higher in serum and/or urine of term and preterm newborns with AKI, especially in the course of perinatal asphyxia.Conclusion:In this systematic review of the literature, we have highlighted the usefulness of biomarkers in predicting tubular and/or glomerular injury in newborns. However, novel biomarkers need to prove their clinical applicability, accuracy, and cost-effectiveness prior to their implementation in clinical practice.
NSE, S100B and MMP9 Expression Following Reperfusion after Carotid Artery Stenting
Authors: Yuan Xiaofan , Wang Jianhong , Wang Duozi , Yang Shu , Yu Nengwei , Guo Fuqiang 
Published: March 2019

Objective:

Previous studies have shown that the neuron-specific- enolase (NSE), S100B protein (S100B) and matrix metalloproteinase-9 (MMP9) are specific markers for studying cerebral injury. This study was aimed to demonstrate these biomarkers for their correlation with reperfusion after carotid artery stenting (CAS).

Methods:
In this study, a total of 44 patients who were diagnosed unilateral carotid artery stenosis by digital subtraction angiography (DSA) and underwent CAS, were selected as the operation groups. The patients' blood samples were collected at three different time points: T1, prior to operation; T2, next morning after operation (24 hours); T3, three days after operation (72 hours); All of the patients with the operation received computed tomography perfusion (CTP) at T1 and T3. The second group of 12 patients, who were excluded for carotid artery stenosis by DSA, were assigned to be the control group; Blood samples of these patients were collected at T1. The concentrations of NSE, S100B and MMP9 in serum from patients of both groups were detected by ELISA.

Results:

All of the operations were implanted in stents successfully without complications. (1) After CAS, rCBF increased while rMTT and rTTP decreased. (2) The concentrations of NSE, S100B and MMP9 in the serum decreased gradually (T1>T2>T3). There was no significant difference between the control group and the operation group at T1 (P>0.05) on their concentrations of NSE, S100B and MMP9 in the serum. When compared among the operation groups, the concentrations of NSE, S100B and MMP9 in the serum at T1 and T3 showed significant difference (P < 0.05). (3) Correlation analysis among the operation groups indicated that NSE, S100B, MMP9 and rCBF were positively correlated before operation (r = 0.69, 0.58 and 0.72, respectively, P < 0.05), as well as after operation (r = 0.75, 0.65 and 0.60, respectively, P < 0.05).

Conclusion:

We concluded that the concentrations of NSE, S100B and MMP9 in serum decreased with the improvement of cerebral reperfusion after CAS. NSE, S100B and MMP9 can be used as laboratory biochemical markers to evaluate the improvement of reperfusion after CAS. The results very well complement the imaging methods, such as CTP.
Does Intraneuronal Accumulation of Carboxyl-terminal Fragments of the Amyloid Precursor Protein Trigger Early Neurotoxicity in Alzheimer’s Disease?
Authors: Lauritzen I. , Pardossi-Piquard R. , Bourgeois A. , Bécot A. , Checler F. 
Published: March 2019

Background:
Alzheimer’s disease (AD) is associated with extracellular accumulation and
aggregation of amyloid β (Aβ) peptides ultimately seeding in senile plaques. Recent data show that their
direct precursor C99 (βCTF) also accumulates in AD-affected brain as well as in AD-like mouse models.
C99 is consistently detected much earlier than Aβ, suggesting that this metabolite could be an early
contributor to AD pathology. C99 accumulates principally within endolysosomal and autophagic structures
and its accumulation was described as both a consequence and one of the causes of endolysosomalautophagic
pathology, the occurrence of which has been documented as an early defect in AD. C99 was
also accompanied by C99-derived C83 (αCTF) accumulation occurring within the same intracellular
organelles. Both these CTFs were found to dimerize leading to the generation of higher molecular
weight CTFs, which were immunohistochemically characterized in situ by means of aggregate-specific
antibodies.

Discussion:
Here, we discuss studies demonstrating a direct link between the accumulation of C99 and
C99-derived APP-CTFs and early neurotoxicity. We discuss the role of C99 in endosomal-lysosomalautophagic
dysfunction, neuroinflammation, early brain network alterations and synaptic dysfunction as
well as in memory-related behavioral alterations, in triple transgenic mice as well as in newly developed
AD animal models.

Conclusion:
This review summarizes current evidence suggesting a potential role of the β -secretasederived
APP C-terminal fragment C99 in Alzheimer’s disease etiology
Hamzah Al-Fansuri: A Figure of Malay-Indonesian Philosophical Mysticism and Sufi Literature
Authors: Harahap Khoirul Amru 
Published: March 2019

This paper discusses Hamzah al-Fansuri and his mysticsm philosophical thought and his sufi literature. His mysticism philosopical thought was very controversial that it raises debates in in his era. One of the hardest figure that attack his mysticism philosophical thought was Nuruddin ar-Raniri. This sunni’s mysticism figure considered al-Fansuri a deviate mysticism, zindiq and mulhid (heathen). Mysticism concept he practiced was the concept of wahdah al-wujud or known as wujudiyah concept, which is mostly affected by Ibnu ‘Arabi. Al-Fansuri’s Wujudiyah concept is a concept stating that wujud (existency) is essentially one, even though it seems a lot. All things that are seen a lot by the sense organ, actually just appearance of a form of existency, Allah. However, al-Fansuri separated it between intrinsic form and inherent form. Inherent form is actually nothing, it can be fana’ at every time, and it does not exist without an essential being. Even though he practiced wujudiyah concept, he is strongly refused ittihad concept (the united of the sufi with God) and hulul concept (God put a place from the body of someone). Meanwhile, his sufi literature has 6 characteristics: 1. He used authorship markers. 2. He quoted a lot of verses of Quran, hadith, and Arabic words. 3. He put his name and nick name in the end of his poetry ties. 4. He used imageries and metaphorical symbolic. 5. He was clever in joining diction with rhythm in a balanced way. 6. He was clever in joining metaphysics sense, logical and sufi aesthetic in his poems.