bluuebunny/crossref_metadata_2025_split huggingface dataset:
Disturbance of consciousness due to hyperammonemia and lactic acidosis during mFOLFOX6 regimen
Authors: Fukuda Masafumi , Nabeta Masakazu , Muta Takanori , Cho Tomonori , Shimamatsu Yutaka , Shimotsuura Yasutaka , Fukami Kei , Takasu Osamu 
Published: August 2020

Abstract

Introduction:
FOLFOX therapy is the main chemotherapy regimen for colorectal cancer. Peripheral neuropathy, hematotoxicity, and digestive symptoms are known to be the most frequent adverse events. Hyperammonemia and lactic acidosis rarely occur simultaneously during treatment with FOLFOX therapy; the number of case reports is limited worldwide. We report a case of disturbance of consciousness, considered to be caused by hyperammonemia and lactic acidosis that occurred during treatment with mFOLFOX6 therapy that was administered as postoperative adjuvant treatment for rectal cancer.

Patient concerns:
This case was of a 71-year-old man who had been receiving oral treatment for chronic kidney disease and diabetes mellitus. Laparoscopic low anterior resection and artificial anal construction surgery were performed for stage III rectal cancer. As adjuvant postoperative therapy, mFOLFOX6 therapy was started but was followed by a disturbance of consciousness.

Diagnoses:
Results of the blood tests revealed notable hyperammonemia (ammonia level, 1,163 μg/dl) and lactic acidosis (pH 7.207; lactate, 17.56 mmol/L); however, imaging diagnosis did not reveal intracranial lesions that could cause disturbance of consciousness.

Interventions:
For hyperammonemia, branched-chain amino acid agents and Ringers solution supplementation were administered. For acidosis, 7% sodium hydrogen carbonate was administered as treatment.

Outcomes:
The disturbance of consciousness improved within 12 hours of initiating the treatment, and the patient was discharged with no sequelae on 7th day after hospitalization.

Conclusion:
In patients with chronic kidney disease, FOLFOX regimen may confer risks of hyperammonemia and lactic acidosis.
Treatment in disproportionately minority hospitals is associated with an increased mortality in end-stage liver disease
Authors: Brahmania Mayur , Alotaibi Ammar , Mooney Owen , Rush Barret 
Published: August 2020

Background
Racial and ethnic disparities are a barrier in delivery of healthcare across the USA. Care for minority patients tends to be clustered into a small number of providers at minority hospitals, which has been associated with worse clinical outcomes in several conditions. However, the outcomes of treatment in patients with end-stage liver disease (ESLD) at predominately minority hospitals are unknown. We investigated the burden of the problem.

Methods
We utilized the nationwide in-patient sample (NIS) to conduct a retrospective nationwide cohort analysis. All patients >18 years of age admitted with ESLD were included in the analysis. A multivariate logistic regression model was used to study the mortality rate among patients with ESLD treated at minority hospitals compared to nonminority hospitals.

Results
A total of 53 281 467 hospitalizations from the 2008 to 2014 NIS were analyzed. There were 163 470 patients with ESLD that met inclusion criteria. In-hospital mortality rates for all races were 8.0 and 8.1% in black and Hispanic minority hospitals, respectively, compared to 7.3% in nonminority hospitals (P < 0.01). On multivariate analysis, treatment of ESLD in black and Hispanic minority hospitals was associated with 11% [odds ratio (OR), 1.11; 95% confidence interval (CI), 1.03–1.20; P < 0.01] and 22% (OR, 1.22; 95% CI, 1.09–1.37; P < 0.01) increased odds of death, respectively, compared to treatment in nonminority hospitals regardless of patient’s race.

Conclusion
Patients with ESLD treated at minority hospitals are faced with an increased mortality rate regardless of patient’s race. This study highlights another quality gap that needs improvement to affect overall survival among patients with ESLD.
The defining of the reference range of liver stiffness and fat attenuation parameter for healthy Chinese children
Authors: Zeng Fansen , Wang Haiyan , Li Xufang 
Published: August 2020

Aims
We aimed to establish the basal reference levels of liver stiffness and accumulated fat in healthy Chinese children.

Methods
To obtain the liver stiffness measurement (LSM) and the fat attenuation parameter (FAP) levels, the transient elastography (FibroTouch) was used in pediatric patients aged 0 to 18 years. The patients were divided into groups as follows: newborns/infants (0–2 years), preschool children (3–5 years), elementary school children (6–11 years), and adolescents/teenagers (12–18 years). The weight, height and fasting liver function tests were performed. FibroTouch and abdominal ultrasonography were performed.

Results
The livers of 521 out of 1362 children (329 male; median age, 4.6 years; age range 0.2–17.6 years) were examined with the FibroTouch. The LSM reference range was 1.5–5.9 kPa, and the FAP reference range was 159.6–217.1 dB/m in healthy children from 0 to 18 years of age. The median LSM value was higher in males than that in females (3.5 vs. 3.2 kPa, respectively; P = 0.01).

Conclusion
For healthy children from 0 to 18 years in southern China, the LSM reference range was 1.5–5.9 kPa, and the FAP reference range was 159.6–217.1 dB/m. The LSM values were age-dependent in children from 3 to 18 years old, and the FAP values were age-independent in children from 0 to18 years old.
Effectiveness of combined therapy radiofrequency ablation/transarterial chemoembolization versus transarterial chemoembolization/radiofrequency ablation on management of hepatocellular carcinoma
Authors: El dorry Ahmed Kaml , Shaker Mohammed Kamal , EL-Fouly Nevien Fouad , Hussien Ahmed , El-Folly Runia Fouad , El Fouly Amr Hafez , Abd El Tawab Karim 
Published: August 2020

Background
One of the most widespread cancer-associated death worldwide is Hepatocellular carcinoma. Concerning hepatic malignant tumor staging system known as Barcelona clinic of liver cancer, a superior curative response could be carried out by combined techniques [radiofrequency ablation (RFA) and transarterial chemoembolization (TACE)] for stage B comparing with TACE alone as palliative monotherapy.

Objectives
To discuss the merging effect of RFA followed by TACE and vice versa on objective response, overall survival, local recurrence and tumor-free survival.

Patients and methods
Sixty-eight cases included with hepatic tumor on top of chronic liver disease post-viral infection and divided into two groups according to different combined treatment modality; first cohort included 34 patients treated with TACE followed by RFA, while the second one included 34 patients treated with RFA followed by TACE for two lesions or single medium-sized lesion stage.

Results
Complete response and objective response rates were 91% and 82% after TACE/RFA and 100% and 91% after RFA/TACE, respectively. Regarding Milan criteria, there was significant downstaging after RFA/TACE (P < 0.05). First and second overall survival rates were 85% and 65% after TACE/RFA versus 100% and 74%, respectively, after RFA/TACE (P > 0.05). Kaplan–Meier curve as regards disease-free survival rate, median time were 17.1 months [95% confidence interval (CI) 12.2–22.0] in TACE/RFA and 23.2 (95% CI 18.1–28.2) months in RFA/TACE (P > 0.05).

Conclusion
RFA/TACE showed effective complete response, downstaging, disease-free survival and overall survival for the treatment of hepatic malignant tumors.
The value of 99mTc-labeled galactosyl human serum albumin single-photon emission computerized tomography/computed tomography on regional liver function assessment and posthepatectomy failure prediction in patients with hilar cholangiocarcinoma
Authors: Huang Xin , Chen Yingmao , Shao Mingzhe , Li Can , Zhang Aiqun , Dong Jiahong , Xiang Canhong 
Published: August 2020

Background
The aim was to evaluate the value of 99mTc-labeled galactosyl human serum albumin (99mTc-GSA) with single-photon emission computerized tomography (SPECT) in the preoperative assessment of regional liver function and prediction of posthepatectomy liver failure (PHLF) in patients with hilar cholangiocarcinoma (hCCA).

Methods
Patients with hCCA who underwent 99mTc-GSA SPECT/computed tomography (CT) before hepatectomy were included. The liver functional parameters of functional liver density (FLD) and predictive residual index (PRI) were calculated based on 99mTc-GSA SPECT/CT. PHLF was defined according to the International Study Group of Liver Surgery criteria. Univariate and multivariate analyses were used to analyze the risk factors for PHLF. The prediction of PHLF was calculated using receiver operating characteristic curve.

Results
A total of 34 patients were included, 23 of whom underwent preoperative biliary drainage. FLD was significantly higher in patients with drained lobes than that in patients with undrained lobes (0.615 ± 0.190 versus 0.500 ± 0.211, P < 0.05). Sixteen patients suffered PHLF. The ratio of future remnant to total morphological liver volume, future remnant FLD, and PRI differed significantly in patients with and without PHLF according to univariate analysis. PRI was identified as the only independent factor for prediction of PHLF according to multivariate analysis. With a PRI of 0.78, it was possible to predict PHLF with a sensitivity of 83% and a specificity of 93%.

Conclusions

99mTc-GSA SPECT/CT can accurately assess regional liver function and is better able to predict PHLF than conventional methods in patients with hCCA.
68Ga-DOTATATE and 123I-mIBG as imaging biomarkers of disease localisation in metastatic neuroblastoma: implications for molecular radiotherapy
Authors: Gains Jennifer E. , Aldridge Matthew D. , Mattoli Maria Vittoria , Bomanji Jamshed B. , Biassoni Lorenzo , Shankar Ananth , Gaze Mark N. 
Published: August 2020

Purpose
Iodine-131-labelled meta-iodobenzylguanidine (131I-mIBG) and lutetium-177-labelled DOTATATE (177Lu-DOTATATE) are used for molecular radiotherapy of metastatic neuroblastoma. These are taken up by the noradrenaline transporter (NAT) and the somatostatin receptor subtype 2 (SSTR-2), respectively. Scintigraphy of iodine-123-labelled meta-iodobenzylguanidine (123I-mIBG) and gallium-68 DOTATATE (68Ga-DOTATATE) PET are used to select patients for therapy. These demonstrate the extent and location of tumour, and avidity of uptake by cells expressing NAT and SSTR-2, respectively. This study compared the similarities and differences in the anatomical distribution of these two imaging biomarkers in an unselected series of patients with metastatic neuroblastoma undergoing assessment for molecular radiotherapy.

Methods
Paired whole-body planar 123I-mIBG views and 68Ga-DOTATATE maximum intensity projection PET scans of metastatic neuroblastoma patients were visually compared. The disease extent was assessed by a semiquantitative scoring method.

Results
Paired scans from 42 patients were reviewed. 68Ga-DOTATATE scans were positive in all patients, 123I-mIBG scans were negative in two. In two patients, there was a mismatch, with some lesions identified only on the 123I-mIBG scan, and others visible only on the 68Ga-DOTATATE scan.

Conclusion

68Ga-DOTATATE and 123I-mIBG scans yield complementary information. For a more comprehensive assessment, consideration could be given to the use of both 123I-mIBG and 68Ga-DOTATATE imaging scans. Because of the heterogeneity of distribution of molecular targets revealed by these techniques, a combination of both 131I-mIBG and 177Lu-DOTATATE molecular radiotherapy may possibly be more effective than either alone.
18F-FDG PET/CT for primary staging of patients with testicular germ cell tumors: the predictors of 18F-FDG PET positivity and prognostic value of PET derived metabolic parameters
Authors: Aydos Uğuray , Tahtaci Gözde , Özgür Akdemir Ümit , Özet Ahmet 
Published: August 2020

Purpose
The aim of this study was to investigate the role of histopathologic and biochemical parameters for predicting 18F-FDG PET positivity for primary staging in patients with newly diagnosed testicular germ cell tumors (TGCT). It was also aimed to evaluate the prognostic value of PET derived metabolic features in this patient group.

Materials and methods
The imaging findings of 51 patients who were diagnosed as TGCT and underwent 18F-FDG PET/CT for primary staging after surgery between 2009 and 2019 were evaluated retrospectively. In terms of the presence of 18F-FDG uptake consistent with metastasis, the patients were divided into two groups as ‘PET-positive’ and ‘PET-negative’. In ‘PET-positive’ patients, highest maximum standardized uptake values of metastatic lesions, whole-body total metabolic tumor volumes (wb-TMTV) and total lesion glycolysis (wb-TLG) were extracted. Pathological tumor types, pathological T stages (pT), tumor marker (TM) levels (AFP, hCG, LDH) after orchiectomy and overall survival (OS) times in months were also recorded. The predictive value of variables for OS was evaluated using the Kaplan-Meier survival analysis.

Results
PET positivity was observed in 28% of pT1 and in 83.3% in pT2-T3 tumors (P < 0.001). In the logistic regression analysis to predict ‘PET positivity’, pT stage and ≥2 elevated TMs were found as independent significant predictors. Survival analysis demonstrated that wb-TMTV, wb-TLG and TMs were significantly associated with prognosis for OS.

Conclusion
The likelihood of PET positivity was significantly higher in patients with elevated TMs and pT2-T3 stages compared to those with normal TMs and pT1 tumors. PET derived quantitative metabolic-volumetric parameters can be used as biomarkers to identify patients with poor prognosis in TGCT.
Preoperative detection of sentinel lymph nodes with hybrid SPECT/computed tomography imaging may improve the accuracy of sentinel lymph node biopsies in patients with early stages of cancer of the oesophagus or gastro-oesophageal junction
Authors: Gabrielson Stefan , Tsai Jon A. , Celebioglu Fuat , Nilsson Magnus , Rouvelas Ioannis , Lindblad Mats , Bjäreback Annie , Tomson Artur , Axelsson Rimma 
Published: August 2020

Objectives
The aim of this study was to investigate the sentinel lymph node biopsy (SLNB) method in patients with cancer of the oesophagus or gastro-oesophageal junction (GOJ) guided by preoperative hybrid single-photon emission tomography/computed tomography (SPECT/CT) lymphoscintigraphy.

Methods
Thirty-nine patients with stage T1–T3, any N-stage, M0 cancer of the oesophagus or GOJ planned for curatively intended esophagectomy underwent preoperative SPECT/CT lymphoscintigraphy following endoscopically guided submucosal injection of radiocolloid and intraoperative radio-guided SLNB using a hand-held gamma scintillation device.

Results
The detection rate in preoperative SPECT/CT imaging was 88%. The median number of detected SLN stations in preoperative imaging was 1 (range 0–4). At least one suspected SLN was identified in all intraoperative SLNP procedures. In six cases, no lymph nodes were identified in the SLNB. In six cases, the SLNB was false negative. The sensitivity for successful SLNB procedures was 20%, the specificity was 100% and the accuracy was 75%.

Conclusions
Preoperative SLN mapping using SPECT/CT yields a high number of detected SLN stations compared to previous studies using planar imaging. The accuracy of the SLNB method in patients with predominantly ≥T3-stage tumours and with a history of previous neoadjuvant treatment is poor, and the method is not recommended in these patient groups.
Polyp Progression in Paediatric Patients With Familial Adenomatous Polyposis: A Single-centre Experience
Authors: Anele Chukwuemeka C. , Xiang Jinpo , Martin Isabel , Hawkins Menna , Clark Susan K. , Faiz Omar D. , Latchford Andrew , Hyer Warren 
Published: August 2020

ABSTRACT

Objectives:
Prophylactic colectomy at a premalignant stage is the cornerstone of management of familial adenomatous polyposis (FAP). Before surgery, colonoscopy surveillance is recommended in children with FAP. This study aimed to examine the natural history of FAP in children by evaluating adenoma progression and factors influencing timing of colectomy.
Method: Patients with FAP younger than 18 years at first surveillance colonoscopy and who had undergone more than 1 colonoscopy were identified. Demographic, endoscopic, genetic, and surgical data were retrieved. Cumulative adenoma (polyp) counts were obtained while accounting for any polypectomies during the study period. The rate of polyp progression and factors influencing the timing of colectomy were evaluated.

Results:
Eighty-four patients (50% boys; mean age at first colonoscopy 13 years [standard deviation 1.97]) were identified, of which 83 had a family history of FAP. At first colonoscopy, 67 (79%) had <100 adenomas and 29 (35%) had colonic polyps identified despite rectal sparing. The median rate of polyp progression per patient was 12.5 polyps/year (range 0–145). Of the 45 (54%) patients who had undergone surgery, 41 (91%) underwent colectomy with ileorectal or ileodistal sigmoid anastomosis. Polyp progression did not alter the choice of surgical intervention in any patient.

Conclusion:
Our results suggest that adenoma number remains relatively stable in the majority of children under surveillance. Tailored surveillance intervals according to phenotype are a more appropriate strategy as recommended by recently published guidelines.
Abnormal Bilirubin Metabolism in Patients With Sodium Taurocholate Cotransporting Polypeptide Deficiency
Authors: Yan Yan Yan , Wang Meng Xuan , Gong Jing Yu , Liu Lang Li , Setchell Kenneth D.R. , Xie Xin Bao , Wang Neng Li , Li Wenhui , Wang Jian-She 
Published: August 2020

ABSTRACT

Objectives:
The aim of the study was to explore the significance of sodium taurocholate cotransporting polypeptide (NTCP) deficiency and its clinical features in Chinese children presenting with isolated persistent hypercholanemia.

Methods:
The exon and adjacent regions of SLC10A1, the gene encoding NTCP, were sequenced in 33 Chinese children presenting with isolated hypercholanemia. Clinical history and medical data were reviewed. Growth milestones were compared with the national standard. The serum direct bilirubin concentration at last follow-up was compared with age- and sex-matched controls.

Results:
A variant, c.800C>T, p. S267F of SLC10A1 was detected in all subjects; 30 patients were homozygotes and 3 were compound heterozygotes. Nine patients presented with transient neonatal cholestasis, and 1 with a persistent mild conjugated hyperbilirubinemia. The serum direct bilirubin level in NTCP-deficient patients was significantly higher than age- and sex-matched controls even after the neonatal cholestasis stage (2.85 ± 1.50 vs 1.49 ± 0.70 μmol/L, P
= 0.00008). No growth delay or other severe long-term clinical consequences were observed.

Conclusions:
NTCP deficiency is the exclusive or major cause of isolated hypercholanemia in Han Chinese children, with c.800C>T the major contributing genetic variation. The defect may affect bilirubin metabolism and present as transient neonatal cholestasis and/or persistent mild conjugated hyperbilirubinmia, but with no apparent long-term clinical consequences.
The Role of Cannabinoid Receptor Type 2 in the Bone Loss Associated With Pediatric Celiac Disease
Authors: Tortora Chiara , Punzo Francesca , Argenziano Maura , Di Paola Alessandra , Tolone Carlo , Strisciuglio Caterina , Rossi Francesca 
Published: August 2020

ABSTRACTObjectives:In this study, we investigated the role of the cannabinoid receptor type 2 (CB2) in the bone loss associated with celiac disease (CD) evaluating the effect of its pharmacological modulation on osteoclast activity. We previously demonstrated a significant association between the CB2 Q63R variant and CD, suggesting it as a possible disease biomarker. Moreover, CB2 stimulation is beneficial for reducing osteoclast activity in several bone pathologic conditions.Methods:In vitro osteoclasts(OCs) were differentiated from peripheral blood mononuclear cells of healthy donors, CD children at diagnosis and after 1 year of gluten-free diet (GFD) and characterized by real-time PCR and western blot for the expression of CB2 and specific osteoclastic markers, TRAP and Cathepsin K. TRAP assay and Bone Resorption assay were performed to evaluate osteoclast activity before and after 48 h exposure to CB2 selective drugs (JWH-133 and AM630) and Vitamin D.Results:We found in CD patients an osteoclast hyperactivation and low levels of CB2. CB2 stimulation with JWH-133 agonist is more effective than Vitamin D in reducing osteoclast activity whereas CB2 blockade with AM630 increases osteoclast activation. The anti-osteoporotic effect of JWH-133 decreases when used in co-treatment with vitamin D. GFD reduces osteoclast activity without restore CB2 expression.Conclusions:CB2 could be a molecular marker to predict the risk of bone alterations in CD and a pharmacological target to reduce bone mass loss in patients who need a direct intervention on bone metabolism, in addition to the GFD.
Activated CD8 T‐cell Hepatitis in Children With Indeterminate Acute Liver Failure
Authors: Chapin Catherine A. , Melin‐Aldana Hector , Kreiger Portia A. , Burn Thomas , Neighbors Katie , Taylor Sarah A. , Ostilla Lorena , Wechsler Joshua B. , Horslen Simon P. , Leonis Mike A. , Loomes Kathleen M. , Behrens Edward M. , Squires Robert H. , Alonso Estella M. ,   Pediatric Acute Liver Failure Study Group
Published: August 2020

ABSTRACTObjectives:In many pediatric acute liver failure (PALF) cases, a diagnosis is not identified, and the etiology is indeterminate (IND‐PALF). Our pilot study found dense CD8 T‐cell infiltrates and increased T‐cell clonality in liver specimens from IND‐PALF patients. We aimed to validate these findings in a multicenter cohort with investigators blinded to diagnosis.Methods:PALF Study Group registry subjects with IND‐PALF (n = 37) and known diagnoses (DX‐PALF) (n = 18), ages 1 to 17 years, with archived liver tissue were included. Liver tissue slides were stained for T cells (CD8 and CD4), B cells (CD20), macrophages (CD163), perforin, and tissue resident‐memory T cells (Trm, CD103), and scored as minimal, moderate, or dense. Lymphocytes were isolated from frozen liver tissue for T‐cell receptor beta (TCRβ) sequencing.Results:Dense hepatic CD8 staining was found in significantly more IND‐PALF (n = 29, 78%) compared with DX‐PALF subjects (n = 5, 28%) (P = 0.001). IND‐PALF subjects were more likely to have dense or moderate perforin (88% vs 50%, P = 0.03) and CD103 (82% vs 40%, P = 0.02) staining compared with DX‐PALF subjects. TCRβ sequencing of 15 IND‐PALF cases demonstrated increased clonal overlap compared with 6 DX‐PALF cases (P = 0.002).Conclusions:Dense infiltration of effector Trm CD8 T cells characterizes liver tissue from IND‐PALF subjects. Increased clonality suggests the T‐cell expansion is antigen(s)‐driven as opposed to a nonspecific inflammatory response. These findings support CD8 staining as a new biomarker of the activated CD8 T‐cell PALF phenotype. Future studies are needed to characterize potential antigens, host risk factors, and inflammatory pathways with the goal of developing targeted therapies.