Database : MEDLINE
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[PMID]: 29507150
[Ti] Title:Correction for Wani et al., Familial hypercatabolic hypoproteinemia caused by deficiency of the neonatal Fc receptor, FcRn, due to a mutant ß -microglobulin gene.
[So] Source:Proc Natl Acad Sci U S A;103(27):10526, 2006 Jul 05.
[Is] ISSN:1091-6490
[Cp] Country of publication:United States
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE; PUBLISHED ERRATUM
[Em] Entry month:1803
[Cu] Class update date: 180311
[Lr] Last revision date:180311
[St] Status:In-Data-Review
[do] DOI:10.1073/pnas.0604332103

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[PMID]: 29474904
[Au] Autor:Khalil SR; Mohammed AT; Abd El-Fattah AH; Zaglool AW
[Ad] Address:Forensic Medicine and Toxicology Department, Faculty of Veterinary Medicine, Zagazig University, Egypt. Electronic address: resamah@zu.edu.eg.
[Ti] Title:Intermediate filament protein expression pattern and inflammatory response changes in kidneys of rats receiving doxorubicin chemotherapy and quercetin.
[So] Source:Toxicol Lett;288:89-98, 2018 Feb 21.
[Is] ISSN:1879-3169
[Cp] Country of publication:Netherlands
[La] Language:eng
[Ab] Abstract:The aim of this study was to explore the potential effects of quercetin (QUR) on doxorubicin (DOX)-induced nephrotoxicity. Fifty male rats were assigned to five groups (10 rats each): a control group, a DOX-treated group (total dose, 15 mg/kg bw, intraperitoneally), a QUR-treated group (50 mg/kg bw/day, orally), a prophylaxis co-treated group, and a therapeutic co-treated group. Biochemical parameters and renal function were measured. Moreover, kidney tissues were homogenized for inflammatory marker evaluation and real-time qPCR analysis to determine the changes in intermediate filament protein mRNA levels (desmin, vimentin, connexin 43 and nestin). QUR exhibited a significant nephroprotective effect, particularly when it was administered prior to and simultaneously with DOX treatment (prophylaxis co-treated group). This role was biochemically demonstrated by the significant modulation of DOX-induced body weight loss, hypoproteinemia, and elevated serum creatinine and urea. Moreover, QUR attenuated the inflammatory response as shown by decreased renal nitric oxide, tumor necrosis factor-α production and myeloperoxidase activity elicited by DOX injection. These biochemical improvements were accompanied by a significant histopathological restoration of rat kidney tissue and successful down-regulation of the intermediate filament protein mRNA levels, indicating amelioration of DOX-induced podocyte injury. Taken together, these results conclusively demonstrated that QUR administration has a prophylactic effect on DOX-induced injury in the rat kidney.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180309
[Lr] Last revision date:180309
[St] Status:Publisher

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[PMID]: 29517436
[Au] Autor:O'Connor MR; Garner MM
[Ti] Title:IRON STORAGE DISEASE IN AFRICAN GREY PARROTS ( PSITTACUS ERITHACUS) EXPOSED TO A CARNIVOROUS DIET.
[So] Source:J Zoo Wildl Med;49(1):172-177, 2018 Mar.
[Is] ISSN:1042-7260
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Within a 2-wk period, three African grey parrots ( Psittacus erithacus) presented for emergency treatment. All three parrots had depressed behavior, an inability to fly, and significant weight loss. Plasma chemistry abnormalities included severe hypoproteinemia and elevated liver enzymes in all parrots. Two of the parrots died, and histologic examination with hematoxylin and eosin and Prussian blue stains revealed severe hepatic iron storage. Quantitative analysis confirmed high hepatic iron concentrations. Iron accumulation was attributed to ingestion of a carnivorous bird diet or selectively eating too much fruit and vegetables high in ascorbic acid. Management entailed husbandry changes including switching the remaining parrots to a low-iron diet. Psittacine species exposed to carnivorous bird diets are at risk of developing iron storage disease.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1803
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Data-Review
[do] DOI:10.1638/2016-0266R1.1

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[PMID]: 29187749
[Au] Autor:Gómez-Hoyos E; Fernández-Peña S; Cuesta M; Ortolá A; Matía P; Pérez-Ferre N; De Luis D; Calle-Pascual A; Rubio MÁ; Runkle-De la Vega I
[Ad] Address:Endocrinology and Nutrition Department, Pharmacy Department, Hospital Clínico Universitario de Valladolid-IEN, Facultad de Medicina Universidad de Valladolid, Valladolid, Spain. emiliagomezhoyos@gmail.com.
[Ti] Title:Hyponatremia in patients receiving parenteral nutrition: the importance of correcting serum sodium for total proteins. The role of the composition of parenteral nutrition in the development of hyponatremia.
[So] Source:Eur J Clin Nutr;72(3):446-451, 2018 Mar.
[Is] ISSN:1476-5640
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:BACKGROUND/OBJECTIVES: Hyponatremia is the most common electrolyte disorder, and is associated with high-morbimortality rates. The true prevalence of hyponatremia in patients on parenteral nutrition (PN) is unknown, and the relationship between PN composition and development of hyponatremia has yet to be studied. Hypoproteinemia, a common finding in patients receiving PN, induces an overestimation of serum sodium (SNa) levels, when using indirect electrolyte methodology. Thus, SNa should be corrected for serum total protein levels (TP). The objective was to accurately determine the prevalence of hyponatremia (indirect SNa corrected for PT) and evaluate the relationship between the composition of PN and the development of hyponatremia. SUBJECTS/METHODS: Medical records of 222 hospitalized patients receiving total PN during a 7-month period were reviewed. Composition of PN, indirect SNa-mmol/l-, and SNa corrected for TP (SNa-TP)-mmol/l-, both upon initiation and during PN administration, were analyzed. RESULTS: Hyponatremia (SNa < 135 mmol/l) was present in 81% of subjects when SNa was corrected for TP, vs. 43% without correction (p = 0.001). In total 64% of patients that were eunatremic upon initiation of PN developed hyponatremia during PN administration, as detected by SNa-TP, vs. 28% as detected by uncorrected SNa (p < 0.001). There were no significant differences in volume, osmolarity, sodium or total osmols administered in PN between patients who developed hyponatremia and those who remained eunatremic. CONCLUSIONS: A majority of patients receiving PN present hyponatremia, when indirect SNa levels are corrected for TP. The development of hyponatremia during PN is not related to the composition of the PN.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 180308
[Lr] Last revision date:180308
[St] Status:In-Data-Review
[do] DOI:10.1038/s41430-017-0026-5

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[PMID]: 29465563
[Au] Autor:Marginean CO; Melit LE; Horvath E; Gozar H; Chincesan MI
[Ad] Address:Department of Pediatrics I.
[Ti] Title:Non-Hodgkin lymphoma, diagnostic, and prognostic particularities in children - a series of case reports and a review of the literature (CARE compliant).
[So] Source:Medicine (Baltimore);97(8):e9802, 2018 Feb.
[Is] ISSN:1536-5964
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:RATIONALE: Non-Hodgkin lymphoma remains an unpredictable condition in pediatric patients. PATIENT CONCERNS: Our first case describes an 8-year-old boy with a history of iron deficiency anemia, admitted in our clinic for recurrent abdominal pain, weight loss, loss of appetite, diarrheic stools, and fever. The second case also describes an 8-year-old boy admitted for abdominal pain and vomiting. The 3rd case refers to a 4 years and 10 months old boy admitted in our clinic with abdominal pain and loss of appetite, who was initially admitted in the Pediatrics Surgery Clinic with the suspicion of appendicitis. Our 4th patient was a 5-year-old boy admitted in our clinic for abdominal pain and intermittent diarrheic stools. DIAGNOSES: In the first case, the laboratory tests showed anemia, thrombocytosis, elevated inflammatory biomarkers, a low level of iron, and hypoproteinemia. The abdominal ultrasound and CT exam revealed an abdominal mass, and the histopathological exam established the diagnosis of diffuse large B-cell lymphoma of the bowel. In the second case, the laboratory tests pointed out anemia, elevated ESR and lactate dehydrogenase level, while both abdominal ultrasound and CT exams showed an abdominal mass. The histopathological exam confirmed the diagnosis of Burkitt lymphoma. Regarding our 3rd case, the laboratory findings revealed leukocytosis, anemia, thrombocytosis, increased inflammatory biomarkers, elevated LDH, and a low level of iron. The abdominal ultrasound and the CT scan revealed an abdominal mass which, according to the histopathological exam, was a Burkitt lymphoma. Due to the cranial CT findings the patient was diagnosed with IV stage Burkitt lymphoma with central nervous system metastases. In our 4th patients we found leukocytosis, anemia, mildly increased inflammatory biomarkers, a high level of LDH, hypoproteinemia, and a low level of serum Ir. Both ultrasound and abdominal CT exams were negative, but the exploratory laparotomy identified an abdominal mass, and according to the histopathological exam the patient was diagnosed with Burkitt lymphoma. INTERVENTIONS: All the patients followed chemotherapy (B-NHL BFM 04 protocol) and supportive treatment. OUTCOMES: The first patient died approximately 4 months after the completion of chemotherapy due to tumor relapse, the second patient died after the first cure of chemotherapy and the fourth patient died at approximately 2 years after the diagnosis. The third patient is recurrence-free after 2 years. LESSONS: Despite the advances in the management, NHL remains a fatal condition in pediatrics.
[Mh] MeSH terms primary: Lymphoma, Non-Hodgkin/diagnosis
[Mh] MeSH terms secundary: Abdominal Pain/etiology
Anemia, Iron-Deficiency/etiology
Antineoplastic Combined Chemotherapy Protocols/therapeutic use
Asparaginase/therapeutic use
Child
Child, Preschool
Daunorubicin/therapeutic use
Diarrhea/etiology
Fatal Outcome
Humans
Lymphoma, Non-Hodgkin/complications
Lymphoma, Non-Hodgkin/drug therapy
Male
Prednisone/therapeutic use
Prognosis
Treatment Outcome
Vincristine/therapeutic use
Vomiting/etiology
Weight Loss
[Pt] Publication type:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Nm] Name of substance:5J49Q6B70F (Vincristine); EC 3.5.1.1 (Asparaginase); VB0R961HZT (Prednisone); ZS7284E0ZP (Daunorubicin)
[Em] Entry month:1802
[Cu] Class update date: 180227
[Lr] Last revision date:180227
[Js] Journal subset:AIM; IM
[Da] Date of entry for processing:180222
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000009802

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[PMID]: 29293605
[Au] Autor:Ha YJ; Hur J; Go DJ; Kang EH; Park JK; Lee EY; Shin K; Lee EB; Song YW; Lee YJ
[Ad] Address:Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Republic of Korea.
[Ti] Title:Baseline peripheral blood neutrophil-to-lymphocyte ratio could predict survival in patients with adult polymyositis and dermatomyositis: A retrospective observational study.
[So] Source:PLoS One;13(1):e0190411, 2018.
[Is] ISSN:1932-6203
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Recent studies have suggested that neutrophil-to-lymphocyte ratio (NLR) and C-reactive protein-to-albumin ratio (CAR) are emerging markers of disease activity and prognosis in patients with chronic inflammatory diseases, cardiovascular diseases, or malignancies. Therefore, we investigated the clinical significance and prognostic value of the NLR and CAR in adult patients with polymyositis and dermatomyositis. The medical records of 197 patients with newly diagnosed polymyositis/dermatomyositis between August 2003 and November 2016 were retrospectively reviewed. Survival and causes of death were recorded during an average 33-month observational period. Clinical and laboratory findings were compared between survivors and non-survivors. Using receiver operating characteristic curves, the NLR and CAR cut-off values for predicting survival were calculated. Univariate and multivariate analyses using Cox proportional hazard models were performed to identify factors associated with survival. Twenty-six patients (13.2%) died during the study period, and the 5-year survival-rate was estimated to be 82%. The non-survivor group exhibited older age and a higher prevalence of interstitial lung disease (ILD), acute interstitial pneumonia, and acute exacerbation of ILD compared to that in the survivor group. NLR and CAR values were significantly higher in the non-survivors and in patients with polymyositis/dermatomyositis-associated ILD, and the death rates increased across NLR and CAR quartiles. Furthermore, when stratified according to the NLR or CAR optimal cut-off values, patients with a high NLR (>4.775) or high CAR (>0.0735) had a significantly lower survival rate than patients with low NLR or CAR, respectively. In addition, old age (>50 years), the presence of acute interstitial pneumonia, hypoproteinemia (serum protein <5.5 g/dL), and high NLR (but not high CAR) were independent predictors for mortality. The results indicate that a high NLR is independently associated with worse overall survival. Thus, the baseline NLR level may be a simple, cost-effective prognostic marker in patients with polymyositis/dermatomyositis.
[Mh] MeSH terms primary: Dermatomyositis/blood
Lymphocytes/pathology
Neutrophils/pathology
Polymyositis/blood
[Mh] MeSH terms secundary: Adult
Dermatomyositis/pathology
Female
Humans
Male
Polymyositis/pathology
Retrospective Studies
Survival Analysis
[Pt] Publication type:JOURNAL ARTICLE; OBSERVATIONAL STUDY
[Em] Entry month:1802
[Cu] Class update date: 180221
[Lr] Last revision date:180221
[Js] Journal subset:IM
[Da] Date of entry for processing:180103
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0190411

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[PMID]: 29411151
[Au] Autor:Hardiansyah D; Ng CM
[Ad] Address:Department of Pharmaceutical Sciences, College of Pharmacy, University of Kentucky, BioPharm Building, Room 341, 789 S. Limestone, Lexington, Kentucky, 40536, USA.
[Ti] Title:Two-Pore Minimum Physiologically-based Pharmacokinetic Model to Describe the Disposition of Therapeutic Monoclonal IgG Antibody in Humans.
[So] Source:Pharm Res;35(3):47, 2018 Feb 06.
[Is] ISSN:1573-904X
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:PURPOSE: The aim of this study was to develop a two-pore minimum physiologically-based pharmacokinetic (mPBPK) model in describing the pharmacokinetic (PK) of therapeutic monoclonal antibody (TMAb) in human subjects. METHODS: PK data used in this study were endogenous/exogenous native IgG and two TMAbs (palivizumab and Motavizumab-YTE) in normal volunteer or familial hypercatabolic hypoproteinemia (FIHH) patient. Several important components were implemented to overcome the limitations of the early mPBPK model, e.g. two-pore model to describe the transcapillary transport of IgG from vascular to interstitial space. Six mPBPK models with different osmotic reflection coefficient (OFC) of transcapillary transport, endocytosis rates (ETR) and plasma clearance for the TMAbs/IgG were tested and the best model was selected using AICc values. RESULTS: The final model consisted of different OFC and ETR values for native IgG and TMAbs, supporting the hypothesis that the dynamics in the endosomal space had an important role in the compliant FcRn salvage mechanism to determine the clearance of TMAbs. The estimated FcRn concentration of FIHH subjects was 2.72 µmol/l. The final two-pore mPBPK model has a better performance for native IgG than previously developed mPBPK model. CONCLUSIONS: The final two-pore mPBPK model not only overcome the limitations of the early mPBPK model but also has a better performance to describe the disposition of the IgG antibody in human subjects.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180209
[Lr] Last revision date:180209
[St] Status:In-Data-Review
[do] DOI:10.1007/s11095-017-2292-2

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[PMID]: 29394556
[Au] Autor:Hiraki S; Kanesada K; Harada T; Tada K; Fukuda S
[Ad] Address:Dept. of Surgery, UBE Industries, LTD. Central Hospital.
[Ti] Title:[A Case of Huge Colon Cancer Accompanied with Severe Hypoproteinemia].
[So] Source:Gan To Kagaku Ryoho;44(12):1129-1131, 2017 Nov.
[Is] ISSN:0385-0684
[Cp] Country of publication:Japan
[La] Language:jpn
[Ab] Abstract:We report a case of huge colon cancer accompanied with severe hypoproteinemia. A7 4-year-old woman was referred to our hospital because of abdominal fullness. Blood examinations revealed anemia(hemoglobin 8.8 g/dL)and sever hypopro- teinemia(total protein 4.5 g/dL, albumin 1.1 g/dL). Computed tomography examination of abdomen revealed ascites and large tumor(12.5×10.5 cm)at the right side colon. By further examinations ascending colon cancer without distant metastasis was diagnosed, then we performed right hemicolectomy and primary intestinal anastomosis by open surgery. Ahuge type 1 tumor(18×12 cm)was observed in the excised specimen, which invaded to terminal ileum directly. The tumor was diagnosed moderately differentiated adenocarcinoma without lymph node metastasis(pT3N0M0, fStage II ). Postoperative course was uneventful and serum protein concentration recovered gradually to normal range. Protein leakage from the tumor cannot be proved by this case, so we can't diagnose as protein-losing enteropathy, but we strongly doubt this etiology from postoperative course in this case.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180203
[Lr] Last revision date:180203
[St] Status:In-Data-Review

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[PMID]: 29342997
[Au] Autor:Xu LJ; Luo YY; Yu JD; Lou JG; Fang YH; Chen J
[Ad] Address:Department of Gastroenterology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang 310003, China.
[Ti] Title:[X-linked inhibitor of apoptosis deficiency manifested as Crohn's disease: a case report and literature review].
[So] Source:Zhonghua Er Ke Za Zhi;56(1):43-47, 2018 Jan 02.
[Is] ISSN:0578-1310
[Cp] Country of publication:China
[La] Language:chi
[Ab] Abstract:To analyze the clinical characteristics of X-linked inhibitor of apoptosis (XIAP) deficient patients with clinical manifestation of Crohn's disease. Clinical manifestations, laboratory investigations, genetic testing and therapeutic interventions of one case of XIAP deficiency who was admitted to Department of Gastroenterology in Children's Hospital, Zhejiang University School of Medicine in May 2016 were summarized. PubMed and Chinese database for articles published from January 2016 to June 2017 were searched using the key words of'Crohn's disease'and'XIAP', and the relevant literature was reviewed. The case we reported was a 6-year-1-month-old boy with recurrent bloody stool for 2 months, and abdominal pain with fever for 2 weeks. The patient had a past history of hemophagocytic lymphohistiocytosis (HLH) and epilepsy in the past one year. Complete blood cell count showed mild anemia (Hb108 g/L). The patient had an elevated high-sensitivity C reactive protein (86 mg/L) and erythrocyte sedimentation rate (46 mm/1h) . White blood cells, pus cells and red blood cells were found on routine stool examination. Biochemical panel showed hypoalbuminemia (25.2 g/L) , elevated transaminase (alanine aminotransferase 175 U/L, aspartate transaminase 229 U/L) , hypertriglyceridemia (4.41 mmol/L) , and hyperferritinemia (>1 650.0 µg/L) . Magnetic resonance enterography revealed the intestinal wall thickening and increased enhancement in parts of illeum and colon. Capsule endoscopy revealed multiple ulcers in jejunum. Colonoscopy showed multiple ulcers in colon and the pathological examination revealed chronic inflammation in mucosa of terminal ileum and colon, which was combined with partial necrosis and ulceration. Some phagocytes were seen in bone marrow smears. The patient was given multiple diagnoses, including hemophagocytic lymphohistiocytosis, Crohn's disease, sepsis, epilepsy, severe malnutrition, and hypoproteinemia. The pediatric Crohn's disease activity index (PCDAI) was 37.5. Genetic testing identified a hemizygotic mutation of c.910G>T chrX:123022501 p.G304X in XIAP. The parents had no such mutation. The patient showed response to infliximab with oral intake of mercaptopurine and corticosteroids, and had remission with PCDAI of 0. There were 9 relevant articles (Chinese 0 English 9), which showed 33.3% XIAP deficient patients manifested with inflammatory bowel disease(IBD), who might have other manifestations such as hemophagocytic lymphohistiocytosis or splenomegaly simultaneously or sequentially. Those patients showed poor response to monotherapy. XIAP deficient patients have various clinical manifestations. Genetic testing is important to those male pediatric IBD patients who have the complicated symptoms or little response to standard therapy.
[Pt] Publication type:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180118
[Lr] Last revision date:180118
[St] Status:In-Process
[do] DOI:10.3760/cma.j.issn.0578-1310.2018.01.011

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[PMID]: 29339590
[Au] Autor:Ciorogar G; Bartos A; Bartos D; Vesa SC; Pop M; Herdean A; Betea I; Ciorogar A; Mois E; Zaharie F; Iancu C
[Ti] Title:Rectal cancer: factors predicting short outcomes after radical anterior resection.
[So] Source:Ann Ital Chir;88:505-513, 2017.
[Is] ISSN:2239-253X
[Cp] Country of publication:Italy
[La] Language:eng
[Ab] Abstract:AIM: This study analyzes risk factors implicated in postoperative complications and mortality after anterior resection in rectal cancer. MATERIAL AND METHODS: A total number of 378 patients with anterior rectal resection, diagnosed with rectal cancer and admitted at the IIIrd Surgery Clinic, "Octavian Fodor" Regional Institute of Gastroenterology and Hepatology, Romania, between 2009 and 2016. The inclusion criteria were anterior rectal resections with curative visa for rectal cancer. The complications we assessed are the following: anastomotic fistula, intra-abdominal infections, postoperative bowel obstruction and wound infection. RESULTS: There was statistical significance regarding male gender, emergency hospitalization, hypoproteinemia and the resumption of intestinal transit. Anterior rectal resection of tumors located on the middle rectum was associated with high rate of anastomotic fistula. Patients with manual suture of anastomosis developed intraabdominal abscess more frequently. In the multivariate analysis, hypoproteinemia and a number of lymph nodes >1 remained independently associated with the occurrence of wound infection. The 30-day mortality rate was 4.8% with 18 deaths and morbidity rate 20.6% with 78 cases. CONCLUSIONS: Major complications after radical resection for rectal cancer are dependent on multiple variables such as male patients, those admitted in emergency and patients with hypoproteinemia. Location of tumor on middle rectum, manual suture of anastomosis, number of lymph nodes > 1 were associated with high rate of morbidity. Patients with coronary heart disease and diabetes mellitus didn't had statistical significance, but the rate of morbidity and mortality remains high in this groups. KEY WORDS: Complications, Radical anterior resection, Rectal cancer, Risk factors.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1801
[Cu] Class update date: 180117
[Lr] Last revision date:180117
[St] Status:In-Process


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