JIMD reports最新文献_第4页

Holocarboxylase Synthetase Deficiency: A Second Case Report With Neonatal Cholestatic Liver Disease 全新羧化酶合成酶缺乏：新生儿胆汁淤积性肝病第二例报告

IF 1.8

JIMD reports Pub Date : 2025-12-10 DOI: 10.1002/jmd2.70051

Sophie Manoy, Claire Murray, Matthew Lynch, Tahlee Minto, Kelvin Choo, Carolyn Bursle, Michelle Lipke, Jim McGill, Anita Inwood, David Coman

引用次数: 0

Liver Transplantation and Other Hepatically Directed Therapies Do Not Change the Biochemical Phenotype nor Halt Progression of Leukodystrophy due to Biallelic HMBS Variants: A Case Report 肝移植和其他肝定向治疗不能改变生化表型，也不能阻止双等位基因HMBS变异引起的白质营养不良的进展：一个病例报告。

IF 1.8

JIMD reports Pub Date : 2025-12-09 DOI: 10.1002/jmd2.70056

Jeremy Clark, Joel Smith, Yoke Leong, Virginia Cronin, Ingrid Winship, Gayle Ross, Timothy Fazio

{"title":"Liver Transplantation and Other Hepatically Directed Therapies Do Not Change the Biochemical Phenotype nor Halt Progression of Leukodystrophy due to Biallelic HMBS Variants: A Case Report","authors":"Jeremy Clark, Joel Smith, Yoke Leong, Virginia Cronin, Ingrid Winship, Gayle Ross, Timothy Fazio","doi":"10.1002/jmd2.70056","DOIUrl":"10.1002/jmd2.70056","url":null,"abstract":"Leukodystrophy due to biallelic HMBS variants is a rare condition distinct from acute intermittent porphyria (AIP). It is characterised by progressive leukoencephalopathy rather than acute attacks of neurovisceral symptoms. We report the ongoing clinical progression of a patient with leukodystrophy due to homozygous variants in HMBS [c.251C>A, p.Ala84Asp] despite liver transplantation. We demonstrate that porphyrin precursor levels are unchanged following liver transplantation in the periphery and that porphyrin precursor levels are constitutively elevated in the cerebrospinal fluid and are not reduced by haem arginate therapy. Liver transplantation and hepatically directed therapies are not likely to be effective for leukodystrophy due to biallelic HMBS variants. Alternative treatment strategies should be developed for effective management of this disorder. One-liner: Leukodystrophy due to biallelic HMBS variants is unlikely to be cured by liver transplantation or other hepatically directed therapies.","PeriodicalId":14930,"journal":{"name":"JIMD reports","volume":"67 1","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12688452/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145723326","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Course of Pregnancy in a Woman With Familial Chylomicronemia Syndrome Treated With Plozasiran, a Small Interfering RNA Against ApoC3 一种抗apo3的小干扰RNA plzasiran治疗家族性乳糜微粒血症综合征的妊娠过程

IF 1.8

JIMD reports Pub Date : 2025-12-04 DOI: 10.1002/jmd2.70052

Miriam Larouche, Diane Brisson, Nathalie Roy, Claudy Grenon, Paul Poirier, Ma'an Muhsin, Daniel Gaudet

{"title":"Course of Pregnancy in a Woman With Familial Chylomicronemia Syndrome Treated With Plozasiran, a Small Interfering RNA Against ApoC3","authors":"Miriam Larouche, Diane Brisson, Nathalie Roy, Claudy Grenon, Paul Poirier, Ma'an Muhsin, Daniel Gaudet","doi":"10.1002/jmd2.70052","DOIUrl":"https://doi.org/10.1002/jmd2.70052","url":null,"abstract":"Persistent chylomicronemia is associated with severe hypertriglyceridemia (sHTG) and plasma triglycerides (TG) levels sustainably > 10 mmol/L (880 mg/dL) despite lipid lowering therapies. The main risk of persistent chylomicronemia is acute pancreatitis (AP). During the second and third trimester of pregnancy, TG levels significantly increase, which represents a serious risk of AP in women with preexisting chylomicronemia. New emerging therapies such as plozasiran, a GalNAc-conjugated small interfering RNA (siRNA) against ApoC3, are developed to manage persistent chylomicronemia, but no data are currently available on their safety and efficacy during pregnancy. We report herein the case of a woman with persistent chylomicronemia randomized in the PALISADE study to receive plozasiran 25 mg quarterly, who had an unplanned pregnancy during the clinical trial. The 34-year-old patient received one dose of plozasiran 8 weeks before conception and the experimental treatment was ceased afterwards. The pregnancy went well, TG levels did not rise above 10 mmol/L (880 mg/dL) during the whole pregnancy, even during the last two trimesters where TG levels usually increase two- to four-fold from baseline and she did not experience any AP episode. She delivered a healthy baby at 39 weeks. This case suggests that plozasiran might be safe for the mother and the fetus and could prevent incremental pregnancy-driven TG elevation and occurrence of AP in women with sHTG. This is consistent with the long duration of action and hepatic half-life of plozasiran in clinical studies where TG levels remained sustainably lower than baseline > 9 months after the last injection.","PeriodicalId":14930,"journal":{"name":"JIMD reports","volume":"67 1","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jmd2.70052","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145686165","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Successful Management of Two Consecutive Pregnancies With Maternal–Fetal Phenylketonuria: Lessons From Clinical Practice 连续两次妊娠伴母胎苯丙酮尿症的成功治疗：临床经验教训

IF 1.8

JIMD reports Pub Date : 2025-12-03 DOI: 10.1002/jmd2.70054

Per Lundkvist, Karin Blom Malmberg, Linda Lindström, Andreas Kindmark

{"title":"Successful Management of Two Consecutive Pregnancies With Maternal–Fetal Phenylketonuria: Lessons From Clinical Practice","authors":"Per Lundkvist, Karin Blom Malmberg, Linda Lindström, Andreas Kindmark","doi":"10.1002/jmd2.70054","DOIUrl":"https://doi.org/10.1002/jmd2.70054","url":null,"abstract":"Phenylketonuria (PKU) is an autosomal recessive disorder caused by a deficiency of phenylalanine hydroxylase (PAH), leading to the accumulation of phenylalanine (Phe) and an increased risk of developmental disorders. Treatment involves a Phe-restricted diet, amino acid supplements, and for a subset of patients, a tetrahydrobiopterin (BH4) chaperone. Managing PKU during pregnancy is challenging due to changing protein and energy needs, stricter Phe control, nausea, and unpalatable supplements. In rare cases of simultaneous maternal and fetal PKU, Phe tolerance may increase less throughout gestation, raising the demands on the patient and caregivers. There are few reports and no guidelines on the management of PKU during pregnancies in which both mother and fetus have PKU, hereafter referred to as maternal-fetal PKU (mfPKU). This report outlines our approach for successfully managing two consecutive mfPKU pregnancies. We emphasize a patient-centered approach, focusing on patient education and close collaboration with a multidisciplinary metabolic team. This involves regular monitoring of body weight, blood Phe levels, and calorie intake through an online food diary to tailor individual recommendations for natural protein restriction and amino acid supplements.","PeriodicalId":14930,"journal":{"name":"JIMD reports","volume":"67 1","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-12-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jmd2.70054","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145659676","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Female Patients With Mucopolysaccharidosis II (MPS II): Insights From the Hunter Outcome Survey 女性粘多糖病II (MPS II)：来自Hunter结果调查的见解

IF 1.8

JIMD reports Pub Date : 2025-12-02 DOI: 10.1002/jmd2.70046

Barbara K. Burton, Hernan Amartino, Roberto Giugliani, Christoph Kampmann, Julian Raiman, Maurizio Scarpa, Anna Tylki-Szymańska, Jennifer Audi, Jaco Botha, Siddarth Jain, Joseph Muenzer

{"title":"Female Patients With Mucopolysaccharidosis II (MPS II): Insights From the Hunter Outcome Survey","authors":"Barbara K. Burton, Hernan Amartino, Roberto Giugliani, Christoph Kampmann, Julian Raiman, Maurizio Scarpa, Anna Tylki-Szymańska, Jennifer Audi, Jaco Botha, Siddarth Jain, Joseph Muenzer","doi":"10.1002/jmd2.70046","DOIUrl":"https://doi.org/10.1002/jmd2.70046","url":null,"abstract":"Mucopolysaccharidosis II is a rare, X-linked disease, with very few reports of affected female patients. Natural history data describe a predominantly male population, and appropriate disease characterization in female patients is lacking. This analysis explores the somatic disease burden and clinical progression of female patients with MPS II enrolled in the Hunter Outcome Survey (HOS; NCT03292887), a global disease registry. In total, 15 female patients were identified, representing 1.1% of the total patients in HOS. The median ages at first symptom onset and diagnosis were 1.8 and 3.1 years, respectively. A total of 8/14 (57.1%) of patients had cognitive impairment at the latest visit. X-chromosome abnormalities were reported in two patients. Most patients (11/15, 73.3%) had received at least one dose of idursulfase, which was generally well tolerated; no serious adverse events during follow-up were considered treatment-related. Musculoskeletal and ear symptoms were present in all 14 patients with data recorded. Almost all females also experienced neurological, abdominal/gastrointestinal, and pulmonary disease, similar to the symptomatology reported in males. Most patients underwent surgery (41 procedures in 12 patients). Two participants had a male sibling with MPS II who was also enrolled in HOS. Both sibling sets had missense variants and demonstrated several differences in signs/symptoms between the male and female siblings. Notably, only the female siblings displayed cognitive impairment. This report illustrates the disease burden in female patients with MPS II, helping to inform clinicians about the likely prognosis for this extremely rare subgroup of patients.","PeriodicalId":14930,"journal":{"name":"JIMD reports","volume":"67 1","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-12-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jmd2.70046","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145659589","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Elevated Transaminases: Does It Always Warrant a Liver Biopsy? Lessons Learned From Pompe Disease 转氨酶升高：是否一定需要肝活检？从庞贝病中吸取的教训。

IF 1.8

JIMD reports Pub Date : 2025-11-24 DOI: 10.1002/jmd2.70050

Alicia Khazzeka, Rebecca L. Koch, Jeong-A. Lim, Jeremy D. Ward, Jessica Doxey, William R. Jeck, Priya S. Kishnani

{"title":"Elevated Transaminases: Does It Always Warrant a Liver Biopsy? Lessons Learned From Pompe Disease","authors":"Alicia Khazzeka, Rebecca L. Koch, Jeong-A. Lim, Jeremy D. Ward, Jessica Doxey, William R. Jeck, Priya S. Kishnani","doi":"10.1002/jmd2.70050","DOIUrl":"10.1002/jmd2.70050","url":null,"abstract":"Pompe disease (PD) is an autosomal recessive disorder caused by pathogenic variants in GAA, resulting in acid alpha-glucosidase (GAA) deficiency and lysosomal glycogen accumulation. PD is classified into infantile-onset (IOPD), characterized by cardiomyopathy and death within the first year if untreated, and late-onset (LOPD), which presents with gradual muscle weakness at variable ages. Incidentally elevated transaminase levels are common in LOPD and reflect muscle injury rather than liver damage. Creatine kinase (CK) levels are often elevated too, further indicating a myopathic origin. However, these elevated transaminases may be misattributed to liver disease, prompting a liver biopsy. Three charts of patients who underwent liver biopsies prior to a LOPD diagnosis were reviewed, including clinical presentations and diagnostic workups. Liver histology from these biopsies was evaluated and compared to liver pathology in a GAA knockout mouse model. All cases had elevated transaminases and underwent a liver biopsy. One biopsy was normal, while two showed non-specific hepatocyte glycogen accumulation. In all cases, aspartate transaminase (AST) levels were higher than alanine transaminase (ALT) levels and CK levels were elevated. Enzyme testing demonstrated GAA deficiency, and genetic testing identified biallelic variants in GAA, confirming the diagnosis. These cases highlight the importance of meticulous phenotyping before liver biopsy. A muscle origin should be considered when AST:ALT > 1 with elevated CK levels. Neuromuscular gene panels and GAA enzyme testing offer a non-invasive diagnostic approach in LOPD. Notably, even when glycogen accumulation is observed in the liver histologically, liver disease is not associated with PD.","PeriodicalId":14930,"journal":{"name":"JIMD reports","volume":"66 6","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-11-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12643788/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145604184","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Correction to “Farber's Lipogranulomatosis: Multimodal Therapy With Tocilizumab and Consolidative HSCT Improves Assessment, and Long-Term Outcome” 对“法伯氏脂肪肉芽肿病：托珠单抗和巩固性造血干细胞移植的多模式治疗改善了评估和长期结果”的修正。

IF 1.8

JIMD reports Pub Date : 2025-11-10 DOI: 10.1002/jmd2.70049

引用次数: 0

Severe Neurological Presentation in Siblings With COQ5-Related Primary Coenzyme Q10 Deficiency: Expanding Clinical and Molecular Spectrum coq5相关的初级辅酶Q10缺乏的兄弟姐妹的严重神经症状：扩大临床和分子谱。

IF 1.8

JIMD reports Pub Date : 2025-11-05 DOI: 10.1002/jmd2.70038

Parith Wongkittichote, Rachel M. Guerra, Daniel J. Wegner, Samantha Toy, Jacqueline A. Hauer, David J. Pagliarini, Jorge L. Granadillo

{"title":"Severe Neurological Presentation in Siblings With COQ5-Related Primary Coenzyme Q10 Deficiency: Expanding Clinical and Molecular Spectrum","authors":"Parith Wongkittichote, Rachel M. Guerra, Daniel J. Wegner, Samantha Toy, Jacqueline A. Hauer, David J. Pagliarini, Jorge L. Granadillo","doi":"10.1002/jmd2.70038","DOIUrl":"10.1002/jmd2.70038","url":null,"abstract":"Coenzyme Q10 (CoQ10) is a coenzyme and antioxidant involved in multiple bioenergetic and biosynthetic processes, particularly within mitochondria. The biosynthesis of CoQ10 is a tightly regulated process that involves multiple enzymes, including the methyltransferase COQ5. Genetic defects in COQ5 have recently been associated with autosomal recessive COQ5-related primary CoQ10 deficiency. The clinical manifestations of seven individuals previously reported were primarily neurological and ophthalmological. Here, we report two siblings with profound developmental delay and brain imaging consistent with multistage strokes. Clinical exome sequencing revealed compound heterozygous variants in COQ5, including one frameshift deletion and one missense variant. Our functional complementation studies demonstrate that a Saccharomyces cerevisiae COQ5 ortholog harboring the corresponding missense variant fails to fully rescue coq5∆ CoQ6 production, leading to the accumulation of CoQ biosynthetic intermediates. After the diagnosis, CoQ10 supplementation was started on the proband, leading to subjective clinical improvement. We describe new cases of COQ5-related primary CoQ10 deficiency and expand the phenotypic and molecular spectrum of the disease. We also establish a yeast system to evaluate the effects of the variants in COQ5 and support the use of CoQ10 supplementation for patients with COQ5-related primary CoQ10 deficiency.","PeriodicalId":14930,"journal":{"name":"JIMD reports","volume":"66 6","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12588166/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145458756","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Small Molecules as Alternate Substrates for 3-Methylglutaconylation 小分子作为3-甲基谷酰化的替代底物。

IF 1.8

JIMD reports Pub Date : 2025-10-15 DOI: 10.1002/jmd2.70047

Elizabeth A. Jennings, Irina Romenskaia, Robert O. Ryan

{"title":"Small Molecules as Alternate Substrates for 3-Methylglutaconylation","authors":"Elizabeth A. Jennings, Irina Romenskaia, Robert O. Ryan","doi":"10.1002/jmd2.70047","DOIUrl":"10.1002/jmd2.70047","url":null,"abstract":"The leucine catabolism pathway intermediate, trans-3-methylglutaconyl (3MGC) CoA, is susceptible to a series of non-enzymatic reactions that generate organic acid waste products and protein 3MGCylation. These reactions proceed when inborn errors of metabolism (IEM) in HMGCL or AUH lead to enzyme deficiencies. When trans-3MGC-CoA levels rise, a portion of this metabolite pool isomerizes to cis-3MGC-CoA, forming a diastereomer that is capable of spontaneous intramolecular cyclization, yielding 3MGC anhydride and free CoA. 3MGC anhydride can undergo hydrolysis to 3MGC acid or react with protein lysine residues to 3MGCylate proteins. The present study was designed to examine the ability of small molecules to react with 3MGC anhydride. An antibody directed against 3MGC was employed in vitro experiments designed to assess the ability of candidate biomolecules to attenuate the immunoblot signal intensity of 3MGCylated bovine serum albumin (BSA). When trans-3MGC-CoA was incubated in the presence of glycine, glucosamine, ethanolamine, or glutathione, each of these free amino group-containing molecules, but not N-acetylglucosamine or choline, induced a concentration-dependent decrease in 3MGCylated BSA immunoblot signal intensity. It is concluded that 3MGC anhydride can react with primary amine-containing metabolites to acylate them.","PeriodicalId":14930,"journal":{"name":"JIMD reports","volume":"66 6","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12527551/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145307967","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Treatment of Single Patient With PMM2-Congenital Disorder of Glycosylation With Govorestat (AT-007), an Aldose Reductase Inhibitor 醛糖还原酶抑制剂Govorestat （AT-007）治疗单例pmm2先天性糖基化障碍

IF 1.8

JIMD reports Pub Date : 2025-10-12 DOI: 10.1002/jmd2.70043

Elizabeth R. Jalazo, Leigh Anne Weisenfeld, Anna Ligezka, Riccardo Perfetti, Joseph Muenzer

{"title":"Treatment of Single Patient With PMM2-Congenital Disorder of Glycosylation With Govorestat (AT-007), an Aldose Reductase Inhibitor","authors":"Elizabeth R. Jalazo, Leigh Anne Weisenfeld, Anna Ligezka, Riccardo Perfetti, Joseph Muenzer","doi":"10.1002/jmd2.70043","DOIUrl":"10.1002/jmd2.70043","url":null,"abstract":"Aldose reductase inhibitors (ARI) have been identified as a potential treatment for phosphomannomutase-2 congenital disorder of glycosylation (PMM2-CDG), a serious condition for which no treatments are approved. We treated a single patient for 36 months 30 months of age at enrollment, under a single-patient investigational new drug expanded access request, with govorestat (AT-007), a novel, highly selective, once daily, brain penetrant ARI at a starting dose of 1 mg/kg oral suspension, which was escalated to 30 mg/kg. The primary endpoint was safety. Secondary assessments included liver transaminases, factor XI, antithrombin III, and whole blood and urine sorbitol. Clinical outcomes were also assessed, including Nijmegen Pediatric CDG Rating Scale (NPCRS), Bayley Scales of Infant Development, and Vineland Adaptive Behavioral Scale. Govorestat was well tolerated; no adverse effects were noted. Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) levels improved from a pre-treatment 12-month average of 205 and 268 U/L to 63 and 68 U/L, respectively, averaged over 36 months of govorestat treatment at 30 mg/kg. Antithrombin III and factor XI fluctuated with illness throughout the study period but overall increased by 60%–100%, approaching the normal range (> 83% activity) at the 5 mg/kg dose. Whole blood sorbitol decreased in a dose-dependent fashion, normalizing at the 30 mg/kg dose. The NPCRS improved by 9 points (46%) over the course of treatment. In conclusion, our patient tolerated govorestat without safety concerns. Improvements in liver transaminases, clotting factors, and whole blood sorbitol were observed along with improvements in clinical measures. These findings support further study of govorestat as a potential treatment for PMM2-CDG.","PeriodicalId":14930,"journal":{"name":"JIMD reports","volume":"66 6","pages":""},"PeriodicalIF":1.8,"publicationDate":"2025-10-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12516010/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145292148","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0