Journal of rare diseases (Berlin, Germany)最新文献

英文中文

A brief insight into the rare diseases in Egypt 埃及罕见病简介

Journal of rare diseases (Berlin, Germany)

Pub Date : 2023-05-01 DOI: 10.1007/s44162-023-00010-1

Tarek Taha, Dina Ahmed, Zaynab El-Gammal, Gehad Atef Oura, S. Elshenawy, Yasmine Gaber, Tarek Elnagdy, Khaled Amer

引用次数: 1

Three-dimensional radiographic features of craniometaphyseal dysplasia—a comparative CBCT study 颅骨干骺端发育不良的三维影像学特征——CBCT对比研究

Journal of rare diseases (Berlin, Germany)

Pub Date : 2023-04-03 DOI: 10.1007/s44162-023-00009-8

K. S. Muttanahally, I. Chen, E. Reichenberger, A. Tadinada, A. Vaddi

引用次数: 0

Acute intermittent porphyria and spinal muscular atrophy: two rare diseases seen in one patient 急性间歇性卟啉症和脊髓性肌萎缩：一例患者的两种罕见疾病

Journal of rare diseases (Berlin, Germany)

Pub Date : 2023-03-01 DOI: 10.1007/s44162-023-00007-w

Sude Çavdaroğlu, Ilayda Altun, Elif Bilge Atasay, Gulshan Yunisova, P. Oflazer, G. Sezgin

引用次数: 0

Luteolin promotes KAT6A gene expression 木犀草素促进KAT6A基因表达

Journal of rare diseases (Berlin, Germany)

Pub Date : 2023-02-22 DOI: 10.1007/s44162-023-00008-9

P. Brun, M. Dettin, R. Vettor

引用次数: 0

Adult presentations of variable kidney and liver phenotypes secondary to biallelic PKHD1 pathogenic variants 双等位基因PKHD1致病变异体继发的可变肾脏和肝脏表型的成人表现

Journal of rare diseases (Berlin, Germany)

Pub Date : 2023-01-02 DOI: 10.1007/s44162-022-00002-7

Ananya Das, P. Mead, J. Sayer

引用次数: 0

Many lessons still to learn about autosomal dominant polycystic kidney disease. 关于常染色体显性遗传性多囊肾病还有很多值得学习的地方。

Journal of rare diseases (Berlin, Germany)

Pub Date : 2023-01-01 Epub Date: 2023-09-01 DOI: 10.1007/s44162-023-00017-8

Sarah Orr, John A Sayer

We are still learning the genetic basis for many rare diseases. Here we provide a commentary on the analysis of the genetic landscape of patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD), one of the most common genetic kidney diseases. Approaches including both phenotype first and genotype first allows some interesting and informative observations within this disease population. PKD1 and PKD2 are the most frequent genetic causes of ADPKD accounting for 78% and 15% respectively, whilst around 7-8% of cases have an alternative genetic diagnosis. These rarer forms include IFT140, GANAB, PKHD1, HNF1B, ALG8, and ALG9. Some previously reported likely pathogenic PKD1 and PKD2 alleles may have a reduced penetrance, or indeed may have been misclassified in terms of their pathogenicity. This recent data concerning all forms of ADPKD points to the importance of performing genetics tests in all families with a clinical diagnosis of ADPKD as well as those with more atypical cystic kidney appearances. Following allele identification, performing segregation analysis wherever possible remains vital so that we continue to learn about these important genetic causes of kidney failure.

我们仍在了解许多罕见疾病的遗传基础。在这里，我们对常染色体显性多囊肾病（ADPKD）患者的遗传状况进行了分析，ADPKD是最常见的遗传性肾脏疾病之一。包括表型优先和基因型优先的方法允许在该疾病人群中进行一些有趣和有信息的观察。PKD1和PKD2是ADPKD最常见的遗传原因，分别占78%和15%，而约7-8%的病例有其他遗传诊断。这些罕见的形式包括IFT140、GANAB、PKHD1、HNF1B、ALG8和ALG9。一些先前报道的可能致病的PKD1和PKD2等位基因可能外显率降低，或者实际上可能在致病性方面被错误分类。这项关于所有形式ADPKD的最新数据表明，在所有临床诊断为ADPKD以及更非典型囊性肾表现的家庭中进行遗传学测试的重要性。在等位基因鉴定之后，尽可能进行分离分析仍然至关重要，这样我们才能继续了解肾衰竭的这些重要遗传原因。

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引用次数: 0

Use of whole genome sequencing to determine the genetic basis of visceral myopathies including Prune Belly syndrome. 使用全基因组测序来确定包括Prune-Belly综合征在内的内脏肌病的遗传基础。

Journal of rare diseases (Berlin, Germany)

Pub Date : 2023-01-01 Epub Date: 2023-06-05 DOI: 10.1007/s44162-023-00012-z

Robert M Geraghty, Sarah Orr, Eric Olinger, Ruxandra Neatu, Miguel Barroso-Gil, Holly Mabillard, Genomics England Research Consortium, Ian Wilson, John A Sayer

Objectives/aims: The visceral myopathies (VM) are a group of disorders characterised by poorly contractile or acontractile smooth muscle. They manifest in both the GI and GU tracts, ranging from megacystis to Prune Belly syndrome. We aimed to apply a bespoke virtual genetic panel and describe novel variants associated with this condition using whole genome sequencing data within the Genomics England 100,000 Genomes Project.Methods: We screened the Genomics England 100,000 Genomes Project rare diseases database for patients with VM-related phenotypes. These patients were screened for sequence variants and copy number variants (CNV) in ACTG2, ACTA2, MYH11, MYLK, LMOD1, CHRM3, MYL9, FLNA and KNCMA1 by analysing whole genome sequencing data. The identified variants were analysed using variant effect predictor online tool, and any possible segregation in other family members and novel missense mutations was modelled using in silico tools. The VM cohort was also used to perform a genome-wide variant burden test in order to identify confirm gene associations in this cohort.Results: We identified 76 patients with phenotypes consistent with a diagnosis of VM. The range of presentations included megacystis/microcolon hypoperistalsis syndrome, Prune Belly syndrome and chronic intestinal pseudo-obstruction. Of the patients in whom we identified heterozygous ACTG2 variants, 7 had likely pathogenic variants including 1 novel likely pathogenic allele. There were 4 patients in whom we identified a heterozygous MYH11 variant of uncertain significance which leads to a frameshift and a predicted protein elongation. We identified one family in whom we found a heterozygous variant of uncertain significance in KCNMA1 which in silico models predicted to be disease causing and may explain the VM phenotype seen. We did not find any CNV changes in known genes leading to VM-related disease phenotypes. In this phenotype selected cohort, ACTG2 is the largest monogenic cause of VM-related disease accounting for 9% of the cohort, supported by a variant burden test approach, which identified ACTG2 variants as the largest contributor to VM-related phenotypes.Conclusions: VM are a group of disorders that are not easily classified and may be given different diagnostic labels depending on their phenotype. Molecular genetic analysis of these patients is valuable as it allows precise diagnosis and aids understanding of the underlying disease manifestations. We identified ACTG2 as the most frequent genetic cause of VM. We recommend a nomenclature change to 'autosomal dominant ACTG2 visceral myopathy' for patients with pathogenic variants in ACTG2 and associated VM phenotypes.Supplementary information: The online version contains supplementary material av

目的：内脏肌病（VM）是一组以平滑肌收缩性差或收缩性差为特征的疾病。它们在胃肠道和GU道都有表现，从巨胱氨酸到Prune-Belly综合征。我们的目标是在英国基因组学100000基因组项目中应用定制的虚拟遗传小组，并使用全基因组测序数据描述与这种疾病相关的新变体。方法：我们筛选了英国基因组学10000基因组项目罕见病数据库中VM相关表型的患者。通过分析全基因组测序数据，筛选这些患者的ACTG2、ACTA2、MYH11、MYLK、LMOD1、CHRM3、MYL9、FLNA和KNCMA1的序列变异和拷贝数变异（CNV）。使用变异效应预测在线工具对已识别的变异进行分析，并使用计算机工具对其他家族成员中任何可能的分离和新的错义突变进行建模。VM队列还用于进行全基因组变异负荷测试，以确定该队列中的确认基因关联。结果：我们确定了76名表型与VM诊断一致的患者。表现的范围包括大孢子虫/微结肠发育不全综合征、Prune-Belly综合征和慢性假性肠梗阻。在我们鉴定出杂合ACTG2变异体的患者中，有7例可能具有致病性变异体，包括1例新的可能致病等位基因。在4名患者中，我们发现了一个意义不确定的杂合MYH11变体，该变体导致移码和预测的蛋白质延伸。我们确定了一个家族，在该家族中，我们在KCNMA1中发现了一个意义不确定的杂合变体，在计算机模型中，该变体预测是致病的，并可能解释所见的VM表型。我们没有在已知基因中发现任何导致VM相关疾病表型的CNV变化。在这个表型选择的队列中，ACTG2是VM相关疾病的最大单基因原因，占队列的9%，这得到了变异负荷测试方法的支持，该方法将ACTG2变异确定为VM相关表型的最大贡献者。结论：VM是一组不易分类的疾病，根据其表型可能会被赋予不同的诊断标签。对这些患者进行分子遗传学分析是有价值的，因为它可以精确诊断并有助于了解潜在的疾病表现。我们确定ACTG2是VM最常见的遗传原因。我们建议将ACTG2致病性变异和相关VM表型的患者命名为“常染色体显性ACTG2内脏肌病”。补充信息：在线版本包含补充材料，可访问10.1007/s44162-023-00012-z。

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引用次数: 0

Retrospective analysis of COVID-19 patients developing otherwise rare complications. 发生罕见并发症的COVID-19患者的回顾性分析。

Journal of rare diseases (Berlin, Germany)

Pub Date : 2023-01-01 DOI: 10.1007/s44162-023-00006-x

Kevin Stepanek, Pritha Chitagi

Background: Over the last 2 years, it has been felt that there was a disproportionate incidence of complications including pneumothorax, pneumomediastinum, and renal disease necessitating dialysis in patients with COVID-19 as compared to patients without COVID-19.

Methods: In a retrospective cohort, all patients were admitted to St. Joseph Mercy Oakland Hospital in Pontiac, Michigan, between March 2020 and November 2021. The data collected included age, sex, BMI, length of stay, COVID-19 PCR result, diagnosis of pneumothorax, diagnosis of pneumomediastinum, diagnosis of renal failure, orders for dialysis, and orders for mechanical ventilation.

Results: Nine thousand five hundred twenty-two patients are included in this study, with 35.6% (3,392 patients) COVID-19 suspected or confirmed positive and 64.4% (6130 patients) confirmed COVID-19 negative. There were 29 cases of pneumomediastinum and 24 cases of pneumothorax, none of which occurred in intubated patients. The incidence of pneumomediastinum (p = 0.001), CODE BLUE (p = 0.01), and mechanical ventilation (p = 0.001) was significantly higher in the COVID-19 positive/suspected group. There was no significant difference in incidence of pneumothorax (p = 0.294). The incidence of dialysis was significantly higher (p < 0.0001) in the COVID-19 negative group.

Conclusions: In review of prior literature and proposed mechanisms, we believe that it was possibly the damage that SARS-CoV-2 inflicts upon lung parenchyma that led to the increased incidence of pneumomediastinum. Given our mixed findings of incidences of pneumomediastinum, pneumothorax, and dialysis, our hope is to remain vigilant to uncover further disease associations and/or complications as more COVID-19 case data becomes available.

背景:在过去的2年里，人们认为与没有COVID-19的患者相比，COVID-19患者的并发症发生率不成比例，包括气胸、纵隔气肿和需要透析的肾脏疾病。方法:在一项回顾性队列研究中，所有患者于2020年3月至2021年11月期间入住密歇根州庞蒂亚克的圣约瑟夫慈悲奥克兰医院。收集的数据包括年龄、性别、BMI、住院时间、COVID-19 PCR结果、气胸诊断、纵隔肺炎诊断、肾功能衰竭诊断、透析医嘱、机械通气医嘱。结果:本研究共纳入9522例患者，其中新冠肺炎疑似或确诊病例3392例(35.6%)，阴性6130例(64.4%)。其中纵隔气肿29例，气胸24例，均无气管插管患者发生。COVID-19阳性/疑似组纵隔气肿(p = 0.001)、CODE BLUE (p = 0.01)和机械通气(p = 0.001)的发生率均显著高于对照组。两组间气胸发生率无统计学差异(p = 0.294)。COVID-19阴性组透析发生率显著高于阴性组(p < 0.0001)。结论:在回顾既往文献和提出的机制后，我们认为可能是SARS-CoV-2对肺实质的损伤导致纵隔肺炎的发生率增加。鉴于我们对纵隔气肿、气胸和透析发病率的调查结果不一，我们希望随着更多COVID-19病例数据的出现，保持警惕，发现进一步的疾病关联和/或并发症。

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引用次数: 0

JORD Editor-in-Chief’s welcome: rare diseases from science to practice JORD总编辑欢迎:罕见病从科学走向实践

Journal of rare diseases (Berlin, Germany)

Pub Date : 2022-12-01 DOI: 10.1007/s44162-022-00001-8

Neveen A. Soliman

引用次数: 0

The impact of consanguinity on human health and disease with an emphasis on rare diseases 血缘关系对人类健康和疾病的影响，重点是罕见疾病

Journal of rare diseases (Berlin, Germany)

Pub Date : 2022-12-01 DOI: 10.1007/s44162-022-00004-5

G. Temaj, N. Nuhii, J. Sayer

引用次数: 6

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