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43 results on '"Wortmann, Saskia B"'

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1. Repurposing empagliflozin in individuals with glycogen storage disease Ib: A value‐based healthcare approach and systematic benefit‐risk assessment

2. Beyond genetics: Deciphering the impact of missense variants in CAD deficiency

4. MOGS‐ CDG: Quantitative analysis of the diagnosticGlc 3 Mantetrasaccharide and clinical spectrum of six new cases

5. How to proceed after “negative” exome: A review on genetic diagnostics, limitations, challenges, and emerging new multiomics techniques

7. Variants in Mitochondrial ATP Synthase Cause Variable Neurologic Phenotypes

8. Expanding the phenotypic spectrum of BCS1L‐related mitochondrial disease

9. Congenital disorders of glycosylation with defective fucosylation

10. Mutations inHID1Cause Syndromic Infantile Encephalopathy and Hypopituitarism

11. Austrian study shows that delays in accessing acute paediatric health care outweighed the risks of COVID‐19

13. Variants in NGLY1 lead to intellectual disability, myoclonus epilepsy, sensorimotor axonal polyneuropathy and mitochondrial dysfunction

15. Mitochondrial DNA mutation analysis from exome sequencing—A more holistic approach in diagnostics of suspected mitochondrial disease

16. Biallelic mutations in PIGP cause developmental and epileptic encephalopathy

19. Next-generation metabolic screening: targeted and untargeted metabolomics for the diagnosis of inborn errors of metabolism in individual patients

21. The role of the clinician in the multi-omics era: are you ready?

22. Progressive deafness–dystonia due to SERAC1 mutations: A study of 67 cases

23. Clinical, morphological, biochemical, imaging and outcome parameters in 21 individuals with mitochondrial maintenance defect related to FBXL4 mutations

25. High prevalence of complementary and alternative medicine use in patients with genetically proven mitochondrial disorders

27. Perinatal and early infantile symptoms in congenital disorders of glycosylation

28. 3‐Methylglutaconic aciduria—lessons from 50 genes and 977 patients

30. Nijmegen paediatric CDG rating scale: a novel tool to assess disease progression

34. MOGS-CDG: Quantitative analysis of the diagnostic Glc 3 Man tetrasaccharide and clinical spectrum of six new cases.

35. A scoring system predicting the clinical course of CLPB defect based on the foetal and neonatal presentation of 31 patients.

36. Mild orotic aciduria in UMPS heterozygotes: a metabolic finding without clinical consequences.

37. Previously Unreported Biallelic Mutation in DNAJC19: Are Sensorineural Hearing Loss and Basal Ganglia Lesions Additional Features of Dilated Cardiomyopathy and Ataxia (DCMA) Syndrome?

38. Atypical Clinical Presentations of TAZ Mutations: An Underdiagnosed Cause of Growth Retardation?

39. SUCLA2 Deficiency: A Deafness-Dystonia Syndrome with Distinctive Metabolic Findings (Report of a New Patient and Review of the Literature).

40. High prevalence of complementary and alternative medicine use in patients with genetically proven mitochondrial disorders.

41. Inborn errors of metabolism in the biosynthesis and remodelling of phospholipids.

42. Leucine Loading Test is Only Discriminative for 3-Methylglutaconic Aciduria Due to AUH Defect.

43. The 3-methylglutaconic acidurias: what's new?

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