LIVIVO - Search results -

Search results

Result 1 - 10 of total 40

Search options

Article: Homocysteine and methylmalonic acid in Phenylketonuria patients.

Hoss, Giovana Regina Weber / Sperb-Ludwig, Fernanda / Tonon, Tássia / Poloni, Soraia / Behringer, Sidney / Blom, Henk J / Maillot, François / Schwartz, Ida Vanessa Doederlein

Genetics and molecular biology

2024 Volume 46, Issue 3 Suppl 1, Page(s) e20230103

Abstract: Hyperhomocysteinemia and vitamin B12 deficiency have been reported in patients with phenylketonuria. In this study, total homocysteine (tHcy) and methylmalonic acid (MMA) levels were analyzed in samples from 25 phenylketonuria (PKU) patients. Comparisons ...

Abstract	Hyperhomocysteinemia and vitamin B12 deficiency have been reported in patients with phenylketonuria. In this study, total homocysteine (tHcy) and methylmalonic acid (MMA) levels were analyzed in samples from 25 phenylketonuria (PKU) patients. Comparisons were made between pre- and post-treatment values (n= 3); on treatment values, between periods with high and normal/low phenylalanine (Phe) levels (n= 20); and in women before, during and after pregnancy (n= 3). THcy levels decreased after treating PKU with metabolic formula (p=0.014). Except for a pregnant woman before pregnancy, none of the patients had tHcy values above the normal range. In fact, tHcy was < 5 μmol/L in 34% of the samples. We observed a decrease in Phe, tHcy, and tyrosine levels during pregnancy. MMA levels did not differ significantly, with values remaining in the normal range. These data indicate that there was no B12 deficiency in patients who adhere to the diet. In conclusion, in PKU patients treated with metabolic formula, tHcy is frequently not elevated, remaining even in the lower normal range in some patients. Thus, clinical follow-up and adherence to dietary treatment are crucial to prevent B12 deficiency.
Language	English
Publishing date	2024-04-05
Publishing country	Brazil
Document type	Journal Article
ZDB-ID	1445712-x
ISSN	1678-4685 ; 1415-4757
ISSN (online)	1678-4685
ISSN	1415-4757
DOI	10.1590/1678-4685-GMB-2023-0103
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 3079: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (2.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

Details ▾
- See ZB MED holdings
- Order with fees

Article: In vitro substrate reduction, chaperone and immunomodulation treatments reduce heparan sulfate in mucolipidosis III human fibroblasts.

Sperb-Ludwig, Fernanda / Ludwig, Nataniel Floriano / Rizowy, Gustavo Mottin / Velho, Renata Voltolini / Schwartz, Ida Vanessa Doederlein

Genetics and molecular biology

2023 Volume 46, Issue 3 Suppl 1, Page(s) e20230117

Abstract: Mucolipidosis II and III (MLII and MLIII) are autosomal recessive diseases caused by pathogenic variants in GNPTAB and GNPTG genes that lead to defects in GlcNAc-1-phosphotransferase. This enzyme adds mannose 6-phosphate residues to lysosomal hydrolases, ...

Abstract	Mucolipidosis II and III (MLII and MLIII) are autosomal recessive diseases caused by pathogenic variants in GNPTAB and GNPTG genes that lead to defects in GlcNAc-1-phosphotransferase. This enzyme adds mannose 6-phosphate residues to lysosomal hydrolases, which allows enzymes to enter lysosomes. Defective GlcNAc-1-phosphotransferase causes substrate accumulation and inflammation. These diseases have no treatment, and we hypothesized that the use of substrate reduction therapy and immunomodulation may be beneficial at the cell level and as a future therapeutic approach. Fibroblasts from two patients with MLIII alpha/beta and 2 patients with MLIII gamma as well as from one healthy control were treated with 10 µM miglustat, 20 µM genistein, and 20 µM thalidomide independently. ELISA assay and confocal immunofluorescence microscopy were used to evaluate the presence of heparan sulfate (HS) and the impact on substrate accumulation. ELISA assay showed HS reduction in all patients with the different treatments used (p=0.05). HS reduction was also observed by immunofluorescence microscopy. Our study produced encouraging results, since the reduction in substrate accumulation, even partial, may offer benefits to the phenotype of patients with inborn errors of metabolism.
Language	English
Publishing date	2023-12-04
Publishing country	Brazil
Document type	Journal Article
ZDB-ID	1445712-x
ISSN	1678-4685 ; 1415-4757
ISSN (online)	1678-4685
ISSN	1415-4757
DOI	10.1590/1678-4685-GMB-2023-0117
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 3079: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (2.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Epidemiological aspects of hereditary fructose intolerance: A database study.

Pinheiro, Franciele C / Sperb-Ludwig, Fernanda / Schwartz, Ida V D

Human mutation

2021 Volume 42, Issue 12, Page(s) 1548–1566

Abstract: Hereditary fructose intolerance (HFI) is an inborn error of fructose metabolism of autosomal recessive inheritance caused by pathogenic variants in the ALDOB gene that lead to aldolase B deficiency in the liver, kidneys, and intestine. Patients manifest ... ...

Abstract	Hereditary fructose intolerance (HFI) is an inborn error of fructose metabolism of autosomal recessive inheritance caused by pathogenic variants in the ALDOB gene that lead to aldolase B deficiency in the liver, kidneys, and intestine. Patients manifest symptoms, such as ketotic hypoglycemia, vomiting, nausea, in addition to hepatomegaly and other liver and kidney dysfunctions. The treatment consists of a fructose-restricted diet, which results in a good prognosis. To analyze the distribution of ALDOB variants described in patients and to estimate the prevalence of HFI based on carrier frequency in the gnomAD database, a systematic review was conducted to assess ALDOB gene variants among patients with HFI. The prevalence of HFI was estimated from the carrier frequency of variants described in patients, as well as rare variants predicted as pathogenic by in silico tools. The p.(Ala150Pro) and p.(Ala175Asp) variants are the most frequent and are distributed worldwide. However, these variants have particular distribution patterns in Europe. The analysis of the prevalence of HFI showed that the inclusion of rare alleles predicted as pathogenic is a more informative approach for populations with few patients. The data show that HFI has a wide distribution and an estimated prevalence of ~1:10,000.
MeSH term(s)	Alleles ; Fructose Intolerance/diagnosis ; Fructose Intolerance/epidemiology ; Fructose Intolerance/genetics ; Fructose-Bisphosphate Aldolase/genetics ; Humans ; Liver/pathology ; Mutation
Chemical Substances	Fructose-Bisphosphate Aldolase (EC 4.1.2.13)
Language	English
Publishing date	2021-09-24
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Systematic Review
ZDB-ID	1126646-6
ISSN	1098-1004 ; 1059-7794
ISSN (online)	1098-1004
ISSN	1059-7794
DOI	10.1002/humu.24282
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Zs.A 3586: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (2.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article: Characterization of the 3'UTR of the BTD gene and identification of regulatory elements and microRNAs.

Silva, Gerda Cristal Villalba / Borsatto, Taciane / Schwartz, Ida Vanessa Doederlein / Sperb-Ludwig, Fernanda

Genetics and molecular biology

2022 Volume 45, Issue 1, Page(s) e20200432

Abstract: Reduced biotinidase activity is associated with a spectrum of deficiency ranging from total deficiency to heterozygous levels, a finding that is not always explained by the pathogenic variants observed in the BTD gene. The investigation of miRNAs, ... ...

Abstract	Reduced biotinidase activity is associated with a spectrum of deficiency ranging from total deficiency to heterozygous levels, a finding that is not always explained by the pathogenic variants observed in the BTD gene. The investigation of miRNAs, regulatory elements and variants in the 3'UTR region may present relevance in understanding the genotype-phenotype association. The aims of the study were to characterize the regulatory elements of the 3'UTR of the BTD gene and identify variants and miRNAs which may explain the discrepancies observed between genotype and biochemical phenotype. We evaluated 92 individuals with reduced biotinidase activity (level of heterozygotes = 33, borderline = 35, partial DB = 20 or total DB= 4) with previously determined BTD genotype. The 3'UTR of the BTD gene was Sanger sequenced. In silico analysis was performed to identify miRNAs and regulatory elements. No variants were found in the 3'UTR. We found 97 possible miRNAs associated with the BTD gene, 49 predicted miRNAs involved in the alanine, biotin, citrate and pyruvate metabolic pathways and 5 genes involved in biotin metabolism. Six AU-rich elements were found. Our data suggest variants in the 3'UTR of BTD do not explain the genotype-phenotype discrepancies found in Brazilian individuals with reduced biotinidase.
Language	English
Publishing date	2022-02-14
Publishing country	Brazil
Document type	Journal Article
ZDB-ID	1445712-x
ISSN	1678-4685 ; 1415-4757
ISSN (online)	1678-4685
ISSN	1415-4757
DOI	10.1590/1678-4685-GMB-2020-0432
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Zs.A 3079: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (2.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article ; Online: Glycogen Storage Disease: Expert Opinion on Clinical Diagnosis Revisited after Molecular Testing.

de Marchi, Rafael / Nalin, Tatiele / Sperb-Ludwig, Fernanda / Pinheiro, Franciele Cabral / Schwartz, Ida Vanessa Doederlein / Steiner, Carlos Eduardo

Genes

2023 Volume 14, Issue 12

Abstract: This study sought to analyze whether an accurate diagnosis of the type and subtype of hepatic Glycogen Storage Diseases (GSDs) could be performed based on general clinical and biochemical aspects via comparing the proposed diagnostic hypotheses with the ... ...

Abstract	This study sought to analyze whether an accurate diagnosis of the type and subtype of hepatic Glycogen Storage Diseases (GSDs) could be performed based on general clinical and biochemical aspects via comparing the proposed diagnostic hypotheses with the molecular results. Twelve physicians with experience in hepatic GSDs reviewed 45 real cases comprising a standardized summary of clinical and laboratory data. There was no relation between the hit rate and the time since graduation, the time of experience in GSD, and the number of patients treated during their careers. The average assertiveness was 47%, with GSD Ia and Ib being the best-identified types, while no expert correctly identified GSD IXc. Underage investigation for later manifestations, incomplete clinical description, and complementary analysis, the overvaluation of a specific clinical finding ("false positive") or the discarding of the diagnosis in the absence of it ("false negative"), as well as the lack of knowledge of the rarest GSD types, may have impacted the accuracy of the assessment. This study emphasized that characteristics considered as determinants in identifying the specific types or subtypes of GSD are not exclusive, thus becoming factors that may have induced the evaluators to misdiagnose.
MeSH term(s)	Humans ; Expert Testimony ; Glycogen Storage Disease/diagnosis ; Glycogen Storage Disease/genetics ; Glycogen Storage Disease Type I/diagnosis ; Molecular Diagnostic Techniques
Language	English
Publishing date	2023-12-15
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2527218-4
ISSN	2073-4425 ; 2073-4425
ISSN (online)	2073-4425
ISSN	2073-4425
DOI	10.3390/genes14122219
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Article ; Online: KHK inhibition for the treatment of hereditary fructose intolerance and nonalcoholic fatty liver disease: a double-edged sword.

Pinheiro, Franciele Cabral / Sperb-Ludwig, Fernanda / Schwartz, Ida Vanessa Doederlein

Cellular and molecular life sciences : CMLS

2020 Volume 77, Issue 17, Page(s) 3465–3466

MeSH term(s)	Fructose ; Fructose Intolerance ; Humans ; Non-alcoholic Fatty Liver Disease
Chemical Substances	Fructose (30237-26-4)
Language	English
Publishing date	2020-06-26
Publishing country	Switzerland
Document type	Journal Article ; Comment
ZDB-ID	1358415-7
ISSN	1420-9071 ; 1420-682X
ISSN (online)	1420-9071
ISSN	1420-682X
DOI	10.1007/s00018-020-03575-y
Database	MEDical Literature Analysis and Retrieval System OnLINE

Full text online

Accessible to users with ZB MED library card

In stock of ZB MED Cologne/Königswinter

Zs.A 195: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾

Article ; Online: Classical homocystinuria: A common inborn error of metabolism? An epidemiological study based on genetic databases.

Weber Hoss, Giovana R / Sperb-Ludwig, Fernanda / Schwartz, Ida V D / Blom, Henk J

Molecular genetics & genomic medicine

2020 Volume 8, Issue 6, Page(s) e1214

Abstract: Background: Biallelic pathogenic variants in CBS gene cause the most common form of homocystinuria, the classical homocystinuria (HCU). The worldwide prevalence of HCU is estimated to be 0.82:100,000 [95% CI, 0.39-1.73:100,000] according to clinical ... ...

Abstract	Background: Biallelic pathogenic variants in CBS gene cause the most common form of homocystinuria, the classical homocystinuria (HCU). The worldwide prevalence of HCU is estimated to be 0.82:100,000 [95% CI, 0.39-1.73:100,000] according to clinical records and 1.09:100,000 [95% CI, 0.34-3.55:100,000] by neonatal screening. In this study, we aimed to estimate the minimal worldwide incidence of HCU. Methods: The 25 most common pathogenic alleles of HCU were identified through a literature review. The incidence of HCU was estimated based on the frequency of these common pathogenic alleles in a large genomic database (gnomAD). Results: The minimum worldwide incidence of HCU was estimated to be ~0.38:100,000, and the incidence was higher in Europeans non-Finnish (~0.72:100,000) and Latin Americans (~0.45:100,000) and lower in Africans (~0.20:100,000) and Asians (~0.02:100,000). Conclusion: Our data are in accordance with the only published metanalysis on this topic. To our surprise, the observed incidence of HCU in Europeans was much lower than those described in articles exploring small populations from northern Europe but was similar to the incidence described on the basis of neonatal screening programs. In our opinion, this large dataset analyzed and its population coverage gave us greater precision in the estimation of incidence.
MeSH term(s)	Adult ; Cystathionine beta-Synthase/genetics ; Databases, Genetic/statistics & numerical data ; Europe ; Gene Frequency ; Homocystinuria/epidemiology ; Homocystinuria/ethnology ; Homocystinuria/genetics ; Humans ; Incidence ; Infant, Newborn ; Neonatal Screening
Chemical Substances	Cystathionine beta-Synthase (EC 4.2.1.22)
Language	English
Publishing date	2020-03-30
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2734884-2
ISSN	2324-9269 ; 2324-9269
ISSN (online)	2324-9269
ISSN	2324-9269
DOI	10.1002/mgg3.1214
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

Article ; Online: Effect of BTD gene variants on in vitro biotinidase activity.

Borsatto, Taciane / Sperb-Ludwig, Fernanda / Blom, Henk J / Schwartz, Ida V D

Molecular genetics and metabolism

2019 Volume 127, Issue 4, Page(s) 361–367

Abstract: Introduction: Biotinidase deficiency (BD), an autosomal recessive disease, is classified into profound (activity ... C (p.Asp444His), which is associated ... ...

Abstract	Introduction: Biotinidase deficiency (BD), an autosomal recessive disease, is classified into profound (activity <10%) or partial BD (activity 10-30%). The most frequent variant in patients worldwide is c.1330G > C (p.Asp444His), which is associated with partial BD. In vivo studies indicate that this variant reduces the biotinidase activity by 50%. The objective of this study was to evaluate the in vitro effect of p.Asp444His and of five novel variants identified among Brazilian individuals showing low activity of biotinidase in serum. Methods: The variants c.119 T > C (p.Leu40Pro), c.479G > A (p.Cys160Tyr), c.664G > A (p.Asp222Asn), c.1330G > C (p.Asp444His), c.1337 T > C (p.Leu446Pro), c.1466A > G (p.Asn489Ser) and the wild type (wt) BTD gene were expressed in HEK 293 cells. Biotinidase activity was quantified by colorimetric method in cells homogenates and culture medium. The wtBTD activity was considered 100%. Results: The p.Leu40Pro, p.Cys160Tyr and p.Leu446Pro variants were associated to impaired biotinidase activity (activity in cells: 33%, 14%, 0%, respectively; activity in medium: 7%, 0.3%, 2%, respectively) and undetectable amount of protein in intra and extracellular space. The p.Asn489Ser variant had these effects restricted to the extracellular space (activity in medium: 43%), and the p.Asp222Asn variant showed normal activity. The expression of p.Asp444His variant resulted in detectable protein and slightly reduced activity only in cells (activity in cells: 46%; activity in medium: 115%). Conclusion: Our findings suggest that the p.Leu40Pro, p.Cys160Tyr and p.Leu446Pro variants are deleterious; the p.Asn489Ser is probably related to a mild biochemical phenotype; and p.Asp222Asn variant is probably not deleterious. The p.Asp444His variant seems to code for a protein with variable activity.
MeSH term(s)	Alleles ; Biotinidase/genetics ; Biotinidase/metabolism ; Biotinidase Deficiency/genetics ; Colorimetry ; Gene Expression ; Genetic Variation ; HEK293 Cells ; Humans ; Mutation
Chemical Substances	Biotinidase (EC 3.5.1.12)
Language	English
Publishing date	2019-07-17
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1418518-0
ISSN	1096-7206 ; 1096-7192
ISSN (online)	1096-7206
ISSN	1096-7192
DOI	10.1016/j.ymgme.2019.07.006
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Zs.A 617: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article ; Online: Chitotriosidase on treatment-naïve patients with Gaucher disease: A genotype vs phenotype study.

Sperb-Ludwig, Fernanda / Heineck, Bianca Lúcia / Michelin-Tirelli, Kristiane / Alegra, Taciane / Schwartz, Ida Vanessa Doederlein

Clinica chimica acta; international journal of clinical chemistry

2019 Volume 492, Page(s) 1–6

Abstract: Background: Chitotriosidase (ChT) is used as a biomarker for the follow-up of patients with Gaucher disease (GD), once his activity is extremely elevated and declines during ERT. However, some variants in the CHIT1 gene affect ChT activity.: Methods: ...

Abstract	Background: Chitotriosidase (ChT) is used as a biomarker for the follow-up of patients with Gaucher disease (GD), once his activity is extremely elevated and declines during ERT. However, some variants in the CHIT1 gene affect ChT activity. Methods: To assess association between ChT genotype, and clinical/biochemical features of GD were performed CHIT1 genotyping for: c.1049_1072dup24, p.Gly102Ser, p.Gly354Arg, c.1155_1156 + 2delGAGT, c.1156 + 5_1156 + 8delGTAA, p.Ala442Val/Gly and the rearrangement delE/I-10. Results: Were evaluated 42 patients with GD from Southern Brazil. Pretreatment ChT activity was available for 32 patients. Allelic frequencies found for dup24, p.Gly102Ser and p.Ala442Gly were 0.14, 0.32 and 0.12, respectively. Only one patient presented reduced ChT activity (dup24 homozygous). Comparison between wild homozygous and heterozygous for dup24 showed that both differ in relation to the ChT activity before (15,230 vs 6936 nmol/h/mL, p < .001), but not after treatment (5212 vs 3045 nmol/h/mL, p = .227). Conclusions: Pretreatment ChT activity was not correlated with clinical/biochemical features. There was a reduction of 63% in the ChT activity after 12 months on treatment (p < .001). There is no evidence that higher ChT levels are associated with a more severe symptomatology in untreated GD patients. The pretreatment ChT levels appear to be mainly dependent on the presence/absence of the dup24 allele.
MeSH term(s)	Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Gaucher Disease/enzymology ; Gaucher Disease/genetics ; Gene Frequency ; Genotype ; Hexosaminidases/genetics ; Humans ; Male ; Middle Aged ; Phenotype ; Young Adult
Chemical Substances	Hexosaminidases (EC 3.2.1.-) ; chitotriosidase (EC 3.2.1.-)
Language	English
Publishing date	2019-01-26
Publishing country	Netherlands
Document type	Journal Article
ZDB-ID	80228-1
ISSN	1873-3492 ; 0009-8981
ISSN (online)	1873-3492
ISSN	0009-8981
DOI	10.1016/j.cca.2019.01.018
Database	MEDical Literature Analysis and Retrieval System OnLINE

In stock of ZB MED Cologne/Königswinter

Ud II Zs.173: Show issues

Location:
Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
ab Jg. 2022: Lesesaal (EG)

Order via subito

Details ▾
- See ZB MED holdings
- Order with fees

Article: The rs2229611 (

Pinheiro, Franciele Cabral / Sperb-Ludwig, Fernanda / Fagundes Verch, Juliana Maria / Dos Santos, Bruna Bento / de Souza, Carolina Fischinger Moura / Schwartz, Ida Vanessa Doederlein

Molecular genetics and metabolism reports

2020 Volume 25, Page(s) 100659

Abstract: The rs2229611 SNP ( ...

Abstract	The rs2229611 SNP (
Language	English
Publishing date	2020-10-20
Publishing country	United States
Document type	Journal Article
ZDB-ID	2821908-9
ISSN	2214-4269
ISSN	2214-4269
DOI	10.1016/j.ymgmr.2020.100659
Database	MEDical Literature Analysis and Retrieval System OnLINE

Order via subito

To top

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

Order via subito

Full text online

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

In stock of ZB MED Cologne/Königswinter

Order via subito

More links

Kategorien

Order via subito