Age Dependency of Serum Acid Phosphatase in Controls and
Gaucher Patients

Magalhães, João P.; Pinto, Ronei Silveira; Lemos, Marina de Paiva; Miranda, María; Poënaru, Livia

doi:10.1159/000469457

Cited by 8 publications

(5 citation statements)

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“…It was documented that the TRAP level could differ as a function of age [42]; however, the respective age means weren't significantly different between our ERT and SRT patients. Considering biomarker values, the disease burden, including bone involvement, was significantly higher within our SRT cohort.…”

Section: Discussioncontrasting

confidence: 59%

Long-Term Evaluation of Biomarkers in the Czech Cohort of Gaucher Patients

Malinová,

Poupětová,

Řeboun

et al. 2023

IJMS

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A personalized treatment decision for Gaucher disease (GD) patients should be based on relevant markers that are specific to GD, play a direct role in GD pathophysiology, exhibit low genetic variation, reflect the therapy, and can be used for all patients. Thirty-four GD patients treated with enzyme replacement therapy (ERT) or substrate reduction therapy (SRT) were analyzed for platelet count, chitotriosidase, and tartrate-resistant acid phosphatase activity in plasma samples, and quantitative measurement of Lyso-Gb1 was performed in dried blood spots. In our ERT and SRT study cohorts, plasma lyso-GL1 correlated significantly with chito-triosidase (ERT: r = 0.55, p < 0.001; SRT: r = 0.83, p < 0.001) and TRAP (ERT: r = 0.34, p < 0.001; SRT: r = 0.88, p < 0.001), irrespective of treatment method. A platelet count increase was associated with a Lyso-Gb1 decrease in both treatment groups (ERT: p = 0.021; SRT: p = 0.028). The association of Lyso-Gb1 with evaluated markers was stronger in the SRT cohort. Our results indicate that ERT and SRT in combination or in a switch manner could offer the potential of individual drug effectiveness for particular GD symptoms. Combination of the key biomarker of GD, Lyso-Gb1, with other biomarkers can offer improved response assessment to long-term therapy.

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Section: Discussioncontrasting

confidence: 59%

Long-Term Evaluation of Biomarkers in the Czech Cohort of Gaucher Patients

Malinová,

Poupětová,

Řeboun

et al. 2023

IJMS

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“…TRAP baseline was higher for the group diagnosed before 15 years than those diagnosed later, which seems to support that the TRAP level could differ as a function of age [32]. The respective median ages for the groups of patients diagnosed before and after 15 years were 21 and 40 years, which partially explains these findings.…”

Section: Discussionmentioning

confidence: 66%

Bone events and evolution of biologic markers in Gaucher disease before and during treatment

et al. 2010

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IntroductionKnown biomarkers of Gaucher-disease activity are platelets, chitotriosidase, angiotensin-converting enzyme (ACE), tartrate-resistant acid phosphatase (TRAP) and ferritin. The aim of this study was to retrospectively evaluate the frequency of bone events (BE) and biomarker changes during two periods: diagnosis to first enzyme-replacement therapy (ERT) and the latter to the closing date.MethodsBE of 62 treated patients, among the 73-patient cohort followed at Beaujon Hospital, Clichy, France, were described with Kaplan-Meier curves, and linear-mixed models were used to analyze their biomarker changes and the influence of several covariates (splenectomy, diagnosis year, genotype, age at diagnosis and sex).ResultsBE occurred before (54 events in 21 patients), but also during, ERT (12 events in 10 patients), with respective frequencies (95% confidence interval) at 10 years of 22.4% (13.3 to 36.3) and 20.0% (10.2 to 36.9). Biomarker slope changes before and during ERT differed significantly for platelets (+190/mm3/year and 7,035/mm3/year, respectively; P < 0.0001) and ferritin (+4% and -14%; P < 0.0001). High ferritin levels and low platelet counts at ERT onset were significantly associated with BE during ERT (P = 0.019 and 0.039, respectively). Covariates significantly influenced biomarker changes (baseline and/or slope): splenectomy affected platelets (baseline and changes), TRAP changes and chitotriosidase changes; diagnosis date influenced ACE and TRAP baseline values; and genotype influenced chitotriosidase baseline and changes.ConclusionsPlatelet counts and ferritin levels and their slope changes at ERT onset seem to predict BE during treatment. Biomarker baseline values and changes are dependent on several covariables.

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“…TRAP activity was measured in plasma separated from heparinised peripheral blood (22). Acidified plasma was incubated with 5 mmol/l 4-methylumbelliferyl-phosphate substrate, prepared in 0.1 mol/l sodium citrate buffer, pH 6.0, with 0.02 mol/l tartaric acid.…”

Section: Tartrate Resistant Acid Phosphatase (Trap) Activitymentioning

confidence: 99%

T Cell Numbers Relate to Bone Involvement in Gaucher Disease

Lacerda

Arosa

Lacerda

et al. 1999

Blood Cells, Molecules, and Diseases

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ABSTRACT:The major elements of bone pathology in Gaucher disease are a failure of osteoclast and osteoblast function, resulting in osteopenia and also osteonecrosis. T lymphocytes have recently been found to be involved in the regulation of osteoblast/osteoclast activity in vitro. In the present report the peripheral blood T major lymphocyte subsets were investigated in a group of genotyped type 1 Gaucher disease patients. A total of 31 patients were studied: 21 non-splenectomized (5 N370S homozygotes) and 10 splenectomized (of whom 1 was a N370S homozygote). The results show that non-splenectomized patients present a decrease in absolute numbers of peripheral blood T lymphocytes, specially the CD4 ϩ T subset. However, when patients were analyzed with respect to the presence of bone disease, the number of CD8 ϩ T lymphocytes was found to be statistically significantly lower in patients presenting bone involvement. Furthermore, lower numbers of CD8 ϩ T lymphocytes were significantly correlated with higher levels of plasma tartrate resistant acid phosphatase (TRAP) activity, a putative marker of osteoclast cell activity. These in vivo findings are in agreement with the results reached in vitro by others.

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Age Dependency of Serum Acid Phosphatase in Controls and Gaucher Patients

Abstract: Serum acid phosphatase activity has been used as a complementary test for the diagnosis of Gaucher’s disease. This study suggests that phosphatase activity is age dependent. The use of controls in the patients’ age is strongly recommended.

Cited by 8 publications

References 1 publication

Long-Term Evaluation of Biomarkers in the Czech Cohort of Gaucher Patients

Long-Term Evaluation of Biomarkers in the Czech Cohort of Gaucher Patients

Bone events and evolution of biologic markers in Gaucher disease before and during treatment

T Cell Numbers Relate to Bone Involvement in Gaucher Disease

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