Bacteriophages of Clostridium botulinum

1972 ◽  
Vol 18 (1) ◽  
pp. 67-76 ◽  
Author(s):  
C. E. Dolman ◽  
Eva Chang
Keyword(s):  
Clostridium Botulinum ◽  
Phage Type ◽  
Phage Group ◽  
Flexible Tail ◽  
Group 4 ◽  
Group 3 ◽  
Type C ◽  
Group 2 ◽  
Group 1

Temperate bacteriophages of diverse morphology were demonstrated by electron microscopy in toxigenic cultures of Clostridium botulinum. The 41 strains examined included 23 type E and multiple representatives of all other types. Cultures induced with mitomycin-C generally gave better yields, but phages were also demonstrable in untreated cultures.A provisional grouping of toxigenic types into four categories is suggested, based mainly upon associated phage patterns. Group 1 comprises types A, B, and F (all proteolytic), many of whose cultures showed an icosahedral contractile phage; others contained a "bullrushy" phage with elongated head and long flexible tail; some strains yielded both. Group 2, types B and F (non-proteolytic), were associated with icosahedral contractile phages; the latter also had an octahedral flexible phage. Group 3, types C and D, yielded conspicuously large phages with octahedral heads and very long sheathed tails. One type C strain produced a long-tailed icosahedral phage. Type E phages constituted group 4. These were icosahedral with tails generally contracted but sometimes flexible, often accompanied by superfluous sheathed tail-like structures resembling certain bacteriocins. Although non-toxigenic "OS" mutants of types A, B, E, and F were phageless, two non-toxic type E strains yielded phages. The possible role of lysogeny in the toxigenicity of certain types of this species is likely to prove difficult to elucidate.

P5392Epicardium derived cells promote sympathetic ganglionic outgrowth towards myocardium in vitro

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
Y Ge ◽  
A M Smits ◽  
J C Van Munsteren ◽  
T Van Herwaarden ◽  
A M D Vegh ◽  
...  
Keyword(s):  
Neurite Outgrowth ◽  
Autonomic Innervation ◽  
Cardiac Damage ◽  
Group 4 ◽  
Group 3 ◽  
Cervical Ganglia ◽  
Group 2 ◽  
Group 1

Abstract Background The autonomic nerve system is essential to maintain homeostasis in the body. In the heart, autonomic innervation is important for adjusting the physiology to the continuously changing demands such as stress responses. After cardiac damage, excessive neurite outgrowth, referred to as autonomic hyperinnervation, can occur which is related to ventricular arrhythmias and sudden cardiac death. The cellular basis for this hyperinnervation is as yet unresolved. Here we hypothesize a role for epicardium derived cells (EPDCs) in stimulating sympathetic neurite outgrowth. Purpose To investigate the potential role of adult EPDCs in promoting sympathetic ganglionic outgrowth towards adult myocardium. Method Fetal murine superior cervical ganglia were dissected and co-cultured with activated adult mesenchymal epicardium-derived cells (EPDCs) or/and adult myocardium in a 3D collagen gel culture system. Four experiment groups were included: Group 1: Vehicle cultures (ganglia cultured without EPDC/myocardium) (n=48); Group 2: ganglia co-cultured with EPDCs (n=38); Group 3: ganglia co-cultured with myocardium (n=95); and group 4: ganglia co-cultured with both EPDCs and myocardium (n=96). The occurrence of neurite outgrowth was assessed in each group. The density of neurites that showed directional sprouting (i.e. sprouting towards myocardium) was assessed as well with a semi-automatic quantification method. Finally, sub-analyses were made by taking gender into account. Results Cervical ganglia cultured with EPDCs alone (group 2) showed increased neurite outgrowth compared to vehicle cultures (group 1), however the neurites did not show directional sprouting towards EPDCs. When co-cultured with myocardium (group 3), directional neurite outgrowth towards myocardium was observed. Compared to the ganglia-myocardium co-cultures, directional outgrowth was significantly increased in co-cultures combining myocardium and EPDCs (group 4), and the neurite density was also significantly augmented. Comparison between males and female ganglia demonstrated that more neurite outgrowth occurred in female-derived ganglia than in male-derived ganglia under the same co-culture conditions. Conclusion Activated adult EPDCs promote sympathetic ganglionic outgrowth in vitro. Sex differences exist in the response of ganglia to EPDCs, and female-derived ganglia appear more sensitive to EPDC-signalling. Results support a role of EPDCs in cardiac autonomic innervation and open avenues for exploring of their role in ventricular hyperinnervation after cardiac damage.

P–110 Does the Body Mass Index affect sperm quality?

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
I Badalotti-Teloken ◽  
C Teloken ◽  
V Dornelles ◽  
A Arent ◽  
A Petracco ◽  
...  
Keyword(s):  
Negative Impact ◽  
Sperm Quality ◽  
The Body ◽  
Semen Parameters ◽  
Healthy Weight ◽  
Group 4 ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Abstract Study question Does the body mass index (BMI) have an impact on semen analysis results? Summary answer The increase in BMI has a negative impact on sperm motility. What is known already Obesity is an increasingly prevalent health condition worldwide and can affect male fertility in various ways. It is known that obesity can cause testicular inflammation, higher testicular temperature, hypogonadism, sperm DNA fragmentation, and erectile dysfunction. However, there are still conflicting data regarding the correlation between BMI and semen parameters in the seminal analysis. Study design, size, duration Observational, cross-sectional, retrospective study using data from 1147 patients seen at a private infertility clinic between 2010 and 2020. The data were collected from electronic records in a prospective database. Participants/materials, setting, methods Patients were divided according to BMI (healthy weight, overweight, obesity classes I, II, III), and their seminal profiles were compared, according to 2010’s World Health Organization’s parameters. Cancer, cryptorchidism, viral orchitis, altered karyotype, Y chromosome microdeletions, vasectomy reversion, and testosterone use were excluding factors. Student t-tests and multiple linear regression were used for statistical analysis. The results were adjusted for age, alcohol, tobacco, and drug use, medication intake, physical activity, comorbidities, and scrotum heat factors. Main results and the role of chance From a total of 1384 patients, 219 were excluded. The BMI varied between 18,9 and 50,8 kg/m². From the 1147 patients, 297 had BMI 18.5–24.9 kg/m² (healthy weight, group 1), 611 had BMI 25–29.9 kg/m² (overweight, group 2), 179 had BMI 30–34.5 kg/m² (obese, group 3), 60 had BMI ≥ 35 kg/m² (extremely obese, group 4). The mean age for groups 1 through 4 was 37.6, 38.5, 38.2, and 36.5 years old. The comparison of the groups’ seminal parameters shows a significant decrease in progressive and total motility in patients with BMI ≥ 35 kg/m².The progressive motility was 43.8% in group 1, 44.1% in group 2, 42.4% in group 3, and 35.2% in group 4 (p = 0.07) and the total motility was 54.4%, 54.1%, 53.6%, and 45.9%, respectively (p = 0.012). The complementary analysis determined BMI 29 kg/m² as the cutoff for negative impact on progressive motility (p = 0.044) and 31 kg/m² on total motility (p = 0.036). The results were still significant after age, use of cannabis, and hypertension adjustments – the other possible interfering factors were not significant. Limitations, reasons for caution Besides the fact that this was a retrospective study, it also has a smaller sample size of patients with extreme obesity. This is probably related to the fact that the patients seeking reproductive treatment in a private clinic have a greater purchasing power and lower prevalence of obesity. Wider implications of the findings: In this study, sperm quality is negatively affected by BMI, with impairment since 29 kg/m² for progressive and 31 kg/m² for total motility. Our data support the potential deleterious role of obesity on semen parameters, reinforcing the importance of weight control in infertility prevention. Trial registration number Not applicable

scholarly journals Exploring the Pathological Role of Collagen in Paravertebral Muscle in the Progression of Idiopathic Scoliosis

2020 ◽  
Vol 2020 ◽  
pp. 1-12
Author(s):  
Haidong Peng ◽  
Feng Jin ◽  
Depeng Meng ◽  
Jun Li ◽  
Shuhan Yu ◽  
...  
Keyword(s):  
Idiopathic Scoliosis ◽  
Cobb Angle ◽  
Muscle Stiffness ◽  
Concave Side ◽  
Paravertebral Muscle ◽  
Group 4 ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Background. Paravertebral muscle (PVM) is considered as a contributing factor of idiopathic scoliosis (IS); collagen is crucial for maintaining the mechanical properties of PVM, but only a few researches have described this field. In this study, we observed the muscle stiffness of PVM and the curvature of the spine by adjusting the content of collagen in PVM of rats and explored the role of collagen in the progression of IS. Methods. 32 female Sprague Dawley rats were randomly divided into four groups: neutralizing antibody (NA) group (group 1), normal control group (group 2), IS group (group 3), and IS with NA group (group 4). TGF-β1 NA was injected into PVM in group 1 and group 4, while Normal saline in group 2 and group 3. The Cobb angle and muscle stiffness were measured before and after injection; the rats were sacrificed at one week after injection, and performed histological, Western Blot, and qRT-PCR examinations. Results. X-rays showed that scoliosis occurred in group 1 and relieved in group 4. The stiffness of PVM was decreased significantly on the convex side in group 1, while on the concave side in group 4. The expression of TGF-β1 and COL1 on the concave side in IS rats (group 3) was significantly increased than that in normal rats (group 2), the concentration of COL1 and COL3 in group 3 was significantly higher than that in group 2, and the addition of TGF-β1 NA significantly downregulated COL1 and COL3 in group 1 and group 4. The concentration of COL1 in convex PVM was negatively related to Cobb angle in group 1 and group 2, and in concave PVM was positively related to Cobb angle in group 3 and group 4. However, no significant correlation was found between COL3 and Cobb angle in group 3 and group 4. Conclusions. Asymmetric biomechanical characteristics of PVM was an important etiological factor of IS, which was directly correlated with collagen, it could be adjusted by local intramuscular injecting of TGF-β1 NA, and finally had an effect on the shape of the spine.

Role of TiF4 in Microleakage of Silorane and Methacrylatebased Composite Resins in Class V Cavities

2016 ◽  
Vol 17 (3) ◽  
pp. 240-247
Author(s):  
Zahra Jowkar ◽  
Fatemeh Koohpeima ◽  
Farahnaz Sharafeddin ◽  
Samaneh Ahmadzadeh ◽  
Mohammad Javad Mokhtari ◽  
...  
Keyword(s):  
Resin Composite ◽  
Composite Resins ◽  
Class V ◽  
Group 4 ◽  
The Social ◽  
Significant Difference ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

ABSTRACT Aim This study investigated the effect of TiF4 solution pretreatment on microleakage of silorane and nanofilled methacrylatebased composites in class V cavities. Materials and methods Forty-eight intact premolar teeth were randomly allocated to four groups of 12 teeth. Restorative techniques after standard class V tooth preparations were as follows: Group 1, Filtek P90 composite; group 2, Filtek Z350 XT; group 3, TiF4 solution pretreatment and Filtek P90 composite; group 4, TiF4 solution pretreatment and Filtek Z350 XT. After storing the specimens in distilled water at 37°C for 24 hours and followed by immersion of the specimens in a 0.5% basic-fuchsin solution for 24 hours, they were sectioned buccolingually to obtain four surfaces for each specimen for analysis of microleakage using a stereomicroscope. Data analysis was performed using Kruskal-Wallis test to compare the four groups and the Mann- Whitney test for paired comparisons with Statistical Package for the Social Sciences (SPSS) version 17 software. Results At the enamel margins, microleakage score of the Filtek Z350 XT group was lower than those of the Filtek P90 with and without the application of the TiF4 (p = 0.009 and p = 0.031 respectively). At the dentin margins, groups 3 and 4 (TiF4+Filtek P90 and TiF4+Filtek z350 XT respectively) showed significantly lower microleakage than group 1 (Filtek P90). However, there was no significant difference between other groups (p > 0.05). Conclusion At the enamel margins, microleakage score of the silorane-based composite was more than that of the nanofilled composite. No significant differences were observed between the other groups. At the dentin margins, for the silorane-based composite restorations, TiF4 solution pretreatment resulted in significantly lower microleakage. However, the similar result was not observed for Filtek Z350 XT. Also, no significant difference was observed between microleakage scores of Filtek P90 and Filtek Z350 XT with or without TiF4 pretreatment. Clinical significance In spite of better mechanical and physical properties of modern composites than earlier methacrylate-based composites, polymerization shrinkage has been remaining as one of the main shortcomings of them. Different methods, such as using new low shrinkage resin composites and different dentin pretreatments, have been suggested to overcome this problem. This study evaluated the effect of TiF4 as pretreatment on microleakage of class V tooth preparations restored with a nanocomposite and a silorane-based resin composite. How to cite this article Koohpeima F, Sharafeddin F, Jowkar Z, Ahmadzadeh S, Mokhtari MJ, Azarian B. Role of TiF4 in Microleakage of Silorane and Methacrylate-based Composite Resins in Class V Cavities. J Contemp Dent Pract 2016;17(3):240-247.

In-vivo evaluation of the effect of cyanoacrylate on prosthetic vascular graft infection – does cyanoacrylate increase the severity of infection?

VASA ◽  
2020 ◽  
Vol 49 (4) ◽  
pp. 281-284
Author(s):  
Atıf Yolgosteren ◽  
Gencehan Kumtepe ◽  
Melda Payaslioglu ◽  
Cuneyt Ozakin
Keyword(s):  
Vascular Graft ◽  
Graft Infection ◽  
Vascular Graft Infection ◽  
Group 4 ◽  
Significant Difference ◽  
Group 3 ◽  
Group 2 ◽  
Prosthetic Vascular Graft Infection ◽  
Group 1

Summary. Background: Prosthetic vascular graft infection (PVGI) is a complication with high mortality. Cyanoacrylate (CA) is an adhesive which has been used in a number of surgical procedures. In this in-vivo study, we aimed to evaluate the relationship between PVGI and CA. Materials and methods: Thirty-two rats were equally divided into four groups. Pouch was formed on back of rats until deep fascia. In group 1, vascular graft with polyethyleneterephthalate (PET) was placed into pouch. In group 2, MRSA strain with a density of 1 ml 0.5 MacFarland was injected into pouch. In group 3, 1 cm 2 vascular graft with PET piece was placed into pouch and MRSA strain with a density of 1 ml 0.5 MacFarland was injected. In group 4, 1 cm 2 vascular graft with PET piece impregnated with N-butyl cyanoacrylate-based adhesive was placed and MRSA strain with a density of 1 ml 0.5 MacFarland was injected. All rats were scarified in 96th hour, culture samples were taken where intervention was performed and were evaluated microbiologically. Bacteria reproducing in each group were numerically evaluated based on colony-forming unit (CFU/ml) and compared by taking their average. Results: MRSA reproduction of 0 CFU/ml in group 1, of 1410 CFU/ml in group 2, of 180 200 CFU/ml in group 3 and of 625 300 CFU/ml in group 4 was present. A statistically significant difference was present between group 1 and group 4 (p < 0.01), between group 2 and group 4 (p < 0.01), between group 3 and group 4 (p < 0.05). In terms of reproduction, no statistically significant difference was found in group 1, group 2, group 3 in themselves. Conclusions: We observed that the rate of infection increased in the cyanoacyrylate group where cyanoacrylate was used. We think that surgeon should be more careful in using CA in vascular surgery.

Does Perioperative Hemoglobin A1c Level Affect the Incidence, Pattern and Mortality of Lower Extremity Amputation?

2019 ◽  
Vol 17 (4) ◽  
pp. 354-364
Author(s):  
Hassan Al-Thani ◽  
Moamena El-Matbouly ◽  
Maryam Al-Sulaiti ◽  
Noora Al-Thani ◽  
Mohammad Asim ◽  
...  
Keyword(s):  
Glycemic Control ◽  
Lower Extremity ◽  
Hazard Ratio ◽  
Lower Extremity Amputation ◽  
Group 4 ◽  
Group 3 ◽  
Group 5 ◽  
Group 2 ◽  
Extremity Amputation ◽  
Group 1

Background: We hypothesized that perioperative HbA1c influenced the pattern and outcomes of Lower Extremity Amputation (LEA). Methods: A retrospective analysis was conducted for all patients who underwent LEA between 2000 and 2013. Patients were categorized into 5 groups according to their perioperative HbA1c values [Group 1 (<6.5%), Group 2 (6.5-7.4%), Group 3 (7.5-8.4%), Group 4 (8.5-9.4%) and Group 5 (≥9.5%)]. We identified 848 patients with LEA; perioperative HbA1c levels were available in 547 cases (Group 1: 18.8%, Group 2: 17.7%, Group 3: 15.0%, Group 4: 13.5% and Group 5: 34.9%). Major amputation was performed in 35%, 32%, 22%, 10.8% and 13.6%, respectively. Results: The overall mortality was 36.5%; of that one quarter occurred during the index hospitalization. Mortality was higher in Group 1 (57.4%) compared with Groups 2-5 (46.9%, 38.3%, 36.1% and 31.2%, respectively, p=0.001). Cox regression analysis showed that poor glycemic control (Group 4 and 5) had lower risk of mortality post-LEA [hazard ratio 0.57 (95% CI 0.35-0.93) and hazard ratio 0.46 (95% CI 0.31-0.69)]; this mortality risk persisted even after adjustment for age and sex but was statistically insignificant. The rate of LEA was greater among poor glycemic control patients; however, the mortality was higher among patients with tight control. Conclusion: The effects of HbA1c on the immediate and long-term LEA outcomes and its therapeutic implications need further investigation.

Association between cervical disc disease and lesions of multiple sclerosis

2021 ◽  
pp. 197140092098356
Author(s):  
Marwan Alkrenawi ◽  
Michael Osherov ◽  
Azaria Simonovich ◽  
Jonathan Droujin ◽  
Ron Milo ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Cervical Disc ◽  
Cervical Disc Disease ◽  
Disc Disease ◽  
Group 4 ◽  
Demyelinating Lesions ◽  
Grade Ii ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Background Cervical discopathy and demyelinating lesions often co-exist in patients with multiple sclerosis (MS). Our study examines the possible association between these two pathologies. Methods Medical records and cervical magnetic resonance imaging scans of MS patients with cervical discopathy who were seen at our MS clinic during 2018 were retrospectively reviewed. The severity of the disc disease was classified as grade I (no compression), grade II (compression of the dural sac) and grade III (cord compression). The spinal cord in each scan was divided into six segments corresponding to the intervertebral space of the spine (C1–C6). Each segment was defined as containing demyelinating lesion and disc pathology (group 1), demyelinating lesion without disc pathology (group 2), disc pathology without demyelinating lesion (group 3) and no demyelinating lesion or disc pathology (group 4). Fisher’s exact test was used to test the association between demyelinating lesions and disc pathology. Results Thirty-four MS patients with cervical discopathy were included in the study (26 females; average age 42.9 ± 13.7 years; average disease duration 8.4 ± 5.4 years). A total of 204 spinal cord segments were evaluated. Twenty-four segments were classified as group 1, 27 segments as group 2, 52 segments as group 3 and 101 segments as group 4. There was no association between demyelinating lesions and the grade of disc disease ( p = 0.1 for grade I, p = 0.3 for grade II and p = 1 for grade III disc disease). Conclusion Our study did not find any association between cervical disc disease and demyelinating spinal cord lesion.

scholarly journals Association between serum oestradiol level on the hCG administration day and neonatal birthweight after IVF-ET among 3659 singleton live births

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yu Liu ◽  
Jing Li ◽  
Wanyu Zhang ◽  
Yihong Guo
Keyword(s):  
Birth Weight ◽  
Ovarian Stimulation ◽  
Group 4 ◽  
Group 6 ◽  
Group 3 ◽  
Group 5 ◽  
Group 2 ◽  
The Impact ◽  
Ivf Et ◽  
Group 1

AbstractOestradiol, an important hormone in follicular development and endometrial receptivity, is closely related to clinical outcomes of fresh in vitro fertilization-embryo transfer (IVF-ET) cycles. A supraphysiologic E2 level is inevitable during controlled ovarian hyper-stimulation (COH), and its effect on the outcome of IVF-ET is controversial. The aim of this retrospective study is to evaluate the association between elevated serum oestradiol (E2) levels on the day of human chorionic gonadotrophin (hCG) administration and neonatal birthweight after IVF-ET cycles. The data of 3659 infertile patients with fresh IVF-ET cycles were analysed retrospectively between August 2009 and February 2017 in First Hospital of Zhengzhou University. Patients were categorized by serum E2 levels on the day of hCG administration into six groups: group 1 (serum E2 levels ≤ 1000 pg/mL, n = 230), group 2 (serum E2 levels between 1001 and 2000 pg/mL, n = 524), group 3 (serum E2 levels between 2001 and 3000 pg/mL, n = 783), group 4 (serum E2 levels between 3001 and 4000 pg/mL, n = 721), group 5 (serum E2 levels between 4001 and 5000 pg/mL, n = 548 ), and group 6 (serum E2 levels > 5000 pg/mL, n = 852). Univariate linear regression was used to evaluate the independent correlation between each factor and outcome index. Multiple logistic regression was used to adjust for confounding factors. The LBW rates were as follows: 3.0% (group 1), 2.9% (group 2), 1.9% (group 3), 2.9% (group 4), 2.9% (group 5), and 2.0% (group 6) (P = 0.629), respectively. There were no statistically significant differences in the incidences of neonatal LBW among the six groups. We did not detect an association between peak serum E2 level during ovarian stimulation and neonatal birthweight after IVF-ET. The results of this retrospective cohort study showed that serum E2 peak levels during ovarian stimulation were not associated with birth weight during IVF cycles. In addition, no association was found between higher E2 levels and increased LBW risk. Our observations suggest that the hyper-oestrogenic milieu during COS does not seem to have adverse effects on the birthweight of offspring after IVF. Although this study provides some reference, the obstetric-related factors were not included due to historical reasons. The impact of the high estrogen environment during COS on the birth weight of IVF offspring still needs future research.

scholarly journals Control of Vulval Competence and Centering in the Nematode Oscheius sp. 1 CEW1

Genetics ◽  
2003 ◽  
Vol 163 (1) ◽  
pp. 133-146 ◽  
Author(s):  
Sophie Louvet-Vallée ◽  
Irina Kolotuev ◽  
Benjamin Podbilewicz ◽  
Marie-Anne Félix
Keyword(s):  
Germ Line ◽  
Specific Mechanism ◽  
C Elegans ◽  
Group 4 ◽  
Cell Ablation ◽  
Group 3 ◽  
Group 2 ◽  
P Cells ◽  
Large Category ◽  
Group 1

Abstract To compare vulva development mechanisms in the nematode Oscheius sp. 1 to those known in Caenorhabditis elegans, we performed a genetic screen for vulva mutants in Oscheius sp. 1 CEW1. Here we present one large category of mutations that we call cov, which affect the specification of the Pn.p ventral epidermal cells along the antero-posterior axis. The Pn.p cells are numbered from 1 to 12 from anterior to posterior. In wild-type Oscheius sp. 1 CEW1, the P(4-8).p cells are competent to form the vulva and the progeny of P(5-7).p actually form the vulva, with the descendants of P6.p adopting a central vulval fate. Among the 17 mutations (defining 13 genes) that we characterize here, group 1 mutations completely or partially abolish P(4-8).p competence, and this correlates with early fusion of the Pn.p cells to the epidermal syncytium. In this group, we found a putative null mutation in the lin-39 HOM-C homolog, the associated phenotype of which could be weakly mimicked by injection of a morpholino against Osp1-lin-39 in the mother’s germ line. Using cell ablation in a partially penetrant competence mutant, we show that vulval competence is partially controlled by a gonadal signal. Most other mutants found in the screen display phenotypes unknown in C. elegans. Group 2 mutants show a partial penetrance of Pn.p competence loss and an abnormal centering of the vulva on P5.p, suggesting that these two processes are coregulated by the same pathway in Oscheius sp. 1. Group 3 mutants display an enlarged competence group that includes P3.p, thus demonstrating the existence of a specific mechanism inhibiting P3.p competence. Group 4 mutants display an abnormal centering of the vulval pattern on P7.p and suggest that a specific mechanism centers the vulval pattern on a single Pn.p cell.

scholarly journals Hypertrophy of unaffected cardiomyocytes correlates with severity of cardiomyopathy in female patients with Fabry disease

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Cristina Chimenti ◽  
Romina Verardo ◽  
Andrea Frustaci
Keyword(s):  
Fabry Disease ◽  
Wall Thickness ◽  
Left Ventricular ◽  
Female Patients ◽  
Maximal Wall Thickness ◽  
Group 4 ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Abstract Aim To investigate the contribution of unaffected cardiomyocytes in Fabry disease cardiomyopathy. Findings Left ventricular (LV) endomyocardial biopsies from twenty-four females (mean age 53 ± 11 ys) with Fabry disease cardiomyopathy were studied. Diagnosis of FD was based on the presence of pathogenic GLA mutation, Patients were divided in four groups according with LV maximal wall thickness (MWT): group 1 MWT ≤ 10.5 mm, group 2 MWT 10.5–15 mm, group 3 MWT 16–20 mm, group 4 MWT > 20 mm. At histology mosaic of affected and unaffected cardiomyocytes was documented. Unaffected myocytes’ size ranged from normal to severe hypertrophy. Hypertrophy of unaffected cardiomyocytes correlated with severity of MWT (p < 0.0001, Sperman r 0,95). Hypertrophy of unaffected myocytes appear to concur to progression and severity of FDCM. It is likely a paracrine role from neighboring affected myocytes.

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