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Plastic and Reconstructive Surgery Feb 2024Dupuytren disease is associated with significant comorbidity and mortality, and it has no existing prevention strategies. It is unclear which modifiable risk factors are...
BACKGROUND
Dupuytren disease is associated with significant comorbidity and mortality, and it has no existing prevention strategies. It is unclear which modifiable risk factors are most amenable for prevention. This study aimed to determine the strength of modifiable risk factors for Dupuytren disease, and to investigate associations with other diseases.
METHODS
Using UK Biobank data, this case-control study analyzed the association between phenotypic variables and Dupuytren disease through multivariable logistic regression. Exposures assessed were age, sex, body mass index, waist-to-hip ratio, Townsend deprivation index, smoking status, alcohol intake, diabetes mellitus, hypertension, cancer, liver disease, respiratory disease, rheumatoid arthritis, epilepsy, psoriasis, and gout.
RESULTS
There were 4148 cases and 397,425 controls. Male sex (OR, 3.23; 95% CI, 2.90 to 3.60; P = 1.07 × 10 -100 ), increasing age (OR, 1.08; 95% CI, 1.07 to 1.08; P = 6.78 × 10 -167 ), material deprivation (OR, 1.01; 95% CI, 1.00 to 1.02; P = 0.0305), high-density lipoprotein cholesterol (OR, 1.76; 95% CI, 1.58 to 1.96; P = 3.35 × 10 -24 ), smoking exposure, and alcohol intake were all associated with increased odds of Dupuytren disease. With increasing obesity class, there was approximately 25% decreased odds (OR, 0.774; 95% CI, 0.734 to 0.816; P = 4.71 × 10 -21 ). Diabetes with microvascular or end-organ complications was associated with more than 2.5 times increased odds of Dupuytren disease (OR, 2.59; 95% CI, 1.92 to 3.44; P = 1.92 × 10 -10 ). Within this group, increasing hemoglobin A1c values by 10 mmol/mol, or 0.9%, increased the odds by 31% (OR, 1.31; 95% CI, 1.13 to 1.51; P = 2.19 × 10 -4 ).
CONCLUSION
Diabetes and poor glycemic control are major risk factors for Dupuytren disease, which present an opportunity for prevention.
CLINICAL QUESTION/LEVEL OF EVIDENCE
Risk, III.
Topics: Humans; Male; Dupuytren Contracture; Case-Control Studies; Biological Specimen Banks; UK Biobank; Risk Factors; Diabetes Mellitus
PubMed: 37257135
DOI: 10.1097/PRS.0000000000010774 -
International Journal of Clinical... 2023Hypergenetic molars are uncommon in children and adolescents. Furthermore, the presence of multiple eumorphic supernumerary molars (SNMs) in children is sporadic. The...
BACKGROUND
Hypergenetic molars are uncommon in children and adolescents. Furthermore, the presence of multiple eumorphic supernumerary molars (SNMs) in children is sporadic. The author, therefore, reports a nonsyndromic case of multiple supplemental SNMs in a child.
CASE DESCRIPTION
A ten year old boy primarily complaining of cheek biting was found to have six molars on the maxillary left quadrant. Following investigations and case discussion, four molars and fibroma were surgically removed under general anesthesia.
DISCUSSION
This paper discusses the prevalence and management of SNMs in the pediatric population while documenting the first case of multiple eumorphic SNMs in the mixed dentition.
HOW TO CITE THIS ARTICLE
Venkatesh SP. Pediatric Molar Hyperdontia: A Descriptive Case Report. Int J Clin Pediatr Dent 2023;16(5):763-768.
PubMed: 38162231
DOI: 10.5005/jp-journals-10005-2658 -
Cureus Sep 2023Cemento-ossifying fibroma is a benign fibro-osseous lesion arising from the periodontal ligament and has the potential to form cementum and bone in the periodontal...
Cemento-ossifying fibroma is a benign fibro-osseous lesion arising from the periodontal ligament and has the potential to form cementum and bone in the periodontal ligament. Cemento-ossifying fibroma is a painless, pedunculated, or sessile, smooth exophytic growth arising attached to the gingival tissues. We present a case of cemento-ossifying fibroid epulis in the posterior maxilla attached to the interdental gingiva between the 26 and 27 region buccally in a 52-year-old female patient managed with surgical excision of the lesion, extraction of the involved teeth, curettage, and palatal obturator while under general anesthesia. The patient was followed up post-operatively, healing was satisfactory, there were no signs of infection, and no recurrence was noted in the six-month follow-up period.
PubMed: 37905253
DOI: 10.7759/cureus.46167 -
Cancer Science Feb 2024Desmoid tumors (DTs), also called desmoid-type fibromatoses, are locally aggressive tumors of mesenchymal origin. In the present study, we developed a novel mouse model...
Desmoid tumors (DTs), also called desmoid-type fibromatoses, are locally aggressive tumors of mesenchymal origin. In the present study, we developed a novel mouse model of DTs by inducing a local mutation in the Ctnnb1 gene, encoding β-catenin in PDGFRA-positive stromal cells, by subcutaneous injection of 4-hydroxy-tamoxifen. Tumors in this model resembled histologically clinical samples from DT patients and showed strong phosphorylation of nuclear SMAD2. Knockout of SMAD4 in the model significantly suppressed tumor growth. Proteomic analysis revealed that SMAD4 knockout reduced the level of Cysteine-and-Glycine-Rich Protein 2 (CSRP2) in DTs, and treatment of DT-derived cells with a TGF-β receptor inhibitor reduced CSRP2 RNA levels. Knockdown of CSRP2 in DT cells significantly suppressed their proliferation. These results indicate that the TGF-β/CSRP2 axis is a potential therapeutic target for DTs downstream of TGF-β signaling.
Topics: Animals; Humans; Mice; beta Catenin; Fibromatosis, Aggressive; LIM Domain Proteins; Mice, Knockout; Muscle Proteins; Nuclear Proteins; Proteomics; Transforming Growth Factor beta; Up-Regulation
PubMed: 38041233
DOI: 10.1111/cas.16037 -
Journal of Clinical Research in... Nov 2023Tumor-induced osteomalacia (TIO) is a rare, paraneoplastic disorder of hypophosphatemia associated with elevated tumor-produced fibroblast growth factor 23 (FGF23).... (Review)
Review
Tumor-induced osteomalacia (TIO) is a rare, paraneoplastic disorder of hypophosphatemia associated with elevated tumor-produced fibroblast growth factor 23 (FGF23). Maxillofacial tumors are rarely involved in TIO, especially maxillary TIO in children. We present a 14-year-old boy with osteomalacia and high serum levels of FGF23, a hormone associated with decreased phosphate resorption, due to a maxillary tumor. The patient was treated with oral phosphorus and calcitriol, and surgical removal of the tumor was performed. After 21 months follow-up, he was pain free and had returned to full activity. We review the reported pediatric cases of TIO in the maxillofacial and oral region and discuss the management of these patients considering the published evidence.
Topics: Male; Humans; Child; Adolescent; Osteomalacia; Fibroma, Ossifying; Paraneoplastic Syndromes; Neoplasms
PubMed: 35135186
DOI: 10.4274/jcrpe.galenos.2022.2021-8-14 -
Journal of Oral Pathology & Medicine :... Sep 2023PEA3 transcription factor has been identified as a downstream target of the MAPK and PI3K pathways, and PEA3 overexpression has been observed in a variety of tumor...
BACKGROUND
PEA3 transcription factor has been identified as a downstream target of the MAPK and PI3K pathways, and PEA3 overexpression has been observed in a variety of tumor types. We aimed to evaluate PEA3 expression in odontogenic cysts and tumors and compare the expression among odontogenic lesions. In addition, the correlations between PEA3 expression and clinicopathological characteristics of conventional ameloblastoma and unicystic ameloblastoma were investigated.
METHODS
This study was performed on 165 samples of odontogenic cysts and tumors including 20 dentigerous cysts, 20 odontogenic keratocysts, 16 adenomatoid odontogenic tumors, 5 ameloblastic fibromas, 45 unicystic ameloblastomas, and 59 conventional ameloblastomas. The sections were immunohistochemically stained with mouse monoclonal anti-PEA3 antibody and PEA3 expression was evaluated as the immunoreactive score.
RESULTS
PEA3 expression was absent in all dentigerous cysts (DCs) and odontogenic keratocysts, while all adenomatoid odontogenic tumors showed either no (75%) or low (25%) expression of PEA3. Most of the ameloblastic fibromas (60%) displayed no PEA3 expression. A high expression of PEA3 was observed in a substantial number of unicystic ameloblastomas (48.9%) and conventional ameloblastomas (49.2%) in our study. PEA3 expression in DCs, odontogenic keratocysts and adenomatoid odontogenic tumors were significantly different from that in conventional ameloblastomas and that in unicystic ameloblastomas (p < 0.05). The expression of PEA3 was significantly different in the age groups of unicystic ameloblastomas and histological subtypes of conventional ameloblastomas (p < 0.05).
CONCLUSION
PEA3 overexpression is predominant in unicystic ameloblastomas and conventional ameloblastomas compared to other odontogenic lesions, indicating a pivotal role of PEA3 as a downstream effector of MAPK pathway in these two odontogenic lesions.
Topics: Ameloblastoma; Dentigerous Cyst; Fibroma; Jaw Neoplasms; Odontogenic Cysts; Odontogenic Tumors; Phosphatidylinositol 3-Kinases; Humans
PubMed: 37549030
DOI: 10.1111/jop.13476 -
Journal of Veterinary Dentistry Dec 2023A 6-month-old intact male Dalmatian mix puppy was presented for the evaluation of left maxillary swelling due to a suspected cyst and an unerupted left maxillary canine...
A 6-month-old intact male Dalmatian mix puppy was presented for the evaluation of left maxillary swelling due to a suspected cyst and an unerupted left maxillary canine tooth. Removal of the unerupted left maxillary canine tooth (204) and enucleation of the cyst was performed, followed by histological analysis, which identified the maxillary swelling to be a cystic ameloblastic fibroma. Ameloblastic fibromas are rare in companion animals, and to the best of the authors' knowledge, this is the first cystic variant reported in dogs. The clinical, radiographic, cone beam computed tomography, and histological findings of this case are discussed and compared with the findings of previously documented human and domestic animal cases.
PubMed: 38105440
DOI: 10.1177/08987564231219100 -
Mymensingh Medical Journal : MMJ Apr 2024Hand Surgery is a specialized branch of Plastic and Reconstructive surgery. There are many conditions that require hand surgery, for example, congenital deformity,... (Observational Study)
Observational Study
Hand Surgery is a specialized branch of Plastic and Reconstructive surgery. There are many conditions that require hand surgery, for example, congenital deformity, electric or flame burn, mechanical or road traffic injury, and post burn or post traumatic deformity. A retrospective observational study was conducted in the department of Burn and Plastic surgery, Mymensingh Medical College Hospital, Bangladesh during a 2 years period extending from 9th September 2021 to 8th September 2023. The objective of this study was to see the hand surgery status in a tertiary hospital of Bangladesh during the post Covid pandemic period. During this period 236 hand surgery procedures were performed in 176 patients. The age of the patients ranged from 02 to 78 years (Mean 31.14±1.52). One hundred and four (59.0%) were male and 72(41.0%) were female. Thirty-four (19.32%) patients had co-morbidities e.g., Epilepsy, Diabetes Mellitus, Chronic Kidney Diseases and HBsAg +ve. Causes of surgery included, wound due to electric burn 49(27.84%), flame burn 36(20.45%), post traumatic 24(13.64%), post infective 11(06.25%), tumor excision 02(2.24%), Dupuytren's contracture 03(1.70%), congenital anomalies 06(3.41%), post burn scar contractures 41(23.29%), nerve injury 01(00.57%) and carpal tunnel syndrome 01 (00.57%). Procedures were performed: post burn scar contracture release 41(17.37%), syndactly release 06(2.54%), release of post traumatic contracture 06(2.54%), carpal tunnel release 01(00.42%), release of Dupuytren's contracture 03(01.27%), nerve repair 01(00.42%), debridement, amputation and Fillet flap 29(12.29%), split thickness skin graft 46 (19.49%), V-Y advancement flap 06(2.54%), transposition flap 18(07.63%), cross finger flap 16 (06.78%), reverse cross finger flap 02 (00.85%), first dorsal metacarpal artery (FDMA) flap 05 (02.12%), reverse FDMA flap 01 (00.42%), metacarpal artery perforator flap 08(3.39%), radial artery perforator flap 04(01.69%), posterior interosseous artery flap 05(2.12%), abdominal flap 11(04.46%) and flap division and insetting 27(11.44%). Outcome of surgery was satisfactory in 225(95.34%) and 11(04.46%) cases had complications (p value 0.453), which was not significant. So, it can be concluded that the outcome of various types of hand surgery procedures in tertiary hospital of northern Bangladesh during the post Covid period was satisfactory overall.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Female; Humans; Male; Middle Aged; Young Adult; Bangladesh; Cicatrix; Dupuytren Contracture; Perforator Flap; Tertiary Care Centers; Treatment Outcome; Retrospective Studies
PubMed: 38557517
DOI: No ID Found -
European Radiology Feb 2024To assess the safety and efficacy of magnetic resonance-guided focused ultrasound (MRgFUS) for the treatment extra-abdominal desmoids.
OBJECTIVE
To assess the safety and efficacy of magnetic resonance-guided focused ultrasound (MRgFUS) for the treatment extra-abdominal desmoids.
METHODS
A total of 105 patients with desmoid fibromatosis (79 females, 26 males; 35 ± 14 years) were treated with MRgFUS between 2011 and 2021 in three centers. Total and viable tumors were evaluated per patient at last follow-up after treatment. Response and progression-free survival (PFS) were assessed with (modified) response evaluation criteria in solid tumors (RECIST v.1.1 and mRECIST). Change in Numerical Rating Scale (NRS) pain and 36-item Short Form Health Survey (SF-36) scores were compared. Treatment-related adverse events were recorded.
RESULTS
The median initial tumor volume was 114 mL (IQR 314 mL). After MRgFUS, median total and viable tumor volume decreased to 51 mL (95% CI: 30-71 mL, n = 101, p < 0.0001) and 29 mL (95% CI: 17-57 mL, n = 88, p < 0.0001), respectively, at last follow-up (median: 15 months, 95% CI: 11-20 months). Based on total tumor measurements (RECIST), 86% (95% CI: 75-93%) had at least stable disease or better at last follow-up, but 50% (95% CI: 38-62%) of remaining viable nodules (mRECIST) progressed within the tumor. Median PFS was reached at 17 and 13 months for total and viable tumors, respectively. NRS decreased from 6 (IQR 3) to 3 (IQR 4) (p < 0.001). SF-36 scores improved (physical health (41 (IQR 15) to 46 (IQR 12); p = 0.05, and mental health (49 (IQR 17) to 53 (IQR 9); p = 0.02)). Complications occurred in 36%, most commonly 1/2 degree skin burns.
CONCLUSION
MRgFUS reduced tumor volume, reduced pain, and improved quality of life in this series of 105 patients with extra-abdominal desmoid fibromatosis.
CLINICAL RELEVANCE STATEMENT
Imaging-guided ablation is being increasingly used as an alternative to surgery, radiation, and medical therapy for the treatment of desmoid fibromatosis. MR-guided high-intensity focused ultrasound is an incisionless ablation technique that can be used to reduce tumor burden effectively and safely.
KEY POINTS
• Desmoid fibromatosis was treated with MR-guided high-intensity focused ultrasound in 105 patients. • MR-guided focused ultrasound ablation reduced tumor volume and pain and improved quality of life. • MR-guided focused ultrasound is a treatment option for patients with extra-abdominal desmoid tumors.
Topics: Humans; Male; Female; Fibromatosis, Aggressive; Retrospective Studies; Quality of Life; High-Intensity Focused Ultrasound Ablation; Pain; Treatment Outcome
PubMed: 37615768
DOI: 10.1007/s00330-023-10073-9 -
Tumori Oct 2023Desmoid-type fibromatosis are rare intermediate tumors in children and adolescents. Owing to local aggressiveness and relapse, systemic treatment for symptomatic...
BACKGROUND
Desmoid-type fibromatosis are rare intermediate tumors in children and adolescents. Owing to local aggressiveness and relapse, systemic treatment for symptomatic advanced or progressive forms is recommended. Following promising results in adult patients, oral vinorelbine is investigated in young patients.
METHODS
A retrospective review of young patients (<25 years old) with advanced or progressive desmoid type fibromatosis treated with oral vinorelbine in eight large centers of the Société Française des Cancers de l'Enfant was performed. In addition to tumor assessment according to RECIST 1.1, pre-treatment and during-treatment imagery were reviewed centrally to assess tumor volume and estimate fibrosis score through the change in percentage in hypoT2 signal intensity.
RESULTS
From 2005 to 2020, 24 patients (median age 13.9 years [range, 1.0-23.0]) received oral vinorelbine. Median number of prior systemic lines of treatment was 1 (range, 0-2), mainly based on intravenous low dose methotrexate and vinblastine. Before vinorelbine initiation, all patients had a progressive disease: radiological for 19, radiological and clinical (pain) for three and only clinical for two. Oral vinorelbine was delivered for a median duration of 12 months (range, 1-42). The toxicity profile was favorable, with no grade 3-4 event. Overall response estimated on 23 evaluable patients according to RECIST 1.1 criteria was three partial responses (13%), 18 stabilization (78%) and two progressive disease (9%). Overall progression-free survival was 89.3% (95% confidential intervals 75.2-100) at 24 months. Four stable tumors according to standard RECIST criteria displayed a partial response with > 65% tumor volume reduction. Among 21 informative patients, the estimated fibrosis score decreased for 15 patients, was stable for four patients and increased for two patients.
CONCLUSION
Oral vinorelbine seems to be effective to control advanced or progressive desmoid type fibromatosis in young patients, with a well-tolerated profile. These results support testing this drug as first-line alone or in combination to improve response rate while preserving quality of life.
Topics: Adult; Child; Adolescent; Humans; Vinorelbine; Fibromatosis, Aggressive; Quality of Life; Antineoplastic Combined Chemotherapy Protocols; Neoplasm Recurrence, Local; Vinblastine; Fibrosis; Treatment Outcome
PubMed: 37114926
DOI: 10.1177/03008916231169806