ABCG2 Expression as a Potential Survival Predictor in Human Gliomas.

Gliomas are notably challenging to treat due to their invasive nature and resistance to conventional therapies. The ABCG2 protein has attracted attention for its role in multidrug resistance, complicating treatment effectiveness. This study scrutinized the relationship between ABCG2 expression and glioma grade and the role of ABCG2 in the process of glioma progression, aiming to evaluate ABCG2 expression as a predictive factor of tumor progression and patient survival. Conducted at Dubrava University Hospital, Zagreb, Croatia, the study analyzed 152 glioma specimens from 2013 to 2022, assessing ABCG2 expression alongside standard clinical markers. A significant association was found between patients' survival and the ABCG2 profile (p = 0.003, r = 0.24), separately for patients who underwent chemotherapy (p = 0.0004, r = 0.32) and radiotherapy (p = 0.003, r = 0.29). Furthermore, the ABCG2 profile was significantly associated with disease progression (p = 0.007, r = 0.23), tumor grade (p = 0.0002, r = 0.31), and Ki67 expression (p = 0.0004, r = 0.31). ABCG2-positive tumor cells only showed association with Ki67 expression (p = 0.002, r = 0.28). The ABCG2 profile was found to affect the overall patient survival (p = 0.02) and represent a moderate indicator of tumor progression (p = 0.01), unlike the percentage of ABCG2-positive tumor cells. ABCG2 may serve as a marker of angiogenesis and vascular abnormalities within tumors, predicting glioma progression and treatment response. Targeting ABCG2 could enhance chemoradiotherapy efficacy and improve patient outcomes, which highlights its value in assessing tumor aggressiveness and designing treatment strategies.

Necrotic Cells from Head and Neck Carcinomas Release Biomolecules That Are Activating Toll-like Receptor 3.

Tumor necrosis is a recurrent characteristic of head and neck squamous cell carcinomas (HNSCCs). There is a need for more investigations on the influence of biomolecules released by these necrotic foci in the HNSCC tumor microenvironment. It is suspected that a fraction of the biomolecules released by necrotic cells are damage-associated molecular patterns (DAMPs), which are known to be natural endogenous ligands of Toll-like receptors (TLRs), including, among others, proteins and nucleic acids. However, there has been no direct demonstration that biomolecules released by HNSCC necrotic cells can activate TLRs. Our aim was to investigate whether some of these molecules could behave as agonists of the TLR3, either in vitro or in vivo. We chose a functional approach based on reporter cell exhibiting artificial TLR3 expression and downstream release of secreted alkaline phosphatase. The production of biomolecules activating TLR3 was first investigated in vitro using three HNSCC cell lines subjected to various pronecrotic stimuli (external irradiation, serum starvation, hypoxia and oxidative stress). TLR3 agonists were also investigated in necrotic tumor fluids from five oral cancer patients and three mouse tumor grafts. The release of biomolecules activating TLR3 was demonstrated for all three HNSCC cell lines. External irradiation was the most consistently efficient stimulus, and corresponding TLR3 agonists were conveyed in extracellular vesicles. TLR3-stimulating activity was detected in the fluids from all five patients and three mouse tumor grafts. In most cases, this activity was greatly reduced by RNAse pretreatment or TLR3 blocking antibodies. Our data indicate that TLR3 agonists are consistently present in necrotic fluids from HNSCC cells and mainly made of dsRNA fragments. These endogenous agonists may induce TLR3, which might lead to a protumorigenic effect. Regarding methodological aspects, our study demonstrates that direct investigations-including functional testing-can be performed on necrotic fluids from patient tumors.

Preliminary Comparison of Molecular Antioxidant and Inflammatory Mechanisms Determined in the Peripheral Blood Granulocytes of COVID-19 Patients.

The aim of this study was to evaluate selected parameters of redox signaling and inflammation in the granulocytes of COVID-19 patients who recovered and those who died. Upon admission, the patients did not differ in terms of any relevant clinical parameter apart from the percentage of granulocytes, which was 6% higher on average in those patients who died. Granulocytes were isolated from the blood of 15 healthy people and survivors and 15 patients who died within a week, and who were selected post hoc for analysis according to their matching gender and age. They differed only in the lethal outcome, which could not be predicted upon arrival at the hospital. The proteins level (respective ELISA), antioxidant activity (spectrophotometry), and lipid mediators (UPUPLC-MS) were measured in the peripheral blood granulocytes obtained via gradient centrifugation. The levels of Nrf2, HO-1, NFκB, and IL-6 were higher in the granulocytes of COVID-19 patients who died within a week, while the activity of cytoplasmic Cu,Zn-SOD and mitochondrial Mn-SOD and IL-2/IL-10 were lower in comparison to the levels observed in survivors. Furthermore, in the granulocytes of those patients who died, an increase in pro-inflammatory eicosanoids (PGE2 and TXB2), together with elevated cannabinoid receptors 1 and 2 (associated with a decrease in the anti-inflammatory 15d-PGJ2), were found. Hence, this study suggests that by triggering transcription factors, granulocytes activate inflammatory and redox signaling, leading to the production of pro-inflammatory eicosanoids while reducing cellular antioxidant capacity through SOD, thus expressing an altered response to COVID-19, which may result in the onset of systemic oxidative stress, ARDS, and the death of the patient.

Dynamometric outcomes of the donor site leg after vastus lateralis free flap harvest.

The vastus lateralis muscle is one of the four muscles that make up the quadriceps femoris muscle, and it is also the largest of them. Some studies have shown that patients experience lower quality of life and muscle weakness after surgical treatment of thigh muscles in order to perform reconstructive surgery of the head and neck. The aim of our study was to assess the quantitative and qualitative function of the lower extremities using an isokinetic dynamometer and a validated questionnaire on subjective difficulties in patients who underwent reconstructive surgery with the vastus lateralis free flap. Fourteen participants aged 20-70 years who suffered from malignant tumor in the head and neck region. The free vastus lateralis flap was used for reconstruction. All patients underwent isokinetic dynamometric measurement with the isokinetic dynamometer to test the isokinetic functions of the thigh muscles after surgery. In addition, subjective assessment of the lower extremities was performed using the validated questionnaire Lower Extremity Functional Scale. All isometric dynamometer measurements of the donor leg were compared with those of the unoperated leg. Peak torque and average power were significantly lower in the operated leg compared with the unoperated leg after 60°/s extension (p = 0.018 for peak torque, p = 0.021 for average power) and 180°/s extension (p = 0.019 for peak torque, p = 0.015 for average power). On the other hand, there was no statistically significant difference in dynamometer measurements after 60°/s flexion (p = 0.700 for peak torque, p = 0.854 for average power, and 180°/s flexion (p = 0.634 for peak torque, p = 0.571 for average power). The median value for the LEFS was 65.5 (40.00-71.25). The results of this study showed that there is a significant deterioration regarding the biophysical properties of the operated leg after harvesting vastus lateralis free flap.

Hemoglobin-Albumin-Lymphocyte-Platelet (HALP) Score as a Predictive Model for the Success of Reconstruction of Head and Neck Defects with Free Microvascular Flaps.

Significant advances in reconstructive head and neck surgery with free microvascular flaps have had a positive impact on esthetic outcomes and quality of life. However, complications still occur in some patients. This study investigated the influence of the Hemoglobin, Albumin, Lymphocyte, and Platelet Score (HALP score), an immunonutritive marker, on complications and flap success. The retrospective analysis included 194 patients who underwent reconstruction of head and neck defects with free microvascular flaps. The HALP score correlated strongly with overall complications, including flap necrosis, infection, fistula, and hematoma. Hemoglobin, albumin, lymphocytes, and platelets individually showed associations with specific complications. HALP score was an extremely strong predictor of complications (AUC = 0.85). HALP score may be valuable for assessing patient status and predicting complications in microvascular free-flap reconstruction to allow timely interventions and improve outcomes. Further research is needed to investigate additional predictors and improve postoperative care.

Nuclear Epidermal Growth Factor Receptor Overexpression as a Survival Predictor in Oral Squamous Cell Carcinoma.

The aim of this study was to determine, by immunohistochemical methods, the expression of nEGFR and markers of cell proliferation (Ki-67), cell cycle (mEGFR, p53, cyclin D1), and tumor stem cells (ABCG2) in 59 pathohistological samples of healthy oral mucosa, 50 oral premalignant changes (leukoplakia and erythroplakia), and 52 oral squamous cell carcinomas (OSCC). An increase in the expression of mEGFR and nEGFR was found with the development of the disease (p < 0.0001). In the group of patients with leukoplakia and erythroplakia, we found a positive correlation between nEGFR and Ki67, p53, cyclin D1, and mEGFR, whereas in the group of patients with OSCC, we found a positive correlation between nEGFR and Ki67, mEGFR (p < 0.05). Tumors without perineural (PNI) invasion had a higher expression of p53 protein than tumors with PNI (p = 0.02). Patients with OSCC and overexpression of nEGFR had shorter overall survival (p = 0.004). The results of this study suggest a potentially important independent role of nEGFR in oral carcinogenesis.

Retrospective Longitudinal Study on Changes in Atmospheric Pressure as a Predisposing Factor for Odontogenic Abscess Formation.

In our retrospective longitudinal study based on the data from 292 patients, we wanted to investigate whether there was an association between weather conditions and the occurrence of odontogenic abscesses (OA) requiring hospitalization. In the adult group (249 patients), the incidence of severe OA was highest in winter (32.9%) during January (11.6%), with the most common localizations being the perimandibular (35.7%) and submandibular (23.3%) regions. We found that changes in mean daily atmospheric pressure five days before hospitalization showed a positive association with the occurrence of OA, especially pressure variations greater than 12 hPa. Atmospheric pressure changes two and five days before hospitalization were also found to be moderate predictors of complications during treatment. Antibiogram analysis revealed resistance of streptococci to clindamycin in 26.3%. In the pediatric group, OA were also most frequent in winter (30.2%), and the perimandibular region (37.2%) and the canine fossa (20.9%) were the most frequent abscess localizations, while an association with meteorological parameters was not demonstrated. Clinical experience teaches us that weather change influences the occurrence of severe OA requiring hospitalization, which we confirmed in this research. To our knowledge, our study is the first to provide a threshold and precise time frame for atmospheric pressure changes.

IS SURGICAL TREATMENT OF VELOPHARYNGEAL INSUFFICIENCY BY MYOMUCOSAL POSTERIOR PHARYNGEAL FLAP A CONTRAINDICATION FOR NASAL INTUBATION? A NARRATIVE REVIEW.

Velopharyngeal insufficiency is a disorder where the soft palate directs the air through the nose. It is often present in patients with previous cleft or short palate, but also in many other conditions. Symptoms are primarily to be found in speech, with very distinct nasal sound. After clinical evaluation and nasal endoscopy, surgery is considered. Several surgical techniques are in use, with posterior pharyngeal flap pharyngoplasty being most widely used. This method leaves the base of the posterior pharyngeal flap attached to the posterior pharyngeal wall, with two lateral ports on each side of the flap. Permanent nasopharyngeal obstruction is a very challenging pathology for anesthesiologists in case of mandatory nasal intubation since it is a relative contraindication for nasal intubation. Patients with previous palatoplasty will regularly appear in our routine anesthetic practice, in all surgical segments. The high risk of damage to the flap with possible bleeding can put the anesthesiologist in a very unpleasant situation if not aware of the permanent effect of this surgery. During preanesthetic assessment, if there is information on a previous pharyngoplasty, one should consider alternative options for nasotracheal intubation. All nasal insertion procedures must be either avoided or carried out with great caution, under fiberoptic visual control.

Unrecognized Lower Jaw Fracture in a Physically and Emotionally Abused Child.

Diagnosing child abuse still poses a challenge for healthcare professionals. We will present a case in which a physically and emotionally abused four-year-old boy had an undiagnosed lower jaw fracture for almost eight months. The child first presented with a peri-mandibular swelling and was treated with antibiotics prescribed by his doctor of dental medicine. After not having responded to antibiotic treatment, the boy was referred to the Department of Oral and Maxillofacial Surgery, accompanied by his birth mother. The deciduous mandibular molars with deep cavities were extracted since we thought that they were the reason for swelling. After several visits with his foster mother, the child was finally diagnosed with a lower jaw fracture and a psychosomatic disorder. Physical abuse by his birth parents was later on confirmed, and the consequences were insufficient growth, non-perception of pain and reduced speech development. After having been with his foster mother for almost a year, the child learned to complain when he was in pain. This report emphasizes the importance of taking a detailed medical history along with a thorough clinical examination.

Significance of nuclear EGFR and ABCG2 expression in malignant transformation of oral potentially malignant disorders.

BACKGROUND: The purpose of this study was to investigate the expression of nuclear EGFR (nEGFR) and the stem cell marker ABCG2 in oral leukoplakia (OL) and oral erythroplakia (OE) and to assess their significance as prognostic biomarkers for malignant transformation. METHODS: In this study we included 50 patients with oral potentially malignant disorders (OPMD), 31 with OL and 19 with OE, in whom we examined the expression of nEGFR and ABCG2 by immunohistochemical methods. RESULTS: Twenty-one (42%) of 50 patients with OL and OE developed oral squamous cell carcinoma (OSCC). The malignant transformation was increased 12,84-fold (95% CI, 2.15-76.44, p = 0.005) in OPMD expressing both ABCG2 and nEGFR. Expression of nEGFR is a strong indicator of malignant transformation, unlike ABCG2 expression, respectively. CONCLUSIONS: Determining the co-expression of the biomarkers nEGFR and ABCG2 in OPMD may serve us to determine the risk of malignant transformation in OSCC.

Diversified Effects of COVID-19 as a Consequence of the Differential Metabolism of Phospholipids and Lipid Peroxidation Evaluated in the Plasma of Survivors and Deceased Patients upon Admission to the Hospital.

As a result of SARS-CoV-2 infection, inflammation develops, which promotes oxidative stress, leading to modification of phospholipid metabolism. Therefore, the aim of this study is to compare the effects of COVID-19 on the levels of phospholipid and free polyunsaturated fatty acids (PUFAs) and their metabolites produced in response to reactions with reactive oxygen species (ROS) and enzymes (cyclooxygenases-(COXs) and lipoxygenase-(LOX)) in the plasma of patients who either recovered or passed away within a week of hospitalization. In the plasma of COVID-19 patients, especially of the survivors, the actions of ROS and phospholipase A2 (PLA2) cause a decrease in phospholipid fatty acids level and an increase in free fatty acids (especially arachidonic acid) despite increased COXs and LOX activity. This is accompanied by an increased level in lipid peroxidation products (malondialdehyde and 8-isoprostaglandin F2α) and lipid mediators generated by enzymes. There is also an increase in eicosanoids, both pro-inflammatory as follows: thromboxane B2 and prostaglandin E2, and anti-inflammatory as follows: 15-deoxy-Δ-12,14-prostaglandin J2 and 12-hydroxyeicosatetraenoic acid, as well as endocannabinoids (anandamide-(AEA) and 2-arachidonylglycerol-(2-AG)) observed in the plasma of patients who recovered. Moreover, the expression of tumor necrosis factor α and interleukins (IL-6 and IL-10) is increased in patients who recovered. However, in the group of patients who died, elevated levels of N-oleoylethanolamine and N-palmitoylethanolamine are found. Since lipid mediators may have different functions depending on the onset of pathophysiological processes, a stronger pro-inflammatory response in patients who have recovered may be the result of the defensive response to SARS-CoV-2 in survivors associated with specific changes in the phospholipid metabolism, which could also be considered a prognostic factor.

Lipidomics Revealed Plasma Phospholipid Profile Differences between Deceased and Recovered COVID-19 Patients.

Thorough understanding of metabolic changes, including lipidome alteration, associated with the development of COVID-19 appears to be crucial, as new types of coronaviruses are still reported. In this study, we analyzed the differences in the plasma phospholipid profiles of the deceased COVID-19 patients, those who recovered and healthy people. Due to identified abnormalities in plasma phospholipid profiles, deceased patients were further divided into two subgroups (D1 and D2). Increased levels of phosphatidylethanolamines (PE), phosphatidylcholines (PC) and phosphatidylserines (PS) were found in the plasma of recovered patients and the majority of deceased patients (first subgroup D1) compared to the control group. However, abundances of all relevant PE, PC and PS species decreased dramatically in the plasma of the second subgroup (D2) of five deceased patients. These patients also had significantly decreased plasma COX-2 activity when compared to the control, in contrast to unchanged and increased COX-2 activity in the plasma of the other deceased patients and recovered patients, respectively. Moreover, these five deceased patients were characterized by abnormally low CRP levels and tremendous increase in LDH levels, which may be the result of other pathophysiological disorders, including disorders of the immune system, liver damage and haemolytic anemia. In addition, an observed trend to decrease the autoantibodies against oxidative modifications of low-density lipoprotein (oLAb) titer in all, especially in deceased patients, indicate systemic oxidative stress and altered immune system that may have prognostic value in COVID-19.

Distribution of Pathogens and Predictive Values of Biomarkers of Inflammatory Response at ICU Admission on Outcomes of Critically Ill COVID-19 Patients with Bacterial Superinfections-Observations from National COVID-19 Hospital in Croatia.

BACKGROUND: Superinfections contribute to mortality and length of stay in critically ill COVID-19 patients. The aim of this study was to determine the incidence and pathogen distribution of bacterial and fungal superinfections of the lower respiratory tract (LRTI), urinary tract (UTI) and bloodstream (BSI) and to determine the predictive value of biomarkers of inflammatory response on their ICU survival rates. METHODS: A retrospective observational study that included critically ill COVID-19 patients treated during an 11-month period in a Croatian national COVID-19 hospital was performed. Clinical and diagnostic data were analyzed according to the origin of superinfection, and multivariate regression analysis was performed to determine the predictive values of biomarkers of inflammation on their survival rates. RESULTS: 55.3% critically ill COVID-19 patients developed bacterial or fungal superinfections, and LRTI were most common, followed by BSI and UTI. Multidrug-resistant pathogens were the most common causes of LRTI and BSI, while Enterococcus faecalis was the most common pathogen causing UTI. Serum ferritin and neutrophil count were associated with decreased chances of survival in patients with LRTI, and patients with multidrug-resistant isolates had significantly higher mortality rates, coupled with longer ICU stays. CONCLUSION: The incidence of superinfections in critically ill COVID-19 patients was 55.3%, and multidrug-resistant pathogens were dominant. Elevated ferritin levels and neutrophilia at ICU admission were associated with increased ICU mortality in patients with positive LRTI.

The Impact of Severe COVID-19 on Plasma Antioxidants.

Several studies suggested the association of COVID-19 with systemic oxidative stress, in particular with lipid peroxidation and vascular stress. Therefore, this study aimed to evaluate the antioxidant signaling in the plasma of eighty-eight patients upon admission to the Clinical Hospital Dubrava in Zagreb, of which twenty-two died within a week, while the other recovered. The differences between the deceased and the survivors were found, especially in the reduction of superoxide dismutases (SOD-1 and SOD-2) activity, which was accompanied by the alteration in glutathione-dependent system and the intensification of the thioredoxin-dependent system. Reduced levels of non-enzymatic antioxidants, especially tocopherol, were also observed, which correlated with enhanced lipid peroxidation (determined by 4-hydroxynonenal (4-HNE) and neuroprostane levels) and oxidative modifications of proteins assessed as 4-HNE-protein adducts and carbonyl groups. These findings confirm the onset of systemic oxidative stress in patients with severe SARS-CoV-2, especially those who died from COVID-19, as manifested by strongly reduced tocopherol level and SOD activity associated with lipid peroxidation. Therefore, we propose that preventive and/or supplementary use of antioxidants, especially of lipophilic nature, could be beneficial for the treatment of COVID-19 patients.

Quality of life following maxillofacial trauma in the elderly: a multicenter, prospective study.

BACKGROUND/AIMS: When facial trauma involves elderly patients, the possible presence of frailty and comorbidities in victims of trauma may worsen the posttraumatic symptoms and decrease quality of life. The aim of this multicenter study was to assess the quality of life following surgical or non-operative management of maxillofacial trauma in elderly patients. MATERIALS AND METHODS: This cohort study was based on the administration of validated self-administered questionnaires to all the geriatric patients (70 years or more) with facial fractures from the involved maxillofacial surgical units across Europe, since 1st January 2019 to 31st June 2019. The following questionnaires were administered: SF36 questionnaire; the VFQ-25 questionnaire; the Oral Health Impact Profile - 14 (OHIP14). Outcome variables were VFQ-25 and OHIP-14 results. RESULTS: A total of 37 patients (14 male and 23 female patients) met the inclusion criteria and were included in the study. Elderly patients had an improvement in almost all the categories examined by the SF-36 questionnaire 6 months after trauma, with the only exception of a worsening as for role limitations due to physical health. An improvement was observed in almost all the categories at SF-36 test. A worsening of scores of OHIP-14 for all the considered dimensions in the whole study population was observed too. CONCLUSIONS: Elderly patients following facial trauma experience significant emotional, social, and functional disturbances. We observed that emotional problems, energy/fatigue, social functioning, and generally social limitations played a great role in the decrease of QoL in elderly patients following maxillofacial trauma.

Preliminary Findings on the Association of the Lipid Peroxidation Product 4-Hydroxynonenal with the Lethal Outcome of Aggressive COVID-19.

Major findings of the pilot study involving 21 critically ill patients during the week after admission to the critical care unit specialized for COVID-19 are presented. Fourteen patients have recovered, while seven passed away. There were no differences between them in respect to clinical or laboratory parameters monitored. However, protein adducts of the lipid peroxidation product 4-hydroxynonenal (HNE) were higher in the plasma of the deceased patients, while total antioxidant capacity was below the detection limit for the majority of sera samples in both groups. Moreover, levels of the HNE-protein adducts were constant in the plasma of the deceased patients, while in survivors, they have shown prominent and dynamic variations, suggesting that survivors had active oxidative stress response mechanisms reacting to COVID-19 aggression, which were not efficient in patients who died. Immunohistochemistry revealed the abundant presence of HNE-protein adducts in the lungs of deceased patients indicating that HNE is associated with the lethal outcome. It seems that HNE was spreading from the blood vessels more than being a consequence of pneumonia. Due to the limitations of the relatively small number of patients involved in this study, further research on HNE and antioxidants is needed. This might allow a better understanding of COVID-19 and options for utilizing antioxidants by personalized, integrative biomedicine approach to prevent the onset of HNE-mediated vitious circle of lipid peroxidation in patients with aggressive inflammatory diseases.

The Acceptance of Cosmetic Surgery Scale (Acss) and Its Correlations with Psychological Characteristics among the Croatian Population.

BACKGROUND: This study aimed to examine the results of Acceptance of Cosmetic Surgery Scale among the Croatian population and its correlations with other scales and demographic data. SUBJECT AND METHODS: The sample consisted of 420 people who voluntarily filled out an online questionnaire. They completed demographic questions and four scales: Acceptance of Cosmetic Surgery Scale (ACSS), Rosenberg Self-Esteem Scale (RSES), Satisfaction With Life Scale (SWLS), and Body Appreciation Scale-2 (BAS-2). RESULTS: ACSS scale showed five statistically significant differences between genders and a higher overall score in women, but no significant differences were recorded in three ACSS subscales and the overall ACSS score. In contrast to men, women respondents recorded a significant negative correlation between ACSS subscales score (Social, Consider) and BAS-2 and Satisfaction with life score, while a positive correlation was recorded with BMI. CONCLUSION: The ACSS score among the Croatian population was higher than the results among the Italian and Serbian population, and similar to the original American study, which tells us that the Croatian population accepts and considers cosmetic surgery a lot. Furthermore, our results are important for practitioners and patients because they revealed correlations between ACSS scores and the self-thinking scale.

Challenges in the diagnostics and treatment of ectopic ameloblastic carcinoma: a case report.

Ameloblastic carcinoma (AC) is a rare and aggressive malignant epithelial odontogenic tumor, most commonly located in the mandible or maxilla. An extremely rare extragnathic localization of AC with no connection to the jaws, ectopic ameloblastic carcinoma (EAC), has so far been described only three times. This report presents a 64-year-old male with skull base ameloblastic carcinoma and offers a review of diagnostic and treatment challenges related to EAC. Because of its rarity and histological similarity to other tumors, EAC is often misdiagnosed. This is why we established a pathohistological and immunohistochemical profile of EAC that differentiates it from histologically similar tumors. The most frequently used EAC treatment is radical surgical resection, but the majority of reviewed reports described local recurrence. Taking into consideration new scientific discoveries on the molecular pathogenesis of ameloblastoma, we are the first to have performed BRAF mutation analysis in an EAC patient. BRAF inhibitors offer promising results in the treatment of BRAF-positive ameloblastomas and should continue to be researched in AC and EAC patients. Finally, EAC should be considered in differential diagnosis of head and neck tumors outside the jaws.

Gingival Hypertrophy in a Child with Hyaline Fibromatosis Syndrome.

Hyaline fibromatosis syndrome (HFS) is a rare autosomal recessive genetic disorder characterized by accumulation of hyalinized fibrous tissue with cutaneous, mucosal, osteoarticular, and systemic involvement. The condition is caused by a mutation of ANTXR2 gene that results in a faulty synthesis of a transmembrane protein which leads up to excessive deposition of hyaline material in extracellular space. The first signs may be present at birth or appear during infancy, and joint stiffness is the first, most common, symptom. Other manifestations include joint contractures, hyperpigmented macules over bony prominences of the joints, and gingival hypertrophy. The symptom that raises suspicion of HFS is present later, along with subcutaneous growths. The progression of the disease includes enteropathy with extensive protein loss, chronic diarrhea and frequent infections. We present a case of a five-year-old girl with severe gingival hypertrophy that caused difficulties in eating and speaking. To the best of our knowledge, this is also the first patient in Croatia with a confirmed ANTXR2 gene mutation described in the literature.

Management of maxillofacial trauma in the elderly: A European multicenter study.

BACKGROUND/AIMS: Management of maxillofacial trauma in the geriatric population poses a great challenge due to anatomical variations and medical comorbidities. The aim of this study was to analyze the management variables, timing, and outcomes of facial fractures in elderly patients (aged 70 years or more) at several European departments of oral and maxillofacial surgery. MATERIALS AND METHODS: This study was based on a systematic computer-assisted database that allowed the recording of data from all geriatric patients with facial fractures from the involved maxillofacial surgical units across Europe between 2013 and 2017. RESULTS: A total of 1334 patients were included in the study: 665 patients underwent closed or open surgical treatment. A significant association (P < .005) was found between the presence of concomitant injuries and a prolonged time between hospital admission and treatment. The absence of indications to treatment was associated with comorbidities and an older age (P < .000005). CONCLUSIONS: Elderly patients require specific attention and multidisciplinary collaboration in the diagnosis and sequencing of trauma treatment. A prudent attitude may be kept in selected cases, especially when severe comorbidities are associated and function is not impaired.