Efficacy of Elexacaftor-Tezacaftor-Ivacaftor on chronic rhinosinusitis in cystic fibrosis.

PURPOSE: Our work aims to add evidence on the effectiveness of Elexacaftor-Tezacaftor-Ivacaftor on chronic rhinosinusitis in cystic fibrosis. MATERIALS AND METHODS: We conducted an observational retrospective cohort study at the Cystic Fibrosis Center of a tertiary care hospital to investigate the effect of Elexacaftor-Tezacaftor-Ivacaftor on chronic rhinosinusitis in cystic fibrosis patients, aged 12 or older. The study's endpoints were the change in the occurrence of acute exacerbations of chronic rhinosinusitis, and the variation of the endoscopic and radiologic findings scored using the Lund-Kennedy endoscopic scale, Lund-Mackay, and modified Lund-Mackay radiologic scales, in patients who underwent both pre-treatment and post-treatment examinations. RESULTS: The study population comprised 136 patients, of which 28 underwent both pre-treatment and post-treatment nasal endoscopy and 15 had pre- and post-treatment CT scans. Elexacaftor-Tezacaftor-Ivacaftor provided a significant improvement in chronic rhinosinusitis. The mean number of acute exacerbations of chronic rhinosinusitis per year in the pre-treatment time was 0.55 versus 0.35 during the treatment (p < 0.0021). The Lund-Kennedy scale had a pre-treatment average score of 4.21 points versus 1.5 points after the start of Elexacaftor-Tezacaftor-Ivacaftor (p < 0.0001). The average Lund-Mackay and modified Lund-Mackay scores in the pre-treatment time were respectively 14.6 and 16.45 points; and after the start of the therapy, they became 5.87 and 6.73 (p < 0.0001). CONCLUSION: Elexacaftor-Tezacaftor-Ivacaftor was associated with fewer acute exacerbations of chronic rhinosinusitis, and a significant improvement of chronic rhinosinusitis evaluated endoscopically and radiologically. To our knowledge, this is the first study investigating the change in the occurrence of acute exacerbation of chronic rhinosinusitis in patients affected by cystic fibrosis in therapy with Elexacaftor-Tezacaftor-Ivacaftor.

Lung abscess as a complication of Lemierre Syndrome in adolescents: a single center case reports and review of the literature.

BACKGROUND: Fusobacterium necrophorum is an anaerobic, gram-negative, non-motile, filamentous, non-spore forming bacillus found in the oral cavity, gastrointestinal tract, and female genital tract, responsible of a rare disease named Lemierre Syndrome, characterized by septic thrombophlebitis of the internal jugular vein, which mainly affects previously healthy adolescents and young adults; some risk factors are reported, as smoking or primary viral or bacterial infection leading to the disruption of mucosa. The syndrome originates commonly from an upper respiratory infection such as pharyngotonsillitis, acute otitis media, cervical lymphadenitis, sinusitis, or odontogenic abscess, and may result in multiorgan metastasis, more frequently leading to pulmonary complications, especially lung abscesses. CASE PRESENTATION: We describe two cases of adolescents with atypical Lemierre Syndrome evaluated in a tertiary care center, one with a confirmed infection by Fusobacterium necrophorum and one with a presumptive diagnosis based on clinical features, who developed lung abscesses needing a prolonged antibiotic course and hospitalization. Of interest, both were user of electronic cigarette, configuring a possible new risk factor. The proper diagnosis of Lemierre Syndrome is often difficult to establish, so a high degree of suspicion is needed, especially in the case of lung abscesses in otherwise healthy adolescents. CONCLUSION: The current study will contribute to providing insight into Lemierre Syndrome clinical presentation and management in adolescents, promoting awareness for a rare but potentially fatal disease. Moreover, it suggests a possible relationship between Lemierre syndrome and the use of electronic cigarette, that should be investigated by future studies.

Endoscopic endonasal repair of bilateral choanal atresia in a 1200 g preterm infant: Is it feasible? (With video).

INTRODUCTION: Bilateral choanal atresia (CA) is a rare congenital anomaly, that causes neonatal respiratory distress. No consensus exists regarding the most appropriate surgical approach in low-birth weight preterm infants. CASE REPORT: We present (with video) the case of a male born at 29 weeks' gestation, 1200 g, affected by bilateral CA. He was successfully treated with an endoscopic transnasal approach performed on day 4 of life which allowed a very early extubation. Otologic instruments and 2.7 mm wide endoscope were used to be able to operate in very narrow nasal cavities. Thulium LASER® was used to limit bleeding. At the follow-up visit, four months after surgery, the neochoana was widely patent. DISCUSSION: In low-birth weight preterm infants, endoscopic transnasal surgery (ETS) may be considered technically not feasible or with a high risk of early restenosis. With early ETS, we were able to avoid the morbidity of a prolonged intubation and sedation. CONCLUSION: Bilateral CA is a life threatening and challenging clinical entity, particularly difficult to treat in low-birth preterm infants. Early surgery to reduce intubation and sedation is preferable, but requires experienced teams with a surgical technique and instruments tailored to the narrowness of the nasal cavity.

Acquired subglottic cysts in children: A rare and challenging clinical entity. A systematic review.

INTRODUCTION: Subglottic cysts (SGCs) are a rare cause of laryngeal stenosis that mainly seem to affect preterm infants with an intubation history. PURPOSE: To review the related literature and compare different management protocols for patients presenting SGCs, as well as briefly report our clinical case treated at Verona University Hospital by Pediatric Airways Team. METHODS: The articles resulting from a PubMed and MEDLINE search were analysed and selected using previously established criteria. A systematic review of the selected papers was conducted following PRISMA guidelines. RESULTS: The search yielded 571 related articles; cross-checking of articles led to the identification and exclusion of 239 duplicates. The remaining 332 papers were screened according to previously established eligibility criteria. The final number of selected articles was 13. CONCLUSIONS: Well-planned teamwork, with active collaboration between the ENT specialists, pediatricians and anesthesiologists, is the key to achieve multidisciplinary management of patients diagnosed with SGCs. Long-term follow-up is crucial considering the high recurrence rate of this disease.

3D exoscopic surgery of lateral skull base.

PURPOSE: The aim of the study is to assess whether the 3D exoscopic surgery technique could be used in lateral skull base surgery and if it could ultimately replace the microscope in the future. METHODS: This is a retrospective study in which were included 24 patients affected by lateral skull base pathologies, who underwent surgery using the 3D exoscope or the operative microscope at the Department of Otolaryngology-Head and Neck Surgery at the University Hospital of Verona. The exoscope and microscope groups each included 12 cases. The feasibility of all the surgical steps solely using the 3D exoscope was evaluated. The exoscope group and microscope group were compared taking into account the following factors: time of the surgery, facial and hearing functions outcomes, as well as the intraoperative and postoperative complications. RESULTS: No intraoperative complication occurred during all the procedures. Postoperatively, only one minor complication emerged. The average operative time was 289 in the exoscope group and 313 min in the microscope one. No significant statistical differences were identified between the two groups (p > 0.05). The facial and hearing function outcomes were fully comparable. CONCLUSION: Our experience demonstrated that the exclusive use of the 3D exoscope, as that of the traditional microscope during lateral skull base surgery, is feasible for all open approaches. The use of the 3D exoscopic technique is very promising for future lateral skull base surgeries.

Retained sponges in abdomen: an analysis of the judgments of the Italian Supreme Court.

The administration of justice in Italy includes first, second and third instance. The first and second instances are represented by the Court of First Instance and the Court of Appeal: these are judgment of Romamerit. The court of last appeal for both the civil and the criminal jurisdiction is the Court of Cassation, the Italian Supreme Court. It is a court of legitimacy that should provide for a consistent and uniform interpretation of the law and that only on points of law, not on factual evidence. The Court of Cassation can confirm the sentence of second instance, can dismiss it without referral, can deciding and closing the trial definitively, or dismiss it referring the case to the judge of merit that must decide according to the principles set out in the legitimacy. The aim of this study is to analyze the Supreme Cassation Court's judgments on the legal proceedings about retained sponges in abdomen.

Double inherited thrombophilias and adverse pregnancy outcomes: fashion or science?

AIM: To determine to what extent double inherited thrombophilias are associated with adverse obstetric complications correlated with fetoplacental insufficiency, such as preeclampsia, hemolytic anemia elevated liver enzymes and low platelet count (HELLP) syndrome, gestational hypertension, fetal growth restriction (FGR), intrauterine death (ID), abruptio placentae and disseminated intravascular coagulopathy. METHODS: Pregnant women coming to delivery were retrospectively divided into two groups: group A (controls) and group B (cases). Patients belonging to group B had one of the following: severe preeclampsia, HELLP syndrome, gestational hypertension, FGR, intrauterine death, abruptio placentae of disseminated intravascular coagulopathy. We detected methylenetetrahydrofolate reductase (MTHFR) A1298C, MTHFR C677T, factor V Leiden, PAI-1, mutant prothrombin G20210A, plasma homocysteine, antithrombin III, protein S and activated protein C resistance. Normal pregnant women or pregnant women with double defects were enrolled in this study. RESULTS: The combination of MTHFR C677T mutation with PAI-1 (5G/5G) mutation was significantly linked with the occurrence of ID. HELLP syndrome was significantly related to the simultaneous presence of factor VIII and X mutations. The combination of MTHFR C677T with factor VIII mutation and the combination of factor II and V mutations were significantly related to the occurrence of abruptio placentae. We found an association between double isoforms MTHFR mutation and FGR. CONCLUSION: It seems that some thrombophilias and a combination of thrombophilic factors carry a greater risk than others for a given adverse outcome. Further studies are needed to check the link between thrombophilic gene mutations and adverse pregnancy outcomes, such as recurrent miscarriages and deep venous thrombosis.

Could birthweight prediction models be improved by adding fetal subcutaneous tissue thickness?

AIM: The aims of the study were to: (i) compare the accuracy of standard ultrasonic algorithms in the estimation of fetal weight and; (ii) test two new algorithms in order to improve the global performance of birthweight prediction by adding fetal subcutaneous tissue thickness. METHODS: We enrolled 398 patients who were between 34 and 42 weeks' gestation. Routine ultrasonographic biometric parameters as well as subcutaneous tissue thickness ultrasound parameters were measured. Correlation matrices between ultrasound parameters, in order to evaluate the degree of multicollinearity between these parameters, were assessed to develop a stepwise multiple regression birthweight predictive model. RESULTS: Contributions of single ultrasound measurements in predicting birthweight were examined, by fitting Log-transformed birthweight versus single ultrasound measurements. We found that the mid-thigh tissue area was able to significantly improve the performance of the birthweight prediction process when added to the other standard ultrasound measurements. We derived two new algorithms which appeared to be better at predicting birthweight. Furthermore there was a lower minimum absolute estimation error noted when compared to other reported formulae. CONCLUSIONS: Our algorithms showed that the addition of the mid-thigh tissue evaluation in birthweight prediction was valuable in comparison to birthweight prediction models which are based on routine ultrasound parameters.

Ultrasound-determined fetal subcutaneous tissue thickness for a birthweight prediction model.

AIM: The aim of this study was to explore a birthweight prediction model using ultrasound determined tissue thickness (SCTT) parameters. METHODS: We measured routine ultrasonographic biometric parameters and in addition, fetal SCTT in 201 healthy singleton pregnancies. Mid-arm fat and lean mass, mid-thigh fat and lean mass, subscapular fat mass and abdominal fat mass (AFM) were measured in order to calculate a birthweight prediction model. Ultrasound measurements were analyzed using an 'anovarepeated measures model'. The growth rate (beta-slope) of the selected parameters was computed and the correlation coefficient with the birthweight and the Kendall rank correlation tau, were calculated. RESULTS: From the ultrasound determined SCTT parameters, only abdominal circumference (AC), AFM, and MTLM showed a statistically significant trend. The beta-slope of mid-thigh lean mass was excluded since it exhibited significant correlation with the beta-slope of AFM. The final regression model could be calculated as: birthweight (gr.) = intercept +alpha(1)(AFM beta-slope) + alpha(2)(AC beta-slope), where alpha(1), alpha(2) represent regression coefficients. CONCLUSIONS: We provide a graphical birthweight prediction model for clinical practice using conventional and specific ultrasound measurements of fetal subcutaneous tissue thickness. This model is based upon an overall analysis of the ultrasound estimated body components.

Single inherited thrombophilias and adverse pregnancy outcomes.

INTRODUCTION: Inherited thrombophilia is believed to be a multiple gene disease with more than one defect. We aimed to determine the association between single thrombophilic patterns and a variety of pregnancy diseases. METHODS: 284 pregnant women were recruited for the present study and were divided in two groups: A group (176 controls) and B group (108 cases). Patients belonging to the B group had one of the following: severe pre-eclampsia, hemolysis, hepatic enzymes increase, hypertension and low platelet count (HELLP) syndrome, gestational hypertension, fetal growth restriction, intrauterine death, abruptio placentae and disseminated intravascular coagulopathy. To detect methylenetetrahydrofolate reductase (MTHFR) A1298C, MTHFR C677T, factor V Leiden, PAI-1, mutant prothrombin G20210A, an inverse hybridization technology was used. Plasma homocysteine, antithrombin (AT) III and protein S were determined. A modified functional activated protein C resistance was detected. RESULTS: MTHFR C677T and hyperhomocysteinemia were more prevalent than other thrombophilias. Deficiency in AT III was significantly linked with pre-eclampsia (relative risk 0.88; 95% CI 0.83-0.94). Activated protein C resistance (APCR) was significantly related to the abruptio placentae (relative risk 0.71; 95% CI 0.61-0.82). COMMENTS: Apart from the linkage between AT III deficiency and the occurrence of pre-eclampsia, and apart from the increased risk of abruptio placentae in pregnant women with altered APCR, we obtained findings in contrast with some of the published literature. In our case series, no association of pre-eclampsia with factor V Leiden or with prothrombin gene mutation was found.

Amniotic fluid lamellar body counts for the determination of fetal lung maturity: an update.

AIM: To reassess the cut-off value for lamellar body counts (LBs) for fetal lung maturity (FLM) over a 10-year study period. PATIENTS AND METHODS: 178 pregnancies were selected under strict inclusion criteria and delivered within 48 h from amniocentesis. FLM was determined by amniotic fluid LBs in centrifuged samples (300 x g for 10 min) in a commercially available Coulter Counter. Cases beyond 37 weeks were excluded. RESULTS: Mean gestational age was 33.5+/-3.0 weeks at amniocentesis and 33.7+/-3.0 weeks at birth. After reassessing the best compromise between sensitivity and specificity for all cases using the receiver operating characteristic (ROC) procedure, an FLM cut-off value of < or = 22,000/microL was obtained. Diagnostic accuracy (and confidence interval, CI) was: sensitivity, 73% (60.0-83.6%); specificity, 81.7% (CI 73.6-88.1%); positive predictive value, 66.2%; and negative predictive value, 86.0%. CONCLUSION: No significant change in FLM cut-off for LBs was found when comparing the value from this study and the results of our earlier report presented in 1996 (< or = 22,000 vs. < or = 20,000/microL), although the new value may be more accurate, since it is based on neonatal outcome with the exclusion of cases in which the diagnosis of FLM is seldom warranted, i.e., > 37 weeks' gestational age.