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1.
Sci Prog ; 106(4): 368504231215591, 2023.
Article in English | MEDLINE | ID: mdl-38055322

ABSTRACT

Introduction: Post-adenotonsillectomy (PAT) bleeding, a life-threatening surgical complication, remains unpredictable despite preoperative blood tests. Every surgeon would like predictive markers for this complication of one of the most common procedures performed in pediatric ear, nose, and throat (ENT). Objective: The purpose of the study is to see whether the results of the blood tests we perform routinely preoperatively in children undergoing adenotonsillectomy (AT) (lymphocyte count and percentage, C reactive protein, fibrinogen, or coagulation variables International Normalized Ratio and activated partial thromboplastin time) can potentially predict early post-AT bleeding. Focus has been placed on the presence of relative lymphocytosis (a value of lymphocyte percentage above 55%) in the blood cell count of the patients and its possible connection to postoperative hemorrhage. Method: We conducted an observational retrospective study on 801 children undergoing adenoidectomy, tonsillectomy, or AT over a period of 6 months in our ENT department. Statistical analysis was performed to compare the data. Results: we did not find a statistically significant correlation between preoperative blood markers (coagulation or inflammatory) and early post-AT bleeding. An important blood marker in relation to PAT bleeding appears to be relative lymphocytosis. Relative lymphocytosis has a weak predictive value of early postoperative bleeding in children with AT (sensitivity of only 31.58%, but acceptable specificity of above 80%). In other words, 80% of patients without relative lymphocytosis will not bleed in the first 24 h postoperatively. Children with relative lymphocytosis may need tighter surveillance in the first 24 h after AT. Conclusions: Relative lymphocytosis has a weak predictive value of early postoperative bleeding in children with AT children.


Subject(s)
Lymphocytosis , Tonsillectomy , Humans , Child , Adenoidectomy , Tonsillectomy/adverse effects , Retrospective Studies , Hemorrhage , Hematologic Tests
2.
J Pers Med ; 13(11)2023 Nov 04.
Article in English | MEDLINE | ID: mdl-38003892

ABSTRACT

Collagen VI-related disorders constitute a spectrum of severities from the milder Bethlem myopathy (BM) to the Ullrich congenital muscular dystrophy (UCMD), which is more severe, and an intermediate form characterized by muscle weakness that begins in infancy. Affected children are able to walk, although walking becomes increasingly difficult starting in early adulthood. They develop contractures in the ankles, elbows, knees, and spine in childhood. In some affected cases, the respiratory muscles are weakened, requiring mechanical ventilation, particularly during sleep. Individuals with collagen VI-related myopathy are at risk of restrictive lung disease and sleep-disordered breathing due to the development of scoliosis associated with neuromuscular weakness. Typical signs of respiratory failure are not always present, and some patients are unaware that their respiratory muscles have become weaker. Here, we report a case of an intermediate form of collagen VI-related myopathy confirmed by next-generation sequencing. The girl presented morning headache, irritability, and aggressiveness, and because of these main symptoms, she was referred by the neurologist for respiratory evaluation. The result of spirometry was associated with hypoventilation shown during sleep studies, indicating the necessity to initiate home non-invasive ventilation (NIV) with immediate improvement in the symptoms. Neuromuscular disorders (NMDs) have a great impact on sleep, but only very few studies evaluating sleep quality in young patients with collagen VI-related myopathy have been described. Daytime symptoms of sleep-disordered breathing may include irritability, emotional lability, and poor attentiveness, but these can be overseen by the severity of other complex medical problems in patients with collagen VI-related myopathy. We underline the importance of the close monitoring of respiratory function, sleep evaluation, and decision making to support the NIV treatment of other collagen VI-related myopathy variant-specific patients. Early recognition of sleep disturbances and initiation of respiratory support can preserve or enhance the quality of life for patients and their caregivers. Routine screening for identification of emotional distress should be instituted in the clinical practice using validated psychological measures in a multidisciplinary approach with different intervention strategies for both patient and parent when necessary.

3.
Medicina (Kaunas) ; 59(9)2023 Aug 24.
Article in English | MEDLINE | ID: mdl-37763653

ABSTRACT

Introduction: Adenoidectomy is often the first major surgical challenge for the child's haemostatic system, and controlling intraoperative bleeding can be a challenge for the surgeon. Different methods have been used intraoperatively by surgeons in order to enhance haemostasis. The cold air effect (continuous suctioning) has been used by some surgeons during adenoidectomy; however, no documentation of its haemostatic effect has been made. Objectives: Our prospective randomised controlled study enrolled a sample of 140 children undergoing adenoidectomy, and we studied the effect of continuous suctioning on the duration of haemostasis in paediatric adenoidectomy. Materials and Methods: We evaluated the effect of using continuous suctioning during haemostasis at the end of adenoidectomy procedures, comparing variables such as total surgery time, total haemostasis time, and intraoperative blood loss, between two groups: 70 adenoidectomy procedures where no continuous suctioning was used to enhance haemostasis versus the other 70 patients where continuous suctioning was the haemostatic method employed. RESULTS: After statistical analysis of the recorded data, we found that the total duration of adenoidectomy, the duration of haemostasis in adenoidectomy, and the intraoperative blood loss were significantly lower in patients in whom cold air was used for haemostasis. Intraoperative haemostasis failure (and consequent use of electrocautery for haemostasis) was more frequent in patients in whom no suctioning was used; as for the rates of postoperative primary bleeding after adenoidectomy, they were similar in both groups of patients, regardless of the technique used for haemostasis. Conclusions: The use of continuous suctioning during adenoidectomy haemostasis significantly shortens total surgical and haemostasis time, reduces intraoperative blood loss, and reduces the incidence of haemostasis failure (with the consequent need for bipolar electrocautery haemostasis).


Subject(s)
Adenoidectomy , Hemostatics , Humans , Child , Adenoidectomy/adverse effects , Blood Loss, Surgical/prevention & control , Prospective Studies , Postoperative Hemorrhage/etiology , Hemostatics/therapeutic use , Hemostasis
4.
Clin Pathol ; 16: 2632010X231195231, 2023.
Article in English | MEDLINE | ID: mdl-37599665

ABSTRACT

Introduction: Teratomas, tumors derived from all 3 fetal germ cell layers, are rarely located in the head and neck region and extremely rare in the Eustachian tube area. Case report: The authors present the case of a 2 years old child with chronic ear discharge. Referral diagnosis was tumor or cyst of the temporal bone. Intraoperatively, a solid, hairy tumor located inside the Eustachain tube, with extension into the middle ear space was discovered and removed. Histology revealed a mature teratoma. Review of the literature showed reports of rare similar cases of Eustachian tube teratomas, differentiating themfrom hairy polyps of the rhynopharynx. The imaging of our patient, the surgical techique for removal of the tumor and long term follow-up are detailed. Conclusions: Teratomas located inside the Eustachian tube are exceptional. Their removal should be carefully planned in view of their long term anatomical sequelae and in order to avoid long term postoperative morbidity.

5.
Cancers (Basel) ; 15(15)2023 Jul 26.
Article in English | MEDLINE | ID: mdl-37568611

ABSTRACT

No clinical studies have investigated the effect of radioiodine (131I)-targeted therapy on the neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) as inflammatory response markers in patients with differentiated thyroid cancer (DTC) associated with type 2 diabetes mellitus (T2DM) and obesity. This study aimed to assess the relationship between blood radioactivity, body mass index (BMI), and peripheral blood cells three days after 131I intake in 56 female patients without T2DM (DTC/-T2DM) vs. 24 female patients with T2DM (DTC/+T2DM). Blood radioactivity, measured three days after 131I intake, was significantly lower in the DTC/+T2DM than in the DTC/-T2DM patients (0.7 mCi vs. 1.5 mCi, p < 0.001). The relationship between blood radioactivity and BMI (r = 0.83, p < 0.001), blood radioactivity and NLR (r = 0.53, p = 0.008), and BMI and NLR (r = 0.58, p = 0.003) indicates a possible connection between the bloodstream 131I uptake and T2DM-specific chronic inflammation. In patients without T2DM, 131I therapy has immunosuppressive effects, leading to increased NLR (19.6%, p = 0.009) and PLR (39.1%, p = 0.002). On the contrary, in the chronic inflammation context of T2DM, 131I therapy amplifies immune metabolism, leading to a drop in NLR (10%, p = 0.032) and PLR (13.4%, p = 0.021). Our results show that, in DTC/+T2DM, the bidirectional crosstalk between neutrophils and obesity may limit 131I uptake in the bloodstream. Considering the immune response to 131I therapy, the two groups of patients can be seen as a synchronous portrait of two sides. The explanation could lie in the different radiosensitivity of T and B lymphocytes, with T lymphocytes being predominant in patients with DTC/-T2DM and, most likely, B lymphocytes being predominant in T2DM.

6.
Medicina (Kaunas) ; 59(4)2023 Apr 10.
Article in English | MEDLINE | ID: mdl-37109697

ABSTRACT

Background and objectives: Bipolar electrocautery is commonly used to control bleeding after cold-instrument pediatric adenoidectomy, but the surgeon should be aware of the possible side effects. OBJECTIVE: The aim of our study is to investigate the effects of bipolar electrocautery when used for bleeding control at the end of an adenoidectomy procedure. Materials and Methods: We evaluated the effect of electrocautery on postoperative pain, velopharyngeal insufficiency symptoms, postoperative nasal obstruction, and rhinorrhea in a group of 90 children undergoing adenoidectomy in our ENT department over a period of 3 months. Results: After statistically analyzing the data, we found that the duration of postoperative pain, the duration of rhinorrhea and nasal obstruction, and the duration of painkiller administration, as well as the velopharyngeal insufficiency symptoms, were significantly longer in patients in whom electrocautery was used for hemostasis. A significantly higher incidence of posterior neck pain and halitosis (oral malodor) was noted in the patients in whom electrocautery was used for adenoidectomy hemostasis. Conclusions: Bipolar electrocautery use should be limited during pediatric adenoidectomy hemostasis because of the possible side effects: longer postoperative pain, prolonged nasal obstruction, rhinorrhea and velopharyngeal insufficiency, and halitosis. We noted some side effects that were specific to electrocautery use during adenoidectomy: posterior neck pain and oral malodor. Acknowledging the risk for these symptoms can help to alleviate the anxiety of both the parents and the patients regarding the expected postoperative outcomes.


Subject(s)
Halitosis , Nasal Obstruction , Velopharyngeal Insufficiency , Child , Humans , Adenoidectomy/adverse effects , Adenoidectomy/methods , Halitosis/etiology , Nasal Obstruction/complications , Velopharyngeal Insufficiency/complications , Neck Pain/complications , Pain, Postoperative/etiology , Pain, Postoperative/epidemiology , Electrocoagulation/adverse effects , Electrocoagulation/methods , Hemostasis
7.
Medicina (Kaunas) ; 59(3)2023 Feb 25.
Article in English | MEDLINE | ID: mdl-36984462

ABSTRACT

Background: The persistent stapedial artery (PSA) is a rare congenital vascular malformation involving the middle ear. It is usually associated with pulsatile tinnitus and/or conductive hearing loss and can account for multiple risks during middle ear surgery. Case Report: we present a case of a 9-year-old male child with conductive hearing loss and persistent stapedial artery in his right ear, who was admitted to our ENT Department for hearing loss. During surgery, we discovered PSA along with congenital stapes agenesis and oval window atresia, as well as an abnormal trajectory of the mastoid segment of the facial nerve. After ossicular reconstruction (transcanal total ossicular replacement prosthesis) with cochleostomy, no surgical complications were recorded and hearing improvement was monitored by pre- and postoperative audiometry. Conclusion: Stapedial artery is a rare anatomical middle ear abnormality that can prevent proper surgical hearing restoration and can be associated with other simultaneous temporal bone malformations.


Subject(s)
Ossicular Prosthesis , Stapes , Male , Child , Humans , Stapes/abnormalities , Stapes/blood supply , Hearing Loss, Conductive/etiology , Hearing Loss, Conductive/surgery , Ear, Middle/abnormalities , Ear, Middle/surgery , Arteries/abnormalities
8.
Clin Case Rep ; 11(2): e6973, 2023 Feb.
Article in English | MEDLINE | ID: mdl-36817310

ABSTRACT

Pediatric tracheostomy in COVID-19 patients is a rarity. Joubert syndrome is a rare genetic disease, involving a lack of muscle control. We report the case of a child with Joubert syndrome and a severe form of COVID-19 infection, in whom we performed tracheostomy in order to replace prolonged intubation and mechanical ventilation; successful decannulation was performed after 12 months. Successful decannulation is still possible in a child with severe comorbidities (Joubert syndrome) even if it might take much longer than in patients without comorbidities.

9.
Clin Case Rep ; 10(12): e6715, 2022 Dec.
Article in English | MEDLINE | ID: mdl-36523389

ABSTRACT

Moebius Syndrome is a rare multifactorial condition defined by congenital complete or partial VII and VIth cranial nerves palsy and other physical abnormalities. We present the case of a 3 months old infant with Moebius sequence and breathing and eating difficulties, managed by tracheostomy and laryngoplasty.

10.
Medicina (Kaunas) ; 58(9)2022 Sep 09.
Article in English | MEDLINE | ID: mdl-36143929

ABSTRACT

(1) Background: In this paper, we report on three cases of hypoacusis as part of a complex phenotype and some rare gene variants. An extensive review of literature completes the newly reported clinical and genetic information. (2) Methods: The cases range from 2- to 11-year-old boys, all with a complex clinical picture and hearing impairment. In all cases, whole exome sequencing (WES) was performed, in the first case in association with mitochondrial DNA study. (3) Results: The detected variants were: two heterozygous variants in the TWNK gene, one likely pathogenic and another of uncertain clinical significance (autosomal recessive mitochondrial DNA depletion syndrome type 7-hepatocerebral type); heterozygous variants of uncertain significance PACS2 and SYT2 genes (autosomal dominant early infantile epileptic encephalopathy) and a homozygous variant of uncertain significance in SUCLG1 gene (mitochondrial DNA depletion syndrome 9). Some of these genes have never been previously reported as associated with hearing problems. (4) Conclusions: Our cases bring new insights into some rare genetic syndromes. Although the role of TWNK gene in hearing impairment is clear and accordingly reflected in published literature as well as in the present article, for the presented gene variants, a correlation to hearing problems could not yet be established and requires more scientific data. We consider that further studies are necessary for a better understanding of the role of these variants.


Subject(s)
DNA Helicases , Hearing Loss , DNA Helicases/genetics , DNA, Mitochondrial/genetics , Hearing Loss/genetics , Humans , Mitochondrial Proteins , Mutation/genetics , Romania
11.
Cancers (Basel) ; 14(10)2022 May 10.
Article in English | MEDLINE | ID: mdl-35625965

ABSTRACT

Radioiodine (131I) therapy for differentiated thyroid cancer (DTC) involves exposure of the whole body, including the heart, to ionizing radiation. This exposure to the subsequent risk of heart disease is uncertain, especially in patients with DTC associated with type 2 diabetes mellitus (DTC/+T2DM). The current study aimed to assess the relationship between left ventricular ejection fraction (LVEF), high cumulative 131I dose, and peripheral blood parameters in patients with DTC/−T2DM and DTC/+T2DM. The study enrolled 72 female patients with DTC/−T2DM and 24 with DTC/+T2DM who received cumulative 131I doses above 150 mCi (5.55 GBq). LVEF was lower in patients with concomitant T2DM than those without (p < 0.001). The cumulative 131I dosage was inversely correlated with LVEF only in DTC/−T2DM patients (r = −0.57, p < 0.001). In the DTC/+T2DM group, LVEF was negatively associated with absolute platelet count (r = −0.67, p < 0.001) and platelet-to-lymphocyte ratio (r = −0.76, p < 0.001). Our results demonstrate that exposure to high cumulative 131I doses has different cardiovascular effects in DTC/−T2DM and DTC/+T2DM.

12.
Cancers (Basel) ; 14(8)2022 Apr 09.
Article in English | MEDLINE | ID: mdl-35454805

ABSTRACT

Publications investigating the effect of radioactive iodine (131I) therapy on the circulating peripheral blood cells in patients with differentiated thyroid cancer (DTC) are limited to blood samples collected more than 92 h after 131I. Studies conducted on blood samples collected up to 92 h are rare due to the radioactive contamination risk. This research aimed to assess the relationship between the prescribed 131I activity, human whole blood activity, and peripheral blood cells at many time points (6, 22, 46, 69, and 92 h after 131I). The study enrolled 50 female patients with DTC who received a 131I median activity of 90.54 mCi (3.35 GBq). The neutrophil-to-lymphocyte ratio (NLR) was measured as an inflammatory marker. 131I uptake in the residual thyroid tissue peaked after 46 h. Blood activity decreased in the first 46 h and increased 69 h after the 131I intake. Blood activity was associated with the absolute lymphocyte count and the NLR at 69 h (r = −0.49 and r = 0.52, p < 0.001). Our results demonstrate that the time interval between 46 and 69 h should be associated with the release of hematological inflammatory mediators, such as neutrophils and lymphocytes, to eradicate tumor cells in response to 131I therapy.

13.
Exp Ther Med ; 22(4): 1113, 2021 Oct.
Article in English | MEDLINE | ID: mdl-34504567

ABSTRACT

No prospective study has specifically examined the serum levels of matrix metalloproteinase-9 (MMP-9) and tissue inhibitor of metalloproteinase-1 (TIMP-1) in the early shock phase of burn-injured patients. Thus, we aimed to detect early changes, activity dynamics, and the predictive value of MMP-9, TIMP-1, and the MMP-9/TIMP-1 ratio to better understand the early repair mechanisms for the development of future therapies for patients with thermal burns. Twenty-five patients with a total body surface area (TBSA) affected by burn <25%, and 30 healthy subjects were enrolled in the study. Serum levels of MMP-9 and TIMP-1 were determined by the ELISA method. Our results showed that MMP-9 concentrations increased immediately after injury and remained on a plateau. In contrast, TIMP-1 showed an upward trend throughout the 7-day study period, and the time course of the MMP-9/TIMP-1 ratio followed the inverse dynamics of TIMP-1. Analysis of the areas under the receiver operating characteristic (ROC) curves (AUC) showed that patients with burn wounds tended to have a MMP-9 value higher than 421.5 ng/ml (AUC=0.979), TIMP-1 value higher than 231.6 ng/ml (AUC=0.908), and MMP-9/TIMP-1 ratio higher than 2.31 (AUC=0.959) (P<0.001). Our findings suggest that although the variations in the two biomarkers were different regarding the time of the initial insult, their ratio is a specific and sensitive indicator of burn evolutivity in patients with a TBSA affected by a burn <25%.

14.
Exp Ther Med ; 22(4): 1062, 2021 Oct.
Article in English | MEDLINE | ID: mdl-34434276

ABSTRACT

Burn injuries can trigger tissue changes that can explain the variation in the level of different biochemical markers that can be recorded both locally or systemically. Some events observed in burn wounds such as vascular hyperpermeability have been associated with the release of matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs) after trauma. Because it is unknown whether the serum levels of MMP-9 and TIMP-1 are a consequence of these destructions or a local response to thermal damage, we decided to follow their dynamics. Twenty-five patients (mean age 49.40±17.55 years) with a total body surface area (TBSA) affected by a thermal burn of <25% and 30 healthy subjects (mean age 49.70±8.04 years) were enrolled in the present study. Enzyme immunoassays were used to measure the serum levels of MMP-9 and TIMP-1. Our results showed that MMP-9 was increased 6.25-fold immediately after injury compared to the controls and remained on a plateau throughout the 7-day monitoring period. TIMP-1 showed an upward trend with an increase of 49.52% on the seventh day after triggering insult. The time-course of the MMP-9/TIMP-1 ratio followed the inverse dynamics of TIMP-1 starting from a ratio value measured at admission 3.82-fold higher than the one observed in the healthy volunteers and a highly statistically significant correlation between the values measured at different time-points during the monitoring period (P<0.001). The results of this retrospective study indicate that the MMP-9/TIMP-1 ratio may provide information on local changes over time, starting from the triggering insult, and may be considered as a predictive biomarker of burn evolutivity.

15.
Biomedicines ; 9(8)2021 Aug 16.
Article in English | MEDLINE | ID: mdl-34440229

ABSTRACT

Otorhinolaryngology is a vast domain that requires the aid of many resources for optimal performance. The medical devices utilized in this branch share common problems, such as the formation of biofilms. These structured communities of microbes encased in a 3D matrix can develop antimicrobial resistance (AMR), thus making it a problem with challenging solutions. Therefore, it is of concern the introduction in the medical practice involving biomaterials for ear, nose and throat (ENT) devices, such as implants for the trachea (stents), ear (cochlear implants), and voice recovery (voice prosthetics). The surface of these materials must be biocompatible and limit the development of biofilm while still promoting regeneration. In this respect, several surface modification techniques and functionalization procedures can be utilized to facilitate the success of the implants and ensure a long time of use. On this note, this review provides information on the intricate underlying mechanisms of biofilm formation, the large specter of implants and prosthetics that are susceptible to microbial colonization and subsequently related infections. Specifically, the discussion is particularized on biofilm development on ENT devices, ways to reduce it, and recent approaches that have emerged in this field.

16.
Cancers (Basel) ; 13(14)2021 Jul 19.
Article in English | MEDLINE | ID: mdl-34298820

ABSTRACT

Autoimmune thyroiditis (AIT) may impair radioiodine (131I) uptake in papillary thyroid cancer (PTC). Finding the mechanisms that govern immune cells during 131I therapy of PTC with concomitant AIT (PTC + AIT) could provide a rationale. Our study aimed to evaluate the effects of 131I on anti-thyroglobulin antibodies (TgAb), matrix metalloproteinase-9 (MMP-9) and its tissue inhibitor TIMP-1 and tumor necrosis factor-α (TNF-α) and its receptors TNFR1 and TNFR2, in PTC and PTC + AIT patients. Peripheral blood was collected from 56 female patients with PTC and 32 with PTC + AIT before and 4 days after 131I (3.7 GBq). The serum levels of TgAb, MMP-9, TIMP-1, TNF-α, TNFR1 and TNFR2 were measured by ELISA. The mean radioactivity of blood samples collected after 131I intake was higher in the PTC + AIT group than in PTC (p < 0.001). In the PTC + AIT group, TNF-α/TNFR1 and TNF-α/TNFR2 ratios decreased by 0.38-fold and 0.32-fold after 131I and were positively correlated with the MMP-9/TIMP-1 ratio (r = 0.48, p = 0.005, and r = 0.46, p = 0.007). In the PTC group, TNF-α/TNFR1 and TNF-α/TNFR2 ratios increased by 3.17-fold and 3.33-fold and were negatively correlated with the MMP-9/TIMP-1 ratio (r = -0.62, p < 0.001 and r = -0.58, p < 0.001). Our results demonstrate that TNF-α may exert different antitumor effects in response to 131I therapy depending on the patient's immune profile.

17.
Pharmaceutics ; 13(6)2021 May 27.
Article in English | MEDLINE | ID: mdl-34072188

ABSTRACT

Thousands of microorganisms coexist within the human microbiota. However, certain conditions can predispose the organism to the overgrowth of specific pathogens that further lead to opportunistic infections. One of the most common such imbalances in the normal oral flora is the excessive growth of Candida spp., which produces oral candidiasis. In immunocompromised individuals, this fungal infection can reach the systemic level and become life-threatening. Hence, prompt and efficient treatment must be administered. Traditional antifungal agents, such as polyenes, azoles, and echinocandins, may often result in severe adverse effects, regardless of the administration form. Therefore, novel treatments have to be developed and implemented in clinical practice. In this regard, the present paper focuses on the newest therapeutic options against oral Candida infections, reviewing compounds and biomaterials with inherent antifungal properties, improved materials for dental prostheses and denture adhesives, drug delivery systems, and combined approaches towards developing the optimum treatment.

18.
Nanomaterials (Basel) ; 11(5)2021 May 17.
Article in English | MEDLINE | ID: mdl-34067544

ABSTRACT

The inner ear is sensitive to various infections of viral, bacterial, or fungal origin, which, if left untreated, may lead to hearing loss or progress through the temporal bone and cause intracranial infectious complications. Due to its isolated location, the inner ear is difficult to treat, imposing an acute need for improving current therapeutic approaches. A solution for enhancing antimicrobial treatment performance is the use of nanoparticles. Different inorganic, lipidic, and polymeric-based such particles have been designed, tested, and proven successful in the controlled delivery of medication, improving drug internalization by the targeted cells while reducing the systemic side effects. This paper makes a general presentation of common inner ear infections and therapeutics administration routes, further focusing on newly developed nanoparticle-mediated treatments.

19.
J Clin Med ; 10(10)2021 May 17.
Article in English | MEDLINE | ID: mdl-34067548

ABSTRACT

Background-Children with genetic disorders have multiple anatomical and physiological conditions that predispose them to obstructive sleep apnea syndrome (OSAS). They should have priority access to polysomnography (PSG) before establishing their therapeutic protocol. We analyzed the prevalence and the severity of OSAS in a particular group of children with genetic disorders and strengthened their need for a multidisciplinary diagnosis and adapted management. Methods-The retrospective analysis included children with genetic impairments and sleep disturbances that were referred for polysomnography. We collected respiratory parameters from sleep studies: apnea-hypopnea index (AHI), SatO2 nadir, end-tidal CO2, and transcutaneous CO2. Subsequent management included non-invasive ventilation (NIV) or otorhinolaryngological (ENT) surgery of the upper airway. Results-We identified 108 patients with neuromuscular disorders or multiple congenital anomalies. OSAS was present in 87 patients (80.5%), 3 of whom received CPAP, 32 needed another form of NIV during sleep, and 15 patients were referred for ENT surgery. The post-therapeutic follow-up PSG parameters confirmed the success of the treatment. Conclusions-The upper airway obstruction diagnostics and management for children with complex genetic diseases need a multidisciplinary approach. Early detection and treatment of sleep-disordered breathing in children with genetic disorders is a priority for improving their quality of life.

20.
Eur Arch Otorhinolaryngol ; 278(8): 2723-2732, 2021 Aug.
Article in English | MEDLINE | ID: mdl-32897440

ABSTRACT

PURPOSE: Hearing preservation cochlear implantation is an established procedure in patients with low-frequency residual hearing, especially in pediatric cochlear implantation. More delicate, thin electrode arrays can minimize damage in the inner ear and enhance the possibility for residual hearing preservation. The Cochlear® CI532 electrode has been reported as an electrode with the potential for residual hearing preservation. No similar studies pertaining to hearing preservation in pediatric patients have appeared to date. The aim of this study was to investigate whether the Cochlear® CI532 Slim Modiolar electrode allows the preservation of low-frequency residual hearing in children undergoing cochlear implantation. METHODS: In this multicenter, nonrandomized, prospective clinical cohort study, medical data of 14 pediatric patients implanted with the CI532 were collected. All patients had residual low-frequency hearing (preoperative audiogram or ABR with at least one threshold better than 90 dB HL at 125, 250, 500, or 1000 Hz). Postoperative thresholds were obtained 1, 3, 6, and 12 months after cochlear implantation. RESULTS: Based on the HEARRING classification, 78.6% of children (11/14) had complete hearing preservation at the last follow-up visit (12 months after CI, or if not available, 6 months). A total of 21.4% (3/14) had partial hearing preservation. At the last follow-up visit, neither minimal hearing preservation nor loss of hearing was observed. Functional low-frequency hearing was preserved in 13 out of 14 patients (93%). CONCLUSIONS: The residual hearing preservation results in children were superior to the results previously reported in adults.


Subject(s)
Cochlear Implantation , Cochlear Implants , Speech Perception , Audiometry, Pure-Tone , Auditory Threshold , Child , Cohort Studies , Hearing , Humans , Prospective Studies , Retrospective Studies , Treatment Outcome
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