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1.
Pediatr Rev ; 45(4): 188-200, 2024 Apr 01.
Article in English | MEDLINE | ID: mdl-38556515

ABSTRACT

Nasal obstruction, rhinorrhea, and epistaxis are common presenting concerns in primary care clinics. Nasal disorders affect the quality of life for many children and families. Rarely, these complaints may represent a life-threatening condition among infant obligate nasal breathers or cases of unusual pathology. The most common causes of rhinorrhea and nasal obstruction vary by age and include physiologic, infectious, allergic, foreign body, irritant, and traumatic causes. Less commonly, children may have congenital malformations, sinonasal masses, or autoimmune disease. The most common causes of epistaxis are inflammatory, environmental, and traumatic causes and medication misuse, but rarely, children may have predisposing anatomic, hematologic, or vascular abnormalities or even sinonasal tumors. In this article, we provide a thorough review of the common nasal disorders treated every day in primary care clinics and mention briefly some of the rare but serious cases that may be overlooked without considering a full differential diagnosis.


Subject(s)
Nasal Obstruction , Nose Diseases , Child , Infant , Humans , Nasal Obstruction/diagnosis , Nasal Obstruction/etiology , Nasal Obstruction/therapy , Epistaxis/therapy , Epistaxis/complications , Quality of Life , Nose Diseases/complications , Rhinorrhea
2.
Lancet Haematol ; 11(1): e27-e37, 2024 Jan.
Article in English | MEDLINE | ID: mdl-37980924

ABSTRACT

BACKGROUND: Paediatric patients with acute lymphoblastic leukaemia or lymphoma are at increased risk of venous thromboembolism resulting in increased mortality and morbidity. We hypothesised that apixaban, a direct oral anticoagulant, would safely reduce venous thromboembolism in this patient population. METHODS: PREVAPIX-ALL was a phase 3, open-label, randomised, controlled trial conducted in 74 paediatric hospitals in 9 countries. Participants aged 1 year or older to younger than 18 years with newly diagnosed acute lymphoblastic leukaemia (pre-B cell or T cell) or lymphoblastic lymphoma (B cell or T cell immunophenotype) and a central venous line in place throughout induction were randomly assigned 1:1 to standard of care (SOC, ie, no systemic anticoagulation) or weight-adjusted twice-daily apixaban during induction. Randomisation was performed centrally and stratified by age (those <10 years or those ≥10 years). Participants weighing 35 kg or less were administered 2·5 mg twice daily of apixaban as a 2·5 mg tablet, 0·5 mg tablets, or 0·4 mg/mL oral solution, while those weighing more than 35 kg were administered weight-adjusted prophylactic doses using 0·5 mg tablets or the 0·4 mg/mL oral solution twice daily. Primary outcomes were assessed by a blinded central adjudication committee. The primary efficacy outcome for the intention to treat population was the composite of symptomatic or clinically unsuspected venous thromboembolism, the primary safety outcome was major bleeding, and secondary safety outcomes included clinically relevant non-major (CRNM) bleeding. Patients were screened for venous thromboembolism by ultrasound and echocardiogram at the end of induction. The trial was registered with ClinicalTrials.gov (NCT02369653) and is now complete. FINDINGS: Between Oct 22, 2015, and June 4, 2021, 512 participants were randomly assigned and included in analyses (222 [43%] female and 290 [57%] male; 388 [76%] White, 52 [10%] Asian, 24 [5%] Black or African American, and 48 [9%] other races; and 122 [24%] Hispanic or Latino ethnicity). During a median follow-up period of 27 days (IQR 26-28), 31 (12%) of 256 patients on apixaban had a composite venous thromboembolism compared with 45 (18%) of 256 participants receiving SOC (relative risk [RR] 0·69, 95% CI 0·45-1·05; p=0·080). Two major bleeding events occurred in each group (RR 1·0, 95% CI 0·14-7·01; p=1·0). A higher incidence of CRNM bleeding, primarily grade 1 or 2 epistaxis, occurred in the apixaban group (11 [4%] of 256 participants) compared with the SOC group (3 [1%] of 256; RR 3·67, 95% CI 1·04-12·97, p=0·030). The most frequent grade 3-5 adverse events in both groups were thrombocytopenia (n=28 for the apixaban group and n=20 for the SOC group) or platelet count decreased (n=49 and n=45), anaemia (n=77 and n=74), febrile neutropenia (n=27 and n=20), and neutropenia (n=16 and n=17) or neutrophil count decreased (n=22 and n=25). Five deaths occurred, which were due to infection (n=3 in the SOC group), cardiac arrest (n=1 in apixaban group), and haemorrhagic cerebral sinus vein thrombosis (n=1 in the SOC group). There was one apixaban-related death (coagulopathy and haemorrhage after cardiac arrest of unknown cause). INTERPRETATION: PREVAPIX-ALL is, to our knowledge, the first trial assessing primary thromboprophylaxis using a direct oral anticoagulant in paediatric patients with acute lymphoblastic leukaemia or lymphoma. No statistically significant treatment benefit was identified in participants receiving apixaban. Major and CRNM bleeding were infrequent overall, but a higher incidence of CRNM bleeding (primarily epistaxis in younger children) occurred in participants receiving apixaban. For patients deemed to be at particularly high risk of thrombosis, PREVAPIX-ALL provides encouraging safety data for the use of apixaban in clinical settings in which the potential benefits are thought to outweigh the risk of bleeding. FUNDING: Bristol Myers Squibb-Pfizer Alliance.


Subject(s)
Heart Arrest , Lymphoma , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Thrombosis , Venous Thromboembolism , Humans , Male , Female , Child , Anticoagulants/adverse effects , Venous Thromboembolism/etiology , Venous Thromboembolism/prevention & control , Venous Thromboembolism/drug therapy , Epistaxis/chemically induced , Epistaxis/complications , Epistaxis/drug therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Thrombosis/drug therapy , Lymphoma/drug therapy , Heart Arrest/chemically induced , Heart Arrest/complications , Heart Arrest/drug therapy , Treatment Outcome
3.
Front Immunol ; 14: 1321182, 2023.
Article in English | MEDLINE | ID: mdl-38143764

ABSTRACT

Hereditary hemorrhagic telangiectasia (HHT) is a rare inherited disease due to heterozygous loss-of-function mutations on the BMP9/10 pathway (ENG, ACVRL1 or MADH4 mainly). HHT endothelial cells are prone to lose their quiescence, leading to progressive appearance of numerous telangiectases on skin and mucosa (complicated by epistaxis and anemia), and to larger arteriovenous malformations in lungs, liver and brain. HHT is also associated with T lymphocyte abnormalities, which are currently poorly understood. We quantified by flow-cytometry the main T lymphocyte circulating subsets in 40 HHT patients and 20 matched healthy controls. Immunostaining was done on 2 HHT skin telangiectases. Disruptions in T lymphocyte homeostasis was observed, characterized by increases in subsets known to promote angiogenesis: Th2 (1.38% vs 1.15%, p=0.021), Th17 (0.32% vs 0.22%, p=0.019 2) and Treg (4.94% vs 3.51%, p= 0.027). T angiogenic lymphocytes (Tang), defined as CD3+CD31+CXCR4+ T cells, were at similar levels in both groups, but the proportion of VEGF-A+ Tang after stimulation was higher in the HHT group compared to controls (68.2% vs 44.9%, p=0.012). The global HHT T lymphopenia predominantly affected the effector memory T-helper cells (200 vs 270 cells/mm3, p=0.017), and the lymphocytic infiltrate around HHT telangiectases consisted of memory T-helper cells. The Th17 circulating subset was positively correlated with the monthly epistaxis duration (r coefficient: +0,431, p=0.042), prospectively assessed. HHT T-helper lymphocytes are affected by several pro-angiogenic changes, potentially resulting from their recruitment by abnormal endothelial cells. They could constitute a biologically relevant source of VEGF-A and a valuable therapeutic target in HHT.


Subject(s)
Telangiectasia, Hereditary Hemorrhagic , Telangiectasis , Humans , Telangiectasia, Hereditary Hemorrhagic/genetics , Epistaxis/complications , Endothelial Cells , Vascular Endothelial Growth Factor A , Telangiectasis/complications , T-Lymphocytes, Helper-Inducer , Activin Receptors, Type II
4.
Ter Arkh ; 95(7): 580-585, 2023 Sep 29.
Article in Russian | MEDLINE | ID: mdl-38159009

ABSTRACT

Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant disease. It is characterized by vascular dysplasia with the formation of telangiectasias on the skin, mucous membranes of the respiratory and digestive tracts, arteriovenous malformations (AVMs) in the internal organs, which is manifested by bleeding. Diagnosis is based on Curacao criteria: recurrent and spontaneous nosebleeds, multiple telangiectases on the characteristic localizations, AVMs in one or more of the internal organs, a family history of HHT (i.e. first-degree relative who meets these same criteria for definite HHT). Therapy is aimed at preventing and stopping gastrointestinal, nosebleeds, correction of iron deficiency anemia. A promising method of therapy is the use of angiogenesis inhibitors, in particular bevacizumab. The article presents a description of a clinical case of HHT in a 49-year-old woman with telangiectisia on the mucous membrane of the tongue, gastrointestinal tract and liver AVMs.


Subject(s)
Anemia, Iron-Deficiency , Telangiectasia, Hereditary Hemorrhagic , Female , Humans , Middle Aged , Telangiectasia, Hereditary Hemorrhagic/complications , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Telangiectasia, Hereditary Hemorrhagic/drug therapy , Anemia, Iron-Deficiency/drug therapy , Epistaxis/complications , Epistaxis/drug therapy , Bevacizumab/therapeutic use , Angiogenesis Inhibitors
5.
J Med Case Rep ; 17(1): 472, 2023 Nov 14.
Article in English | MEDLINE | ID: mdl-37957719

ABSTRACT

BACKGROUND: Congenital erythropoietic porphyria (CEP), also known as pink tooth or Gunther disease, is a rare hereditary disorder caused by an enzyme mutation in the heme biosynthesis pathway, which leads to the accumulation of immature and non-physiological protoporphyrin rings in various tissues. CEP is characterized by sun-exposed bullous skin lesions, hemolytic anemia, red/brown urine, and teeth staining. CASE PRESENTATION: We present a unique case of a 10-year-old Asian boy with CEP who presented with recurrent epistaxis, an unusual presentation for this condition. Based on clinical presentation and laboratory findings, including elevated urine uroporphyrin and coproporphyrin I and III levels, microcytic anemia, a higher red cell distribution width (RDW), and a lower platelet count, a thorough assessment and detailed workup resulted in a diagnosis of CEP. The patient underwent a successful splenectomy and recovered without any complications. CONCLUSION: This case report aims to raise awareness among healthcare professionals about the uncommon and atypical presentation of CEP and its management options.


Subject(s)
Anemia, Hemolytic , Porphyria, Erythropoietic , Male , Humans , Child , Porphyria, Erythropoietic/complications , Porphyria, Erythropoietic/diagnosis , Porphyria, Erythropoietic/genetics , Epistaxis/complications , Mutation
6.
Ren Fail ; 45(2): 2282710, 2023.
Article in English | MEDLINE | ID: mdl-37975167

ABSTRACT

A 68-year-old male, who was undergoing XELOX plus trastuzumab therapy for gastric cancer, developed proteinuria, hematuria, and progressive increase in creatinine after 3 months. Subsequently, the patient also experienced hemoptysis, nasal bleeding. Chest CT examination shown pulmonary hemorrhage. The MRI of the nasopharynx ruled out nasopharyngeal cancer recurrence. The MPO and PR3 were elevated, and renal biopsy confirmed ANCA-related vasculitis, which affected the lungs, kidneys, and nasopharynx. Based on the review of the patient''s medical history and medication, it is believed that ANCA-related vasculitis was caused by XELOX plus trastuzumab chemotherapy, but it is difficult to confirm which specific drug caused it. After stopping XELOX plus trastuzumab chemotherapy, glucocorticoids and cyclophosphamide was given, the patient''s pulmonary hemorrhage and nasal bleeding stopped, and the lung lesions were absorbed. The renal function also improved. The patient later experienced pulmonary infection again, and tNGS indicated Legionella pneumophila and pulmonary tuberculosis infection. Despite anti-infection treatment, steroid dose was rapidly reduced. Ultimately, the patient gave up on treatment and eventually died.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Kidney Diseases , Lung Diseases , Nasopharyngeal Neoplasms , Male , Humans , Aged , Oxaliplatin , Antibodies, Antineutrophil Cytoplasmic , Trastuzumab/adverse effects , Capecitabine , Epistaxis/complications , Nasopharyngeal Neoplasms/complications , Neoplasm Recurrence, Local/complications , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/chemically induced , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications , Lung Diseases/chemically induced , Kidney Diseases/complications , Peroxidase
7.
BMC Urol ; 23(1): 173, 2023 Oct 27.
Article in English | MEDLINE | ID: mdl-37891557

ABSTRACT

BACKGROUND: To investigate the association between erectile dysfunction (ED) as well as epistaxis (ES) in relation to the extent of iliac atherosclerosis. METHODS: In this retrospective cross-sectional study, all consecutive male patients treated at our institution from 01/2016 to 12/2020 undergoing abdominal CT scan were evaluated. Patients (n = 1272) were invited by mail to participate in the study in returning two questionnaires for the evaluation of ED (IIEF-5) and ES. Patients who returned filled-in questionnaires within a 3-month deadline were included in the study. The extent of atherosclerosis in the common iliac artery (CIA) and the internal iliac artery (IIA) was assessed by calcium scoring on unenhanced CT. Stratification of results was performed according to reported IIEF-5 scores and consequential ED groups. RESULTS: In total, 437 patients (34.4% of contacted) met the inclusion criteria. Forty-two patients did not fulfill predefined age requirements (< 75 years) and 120 patients had to be excluded as calcium scoring on nonenhanced CT was not feasible. Finally, 275 patients were included in the analysis and stratified into groups of "no-mild" (n = 146) and "moderate-severe" (n = 129) ED. The calcium score (r=-0.28, p < 0.001) and the number of atherosclerotic lesions (r=-0.32, p < 0.001) in the CIA + IIA showed a significant negative correlation to the IIEF-5 score, respectively. Patients differed significantly in CIA + IIA calcium score (difference: 167.4, p < 0.001) and number of atherosclerotic lesions (difference: 5.00, p < 0.001) when belonging to the "no-mild" vs. "moderate-severe" ED group, respectively. A multivariable regression model, after adjusting for relevant baseline characteristics, showed that the number of atherosclerotic CIA + IIA lesions was an independent predictor of ED (OR = 1.05, p = 0.036), whereas CIA + IIA calcium score was not (OR = 1.00031, p = 0.20). No relevant correlation was found between ES episodes and IIEF-5 scores (r=-0.069, p = 0.25), CIA + IIA calcium score (r=-0.10, p = 0.87) or number of atherosclerotic CIA + IIA lesions (r=-0.032, p = 0.60), respectively. CONCLUSIONS: The number of atherosclerotic lesions in the iliac arteries on nonenhanced abdominal CT scans is associated with the severity of ED. This may be used to identify subclinical cardiovascular disease and to quantify the risk for cardiovascular hazards in the future. TRIAL REGISTRATION: BASEC-Nr. 2020 - 01637.


Subject(s)
Atherosclerosis , Erectile Dysfunction , Humans , Male , Aged , Erectile Dysfunction/diagnostic imaging , Erectile Dysfunction/complications , Iliac Artery/diagnostic imaging , Retrospective Studies , Calcium , Cross-Sectional Studies , Epistaxis/complications , Atherosclerosis/complications , Atherosclerosis/diagnostic imaging , Tomography, X-Ray Computed
8.
Zhonghua Jie He He Hu Xi Za Zhi ; 46(6): 558-564, 2023 Jun 12.
Article in Chinese | MEDLINE | ID: mdl-37278169

ABSTRACT

Objective: To describe the clinical characteristics, diagnosis, genetic features and treatment of hereditary pulmonary hypertension complicated with suspected hereditary hemorrhagic telangiectasia (HHT). Methods: Firstly, we summarized and analyzed the clinical data of two cases of suspected HHT admitted to the Department of Pulmonary and Critical Care Medicine, the Second Xiangya Hospital, Central South University. Secondly, the genes of peripheral blood of patients and their families were completely sequenced and sanger sequencing was performed to verify the variation sites, and then the mRNA deletion caused by the variation was further verified. Thirdly, "HHT" "FPAH" and "BMPR2 gene variation" were used as keywords,and the related literatures of Wanfang database and PubMed database from January 2000 to November 2021 were searched and reviewed. Results: We found two patients in a family from Yiyang, Hunan province, who had symptoms of hemoptysis or pulmonary hypertension without epistaxis or other clinical features of HHT. However, both patients had pulmonary vascular abnormalities and pulmonary hypertension in their lungs. We found that BMPR2 gene variation (NM_001204.7:c.1128+1G>T) was positive and ENG, ACVRL1 and SMAD4 genes were negative. Family analysis and Sanger verification were carried out on 16 individuals in 4 generations of the family (7 of whom were found to carry the mutant gene), and then transcriptional level mRNA sequencing further confirmed that the variation resulted in the deletion of exon 8 and exon 9, and amino acid sequence estimation revealed that the amino acids of the protein from 323 to 425 were deleted. We thought that the incomplete translation of BMPR2 gene could lead to BMPRⅡ dysfunction. Therefore, it was diagnosed as hereditary pulmonary hypertension with suspected HHT. Both patients were suggested to reduce the pulmonary artery pressure, and at the same time, the whole-body imaging examination should be performed to screen other arteriovenous malformations, and the annual cardiac color Doppler ultrasound should be reviewed to evaluate the changes of pulmonary artery pressure. Conclusions: Hereditary pulmonary hypertension (HPAH) is a group of diseases with increasing pulmonary vascular resistance caused by genetic factors, including familial PAH and simple PAH. Variation in the BMPR2 gene is an important pathogenic factor of HPAH. Therefore, we should pay attention to the inquiry of family history when we clinically encounter young patients with pulmonary hypertension. If the cause is unknown, genetic testing is recommended. HHT is a rare autosomal dominant genetic disease. The possibility of this disease should be considered in clinical manifestations such as familial pulmonary vascular abnormality, pulmonary hypertension and recurrent epistaxis. There is no effective specific treatment for HPAH and HHT, which are treated symptomatically (including blood pressure reduction and hemostasis, etc.). It is suggested for these patients that pulmonary artery pressure should be dynamically monitored and have genetic counseling before giving birth.


Subject(s)
Hypertension, Pulmonary , Telangiectasia, Hereditary Hemorrhagic , Pregnancy , Humans , Female , Hypertension, Pulmonary/diagnosis , Epistaxis/complications , Telangiectasia, Hereditary Hemorrhagic/genetics , Telangiectasia, Hereditary Hemorrhagic/complications , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Lung/pathology , Mutation , Bone Morphogenetic Protein Receptors, Type II/genetics , Activin Receptors, Type II/genetics
9.
Int Arch Allergy Immunol ; 184(9): 882-892, 2023.
Article in English | MEDLINE | ID: mdl-37290409

ABSTRACT

INTRODUCTION: Allergic rhinitis (AR) in children is associated with various comorbidities, posing challenges for treatment and management. There have been few investigations of these multimorbidities in Chinese children with AR. Here, we investigated the prevalence of multimorbidities in children with moderate to severe AR and analyzed the influencing factors using real-world data. METHODS: In total, 600 children who visited the outpatient clinic of our hospital and were diagnosed with moderate-severe AR were prospectively enrolled. All children underwent allergen detection and electronic nasopharyngoscopy. Parents or guardians completed a questionnaire that included age, sex, mode of delivery, feeding pattern, and familial history of allergy. The multimorbidities investigated included atopic dermatitis (AD), asthma, allergic conjunctivitis (AC), chronic rhinosinusitis (CRS), adenoid hypertrophy (AH), tonsil hypertrophy (TH), recurrent epistaxis, and recurrent respiratory tract infections (RRTIs). RESULTS: The AR multimorbidities reported in children were as follows: recurrent epistaxis (46.5%), AC (46.3%), AD (40.7%), asthma (22.5%), RRIs (21.3%), CRS (20.5%), AH (19.7%), and TH (12.5%). In univariate logistic regression analysis, age (<6 years), birth mode, familial history of allergy, and single dust mite allergy were associated with AR multimorbidity (p < 0.05). Multivariate logistic regression revealed that a familial history of allergy was an independent risk factor for AC (odds ratio [OR] = 1.539, 95% confidence interval [CI]: 1.104-2.145) and AH (OR = 1.506, 95% CI: 1.000-2.267) (p < 0.05). Age (<6 years) was independently associated with the risk of AD (OR = 1.405, 95% CI: 1.003-1.969) and RRTIs (OR = 1.869, 95% CI: 1.250-2.793) (p < 0.05), cesarean section with AR and CRS risk (OR = 1.678, 95% CI: 1.100-2.561), and single dust mite allergy with asthma (OR = 1.590, 95% CI: 1.040-2.432) and CRS (OR = 1.600, 95% CI: 1.018-2.515) risk (p < 0.05). Further, non-dust mite allergy was independently associated with AR and CRS (OR = 2.056, 95% CI: 1.084-3.899). CONCLUSION: AR was found to be accompanied by different comorbidities, including both allergic and non-allergic comorbidities, complicating disease treatment. These findings demonstrated that age (<6 years), familial history of allergy, types of allergens, and cesarean section were risk factors for different multimorbidities associated with AR.


Subject(s)
Asthma , Conjunctivitis, Allergic , Dermatitis, Atopic , Respiratory Tract Infections , Rhinitis, Allergic , Sinusitis , Pregnancy , Humans , Child , Female , Multimorbidity , Cesarean Section/adverse effects , Epistaxis/complications , Rhinitis, Allergic/epidemiology , Rhinitis, Allergic/complications , Asthma/etiology , Allergens , Dermatitis, Atopic/epidemiology , Conjunctivitis, Allergic/epidemiology , Sinusitis/epidemiology , Chronic Disease , Respiratory Tract Infections/complications , Hypertrophy/complications
10.
BMC Endocr Disord ; 23(1): 107, 2023 May 12.
Article in English | MEDLINE | ID: mdl-37173679

ABSTRACT

BACKGROUND: Giant prolactinoma (> 4 cm in dimension) is a rare disorder. Invasive macroprolactinoma has the potential to cause base of skull erosion and extend into the nasal cavity or even the sphenoid sinus. Nasal bleeding caused by intranasal tumor extension is a rare complication associated with invasive giant prolactinoma. We report a case of giant invasive macroprolactinoma with repeated nasal bleeding as the initial symptom. CASE PRESENTATION: A 24-year-old man with an invasive giant prolactinoma in the nasal cavity and sellar region who presented with nasal bleeding as the initial symptom, misdiagnosed as olfactory neuroblastoma. However, markedly elevated serum prolactin levels (4700 ng/mL), and a 7.8-cm invasive sellar mass confirmed the diagnosis of invasive giant prolactinoma. He was treated with oral bromocriptine. Serum prolactin was reduced to near normal after 6 months of treatment. Follow-up magnetic resonance imaging showed that the sellar lesion had disappeared completely and the skull base lesions were reduced. CONCLUSION: This case is notable in demonstrating the aggressive nature of untreated invasive giant prolactinomas which can cause a diagnostic difficulty with potential serious consequences. Early detection of hormonal levels can avoid unnecessary nasal biopsy. Early identification of pituitary adenoma with nasal bleeding as the first symptom is particularly important.


Subject(s)
Pituitary Neoplasms , Prolactinoma , Male , Humans , Young Adult , Adult , Prolactinoma/diagnosis , Prolactinoma/diagnostic imaging , Epistaxis/complications , Epistaxis/drug therapy , Prolactin , Pituitary Neoplasms/diagnosis , Pituitary Neoplasms/diagnostic imaging , Bromocriptine/therapeutic use , Magnetic Resonance Imaging
11.
Ann Med ; 55(1): 2200257, 2023 12.
Article in English | MEDLINE | ID: mdl-37074291

ABSTRACT

Aim: To investigate the treatment of intractable epistaxis after radiotherapy for nasopharyngeal carcinoma (NPC).Methods: This review focuses on the anatomy and pathophysiology, mechanism, and clinical treatments of epistaxis after NPC radiotherapy.Results: For treating NPC, radiation therapy is the primary therapeutic modality. However, radiotherapy can lead to varied degrees of harm to the neighboring tissues and is correlated with numerous complications. Among these complications, epistaxis is a common occurrence after NPC radiotherapy, owing to damage to the surrounding tissues caused by radiotherapy. Unfortunately, epistaxis, particularly carotid blowout, can have a dangerous course and a high mortality rate. Accurate understanding of epistaxis following radiotherapy, prompt bleeding cessation, and reduction of bleeding volume are key considerations. Nasal tamponade is a crucial rescue treatment, while tracheotomy is an active and effective method. Intravascular balloon embolization is a reliable and effective treatment method for ICA hemorrhage, and vascular embolization is the primary approach for treating external carotid artery maxillary bleeding. Implantation of a covered stent can achieve hemostasis without altering hemodynamics.Conclusion: A comprehensive approach utilizing these methods can improve the success rate of treating nosebleeds following NPC radiotherapy.HighlightsThe mortality rate for carotid blowout following radiotherapy for NPC is high.Radiation therapy and tumor condition are correlated with epistaxis in NPC.Treatment methods for NPC-related epistaxis include posterior nostril tamponade, endoscopic hemostasis, DSA, selective vascular embolization, and stent implantation.The use of a covered stent for NPC-related carotid blowout achieves hemostasis without altering blood perfusion.Effective and timely application of various hemostasis methods is key to improving the success rate of rescue, considering the characteristics of NPC-related epistaxis.


Subject(s)
Embolization, Therapeutic , Nasopharyngeal Neoplasms , Humans , Nasopharyngeal Carcinoma/complications , Nasopharyngeal Carcinoma/therapy , Nasopharyngeal Neoplasms/complications , Nasopharyngeal Neoplasms/radiotherapy , Epistaxis/therapy , Epistaxis/complications , Carotid Arteries , Embolization, Therapeutic/adverse effects , Embolization, Therapeutic/methods
12.
J Med Genet ; 60(9): 905-909, 2023 09.
Article in English | MEDLINE | ID: mdl-36813543

ABSTRACT

BACKGROUND: EPHB4 loss of function is associated with type 2 capillary malformation-arteriovenous malformation syndrome, an autosomal dominant vascular disorder. The phenotype partially overlaps with hereditary haemorrhagic telangiectasia (HHT) due to epistaxis, telangiectases and cerebral arteriovenous malformations, but a similar liver involvement has never been described. METHODS: Members of the French HHT network reported their cases of EPHB4 mutation identified after an initial suspicion of HHT. Clinical, radiological and genetic characteristics were analysed. RESULTS: Among 21 patients with EPHB4, 15 had a liver imaging, including 7 with HHT-like abnormalities (2 female patients and 5 male patients, ages 43-69 years). Atypical epistaxis and telangiectases were noted in two cases each. They were significantly older than the eight patients with normal imaging (median: 51 vs 20 years, p<0.0006).The main hepatic artery was dilated in all the cases (diameter: 8-11 mm). Six patients had hepatic telangiectases. All kind of shunts were described (arteriosystemic: five patients, arterioportal: two patients, portosystemic: three patients). The overall liver appearance was considered as typical of HHT in six cases.Six EPHB4 variants were classified as pathogenic and one as likely pathogenic, with no specific hot spot. CONCLUSION: EPHB4 loss-of-function variants can be associated with HHT-like hepatic abnormalities and should be tested for atypical HHT presentations.


Subject(s)
Intracranial Arteriovenous Malformations , Telangiectasia, Hereditary Hemorrhagic , Male , Humans , Female , Telangiectasia, Hereditary Hemorrhagic/complications , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Telangiectasia, Hereditary Hemorrhagic/genetics , Epistaxis/complications , Liver , Mutation
13.
Laryngoscope ; 133(10): 2502-2510, 2023 10.
Article in English | MEDLINE | ID: mdl-36683553

ABSTRACT

INTRODUCTION: Sinonasal inverted papillomas (IP) can undergo transformation into IP-squamous cell carcinomas (IP-SCC). More aggressive treatment plan should be established when IP-SCC is suspected. Nevertheless, inaccuracy of the preoperative punch biopsy results to detect IP-SCC from IP raises the need for an additional strategy. The present study aimed to investigate significant clinicoradiological remarks associated with IP-SCC than IP. MATERIAL AND METHODS: Postoperative surgical specimens obtained from patients with confirmed IP or IP-SCC at a single tertiary medical center from 1997 to 2018 were retrospectively evaluated. Patients' demographic and clinical characteristics, preoperative in-office punch biopsy results, and preoperative computed tomography (CT) or magnetic resonance images were reviewed. Univariate and multivariate analyses were performed to assess the odds ratio (OR) associated with IP-SCC. The area under the curve (AUC) in the receiver Operating Characteristic (ROC) curve was calculated in the prediction model to discriminate IP-SCC from IP. RESULTS: The study included 44 IP-SCC and 301 patients with IP. The diagnostic sensitivity of in-office punch biopsy to detect IP-SCC was 70.7%. Multivariate analysis showed that factors significantly associated with IP-SCC included tobacco smoking >10PY (adjusted-OR [aOR]: 4.1), epistaxis (aOR: 3.4), facial pain (aOR: 4.2), bony destruction (aOR: 37.6), bony remodeling (aOR: 36.3), and invasion of adjacent structures (aOR: 31.6) (all p < 0.05). Combining all significantly related clinicoradiological features, the ability to discriminate IP-SCC from IP reached an AUC of 0.974. CONCLUSION: IP patients with a history of tobacco smoking, facial pain, epistaxis, and bony destruction, remodeling, or invasion of an adjacent structure on preoperative images may be at higher risk for IP-SCC. LEVEL OF EVIDENCE: 3 Laryngoscope, 133:2502-2510, 2023.


Subject(s)
Carcinoma, Squamous Cell , Nose Neoplasms , Papilloma, Inverted , Paranasal Sinus Neoplasms , Humans , Papilloma, Inverted/surgery , Nose Neoplasms/pathology , Epistaxis/complications , Retrospective Studies , Paranasal Sinus Neoplasms/diagnostic imaging , Paranasal Sinus Neoplasms/surgery , Paranasal Sinus Neoplasms/complications , Squamous Cell Carcinoma of Head and Neck/complications , Carcinoma, Squamous Cell/pathology , Facial Pain
14.
Laryngoscope ; 133(10): 2553-2557, 2023 10.
Article in English | MEDLINE | ID: mdl-36688270

ABSTRACT

OBJECTIVE: The treatment of nasal foreign bodies involves safe and reliable removal. Few reports have investigated the relationship between equipment and the incidence of complications. METHODS: This retrospective study included 300 patients with nasal foreign bodies (average: 3.28 years, interquartile range: 2-4 years). Patients' background, characteristics of nasal foreign body, equipment to remove the nasal foreign body, and complications were obtained from medical records. Statistical analysis was performed using Pearson's chi-square test for associated factors and the incidence of epistaxis among the complications. RESULTS: Nasal foreign bodies were found and removed in 256 patients. Forceps, hooks, suction, modified paper clips, and cotton swabs were mainly used to remove the nasal foreign bodies. Epistaxis due to the removal procedure was observed in 26 patients. The occurrence of epistaxis differed depending on the equipment (p = 0.077) and was less frequent in suction and paper clips than in forceps (p < 0.05 and p = 0.077). Epistaxis was not observed when a cotton swab was used. Aspiration and septal perforation were not observed. A statistical relationship was not detected between the hardness of foreign bodies and the occurrence of epistaxis (p = 0.251). The incidence of epistaxis was higher in cases nasal foreign bodies remained for 1 day and over than in cases foreign bodies were removed within 1 day (p < 0.05). CONCLUSIONS: This study revealed that suction, modified paper clips, and cotton swabs could be beneficial options for minimizing complications in the removal of nasal foreign bodies. LEVEL OF EVIDENCE: 4 Laryngoscope, 133:2553-2557, 2023.


Subject(s)
Foreign Bodies , Nose , Humans , Retrospective Studies , Epistaxis/etiology , Epistaxis/complications , Surgical Instruments/adverse effects , Foreign Bodies/epidemiology , Foreign Bodies/etiology , Foreign Bodies/surgery
15.
Pediatr Emerg Care ; 39(5): 342-346, 2023 May 01.
Article in English | MEDLINE | ID: mdl-36706230

ABSTRACT

OBJECTIVE: The aim of this study is to examine the reasons and the methods of approach to the patients for the ear nose and throat (ENT) consultations requested from the patients who applied to the pediatric emergency department. METHODS: The files of 351 patients who applied to the pediatric emergency outpatient clinic and were asked for consultation from the ENT clinic were reviewed retrospectively. Demographic data, complaints on admission, diagnostic examinations, diagnoses, treatment methods, and hospitalizations were recorded. RESULTS: Of the patients included in the study, 190 (54.1%) were female and 161 (45.9%) were male. The median age of the patients was 4.0 years (3.0-8.0 years). The most common diagnoses after ENT examination are; 120 patients (34.2%) had foreign body (FB) in the nose, 58 patients (16.5%) had FB in the ear, 16 patients (4.6%) had FB in the throat, 16 patients (4.6%) had epistaxis, and 15 patients (4.3%) had Bell's palsy. According to age group, it was determined that FB in the nose and ear was more common in the 0- to 5- and 6- to 11-year age group, and Bell's palsy, FB in the ear and epistaxis were more common in the 12- to 17-year age group. A normal examination was also an important finding in 83 of the patients (23.6%). CONCLUSIONS: Foreign bodies are the most common reason for admission to the emergency services in children, and it is frequently seen between 0 and 5 years of age. Informing and raising awareness of parents on this topic will reduce both unwanted complications and ENT consultations along with admission to pediatric emergency services.


Subject(s)
Bell Palsy , Facial Paralysis , Foreign Bodies , Child , Humans , Male , Female , Child, Preschool , Pharynx , Tertiary Care Centers , Epistaxis/complications , Bell Palsy/complications , Retrospective Studies , Foreign Bodies/diagnosis , Foreign Bodies/epidemiology , Foreign Bodies/therapy , Referral and Consultation , Emergency Service, Hospital , Nose
16.
Aesthetic Plast Surg ; 47(1): 294-300, 2023 02.
Article in English | MEDLINE | ID: mdl-35941387

ABSTRACT

OBJECTIVE: In this study, we aimed to investigate the effects of the Ankaferd Blood Stopper (ABS) on nasal bleeding, pain, eating disorders, and sleeping problems in patients undergoing septoplasty. METHODS: The prospective study included 100 patients who underwent septoplasty surgery. Patients were divided into two groups based on the chronological order of hospital admission: Group I was administered the ABS, and Group II was administered a standard Invotec Merocel nasal tampon. In both groups, the intensity of pain experienced by patients postoperatively during tampon use and tampon removal was assessed using the visual analogue scale (VAS). RESULTS: A significant difference was found between the two groups with regard to VAS scores assessed during tampon use. In the ABS group, the VAS scores assessed during tampon use were significantly lower, and the incidence of nasal obstruction, eating disorders, dripping-type bleeding before tampon removal, and sleeping problems was significantly lower in the ABS group than in the Merocel group. CONCLUSIONS: The ABS may be a viable alternative to other nasal packs, as it did not lead to a significant reduction in postoperative patient comfort during tampon use and caused a lower incidence of postoperative pain, eating disorders, sleeping problems, and nasal obstruction than standard Merocel tampons. LEVEL OF EVIDENCE III: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .


Subject(s)
Nasal Obstruction , Rhinoplasty , Humans , Nasal Obstruction/surgery , Nasal Septum/surgery , Prospective Studies , Postoperative Hemorrhage , Rhinoplasty/adverse effects , Epistaxis/complications , Epistaxis/surgery , Pain, Postoperative
17.
J Neurointerv Surg ; 15(10): 1050-1054, 2023 Oct.
Article in English | MEDLINE | ID: mdl-36137744

ABSTRACT

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder characterized by recurrent epistaxis (nose bleeds), mucosal telangiectasias (spider veins), and arteriovenous malformations. Although HHT affects all racial groups, few studies have explored racial disparities among patients with HHT. METHODS: We performed a retrospective chart review of HHT patients who were seen at a single academic center between July 1, 2014 and January 1, 2022. The primary outcomes of this study were the Epistaxis Severity Score (ESS) and the presence of pulmonary, cerebral, gastrointestinal, spinal, and hepatic arteriovenous malformations (AVMs). We analyzed racial differences using t-tests and analysis of variance (ANOVA) for continuous variables, and chi-squared tests for categorical variables. We then performed multivariable linear and logistic regressions on outcomes. RESULTS: Our review identified 35 Asian, 6 Black or African American, 72 Hispanic or Latino, and 244 White or Caucasian patients who met the inclusion criteria. Through an analysis of variance model, race/ethnicity was not significantly associated with ESS. Two univariable logistic regression models between race and both pulmonary and brain AVMs showed that race was associated with the incidence of pulmonary AVMs (p<0.01), with Asian patients at a 2.3-fold increased risk of pulmonary AVMs compared with White patients (p=0.03). Race was also associated with the incidence of cerebral AVMs (p<0.01) with Hispanic or Latino patients at a 4.8-fold increased risk compared with White patients (p<0.01). CONCLUSION: Patients who identified as Asian may have higher rates of pulmonary AVMs while patients identifying as Hispanic or Latino may have more cerebral AVMs. The correlations may be important for identifying risk factors in certain patient populations.


Subject(s)
Intracranial Arteriovenous Malformations , Telangiectasia, Hereditary Hemorrhagic , Humans , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Telangiectasia, Hereditary Hemorrhagic/complications , Telangiectasia, Hereditary Hemorrhagic/epidemiology , Retrospective Studies , Epistaxis/complications , Intracranial Arteriovenous Malformations/complications
18.
Laryngoscope ; 133(8): 1834-1838, 2023 08.
Article in English | MEDLINE | ID: mdl-36354237

ABSTRACT

OBJECTIVE: Endoscopic sinus surgery (ESS) is the procedure of choice for chronic rhinosinusitis (CRS). Adhesions are the most common postoperative complications, causing recurrent disease and revision surgery. Postoperative care is thus essential for the healing of the operated cavity. A wide variety of packing materials are used to prevent bleeding and adhesions postoperatively. Two main absorbable packing materials are used: Foam-based packs (e.g., Posisep and Nasopore) and gel-based packs (PureRegen Gel - PRG). The current study is a randomized, blinded, prospective analysis of cavity healing using Posisep and PRG in ESS, aiming to compare the pros and cons of the two. METHODS: Patients with bilateral symmetric CRS were recruited for the study. At the end of surgery, one side was randomly packed with Posisep, whereas the other was packed with PRG. The postoperative cavity cleaning was video recorded and a blinded physician evaluated the mucosal healing. RESULTS: The side packed with Posisep had significantly less middle turbinate (MT) lateralization and adhesions yet dissolved significantly slower than the PRG, causing more mucosal edema. Severe MT scarring requiring recurrent medialization and adhesiolysis was exclusively observed in the PRG group. All differences were observed in the early postoperative period (up to 12 weeks after surgery). By that time, only the MT position was significantly different between groups, despite recurrent adhesiolysis and medialization. CONCLUSION: The authors recommend using Posisep for MT support only when it is unstable or lateralized. Packing the surgical cavity in other cases with PRG is more beneficial. LEVEL OF EVIDENCE: 2 Laryngoscope, 133:1834-1838, 2023.


Subject(s)
Paranasal Sinuses , Rhinitis , Sinusitis , Humans , Paranasal Sinuses/surgery , Prospective Studies , Sinusitis/surgery , Sinusitis/complications , Epistaxis/complications , Endoscopy/methods , Chronic Disease , Rhinitis/surgery , Rhinitis/complications , Treatment Outcome
19.
Article in English | MEDLINE | ID: mdl-36360682

ABSTRACT

BACKGROUND: Transcatheter left atrial appendage closure (LAAC) is performed in patients unsuitable for long-term anticoagulation, predominantly due to prior bleeding events. The study aimed to investigate the incidence and predictors of clinically significant bleeding (CSB) post-LAAC. METHODS: Consecutive patients after LAAC with an Amplatzer or WATCHMAN device were analyzed (05.2014-11.2019). Bleeding was classified as CSB when associated with at least one of the following: death, ≥2 g/dL hemoglobin drop, ≥2 blood units transfusion, critical anatomic site, or hospitalization/invasive procedure. RESULTS: Among 195 patients (age 74 (68-80), 43.1% females, HAS-BLED score 2.0 (2.0-3.0)), during median follow-up of 370 (IQR, 358-392) days, there were 15 nonprocedural CSBs in 14 (7.2%) patients. Of those, 9 (60.0%) occurred during postprocedural dual antiplatelet therapy (DAPT) (median 46 (IQR: 16-60) days post-LAAC) vs. 6 (40%) after DAPT discontinuation (median 124 (81-210) days post-LAAC), translating into annualized CSB rates of 14.0% (per patient-year on DAPT) vs. 4.6% (per patient-year without DAPT). In 92.9% (13/14) of patients, the post-LAAC nonprocedural CSB was a recurrence from the same site as bleeding pre-LAAC. In the multivariable model, admission systolic blood pressure (SBP) > 127 mmHg (HR = 10.73, 1.37-84.26, p = 0.024), epistaxis history (HR = 5.84, 1.32-25.89, p = 0.020), permanent atrial fibrillation (AF) (HR = 4.55, 1.20-17.20, p = 0.025), and prior gastrointestinal bleeding (HR = 3.35, 1.01-11.08, p = 0.048) predicted post-LAAC CSB. CONCLUSIONS: Nonprocedural CSBs after LAAC, with a similar origin as the pre-LAAC bleedings, were observed predominantly during postprocedural DAPT and predicted by elevated admission SBP, prior epistaxis, permanent AF, and gastrointestinal bleeding history. Whether a more reserved post-LAAC antiplatelet regimen and stringent blood pressure control may improve LAAC outcomes remains to be studied.


Subject(s)
Atrial Appendage , Atrial Fibrillation , Stroke , Female , Humans , Aged , Male , Atrial Appendage/surgery , Incidence , Platelet Aggregation Inhibitors/therapeutic use , Epistaxis/complications , Stroke/epidemiology , Treatment Outcome , Atrial Fibrillation/epidemiology , Atrial Fibrillation/therapy , Atrial Fibrillation/complications , Gastrointestinal Hemorrhage
20.
Lakartidningen ; 1192022 08 24.
Article in Swedish | MEDLINE | ID: mdl-36082917

ABSTRACT

Hereditary haemorrhagic telangiectasia (HHT, Osler disease) is an autosomal dominant disease with a prevalence of about 1 in 5 000. The most common symptom is epistaxis in 90 percent of patients, with an average onset at the age of 12 years. Pulmonary arteriovenous malformations are present in 15-35 percent of patients and are associated with embolic complications, such as stroke and cerebral abscesses.  No causative treatment for HHT exists. Iron deficiency anaemia is a common complication. It is treated with oral or intravenous iron replacement depending on the response to tranexamic acid and local treatments. Bevacizumab has been reported to be effective in reducing bleeding complications as well as hepatic and cardiac failure. A multidisciplinary center for the treatment of HHT was established at the University Hospital in Uppsala in 2009.


Subject(s)
Pulmonary Veins , Telangiectasia, Hereditary Hemorrhagic , Bevacizumab/therapeutic use , Child , Epistaxis/complications , Humans , Pulmonary Artery , Telangiectasia, Hereditary Hemorrhagic/complications , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Telangiectasia, Hereditary Hemorrhagic/drug therapy
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