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1.
Eur J Neurol ; 31(4): e16176, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38064178

ABSTRACT

BACKGROUND AND PURPOSE: Pulmonary arteriovenous malformations (PAVMs) may cause recurrent brain abscess. The primary aim was to determine the prevalence of PAVM amongst survivors of brain abscess. The proportion with cardiac right-to-left shunts was also assessed post hoc. METHODS: This was a cross-sectional population-based study of adult (≥18 years) survivors of cryptogenic bacterial brain abscess in Denmark from 2007 through 2016. Patients were invited for bubble-echocardiography to detect vascular right-to-left shunting and, if abnormal, subsequent computed tomography thorax for diagnosis of PAVM. Data are presented as n/N (%) or median with interquartile range (IQR). RESULTS: Study participation was accepted by 47/157 (30%) eligible patients amongst whom two did not appear for scheduled bubble-echocardiography. The median age of participants was 54 years (IQR 45-62) and 19/57 (33%) were females compared with 59 years (IQR 48-68, p = 0.05) and 41/85 females (48%, p = 0.22) in non-participants. Bubble-echocardiography was suggestive of shunt in 10/45 (22%) participants and PAVM was subsequently confirmed by computed tomography in one patient with grade 1 shunting. The corresponding prevalence of PAVM was 2% (95% confidence interval 0.06-11.8) amongst all examined participants. Another 9/45 (20%) were diagnosed with patent in persistent foramen ovale (n = 8) or atrial septum defect (n = 1), which is comparable with the overall prevalence of 25% amongst adults in the Danish background population. CONCLUSIONS: Undiagnosed PAVM amongst adult survivors of cryptogenic bacterial brain abscess is rare but may be considered in select patients. The prevalence of cardiac right-to-left shunts amongst brain abscess patients corresponds to the prevalence in the general population.


Subject(s)
Arteriovenous Fistula , Arteriovenous Malformations , Brain Abscess , Pulmonary Artery/abnormalities , Pulmonary Veins/abnormalities , Telangiectasia, Hereditary Hemorrhagic , Adult , Female , Humans , Middle Aged , Male , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Telangiectasia, Hereditary Hemorrhagic/epidemiology , Cross-Sectional Studies , Arteriovenous Malformations/diagnosis , Arteriovenous Malformations/etiology , Brain Abscess/complications , Brain Abscess/epidemiology
2.
Eur J Neurol ; 31(2): e16128, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37955551

ABSTRACT

BACKGROUND AND PURPOSE: Hereditary haemorrhagic telangiectasia (HHT) is a genetic disease with fragile blood vessels and vascular malformations, potentially causing neurological manifestations, including stroke and cerebral abscesses. The study aimed to investigate neurological manifestations in the Danish HHT database, focusing on pulmonary arteriovenous malformations (PAVMs) as a risk factor for cerebral events. METHODS: Retrospective analysis of the Danish HHT database was conducted, cross-referencing neurological outcomes with the Danish Apoplexy Register for accuracy. Patients were stratified by HHT type. Primary outcomes included ischaemic stroke, transient ischaemic attack and cerebral haemorrhage. Secondary outcomes comprised age, age at HHT diagnosis, age at cerebral ischaemic event, and PAVM and cerebral arteriovenous malformation status. RESULTS: Six hundred and sixty-four HHT patients were included. PAVM was diagnosed in 54% of patients, with higher prevalence in HHT type 1 (70%) compared to HHT type 2 (34%) and juvenile polyposis HHT (66%). Ischaemic stroke or transient ischaemic attack occurred in 12.5%, with a higher risk associated with macroscopic PAVM. Logistic regression showed a nearly 10 times increased risk of ischaemic stroke with macroscopic PAVM. Cerebral abscesses occurred in 3.2% of patients, all with macroscopic PAVM. Incomplete PAVM closure increased cerebral abscess risk. CONCLUSION: This study provides valuable insights into the prevalence of neurological manifestations and vascular events in HHT patients. The presence of PAVM was associated with an increased risk of ischaemic stroke, highlighting the importance of early screening and intervention. The findings emphasize the need for comprehensive management strategies targeting both vascular and neurological complications in HHT patients, especially regarding secondary stroke prevention.


Subject(s)
Brain Abscess , Brain Ischemia , Ischemic Attack, Transient , Ischemic Stroke , Stroke , Telangiectasia, Hereditary Hemorrhagic , Humans , Telangiectasia, Hereditary Hemorrhagic/complications , Telangiectasia, Hereditary Hemorrhagic/epidemiology , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Retrospective Studies , Ischemic Attack, Transient/complications , Ischemic Attack, Transient/epidemiology , Brain Ischemia/complications , Brain Ischemia/epidemiology , Stroke/complications , Stroke/epidemiology , Ischemic Stroke/complications , Brain Abscess/complications , Brain Abscess/epidemiology
3.
Orphanet J Rare Dis ; 18(1): 354, 2023 Nov 14.
Article in English | MEDLINE | ID: mdl-37964294

ABSTRACT

BACKGROUND: Patients with the rare disease; Hereditary haemorrhagic telangiectasia (HHT) often bleed from telangiectatic lesions in mucosal surfaces. Studies suggest that impaired platelet function may also play a role in their bleeding tendency. The aim of the present study was to investigate whether HHT-patients with epistaxis have impaired platelet function. METHOD: We conducted a case-control study based on a sample size calculation and included 22 HHT-patients (inclusion criteria: epistaxis severity score ≥ 4, no intake of medicine affecting platelet function the last 5 days, HHT-type 1 or 2, age ≥ 18 years) and 20 controls. We assessed the platelet function with standard haemostasis parameters, flow cytometry (platelet function and micro aggregation), rotational thromboelastometry and Platelet Function Analyzer 200. RESULTS: We found no significant difference in mean platelet volume and immature platelet fraction and no difference in platelet activation as measured by exposure of CD62P, CD63P and PAC1 binding. Nor did we find a significant difference in platelet aggregation response in HHT-patients compared with the control group for all agonists (thrombin receptor activating peptide, adenosine diphosphate and collagen-related peptide). The PFA-200 analysis was without difference between the two groups and thromboelastometry showed no impairment of global haemostasis. CONCLUSION: Reduced platelet function is unlikely to contribute to the frequent and long bleeding episodes that HHT-patients suffer from. We propose that further studies should focus on whether patients with HHT have hypercoagulability.


Subject(s)
Telangiectasia, Hereditary Hemorrhagic , Humans , Adolescent , Epistaxis , Case-Control Studies , Disease Susceptibility
4.
Front Oncol ; 13: 1193730, 2023.
Article in English | MEDLINE | ID: mdl-37274287

ABSTRACT

Eosinophilic granulocytes are normally present in low numbers in the bloodstream. Patients with an increased number of eosinophilic granulocytes in the differential count (eosinophilia) are common and can pose a clinical challenge because conditions with eosinophilia occur in all medical specialties. The diagnostic approach must be guided by a thorough medical history, supported by specific tests to guide individualized treatment. Neoplastic (primary) eosinophilia is identified by one of several unique acquired genetic causes. In contrast, reactive (secondary) eosinophilia is associated with a cytokine stimulus in a specific disease, while idiopathic eosinophilia is a diagnosis by exclusion. Rational treatment is disease-directed in secondary cases and has paved the way for targeted treatment against the driver in primary eosinophilia, whereas idiopathic cases are treated as needed by principles in eosinophilia originating from clonal drivers. The vast majority of patients are diagnosed with secondary eosinophilia and are managed by the relevant specialty-e.g., rheumatology, allergy, dermatology, gastroenterology, pulmonary medicine, hematology, or infectious disease. The overlap in symptoms and the risk of irreversible organ involvement in eosinophilia, irrespective of the cause, warrants that patients without a diagnostic clarification or who do not respond to adequate treatment should be referred to a multidisciplinary function anchored in a hematology department for evaluation. This review presents the pathophysiology, manifestations, differential diagnosis, diagnostic workup, and management of (adult) patients with eosinophilia. The purpose is to place eosinophilia in a clinical context, and therefore justify and inspire the establishment of a multidisciplinary team of experts from diagnostic and clinical specialties at the regional level to support the second opinion. The target patient population requires highly specialized laboratory analysis and therapy and occasionally has severe eosinophil-induced organ dysfunction. An added value of a centralized, clinical function is to serve as a platform for education and research to further improve the management of patients with eosinophilia. Primary and idiopathic eosinophilia are key topics in the review, which also address current research and discusses outstanding issues in the field.

5.
Ugeskr Laeger ; 185(13)2023 03 27.
Article in Danish | MEDLINE | ID: mdl-36999292

ABSTRACT

Hereditary haemorrhagic telangiectasia is a genetic disease, causing abnormal formations of blood vessels in skin, mucus membranes, lungs, liver, and brain. In the liver, the disease results in shunting of blood, bypassing the capillary bed. Recent studies have shown that the prevalence of liver shunts are more frequent than previously suggested. The patients present with symptoms related to high-output cardiac failure causing dyspnoea and oedema. Liver shunts can be shown using CT-scans and ultrasonography. The only curable treatment is a liver transplant; however, it is the last treatment option, as argued in this review.


Subject(s)
Liver Transplantation , Telangiectasia, Hereditary Hemorrhagic , Humans , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Telangiectasia, Hereditary Hemorrhagic/diagnostic imaging , Liver , Tomography, X-Ray Computed , Skin
6.
Int J Dermatol ; 61(12): 1497-1505, 2022 Dec.
Article in English | MEDLINE | ID: mdl-35792874

ABSTRACT

BACKGROUND: The diagnostic criteria for hereditary hemorrhagic telangiectasia (HHT) include the presence of telangiectatic lesions in common sites (nose, fingers, oral cavity, and lips). Telangiectatic lesions are described as red spots in the skin, but this description is inadequate. Few studies have investigated the characteristics of telangiectatic lesions in HHT, and we aimed to describe the distribution and morphology of telangiectatic lesions in HHT patients. METHODS: We reviewed the telangiectatic lesions in 34 adult HHT patients seen at our HHT center. Photo documentation was used to describe the morphology and distribution of the lesions. RESULTS: The telangiectatic lesions in both HHT1 and HHT2 patients were predominantly round and either flat or slightly elevated. However, elongated flat lesions and larger round, elevated lesions were also observed. Patients with HHT1 had more lesions in the mucosa (tongue and oral cavity) compared with HHT2 patients. There was no difference between HHT1 and HHT2 patients in the total number of lesions in the skin and mucosa. CONCLUSIONS: The typical round, flat telangiectatic lesion is the most common lesion in HHT, but it is very often accompanied by elevated or elongated lesions. The total number of lesions did not vary between gender, but women had significantly more lesions in the mucosa (p = 0.027). The presentation of telangiectatic lesions may vary a little between HHT1 and HHT2 patients but not in such a way that allows the morphology and location of the lesions to predict the HHT subtype.


Subject(s)
Telangiectasia, Hereditary Hemorrhagic , Adult , Humans , Female , Telangiectasia, Hereditary Hemorrhagic/complications , Skin , Mucous Membrane
7.
Ugeskr Laeger ; 184(20)2022 05 16.
Article in Danish | MEDLINE | ID: mdl-35656602

ABSTRACT

In this case report, it was suspected that a four-year-old girl had an impacted toy in her nose. Initially, she was refused consultation due to a COVID-19 positive parent. Three days later she presented with nasal secretion and facial oedema. At the hospital, an examination revealed an impacted battery and consequent necrosis of nasal structures. The battery was removed in general anesthesia. Afterwards, the girl made fine recovery but developed a permanent septal perforation. Frequent alterations in COVID-19 guidelines challenge doctors to distinguish conditions crucial for evaluation. Batteries are uncommon for nasal impaction but may lead to severe morbidity if left undiscovered.


Subject(s)
Angioedema , COVID-19 , Anesthesia, General , Child, Preschool , Female , Hospitals , Humans , Nose
8.
Ugeskr Laeger ; 184(19)2022 05 09.
Article in Danish | MEDLINE | ID: mdl-35593371

ABSTRACT

Capillary malformations - arteriovenous malformation, hereditary hemorrhagic telangiectasia and Sturge-Weber syndrome - are rare diseases in which cutaneous capillary malformations (CM) may be associated with cerebral vascular malformations. The clinical presentation of each disease is described with focus on how to distinguish them in the clinic and differential diagnoses are listed. This review finds that upon thorough and careful examination of patients, cutaneous CM might be a diagnostic hallmark for underlying disease and therefore a significant clinical observation.


Subject(s)
Arteriovenous Malformations , Skin Diseases, Vascular , Telangiectasia, Hereditary Hemorrhagic , Vascular Malformations , Arteriovenous Malformations/complications , Arteriovenous Malformations/diagnostic imaging , Capillaries/abnormalities , Humans , Port-Wine Stain , Skin Diseases, Vascular/diagnosis , Telangiectasia, Hereditary Hemorrhagic/complications , Telangiectasia, Hereditary Hemorrhagic/diagnosis , Vascular Malformations/complications
9.
Dan Med J ; 69(4)2022 Mar 04.
Article in English | MEDLINE | ID: mdl-35319447

ABSTRACT

INTRODUCTION: Patients with eosinophilia (an increased number of eosinophilic granulocytes > 0.5 × 108/l in the blood) are encountered in all medical specialties and frequently need thorough workup to identify the eliciting causes and decide whether treatment is indicated. In Denmark, highly specialised centres for eosinophilic diseases or conditions have been established to provide a foundation for the management of complicated cases. Here, we present experiences from such a multidisciplinary centre. METHODS: This was a retrospective study of all patients seen in our tertiary centre for eosinophilia in the 2016-2019 period. RESULTS: Referrals mainly derived from specialised secondary care and to a lesser degree from primary care physicians. Patients were either asymptomatic or exhibited symptoms from up to three organ systems and presented a median eosinophil count of 1.7 × 108/l. Up to eight new clonality analyses or imaging studies per patient were performed after referral. One of these, T-cell receptor analysis, was performed frequently but provided limited information, whereas, e.g., flow cytometry proved more clinically applicable owing to its broader diagnostic range. In total, 51 patients were evaluated and classified as secondary (59%), myeloid neoplasm with PDGFRA rearrangement (2%), idiopathic hypereosinophilic syndrome (31%) and idiopathic hypereosinophilia (8%). CONCLUSION: The value of a multidisciplinary and versatile approach in a highly specialised centre has a positive impact on diagnostic processes as well as on the evaluation of treatment need. FUNDING: none. TRIAL REGISTRATION: not relevant.


Subject(s)
Hypereosinophilic Syndrome , Humans , Hypereosinophilic Syndrome/diagnosis , Hypereosinophilic Syndrome/genetics , Referral and Consultation , Retrospective Studies
10.
Orphanet J Rare Dis ; 17(1): 8, 2022 01 06.
Article in English | MEDLINE | ID: mdl-34991676

ABSTRACT

BACKGROUND: Hereditary Haemorrhagic Telangiectasia (HHT) is an autosomal dominant disorder characterized by several clinical symptoms including epistaxis, arteriovenous malformations (AVM), and telangiectasia. In 2018, European Reference Network for Rare Vascular Diseases (VASCERN) recommended five outcome measures for HHT-patients to guide health care providers, some with limited experience in treating HHT, and thereby maximizing the number of HHT-patients receiving good care. The outcome measures cover the following aspects: (1) 90% of the patients should receive a pulmonary AVM (PAVM) screening; (2) 90% of the patients should receive written advice on nosebleed; (3) 70% should be assessed for iron deficiency; (4) 100% of the patients should receive written advice on antibiotic (AB) prophylaxis prior to dental and surgical procedures, and (5) 100% of relevant patients should receive written advice on pregnancy. We have introduced the outcome measures as Benchmarks in our HHT-centre and wanted to evaluate the extend of implementation we have achieved. We constantly struggle to secure the best possible treatment of our HHT-patients. METHODS: The study was a non-interventional retrospective study. Data was collected manually from patient records and from the Danish HHT-database. RESULTS: A total of 180 HHT-patients were included, all diagnosed in the period from January 1st, 2016, to December 31st, 2020. All patients were screened for PAVM. We could confirm that 66% of patients who had epistaxis received thoroughly advice. Assessment for iron deficiency was performed in 80% of the adult patients. Thoroughly advice on AB prophylaxis was documented in 75%. Thoroughly advice on pregnancy was documented in 80% of female patients 15-45 years of age. There were no significant differences over time for any of the outcome measures. CONCLUSIONS: The Danish HHT-centre reached the target threshold for outcome measures 1 and 3. It could not be documented that the target thresholds for outcome measures 2, 4, and 5 were achieved. As information and education are a very important part of HHT care, focus on and documentation that all patients receive the relevant advice must be a priority in order to ensure best care.


Subject(s)
Arteriovenous Malformations , Telangiectasia, Hereditary Hemorrhagic , Adult , Arteriovenous Malformations/diagnosis , Denmark , Female , Humans , Outcome Assessment, Health Care , Pregnancy , Retrospective Studies , Telangiectasia, Hereditary Hemorrhagic/diagnosis
11.
BMJ Case Rep ; 14(5)2021 May 25.
Article in English | MEDLINE | ID: mdl-34035019

ABSTRACT

A 21-year-old, otherwise healthy, female patient was admitted with haemoptysis. Chest X-ray and CT found a consolidated right middle pulmonary lobe. Catheter angiography of ascending aorta visualised two hypertrophic and tortuous branches of the right internal mammary artery with a fistula to the right superior pulmonary vein. The inflow was embolised with coils. Catheter angiography of descending aorta found hypertrophic right bronchial arteries and right phrenic artery supplying a web-like network of vessels, which drained to the right superior pulmonary vein with discrete filling of an accessory right middle pulmonary vein. CT angiography with a catheter for contrast administration in the ascending aorta was performed for characterisation. After two additional episodes of haemoptysis, right middle lobe lobectomy was performed. Perioperatively pulmonary artery blood supply to the right middle pulmonary lobe was absent and an atretic accessory middle pulmonary vein was seen. The patient was discharged 7 days afterwards without sequelae.


Subject(s)
Fistula , Mammary Arteries , Pulmonary Veins , Adult , Anastomosis, Surgical , Female , Hemoptysis/etiology , Hemoptysis/therapy , Humans , Mammary Arteries/diagnostic imaging , Pulmonary Artery , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/surgery , Young Adult
12.
Ugeskr Laeger ; 183(20)2021 05 17.
Article in Danish | MEDLINE | ID: mdl-33998447

ABSTRACT

Patients with hereditary haemorrhagic telangiectasia (HHT) are known to suffer from cerebral arteriovenous malformations (CAVMs). In this review, we explore existing literature for bleeding risk, interventional therapy and neuroradiological features in HHT-related CAVMs. Studies estimate the annual intracerebral haemorrhage rate of CAVMs in HHT patients to be 0.667-1.014%. The clinician must balance bleeding risk and the non-negligible procedural risks of interventional therapy. We recommend, in agreement with European guidelines, that screening of asymptomatic HHT patients should only be carried out after careful information.


Subject(s)
Intracranial Arteriovenous Malformations , Telangiectasia, Hereditary Hemorrhagic , Cerebral Hemorrhage , Humans , Intracranial Arteriovenous Malformations/diagnosis , Intracranial Arteriovenous Malformations/diagnostic imaging , Mass Screening , Research , Telangiectasia, Hereditary Hemorrhagic/complications , Telangiectasia, Hereditary Hemorrhagic/diagnosis
13.
Immunol Res ; 68(4): 198-203, 2020 08.
Article in English | MEDLINE | ID: mdl-32681498

ABSTRACT

The purpose of this study was to investigate the risk of the patient having: (1) TB, (2) sarcoidosis, (3) atypical mycobacteria, or (4) malignant disease, if FNAC or histology from a cervical lymph node shows granulomatous inflammation (GI). And to elucidate clinical characteristics associated with these causes of GI, patients with a pathological diagnosis of GI in head and neck lymph nodes were identified though a search of the Danish national pathology database. Charts were reviewed to identify the final clinical diagnosis and specific clinical characteristics. For the most common clinical diagnoses, association to clinical characteristics was analyzed using logistic regression (Odense University Hospital January 2006 to December 2015). We included 121 patients. Clinical diagnoses fell into the following categories: sarcoidosis (26%), tuberculosis (TB) (22%), cat scratch disease (6%), atypical mycobacteriosis (7%), malignancy (2%), and other (4%). In 33% of cases, the diagnosis was unknown. In the pediatric group, atypical mycobacteriosis was the most frequent clinical diagnosis (50%). TB and sarcoidosis were dependent variables in regression analysis. Characteristics significantly related to TB were histology showing necrotizing GI, gland localization in level 3-6, and origin other than Danish and TB being the tentative diagnosis. Characteristics significantly related to sarcoidosis were histology showing non-necrotizing GI, gland localization in level 3-6, the patient being of Danish origin, and unknown duration of symptoms. TB and sarcoidosis were the most common clinical diagnoses, and they were associated with specific clinical characteristics. In a third of cases, a specific clinical diagnosis was never given.


Subject(s)
Granuloma/epidemiology , Lymph Nodes/pathology , Lymphadenitis/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Biopsy , Cat-Scratch Disease , Child , Child, Preschool , Denmark/epidemiology , Female , Granuloma/immunology , Granuloma/microbiology , Granuloma/pathology , Head , Head and Neck Neoplasms/epidemiology , Head and Neck Neoplasms/immunology , Head and Neck Neoplasms/pathology , Humans , Incidence , Infant , Lymph Nodes/immunology , Lymph Nodes/microbiology , Lymphadenitis/immunology , Lymphadenitis/microbiology , Lymphadenitis/pathology , Male , Middle Aged , Mycobacterium Infections, Nontuberculous/complications , Mycobacterium Infections, Nontuberculous/epidemiology , Mycobacterium Infections, Nontuberculous/immunology , Mycobacterium Infections, Nontuberculous/microbiology , Neck , Nontuberculous Mycobacteria/isolation & purification , Retrospective Studies , Risk Assessment/methods , Risk Assessment/statistics & numerical data , Sarcoidosis/complications , Sarcoidosis/epidemiology , Sarcoidosis/immunology , Tuberculosis/complications , Tuberculosis/epidemiology , Tuberculosis/immunology , Tuberculosis/microbiology , Young Adult
14.
Eur Arch Otorhinolaryngol ; 277(3): 791-800, 2020 Mar.
Article in English | MEDLINE | ID: mdl-31845036

ABSTRACT

BACKGROUND: Nasal closure, also known as the modified Young's procedure was introduced in Denmark in 2008, as a surgical solution to severe epistaxis in patients with hereditary haemorrhagic telangiectasia (HHT). The objective of this study was to report the overall satisfaction of the procedure from a patient's point of view as well as the occurrence of complications. METHODS: All the HHT patients who underwent nasal closure from 2008 to 2018 were included in the study. The patients were evaluated for postoperative complications and subjective outcome using Glasgow Benefit Inventory (GBI). RESULTS: Ten patients were included in the study and were observed for a mean of 64 months. None of the patients was completely free of complications, and reversal was requested in a single case. Haemoglobin levels rose with an average of 2.8 g/dl. The average GBI score after surgery was 38.05. Nine of ten patients would recommend nasal closure to fellow HHT patients. CONCLUSION: Nasal closure is highly recommended among patients, but due to the rate of postoperative complications, the procedure should be reserved for a carefully selected group of HHT patients.


Subject(s)
Epistaxis/surgery , Nasal Surgical Procedures/methods , Nose/surgery , Telangiectasia, Hereditary Hemorrhagic/surgery , Adult , Aged , Cohort Studies , Denmark , Epistaxis/etiology , Female , Humans , Male , Middle Aged , Nasal Mucosa/surgery , Nasal Surgical Procedures/adverse effects , Patient Reported Outcome Measures , Patient Satisfaction , Surgical Flaps/surgery , Telangiectasia, Hereditary Hemorrhagic/complications , Treatment Outcome
15.
Ugeskr Laeger ; 181(18)2019 04 29.
Article in Danish | MEDLINE | ID: mdl-31036144

ABSTRACT

In this review, the importance of correct diagnosis of glomuvenous malformations (GVM) is emphasised, and different treatment modalities are discussed. GVM are simple venous malformations located in the skin and subcutis, and GVM has a characteristic blue to reddish cobblestone-like appearance with not fully compressible elements, which can be associated with pain on palpation. Clinically, GVM differ from blue rubber bleb naevus syndrome and common/familial cutaneo-mucosal venous malforma-tions in appearance, compressibility and in having a normal D-dimer concentration.


Subject(s)
Glomus Tumor , Skin Neoplasms , Glomus Tumor/diagnosis , Glomus Tumor/surgery , Humans , Paraganglioma, Extra-Adrenal , Skin Neoplasms/diagnosis , Skin Neoplasms/surgery
16.
Dan Med J ; 66(3)2019 Mar.
Article in English | MEDLINE | ID: mdl-30864547

ABSTRACT

INTRODUCTION: The National Clinical Guideline for Ton-sillectomy (Danish Health Authority, 2016) suggests inpatient admission after elective tonsil surgery in patients aged < 4 years at the time of surgery. We aimed to evaluate the safety of tonsil surgery as day surgery in children ≤ 4 years of age. METHODS: The charts of 414 patients aged 2-15 years who underwent elective tonsillectomy, adenotonsillectomy or tonsillotomy at Svendborg Hospital, Denmark, from February 2010 to April 2015 were reviewed in a retrospective cohort. We investigated post-operative complications, defined as post-operative haemorrhage and unplanned contacts or revisits to the hospital. RESULTS: A total of 389 patients were divided into two groups by age (≤ 4/> 4 years). In all, 108 patients contacted or revisited the hospital after discharge. Patients aged ≤ 4 years accounted for the majority of contacts unrelated to bleeding and associated with complaints of pain and pain medication. Haemorrhage occurred in 22 (5.7%) patients, 19 of whom were > 4 years. The rates of haemorrhage, readmission and secondary surgery were significantly higher in patients aged > 4 years than in the remaining patients. CONCLUSIONS: Patients ≤ 4 years experienced significantly less haemorrhage but had more unplanned contacts than patients > 4 years. For reassurance of caretakers, easy access to telephone contact with hospital staff in the post-operative period is important. FUNDING: none. TRIAL REGISTRATION: not relevant.


Subject(s)
Day Care, Medical , Palatine Tonsil/surgery , Postoperative Complications/epidemiology , Tonsillectomy/adverse effects , Adolescent , Age Factors , Child , Child, Preschool , Denmark , Female , Humans , Logistic Models , Male , Multivariate Analysis , Pain/epidemiology , Patient Readmission/statistics & numerical data , Postoperative Hemorrhage/epidemiology , Retrospective Studies
17.
Cardiovasc Intervent Radiol ; 42(8): 1102-1109, 2019 Aug.
Article in English | MEDLINE | ID: mdl-30887104

ABSTRACT

PURPOSE: The present study is a register-based observational study of an unselected consecutive patient cohort with pulmonary arteriovenous malformations (PAVMs) from a single national hereditary hemorrhagic telangiectasia and PAVM embolization center. The aim was to investigate the frequency of re-embolizations and the clinical outcome after embolization with the use of different embolization materials further, to define which PAVM morphology and size of feeding arteries that most often were re-embolized, and to estimate the clinical outcome of the patients including those that were re-embolized. METHODS: The population was included from 1996 until 2016 and was made up of a total of 136 patients with 322 PAVMs. Median follow-up was 38.3 (0.3-241 months). RESULTS: The re-embolization rate was 9.3%. None of the PAVMs treated with detachable silicone balloons were re-embolized, while 4.5% treated with vascular plugs and 11.7% treated with coils were re-embolized (p=0.07). In total, 16/74 complex PAVMs were re-embolized compared with 14/248 simple PAVMs. In big-sized feeding arteries ≥ 6mm, 16/112 were re-embolized compared with 14/210 with smaller-sized feeding arteries. Out of the 30 re-embolized PAVMs, 23 resulted in a successful clinical outcome. CONCLUSIONS: Our results suggest that standard coils probably should not be the first choice for embolization of PAVMs, and vascular plug alone or in combination with coils might be a better primary option for embolization in these patients. LEVEL OF EVIDENCE: Level 3A, non-randomized case controlled cohort/follow-up study.


Subject(s)
Arteriovenous Fistula/therapy , Arteriovenous Malformations/therapy , Embolization, Therapeutic/methods , Pulmonary Artery/abnormalities , Pulmonary Veins/abnormalities , Adult , Cohort Studies , Denmark , Female , Follow-Up Studies , Humans , Male , Pulmonary Artery/diagnostic imaging , Pulmonary Veins/diagnostic imaging , Radiography, Interventional/methods , Retrospective Studies , Treatment Outcome
18.
Orphanet J Rare Dis ; 13(1): 223, 2018 12 14.
Article in English | MEDLINE | ID: mdl-30547819

ABSTRACT

BACKGROUND: Hereditary Haemorrhagic Telangiectasia (HHT) is an autosomal dominant genetic disorder with a wide variety of clinical manifestations due to the presence of multiple arteriovenous malformations in various tissues and organs. OBJECTIVE: To study the need for hospital admittance in a group of HHT patients and matched controls during a 20 years follow-up period commencing in 1995. METHODS: All HHT patients in the County of Funen, Denmark, were included. For each patient, three age and sex matched controls were identified at the time of enrolment. Data on all hospitalisations were extracted from the national health registers and compared with clinical records. The hospitalisations were grouped as HHT relevant or not HHT relevant based on the discharge diagnosis (International Classification of Diseases, ICD10) and with particular focus on infections, bleedings and thromboembolic events. Patients with HHT were compared with controls concerning the first time incidence of each discharge diagnosis. RESULTS: We included 73 HHT patients and 219 controls of which one control was lost to follow-up. HHT-patients had significantly more hospitalisations per person caused by infections in joints and bones, but not caused by infections in general. Bleeding episodes were, as expected, more frequent among the HHT-patients. The study revealed a similar incidence of abscesses and thromboembolisms, including in the central nervous system, among the HHT patients and controls. CONCLUSIONS: Based on this study Danish HHT patients had an increased comorbidity of infections in joints and bones and of bleeding episodes. However, the incidence of thromboembolisms, cerebral abscesses and other conditions commonly considered related to HHT was comparable between the patients and the controls. The patients included in this study were closely monitored at a highly specialised HHT Centre where they received relevant diagnostic evaluation, treatment and counselling. Since this is assumed to benefit the overall health of the patients, it may explain why these patients were less prone to comorbidity than other studies have suggested.


Subject(s)
Telangiectasia, Hereditary Hemorrhagic/epidemiology , Adult , Aged , Denmark/epidemiology , Follow-Up Studies , Hospitalization/statistics & numerical data , Humans , Incidence , Middle Aged
19.
BMJ Case Rep ; 20182018 Jan 17.
Article in English | MEDLINE | ID: mdl-29348274

ABSTRACT

We present two 11-year-old girls with chronic recurrent multifocal osteomyelitis, treated with adalimumab. Both developed severe intracranial complications to sinusitis. Patient 1 had been treated with adalimumab for 15 months when she developed acute sinusitis complicated by an orbital abscess, forehead swelling, a subdural empyema and osteomyelitis of the frontal bone. She was treated with a rhinosurgical and neurosurgical approach with intravenous antibiotics.Patient 2 had been in adalimumab treatment for 10 weeks. Adalimumab was discontinued 8 weeks prior to developing subdural empyema and subcortical abscesses in combination with sinusitis. She was treated with endoscopic sinus surgery and intravenous antibiotics. Both patients had developed psoriasis and episodes of infection during treatment. They were non-septic and had low fever on presentation. None of the patients suffered any long-term neurological sequelae. The immunosuppressive treatment with adalimumab is considered to be the cause of the sinogenic intracranial complications in our cases.


Subject(s)
Adalimumab/adverse effects , Anti-Inflammatory Agents/adverse effects , Brain Diseases/chemically induced , Osteomyelitis/drug therapy , Sinusitis/chemically induced , Abscess/chemically induced , Acute Disease , Brain Abscess/chemically induced , Child , Empyema, Subdural/chemically induced , Female , Humans , Orbital Diseases/chemically induced
20.
Otolaryngol Head Neck Surg ; 153(4): 636-43, 2015 Oct.
Article in English | MEDLINE | ID: mdl-25676152

ABSTRACT

OBJECTIVE: The pathological picture may differ considerably between diagnostic subgroups of children with otitis media receiving ventilating tubes. The aims of this study are to investigate differences in quality of life among diagnostic subgroups of children treated with ventilating tubes and to investigate possible predictors for clinical success. STUDY DESIGN: Longitudinal observational study. SETTING: Secondary care units. METHODS: Four hundred ninety-one families were enrolled in the study. The Otitis Media-6 questionnaire was applied in the assessment of child quality of life. Caregivers completed questionnaires at 7 time points from before treatment to 18-month follow-up. Logistic regression analysis was used to investigate possible predictors for clinical success. RESULTS: Response rates ranged from 96% to 81%; diagnostic distribution: 15% recurrent acute otitis media (rAOM), 47% otitis media with effusion (OME), and 38% mixed diagnosis of rAOM and OME (rAOM/OME). There were no significant differences between children diagnosed with rAOM and children diagnosed with rAOM/OME. However, these children had a significantly poorer quality of life at baseline compared with children diagnosed with only OME. Factors associated with clinical success included a diagnosis of rAOM, number of interrupted nights, physician visits, and canceled social activities due to OM. CONCLUSIONS: Results highlight the importance of distinguishing between diagnostic subgroups of children having ventilating tube treatment. A diagnosis of rAOM was found to predict baseline quality of life. Children with rAOM with or without OME were found to suffer significantly more than children with only OME before treatment. Factors associated with disease severity were found to predict clinical success.


Subject(s)
Middle Ear Ventilation/instrumentation , Otitis Media/surgery , Quality of Life , Child, Preschool , Female , Humans , Longitudinal Studies , Male , Otitis Media/classification , Otitis Media/diagnosis , Surveys and Questionnaires
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