422 Atrioventricular blocks in children: clinical aspects and special features in a cohort of Italian patients

Abstract Aims The natural history of atrioventricular blocks (AVBs) in children is not completely clarified, with particular regard for isolated, acquired, and non-immune disorders. Moreover, there is still concern on when—and not on if—a pacemaker (PM) implantation is indicated. In this retrospective study, we investigated diagnostic and therapeutic approach to AVBs in children and we described complications occurred in PM recipients. Methods and results We analysed 73 patients with a diagnosis of AVB who were submitted to a regular follow-up between 2015 and 2020 at the Paediatric Cardiology and Cardiac Surgery Unit of an Italian referral centre. Forty-four patients (60%) were diagnosed with a complete AVB and after a mean follow-up of 8.2 ± 6.8 years, 35 patients (79.5%) had received a permanent PM. AVB was asymptomatic in 52 children (71.2%) and a congenital heart disease was present in 13 cases (17.8%). Anti-SSA/B autoantibodies were detected in eight patients (11%) with complete block and their prognostic role was substantially redefined. The mean interval between diagnosis and implantation and the complication rate in PM recipients were similar to data available on literature, regardless of the age at diagnosis. Conclusions From a diagnostic point of view, we identified several differences between partial and complete AVBs. As regards the timing of pacing therapy, the moment of implantation should be accurately chosen but should not depend only on the age at diagnosis or the presence of maternal autoantibodies, since the incidence of device-related complications was independent from those features.

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POS0119 RENAL INVOLVEMENT AND NEED OF RENAL REPLACEMENT THERAPY IN ANCA ASSOCIATED VASCULITIS IN A SPANISH SINGLE-CENTER STUDY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.345 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 270.2-271

Author(s):

J. Álvarez Troncoso ◽

J. C. Santacruz Mancheno ◽

A. Díez Vidal ◽

S. Afonso Ramos ◽

A. Noblejas Mozo ◽

...

Keyword(s):

Renal Replacement Therapy ◽

Replacement Therapy ◽

Granulomatosis With Polyangiitis ◽

Renal Involvement ◽

Age At Diagnosis ◽

Systemic Involvement ◽

Risk Of Death ◽

Anca Associated Vasculitis ◽

The Mean

Background:Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EPGA). Renal involvement is frequent in AAV and is an important factor for morbidity and mortality.Objectives:The main objective of this study was to analyze the demographic, clinical, histological and therapeutic characteristics of renal involvement in patients with AAV and the risk of renal replacement therapy (RRT) or death.Methods:Retrospective observational study of 56 patients with AAV fulfilling classificatory criteria and renal involvement diagnosed between 1995 and 2020 from a Spanish tertiary centre. We studied the histological involvement (according to the 2010 classification in focal, crescentic, mixed or sclerotic), immunofluorescence (IF) and the treatment received with the risk of RRT or death.Results:We included 56 patients diagnosed with AAV and renal involvement. The mean age was 61.08±4.05 years; 58.9% were women. The mean follow-up time of these patients was 16.14± 8.80 years. Only 57.1% of patients presented systemic involvement.Most frequent non-renal AAV manifestations were lung involvement (39.3%), central nervous system (30.4%), otorhinolaryngology (ORL) (14.3%), skin (8.9%) and cardiac involvement (8.9%). Main immunological findings were ANCA-MPO+ (69.6%), ANCA-PR3+ (23.2%), ANCA-negative (5.4%). Low C3 was found in 19.6% patients. Histologic classification (HC) and need of RRT is described in table 1. Main HC in renal AAV was crescentic, mixed, focal and sclerotic respectively. Eight patients had not biopsy performed. IF was positive for C3 deposits in 20 patients (35.7%). Half of the patients presented <50% normal glomeruli.The treatment of renal involvement in AAV in our cohort was as follows: 83.9% (47) corticosteroids (CS) and cyclophosphamide (of which 40 received intravenous and 7 oral cyclophosphamide; and 12 patients associated plasma exchange (PE) with this treatment), 5.36% CS alone, 2 patients received CS and mycophenolate; 1 CS and rituximab, 1 CS and PE, and 2 patients received no treatment. A total of 13 patients received PE and 18 RRT. The mean time to RRT was 65.44±32.72 months. Relapses were not uncommon, 33.93% of the patients presented ≥1 relapse and 10.71% presented ≥2.Infections were very frequent since they were present in 91.07% of the patients. Other frequent non-immunological complications observed in the follow-up of these patients were thrombosis in 31.14%, cardiovascular events in 28.57% and cancer in 19.64%.Patients with ANCA-PR3+ were younger at diagnosis (p<0.001), were more likely to present cardiac (p=0.045) and ORL involvement (p<0.001). However, neither ANCA-PR3+ nor ANCA-MPO+ were specifically associated with the need of RRT or higher risk of death in our cohort. Use of CS alone for the treatment of renal AAV was associated with higher mortality (p=0.006) but CS and cyclophosphamide with lower mortality (p=0.044). ANCA-negative patients were more likely to receive no treatment. Having <50% normal glomeruli and C3 deposits on IF were associated with an increased need for RRT. Presenting focal disease on HC was protective for the need of RRT. Older age at diagnosis, systemic involvement of AAV and need of RRT was associated with higher mortality.Conclusion:AAVs are complex vasculitides with frequent renal involvement. Increased C3 deposition, non-focal histological forms, and <50% normal glomeruli were related to the need for RRT. In turn, the need for RRT, a later age at diagnosis, and systemic involvement were associated with higher mortality. Holistic and multidisciplinary early management of AAVs in experience centers can help improve renal prognosis and decrease mortality.References:[1]Binda et al. ANCA-associated vasculitis with renal involvement. J Nephrol. 2018 Apr;31(2):197-208.[2]Kronbichler et al. Clinical associations of renal involvement in ANCA-associated vasculitis. Autoimmun Rev. 2020 Apr;19(4):102495.Disclosure of Interests:None declared

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Sialendoscopy With Intraductal Steroid Irrigation in Patients With Sialadenitis Without Sialoliths

Ear Nose & Throat Journal ◽

10.1177/0145561319841207 ◽

2019 ◽

Vol 98 (5) ◽

pp. 291-294 ◽

Cited By ~ 4

Author(s):

Saudamini J. Lele ◽

Mickie Hamiter ◽

Torrey Louise Fourrier ◽

Cherie-Ann Nathan

Keyword(s):

Treatment Option ◽

Case Series ◽

Complete Response ◽

Definitive Treatment ◽

Invasive Technique ◽

Effective Treatment Option ◽

Patient Reported ◽

History Of ◽

The Mean

Sialendoscopy has emerged as a safe, effective and minimally invasive technique for management of obstructive and inflammatory salivary gland disease. The aim of our study was to analyze outcomes of sialendoscopy and steroid irrigation in patients with sialadenitis without sialoliths. We performed a retrospective analysis of patients who underwent interventional sialendoscopy with steroid irrigation from 2013 to 2016, for the treatment of sialadenitis without sialolithiasis. Twenty-two patients underwent interventional sialendoscopy with ductal dilation and steroid irrigation for the treatment of sialadenitis without any evidence of sialolithiasis. Conservative measures had failed in all. Eleven patients had symptoms arising from the parotid gland, 4 patients had symptoms arising from the submandibular gland, while 6 patients had symptoms in both parotid and submandibular glands. One patient complained of only xerostomia without glandular symptoms. The mean age of the study group which included 1 male and 21 females was 44.6 years (range: 3-86 years). Four patients had autoimmune disease, while 7 patients had a history of radioactive iodine therapy. No identifiable cause for sialadenitis was found in the remaining 11 patients. The mean follow-up period was 378.9 days (range: 16-1143 days). All patients underwent sialendoscopy with ductal dilation and steroid irrigation. Twelve patients showed a complete response and 9 patients had a partial response, while 1 patient reported no response. Only 3 patients required repeat sialendoscopy. The combination of sialendoscopy with ductal dilation and steroid irrigation is a safe and effective treatment option for patients with sialadenitis without sialoliths refractory to conservative measures. Prospective studies with a larger case series are needed to establish its role as a definitive treatment option.

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Congenital Temporomandibular Joint Ankylosis: Clinical Characterization and Natural History of Four Unrelated Affected Individuals

The Cleft Palate-Craniofacial Journal ◽

10.1597/04-027.1 ◽

2005 ◽

Vol 42 (6) ◽

pp. 694-698 ◽

Cited By ~ 4

Author(s):

Vera Lúcia Gil-da-Silva-Lopes ◽

Daniela Varela Luquetti

Keyword(s):

Natural History ◽

Temporomandibular Joint ◽

Clinical Aspects ◽

Temporomandibular Joint Ankylosis ◽

History Of ◽

Joint Ankylosis

The objective of this study was to describe four unrelated patients affected by congenital temporomandibular joint ankylosis. Clinical delineation, natural history, and possible etiologies are discussed. Clinical aspects that are important for follow-up are outlined.

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Management of ureteropelvic junction obstruction in horseshoe kidneys by an assortment of laparoscopic options

Canadian Urological Association Journal ◽

10.5489/cuaj.3111 ◽

2015 ◽

Vol 9 (11-12) ◽

pp. 775 ◽

Cited By ~ 5

Author(s):

Pejman Shadpour ◽

H. Habib Akhyari ◽

Robab Maghsoudi ◽

Masoud Etemadian

Keyword(s):

Ureteropelvic Junction Obstruction ◽

Left Kidney ◽

Operation Time ◽

Ureteropelvic Junction ◽

Laparoscopic Pyeloplasty ◽

Referral Centre ◽

Laparoscopic Management ◽

The Mean ◽

Crossing Vessels

Introduction: We report our experience with laparoscopic management of ureteropelvic junction obstruction in horseshoe kidneys.Methods: Between February 2004 and March 2014, 15 patients with horseshoe kidneys and symptomatic ureteropelvic junction obstruction underwent laparoscopic management at our national referral centre. Depending on the anatomy and presence of obtrusive vessels or isthmus, we performed either dismembered, Scardino or Foley YV pyeloplasty, or Hellstrom vessel transposition. Patients were initially evaluated by ultrasonography, then diuretic scintiscan at 4 to 6 months, and followed by yearly clinical and sonographic exams.Results: This study included 11 male and 4 female patients between the ages of 4 to 51 year (average 17.7). The left kidney was involved in 12 patients (80%). Operation time was 129 minutes (range: 90–186), and patients were discharged within 2.8 days (range: 1–6). Although 8 (53.3%) patients had crossing vessels, of which 6 required transposing, the Hellstrom technique was solely used in 3 cases, of which notably 1 case failed to resolve and required laparoscopic Hynes within the next year. Eight cases underwent dismembered pyeloplasty, 2 Foley YV, 1 Scardino flap and 1 required isthmectomy and vessel suspension. At the mean follow-up of 60 (range: 18–120) months, the overall success rate was 93.3%.Conclusions: To our knowledge, this represents the largest report on laparoscopic pyeloplasty for horseshoe kidneys, providing the longest follow-up. Our findings confirm prior reports supporting laparoscopy and furthermore show that despite the prevalence of crossing vessels, transposition alone is seldom sufficient.

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Frequency, Predictors, and Outcome of Seizures in Patients With Myelomeningocele: Single-Center Retrospective Cohort Study

Journal of Child Neurology ◽

10.1177/08830738211053132 ◽

2021 ◽

pp. 088307382110531

Author(s):

Cemal Karakas ◽

Emin Fidan ◽

Kapil Arya ◽

Troy Webber ◽

Joan B. Cracco

Keyword(s):

Good Prognosis ◽

Cortical Atrophy ◽

Seizure Freedom ◽

Single Center ◽

Seizure Onset ◽

Shunt Procedure ◽

History Of ◽

The Mean

To determine the frequency, predictors, and outcomes of seizures in patients with myelomeningocele, we retrospectively analyzed the data from patients with myelomeningocele followed longitudinally at a single center from 1975 to 2013. We identified a total of 122 patients (61% female). The mean follow-up duration was 11.1 years (minimum-maximum = 0-34.5 years, SD = 8.8, median = 9.1 years). A total of 108 (88.5%) patients had hydrocephalus, and 98 (90.7%) of those patients required a ventriculoperitoneal shunt procedure. Twenty-four (19.7%) patients manifested with seizures, 23 of whom had hydrocephalus. The average age of seizure onset was 4.8 years (median 2 years of age). Falx dysgenesis ( P = .004), lumbar myelomeningocele ( P = .007), and cortical atrophy ( P = .028) were significantly associated with epileptic seizure development. The average seizure-free period at the last follow-up in patients with a history of myelomeningocele and seizures was 8.1 years. We conclude that myelomeningocele patients with seizures have an overall good prognosis with considerable long-term seizure freedom.

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Palliative Treatment of Hepatocellular Carcinoma with Transcatheter Arterial Embolization

Acta Radiologica ◽

10.1177/028418519303400106 ◽

1993 ◽

Vol 34 (1) ◽

pp. 26-29 ◽

Cited By ~ 4

Author(s):

S. Savastano ◽

G. P. Feltrin ◽

D. Neri ◽

P. da Pian ◽

M. Chiesura-Corona ◽

...

Keyword(s):

Hepatocellular Carcinoma ◽

Transcatheter Arterial Embolization ◽

Palliative Treatment ◽

Arterial Embolization ◽

Percutaneous Ethanol Injection ◽

Ethanol Injection ◽

History Of ◽

The Mean ◽

Hcc Recurrence

Thirty-three consecutive patients with previously untreated hepatocellular carcinoma (HCC) and 6 patients with recurrent HCC were treated with transcatheter arterial embolization (TAE). The patients were not eligible for surgical resection or percutaneous ethanol injection. TAE was performed with Lipiodol Ultra-Fluid, epidoxorubicin and Gelfoam, with a mean of 1.7 treatments per patient. CT was performed 15 days after TAE. The mean cumulative survival was 14.2 months in patients with previously untreated HCC. The survival of patients stages Okuda I and II did not differ significantly (p > 0.05); tumor size did not affect survival (p > 0.05). Two patients with recurrent HCC died 7.0 and 9.3 months after the diagnosis of tumor recurrence; the remaining 4 patients are still alive with a maximum follow-up of 22.5 months from the diagnosis of HCC recurrence. Ten complications occurred in 8 patients, and were controlled by medical therapy. Eleven patients died during the study; no death was related to TAE. The series was not randomized, but comparison with the natural history of HCC suggests that TAE is effective as palliative treatment of advanced or recurrent HCC.

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Complications in Mandibular Midline Distraction

Craniomaxillofacial Trauma & Reconstruction ◽

10.1055/s-0037-1600902 ◽

2017 ◽

Vol 10 (3) ◽

pp. 204-207 ◽

Cited By ~ 2

Author(s):

Jan Pieter de Gijt ◽

Atilla Gül ◽

Eppo B. Wolvius ◽

Karel G.H. van der Wal ◽

Maarten J. Koudstaal

Keyword(s):

Retrospective Cohort ◽

Mental Nerve ◽

Safe Method ◽

Congenital Deformities ◽

Area Of Interest ◽

Sensory Disturbances ◽

History Of ◽

The Mean ◽

New Surgical Technique

Mandibular midline distraction (MMD) is a relatively new surgical technique for correction of transverse discrepancies of the mandible. This study assesses the amount and burden of complications in MMD. A retrospective cohort study was performed on patients who underwent MMD between 2002 and 2014. Patients with congenital deformities or a history of radiation therapy in the area of interest were excluded. Patient records were obtained and individually assessed for any complications. Complications were graded using the Clavien-Dindo classification system (CDS). Seventy-three patients were included of which 33 were males and 40 were females. The mean follow-up was 2.1 years. Twenty-nine patients had minor complications, grades I and II. Two patients had a grade IIIa and three patients had a grade IIIb complication. Common complications were pressure ulcers, dehiscence, and (transient) sensory disturbances of the mental nerve. This study shows that although MMD is a relatively safe method, complications can occur. Mostly the complications are mild, transient, and manageable without the need for any reoperation.

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Incidence and prognosis of patients with spinal metastasis as the initial manifestation of malignancy

The Bone & Joint Journal ◽

10.1302/0301-620x.101b11.bjj-2018-1600.r2 ◽

2019 ◽

Vol 101-B (11) ◽

pp. 1379-1384 ◽

Cited By ~ 3

Author(s):

Jin-Sung Park ◽

Se-Jun Park ◽

Chong-Suh Lee

Keyword(s):

Surgical Treatment ◽

Spinal Metastasis ◽

Spinal Disease ◽

Primary Malignancy ◽

Initial Manifestation ◽

Kaplan Meier ◽

History Of ◽

The Mean ◽

The Difference

Aims This study aimed to evaluate the incidence and prognosis of patients with spinal metastasis as the initial manifestation of malignancy (SM-IMM). Patients and Methods We retrospectively reviewed the electronic medical records of 338 patients who underwent surgical treatment for metastatic spinal disease. The enrolled patients were divided into two groups. The SM-IMM group included patients with no history of malignancy whose site of primary malignancy was diagnosed after the identification of spinal metastasis. The other group included patients with a history of treatment for primary malignancy who then developed spinal metastasis (SM-DTM). The incidence of SM-IMM by site of primary malignancy was calculated. The difference between prognoses after surgical treatment for SM-IMM and SM-DTM was established. Results The median follow-up period was 11.5 months (interquartile range (IQR) 3.2 to 13.4) after surgical treatment. During the follow-up period, 264 patients died; 74 patients survived. The SM-IMM group consisted of 94 patients (27.8%). The site of primary malignancy in the SM-IMM group was lung in 35/103 patients (34.0%), liver in 8/45 patients (17.8%), kidney in 10/33 patients (30.3%), colorectum in 3/29 patients (10.3%), breast in 3/22 patients (13.6%), prostate in 3/10 patients (30%), thyroid in 4/8 patients (50%), and ‘other’ in 28/88 patients (31.8%). On Kaplan–Meier survival analysis, the SM-IMM group showed a significantly longer survival than the SM-DTM group (p = 0.013). The mean survival time was 23.0 months (95% confidence interval (CI) 15.5 to 30.5) in the SM-IMM group and 15.5 months (95% CI 11.8 to 19.2) in the SM-DTM group. Conclusion Of the 338 enrolled patients who underwent surgical treatment for spinal metastasis, 94 patients (27.8%) underwent surgical treatment for SM-IMM. The SM-IMM group had an acceptable prognosis with surgical treatment. Cite this article: Bone Joint J 2019;101-B:1379–1384.

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Moment formulas for the Markov renewal branching process

Advances in Applied Probability ◽

10.2307/1425930 ◽

1976 ◽

Vol 8 (4) ◽

pp. 690-711 ◽

Cited By ~ 54

Author(s):

Marcel F. Neuts

Keyword(s):

Steady State ◽

Numerical Computation ◽

Busy Period ◽

Branching Process ◽

Queueing Models ◽

Absorption Time ◽

The Mean ◽

The Moment

There are many queueing models in which there appears a semi-Markov matrix G(·), whose entries are absorption-time distributions in a Markov renewal branching process. The role of G(·) is similar to that of the busy period in the simple M/G/1 model. The computation of various quantities associated with G(·) is however much more complicated. The moment matrices, and particularly the mean matrix of G(·), are essential in the construction of general and mathematically well-justified algorithms for the steady-state distributions of such queues.This paper discusses the moment matrices of G(·) and algorithms for their numerical computation. Its contents are basic to the algorithmic solutions to several queueing models, which are to be presented in follow-up papers.

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Feasibility of Powered Intracapsular Tonsillectomy in Pediatric Patients with Tonsil Problem, Including Recurrent Tonsillitis: A Single Surgeon’s Experience

ORL ◽

10.1159/000509479 ◽

2020 ◽

Vol 82 (6) ◽

pp. 335-342

Author(s):

Heejin Kim ◽

Sung Won Li ◽

Sung Min Park ◽

Sung Kyun Kim ◽

Seok Jin Hong ◽

...

Keyword(s):

Obstructive Sleep Apnea ◽

Sleep Apnea ◽

Postoperative Pain ◽

Pediatric Patients ◽

Recurrent Tonsillitis ◽

Obstructive Sleep ◽

History Of ◽

The Mean ◽

Intracapsular Tonsillectomy

Introduction: Powered intracapsular tonsillectomy (PIT) is a technique that protects the tonsillar capsule by using a microdebrider, resulting in faster wound-healing and reduced suffering. Many studies have found PIT to be effective, particularly in pediatric patients with obstructive sleep apnea (OSA). However, previous studies have not included patients with a history of recurrent tonsillitis. Objective: The aim of this study was to determine the efficacy of PIT in pediatric patients even with a history of recurrent tonsillitis, and therefore, we want to expand the indication for PIT and reveal its safety. Methods: A total of 886 pediatric patients underwent PIT between February 2013 and March 2016. All patients rated their postoperative pain using a visual analog scale (VAS) and completed the Korean obstructive sleep apnea (KOSA)-18 questionnaire for assessment of their quality of life (QOL). There were 539 males and 347 females. Their mean age was 6.2 years (range 2–14 years). The majority (77.7%) underwent the operation for OSA, and the rest (22.3%) had a history of recurrent tonsillitis. To compare the efficacy of PIT with traditional tonsillectomy, we selected 191 patients who underwent extracapsular tonsillectomy (ECT), a conventional technique, during the same time period. The median follow-up period was 16.7 months. During the follow-up period, instances of delayed bleeding and recurrent pharyngitis were monitored. Results: In comparison to the patients who underwent ECT, the PIT group showed significantly fewer cases of postoperative bleeding (p = 0.027). Thirteen patients in the PIT group (1.5%) visited the hospital during the follow-up period for pharyngitis, while 8 in the ECT group (4.2%) visited for pharyngitis. The mean postoperative pain score, as assessed by a VAS, was 4.6 ± 3.2, and pain improved within an average of 2.9 days after surgery in the PIT group. The mean KOSA-18 score for the QOL of the patients was 65.9 preoperatively and 35.6 postoperatively in the PIT group. Conclusions: Pediatric tonsillectomy using PIT is valid for reducing postoperative pain and improving the QOL of OSA patients. PIT is also effective and safe for patients with a history of recurrent tonsillitis.

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