High degree atrioventricular block in a marathoner with 5-year follow-up

Cerebellotrigeminal dermal (CTD) dysplasia is a rare neurocutaneous disorder characterized by a triad of symptoms: bilateral parieto-occipital alopecia, facial anesthesia in the trigeminal area, and rhombencephalosynapsis (RES), confirmed by cranial magnetic resonance imaging. CTD dysplasia is also known as Gómez-López-Hernández syndrome. So far, only 35 cases have been described with varying symptomatology. The etiology remains unknown. Either spontaneous dominant mutations or de novo chromosomal rearrangements have been proposed as possible explanations. In addition to its clinical triad of RES, parietal alopecia, and trigeminal anesthesia, CTD dysplasia is associated with a wide range of phenotypic and neurodevelopmental abnormalities.Treatment is symptomatic and includes physical rehabilitation, special education, dental care, and ocular protection against self-induced corneal trauma that causes ulcers and, later, corneal opacification. The prognosis is correlated to the mental development, motor handicap, corneal–facial anesthesia, and visual problems. Follow-up on a large number of patients with CTD dysplasia has never been reported and experience is limited to few cases to date. High degree of suspicion in a child presenting with characteristic alopecia and RES has a great importance in diagnosis of this syndrome.

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Long-term outcomes in young patients with atrioventricular block of unknown aetiology

European Heart Journal ◽

10.1093/eurheartj/ehab060 ◽

2021 ◽

Author(s):

Johnni Resdal Dideriksen ◽

Morten K Christiansen ◽

Jens B Johansen ◽

Jens C Nielsen ◽

Henning Bundgaard ◽

...

Keyword(s):

Heart Failure ◽

Atrioventricular Block ◽

Ventricular Tachyarrhythmia ◽

Pacemaker Implantation ◽

Young Patients ◽

Composite Endpoint ◽

Unknown Aetiology ◽

Successful Resuscitation

Abstract Aims Atrioventricular block (AVB) of unknown aetiology is rare in the young, and outcome in these patients is unknown. We aimed to assess long-term morbidity and mortality in young patients with AVB of unknown aetiology. Methods and results We identified all Danish patients younger than 50 years receiving a first pacemaker due to AVB between January 1996 and December 2015. By reviewing medical records, we included patients with AVB of unknown aetiology. A matched control cohort was established. Follow-up was performed using national registries. The primary outcome was a composite endpoint consisting of death, heart failure hospitalization, ventricular tachyarrhythmia, and cardiac arrest with successful resuscitation. We included 517 patients, and 5170 controls. Median age at first pacemaker implantation was 41.3 years [interquartile range (IQR) 32.7–46.2 years]. After a median follow-up of 9.8 years (IQR 5.7–14.5 years), the primary endpoint had occurred in 14.9% of patients and 3.2% of controls [hazard ratio (HR) 3.8; 95% confidence interval (CI) 2.9–5.1; P < 0.001]. Patients with persistent AVB at time of diagnosis had a higher risk of the primary endpoint (HR 10.6; 95% CI 5.7–20.0; P < 0.001), and risk was highest early in the follow-up period (HR 6.8; 95% CI 4.6–10.0; P < 0.001, during 0–5 years of follow-up). Conclusion Atrioventricular block of unknown aetiology presenting before the age of 50 years and treated with pacemaker implantation was associated with a three- to four-fold higher rate of the composite endpoint of death or hospitalization for heart failure, ventricular tachyarrhythmia, or cardiac arrest with successful resuscitation. Patients with persistent AVB were at higher risk. These findings warrant improved follow-up strategies for young patients with AVB of unknown aetiology.

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Longevity in Patients With High Degree Atrioventricular Block Paced in the Atrial Synchronous or the Fixed Rate Ventricular Inhibited Mode

Pacing and Clinical Electrophysiology ◽

10.1111/j.1540-8159.1992.tb06500.x ◽

1992 ◽

Vol 15 (3) ◽

pp. 304-313 ◽

Cited By ~ 35

Author(s):

CECILIA LINDE-EDELSTAM ◽

BO GULLBERG ◽

ROLF NORLANDER ◽

S. KENNETH PEHRSSON ◽

MARTEN ROSENQVIST ◽

...

Keyword(s):

Atrioventricular Block ◽

Fixed Rate ◽

High Degree

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Clinical and genetic analysis of KATP variants with heart failure risk in patients with decreased serum ApoA-I levels

The Journal of Clinical Endocrinology & Metabolism ◽

10.1210/clinem/dgab336 ◽

2021 ◽

Author(s):

Cheng Liu ◽

Yanxian Lai ◽

Jingxian Pei ◽

Huiling Huang ◽

Junfang Zhan ◽

...

Keyword(s):

Heart Failure ◽

Potassium Channels ◽

Vascular Reactivity ◽

Expression Profiles ◽

Channel Coupling ◽

Lower Serum ◽

Increased Risk ◽

High Degree ◽

Generation Sequencing

Abstract Context Lower serum concentration of apolipoprotein A-I (ApoA-I) is causally associated with heart failure (HF) risk. ATP-sensitive potassium channels (KATP), as a gating channel coupling vascular reactivity and metabolism with ischemic protection, become a new potential target of management for HF. The KATP gene sequence is highly polymorphic and high degree of genetic heterogeneity. Objective To determine whether ATP-sensitive potassium channels (KATP) variants predict the risks of decreased ApoA-I concentration and its related HF. Design, Patients, Settings A total of 634 subjects, including 317 subjects with decreased ApoA-I concentration (< 120 mg/dL) and 317 counterpart subjects (≥ 120 mg/dL), were retrospectively selected. Methods 5 KATP variants were genotyped through MassARRAY platform. The exosome-derived microRNAs (exo-miRs) expression profiles were identified by next-generation sequencing, and the top 10 DE exo-miRs were verified using qPCR in a validation cohort of 240 subjects with decreased ApoA-I concentration. Results KATP rs141294036 was related to increased risk of lower ApoA-I levels (adjusted OR=1.95, P=0.002) and HF incidence (adjusted OR=2.38, P=0.009), especially HFpEF (adjusted OR=2.13, P=0.015). After median 48.6-months follow-up, participants carrying CC genotype of rs141294036 was associated with elevated HF re-hospitalization risk (adjusted HR=1.91, P=0.005). 36 exo-miRs were significantly differentially expressed between different genotypes of rs141294036 in subjects with lower ApoA-I levels, but only 5 exo-miRs (miR-31-5p, miR-126-5p, miR-106a-5p, miR-378i and miR-181c-5p) were further confirmed. Conclusions The KATP rs141294036 was associated with increased risks of lower ApoA-I levels, HF incidence (especially HFpEF) and HF re-hospitalization, involving in those 5 confirmed exo-miRs and its related metabolic pathways.

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Predictors of Acute Kidney Injury in Patients with High-degree Atrioventricular Block

Kosuyolu Heart Journal ◽

10.51645/khj.20035 ◽

2020 ◽

Vol 23 (3) ◽

pp. 157-162

Author(s):

Ayça Gümüşdağ ◽

Koray Demir ◽

Özlem Yıldırımtürk ◽

Emrah Bozbeyoğlu ◽

Ömer Kozan

Keyword(s):

Acute Kidney Injury ◽

Atrioventricular Block ◽

Kidney Injury ◽

High Degree

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Pediatrician Participation in Medicaid: 1978 to 1989

PEDIATRICS ◽

10.1542/peds.85.4.567 ◽

1990 ◽

Vol 85 (4) ◽

pp. 567-577 ◽

Cited By ~ 2

Author(s):

Beth K. Yudkowsky ◽

Jenifer D. C. Cartland ◽

Samuel S. Flint

Keyword(s):

Policy Options ◽

Medicaid Program ◽

Limited Participation ◽

Medicaid Reimbursement ◽

American Academy Of Pediatrics ◽

Private Market ◽

Medicaid Beneficiaries ◽

High Degree ◽

Medicaid Children

Optimal pediatrician participation in the Medicaid program is essential if the full benefits of recent eligibility expansions are to be realized. A 1989 national survey of the members of the American Academy of Pediatrics (n = 940), designed as a follow-up to similar studies conducted in 1978 and 1983, was an examination of the factors that influence pediatrician participation. Between 1978 and 1989: (1) basic participation (treating any Medicaid beneficiaries) declined to 77% from 85%; (2) limited participation (seeing only some Medicaid beneficiaries who request care) increased from 26% to 39.4%; and (3) extent of participation (the percentage of a pediatrician's patients who are Medicaid beneficiaries) increased from 15.7% to 19.4%. A dichotomous conceptualization of participation (restricted or unrestricted) was developed. By this definition, only 56% of pediatricians allowed comparable access to their practices for both Medicaid and private patients. Low reimbursement and slow payments discouraged participation. Medicaid reimbursement to pediatricians was approximately equal to their overhead costs. However, a high degree of willingness to care for Medicaid children remains if fees are increased to within 11% to 16% of the private market level. Policy options to enhance participation are discussed.

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Pacemaker use for the treatment of reflex-mediated syncope: 40-year experience at a single paediatric institution

Cardiology in the Young ◽

10.1017/s1047951121004340 ◽

2021 ◽

pp. 1-6

Author(s):

Thomas Huang ◽

Edward O’Leary ◽

Mark E. Alexander ◽

Laura Bevilacqua ◽

Francis Fynn-Thompson ◽

...

Keyword(s):

Young Adults ◽

Atrioventricular Block ◽

Sinus Bradycardia ◽

Pacemaker Implantation ◽

Complication Rates ◽

Single Centre Experience ◽

Procedural Complications ◽

Children And Young Adults ◽

Dual Chamber Pacemakers

Abstract Introduction: Reflex-mediated syncope occurs in 15% of children and young adults. In rare instances, pacemakers are required to treat syncopal episodes associated with transient sinus pauses or atrioventricular block. This study describes a single centre experience in the use of permanent pacemakers to treat syncope in children and young adults. Materials and methods: Patients with significant pre-syncope or syncope and pacemaker implantation from 1978 to 2018 were reviewed. Data collected included the age of presentation, method of diagnosis, underlying rhythm disturbance, age at implant, type of pacemaker implanted, procedural complications and subsequent symptoms. Results: Fifty patients were identified. Median age at time of the first syncopal episode was 10.2 (range 0.3–20.4) years, with a median implant age of 14.9 (0.9–34.3) years. Significant sinus bradycardia/pauses were the predominant reason for pacemaker implant (54%), followed by high-grade atrioventricular block (30%). Four (8%) patients had both sinus pauses and atrioventricular block documented. The majority of patients had dual-chamber pacemakers implanted (58%), followed by ventricular pacemakers (38%). Median follow-up was 6.7 (0.4–33.0) years. Post-implant, 4 (8%) patients continued to have syncope, 7 (14%) had complete resolution of their symptoms, and the remaining reported a decrease in their pre-syncopal episodes and no further syncope. Twelve (24%) patients had complications, including two infections and eight lead malfunctions. Conclusions: Paediatric patients with reflex-mediated syncope can be treated with pacing. Complication rates are high (24%); as such, permanent pacemakers should be reserved only for those in whom asystole from sinus pauses or atrioventricular block has been well documented.

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Takotsubo syndrome presenting with high-degree atrioventricular block with ventricular asystole

Journal of Cardiology Cases ◽

10.1016/j.jccase.2021.09.004 ◽

2021 ◽

Author(s):

Hyobae Kown ◽

Jongkwon Seo ◽

Byung Gyu Kim ◽

Gwang Sil Kim ◽

Moo-Nyun Jin ◽

...

Keyword(s):

Atrioventricular Block ◽

Takotsubo Syndrome ◽

High Degree

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10 Years' Experience in Balanitis Xerotica Obliterans: A Single-Institution Study

European Journal of Pediatric Surgery ◽

10.1055/s-0038-1668562 ◽

2018 ◽

Vol 29 (03) ◽

pp. 302-306 ◽

Cited By ~ 3

Author(s):

Akhilesh Pradhan ◽

Radhika Patel ◽

A. Said ◽

Manasvi Upadhyaya

Keyword(s):

Average Duration ◽

Age Group ◽

Histological Findings ◽

Single Institution ◽

Exact Test ◽

Balanitis Xerotica Obliterans ◽

Tertiary Teaching ◽

Tertiary Teaching Hospital ◽

High Degree

Introduction Balanitis xerotica obliterans (BXO) is uncommon in children. Diagnosis of the condition is almost always clinical and supported by histology. Our aim was to evaluate the outcomes of children undergoing circumcision for BXO and explore the correlation between surgical and histological findings. Materials and Methods A 10-year retrospective review (2007–2017) of all children, aged 16 and less, undergoing circumcision at a tertiary teaching hospital was conducted. Statistical analysis was performed using Fisher's exact test. Results BXO occurred in 91/1025 (8.9%) children. The highest incidence of BXO was in the 5 to 10 age group (13.3%; p < 0.0001). The commonest symptom was foreskin scarring (62.6%). Intraoperatively, involvement of foreskin alone was seen in 26.4%, foreskin and meatus in 47.2%, and foreskin, meatus, and glans in 26.4%. Preoperatively, 24.2% of patients were prescribed steroid cream. Histologically, all patients showed microscopic changes confirming BXO. A total of 87.9% of patients only had a circumcision, and 11% required a meatal procedure along with the circumcision. Postoperatively, 19.8% of patients required a further procedure after an average duration of 5.8 months (range: 2–12 months); the majority of whom (83.3%) were prescribed postoperative steroid cream. Conclusion Clinical correlation by surgeons has a high degree of accuracy (>90%). Meatal involvement is more common than previously reported. The use of pre- or postoperative steroids does not obviate the need for further surgical procedures. Patients can have recurrent symptoms 1 year following surgery, and prolonged follow-up is necessary.

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