Cardiac pacing in patients with familial dysautonomia

Bartoletti, Angelo

doi:10.1007/s10286-005-0242-2

Cited by 6 publications

(1 citation statement)

References 40 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Autonomic dysfunction is documented in cases of lymphoma, 5 but it is important to clarify whether this is a paraneoplastic manifestation or a consequence of chemotherapy 7,12 because, in the first hypothesis, chemotherapy can cure the alteration, while in the second one it may exacerbate the dysfunction, with all the well-known consequences. In fact, autonomic impairment is responsible for a higher mortality rate due to cardiovascular disease, such as sudden death resulting from cardiac arrhythmia.…”

Section: Discussionmentioning

confidence: 99%

Autonomic Dysfunction in Hodgkin and Non-Hodgkin Lymphoma. A Paraneoplastic Syndrome?

et al. 2010

View full text Add to dashboard Cite

We wanted to determine whether autonomic dysfunction in patients with lymphoma is related to chemotherapy or represent a paraneoplastic syndrome. 40 patients with current or cured Hodgkin or non-Hodgkin lymphoma and 40 healthy controls, matched for age, gender, hypertension and diabetes mellitus underwent autonomic evaluation (Deep Breath, Valsalva Maneuver, Hand Grip, Lying to Standing, Tilt Test). Current patients also suffering from diabetes or hypertension, or still on chemotherapy revealed autonomic changes, while cured or healthy subjects did not. Autonomic dysfunction in lymphoma is a transient manifestation of a paraneoplastic syndrome.

show abstract

Section: Discussionmentioning

confidence: 99%

Autonomic Dysfunction in Hodgkin and Non-Hodgkin Lymphoma. A Paraneoplastic Syndrome?

et al. 2010

View full text Add to dashboard Cite

show abstract

Familial dysautonomia: Frequent, prolonged and severe hypoxemia during wakefulness and sleep

Weese‐Mayer

Kenny

Bennett

et al. 2008

Pediatric Pulmonology

View full text Add to dashboard Cite

Sudden unexplained deaths have been reported in 13% [corrected] of Familial Dysautonomia (FD) subjects. To characterize cardiorespiratory dysregulation in children with FD that might contribute to potential sudden death, respiratory inductance plethysmography (chest/abdomen), ECG, hemoglobin saturation, and pulse waveform (VivoMetrics, Inc.) were recorded in the home during daytime wakefulness and overnight sleep in 25 children with IKBKAP mutation-confirmed FD and 25 age-, and gender-matched controls. Breath-to-breath and beat-to-beat characterization of breathing, hemoglobin saturation, and heart rate was conducted. Children with FD had more frequent, prolonged, and severe episodes of hypoxemia than matched controls, awake and asleep. Though a small percent of the study time revealed bradycardia and apnea, the hypoxemia was the most prevalent pattern in FD and rarely occurred with related bradycardia. Though infrequent with desaturation or bradycardia, apnea was more prevalent in FD subjects than controls, and more apparent during sleep than wakefulness. Children with FD have cardiorespiratory dysregulation during wakefulness and sleep, likely representing alveolar hypoventilation. We hypothesize that the related repeated hypoxemia (and presumed related hypercarbia) may render individuals with FD more vulnerable to sudden death.

show abstract

Documented Transient Third-Degree Atrioventricular Block and Asystole in a Child with Familial Dysautonomia

2007

View full text Add to dashboard Cite

An 11-year-old boy with familial dysautonomia presented with palpitations. Continuous 24-h Holter monitoring revealed intermittent high-grade atrioventricular block and asystole. The unopposed parasympathetic tone in patients with dysautonomia may make them susceptible to bradycardia and atrioventricular block. We recommend routine 24-h Holter monitoring screening and, when indicated, consideration of pacemaker implantation to reduce the high risk of sudden death phenomena in this patient population.

show abstract

Cardiac pacing in patients with familial dysautonomia

Cited by 6 publications

References 40 publications

Autonomic Dysfunction in Hodgkin and Non-Hodgkin Lymphoma. A Paraneoplastic Syndrome?

Autonomic Dysfunction in Hodgkin and Non-Hodgkin Lymphoma. A Paraneoplastic Syndrome?

Familial dysautonomia: Frequent, prolonged and severe hypoxemia during wakefulness and sleep

Documented Transient Third-Degree Atrioventricular Block and Asystole in a Child with Familial Dysautonomia

Contact Info

Product

Resources

About