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Suggested surveillance for individuals with congenital central hypoventilation syndrome (CCHS)

	At diagnosis	Follow-up
Polysomnography (including endogenous and exogenous gas challenges)	X	0 to 3 years: Every 6 months 3 years and older: At least annually
Echocardiogram	X	0 to 3 years: Every 6 months 3 years and older: At least annually
Hemoglobin, hematocrit, reticulocytes, blood gas including HCO₃	X	0 to 3 years: Every 6 months 3 years and older: At least annually
Electrocardiography (72-hour continuous monitoring)	X	0 to 3 years: Every 6 months 3 years and older: At least annually
Neurocognitive testing	X	0 to 3 years: Every 6 months 3 years and older: Annually
Neural crest tumor surveillance
NPARM mutations: Chest and abdominal imaging, urine catecholamines (for neuroblastoma)	X	0 to 2 years: Every 3 months 2 to 7 years: Every 6 months
Long PARM mutations (20/28 and longer): Chest and abdominal imaging (for ganglioneuromas and ganglioneuroblastomas)	X	Annually
Short PARM mutations (20/24, 20/25, 20/26, or 20/27)	Not required	Not required

This table outlines the approach to surveillance suggested in the American Thoracic Society (ATS) statement. Evidence for these guidelines is based on the ATS authors' experience rather than randomized trials or cohort studies. Other approaches to monitoring may be appropriate depending on the characteristics and history of the individual patient and practicality within the local health care system.

CCHS: congenital central hypoventilation syndrome; HCO₃: bicarbonate; NPARM: non-polyalanine repeat mutations; PARM: polyalanine repeat mutations; ATS: American Thoracic Society.

Source: Based on recommendations in: Weese-Mayer DE, Berry-Kravis EM, Ceccherini I, et al. An official ATS clinical policy statement: Congenital central hypoventilation syndrome: genetic basis, diagnosis, and management. Am J Respir Crit Care Med 2010; 181:626.

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