ESPE Abstracts (2015) 84 P-2-258

Cardiac Autonomic Neuropathy is Highly Predictive for Survival in Children with Mauriac Syndrome

Margarita Arshinkova, Olga Slavcheva & Maia Konstantinova

University Pediatric Hospital, Sofia, Bulgaria

Background: Diabetic autonomic neuropathy (DAN) is predictive for subsequent mortality mainly due to terminal renal insufficiency. DAN is screened by cardiac tests based on heart rate variability (HRV).

Aim: To assess cardiac autonomic neuropathy (CAN) and its predictive value for survival in children with Mauriac syndrome.

Material and methods: The study included 12 patients with Mauriac syndrome (growth retardation, hepatomegaly and delayed puberty, seven boys, five girls). Ten were born 1980–1987 and two children 2005-2007. Time-domain and frequency-domain analyzes of R-R intervals of ECG at rest and during sympathetic and vagal stimuli were employed. Deviations out of referent 25–75 percentiles or ±2 standard deviation scores (SDSs) were accepted as abnormal for heart rate (HR), HRV at rest, deep breathing (DB) and Valsalva maneuver and for total power (TP) and low/high frequency (LF/HF) of spectral analysis. The results were compared with 346 healthy controls and 202 diabetic children with normal growth.

Results: The children with Mauriac syndrome had mean age at diagnosis 3.4±2.5 year (eight months to 8.3 years), younger than other diabetic children (P<0.05); mean age at the investigation 15.3±2.7 (9.7 – 17.9 years); diabetes duration 12.1±3 years (7.6 – 15.9); mean HbA1c 10.9±2.17% (4.2–6). Growth retardation was −3,27±0.92 SDS. They had one or more late diabetic complications. The deviations below 25th percentile for TP, CV, DB, Valsalva and over 75th percentile for HR and LF/HF were 100% in the group. Mean SDSs for all HRV parameters were statistically different from healthy controls and diabetic children with normal growth/no late complications. (P < 0.05). At present, 8 out of 10 patients born before 1987 were not alive. Main reason for death was terminal renal insufficiency.

Conclusion: Children with Mauriac syndrome developed CAN before age of 18 years together with other late complications. CAN predicted poor survival.

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