Abstract | OBJECTIVE: DESIGN: A prospective cohort study. SETTING: A pediatric tertiary hospital in Beijing; all patients were followed until death or June 2013. SUBJECTS: OUTCOME MEASURES: The data of clinical characteristics and pretreatment biochemical parameters were collected. The Cox regression analysis was performed to identify independent risk factors for survival of patients with methylmalonic acidemia and homocystinuria. The best cutoff values for these independent factors were determined by the receiver characteristic curve. RESULTS: Newborn onset (OR=6.856, 95%CI=2.241-20.976, P=0.001), high level of methylmalonic acid in urine (OR=1.022, 95%CI=1.011-1.033, P<0.001), and high level of urea in serum (OR=1.083, 95%CI=1.027-1.141, P=0.003) were independent negative risk factors for survival of patients with methylmalonic acidemia and homocystinuria. The cutoff values of maximum predictive accuracy of methylmalonic acid in urine and urea in serum were respectively 5.41 mmol/mmol creatinine and 7.80 mmol/L by receiver operating characteristic curve analysis. CONCLUSIONS:
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Authors | Li Qiliang, Song Wenqi, Wang Quan, Yang Xinying, Li Jiuwei, Sun Qiang, Peng Xiaoxia, Wang Peichang |
Journal | Indian pediatrics
(Indian Pediatr)
Vol. 52
Issue 2
Pg. 119-24
(Feb 2015)
ISSN: 0974-7559 [Electronic] India |
PMID | 25691178
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Topics |
- Amino Acid Metabolism, Inborn Errors
(complications, diagnosis, epidemiology, mortality)
- Beijing
(epidemiology)
- Female
- Homocystinuria
(complications, diagnosis, epidemiology, mortality)
- Humans
- Male
- Prognosis
- Prospective Studies
- Treatment Outcome
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