Carbohydrate counting in the nutritional treatment of Citrullinemia Type II: Case report

Summary Background & aims Citrullinemia Type II is a hereditary disease characterized by citrine and arginine succinate deficiency in the liver. Here we report the first Brazilian patient described with Citrullinemia Type II in Brazil and the diet therapy approach in his case, based on carbohydrate counting. Methods The study was conducted at the University Hospital, Faculty of Medicine of Ribeirao Preto, University of Sao Paulo, between March and September 2011. A 2000 kcal diet with carbohydrate counting and containing (10–45–45%) proteins, carbohydrates and lipids, respectively, was prescribed. Energy and macronutrient consumption were described before and after the carbohydrate counting and the influence of dietary plan on blood ammonia levels were evaluated by laboratory analyses. Descriptive analysis of the data was performed using the median and interquartile range (IQR). Results Median carbohydrate intake before and after the counting method was 287 g (IQR = 55 g) and 257 g (IQR = 63 g), respectively. Median serum ammonia levels before and after the counting method were 106 μg/dL and 48 μg/dL (reference value of 80 μg/dL), respectively Conclusions Carbohydrate counting was satisfactory for the nutritional treatment of the patient and contributed to the reduction of serum ammonia levels.