... with metabolic diseases with sufficient resources is essential. Prevention of Primary Manifestations Dietary restriction of lysine intake ... threshold for hospital admission (see Treatment of Manifestations ). Prevention of Secondary Complications One of the most important ...

... poor nutrition, and risk of aspiration ( Figure 2b ) Prevention of Primary Manifestations Cystine depletion therapy with cysteamine ... or postpones retinal deterioration [ Tsilou et al 2006 ]. Prevention of Secondary Complications Affected individuals who have undergone ...

... but rather support optimal speech and language development. Prevention of Primary Manifestations Prevention of hyperammonemia is achieved ... based on metabolic control (see Treatment of Manifestations ). Prevention of Secondary Complications One of the most important ...

... behavior can be addressed by a pediatric psychiatrist. Prevention of Primary Manifestations Medical and Dietary Prevention of Hyperammonemia In neonatal-onset OTC deficiency diagnosed ...

... and care should be coordinated with FA experts. Prevention of Primary Manifestations Human papilloma virus (HPV) vaccination ... the risk of oral cancer in all individuals. Prevention of Secondary Complications Individuals with FA treated with ...

... PT = physical therapy; RDP = rapid-onset dystonia-parkinsonism Prevention of Primary Manifestations Note: Pallidal deep brain stimulation ... Brücke et al 2014 ; Brashear A, personal communication]. Prevention of Secondary Complications Table 4. Prevention of Primary ...

... Morton et al 2002 , Zinnanti et al 2009 ]. Prevention of Primary Manifestations Table 9. Prevention of Primary ... chain alpha-ketoacid dehydrogenase (BCKD) mutants is controversial. Prevention of Secondary Complications Any trauma care or surgical ...

... with metabolic diseases with sufficient resources is essential. Prevention of Primary Manifestations High carbohydrate intake (typically >60%), ... low threshold for hospital admission (see Table 7 ). Prevention of Secondary Complications One of the most important ...

... formal developmental evaluation and intervention before school age. Prevention of Primary Manifestations Prevention of primary manifestations (see ... birth; treatment and monitoring may then start immediately. Prevention of Secondary Complications Prevention or reduction of serious ...

... control. This may require years of intensive therapy. Prevention of Primary Manifestations See Table 8 . Prevention of Secondary Complications Because bone mineral density in ...