ABSTRACT
Use and misuse of EEG Electroencephalogram (EEG) has an important role in the investigation of patients with different neurological disorders, especially epilepsy. Knowledge about the method, correct indications, its strength/weakness and the actual diagnosis is crucial to get the best out of EEG. The study of EEG-requests at a Swedish hospital during one year showed that 78% were consistent with guidelines and 86% of the acute requests were medically motivated or justifiable.
Subject(s)
Electroencephalography/statistics & numerical data , Referral and Consultation/standards , Adolescent , Brain Diseases/diagnosis , Child , Child, Preschool , Encephalitis/diagnosis , Epilepsy/diagnosis , Guideline Adherence/statistics & numerical data , Headache/diagnosis , Hospital Departments/statistics & numerical data , Humans , Infant , Pediatrics , Seizures/diagnosis , Sweden , Unnecessary ProceduresABSTRACT
AIM: Prader-Willi syndrome is a neurogenetic disorder, with characteristics such as obesity, short stature, muscular weakness, intellectual deficiencies and deviant social behaviour. This study evaluated whether growth hormone treatment of children with Prader-Willi syndrome resulted in possible and lasting effects on their cognition and behaviour. METHODS: We randomised six girls and 13 boys to either a treatment group or a control group. The treatment group received growth hormone (Genotropin(®) 0.033 mg/kg/day) for 2 years, while the control group did not receive treatment in the first year and then received a double dose in the second year. Treatment was then stopped in both groups for 6 months. RESULTS: Both groups showed the same intellectual disabilities at the start of the study, and no difference was found after the first and second years. The parents reported that the children showed increased vitality during treatment. When treatment was stopped, the children showed a marked exacerbation of behavioural problems, a significant increase in body fat and a decrease in insulin-like growth factor 1 levels. CONCLUSION: We believe this is the first study to show that abrupt-ceasing growth hormone treatment led to a successive deterioration in behavioural problems in children with Prader-Willi syndrome.
Subject(s)
Child Behavior/drug effects , Cognition/drug effects , Human Growth Hormone/therapeutic use , Prader-Willi Syndrome/drug therapy , Child , Child, Preschool , Female , Human Growth Hormone/pharmacology , Humans , Insulin-Like Growth Factor I/metabolism , Male , Prader-Willi Syndrome/blood , Prader-Willi Syndrome/psychology , Prospective StudiesABSTRACT
Aarskog syndrome is an X-linked disorder characterized by faciogenital dysplasia and short stature. The present study set out to determine the effect of growth hormone (GH) therapy in patients with Aarskog syndrome enrolled in KIGS--the Pharmacia International Growth Database. Twenty-one patients (20 males) were evaluated. Median age at start of treatment was 8.3 years (10-90th percentiles, 5.1-14.1 years) and median height SDS was -2.8 (10-90th percentiles, -2.1 to -3.7). The median dose of GH was 0.22 mg/kg/week (10-90th percentiles, 0.15-0.30 mg/kg/week) given at a median frequency of six (4-7) times per week. Prepubertal patients were followed longitudinally for 1 year (n = 13) or 3 years (n = 7). After 1 year, the median height SDS had improved from -2.8 to -2.3 in 13 patients. After 3 years, height SDS had improved significantly (p <0.05) to -1.8 (10-90th percentiles, -2.1 to -1.1) in the seven patients. No adverse events were noted. Although final height data for these patients are still awaited, the present results support the use of GH to promote growth in children with Aarskog syndrome.
