Tourette syndrome in children: an updated review.

Tourette syndrome (TS) is a common neuropsychiatric disorder in children characterized by multiple motor and vocal tics that fluctuate in severity and lasting for at least 1 year. Boys are more commonly affected than girls. Symptoms usually begin with simple motor or vocal tics which then evolve into more complex motor and vocal tics over time. Premonitory sensory urges are common in children over the age of 8 years, and these urges help distinguish tics from symptoms of other movement disorders. Common comorbidities of TS include attention deficit hyperactivity disorder, obsessive-compulsive disorder and learning difficulties. Several genes have been assessed as candidate genes for TS; environmental factors such as stress and streptococcal infections might also contribute to its etiology. The pathophysiology of TS mainly involves dysfunction of basal ganglia-related circuits and hyperactive dopaminergic innervations. A thorough history assessment and neurological examination are important for the correct diagnosis and differentiation from other movement disorders. Treatment for TS should focus on improving the patient's social functioning, minimizing the impairment from cormobid disorders, and controlling tics, if they are severe. Commonly used medications for TS include a2-adrenergic agonists and atypical neuroleptics. Habit reversal therapy is an effective option for TS, and repetitive transcranial magnetic stimulation may be a promising approach for severe cases.

Comparison of growth in primary Fanconi syndrome and proximal renal tubular acidosis.

To compare the difference between primary proximal renal tubular acidosis (PRTA) and Fanconi syndrome (FS), and to find out possible risk factors for growth retardation, we studied the long-term growth, clinical, laboratory, and radiological findings associated with the treatment of six children with primary FS and 15 children with PRTA. The ages of the children with FS were much older than those with PRTA at initial diagnosis (7.03+/-3.82 vs. 1.63+/-1.56 years). The height standard deviation score (SDS) at the start of treatment was significantly lower in FS than in PRTA. Catch-up growth was noted in PRTA at the end of follow-up (initial height SDS -2.13+/-1.10 vs. last height SDS -1.33+/-1.43, P=0.023 by paired t-test), whereas apparent linear growth impairment was found in FS in terms of overall growth velocity index (82.70+/-8.37%) and height SDS (initial -3.25+/-0.95 vs. last -3.15+/-0.31, P=0.791). There was also a higher rate of rickets occurrence in FS (3/6 vs. 0/15 in PRTA). Hypophosphatemia during the follow-up period was more frequent for FS than PRTA (69.2+/-26.1% vs. 7.0+/-25.8%, P<0.001), whereas metabolic acidosis (blood HCO(3)<20 mmol/l) was less efficiently corrected in PRTA (49.1+/-20.5% vs. 25.2+/-21.6% in FS, P=0.028). Moreover, the height Delta SDS correlated well with the mean serum P level during the treatment period in these patients (R=0.528, P=0.014 for all children; R=0.917, P=0.01 for FS patients). Our data suggest that metabolic acidosis may not be the sole factor causing growth impairment in FS. Correction of metabolic acidosis may indeed improve growth in PRTA but not in FS. This study indicates that factors other than metabolic acidosis, such as phosphate depletion and delayed diagnosis/treatment, should be considered to be important causes of growth retardation in FS.

Effective ultrasonographic predictor for the diagnosis of acute lobar nephronia.

BACKGROUND: Correct identification of acute lobar nephronia (ALN) is necessary to prevent progression to renal abscess. The goal of this retrospective study was to determine whether the sonographic finding of severe nephromegaly (i.e. renal length greater than mean + 3 sd) is a preselection criterion for computed tomographic (CT) scanning in diagnosing pediatric ALN among children with an acute upper urinary tract infection. DESIGN/METHODS: We evaluated a new imaging work-up scheme to detect pediatric ALN. All patients with urinary tract infection were evaluated with ultrasonography. If a markedly enlarged kidney or focal mass was present sonographically, CT scanning was done immediately. CT scanning was also performed when the patient had borderline nephromegaly and remained febrile for 72 h after start of antibiotic treatment. ALN diagnosis was made on the basis of positive CT findings. RESULTS: Thirty patients with ALN (13 left, 7 right, 10 bilateral) and one with acute pyelonephritis were identified. ALN in all patients resolved after 3 weeks of antibiotic treatment. Thirty-nine of the 62 kidneys evaluated showed severe nephromegaly, and 10 had focal renal masses. With CT diagnosis of ALN as the reference standard, the sensitivity of severe nephromegaly was 90.0% and the specificity was 86.4%. When the focal renal mass was added as a combining predictor, the sensitivity further increased to 95%. CONCLUSIONS: Pediatric ALN was effectively predicted using sonographic findings of severe nephromegaly and/or focal mass before CT scanning.