L-dopa is a potent stimulator of cortisol in short children.

AIMS: In this study, we evaluated the diagnostic usefulness of oral L-dopa as a stimulatory agent for cortisol. METHODS: In 27 short children that were evaluated for possible growth hormone deficiency (GHD), the levels of serum GH and cortisol were determined after oral L-dopa administration and after i.m. glucagon administration. We defined cortisol concentrations >18 µg/dl (496 nmol/l) as adequate response. Peak GH concentration <10 ng/ml in both tests defined GHD. RESULTS: Twenty-five out of the 27 children (93%) studied showed a normal cortisol response, i.e. a peak serum cortisol >18 µg/dl in the L-dopa test, whereas 19 children (70%) had a normal cortisol response after stimulation with glucagon. In the children with normal cortisol response in both tests, the mean peak serum cortisol concentration was 28.7 (SD 1.59) after L-dopa and 26.65 (SD 1.26) µg/dl after glucagon administration. There was no statistically significant difference in peak serum cortisol response to L-dopa between GH-deficient and GH-sufficient children [25.90 (SD 4.9) vs. 29.87 (SD 9.9) µg/dl, respectively]. CONCLUSIONS: These results clearly suggest that L-dopa administration is a potent stimulus for cortisol secretion at least in short children.

The diversity of juvenile sarcoidosis symptoms.

We report a case of juvenile sarcoidosis, emphasizing the variety of clinical manifestations. The child had uveitis, which is among the most common manifestations of the disease. However, fever of unknown origin, glomerulonephritis and lymphadenopathy were also noticed, underscoring the diversity of the clinical spectrum of the disease.

Menetrier's disease associated with Helicobacter pylori: three cases with sonographic findings and a literature review.

Menetrier's disease is a rare cause of protein-losing gastropathy characterised by hypertrophy of the gastric folds. Three cases are reported. Helicobacter pylori was detected by culture and CLO test in two cases and on follow-up gastroscopy in the other. The first two patients were given eradication therapy and hypertrophic gastropathy resolved whereas in the third the disease remitted prior to the eradication treatment. Typical sonographic features were thickened gastric wall with preservation of the wall stratification. A review of the literature found eight cases of Menetrier's disease associated with H. pylori in children and these cases are briefly reviewed. H. pylori infection should be considered in all children with Menetrier's disease and, if isolated, eradication treatment should be administered.

Uveitis: an isolated complication of post-streptococcal syndrome.

A child with anterior uveitis as the sole manifestation of group A streptococcal infection is described. There was a history of a 'viral' upper respiratory tract infection 2 weeks before the onset of uveitis. A post-streptococcal phenomenon was diagnosed on the basis of serial ASO titre (ASOT) monitoring. There are few reports of patients with post-streptococcal uveitis. ASOT monitoring should be included in the work-up of uveitis of undetermined aetiology.

Long term follow up of a woman with classic form of Ehlers-Danlos syndrome associated with rare manifestations and review of the literature.

We present the case of a 46 year-old woman with the classic type of Ehlers-Danlos syndrome who developed the rare manifestations of colon diverticula and mitral valve prolapse. We emphasize on clinical features and complications associated to this type of the syndrome, which gradually developed in our patient. A review of the literature referring to the epidemiology, molecular basis and manifestations of the classic type Ehlers-Danlos syndrome is also discussed.

Is there an association between wheezing and pneumonia?

Objective The aim of this study was to investigate whether there is a relationship in school aged children between wheezing and pneumonia prior, during, or following the pneumonia episode. Patients-Methods One hundred and three children with community acquired pneumonia who were hospitalised were recruited along with 55 controls. Results During hospitalisation wheezing was audible in 11/103 (10.6%) patients with pneumonia and in none of the controls (p=0.009). Wheezing ever or asthma was elicited in 29/103(28%) patients with pneumonia and in 8/55 (14.5%) of the controls and this difference was not significant. Two years after the hospitalisation with pneumonia, wheezing episodes occurred in 12/103 with pneumonia and 1/55 of the controls (p=0.034). Among those who developed asthma following pneumonia 11/12 also had wheezing prior to pneumonia. Conclusion There is an excess of wheezing prior, during, and after an episode of pneumonia in school aged children and therefore children with pneumonia should be followed up carefully

No disponible

Is there an association between wheezing and pneumonia?

OBJECTIVE: The aim of this study was to investigate whether there is a relationship in school aged children between wheezing and pneumonia prior, during, or following the pneumonia episode. PATIENTS-METHODS: One hundred and three children with community acquired pneumonia who were hospitalised were recruited along with 55 controls. RESULTS: During hospitalisation wheezing was audible in 11/103 (10.6%) patients with pneumonia and in none of the controls (p=0.009). Wheezing ever or asthma was elicited in 29/103(28%) patients with pneumonia and in 8/55 (14.5%) of the controls and this difference was not significant. Two years after the hospitalisation with pneumonia, wheezing episodes occurred in 12/103 with pneumonia and 1/55 of the controls (p=0.034). Among those who developed asthma following pneumonia 11/12 also had wheezing prior to pneumonia. CONCLUSION: There is an excess of wheezing prior, during, and after an episode of pneumonia in school aged children and therefore children with pneumonia should be followed up carefully.

Splitting the dose of neonatal BCG vaccination: a naïve practice.

Bilateral axillary lymphadenitis complicating BCG vaccination in both arms (0.025 ml) in an immunocompetent 13-month-old boy is described. Prompt administration of isoniazid and erythromycin did not prevent suppuration of the affected lymph nodes. It was managed by bilateral surgical excision of the nodes.

Clinical notations on bacteremic cavitating pneumococcal pneumonia in nonvaccinated immunocompetent children.

We describe 10 children who developed lung cavitation during the treatment of bacteremic pneumococcal lobar pneumonia. Chest CT scan showed extensive consolidation with several small lucencies and air-filled cavities. Invasive procedures like aggressive pleural intervention were not needed and the children recovered attaining normal lung function on long-term follow-up.

Endothelin 1 levels in relation to clinical presentation and outcome of Henoch Schonlein purpura.

BACKGROUND: Henoch Schonlein purpura (HSP) is a common vasculitis of small vessels whereas endothelin-1 (ET-1) is usually reported elevated in vasculities and systematic inflammation. The aim of the present study was to investigate whether ET-1 levels are correlated with the clinical presentation and the outcome of HSP. METHODS: The study sample consisted of thirty consecutive patients with HSP. An equal number of healthy patients of similar age and the same gender were served as controls. The patients' age range was 2-12.6 years with a mean +/- SD = 6.3 +/- 3 years. All patients had a physical examination with a renal, and an overall clinical score. Blood and urinary biochemistry, immunology investigation, a skin biopsy and ET-1 measurements in blood and urine samples were made at presentation, 1 month later and 1 year after the appearance of HSP. The controls underwent the same investigation with the exception of skin biopsy. RESULTS: ET-1 levels in plasma and urine did not differ between patients and controls at three distinct time points. Furthermore the ET-1 were not correlated with the clinical score and renal involvement was independent from the ET-1 measurements. However, the urinary ET-1 levels were a significant predictor of the duration of the acute phase of HSP (HR = 0.98, p = 0.032, CI0.96-0.99). The ET-1 levels did not correlate with the duration of renal involvement. CONCLUSION: Urinary ET-1 levels are a useful marker for the duration of the acute phase of HSP but not for the length of renal involvement.

Maturational tempo differences in relation to the timing of the onset of puberty in girls.

AIM: To determine maturational tempo differences related to the timing of pubertal onset in girls. METHODS: We studied longitudinally 307 prepubertal Greek schoolgirls. Age at onset of puberty (B2), peak height velocity (PHV) and age at menarche were recorded. Age at onset of puberty quartiles were determined in order to define girls as early maturers (<9.2 years) and late maturers (>10.6 years). Due to dropout from the study, results for menarche are reported for 182 girls. RESULTS: Median (1st-3rd quartile) time span from B2 to PHV was longer for early maturers than for average and late maturers, 1.6 (1.0-2.2) years, 1.1 (0.7-1.7) years and 0.9 (0.3-1.1) years, respectively, p < 0.001). There was no significant difference in PHV, in the time interval from PHV to menarche and in adult height (for 75 girls) with respect to whether girls matured early, average or late. CONCLUSION: A compensatory delay in the maturational tempo in early-maturing girls and a catch-up mechanism in late-maturing girls was shown. The maturational differences manifest as differences in the time span from the onset of puberty to PHV. Early pubertal maturation in girls does not compromise adult height and is not always followed by early menarche.

Prevalence of overweight and obesity in young Greek men.

We determined the prevalence of overweight and obesity in young Greek men in 2006 and examined variations related to their place of residence and educational level. Body height and weight were measured in 2568 conscripts of the Greek army, aged 19-26 years. The calculated body mass index (BMI, kg m(-2)) was correlated to their socio-demographic characteristics, i.e. level of education and place of residence (urban or rural). Overweight and obesity were defined according to the World Health Organization classification. Mean BMI (standard deviation) of the conscripts was 24.7 (4.2). The prevalence of overweight (30 > BMI > or = 25 kg m(-2)) was 28.5% and correlated positively with a higher educational level, whereas the prevalence of obesity (BMI > or = 30 kg m(-2)) was 10.4% and correlated positively with a lower educational level. Our data were compared with those of similar studies performed in the years 1969: BMI 23.8 (1.4) (P < 0.0001) and 1990: BMI 23.8 (2.9) (P < 0.0001), showing a positive secular trend for BMI in Greek conscripts in the last 16 years. In conclusion, we documented an alarmingly high prevalence of overweight and obesity among young Greek men.

Steroid hormones polymorphisms and cholelithiasis in Greek population.

BACKGROUND: Genetic variation in genes involved in steroid biosynthesis, metabolism and signal transduction have been suggested to play a role in gallstone disease. METHODS: To elucidate the possible role of genetic variation in the estrogen receptors alpha and beta (ER-alpha, ER-beta) and androgen receptor (AR) genes in breast cancer risk, the -1174(TA)n, c.1092+3607(CA)(n) and c.172(CAG)n repeat polymorphisms of the three genes were studied. A case-control cohort of 99 patients with cholelithiasis and 179 controls were used. RESULTS: No significant difference was observed in the frequency distribution of -1174(TA)(0-26) in the ER-alpha gene between patients and controls, while a significant difference was observed in the frequency distribution of repeat polymorphism c.1092+3607(CA)5-27 and c.172(CAG)5-32 in the ER-beta gene and AR gene, respectively (P< or =0.001 and P=0.05, respectively). A significant difference was observed in the repeat genotype distribution (SS, SL, LL) in the (CA)n of the ER-beta gene (P<0.0001) and in the (CAG)n of the AR gene (P< or =0.0001). A significantly decreased odds ratio for cholelithiasis risk was observed in individuals having the SL and LL genotype for ER-beta gene compared with SS genotype (OR=0.212; 95% CI 0.105-0.426; P<0.0001 and OR=0.042; 95% CI 0.018-0.097, respectively) and LL genotype for AR gene (OR=0.622; 95% CI 0.345-1.121; P=0.114 and OR=0.287; 95% CI 0.151-0.543, P<0.0001, respectively). This protective effect of SL and LL genotypes for ER-beta and LL for AR gene remained evident (P<0.0001 for all of them) even after adjustment for various risk factors. CONCLUSIONS: In conclusion an association for cholelithiasis risk between short alleles for both c.1092+3607(CA)5-27 and c.172(CAG)5-32 repeat polymorphisms of the ER-beta and AR was found in individuals of Greek descent.

Clinical expression of co-inherited Dubin-Johnson and thalassaemic heterozygous states.

Dubin-Johnson syndrome is a worldwide prevalent familial conjugated hyperbilirubinaemia. The identification of multidrug resistance-associated protein 2 provided an understanding of the complex metabolic impairment involved in this syndrome. We report the first family with modified clinical expression of Dubin-Johnson heterozygous state due to genotypic interaction with co-inherited beta and deltabeta thalassaemia, an interaction that has never been described.

The effect of inhaled budesonide on adrenal and growth suppression in asthmatic children.

The present authors evaluated adrenal reserve in asthmatic children on long-term inhaled corticosteroids and whether possible adrenal suppression could be predicted by growth retardation. Low-dose synacthen test (0.5 microg x 1.73 m(-2)) was performed in 72 asthmatic children with a median age of 9.4 (range 4.2-15.7) yrs on long-term treatment (median 18 (range 6-84) months) with low-to-moderate doses (median 363 (range 127-1012) microg x m(-2)) of inhaled budesonide, as well as in 30 controls. Adrenal suppression was considered as a peak serum cortisol <495 nmol x L(-1). The current authors calculated height standard deviation score (HSDS) at the time of testing and height velocity SDS (HVSDS) in the preceding year. Mean HSDS was 0.06+/-1.3 and HVSDS was -0.9+/-2.3. Adrenal suppression was disclosed in 15 asthmatic children (20.8%). There were no differences in HSDS and HVSDS between children with and without adrenal suppression. There was no correlation between peak cortisol response and dose or duration of treatment. However, a positive relationship between HVSDS and duration of treatment was noted. These data suggest that long-term treatment of asthmatic children with low and moderate doses of inhaled budesonide may result in mild adrenal suppression that cannot be predicted by growth deceleration. The negative influence of inhaled corticosteroids on growth becomes less the longer the duration of treatment.

Invasive meningococcal disease presenting as Henoch-Schonlein purpura.

Henoch-Schonlein purpura (HSP) is an acute systemic form of vasculitis that has been associated with a number of viral and bacterial infections. Described here are the cases of two children with invasive meningococcal disease who presented with clinical and laboratory findings typical of HSP. Meningococcal infection may have been the trigger for the manifestation of HSP in these patients.