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.

Association of extracurricular sports participation with obesity in Greek children.

AIM: Childhood obesity has become a modern epidemic with escalating rates. The aim of our study was the assessment of the association between extracurricular sports participation with the obesity status among Greek schoolchildren aged 10-12 years. METHODS: Seven-hundred children (323 boys) aged 10-12 years were evaluated through a standardized questionnaire. Several lifestyle, dietary and physical activity attributes were recorded. Children according to the median hours of participation in after-school structured physical activity were classified as participating for more or less than 3 hours per week. Body height and mass were measured and body mass index was calculated in order to classify children as overweight or obese according to IOTF classification. Multiple logistic regression analysis was used in order to evaluate the association between the participation of more than 3 hours per week in structured physical activity after school with overweight or obesity. RESULTS: A total of 48.9% of the boys and 31.8% of the girls were participating for more than 3 hours per week (P<0.001) in extracurricular sport activities. Moreover, 33.9 % of the boys and 22.1 % of the girls were classified as overweight and 9.4% of the boys and 8.6% of the girls as obese (P=0.006). Girls who participated in excess of 3 hours in extracurricular sport activities were 59% less likely to be overweight or obese than their non participating counterparts, adjusted for several confounders (adjusted OR: 0.41, 95% CI: 0.20-0.83). CONCLUSION: Participation in extracurricular sports activity is inversely related to overweight or obesity in 10-12 years old Greek girls.

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.

Dysregulation of the stress response in asthmatic children.

The stress system co-ordinates the adaptive responses of the organism to stressors of any kind. Inappropriate responsiveness may account for increased susceptibility to a variety of disorders, including asthma. Accumulated evidence from animal models suggests that exogenously applied stress enhances airway reactivity and increases allergen-induced airway inflammation. This is in agreement with the clinical observation that stressful life events increase the risk of a new asthma attack. Activation of the hypothalamic-pituitary-adrenal (HPA) axis by specific cytokines increases the release of cortisol, which in turn feeds back and suppresses the immune reaction. Data from animal models suggest that inability to increase glucocorticoid production in response to stress is associated with increased airway inflammation with mechanical dysfunction of the lungs. Recently, a growing body of evidence shows that asthmatic subjects who are not treated with inhaled corticosteroids (ICS) are likely to have an attenuated activity and/or responsiveness of their HPA axis. In line with this concept, most asthmatic children demonstrate improved HPA axis responsiveness on conventional doses of ICS, as their airway inflammation subsides. Few patients may experience further deterioration of adrenal function, a phenomenon which may be genetically determined.

Secular trend in body height of schoolchildren in Northeast Attica, Greece.

AIM: To examine the secular trend for body height in schoolchildren in Northeast Attica, Greece. METHODS: In 2005 we studied the growth of 4,131 Greek schoolchildren (2,054 boys) aged 5.5-12.6 years. The data were compared with those of a study performed in 1994/95. We also studied the growth of 236 immigrant schoolchildren (95 boys and 141 girls). RESULTS: For boys in the present study mean height (SD) was 135.6 (11.0) vs 133.8 (10.4) cm in the 1994/95 study, p < 0.001, and for girls of the present study mean height was 135.2 (11.7) vs 133.3 (10.8) cm in 1994/95, p < 0.001. The increase in height was present at almost all ages. There was a significant difference in height between Greek and immigrant boys, 135.6 (11.0) cm and 133.0 (10.2) cm, respectively, p < 0.05. However, there was no significant difference in height between Greek and immigrant girls, 135.2 (11.7) and 133.8 (12.4) cm, respectively. CONCLUSION: A secular trend in height is still taking place in Greek schoolchildren that is mostly attributed to an increase in height from early life.

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.

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.

Hereditary vitamin D-resistant rickets in Greek children: genotype, phenotype, and long-term response to treatment.

We describe four patients (two pairs of children from two unrelated kindreds) from a Greek island, suffering from hereditary vitamin D-resistant rickets (HVDRR) with alopecia. There were two different homozygous mutations in the vitamin D receptor (VDR) gene of the affected members of the two kindreds that resulted in a truncated or missing receptor. The disorder began in early infancy with similar clinical, biochemical and radiological findings in all four patients, namely, alopecia (which provided the initial diagnostic evidence for HVDRR), rachitic deformities, hypocalcemia, hypophosphatemia, secondary hyperparathyroidism, and elevated serum levels of 1,25-dihydroxyvitamin D; however, the patients of kindred B had a more severe clinical expression. Treatment options include oral or intravenous calcium and active vitamin D metabolites. The response varies widely in different cases. Our patients were initially treated with high doses of 1alpha(OH)D3 and oral calcium supplementation. Kindred A patients had a satisfactory response to this regimen, while kindred B patients presented clinical and biochemical improvement when 1alpha(OH)D3 was changed to 1,25(OH)2D3. In the older patients of each kindred, treatment requirements gradually decreased during puberty, and therapy was finally discontinued before adulthood.

Discrepancy between neuroimaging findings and clinical phenotype in Alexander disease.

We present a case of infantile-onset Alexander disease (AD) with a novel glial fibrillary acidic protein mutation but without clinical evidence of neurologic deterioration. Brain MRI studies showed typical AD findings and increasing size of frontal cavitations. Serial proton MR spectroscopy demonstrated high levels of myo-inositol and lactic acid and decreasing levels of N-acetylaspartate. The degree of demyelination and the timing of the axonal degeneration may determine phenotypic severity of the disease. Conventional neuroimaging techniques cannot always predict the outcome.

Estimates of dose to the bladder during direct radionuclide cystography: concise communication.

We estimate the absorbed dose to the bladder walls, Db, due to [99mTc]pertechnetate direct radionuclide cystography, with due consideration to the dynamic nature of the examination. Our analytical method resulted in Db values expressed as functions of the bladder filling time. These values are compared with published data based on a static bladder model. It is shown that the Db value depends strongly on the protocol under which the examination is carried out. It is also shown that the normal saline flow rate and the time of the administration of the radioactive material can be adjusted so that the radiation burden may be greatly reduced.

Hydatid disease in Greek children.

The surgical incidence of hydatidosis in the Greek population aged 0-18 years was investigated. Discharge and operating room records from 1 January 1969 through 31 December 1975 in all hospitals of the country were reviewed. Data were statistically analyzed according to geographical and annual distribution of the disease, age, sex, and organ affected. Of a total of 4,566 patients with hydatidosis, 534 (11.7%) were 0-18 years old. By extrapolation, operations in this age group were performed at a rate of 2.7/100,000 population per year. Surgical incidence of hydatidosis varies among different geographical areas in Greece; its percentage is highest in rural areas. The disease is more frequent in boys than in girls (P less than 0.01). Lungs and liver are the most commonly involved organs (94.2%). Lung involvement is more frequent in boys (73.0%) than in girls (50.5%) (P less than 0.001).

Statistical aspects of hydatid disease in Greek adults.

A retrospective study was undertaken to estimate the incidence of hydatidosis among Greeks over 18 years of age. Discharge and operating room records in all community and private hospitals during the period 1 January 1969 to 31 December 1975 were reviewed, and new hydatid patients were registered. Hydatid disease was diagnosed in 4,202 adults. The average annual incidence of all hospital cases was 9.77 per 100,000 population. The diagnosis was confirmed at surgery in 3,397 patients (80.84%). The average annual surgical incidence was 7.89 per 100,000 inhabitants. The incidence of the disease varied among different geographic areas in the country; its percentage was highest in rural areas. In this series, both sexes were equally involved (P greater than 0.1). Solitary organ involvement occurred in 95.88% of cases. Cysts were localized in the liver in 69.64% and in the lungs of 23.43% of the cases with single organ involvement. Liver involvement was more frequent in women, while lung involvement was more frequent in men (P less than 0.001).

Hydatid disease in asymptomatic young carriers in northern Greece.

An epidemiological survey to determine the prevalence of echinococcosis in children and adolescents was made in Grevena County of northern Greece. Counterimmunoelectrophoresis was used to screen 2,736 school children whose ages ranged from 6-18 years; among them, three asymptomatic carriers were identified. The diagnosis was subsequently confirmed by liver radioisotope scanning. By extrapolation, we estimate that the point prevalence of hydatid disease in the asymptomatic population of the study area is 110 per 100,000.

Idiopathic intracranial hypertension in children.

We studied prospectively the etiology, clinical presentation, and outcome of idiopathic intracranial hypertension in 36 patients (20 boys and 16 girls) aged 3.5 months to 14 years. The etiology was identified in 28 (77.7%) patients. The most common predisposing factor was middle-ear infection, followed by obesity. Of the 36 cases seen, 26 presented with the classic picture of headaches, papilledema, and elevated cerebrospinal fluid pressure; 8 children had intracranial hypertension in the absence of papilledema and 2 had fundoscopic evidence of papilledema with normal cerebrospinal fluid pressure initially. In four children resolution of intracranial hypertension occurred with removal of the causative agent or appropriate treatment of the underlying condition. In 8 of 17 patients intracranial hypertension resolved with acetazolamide therapy and in 22 of 24 patients it resolved with corticosteroids in combination with acetazolamide. Subnormal visual acuity, narrowing of visual fields, or both were present on the initial examination in 10 patients; 2 of them, who presented with loss of vision, have permanent visual impairment. Four additional patients presented a transient, mild impairment of visual acuity during treatment. Our study confirms the wide clinical spectrum and the difficulties encountered in diagnosis of idiopathic intracranial hypertension. A wide variety of etiologic associations should be investigated to provide definitive therapy. Loss of visual function is the only serious complication.

Calcium sensing receptor in pregnancies complicated by gestational diabetes mellitus.

INTRODUCTION: Infants born from mothers with Gestational diabetes mellitus (GDM) experience several complications, including a higher rate of postnatal hypocalcemia. In this study, we investigated the association between calcium sensing receptor (CaSR) and neonatal hypocalcemia observed in GDM pregnancies. METHODS: Our study consisted of 58 pregnant women with GDM and 40 healthy women and their neonates. CaSR placental expression was evaluated with immunohistochemistry and Western Blot. Three CaSR single nucleotide polymorphisms, A986S, R990G, Q1011E, were evaluated in neonate's genomic DNA. Serum Ca, P, Mg, 25(OH)D and PTH were measured in cord blood and at 2nd day of life. RESULTS: GDM neonates had lower mean cord blood Ca levels than controls (2.47 ± 0.21 mmol/l vs 2.59 ± 0.13 mmol/l, p = 0.001) while 15.5% developed postnatal hypocalcemia. CaSR expression was lower in GDM than in healthy placentas (p < 0.001). In the GDM group, reduced CaSR immunostaining in the syncytiotrophoblast (p = 0.042) and extravillous cytotrophoblasts (p = 0.002) was associated with lower Ca cord blood levels. Moreover, the absence of the S allele of the A986S polymorphism was associated with lower serum Ca levels both at birth (AA:2.41 ± 0.23 mmol/l, AS + SS: 2.57 ± 0.12 mmol/l, p = 0.002) and at 2nd day of life (AA:2.05 ± 0.22 mmol/l, AS + SS: 2.20 ± 0.18 mmol/l, p = 0.019). CONCLUSIONS: Our results showed that CaSR is under-expressed in GDM compared with healthy placentas and this alteration may be associated with the lower Ca levels measured in cord blood of GDM infants. Placental CaSR seems to exert a local effect in fetal Ca homeostasis, which is dissociated from its contribution to the regulation of Ca homeostasis in postnatal life.