Down syndrome patients with pulmonary hypertension have elevated plasma levels of asymmetric dimethylarginine

Down syndrome (DS) patients have an increased risk of developing pulmonary hypertension (PH). Increased plasma levels of asymmetric dimethylarginine (ADMA) may contribute to vascular dysfunction in adults with idiopathic pulmonary hypertension. Our goal was to test the hypothesis that DS patients with PH have higher plasma levels of ADMA than DS patients without PH. DS patients with definitive PH (n = 6) and DS patients with no evidence of PH (n = 12) were studied. Plasma levels of arginine, ADMA, and nitrite/nitrate (NOx; stable metabolites of nitric oxide (NO)) were measured. Plasma arginine concentration was lower (p < 0.05) in PH patients (23 ± 11 μM) versus non-PH patients (46 ± 24 μM). Plasma ADMA concentration was higher (p < 0.005) in PH patients (18.0 ± 4.2 μM) versus non-PH patients (8.6 ± 5.9 μM). Plasma NOx was lower (p < 0.05) in PH patients (4.5 ± 1.7 μM) versus non-PH patients (8.5 ± 7.3 μM). These results are consistent with ADMA contributing to lower NO production in DS patients with PH and suggest that ADMA levels may be a potential biomarker for PH in DS patients.     

The number and function of circulating endothelial progenitor cells in patients with Kawasaki disease

Kawasaki disease (KD) is associated with coronary artery injury. Studies have shown that the endothelial progenitor cell (EPC) participates in the process of arterial repair. Data have been reported that the number of EPC increased significantly in the subacute phase of KD. However, until now, there are no data about the functions of EPC in KD patients. The present study was designed to further investigate the number and functions of EPC in KD. Ten KD patients in the acute phase and ten healthy volunteers were recruited and attributed to the KD group and control group, respectively. The circulating CD34/kinase insert domain-containing receptor double positive cells were evaluated in the two groups using flow cytometry. In vitro assays were used to measure the functions of EPC, including proliferation, adhesion, and migration activities. The plasma levels of nitric oxide (NO), tumor necrosis factor-α (TNF-α), and high sensitivity C-reactive protein (hs-CRP) were also assessed in both groups. The number of EPC in the KD group was significantly higher than that of the control group (0.021 ± 0.007% vs. 0.014 ± 0.003%, P < 0.05). The migratory response of EPC was significantly decreased in the KD group, compared with that of the control group (5.50 ± 1.78 vs. 3.40 ± 1.35 cells/high power field, P < 0.01). Similarly, the proliferative and adhesive activities of EPC in the KD group were also decreased (0.47 ± 0.08 vs. 0.66 ± 0.07, P < 0.01; 6.5 ± 2.12 vs. 11.2 ± 2.04 cells/high power field, P < 0.01). The plasma NO, TNF-α, and hs-CRP levels in the KD group were higher than those of the control group (54.10 ± 11.78 vs. 38.80 ± 11.10 μmol/l, P < 0.01; 48.20 ± 7.42 vs. 37.00 ± 11.12 pg/ml, P < 0.05; 87.10 ± 30.18 vs. 5.30 ± 3.37 mg/l, P < 0.01). The number of circulating EPC positively correlated with the level of NO (r = 0.92, P < 0.001), and the functions of EPC negatively correlated with the levels of TNF-α and hs-CRP, respectively. In Kawasaki disease, the number of EPC was enhanced and the functions of EPC were attenuated. The two-way regulation of circulating EPC in KD patients may be associated with the disorders of cytokines or messengers in KD patients.     

Airway Pressure Release Ventilation: An Alternative Ventilation Mode for Pediatric Acute Hypoxemic Respiratory Failure

Airway pressure release ventilation (APRV) is a relatively new mode of mechanical ventilation. The use of this model of ventilation in pediatrics has been limited. The authors describe their experience with this mode of ventilation in a series of pediatric hypoxemic respiratory failure patients. Three patients with acute hypoxemic respiratory failure (AHRF) were treated with APRV, when oxygenation did not improve with pressure control ventilation (PCV). The mean age of the patients was 5.8 ± 1.3 months. Fractional oxygen concentration decreased from 0.97 ± 0.02 for PCV to 0.68 ± 0.12 for APRV, peak airway pressure fell from 36.6 ± 11.5 cm H₂O for PCV to 33.3 ± 5.7 cm H₂O for APRV, mean airway pressure increased from 17.9 ± 5.9 cmH₂O for PCV to 27 ± 2.6 cmH₂O for APRV and release tidal volume increased from 8.3 ± 1.5 mL/kg for PCV to 13.2 ± 1.1 mL/kg for APRV after 1 h. APRV may improve oxygenation in pediatric AHRF when conventional mechanical ventilation fails. The APRV modality may provide better oxygenation with lower peak airway pressure.     

Clinical characteristics of acute mitral regurgitation due to ruptured chordae tendineae in infancy—experience at a single institution

In infants, acute mitral regurgitation resulting from ruptured chordae tendineae is very rare, but often fatal. There are a few case reports, but the characteristics and etiology of chordae tendineae rupture have not been elucidated. Our aim was to determine the clinical characteristics of idiopathic acute mitral regurgitation due to chordal rupture in infancy. A retrospective analysis was performed on ten consecutive patients, with a mean onset age of 4.6 ± 1.3 months. Despite nonspecific initial symptoms, all patients developed respiratory distress and four required resuscitation within a few days (mean, 1.8 ± 1.8 days). Chest radiographs showed pulmonary congestion with a normal or mildly increased cardiothoracic ratio in all ten patients. Laboratory data and electrocardiograms showed nonspecific findings. Echocardiography revealed ruptured chordae in all patients; locations were anterior (50%), posterior (20%), and both (30%). Surgical intervention was performed within 24 h of admission in eight patients (mean, 3.6 ± 5.1 h). Pathological findings included inflammatory cells in six specimens and myxomatous degeneration in two. No bacteria were isolated from preoperative blood cultures, pathological tissues, or excised tissue cultures. Autoantibody levels were insignificant. Three preoperatively resuscitated patients developed neurological sequelae and arrhythmias occurred in four after mitral valve replacement. Acute onset and rapid deterioration in patients with ruptured chordae tendineae necessitates early surgical intervention to improve outcomes. Though the etiology remains unknown, onset is in infants approximately 4 months of age, suggesting a definite disease entity.     

Spectrum of Gastritis in Celiac Disease in Childhood

Celiac disease (CD) may cause changes throughout the gastrointestinal tract. The pathology is best described in the distal duodenum and jejunum. It is also associated with lymphocytic gastritis (LG) and varioliform gastritis in adults and children, but the histologic spectrum in the gastric biopsy and the clinical implications are undefined. In this report we relate our experience with the clinical, endoscopic, and histologic changes in gastric biopsies in CD in childhood. Slides (hematoxylin and eosin stained) were reviewed from 33 celiac children, 5 having had more than 1 gastric biopsy during a 7-year period. Gastric intraepithelial lymphocyte (IEL) counts were compared with those of 10 histologically normal controls (normal range, 1–7 IEL/100 antral or body epithelial cells) and 10 nonceliac chronic gastritis (CG) biopsies without H. pylori (normal range, 1–19 IEL/100 antral cells), noting changes in the epithelium and lamina propria (LP). LG was present in 29/33 initial biopsy sets. Fifteen of 29 showed LG/CG. The IEL number was greater in LG/CG than in LG only (27.2 ± 9.3, n = 14 vs. 18.6 ± 13.4, n = 15 in the antrum; 23.5 ± 2.8, n = 4 vs. 13.0 ± 8.4 in the body). In CD the difference between these mean values and those of normal and nonceliac CG controls was statistically significant. In CG/LG the inflammatory infiltrate was predominantly diffuse/superficial in the LP; mucin depletion was noted in 11/15. The IELs were in the LG/CG range in two CG controls. The IELs were normal at follow-up in five cases. There were no statistically significant differences between the groups with respect to clinical parameters or gastric endoscopic findings. No child had varioliform gastritis. We conclude that in CD children, the stomach is endoscopically unremarkable but may show LG, or LG/CG with or without mucin depletion, or occasionally appear normal. Gastric histology returned to normal with gluten withdrawal. Normal gastric histology is not typical, but does not exclude CD. Received January 20, 1998; accepted January 14, 1999.     

Virtual cardiotomy based on 3-D MRI for preoperative planning in congenital heart disease

Background  Patient-specific preoperative planning in complex congenital heart disease may be greatly facilitated by virtual cardiotomy. Surgeons can perform an unlimited number of surgical incisions on a virtual 3-D reconstruction to evaluate the feasibility of different surgical strategies. Objective  To quantitatively evaluate the quality of the underlying imaging data and the accuracy of the corresponding segmentation, and to qualitatively evaluate the feasibility of virtual cardiotomy. Materials and methods  A whole-heart MRI sequence was applied in 42 children with congenital heart disease (age 3 ± 3 years, weight 13 ± 9 kg, heart rate 96 ± 21 bpm). Image quality was graded 1–4 (diagnostic image quality ≥2) by two independent blinded observers. In patients with diagnostic image quality the segmentation quality was also graded 1–4 (4 no discrepancies, 1 misleading error). Results  The average image quality score was 2.7 – sufficient for virtual reconstruction in 35 of 38 patients (92%) older than 1 month. Segmentation time was 59 ± 10 min (average quality score 3.5). Virtual cardiotomy was performed in 19 patients. Conclusion  Accurate virtual reconstructions of patient-specific cardiac anatomy can be produced in less than 1 h from 3-D MRI. The presented work thus introduces a new, clinically feasible noninvasive technique for improved preoperative planning in complex cases of congenital heart disease.     

Clinical significance of plasma endothelin levels in patients with biliary atresia

Biliary atresia (BA) is the end-result of a destructive inflammatory process that affects intra- and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tracts with the development of biliary cirrhosis and portal hypertension (PH). Endothelins (ET) are 21-amino-acid peptides of endothelial origin with potent vasoconstrictor activity that bind to various cells of the liver. Nothing is presently known about plasma ET levels in BA. The aim of this study was to determine the clinical significance of plasma ET levels in patients with BA after hepatic portoenterostomy (Kasai's procedure) and to correlate these with liver function tests (LFT) and PH. We measured plasma concentrations of ET in 19 patients with BA (5 boys and 14 girls; mean age 11.6 ± 5.5 years) after portoenterostomy and 10 age-matched controls. Patients were grouped according to outcome based on LFT: group A consisted of 9 patients with an ‘‘unfavorable outcome” and Group B 10 patients with a “favorable outcome”. The plasma ET levels were measured using a highly sensitive and specific enzyme immunometeric assay (EIA). No patient had ascites or hepatorenal syndrome. Plasma ET levels were significantly higher in patients with BA than in controls (3.42 ± 0.42 vs 1.75 ± 0.39 pg/ml, respectively; P < 0.01) and in patients in group A than in group B. (3.75 ± 0.25 vs 3.06 ± 0.23 pg/ml, respectively; P < 0.01). In group A, plasma ET levels were higher in patients with PH (n = 4) than in those without PH (n = 5) (3.99 ± 0.06 vs 3.64 ± 0.22 pg/ml, respectively; P < 0.05). We conclude that plasma ET levels are high in patients with BA, especially those with severe biliary cirrhosis, and that ET may partially contribute to development of PH in BA. The results of the present study also suggest that plasma ET concentrations may be a useful marker in the follow-up of patients with BA. Accepted: 12 September 1997     

Contribution of the blood glucose level in perinatal asphyxia

This is a comparative study between 60 asphyxiated newborns (cases) and 60 normal neonates (controls) in respect of their plasma glucose and uric acid levels and also their clinical and neurological status. The mean plasma glucose level was significantly lower (35.1 ± 11.4 mg/dl vs. 56.9 ± 5.5 mg/dl; P < 0.001) and the mean serum uric acid level was higher (8.0 ± 1.2 mg/dl vs. 4.5 ± 0.83 mg/dl; P < 0.001) in the asphyxiated group when compared to the controls. Within the perinatal asphyxia group, the plasma glucose level and Apgar scores showed a significant positive linear correlation (r = 0.740, P < 0.001), whereas a significant negative linear correlation was observed between the glucose level and different stages of hypoxic ischemic encephalopathy (HIE) (r = −0.875, P < 0.001). Although a strong positive linear correlation was found between uric acid and HIE stages (r = 0.734, P ≤ 0.001), the linear correlation between uric acid and Apgar scores (r = −0.885, P < 0.001) and uric acid and the plasma glucose level (r = −0.725, P < 0.001) were found to be significantly negative among the cases. Conclusion: The severity of encephalopathy and cellular damage varies with the severity of hypoglycemia.     

Final height, gonadal function and bone mineral density of adolescent males with central precocious puberty after therapy with gonadotropin-releasing hormone analogues

Few data are available on the outcome of boys with central precocious puberty (CPP) treated with gonadotropin-releasing hormone (GnRH) analogues. We report on final height, endocrine and exocrine testicular function, and bone mineral density (BMD) in nine males (age 16.7 ± 1.5 years) treated with GnRH analogues from the age 6.0 ± 1.8 years for a mean period of 5.6 ± 2.4 years. The following parameters were evaluated: final height, serum gonadotropin and gonadal steroid levels, spermarche, semen analysis, area and volumetric BMD. Final height (−0.4 ± 1.1 SDS) was significantly higher than pre-treatment predicted adult height (−2.0 ± 1.2 SDS) and not significantly different than midparental height (−0.1 ± 0.8 SDS). Pubertal response of gonadotropins to GnRH test occurred within 1.5 years (mean 0.7 ± 0.4 years) and spermarche (n=7) from 0.7 to 3 years (1.8 ± 0.9 years) after the discontinuation of GnRH analogue therapy. No alteration in semen analysis was found (n=6, sperm count, 10⁶/ml: 52.0 ± 18.7; normal motility (%): 49.5 ± 18.7; atypical morphology (%): 44.5 ± 11.4). Area and volumetric BMD were not reduced (0.2 ± 1.0 SDS and −0.1 ± 0.9 SDS, respectively). Conclusion Long-term treatment with gonadotropin-releasing hormone analogues improves final height in boys with central precocious puberty. Post-therapy data demonstrating normal endocrine and exocrine testicular function support the safety of gonadotropin-releasing hormone analogues on reproductive function. Long-term pharmacological suppression of testicular function in childhood does not impair bone mineral density in late adolescence. Received: 4 May 1999 / Accepted: 30 November 1999     

The effect of colchicine on physical growth in children wıth familial mediterranean fever

Familial Mediterranean fever (FMF) is an autosomal recessive disease, characterized by recurrent, self-limited attacks of fever with serositis involving the peritoneum, pleura, and joints. There is very scarce information on physical growth of affected children. The aim of this study was to determine whether there is significant improvement in growth parameters in FMF patients after colchicine treatment. Patient files were retrospectively evaluated and patients that used colchicine for more than 1 year were included in the study. Demographic features, clinical findings before and after colchicine therapy, duration and dosage of therapy, weight, height, parentally adjusted height, and body mass index before and after colchicine therapy were noted and transformed into standard deviation scores (SDS). The study group consisted of 50 FMF (25 male and 25 female) patients. Median age at the time of diagnosis was 6.5 years. Median follow-up period was 3.6 (1–12.5) years. Mean height SDS increased from −0.19 ± 1.01 to 0.13 ± 0.99 (p = 0.026), and mean parentally adjusted height increased from −0.18 ± 1.23 to 0.13 ± 1.24 (p = 0.027), and both of them were found to be statistically significant. Mean body mass index SDS increased from −0.61 ± 1.32 to −0.32 ± 1.33, but this improvement was statistically insignificant (p = 0.18). In this study, we found that colchicine significantly improved height development in FMF patients.     

Clinical features and prognoses of 23 patients with chronic granulomatous disease followed for 21 years by a single hospital in Japan

In this paper, we examined the details of severe infections, treatment efficacies, and the prognoses of 23 Japanese patients with chronic granulomatous disease (CGD). We described the mean ages at diagnosis and follow-up, which were 2.8 years (range, 0.7–10 years) and 14.9 years (range, 0.2–28.4 years), respectively. There were three deaths, two from Aspergillus pneumonia and one from liver abscess. Eighteen of the 23 patients (78%) had a complete loss of gp91phox, and three had p22-phox and one had p67phox deficiencies. Aspergillus species were found in 45% of 174 severe infections. The mean height and weight of the 20 surviving patients were −0.8 ± 1.3SD and −1.9 ± 1.9SD below the means for age, respectively. Short stature and underweight (below the 10th percentile of the means) for age were seen in 22% and 17% of the patients, respectively. This growth retardation reflects the severity of the disease. At 20 years of age, there was 87% survival. Ongoing prophylaxis with trimethoprim-sulfamethoxazole (TMP-SMX) or antifungal drugs was given in 16 and 11 patients, respectively. Interferon-gamma (IFN-gamma) was given once a week to 14 patients. Four patients underwent hematopoietic stem cell transplantation (HSCT) and are currently well. There were infections observed in three of 21 identified related carriers of X-linked CGD. A carrier with a liver abscess had 5% normal neutrophils during the acute phase of infection, which returned to 40% normal neutrophils after recovery. The high survival rate in this hospital results from regular follow-up and prophylaxis with TMP-SMX and anti-fungal drugs beginning at the time of diagnosis, along with treatment with weekly IFN-gamma.     

Age effect on whole blood cyclosporine concentrations following oral administration in children with nephrotic syndrome

The aim of this study was to investigate age-related pharmacokinetic differences of cyclosporine (CyA) in children with nephrotic syndrome. Whole blood concentrations of CyA were monitored for a total of 96 times in 36 cases. The 25 male and 11 female patients ranged in age from 1.9 to 19.7 years with a mean age of 9.1 years. Renal biopsy showed minimal change in 33 patients and focal segmental glomerulosclerosis in three patients. CyA was orally administered in two divided doses just before meals. The doses of CyA administered were adjusted such that the target value for blood concentration at 2 h post-dose (C2) was 400–450 ng/ml. The 96 subjects were divided into three groups according to age: group I, 1–5 years (n = 30); group II, 6–10 years (n = 34); and group III, ≥ 11 years (n = 32). In all subjects, peak levels (Cmax) of CyA were reached at C1 or C2. There was no significant difference between the groups for C2, area under the whole blood concentration–time curve up to 4 h post-dose (AUC0–4), and Cmax. The mean CyA doses of groups I, II, and III were 4.8 ± 1.0 mg/kg/day, 3.8 ± 0.9 mg/kg/day, and 3.0 ± 0.6 mg/kg/day, respectively, and there were significant differences between every two groups. In addition, the dose-normalized Cmax (Cmax/dose) and AUC0–4 (AUC0–4/dose) values were significantly lower in the younger group than in the older group. These findings suggested that in children, when the same concentration is targeted, the required CyA dose would vary according to age but would be significantly higher for the younger children.     

Precision of continuous neonatal ventilator respiratory mechanics is improved with selected optimal respiratory cycles

Given their high apparent variability, bedside continuous respiratory mechanics (RM) parameters [excepting tidal volume (V _T)] remain infrequently used for adjustment of neonatal ventilatory settings. RM parameters provided by ventilator (VRC) from ten recordings of newborns [10 min in synchronised intermittent mandatory ventilation and 10 min in assist/control (A/C)] were compared to those computed from visually selected assisted leak-free optimal respiratory cycles (SRC). Mean values, variability and ability to distinguish patients were compared between VRC and SRC. Dynamic resistances were more correlated (r ² = 0.95) than compliances (r ² = 0.42). V _Ts were correlated only in A/C (r ² = 0.78). C20/C was significantly higher in VRC (1.81 ± 0.67) than in SRC (1.23 ± 0.36) and frequently out of neonatal reference range. In A/C ventilation, V _T was higher in VRC (5.6 ± 1.8 ml/kg) than in SRC (4.8 ± 1.0 ml/kg) (p < 0.05). Displayed V _Ts do not reflect those found in optimal assisted breaths and therefore have incomplete value in assessing adequacy of ventilator settings. The variability of RM parameters provided by the ventilator is large, and coefficients of variation were significantly lower with optimal respiratory cycles (for resistance, compliance, V _T and C20/C; 27%, 26%, 18%, 24% in SRC and 36%, 35%, 40% and 33% in VRC). Selecting optimal cycles yields RM with two to three times higher discriminating power between patients. Conclusion: Current ventilator’s RM parameters have limited clinical use. Using optimal breaths to calculate RM parameters improves precision and discriminating power. For integration to ventilatory care, automation of this selection must be implemented first.     

Intrapleural streptokinase treatment in children with empyema

Our aim was to compare intrapleural streptokinase (SK) treatment and simple tube drainage in the treatment of children with complicated parapneumonic pleural effusion. A retrospective review of medical records included patient demographics, clinical presentation, biochemical and microbial studies of pleural effusion, radiographic evaluation of chest tube drainage, use of fibrinolytic agents and type of surgical intervention. During the 2.5-year period (1999–2002), 53 children (29 M, 24 F) with complicated parapneumonic effusions or empyema were identified. Closed tube drainage and antibiotic treatment were administered to patients with a diagnosis of complicated parapneumonic effusion (n = 24) until October 2000; after that time point, intrapleural streptokinase was added to this regimen (n = 29). The median age at the time of presentation was 2.5 years (range: 5 months-14.6 years). There were no significant differences in terms of clinical outcomes between the two groups. The average length of hospital stay was 19.1 ± 5.5 and 21.9 ± 11.2 days for the drainage and streptokinase groups, respectively; the time to afebrile state after admission was 5.8 ± 4.1 and 7.6 ± 7.5 days. The percentage of patients who eventually required surgical intervention was 8.3% for the drainage group and 20.6% for the streptokinase group. In conclusion, in the treatment of complicated parapneumonic effusions or empyema, the adjunctive treatment with intrapleural SK does not significantly reduce durations of fever, chest tube drainage and hospital stay, and the need for surgery, regardless of the stage of the disease, compared to simple closed tube drainage.     

Early intravenous immunoglobin (two-dose regimen) in the management of severe Rh hemolytic disease of newborn—a prospective randomized controlled trial

Phototherapy is the standard treatment in moderately severe hemolytic disease of newborn (HDN), whereas exchange transfusion (ET) is the second line in progressive cases. Intravenous immunoglobin (IVIG) has been suggested to decrease the need for ET. We aimed at assessing the efficacy of early two-dose regimens of IVIG to avoid unnecessary ET in severe Rh HDN. The study included 90 full-term neonates with Rh incompatibility unmodified by antenatal treatment and not eligible for early ET and which were randomly assigned into one of three groups: group (I), treated by conventional method; groups IIa and IIb received IVIG once at 12 h postnatal age if PT was indicated, in a dose of 0.5 and 1 g/kg, respectively. Analysis revealed 11 neonates (22%) in the conventional group and 2 (5%) in the intervention group who administered low-dose IVIG at 12 h, while none in group IIb required exchange transfusion (p = 0.03). Mean bilirubin levels were significantly lower during the first 96 h in the intervention group compared to the conventional group (p < 0.0001). Shorter duration of phototherapy (52.8 ± 12.39 h) and hospital stay (3.25 ± 0.71 days) in the IVIG group compared to conventional group (84 ± 12.12 h and 4.72 ±0.78 days, p < 0.0001, respectively) were observed. We conclude that IVIG administration at 12 h was effective in the treatment of severe Rh HDN; the low-dose IVIG (0.5 g/kg) was as effective as high dose (1 g/kg) in reducing the duration of phototherapy and hospital stay, but less effective in avoiding exchange transfusion.     

Growth hormone secretion, puberty and adult height after cranial irradiation with 18 Gy for leukaemia

The dose of prophylactic cranial irradiation given to patients for acute lymphoblastic leukaemia has been decreased from 24 to 18 Gy, but the beneficial effect of this decrease on growth is controversial. This study compares the growth hormone (GH) secretion and growth of 35 patients (20 boys) given 18 Gy at 3.7 ± 0.3 (SE) years, and routinely evaluated 5.4 ± 0.4 years after irradiation to define the indications for GH treatment in these patients. Of these, 63% had a low GH peak (<10 μg/l) after one (22 cases) or two (17 cases) stimulation tests. The plasma concentrations of insulin-like growth factor I and its GH-dependent binding protein were normal for age in all but two cases. The height changes between irradiation and evaluation were correlated with the GH peaks (P < 0.03) and were concordant, except in patients with early puberty. This occurred in 16 patients including all 12 girls irradiated before 4 years of age. A significant (P < 0.03) reduction in height (SD) between irradiation and adult height occurred in untreated GH-deficient patients (−1 ± 0.3, n = 6), but not in GH-deficient patients given GH (−0.6 ± 0.3, n = 8) or in those with normal GH peak (−0.4 ± 0.3, n = 7). Conclusion In children irradiated for acute lymphoblastic leukaemia, GH deficiency is frequent after 18 Gy but its impact on adult height is smaller than after higher doses. We suggest that the indications for gonadotropin releasing hormone analogue therapy should be broad in patients with early or rapidly progressing puberty and those for GH therapy in those patients with a below average constitutional height before irradiation. Received: 17 November 1997 / Accepted: 9 February 1998     

Prevalence of vitamin D deficiency rickets in the eastern part of Turkey

Turkey, especially its eastern part, has been accepted as endemic for vitamin D deficiency rickets (VDDR). In a study performed by our team in the region in 1998, the incidence of VDDR was 6.09% in children aged between 0–3 years. In 2005, the Ministry of Health initiated a free vitamin D supplementation campaign nationwide for every infant to eradicate VDDR. In this study, we aimed to investigate the prevalence of VDDR in children aged between 0–3 years in order to evaluate the effectiveness of this campaign. Between March 2007 and February 2008, 39,133 children aged between 0–3 years who were brought to different pediatric outpatient clinics in Erzurum, Turkey, were examined for VDDR. VDDR diagnosis was made by radiological and biochemical findings in the cases who were initially suspected of having clinical VDDR. During a one-year period, 39 (0.099%) of the 39,133 patients were diagnosed with VDDR. None of the cases with rickets was taking vitamin D supplementation. The most frequent physical findings were rachitic rosary, enlargement of the wrists, and craniotabes. The laboratory findings of the cases were compatible with VDDR; serum calcium (Ca) 7.5 ± 1.9 mg/dL, PO₄ 4.4 ± 1.3 mg/dL, alkaline phosphatase (ALP) 1,341 ± 823, 25-hydroxyvitamin D (25 (OH) D) 5.8 ± 2.9 ng/mL, intact parathyroid hormone (iPTH) 240 ± 106 pg/mL. It was concluded that, although VDDR has been a continuing childhood health problem, a nationwide free vitamin D supplementation campaign initiated by the government appeared to be effective in eliminating VDDR. Behzat Ozkan and Hakan Doneray should be regarded as the first authors. An erratum to this article can be found at     

Reduced physical activity level and cardiorespiratory fitness in children with chronic diseases

We aimed to compare physical activity level and cardiorespiratory fitness in children with different chronic diseases, such as type 1 diabetes mellitus (T1DM), obesity (OB) and juvenile idiopathic arthritis (JIA), with healthy controls (HC). We performed a cross-sectional study including 209 children: OB: n = 45, T1DM: n = 48, JIA: n = 31, and HC: n = 85. Physical activity level was assessed by accelerometer and cardiorespiratory fitness by a treadmill test. ANOVA, linear regressions and Pearson correlations were used. Children with chronic diseases had reduced total daily physical activity counts (T1DM 497 ± 54 cpm, p = 0.003; JIA 518 ± 28, p < 0.001, OB 590 ± 25, p = 0.003) and cardiorespiratory fitness (JIA 39.3 ± 1.7, p = 0.001, OB 41.7 ± 1.2, p = 0.020) compared to HC (668 ± 35 cpm; 45.3 ± 0.9 ml kg⁻¹ min⁻¹, respectively). Only 60.4% of HC, 51.6% of OB, 38.1% of JIA and 38.5% of T1DM children met the recommended daily 60 min of moderate-to-vigorous physical activity. Low cardiorespiratory fitness was associated with female gender and low daily PA. Conclusion: Children with chronic diseases had reduced physical activity and cardiorespiratory fitness. As the benefits of PA on health have been well demonstrated during growth, it should be encouraged in those children to prevent a reduction of cardiorespiratory fitness and the development of comorbidities.