Atrial septal aneurysms in childhood: prevalence, classification, and concurrent abnormalities.

OBJECTIVES: This study sought to investigate the prevalence of atrial septal aneurysms in the paediatric population and to define coexisting abnormalities and their incidence. BACKGROUND: Few papers refer to the prevalence of atrial septal aneurysms in childhood. METHODS: We enrolled a total of 4522 children aged more than 12 months who underwent a transthoracic echocardiography. Atrial septal aneurysm was defined as a protrusion of the interatrial septum or part of it >15 mm beyond the plane of the atrial septum or phasic excursion of the interatrial septum during the cardiorespiratory cycle of at least 15 mm in total amplitude and a diameter of the base of the aneurysm of at least 15 mm. RESULTS: Atrial septal aneurysms were found in 47 children (1.04%). They involved almost the entire septum in 14 patients (28.89%) and were limited to the fossa ovalis in 33 (71.11%). An atrial septal aneurysm was an isolated structural defect in 17 (35.56%). In 30 (64.44%) patients, it was associated with interatrial shunting - atrial septal defect and patent foramen ovale. At the echo follow-up after a year, no changes were recorded. CONCLUSIONS: Prevalence of atrial septal aneurysms is almost 1%. The most common abnormalities associated are interatrial shunts, that is, a patent foramen ovale and an atrial septal defect. From a medical point of view, it is suggested that no action is to be taken during childhood, as a child with an atrial septal aneurysm is not at increased risk compared with a child without one. Follow-up is scheduled on an individual basis.

In vitro fertilization and risk of childhood leukemia in Greece and Sweden.

BACKGROUND: Cancer risk in children born after in vitro fertilization (IVF) remains largely unknown. We aimed to investigate risk of leukemia and lymphoma following IVF using two nationwide datasets. METHODS: The hospital-based case-control study in Greece derived from the National Registry for Childhood Hematological Malignancies (1996-2008, 814 leukemia and 277 lymphoma incident cases with their 1:1 matched controls). The Swedish case-control study was nested in the Swedish Medical Birth Register (MBR) (1995-2007, 520 leukemia and 71 lymphoma cases with their 5,200 and 710 matched controls) with ascertainment of incident cancer cases in the National Cancer Register. Study-specific and combined odds ratios (OR) were estimated using conditional logistic regression, with adjustment for possible risk factors. RESULTS: Nationwide studies pointed to similar size excess risk of leukemia following IVF, but to a null association between IVF and lymphoma. The proportion of leukemia cases conceived through IVF was 3% in Greece and 2.7% in Sweden; prevalence of IVF in matched controls was 1.8% and 1.6%, respectively. In combined multivariable analyses, the increased risk of leukemia was confined to age below 3.8 years (OR = 2.21; 95% confidence interval, CI: 1.27-3.85) and to acute lymphoblastic leukemia (ALL) (OR = 1.77; 95% CI: 1.06-2.95) with no sufficient evidence of excess risk for other leukemias (OR = 1.34; 95% CI: 0.38-4.69). Following IVF, OR for ALL was 2.58 (95% CI: 1.37-4.84) before age 3.8 and 4.29 (95% CI: 1.49-12.37) before age 2 years. CONCLUSIONS: IVF seems to be associated with increased risk of early onset ALL in the offspring.

Neonatal haemostatic parameters in correlation to gestational age and birth weight.

INTRODUCTION: The aim of our study was to establish reference ranges for neonatal coagulation and fibrinolysis parameters and to investigate their relationship with gestational age (GA) and birth weight (BW). METHODS: A single-centre prospective study was conducted in all healthy neonates born in our hospital during the study period, excluding those with maternal or neonatal disorders and diseases that affect haemostasis. The following parameters were measured: fibrinogen, prothrombin time (PT), international normalized ratio (INR), activated partial thromboplastin time (aPTT) as well as factors II, V, VII, VIII, IX, X, XI and XII, von Willebrand (vWF), protein C, free protein S, antithrombin (AT), activated protein C resistance (APCr), tissue plasminogen activator (tPA) and plasminogen activator inhibitor-1 (PAI-1). RESULTS: Study population consisted of 327 neonates. Fibrinogen, AT III, proteins C and S, PAI-1, vWF and factors II, V, VIII, IX, XI and XII were positively correlated, while PT, aPPT, INR, APCr and tPA were negatively correlated with GA and BW. Proteins C and S, factors II, VIII, IX, XI and vWF, as well AT III and PAI-1 had a significant positive linear correlation with GA, while aPTT had a significant negative one. Fibrinogen, and factors V, VII and XII had a significant positive linear correlation with BW, while factor VIII, tPA, as well PT and INR had a significant negative one. CONCLUSION: Fibrinogen, AT III, proteins C and S, PAI-1, vWF and factors II, V, VIII, IX, XI and XII increase with GA and BW.

Candida hellenica var. hellenica as a possible cause of respiratory infection in a child with acute myeloid leukemia.

Candida hellenica var. hellenica (teleomorph Zygoascus meyerae) is a member of the genus Zygoascus that comprises species isolated from environmental sources such as damaged grapes. A case of a possible pneumonia due to this uncommon yeast in a pediatric oncology patient suffering from acute myeloid leukemia is described. To our knowledge, this is the first report concerning the isolation of the species from a pediatric patient and the second in humans.

Holoprosencephaly and diabetes insipidus in a 3-month-old infant.

Holoprosencephaly is a developmental defect caused by incomplete cleavage of the embryonic forebrain structures during early embryogenesis. We describe a 3-month-old boy with median cleft palate, surgically reconstructed cleft lip, hypotelorism with a flat nose, cryptorchidism, clubfoot, and microcephaly. During the laboratory investigation, his blood sodium level was 154 mmol/L and urine specific gravity was 1.007. Serum osmolarity was 317 mOsm/kg and urine osmolarity was 268 mOsm/kg. Given these findings and the clinical response to vasopressin, diagnosis of central diabetes insipidus was made. Magnetic resonance imaging revealed semilobar holoprosencephaly. The patient responded very well to vasopressin treatment with restoration of serum electrolytes, which remained within normal limits on follow-up. In case of midline facial defects accompanied by hypotelorism with or without developmental delay, the brain should be imaged to confirm its morphology and investigations should be directed by a high index of suspicion of associated endocrinologic dysfunctions.

Enterococcus faecalis: an unusual cause of meningitis in a child with non-Hodgkin lymphoma.

Enterococcal meningitis is an uncommon disease in children, most frequently reported in infants or in children with central nervous system pathology. We report a rare case of Enterococcus faecalis meningitis in an 11-year-old child with non-Hodgkin lymphoma. The patient during the course of chemotherapy became neutropenic, febrile, agitated, and disoriented with clinical signs of meningeal irritation. Culture of cerebrospinal fluid yielded Enterococcus faecalis. The patient was successfully treated with ampicillin without any neurological defects.

Increased expression of multidrug resistance gene (MDR1) at relapse in a child with acute lymphoblastic leukemia.

Modern treatment protocols lead to complete remission in a high proportion of patients with childhood acute lymphoblastic leukemia (ALL). However, a large number of them show a relapse of the disease. Treatment failure in these patients is mainly attributable to de novo or acquired resistance to a wide variety of cytotoxic drugs, which is called multi drug resistance (MDR). Expression of multi drug resistance 1 gene (MDR1) is implicated in the drug-resistance mechanism. In order to contribute further information we present a rare case of a 15-month old girl with newly diagnosed CALLA positive pre-B acute lymphoblastic leukemia with favourable prognostic factors at diagnosis who experienced a relapse of the disease. Using reverse transcriptase polymerase chain reaction method, m-RNA expression of the MDR1 gene upon relapse, was five-fold compared with that at diagnosis. This is the first report on increased mRNA expression at relapse in a paired sample of a child with ALL in our region.

Spinal epidural extraskeletal Ewing sarcoma in an adolescent boy: a case report.

Extraskeletal Ewing sarcoma (EES) represents a rare soft tissue malignant neoplasm histologically similar to skeletal Ewing sarcoma. It occurs mainly in adolescents and young adults and commonly affects the paravertebral regions. The differential diagnosis includes other small, blue round cells tumors. The authors report a case of an EES involving the spinal epidural and paravertebral spaces in an adolescent boy. EES diagnosis was confirmed by features of histologic analysis and immunohistochemistry and by the presence of the t(11;22)(q24;q12) chromosomal translocation by reverse transcriptase-polymerase chain reaction.

Bone mineral density in survivors of childhood acute lymphoblastic leukemia.

The aim of our study was to evaluate bone metabolism with measurement of bone mineral density (BMD) after management (chemo-, radiotherapy) for childhood acute lymphoblastic leukemia (ALL). Bone mineral density (g/cm2) of lumbar spine was measured by dual energy X-ray absorptiometry (Norland bone densitometer) in 18 children with ALL and a median of 34 months' post-diagnosis with no history of relapse, secondary malignancy, or transplantation. In addition, patients' BMDs were correlated with particular attention to age, sex and time (years) from completion of chemotherapy. The results were compared with healthy age- and sex-matched controls of the same population and expressed as standard deviation scores (SDS). Mean age of children was 9.8 +/- 3.7 years. Of 18 children (10 boys and 8 girls), 13 were grouped as standard and 5 as high-risk, respectively. Based on z-score values, 9 were classified as normal (z-score <1 SD), 7 as osteopenic (z-score 1-2.5 SD) and 2 as osteoporotic (z-score >2.5 SD). Children with ALL had reduced lumbar BMDs (z score -0.99) in comparison to healthy controls (z score -0.14) (p=0.011), which is indicative of relative osteopenia. Moreover, the reduced BMD was associated with patient age (z score -0.14 and -1.52 for ages <10 and >10 years, respectively, p=0.016). Reduced BMD was not correlated with time from completion of chemotherapy (p=0.33), risk group (p=0.9) and sex (p=0.3). We conclude that children's BMDs are reduced after completion of chemotherapy for ALL. The causes are multifactorial and mainly related to antineoplastic treatments, such as corticosteroids and methotrexate, physical inactivity and cranial irradiation. We suggest that further studies are needed to evaluate the long-term effect on BMD in these children and to prevent pathological fractures later in life.

Fungal brain abscesses in leukemia.

Invasive fungal infections remain a life threatening complication in children with hematological malignancies. The brain represents a common site of hematogenously disseminated infections from an extracranial focus. We report our experience in the diagnosis, radiological aspects and therapeutic approach of fungal brain abscesses in 2 children receiving chemotherapy for acute lymphoblastic leukemia (ALL).

Associations of Apolipoprotein A, High-Sensitivity C-Reactive Protein and Fasting Plasma Insulin in Obese Children With and Without Family History of Cardiovascular Disease.

BACKGROUND: The worldwide prevalence of childhood obesity has increased from 4.2% to 6.7% during the last two decades. Pediatric obesity is a major health problem, which is dramatically increasing in Greece. A variety of inflammatory variables have been also found to associate with cardiometabolic (CV) risk in obese children. The purpose of this study was to identify and examine the effects of possible CV risk factors in obese and non-obese children with and without family history (FH) of cardiovascular disease (CVD). METHODS: Sixty-eight (68) healthy children and adolescents aged 7 - 13 years participated in the study. Anthropometrical and biochemical indexes were obtained from all children as well as FH of CVD. RESULTS: Systolic blood pressure (SBP), total cholesterol (TC), triglyceride (TG), high-sensitivity C-reactive protein (hsCRP), fasting plasma insulin (FPI) and homeostasis model assessment of insulin resistance (HOMA-IR) levels were found statistically significantly higher in the obese group compared to the non-obese one. High-density lipoprotein (HDL) levels were observed to be statistically significantly lower in the obese children compared to their normal peers. CONCLUSIONS: Apolipoprotein A, hsCRP and FPI levels were significantly higher in the obese children with FH of CVD compared to the ones without FH of CVD. TC and SBP were found to be independently associated with obesity (odds ratio (OR): 1.965, 95% confidence interval (CI): 1.935 - 2.97, P < 0.031 and OR: 1.045, 95% CI: 1.016 - 1.074, P < 0.002, respectively).

Reduced exercise capacity in Greek children with mild to moderate obstructive sleep apnea syndrome.

INTRODUCTION: Obstructive sleep apnea syndrome (OSAS) is a common disease that is increasingly recognized among pediatric population. The exercise capacity of adults with OSAS has been demonstrated to be impaired, but there are no data about pediatric exercise response. AIM: The aim of this study was to evaluate cardiopulmonary response to exercise in children with OSAS and to correlate exercise capacity and severity of OSAS. METHODS: Twenty-seven children with habitual snoring (Group A) (mean age 10.5 ± 1.8 years) referred for overnight polysomnography and 13 apparently healthy controls (mean age 11 ± 1.5 years) were recruited. According to the apnea hypopnea index (AHI) group A consisted of 15 (55.6%) children with mild OSAS and 12 (44.4%) with moderate-severe OSAS. All children completed a maximal ramping cardiopulmonary exercise test (CPET) on cycle ergometer. RESULTS: According to CPET children with OSAS had significantly lower VO2max (40.3 ± 8.4 ml/kg/min vs. 47.6 ± 7.9 ml/kg/min, P = 0.013) significantly lower VO2max (%) (77.7 ± 15 vs. 92.9 ± 10.5, P = 0.002), lower maximum heart-rate at peak exercise (86.6 ± 8.8 beat/min vs. 90.6 ± 7.2 beat/min) and higher systolic blood pressure level at peak exercise (145 ± 27.4 mmHg vs. 143.92 ± 20 mmHg) compared to control group. CONCLUSION: The present study demonstrates that young patients with OSAS, even with mild OSAS, had reduced exercise capacity as compared to control group.

Socioeconomic status, area remoteness, and survival from childhood leukemia: results from the Nationwide Registry for Childhood Hematological Malignancies in Greece.

The aim of the present nationwide Greek study is to assess whether survival from acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML) is modified by socioeconomic status (SES) and area remoteness. Detailed precoded information derived from a personal interview conducted by specially trained health personnel with the child guardians was available for 883 ALL and 111 AML incident childhood cases registered in the Nationwide Registry for Childhood Hematological Malignancies during the period 1996-2010. Parental socioprofessional category was recorded on the basis of ISCO68 and ISCO88 codes; the exact traveling distance between residence and the treating hospital was ad hoc calculated. Multivariate Cox's proportional hazards models were applied to examine the mutually adjusted associations between survival and potential predictors. Children from a lower parental socioprofessional category experienced 40% worse survival (P=0.02) independent of age, sex, and ALL subtype, whereas those whose parents were married had better outcomes (rate ratio: 0.47, P=0.01). Urbanization of residence at diagnosis or 'residence to treating hospital' distance was not nominally associated with survival from ALL. By contrast, no noteworthy associations implicating SES were found for AML survival, probably because of the burden of the disease and small numbers. Lower SES indicators and a single-parenthood family milieu seem to be independently associated with unfavorable outcomes from childhood ALL. Area remoteness might not be a significant outcome predictor during recent years, following considerable improvements in the motorway infrastructures and care delivery patterns. This study may provide a valuable snapshot capturing the impact of socioeconomic covariates before the burst of the Greek financial crisis.