PPHN and oxidative stress: a review of literature.

Persistent pulmonary hypertension of the neonate is a multifactorial condition characterized by maladaptive pulmonary vascular remodeling and abnormal contractile reactivity. This review evaluates the role of oxidative stress and antioxidant treatment on the persistent pulmonary hypertension of the neonate.

Pediatric urolithiasis.

Urolithiasis is a well-known condition that can affect any part of the urinary tract. With a rate of 3-5% the incidence of upper urinary tract for long has been higher in adults (1-3), but recently it has increased among children reaching 3,3% . Indeed, more than 1% of all urinary stones are seen in patients aged less than 18 years (4). Pediatric urolithiasis is endemic in Turkey and Far East and it is probably due to malnutrition and racial factors (5). The spontaneous stone passage is more likely in children than in adults, indeed ureteral calculi spontaneously pass into 41-63% of children (1). Rate of stone passage depends on size and stone location in the urinary system. Stones sized less than 5 mm have a passage rate ranging from 40% to 98%, whilst stones > 5 mm have between 55% and 50% (6). In the last decade, the use of alpha blockers has proven well efficacious in helping spontaneous passage of distal ureteric stones in adults (7-9). The latest EAU guidelines support their use in adults while remain vague about their use in children because of unclear safety and efficacy (4). In search of evidence supporting or not the use of medical expulsive therapy in children we reviewed the literature dealing with the management of urolithiasis in pediatric patients. The primary aim of the present study was to evaluate the efficacy of medical expulsive therapy (MET), defined as stone expulsion rate, with a-blockers compared to a control group. The secondary aim was to assess the safety, defined as side effects rate, of MET compared to a control group.

Monosymptomatic enuresis: the therapeutic weapons.

Nocturnal enuresis (NE) was defined by the World Health Organization (ICD-10) and the American Psychiatric Association (DSM-5) as bed-wetting in children aged >5 years. In cases of mental retardation, the developmental age may be equivalent to 5 years. In this review, we focus on the current knowledge about the etiology of enuresis and the most recent therapeutical options. Both non-pharmacological and pharmacological therapies are included, although the relative effectiveness of each remains uncertain. To date, motivational, alarm and drug therapies are the mainstay of treatment. Alarm therapy remains the first-line treatment modality for NE, while desmopressin is the most commonly used medical treatment.

Unusual presentation of Henoch-Schönlein purpura.

Henoch Schonlein Purpura (HSP) is a systematic IgA-mediated vasculitic disease that affects the small vessels of the skin, the joints, the gastrointestinal tract and the kidneys (1). It is the most common childhood vaculitis, with an incidence estimated at 3-26 per 100,000 children, and with a male-to-female ratio of 2:1 (2-6). The 90% of patients are under 10 years of age, with a mean age of 4 years (4). It seems to be most common in fall and winter in children, and summer and winter in adults (7). Recent studies suggested a strong genetic predisposition in individuals with immunoglobulin Avasculitis (IgAV) associated to HLA class II region. Clinically, the non-thrombocytopenic purpura often located on lower extremities and buttocks is the essential element for the diagnosis of HSP. Treatment is supportive, because the disease is usually benign and self-limited. Indeed, in children, the prognosis is good, with a self-limited course and without any complications and after a median follow-up of 12 months, complete recovery was obtained in 83% of the IgAV patients (4, 8). The aim of our study is to describe some atypical presentations of the HSP in children.

Local therapy with ozone in the management of the exit site in a patient undergoing peritoneal dialysis.

The natural history of children with end stage renal disease is dialysis until a transplant can be done. There are two types of dialysis: hemodialysis and peritoneal dialysis (1). Peritoneal dialysis is preferred in young children because getting the vascular access for hemodialysis is challenging (2). Catheters should be surgically placed in a paramedian or lateral abdominal region with an extremity located in Douglas' pouch.

Hemodialysis in children: how, when and why.

End-stage renal diseases requiring chronic dialysis are rare in childhood and adolescence, but they are associated with high mortality and impaired quality of life (1, 2). The most common disease that causes chronic kidney disease (CKD) is primary glomerular disease (GD), followed by congenital abnormalities of the kidney and urinary tract, cystic, hereditary or congenital disorders and, more rarely, secondary GD. However, patients with secondary GD, urologic disorders, and metabolic diseases have greater mortality risk than patients with primary GD (3). Here, we focused on the different options of treatment available, and specifically we compared peritoneal dialysis and hemodialysis, showing pros and cons between them.

Accuracy of CT and MRI to assess resection margins in primary malignant bone tumours having histology as the reference standard.

AIM: To evaluate the accuracy of magnetic resonance imaging (MRI) and computed tomography (CT) in assessing the resection margins of primary malignant bone tumours. MATERIALS AND METHODS: Resected primary malignant bone tumour specimens removed from 46 patients (27 male; mean age: 48±22 years) were imaged using MRI (fat-saturated proton density-weighted and three-dimensional fat-suppressed T1-weighted gradient-recalled-echo) and CT immediately after surgery. A radiologist and an orthopaedist evaluated bone and soft-tissue margins of the specimens on both examinations. Histological evaluation was performed by a senior orthopaedic oncology pathologist. Margins were classified as R0 (safe margins), R1 (residuals between 0 and 1 mm), and R2 (macroscopic residuals). Cohen's k, chi-square, and McNemar's statistics were used. RESULTS: Having histology as the reference standard, reproducibility of the radiologist ranged from moderate (k=0.544) to substantial (k=0.741) for bone and soft-tissue margins on CT, respectively, while that of the orthopaedist ranged from fair (k=0.316) to moderate (k=0.548). When comparing R2 and R0+R1 scores, reproducibility of readers' evaluation of bone margins increased ranging from substantial (k=0.655) to perfect (k=1.000). Inter-reader agreement ranged from fair (k=0.308) to substantial (k=0.633). Accuracy of the radiologist and orthopaedist ranged from 76% to 83% and from 68% to 72%, respectively. When comparing R2 and R0+R1 scores, the accuracy of both readers ranged from 83% to 100%. There was no association between local recurrence and margin scores of histology, MRI, and CT (p≥0.058). CONCLUSIONS: MRI and CT may be useful for extemporaneous analysis of resection margins of primary malignant bone tumours, although wide accuracy variability between the different imaging methods was observed.

Relationship between metabolic syndrome and platelet responsiveness to leptin in overweight and obese patients.

OBJECTIVE: To verify whether platelet responsiveness to leptin is associated with metabolic syndrome risk factors. DESIGN: Cross-sectional study. SUBJECTS: We studied 169 consecutive patients, mean age=43.6+/-9.9 years, with overweight (N=57) or obesity (N=112). MEASUREMENTS: Cluster analysis was used to generate three clusters based on platelet responsiveness to increasing doses of leptin. Profiles of metabolic syndrome risk factors of the three clusters were compared by discriminant analysis. RESULTS: Platelet responsiveness to leptin was absent in cluster 1, whereas cluster 3 had the greatest platelet aggregation response to leptin pre-incubation. Plasma leptin levels significantly decreased from cluster 1 to cluster 3 in both gender. Patients in cluster 2 had an intermediate profile of leptin responsiveness. Highest body mass index (BMI) values were more frequent in non-responders, whereas the prevalence of high waist circumference, as well as hypertriglyceridemia and hypertension, increased with increasing responsiveness to leptin from cluster 1 to cluster 3. Pattern of metabolic syndrome risk factors qualified as group specific in 69.0% of the cluster 1, 54.9% of the cluster 2 and 55.8% of the cluster 3. Circulating leptin, waist circumference, plasma triglycerides and BMI defined distinctive patterns of metabolic syndrome risk factors in the clusters. CONCLUSIONS: In overweight and obese outpatients, metabolic syndrome risk factors parallel to some extent platelet responsiveness to leptin. Such a correlation involves plasma leptin levels, waist circumference, plasma triglycerides and BMI, and may contribute to the excess risk of cardiovascular events in overweight and obese patients.

Intracellular polyamine levels are involved in NMDA-evoked nitric oxide production in chick retina cells.

The NMDA-sensitive glutamate receptor complex can be modulated by numerous drugs and endogenous substances such as polyamines. We studied the pathway of arginine/nitric oxide/cyclic GMP in cultured chick retina cells through NMDA receptor activation, seen as a function of both differentiation stages of culture and intracellular polyamine levels. In our experimental conditions, the nitric oxide synthase activity was stimulated by NMDA from three to four times between embryonic day (E) 8 plus 5 days in vitro (C) and E8C7. The NMDA response was blocked by MK-801 (10 microM) by >60% at stage E8C5. During culture differentiation, the NMDA-induced increase in nitric oxide synthase activity at the E8C5 stage was blocked by preliminary incubation (24 h) of the cells with alpha-difluoromethylornithine, the inhibitor of polyamine biosynthesis. This effect was assessed by a reduction of NMDA-evoked cyclic GMP formation in polyamine-depleted retina cells. Thus, intracellular polyamine levels are involved in NMDA-evoked nitric oxide production. Our results indicate that (a) the developmental pattern of polyamine levels can be associated with the modulation of NMDA-evoked events and (b) the NMDA-mediated effects have been reduced in alpha-difluoromethylornithine-treated cell cultures. These observations provide evidence for a physiological interaction between polyamines and NMDA-sensitive glutamate receptors during differentiation stages of cultured chick retina cells.