Urinary tract infection (UTI) is one of the most common bacterial infections in pediatrics. Delay in diagnosis and treatment can cause significant morbidity. The physician's knowledge regarding the symptoms, microorganisms that caused UTI, and effective antibiotics in a geographical area can help them to select the appropriate antibiotics. This study was performed to determine the prevalence of bacteria that cause UTI and their susceptibility to common antibiotics as well as the common symptoms and associated factors in children of Shiraz, Southern Iran.This cross sectional study was performed among 202 children with UTI, aged 2 months to 18 years old, between August and November 2014 in pediatric medical centers of Shiraz University of Medical Sciences. Urine samples were collected using urinary catheter or suprapubic in children < 2 years and mid-stream in children over 2 years, respectively. The type of micro-organisms causing UTI was determined and evaluation of antibiotic susceptibility for each organism was assayed by the Kirby Bauer method using antibiogram test. Patient's information was collected through checking the medical documents and interview with parents.Our results showed that the frequency of UTI was significantly higher in girls (70.3%) than in boys. The most commonly discovered pathogens were Escherichia coli (E coli) (51.5%), followed by Klebsiella spp. (16.8%), and Enterococcus spp. (9.9%). Overall susceptibility test showed the highest resistance to ampicillin (81.2%) and cotrimoxazole (79.2%), and the highest sensitivity to imipenem (90.1%) and Gentamicin (65.3%). Gram negative and positive bacteria showed the highest antibiotic resistance to amoxicillin (83.8%) and clindamycin (100%), respectively. In addition, production of extended spectrum beta lactamase (ESBL) was 69.2% and 30.8% in E coli and Kelebsiella respectively.The efficacy of third generation of the cephalosporins was reduced because of the high rate of production of ESBL and drug resistance. These results inform the physician as to which antibiotics are appropriate to prescribe for the patient, as well as urine culture reports and following the patient's clinical response so that high antimicrobial resistance is not developed at the community level.

Hypertension is a common complication after renal transplantation and is associated with increased risk of cardiovascular disease. The aim of the current study was to investigate the diurnal blood pressure pattern and its relation to structural and functional cardiac changes in renal transplant recipients. Sixty-six stable renal transplant patients (34 female, 32 male), aged 7 to 25 years (mean 17.4±4.3 years) were enrolled in this study. Cardiac function assessed by tissue Doppler echocardiography and blood pressure measurement performed using both the ambulatory and the casual method. Hypertension was demonstrated in 57% of recipients by the casual method and in 75.7% by ambulatory blood pressure monitoring (ABPM). The efficacy of BP control among patients on antihypertensive drugs was 60%. The prevalence of non-dipping was 73%. There was significant inverse correlation between systolic or diastolic day-time or night-time BP index and post-transplant duration (p<0.001, r=-0.386), but no correlation between ABP parameters and BMI, gender, and eGFR. There was a significant relationship between all ABP parameters and left ventricular mass index (LVMI) (p=0.025-0.007, r=0.28-0.38). LVMI was significantly higher in hypertensive than in normotensive cases (p=0.034). There was no difference in diastolic function between hypertensive and normotensive patients or between patients with and without left ventricular hypertrophy (LVH). In conclusion, our study showed the advantage of ABPM over the casual method of diagnosis of hypertension. LVH is common in transplant patients and is likely associated with arterial hypertension. Hypertension and LVH cannot differentiate transplant patients with diastolic malfunction.

Urinary tract infection is common in children. The available gold standard methods for diagnosis, Tc-99m dimercaptosuccinic acid (DMSA) scan and computed tomography (CT) are invasive and expensive. This study was performed to assess the role of power Doppler ultrasound (PDU) for diagnosis of acute pyelonephritis (APN) compared with Tc-99m DMSA scan. A prospective study was conducted in 34 children with the mean age of 2.8+/-2.7 years who were hospitalized with the first episode of febrile urinary tract infection. All children were examined in the first 3 days of admission by PDU and Tc-99m DMSA scan. Patients with congenital structural anomaly were excluded. Each kidney was divided into three zones. The comparison between PDU and DMSA scan was performed on the basis of patients and renal units. According to the patient's number, sensitivity, specificity, positive and negative predictive values, and accuracy of PDU were 89%, 53%, 70%, 80%, and 74%, respectively, but based on the renal units, changed to 66%, 81%, 46%, 91%, and 79%, respectively. Although PDU has the potential for identifying APN in children, it is still soon to replace DMSA scan.

Schimke immuno-osseous dysplasia (SIOD) is a multisystemic disorder with prominent skeletal, renal, immunological, and ectodermal abnormalities. It is caused by mutations of SMARCAL1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1), which encodes a DNA stress response protein. To determine the relationship of this function to the SIOD phenotype, we profiled the cancer prevalence in SIOD and assessed if defects of nucleotide excision repair (NER) and of nonhomologous end joining (NHEJ), respectively, explained the ectodermal and immunological features of SIOD. Finally, we determined if Smarcal1del/del mice had hypersensitivity to irinotecan (CPT-11), etoposide and hydroxyurea (HU) and whether exposure to these agents induced features of SIOD. Among 71 SIOD patients, three had non-Hodgkin lymphoma (NHL) and one had osteosarcoma. We did not find evidence of defective NER or NHEJ; however, Smarcal1-deficient mice were hypersensitive to several genotoxic agents. Also, CPT-11, etoposide and HU caused decreased growth and loss of growth plate chondrocytes. These data, which identify an increased prevalence of NHL in SIOD and confirm hypersensitivity to DNA damaging agents in vivo, provide guidance for the management of SIOD patients.

BackgroundArteriosclerosis and emphysema develop in individuals with Schimke immuno-osseous dysplasia (SIOD), a multisystem disorder caused by biallelic mutations in SMARCAL1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1). However, the mechanism by which the vascular and pulmonary disease arises in SIOD remains unknown.MethodsWe reviewed the records of 65 patients with SMARCAL1 mutations. Molecular and immunohistochemical analyses were conducted on autopsy tissue from 4 SIOD patients.ResultsThirty-two of 63 patients had signs of arteriosclerosis and 3 of 51 had signs of emphysema. The arteriosclerosis was characterized by intimal and medial hyperplasia, smooth muscle cell hyperplasia and fragmented and disorganized elastin fibers, and the pulmonary disease was characterized by panlobular enlargement of air spaces. Consistent with a cell autonomous disorder, SMARCAL1 was expressed in arterial and lung tissue, and both the aorta and lung of SIOD patients had reduced expression of elastin and alterations in the expression of regulators of elastin gene expression.ConclusionsThis first comprehensive study of the vascular and pulmonary complications of SIOD shows that these commonly cause morbidity and mortality and might arise from impaired elastogenesis. Additionally, the effect of SMARCAL1 deficiency on elastin expression provides a model for understanding other features of SIOD.

A 10-year-old boy with systemic lupus erythematosus (SLE) developed abrupt right upper quadrant pain and vomiting during the course of his active disease. Antiphospholipid antibody was negative and the C3 level was low. Abdominal sonography showed cholecystitis with sludge balls in the gallbladder. He was treated by high-dose prednisolone with ceftriaxone and metronidazole IV for 3 days but due to poor response, cholecystectomy was performed and no stone was identified. Histopathologic examination showed vasculitis in the medium-sized arteries of the gallbladder wall. He was doing well at the 9-month follow-up after the operation. This report describes the first pediatric case of SLE with acalculous cholecystitis caused by vasculitis of the gallbladder.

Despite increasing reports on nonionic contrast media-induced nephropathy (CIN) in hospitalized adult patients during cardiac procedures, the studies in pediatrics are limited, with even less focus on possible predisposing factors and preventive measures for patients undergoing cardiac angiography. This prospective study determined the incidence of CIN for two nonionic contrast media (CM), iopromide and iohexol, among 80 patients younger than 18 years and compared the rates for this complication in relation to the type and dosage of CM and the presence of cyanosis. The 80 patients in the study consecutively received either iopromide (group A, n = 40) or iohexol (group B, n = 40). Serum sodium (Na), potassium (K), and creatinine (Cr) were measured 24 h before angiography as baseline values, then measured again at 12-, 24-, and 48-h intervals after CM use. Urine samples for Na and Cr also were checked at the same intervals. Risk of renal failure, Injury to the kidney, Failure of kidney function, Loss of kidney function, and End-stage renal damage (RIFLE criteria) were used to define CIN and its incidence in the study population. Accordingly, among the 15 CIN patients (18.75%), 7.5% of the patients in group A had increased risk and 3.75% had renal injury, whereas 5% of group B had increased risk and 2.5% had renal injury. Whereas 33.3% of the patients with CIN were among those who received the proper dosage of CM, the percentage increased to 66.6% among those who received larger doses, with a significant difference in the incidence of CIN related to the different dosages of CM (p = 0.014). Among the 15 patients with CIN, 6 had cyanotic congenital heart diseases, but the incidence did not differ significantly from that for the noncyanotic patients (p = 0.243). Although clinically silent, CIN is not rare in pediatrics. The incidence depends on dosage but not on the type of consumed nonionic CM, nor on the presence of cyanosis, and although CIN usually is reversible, more concern is needed for the prevention of such a complication in children.

Long-term survival after successful renal transplantation is shortened by cardiovascular disease. Cardiovascular disease is a main cause of morbidity and death among children and young adults after renal transplantation. The aim of our study was to measure the carotid intima media thickness (cIMT) and determine its relationship to the risk factors for early arteriopathy in renal transplant recipients. Sixty-six stable renal transplant patients (36 female and 30 male), 7-25 years of age (mean 18.3 +/- 4.5 years) were enrolled in this study. The cIMT was measured by high-resolution B mode ultrasonography in multiple projections. The results were correlated with clinical and paraclinical parameters, including age, gender, body mass index (BMI), blood pressure, glomerular filtration rate (GFR), duration of dialysis, duration of chronic kidney disease (CKD), post-transplantation interval, calcium-phosphate (CaxP) product, cumulative dose of Ca-based P binder and calcitriol, lipid profile, uric acid, and cyclosporine level. The mean post-transplantation follow-up period was 64 +/- 40 months. The mean cIMT standard deviation score (SDS) of the patients and the control group was 0.60 +/- 0.81 mm (range -1.10 mm to 2.75 mm) and -1.25 +/- 0.95 mm (range -3.23 mm to 0.26 mm), respectively. Renal transplant recipients had a significantly greater cIMT than that of the controls (P < 0.001). Among several risk factors, there were positive correlations between cIMT SDS and gender, and cumulative dose of calcitriol (P = 0.02 and P = 0.02, respectively). In conclusion, subclinical atherosclerosis is present in young transplant recipients. Non-invasive monitoring of cIMT in renal transplant patients for the detection of early vascular lesions might be of value in preventing cardiovascular disease. Further studies are needed to see if proper monitoring of vitamin D therapy before and after transplantation could be helpful in the prevention of arteriopathy in renal transplant recipients.