Changes in structure, interstitial cajal-like cells and apoptosis of smooth muscle cells in congenital ureteropelvic junction obstruction

The goal of this study is to evaluate some structural changes in muscular, collagenous and neural components as well as expression of Cajal-like cells and apoptosis of smooth muscle cells in congenital ureteropelvic junction obstruction [UPJO]. Tissue specimens were obtained from 25 patients with UPJO and compared with normal ureteropelvic junction regions of 19 autopsies. In paraffin embedded sections the amount of Cajal-like cells, density of nerve fibers and smooth muscle cell apoptosis [using immunohistochemical staining] were determined. Collagen deposition and muscular components were stained by Trichrome-Masson staining and evaluated by image analysis techniques. Arrangement of muscular bundles was also evaluated qualitatively. The number of Cajal-like cells was significantly lower in patients than in controls. The apoptotic score and mean number of nerve fibers were not statistically different for the two groups. Arrangement of muscular fibers was more irregular in patients than in controls [P<0.001]. Collagen deposition was significantly higher in patients than in controls [P<0.001]. The mean amount of muscular component was lower in patients than in normal ones. [P= 0.09] We found significant pathologic changes in congenital ureteropelvic junction obstruction such as decrease in Cajal-like cells, increase in collagen deposition and irregular arrangement of muscle fibers

Severe hypertension and encephalopathy due to renin- producing hepatoblastoma

Paraneoplastic syndromes result from secretion of hormones, peptides or cytokines by tumor or immune cross-reactivity between malignant and normal tissues. These conditions are rare in children, but when the clinical presentation of patients with a tumor is unusual, these syndromes should be emphasized. Extrarenal tumors with renin-secretion are rare in children. They may be related to paraneoplastic syndromes. We report a 22-month-old infant with hepatoblastoma presented with severe hypertension and related neurologic symptoms due to high plasma renin activity. To the best of our knowledge, this is the second report of renin producing hepatoblastoma in the literature. However, due to lack of laboratory facilities such as immunohistochemical study or polyclonal antibody for human renin activity, we could not prove the secretion of renin just by tumor tissue cells, but this potentiality is very likely. Other intensive investigations did not show any other origin for rennin secretion or hypertension in this patient

Her2/neu expression in Wilms' tumor and correlation with histopathologic findings

Wilms' tumor is an emberyonal tumor arising from remnants of immature renal tissue. Her2/neu is an onco-protein which mediates cellular proliferation, differentiation and survival. In the current study, we analyzed Her2/neu expression in 40 Wilms' tumors. The clinico-demographic data of 40 patients with Wilms' tumor were retrieved. Immunohistochemical staining for HER2/neu was performed. Her2/neuimmunoreactivity was evaluated by Canadian Consensus 2007 scoring system. Among the 38 specimens with epithelial component, 68.5% were positive for Her2/neu, whereas there was immunoreactivity in 37% of 38 blastemal, and 12% of 31 stromal components. The Her2/neu expression was significantly higher in early stages [81.5%] than in advanced stages [36.4%] in epithelial component, but not in other components. This study suggested that Her2/neuexpression is associated with epithelial cell differentiation accompanied by lower stages of tumor. No significant relationship was found between Her2/neu positivity and tumor size and patient's age and gender

Inequalities in cancer distribution in Tehran; a disaggregated estimation of 2007 incidence by 22ÿdistricts

Cancer is the third cause of death in Iran, with an increasing incidence projected for the next decade. This study aimed to provide a disaggregated viewpoint on cancer incidence in all 22 districts of Tehran, using the Geographic Information System [GIS]. Identifying clusters of cancers may assist in recognizing the cause of the disease, visualizing patterns of cancer distribution, the potential disparities, and help in the provision of early detection programs and equitable, curative, and palliative services. According to the 2007 - 2008 Cancer Registry Data published by the Ministry of Health, there were 7948 new cancer cases diagnosed in Tehran. Data were collected from all pathology centers and hospitals, either public or private facilities, in Tehran. These were classified into 31 main categories according to the expert panels and available resources. The population of the districts and neighborhoods were obtained from the Iran Statistical Center and the Municipally of Tehran, respectively. Home addresses and phones were extracted from the database and imported to GIS. The Age-Standardized Rate [ASR] was calculated using both the new world standard population [2000 - 2025] and the Iran population. Overall, the cancer incidence rate and ASR were 101.8 and 94.775 per 100,000 people, respectively. The maximum cancer incidence rates in both sexes were in districts 6, 3, 1, and 2, whereas, the maximum ASRs were in districts 6, 1, 2, and 3. District 6 accommodated the highest ASRs in both the sexes. Common cancers were breast, skin, colorectal, stomach, and prostate. The ASR in men and women were 129.954 and 114.546 per 100,000 population. This report provides an appropriate guide to estimate the cancer distribution within the districts of Tehran. Higher ASR in districts 6, 1, 2, and 3, warrant further research, to obtain robust population-based incidence data and also to investigate the background predisposing factors in the specified districts

Helicobacter pylori infection in pediatric candidates for kidney transplantation

Chronic kidney failure was suggested to have a protective effect against Helicobacter pylori infection in adults. However, data about this effect in children is lacking. This study was designed to ascertain the prevalence, endoscopic findings, and histopathological features accompanying the Helicobacter pylori infection in children with end-stage renal disease. Data were collected from 117 children with end-stage renal disease aged 5 to 18 years that underwent routine upper gastrointestinal endoscopy before kidney transplantation between 1998 and 2009. The specimens that were taken from the antrum were stained with hematoxylin-eosin and Giemsa to detect Helicobacter pylori. Gastrointestinal symptoms were reported in 12% of the patients. Helicobacter pylori was detected in 24% of the children. The prevalence of Helicobacter pylori infection was high in children with abnormal endoscopic findings [P = .02]. There was no correlation between Helicobacter pylori infection and gender, dialysis status, duration of dialysis, underlying diseases, and gastrointestinal symptoms. Helicobacter pylori infection had a significant correlation with histopathological features [P = .005], age older than 10 years [P = .003], and upper gastrointestinal endoscopic findings [P = .001]. In this study, Helicobacter pylori infection had a high prevalence in children with end-stage renal disease, especially in older ones. The majority of children with Helicobacter pylori infection were asymptomatic, while they had abnormal findings on upper gastrointestinal endoscopy and chronic active gastritis features in histopathological assessment

role of electron microscopy for the diagnosis of childhood glomerular diseases

Optimum diagnosis of glomerulopathies requires light microscopy, immunofluorescence and electron microcopy. In fact electron microscopy has a confirmatory role in glomerular diseases. It provides more information for patient management and can rule out other diseases. The goal of the present study is analysis the necessity of electron microscopy for the diagnosis of childhood glomerulopathies. 134 cases of renal biopsy with some clinical data retrospectively were reviewed. The contribution of electron microscopy to the final diagnosis was graded as necessary - diagnosis could not be reached without it, supportive - it increased the level of confidence in the final diagnosis and noncontributory - the diagnosis don't need electron microscopy for confirmation. The contribution of electron microscopy to the final diagnosis was necessary in 51 cases [38%], supportive in 40 cases [=30%] and noncontributory in 43 cases [32%]. In conclusion the results showed in about 68% of childhood glomerulopathies the ultrastructural study was necessary or supportive, so electron microscopy still remains an important tool in diagnosis of childhood glomerulopathies

Sonographic value in the diagnosis of gestational trophoblastic disease

Hydatiform moles are a group of fertilization disorders characterized by an abnormal growth of chorionic tissues. It has an incidence of 1/347 in Iran. Early diagnosis of disease is of clinical importance. The aim of this study was to investigate the sensitivity of sonography in evaluation of Gestational Trophoblastic disease [GTD]. Across sectional hospital based study was designed. A total number of 95 women with sonography diagnosis of gestational trophoblastic disease, missed abortion, incomplete abortion and blighted ovum participated in the study. The patients underwent hysterectomy; the type of disorders was confirmed by pathology. Ninety five patients participated in the study with the mean age of 24.05, Eighty nine of them with Complete Hydatiform Mole [CHM] diagnosis and six of them with Partial Hydatiform Mole [PHM]. There was a 87.5% sensitivity for diagnosis of molar pregnancies totally when there was 91.3% sensitivity in the diagnosis of CHM and 60% sensitivity in the diagnosis of PHM. These findings showed that sonography is an effective diagnostic tool in the diagnosis of molar pregnancies; however it is more sensitive in the diagnosis of CHM than PHM. Further studies are warranted to elucidate the role of ultrasonography in future

[Distribution of cancer incidence in districts and neighbourhoods of a number of Tehran districts in 1386]

The global burden of cancer continues to increase largely because of the aging and growth of the world population alongside an increasing adoption of cancer-causing behaviors, particularly smoking and various environmental factors. A variety of cancers data in special geographic areas can help define medical programs for treatment and screening of high-risk groups. Since cancer types has not been reported within districts and neighbourhoods of Tehran city so far, this study could be considered as the first in this respect, which defines cancer epidemiology in the catchment area, and on the other hand, paves the provision of equitable services patient who need these facilities. This is the cross sectional study that shows cancer distribution patern. Incident cases from Tehran residents covered by the Iran University of Medical Sciences were obtained, which included almost 55% of the total cancer incidence in Tehran in 2007. Districts covered by the university were located in west and southwest of Tehran including districts 2,5,6,9,18,21, and 22 cases of other districts refered to Iran university that identified incidence cancer but results must interpreted with caution since those resident districts have not been covered population data were obtained from the previous census in 2006. Age and sex standard rate were calculated based on Iranian standard population. Cancers distribution maps were developed using available adresses, indicating districts and neighbourhoods in GIS. Common cancers diffrence within covered districts was tested by Fisher exact test. Cancer incidence in specified districts of Tehran was 72.822 in 100000 population, where district 6 had the highest incidence [ASR=90.552] followed by district 2 [ASR=71.503] and the least incidence was in district 18 [ASR=34.991]. This diffrence was significant within districts by Fisher exact test. The highest ASRs in women was 87.517 in districts 6 and 2 [71.621] and in male respectivly 94.683 and 70.919. Highest incidence within neighbourhoods were Abasabad, Qaemmaqam and Gand in males and Ddaneshgah, Arjantin and Shiraz in females. High cancers ASR consisted of breast [9.018], colorectal [4.94], prostate [4.174], stomach [3.711] and skin [3.522]. More cancer aggregation, which was observed in districts 2 and 6 within the university catchment area and districts 1 and 3 out of this territory, warrants more researches to investigate what factors in the specified districts and neighbourhoods has led to this condition

value of serum uric acid as a mortality prediction in critically ill children

The role of initial serum uric acid on admission in critically ill patients is controversial; we presumed that uric acid level can predict the mortality of the admitted patients to intensive care unit as a simple test. Totally 220 consecutively admitted children [96 girls, 124 boys] with mean age 3.5 years, who were at least 24 hours in pediatric intensive care unit [PICU], were enrolled in a prospective cohort study during January 2006 to December 2007. The subsequent PICU admission in the same hospitalization, those who were discharged from the hospital and then re-admitted to the PICU during the observation period, and the patients with chronic renal failure were excluded. Serum uric acid level was measured during the first day of PICU admission. Death or transfer from PICU was considered as final outcome. The statistical analysis was done by suing linear regression analysis, ROC curve, student t-test, and Chi-square. P value less than 0.05 was considered significant. From 44 patients who had serum uric acid level more than 8 mg/dl, 17 cases died showing with a higher relative risk of 1.88, higher mortality [P<0.05]. The relative risk of death in patients who had serum uric acid > 8mg/dl and needed vasopressor was 1.04, and in those under mechanical ventilation 1.33. In patients who scored pediatric risk of mortality of > 38 it was 1.4, and in septic cases 4 [P<0.05]. Stepwise linear regression analysis showed that mainly the need for mechanical ventilation [P=0.001] and vasopressor had statistically significant correlation with the poor outcome [P=0.001]. Uric acid level during the first day of intensive critical care admission is not an independent risk of mortality in PICU. Need for mechanical ventilation or inotropic agents was associated with poor outcome and only higher uric acid level in sepsis played an additive risk factor role

Management and outcome of steroid-resistant nephrotic syndrome in children

Steroid-resistant nephrotic syndrome [SRNS] is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes. We assessed 73 children with SRNS admitted to Ali Asghar Children Hospital in Tehran, Iran. Their clinical presentations, treatment, and disease courses were reviewed. The mean follow-up duration was 6.0 +/- 4.2 years. Moreover, survival times were calculated and the Cox regression method was used to determine variables able to predict survival of the kidneys. Age at the onset of the disease, sex, and hematuria were not predictive of the response to treatment with immunosuppressive drugs in the children with SRNS. The type of resistance [early or late] was associated with the responsiveness to immunosuppressives. Response to any of the immunosuppressive drugs determined the responsiveness to other immunosuppressive drugs. Cyclosporine was more effective than cyclophosphamide as initial therapy. The mean kidney survival time was 11.62 years. Kidney survival rates were 94.6%, 70.0%, 56.0%, and 34.0% at 1, 5, 10, and 15 years, respectively, in patients with initial resistance to steroid, while these were 100%, 100%, 83.0%, and 83.0% in those with late resistance, respectively [P = .03]. We showed that patients with late steroid resistance had better response to immunosuprressive drugs than patients with early resistance. We also showed that resistance to immunosuppressive therapies increased the risk of resistance to other immunosuppressive drugs. Achievement of complete or partial remission with any therapy reduced the risk of ESRD

Quantification of proteinuria with urinary protein to osmolality ratios in children with and without renal insufficiency

Spot urine is recommended as an accurate method to determine proteinuria in children and adults. However, urinary excretion of creatinine may vary in newborns and spot urine may be influenced by the hydration-dehydration condition of patients. The study was done to assess the validity of the urine protein to osmolality ratio versus the urine protein to creatinine ratio in health and disease conditions. We studied the correlation of the urine protein-osmolality ratio [Uprot/Uosm] and the urine protein to creatinine ratio [Up/Ucr] and compared results with the 24-hour urinary protein excretion. Three groups were compared: children with normal renal function and without proteinuria [group 1, n=53], children with normal renal function and with proteinuria [group 2, n=52] and patients with renal insufficiency [group 3, n=45]. Early morning urine samples and 24-hour urine specimens were collected for protein, creatinine, and osmolality. The optimal cutoff value of the Uprot/Uosm ratio was determined to be 0.33 mg/L/mosm/kgH2O for abnormal proteinuria and 1.75 mg/L/mosm/kgH2O for nephrotic range proteinuria. In comparing ROC curves, we found no differences between the Uprot/Uosm and Up/Ucr ratios in detecting abnormal proteinuria or nephrotic syndrome in children with normal or decreased renal function [P>0.05]. Both the Uprot/Uosm and Up/Ucr ratios from random urine specimens are good predictors of 24-hour urinary total protein excretion in children with and without renal insufficiency