Medical Journal of The Islamic Republic of Iran (MJIRI)

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The purpose of this study has been to evaluate various factors influencing prognosis in children with hemolytic-uremic syndrome (HUS). Forty children with classical picture of HUS were seen in 1986-1 991. Boys and girls were equally affected, aged from two months to ten years. In 35 patients (87.5%) there was a history of diarrhea which was bloody in25. All were treated with peritoneal dialysis within the first 24 hours. Fresh frozen plasma (FFP) was transfused in the first two days for all except II patients for whom it was transfused in the third to fifth day of admission. Fifteen patients died (37.5%) of these, 12 (80%) had diarrhea for longer than 7 days, II (73%) had prominent neutrophilia, and 9 (60%) had significant neurological symptoms. Eleven of the fifteen patients had been transfused with FFP after the third clay of hospitalization. Statistical analysis of data relating to mortality revealed the following regarding prognostic factors in HUS among children: mortality is higher in those with longer prodromal period (p<0.001), in those with bloody diarrhea (p<0.025), in patients with prominent neutrophilia (p<0.001), and in those who had delayed treatment with FFP (p<0.001). Prognosis was not affected by age, sex, or season of presentation.

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Most current references recommend divided doses of prednisolone for the initial treatment of idiopathic minimal change nephrotic syndrome in children, with relapse occuring in the majority of them, but there is little experience concerning single-dose prednisolone therapy, especially considering the relapse rate. In this prospective study on 36 consecutive children with primary nephrotic syndrome, prednisolone (2 mg/kg/day) was used as a single daily dose in 17 patients (SD group), or divided into 3 doses in 19 cases (DD group) who were randomly selected, and relapse rates were compared. The mean age of the patients was 6 years (range 15 months-I3 years)• and there was no statistically significant difference between the two groups considering age, sex, clinical presentation, laboratory findings at the time of admission and prednisolone side effects. After 4 weeks of full dose prednisolone therapy, the drug was changed to 2 mg! kg as a single dose every other day in both groups, irrespective of the response. During the third month of therapy, the drug was reduced to 1 mg/kg every other day and it was discontinued after 4 weeks. Relapse rate was compared in steroid responsive patients in both groups (14 in the SD and 11 in the DD group) who were matched for age, sex and paraclinical findings. During the first year of follow-up, in those who were initially steroid responsive, 2 patients in the SD and 6 in the DD group had no relapse. The number of total relapses/year in the SD and DD group were 19 for 12 patients and 8 for 5 patients respectively (p

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A case of renal tubular acidosis (RTA) who had gradual onset of paralysis of lower extremities and persisted for a long time is presented. His primary workups were mistaken for muscular dystrophy. Eventually, an abdominal ultrasonography revealed small stones in both of the kidneys and his workup for the stones were in favor of distal renal tubular acidosis and advanced rickets with hypokalemia. He had a dramatic response to alkaline therapy. There are many reports of periodic paralysis in RTA but no report of prolonged paralysis is found in the literature.

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 ABSTRACT

 Background: The efficacy of anticonvulsants after a first seizure is uncertain the more predictable the time and the type of recunent seizure, the more preventable the probable events. This study was conducted to evaluate sirnilmity of type and time of a first seizure and its recutTence in children.

 Methods: 174 children with at least two separate seizures were taken into account.

 Results: Overall in 90.5% the sleep-wake state at the second seizure was the same as the first one, while the seizure type was consistent from the first seizure to the second with 95.9% being the same.

 Conclusion: This infonnation should be helpful in 1) counseling parents after their child has had a first seizure, and 2) in answering the question whether treatment should be started in a child presenting with a first epi Ieptic seizure.

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Absract

This study investigated a 63-day boy with end stage renal disease and abdominal

cysts. The antenatal sonography detected anhydraminos, posterior urethral valve,

and cystic dysplastic kidneys. Voiding cystourethrogram revealed two obstructive

giant diverticula which at first looked like enlarged renal pelvis. The patient had persistent

urinary tract infection and perforation of diverticula. The dialysis was ineffective

because of leakage, immeasurable inflow and dwell volume, peritonitis and tunnel

infection. As a result, the availability of automated peritoneal dialysis for infants

is recommended to reduce morbidity and increasing the survival rate. Nonetheless

the giant bladder diverticules might be better managed by diverticulectomy procedure.

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 Abstract

 Background: Clinical Risk Index of Babies (CRIB), Score for Neonatal Acute Physiology

 (SNAP), an update of the Clinical Risk Index for Babies score (CRIB II) and

 Score for Neonatal Acute Physiology - Perinatal Extension (SNAP-PE) are scoring devices

 developed in neonatal intensive care units. This study reviewed these scoring systems

 in critically ill neonates to determine how well they could predict mortality.

 Methods: This prospective cohort study was conducted at the neonatal intensive care

 units of Mofid and Mahdieh hospitals between March 2006 and May 2009. We evaluated

 CRIB, CRIB II, SNAP, SNAPII and SNAP-PE score for each neonate and the final

 scores were then obtained. The predictive accuracy of these parameters were expressed

 as area under the receiver operative characteristic curve, sensitivity, specificity, positive

 predictive value and negative predictive value.

 Results: Of 404 neonate evaluated 53% were male. Primary diagnoses were respiratory

 distress syndrome, gastrointestinal obstruction, sepsis, prematurity, and neuromuscular

 diseases. The authors detected mortality in 20.5% and found a significant difference

 in scoring systems between survived and death groups. The mean CRIB score in

 survived neonates was 2.57±3.66 and in death neonates 8.43±4.66 (p value<0.001). We

 also found that the SNAP score had the highest area under the curve and the highest sensitivity,

 specificity, positive predictive value, negative predictive value and we had the

 lowest score for CRIB II.

 Conclusion: We concluded that the neonatal scoring systems could be a useful tool

 for prediction of mortality in NICUs and SNAP can predict the mortality better than the

 others.

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  Background :Attention Deficit Hyperactivity Disorder (ADHD) is the most common childhood neurological disorder. This disorder is more prevalent in some chronic diseases. The aim of this study was to investigate ADHD in children with early stages of chronic kidney disease (CKD) and to compare it with healthy children.

  Methods : Seventy five 5-16-year-old children with early stages of CKD (stage 1, 2 and 3) and 75 healthy children without CKD were included in this case – control study as case and control groups, respectively. The participants were selected from those children who were referred to the pediatric clinic of Amir Kabir Hospital of Arak (Iran) in the form of simple probability and based on inclusion and exclusion criteria. ADHD was diagnosed using Conner's Parent Rating Scale – 48 (CPRS-48) and DSM-IV criteria and was confirmed by a psychologist consultant. Data were analyzed by Binomial test in SPSS18.

  Results : ADHD inattentive type was observed in 8 cases (10.6%) with CKD and 2 controls (2.6%) (p= 0.109). Moreover, in the case and control groups, 7 (9.3%) and 6 (8%) children were affected by ADHD hyperactive-impulsive type (p= 0.997), and 9 (12%) and 12 (16%) children were affected by ADHD mixed type (p= 0.664), respectively.

  Conclusion : No differences were found between the prevalence of ADHD in the children with early stages of CKD and the control group. However, due to the importance of the relationships between different types of psychiatric disorders and CKD and lack of enough evidence concerning the relationship between ADHD and different stages of CKD in children, conducting further studies in this field is recommended.

  

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Background: The invasive, expensive, and time-consuming nature of radiological examinations for vesicoureteral reflux (VUR) has compelled researchers to search for new markers to predict VUR. This study was designed to evaluate the usefulness of serum and urine concentrations of neutrophil gelatinase-associated lipocalin (NGAL) in predicting the existence of VUR.
   Methods: This cross-sectional study involved all patients with a first febrile urinary tract infection (UTI) referred to Ali Asghar Children’s Hospital. Each patient included in the study had clinical symptoms of pyelonephritis and a positive urine culture. The patients were divided into 2 groups: VUR and non-VUR. The serum and urinary NGAL levels were calculated in both groups. The receiver operating characteristic (ROC) curve was used to look for serum and urinary NGAL cut-points that differentiated the VUR group from the non-VUR group.
   Results: Among the 40 children in the study, 23 belonged to the VUR group. The median age was 2.5 years (range, 0.3-8 years), and 35 patients were girls. ROC curve analysis showed that only the urinary NGAL level was significantly related to VUR. There was no association between serum NGAL levels and VUR. According to the ROC curve, a urinary NGAL level cut-off value of 15 ng/mL was likely to be diagnostic of VUR with 82.6% sensitivity and 58.8% specificity.
   Conclusion: The urinary NGAL level, specifically with a cut-off value of 15 ng/mL, can indicate the existence of VUR in patients with UTI with near-acceptable levels of sensitivity and specificity.