Iranian Journal of Pediatrics
Year:2007 | Volume:17 | Issue:4|Papers Count:18

Objective: This study aims to assess the utility of a scoring system as predictor of neonatal mortality rate among the neonates admitted within one year to the neonatal intensive care unit (NICU) of the Children’s Medical Center in Tehran, Iran. Material & Methods: Data were gathered from 213 newborns admitted to the NICU from September 2003 to August 2004. In addition to demographic data, Apgar scores at 1 minute and 5 minutes, history and duration of previous hospitalization, initial diagnosis and final diagnosis, and scoring system by using the score for the neonatal acute physiology-perinatal extension II (SNAP-PE II) were carried out within 12 hours after admission to the NICU. All of the parameters were prospectively applied to the admitted newborns. The exclusion criteria were discharge or death in less than 24 hours after NICU admission. Findings: 198 newborn infants met the inclusion criteria. The mean and standard deviation (SD) of the variables including postnatal age, birth weight, SNAP, and finally Apgar scores at 1 minute and 5 minutes of neonates under this study were 7.6 (0.5) days, 2479.8 (29.4) grams, 21.6 (1.1), 7.47( 0.08), and 7.71 (0.06), respectively. Twenty five of the 198 patients died (12.6%). Gestational age (P=0.03), birth weight (P=0.02), Apgar score at 5 minutes (0.001), and SNAP-PE II (P=0.04) were significantly related to the mortality rate. By Analyzing through logistic regression to evaluate the predictive value of these variables in relation to the risk of mortality, it was shown that only SNAPPE II and Apgar score at 5 minutes could significantly predict the neonatal mortality.Conclusion: According to this study SNAP-PE II and Apgar score at 5 minutes can be used to predict mortality among the NICU patients. SNAP-PE II score had the best performance in predicting mortality in this study. More studies with larger samples are suggested to evaluate all of the abovementioned parameters among neonates who are admitted to NICUs countrywide.

Objective: The face is involved in many syndromes of dysmorphogenesis. The soft tissue facial landmarks are known to be influenced by age, race and sex and it is imperative to identify a balance between them before reconstructive surgery. Further, with knowledge of standard facial traits, an individualized norm can be established to optimize facial attractiveness. Material & Methods: Measurements were made on 60 infants (30 males and 30 females) aged between 1-4 months and 60 newborns (30 males and 30 females) with a view to establish the criterion of facial anthropometry for this age group in North Indians. A special emphasis was given in this study to sexual dimorphism. Findings: The mean value and range for all the parameters was determined for the North Indian population. A statistically significant sexual dimorphism was noted to exist in ear length (P<0.01) and length of the philtrum (P<0.05). Philtral-commissural ratio was determined for North Indians, philtrum width=oral width/3.2.Conclusion: The local values derived from well defined populations should be used as reference in the evaluation of a case with dimorphic features. This study demonstrates the existence of a partial positive correlation between philtral width and oral width, i.e. when one increases the other also increases and vice versa. The Philtral–commissural relationship is expected to assist in planning philtral construction in cleft lip patients.

Objective: Pain in neonates can be associated with various risks. So, it seems essential to find a simple and acceptable method for relieving pain. Pharmacologic agents are not recommended in neonates for pain relief in minor procedures but nonpharmacologic interventions like Kangaroo Care (KC) is found to be effective. The objective of this study was to assess the efficacy of KC on physiologic responses to the pain of an intramuscular injection of vitamin K in term neonates. Material & Methods: One hundred healthy term neonates were enrolled for this study during 2 months. The inclusion criteria were gestational age 37-42 weeks, normal vaginal delivery, birth weight 2500-4000 gr, age more than 2 hours and Apgar score more than 7 at 1 minute. They were randomly assigned to intervention and control groups. In the intervention group, the neonate was held in KC for 10 minutes before the injection until 3 minutes after injection. In the control group, the neonate was in the prone position in the isolate. The primary outcome measures were heart rate and blood oxygen saturation rate before, during and 3 minutes after injection.Findings: The heart rate during and 3 minutes after injection for neonates given KC were significantly lower than for neonates in control group (P<0.001). The blood oxygen saturation rate during and 3 minutes after injection for neonates given KC were significantly higher than for neonates in control group (P<0.001).Conclusion: KC given before injection seems to effectively decrease pain from injection. It is a simple, safe and effective analgesic and should be considered for minor invasive procedures in term neonates.

Introduction: There are many advantages of breast milk for infants. The impact of Ramadan fasting of breast-feeding mothers on their exclusively breast-fed infants' growth is still not clear. The objective of this study is to determine the impact of maternal Ramadan fasting on growth parameters of exclusively breast-fed infants. Methods: This cohort study was conducted during Ramadan and five months after Ramadan on 116 healthy, exclusively breast-fed infants aged 15 days to 6 months. 36 infants, whose mothers fasted throughout Ramadan (case group) and 80 infants, whose mothers did not fast (control group), were enrolled in the study. All infants underwent periodic physical examinations, twice in Ramadan, 3 times in the second month and twice monthly in the next 4 months. The data analyses were done using a repeated measure analysis of variance. Statistical significance was defined as P<0.05. Analyses were performed using SPSS statistical package and repeated measures ANOVA. Findings: All growth parameters increased during the study period (P<0.05), rate of increase being the same for both groups (P>0.05). There is obvious increasing trend for all growth parameters; this trend depends on age and is almost similar for both groups.Conclusion: Ramadan fasting by breast-feeding mothers did not adversely affect the growth parameters of exclusively breast-fed infants in short-term.

Objective: Determining prevalence of idiopathic hypercalciuria (IH) in primary school children of Tehran. Material & Methods: We evaluated 778 primary school children (age 6-11, mean 9.1 years) in two steps: first (Screening test), we measured urine calcium to urine creatinine ratio (UCa/UCr) and in the second step (Definitive test), for those children who had UCa/UCr ratio more than 0.21 mg/mg we measured 24 hours urine calcium excretion. Children with secondary forms of hypercalciuria were excluded from the study. Children with idiopathic hypercalciuria were evaluated for manifestations of IH. Findings: Among 778 children, 195 (25.1%) had UCa/UCr ratio more than 0.21 mg/mg, but from these 195 children only 128 children delivered 24 hours urine samples. Among these 128 children for whom 24 hours urine calcium measurements were done 28 children excreted more than 4mg/kg/day calcium without hypercalcemia or any other known causes of hypercalciuria and we defined them as having idiopathic hypercalciuria. If all 195 suspicious cases of IH had delivered 24-hrs urine samples we would have 42 cases of IH. Conclusion: Prevalence of IH in our children was 5.4% and its manifestations were: hematuria, dysuria, recurrent abdominal pain, urinary incontinence, urgency, urinary tract infections and urolithiasis.

Objective: In medicine, especially in the field of pediatrics, it is necessary to perform regular epidemiologic studies in each geographic region. We designed this study in Nemazee Hospital, Shiraz (the major referral pediatric center in Southern Iran), to determine the disease spectrum, the mortality and also the rate of matching final diagnosis with the initial impression in pediatric wards. Material & Methods: All children older than 1 month (2731) who were admitted in Nemazee Hospital (except for surgical wards), from November 2005 to October 2006, were studied through questionnaire interviewing on admission and were followed till discharge or expire time. In some instances that final diagnosis or cause of death was unclear; we followed them through their files in clinics up to 1 year. Findings: Among 3057 admissions (related to 2731 patients), 56.8% were males (male to female ratio: 1.31). Patients’ age was 31 days to 25 years (mean: 5.16 years, SD: 4.98 years) and the significant portion was less than 2 years (1270= 44.2%). Admission duration varied from 1 to 62 days (mean: 5 days, SD: 5.13 days). The most common diseases were seizure disorder (315=11.1%), pneumonia (235=7.7%), febrile convulsion (136=4.79%) and gastroenteritis (128=4.18%). 17.93% of admissions came out of Fars Province (18 Provinces) with digestive system (19.1%), infectious (18.5%) and nervous system diseases (15.0%) as the most frequent referred patients. In 90.6% of admissions final diagnosis was clarified in the hospital (ranged from 86.45% in infectious ward to 93.4% in cardiology ward) (P=0.01). The total mortality rate was 5.30%; this rate was 9.49% for patients younger than 1 year and 5.0% in older patients (P<0.001).Conclusion: The most common age of admission in pediatric wards was less than one year. The most common diseases were seizure disorder, pneumonia and gastroenteritis. Congenital heart disease, sepsis and pneumonia were the most common diseases leading to death.

Objective: Although abdominal mass in neonates is not common, it is very critical to detect it promptly. 2/3 of neonatal abdominal masses belong to kidney. Correct diagnosis and immediate appropriate management can prevent morbidity and mortality. This study pays special attention to incidence, associated anomalies and mortality rate in those neonates who suffered from palpable kidney mass. Material & Methods: During a period of 10 years 48250 neonates were examined for abdominal mass by neonatalogists or pediatric residents. Sonography in suspected cases of abdominal mass was performed to confirm the diagnosis. In all cases of neonatal death, autopsy was performed. Findings: Thirty out of 48250 neonates had abdominal mass presenting as nephromegaly (1/1600). Polycystic kidneys (PCK) were the most common cause of renal enlargement followed by multicystic kidney disease (MCK) and hydronephrosis.Conclusion: It was revealed that renal mass contained most of abdominal mass in our study andmortality (73%) was higher than other reports. Also, we found that PCK was the most common abdominal mass in our cases. Lung hypoplasia was the most common cause of death in PCK cases.

Objective: Pyloric atresia (PA) is a rare congenital anomaly that constitutes approximately 1% of all intestinal atresias, and its incidence is approximately 1 in 100,000 live births. PA may occur as an isolated condition or associated with other abnormalities, the most common being Junctional epidermolysis bullosa (EB). Evidence suggests that PA-EB is a distinct entity. In this report, we present 5 cases of pyloric atresia associated with Junctional epidermolysis bullosa, 4 of whom survived after surgery. Cases Presentation: Prospective evaluation of 5 patients with pyloric atresia associated with Epidermolysis bullosa undergoing therapeutic surgery. Biopsy of the fresh bulla was compatible with Junctional EB in all 5 patients. All patients underwent laparatomy after stabilization. Four neonates underwent gastroduodenostomy, and one patient had excision of membrane and pyloroplasty. Out of 5 neonates, 4 survive and one died from fulminant septicemia 12 days after operation.Conclusion: Although association of PA with EB has been reported to be fatal, recently there have been encouraging reports of survival among these patients. These 5 patients underwent surgery and survived, and are doing well on follow up.

Background: Lethal toxic encephalopathy of shigellosis (Ekiri syndrome) is a rare complication of the shigella infection presented with fever, severe toxicity, seizure and diffuse brain edema, coma and death in the absence of dehydration and is nearly always lethal. This report is about a child who has survived the Ekiri syndrome. Case presentation: A three-year old child was admitted to the emergency ward because of fever, one attack of generalized tonic-clonic seizure, drowsiness and diarrhea with no signs of dehydration. The differential diagnosis was meningitis, shigellosis and atypical febrile convulsion. All test results for blood, urine, stool and cerebro-spinal fluid samples were normal except for serum sodium (119 mmol/l) and stool exam (many RBCs). He was treated with anticonvulsants because of the recurrent seizures and ceftriaxone with probability of shigellosis. The emergency brain CT scan showed diffuse brain edema. After 48 hours the consciousness was improved. Stool culture showed the growth of Shigella flexneri. Second brain CT scan revealed a dramatic decrease of the brain edema. Conclusion: Because neurological symptoms are among the extra intestinal complications of the shigellosis, it is recommended to put this disease in the list of differential diagnosis of unexplained neurological signs in endemic areas and consider the specific medications in the treatment programs. 

Background: Ovarian cysts are the most frequent, prenatally diagnosed intra-abdominal cysts. Prenatal ultrasonography allows diagnosis of ovarian cysts and may suggest antenatal complications. The management of fetal ovarian cysts is still controversial despite the improvement in prenatal diagnosis with ultrasonography. Some studies suggest an aggressive management, while others plead for a conservative one. Case report: We report on the first successfully treated large neonatal ovarian cyst from Iran. She was a normal-term infant with respiratory distress due to the large size of the ovarian cyst diagnosed prenatally. We didn’t find any other related anomaly in the neonate. Conclusion: Neonatal simple ovarian cysts are rare, but may cause complications. Most small simple cysts regress spontaneously; surgical intervention is reserved for complicated or large size ovarian cysts.

Background: Biliary atresia (BA) is a rare disease and the end result of a destructive inflammatory process in bile ducts, leading to fibrosis and liver cirrhosis. It has two forms: 1) syndromic or fetal or embryonic (10-35%) with various congenital anomalies, 2) non syndromic (70-90%), in which BA is an isolated anomaly. In this article we report on an infant with the first form of BA in which diagnosis and operation was not based on routine liver biopsy but on associated malformations and clinical features that were highly suggestive of embryonal form of biliary atresia. Case Presentation: A 70-day old infant with syndromic BA, levocardia, situs inversus and polysplenia. He developed jaundice in 4th day of life, liver was not palpable. Kasai operation was not effective. He developed liver cirrhosis at 3 months of age.Conclusion: Syndromic type of EHBA is a very rare disease with a worse outcome than non syndromic type of BA. Early diagnosis is important and may be difficult as the liver sometimes could not be palpable because of its malposition.

Introduction: Menkes disease is a rare X-linked recessive disorder of copper metabolism. It is characterized by progressive cerebral degeneration with psychomotor deterioration, hypothermia, seizures and characteristic facial appearance with hair abnormalities. Case Presentation: We report on two cases of classical Menkes disease with typical history, (progressive psychomotor deterioration and seizures), clinical manifestations (cherubic appearance, with brittle, scattered and hypopigmented scalp hairs), and progression. Light microscopic examination of the hair demonstrated the pili torti pattern. The low serum copper content and ceruloplasmin confirmed the diagnosis.Conclusion: Menkes disease is an under-diagnosed entity, being familiar with its manifestation and maintaining high index of suspicion are necessary for early diagnosis.

Rotational deformities are common lower extremity abnormalities in children. Rotational problems include in-toeing and out-toeing. In-toeing is caused by one of the three types of deformity: metatarsus adductus, internal tibial torsion, and increased femoral anteversion. Out-toeing is less common than intoeing, and its causes are similar but opposite to those of intoeing. These include femoral retroversion and external tibial torsion. An accurate diagnosis can be made with careful history and physical examination, which includes torsional profile (a four- component composite of measurements of the lower extremities). Charts of normal values and values with two standard deviations for each component of the torsional profile are available. In most cases, the abnormality improves with time. A careful physical examination, explanation of the natural history, and serial measurements are usually reassuring to the parents. Treatment is usually conservative. Special shoes, cast, or braces are rarely beneficial and have no proven efficacy. Surgery is reserved for older children with deformity from three to four standard deviations from the normal.