Thyroid Hormone Levels in Preterm Neonates with Birth Weight Less than 2500 g, Treated with Phenobarbital.

Objectives: Indicatively, phenobarbital can impair thyroid function in adults and children. The present research aims to evaluate the thyroid hormone levels in preterm neonates who had received phenobarbital treatment. Materials &Methods: This study was conducted on preterm neonates who weighed less or equal to 2500 g when phenobarbital was prescribed for treatment in the first 15 days of life. TSH and total T4 measurements were performed before and three months after initiation of phenobarbital. Results: In this study, the sum of preterm neonates stood at 94, of which 53 were girls, with a mean birth weight of 2004.41 ± 315.41g. Weight of 8.5% were under 1500 g. The mean gestational age was estimated at 33.64 ± 2.01 weeks. Mean T4 levels were 12.24 ± 1.96 and 12.07 ± 1.95 (p=0.334), and mean TSH levels were 5.34 ± 2.14 and5.15 ± 2.15 (p=0.376) before and after prescribing phenobarbital, respectively. The same results were compared based on sex, gestational age, birth weight, and height for T4 and TSH and T4 based on head circumference. The only significant difference was TSH in preterm infants with head circumference <32 cm before and after prescribing phenobarbital (p=0.030). Conclusion: In preterm newborns that had less than 2500 g birth weight, phenobarbital did not significantly alter the serum thyroid hormone levels.

Evaluation of therapeutic effect of oral Ursodeoxycholic Acid on indirect hyperbilirubinemia in term neonates undergoing phototherapy: A randomized controlled clinical trial.

INTRODUCTION AND AIMS: Phototherapy is the most common treatment modality of neonatal hyperbilirubinemia. We aimed to evaluate the therapeutic effect of oral Ursodeoxycholic Acid (UDCA) on indirect hyperbilirubinemia in term neonates undergoing phototherapy. MATERIALS AND METHODS: This randomized controlled clinical trial was performed on 106 full-term neonates with jaundice who were admitted to the neonatal ward of 17 Shahrivar Hospital in Rasht, Iran. The neonates were randomly assigned to two groups of intervention (10 mg/kg UDCA+phototherapy) and control (phototherapy alone). Total serum bilirubin (TSB) was measured at the time of admission, during first 12, 24, and 48 hours after admission and at the time of discharge. The duration of hospitalization and side effects were also assessed in both groups. IBM SPSS Statistics for Windows, version 20 was used to analyze the data. RESULTS: Results showed that in the intervention group, 28 (52.8%) of neonates were boys with the mean age of 5.1±1.25 days. While, in the control group 29 (54.7%) of them were boys with the mean age of 5.19±2.26 days. Bilirubin levels in both groups decreased significantly after hospitalization (at 12, 24 and 48 hours) (P <0.001). The mean of bilirubin at 12, 24 and 48 hours in the intervention and control groups were 17.1, 13.2, 10.2 mg / dl and 17.1, 14.2 and 11.3 mg / dl, respectively. At the time of discharge, TSB in the former compared to the latter group was significantly reduced (7.74± 1.39 vs. 8.67±1.35) (P = 0.001). In addition, the duration of hospitalization was considerably shorter in the intervention compared to the control group (P = 0.038) and no side effects were observed. CONCLUSIONS: Administering UDCA plus phototherapy reduced TSB and length of hospital stay with proper safety and efficacy. Therefore, it seems that this combination can be an appropriate treatment modality in neonatal hyperbilirubinemia.

Neurofibromatosis-Noonan syndrome and growth deficiency in an Iranian girl due to a pathogenic variant in NF1 gene.

BACKGROUND: Mutations in NF1 gene could cause allelic disorders with clinical spectrum of Neurofibromatosis type 1 to Noonan syndrome. Here, a 7-year-old Iranian girl is described with Neurofibromatosis-Noonan syndrome due to a pathogenic variant in NF1 gene. METHODS: Clinical evaluations were performed along with genetic testing using whole exome sequencing (WES). The variant analysis including pathogenicity prediction was also done using bioinformatics tools. RESULTS: The chief compliant of the patient was short stature and lack of proper weight gain. Other symptoms were developmental delay, learning disability, inadequate speech skill, broad forehead, hypertelorism, and epicanthal folds, low set ears and webbed neck. A small deletion, c.4375-4377delGAA, was found in NF1 gene using WES. This variant was classified as pathogenic according to ACMG. CONCLUSIONS: NF1 variants may show variable phenotypes among the patients; identifying such variants is helpful in therapeutic management of the disease. WES is considered as an appropriate test to diagnose Neurofibromatosis-Noonan syndrome.

Diagnostic value of IL-6, CRP, WBC, and absolute neutrophil count to predict serious bacterial infection in febrile infants.

Since clinical manifestations of most febrile infants younger than three months old are nonspecific, differentiation of Serious Bacterial Infection (SBI) from self-limiting viral illness is a significant challenge for pediatricians. This study was performed to assess the diagnostic value of white blood cell count (WBC), Absolute Neutrophil Count (ANC), Interleukin -6 (IL-6) and C-reactive protein (CRP) level to predict SBI in febrile infants younger than three months old who were hospitalized. This was a diagnostic test validation study. In this prospective study, 195 febrile infants admitted to 17 Shahrivar Hospital underwent a full sepsis workup including blood, urine, cerebrospinal fluid cultures and chest radiography. WBC count, ANC and CRP and Il-6 level were measured in all patients. Serum IL-6 concentration was measured by Enzyme-linked Immunosorbent Assay test. Then diagnostic, values of these tests for predicting SBI was compared with each other. Of total cases, 112 (57.4%) infants were male. SBI was diagnosed in 29 (14.9%) patients. The most common type of SBI was Urinary Tract Infection (UTI). Serum IL-6 (³20pg/dl) had sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of 79/1%, 91.6%,75.4%, 60.3%, respectively and for CRP (³ 10mg/l) values were 81.6%, 89.8%, 78.2%, and 52%,respectively. The predictive values of CRP and IL-6 were higher than WBC and ANC. IL-6 and CRP are more valid and better diagnostic markers for predicting SBI than WBC count and ANC. CRP level seems to be an accessible and cost-effective marker for early diagnosis of SBI. Since by no marker we can totally rule out SBI in febrile infants < three months of age, it is recommended to administer systemic antibiotics until culture results become available.

Biotinidase Deficiency in Newborns as Respiratory Distress and Tachypnea: A Case Report.

OBJECTIVE: Biotin is a coenzyme composed of four carboxylases. It presents in amino acid catabolism, fatty acid synthesis, and gluconeogenesis. Biotinidase recycles the vitamin biotin. A biotinidase deficiency is a neurocutaneous disorder with autosomal recessive inheritance. The symptoms can be successfully treated or prevented by administering pharmacological doses of biotin. Although, according to neonatal prenatal medicine (2011), a biotinidase deficiency does not manifest during the neonatal period. In this study, we report on a case of biotinidase deficiency in the first week of birth. CASE REPORT: A 3100 g term boy was born via cesarean section. After 3 days, he was referred to the 17th Shahrivar Hospital with the chief complaint of tachypnea and grunting. Laboratory results revealed that liver and renal function tests, serum electrolytes, and blood indexes except ammonia were all normal. Within few days after the administration of oral biotin, the patient showed dramatic improvement and was discharged. However, within 4 months he was admitted two other times with the complaints of diarrhea and pneumonia. Unfortunately, he expired after 4 months. According to our results, it seems that clinicians should accurately assess suspicious patients and even assess infants for biotinidase deficiency.

Incidence and Risk Factors for Retinopathy of Prematurity in North of Iran.

PURPOSE: To report the incidence and risk factors for retinopathy of prematurity (ROP) among preterm infants referred to Amiralmomenin Eye Hospital, Rasht, Iran. METHODS: This cross-sectional retrospective study included all preterm infants with birth weight ≤2500 g and/or gestational age ≤36 weeks who had been referred to our facility for ROP screening over a five year period from September 2005 to September 2010. Possible risk factors and findings related to eye examinations were extracted and analyzed. RESULTS: Among 310 infants, ROP was diagnosed in 64 (20.6%) of referred preterm infants (95% CI: 17.7%-23.5%); these included stage I in 48%, stage 2 in 29%, and stage 3 or higher disease in 23% of subjects. Mean gestational age (GA) and birth weight (BW) in the ROP-affected infants was 30.18 ± 2.28 weeks and 1,422.8 ± 420.8 g, respectively. Low BW, low GA, oxygen therapy, phototherapy, blood transfusion and apnea were risk factors for ROP. After logistic regression analysis, only low GA and low BW were independently associated with the condition. CONCLUSION: ROP is a relatively common finding in preterm infants of Guilan Province in the North of Iran. Low BW and low GA were significant risk factors for the disease.

Entrobacter, the most common pathogen of neonatal septicemia in rasht, iran.

OBJECTIVE: Bacterial sepsis continues to be a major cause of morbidity and mortality in newborns. Bacterial pathogens of neonatal septicemia may vary from one country to another and within a country from one hospital or region to another. Both gram-negative and gram-positive bacteria are responsible in neonatal sepsis. This study was undertaken to determine the prevalent bacterial agents of neonatal sepsis and their antimicrobial susceptibility in a teaching hospital, Rasht, from February 2008 to February 2010. METHODS: This prospective study includes 611 newborns admitted with the probable diagnosis of septicemia. We studied the cases with positive blood culture, the pathogens and antibiotic resistance to different antibiotics. FINDINGS: Among 611 hospitalized newborns, 64 (10.6%) cases had positive blood culture. The commonest pathogens were Entrobacter (78.1%) and Klebsiella (6.2%). CONCLUSION: According to the results, low birth weight and prematurity were associated with higher risk of sepsis significantly. The most common pathogen was Enterobacter. Treatment with effective antibiotics (e.g. gentamicin, cost effective and easily available) and hygienic care in the neonatal unit are recommended to eliminate the infectious factors especially Entrobacter.

Assessment of febrile neonates according to low risk criteria for serious bacterial infection.

OBJECTIVE: It is propounded that febrile neonates with low risk criteria (LRC) can be carefully observed without parenteral antimicrobial therapy; but yet, reliability of LRC to exclude serious bacterial infection (SBI) is uncertain. METHODS: The records of all febrile term neonates, seen in the emergency room and admitted in neonatal ward of 17 Shahrivar children's hospital of Rasht, Iran from January 2004 to January 2009 were reviewed. All of them underwent full sepsis workup. The prevalence of SBI in total population and LRC positive and negative neonates were calculated FINDINGS: A total of 202 records of previously healthy febrile neonates were evaluated. SBI was shown in 38 (18.8%). The most common type of SBI was urinary tract infection (UTI). Sixty-two (31%) neonates had LRC, and only one (1.6%) had SBI (UTI with E. coli). SBI was significantly more common in neonates without LRC (26.6% versus 1.6%, P<0.001). The negative predictive value (NPV) of LRC to exclude SBI was 98.4% ((95%)confidence interval: 96.7% to 100%). CONCLUSION: These findings suggest that LRC may be relied upon to exclude SBI in febrile neonates. We propose that all febrile neonates be admitted, ill or LRC negative neonates should undergo a full sepsis work up and be administered systemic antibiotics immediately. LRC positive neonates should be under close observation.