Low-Dose Temporal Bone CT in Infants and Young Children: Effective Dose and Image Quality

The temporal bone is ideal for low-dose CT because of its intrinsic high contrast. The aim of this study was to retrospectively evaluate image quality and radiation doses of a new low-dose versus a standard high-dose pediatric temporal bone CT protocol and to review dosimetric data from the literature.

Prediction of codeine toxicity in infants and their mothers using a novel combination of maternal genetic markers

Substantial variation exists in response to standard doses of codeine ranging from poor analgesia to life-threatening central nervous system (CNS) depression. We aimed to discover the genetic markers predictive of codeine toxicity by evaluating the associations between polymorphisms in cytochrome P450 2D6 (CYP2D6), UDP-glucuronosyltransferase 2B7 (UGT2B7), P-glycoprotein (ABCB1), mu-opioid receptor (OPRM1), and catechol O-methyltransferase (COMT) genes, which are involved in the codeine pathway, and the symptoms of CNS depression in 111 breastfeeding mothers using codeine and their infants. A genetic model combining the maternal risk genotypes in CYP2D6 and ABCB1 was significantly associated with the adverse outcomes in infants (odds ratio (OR) 2.68; 95% confidence interval (CI) 1.61-4.48; P(trend) = 0.0002) and their mothers (OR 2.74; 95% CI 1.55-4.84; P(trend) = 0.0005). A novel combination of the genetic and clinical factors predicted 87% of the infant and maternal CNS depression cases with a sensitivity of 80% and a specificity of 87%. Genetic markers can be used to improve the outcome of codeine therapy and are also probably important for other opioids sharing common biotransformation pathways.

Nanoduct(R) sweat testing for rapid diagnosis in newborns, infants and children with cystic fibrosis

Determination of chloride concentration in sweat is the current diagnostic gold standard for Cystic Fibrosis (CF). Nanoduct(R) is a new analyzing system measuring conductivity which requires only 3 microliters of sweat and gives results within 30 minutes. The aim of the study was to evaluate the applicability of this system in a clinical setting of three children's hospitals and borderline results were compared with sweat chloride concentration. Over 3 years, 1,041 subjects were tested and in 946 diagnostic results were obtained. In 95 children, Nanoduct(R) failed (9.1% failure rate), mainly due to failures in preterm babies and newborns. Assuming 59 mmol/L as an upper limit of normal conductivity, all our 46 CF patients were correctly diagnosed (sensitivity 100%, 95% CI: 93.1-100; negative predicted value 100% (95% CI: 99.6-100) and only 39 non CF's were false positive (39/900, 4.3%; specificity 95.7%, 95%CI: 94.2-96.9, positive predicted value 54.1% with a 95%CI: 43.4-65.0). Increasing the diagnostic limit to 80 mmol/L, the rate fell to 0.3% (3/900). CF patients had a median conductivity of 115 mmol/L; the non-CF a median of 37 mmol/L. In conclusion, the Nanoduct(R) test is a reliable diagnostic tool for CF diagnosis: It has a failure rate comparable to other sweat tests and can be used as a simple bedside test for fast and reliable exclusion, diagnosis or suspicion of CF. In cases with borderline conductivity (60-80 mmol/L) other additional methods (determination of chloride and genotyping) are indicated.

The Ross procedure in infants and young children

BACKGROUND: This study reviews our experience with the Ross procedure in infants and young children. METHODS: From September 1993 to September 2004, 52 children less than 15 years of age underwent a Ross procedure. The patients ranged in age from 4 days to 15 years old (median, 5 years). Fifteen patients (29%) were less than 2 years of age. The predominant indication for the Ross procedure was aortic stenosis. Sixteen patients underwent a Ross-Konno procedure for severe left ventricular outflow tract obstruction. Thirty-four patients had 48 previous interventions. Preoperatively, 6 patients showed severe left ventricular dysfunction, and 2 of the patients required ventilation and inotropic support. Concomitant procedures were performed in 8 patients. Three patients had a mitral valve replacement, 2 patients had a ventricular septal defect closure and an aortic arch reconstruction, 2 patients had aortic arch reconstructions, and 1 patient had resection of a coarctation and a ventricular septal defect closure. RESULTS: Patients were followed up for a median of 43 months (range, 1 to 130). Overall survival was 85% +/- 5% at 1 and 82% +/- 5% at 2, 5, and 10 years. Hospital mortality was 5 of 52 patients (9.6%). All deaths occurred in neonates or infants less than 2 months of age, who needed urgent surgery. Three patients died late of noncardiac causes. At last follow-up, all patients were classified in New York Heart Association functional class I or II. No patient had endocarditis of the autograft or the right ventricular outflow tract replacement. During the follow-up, no event of thrombembolism was observed. No patient required the insertion of a permanent pacemaker. Overall freedom from reoperation is 57% +/- 15% at 10 years. One patient required the replacement of the autograft at 6 months postoperatively. The development of mild aortic insufficiency was observed in 24 patients, and moderate aortic insufficiency in 1 patient during follow-up. Freedom from reoperation for the right ventricular outflow tract replacement is 60% +/- 15% at 10 years. CONCLUSIONS: The Ross procedure represents an attractive approach to aortic valve disease in young children. However, a high early mortality rate has to be considered when performing this procedure in neonates or infants who present in critical preoperative condition.

Forced oscillation technique in infants and young children

Due to its non-invasive character, the forced oscillation technique has gained importance in clinical research in infants and young children. Standardisation has enabled systematic and comparable measurements to be made in different laboratories throughout the world. The theoretical conditions are now fulfilled for use of these techniques in the clinical environment. This review discusses the principles, usefulness and pitfalls of various forced oscillation techniques in a research and clinical environment and the present and future clinical applications in children. It will focus particularly on the role of infant and preschool lung function as forced oscillation only requires minimal cooperation.

Validation of multiple-breath washout equipment for infants and young children.

INTRODUCTION The new ATS/ERS consensus report recommends in vitro validation of multiple-breath inert gas washout (MBW) equipment based on a lung model with simulated physiologic conditions. We aimed to assess accuracy of two MBW setups for infants and young children using this model, and to compare functional residual capacity (FRC) from helium MBW (FRCMBW ) with FRC from plethysmography (FRCpleth ) in vivo. METHODS The MBW setups were based on ultrasonic flow meter technology. Sulfur hexafluoride and helium were used as tracer gases. We measured FRC in vitro for specific model settings with and without carbon dioxide and calculated differences of measured to generated FRC. For in vivo evaluation, difference between FRCMBW and FRCpleth was calculated in 20 healthy children, median age 6.1 years. Coefficient of variation (CV) was calculated per FRC. RESULTS In the infant model (51 runs, FRC 80-300 ml), mean (SD) relative difference between generated and measured FRCs was 0.7 (4.7) %, median CV was 4.4% for measured FRCs. In the young child model, one setting (8 runs, FRC 400 ml) showed a relative difference of up to 13%. For the remaining FRCs (42 runs, FRC 600-1,400 ml), mean (SD) relative difference was -2.0 (3.4) %; median CV was 1.4% for measured FRCs. In vivo FRCpleth exceeded FRCMBW values by 37% on average. CONCLUSIONS Both setups measure lung volumes in the intended age group reliably and reproducibly. Characteristics of different techniques should be considered when measuring lung volumes in vivo. Pediatr Pulmonol. © 2014 Wiley Periodicals, Inc.

Multiple breath washout is feasible in the clinical setting and detects abnormal lung function in infants and young children with cystic fibrosis.

BACKGROUND Cystic fibrosis (CF) lung disease starts in the first months of life often before the onset of clinical symptoms. Multiple breath washout (MBW) detects abnormal lung function in infants and young children in the laboratory setting. OBJECTIVE The aim of this study was to determine the feasibility of MBW in 0- to 4-year-old children with CF and non-CF controls in the clinical setting. METHODS Fourteen children with CF (mean age 1.3 ± 1.0 years) and 26 age-matched non-CF controls were sedated with chloral hydrate and MBW was performed with sulfur hexafluoride. RESULTS MBW measurements were successful in 27 of 40 children (67.5%). The mean lung clearance index (LCI) was significantly higher in CF patients compared to non-CF controls (p = 0.006). Further, the frequency of elevated LCI (z-score >1.96) was significantly increased in CF patients compared to controls (p = 0.0003). CONCLUSIONS We conclude that MBW is feasible and sensitive to detect abnormal lung function in infants and young children with CF in the clinical setting.

Transient Benign Hyperphophatasemia - A Systematic Review of The Literature

BACKGROUND:: Sometimes, a temporary increase in alkaline phosphatase level is found in healthy infants and toddlers without evidence of liver or bone disease. The condition is customarily termed transient benign hyperphosphatasemia of infancy and early childhood. Most textbooks do not refer to the condition. METHODS:: We completed a systematic review of the literature using the principles underlying the UK Economic and Social Research Council guidance on the conduct of narrative synthesis and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. RESULTS:: The 142 reports retained for analysis included 813 cases (male:female ratio = 1.1:1.0): 80 in subjects >18 years and 733 in subjects ≤18 years of age. The alkaline phosphatase ratio, calculated by dividing the measured level by the upper normal limit, was ≥5.0 in ≈70% and the duration of the elevation ≤4 months in 80% of the cases. Transient benign hyperphosphatasemia often followed a benign infection but available data fail to demonstrate a causal link. The prevalence of transient benign hyperphosphatasemia ranged 1.1-3.5% in infants 2 to 24 months of age. CONCLUSIONS:: Transient benign hyperphosphatasemia is likely the most common cause of hyperphosphatasemia among healthy infants and toddlers. Sometimes, it also occurs in older children and adults, indicating that the traditional term transient benign hyperphosphatasemia of infancy and early childhood might not be correct. The elevation in alkaline phosphatase persists for >4 months in ≈20% of the cases. Recognition of this benign condition is crucial to avoid unnecessary investigations.

[Examination of urine in the child]

The examination of urine in children can be very complex, due to the difficulty to obtain clean urine specimens in infants and toddlers. Clean catch is an easy system to obtain urine but patience is needed. Transurethral catheterization or suprapubic aspiration is useful in infants and toddlers with sign of pyelonephritis. Urine bag specimens are not useful in the diagnosis of urinary tract infection because of the high rate of false positive cultures. The 24 hours urine collection is frequently replaced by a spot urine and the ratio of the measured substances with the urine creatinine are calculated. Urine microscopy is needed for the evaluation of pathological results in the dipstick testing: confirm that red urine is due to haematuria by demonstration of red blood cells on urine microscopy, dysmorphic cells and red-cell casts are pathognomonic of glomerular bleeding, white-cell casts signify glomerular inflammation and bacteria are easily seen in unstained urine. A urine culture is pathologic if the colony count exceeds 10(4) in the transurethral catheterization or clean void. In the suprapubic aspiration is any number of colony pathologic. Urate crystals in the urine of infants may cause a pink discoloration to nappies. Urine screenings are not very useful and should be performed only at the age of 5 years or by sexual-active adolescents.

Validation of the LittlEARS((R)) Auditory Questionnaire in children with normal hearing

OBJECTIVES: With more children receiving cochlear implants during infancy, there is a need for validated assessments of pre-verbal and early verbal auditory skills. The LittlEARS Auditory Questionnaire is presented here as the first module of the LittlEARS test battery. The LittlEARS Auditory Questionnaire was developed and piloted to assess the auditory behaviour of normal hearing children and hearing impaired children who receive a cochlear implant or hearing aid prior to 24 months of age. This paper presents results from two studies: one validating the LittlEARS Auditory Questionnaire on children with normal hearing who are German speaking and a second validating the norm curves found after adaptation and administration of the questionnaire to children with normal hearing in 15 different languages. METHODS: Scores from a group of 218 German and Austrian children with normal hearing between 5 days and 24 months of age were used to create a norm curve. The questionnaire was adapted from the German original into English and then 15 other languages to date. Regression curves were found based on parental responses from 3309 normal hearing infants and toddlers. Curves for each language were compared to the original German validation curve. RESULTS: The results of the first study were a norm curve which reflects the age-dependence of auditory behaviour, reliability and homogeneity as a measure of auditory behaviour, and calculations of expected and critical values as a function of age. Results of the second study show that the regression curves found for all the adapted languages are essentially equal to the German norm curve, as no statistically significant differences were found. CONCLUSIONS: The LittlEARS Auditory Questionnaire is a valid, language-independent tool for assessing the early auditory behaviour of infants and toddlers with normal hearing. The results of this study suggest that the LittlEARS Auditory Questionnaire could also be very useful for documenting children's progress with their current amplification, providing evidence of the need for implantation, or highlighting the need for follow-up in other developmental areas.

Lung-function tests in neonates and infants with chronic lung disease: tidal breathing and respiratory control

This paper is the fourth in a series of reviews that will summarize available data and critically discuss the potential role of lung-function testing in infants with acute neonatal respiratory disorders and chronic lung disease of infancy. The current paper addresses information derived from tidal breathing measurements within the framework outlined in the introductory paper of this series, with particular reference to how these measurements inform on control of breathing. Infants with acute and chronic respiratory illness demonstrate differences in tidal breathing and its control that are of clinical consequence and can be measured objectively. The increased incidence of significant apnea in preterm infants and infants with chronic lung disease, together with the reportedly increased risk of sudden unexplained death within the latter group, suggests that control of breathing is affected by both maturation and disease. Clinical observations are supported by formal comparison of tidal breathing parameters and control of breathing indices in the research setting.

Preference for cute infants does not depend on their ethnicity or species: Evidence from hypothetical adoption and donation paradigms

Results of previous work suggest a preference of adult observers for cute compared to less cute infants. In Study 1 we investigated whether the preference for cute infants depends on the ethnicity and species of the infant. We simultaneously presented two faces (one cute and one less cute) and asked Caucasian participants to choose the infant to whom they would rather give a toy (Task 1) and which infant they would rather adopt (Task 2). The infants were Caucasian or African human babies, or dog puppies. For all face categories and in both tasks we found a strong preference for cute infants. A possible reason for preferring cute infants may be that cute infants look healthier than less cute infants. To investigate whether cuteness is associated with the assessment of health we conducted Study 2. Faces of Caucasian and African infants and dog puppies were rated for cuteness and health. The findings revealed a significant relationship between health and cuteness evaluation across all stimuli. We suggest that one reason why cute infants are preferred might be because they are perceived as being healthier.