The Added Value of the Lateral Chest Radiograph for Diagnosing Community Acquired Pneumonia in the Pediatric Emergency Department.

BACKGROUND: Opinions differ as to the need of a lateral radiograph for diagnosing community acquired pneumonia in children referred to the emergency department. A lateral radiograph increases the ionizing radiation burden but at the same time may improve specificity and sensitivity in this population. OBJECTIVES: To determine the value of the frontal and lateral chest radiographs compared to frontal view stand-alone images for the management of children with suspected community acquired pneumonia seen in a pediatric emergency department. METHODS: Chest radiographs from 451 children with clinically suspected pneumonia were retrospectively reviewed. Interpretation of frontal views was compared to interpretation of combined frontal and lateral view, the latter being the gold standard. RESULTS: Findings consistent with bacterial pneumonia were diagnosed in 94 (20.8%) of the frontal stand-alone radiographs and in 109 (24.2%) of the combined frontal and lateral radiographs. The sensitivity, specificity, positive predictive value, and negative predictive value of the frontal radiograph alone were 86.2%, 93.9%, 81.7%, and 95.5%, respectively. False positive and false negative rates were 15% and 21%, respectively, for the frontal view alone. The number of lateral radiographs needed to diagnose one community acquired pneumonia was 29. CONCLUSIONS: The lateral chest radiograph improves the diagnosis of pediatric community acquired pneumonia to a certain degree and may prevent overtreatment with antibiotics.

[Near fatal attraction of ingested magnets].

We report a case of intestinal perforation in a 20 month old girl following the ingestion of 2 small magnets. Ingestion of multiple magnets constitutes a unique problem. Magnets in adjacent intestinal loops may forcefully attract each other and produce pressure necrosis of the bowel wall, leading to perforation, fistula formation or intestinal obstruction. Therefore, these children should be observed carefully. Early surgical intervention should be considered when clinical symptoms develop, especially when, on sequential abdominal radiographs, there is no change in the magnets' location. Since toys with small magnets are ubiquitous, efforts should be made to increase parents' awareness on the one hand, and to alert toy manufacturers on the other hand.

Who should be reading chest radiographs in the pediatric emergency department?

BACKGROUND: Pediatric emergency department chest radiographs are frequently interpreted by the pediatrician, and patient management is based on this interpretation. OBJECTIVE: The purpose of this study was to assess the chest radiograph interpretation discordance rate between pediatricians and pediatric radiologists and determine its clinical significance. METHODS: This study included 1196 patients admitted off-hours to the pediatric emergency department who had chest radiographs as part of the clinical evaluation. The pediatricians' interpretations of the chest radiographs as appeared in the discharge reports were reviewed and compared with the specialist radiologist's ultimate report. RESULTS: Discordant chest radiograph interpretations were found in 332 (28%) of 1196 patients. Of these, 76 (6.3%) were clinically significant. These included 59 initial diagnoses of normal or viral patterns subsequently interpreted as bacterial pattern by the radiologist and 17 radiographs with findings such as chronic lung disease or abnormal heart shadow overlooked or misinterpreted by the pediatrician. CONCLUSIONS: Although the clinically significant discordant rate was relatively low, daily chest radiograph reassessment by pediatric radiologists in a joint meeting with pediatricians has an added value for patient safety, quality assurance, and mutual training.

Alice in Wonderland syndrome in H1N1 influenza: case report.

The different aspects of the global H1N1 influenza and its complications are currently of great interest. Neurological complications of the disease and its frequency are still unknown. We report a case of an 11-year-old girl who developed Alice in Wonderland syndrome associated with H1N1 influenza. This unique clinical syndrome was previously described in other diseases. The clinician's awareness of the existence of this syndrome in H1N1 influenza might save the child from undergoing extensive diagnostic procedures.

Lessons learned from chlorine intoxications in swimming pools: the challenge of pediatric mass toxicological events.

INTRODUCTION: The classical doctrine of mass toxicological events provides general guidelines for the management of a wide range of "chemical" events. The guidelines include provisions for the: (1) protection of medical staff with personal protective equipment; (2) simple triage of casualties; (3) airway protection and early intubation; (4) undressing and decontamination at the hospital gates; and (5) medical treatment with antidotes, as necessary. A number of toxicological incidents in Israel during the summer of 2005 involved chlorine exposure in swimming pools. In the largest event, 40 children were affected. This study analyzes its medical management, in view of the Israeli Guidelines for Mass Toxicological Events. METHODS: Data were collected from debriefings by the Israeli Home Front Command, emergency medical services (EMS), participating hospitals, and hospital chart reviews. The timetable of the event, the number and severity of casualties evacuated to each hospital, and the major medical and logistical problems encountered were analyzed according to the recently described methodology of Disastrous Incident Systematic Analysis Through-Components, Interactions, Results (DISAST-CIR). RESULTS: The first ambulance arrived on-scene seven minutes after the first call. Emergency medical services personnel provided supplemental oxygen to the victims at the scene and en route when required. Forty casualties were evacuated to four nearby hospitals. Emergency medical services classified 26 patients as mildly injured, 13 as mild-moderate, and one as moderate, suffering from pulmonary edema. Most children received bronchodilators and steroids in the emergency room; 20 were hospitalized. All were treated in pediatric emergency rooms. None of the hospitals deployed their decontamination sites. CONCLUSIONS: Event management differed from the standard Israeli toxicological doctrine. It involved EMS triage of casualties to a number of medical centers, treatment in pediatric emergency departments, lack of use of protective gear, and omission of decontamination prior to emergency department entrance. Guidelines for mass toxicological events must be tailored to unique scenarios, such as chlorine intoxications at swimming pools, and for specific patient populations, such as children. All adult emergency departments always should be prepared and equipped for taking care of pediatric patients.

Exercise challenge test in 3- to 6-year-old asthmatic children.

RATIONALE: The exercise challenge test (ECT) is a common tool to assess exercise-induced asthma (EIA) in school-aged children. EIA has not been explored in the early childhood setting. OBJECTIVE: To assess the existence of EIA in children in this age group. MEASUREMENTS AND MAIN RESULTS: A 6-min, controlled, free-run test was performed in 55 children (age range, 3 to 6 years old) who were classified into the following groups: 30 children in whom asthma had been previously diagnosed (group A); and 25 children with prolonged coughing (group B). Spirometry measurements were obtained before the run, and at 1, 2, 3, 5, 10, and 20 min after the run. A positive finding of EIA was defined as a 13% decrease from baseline FEV(1) or baseline forced expiratory volume in the first 0.5 s (FEV(0.5)). The actual duration of each run was age-related (mean [+/- SD] duration, 4.8 +/- 0.8 min). The nadir in indexes occurred after a mean time of 2.98 +/- 1.31 min. A positive EIA finding determined by FEV(1) was present in 15 children, and by FEV(0.5) in 34 children. Twenty-six children were from group A, but only 8 children were from group B. Wheezing and/or prolonged expiration were associated with a positive test result in 31 of 34 children. Coughing was frequent in children with both negative and positive ECT findings. CONCLUSION: The present study documents for the first time the presence of EIA in response to a free-run test in early childhood. Our findings suggest that a free-run test for the presence of EIA is suitable, but that the running duration is limited by age. The duration of airflow limitation after exercise is significantly earlier and shorter in young children with asthma compared with older children. FEV(0.5) is a better index than the traditional FEV(1) for describing positive ECT results in young children. The association of wheezing and/or prolonged expiration may help in defining EIA in early childhood in the absence of a spirometer.

Spirometry in early childhood in cystic fibrosis patients.

BACKGROUND: Spirometry data in cystic fibrosis (CF) patients in early childhood is scarce, and the ability of spirometry to detect airways obstruction is debatable. OBJECTIVE: To evaluate the ability of spirometry to detect airflow obstruction in CF patients in early childhood. METHODS: CF children (age range, 2.5 to 6.9 years) in stable clinical condition were recruited from five CF centers. The children performed guided spirometry (SpiroGame; patented by Dr. Vilzone, 2003). Spirometry indices were compared to values of a healthy early childhood population, and were analyzed with relation to age, gender, and clinical parameters (genotype, pancreatic status, and presence of Pseudomonas in sputum or oropharyngeal cultures). RESULTS: Seventy-six of 93 children tested performed acceptable spirometry. FVC, FEV1, forced expiratory flow in 0.5 s (FEV0.5), and forced expiratory flow at 50% of vital capacity (FEF50) were significantly lower than healthy (z scores, mean +/- SD: - 0.36 +/- 0.58, - 0.36 +/- 0.72, - 1.20 +/- 0.87; and - 1.80 +/- 1.47, respectively; p < 0.01); z scores for FEV1 and FVC were similar over the age ranges studied. However, z scores for FEV0.5 and forced expiratory flow at 25 to 75% of vital capacity were significantly lower in older children compared to younger children (p < 0.001), and a higher proportion of 6-year-old than 3-year-old children had z scores that were > 2 SDs below the mean (65% vs 5%, p < 0.03). Girls demonstrated lower FEF50 than boys (z scores: - 2.42 +/- 1.91 vs - 1.56 +/- 1.23; p < 0.001). Clinical parameters evaluated were not found to influence spirometric indices. CONCLUSIONS: Spirometry elicited by CF patients in early childhood can serve as an important noninvasive tool for monitoring pulmonary status. FEV0.5 and flow-related volumes might be more sensitive than the traditional FEV1 in detecting and portraying changes in lung function during early childhood.

N-terminal pro B-type natriuretic peptide (N-BNP) levels in cystic fibrosis patients.

BACKGROUND: The diagnosis of right heart failure and pulmonary hypertension in cystic fibrosis (CF) patients with advanced pulmonary disease is sometimes difficult on clinical grounds alone. B-type natriuretic peptide (BNP) & N-terminal pro-B-type natriuretic peptide (N-BNP) levels were found to be useful in differentiating heart failure from various pulmonary diseases. However, its level was never measured in CF patients. The aim of this study was to measure N-BNP level in CF patients without heart failure. METHODS: The study included 49 patients. Of these, 32 had CF and 17 were control subjects who were matched by age and sex variables to the study group. We looked for a correlation between N-BNP and lung function test, genetic profile, height percentiles, and weight percentiles. N-BNP level was measured using an immunoassay that contains polyclonal antibodies that recognize epitopes located in the N-terminal part of proBNP. RESULTS: N-BNP level among CF patients without heart failure, after age and sex adjustments, was similar to the control group (Median: 47 pg/ml vs. 38 pg/ml, P = 0.248, interquartile range: 33-99 pg/ml vs. 31-76 pg/ml). A correlation between N-BNP level to age was found in both groups (CF: R = -0.398; P = 0.024, CONTROL: R = -0.054; P = 0.024). There was no correlation between N-BNP level to FEV1, O2 saturation and nutritional status. Among CF patients, eight (25%) had a mildly elevated N-BNP level whereas none was found in the control group (P = 0.038). CONCLUSION: We conclude that N-BNP level among CF patients is similar to the normal population and that it has no correlation to lung function impairment. Therefore, measurement of elevated N-BNP level in CF patients might be a predictor to the development of pulmonary hypertension and heart failure.

The impact of educational intervention programs on pain management in a pediatric emergency department.

Management of pain and anxiety is an important part of patient care in the pediatric emergency department (ED). Even though it has improved significantly over the past few years, it is still suboptimal. The objective of this study was to evaluate the effect of informal and formal education on pain and anxiety management in the pediatric ED. Management of pain and anxiety was assessed by comparing the use of analgesics and sedatives during three phases: A) year 2000 (baseline), B) years 2001-2002 (informal teaching) and C) year 2004 (following a structured simulation-based training in pediatric sedation and analgesia). During period B there was a significant increase in the yearly use of eutectic mixture of local anesthetics (EMLA) (RR=2.63, CI 1.23-5.6), ibuprofen (RR=14.16, CI 8.73-22.98), midazolam (RR=1.68, CI 1.39-2.03) and nitrous oxide (N2O) in comparison with period A, with an additional increment of the first three medicines during period C. There was no change in the use of ketamine, morphine and meperidine during period B. Whereas, during period C, a significant increase in the use of ketamine and morphine was demonstrated (RR=24.56, CI 10.71-56.3 and RR=3.07, CI 2.12-4.44, respectively), while the use of meperidine (RR=0.68, CI 0.49-0.94) and N2O (RR=0.46, 95% CI 0.32-0.67) declined significantly. Educational interventions have a clear impact on pain and anxiety management demonstrated by the subsequent change in the use of sedatives and analgesics and should be provided to pediatric ED physicians. Informal teaching affected mainly the use of milder sedatives and analgesics, while formal structured training influenced the use of opioids and dissociative agents.

The practice of pediatric procedural sedation and analgesia in the emergency department.

The treatment of acute pain and anxiety in children undergoing therapeutic and diagnostic procedures in the emergency department has improved dramatically over the last few years. The availability of noninvasive monitoring devices and the use of short-acting sedative and analgesic medications enable physicians to conduct safe and effective sedation and analgesia treatment. In today's practice of pediatric emergency medicine, sedation and analgesia has been considered as the standard of care for procedural pain. The following article describes the basic principles of pediatric procedural sedation and analgesia in the emergency department.

The role of computer games in measuring spirometry in healthy and "asthmatic" preschool children.

STUDY OBJECTIVES: To explore the role of respiratory interactive computer games in teaching spirometry to preschool children, and to examine whether the spirometry data achieved are compatible with acceptable criteria for adults and with published data for healthy preschool children, and whether spirometry at this age can assess airway obstruction. DESIGN: Feasibility study. SETTINGS: Community kindergartens around Israel and a tertiary pediatric pulmonary clinic. PARTICIPANTS: Healthy and asthmatic preschool children (age range, 2.0 to 6.5 years). INTERVENTION: Multi-target interactive spirometry games including three targets: full inspiration before expiration, instant forced expiration, and long expiration to residual volume. MEASUREMENTS AND RESULTS: One hundred nine healthy and 157 asthmatic children succeeded in performing adequate spirometry using a multi-target interactive spirometry game. American Thoracic Society (ATS)/European Respiratory Society spirometry criteria for adults for the start of the test, and repeatability were met. Expiration time increased with age (1.3 +/- 0.3 s at 3 years to 1.9 +/- 0.3 s at 6 years [+/- SD], p < 0.05). FVC and flow rates increased with age, while FEV1/FVC decreased. Healthy children had FVC and FEV1 values similar to those of previous preschool studies, but flows were significantly higher (> 1.5 SD for forced expiratory flow at 50% of vital capacity [FEF50] and forced expiratory flow at 75% of vital capacity [FEF75], p < 0.005). The descending part of the flow/volume curve was convex in 2.5- to 3.5-year-old patients, resembling that of infants, while in 5- to 6-year-old patients, there was linear decay. Asthma severity by Global Initiative for Asthma guidelines correlated with longer expiration time (1.7 +/- 0.4 s; p < 0.03) and lower FEF50 (32 to 63%; p < 0.001) compared to healthy children. Bronchodilators improved FEV1 by 10 to 13% and FEF50 by 38 to 56% of baseline. CONCLUSIONS: Interactive respiratory games can facilitate spirometry in very young children, yielding results that conform to most of the ATS criteria established for adults and published data for healthy preschool children. Spirometric indexes correlated with degree of asthma severity.

Pregnancies and outcome in women with cystic fibrosis.

BACKGROUND: Along with the increased life expectancy in cystic fibrosis and the remarkable progress in its management and therapy, issues of female fertility and pregnancy are frequently raised. These include infertility, severity of lung disease, pancreatic insufficiency, poor nutritional status, glucose intolerance and diabetes, drug safety, and long-term maternal and neonatal outcome. OBJECTIVE: To describe the experience of our CF center in the management of CF pregnant woman from 1977 to 2004. METHODS: We analyzed 27 years of records (1977-2004) of the national CF registry of all CF women who wished to conceive and became pregnant. RESULTS: Eight CF women (mean age 24 +/- 4.5 years) who wished to conceive had 11 pregnancies and delivered 12 neonates. The pregestational results of forced expiratory volume per 1 second varied significantly among patients (59 +/- 23%), yet most (10/11) stayed stable throughout the pregnancy course. Maternal deterioration in CF condition occurred in only one mother, necessitating cesarean section. In 9 of the 11 pregnancies the women were pancreatic-insufficient. Of the 11 pregnancies, 2 CF women had diabetes mellitus and 3 developed gestational diabetes. One pregnancy occurred in a mother with a transplanted lung. Of the 12 neonates, 3 were preterm and one was born with esophageal atresia. No miscarriages, terminations or neonatal mortalities occurred. Although most of the CF mothers had FEV1 below 55% before pregnancy, the maternal and neonatal outcome was favorable and lung function tests generally remained stable. CONCLUSIONS: We conclude that pregnancy in CF is feasible with a positive maternal and neonatal outcome. Early participation of the CF physician in the wish of the CF woman to reproduce is required. The integration of an intensive multidisciplinary approach during pregnancy, which includes close follow-up of maternal and fetal condition by the various specialists, should ensure an optimal outcome.

Back-to-school upper respiratory infection in preschool and primary school-age children in Israel.

BACKGROUND: Increased upper respiratory infection (URI) among children at the beginning of school year is well known to parents and pediatricians. However, this phenomenon is not well documented or characterized. METHODS: Computerized datasets from a large health maintenance organization in Israel were used to calculate the weekly rates of URI among children 3-14 years old for the years 2007-2012. In addition, nasopharyngeal swabs were collected in 2010-2012 from children with URI symptoms and controls during school opening time. Swabs were tested by real-time polymerase chain reaction for the presence of respiratory viruses. RESULTS: Time-series analysis demonstrated a peak of URI in September each year. The peaks reached their height 2 weeks after school opening and returned to baseline within 4-7 weeks. The main 3 viruses detected both in URI patients and in healthy controls during the first weeks of school opening were rhinovirus, adenovirus and enterovirus. The detection rate of any respiratory virus, and of rhinovirus in particular, was significantly higher among cases than among controls (54% vs. 16%, P < 0.001 for any virus, and 35% vs. 6.0%, P < 0.01 for rhinovirus). When adjusting for age and sex cases had 5.8 times more viral detection when compared with controls. Upper respiratory symptoms were significantly more prevalent among the virus-positive cases when compared with negative ones. CONCLUSIONS: Back-to-school illness consisting of URI has a distinct epidemiological pattern demonstrating a rapid rise peaking within 2 weeks of school opening and is associated predominantly with rhinovirus.

Endobronchial adrenaline: should it be reconsidered? Dose response and haemodynamic effect in dogs.

BACKGROUND: Tracheal drug administration is a route for drug delivery during cardiopulmonary resuscitation when intravenous access is not immediately available. However, tracheal adrenaline (epinephrine) injection has been recently shown to be associated with detrimental decrease in blood pressure. This was attributed to exaggerated early beta2 mediated effects unopposed by alpha-adrenergic vasoconstriction. We hypothesized that endobronchial adrenaline administration is associated with better drug absorption, which may abolish the deleterious drop of blood pressure associated with tracheal drug administration. OBJECTIVE: To determine haemodynamic variables after endobronchial adrenaline administration in a non-arrest canine model. DESIGN: Prospective, randomized, laboratory study. METHODS: Adrenaline (0.02, 0.05, 0.1 mg/kg) diluted with normal saline was injected into the bronchial tree of five anaesthetized dogs. Injection of 10-ml saline served as control. Heart rate, blood pressure and arterial blood gases were monitored for 60 min after drug instillation. The protocol was repeated after 1 week. RESULTS: Adrenaline at a dose of 0.02 mg/kg produced only a minor initial decrease in diastolic (from 90 +/- 5 to 78 +/- 3 mmHg, P=0.05), and mean blood pressure (from 107 +/- 4 to 100 +/- 3 mmHg, P=0.05), in all dogs. This effect lasted less then 30 s following the drug administration. In contrast, higher adrenaline doses (0.05 and 0.1 mg/kg) produced an immediate increase in diastolic (from 90 +/- 5 to 120 +/- 7 mmHg; and from 90 +/- 5 to 170 +/- 6 mmHg, respectively), and mean blood pressure (from 107 +/- 4 to 155 +/- 10 mmHg; and from 107 +/- 4 to 219 +/- 6 mmHg, respectively). All adrenaline doses resulted in an immediate increase in systolic blood pressure and pulse. Endobronchial administration of saline (control) affected none of the haemodynamic variables. CONCLUSIONS: In a non-arrest model, endobronchial adrenaline administration, as opposed to the effect of tracheal adrenaline, produced only a minor decrease in diastolic and mean blood pressure. We suggest that endobronchial adrenaline administration should be investigated further in a CPR low-flow model when maintaining adequate diastolic pressure may be crucial for survival.

Reversal of digital clubbing after lung transplantation in cystic fibrosis patients: a clue to the pathogenesis of clubbing.

Digital clubbing is a common sign in cystic fibrosis (CF) and in a variety of other diseases. However, its pathogenesis remains obscure. In diseases other than CF, regression of clubbing has been noted after cure of the underlying disease. The aim of this study was to assess whether clubbing is reversible in CF patients after lung transplantation. Digital clubbing was investigated in 3 CF patients, prior to and after lung transplantation. Distal phalangeal depth (DPD) and interphalangeal depth (IPD) of the index finger were measured using a skinfold caliper, and the DPD/IDP ratio was calculated. The mean DPD/IDP ratio was 1.08 +/- 0.05 prior to transplantation and 1.00 +/- 0.06, 0.96 +/- 0.06, 0.92 +/- 0.04, and 0.89 +/- 0.07 at 3, 6, 12, and 24 months after transplantation, respectively. In all 3 patients, the DPD/IDP ratio was greater than 1 before transplantation. In 2 patients, this ratio decreased to less than 1 within 3 months, and in the third patient within 9 months after surgery. We conclude that digital clubbing is reversible in CF patients who undergo lung transplantation. Regression was usually noted during the first 3 months posttransplantation. This could be explained either by adequate inactivation of a circulating clubbing-inducing molecule by the normal transplanted lungs, or by removal of the diseased lungs in which this presumably causative substance was produced.

Liver cirrhosis and portal hypertension in cystic fibrosis.

OBJECTIVES: Liver disease is the second cause of death in cystic fibrosis. The most deleterious complication of liver disease is portal hypertension, which has an estimated prevalence of up to 8%. Portal hypertension may manifest itself by splenomegaly, hypersplenism, gastro-oesophageal bleeding and ascites. The aim of our study was to determine the prevalence, risk factors and invasive management of portal hypertension at our centre. METHODS: One hundred and fifty patients with cystic fibrosis were followed up between 1975 and 2000 in the national cystic fibrosis centre in Israel. Forty patients (27%) had liver disease. All underwent clinical evaluation and laboratory and imaging studies. RESULTS: Portal hypertension was diagnosed in 10 patients (7%), of whom eight were male. The mean age at diagnosis was 11 years (range, 4-17 years). All had severe mutations of the cystic fibrosis transmembrane conductance regulator gene (the CFTR gene), pancreatic insufficiency, meconium ileus or distal intestinal obstruction syndrome and variceal bleeding. Seven patients underwent sclerotherapy to control acute bleeding. Four underwent portosystemic shunting (functioning up to 37 years). Two patients with severe lung and liver disease underwent transjugular intrahepatic portosystemic shunting, which provided bleeding control, but both died while waiting for lung/liver transplantation. One patient underwent liver transplantation due to liver failure and still had good liver and lung function 10 years later. CONCLUSIONS: Portal hypertension is more common among Israeli patients with cystic fibrosis. The unique genetic composition of our population may explain this phenomenon. Risk factors include male gender, pancreatic insufficiency, severe CFTR mutations, meconium ileus and meconium ileus equivalent. Sclerotherapy is the main option to control oesophageal variceal bleeding, while portosystemic shunts offer a prolonged alternative treatment for refractory bleeding. A transjugular intrahepatic portosystemic shunt and liver transplantation may also be effective, but further research is required in order to establish their role.

Oligohydrosis and hyperthermia: pilot study of a novel topiramate adverse effect.

A 6-year-old boy with partial complex seizures developed recurrent episodes of hyperthermia 2 months after topiramate was introduced into his antiepilepsy drug regimen. Further investigation revealed that the febrile episodes were related to environmental temperature and physical activity. A pilocarpine iontophoresis sweat test showed that the amount of sweat produced by the child was 5% that of age-matched controls. Topiramate discontinuation resulted in the disappearance of febrile episodes and normalization of sweat quantity in repeat sweat testing. Based on this observation and the previous data on zonisamide and isolated case reports on topiramate-related hyperthermia and the effect on sweat production, topiramate was suspected of causing oligohydrosis. A pilot study was carried out involving 13 additional children and young adults (age range 1-37 years) receiving topiramate. All patients were directly questioned regarding symptoms of decreased sweating and heat intolerance, went through a pilocarpine iontophoresis sweat test, and were compared with 14 age-matched controls who went through the sweat test for unrelated reasons. Nine of the patients were found to have reduced sweat quantity on the pilocarpine iontophoresis sweat test (including index case) (mean 0.089 g/30 minutes, SD 0.082; age-matched control: mean 0.21 g/30 minutes, SD 0.06). Eight of them were children (below 16 years). However, only three patients revealed symptoms related to heat intolerance. Topiramate is most likely responsible for decreased sweat production as detected by a pilocarpine iontophoresis sweat test. The effect seems to be more significant in children than in adults. There is a discrepancy between test results and clinical symptoms. Interestingly, oligohydrosis was found to be a relatively common side effect of zonisamide. Both zonisamide and topiramate share a carbonic anhydrase inhibitor activity. The significance of oligohydrosis in hot climates should not be underestimated. Its extent, the role of sweat test prediction, and clinical significance during topiramate treatment should be further estimated.

The effect of fasting practice on sedation with chloral hydrate.

OBJECTIVES: Infants undergo various painless imaging procedures frequently. Mild sedation is required in such cases to reduce anxiety as well as to ensure optimal performance of the procedure. The most frequently used sedative as a single drug is chloral hydrate. The issue of preprocedural fasting is a subject of contention. The purpose of this study was to assess the effect of the American Academy of Pediatrics (AAP)/American Society of Anesthesiology (ASA) fasting practice guidelines on the efficacy and success of the sedation with chloral hydrate. METHODS: The sedation records of 200 infants from 2 hospitals who underwent auditory brainstem response for evaluation of hearing loss were evaluated retrospectively. In the first hospital (group A), strict nulla per os (NPO) guidelines were followed in accordance with the guidelines published by the AAP/ASA, whereas in the second hospital (group B), no fasting period was required. All children were premedicated with chloral hydrate. We evaluated the sedation failure rate, total dose of chloral hydrate needed, adverse effects, overall sleep time, and time to discharge. RESULTS: The average fasting period as expected was significantly longer in group A patients than in group B patients (5.7 +/- 1.7 vs. 2 +/- 0.2 hours; P < 0.001). Group A patients demonstrated a significantly higher failure rate to achieve sedation with the first dose of chloral hydrate compared with group B patients (21% vs.11%; P = 0.03), hence needing higher doses (83 +/- 31 vs. 61 +/- 21 mg/kg; P < 0.01), and were sedated for longer periods (103 +/- 42 vs. 73 +/- 48 minutes; P < 0.001) and discharged later. No difference was found in the adverse effect rate. CONCLUSION: Fasting was associated with an increased failure rate of the initial sedation. As a consequence, an increased total dose of chloral hydrate was required inducing a prolonged sedation time. Presumably, this is a result of the fact that a hungry child is irritable and therefore more difficult to sedate.

Long-term non-invasive positive pressure ventilation among cystic fibrosis patients awaiting lung transplantation.

BACKGROUND: Pulmonary disease is the most frequent cause of morbidity and mortality in cystc fibrosis patients. New techniques such as non-invasive positive pressure ventilation have resulted in prolongation of life expectancy in CF patients with end-stage lung disease. OBJECTIVES: To determine the role of NIPPV in CF patients awaiting lung transplantation. METHODS: Between 1996 and 2001 nine CF patients (5 females) with end-stage lung disease were treated with bi-level positive airway pressure ventilation in the "spontaneous" mode. RESULTS: The patients' mean age at initiation of BiPAP was 15 years (range 13-40 years) and the mean duration of BiPAP usage was 8 months (range 3-16 months). Four patients underwent successful lung transplantation, three patients died while awaiting transplantation, and the remaining two are still on NIPPV while waiting for transplantation. Patients' body mass index increased significantly (P < 0.05) during BiPAP therapy (from 16.1 to 17.2 kg/m2). Blood pH, PaCO2, and bicarbonate improved significantly (from 7.31 to 7.38, 90.8 to 67.2 mmHg, and 48.9 to 40.3 mEq/L, respectively). Pulmonary function tests were not affected by BiPAP usage. The patients experienced a significant alleviation in morning headaches and improvement in quality of sleep (P < 0.003). There were no major complications during BiPAP usage. CONCLUSIONS: We demonstrated that long-term NIPPV can stabilize and improve physiologic parameters such as ventilation, arterial blood gases and body mass index, as well as subjective symptoms such as sleep pattern, daily activity level, and morning headaches in CF patients with end-stage lung disease. Further prospectively controlled studies are needed to evaluate the potential of BiPAP therapy and its influence on morbidity and mortality in the post-lung transplantation period.