Polycythemia as an Uncommon Finding in 2 Children, One With Systemic Capillary Leak Syndrome and the Other With Protein-losing Enteropathy Caused by CD55 Deficiency.

We report 2 children with distinct causes of polycythemia, 1 from systemic capillary leak syndrome (SCLS) and the other from protein-losing enteropathy (PLE) caused by CD55 deficiency. There is only a single case series about polycythemia in children with SCLS, but none on polycythemia in children with PLE. We present a 10-year-old girl with hypoalbuminemia, polycythemia, and edema who died as a result of an SCLS attack and a 1-year-old girl with PLE who was successfully treated with eculizumab. Our experience suggests that hematologists should be alert for SCLS and PLE in children with relative polycythemia.

The Burden of Burnout Syndrome in Pediatric Intensive Care Unit and Pediatric Emergency Department: A Multicenter Evaluation.

OBJECTIVE: The objective of this study was to detect variables associated with burnout syndrome (BS) in pediatric intensive care units (PICUs) and pediatric emergency medicine departments (PEDs) in high-volume centers from different parts of Turkey. METHODS: An observational, cross-sectional multicenter study was performed. The Maslach Burnout Inventory scale was administered to all of health care providers working in PICUs and PEDs. In this study, health care providers were defined as physicians, nurses, and other staff (secretaries, cleaning and patient care staff) working in PICU and PEDs. RESULTS: A total of 570 participants completed the survey. The major finding of this study was that 76.1% (n = 434) of PICU and PED health care professionals had BS. The most prominent subscale of BS was emotional exhaustion (62.5%). The rate of BS was higher among health care providers working in PEDs compared with PICUs (79.1% vs 73.7%, P = 0.04). The frequency of BS according to emotional exhaustion and depersonalization subscales was higher in health care providers of PEDs. The rate of BS was also significantly higher in younger employees, females, those working 51 or more hours totally in a week, those having a low monthly salary, those single or divorced, those without children, those with no childcare at home, those not owning a home, those not doing regular exercise and not having regular breakfast, those with total employment time of less than 1 year, and those not having a car or not having a hobby. In PEDs, when the daily evaluated number of patients was equal to or more than 44 (sensitivity, 88%; specificity, 66%), it predicted the occurrence of BS. In PICUs, when the number of patients cared for by 1 nurse was equal to or more than 3, it predicted the occurrence of BS (sensitivity, 78%; specificity, 62%). CONCLUSIONS: By creating early intervention programs to prevent BS, shortages of health care professionals can be avoided and the costs of health care expenditures related to infections can be decreased.

Potential predictors of relapse after treatment of asthma exacerbations in children.

BACKGROUND: Knowledge of factors that affect relapse will allow close monitoring of patients at risk, resulting in a decreased rate of readmission to the emergency department. OBJECTIVE: To determine risk factors associated with relapse within 7 days after treatment of asthma exacerbations in children. METHODS: This was a multicenter, prospective study of children with asthma attacks. Patients between the ages of 6 months and 17 years who met the criteria between June 2009 and September 2012 were considered. RESULTS: The study included 1177 patients (775 males [65.8%]) with a mean (SD) age of 70.72 (48.24) months. Of them, 199 (16.9%) had a relapse within 1 week after being discharged from the hospital. Factors independently associated with relapse identified by a logistic regression model for the 1,177 study visits were having taken a short-acting inhaled ß2-agonist within 6 hours before admission (odds ratio [OR], 2.43; 95% confidence interval [CI], 1.728-3.426; P = .001), presence of retraction on physical examination (OR, 1.76; 95% CI, 1.123-2.774; P = .01), no prescription for high-dose inhaled steroids on release (OR, 2.02; 95% CI, 1.370-3.002; P < .001), and not being given a written instructional plan (OR, 1.55; 95% CI, 1.080-2.226; P = .02). CONCLUSION: Whereas having taken short-acting ß2-agonists within 6 hours before admission and the presence of retractions on physical examination increased the risk of relapse after treatment of the acute attack, being given high-dose inhaled steroids and a written instructional plan when being sent home reduced the risk.

Pediatricians manage anaphylaxis poorly regardless of episode severity.

BACKGROUND: No information exists on how the knowledge or the practice of pediatricians regarding anaphylaxis episodes vary with episode severity. The aim of this study was to assess and compare pediatrician knowledge on the management of mild and severe anaphylaxis using clinical scenarios and to determine factors that affect their decisions. METHODS: A questionnaire consisting of eight questions on the diagnosis and management of anaphylaxis was distributed at two national congresses. A uniform answer box including possible response choices was given below each question, and respondents were asked to check the answers that they thought appropriate. The management of mild and severe anaphylaxis was examined using two clinical case scenarios involving initial treatment, monitoring, and discharge recommendations. RESULTS: Four hundred and ten questionnaires were analyzed. The percentage of pediatricians who correctly answered all questions on the management of mild and severe anaphylaxis scenarios was 11.3% and 3.2%, respectively. Pediatricians did significantly better with initial treatment, but they were less knowledgeable with respect to observation time and discharge criteria in the mild anaphylaxis case scenario compared with the severe one (both P < 0.001). Multiple logistic regression analysis identified only working in an emergency department or intensive care unit as significantly predicting correct diagnosis of anaphylaxis among pediatricians (P = 0.01, 95% confidence interval: 0.11-0.57). No pediatrician-related factors predicted physician knowledge on the management of anaphylaxis. CONCLUSIONS: Pediatricians have difficulty with different steps in managing mild and severe anaphylaxis. Their deficiencies in management may result in failure to prevent recurrences of mild anaphylaxis and may increase mortality in severe anaphylaxis.

Management preferences of pediatricians in moderate and severe acute asthma.

OBJECTIVE: To assess and compare management preferences of physicians for moderate and severe acute asthma based on case scenarios and to determine the factors influencing their decisions. METHODS: A questionnaire based on the Global Initiative on Asthma (GINA) guideline and comprising eight questions on management of acute asthma was delivered to participants of two national pediatric congresses. Management of moderate and severe acute asthma cases was evaluated by two clinical case scenarios for estimation of acute attack severity, initial treatment, treatment after 1h, and discharge recommendations. A uniform answer box comprising the possible choices was provided just below the questions, and respondents were requested to tick the answers they thought was appropriate. RESULTS: Four-hundred and eighteen questionnaires were analyzed. All questions regarding moderate and severe acute asthma case scenarios were answered accurately by 15.8% and 17.0% of physicians, respectively. The initial treatment of moderate and severe cases was known by 100.0% and 78.2% of physicians, respectively. Knowledge of the appropriate plan for treatment after 1h was low both for moderate (45.0%) and severe attacks (35.4%). Discharge recommendations were adequate in 32.5% and 70.8% of physicians for moderate and severe attacks, respectively. Multiple logistic regression analysis revealed that working at a hospital with a continuing medical education program was the only significant predictor of a correct response to all questions regarding severe attacks (p = .04; 95%CI, 1.02-3.21). No predictors were found for information on moderate attacks. CONCLUSIONS: Pediatricians have difficulty in planning treatment after 1 hour both for moderate and severe asthma attacks. Postgraduate education programs that target physicians in hospitals without continuing medical education facilities may improve guideline adherence.

Neutrophil gelatinase-associated lipocalin as a follow-up marker in critically ill pediatric patients with established acute kidney injury.

AIM: To assess the utility of neutrophil gelatinase-associated lipocalin (NGAL) in both urine and serum as a follow-up marker for the discrimination of prerenal acute kidney injury (AKI) from intrinsic AKI in critically ill pediatric patients with established AKI at the time of patient presentation. PATIENTS AND METHODS: This was a prospective cohort study of a heterogeneous group of critically ill children in the pediatric intensive care unit (PICU). Serum creatinine (SCr) values were obtained daily as part of routine patient care. AKI was defined as a 50% or greater increase in SCr from baseline and classified as prerenal and intrinsic AKI. RESULTS: A total of 32 critically ill children (mean age: 105 ± 71.7 months, 56% female) with established AKI were included to the study. Area under curve (AUC) for urine and serum NGAL to distinguish prerenal AKI from intrinsic AKI was 0.94, 95% confidence interval (CI): 0.869-1.02 (p < 0.001) and 0.86, 95% CI: 0.71-1.02 (p = 0.002), respectively. CONCLUSION: In a heterogeneous group of critically ill children with established AKI, we found that NGAL in both urine and serum at the time of patient presentation discriminated intrinsic AKI from prerenal AKI.

Drug-induced movement disorders in children at paediatric emergency department: 'dystonia'.

AIM: To examine cases with drug-induced dystonic reactions (DIDRs), to identify the complaints of the application, to classify the drugs causing those dystonic reactions (DRs) and to determine the treatment options and protective measures to prevent DIDRs. METHOD: The authors retrospectively analysed 55 cases with DIDRs at paediatric emergency department (PED) in a 5-year period. RESULTS: The mean age of the patients was 145.07±56.30 months, and of the 55 cases, 28 cases (50.9%) were boys. Antiemetics and antipsychotics were the most common causes of DIDRs. 35 (70%) patients developed DIDRs at therapeutic doses. Treatment side effect was the most common cause of the DIDRs (78.2%). The most common DIDRs were abnormal postures of the head and neck (56.6%). Laryngospasm was observed only in four cases (7.3%) that used either antipsychotics or psychostimulants. 51 (92.7%) children were treated with parenteral diphenhydramine successfully. CONCLUSION: Dystonia is a common side effect of certain drugs, even when therapeutic doses are administered. Although the most common DIDRs were abnormal postures of the head and neck, rare life-threatening conditions, may develop particularly due to use of antipsychotics. In treatment, diphenhydramine could effectively be used through parenteral way to eliminate the cholinergic effects of those drugs. However, the easiest and the safest way to prevent the development of DRs is to avoid unnecessary drug usage. In conclusion, physicians should be aware that antiemetic and antipsychotic drugs are associated with DRs in normal doses and that those drugs should be prescribed with a correct indication.

Is acute dystonia an emergency? Sometimes, it really is!

Most cases of acute dystonia are mild and easy to manage; nevertheless, some of them can be fatal because of the involvement of certain muscle groups such as the laryngeal muscles, thus requiring urgent intervention. In the literature, approach to life-threatening acute dystonia has not been investigated thoroughly, although the diagnosis is a challenge, and treatment should be offered immediately. Herein the management of life-threatening acute dystonia is discussed via 2 case reports.

Inability to walk, disequilibrium, incoherent speech, disorientation following the instillation of 1% cyclopentolate eyedrops: case report.

A 4-year-old boy, who had no prior history of convulsions, presented with inability to walk, disequilibrium, dysarthria (incoherent speech), and impaired cognition (disorientation) following the instillation of 1% cyclopentolate, a commonly used mydriatic in pediatric practice. This case demonstrates the uncommon, although serious, atropine-like adverse effect of cyclopentolate eyedrops in usual dosage in child.

Iatrogenic water intoxication during pelvic ultrasonography in a patient with diabetes insipidus.

Pelvic ultrasonography (US) is a simple and non-invasive radiologic test to evaluate the pelvic organs. It requires a full bladder for better visualization. Our case is a 14-year-old female with diabetes insipidus (DI) who admitted to the pediatric emergency service with the complaints of seizure and agitation after drinking 4 liters of water in one hour for a pelvic US examination due to work-up for delayed puberty. Her biochemical and clinical evaluation revealed water intoxication (WI). To our knowledge, this is the first WI case developed in a patient with DI. Here, we discuss the underlying factors leading to this complication and recommended an approach to obtain a better sonographic image without necessitating oral water intake to fill the urinary bladder.

Make-up of the epinephrine autoinjector: the effect on its use by untrained users.

BACKGROUND: Part of the problems related to proper use of the epinephrine autoinjector may be related to the design of the autoinjector itself. AIM: We investigated whether minor modifications in the design of the currently available epinephrine autoinjector ease its use and abrogate common use errors. METHODS: All interns other than those who had previously worked in allergy department in a medical school were invited to the study. Two identical epinephrine autoinjector trainers (Epipen trainer(®) ) were used, one of which was modified by changing the gray safety cap to red and placing a yellow arrow pointing to the black injection tip. A written and visual instruction sheet for each trainer was provided. Participants were asked to demonstrate the use of the Epipen trainer either with the original or with the modified one. They were scored and timed for their demonstration. RESULTS: Out of the 224 interns who were invited to participate, one hundred and sixty-four interns (73.2%) participated in the study. The number of participants correctly demonstrating the use of epinephrine autoinjectors was 22.6% and 65% in unmodified and modified trainer groups, respectively (p < 0.001). The mean time to administer trainers was 26.78 ± 10.6 and 15.88 ± 2.55 s; total median scores were 3.08 ± 1.48 and 4.47 ± 0.84 in unmodified and modified groups, respectively (p < 0.001 for both). Significantly fewer participants had presumptive unintentional injection injury while using modified (5%) compared with unmodified trainer (45.2%) (p < 0.001). CONCLUSION: Few and simple modifications in the design of epinephrine autoinjector were found effective in increasing its correct use and decreasing common use errors by untrained users. (Clinical trials identifier: NCT01217138).

Training of trainers on epinephrine autoinjector use.

The majority of physicians do not know how to use epinephrine autoinjectors. This displays that current education of physicians on anaphylaxis is inadequate for a thorough practice. We hypothesize that a theoretical lecture together with a practical session on epinephrine autoinjector use will improve its proper use by physicians. Residents, specialists, and consultants from General Pediatrics excluding allergists and allergy fellows were included in this study. All physicians were given an eight-item questionnaire followed by a practical session scoring and timing ability to use epinephrine autoinjector trainer. This ensued with one-to-one hands-on training on correct autoinjector use. Finally, a joint theoretical lecture on anaphylaxis including re-demonstration of epinephrine autoinjector use was given. All physicians were scored a second time on use of epinephrine autoinjector 6 months later. One hundred fifty-one of 196 participants completed all steps of the study in four tertiary hospitals. Correct use of epinephrine autoinjector improved from 23.3% to 74.2%, mean score from 3.49 ± 1.14 to 4.66 ± 0.65, need for prospectus from 91.4% to 29.1%, and mean time to administer autoinjector from 28.01 ± 6.22 s to 19.62 ± 5.01 s (p < 0.001 for each). The rate of most common mistakes during autoinjector use decreased but the ranking did not change. An integrated theoretical and practical education increased correct of epinephrine autoinjector use by physicians. Ongoing mistakes despite this education may be related with its design.

Clinical and epidemiological features of Turkish children with 2009 pandemic influenza A (H1N1) infection: experience from multiple tertiary paediatric centres in Turkey.

BACKGROUND: In April 2009 a novel strain of human influenza A, identified as H1N1 virus, rapidly spread worldwide, and in early June 2009 the World Health Organization raised the pandemic alert level to phase 6. Herein we present the largest series of children who were hospitalized due to pandemic H1N1 infection in Turkey. METHODS: We conducted a retrospective multicentre analysis of case records involving children hospitalized with influenza-like illness, in whom 2009 H1N1 influenza was diagnosed by reverse-transcriptase polymerase chain reaction assay, at 17 different tertiary hospitals. RESULTS: A total of 821 children with 2009 pandemic H1N1 were hospitalized. The majority of admitted children (56.9%) were younger than 5 y of age. Three hundred and seventy-six children (45.8%) had 1 or more pre-existing conditions. Respiratory complications including wheezing, pneumonia, pneumothorax, pneumomediastinum, and hypoxemia were seen in 272 (33.2%) children. Ninety of the patients (11.0%) were admitted or transferred to the paediatric intensive care units (PICU) and 52 (6.3%) received mechanical ventilation. Thirty-five children (4.3%) died. The mortality rate did not differ between age groups. Of the patients who died, 25.7% were healthy before the H1N1 virus infection. However, the death rate was significantly higher in patients with malignancy, chronic neurological disease, immunosuppressive therapy, at least 1 pre-existing condition, and respiratory complications. The most common causes of mortality were pneumonia and sepsis. CONCLUSIONS: In Turkey, 2009 H1N1 infection caused high mortality and PICU admission due to severe respiratory illness and complications, especially in children with an underlying condition.

The first report of West Nile virus infection in a child from Turkey.

Seroprevalence data indicate that West Nile virus (WNV) activity is present in Turkey; however, no pediatric cases of WNV infection have been reported to date. WNV is an emerging flavivirus in Turkey, and in September2010, the Turkish Ministry of Health reported the first seven cases of laboratory-confirmed WNV infection from five different cities in the western part of the country. This is the first report of a child from central Anatolia, indicating the existence of the virus in other regions of the country.

The comparison and diagnostic accuracy of different types of thermometers.

BACKGROUND: Fever is one of the leading causes of hospital admissions in children. Although there are many ways to measure body temperature, the optimal method and the anatomic site are still controversial. In this study, we aimed to evaluate the performance of new methods of measuring body temperature and to compare the accuracy, sensitivity and specificity of these methods. METHODS: The body temperatures of the patients who were hospitalized as inpatients or who presented to the emergency room as outpatients between November 2014- March 2015 were measured and recorded. Mercury and digital axillary measurements, tympanic, temporal artery and non-contact skin temperatures were measured. Measurements were compared with each other. RESULTS: According to our results temperature tends to increase over time for up to 8 minutes after placement when using axillary thermometers. Non-contact skin thermometers should be used only for follow-up of patients with fever, because of their low sensitivity and low negative predictivity. At the first examination, tympanic thermometers and axillary thermometers may be preferable for the diagnosis of fever. CONCLUSIONS: According to our results, using non-contact thermometers seems feasible and logical during the follow-up ofpatients with fever, but not in cases whose exact body temperature should be known. For the first examination of the patient to diagnose fever, tympanic thermometers and axillary thermometers may be preferable. Future studies are warranted to expose the optimum way of measuring body temperature in children.

Pediatricians' Attitude in Management of Acute Bronchiolitis: Did Guidelines Overcome Practices?

Background: Acute bronchiolitis is one of the most common diseases of early childhood. There are many recent changes in the treatment of acute bronchiolitis. The aim of this study is to evaluate treatment approaches to acute bronchiolitis among clinicians and to observe compliance with clinical guidelines. Materials and Methods: Our study was designed as a multicenter cross-sectional descriptive study. A cohort of pediatric residents, fellows, and attendants were surveyed with a questionnaire including general and occupational characteristics of pediatricians and treatment choices in acute bronchiolitis. Results: A total of 713 questionnaires were collected. Most commonly applied treatment among pediatricians was inhaled salbutamol, followed by intravenous hydration, hypertonic saline, and inhaled steroid. Most commonly preferred treatment in the management of mild bronchiolitis was oral hydration and inhaled salbutamol in severe bronchiolitis. Conclusion: Although recent guidelines for the treatment of acute bronchiolitis does not support the use of many different therapies, pediatricians still tend to use them, especially bronchodilators, corticosteroids, and antibiotics.

An unusual cause of stridor: congenital laryngeal web.

Respiratory distress and stridor are some of the common presenting symptoms for children in Pediatric Emergency Department. Most of these children are diagnosed as having common illnesses such as laryngitis, croup or laryngomalacia. However, Pediatric Emergency physicians must keep in mind other rare respiratory diseases other than laryngitis or croup in the differential diagnosis of stridor. Stridor may occur due to congenital and acquired diseases. Laryngeal web is one of the rare congenital causes of stridor, which usually presents in the first weeks of life; however, it is very rarely diagnosed in the later period. Herein, we report a one-year-old boy who was evaluated for croup and was diagnosed as having laryngeal web.

Outcome of out-of-hospital cardiopulmonary arrest in children: A multicenter cohort study.

Kurt F, Kendirli T, Gündüz RC, Kesici S, Akça H, Sahin S, Kalkan G, Derbent M, Tuygun N, Ödek Ç, Gültekin-Keser A, Oguz S, Polat E, Derinöz O, Tekin D, Teksam Ö, Bayrakci B, Suskan E. Outcome of out-of-hospital cardiopulmonary arrest in children: A multicenter cohort study. Turk J Pediatr 2018; 60: 488-496. The aim of this study was to evaluate the demographic characteristics of children who experienced out-of-hospital cardiopulmonary arrest (CPA), and to assess the impact of the bystander cardiopulmonary resuscitation (CPR) on the survival rate of witnessed arrests and the effects of the arrest and CPR durations on the neurological outcomes. This multicenter, retrospective study included a total of 182 patients who underwent CPR for out-of-hospital CPA between January 2008 and December 2012 at six centers in Ankara, Turkey. The median [interquartile range (IQR)] age was 22 (5-54) months; 60.4% of the patients were males, and 44% were younger than one year of age. The witnessed arrest rate was 75.8% (138/182) and the rate of bystander CPR was 13.9% (13/93). In these patients the rate of the return of spontaneous circulation (ROSC) was higher (76.9%). Following resuscitation in the patients for whom the spontaneous circulation was able to be returned, the median (IQR) duration of arrest was 5 (1- 15) min, while it was 15 (5-40) min for the remaining patients (p < 0.001). The ROSC rate was 94.9% in patients who underwent CPR for less than 20 min and 22% in patients requiring CPR longer than 20 min (p < 0.001). Survival to hospital discharge was 14.3%. Of these patients, 57.7% experienced neurological disability. The short duration of an arrest and the presence of CPR are both critical for survival. We suggest that a witness to the CPA, performing early and efficient CPR, yields better results.

Age-Specific Frequencies and Characteristics of Ovarian Cysts in Children and Adolescents.

OBJECTIVE: The aim of the present study was to document ovarian cyst frequency and characteristics as well as distribution of these parameters with respect to age in children and adolescents. METHODS: We retrospectively analyzed the medical records of 1009 girls between the ages of 5-18 years who presented to our pediatric emergency department (PED) with pelvic pain and therefore underwent pelvic ultrasound examination between June 2011 and May 2014. RESULTS: In total, 132 of 1009 girls (13.1%) were identified as having ovarian cysts ≥1 cm in diameter. The frequency of ovarian cysts was found to be 1.8% (6/337) in children aged 5-9 years and 18.8% (126/672) in those aged 10-18 years. All the cysts detected in children aged 5-9 years were small (<3 cm) and simple with age-specific frequencies ranging between 1.5-2.7%. With the onset of adolescence, ovarian cyst frequency started to increase with age and ranged between 3.8-31.3% throughout adolescence. Age of peak ovarian cyst frequency was 15 years with a rate of 31.3%. Large ovarian cysts (>5 cm) were identified in 19 adolescents (15.1%) with most occurring during middle adolescence. Of the 19 adolescents, five were found to have cyst-related significant ovarian pathologies including cystadenoma (n=3) and ovarian torsion (n=2). CONCLUSION: In children aged 5-9 years, ovarian cysts were infrequent and small (<3 cm). Peak ovarian cyst frequency was detected at the age of 15 years. All patients diagnosed with cyst-related significant ovarian pathologies were adolescents having a cyst >5 cm in diameter with a complex appearance in most.

Use of the bayley infant neurodevelopmental screener with premature infants.

OBJECTIVE: To examine the utility of the Bayley Infant Neurodevelopmental Screener (BINS) for the neurodevelopmental follow up of high-risk preterms. METHODS: The study group consisted of 122 preterm infants of the gestational ages between 26 and 37 weeks. Medical factors such as; mean birth weight, gestational age, gender, birth place and type, number of multiple pregnancy, days of hospitalization and oxygen therapy, use of antenatal steroids, and occurrence of sepsis for each patient were evaluated. The neurodevelopmental examination and BINS administration was made at each visit to the patients whose ages were adjusted for prematurity. RESULTS: At the corrected age of 7-10 months patients at 26-29 GA had significantly lower total means of BINS scores when compared to those of the other two groups at 30-32 and 33-37 GA (P<0.01 and P<0.001). At the corrected age of 3-4 months, the total means of the BINS scores of the patients at 30-32 GA were lower than that of the patients at 33-37 GA (P<0.009). at 7-10 months the scores of the items defining the expressive and cognitive functions of the patients at 26-29 GA were lower than those of the patients at 30-32 GA (P<0.05) and 33-37 GA (P<0.05). At 16-20 months the mean scores of the items of expressive functions were significantly lower in patients of the 26-29 GA compared to those of the patients of 33-37 GA (P<0.03). CONCLUSION: As data accumulates, BINS will turn out to be a quick way of determining infants at risk of developmental delay in many aspects of neurologically high risk conditions.