Behcet Uz Children Research Articles

Pediatric Status Epilepticus Severity Score (STEPSS): Predictive Performance of Functional Outcomes: A Prospective Single-Center Study.

Objective: To prospectively investigate the predictive value of the modified Status Epilepticus Severity Score (STESS) for pediatric use (STEPSS) regarding unfavorable outcomes in the short term. Methods: Patients diagnosed as status epilepticus in the emergency department between January 2019 and June 2021 at a tertiary center of the University of Health Sciences, Dr. Behcet Uz Children's Hospital, were included in the study. The patients were followed up in the emergency department, neurology clinic, and pediatric intensive care unit until discharge. Demographic and clinical characteristics, STEPSS, and Pediatric Overall Performance Category Scale (POPC) scores were calculated. We defined a Pediatric Overall Performance Category Scale score ≥3 as an unfavorable outcome. We compared the effect of STEPSS on unfavorable outcomes and mortality. Results: 124 children were included. The median age was 33 months (interquartile range 16.2-84.7). Seventy-two (58.1%) patients had acute symptomatic etiology. We found that the STEPSS score with the receiver operating characteristic curve (area under the curve = 0.917, P < .001) could predict unfavorable outcomes (Pediatric Overall Performance Category Scale score ≥3) in children with status epilepticus. The Youden index (0.76) showed that a STEPSS score >2 was the optimal cutoff point for an unfavorable outcome. We found STEPSS useful in predicting mortality (area under the curve = 0.853, P < .001). The Youden index (0.58) indicated that a STEPSS >2 was the optimal cutoff for mortality: sensitivity 0.90 (95% confidence interval [CI] 0.58-0.99), specificity 0.67 (95% CI 0.57-0.77), positive predictive value 0.21, negative predictive value 0.98, positive likelihood ratio 2.7, negative likelihood ratio 0.14. Conclusion: We determined that STEPSS can be predicted unfavorable outcomes and mortality. We think that STEPSS can be used as a useful clinical score with further studies and external validations.

Evaluation of Newborns with Non-COVID-19 Pneumonia Hospitalized in the Neonatal Intensive Care Unit during the COVID-19 Pandemic, Turkey, Izmir 2020–2021

Abstract Objective In this study, we aimed to compare the clinical, laboratory, and radiological findings of noncoronavirus disease 2019 (COVID-19) viral agents in newborn infants hospitalized for lower respiratory tract infection during the COVID-19 pandemic. Methods This prospective cross-sectional study conducted between 11 March 2020 and 31 July 2021 included neonates with lower respiratory tract infections admitted to the neonatal intensive care unit of the Dr. Behcet Uz Children's Hospital. Nasopharyngeal swab samples were taken from all hospitalized patients for multiplex respiratory polymerase chain reaction (PCR) and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) PCR. The detection of respiratory viral pathogens was performed by multiplex real-time PCR assay (Bosphore Respiratory Pathogens Panel Kit V4, Anatolia Geneworks, Turkey). Infants with SARS-CoV-2 PCR positivity were excluded from the study. Patients' data were obtained from the electronic medical registry system. The non-COVID-19 viruses of the cases were analyzed according to seasonal variation (in/off-season). The pulmonary findings of the cases were classified as normal, infiltration, air bronchogram, and reticulogranular appearance at the time of admission. Results A total of 80 infants were included during the study period. A multiplex PCR test was performed to identify viral agents affecting the lower respiratory tract of infants; it was determined that 31% (25 out of 80) were respiratory syncytial virus (RSV), 41% (33 out of 80) were rhinovirus (Rhino), and the remaining portion (28%, 22 out of 80) were other viral agents (enterovirus, bocavirus, adenovirus, influenza, and parainfluenza). Compared with Rhino and other viral agents, RSV was detected most frequently in seasonal hospitalizations (p &lt; 0.05). When chest radiography and laboratory findings were evaluated, the rate of “infiltration” /“lymphopenia” was significantly associated with infants with RSV lower respiratory tract infections (p &lt; 0.05). Conclusion During the pandemic period, RSV affected the prognosis in intensive care unit admissions due to lower respiratory tract infection in newborns.

The assesment of prothrombotic potential using thrombin generation assay in pediatric patients with nephrotic syndrome: preliminary study.

Nephrotic syndrome (NS) is a common kidney disease associated with an increased risk of thrombotic events. The aim of this study was to assess the prothrombotic potential of patients with NS using the thrombin generation assay (TGA). A total of 35 patients with NS, who were followed in the Division of Pediatric Nephrology in Behcet Uz Children`s Hospital, were included in the study. After the patients with Steroid Resistant NS (n:3) were excluded, 32 patients in total were evaluated for TGA. Patients were primarily classified according to their response to corticosteroid therapy. The control group consisted of 34 healthy volunteers with similar gender and age distribution to the patients. Blood urine nitrogen, creatinine, albumin, triglyceride, cholesterol, 24-hour proteinuria, platelets, erythrocyte sedimentation rate, C-reactive protein and thrombin generation values in activation and remission period of NS were compared. Moreover, TGA values of the patients in their remission period were compared with the values of those in the control group. Endogenous thrombin potential (ETP) and peak thrombin levels were significantly higher in the activation period than remission period of NS. Additionally, after the patients achieved remission, their ETP was still higher than the control group. There was a negative correlation between both ETP and peak thrombin levels of patients with serum albumin, whereas a significant positive correlation was detected with platelet levels. Thromboembolic events were not observed in any of the patients during follow-up. Nephrotic syndrome is strongly associated with hypercoagulopathy as assessed by TGA during active NS. The present study reinforces the usefulness of TGA as a marker of hypercoagulability in pediatric patients with NS. Further studies are needed in this regard.

Incomplete Antenatal Steroid Therapy Can Enhance Outcomes in Preterm Infants with Emergency Conditions

OBJECTIVE: We aimed to investigate the role of incomplete of antenatal steroid therapy by comparing with no and complete steroid exposure on mortality and morbidity in preterm infants.STUDY DESIGN: This is a prospective, an observational study which includes preterm infants of 32 weeks of gestation and/or ≤1500 grams who were referred to Izmir Dr. Behcet Uz Children's Hospital NICU during the one year period. Infants were divided into three groups according to the administration of antenatal steroid as those who received incomplete-dose antenatal steroid, complete dose steroid therapy and those with no steroid exposure. Intubation at the delivery room, surfactant requirement, the inotropic requirement in the first 72 hours and morbidities associated with prematurity were determined as the primary results. Mortality and bronchopulmonary dysplasia at discharge and stage ≥2 retinopathy were analyzed as secondary outcomes.RESULTS: We found that 54 infants were born with an incomplete dose, 55 infants with complete dose and 38 infants with no steroid therapy. Surfactant requirement, inotropic requirement, and hemodynamically significant patent ductus arteriosus were lower in the steroid exposure group leading to a statistical difference (p &lt;0.05). Also, it was found that mortality and Bronchopulmonary dysplasia was lower in the incomplete and complete dose steroid groups, leading to a statistical difference (p &lt;0.05).CONCLUSION: We speculate that even incomplete dose steroid may reduce mortality by reducing respiratory distress syndrome.

A step to infant oral health promotion intervention among parents.

Providing oral health care to children from infant to toddler age can be achieved by educating and informing parents about their children&#x2019;s oral health. The aims of this study were to determine the basic knowledge of oral health and to evaluate the efficacy of an infant oral health education programme among parents in Turkey. Study Design: One hundred and fifty parents of infants between 3 and 12 months of age attending the well-baby clinic, Dr. Behcet Uz Children&#x2019;s Hospital (Izmir, Turkey), participated in the study. A pre-test questionnaire was administered to each parent to assess their infant oral health knowledge. The educational intervention was in the form of a 30-minute interactive explanation of a PowerPoint presentation. The parents completed a post-test after the education in the same session (a questionnaire was administered). The range of correct answers on the pre-test was 7&#x2013;20 of 26 with a mean of 13.7 (SD=2.76). The range of correct answers on the post-test was 12&#x2013;26 of 26 with a mean of 21.2 (SD=3.34). The difference of correct responses between before and after education was found to be statistically significant (p&#x3C;0.01). Under the conditions of this study, educational presentation improved parents&#x2019; knowledge on infant oral health care and provided anticipatory guidance.

Do microdeletions lead to immune deficiency?

IntroductionMicrodeletion syndromes may be accompanied by immunological disorders. This study aimed to evaluate the clinical and laboratory data as well as the immune functions of patients diagnosed with a microdeletion syndrome.Material and methods39 patients diagnosed with microdeletion syndrome who were monitored at the Pediatric Genetics and Immunology clinics at Dr. Behcet Uz Children’s Hospital were included in this study. All data for this research were obtained from patient records and by individual consultation with their parents.ResultsOf the 39 patients, 15 were monitored for a diagnosis of Williams syndrome, 12 for DiGeorge syndrome, 4 for Prader-Willi syndrome, 2 for Wolf-Hirschhorn syndrome, 1 for a 1p36 deletion, 1 for Smith-Magenis syndrome, 2 for Trichorhinophalangeal syndrome type 2 (TRPS2), and 2 for Cri-du-chat syndrome. Of these 39 patients, 10 (25.6%) had a medical history of frequent upper respiratory tract infections. One of the cases with TRPS2 and another with Smith-Magenis syndrome had previously received intravenous antibiotic therapy for infectious disease. Five of the 12 patients with DiGeorge syndrome had low T lymphocytes. Two of the patients with DiGeorge syndrome with a history of frequent infections, with hypogammaglobinemia, and low lymphocytes were receiving regular intravenous immunoglobulin (IVIG) replacement.ConclusionsIt must be taken into account that patients with microdeletion syndromes, especially those with DiGeorge syndrome, may also have immunodeficiencies; therefore, these patients should be closely monitored to prevent development of any complications.

S.B.Ü. Dr. Behçet Uz Çocuk Hastalıkları ve Cerrahisi Eğitim ve Araştırma Hastanesi’nde 2018 Yılı İçinde Yapılan Bildirimi Zorunlu Bulaşıcı Hastalıklar Sürveyans Verilerinin Değerlendirilmesi

S.B.Ü. Dr. Behçet Uz Çocuk Hastalıkları ve Cerrahisi Eğitim ve Araştırma Hastanesi’nde 2018 Yılı İçinde Yapılan Bildirimi Zorunlu Bulaşıcı Hastalıklar Sürveyans Verilerinin Değerlendirilmesi

Etiology of Fever in Neonates: Is It Just Due to Infection?

Objectives Fever is an abnormal elevation of body temperature that occurs as part of a specific biologic response that is mediated and controlled by the central nervous system. In neonates, it may indicate a serious underlying disease. With this study, we aimed to investigate the causes of fever in neonates. Methods Medical charts were reviewed retrospectively for all term newborn infants treated at the Behcet Uz Children's Hospital between 31 December 2007 and 31 December 2009 who subsequently developed fever during their first 28 days. The history of the newborn included type of feeding (formula or breast); weight loss, environmental temperature, and symptoms of infection (lethargy-poor suck, irritability, vomiting, abdominal distention, respiratory distress, and diarrhea) were noted; and physical examination also established. The standard laboratory workup for febrile infants included blood, cerebrospinal fluid, urine and stool cultures, blood count, C-reactive protein, and analysis of serum electrolytes. Results It was determined that the most admissions were made in summer and most frequently in July. The most frequent cause of fever was infection, followed by unexplained fever and dehydration. Clinical sepsis was more frequent than proven sepsis. Risk for neonatal infection was increased by normal spontaneous vaginal birth, whereas the risk for dehydration was increased by cesarean section. Conclusions Environmental factors and dehydration may also be a factor in the newborn who is admitted for fever. But given our results, a newborn with a complaint of fever should be managed as an infection until proven otherwise.

Does Allergen-Specific Immunotherapy Prevent New Sensitizations in Childhood Allergic Airway Diseases?

Objective: Specific immunotherapy (SIT) is a well-established treatment method that is suggested to alter the natural course of the disease. Although the mechanisms remain unclear, recent studies have shown that allergen-specific immunotherapy may reduce the risk of new sensitizations. The aim of this study was to evaluate new sensitizations in children who underwent SIT for allergic airway diseases: allergic rhinitis, allergic asthma or both. Materials and Methods: This retrospective observational study was conducted at Behcet Uz Children’s Hospital, among children with an allergic airway disorder due to monosensitization. The SIT group consisted 67 patients who had been offered, and accepted, and thus underwent SIT regularly during four years while the control group consisted of 57 patients who had been offered but refused SIT, and thus had not undergone SIT and were treated with medications only. The allergens that were negative at the first skin prick test but positive at the second prick test were accepted as ‘new sensitization’. Results: The SIT group consisted of 67 (mean age ±SD: 15.27±3.15; Male /Female: 42/25) while the control group included 57 (mean age ± SD: 12.61 ± 2.69; Male /Female: 35/22) monosensitized patients with allergic airway disorder. There was no significant difference between SIT group and controls considering age and gender, current airway disease and current responsible allergen (p>0.05). The rate of new allergen sensitization in the immunotherapy group was 26/67 (38.8%) and slightly higher than control group (14/57; 24.6%) but the difference was not significant, statistically (p=0.12). The most common allergens causing new sensitization were pollens of grass (12/40), animal dander (7/40) and olive tree (6/40). When newly sensitized patients were evaluated, the atopy incidence was significantly higher than patients with no new sensitizations (32/40 vs 8/40). Conclusion: We suggest that SIT may not prevent new sensitizations in childhood allergic airway diseases in monosensitized patients. The most important risk factor in developing new sensitization is presence of atopic disease in the parents.

Neurodevelopmental Outcome of Children with Congenital Hypothyroidism Diagnosed in a National Screening Program in Turkey.

To study the factors affecting a neurodevelopmental status of children with congenital hypothyroidism, diagnosed on national screening program. The study was performed in the Pediatric Endocrinology Department of Dr. Behcet Uz Children's Hospital between May 2012 and May 2013. Children with congenital hypothyroidism, aged between 24 and 36 months, diagnosed by national screening program were included in the study group. Healthy subjects at the same age group consisted of the control group. For the neurodevelopmental evaluation, Bayley Scale of Infant Development- II (BSID-II) was used. Factors possibly effective on neurodevelopment were evaluated. 42 patients and 40 healthy children (mean (SD) age, 29.4 (3.7) and 29.2 (3.5), respectively were included in the study. The mean MDI score [92.6 (7.07) vs 97.1 (9.69), P=0.14)] and the mean PDI score [97.8 (15.68) vs 99.1 (10.57), P=0.66)] in the study group and control group were not significantly different. Among the patient, 4.6% and 4.7% children were moderately retarded as per the MDI scores and PPI scores, respectively. The sex, socioeconomic status, birth weight, screening levels of TSH, severity of the congenital hypothyroidism, initiation time and the dosage of thyroid hormone replacement, length of the normalization period of TSH, and adherence to treatment were not found to affect the MDI and PDI scores of the patients. Some children with congenital hypothyrodism may have mild to moderate neurodevelopmental retardation, despite the early diagnosis and treatment, and thus need to be under regular follow-up for neurodevelopmental status.

Anafilakside Riskli Grup: İnfant Anafilaksisi

Objective: The infantile group that is accepted to be at risk of anaphylaxis has a special importance since they are unable to express their complaints and also the signs and symptoms of anaphylaxis can be confused with common symptoms of infants such as drooling, vomiting, diarrhea and itching. However, early detection of the signs and symptoms of anaphylaxis is crucial for effective diagnosis and treatment. The aim of study was to draw attention to infants anaphylaxis. Materials and Methods: All of the patients in the infant group (n=15) among a total of 35 anaphylaxis cases that we followed in the Pediatric Allergy Clinic of Dr. Behcet Uz Children’s Hospital between the years 2011-2015 were included in the study. Demographic and clinical features of the patients were evaluated. Results: 9 of 15 (60%) patients involved in the study were male and 6 (40%) were female, and the average age of anaphylaxis was 8.13±6.83 (min 1-max 24) months. In 93% of cases (n=14) the trigger was food and in 7% of cases (n=1) the trigger was not determined. The most frequently responsible foods were respectively cow’s milk (67%) and egg (20%). In 7 patients (47%), there were respiratory system and skin manifestations, in 7 patients (47%) 3 system involvement and in 1 patient respiratory and gastrointestinal system involvement has been observed. In only 3 cases (20%) the blood pressure was measured and also epinephrine was administered to only 3 cases (20%) in the hospital. In only 3 of the patients (20%), an epinephrine injector was prescribed after anaphylaxis. Conclusion: It was detected that the most common triggers are cow’s milk and egg, the subjective symptoms are frequently overlooked, an atopic nature is a risk factor and physicians’ rates of blood pressure measurement, epinephrine administration and EpiPen autoinjector prescription are low.

Chicken or the egg: the dilemma of allergic rhinitis versus adenoid hypertrophy

There is no consensus as to whether allergic rhinitis (AR) is a cause of adenoid hypertrophy (AH). This study evaluated the role of allergy in AH, and it explored the role of nasal endoscopy and the history of diagnosing AR in children empirically. This study enrolled 155 children consecutively in İzmir Behcet Uz Children`s Hospital between January and September 2013. Of the patients, 101 (65.2%) had a positive skin prick test. Multiple allergen sensitivity was identified in 76 (75.2%) of these patients. The history items of itching and sneezing had predictive value for empirically diagnosing AR in children. Of the nasal examination findings, only the nasal secretion characteristics had significant predictive value. AH and AR had a significant negative correlation. In this study, AH and AR were inversely related. The characteristics of the nasal secretions, itching, sneezing, were predictors of AR in children.

Chicken or the egg: the dilemma of allergic rhinitis versus adenoid hypertrophy.

There is no consensus as to whether allergic rhinitis (AR) is a cause of adenoid hypertrophy (AH). This study evaluated the role of allergy in AH, and it explored the role of nasal endoscopy and the history of diagnosing AR in children empirically. This study enrolled 155 children consecutively in İzmir Behcet Uz Children`s Hospital between January and September 2013. Of the patients, 101 (65.2%) had a positive skin prick test. Multiple allergen sensitivity was identified in 76 (75.2%) of these patients. The history items of itching and sneezing had predictive value for empirically diagnosing AR in children. Of the nasal examination findings, only the nasal secretion characteristics had significant predictive value. AH and AR had a significant negative correlation. In this study, AH and AR were inversely related. The characteristics of the nasal secretions, itching, sneezing, were predictors of AR in children.

The Importance of Hypersensitivity in Childhood Acute Lymphoblastic Leukemia

BackgroundAcute lymphoblastic leukemia (ALL) remains the most common pediatric malignancy in the world. Overall survival rates have increased from 30% in the 1960s to 90% in past 10 years. Enabling this dramatic improvement are the multi-agent chemotherapeutic regimens in which asparaginase is cornerstone. Unfortunately, hypersensitivity to asparaginase has been a commonly-reported adverse event, associated with inferior outcomes in ALL.MethodsBetween January 1, 2005 and December 31, 2012, 97 patients were enrolled in the study. Selection criteria included those were diagnosed with ALL and given the ALL-BFM-2000 protocol at Dr. Behcet Uz Children’s Hospital. Data regarding age at diagnosis, sex, follow-up duration, hypersensitivity of L- asparaginase, status of relapse and outcome were gathered from the patients’ files. The event-free survival (EFS) and the overall survival (OS) of the patients who developed and did not develop hypersensitivity to L- asparaginase were compared. Peg-asparaginase was given to patients who developed L- asparaginase hypersensitivity, and erwinia asparaginase was given if the patients developed peg-asparaginase. The asparaginase activity could not be measured. Kaplan mayer test was used to calculate the EFS and the OS.ResultsA total of 97 patients were evaluated. Thirty-two were standard risk, 49 were moderate risk, and 15 were high risk. 61 patients were male, and 36 were female. The median age (range) at diagnosis was 5 (1-15) years. Median (range) follow-up duration was 5.1 (0.08-7.3) years. Of the 97 patients, 28 (29.2%) developed L-asparaginase hypersensitivity. The event-free survival was 84.5 ± 4.6%, and 62.6 ± 9.4% for patients who developed and did not develop hypersensitivity, respectively (p=0.01). The overall survival rates were 89.2 ± 3.9 % and 74.6 ± 8.3% for patients who developed and did not develop hypersensitivity, respectively (p=0,07). [Display omitted] ConclusionsThis study demonstrated that EFS was lower in patients with L-asparaginase hypersensitivity than those without hypersensitivity, although there were no differences in terms of OS. All of the patients with hypersensitivity received a sufficient dose of peg-asparaginase or erwinia asparaginase according to the ALL-BFM-2000 protocol. The lower EFS for patients who were switched to peg-asparaginase because of L- asparaginase may be explained by silent inactivation of peg-asparaginase. If the activity of asparaginase could not be measured, it could be preferable to switch to erwinia asparaginase. DisclosuresNo relevant conflicts of interest to declare.

Propofol Infusion Syndrome or Adrenoleukodystrophy?

*Anesthesiology and Reanimation Clinic, Tepecik Research and Training Hospital; and †Anesthesia and Reanimation and ‡Pediatrics Clinics, BehcetUz Children’s Hospital, Izmir, Turkey.

A novel mutation, IVS13+5G&gt;A, in Ellis-van Creveld syndrome associated with haemophagocytic lymphohistiocytosis

Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Departments of Medical Genetics, Pediatric Hematology, Faculty of Medicine, Ege University, Departments of Medical Genetics, Pediatric Hematology, Dr Behcet Uz Children’s Hospital, Izmir, Turkey and Molecular Genetics Laboratory, CSS-Mendel Institute, Rome, Italy Correspondence to Ayca Aykut, MD, PhD, Department of Medical Genetics, Faculty of Medicine, Ege University, 35100 Bornova, Izmir, Turkey Tel: + 90 232 390 3961; fax: + 90 232 390 3971; e-mail: aycaaykut@hotmail.com

Cow’s milk allergy in children: 9 years experience of Dr. Behcet Uz Children’s hospital, Izmir

Objective: Cow’s milk allergy is the most frequent food allergy in childhood. Delayed diagnosis may cause a number of systemic dysfunctions, while incorrect diagnosis may result in deficient nutrition of the mother and the child. As in other types of allergies, food allergy is also a growing problem in recent years. Our aim was to determine the characteristics of our patients allergic to cow’s milk, and see if there was an increasing trend. Material and Methods: The study was of retrospective and cross-sectional design. Patient data were collected from Pediatric Allergy Department patient files. Cases of food allergy were reviewed and patients fulfilling the criteria of cow’s milk allergy were included in the study. Results: A total of 105 cases with cow’s milk allergy out of 264 food allergies who were followed up between 2003-2011 were investigated. Most of the patients were under 2 years of age (78.1%), were males (62.9%) and presented with a history of allergy (79.1%). IgE mediated cases constituted the majority (75.2%). More than half of the patients admitted in 2010 and 2011. This was mostly due to the increased number of patients less than twelve months of age (p=0.015). Most frequently involved system was skin (67.5%) and multisystem involvement was 15.7% Conclusion: A detailed history is the key element of diagnosing cow’s milk allergy. There is a considerable number of cases within the first year of life which may present with different signs and symptoms. Although not a life threatening situation, correct diagnosis is vital as nutrition is a key component in the development and growth of child.

The evaluation of muscle biopsy findings in children with neuromuscular disorders

Objective: The studies considering the clinical and histopathological features of childhood muscle disease are scarce in number worldwide. This study aims to examine the histopathological profile of the children’s muscle biopsies evaluated at the Behcet Uz Children’s Hospital (BUCH) and determine their correlation with clinical diagnosis. Methods: We retrospectively reviewed 323 children who underwent muscle biopsy examination between 2004 and 2011 at pathology laboratory of Izmir BUCH. All patients were clinically diagnosed as neuromuscular disorders and biopsy specimens were collected from 5 different centers of neurological disorders. Results: The mean age of the patients was 6.15 years (3 weeks-18 years). Only 22 cases (6.8%) were older than 14 and 39 (12.07%) were younger than twelve months. Spinal muscular atrophy (n=11; 28.2%) was the most common disease in the infantile group. Dystrophies were diagnosed in 148 (45.7%) patients and 102 of them were dystrophinopathies. Other disorders were 23 primary, 16 inflammatory and 19 metabolic/mitochondrial myopathies and 28 neuropathies. Seventy three (22.9%) patients had similarly affected family members. Consanguinity rate was 25.1%. Serum enzymes alterations, EMG pathologies and fiber type disproportion were determined in 109 (33.7%), 188 (58.2%) and 85 (26.3%) of the patients respectively. There were statistical significant correlations between the types of disease and serum enzyme levels (p= 0.001), and age (p=0.015) of the patients. Conclusion: The present study revealed the panorama of the childhood muscle diseases in western Turkey. We conclude that if avoidance from unnecessary diagnostic and therapeutic interventions was desired, we must be aware of the limitation and diagnostic value of muscle biopsy evaluations in childhood muscle diseases.

Persistan Atelektazili Yenidoğanlarda İntratrakeal ve İnhale Rekombinan İnsan Deoksiribonükleaz (rhDNase) Uygulaması: Bir Kurtarma Tedavisi

Objective: Atelectasis increases the risk of secondary pulmonary infections related with prolonged artificial ventilation. Therefore, it requires early and aggressive treatment in newborns cared in neonatal intensive care units (NICUs). Current treatment of atelectasis consists of certain conventional modalities. However, there is still no evidence-based, ''gold standard'' treatment. Use of recombinant human deoxyribonuclease (rhDNase) is a new concept in NICUs. In this study, we aimed to compare and evaluate the clinical and radiological changes in infants who received nebulized or intratracheal rhDNase for persistent atelectasis unresponsive to conventional treatment options. Material and Methods: This study was conducted in 23 newborns hospitalized at the NICU of Dr. Behcet Uz Children's Hospital. Twelve intubated patients received 1.25 mg rhDNase mixed with 1:1 0.9% saline intratracheally, whereas 11 non-intubated patients received the drug via a jet nebulizer. A second dose was administered 4 hours after the initial dose. Chest physiotherapy and tracheal aspiration was performed 1 hour after the second dose. The same protocol was repeated on the second day. Clinical and radiological responses were evaluated separately. Results: Positive radiological and clinical responses to rhDNase and recurrence of atelectasis in the whole study group were 78.3%, 56.3% and 16.7% respectively. Nebulized route was more successful than the intratracheal route. Response to rhDNase was better in cases with upper lung lobe involvement. Conclusion: Both nebulized and intratracheal rhDNase administrations are successful without any adverse reactions for the treatment of persistent atelectasis, especially in neonates with viscous secretions and pneumonia with upper lobe atelectasis.

Comparison of Oral and Intravenous Ibuprofen for Medical Closure of Patent Ductus Arteriosus: Which One Is Better?

Intravenous ibuprofen is an expensive drug that is being used currently for treating and preventing patent ductus arteriosus. Although oral ibuprofen is much cheaper, there is limited data published about its safety and efficacy. The aim of this study was to compare two forms of ibuprofen in terms of safety and efficacy in closure of patent ductus arteriosus. This is a single-center retrospective study. Data were collected from patients' files of preterm infants who were hospitalized at the Neonatal Intensive Care Unit of Dr. Behcet Uz Children's Hospital between April 2009 and June 2010. Six hundred sixty infants were evaluated by echocardiography between 24 and 48 postnatal hours. Clinically and hemodynamically significant ductus arteriosus was defined in 66 infants with gestational age less than 32 weeks and birth weight less than 1500 g. Oral or intravenous ibuprofen (loading dose: 10 mg/kg on day 1, followed by maintenance dose: 5 mg/kg on days 2 and 3) was administered. Treatment success was defined as a completely closed duct without reopening on follow-up. Drug-associated renal, gastrointestinal, cerebral, hematological, and metabolic side effects were monitored and compared between treatment groups. Ductal closure rates were 100% and 97.6%, respectively, in the oral and intravenous groups. Hypernatremia was the remarkable side effect in the intravenous group, whereas bronchopulmonary dysplasia and septicemia were prominent in the oral group. No statistically significant difference could be demonstrated between the groups in terms of mortality rates. Oral ibuprofen therapy is as efficacious as intravenous ibuprofen with some concerns about increased sepsis and bronchopulmonary dysplasia incidence. However, comprehensive and large-scale pharmacokinetic studies are required in order to prove this efficacy. On the other hand, intravenous ibuprofen still remains to be the drug of choice for patent ductus arteriosus but only with meticulous control of serum sodium levels in smaller infants.