Objective: Poor adherence to medications is an important problem, especially in patients with chronic diseases such as familial Mediterranean fever (FMF). In this study, we aimed to evaluate medication adherence in pediatric FMF patients and investigate the relationship between disease perception and treatment compliance.
Method: Pediatric patients (<18 years old) with a diagnosis of FMF and at least six months of follow-up period participated in the study. Patient perceptions of illness and medication adherence were measured via a brief illness perception questionnaire (brief IPQ) and the Medication Adherence Scale in FMF Patients (MASIF), respectively.
Results: A total of 50 patients (46% girls, n: 23) with a median age of 9.9 (IQR 5.9-15.8) years were included. The median age at diagnosis was 5.4 (IQR 3.2-10.1) years. Twenty-eight patients (56%) had good medication adherence (the MASIF score>60), while 44% of the patients were non-adherent to the treatment. Patients aged under 12 years of age were more adherent to colchicine treatment than those over 12 years of age (68.2% vs. 25.0%, p= 0.002). The comparison of the illness perception and medication adherence revealed higher brief IPQ total scores in patients who adhered to the treatment (median 48.5 vs 52.5, p=0.037). We found significant differences in timeline scores between patients who were adherent and those who were non-adherent to the treatment (p=0.01).
Conclusion: Non-adherence to medication is an important and widespread problem, particularly among adolescent patients. Perceptions of the illness and beliefs about the duration of the disease may affect adherence to treatment. Medication adherence should be routinely assessed at follow-up visits and educational interventions might improve adherence to the treatment.

Objective: Hypospadias is defined as incomplete closure of the penile structures during embryogenesis. Surgical correction induces various complications, including urethrocutaneous fistula (UCF). The aim of this study was to determine the risk factors for the occurrence of UCF in children undergoing hypospadias repair under caudal anesthesia.
Method: The medical records of children undergoing hypospadias repair between January 2013 and July 2018 were included. Data on patients’ age, body weight, height, type of repair procedure, type of hypospadias, duration of surgery, and hospitalization, and postoperative complications were analyzed.
Results: The mean age of the 122 patients was 4.8±3.7 years. The type of surgery performed was tubularized incised plate urethroplasty (Snodgrass) in 90 (73.8%) and meatal advancement and glanuloplasty (MAGPI) in 32 (26.2%) children. Sixteen (13.1%) children had postoperative complications, all of which were UCF. No statistical association was found between postoperative UCF and patient variables.
The most common complication of hypospadias repair is UCF, which occurs mostly in the immediate postoperative period.
Conclusion: Hypospadias repair can result in complications. UCF remains a significant problem in the postoperative period. All patients underwent caudal block and despite the previous literature, we experienced lower rates of penile engorgement and postoperative UCF. These results showed that there was no cause-and-effect relationship between the caudal block and UCF. We think that the development of a urethrocutaneous fistula is mostly related to surgical causes and well-designed, prospective, and controlled studies are required to elucidate this issue.

Objective: The evaluation of patients with neonatal cholestasis is difficult due to the variety of cholestatic syndromes and non-specific clinical findings. It is important to recognize treatable diseases promptly. The aim of this study is to draw attention to suspicious markers in order to diagnose treatable metabolic diseases.
Method: The presented retrospective study included patients with cholestasis in the first three months of life. The study was conducted between 2018 and 2021 at Diyarbakır Children’s Hospital, Türkiye.
Results: 253 patients presenting with neonatal cholestasis were retrospectively evaluated. 174 patients (68.77%) were examined for intrahepatic cholestasis. 16.6% of the patients were diagnosed with an infection, 13.43% with TPN-related cholestasis, 8.3% with IEM, 7.11% with cystic fibrosis, 4.74% with endocrinopathy, 4.34% patients with Alpha-1 antitrypsin deficiency, 2.76% with idiopathic neonatal hepatitis, 1.97% with genetic syndrome, 1.58% with PFIC, and 0.79% patients with Alagille syndrome. IEM-related patients (21) were diagnosed with tyrosinemia type 1, galactosemia, Niemann-Pick type A, glycogen storage disease type 3, peroxisomal disorders, fatty acid oxidation defects, mitochondrial DNA depletion syndrome, citrine deficiency, Niemann-Pick Type C and bile acid synthesis defect. Plasma tyrosine and methionine levels were high in patients with not only tyrosinemia type 1, but also galactosemia and citrine deficiency. Therapeutic plasma exchange was performed in two patients with fatty acid oxidation disorders.
Conclusion: Neonatal cholestasis poses a diagnostic challenge for clinicians. Delayed referral to a specialist for treatable metabolic diseases may increase mortality and morbidity. IEMs are observed more frequently in the etiologies of neonatal cholestasis in Türkiye due to high parental consanguinity and inadequate newborn screening programs. Treatable disorders should be considered early, as therapeutic interventiosn can be lifesaving. It also helps in genetic counseling, prenatal diagnosis for future pregnancies.

Objective: Inborn errors of metabolism (IEM) are a rare, inherited, heterogeneous group of diseases that are mostly symptomatic in the pediatric age group. Late diagnosis and delays in intervention can result in acute metabolic decompensation, progressive neurological damage, or death. IEM patients are responsible for significantly increased morbidity and mortality in intensive care units. Rapid, aggressive, and supportive treatment in pediatric intensive care units can reduce morbidity and mortality in IEM patients.
Method: Patients diagnosed with IEM and/or diagnosed during hospitalization in the tertiary Pediatric Intensive Care Unit (PICU) between February 2021 and November 2022 were retrospectively analyzed. During this period, 962 hospitalized patients were screened and patients with a diagnosis of IEM were included in the study. Demographic data, laboratory analysis, treatment characteristics, PICU, and length of hospital stay were recorded retrospectively.
Results: Twenty-three patients diagnosed with IEM were included in the study. The mean age of the patients was 48 months, and the majority of participants were female. 5/23 patients were followed up with the diagnosis of intoxication type, 10/23 patients with energy metabolism disorder type, and 8/23 patients with complex molecule disorder type. The median lactate level was (6.7 mmol/L, range: 0.8-32) higher in patients (7/23) who died in the PICU than in those who survived (p=0.016). Continuous renal replacement therapy was used in 6/23 (26%) patients, and invasive mechanical ventilation was applied to 3/23 (56.5%) patients.
Conclusion: IEM patients are challenging for pediatric intensive care professionals at the diagnostic and therapeutic levels. Undiagnosed patients at the time of admission to the PICU require a high degree of suspicion for prompt diagnosis and treatment. It is thought that the newborn screening program should be expanded. Aggressive and supportive treatment and specific metabolic disease treatment can be lifesaving, but these patients still have a high mortality rate.

Objective: Prevention and management of malnutrition are important in critically ill children. Parenteral nutrition (PN) is considered for patients who cannot tolerate enteral feeding. There are many reasons why PN cannot be delivered in the prescribed amount. We aimed to evaluate whether PN is delivered as prescribed in the pediatric intensive care units and to reveal the reasons for failure.
Method: Demographics, pediatric risk of mortality (PRISM) III scores, predicted death rates (PDR), indications for PN, duration of PN, vascular access site, daily amount of prescribed and delivered PN, reasons for not receiving PN as prescribed, and whether renal replacement therapy (RRT) was received were noted. The delivered/prescribed PN volume ratio was compared by gender, age, PRISM III score, PDR, indications for PN, duration of PN, and vascular access site.
Results: The most common indication for PN was failing to meet the targeted energy enterally (n=51, 69.9%). The duration of PN was ≤ 7 days in 40 (54.8%) patients and the type of vascular access was jugular venous catheter in 46 (63%) patients. 16 (21.9%) patients received RRT. PN was administered for 906 PN-days and the patients received the prescribed volume on 698 PN-days (77%). The most common reasons for not receiving the PN volume as prescribed were volume restrictions (n= 29, 39.7%) and electrolyte imbalance (n=13, 17.8%). Age, gender, weight, duration of PN, vascular access site, receiving RRT, PRISM III score, and PDR were not associated with receiving more than 0.8 of the prescribed PN volume. All gastrointestinal surgery patients received more than 0.8 of the prescribed amount.
Conclusion: In about a quarter of PN-days, the prescribed volume could not be delivered, often due to volume restrictions in the pediatric intensive care units. Setting the correct nutritional targets, individualizing nutritional support, and preventing and overcoming obstacles on the way to the targets may improve outcomes.

Objective: The purpose of this study was to identify the intrahospital transport practices of pediatric intensive care units and the adverse events that occurred during intrahospital transport in Türkiye.
Method: In this descriptive study, a questionnaire with 22 questions was used, which was created by the researchers based on the relevant literature. The questionnaire was filled out electronically.
Results: The study included 26 centers from 13 different provinces. In terms of intrahospital transport practices, 53.8% of the units lacked a written protocol for patient transport, and 92.3% did not utilize a transport preparation checklist. It was determined that in 65.4% of the units, a nurse accompanied a physician during transport. Examining the adverse events during intrahospital transport, findings reveal that 96.2% of the units reported a decrease in oxygen saturation, 80.8% hypotension, 73.1% hypothermia, 61.5% unplanned extubation, and 61.5% cardiac arrest. It was found that 7.7% of the units had an accident with mortality during transportation.
Conclusion: As a result of our study, it has been determined that many of the measures recommended in the literature to ensure the safe transport of intensive care patients are implemented at varying rates, and adverse events occur during intrahospital transport. In pediatric intensive care units, it is crucial to utilize a written in-hospital transport protocol when transporting pediatric patients and to enhance monitoring procedures during transportation.

Objective: Hereditary Angioedema (HAE) is a rare but life-threatening disease. It is aimed to present data on the clinical characteristics of our pediatric patients with HAE, whose symptoms usually start in childhood, but the delay in diagnosis is still a serious problem.
Method: Clinical and laboratory findings, family histories, and clinical characteristics of 14 patients with HAE diagnosed in our clinic between 1998-2019 were analyzed.
Results: Half of our patients diagnosed with HAE were girls, 78.5% of them were diagnosed with HAE type 1, and 21.4% were HAE type 2. All our patients had a family history, and 10 of them were diagnosed based on their family history. The mean age at diagnosis was 9.7±4.4 years and the mean age at the onset of the first angioedema symptom was 5.3±1.8 years. The delay in diagnosis was 4.4±4.1 years. The swollen areas included extremities (78.5%), abdominal attacks (71.4%), facial edema (57.1%), and laryngeal edema (21.4%). C4 levels were low in all patients. The mean C1 esterase inhibitor level was 0.69±0.08 g/l for HAE type 2 and 0.08±0.04 g/l for HAE type 1. The mean C1 esterase inhibitor functional activity level was 18.6±10.4% in HAE type 2.
Conclusion: Early diagnosis of the disease is critical for reducing morbidity and mortality due to attacks. There are very few studies in Türkiye that focus exclusively on pediatric HAE patients. Sharing our patients’ clinical findings and treatment plans for this rare disease is crucial for bringing the disease to light and raising awareness.

Objective: Pro-B type brain natriuretic peptide (proBNP) is released from cardiac ventricular myocytes as a result of increased volume and pressure. Troponin T plays a role in the contraction process. Both proteins may be elevated in many cardiac and non-cardiac conditions. Our aim is to evaluate troponin T values and cardiac findings of the patients in pediatric intensive care unit (PICU) with elevated proBNP levels.
Method: Patients with high proBNP values who were admitted to the PICU between January 2022 and January 2023 were included in the study. The clinical diagnoses, proBNP, and troponin T values were recorded. Information about the presence of heart disease and the status of systolic functions were obtained from echocardiographic examination reports.
Results: One hundred and ten patients were included in the study. Mean age of the patients was 2.48±3.41 years. Among the patients hospitalized in the pediatric intensive care unit, 41% had lower respiratory tract infections, and 20% had heart disease. The mean proBNP values were 11827.06±12652.82 ng/l, and troponin T was 201.41±737.74 ng/l. Ejection fraction (EF) was normal in 75% of the patients. The mean values of proBNP and troponin T in the patients with normal EF were 7284.74±8437.16 ng/l and 49.67±73.15 ng/l while the mean values of proBNP and troponin were 25129±13659.24 ng/l and 645.8±1380.74 ng/l in the patients with decreased EF (p<0.05, for both). ProBNP and troponin T values of the patients with decreased EF accompanied with or without heart disease were higher than those in the group with normal EF without existing heart disease (p<0.0001, for all). It was observed that decreased EF value was more common in cases who have proBNP>16314 ng/l and troponin T >114 ng/l (p=0.0031, p<0.0001, respectively).
Conclusion: ProBNP and troponin T values increase in many cardiac and non-cardiac diseases. However, quite high values of the parameters help to distinguish the patients with cardiac systolic dysfunction.

Background: Congenital Heart Disease (CHD) constitutes a significant cause of morbidity and mortality in newborns. Identifying CHD prenatally and understanding associated risk factors can aid in early diagnosis, intervention, and postnatal management. This study aims to assess risk factors for CHD using prenatal fetal echocardiography (FE) and investigate their correlation with postnatal diagnoses.
Patients and Methods: In this study, we included 993 pregnant women presenting to the pediatric cardiology outpatient clinic between December 2018 and December 2020, considered at risk for CHD. We retrospectively evaluated the cases’ postnatal echocardiography data with detected CHD during fetal echocardiography.
Results: The average age of the patients was 29.8±5.7, and the mean gestational week was 23.61±3.9. Among the pregnant women, 253 (25.47%) were primiparous, 740 (74.53%) were multiparous, 103 cases (9.32%) involved multiple pregnancies, and 259 (26.08%) had chronic diseases. The most common reason for fetal echocardiography referral was the suspicion of CHD in fetuses with dysmorphic findings detected during obstetric ultrasonography. Among the cases, 329 (33.1%) were classified as low-risk, while 664 (66.9%) as high-risk. Among all patients, the most commonly observed prenatal CHD were Ventricular Septal Defects (VSD) (8.2%), Hydrops Fetalis (6.1%), and large Atrial Septal Defects (ASD) (3.9%). The overall prevalence of CHD was 31.6%. The accuracy of postnatal echocardiography in confirming the diagnoses made with fetal echocardiography was 94%.
Conclusion: Prenatal diagnosis of congenital heart diseases is crucial for planning prenatal and postnatal management and providing families with the option of pregnancy termination in severe anomalies. Fetal echocardiography has shown significant potential for early diagnosis of CHD, even in low-risk fetuses, and its inclusion in routine prenatal screenings by increasing the number of experienced specialists and centers could play a crucial role in reducing CHD-related mortality and morbidity rates.

Objective: Healthcare-associated infections (HAIs) are a major issue in neonatal intensive care units (NICUs). The characteristics of HAIs and the distribution of pathogens might also vary. HAI surveillance is important for infection control to determine HAI rates and pathogen characteristics. The purpose of this study was to assess the rates of HAIs, distribution of HAI types, characteristics of the pathogens, and antibiotic susceptibility in the first four years of a newly opened NICU.
Method: In the NICU of Marmara University Pendik Training and Research Hospital, the infection control team identified HAIs and recorded the National Hospital Infection Surveillance Network in accordance with the standards of the Centers for Disease Control and Prevention throughout the period of four years after the unit’s opening. All patients in the first four years of the NICU were included in the study. The capacity of the NICU is 16 incubators and the average nurse/neonate ratio was 1/3 in this period.
Results: During the 4-year study period, 1301 patients were hospitalized in the NICU and 378 HAIs were detected. The overall HAI rate was 29.1% and the density was 21.8 per 1000 patient days. Neonatal groups with birth weights of 750 grams and 751–1000 grams had the highest rates and incidence density of HAIs. The most common HAI pathogens were Klebsiella spp. (27.8%), Staphylococcus spp. (26.2%), Acinetobacter baumannii (5.8%), and Escherichia coli (5.8%).
Conclusion: The risk of HAIs was found to be higher in neonates with a birth weight <1000 grams. In places where HAI rates are high such as NICUs, analyzing the characteristics of HAIs with active surveillance data is an essential component of infection control. This could enhance patient care and increase the survival of preterm infants with low birth weight.

Background: Acute generalized exanthematous pustulosis (AGEP) is a rare cutaneous drug reaction presenting with rapid-onset sterile pustules on edematous erythema.
Case: A 12-year-old female patient with acute gastroenteritis was consulted with complaints of pruritic erythema and high fever developing with small pustules on the 2nd day of ceftriaxone treatment. Lab tests showed an elevated absolute neutrophil count and lymphopenia. Ceftriaxone was discontinued immediately. The fever went away within 24 hours. According to EuroSCAR, the diagnosis of AGEP was confirmed. The skin biopsy was compatible with AGEP. After 6 weeks, a patch test with ceftriaxone was performed. A strong positive reaction to ceftriaxone was detected. Three months later, amoxicillin, amoxicillin-clavulanate, clarithromycin, and trimethoprim/sulfamethoxazole patch tests were performed, all were negative, and provocation tests were also planned.
Conclusion: AGEP is a severe cutaneous drug reaction. We wanted to emphasize that patch tests help identify the responsible drug and find a safe alternative.

The incidence of invasive candidiasis (IC) in neonatal intensive care units (NICUs) has significantly increased. Although C. albicans is still the most common pathogen detected in IC cases (60-75%), the increase in the use of prophylactic antifungal therapies and empirical echinocandin has led to a shift in detected pathogens to non-albicans candida species such as C. glabrata (2-8%). In the past, C. glabrata was considered one of the relatively non-pathogenic saprophytes of the normal flora. However, mucosal and systemic C. glabrata infections have escalated with the increase in the survival rates of premature newborns, prolonged hospitalization, and the widespread use of immunosuppressives and broad-spectrum antibiotics and started to appear more frequently as an important nosocomial pathogen, especially with its natural resistance to the azole antifungals. In this article, we aimed to draw attention to the importance of C. glabrata in NICUs by presenting extremely low-birth-weight premature twins with severe clinical course.