Long-term outcomes of standardized training for caregivers of children with tracheostomies: The IStanbul PAediatric Tracheostomy (ISPAT) project.

BACKGROUND AND OBJECTIVES: Children with tracheostomies are at increased risk of tracheostomy-related complications and require extra care. Standardized training programs for caregivers can improve tracheostomy care and reduce complications. In this study, we compared caregiver knowledge and skill scores after a standardized theoretical and practical training program on tracheostomy care (IStanbul PAediatric Tracheostomy (ISPAT) project) immediately and 1 year post-training and evaluated how this training affected the children's clinical outcomes. MATERIALS AND METHODS: We included 32 caregivers (31 children) who had received standardized training a year ago and administered the same theoretical and practical tests 1 year after training completion. We recorded tracheostomy-related complications and the number and reasons for admission to the healthcare centers. All data just before the training and 1 year after training completion were compared. RESULTS: After 1 year of training completion, the median number of correct answers on the theoretical test increased to 16.5 from 12 at pretest (p < 0.001). Compared with pretest, at 1-year post-training practical skills assessment scores, including cannula exchange and aspiration, were significantly higher (both p < 0.001) and mucus plug, bleeding, and stoma infection reduced significantly (p = 0.002, 0.022, and 0.004, respectively). Hands-on-training scores were better than pretest but declined slightly at 1 year compared to testing immediately after training. Emergency admission decreased from 64.5% to 32.3% (p = 0.013). Hospitalization decreased from 61.3% to 35.5% (p = 0.039). CONCLUSION: Our findings indicate that caregiver training can lead to a persistent increase in knowledge and skill for as long as 1 year, as well as improvements in several measurable outcomes, although a slight decrease in scores warrants annual repetitions of the training program.

Evaluation of 601 children with multisystem inflammatory syndrome (Turk MISC study).

PURPOSE: Due to its link with the 2019 coronavirus, the multisystem inflammatory syndrome in children (MISC) has garnered considerable international interest. The aim of this study, in which MISC patients were evaluated multicenter, and the data of the third period of the Turk-MISC study group, to compare the clinical and laboratory characteristics and outcomes of MISC patients who did and did not require admission to an intensive care unit (ICU). METHODS: This retrospective multicenter observational study was carried out between June 11, 2021, and January 01, 2022. The demographics, complaints, laboratory results, system involvements, and outcomes of the patients were documented. RESULTS: A total of 601 patients were enrolled; 157 patients (26.1%) required hospitalization in the intensive care unit (ICU). Median age was 8 years (interquartile range (IQR) 4.5-11.3 years. The proportion of Kawasaki disease-like features in the ICU group was significantly higher than in the non-ICU group (56.1% vs. 43.2% p = 0.006). The ICU group had considerably lower counts of both lymphocytes and platelets (lymphocyte count 900 vs. 1280 cells × µL, platelet count 153 vs. 212 cells × 103/ µL, all for p< 0.001). C-reactive protein, procalcitonin, and ferritin levels were significantly higher in the ICU group (CRP 164 vs. 129 mg/L, procalcitonin 9.2 vs. 2.2 µg/L, ferritin 644 vs. 334 µg/L, all for p< 0.001). Being between ages 5-12 and older than 12 increased the likelihood of hospitalization in the ICU by four [95% confidence intervals (CI)1.971-8.627] and six times (95% CI 2.575-14.654), respectively, compared to being between the ages 0-5. A one-unit increase in log D-dimer (µg/L) and log troponin (ng/L) was also demonstrated to increase the need for intensive care by 1.8 (95% CI 1.079-3.233) and 1.4 times (95% CI 1.133-1.789), respectively.     Conclusion: By comparing this study to our other studies, we found that the median age of MISC patients has been rising. Patients requiring an ICU stay had considerably higher levels of procalcitonin, CRP, and ferritin but significantly lower levels of lymphocyte and thrombocyte. In particular, high levels of procalcitonin in the serum might serve as a valuable laboratory marker for anticipating the need for intensive care. WHAT IS KNOWN: • Lymphopenia and thrombocytopenia were an independent predictor factors in patients with MISC who needed to stay in intensive care unit. • The possibility of the need to stay in the intensive care unit in patients with MISC who had Kawasaki disease-like findings was controversial compared with those who did not. WHAT IS NEW: • A one-unit increase log D dimer and log troponin was demonstrated to require for intensive care unit by 1.8 and 1.4 times, respectively. • Serum procalcitonin levels had the best performance to predict stay in the intensive care unit stay.

Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?

BACKGROUND: Cystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment. METHODS: This retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment. RESULTS: In 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased. CONCLUSIONS: Patients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide.

Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data.

BACKGROUND: We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV1) decline and LT candidates without rapid FEV1 decline in the last year to identify a preventable cause in patients with such rapid FEV1 decline. METHODS: All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV1 below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV1 decline of more than 20% in the previous year but had other indications for LT (Group 2). Demographic and clinical features were compared between the two groups. RESULTS: Of 1488 patients registered in CFRT, 58 had a need for LT. Twenty patients were included in Group 1 and others in Group 2. Our findings did not reveal any significant variations in treatment, chronic infection status, or complications between the two groups. The average weight z-score was significantly higher in Group 1. Positive correlations were detected between weight z-score and FEV1 in 2017 in Group 1 and between FEV1 values in 2017 and 2018 in Group 2. CONCLUSIONS: There appears to be a relationship between the nutritional status and weight z-scores of CF patients and pulmonary function, which may indirectly affect the need for lung transplantation referral.

Evaluation of diagnostic components and management of childhood pulmonary tuberculosis: a prospective study from Istanbul, Turkey.

INTRODUCTION: The diagnosis of childhood tuberculosis is difficult and most of the patients are diagnosed clinically. The objective of this study is to reveal the diagnostic and therapeutic components of childhood pulmonary tuberculosis and to analyze the changes that occurred in our country over the years. METHODOLOGY: All patients diagnosed with tuberculosis between 2006 and 2016 were included. Demographic characteristics, diagnostic and treatment outcomes were recorded and patients were followed up prospectively. RESULTS: A total of 492 patients were included in the study. 97% had Bacillus Calmette-Guerin vaccine, 36% were diagnosed with microbiologically-confirmed tuberculosis and 64% were diagnosed with clinically-proven tuberculosis. 94% of the patients had symptoms consistent with tuberculosis, all patients had radiologic findings, 74% had a history of tuberculosis contact and 63% had tuberculin skin test positivity. The diagnoses included primary tuberculosis in 62%, secondary tuberculosis in 21%, progressive primary tuberculosis in 13% and miliary tuberculosis in 4%. 48% of the patients received a treatment regimen containing three drugs as the initial treatment, and drug-related side effects developed in 12%. Isoniazid resistance was detected in 13% of the patients and rifampicin resistance was detected in 8%. None of the patients died due to tuberculosis. In the last 50 years in Turkey, the rates of Bacillus Calmette-Guerin vaccination and diagnosis of tuberculosis cases have increased and the mortality rates have decreased over the years. CONCLUSIONS: Our study is one of the few prospective studies and revealed the differences between the recent data and the past 50 years in childhood tuberculosis in Turkey.

The ISPAT project: Implementation of a standardized training program for caregivers of children with tracheostomy.

BACKGROUND: Tracheostomy-related morbidity and mortality mainly occur due to decannulation, misplacement, or obstruction of the tube. A standardized training can improve the skills and confidence of the caregivers in tracheostomy care (TC). OBJECTIVE: Our primary aim was to evaluate the efficiency of standardized training program on the knowledge and skills (changing-suctioning the tracheostomy tube) of the participants regarding TC. MATERIALS AND METHODS: Sixty-five caregivers of children with tracheostomy were included. First, participants were evaluated with written test about TC and participated in the practical tests. Then, they were asked to participate in a standardized training session, including theoretical and practical parts. Baseline and postintervention assessments were compared through written and practical tests conducted on the same day. RESULTS: A significant improvement was observed in the written test score after the training. The median number of correct answers of the written test including 23 questions increased 26%, from 12 to 18 (p < .001). The median number of correct steps in tracheostomy tube change (from 9 to 16 correct steps out of 16 steps, 44% increase) and suctioning the tracheostomy tube (from 9 to 17 correct steps out of 18 steps, 44% increase) also improved significantly after the training (p < .001, for both). CONCLUSION: Theoretical courses and practical hands-on-training (HOT) courses are highly effective in improving the practices in TC. A standardized training program including HOT should be implemented before discharge from the hospital. Still there is a need to assess the impact of the program on tracheostomy-related complications, morbidity, and mortality in the long term.

Lifesaving Treatment of Aortic Valve Staphylococcus aureus Endocarditis: Daptomycin and Early Surgical Therapy.

Infective endocarditis (IE) is an uncommon infection in children. The recommended treatment for native valve endocarditis secondary to methicillin-susceptible Staphylococcus aureus infection is antistaphylococcal penicillins such as nafcillin or oxacillin. If the initial therapy fails in IE, it can lead to catastrophic results. Nowadays, daptomycin is the best alternative antimicrobial agent to treat children with severe infections, when standard antimicrobial therapy does not yield a result. Herein, in this article, we described a case of a 16-year-old boy who had aortic valve S. aureus endocarditis with septic embolization and stroke. The patient was successfully treated only with daptomycin as well as surgical therapy in the early phase of the infection.

Caregiver burden and related factors in caregivers of patients with childhood-onset systemic lupus erythematosus.

OBJECTIVE: Having a child with a chronic illness is a source of stress for the whole family, especially the primary caregiver. The aim of this study was to evaluate the associations between caregiver burden and both the caregiver's and child's psychological symptoms in a cohort of children with systemic lupus erythematosus (SLE). METHODS: Thirty-four patients (aged 9-18 years) with childhood-onset SLE and their caregivers participated in this study. The control group was composed of healthy children and their caregivers. Questionnaires were used to evaluate caregiver burden and the psychological status of parents and children and adolescents with and without SLE. RESULTS: No significant difference was found between the study and control groups for caregiver burden, anxiety and depression in parents, and psychological status in children. Caregiver burden was positively correlated with parent's depression, anxiety, and behavioral and peer problems of the children, and it was negatively correlated with the children's prosocial behaviors. According to regression analyses, the parents' depression and children's peer relationship had a positive effect on caregiver burden scores. CONCLUSION: Physicians should be aware of the presence of psychological symptoms in patients with childhood-onset SLE and their caregivers because it can affect caregiver burden and the caregiver's psychological state. Key points •Caregiver burden was positively correlated with parent's depression and anxiety. •Caregiver burden was positively correlated with children's behavioral and peer problems. •Caregiver burden was negatively correlated with child's prosocial behaviors.

Post-infectious bronchiolitis obliterans in children: Clinical and radiological evaluation and long-term results.

BACKGROUND AND OBJECTIVE: This study aims to evaluate clinical and radiological findings and treatment outcomes of the patients with PIBO. METHODS: One hundred fourteen children were enrolled. Initial demographic and clinical findings were evaluated. Pre- and post-treatment clinical and radiological findings were compared. RESULTS: The median age of the patients at initial pulmonary injury was 7,2 months, the median age at diagnosis was 17.5 months. Persistent wheezing was the most common complaint. Thirty-five patients had mechanical ventilation history. 82,5% of patients had clinical improvement. Bronchiectasis, atelectasis, hyperinflation and air trapping in HRCT improved significantly with treatment. Post-treatment Bhalla scores decreased from 8.3 to 6.5 (p= 0,001). Improvement was observed in radiological and clinical findings after treatment. CONCLUSIONS: This study is one of the largest studies in the literature and one of the few studies that evaluate clinical and radiological outcomes of patients with PIBO.

The Possible Role of IVIG in the Treatment of Atrial Fibrillation Accompanied by Fulminant Myocarditis in a 12-Year-Old Pediatric Patient.

BACKGROUND: Fulminant myocarditis (FM) is a potentially lethal condition in children due to rapid progressive hemodynamic instability and cardiogenic shock. Patients with FM might show different clinical manifestations on emergency department admission. CASE: Herein, we describe the case of a 12-year-old girl who was admitted to our institution's emergency department due to complaints of abdominal pain and incessant vomiting. However, we detected an early onset of atrial fibrillation (AF) accompanied by FM. The patient's condition of AF and severe hemodynamic disorder was successfully treated in our institution's pediatric intensive care unit. CONCLUSION: To the best of our knowledge, this is the first report of the co-occurrence of FM and AF successfully treated in childhood. This case report will serve as a guide for the treatment of cases with FM accompanied by AF.

Subdural empyema, brain abscess, and superior sagittal sinus venous thrombosis secondary to Streptococcus anginosus.

Streptococcus anginosus can be frequently isolated from brain abscesses, but is a rare cause of the liver, lung, and deep tissue abscesses. In this report, we present a patient with subdural empyema, brain abscess, and superior sagittal cerebral venous thrombosis as complications of rhinosinusitis whose purulent empyema sample yielded S. anginosus. A 13-year-old female patient was referred to our pediatric intensive care unit with altered mental status, aphasia, and behavioral change. On a brain computed tomography scan, subdural empyema extending from the left frontal sinus to the frontal interhemispheric area and left hemispheric dura was detected. Intravenous ceftriaxone, vancomycin, and metronidazole treatments were started. Subdural empyema was surgically drained. Postoperative brain magnetic resonance venography imaging showed superior sagittal sinus thrombosis. Cultures obtained from purulent empyema sample revealed S. anginosus. On the third day of hospitalization, a brain computed tomography scan showed brain edema, especially in the left hemisphere and significantly increased subdural empyema that had been previously drained. She was reoperated and decompressive craniectomy was performed. On the fifth day, partial epileptic seizures occurred. Brain magnetic resonance imaging showed a brain abscess on the interhemispheric area. The magnetic resonance imaging findings of abscess formation improved on 30th day and she was discharged on the 45th day after the completion of antibiotic therapy.

Balanced Double Aortic Arch Causing Persistent Respiratory Symptoms Mimicking Asthma in an Infant.

Double aortic arch (DAA) is a common form of complete vascular ring. The condition leads to airway obstruction and compression of the esophagus. A balanced type of DAA is an extremely rare anomaly. The anatomical aberration cannot be easily distinguished by transthoracic echocardiography when an infant has chronic respiratory distress. Herein we presented a case of an 11-month-old infant who had chronic respiratory distress and a balanced type of DAA.

A Rare Presentation of Neurobrucellosis in a 6-Year-Old Pediatric Patient with Sagittal Sinus Thrombosis.

Brucellosis is one of the most common zoonosis worldwide. It is still endemic in many regions of the world. A 6-year-old female was admitted to the emergency department (ED) due to a sudden change in consciousness, urinary incontinence, vomiting, and difficulty in walking. Neurological examination demonstrated abducens nerve paralysis, mild-to-moderate motor deficit in hemiparesis in the left arm. Brain magnetic resonance imaging showed a hemorrhagic focus at the right frontal lobe and thrombosis in the superior sagittal sinus of the brain. The diagnosis of neurobrucellosis was confirmed by identifying Brucella spp. in the blood culture on the day 6 of pediatric intensive care unit admission; thus, trimethoprim-sulfamethoxazole and rifampicin, and ceftriaxone were promptly initiated. Despite neuroprotective management and acetazolamide, the patient's neurological problems and high intracranial pressure (ICP) persisted. An external ventricular drainage tube and a Codman ICP monitor were placed to be on the consent vigilance of the patient's neurological condition. The patient's ICP continued to increase despite the current treatment regimen; therefore, a decompressive bitemporal craniectomy was performed. The ICP level of the patient returned to its normal range immediately after the craniectomy. The patient did not have any notable neurologic sequelae at the first-year follow-up. Neurobrucellosis is a rare complication of systemic brucellosis and may present as meningitis, encephalitis, myelitis, radiculitis, and/or neuritis. Herein, we describe a six-year-old girl with brucellosis complicated with cerebral vein thrombosis. This case illustrates the need for close monitoring of patients with unexplained neurological signs or symptoms for brucellosis in endemic areas.

Development of Myocardial Infarction in a 12-Year-Old Female after the Use of Inhaled Salbutamol.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) accounts for 0.023% of all cases reported in pediatric patients. According to literature, only a handful of ALCAPA patients are able to reach adulthood. Clinical manifestations of ALCAPA range from fatigue during exercise to sudden death in adulthood. Herein, we described a 12-year-old symptomatic patient with ALCAPA who had severe chest pain after using salbutamol treatment for presumed asthma. ALCAPA is one of the curable versions of myocardial ischemia and infarction in childhood. Due to clinical findings in conjunction with electrocardiogram and echocardiography, a computed tomography scan with coronary angiography was performed and the diagnosis of ALCAPA was confirmed. We presented this case because ALCAPA-related myocardial ischemia and infarction in children are rare with only sporadic cases reported. This case illustrated the need for close monitoring and surgery as the best treatment for ALCAPA associated with myocardial infarction.

A 13-Year-Old Boy Who Has Kawasaki Disease Shock Syndrome Presents with Parotitis.

We report a 13-year-old boy who (initially) had symptoms of toxic shock-like syndrome and mumps. Then, the patient was hospitalized in the pediatric intensive care unit (PICU) because of his ongoing hemodynamic instability (low blood pressure of 70/30 mm Hg and capillary refill time of > 4 seconds). During his stay in the PICU, the patient was treated with fluid resuscitation and vasoactive infusion and at the same time was diagnosed with Kawasaki disease shock syndrome (KDSS), when giant right coronary artery aneurysms were detected on echocardiographic examination. This case illustrates the risk of KDSS in patient who carries both parotitis and toxic shock-like syndrome. The clinicians should be cautious about detecting any types of coronary artery aneurysms in such patients. This is the first case of KDSS associated with parotitis reported in the literature.

Pulmonary Edema in the Acute Stage of Rheumatic Fever Treated with Double-Valve Replacement in a Pediatric Patient.

Cardiogenic pulmonary edema (CPE) is a rare clinical condition of acute rheumatic fever (ARF) in the early stage. Generally, CPE can be convalesced by steroid and anticongestive treatment. Herein, we describe a case of a 14-year-old boy with ARF presenting with bilateral pulmonary edema secondary to acute mitral and aortic insufficiency. In this case, the pulmonary edema of ARF was successfully managed by combined surgical replacements of both valves.