A rare cause and an even rarer treatment of hypertension in a 5-year-old boy: Mid-aortic syndrome.

Renal artery stenosis is one of the common vascular diseases that cause hypertension in children. However, renal artery aneurysms and abdominal aortic aneurysms, which may be components of mid-aortic syndrome, are rarely associated with renal artery stenosis. Despite its rarity, early diagnosis and treatment are critical to prevent fatal complications. Currently, non-surgical invasive techniques are considered the first choice for treatment, but in some cases, surgery is inevitable. Here, we present a 5-year-old boy with a mid-aortic syndrome. The patient presented with a history of severe headache and epistaxis 5-6 times a day and was diagnosed with hypertension. A 9 × 9 mm saccular aneurysm on the anterior surface of the abdominal aorta at the level of the left renal artery ostium, and a 12 mm aneurysm in the left renal artery after a stenotic segment at the hilum level was detected in the doppler USG and contrast-enhanced imaging techniques. The patient was operated on electively. We used a PTFE patch to repair the abdominal aorta and, saphenous vein which was taken from his father to repair the renal artery. The patient recovered well and was discharged on the 18th day.

Multisystem inflammatory syndrome in children during severe acute respiratory syndrome coronavirus-2 pandemic in Turkey: A single-centre experience.

AIM: Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection may result in a life-threatening hyperinflammatory condition named multisystem inflammatory syndrome in children (MIS-C). We aimed to assess demographics, clinical presentations, laboratory characteristics and treatment outcomes of patients with MIS-C. METHODS: We performed a retrospective study of patients with MIS-C managed between August 2020 and March 2021 at Dr. Sami Ulus Maternity Child Health and Diseases Training and Research Hospital in Turkey. RESULTS: A total of 45 patients (23 male, 51%) with a median age of 8.7 years (interquartile range: 5.6-11.7 years) were enrolled to study. The SARS-CoV-2 serology was positive in 43 (95%) patients. Organ-system involvement included the dermatologic in 41 (91%), cardiovascular in 39 (87%), hematologic in 36 (80%) and gastrointestinal in 36 (80%) patients. Acute anterior uveitis was diagnosed in nine (20%) patients. Two patients presented with clinical findings of deep neck infection such as fever, neck pain, trismus, swelling and induration on the cervical lymph node. One patient presented with Henoch-Schonlein purpura-like eruption. Coronary artery dilatation was detected in five (11%) patients. For treatment of MIS-C, intravenous immunoglobulin was used in 44 (98%) patients, methylprednisolone in 27 (60%) and anakinra in 9 (20%) patients. The median duration of hospitalisation was nine days. All patients recovered. CONCLUSIONS: Children with MIS-C might have variable clinical presentations. Acute anterior uveitis might be a prominent presentation of MIS-C and require ophthalmological examination. It is essential to make patient-based decisions and apply a stepwise approach for the treatment of this life-threatening disease.

A novel therapeutic approach using the Zipper method to treat chorea in a pediatric-onset systemic lupus erythematosus patient.

Pediatric-onset systemic lupus erythematosus is among the prototypic systemic autoimmune diseases seen in children. Although the neuropsychiatric involvement rate varies during the course of the disease, it is an important cause of morbidity and mortality. The clinical picture of neuropsychiatric SLE (NPSLE) is highly variable, and neurological features can precede systemic findings, leading to some diagnostic difficulties. NPSLE requires early and aggressive immunosuppressive therapy. Some patients can be resistant to immunosuppressive therapy. Chorea is a rare manifestation that occurs in 1.2%-2% of SLE patients and can result from an immunologically mediated mechanism, antiphospholipid autoantibodies or ischemia. Herein we present the first case of pediatric-onset SLE diagnosed with central nervous system involvement and treated with Zipper method. The Zipper method is a new immunomodulation treatment. The clinical findings of the patient, which were resistant to corticosteroids and cyclophosphamide, resolved by this novel treatment.

Successful Treatment With Bortezomib for Refractory and Complicated Acquired Thrombotic Thrombocytopenic Purpura in an Adolescent Girl.

Thrombotic thrombocytopenic purpura (TTP) is a rare, dangerous, life-threatening disease characterized by microangiopathic hemolytic anemia and thrombocytopenia, along with organ dysfunction due to microangiopathy-related ischemia. Plasma exchange and steroids are used for initial treatment, and rituximab is often used in refractive patients. Caplacizumab, cyclophosphamide, and splenectomy are among other treatment options. It has been reported that bortezomib, a proteasome inhibitor, can be used in the management of refractory acquired TTP. Herein, we present a 16-year-old female patient who was monitored for acquired TTP and treated with high-dose steroids, plasma exchange, rituximab, cyclophosphamide, and N-acetylcysteine but developed renal, cardiac, gastrointestinal, and neurologic complications. The girl was then successfully treated with bortezomib, and she has been monitored in remission for 6 months. We consider that bortezomib is a beneficial treatment, especially in patients with refractory TTP.

The skills of defibrillation practice and certified life-support training in the healthcare providers in Turkey.

AIM OF THE STUDY: Successful cardiopulmonary resuscitation and early defibrillation are critical in survival after in- or out-of-hospital cardiopulmonary arrest. The scope of this multi-centre study is to (a) assess skills of paediatric healthcare providers (HCPs) concerning two domains: (1) recognising rhythm abnormalities and (2) the use of defibrillator devices, and (b) to evaluate the impact of certified basic-life-support (BLS) and advanced-life-support (ALS) training to offer solutions for quality of improvement in several paediatric emergency cares and intensive care settings of Turkey. METHODS: This cross-sectional and multi-centre survey study included several paediatric emergency care and intensive care settings from different regions of Turkey. RESULTS: A total of 716 HCPs participated in the study (physicians: 69.4%, healthcare staff: 30.6%). The median age was 29 (27-33) years. Certified BLS-ALS training was received in 61% (n = 303/497) of the physicians and 45.2% (n = 99/219) of the non-physician healthcare staff (P < .001). The length of professional experience had favourable outcome towards an increased self-confidence in the physicians (P < .01, P < .001). Both physicians and non-physician healthcare staff improved their theoretical knowledge in the practice of synchronised cardioversion defibrillation (P < .001, P < .001). Non-certified healthcare providers were less likely to manage the initial doses of synchronised cardioversion and defibrillation: the correct responses remained at 32.5% and 9.2% for synchronised cardioversion and 44.8% and 16.7% for defibrillation in the physicians and healthcare staff, respectively. The indications for defibrillation were correctly answered in the physicians who had acquired a certificate of BLS-ALS training (P = .047, P = .003). CONCLUSIONS: The professional experience is significant in the correct use of a defibrillator and related procedures. Given the importance of early defibrillation in survival, the importance and proper use of defibrillators should be emphasised in Certified BLS-ALS programmes. Certified BLS-ALS programmes increase the level of knowledge and self-confidence towards synchronised cardioversion-defibrillation procedures.

Acute necrotizing encephalopathy of childhood: a single-center experience

Background/aim: Acute necrotizing encephalopathy is a rare type of acute encephalopathy characterized by multi-ocal brain lesions and associated severe neurological findings and various organ dysfunctions may accompany it. Materials and Methods: Patients with acute necrotizing encephalopathy of childhood diagnosed by pediatric neurology and pediatric intensive care at Sami Ulus Maternity, Child Health and Diseases Training and Research Hospital between 2007 and 2020 were included in this study. Results: Nine patients (six females, three males) with a mean age of 4.05 ± 1.94 years (age range 1­6.5) were included in this study. The interval range between fever and encephalopathy in patients was 1­4 days. Influenza A (3H1N1, one untyped) was detected in four patients, influenza B in three patients, and no cause was found in two patients. Major clinical findings other than febrile encephalopathy in all patients were a hemodynamic shock in seven patients, seizures in six patients, vomiting in five patients, dystonia in three patients, and flaccid paralysis in the upper extremity in one patient. Despite all our treatment approaches, including plasmapheresis, moderate to severe neurological sequelae was observed in all of our patients, who survived even with significant radiological improvement. Three patients for whom we could not perform plasmapheresis died. Conclusion: Our study revealed that thalamic involvement increased as the interval shortened, and brainstem involvement increased in patients over four years of age. The presence of persistent vomiting accompanying encephalopathy during the parainfectious period and plasmapheresis treatment being a treatment option that could prevent mortality were cautionary for our study.

Successful management of acute pancreatitis due to apolipoprotein C-II deficiency in a 37-day-old infant.

Familial chylomicronemia is caused by deficiency of lipoprotein lipase or its co-activators. Here, we report an infant with apolipoprotein C-II (APOC2) deficiency, who developed acute pancreatitis 37 days after birth. He presented as abdominal sepsis with fever, irritability and abdominal distention. Amylase levels were low, but lipase levels and imaging findings were consistent with acute pancreatitis. He had severe hypertriglyceridemia (1091 mg/dl). Keeping him nil orally for two days resulted in rapid decrease in triglyceride levels and resolution of the clinical findings. APOC2 gene sequencing revealed a homozygous splice-site mutation (c.55+1G>C). To the best of our knowledge, this patient is not only the youngest reported patient with APOC2 deficiency, but also the youngest such patient who developed pancreatitis. Although he had a severe presentation, invasive methods to treat hypertriglyceridemia were not necessary. We emphasize that clinical findings and amylase levels are not reliable to diagnose pancreatitis in this age group.

Analysis of Intestinal and Nasopharyngeal Microbiota of Children with Meningococcemia in Pediatric Intensive Care Unit: INMACS-PICU Study.

Microbiota composition might play a role in the pathophysiology and course of sepsis, and understanding its dynamics is of clinical interest. Invasive meningococcal disease (IMD) is an important cause of community-acquired serious infection, and there is no information regarding microbiota composition in children with meningococcemia. In this study, we aimed to evaluate the intestinal and nasopharyngeal microbiota composition of children with IMD. Materials and Methods: In this prospective, multi-center study, 10 children with meningococcemia and 10 age-matched healthy controls were included. Nasopharyngeal and fecal samples were obtained at admission to the intensive care unit and on the tenth day of their hospital stay. The V3 and V4 regions of the 16S rRNA gene were amplified following the 16S Metagenomic Sequencing Library Preparation. Results: Regarding the alpha diversity on the day of admission and on the tenth day at the PICU, the Shannon index was significantly lower in the IMD group compared to the control group (p = 0.002 at admission and p = 0.001, on the tenth day of PICU). A statistical difference in the stool samples was found between the IMD group at Day 0 vs. the controls in the results of the Bray-Curtis and Jaccard analyses (p = 0.005 and p = 0.001, respectively). There were differences in the intestinal microbiota composition between the children with IMD at admission and Day 10 and the healthy controls. Regarding the nasopharyngeal microbiota analysis, in the children with IMD at admission, at the genus level, Neisseria was significantly more abundant compared to the healthy children (p < 0.001). In the children with IMD at Day 10, genera Moraxella and Neisseria were decreased compared to the healthy children. In the children with IMD on Day 0, for paired samples, Moraxella, Neisseria, and Haemophilus were significantly more abundant compared to the children with IMD at Day 10. In the children with IMD at Day 10, the Moraxella and Neisseria genera were decreased, and 20 different genera were more abundant compared to Day 0. Conclusions: We first found alterations in the intestinal and nasopharyngeal microbiota composition in the children with IMD. The infection itself or the other care interventions also caused changes to the microbiota composition during the follow-up period. Understanding the interaction of microbiota with pathogens, e.g., N. meningitidis, could give us the opportunity to understand the disease's dynamics.

Evaluation of nutritional status in pediatric intensive care unit patients: the results of a multicenter, prospective study in Turkey.

Introduction: Malnutrition is defined as a pathological condition arising from deficient or imbalanced intake of nutritional elements. Factors such as increasing metabolic demands during the disease course in the hospitalized patients and inadequate calorie intake increase the risk of malnutrition. The aim of the present study is to evaluate nutritional status of patients admitted to pediatric intensive care units (PICU) in Turkey, examine the effect of nutrition on the treatment process and draw attention to the need for regulating nutritional support of patients while continuing existing therapies. Material and Method: In this prospective multicenter study, the data was collected over a period of one month from PICUs participating in the PICU Nutrition Study Group in Turkey. Anthropometric data of the patients, calorie intake, 90-day mortality, need for mechanical ventilation, length of hospital stay and length of stay in intensive care unit were recorded and the relationship between these parameters was examined. Results: Of the 614 patients included in the study, malnutrition was detected in 45.4% of the patients. Enteral feeding was initiated in 40.6% (n = 249) of the patients at day one upon admission to the intensive care unit. In the first 48 h, 86.82% (n = 533) of the patients achieved the target calorie intake, and 81.65% (n = 307) of the 376 patients remaining in the intensive care unit achieved the target calorie intake at the end of one week. The risk of mortality decreased with increasing upper mid-arm circumference and triceps skin fold thickness Z-score (OR = 0.871/0.894; p = 0.027/0.024). The risk of mortality was 2.723 times higher in patients who did not achieve the target calorie intake at first 48 h (p = 0.006) and the risk was 3.829 times higher in patients who did not achieve the target calorie intake at the end of one week (p = 0.001). The risk of mortality decreased with increasing triceps skin fold thickness Z-score (OR = 0.894; p = 0.024). Conclusion: Timely and appropriate nutritional support in critically ill patients favorably affects the clinical course. The results of the present study suggest that mortality rate is higher in patients who fail to achieve the target calorie intake at first 48 h and day seven of admission to the intensive care unit. The risk of mortality decreases with increasing triceps skin fold thickness Z-score.

Croup as a Previously Unrecognized Symptom of COVID-19 in Infants.

Although the most common symptoms of coronavirus disease 2019 (COVID-19) in children are fever and cough, cases of croup associated with COVID-19 are reported in the literature and have increased sharply with the Omicron variant. We present severe acute respiratory syndrome coronavirus 2 as a viral agent in an infant presenting with croup.

Giant Right Atrial Myxoma with Fulminant Progression in an Infant.

Cardiac myxoma is rare in children. Myxomas are exceedingly rare in infancy. Right atrial myxomas were recorded in a small number of case reports involving infants worldwide. We report the case of a 2-month-old infant with giant right atrial myxoma. The case presented to our hospital with respiratory distress, and had pericardial and pleural effusion. Diagnosis of cardiac tumor was made with the aid of computerized tomography scan and echocardiogram. The tumor size was 3.1 × 3.4 × 3.9 cm. The patient worsened rapidly and had sudden cardiac arrest which did not respond to interventions. Postmortem cardiac autopsy confirmed the diagnosis of myxoma on gross examination and histology. This article aims to focus attention to the atypical size and location of this atrial myxoma, causing diagnostic difficulty in this infant.

Increased Severe Cases and New-Onset Type 1 Diabetes Among Children Presenting With Diabetic Ketoacidosis During First Year of COVID-19 Pandemic in Turkey.

Introduction: There have been some significant changes regarding healthcare utilization during the COVID-19 pandemic. Majority of the reports about the impact of the COVID-19 pandemic on diabetes care are from the first wave of the pandemic. We aim to evaluate the potential effects of the COVID-19 pandemic on the severity of diabetic ketoacidosis (DKA) and new onset Type 1 diabetes presenting with DKA, and also evaluate children with DKA and acute COVID-19 infection. Methods: This is a retrospective multi-center study among 997 children and adolescents with type 1 diabetes who were admitted with DKA to 27 pediatric intensive care units in Turkey between the first year of pandemic and pre-pandemic year. Results: The percentage of children with new-onset Type 1 diabetes presenting with DKA was higher during the COVID-19 pandemic (p < 0.0001). The incidence of severe DKA was also higher during the COVID-19 pandemic (p < 0.0001) and also higher among children with new onset Type 1 diabetes (p < 0.0001). HbA1c levels, duration of insulin infusion, and length of PICU stay were significantly higher/longer during the pandemic period. Eleven patients tested positive for SARS-CoV-2, eight were positive for new onset Type 1 diabetes, and nine tested positive for severe DKA at admission. Discussion: The frequency of new onset of Type 1 diabetes and severe cases among children with DKA during the first year of the COVID-19 pandemic. Furthermore, the cause of the increased severe presentation might be related to restrictions related to the pandemic; however, need to evaluate the potential effects of SARS-CoV-2 on the increased percentage of new onset Type 1 diabetes.

Pseudomonas Aeruginosa Sepsis in a Previously Healthy Infant with Subcutaneous Nodules and Mastoid Bone Destruction.

Pseudomonas aeruginosa septicemia is rare in previously healthy children. Skin lesions such as subcutaneous nodules and ecthyma gangrenosum may be the first manifestation of Pseudomonas infection that have rarely been reported. Herein we reported a previously healthy 6-month-old boy patient who presented with suppurative otitis media, multiple nodules, septic shock, and P. aeruginosa was identified in cultures of the blood, skin lesions, and purulent material of his ears.

Thiol-disulphide homeostasis is an oxidative stress indicator in critically ill children with sepsis. / La homeostasis de tiol/disulfuro es un indicador del estrés oxidativo en los niños críticamente enfermos con sepsis.

The aim of this study is to evaluate a novel oxidative stress marker (thiol-disulphide homeostasis) in paediatric sepsis and to determine their effects on the prognosis of sepsis. Patients diagnosed with sepsis (n= 38) and healthy controls (n= 40) were incorporated in the study. Total thiol, native thiol, disulphide, disulphide/total thiol, disulphide/native thiol, and native thiol /total thiol levels were measured in the sepsis and control groups. Additionally, the parameters were compared between survivors and non-survivors in the sepsis group. The levels of hemoglobin, white blood cell, platelet, lactate, and C-reactive protein were measured in patients with sepsis at diagnosis. The paediatric risk of mortality and paediatric logistic organ dysfunction scores of the patients were used to estimate the disease severity. The plasma thiol levels of the patients with sepsis were significantly lower than the control group (p < 0.001). This study showed that thiol/disulphide homeostasis is abnormal in children with sepsis in Paediatric Intensive Care Unit.

Objetivo. Evaluar un novedoso marcador del estrés oxidativo (la homeostasis de tiol /disulfuro) en la sepsis pediátrica y determinar sus efectos sobre el pronóstico de esta afección. Métodos. En el estudio, se incluyeron pacientes con diagnóstico de sepsis y controles sanos. Se midieron las concentraciones de tiol total, tiol nativo, disulfuro, disulfuro /tiol total, disulfuro /tiol nativo y tiol nativo/tiol total en los grupos con sepsis y de referencia. Se compararon los parámetros entre los supervivientes y los no supervivientes del grupo con sepsis. Se midieron las concentraciones de hemoglobina, leucocitos, trombocitos, lactato y proteína C-reactiva en los pacientes con sepsis al momento del diagnóstico. Se utilizaron el puntaje de riesgo de mortalidad pediátrico (Pediatric Risk of Mortality, PRISM) y el puntaje de disfunción orgánica (Pediatric Logistic Organ Dysfunction, PELOD) para estimar la gravedad de la enfermedad. Resultados. En el grupo con sepsis se incluyó a 38 pacientes y en el de referencia, a 40 niños sanos. Las concentraciones plasmáticas de tiol en los pacientes con sepsis fueron significativamente inferiores que las del grupo de referencia (p < 0,001). Conclusión. La homeostasis de tiol/disulfuro fue anormal en los niños con sepsis en la unidad de cuidados intensivos pediátricos.

Six year mortality profile of a Pediatric Intensive Care Unit: associaton between out-of-hours and mortality. / Perfil de mortalidad en una Unidad de Cuidados Intensivos Pediátricos: asociación entre la mortalidad y el período fuera del horario diurno semanal.

INTRODUCTION: Recent improvements in pediatric intensive care units (PICUs) were achieved through trained personnel and better technology, leading to an increase in patient care, survival rates and good prognosis. Nevertheless the quality of care varies according to the availability of human and technical resources. OBJECTIVE: The aim was to determine the correlation of mortality rates with daytime shifts compared to other shift periods (off-hours). METHODS: Work hours were defined as week days between 8:00 a.m. and 5:00 p.m., with in-house attendance of senior staff, and off-hours as week days between 5:00 p.m. and 8:00 a.m., weekends and public holidays, with one resident covering the PICU and senior staff directly available only on-call. Mortality causes in children hospitalized in the intensive care unit were classified. RESULTS: During this period, a total of 2,187 patients were hospitalized in the PICU. A total of 394 patients died; 151 were girls (38.3 %) and 243 boys (61.7 %). Evaluating time of mortality showed that death occurred mostly at out-off hours: 244 (61.9 %) vs. 150 (38.1 %) during the daytime shift, which was statistically significant (p < 0.05). In addition, age, gender and the length of stay in the PICU were not significant (p > 0.05) when daytime was compared to out-off hours. The conditions which accompanied death were evaluated, the relationship between working hours and death was also significant (p < 0.05). More conditions related to mortality were encountered at off-hours. CONCLUSION: Off-hours without 24 hour attendance of senior staff, was associated with higher mortality.

Introducción. El personal capacitado y los avances tecnológicos mejoraron las unidades de cuidados intensivos pediátricos (UCIP); por ende, mejoraron la atención, la supervivencia y el pronóstico. Pero la calidad de la atención varía según la disponibilidad de recursos humanos y técnicos. Objetivo. Determinar la correlación de las tasas de mortalidad dentro y fuera del horario diurno en días de semana. Métodos. Se definió horario de días de semana de 8:00 a. m. a 5:00 p. m., el período fuera del horario fueron de 5:00 p. m. a 8:00 a. m., fines de semana y feriados, con un residente en la UCIP y un médico de planta de guardia pasiva. Se clasificaron las causas de mortalidad en la UCIP. Resultados. Se hospitalizaron 2187 pacientes en la UCIP. Fallecieron 394; 151 niñas (38,3 %) y 243 varones (61,7 %). Según el horario de muerte, la mayoría ocurrió fuera del horario diurno 244 (61,9 %) versus a 150 (38,1 %) durante el turno diurno, una diferencia estadísticamente significativa (p < 0,05). La edad, el sexo y la duración de la hospitalización en la UCIP no fueron significativos (p > 0,05) al comparar el período dentro y fuera del horario diurno. Se evaluaron las afecciones que acompañaron la muerte. La relación entre el horario de médicos de planta y la muerte fue significativa (p < 0,05). Hubo más afecciones asociadas a mortalidad fuera del horario diurno. Conclusión. El período fuera del horario diurno sin médicos de planta estuvo asociado con mayor mortalidad.

Clinical impact and efficacy of bedside echocardiography on patient management in pediatric intensive care units (PICUs): A prospective study.

OBJECTIVE: To determine the indication and necessity of echocardiographic assessment and therapeutic interventions in critically ill children. METHODS: A total of 140 children, including 75 mechanically ventilated (MV) and 65 spontaneously breathing (SB) children, who were admitted consecutively from March to August 2013 were evaluated prospectively. Data regarding the indication for echocardiography and therapeutic approaches used were documented. For evaluating disease severity, the Pediatric Risk of Mortality Score III (PRISM) was ascertained. The correlation between PRISM score and the requirement of echocardiographic evaluations were analyzed. RESULTS: Patients ages were between 45 days to 18 years. The male-to-female ratio was 1.33. In 35.4% patients who underwent echocardiographic evaluation, no definitive alteration occurred in treatment approach, whereas in the remaining 64.6% patients, decisive or supplemental information was gathered. Echocardiography was indicated in 88% MV children and 46.2% SB children. Echocardiographic evaluation was necessary in MV children and there was a positive correlation between the PRISM score and the requirement of echocardiographic assessment (p<0.001). CONCLUSION: Echocardiographic evaluation is an invaluable tool especially in MV children and the requirement of echocardiographic assessment increases according to clinical severity. Basic training for intensivists in this procedure is crucial and needs to be improved and supported in critically ill.