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Sensorineural hearing loss was found in 4 of our patients with a mutation in the ATP6V0A4 gene. Serum ammonia levels were found to be high in 3 patients with mutations in the ATP6V0A4 gene.

Adequate metabolic control is essential for optimal growth and preserved kidney function in distal kidney tubular acidosis patients. Distal kidney tubular acidosis may be associ- ated with hyperammonemia. We recommend keeping potassium levels at high-normal levels to reduce ammonia levels, especially in the absence of acidosis.
Adequate metabolic control is essential for optimal growth and preserved kidney function in distal kidney tubular acidosis patients. Distal kidney tubular acidosis may be associ- ated with hyperammonemia. We recommend keeping potassium levels at high-normal levels to reduce ammonia levels, especially in the absence of acidosis.
Our aim in this study is to reveal the frequency of febrile seizures in patients with Familial Mediterranean Fever and Periodic Fever, Aphthous stomatitis, Pharyngitis, cervical Adenitis syndrome and to compare it to normal population.

Patients with Familial Mediterranean Fever and Periodic Fever, Aphthous stomatitis, Pharyngitis, cervical Adenitis syndrome, who were diagnosed accord- ing to Turkish pediatric Familial Mediterranean Fever diagnostic criteria and Marshall criteria, were enrolled to the study. A form containing questions about febrile seizures history was pre- pared for Familial Mediterranean Fever and Periodic Fever, Aphthous stomatitis, Pharyngitis, cervical Adenitis syndrome patients. Demographic data and febrile seizures history of Periodic Fever, Aphthous stomatitis, Pharyngitis, cervical Adenitis patients were obtained by calling the parents by phone. Familial Mediterranean Fever patients were randomly selected during their routine follow-up. The frequency of febrile seizures in both d than in the general population. This increased frequency of febrile seizures in both periodic syndromes seems to be a result of recurrent fever.
The frequency of febrile seizures in patients with Familial Mediterranean Fever and Periodic Fever, Aphthous stomatitis, Pharyngitis, cervical Adenitis syndrome was found to be significantly higher than in the general population. This increased frequency of febrile seizures in both periodic syndromes seems to be a result of recurrent fever.
In patients with severe traumatic brain injury, it is possible to obtain a good long- term prognosis, prevent secondary injury, and decrease morbidity and mortality with the administration of appropriate treatments in the acute period. This study aims to evaluate the approaches of the pediatric intensive care specialists in Turkey towards the follow-up and treatment of severe traumatic brain injury by using a survey that had been prepared in light of the current pediatric severe traumatic brain injury guideline.

The survey, which included 45 questions, was prepared on the SurveyMonkey® system sent by e-mail to the centers, which were members of the Turkish Society of Pediatric Emergency and Intensive Care Medicine.

A total of 45 centers participated in the survey. In all centers, computed tomography of the brain was found to be the first choice as an imaging method for traumatic brain inju- ries. In terms of hyperosmolar therapy, 30 (66.6%) centers stated that they used 3% hypertonic saline, and 16 (35.5%) centers stated using 3% hypertonic saline+mannitol. Forty (86.96%) cen- ters stated that they did not use prophylactic hyperventilation in the first 48 hours. A total of 44 centers (97.78%) stated that they administered prophylactic antiepileptic drugs in the first 7 days while the most preferred antiepileptic drug was levetiracetam.

The results of our survey supported that the pediatric intensive care specialists in Turkey planned a large percentage of the treatment for patients with severe traumatic brain injury in line with the published traumatic brain injury guidelines.
The results of our survey supported that the pediatric intensive care specialists in Turkey planned a large percentage of the treatment for patients with severe traumatic brain injury in line with the published traumatic brain injury guidelines.
Coronavirus disease 2019 is a major health problem in all age groups. Although most clinical symptoms are respiratory, gastrointestinal symptoms are often reported. This is a major concern for children and has limited research coverage. In this study, we evaluated the frequencies of gastrointestinal symptoms and liver biochemical findings in children with coronavirus disease 2019 and their relationship with clinical course and length of hospital stay.

Demographic data, clinical, and laboratory findings of children with Coronavirus disease 2019 who were followed up by the Department of Pediatric Infectious Diseases between March 2020 and August 2020 were recorded. They were classified accord- ing to age groups as <5 years, 5-10 years, and >10 years. Laboratory findings were analyzed according to age groups. Demographic, clinical, and laboratory findings were compared in both situations, the presence of gastrointestinal symptoms and the presence of elevated liver enzymes. It was considered statistical with coronavirus disease 2019.
Atrial septal defect is a congenital heart disease usually diagnosed in childhood. This study aimed to evaluate the mid-term follow-up results of patients who underwent trans- catheter closure of atrial septal defect by comparing the devices and methods used in the procedure and investigating the complications of this procedure in children.

This study evaluated 232 patient files retrospectively. Of the 232 patients, 24 were excluded from the study due to missing files or data. Also, patients with multi-fenestrated atrial septal defect and aneurismatic septal tissue were excluded from the study. The following data were evaluated follow-up time, patient complaints, symptoms, trans- thoracic echocardiography, and transesophageal echocardiography findings (if performed), the size of the defect as measured by balloon-sizing, the size of the device used in the proce- dure, and major and minor complications.

The study included 208 children who were diagnosed with atrial septal defect. The mean age of the patients was 88.0 ± 56.5 months. Of the patients, 170 (81.7%) had no com- plaints. The success rate of the procedure was found to be 95.7%. While device embolization was the most common major complication, arrhythmia was the most common minor complica- tion. The complication rate was statistically different according to the device type used in the procedure.

Transcatheter closure of atrial septal defect is a safe method for atrial septal defect closure in pediatric patients. The study found that defect diameters measured by differ- ent methods were not correlated with each other. The procedure complication rates differed according to device type.
Transcatheter closure of atrial septal defect is a safe method for atrial septal defect closure in pediatric patients. The study found that defect diameters measured by differ- ent methods were not correlated with each other. The procedure complication rates differed according to device type.
In this study, we sought to describe the clinical, laboratory, and genetic character- istics of patients diagnosed with primary hemophagocytic lymphohistiocytosis. Thus, we aimed to evaluate the early diagnosis and appropriate treatment options for pediatric hemophago- cytic lymphohistiocytosis patients.

Medical records of 9 patients diagnosed with primary hemophago- cytic lymphohistiocytosis between November 2013 and December 2019 were analyzed retro- spectively. Clinical, genetic, and laboratory characteristics, family histories, initial complaints, physical examination findings, age at diagnosis, treatment choices, and clinical follow-up of all patients were investigated.

The mean age at diagnosis was 11 months (range 1.5 months to 17 years). Genetic analysis was performed in all patients, and a disease-related mutation was detected in 8 (89%) of them. Among clinical features, 6 (66%) patients had fever, 5 (56%) had splenomegaly, 4 (44%) had lymphadenopathy, 4 (44%) had skin rash, and 4 (44%) had neumotherapy should be started as early as possible, in order to achieve a disease remission. Patients should be referred to the appropriate bone marrow transplant center for hematopoi- etic stem cell transplantation as soon as they reach the disease remission.
The aim of the study was to evaluate the allergic rhinitis severity, to identify risk fac- tors associated with asthma, and to determine the frequency of comorbid conditions in allergic rhinitis patients with positive skin prick test.

Clinical characteristics of pediatric patients with allergic rhinitis were investigated. The frequency of comorbidities and risk factors for asthma development were investigated.

A total of 120 patients with a mean age of 13.05 ± 3.20 years were included in the study. Dermatophagoides pteronyssinus was the most common source of allergic sensitization (n = 78, 61.0%), whereas mild-persistent disease was the most common type of allergic rhini- tis severity (n = 44, 36.6%). Gedatolisib Sensitization to Dermatophagoides farinea, Dermatophagoides pteronyssinus, and Alternaria was more common in patients with a moderate-severe course of allergic rhinitis than in the mild group (P = .006, P = .008, and P = .005, respectively). The most frequent comorbidity in children with allergic rhinitis derate-severe allergic rhinitis course. Also, having moderate-severe allergic rhinitis severity and persistent allergic rhinitis symptoms are associated with the development of asthma. Awareness of the risk factors could prevent the progression and complications of allergic rhinitis in children.Both "new" and "old" bronchopulmonary dysplasia features overlap in preterm infants with severe bronchopulmonary dysplasia. The optimal ventilation strategy for infants with severe bronchopulmonary dysplasia has not been clarified yet. Principally, the lung is a multi-com- partmental heterogeneous tissue with regionally varying compliance and resistance. Generally, 2 critical strategical errors are common while ventilating infants with established bronchopulmonary dysplasia (i) ventilatory management as if they are still in the acute phase of respiratory distress syndrome and (ii) early extubation attempts with the aim of reducing ventilator-induced lung injury. Considering the heterogeneous character of bronchopulmo- nary dysplasia, although there is no unique formulation for optimal ventilation, the most physi- ologically appropriate ventilation mode may be the combined mode of volume-guaranteed synchronized intermittent mechanical ventilation and pressure support ventilation. With the volume-guaranteed synchronized intermittent mechanical ventilation mode, slow compart- ments of the lung with high resistance and low compliance can be adequately ventilated, while fast compartments having relatively normal resistance and compliance can be venti- lated well with the pressure support ventilation mode. The following settings are advisable frequency = 12-20 breaths per minute, tidal volume = 10-15 mL/min, positive end expiratory pressure = 7-12 cmH2O, and inspiratory to expiratory time ratio = 1 5. Higher oxygen satura- tions such as 92%-95% should be targeted to avoid subsequent pulmonary hypertension. In conclusion, there is no evidence-based ventilation recommendation for infants with severe bronchopulmonary dysplasia. However, given the changing pattern of the disease and the underlying pathophysiology, these infants should not be ventilated as if they were in the acute phase of respiratory distress syndrome.
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