Mortality risk factors among critically ill children with MIS-C in PICUs: a multicenter study.

BACKGROUND: This study evaluated of clinical characteristics, outcomes, and mortality risk factors of a severe multisystem inflammatory syndrome in children admitted to a the pediatric intensive care unit. METHODS: A retrospective multicenter cohort study was conducted between March 2020 and April 2021 at 41 PICUs in Turkey. The study population comprised 322 children diagnosed with multisystem inflammatory syndrome. RESULTS: The organ systems most commonly involved were the cardiovascular and hematological systems. Intravenous immunoglobulin was used in 294 (91.3%) patients and corticosteroids in 266 (82.6%). Seventy-five (23.3%) children received therapeutic plasma exchange treatment. Patients with a longer duration of the PICU stay had more frequent respiratory, hematological, or renal involvement, and also had higher D-dimer, CK-MB, and procalcitonin levels. A total of 16 patients died, with mortality higher in patients with renal, respiratory, or neurological involvement, with severe cardiac impairment or shock. The non-surviving group also had higher leukocyte counts, lactate and ferritin levels, and a need for mechanical ventilation. CONCLUSIONS: In cases of MIS-C, high levels of D-dimer and CK-MB are associated with a longer duration of PICU stay. Non-survival correlates with elevated leukocyte counts and lactate and ferritin levels. We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality. IMPACT: MIS-C is a life-threatening condition. Patients need to be followed up in the intensive care unit. Early detection of factors associated with mortality can improve outcomes. Determining the factors associated with mortality and length of stay will help clinicians in patient management. High D-dimer and CK-MB levels were associated with longer PICU stay, and higher leukocyte counts, ferritin and lactate levels, and mechanical ventilation were associated with mortality in MIS-C patients. We were unable to show any positive effect of therapeutic plasma exchange therapy on mortality.

Role of automated red blood cell exchange in the treatment of aluminum phosphide poisoning: A case report and review of the literature.

Aluminum phosphide (AIP) is a fumigant commonly used in agricultural areas. AIP is frequently misused for suicidal purposes because it is easily accessible. AIP poisoning causes severe metabolic acidosis, resistant hypotension, acute respiratory distress syndrome, and multiorgan failure with cardiogenic shock. Despite supportive management and intensive care, most patients die following AIP ingestion because there is no specific antidote. In this case report we present a 15-year-old female who presented with vomiting, coma and epigastric pain. She developed resistant metabolic acidosis and hypotension due to AIP poisoning. Although supportive treatment did not result in clinical improvement, she was successfully treated with automated red blood cell exchange. Automated red blood cell exchange is a procedure which is used to exchange the patient erythrocyte mass with donor red blood cell. Although automated red blood cell exchange is a preferred treatment method in the complications of sickle cell anemia, some blood diseases and infectious diseases such as malaria and babesiosis, there is little information about its use in poisoning. To the best of our knowledge, this is the first child with AIP poisoning who was treated with automated red blood cell exchange.

The Role of Therapeutic Plasma Exchange in the Treatment of Childhood Intoxication: A Single-Center Experience.

OBJECTIVES: Therapeutic plasma exchange is used to treat neurologic, hematological, renal, and autoimmune diseases with a known or suspected etiopathogenesis. However, there is incomplete understanding of the use of therapeutic plasma exchange in pediatric cases of intoxication. This study investigated 5 years of experience with therapeutic plasma exchange to treat intoxication cases. DESIGN: A retrospective, case series, single-center study. SETTING: PICU of Baskent University, Dr. Turgut Noyan Teaching, and Medical Research Center Hospital in Adana, Turkey. PATIENTS: Fourteen patients diagnosed with intoxication who underwent therapeutic plasma exchange between January 2013 and January 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Data pertaining to 14 patients, including their medical history (exposure to drugs/toxicants), demographics, initial presentation, and severity of clinical symptoms (requirement of mechanical ventilation, Glasgow Coma Scale score, and the pediatric severity of illness score [Pediatric Logistic Organ Dysfunction] were retrospectively reviewed. The most common indication for therapeutic plasma exchange was multiple drug intoxication, followed by amitriptyline, Amanita phalloides mushroom, carbamazepine, mercury, verapamil, and botulism. All patients underwent therapeutic plasma exchange and two patients underwent hemodialysis before therapeutic plasma exchange. There was no mortality or complications related to the therapeutic plasma exchange procedure. Clinical improvement was observed after therapeutic plasma exchange in 13 of the 14 patients; one patient with verapamil intoxication died. CONCLUSIONS: Therapeutic plasma exchange appears to be safe and effective for treating pediatric cases of intoxication, including multidrug and amitriptyline intoxication, and is associated with significant recovery in the majority of severely affected patients. Treatment of intoxication with therapeutic plasma exchange should be guided primarily by the properties of the causative toxic substances/drugs, and consideration of patient age, the severity of clinical symptoms, Pediatric Logistic Organ Dysfunction score and response to initial supportive and antidotal treatment.

Hematologic and bone marrow changes in children with protein-energy malnutrition.

BACKGROUND: All systems in an organism are affected by protein-energy malnutrition (PEM), but one of the worst affected is the hematopoietic system. Today PEM remains a very serious problem in developing countries. We examined the relationships between clinical features, hematological, and bone marrow changes with severe PEM from Turkey. METHOD: We evaluated 34 (11 females and 23 males) consecutive cases of severe PEM, with no underlying diseases aged 3-20 months. The clinical nutritional conditions of the patients were determined using the Wellcome-Trust PEM classification. Ten of the patients were in the Marasmic-Kwashiorkor (M-K) group, 10 were in the Kwashiorkor (KW) group, and 14 were in the Marasmic (M) group. Full blood count, protein, albumin, serum iron (SI), iron-binding capacity (TIBC), ferritin, vitamin B12, folic acid, complement-3 (C3), complement-4 (C4), and bone marrow were investigated in all groups. RESULTS: Anemia was detected in 97% of patients. We determined serum iron levels were low in 67.6% of the patients, TS levels were low in 76.4% of the patients and ferritin levels were low in 20.5%. The level of vitamin B12 was normal in all patients. Bone marrow analysis showed erythroid series hypoplasia in 28.5% of patients in the M group, 50% in the KW group, and 30% in the M-K group. Marrow iron was absent in 58.8% of patients. CONCLUSION: The most common hematologic change in the children with PEM was anemia and major cause of anemia was iron deficiency in this study. Patients with severe PEM have normal Vit B12 and serum folate levels. Most of the patients with severe PEM had normal cellularity with megaloblastic and dysplastic changes in bone marrow due to the inadequate and imbalanced intake of protein and energy.

The Effects of Protein Intake on Clinical Outcome in Pediatric Intensive Care Units.

OBJECTIVE: Factors such as increased metabolic needs and inadequate calorie and protein intake increase the risk of malnutrition in critically ill children admitted to the pediatric intensive care unit. This study aimed to determine the risk of malnutrition and associated clinical outcomes. MATERIALS AND METHODS: Data from all patients aged 1 month to 18 years in 4 pediatric intensive care units in Adana, Turkey, were prospectively collected. Patient anthropometric data, the duration of mechanical ventilation, the length of stay in pediatric intensive care unit, 60-day mortality, nutritional status, and calorie and protein intake were recorded. RESULTS: A total of 111 patients were included in the study. There was a significant difference between survivor and non-survivor patients in terms of calorie and protein intake 48 hours after admission and protein intake on the seventh day after admission (P = .001, P = .000, and P = .003, respectively). No significant correlation was found between the length of pediatric intensive care unit stay, sepsis, and calorie intake in the first week. It was found that 1 g/kg/ day increase in protein intake on the seventh day of intensive care hospitalization decreased the risk of mechanical ventilation by 0.49 times (P = .035; sensitivity: 83.3%; specificity: 34.5%). CONCLUSION: Nutritional status should be evaluated fully in patients admitted to the pediatric intensive care unit and early detected malnutrition should be monitored closely to determine the need for early intervention. The risk of developing malnutrition is high in critically ill children. Providing the necessary energy and protein intake with nutritional therapy affects the clinical course in children with critical illness. Protein intake causes prolongation of mechanical ventilation time, delaying clinical recovery.

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.

Side effects and antibody response of an inactive severe acute respiratory syndrome coronavirus 2 vaccine among health care workers.

OBJECTIVE: This study aims to investigate the antibody response and the side effects of the two-dose inactive SARS-CoV-2 vaccine (CoronaVac, Sinovac, China) among a health care worker population in Turkey. METHODS: This study was a prospective, cross-sectional, single-center study conducted between December 16, 2020, and March 15, 2021. We evaluated the side effects from a questionnaire, and anti-spike immunoglobulin G response to the vaccine (0- and 28-day schedule) using an enzyme-linked immunosorbent assay. RESULTS: A total of 94 of 184 health care workers completed this study. The percentages of participants who were seronegative at baseline and achieved to the seropositivity were 21.3 and 97.9%, respectively, on day 21 after vaccinations. The seropositivity was predominantly detected in 31-45 years of the age group (55.4%, p=0.636), normal body mass index (47.8%, p=0.999), nonsmokers (64.1%, p=0.999), those without any comorbidities (73.9%, p=0.463), and those without any side effects (70.2%, p=0.256). The frequencies of overall side effects within seven days after the first and second doses of CoronaVac were 37.2 and 28.7%, respectively. The most common side effects was localized pain at the injection site (15.7 and 11.6%, respectively). CONCLUSIONS: We found that vaccination by two-dose CoronaVac could elicit a specific humoral response, and it was well tolerated in health care workers. The high seropositivity developed after the second dose attracted attention. Our study will be useful in terms of showing short-term immunity and side effects.

Acute flaccid myelitis outbreak through 2016-2018: A multicenter experience from Turkey.

AIM: We aim to describe the demographic characteristics, etiology, neurophysiology, imaging findings, treatment, prognosis, and prognostic factors of acute flaccid myelitis. METHODS: The clinical data, laboratory test and, magnetic resonance imaging (MRI) results of pediatric patients diagnosed with acute flaccid myelitis according to the Centers for Disease Control criteria between August 1, 2016, and December 31, 2018, from 13 centers in Turkey were reviewed. RESULTS: Of the 34 cases identified, 31 were confirmed (91.2%). Eighteen patients (55.9%) were boys. The median patient age was 4 years (interquartile range 2.5-6.9 years). Most of the patients were admitted in 2018 (n = 27). A preceding history of a febrile illness was reported in all patients, with a median of 4 days (interquartile range 3-7 days) before symptom onset. Thirty-one patients had T2 hyperintensity on spinal MRI, and 18 patients had cerebrospinal fluid pleocytosis. The most common infectious agents were entero/rhinoviruses (n = 5) in respiratory specimens. All patients except one received immunotherapy either alone or in combination. Among 27 patients with follow-up data 24 had persistent weakness. Involvement of four limbs together with an abnormal brain MRI at onset were associated with a poor prognosis. CONCLUSION: The number of patients with acute flaccid myelitis increased since 2012, spiking with every 2-year interval, largely in the pediatric population. The median age decreases with every outbreak. Clinicians should be aware of the clinical picture for early collection of specimens and early start of rehabilitation programs. Further studies are needed to better characterize the etiology, pathogenesis, risk factors, and treatment of this rare condition.

Overview of therapeutic plasma exchange in pediatric neurology: a single-center experience.

Therapeutic plasma exchange (TPE) is used in the treatment of neurological, hematological, renal and autoimmune diseases with known or suspected immune pathogenesis. In comparison with neurological diseases of adults, knowledge about the use of TPE in children is incomplete. We report our experience on TPE in children with neurological diseases in a single institution and describe the underlying etiology, clinical course, treatment and outcome. We retrospectively evaluated 22 consecutive children (12 girls, 10 boys, aged 2-16 years) who underwent TPE in the pediatric intensive care unit between January 2010 and January 2017. There were 135 TPE procedures with median 6 TPE sessions per patient. Fresh frozen plasma was used as a replacement fluid in all cases. Most common indications were inflammatory polyneuropathy followed by acquired demyelinating diseases of the central nervous system. Other indications were autoimmune encephalitis and paraneoplastic limbic encephalitis. No mortality was recorded during TPE. The complication rate was 2.2% and consisted of transient events like hypotension and allergic reactions. Therapetic plasma exchange is one of the safe methods of treatment for neuroimmunological disorders in children, with Guillain-Barré syndrome as the most common indication.

Correction to: Overview of therapeutic plasma exchange in pediatric neurology: a single-center experience.

Unfortunately, the immediate following sentences were incorrectly published in Material and Method section of the original article.

Sadfly fever: two case reports.

Sandfly fever, also known as 'three-day fever' or 'pappataci fever' or 'Phlebotomus fever' is a viral infection that causes self-limited influenza-like symptoms and characterized by a rapid onset. The disease occurs commonly in endemic areas in summer months and especially in August during which sandflies are active. In this article, two siblings who presented with high fever, redness in the eyes, headache, weakness, malaise and inability to walk, who were found to have increased liver function tests and creatine kinase levels and who were diagnosed with sadfly fever with positive sadfly IgM and IgG antibodies are reported because of the rarity of this disease.

Neuromyelitis optica mimics the morphology of spinal cord tumors.

Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system, that predominantly affects the spinal cord and the optic nerve. Its key features include transverse myelitis, commonly associated with extensive inflammation spanning three or more consecutive vertebral segments. Longitudinal extensive spinal cord lesions can also occur in systemic autoimmune diseases, infections, vascular and metabolic disorders, subsequent to irradiation, intramedullary tumors and paraneoplastic myelopathies. We present a case study of an 8-year-old girl seropositive for antibodies against the aquaporin 4 who displayed longitudinal extensive spinal cord lesions, that was initially misdiagnosed as an intramedullary tumor.

FCN2 c.772G>T polymorphism is associated with chronic adenoiditis and/or tonsillitis, but not -4 A>G and -602 G>A.

OBJECTIVE: Ficolins are complement activating peptides that play a role in the initial host defense against infectious pathogens. In the present study, we investigated the relationship between single nucleotide polymorphisms (SNPs) in the ficolin 2 gene (FCN2) and chronic adenotonsillitis in pediatric cases. STUDY DESIGN: Case-control study. METHODS: A total of 101 pediatric patients diagnosed with chronic adenotonsillitis and 100 healthy children were enrolled in the study. Genotypes of FCN2 promoter SNPs - 602 G>A and -4 A>G, and the exonic SNP c.772G>T were determined by light SNP assay after realtime PCR analysis using genomic DNA samples obtained from peripheral blood samples of all participants. RESULTS: Of the 101 chronic tonsillitis patients, 38 were girls and 63 were boys; the mean age was 5.2 ± 2.3 years. The c.772G>T SNP frequency was significantly higher in chronic adenotonsillitis cases compared to the control group (p = 0.00); however, no significant difference was determined at positions -602 G>A or -4 A>G (p > 0.05). CONCLUSIONS: The FCN2 c.772G>T genotype appears to be associated with predisposition to chronic adenotonsillitis in the pediatric age group. This nucleotide change is likely to influence the level of gene expression and contribute to the development of disease.

Serum vitamin B12, folic acid, and homocysteine levels in children with febrile seizure.

The aim of this study is to investigate the associations between febrile seizure and serum levels of vitamin B12, folic acid, and homocysteine. One hundred and four children who presented with febrile seizure and 75 controls who presented with febrile illness unaccompanied by seizure were enrolled into the study. Mean levels of vitamin B12, folic acid and homocysteine were compared between two groups. Mean vitamin B12 level in the febrile seizure group was significantly lower than the control group. The febrile seizure patients with 3 or more had significantly lower serum folic acid than the subgroups with two or one episode only. Serum concentrations of folic acid were significantly lower in the febrile seizure subgroup with body temperature 37.5-39.0˚C at time of convulsion. Low serum vitamin B12 may reduce a child's threshold for seizure and may be a risk factor for febrile seizure. Low serum folic acid level may be predisposed to recurrent febrile seizure.

22q11.2 microdeletion in two adolescent patients who presented with convulsion.

22q11.2 microdeletion which involves DiGeorge syndrome, velo-cardiofacial syndrome and conotruncal anomaly face syndrome occurs as a result of a deletion in the short segment of the long arm of the 22th chromosome. Patients with this syndrome have a wide clinical spectrum including learning difficulty, dysmorphic face, cardiac anomalies, hypocalcemia, hypoparathyroidism, cleft palate, thymus anomalies, immune failure and speech and feeding problems. The number of clinical characteristics which have been reported to be related with this syndrome is higher than 180. All anomalies may not be present in all patients. In this article, a 12-year old female patient who was found to have 22q11.2 microdeletion with mild mental retardation and dysmorphic face and who presented to our hospital because of convulsion and a 13-year old male patient who was found to have 22q11.2 microdeletion with hypocalcemia, hypoparathyroidism, dysmorphic face and mental retardation and who presented to our hospital because of convulsion (it was learned from his history that he was being followed up in another center because of autism) were presented.

Long-term accidental overdose of levetiracetam in an infant.

Levetiracetam is one of the new anticonvulsant drugs that has a high therapeutic index and potential antiepileptogenic effects. Herein, we report a patient with multidrug refractory epilepsy and Ohtahara syndrome who was accidentally administered 300 mg/kg/d for 35 days by her mother. To our knowledge, there are only a few cases of accidental overdose of levetiracetam in pediatric patients reported in the literature, and this case study is the first to report such a high and long-term dose in an infant who showed no adverse effects.

Vici syndrome associated with sensorineural hearing loss and laryngomalacia.

The phenotypically heterogeneous, autosomal recessive Vici syndrome was first described in 1988 in a sister and brother with oculocutaneous albinism, agenesis of the corpus callosum, cataract, cardiomyopathy, cleft lip, and immunodeficiency. Only 14 cases of Vici syndrome have yet been reported, several involving morphologic and functional defects in addition to those described in the initial case. We report on a 3-month-old Turkish girl with Vici syndrome associated with laryngomalacia, further expanding the clinical spectrum. We also review clinical features in all 15 Vici syndrome patients, to distinguish general from less common signs. To the best of our knowledge, this report is the first of a Turkish patient with Vici syndrome.

Acute disseminated encephalomyelitis associated with influenza A H1N1 infection.

Acute disseminated encephalomyelitis is an immune-mediated inflammatory disorder of the central nervous system, characterized by demyelination. Acute disseminated encephalomyelitis predominantly involves the white matter of the brain and spinal cord, and often follows upper respiratory tract infection. We describe a case of acute disseminated encephalomyelitis associated with the influenza A (H1N1) virus. The H1N1 virus usually causes febrile respiratory signs, e.g., fever, cough, and sore throat. Although these signs exhibit a self-limited course, the frequencies of severe complications and death are increasing. To date, only a few reports of acute disseminated encephalomyelitis secondary to the H1N1 virus have been published.