A rare complication of leukostasis with AML M4 patient: Microcirculatory dysfunction of upper extremities.

BACKGROUND: In children with acute myeloid leukemia, the incidence of hyperleukocytosis is 5-33%. Patients with AML and hyperleukocytosis have a higher early mortality rate than patients with nonhyperleukocytic AML because of the increased risk of severe pulmonary and neurologic complications. Leukapheresis provides rapid cytoreduction and reduces early mortality rates. CASE PRESENTATION: In this report, we present a case with microcirculatory failure of upper extremities as a rare symptom of hyperleukocytic AML M4 at initial presentation. CONCLUSIONS: Early diagnosis and treatment of patients with AML admitted to emergency services with these symptoms is too important to prevent from loss of extremities. Most of the complications of hyperleukocytosis can be reversible with early treatment.

Misdiagnosis of multisystem inflammatory syndrome in children: A diagnostic challenge.

AIMS: As the COVID-19 pandemic continues, multisystem inflammatory syndrome in children (MIS-C) maintains its importance in the differential diagnosis of common febrile diseases. MIS-C should be promptly diagnosed because corticosteroid and/or intravenous immunoglobulin treatment can prevent severe clinical outcomes. In this study, we aimed to evaluate clinical presentation, diagnostic parameters and management of MIS-C and compare its clinical features to those of common febrile disease. METHODS: This study was conducted at a tertiary-level university hospital between December 2020 and October 2022. One hundred and six children who were initially considered to have MIS-C disease were included in the study. During the follow-up period in the hospital, when the clinical and laboratory findings were re-evaluated, 38 out of 106 children were diagnosed differently. The clinical and laboratory findings of 68 children followed up with the diagnosis of MIS-C and 38 children who were initially misdiagnosed as MIS-C but with different final diagnoses were retrospectively compared. RESULTS: We identified 68 patients with MIS-C and 38 patients misdiagnosed as MIS-C during the study period. Infectious causes (71%), predominantly bacterial origin, were the most frequently confused conditions with MIS-C. Patients with MIS-C were older and had a more severe clinical course with high rates of respiratory distress, shock, and paediatric intensive care unit admission. While rash and conjunctivitis were more common among patients with MIS-C, cough, abdominal pain and diarrhoea were observed more frequently in patients misdiagnosed as MIS-C. Lower absolute lymphocyte counts, platelet counts and higher C-reactive protein and fibrinogen levels, pathological findings on echocardiography were the distinctive laboratory parameters for MIS-C. Multivariate analysis showed that older age, presence of conjunctivitis, high level of serum CRP and lower platelets were the most discriminative predictors for the diagnosis of MIS-C. CONCLUSION: There are still no specific findings to diagnose MIS-C, which therefore can be confused with different clinical conditions. Further data are needed to assist the clinician in the differential diagnosis of MIS-C and the diagnostic criteria should be updated over time.

Evaluation of thrombosis and bleeding events in the children with left ventricular assist device (L-VAD).

This study aimed to evaluate thrombosis and bleeding events in the children implanted with two types of ventricular assist devices (VAD). A total of 26 paediatric end-stage heart failure patients with the mean age of 11.32 ± 4.17 years, 15 were boys, implanted with a VAD, either the Berlin Heart EXCOR (BHE group; n = 9) or the HeartWare (HW group, n = 17), were included in this retrospective study. Follow up data on bleeding events, thrombosis events, bridge-to-transplantation rates and survival outcome were recorded. Overall, 16(33.3%) bleeding events and 32(66.7%) thrombosis events occurred, while 14(53.8%) patients had at least one thrombotic event and 8(30.8%) patients had at least one bleeding event. BHE and HW groups were similar in terms of number of patients with at least one thrombotic (33.3% vs. 64.7%, p = .218) or bleeding (22.2% vs.35.3%, p = .399) event. Mortality occurred in 9(34.6%) patients and 13(50.0%) patients achieved bridge-to-transplantation, similarly in BHE and HW groups (mortality: 44.4% vs. 29.4%, p = .667 and transplantation: 77.8% vs. 35.3%, p = .097). In conclusion, our findings revealed that VAD application in children with heart failure enables successful heart transplantation achievement with an acceptable risk of bleeding/thromboembolic events in most of cases. More advanced VAD technologies and more successful management for haematologic complications are necessary to improve the transplantation rates in children.

Impact of Decompressive Craniectomy on Diagnosing Brain Death in Children.

OBJECTIVE: In this study, we aimed to evaluate the effects of decompressive craniectomy (DC) on the brain death (BD) determination process in the pediatric intensive care unit (PICU). MATERIALS AND METHODS: All children who were diagnosed with BD in the PICU between 2009 and 2020 were included in this study. Patient demographics, causative mechanisms, BD examinations, and ancillary tests used were recorded. The time intervals (from PICU admission to first BD examination, from first BD examination to BD diagnosis) and number of BD examinations were compared between patients with and without DC. RESULTS: During the study period, 70 pediatric cases were diagnosed with BD among 513 total deaths (13.6%). Their median age was 54.5 months [interquartile range (IQR): 24-140]. Transcranial Doppler ultrasound (TCD) was performed in 66 patients (94.3%). The most common combination of ancillary tests was the apnea test and TCD combination, which was performed in 56 patients (80%). Thirty-four children (48.6%) experienced immediate surgery for DC. Patients with DC had a higher median number of BD examinations than patients without DC [3 (IQR: 2-3) vs. 2 (IQR: 1-2), P < .001]. The patients with DC had a longer time interval between the first examination and declaration of BD than patients without DC [45.5 hours (IQR: 21.7-91.7) versus 15 hours (IQR: 2-31.2), P < .006]. CONCLUSION: Decompressive craniectomy may complicate BD determination and cause challenges for brain death diagnosis based on cerebral flow imaging techniques. The lack of specific recommendations for this patient group in the guidelines causes a delayed diagnosis of BD. Cite this article as: Ekici B, Ersayoglu I, Yazici Özkaya P, Cebeci K, Koç G, Turanli EE. Impact of decompressive craniectomy on diagnosing brain death in children. Turk Arch Pediatr. 2024;59(1):93-97.

Therapeutic plasma exchange in critically ill children: A single center experience.

INTRODUCTION: Therapeutic plasma exchange (TPE) is used in a wide spectrum of diseases in critically ill pediatric patients. We aim to review the indications, complications, safety, and outcomes of critically ill children who received TPE. METHODS: All of the TPE procedures performed in a pediatric intensive care unit providing tertiary care during 19 years (January 2013-January 2023) were evaluated retrospectively. A total of 154 patients underwent 486 TPE sessions. RESULTS: Median age was 6 years (2-12.5) and 35 children had a body weight of <10 kg (22.7%). Number of organ failure was 4 (2-6). Liver diseases were the most common indication for TPE (31.2%) followed by sepsis with multiorgan dysfunction syndrome (27.3%). Overall survival rate was 72.7%. The highest mortality was observed in hemophagocytic lymphohistiocytosis group. Non-survivors had significantly higher number of organ failure (p < 0.001), higher PRISM score (p < 0.001), and higher PELOD score on admission (p < 0.001). Adverse events were observed in 68 (13.9%) sessions. Hypotension (7.8%) and hypocalcemia (5.1%) were the most frequent adverse events. CONCLUSION: TPE is safe for critically ill pediatric patients with experienced staff. Survival rate may vary depending on the underlying disease. Survival decreases with the increase in the number of failed organs.

Sepsis associated acute kidney injury in pediatric intensive care unit.

BACKGROUND: We aimed to compare the acute kidney injury (AKI) incidence in pediatric septic shock patients according to the three different classifications. METHODS: We analyzed retrospectively 52 patients with severe sepsis between January 2019 and December 2019. RESULTS: While 21 patients have been diagnosed with SA-AKI according to the pRIFLE criteria, 20 children have been diagnosed according to the AKIN criteria, and 21 children have been diagnosed according to the KDIGO criteria. Older age, lower platelet count were determined as independently risk factor for SA-AKI. Older age and higher PRISM score were associated with mortality. According to Canonical correlation coefficients, pRIFLE is the most successful classification to distinguish AKI state. The canonical correlation coefficients for pRIFLE, KDIGO, and AKIN were 0.817, 0.648, and 0.615, respectively. CONCLUSION: Although AKI incidence was similar between the three classifications, pRIFLE was the most successful classification to distinguish AKI state.

The first pediatric case of mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) associated with Neisseria meningitidis.

BACKGROUND: Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a clinic-radiological syndrome characterized by neurological deficiencies and reversible magnetic resonance imaging findings in the splenium of the corpus callosum. Usually, it has a good prognosis, and patients recover without any sequelae. A viral infection has caused most MERS cases, and bacterial agents have rarely been reported as a cause of MERS. CASE REPORT: A 5-month-old male was admitted to the hospital with fever, poor feeding, decreased activity and groaning. He had focal seizures and required mechanical ventilation. A lumbar puncture was performed, and nucleic acid amplification tests (NAATs) of cerebral spinal fluid was positive for non-typeable serogroup of Neisseria meningitidis. Cranial magnetic resonance imaging (MRI) performed on day 3 of admission showed T2 hyperintensity and diffusion restriction at the splenium of corpus callosum (SCC). Diffusion restriction in the occipital horn of the left ventricular compatible with empyema was also obvious. According to the findings on cranial MRI, this clinical course was diagnosed as MERS associated with meningitis. His fever resolved in one week, cefotaxime was discontinued after 14 days, and the patient completely recovered. A follow-up MRI performed after three weeks of admission showed complete resolution of the signal intensity changes in the SCC and the occipital horn of the left lateral ventricle. CONCLUSION: To the best of our knowledge, this is the first report of MERS associated with Neisseria meningitidis in children. Bacterial agents as a cause of MERS should be kept in mind, and we should avoid unnecessary treatment strategies due to the good prognosis of MERS in children.