Combined immunoglobulin and plasmapheresis treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES).

Febrile infection-related epilepsy syndrome (FIRES) is a rarely observed and destructive syndrome progressing with resistant seizures or refractory status epilepticus. In this report we present in a treatment procedure with plasmapheresis of a pediatric patient with FIRES and currently unknown etiology in order to contribute to the literature.

Associations Between Transcranial Doppler Flow Velocities, Laboratory Parameters, and Clinical Features in Turkish Children With Sickle Cell Disease.

OBJECTIVE: Stroke is one of the major complications of sickle cell disease (SCD). Stroke features either occlusion of, or stenosis at, the origin of one of the large intracerebral arteries, the internal carotid artery (ICA), and/or the middle cerebral artery (MCA). PURPOSE: We sought correlations between cerebral blood flow velocities and the laboratory and clinical findings of patients with SCD. MATERIALS AND METHODS: Fifty-three pediatric SCD patients (39 with HbSS, 14 with HbSß0) were analyzed. The mean patient age was 12.9±3.9 years. The control group contained 24 healthy individuals. The time-averaged maximum mean velocity (TAMMV) and resistive index (RI) of the MCA, the TAMMVs of the ICA and vertebral artery (VA), and the diameter of the VA were estimated through transcranial Doppler ultrasonography using a 2.5 MHz transducer, in accordance with the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. We evaluated the relationships between the TAMMVs, laboratory parameters, and clinical findings. RESULTS: The mean±SD MCA TAMMV was 161.2±35.4 cm/s in patients with HbSS and 185.8±62.9 cm/s in patients with HbSß0. The mean MCA TAMMV, RI, ICA TAMMV, VA TAMMV, and VA diameter were 168.5±43.9 cm/s, 0.63±0.06, 116.8±25.5 cm/s, 69.2±18.5 cm/s, and 3.5±0.61 mm for all SCD patients, respectively. In the control group, the mean MCA TAMMV, RI, ICA TAMMV, VA TAMMV, and VA diameter were 103.8±28.8 cm/s, 0.53±0.04, 96.4±27.8 cm/s, 40.3±12.1 cm/s, and 3.4±0.6 mm, respectively. Although the differences were not significant, TAMMVs were higher in HbSß0 patients taking hydroxyurea; those with hemoglobin levels <8 g/dL, ferritin levels >1000 ng/dL, mean platelet volume >12 fL, or red cell distribution width >18%; or required chelation, or were below the third percentiles of weight and height. The TAMMV was significantly higher only in SCD patients who complained of headache. CONCLUSIONS: High ferritin and low hemoglobin levels, a high mean platelet volume, a high red cell distribution width, low weight (below the third percentile), and a short height (below the third percentile) may be associated with elevated cerebral blood flow velocities and an increased stroke risk in children with SCD. Children with such features should be closely followed-up through transcranial Doppler ultrasonography examination.

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.

Central venous pressure, global end-diastolic index, and the inferior vena cava collapsibility/distensibility indices to estimate intravascular volume status in critically ill children: A pilot study.

BACKGROUND: The assessment of the volume status in critically ill paediatric patients in intensive care units is vitally important for fluid therapy management. The most commonly used parameter for detecting volume status is still central venous pressure (CVP); however, in recent years, various kinds of methods and devices are being used for volume assessment in intensive care units. OBJECTIVES: We aimed to evaluate the relationship between CVP, the global end-diastolic index (GEDI), and ultrasound measurements of the collapsibility and distensibility indices of the inferior vena cava (IVC) in paediatric patients undergoing Pulse index Contour Cardiac Output (PiCCO) monitoring. METHODS: Fifteen patients receiving PiCCO monitoring were prospectively included in the study. Forty-nine PiCCO measurements were evaluated, and simultaneous CVP values were noted. After each measurement, IVC collapsibility (in spontaneously breathing patients) and distensibility (in mechanically ventilated patients) indices were measured with bedside ultrasound. RESULTS: The mean age was 93.2 ± 61.3 months. Significant and negative correlations of the GEDI were found with the IVC collapsibility index (in spontaneously breathing patients) and the IVC distensibility index (in mechanically ventilated patients) (r = -0.502, p < 0.001; r = -0.522, p = 0.001, respectively). A significant and weakly positive correlation was found between the GEDI and CVP (r = 0.346, p = 0.015), and a significant and negative correlation was found between the IVC collapsibility index and CVP (r = -0.482, p = 0.03). The correlation between the IVC distensibility index and CVP was significant and negative (r = -0.412, p = 0.04). CONCLUSION: The use of PiCCO as an advanced haemodynamic monitoring method and the use of bedside ultrasound as a noninvasive method are useful to evaluate the volume status in critically ill paediatric patients in intensive care. These methods will gradually come to the fore in paediatric intensive care.

A Novel Frameshift Mutation in the FGA Gene (c.196 delT) Leading to Congenital Afibrinogenemia.

BACKGROUND: Congenital afibrinogenemia is characterized by the absence of fibrinogen. Congenital fibrinogen disorders result from several mutations in FGA, FGB, or FGG. Their epidemiology is not well known. OBSERVATION: The present study reports on 2 children with congenital afibrinogenemia. The first child, a male who is now 9 years old, was diagnosed with afibrinogenemia after spontaneous intracranial bleeding at the age of 3 years. The second child is a 2-year-old female cousin of the first patient, who was diagnosed with afibrinogenemia after coagulation tests were carried out due to frequent epistaxis and mucocutaneous bleeding. At follow-up, blood samples of the patients and their parents were sent to the Department of Genetic Medicine and Development, University Medical Center, Switzerland, for polymerase chain reaction analysis. In both patients, the novel homozygous frameshift mutation in the FGA exon 3: c.196 delT was detected. The parents of the patients were both heterozygous for the same mutation. CONCLUSIONS: Congenital afibrinogenemia is a rare coagulation disease. The molecular epidemiology of congenital fibrinogen disorders is complex, and the identification of new mutations will help shed light on this complex molecular structure. Therefore, a genetic analysis that includes more centers is needed.

Somatic regional oxygen saturation as an early marker of intra-abdominal hypertension in critically ill children: a pilot study

Background/aim: Intraabdominal hypertension is a common clinical condition with high mortality and morbidity in pediatric intensive care units. The aim of this study was to test the feasibility of regional tissue oxygenation (rSO2) measurement using near-infrared spectroscopy and to assess the correlation between rSO2 and perfusion markers of intraabdominal hypertension in high-risk pediatric patients. Materials and method: In this prospective observational cohort study in a tertiary pediatric intensive care unit in Çukurova University Faculty of Medicine, a total of 31 patients who were admitted between May 2017 and May 2018 with a risk of intraabdominal hypertension were included. Mesenteric and renal rSO2 measurements were taken and correlations with other tissue perfusion markers including mean arterial pressure, pH, lactate, intraabdominal pressure, abdominal perfusion pressure, mixed venous oxygen saturation, vasoactive inotropic score were assessed. Intraabdominal pressure was measured as ≥10 mmHg in 15 patients (48.3%) and these patients were defined as the group with intraabdominal hypertension. Results: In the group with intraabdominal hypertension, mixed venous oxygen saturation was lower (P = 0.024), vasoactive inotropic score was higher (P = 0.024) and the mean abdominal perfusion pressure value was lower (P = 0.014). In the ROC analysis, the mesenteric rSO2 measurement was the best parameter to predict intraabdominal hypertension with area under the curve of 0.812 (P = 0.003) 95% CI [0.652­0.973]. Conclusion: Monitoring of mesenteric rSO2 is feasible in patients at risk for intraabdominal hypertension. Moreover, both mesenteric regional oxygen and perfusion markers may be used to identify pediatric patients at risk for intraabdominal hypertension.

Effects of Sedation and/or Sedation/Analgesic Drugs Administered during Central Venous Catheterization on the Level of End-tidal Carbon Dioxide Measured by Nasal Cannula in Our PICU.

AIMS AND OBJECTIVES: Sedatives and analgesics are commonly used in pediatric intensive care units during minor invasive procedures. Here, we aimed to measure the changes in end-tidal carbon dioxide (EtCO2) levels with different sedation/analgesic drug administrations (midazolam, ketamine, midazolam + ketamine/fentanyl) during central venous catheterization. MATERIALS AND METHODS: This prospective study included 44 patients who needed sedation/analgesia for central venous catheterization. Patients were sedated with midazolam, ketamine, or midazolam + fentanyl/ketamine. End-tidal carbon dioxide values were measured before and after sedation-analgesia with nasal cannula and recorded from the capnograph. Oxygen saturation (SO2) was monitored by pulse oximetry. Whether respiratory depression occurred during the process was recorded. RESULTS: During the procedure, 15 (34%) patients were given 0.1 mg/kg dose of midazolam described as group I, 18 (41%) patients were given 1 mg/kg dose of ketamine only described as group II, and 11 (25%) patients who could not be effective sedated with a single sedative-analgesic agent were given either 1 mg/kg dose of ketamine or 2 µg/kg dose of fentanyl together with 0.1 mg/kg dose of midazolam described as group III. According to our findings, hypoxia (54.5%) and hypercarbia (45.5%) were detected higher in group III but it was not statistically significant (p = 0.255, p = 0.364). Hypercarbia was detected in 29.5% patients, in 62% of these patients hypercarbia was accompanied by hypoxia, and 38% had only hypercarbia. When presedation and postsedation EtCO2 values were compared, we detected a statistically significant difference in all groups. CONCLUSION: We detected hypercarbia unaccompanied by hypoxemia in 38% patients. And we think that we have identified these patients early due to measurement of EtCO2 by nasal cannula. This study demonstrated that EtCO2 monitoring via nasal cannula is a feasible and practical way to follow ventilation during sedation/analgesia. HOW TO CITE THIS ARTICLE: Aslan N, Yildizdas D, Horoz OO, Arslan D, Coban Y, Sertdemir Y. Effects of Sedation and/or Sedation/Analgesic Drugs Administered during Central Venous Catheterization on the Level of End-tidal Carbon Dioxide Measured by Nasal Cannula in Our PICU. Indian J Crit Care Med 2020;24(8):705-708.

Trientine-induced Rhabdomyolysis in an Adolescent with Wilson's Disease.

BACKGROUND: Drugs are very important in the etiology of nontraumatic rhabdomyolysis. CASE DESCRIPTIONS: A 16-year-old male patient with Wilson's disease was admitted for myoclonic contractions. Oral trientine was started for neurological problems and tremor on the hands due to D-penicillamine 1 month ago. Patient was oligoanuric, and his creatine kinase level was 15197 U/L. Rhabdomyolysis was associated with trientine, and trientine treatment was stopped. Hemodiafiltration was performed. The patient began to urinate on the 24th day. CONCLUSION: This is the first pediatric patient with rhabdomyolysis induced by trientine. Drugs used should be questioned carefully in patients with rhabdomyolysis. HOW TO CITE THIS ARTICLE: Aslan N, Yavuz S, Yildizdas D, Horoz OO, Coban Y, Tumgor G, et al. Trientine-induced Rhabdomyolysis in an Adolescent with Wilson's Disease. Indian J Crit Care Med 2019;23(10):489-490.

Can bispectral index be an early marker in the diagnosis of brain death?

BACKGROUND: The diagnosis of brain death is a clinical condition in which it is difficult to perform confirmatory tests due to the ineligible clinical status of the patient. Prior to confirmatory tests, the use of a BIS monitor to determine the time of brain death is important for organ transplants, cost-effectiveness and reducing stressful wait of the family. OBJECTIVE: This study aimed to use BIS monitoring for early detection of brain death. METHODS: BIS monitoring was performed in 12 patients who were clinically diagnosed with brain death in our intensive care unit during a two-year period. RESULTS: All patients had diffuse brain injury. The BIS score was zero in all patients. However, two patients could not be legally diagnosed with brain death because confirmatory tests could not be performed due to the clinical status. In one patient, the BIS score was zero and blood flow was present on the first computed tomography angiography of the brain; however, the cerebral blood flow was absent on the second imaging after two days. CONCLUSION: It was believed that BIS monitoring could be a parameter to use for detection of brain death in patients with severe brain injury. However, future research is needed in this regard.