Optic nerve sheath diameter and pulsatility index for the diagnosis and follow-up in pediatric traumatic brain injury: a prospective observational cohort study.

PURPOSE: Invasive neuromonitoring could be difficult in children with traumatic brain injury (TBI). This study aimed to determine whether noninvasive intracranial pressure (nICP), calculated via pulsatility index (PI) and optic nerve sheath diameter (ONSD) had correlated with each other and patient outcome. METHODS: All moderate-severe TBI patients were eligible. Patients with a diagnosis of intoxication that did not affect the mental status or cardiovascular system were enrolled as controls. The PI measurements were routinely performed bilaterally on the middle cerebral artery. A software (QLAB's Q-Apps) was used to calculate PI, which further placed the ICP equation of Bellner et al. Linear probe with a 10 MHz frequency transducer to measure ONSD, which further placed the ICP equation of Robba et al. All measurements were performed by a point-of-care ultrasound certified pediatric intensivist under the supervision of a neurocritical care specialist, before and 30 min after a hypertonic saline (HTS) infusion for every 6 h when the patient's mean arterial pressure, heart rate, body temperature, hemoglobin, and blood CO2 levels were within normal ranges. The secondary outcome was the effect of hypertonic saline (HTS) on nICP. Delta-sodium values of each HTS infusion were calculated as a difference between pre- and post-measurements. RESULTS: Twenty-five TBI patients (200 measurements) and 19 controls (57 measurements) were included. Median nICP-PI and nICP-ONSD on admission were significantly higher in the TBI group (11.03 (9.98-12.63), p = 0.004, and 13.14 (12.27-14.64), p < 0.001, respectively). Median nICP-ONSD of severe TBI patients were higher than moderate TBI patients (13.58 (13.14-15.71) and 12.30 (9.83-13.14), respectively, p = 0.013). The median nICP-PI was the same across the type of injury (falls and motor vehicle accidents), while the median nICP-ONSD of the motor vehicle accident group was higher than falls. The first nICP-PI and nICP-ONSD measurements in PICU and admission pGCS were negatively correlated (r = - 0.562, p = 0.003 and r = - 0.582, p = 0.002, respectively). The mean nICP-ONSD during the study period and admission pGCS and GOS-E peds score significantly correlated. However, the Bland-Altman plots showed significant bias between the two methods of ICP except after 5th dose of HTS. All nICP values significantly decreased in time, and it was most obvious after the 5th dose of HTS. No significant correlations were found between delta sodium levels and nICP. CONCLUSION: Noninvasive estimation of ICP is helpful for the management of pediatric severe TBI patients. nICP driven by ONSD is more consistent with clinical findings of increased ICP but not useful as a follow-up tool in acute management because of slow circulation of CSF around the optic sheath. The correlation between admission GCS scores and GOS-E peds score favors ONSD as a good candidate for determining disease severity and predicting long-term outcomes.

Reversible bronchiectasis caused by influenza virus mimicking Williams-Campbell syndrome.

The term bronchiectasis refers to permanent enlargement of the bronchi. It is increasingly diagnosed because of high-resolution computed investigations. It can be congenital or acquired, the latter mostly following infection. Williams-Campbell syndrome is a rare form of congenital non-cystic fibrosis bronchiectasis. Here we report a 5-month-old girl with reversible bronchiectasis treated with extracorporeal membrane oxygenation for acute respiratory distress syndrome (ARDS) caused by influenza virus following surgery for congenital heart disease. Chest CT showed an abnormally large bronchial tree mimicking Williams-Campbell syndrome. At 9 months later, chest CT showed regression of bronchiectasis and normalized caliber of previously collapsed segments in both lungs. This atypical course illustrates that influenza virus can cause reversible bronchiectasis in infants and mimic congenital disease such as Williams-Campbell syndrome.

Multisystem inflammatory syndrome in children during the COVID-19 pandemic in Turkey: first report from the Eastern Mediterranean.

OBJECTIVE: We aimed to describe the typical clinical and laboratory features and treatment of children diagnosed with multisystem inflammatory syndrome in children (MIS-C) and to understand the differences as compared to severe/critical pediatric cases with COVID-19 in an eastern Mediterranean country. METHODS: Children (aged <18 years) who diagnosed with MIS-C and severe/critical pediatric cases with COVID-19 and were admitted to hospital between March 26 and November 3, 2020 were enrolled in the study. RESULTS: A total of 52 patients, 22 patients diagnosed with COVID-19 with severe/critical disease course and 30 patients diagnosed with MIS-C, were included in the study. Although severe COVID-19 cases and cases with MIS-C share many clinical and laboratory features, MIS-C cases had longer fever duration and higher rate of the existence of rash, conjunctival injection, peripheral edema, abdominal pain, altered mental status, and myalgia than in severe cases (p<0.001 for each). Of all, 53.3% of MIS-C cases had the evidence of myocardial involvement as compared to severe cases (27.2%). Additionally, C-reactive protein (CRP) and white blood cell (WBC) are the independent predictors for the diagnosis of MIS-C, particularly in the existence of conjunctival injection and rash. Corticosteroids, intravenous immunoglobulin (IVIG), and biologic immunomodulatory treatments were mainly used in MIS-C cases rather than cases with severe disease course. There were only three deaths among 52 patients, one of whom had Burkitt lymphoma and the two cases with severe COVID-19 of late referral. CONCLUSION: Differences between clinical presentations, acute phase responses, organ involvements, and management strategies indicate that MIS-C might be a distinct immunopathogenic disease as compared to pediatric COVID-19. Conjunctival injection and higher CRP and low WBC count are reliable diagnostic parameters for MIS-C cases. Key Points • MIS-C cases had longer fever duration and higher rate of the existence of rash, conjunctival injection, peripheral edema, abdominal pain, altered mental status, and myalgia than in severe/critical pediatric cases with COVID-19. • Higher CRP and low total WBC count are the independent predictors for the diagnosis of MIS-C. • MIS-C might be a distinct immunopathogenic disease as compared to pediatric COVID-19.

Retrospective evaluation of 85 patients with urea cycle disorders: one center experience, three new mutations.

Objectives Urea cycle disorders (UCDs) are rare hereditary diseases. This study was conducted to help identify the characteristics of UCDs in Turkey. Methods The primary outcome was to determine patient characteristics. Investigating the relationships between the patient outcomes and ammonia levels were the secondary outcomes. Eighty five patients from 79 families, diagnosed with UCD at a single metabolic referral center between 1979 and 2017, were included. Clinical and laboratory data were retrieved retrospectively from hospital records. Results Classical citrullinemia was the most common type of UCD; citrin deficiency and carbamoyl phosphate synthase 1 deficiency (CPS1D) were the rarest. One thirty one hyperammonemic episodes were recorded. The peak ammonia levels were found to be significantly associated with polycythemia and hypocalcemia at presentation. The median peak ammonia values of the patients who died were higher than those of the survivors. The highest mortality rate was in the classical citrullinemia group. The mortality rate of the first hyperammonemic crisis was 28.6%, while it was 6.7% in subsequent episodes with an odds ratio of 4.28 (95% CI: 1.67-11.0) (p=0.001). Forty-four patients underwent genetic analysis and genetic variants were detected in 42 patients (95%). Three of the detected variants have not been previously reported. Conclusions This is the largest UCD series in Turkey and may serve as a guide to clinical, biochemical and genetic features of UCDs in our country. Prevention of hyperammonemia may be the most influential measure to improve long term survival.