Longitudinal Analysis of Amplitude-Integrated Electroencephalography for Outcome Prediction in Hypoxic-Ischemic Encephalopathy.

OBJECTIVE: To investigate the prognostic accuracy of longitudinal analysis of amplitude-integrated electroencephalography (aEEG) background activity to predict long-term neurodevelopmental outcome in neonates with hypoxic-ischemic encephalopathy (HIE) receiving therapeutic hypothermia. STUDY DESIGN: This single-center observational study included 149 neonates for derivation and 55 neonates for validation with moderate-severe HIE and of gestational age ≥35 weeks at a tertiary neonatal intensive care unit. Single-channel aEEG background pattern, sleep-wake cycling, and seizure activity were monitored over 84 hours during therapeutic hypothermia and rewarming, then scored for each 6-hour interval. Neurodevelopmental outcome was assessed using the Bayley Scales of Infant Development, Second Edition. Favorable outcome was defined as having both a Mental Development Index (MDI) score and Psychomotor Development Index (PDI) score ≥70, and adverse outcome was defined as either an MDI or a PDI <70 or death. Regression modeling for longitudinal analysis of repeatedly measured data was applied, and area under the receiver operating characteristic curve (AUC) was calculated. RESULTS: Longitudinal aEEG background analysis combined with sleep-wake cycling score had excellent predictive value (AUC, 0.90; 95% CI, 0.85-0.95), better than single aEEG scores at any individual time point. The model performed well in the independent validation cohort (AUC, 0.87; 95% CI, 0.62-1.00). The reclassification rate of this model compared with the conventional analysis of aEEG background at 48 hours was 18% (24 patients); 14% (18 patients) were reclassified correctly. Our results were used to develop a user-friendly online outcome prediction tool. CONCLUSIONS: Longitudinal analysis of aEEG background activity and sleep-wake cycling is a valuable and accurate prognostic tool.

The Role of Mannose-binding Lectin in Infectious Complications of Pediatric Hemato-Oncologic Diseases.

The complement system is essential for protection against infections in oncologic patients because of the chemotherapy-induced immunosuppression. One of the key elements in the activation of the complement system via the lectin pathway is the appropriate functioning of mannose-binding lectin (MBL) and mannose-binding lectin-associated serine protease 2 (MASP2) complex. The objective of our study was to find an association between polymorphisms resulting in low MBL level and activation of the MBL-MASP2 complex. Also, we aimed at finding a connection between these abnormalities and the frequency and severity of febrile neutropenic episodes in children suffering from hemato-oncologic diseases. Ninety-seven patients had been enrolled and followed from the beginning of the therapy for 8 months, and several characteristics of febrile neutropenic episodes were recorded. Genotypes of 4 MBL2 polymorphisms (-221C/G, R52C, G54D, G57E) were determined by real-time polymerase chain reaction. Activation of the MBL-MASP2 complex was evaluated by enzyme-linked immunosorbent assay at the time of diagnosis and during an infection. The number of febrile neutropenic episodes was lower, and the time until the first episode was longer in patients with normal MBL level than in patients with low MBL level coding genotypes. The MBL-MASP2 complex activation level correlated with the MBL genotype and decreased significantly during infections in patients with low MBL level. Our results suggest that infections after immunosuppression therapy in children suffering from hemato-oncologic diseases are associated with the MBL2 genotype. Our results may contribute to the estimation of risk for infections in the future, which may modify therapeutic options for individuals.

Prevalence of cerebral palsy and data quality assessment of patient documentation at the Clinics of Semmelweis University / A cerebralis paresis elofordulása és a betegdokumentáció minoségi vizsgálata a Semmelweis Egyetem klinikáin

Introduction, aim: Quality control of patient documentation for cerebral palsy (CP) at Semmelweis University. METHOD: In our retrospective audit, we revised patient records for all children born between 2005 and 2015, with suspected CP, registering 673 cases with confirmed CP. Based on the available patient data, we assessed clinical and etiological classification of CP and data availability. RESULTS: Patient records of 86% of children were suitable for clinical classification. Among them, 90.5% were spastic, 7.8% hypotonic, 1.2% dyskinetic and 0.5% ataxic. Among the classifiable spastic cases (98% of all spastic cases), 51% presented with tetraparetic/tetraplegic, 26% diparetic/diplegic and 23% hemiparetic/hemiplegic localization; in the remaining 2%, sufficient data for topological classification was unavailable. Severity assessed on Gross Motor Function Classification System was definable in 82% of cases, 43% showing grade I-II, 28% grade III and 29% grade IV-V impairment. Patient history was specified in 91% of cases. Prematurity was documented in 55%, perinatal asphyxia/hypoxic-ischemic encephalopathy in 31%, intraventricular/intracranial haemorrhage in 27%, multiple births in 19%, intrauterine growth restriction in 18%, intrauterine/perinatal/infancy infection in 15%, congenital malformation in 12%, in vitro fertilisation in 5%, stroke in 3% and CP-associated genetic mutation in 3% of cases. Negative patient history was determined in 16% of children. CONCLUSIONS: Our audit established that clinical documentation of CP is performed based on uniform criteria, detecting missing data primarily in clinical classification and patient history. We propose a patient documentation standard in the clinical care of affected children, which is a prerequisite for unified data recording and a future national CP registry. Orv Hetil. 2020; 161(21): 873-880.