Risks of ACTH therapy for West syndrome following BCG vaccination.

OBJECTIVE: Bacille de Calmette et Guérin (BCG) is a live vaccine for tuberculosis that is administered to all infants in Japan. Adrenocorticotropic hormone (ACTH) therapy for West syndrome (WS) causes immunosuppression and may result in BCG infection after BCG vaccination. We evaluated the safety of ACTH therapy initiated shortly after BCG vaccination. METHODS: We analyzed patients with WS who received ACTH therapy between 2005 and 2018. We evaluated the interval between BCG and ACTH therapy, and the rate of BCG infection during and after ACTH therapy, by retrospective chart review. RESULTS: Seventy-nine patients were included in the analysis. Twenty-three patients received ACTH therapy prior to BCG vaccination. For the remaining 56 patients, the median interval between BCG vaccination and the start of ACTH therapy (BCG-ACTH interval) was 91.5 (range 14-280) days. The BCG-ACTH interval was shorter in patients with unknown than in those with known etiologies. It was <8 weeks in 13 patients (10 with unknown and 3 with known etiologies). The minimum BCG-ACTH interval was 14 days. Six patients with epileptic spasms received BCG vaccinations because physicians did not recognize their seizures. None of the patients developed BCG infection. CONCLUSION: No patients who received ACTH therapy after BCG, even at an interval of 8 weeks, developed BCG infection. The timing of ACTH therapy initiation should be based on the risk of BCG-related adverse events and the adverse effects of any delay.

Effectiveness of lacosamide in children and young adults previously treated with other sodium channel blockers.

PURPOSE: This multicenter study examined the effectiveness and tolerability of lacosamide (LCM) for children and young adults with epilepsy, particularly in patients who had previously been treated with other sodium channel blockers (SCBs) and the difference in effectiveness and tolerability when using other concomitant SCBs. METHODS: We retrospectively studied the clinical information of patients aged <30 years given LCM to treat epilepsy. The effectiveness and adverse events (AEs) of LCM and the other SCBs were investigated. Factors related to the effectiveness and AEs of LCM, such as the number of antiepileptic drugs (AEDs) tried before LCM and concomitantly used SCBs, were also studied. RESULTS: We enrolled 112 patients (median age = 11 years). One year after starting LCM, 29% of the patients were seizure free, and 50% had a ≥50% seizure reduction. Of the patients, 17% experienced AEs, the most common being somnolence. A ≥50% seizure reduction was observed for LCM in 30% of patients in whom other SCBs had not been effective. Lacosamide produced a ≥50% seizure reduction in 35% of the patients taking one concomitant SCB. By contrast, no patients had ≥50% seizure reduction, and 33% developed AEs, when LCM was administered concomitantly with two SCBs. CONCLUSIONS: Lacosamide was effective in 30% of children and young adults in whom other SCBs had not been effective. The effectiveness of LCM may differ from that of other SCBs, and it is worth trying in patients with epilepsy resistant to other AEDs.

MRI findings in children with congenital cytomegalovirus infection retrospectively diagnosed with dried umbilical cord.

PURPOSE: Brain MRI provides important information about suspected congenital CMV infection in neonatally underdiagnosed children. This study aimed to describe MRI findings in children in whom congenital CMV infection was not suspected during the neonatal period and was proven retrospectively. METHODS: We enrolled 31 children referred to the pediatric neurology clinic with neurological symptoms who were proven to have congenital CMV infection based on dried umbilical cord samples. Upon diagnosis, MR and CT images were assessed using the van der Knaap scoring system integrated with additional variables. Two investigators independently assessed all images. RESULTS: The age at diagnosis was < 12 months in 14, 12-24 months in 11, and > 24 months in 6 patients. The initial symptom triggering clinic referral was delayed development in 22, seizure in 5, deafness in 3, and hemiplegia in 1 patient. Of the 31 children, 30 had a white matter (WM) abnormality predominant in the deep WM of the parietal lobe (n = 25). Anterior temporal lesions were observed in 21 children. Cortical lesions were observed in 7 children, suggestive of polymicrogyria. No child had cerebellar or brainstem abnormalities. Brain CT was performed in 22 of 31 children, and 11 showed punctate cerebral calcification in the periventricular and/or deep WM. CONCLUSION: Patients with congenital CMV infection with delayed neurological symptoms show a relatively uniform pattern of parietal-dominant multifocal WM lesions and anterior temporal lesions, with or without polymicrogyria.

Hippocampal diffusion abnormality after febrile status epilepticus is related to subsequent epilepsy.

OBJECTIVE: To assess hippocampal signal changes on diffusion-weighted imaging (DWI) during the acute period after febrile status epilepticus (FSE) and to examine the relationship between DWI and subsequent epilepsy. METHODS: A prospective, multicenter study of children with a first episode of FSE was performed. The patients underwent magnetic resonance imaging (MRI) within 3 days of FSE, and signal intensity was evaluated on DWI. Electroencephalography studies within 3 days of FSE were also assessed. Nine to 13 years after FSE, information on subsequent epilepsy was obtained. RESULTS: Twenty-two children with FSE were evaluated. DWI showed unilateral hippocampal hyperintensity in six patients (27%). Three of six patients with hippocampal hyperintensity had ipsilateral thalamic hyperintensity. On EEG within 3 days of FSE, five of six patients with hippocampal hyperintensity had ipsilateral focal slowing, spikes, or attenuation. Nine to 13 years later, the outcomes could be determined in five patients with hippocampal hyperintensity and in 10 without. All 5 patients with hippocampal hyperintensity had hippocampal atrophy and developed focal epilepsy, whereas only 1 of 10 patients without hippocampal hyperintensity developed epilepsy (P = 0.002). Ictal semiology was concordant with temporal lobe seizures in all patients. Ipsilateral temporal epileptiform abnormalities were seen on EEG in four of five at last follow-up. SIGNIFICANCE: Acute DWI hippocampal hyperintensity was seen in 27% of patients with FSE. Acute DWI hyperintensity suggests cytotoxic edema caused by prolonged seizure activity. Hippocampal DWI hyperintensity is related to mesial temporal lobe epilepsy and can be a target of neuroprotective treatments to prevent the onset of epilepsy.

De novo PHACTR1 mutations in West syndrome and their pathophysiological effects.

Trio-based whole exome sequencing identified two de novo heterozygous missense mutations [c.1449T > C/p.(Leu500Pro) and c.1436A > T/p.(Asn479Ile)] in PHACTR1, encoding a molecule critical for the regulation of protein phosphatase 1 (PP1) and the actin cytoskeleton, in unrelated Japanese individuals with West syndrome (infantile spasms with intellectual disability). We then examined the role of Phactr1 in the development of mouse cerebral cortex and the pathophysiological significance of these two mutations and others [c.1561C > T/p.(Arg521Cys) and c.1553T > A/p.(Ile518Asn)], which had been reported in undiagnosed patients with intellectual disability. Immunoprecipitation analyses revealed that actin-binding activity of PHACTR1 was impaired by the p.Leu500Pro, p.Asn479Ile and p.Ile518Asn mutations while the p.Arg521Cys mutation exhibited impaired binding to PP1. Acute knockdown of mouse Phactr1 using in utero electroporation caused defects in cortical neuron migration during corticogenesis, which were rescued by an RNAi-resistant PHACTR1 but not by the four mutants. Experiments using knockdown combined with expression mutants, aimed to mimic the effects of the heterozygous mutations under conditions of haploinsufficiency, suggested a dominant negative effect of the mutant allele. As for dendritic development in vivo, only the p.Arg521Cys mutant was determined to have dominant negative effects, because the three other mutants appeared to be degraded with these experimental conditions. Electrophysiological analyses revealed abnormal synaptic properties in Phactr1-deficient excitatory cortical neurons. Our data show that the PHACTR1 mutations may cause morphological and functional defects in cortical neurons during brain development, which is likely to be related to the pathophysiology of West syndrome and other neurodevelopmental disorders.

Effect of pressure-controlled inverse ratio ventilation on dead space during robot-assisted laparoscopic radical prostatectomy: A randomised crossover study of three different ventilator modes.

BACKGROUND: Pressure-controlled inverse inspiratory to expiratory ratio ventilation (PC-IRV) is thought to be beneficial for reducing the dead space volume. OBJECTIVE: To investigate the effects of PC-IRV on the components of dead space during robot-assisted laparoscopic radical prostatectomy (RLRP). DESIGN: A randomised crossover study of three different ventilator modes. SETTING: A single university hospital from September 2014 to April 2015. PATIENTS: Twenty consecutive study participants undergoing RLRP. INTERVENTIONS: Patients were ventilated sequentially with three different modes in random order for 30 min: volume control ventilation (VCV; inspiratory to expiratory ratio 0.5), pressure control ventilation (PCV; inspiratory to expiratory ratio 0.5) and PC-IRV. Inverse inspiratory to expiratory ratio was adjusted individually by observing the expiratory flow-time wave to prevent the risk of dynamic pulmonary hyperinflation. MAIN OUTCOME MEASURES: The primary outcome included physiological dead space (VDphys), airway dead space (VDaw), alveolar dead space (VDalv) and shunt dead space (VDshunt). VDphys was calculated by Enghoff's method. We also analysed respiratory dead space (VDresp) and VDaw using a novel analytical method. Then, VDalv and VDshunt were calculated by VDalv = VDresp - VDaw and VDshunt = VDphys - VDresp, respectively. RESULTS: The VDphys/expired tidal volume (VTE) ratio in PC-IRV (29.2 ±â€Š4.7%) was significantly reduced compared with that in VCV (43 ±â€Š8.5%) and in PCV (35.9 ±â€Š3.9%). The VDshunt/VTE in PC-IRV was significantly smaller than that in VCV and PCV. VDaw/VTE in PC-IRV was also significantly smaller than that in VCV but not that in PCV. There was no significant change in VDalv/VTE. CONCLUSION: PC-IRV with the inspiratory to expiratory ratio individually adjusted by the expiratory flow-time wave decreased VDphys/VTE in patients undergoing RLRP. TRIAL REGISTRATION: University Hospital Medical Information Network in Japan 000014004.

Molecular and clinical features of KATP -channel neonatal diabetes mellitus in Japan.

BACKGROUND: There are few reports pertaining to Asian patients with neonatal diabetes mellitus (NDM) caused by activating mutations in the ATP-sensitive potassium channel genes (KATP-NDM). OBJECTIVES: To elucidate the characteristics of Japanese patients with KATP-NDM. METHODS: By the amplification and direct sequencing of all exons and exon-intron boundaries of the KCNJ11 and ABCC8 genes, 25 patients with KATP-NDM were identified from a total of 70 patients with NDM. Clinical data were collected from the medical charts. RESULTS: Sixteen patients had mutations in KCNJ11 and nine in ABCC8. Eight novel mutations were identified; two in KCNJ11 (V64M, R201G) and six in ABCC8 (R216C, G832C, F1176L, A1263V, I196N, T229N). Interestingly, V64M caused DEND (developmental delay, epilepsy, neonatal diabetes) syndrome in our patient, while mutation of the same residue (V64G) had been reported to cause congenital hyperinsulinism. Mutations in ABCC8 were associated with TNDM (4/9) or isolated PNDM (5/9), whereas those in KCNJ11 were associated with more severe phenotypes, including DEND (3/16), iDEND (intermediate DEND, 4/16), or isolated PNDM (6/16). Switching from insulin to glibenclamide monotherapy was successful in 87.5% of the patients. Neurological improvement was observed in two patients, one with DEND (T293N) and one with iDEND (R50P) syndrome. Three others with iDEND mutations (R201C, G53D, and V59M) remained neurologically normal at 5, 1, and 4 years of age, respectively, with early introduction of sulfonylurea. CONCLUSION: Overall, clinical presentation of KATP-NDM in Japanese patients was similar to those of other populations. Early introduction of sulfonylurea appeared beneficial in ameliorating neurological symptoms.

Clinical and genetic diversities of Charcot-Marie-Tooth disease with MFN2 mutations in a large case study.

Charcot-Marie-Tooth disease (CMT) constitutes a heterogeneous group affecting motor and sensory neurons in the peripheral nervous system. MFN2 mutations are the most common cause of axonal CMT. We describe the clinical and mutational spectra of CMT patients harboring MFN2 mutations in Japan. We analyzed 1,334 unrelated patients with clinically suspected CMT referred by neurological and neuropediatric departments throughout Japan. We conducted mutation screening using a DNA microarray, targeted resequencing, and whole-exome sequencing. We identified pathogenic or likely pathogenic MFN2 variants from 79 CMT patients, comprising 44 heterozygous and 1 compound heterozygous variants. A total of 15 novel variants were detected. An autosomal dominant family history was determined in 43 cases, and the remaining 36 cases were reported as sporadic with no family history. The mean onset age of CMT in these patients was 12 ± 14 (range 0-59) years. We observed neuropathic symptoms in all patients. Some had optic atrophy, vocal cord paralysis, or spasticity. We detected a compound heterozygous MFN2 mutation in a patient with a severe phenotype and the co-occurrence of MFN2 and PMP22 mutations in a patient with an uncommon phenotype. MFN2 is the most frequent causative gene of CMT2 in Japan. We present 15 novel variants and broad clinical and mutational spectra of Japanese MFN2-related CMT patients. Regardless of the onset age and inheritance pattern, MFN2 gene analysis should be performed. Combinations of causative genes should be considered to explain the phenotypic diversity.

Effects of lubrication on air-sealing performance of a pediatric cuffed tracheal tube.

BACKGROUND: Lubrication of cuffed tracheal tubes (CTTs) reduces liquid leakage. However, it is not clear how cuff lubrication influences air leakage. We aimed to test the hypothesis that pretreatment with K-Y jelly, a water-soluble lubricant, would improve the air-sealing performance of pediatric CTTs in a model study. METHODS: We placed Parker Flex-Tip™ CTT with 4.0- and 5.0-mm internal diameter (ID) into a tracheal model with 9- and 12-mm ID. The tracheal model was connected to a test lung ventilated in pressure control mode. We compared three cuff lubrication conditions: none (N), water (W), and K-Y jelly (KY). We measured the leak airway pressure (LAWP), defined as the lowest peak airway pressure (PAWP) at which leakage was detected, with the fixed cuff pressure (CP) at 20 cmH2O and varied PAWP. We also measured the leak CP (LCP), defined as the highest CP at which leakage was detected, with fixed PAWP at 25 cmH2O and varied CP. We confirmed air leakage when an apparent elevation of oxygen concentration was detected above the cuff after changing the inspiratory gas from air to oxygen. RESULTS: For both 4.0-mm ID and 5.0-mm ID endotracheal tubes, the KY group showed significantly higher LAWP and lower LCP than the other two groups. For the 4.0-mm ID, median values and ranges of LAWP and LCP were K-Y group: 25 (25) and 15 (15); N group: 5 (5) and 35 (35): and W group: 5 (5) and 35 (15-35) cmH2O. For the 5.0-mm ID, median values and ranges of LAWP and LCP were K-Y group: 25 (15-25) and 15 (15-35); N group: 5 (5) and 35 (35); and W group: 5 (5) and 35 (15-35) cmH2O. Water application did not change these outcomes compared with the N group. CONCLUSION: Pre-treatment of the cuff with K-Y jelly significantly improved the air-sealing performance of a pediatric CTT in our model study.

Effect of head position on the success rate of blind intubation using intubating supraglottic airway devices.

BACKGROUND: To evaluate the effect of head position on the performance of intubating supraglottic airway devices, we compared the success rate of blind intubation in the head-elevated and the pillowless head positions with the LMA Fastrach and the air-Q, and the change of glottic visualization through the air-Q. METHODS: We assigned 193 patients to two groups according to the device used and subgrouped by head position used for intubation: Fastrach/pillowless, Fastrach/head-elevated, air-Q/pillowless, and air-Q/head-elevated. Blind intubation through the Fastrach or the air-Q was attempted up to twice after induction of general anesthesia. Before the attempt at blind intubation with the air-Q, the percentage of glottic opening (POGO) score was also fiberscopically evaluated at the outlet of the device in both head positions in a cross-over fashion. RESULTS: The Fastrach significantly facilitated blind intubation compared with the air-Q in both the pillowless and head-elevated positions: 87.2% in Fastrach/pillowless vs 65.9% in air-Q/pillowless (P=.048), 90% in Fastrach/head-elevated vs 53.7% in air-Q/head-elevated (P<.001). The head-elevated position did not significantly affect the success rate of blind intubation for either device (P=.97 in Fastrach, P=.37 in air-Q). Although the head-elevated position significantly improved the POGO score from the median (10-90 percentile) 60% (0-100%) in the pillowless position to 80% (0-100%) (P=.008), it did not contribute to successful blind intubation with the air-Q. CONCLUSION: Although the head-elevated position improved glottic visualization in the air-Q, the head position had minimal influence on the success rate of blind intubation with either the Fastrach or the air-Q.

Clinical and genetic investigation of 17 Japanese patients with hyperekplexia.

AIM: The aim of the study was to determine clinical and genetic characteristics of Japanese patients with hyperekplexia. METHOD: Clinical courses, responses to antiepileptic drugs, outcomes, and genetic testing were investigated in 17 Japanese patients (nine males, eight females, median age 1y, range birth-45y) with hyperekplexia. RESULTS: In all patients, muscle stiffness and startle responses appeared soon after birth. Only seven patients were diagnosed with hyperekplexia before 1 year of age. Seven patients had been misdiagnosed with other disorders such as epilepsy and adult-onset anxiety neurosis. Umbilical/inguinal hernias were seen in 10 patients. Life-threatening events were noted in four patients. Clonazepam was the most effective drug. Muscle stiffness completely disappeared in 12 patients before 5 years of age, whereas startle responses resolved in only three patients. Mutations in the GLRA1 and GLRB genes were identified in 16 patients and one patient respectively. In 14 patients, the mutation showed autosomal dominant inheritance; in the other three, inheritance was autosomal recessive. p.R271Q of GLRA1 was the most frequent mutation, found in 10 patients. Novel mutations, p.A272P and p.A384P of GLRA1, were detected. Clinical severity and outcome varied even in the same family. INTERPRETATION: Early correct diagnosis is essential for prevention of accidental injuries and to provide appropriate treatments for hyperekplexia. Clonazepam is effective, although the time taken for startle responses to resolve varied.

Effects of sniffing position for tracheal intubation: a meta-analysis of randomized controlled trials.

BACKGROUND: The purpose of this meta-analysis was to validate the efficacy of the sniffing position in the performance of intubation with direct laryngoscopy. METHODS: We searched MEDLINE, the Cochrane Central Register of Controlled Trials, Embase, and Web of Science. Six randomized controlled trials comprising 2759 adult participants were analyzed. The DerSimonian-Laird method was used to calculate pooled relative risk (RR) and the 95% confidence interval (CI) of Cormack-Lehane classification, Intubation Difficulty Scale, success rate of the first intubation, and weighted mean difference of intubation time. RESULTS: Compared with the other head positions, the sniffing position did not improve glottic visualization, success rate of the first intubation, or intubation time. However, the sniffing position was significantly associated with better Intubation Difficulty Scale compared with the simple head extension position. (RR,1.28; 95% CI, 1.15-1.42; p<0.0001) CONCLUSIONS: Although patients do not benefit from the sniffing position in terms of glottic visualization, success rate of the first intubation, or intubation time, the sniffing position can still be recommended as the initial head position for tracheal intubation because the sniffing position provides easier intubation conditions.

Effects of gas composition on the delivered tidal volume of the Avance Carestation.

BACK GROUND: Measurements with various flowmeters are affected by changes in gas mixture density. The Avance Carestation incorporates ventilator feedback controlled by a built-in flowmeter with a variable orifice sensor. We hypothesised that changes in the composition of delivered gas may cause changes in the delivered tidal volume by affecting the flow measurement unless appropriate corrections are made. METHODS: We used 100 % O2, 40 % O2 in N2 and 40 % O2 in N2O as carrier gases with/without sevoflurane and desflurane. We measured delivered tidal volume using the FlowAnalyzer™ PF 300 calibrated with the corresponding gas mixtures during volume control ventilation with 500-ml tidal volume using the Avance Carestation connected to a test lung. RESULTS: Change of carrier gas and addition of sevoflurane and desflurane significantly altered delivered tidal volume. Desflurane 6 % reduced delivered tidal volume by 7.6, 3.6 and 16 % of the pre-set volume at 100 % O2, 40 % O2 in N2 and 40 % O2 in N2O, respectively. Importantly, the Carestation panel indicator did not register these changes in measured expired tidal volume. Ratios of delivered tidal volume to 500 ml correlated inversely with the square root of the delivered gas density. CONCLUSIONS: These results support our hypothesis and suggest that changing gas composition may alter delivered tidal volume of anesthesia machines with built-in ventilators that are feedback-controlled by uncorrected flowmeters due to changes in gas mixture density.

Comparison of intubation performance between the King Vision and Macintosh laryngoscopes in novice personnel: a randomized, crossover manikin study.

PURPOSE: The King Vision laryngoscope is a newly developed video laryngoscope. We conducted a simulation study to evaluate the efficacy of the King Vision in novice personnel. METHODS: Thirty-one registered nurses with no previous experience with tracheal intubation were enrolled. Participants made 6 consecutive attempts at intubation of the manikin's trachea with a Macintosh laryngoscope (MAC) and the King Vision with channeled blade (KVC) and non-channeled blade (KVNC) in a randomized cross-over fashion. The Grading Scale of Intubation Difficulty (GSID) was rated on a 5-point scale. RESULTS: Overall median (range) intubation times (sec) were 16.9 (8.0-60.0) with the MAC, 20.5 (7.2-60.0) with the KVC, and 60.0 (11.0-60.0) with the KVNC. The KVNC required significantly longer intubation time compared with the MAC or the KVC (p < 0.001). Success rate with the KVNC was 47.3 %, which was significantly inferior to that with the MAC (91.4 %) or KVC (86.6 %). Median GSID was 2 (range 1-5) with the KVC and 3 (1-4) with the MAC, which were both significantly lower than the 4 (2-5) with the KVNC (p < 0.001). Esophageal intubation with the MAC occurred in 18 of 186 attempts, whereas no incidents of esophageal intubation occurred with the KVC or KVNC. CONCLUSION: The KVC facilitated intubation by novice personnel without incidence of esophageal intubation. However, intubation times, success rates, and GSID scores were similar to the values obtained with the MAC. These findings suggest that the KVC, but not the KVNC, could be used as an alternative device for intubation by novice personnel.

[Clinical profile of persistent generalized muscle contraction following the insult of developing brain].

OBJECTIVE: Persons with severe motor and intellectual disabilities (SMID) caused by injury to the developing brain sometimes present generalized hypertonia in a specific position with extreme muscle overactivity persisting for most of the time during wakefulness. This "persistent generalized muscle contraction" is often associated with bad humor, sleep disturbance, hyperhidrosis, wasting, elevation of serum creatine kinase levels, regular daytime use of hypnotic or sedative medication, and the necessity to maintain the neck or hip in a flexed position manually. The aim of this study is to elucidate the clinical profile of this condition. METHODS: We retrospectively examined the medical records and brain imaging data of 66 SMID patients in the state of persistent generalized muscle contraction. RESULTS: Most patients could be classified into 2 major categories on the basis of clinical presentation and brain imaging: (A) those with premature birth and bilateral lesion of globus pallidus interna (kernicterus) (n = 16), and (B) those with various widespread bilateral basal ganglia/thalamic and/or cerebral lesions such as hypoxia-ischemia, acute encephalopathy, malformation, etc (n = 50). Group A assumed an asymmetrical tonic-neck-reflex-like position, torsion of the trunk, fluctuation of hypertonia, and better mental development. Three of them exhibited extreme hypertonia resembling status dystonicus. Group B often exhibited persistent and fixed retroflexion of the neck and trunk or opisthotonus. Drugs such as oral muscular relaxants were ineffective in both groups. Injection of botulinum toxin into the cervical and paravertebral muscles partially alleviated symptoms. CONCLUSIONS: Persistent generalized muscle contraction in SMID has at least two different types. Group A has characteristics of severe dystonic hypertonia that could lead to status dystonicus. Group B might have peculiar characteristics of muscle overactivity triggered by wakefulness or discomfort, which probably results from inability to achieve spontaneous muscle relaxation.

Development of a New Method for Evaluating Heat-and-Moisture Exchanger Performance.

BACKGROUND: A model system described in International Organization for Standardization 9360 is the standard method for estimating the humidifying performance of heat-and-moisture exchangers (HMEs). However, there are no reliable bedside methods for evaluating the ongoing humidification performance of HMEs. Therefore, this study aimed to develop 2 clinically applicable methods for estimating the ongoing humidifying performance of HMEs and to evaluate their reliability in a model system. METHODS: Physiologically expired gas was simulated using a heated humidifier, and ventilation was delivered using a ventilator with constant flow through 3 different types of HMEs. Relative humidity (RH) was measured using a capacitive-type moisture sensor. Water content lost during expiration was calculated by integrating absolute humidity (AH), instantaneous gas flow measured at the expiratory outlet of the ventilator, and time. We also calculated the water content released and captured by the HMEs during tidal ventilation by integrating the difference in AH across the HMEs, instantaneous gas flow, and time. RESULTS: We found that the RH, temperature, and AH were almost constant on the expiratory outlet of the ventilator but rapidly varied near the HMEs. The water content lost by the 3 HMEs was associated with the manufacturer-reported values and inversely correlated with the calculated values of the water content exchanged by the HMEs. The water content released and captured by HMEs was closely correlated with the difference in HME weight measured at the end of inspiration and expiration; however, the water content captured by HMEs seemed to be overestimated. CONCLUSIONS: Our results demonstrated that our system was able to detect the differences in the performance of 3 models of HMEs and suggest that our method for calculating water loss is reliable for estimating the water retention performance of HMEs during mechanical ventilation, even in the presence of a constant flow.

Hemodynamic response related to the Airway Scope versus the Macintosh laryngoscope: A systematic review and meta-analysis with trial sequential analysis.

BACKGROUND: It is important to reduce the hemodynamic response during tracheal intubation. We performed a systematic review and meta-analysis of the Airway Scope and Macintosh laryngoscope to determine whether they reduce the hemodynamic responses of heart rate (HR) and mean blood pressure (MBP) after tracheal intubation under general anesthesia. METHODS: We performed a comprehensive literature search of electronic databases for clinical trials comparing hemodynamic response to tracheal intubation. The primary aim of our meta-analyst is to determine if the Airway Scope reduces hemodynamic responses (HR and mean MBP) 60 seconds after tracheal intubation compared to the Macintosh laryngoscope. We expressed pooled differences in hemodynamic responses between the 2 devices as weighted mean differences with 95% confidence intervals. We conducted trial sequential analysis. Secondarily, we investigated the ability of the Airway Scope and Macintosh laryngoscope to reduce hemodynamic responses at 120 seconds, 180 seconds, and 300 seconds after tracheal intubation. RESULTS: We identified clinical trials comparing hemodynamic response via a comprehensive literature search. Of 185 articles found in the search, we selected 8. In comparison to the Macintosh laryngoscope, the Airway Scope significantly reduced HR and MBP at 60 seconds after tracheal intubation (HR; weighted mean difference = -7.29; 95% confidence interval, -10.9 to -3.62; P < .0001; I2 = 57%, MBP; weighted mean difference = -11.5; 95% confidence interval, -20.4 to -2.65; P = .01; I2 = 91%). At the secondary outcome, the Airway Scope significantly reduced the fluctuation of HR after 120 seconds and 180 seconds of tracheal intubation. However, the Airway Scope did not significantly reduce MBP 120 seconds, 180 seconds, and 300 seconds after tracheal intubation. Trial sequential analysis suggested that the total sample size reached the required information size for heart rate. CONCLUSIONS: Our finding suggested that the Airway Scope attenuated hemodynamic responses at 60 seconds after tracheal intubation in comparison with that of the Macintosh laryngoscope. However, the MBP sample size is small and further research is needed.

Reading impairment after neonatal hypoglycemia with parieto-temporo-occipital injury without cortical blindness: A case report.

BACKGROUND: Perinatal brain injury may lead to later neurodevelopmental disorders, whose outcomes may vary due to neuroplasticity in young children. Recent neuroimaging studies have shown that the left parietotemporal area (which includes the left inferior parietal lobe) is associated with phonological awareness and decoding skills, which are essential skills for reading acquisition in children. However, the literature on the effect of perinatal cerebral injury on the development of phonological awareness or decoding ability in childhood is limited. CASE SUMMARY: We report the case of an 8-year-old boy who presented with reading difficulty following a perinatal injury in the parieto-temporal-occipital lobes. The patient was born at term and was treated for hypoglycemia and seizures during the neonatal period. Diffusion-weighted brain magnetic resonance imaging on postnatal day 4 revealed cortical and subcortical hyperintensities in the parieto-temporo-occipital lobe. At the age of 8 years, physical examination was unremarkable, aside from mild clumsiness. Despite occipital lobe injury, the patient had adequate visual acuity, normal eye movement, and no visual field defects. Full-scale intelligence quotient and verbal comprehension index on Wechsler Intelligence Scale for Children-Fourth Edition were 75 and 90, respectively. Further assessment revealed adequate recognition of Japanese Hiragana letters. However, he had significantly slower reading speed in the Hiragana reading test than control children. The phonological awareness test revealed significant errors (standard deviation +2.7) in the mora reversal task. CONCLUSION: Patients with perinatal brain injuries in the parietotemporal area require attention and may benefit from additional reading instructions.

Clinical and radiological features of Japanese patients with a severe phenotype due to CASK mutations.

Heterozygous loss of function mutations of CASK at Xp11.4 in females cause severe intellectual disability (ID) and microcephaly with pontine and cerebellar hypoplasia (MICPCH). However, the longitudinal clinical and radiological course of affected patients, including patterns of postnatal growth, has not been described. Neurodevelopmental and imaging information was retrospectively accrued for 16 Japanese (15 female and 1 male) patients with ID and MICPCH associated with CASK mutations. All records were analyzed; patient age ranged from 2 to 16 years at the time of the most recent examinations. The growth pattern, neurological development, neurological signs/symptoms, and facial features were similar in the 15 female patients. Their head circumference at birth was within the normal range in about half, and their height and weight were frequently normal. This was followed by early development of severe microcephaly and postnatal growth retardation. The patients acquired head control almost normally between 3 and 6 months, followed by motor delay. More than half of the female patients had epilepsy. Their MRIs showed microcephaly, brainstem, and cerebellar hypoplasia in early infancy, and a normal or large appearing corpus callosum. The male patient showed a more severe clinical phenotype. These uniform clinical and radiological features should facilitate an early diagnosis and be useful for medical care of females with ID and MICPCH associated with CASK mutations.

[Survival analysis for patients with severe motor and intellectual disabilities following tracheotomy].

To investigate the survival rate and causes of death in patients with severe motor and intellectual disabilities (SMIDs) that necessitated tracheotomy, we retrospectively analyzed 90 patients who underwent tracheotomy between 1990 and 2009. Indications for tracheotomy in these patients were upper airway obstruction (44 patients), recurrent aspiration pneumonia (28 patients), retained secretions (23 patients), prolonged mechanical ventilation (18 patients), chronic respiratory failure (9 patients), central respiratory failure (5 patients), and gastroesophageal reflux (8 patients). Most of the patients underwent tracheotomy at the age of 0-5 years or 10-19 years. As of April 1, 2010, 28 patients had died. The survival rate was 0.91 at 1 year, 0.74 at 5 years, 0.59 at 10 years, 0.54 at 15 years, and 0.40 at 19 years after tracheotomy. Massive tracheal bleeding due to development of tracheo-innominate artery fistulas occurred in 5 patients, and 4 of them died. They were thirteen years of age or older when they underwent tracheotomy, and developed fistulas after 2 weeks or later. In contrast, 7 patients at high risk for fistula formation, including those that had developed severe tracheomalacia associated with granulation or warning hemorrhages, underwent preventive resection of the innominate artery, and all of them had survived. It is important to regularly evaluate patients with SMIDs who have undergone tracheotomy by using bronchofiberscopy to identify risk factors for tracheoinnominate artery fistulas, a preventable cause of death.