Anesthesia for a child with giant axonal neuropathy.
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Anesthesia↗
Biomedical subjects
J Attias
Publications and source records attributed to J Attias.
Hyperacusis in Williams syndrome: characteristics and associated neuroaudiologic abnormalities.
BACKGROUND: Hyperacusis and phonophobia are common, debilitating symptoms in Williams syndrome (WS), yet little is known about their underlying audiologic and neurologic processes. METHODS: The mothers of 49 subjects with WS were asked to complete the Hyperacusis Screening Questionnaire. Subjects with reported hyperacusis and sufficient developmental capacity underwent comprehensive audiological and brain auditory evoked response (BAER) testing. Findings were compared with those from pair-matched typically developing control subjects. RESULTS: Forty-one of the 49 children with WS (84%) had hyperacusis of moderate to severe degree, which began in infancy. Of these, 21 (mean age 15.8 +/- 5.5 years) were quantitatively tested. Subjects with WS reported discomfort at sound intensities on average 20 dB lower than control subjects. Pure-tone audiometry and distortion products otoacoustic emission test revealed a high-frequency cochlear hearing loss. An absence of ipsilateral acoustic reflex responses to maximum stimulation was significantly more common in the subjects with WS than controls. On BAER testing, the WS group had a significant prolongation in wave I latency. CONCLUSIONS: Hyperacusis in Williams syndrome (WS) is associated with a high-frequency hearing loss resembling the configuration of noise-induced hearing loss. The hyperacusis and hearing loss in WS may stem from a deficiency in the acoustic reflex resulting from auditory nerve dysfunction. Additional mechanisms that may mediate hyperacusis in WS and should be evaluated in future studies include recruitment, malformation of the facial canal, and haploinsufficiency of the elastin gene.
Acoustic Stimulation↗
The prevalence of congenital and early-onset hearing loss in Jordanian and Israeli infants.
The objective of the study was to investigate the prevalence of congenital and early-onset hearing loss, and the influence of the known risk factors for hearing loss on infants in Jordan and Israel. Subjects were a total of nearly 17,000 infants from both countries, including infants with and without risk factors for hearing loss. The hearing screening protocol included distortion product otoacoustic emission, followed in case of repeated OAE referral or high risk (HR) infant by diagnostic auditory brainstem responses. The results indicate that the prevalence and severity of hearing loss amongst Jordanian infants (1.37%) is remarkably higher as compared to the Israeli infants (0.48%). The overall prevalence of bilateral SNHL was seven times more in the Jordanian infants, 18 times in non-risk, and three times in the HR infants relative to the Israeli infants. Risk factors including family history, hyperbilirubinemia, bacterial meningitis, and associated syndromes were more prevalent amongst Jordanian infants. This unique study underscores the importance of sharing and exchanging information to create empirical data to guide health-care providers in adapting protocols to the local constraints in developing countries.
Developing Countries↗
Dysfunction of the auditory efferent system in patients with traumatic brain injuries with tinnitus and hyperacusis.
OBJECTIVES: Tinnitus, hyperacusis and difficulty listening in background noise are common symptoms reported by patients with traumatic brain injury (TBI). The aim of this study was to explore the function of the auditory system in TBI patients with and without auditory complaints but having normal pure-tone audiograms. METHODS: The study consisted of 24 TBI patients with and 10 TBI patients without auditory complaints. In addition, 15 normal controls were included in the study. The function of the auditory system was tested by recording transient otoacoustic emissions (TEOAE) during the presentation of increasing levels of white noise in the contralateral ear. RESULTS: Most of the TBI patients with auditory complaints (87%) showed absent or significantly reduced effect of the auditory efferent system as compared with the TBI patients without auditory complaints and to normal controls. However, the global amplitude of the TEOAE was significantly higher in TBI patients with auditory complaints compared to those without. CONCLUSIONS: Due to its role in peripheral and central auditory activity, dysfunction of the efferent system may be at least partially responsible for these auditory complaints. This study underscores the importance of testing and evaluating the functional integrity of the medial efferent system by an objective and non-invasive method in patients with TBI.
Acoustic Stimulation↗
Reduction in noise-induced temporary threshold shift in humans following oral magnesium intake.
A previous study demonstrated the prophylactic effects of magnesium on noise-induced permanent threshold shift in humans. For the first time, this study explores the effects of magnesium on temporary threshold shift in 20 human subjects, all men (16-37 years, mean age 21 years). The study was conducted in a double-blind manner on the same subjects tested in three different phases (placebo, magnesium, no-drug). The cochlear changes were assessed using both behavioural (audiograms) and objective (otoacoustic emission) measures. Blood samples were collected at the beginning and end of each phase. Amongst subjects and phases, magnesium intake was associated with significantly lower temporary threshold shift, compared with the other two phases, which were reflected both by the behavioural and cochlear measures. A correlation was found between the blood magnesium levels and temporary threshold shift reduction. No side-effects were associated with the oral ingestion of the magnesium. Magnesium provides significant protection against temporary threshold shift, complementing the previous permanent threshold shift human study. Both human noise-induced hearing loss studies introduced a novel, biological, natural agent for prevention and possible treatment of noise-induced cochlear damage in humans.
Acoustic Impedance Tests↗
Monitoring of the sciatic nerve during hamstring lengthening by evoked EMG.
Traction injury to the sciatic nerve can occur during hamstring lengthening. The aim of this study was to monitor the influence of hamstring lengthening on conduction in the sciatic nerve using evoked electromyography (EMG). Ten children with spastic cerebral palsy underwent bilateral distal hamstring lengthening. Before lengthening, the evoked potential was recorded with the patient prone. During lengthening, it was recorded with the knee flexed to 90 degrees, 60 degrees and 30 degrees, and at the end of lengthening with the hip and knee extended. In all patients, the amplitude of the evoked EMG gradually decreased with increasing lengthening. The mean decrease with the knee flexed to 60 degrees was 34% (10 to 77), and to 30 degrees, 86% (52 to 98) compared with the pre-lengthening amplitude. On hip extension at the end of the lengthening procedure, the EMG returned to the pre-lengthening level. Monitoring of the evoked EMG potential of the sciatic nerve during and after hamstring lengthening, may be helpful in preventing traction injury.
Adolescent↗
Pomegranate juice flavonoids inhibit low-density lipoprotein oxidation and cardiovascular diseases: studies in atherosclerotic mice and in humans.
The beneficial health effects attributed to the consumption of fruit and vegetables are related, at least in part, to their antioxidant activity. Of special interest is the inverse relationship between the intake of dietary nutrients rich in polyphenols and cardiovascular diseases. This effect is attributed to polyphenols' ability to inhibit low-density lipoprotein (LDL) oxidation, macrophage foam cell formation and atherosclerosis. Pomegranate polyphenols can protect LDL against cell-mediated oxidation via two pathways, including either direct interaction of the polyphenols with the lipoprotein and/or an indirect effect through accumulation of polyphenols in arterial macrophages. Pomegranate polyphenols were shown to reduce the capacity of macrophages to oxidatively modify LDL, due to their interaction with LDL to inhibit its oxidation by scavenging reactive oxygen species and reactive nitrogen species and also due to accumulation of polyphenols in arterial macrophages; hence, the inhibition of macrophage lipid peroxidation and the formation of lipid peroxide-rich macrophages. Furthermore, pomegranate polyphenols increase serum paraoxonase activity, resulting in the hydrolysis of lipid peroxides in oxidized lipoproteins and in atherosclerotic lesions. These antioxidative and antiatherogenic effects of pomegranate polyphenols were demonstrated in vitro, as well as in vivo in humans and in atherosclerotic apolipoprotein E deficient mice. Dietary supplementation of polyphenol-rich pomegranate juice to atherosclerotic mice significantly inhibited the development of atherosclerotic lesions and this may be attributed to the protection of LDL against oxidation.
Animals↗
Ectodermal dysplasia, ectrodactyly and macular dystrophy (EEM syndrome) in siblings.
We report a brother and sister with ectodermal dysplasia, ectrodactyly, and macular dystrophy (the EEM syndrome). Both children had abnormalities of the hands and the hair, and bilateral macular degeneration. The clinical picture in both is similar to, but less severe than, that described in the previously reported cases of this rare syndrome. Even though the parents are not related, they are both of Jewish Yemenite origin, and the possibility of a common ancestor cannot be ruled out. This would suggest autosomal recessive inheritance. The clinical picture in these patients suggests either variable expression or genetic heterogeneity in the EEM syndrome and further delineates the clinical and genetic spectrum of this condition.
Abnormalities, Multiple↗
Anxiety reaction in children during removal of their plaster cast with a saw.
We have had experience of an 18-month-old boy with a cardiomyopathy who died a few minutes after removal of his cast with a saw, apparently from a malignant cardiac arrhythmia triggered by anxiety. We therefore examined the anxiety reaction to this method of removal of a plaster cast in 20 healthy children; ten were provided with hearing protectors and ten were not. The level of anxiety was assessed by measuring the heart rate, a known physiological indicator of anxiety, before, during and five minutes after removal of the cast. The noise level was also measured. The results showed a mean increase in heart rate during the procedure of 27.9 beats per minute (bpm) (26.9%) in the children with no hearing protectors and 10.4 bpm (11.1%) in children who used hearing protectors (p < 0.001). Five minutes after the procedure the heart rate had returned to the baseline rate in all patients. We recommend that hearing protectors should be used in children undergoing removal of a plaster cast to decrease the anxiety reaction. If possible, clinicians should avoid the use of a saw for this purpose in children with a cardiomyopathy.
Anxiety↗
Attenuation of atherosclerosis in apolipoprotein E-deficient mice by ramipril is dissociated from its antihypertensive effect and from potentiation of bradykinin.
We investigated the mechanism of the antiatherosclerotic effect of the angiotensin-converting enzyme (ACE) inhibitor, ramipril, in the apolipoprotein (apo) E-deficient mice. Mice that received a high dose (5 mg/kg/day) of ramipril supplemented in their drinking water for 10 weeks showed reduced aortic lesion size by 75% compared with placebo-treated mice. At this dosage, ramipril significantly reduced blood pressure from 95+/-5 mm Hg before treatment to 68+/-4 mm Hg at the end of the treatment period. Ramipril also increased the resistance of the mouse low-density lipoprotein (LDL) to CuSO4-induced oxidation, as shown by a prolongation of the lag time required for the initiation of LDL oxidation from 90 min in the placebo-treated mice to >180 min in the ramipril-treated mice. Similarly, a reduction in the maximal LDL-associated conjugated dienes after 180 min of oxidation by 250% in comparison with placebo-treated mice was noted. Ramipril (1 mg/kg/day) that was still adequate to reduce their plasma ACE activity and LDL propensity to lipid peroxidation was insufficient to reduce their blood pressure. This dosage also inhibited the progression of atherosclerosis in the apo E-deficient mice by 74%. The contribution of bradykinin potentiation to the ACE-inhibitor action was assessed by cotreatment of ramipril with the bradykinin B2-receptor antagonist, icatibant (HOE-140, 0.5 mg/kg given subcutaneously twice a day) for a period of 10 weeks. HOE-140 had no effects on ACE activity, LDL lipid peroxidation, blood pressure, or atherosclerosis. In combination with ramipril, no additional effect of HOE-140 on LDL oxidation or on atherosclerosis was noted in comparison with ramipril treatment alone. We thus conclude that the antiatherogenic effect of ramipril in E(0) mice is independent of blood pressure reduction and is not mediated by bradykinin. It seems, therefore, that most of its antiatherosclerotic and antioxidative effects are mediated through the inhibition of angiotensin II production.
Angiotensin-Converting Enzyme Inhibitors↗
Evaluating children's hearing by DPOAEs at 1-10 kHz.
The efficacy of application of DPOAE (distortion product otoacoustic emission) in the evaluation of hearing in children was assessed. 106 children were checked by both DPOAE and ABR. The results show that DPOAE is highly effective in discriminating between hearing disorders and normal hearing. The correct classification rate of normal ears varied between 86.8-96.8% and ears with hearing loss (ABR threshold equal or above 20 dB HL) 87-100%. At the lower frequencies (1-2 kHz) the hit rate was lower. The results of this study suggest that the DPOAE can be used as a rapid hearing screening test for infants and children providing frequency specific information, mostly in the 3-10 kHz frequency range. The effectiveness of DPOAE at 1-10 kHz was evident in children with middle ear dysfunction and in detecting infants with possible high frequency hearing loss. In addition, high frequency DPOAE broadens the range typically available from click-evoked ABR measurements. This information is of increased clinical value not only in terms of speech and language development but also for detecting children who are at high-risk for possible hearing deterioration and who require early intervention.
Acoustic Impedance Tests↗
The angiotensin-converting enzyme inhibitor, fosinopril, and the angiotensin II receptor antagonist, losartan, inhibit LDL oxidation and attenuate atherosclerosis independent of lowering blood pressure in apolipoprotein E deficient mice.
OBJECTIVE: To investigate the possible mechanisms of the antiatherosclerotic effects of the angiotensin-converting enzyme (ACE) inhibitor, fosinopril, in apolipoprotein (apo) E deficient mice. METHODS: Apo E deficient (E0) mice at the age of 8 weeks received either placebo or a high dose (25 mg/kg/d) of fosinopril supplemented in their drinking water. RESULTS: After 12 weeks of treatment, fosinopril reduced the aortic lesion size by 70%, compared with the placebo group. At this dosage, fosinopril significantly reduced blood pressure from 93 +/- 2 mmHg before treatment to 70 +/- 2 mmHg at the end of the treatment period (P < 0.005). Fosinopril also increased the resistance of the mice plasma low density lipoprotein (LDL) to CuSO4-induced oxidation, as shown by a 90% reduction in the LDL content of malondialdehyde (MDA) and also by a prolongation of the lag time required for the initiation of LDL oxidation (from 100 min in the placebo-treated mice to more than 240 min in the fosinopril-treated mice; P < 0.001). In addition, fosinopril inhibited CuSO4-induced oxidation of LDL that was obtained from the aortas of the treated mice, as shown by an 18% and 37% reduction in the LDL content of lipid peroxides and hydroperoxy-cholesterol linoleate, respectively, compared with the placebo-treated mice (P < 0.01). A low dosage of fosinopril (5 mg/kg/d) that was still adequate to reduce their plasma ACE activity and LDL propensity to lipid peroxidation was insufficient to lower their blood pressure. This dosage also reduced the aortic lesion size in the apo E deficient mice by 40% (P < 0.01). CONCLUSIONS: The antiatherogenic effects of fosinopril in apo E deficient mice are due not only to blood pressure reduction but also to the direct inhibition of angiotensin II-dependent effects, which are probably also associated with the inhibition of LDL oxidation.
Angiotensin Receptor Antagonists↗
Angiotensin II atherogenicity in apolipoprotein E deficient mice is associated with increased cellular cholesterol biosynthesis.
Angiotensin II (Ang II) was shown to be an important risk factor for accelerated atherosclerosis. Inhibition of Ang II action on the arterial wall by blocking its production with angiotensin converting enzyme (ACE) inhibitors, or by blocking binding to its receptors on cells with antagonists was shown to attenuate atherogenesis in animal model of atherosclerosis. We questioned whether Ang II atherogenicity is related to a stimulatory effect of Ang II on macrophage cholesterol biosynthesis. Angiotensin II injected intraperitoneally once a day (0.1 ml of 10(-7) M per mouse) for a period of 30 days, to the apolipoprotein E deficient mice increased the atherosclerotic lesion area by 95% (P < 0.01 vs. control), compared to placebo-injected mice, with no significant effect on blood pressure or on plasma cholesterol levels. On using mouse peritoneal macrophages (MPMs) that were harvested after intraperitoneally injection of Ang II, an increased rate of cellular cholesterol biosynthesis (measured as incorporation of [3H]acetate into cholesterol) by up to 90% (P < 0.01 vs. control) was observed. In mice treated with the ACE inhibitor, Fosinopril (25 mg/kg per day) a reduction in their MPM's cholesterol synthesis by up to 70% (P < 0.01 vs. control) was obtained. In vitro studies in human monocyte-derived macrophages (HMDM), in MPMs from control BALB/c mice, and in J-774 A.1 macrophage-like cell line demonstrated up to 44, 34 and 30% stimulation of macrophage cholesterol biosynthesis, respectively, following cell incubation with 10(-7) M Ang II for 18 h at 37 degrees C. The stimulatory effect of Ang II on macrophage cholesterol biosynthesis could be related to its interaction with the macrophage AT1 receptor, as Losartan (10(-5) M), an AT1 blocker, but not PD 123319 (10(-5) M), an AT2 blocker, prevented the stimulatory effect on macrophage cholesterol synthesis. Furthermore, in cells that lack the AT1 receptor (RAW macrophages), Ang II did not increase cellular cholesterol synthesis. Ang II increased macrophage 3-hydroxy-3-methyl glutaryl CoA (HMG CoA) reductase mRNA levels in a dose dependent manner in J-774 A.1 macrophages and in MPM. Losartan, the AT1 receptor antagonist clearly attenuated this mRNA induction. We thus conclude that Ang II stimulation of macrophage cholesterol biosynthesis is related to its interaction with the AT1 receptor, followed by stimulation of macrophage HMG CoA reductase gene expression, which leads to increased cellular cholesterol biosynthesis, and can possibly result in macrophage cholesterol accumulation and foam cell formation.
Angiotensin II↗
Otological evaluation of newborns who failed otoacoustic emission screening.
Early identification of congenital hearing loss and early rehabilitation is extremely important. Otoacoustic emissions (OAE) are an efficient tool for hearing screening. Previous studies using click evoked otoacoustic emissions (CEOAEs) for newborn hearing screening resulted in approximately 70% pass rate, reflecting intact hearing. The aim of our study was to perform a detailed otological evaluation of newborns who failed OAE screening, using otoscopy, tympanometry and ABR. CEOAEs were recorded from 257 newborns prior to their release from the hospital. Those babies who did not pass the CEOAE were examined by DPOAE, otoscopy, tympanometry and ABR, if needed. 73% of all the newborns had CEOAE in both ears. 20% had CEOAE in only one ear. When the test was administered again three days postpartum, the CEOAE pass rate increased; 98% passed in at least one ear. Most of the newborns (84%) who failed had an obstruction of the external ear canal (collapsed ear canal or debris). There was a good correlation between the otoscopy and the tympanometry. Based on the above results, a newborn hearing screening protocol was introduced.
Deafness↗
Antiatherosclerotic and antioxidative effects of captopril in apolipoprotein E-deficient mice.
The effect of the angiotensin-converting enzyme (ACE) inhibitor, captopril, on the development of atherosclerosis was determined in the apolipoprotein (apo) E-deficient mice. These mice develop severe hypercholesterolemia and extensive atherosclerotic lesions on chow diet, similar to those found in humans. Furthermore, in these mice, accelerated atherosclerosis is associated with increased plasma lipid peroxidation, a phenomenon that may play a crucial role in the buildup of the atherosclerotic lesions. Mice received either placebo or 50 mg/kg/day of captopril. After 12 weeks of treatment, captopril reduced the aortic-lesion area by 70% compared with that of the placebo-treated group. Captopril also increased the resistance of low-density lipoprotein (LDL) to CuSO4-induced oxidative stress, as shown by a significant reduction in the LDL content of malondialdehyde (MDA) by 30%, as well as by the prolongation of the lag time required for LDL oxidation from 55 min in the placebo-treated mice to 70 min in the captopril-treated mice, and reduction of the maximum LDL oxidation at 150 min by 35%. In vitro studies demonstrated that preincubation of LDL with captopril, inhibited the onset of CuSO4-induced LDL peroxidation up to 120 min, and reduced the LDL content of MDA by 90%. We conclude that captopril attenuates atherosclerosis in the apo E-deficient mice, and this phenomenon may be related to its inhibitory effect on the plasma LDL oxidation.
Angiotensin-Converting Enzyme Inhibitors↗
Evaluating noise induced hearing loss with distortion product otoacoustic emissions.
This study assessed the clinical efficacy of screening for noise induced hearing loss (NIHL) with distortion product otoacoustic emissions (DPOAE). DPOAEs were recorded from 76 military personnel (137 ears) aged between 17 and 41 years in response to equilevel 70 dB SPL primary stimulating tones. The 2f1-f2 DPOAE levels were correlated with audiometric thresholds at frequencies close to f2. Ears with normal audiograms, but with a history of military noise exposure, had DPOAEs that were significantly decreased in amplitude as compared to the ears of normal hearing non-exposed to noise subjects. These ears also had an increased absence of DPOAEs as compared with the ears of the normal hearing non-exposed to noise subjects. Although, in general, the DPOAE amplitudes and spectral frequency ranges reflected the audiometric NIHL configurations, in a number of cases DPOAEs were present for hearing losses up to 75 dB HL. As a consequence, DPOAEs correlated moderately and negatively with the audiometric thresholds. Applying test criteria designed to logically reflect NIHL, DPOAE sensitivity and specificity levels ranged between 0.51-0.90 and 0.63-0.25, respectively. These findings indicate that DPOAEs, recorded and analysed as described, are not sufficiently sensitive to serve as a single test to identify NIHL.
Acoustic Stimulation↗
Angiotensin II injection into mice increases the uptake of oxidized LDL by their macrophages via a proteoglycan-mediated pathway.
Angiotensin II (Ang-II) has been shown to possess several atherogenic properties including its ability to induce macrophage-mediated oxidation of LDL and to form Ang-II-modified LDL which is taken up by macrophages at enhanced rate. Oxidized-LDL (Ox-LDL) is also taken up by macrophages at enhanced rate via several scavenger receptors, leading to macrophage cholesterol accumulation. In the present study we examined the effect of Ang-II on the uptake of Ox-LDL by peritoneal macrophages derived from Balb/c mice (MPM). Intraperitoneal injection of Ang-II (10(-7) M, once daily for a period of 2 days) to the mice resulted in an increased Ox-LDL uptake up to 60%, in comparison to macrophages from placebo-treated mice. Similar results were obtained when Ang-II (10(-7) M) was injected to the mice twice a week for a period of three months. This Ox-LDL uptake was Ang-II dose-dependent. The cellular uptake of acetylated-LDL (Ac-LDL), another ligand for scavenger receptors, however, was not affected by Ang-II injection to the mice. Furthermore, preincubation of the MPM with the monoclonal antibody, anti CD36, reduced macrophage uptake of Ox-LDL in Ang-II-treated mice by only 11%. Ang-II administration to mice resulted in a 60% increase in the macrophage cellular proteoglycan content. Chondroitinase treatment of MPM decreased Ox-LDL cellular uptake by 20% and by 38% in placebo-treated and Ang-II-treated cells, respectively. We thus conclude that Ang-II administration to mice enhances their macrophage Ox-LDL uptake via its stimulating effect on cellular proteoglycan content and this process can lead to foam cell formation and atherosclerosis.
Angiotensin II↗
The angiotensin-II receptor antagonist, losartan, inhibits LDL lipid peroxidation and atherosclerosis in apolipoprotein E-deficient mice.
The potential antiatherogenic actions of the angiotensin II receptor antagonist, losartan were investigated in apolipoprotein (apo) E deficient mice, an animal model with severe hypercholesterolemia and extensive atherosclerosis. In these animals accelerated atherosclerosis is associated with increased lipid peroxidation which may play a crucial role in the build up of the atherosclerotic lesions. Administration of losartan (25mg/kg/d) to the apo E deficient mice for a 3-month period increased the plasma renin activity 3.5-fold compared to the placebo group. Losartan increased the resistance of LDL to CuSO4-induced oxidative modification as shown by a significant reduction in the LDL content of malondialdehyde by 55% compared to placebo, as well as by the prolongation of the lag time required for LDL oxidation, from 60 min in the placebo-treated mice to more than 140 min in the losartan-treated mice. Losartan reduced significantly the mean atherosclerotic lesion area by 80% compared to the placebo group. We conclude that losartan inhibits LDL lipid peroxidation in the apo E deficient mice and this effect may have an important role in the attenuation of the accelerated atherosclerosis.
Angiotensin Receptor Antagonists↗