bluuebunny/crossref_metadata_2025 huggingface dataset:
Coexisting Muscular Dystrophies and Epilepsy in Children
Authors: Tsao Chang-Yong , Mendel J.R.
Published: June 2007

Muscular dystrophies are composed of a variety of genetic muscle disorders linked to different chromosomes and loci and associated with different gene mutations that lead to progressive muscle atrophy and weakness. Fukuyama congenital muscular dystrophy is frequently associated with partial and generalized epilepsy and congenital brain anomalies, including cobblestone complex and other neuronal migration defects. We report generalized convulsive epilepsy in a boy with normal brain magnetic resonance imaging and Duchenne muscular dystrophy with deletion of dystrophin gene, and we report absence epilepsy with normal brain magnetic resonance imaging in another boy with limb girdle muscular dystrophy with partial calpain deficiency. We, therefore, review coexisting musclar dystrophies and epilepsy in children. In addition to Fukuyama congenital muscular dystrophy, partial or generalized epilepsy has also been reported in the following types of muscular dystrophies, including Duchenne/Becker dystrophy, facioscapulohumeral dystrophy, congenital muscular dystrophy with partial and complete deficiency of laminin α 2 (merosin) chain, and limb girdle muscular dystrophy with partial calpain deficiency. ( J Child Neurol 2006;21:148—150; DOI 10.2310/7010.2006.00035).
Nonprogressive Familial Leukoencephalopathy With Porencephalic Cyst and Focal Seizures
Authors: Blumkin Lubov , Watemberg Nathan , Lev Dorit , Malinger Gustavo , Luckman Yehudit , Ben-Zeev Bruria , Lerman-Sagie Tally
Published: June 2007

Two siblings with a similar white-matter disease but different clinical symptoms are described. The first sibling suffers from nonprogressive spastic hemiparesis secondary to a congenital periventricular porencephalic cyst. Her brother has focal epilepsy. On magnetic resonance imaging, both patients show diffuse white-matter involvement predominantly of the posterior periventricular area. We suggest that this is a familial white-matter disorder with minimal symptoms and no progression in early childhood. ( J Child Neurol 2006;21:145—148; DOI 10.2310/7010.2006.00036).
Characteristic Brain Magnetic Resonance Imaging (MRI) Findings in Neonates With Tuberous Sclerosis Complex
Authors: Arca Gemma , Pacheco Esperanza , Alfonso Israel , Duchowny Michael S. , Melnick Steven J.
Published: June 2007

We describe the brain magnetic resonance imaging (MRI) findings in eight neonates with tuberous sclerosis complex to further delineate the spectrum of characteristic findings in these patients. In addition to the previously described characteristic brain MRI findings in neonates, which included cortical tuber, transmantle dysplasia, subependymal nodules, cerebral subependymal giant cell astrocytomas, white-matter anomalies, and hemimegalencephaly, we found one neonate with cerebellar subependymal giant cell astrocytomas and one patient who had a normal MRI and computed tomographic scan of the brain at 4 days of age but subsequently was found to have cortical tubers and subependymal nodules by MRI. ( J Child Neurol 2006;21:280—285; DOI 10.2310/7010.2006.00103).
Arm Trajectories in Dyskinetic Cerebral Palsy Have Increased Random Variability
Authors: Sanger Terence D.
Published: June 2007

Dyskinetic cerebral palsy results from injury to the basal ganglia early in life. Symptoms can include hyperkinetic or dystonic arm movements that impair function. It is not known whether these movements comprise a small number of specific abnormal motor patterns or whether they are random and variable. We hypothesize that injury to the basal ganglia leads to impaired filtering and removal of undesired neural signals and that lack of appropriate removal of noisy or irrelevant neural signals leads to random and variable arm movements. To test this hypothesis, we quantified the variability in arm trajectories while seven children with dyskinetic cerebral palsy between the ages of 4 and 13 years old made repeated outward reaching movements. We compared the results with those of 21 healthy children between the ages of 5 and 16 years. The best-fit trajectory to the set of reaching movements for each child was taken as the predictable component of movement. We calculated the ratio of the power in the best-fit trajectory to the total variance. This measure is the signal-to-noise ratio, and it quantifies the extent to which trajectories are predictable. We found that children with dyskinetic cerebral palsy had a significantly reduced signal-to-noise ratio compared with healthy children at similar ages. This result shows that there is increased movement variability, and it is consistent with the hypothesis that inadequate removal of noisy signals could be a cause of the movement disorder in dyskinetic cerebral palsy. (J Child Neurol 2006;21:551–557; DOI 10.2310/7010.2006.00113).
Migration Abnormality in the Left Cingulate Gyrus Presenting With Autistic Disorder
Authors: Korkmaz Bariş , Benbir Gülçin , Demirbilek Veysi
Published: June 2007

Autism, characterized by an impairment in communication, including language, narrowly focused interests, and poor sociability, is a neurodevelopmental disorder of still largely unknown pathogenesis. In children with autistic symptomatology, the most consistent functional or anatomic abnormalities are found in the cingulate gyrus, particularly in the anterior regions. Neuronal migration malformations caused by incomplete neuronal migration and characterized by loss of the normal gyral patterns in the cerebral hemispheres and prominent disorganization of the cerebral cortical cytoarchitecture are generally associated with profound neurologic deficits, epilepsy, and autism. In this report, we present a case with an isolated migration abnormality located in the anterior part of the left cingulate gyrus who was admitted with the complaints of epileptic seizures and autism. In addition, the role of the localization of the migration abnormality in the appearance of autistic symptomatology is discussed. (J Child Neurol 2006;21:600—604; DOI 10.2310/7010.2006.00141).
99mTc-HMPAO Brain Perfusion Single-Photon Emission Computed Tomography in Children with Down Syndrome: Relationship to Epilepsy, Thyroid Functions, and Congenital Heart Disease
Authors: Altiay Suriye , Kaya Meryem , Karasalihoglu Serap , Gultekin Aziz , Biner Betul
Published: June 2007

In recent years, it has been possible for patients with Down syndrome to live longer with advanced medical treatment and social support. As a result, the problems of these patients, such as thyroid diseases, leukemia, and Alzheimer disease, would be encountered more frequently. In this study, we aimed to perform the brain perfusion of children with Down syndrome by technetium 99m hexamethylpropylene amine oxime (99mTc-HMPAO) single-photon emission computed tomography (SPECT) and to determine the relationship between brain perfusion and epilepsy, thyroid function tests, congenital heart disease, and level of mental and motor development. Thirty patients with Down syndrome, aged between 1 and 15 years, were included in our study. Demographic data, the existence of epilepsy and congenital heart defects, the level of mental and motor development, serum levels of thyroid hormones, and autoantibodies were determined. All patients underwent computed tomography (CT) and/or magnetic resonance imaging (MRI). Cerebral SPECT was performed in all cases to evaluate the brain perfusion pattern. According to the visual evaluation of cerebral SPECT results, hypoperfusion was detected in 11 cases (37%). Patients with cerebral hypoperfusion (group 1) and patients with normal cerebral perfusion (group 2) were compared. There was no difference between group 1 and group 2 in terms of demographic data, congenital heart defects, IQ levels, thyroid hormones, and autoantibodies, but the incidence of epilepsy was significantly higher in group 1 (P < .001). When motor and mental development levels were compared, it was found that cases in group 1 were significantly more retarded in personal-social and fine motor skills (P < .05). The present study showed that cerebral hypoperfusion in children with Down syndrome is mostly related to epilepsy and the other coexisting conditions, congenital heart disease and hypothyroidism. Patients with cerebral hypoperfusion also have more retarded developmental levels, especially in personal-social and fine motor skills. (J Child Neurol 2006;21:610—614; DOI 10.2310/7010.2006.00144).
Methadone Intoxication in a Child: Toxic Encephalopathy?
Authors: Anselmo Marisol , Rainho António Campos , do Carmo Vale Maria , Estrada João , Valente Rosalina , Correia Manuela , Vieira José Pedro , Barata Deolinda
Published: June 2007

Methadone is used in the treatment of opioid addiction. Acute intoxication can lead to severe consequences and can even be lethal. In several case reports and small series, a presumably toxic leukoencephalopathy is described resulting from inhalation of heroin. We present the case of a 3-year-old boy who ingested methadone accidentally. In a coma with acute obstructive hydrocephalus owing to massive cerebellar edema and supratentorial lesions, he was successfully treated with methylprednisolone and cerebrospinal fluid external drainage. To our knowledge, this is the first report of an encephalopathy associated with synthetic opioid intoxication. (J Child Neurol 2006;21:618—620; DOI 10.2310/7010.2006.00146).
Brief Review of Habit Reversal Training for Tourette Syndrome
Authors: Himle Michael B. , Woods Douglas W. , Piacentini John C. , Walkup John T.
Published: June 2007

It is well established that Tourette syndrome has a neurobiologic origin. Although pharmacotherapy is the most commonly prescribed intervention, there is considerable evidence to support the use of behavior therapy, specifically habit reversal training, as an alternative or adjunct treatment for some individuals with Tourette syndrome. Unfortunately, many professionals are unfamiliar with habit reversal training. The purpose of this review is to provide readers with a brief review of empiric studies on habit reversal training, update readers on the current state and future of behavior therapy for Tourette syndrome, and provide resources for those readers interested in additional information. (J Child Neurol 2006;21:719—725; DOI 10.2310/7010.2006.00158).
Attention-Deficit Hyperactivity Disorder (ADHD) Comorbidity: A Case for ``Pure'' Tourette Syndrome?
Authors: Denckla Martha Bridge
Published: June 2007

More than a decade of research regarding the motoric characteristics of the attention-deficit hyperactivity disorder (ADHD) that accompanies Tourette syndrome has revealed unique anatomic and neurobehavioral differences and highlighted the importance of distinguishing children with this form from the 40% of children with Tourette syndrome who do not have ADHD. This distinction is important in providing guidance to parents and to patients and in formulating expectations for short- and long-term prognoses. In addition, study methodologies that fail to categorize patients in this way and instead involve covarying for dimensional symptoms of ADHD obscure biologically distinctive circuits and clinically meaningful patient characteristics. (J Child Neurol 2006;21:701—703; DOI 10.2310/7010.2006.00164).
Genetics and Epidemiology of Tourette Syndrome
Authors: Keen-Kim Dianne , Freimer Nelson B.
Published: June 2007

Although the genetic basis of Tourette syndrome is well established, uncertainty about how best to define and assess the Tourette syndrome phenotype has hampered efforts to identify the genes responsible for susceptibility to the disorder. In addition, such efforts have typically been underpowered or were undertaken before the technology was available to perform systematic genome-wide genetic investigations. The Tourette Syndrome Association International Consortium on Genetics was formed by more than a dozen research groups from around the world to develop common approaches to phenotyping Tourette syndrome and to pool samples for uniform, well-powered genetic investigations. Several recent advances, including the completion of genome-wide scans of affected sib-pairs and large families, show real promise for identifying Tourette syndrome susceptibility genes. In this review, we describe the key epidemiologic, linkage, and association studies in Tourette syndrome and illustrate the strategies currently being used to identify Tourette syndrome genes. (J Child Neurol 2006;21:665—671; DOI 10.2310/7010.2006.00168).
Bispectral Index Monitoring in Confirmation of Brain Death in Children
Authors: Okuyaz Çetin , Birbiçer Handan , Doruk Nurcan , Atici Aytuğ
Published: June 2007

Although the guidelines for the diagnosis of brain death in children are well established, the diagnosis is still under debate, and further confirmatory tests are required. Performing these confirmatory tests presents some drawbacks, such as high costs, the need for specialized personnel and technology, transportation of patients out of the intensive care unit, and the use of contrast media. Bispectral index monitoring can provide real-time, objective, continuous monitoring of the consciousness level in critically ill children. The aim of this prospective study was to define the role of bispectral index monitoring in the confirmation and diagnosis of brain death. Eight children who had fulfilled the diagnostic criteria of brain death were included in the study. The age of patients ranged from 3 months to 15 years. All patients had electrocerebral silence on their electroencephalographic recordings. After the diagnosis of brain death, at least 2-hour monitoring was performed, and all patients expressed a score of 0, indicating brain death. According to our study, the decrease in bispectral index score to 0 in patients with suspected brain death can support and confirm brain death diagnosis in children and can enable scheduling of expensive tests, such as cerebral angiography, in the appropriate time. Nevertheless, further studies are needed to determine the role of the bispectral index in the diagnosis and confirmation of brain death in children. In this article, we review clinical utility, application time, and interpretation of bispectral index monitoring in confirmation of brain death diagnosis in children. (J Child Neurol 2006;21:799—801; DOI 10.2310/7010.2006.00131).
Proton Magnetic Resonance Spectroscopy and Diffusion-Weighted Imaging in Isolated Sulfite Oxidase Deficiency
Authors: Eichler Florian , Tan Wen-Hann , Shih Vivian E. , Grant P. Ellen , Krishnamoorthy Kalpathy
Published: June 2007

Isolated sulfite oxidase deficiency is a rare autosomal recessive disorder of the newborn that can be mistaken for neonatal asphyxia. Diffusion-weighted imaging of the brain demonstrates widespread diffusion restriction, and proton magnetic resonance spectroscopy shows an elevated lactate level, a decrease in the ratio of N-acetylaspartate to creatine, and a rise in the ratio of choline to creatine. This precedes severe cystic encephalomalacia and suggests that the energy failure associated with neuronal dysfunction and myelin disintegration occurs early in isolated sulfite oxidase deficiency. (J Child Neurol 2006;21:801—805; DOI 10.2310/7010.2006.00174).
Recurrent Absence Status Epilepticus (Spike-and-Wave Stupor) Associated With Lamotrigine Therapy
Authors: Hasan Mariana , Lerman-Sagie Tally , Lev Dorit , Watemberg Nathan
Published: June 2007

We report on the aggravation of absence seizures by lamotrigine leading to absence status epilepticus in a child. The patient is a 10-year-old girl with a history of absence seizures, learning disabilities, and hereditary neuropathy with liability to pressure palsies. Lamotrigine (up to 12 mg/kg/day) was prescribed after a brief course of valproate was associated with restlessness. Long-acting methylphenidate was also administered. The initial response to lamotrigine appeared to be excellent. The first episode of absence status epilepticus occurred during a febrile illness while lamotrigine was being gradually discontinued. Following this event, lamotrigine dose was increased to 10 mg/ kg/day and methylphenidate was continued. Six weeks later, a second absence status epilepticus episode ensued without fever. Sulthiame and clonazepam were substituted for lamotrigine, whereas methylphenidate therapy was continued. A psychiatrist prescribed risperidone 1 month later owing to obsessive-compulsive behavior. Nine months later, she remained free of absence seizures. Whereas the first absence status epilepticus event could have been triggered by fever, the second episode occurred while the daily lamotrigine dose was being increased. Moreover, the patient is seizure free following lamotrigine discontinuation. Hence, it is quite possible that lamotrigine caused seizure aggravation and absence status epilepticus in this child. (J Child Neurol 2006;21:807-809; DOI 10.2310/ 7010.2006.00176).
Sleep Disturbance in Children With Migraine
Authors: Heng Kenneth , Wirrell Elaine
Published: June 2007

The objectives of this study were to determine if (1) children with migraine experience greater sleep disturbances than their siblings, (2) those with more severe migraine have greater levels of sleep disruption, and (3) these sleep disturbances lead to greater behavioral problems and more missed school. Children aged 6 to 18 years with a diagnosis of migraine for > 6 months, who had at least one sibling without migraine in the same age range, were identified through our neurology clinic database or at the time of the clinic visit. Parents completed the (1) demographic, general health, and migraine information questionnaire; (2) Child Sleep Habits Questionnaire; and (3) Behavior Assessment System for Children: Second Edition (BASC-2) Parent Rating Scales for each child. Cases with migraine had higher total sleep (P < .02), sleep delay (P < .03), and daytime sleepiness scores (P < .001) than controls. Cases with more severe migraines had higher total sleep (P < .01) and sleep duration scores (P < .03) than those with milder headaches. In cases, higher total sleep scores predicted greater behavior problems on all four composite scales on the Behavior Assessment System for Children: Second Edition (Externalizing Problems, P < .05; Internalizing Problems, P < .005; Behavior Systems Index, P < .003; and Adaptability Skills, P < .006). We conclude that children with migraine are prone to greater sleep and behavioral disturbances than children without headache. Sleep disorders should be routinely queried and appropriate advice on sleep hygiene provided. (J Child Neurol 2006;21:761—766; DOI 10.2310/7010.2006.00191).
Acute Disseminated Encephalomyelitis in North Indian Children: Clinical Profile and Follow-Up
Authors: Singhi Pratibha D. , Ray Munni , Singhi Sunit , Narendra Kumar Khandelwal
Published: June 2007

Acute disseminated encephalomyelitis in children is not uncommon in developing countries, yet there is little systematic documentation of its clinical profile and follow-up. We studied the clinical and neuroradiologic features of acute disseminated encephalomyelitis in 52 consecutive children. Clinical details, magnetic resonance imaging (MRI) findings, and the results of other investigations were recorded, and children were followed up from 6 to 48 months. A repeat MRI was done after 3 to 4 months, and in those with persistent lesions, another MRI was done after 6 to 7 months of discharge. The mean age at presentation was 6.14 ± 3.17 years, 73.1% were male, and 17 children had a history of antecedent infectious illness or vaccination. Most children had a meningoencephalitic presentation, with sudden-onset motor weakness in 76.9% and seizures in 36.5%. Altered sensorium and pyramidal signs were seen in 55.8% and 80.7% of children, respectively. On MRI, scattered T2-weighted hyperintense lesions were seen, mainly in the subcortical white matter, especially in the parietal (53.8%) and frontal (30.17%) regions. Thalamic, basal ganglia, and callosal lesions were seen in 30.76%, 17.3%, and 13.46% of cases, respectively. Variable contrast enhancement was seen in 48% of those who had contrast MRIs. The response to methylprednisolone was good, with dramatic recovery in 26.9% and marked improvement in 51.9% at discharge. On follow-up, of 44 children, residual smaller MRI lesions were seen in 30. The MRI was repeated at 6 months in children with residual lesions, and it was found that the lesions either disappeared or were significantly reduced after 6 months in 75% of cases. Four children had relapse of acute disseminated encephalomyelitis with new lesions on MRI. All of them responded to methylprednisolone. None of the clinical or neuroradiologic factors at presentation had any significant correlation with relapse. Six months after discharge, no deficits could be found in 61.3% of cases; 15.9% and 4.5% had motor and cognitive deficits, and 9% had multiple deficits. The presentation of pediatric acute disseminated encephalomyelitis in developing countries is similar to that in developed countries. In spite of an aggressive presentation, most children respond well to corticosteroids. MRI lesions disappear or are significantly reduced at 6 months in the majority of cases. (J Child Neurol 2006;21:851—857; DOI 10.2310/7010.2006.00187).
Magnetic Resonance Imaging Study of the Orbitofrontal Cortex in Autism
Authors: Hardan Antonio Y. , Girgis Ragy R. , Lacerda Acioly L.T. , Yorbik Ozgur , Kilpatrick Megan , Keshavan Matcheri S. , Minshew Nancy J.
Published: June 2007

The orbitofrontal cortex is involved in multiple psychologic functions, such as emotional and cognitive processing, learning, and social behavior. These functions are variably impaired in individuals with autism. The present study examined the size of the orbitofrontal cortex, and its medial and lateral subdivisions, using magnetic resonance imaging (MRI) scans obtained from 40 non—mentally retarded individuals with autism and 41 healthy controls. No differences were observed between the two groups on any of the orbitofrontal cortex measurements. However, when compared with controls, a smaller right lateral orbitofrontal cortex was observed in children and adolescents with autism, whereas a larger right lateral orbitofrontal cortex was found in adult patients. Interestingly, a positive relationship was found in the patient group between circumscribed interests and all orbitofrontal cortex structures. The present study suggests the absence of global volumetric abnormalities in the orbitofrontal cortex in autism and indicates that the functional disturbances in this structure might not be related to anatomic alterations. (J Child Neurol 2006;21:866—871; DOI 10.2310/ 7010.2006.00199).
Giant Axonal Neuropathy: Diffusion-Weighted Imaging Features of the Brain
Authors: Alkan Alpay , Sigirci Ahmet , Kutlu Ramazan , Doganay Selim , Erdem Gulnur , Yakinci Cengiz
Published: June 2007

Giant axonal neuropathy is a rare autosomal recessive childhood disorder characterized by a peripheral neuropathy and features of central nervous system involvement. Magnetic resonance imaging (MRI) of an 11-year-old boy with giant axonal neuropathy revealed high signal intensity in the white matter of the cerebrum and cerebellum on T2-weighted imaging. An apparent diffusion coefficient map revealed increased apparent diffusion coefficient values in the periventricular, deep, and cerebellar white matter, basal ganglia, and thalamus. Increased apparent diffusion coefficient values in distinct locations suggest increased mobility of water molecules in the brain of a patient with giant axonal neuropathy. This finding could indicate a myelin disorder such as demyelination. Diffusion-weighted imaging should be performed to reveal apparent diffusion coefficient changes and determine brain involvement in patients with giant axonal neuropathy. (J Child Neurol 2006;21:912—915; DOI 10.2310/7010.2006.00211).
Cockayne Syndrome in Adults: Review With Clinical and Pathologic Study of a New Case
Authors: Rapin Isabelle , Weidenheim Karen , Lindenbaum Yelena , Rosenbaum Pearl , Merchant Saumil N. , Krishna Sindu , Dickson Dennis W.
Published: June 2007

Cockayne syndrome and xeroderma pigmentosum—Cockayne syndrome complex are rare autosomal recessive disorders with poorly understood biology. They are characterized by profound postnatal brain and somatic growth failure and by degeneration of multiple tissues resulting in cachexia, dementia, and premature aging. They result in premature death, usually in childhood, exceptionally in adults. This study compares the clinical course and pathology of a man with Cockayne syndrome group A who died at age 31½ years with 15 adequately documented other adults with Cockayne syndrome and 5 with xeroderma pigmentosum—Cockayne syndrome complex. Slowing of head and somatic growth was apparent before age 2 years, mental retardation and slowly progressive spasticity at 4 years, ataxia and hearing loss at 9 years, visual impairment at 14 years, typical Cockayne facies at 17 years, and cachexia and dementia in his twenties, with a retained outgoing personality. He experienced several transient right and left hemipareses and two episodes of status epilepticus following falls. Neuropathology disclosed profound microencephaly, bilateral old subdural hematomas, white-matter atrophy, tigroid leukodystrophy with string vessels, oligodendrocyte proliferation, bizarre reactive astrocytes, multifocal dystrophic calcification that was most marked in the basal ganglia, advanced atherosclerosis, mixed demyelinating and axonal neuropathy, and neurogenic muscular atrophy. Cellular degeneration of the organ of Corti, spiral and vestibular ganglia, and all chambers of the eye was severe. Rarely, and for unexplained reasons, in some patients with Cockayne syndrome the course is slower than usual, resulting in survival into adulthood. The profound dwarfing, failure of brain growth, cachexia, selectivity of tissue degeneration, and poor correlation between genotypes and phenotypes are not understood. Deficient repair of DNA can increase vulnerability to oxidative stress and play a role in the premature aging, but why patients with mutations in xeroderma pigmentosum genes present with the Cockayne syndrome phenotype is still not known. (J Child Neurol 2006;21:991—1006; DOI 10.2310/7010.2006.00088).
Behavioral and Emotional Problems in Children With Epilepsy
Authors: Freilinger Michael , Reisel Barbara , Reiter Edith , Zelenko Marina , Hauser Erwin , Seidl Rainer
Published: June 2007

The principal purpose of this study was to assess behavioral and emotional problems in children with epilepsy to investigate if specific behavioral and emotional problems are associated with specific medical epilepsy-related factors. The Child Behavior Checklist (CBCL) was used to assess parent-reported behavioral and emotional problems in 108 5- to 18-year-old children with various epilepsy syndromes. Specific medical epilepsy-related factors, such as etiology, age at onset, seizure symptoms, prognosis, seizure frequency, electroencephalography (EEG), and anticonvulsive therapy, were recorded during a regular follow-up examination in our pediatric outpatient epilepsy clinic, and 22.2% of our patients showed moderate to severe behavioral or emotional problems as measured by the Child Behavior Checklist total score. Higher Child Behavior Checklist scores were associated with such specific medical epilepsy-related factors as etiology, age at onset, and polypharmacy. Higher scores on the Social Problem scale were associated with symptomatic epilepsy syndromes and an earlier age at onset. Higher scores on the Social Problems, Attention Problems, and Aggressive Behavior scales were associated with anticonvulsive polytherapy. There were no statistically significant associations between the Child Behavior Checklist scores and seizure symptoms and frequency and EEG at the time of evaluation. The demonstrated frequency of behavioral and emotional problems in children with epilepsy suggests the necessity to address psychosocial issues during the course of clinical treatment. (J Child Neurol 2006;21:939—945; DOI 10.2310/ 7010.2006.00205).
Use of Combined Higb-Frequency Oscillation and Intermittent Mandatory Ventilation in Rabbits with Saline-Lavaged Lungs
Authors: Blanco Carlos E. , Maertzdorf Wiel J. , Walther Frans J.
Published: June 2007

We combined high-frequency oscillation ventilation (HFOV) with intermittent mandatory ventilation (IMV) in rabbits with surfactant-depleted lungs by saline la vage. Thirteen rabbits were initially ventilated with con stant-flow, time-cycled, pressure-limited ventilators and 100% oxygen at a peak inspiratory pressure to keep arterial carbon dioxide tension (PaCOz) below 50 mm Hg. Group 1 (n = 6) received only IMV for 3 hours and Group 2 ( n = 7), combined HFOV/IMV for 3 hours. HFOV was delivered at 15 Hz and a tidal volume of 2 ml/ kg. Arterial blood gases were measured every 15 min utes. In Group 1, arterial oxygen tension (PaO 2) de creased from 71 ± 5 to 60 ± 7 mm Hg (mean ± standard error of the mean), PaCO2 increased from 34 ± 2 to 50 ± 9 mm Hg, and the ventilatory index (PaO 2/mean airway pressure x fraction of oxygen in in spired air) decreased from 5.9 ± 0.8 to 4.3 ± 0.6 mm Hg/cm H2O over the 3-hour period (nonsignificant). In Group 2, PaO2 increased from 61 ± 6 to 141 ± 12 mm Hg (p < .01), PaCO 2 decreased from 35 ± 2 to 25 ± 7 mm Hg (nonsignificant), and the ventilatory index in creased during HFOV/IMV from 5.1 ± 0.6 to 12.7 ± 2.2 mm Hg/cm H2O (p < .001) at a lower mean airway pressure than Group 1 over 3 hours. This indicates that the combination of HFOV and IMV is advantageous in improving pulmonary gas exchange in rabbits' saline- lavaged lungs.
Stress Ulcer Syndrome
Authors: Zuckerman Gary R. , Cort David , Shuman Robert B.
Published: June 2007

Stress ulcer syndrome refers to gastroduodenal erosions or ulcers that develop acutely in relation to major physi ological stress, usually manifested clinically as upper gastrointestinal (UGI) bleeding. These lesions occur most often in the gastric fundus. Endoscopy has shown gastroduodenal mucosal lesions in 75 to 100% of inten sive care unit (ICU) patients within 72 hours of admis sion. Patients at high risk for stress ulcer include those with large body surface area burns, intracranial lesions associated with coma, fulminant hepatic failure, sepsis, and trauma and abdominal, cardiovascular, and thoracic surgery patients. Also considered high risk are ICU pa tients with superimposed complications such as shock, mechanical ventilation for more than 3 days, coagulopa thy, jaundice, and sepsis. Approximately 15% of ICU pa tients will experience UGI bleeding from stress ulcer. Patients bleeding from stress ulcer have an overall mor tality rate approaching 65% compared with 9 to 22% mortality in patients without stress ulcer. When strati fied according to occult blood loss versus clinically significant bleeding, mortality can be as high as 90% in patients overtly bleeding; 30% of deaths are directly related to bleeding. Both antacids and H2 receptor an tagonists are effective in prophylaxis for stress ulcer bleeding.
Pain Relief in the Intensive Care Unit
Authors: Bouckoms A.J.
Published: June 2007

Treatment of severe pain in the intensive care unit first requires an assessment of pain type: nociceptive, cen tral, visceral, or referred. Narcotics given parenterally are the most commonly used agents for severe nocicep tive pain. Attention to potency, lipophilicity, route of administration, and side effects are all important. Cen tral nervous system pain may require nonnarcotic adju vants, anticonvulsants, or monoamine altering drugs for effective analgesia. The concomitance of emotional suf fering with the pain is an important problem to recog nize so that psychiatric disorders are properly treated.
Weaning from Mechanical Ventilation
Authors: Morganroth Melvin L. , Grum Cyril M.
Published: June 2007

For most patients who require mechanical ventilation weaning and extubation is simple. In these patients a variety of strategies can be successful. In addition, sim ple criteria may predict when the patient is ready for extubation. For the small group of patients who require prolonged mechanical ventilation, however, contro versy exists about how best to remove ventilator sup port by weaning, and available data are sparse. Much of the controversy has centered on T-piece weaning ver sus intermittent mandatory ventilation. To date no con trolled study has demonstrated the superiority of either intermittent mandatory ventilation or T-piece weaning in difficult-to-wean patients. In the evolution of this con troversy, concern has developed over the potential for increased inspiratory work and expiratory resistance that may be associated with certain intermittent manda tory ventilation systems. The possibility that significant inspiratory work may occur during assist-control venti lation has also been demonstrated. Respiratory muscle weakness and fatigue is likely important in failure to wean. Other possible causes are failure of the cardiovas cular system and impaired ability of the lung to carry out gas exchange. In this article we first examine criteria and techniques for weaning short-term ventilator pa tients. We then examine criteria to begin the weaning process in prolonged ventilation patients, potential causes of failure to wean, and techniques that can be used to remove ventilator support from patients who are difficult to wean. Much literature has been devoted to techniques and criteria for weaning and extubation of patients from mechanical ventilation. For most patients who require ventilatory support, weaning and extuba tion can be easily accomplished by a variety of tech niques [1-4]. At one referral center 77.2% of all surviving patients were weaned from the ventilator within 72 hours of the onset of mechanical ventila tion, and 91% were weaned within 7 days [1]. Less than 10% of ventilated patients potentially posed problems in weaning from mechanical ventilation. Similarly, at a community hospital, few surviving patients required prolonged ventilatory support [2]. In easy-to-wean patients, Sahn and Lakshminarayan [5] described simple criteria that are predictive of successful discontinuation of ventilator support. For the small group of patients who require pro longed mechanical ventilation, however, minimal data are available. In these patients criteria to deter mine weaning ability or which measurements to follow are not clearly defined. Furthermore, no controlled trials are available to compare the differ ent weaning techniques proposed. In this article we first address routine weaning of the patient who has not required prolonged ventilator support. We then examine the difficult-to-wean patient and dis cuss criteria to begin the weaning process, poten tial causes of failure to wean, and available weaning techniques.
Massive Hemoptysis: Pathogenesis and Management
Authors: Winter Stephen M. , Ingbar David H.
Published: June 2007

Massive hemoptysis is a life-threatening complication of many pulmonary disorders, and it occurs most com monly in association with longstanding inactive tuber culosis, bronchiectasis, lung abscess, bronchogenic car cinoma, and fungal disease. It is generally a neovascular change or local erosive effect of chronic pulmonary dis ease and may originate from either bronchial or pulmo nary circulation. Recurrent bleeding is unpredictable; therefore, diagnostic and therapeutic intervention must be undertaken with urgency. The immediate priorities must be protection of the airway to the nonbleeding lung and localization of the site of hemorrhage prefera bly by bronchoscopy, which has a high yield when per formed during active hemorrhage. Immediate control of bleeding may be obtained by local tamponade with a balloon-tipped Fogarty catheter, use of a double-lumen endotracheal tube, or angiographically guided emboliza tion. Surgical resection is the preferred definitive treat ment for those who meet operative criteria; those who lack adequate pulmonary reserve are candidates for em bolization of sites with persistent bleeding. The high mortality of conservatively treated massive hemoptysis and the current inability to predict which patients will have fatal hemorrhage mandate rapid assessment and intervention.
Analytic Reviews : Nonsurgical Alternatives in the Management of Gallstones
Authors: Albert Michael B. , Fromm Hans
Published: June 2007

Until recently, the only therapeutic option for patients with symptomatic gallstones was surgery. However, sev eral new and innovative nonsurgical approaches are cur rently available, including oral dissolution therapy with the bile salts, ursodeoxycholic and chenodeoxycholic acids, instillation of liquid solvents such as methyl tert- butyl ether directly into the gallbladder or the common bile duct, and extracorporeal shock-wave lithotripsy. We review the role of each of these methods in the management of patients with gallstones as well as the epidemiology, pathogenesis, natural history, and radi ological characteristics of gallstones, all important con siderations when choosing appropriate treatment for the individual patient.
Adrenal Insufficiency in the Intensive Care Setting
Authors: Knowlton Abbie I.
Published: June 2007

In the intensive care setting, the question of adrenal insufficiency arises most frequently in patients whose endogenous adrenal function may be suppressed by pre ceding glucocorticoid therapy or in patients with the acquired immunodeficiency syndrome. Less often the question arises in patients whose adrenal tissue may have been destroyed by autoimmune disease, tuber culosis, meningococcal or other infection, hemorrhage, or replacement by malignancy, or in patients whose adrenal function has been suppressed by certain drugs. Measurements of plasma adrenocorticotrophic hor mone (ACTH), cortisol, aldosterone, and renin levels and the response of cortisol and aldosterone to exoge nously administered ACTH form the basis for clinically evaluating the adequacy of a patient's adrenal function. The naturally occurring glucocorticoid, hydrocortisone, remains the cornerstone of adrenal replacement therapy along with appropriate fluid and electrolyte administra tion. In rare instances the addition of a mineralocor ticoid is necessary. A brief review of the use of mega- dose glucocorticoids in the treatment of sepsis, shock, and the adult respiratory distress syndrome is included.
The Clinical Course of Patients with Atrial Fibrillation and Flutter Admitted to Medical Intensive Care Units
Authors: Herrmann Howard C. , Thibault George E.
Published: June 2007

To better understand the clinical course of patients with atrial fibrillation and flutter who are admitted to medi cal intensive care units (ICUs), we retrospectively re viewed the course of patients with either of these rhythms present on admission. Patients with atrial fibril lation and flutter accounted for 10% of the admissions to a combined intensive and coronary care unit over a 5- year period. These patients had an ICU mortality rate of 9.4%; the ICU deaths occurred exclusively in a subgroup of patients (60%) whose arrhythmia was associated with other serious illnesses. The subgroup of patients presenting with atrial fibrillation and flutter only had a benign course; this subgroup included patients in whom alcohol intake contributed to the rhythm disturbance. Complications occurred in 40% of patients and were predicted with multivariate analysis by (1) the presence of alveolar edema on the initial chest roentgenogram; (2) female sex; (3) diastolic blood pressure less than or equal to 95; (4) chest pain lasting more than 2 hours; and (5) ischemia on the presenting electrocardiogram. Prospective application of these criteria may be helpful in deciding which patients could be initially triaged to non-ICU areas, thereby resulting in more efficient ICU use.
Low Incidence of Extrapyramidal Symptoms in Treatment of Delirium with Intravenous Haloperidol and Lorazepam in the Intensive Care Unit
Authors: Sanders Kathy M. , Minnema Ann Mary , Murray George B.
Published: June 2007

We retrospectively reviewed 11 patients in an intensive care unit who were treated for delirium with intrave nously administered haloperidol and lorazepam. The cu mulative doses for each episode of delirium ranged from 25.5 to 1,929 mg of haloperidol and 19.5 to 2,274 mg of lorazepam. Neuroleptic-induced rigidity developed in 1 patient. After administration of the neuroleptic was dis continued, there were no residual extrapyramidal symp toms.
Diagnosis and Management of Hyponatremia
Authors: Black Robert M.
Published: June 2007

Appropriate treatment of hyponatremic disorders is de pendent on an understanding of the mechanisms that cause these abnormalities. This article offers a patho physiological approach to hypoosmolar syndromes. Common causes of hyponatremia are reviewed with particular emphasis on congestive heart failure, ad vanced liver disease, diuretic use, and the syndrome of inappropriate antidiuretic hormone secretion. New con cepts in treatment are discussed with the aid of clinical examples that emphasize critical information. A number of recent studies have questioned the safety of rapidly correcting hyponatremia; recommendations based on our current understanding of these risks are proposed. Pitfalls in the diagnosis and management of patients with hyponatremic disorders are also discussed.
Flexible Fiberoptic Bronchoscopy in the Intensive Care Unit
Authors: Brandstetter Robert D.
Published: June 2007

For the past 20 years, flexible fiberoptic bronchoscopy (FOB) has been shown to be an important procedure in the diagnosis and management of patients in intensive care units (ICU). In adults, FOB is used therapeutically to remove retained secretions and to correct atelectasis not improved by conservative means. In the pediatric population, however, FOB is mainly used to diagnose tracheal disease in critically ill children. The principal risks of FOB are hypoxemia and dysrhythmias; hemor rhage and pneumothorax may occur as a result of biopsy procedures. In competent hands, these adverse compli cations of FOB are minimal. Although rigid bronchos copy remains pivotal in most pediatric bronchoscopic procedures, massive hemoptysis, foreign body removal, and laser therapy for occluding tumors of the upper airway, flexible FOB has an increasingly important role in the diagnosis and management of these disorders.
Anatomical Considerations in Obtaining Arterial Access
Authors: Mathers Lawrence H.
Published: June 2007

Arterial cannulation and catheterization are routine parts of medical care in intensive care units for neo nates, children, and adults. In this review I describe the anatomical landmarks that facilitate successful catheteri zation of the radial, ulnar, brachial, axillary, femoral, pos terior tibial, dorsalis pedis, umbilical, and superficial temporal arteries. Criteria for selection of each of these arteries for catheterization are discussed. Risks of arte rial puncture or catheterization include hemorrhage, thrombosis, infection, and vascular compromise to sur rounding structures. These risks are reviewed with ref erence to each of the arteries described.
Pulmonary Artery Catheters: An Update
Authors: Voyce Stephen J. , Rippe James M.
Published: June 2007

Pulmonary artery (PA) catheters are widely used in the care of the critically ill. Numerous catheter designs are available, and the list of indications for their use is con stantly expanding. Extensive physiological data are sup plied by catheters. Right atrial, right ventricular, PA, and PA wedge pressure waveforms, as well as cardiac output and several derived parameters (e.g., systemic vascular resistance, pulmonary vascular resistance) are easily measured. Clinical application of these data may aid in diagnosis and management of acutely ill patients. Physi cians using catheters need to be aware of the numerous complications associated with their use. Scrupulous at tention to insertion and maintenance techniques will minimize the incidence of many of these complications. The undefined risk/benefit ratio of PA catheterization has caused controversy among physicians. Prospective studies to define better the risks versus benefits of PA catheters are currently being planned. Physicians using PA catheters should recognize that the catheters have no direct therapeutic benefit and that PA catheterization should neither replace bedside clinical evaluation nor delay treatment of the patient.
The Style Characteristics of the Hwalot, with a Focus on One Robe from the Collection of the Honolulu Academy of Arts
Authors: Kidd Laura K. , Lee Younsoo
Published: June 2007

Since the early Choson dynasty (1392-1910 C.E.), the hwalot has been culturally significant in Korea as a female wedding garment. We examine hwalot using costume history literature from Korean, English, and Japanese sources, and hwalot artifacts from Korean and Hawaiian locations. Hwalot evolved from Chinese costume styles and exhibited stylistic differences from other Korean hanbok. The visual comparison of the hwalot suggest that primary design elements such as silhouette, color, and motifs have symbolic meaning that has changed little since the Choson dynasty. The visual comparison also suggested that secondary design elements such as size, type, and placement of motifs may differ between hwalot without altering the cultural significance of this robe as a wedding garment. We suggest that traditional costume does not remain static but allows limited expression and individuality by the designer or the wearer through changes in applied surface design. Although changes may occur, they are controlled within shared cultural interpretation that do not dilute or distort the recognition and meaning of the garment, confirming the deep and significant role that the garment serves.
Unit Administrators’ Perceived Importance of Faculty Outcomes, and Potential Effect on Productivity
Authors: Laughlin Joan , Kean Rita C.
Published: June 2007

Faculty members invest resources concomitant with their perceptions of the core values, role, and mission of their institutions. Administrators’ perceptions of importance help to establish expectations for faculty performance. We examine what unit administrators consider important relative to a variety of faculty efforts. We compare administrators’ perceptions to faculty publication of scientific, refereed journal articles and raise provocative issues for thought and discussion. Ninety-three unit administrators provided information about their perceptions of importance of outcomes and descriptors about their textiles and clothing programs. Administrators differed in perceptions of importance of various indicators of faculty members’ productivity. These perceptions included four areas of agenda: research; professional; creative and university service; and service agendas. Unit administrators placed publication in scientific, refereed journals at the highest level of importance, yet the average quantity of faculty publications did not reflect that importance. Introducing realities of expectations of the work of the faculty as determined by the institution’s core values, role, and mission are critical to the future of textiles and clothing in higher education.
Indigenous Knowledge in Visual Merchandising of Textile Products
Authors: Ogle Jennifer Paff , Schofield-Tomschin Sherry
Published: June 2007

The purpose of this observational study was to document the indigenous knowledge behind visual merchandising practices used for selling textile products in Mexican craft markets. Data were collected in eight diverse markets of the Oaxaca Valley region. From the emergent design of our project evolved a model illustrating the factors that appear to impact visual merchandising decisions and a series of themes describing the visual merchandising practices evidenced. Themes generated included (a) grouping of similar products, (b) linearity of displays, (c) overlapping of products, (d) use of fixtures, (e) high levels of product density, (f) on-site production of products sold, and (g) wearing of products sold. The findings provide a useful framework for future work related to the marketing of indigenous craft items.