bluuebunny/crossref_metadata_2025 huggingface dataset:
Childhood Form of Adrenoleukodystrophy in the Sucking Infant
Authors: Kodama Soichi , Nishio Hisahide , Yokoyama Sumiyoshi , Matsuo Tamotsu , Yamano Tsunekazu , Shimada Morimi
Published: March 2008

The childhood form of adrenoleukodystrophy (ALD) in a sucking infant was discovered before the appearance of clinical symptoms. C24:0/C22:0 and C26:0/C22:0 ratios in umbilical cord plasma were significantly higher so that very long-chain saturated fatty acid-restricted milk was given. Ultrastructural study on biopsied rectal mucosa revealed rectilinear and/or curved electron-lucent clefts in the histiocytes at 3 months of age, which were also recognized in the elder brother and another boy with symptoms of ALD. However, magnetic resonance imaging and T, and T2 values were within the normal range in cerebral gray matter, cerebral white matter, caudate nucleus, internal capsule, and thalamus.
Brain Calcification in Kallman’s Syndrome
Authors: Malat Jean , Virapongse Chat
Published: March 2008

We report a 21-year-old white male with Kallman’s syndrome (gonadotropic hypogonadism, hyposmia and sensorineural hearing loss) with extensive brain calcification, demonstrated for the first time by computed tomography. The pattern and anatomic distribution of the extensive brain calcification is nonspecific, being indistinguishable from those seen in other causes of brain calcification (principally diseases related to a disturbance in calcium metabolism), despite the normal serum calcium levels found in this patient. This syndrome, therefore, should be considered along with Cockayne’s, Kearns-Sayre and Down’s syndromes, tuberous sclerosis, carbonic anhydrase II deficiency, congenital mental deficiency and idiopathic familial basal ganglia calcification as another developmental cause of brain calcification, which usually show no disturbance in serum calcium level.
Ventriculoperitoneal Shunts in Children: Factors Affecting Shunt Survival
Authors: Liptak Gregory S. , McDonald Joseph V.
Published: March 2008

The survival of ventriculoperitoneal cerebrospinal fluid shunts inserted during the past 11 years at the University of Rochester was reviewed. Half the shunts inserted failed by 23 months after insertion, although shunt survival has improved since 1981. Shunt survival was found to be affected by etiology. For example, children with neural tube defects had longer shunt survival than children with hydrocephalus from other etiologies. Children whose shunts were inserted prior to their first birthday, and children whose shunts were inserted when they weighed less than 3,000 g, had worse shunt survival. The manner in which factors such as age, weight and etiology affect shunt survival are uncertain, yet it is clear that children with hydrocephalus are a heterogeneous group. Studies that fail to consider this heterogeneity may result in invalid conclusions.
Spasmus nutans as a Presenting Sign of an Arachnoid Cyst
Authors: Spaide Richard.F. , Klara Peter M. , Restuccia Robert D.
Published: March 2008

Spasmus nutans, usually believed to be a benign entity, is a disorder of young children consisting of nystagmus, head nodding and an anomalous head position of unknown etiology. We present a patient with spasmus nutans who proved to have a large arachnoid cyst. When the cyst was shunted the nystagmus lessened. Recent studies suggest that the head nodding and anomalous head position are adaptive mechanisms that compensate for the nystagmus and not pathological in themselves. Therefore, spasmus nutans is not a triad of findings, but an acquired nystagmus with associated adaptive responses. Because spasmus nutans implies a benign entity composed of three requisite components, we feel it is an inaccurate term that should be abandoned. A better designation would be acquired nystagmus of infancy, which would not imply a benign etiology.
Sudden Death in Hydrocephalic Spina bifida aperta Patients
Authors: Staal M.J. , Meihuizen-de Regt M.J. , Hess J.
Published: March 2008

Twelve patients of 146 hydrocephalic spina bifida aperta patients, treated with a ventricular drainage system, died suddenly. In 7 of them the cause of death was most probably related to the use of a ventriculovascular shunt, causing pulmonary embolism. Thromboembolic complications were not seen in the 26 hydrocephalic spina bifida patients treated with a ventriculoperitoneal shunt. In 4 other patients shunt malfunction was considered as the cause of sudden death. The case reports of these 11 patients are presented. Pathophysiological mechanisms are discussed and measures for prevention of these two potentially lethal complications are described.
Hypothalamic Hamartoma: A Report of 2 Cases
Authors: Markin Rodney S. , Leibrock Lyal G. , Huseman Carol A. , McComb Rodney D.
Published: March 2008

Two patients with hypothalamic hamartoma presented with isosexual precocious puberty. LHRH challenge showed a pubertal LH response in both cases. Serum FSH responses to LHRH were pubertal in case 1, but prepubertal for case 2. Computed tomography revealed isodense noncontrast-enhancing retrosellar mass lesions in both cases. The tumors were composed of mature neurons and neuroglial tissue. Electron microscopy of the lesions failed to demonstrate dense core (neurosecretory) granules in either case. Subtotal removal of the harmartomas resulted in decreased LH responsiveness to LHRH in both cases. Serum FSH responsiveness to LHRH was not significantly suppressed postoperatively in case 1, and FSH responsiveness to LHRH in case 2 showed exaggerated levels, more typical of very young prepubertal girls. Postoperative magnetic resonance imaging (MRI) scans of both patients are also presented.
Cerebral Palsy Spasticity. Selective Posterior Rhizotomy
Authors: Peacock Warwick J. , Arens Leila J. , Berman Barbara
Published: March 2008

We have performed selective posterior rhizotomies on 60 children with cerebral palsy. The procedure involves lumbar laminectomy with stimulation of the rootlets (fascicles) of the second lumbar to the first sacral posterior roots bilaterally; those rootlets associated with an abnormal motor response, as evidenced by sustained or diffused muscular contraction, are divided leaving intact rootlets associated with a brief localized contraction. The patients were between 20 months and 19 years of age representing all degrees of physical handicap and ranging from profoundly retarded to normal intelligence. Follow-up has been from 1 to 5 years. Each child was assessed pre-and postoperatively and graded in the following categories: muscle tone, power, sitting, standing, walking, upper limb function, and speech. The patients whose function improved most dramatically following rhizotomy were purely spastic and intelligent, were significantly more affected by spasticity in the lower than the upper limbs, had some degree of forward locomotion, and could side-sit independently. Patients with severe athetosis or marked contractures improved least.
Central Nervous System Lymphomatoid Granulomatosis
Authors: Whelan Harry T. , Moore Patricia
Published: March 2008

Lymphomatoid granulomatosis is a vasocentric lymphoreticular proliferative disease. Some patients go on to develop frank neoplasia. Central nervous system involvement with lymphomatoid granulomatosis has been reported previously in the literature. We are presenting a childhood case evaluated with magnetic resonance imaging and x-ray computerized tomography who developed severe tissue destruction with pleomorphic cellular infiltrate and oligoclonal light chains. This report adds information regarding neuroimaging as well as immunopathology data relevant to the basic biology of this disease.
Bilateral Middle Cranial Fossa Arachnoid Cysts
Authors: Nagaya Takashi , Doi Terushige , Katsumata Tsuguo , Kuwayama Naoto
Published: March 2008

A rare case of bilateral arachnoid cysts of the middle cranial fossa in a child successfully treated by partial excision of their walls is reported. Patients with bilateral arachnoid cysts are usually severely disabled, presumably due to primary dysgenesis of the operculum; however, in our case no primary dysgenesis was noted and the operative result was excellent.
Trigeminal Schwannomas in Adolescence
Authors: Cantini Rinaldo , Giorgetti Waifredo , Valleriani Maria , Burchianti Marilena
Published: March 2008

Trigeminal neurinomas, unlike those of the acoustic nerve or of other cranial nerves, are relatively frequent in adolescence, making up 10% of the total. Two cases are described, bringing the number of reports in the world literature up to 13. The clinical, radiological and surgical data on these tumors are analyzed, and some particular aspects present in adolescence are pointed out.
Walker-Warburg Syndrome with Skeletal Muscle Involvement
Authors: Takada Kuniyasu , Becker Laurence E. , Takashima Sachio
Published: March 2008

Three patients with Walker-Warburg syndrome are reported. In all, severe hypotonia and ocular abnormalities were noted soon after birth, followed by a rapid increase of head size. Examination of the brain showed regions of complete agyria, cortical dysplasia of the cerebrum, cerebellar micropolygyria, marked hydrocephalus and aqueduct stenosis. In skeletal muscles, advanced myopathy consistent with dystrophic change was present. The cortical dysplasia in this syndrome is similar to the severest pattern of the cortical dysplasia seen in Fukuyama congenital muscular dystrophy, but ocular abnormalities are rare in the latter. These two syndromes may be genetically distinct, despite the presence of cortical dysplasia and myopathy in both.
Management of Infratentorial Brain Tumors
Authors: Hudgins Roger J. , Edwards Michael S.B.
Published: March 2008

Because 60–70% of brain tumors in children occur in the posterior fossa, the pediatric neurosurgeon must be familia with the specialized techniques necessary for successful surgery in this region. We discuss the preoperative evaluation, surgical techniques, and postoperative management for children harboring infratentorial brain tumors. Using this approach to treatment, we have had no operative mortalities, a 2% rate of infection, a 15% rate of transient neurologic deficit, and a 5% rate of permanent neurologic deficit.
Subdural Empyemas in Children
Authors: Pattisapu Jogi V. , Parent Andrew D.
Published: March 2008

Subdural empyema is a neurosurgical emergency which is rapidly fatal if not recognized and managed promptly. Most series report a 30–40% mortality, and recommend a craniotomy along with aggressive medical therapy. Between 1978 and 1986, 8 children (2 months to 13 years) with subdural empyemas were diagnosed and treated at our institution, and form the basis for this study. Burr hole and catheter drainage was the treatment of choice in 5 children, while craniotomy was required in 1 case of sinusitis with osteomyelitis. Three infants recieved multiple subdural taps via the anterior fontanel. All patients responded to surgical intervention and antibiotic therapy. The average follow-up period was 29 months, and 5 children had no developmental delay, decrease in school performance, or impairment of intellectual function. There were no deaths in our series. Although the surgical management of subdural empyemas remains controversial, it appears that burr hole and catheter drainage is sufficient in most cases. With earlier diagnosis, aggressive antibiotic therapy, and timely surgical intervention, the morbidity and mortality of subdural empyemas have significantly diminished in recent years.
External Ventricular Drainage for Initial Treatment of Neonatal Posthemorrhagic Hydrocephalus: Surgical and Neurodevelopmental Outcome
Authors: Rhodes Torunn T. , Edwards William H. , Saunders Richard L. , Harbaugh Robert E. , Little Carol L.C. , Morgan Linda J. , Sargent Steven K.
Published: March 2008

Thirty-seven infants were treated for posthemorrhagic hydrocephalus (PHH) with external ventricular drainage (EVD) in 1977–1985. The mean age at EVD placement was 13 days; the mean duration of drainage was 21 days, and an average of 1.8 drains was used per patient. Complications were apnea (10%), hemorrhage (8%) and ventriculitis (6%). Ten infants died from pulmonary disease unrelated to the surgery. Thirty-two percent of the survivors did not require a permanent shunt. Neurodevelopmental outcome was evaluated at a mean age of 38 months. Eighteen of the twenty-seven survivors (67%) had normal cognitive function, 3 had borderline and 6 (22%) had moderate to profound mental retardation. Nine of the children (33 %) had a normal neuromuscular exam. One child had mild, 11 had moderate and 6 had severe cerebral palsy. Four children had serious visual impairment. The worst outcomes were in the children with parenchymal or large intraventricular hemorrhages.
Epithelioid Hemangioendothelioma of the Central Nervous System
Authors: Taratuto Ana Lia , Zurbriggen G. , Sevlever G. , Saccoliti M.
Published: March 2008

In 1982, Weiss and Enzinger described a group of soft tissue tumors in adults that they called epithelioid hemangioendothelioma [Cancer 50: 970–981, 1982]. Such tumors have also been found in heart, bone, liver, lung and lymph nodes, and Kepes and Rubinstein described two intracranial examples in adults in 1986 [J. Neuropath, exp. Neurol. 45: 319, 1986]. This report documents clinical and pathological features of an epithelioid hemangioendothelioma in the parietal lobe of a 4-year-old boy.
Sex Hormone Receptors in a Congenital Meningioma
Authors: Carter David A. , Hollenberg Robert D.
Published: March 2008

The authors report a case of a congenital meningioma in a male infant in whom recaptor binding sites for progesterone were significantly elevated.
Significance of Basal Ganglia Calcification on Computed Tomography in Children
Authors: Legido Augustin , Zimmerman Robert A. , Packer Roger J. , Bilaniuk Larissa T. , Siegel Kathy R. , D’Angio Giulio
Published: March 2008

We reviewed 6,428 head computed tomography (CT) scans performed on 4,283 children at our institution over a 3-year period and found basal ganglia calcification (BGC) in 48 (1.1%) of the patients. Their mean age at the time of detection was 5.3 years (range: 0.5–20 years); 16 (33%) patients had cancer, 14 (29%) had tuberous sclerosis or congenital infection and 18 (38%) had other medical conditions. All patients with cancer had been treated with radiation therapy, receiving a mean dose of 4,583 cGy (range: 1,800–5,500 cGy) to the diencephalon, and calcifications first became apparent at a median of 10 months after treatment. Other medical conditions included neonatal asphyxia (3), metabolic disease (3) (Kearns Sayre, MELAS, Krabbe’s), congenital anomalies (3), meningitis (2), Fahr’s disease (1) and others (6). Neurologic symptoms were common in children of all groups, but could not be correlated to BGC changes. Calcium and phosphorus metabolism was evaluated in 19 patients and was abnormal in 1. We conclude that BGC on CT in childhood occur primarily as an aftermath of the cancer treatment or in children with generalized neurologic dysfunction. Many children with BGC are delayed in their development, but calcifications are not directly related to specific forms of neurologic dysfunction. Rather, they appear to serve as markers for more extensive brain damage.
Diastematomyelia – A 40-Year Experience
Authors: Gower David J. , Del Curling O. , Kelly, Jr. David L. , Alexander, Jr. Eben
Published: March 2008

Diastematomyelia is a rare but potentially devastating spinal dysraphism classically characterized as a bony or fibrous spur separating two hemispinal cords. This study reviews our experience with diastematomyelia over a 40-year period from 1947 through 1987, and suggests that the neurologic and orthopedic deficits of diastematomyelia remain stable during extended follow-up after surgery.
Brainstem Glioma with Multiple Intraspinal Metastases during Life: Case Report and Review of the Literature
Authors: Silbergeld D. , Berger M. , Griffin B. , Bleyer A. , Geyer R. , Milstein J. , Eskridge J.
Published: March 2008

We present a case of a 17-year-old male with an unbiopsied brainstem glioma who developed drop metastases a few months following completion of hyperfractionated radiation therapy to the primary site. The literature is reviewed with reference to intraspinal metastases from brainstem gliomas.
Diastematomyelia Associated with Ectopic Renal Tissue
Authors: Gaskill Sarah J. , Kagen-Hallett Katherine , Marlin Arthur E.
Published: March 2008

A case of diastematomyelia associated with ectopic renal tissue is presented. While findings of ectopic renal tissue and Wilms tumor in dysraphic conditions have been reported in the literature, there has been no incorporation of these findings into the theories of the development of neural tube defects. This case supports the hypothesis of neural tube defects secondary to rupture of a previously intact neural tube (secondary neural tube defect). A discussion and review of the literature regarding the hypotheses of primary and secondary neural tube defects is included.
Choroid Plexus Carcinoma of the Fourth Ventricle
Authors: Griffin Brian R. , Stewart Glenn R. , Berger Mitchell S. , Geyer Russell , O’Dell Michael , Rostad Steven
Published: March 2008

An 8-month-old female infant with choroid plexus carcinoma presenting in the fourth ventricle is described. The patient was initially treated with combination chemotherapy due to her young age, but developed local tumor recurrence 13 months after beginning treatment. Staging studies to detect tumor seeding of the cerebrospinal fluid were negative, and the patient received posterior fossa radiotherapy. Although the primary tumor regressed with radiation treatment, widespread disease throughout the spinal subarachnoid space subsequently developed. Choroid plexus carcinoma has a significant tendency to metastasize through the cerebrospinal fluid, and treatment of the entire neuraxis should be strongly considered for patients with this disease.
Neonatal Cerebral Venous Thrombosis
Authors: Hanigan William C. , Tracy Patrick T. , Tadros Wadie S. , Wright Robert M.
Published: March 2008

Five cases of neonatal cerebral venous thrombosis (NCVT) diagnosed by magnetic resonance imaging (MRI) are presented in this report. MRI was specific for the anatomic diagnosis, demonstrating involvement of the superior sagittal sinus in 3 infants or deep venous system in the remaining 2. Four cases were associated with perinatal hypoxia or cranial trauma. Three of these children show significant developmental delays. The increased use of MRI may add significant information on the association of cerebral venous thrombosis with the common systemic illnesses of newborns.
Aggressive Physiologic Monitoring of Pediatric Head Trauma Patients with Elevated Intracranial Pressure
Authors: Kasoff Samuel S. , Lansen Thomas A. , Holder Donovan , San Filippo Joseph
Published: March 2008

The management of pediatric patients with severe head trauma often requires aggressive physiologic monitoring and treatment. As intracranial pressure (ICP) increases, so does mortality. Yet attempts to decrease elevated ICP can cause physiologic and hemodynamic problems. In this retrospective analysis of 25 patients treated with ICP monitoring alone, ICP measurements plus mannitol, or ICP measurements plus pentobarbital (PB) induced coma/mannitol, survival rates were higher than predicted, particularly among the most severely injured. These results were apparently due to the intensive care and aggressive monitoring. Although barbiturates are known to decrease ICP, the adverse effects encountered, including hypotension, cardiovascular depression and arrhythmias, often outweigh the benefits. Hemodynamic abnormalities are unpredictable and monitoring with Swan-Ganz catheter is useful in managing patients with PB-induced comas. Many failures of PB therapy in pediatric patients may be related to undiagnosed decreases in cardiac output, resulting in decreased cerebral blood flow, even with well-controlled ICP.
Patient Data System for Monitoring Shunts
Authors: Frank Edmund , Su Eustacia , Smith Karen
Published: March 2008

Rapidly locating accurate data on a patient’s shunt system is often extremely difficult. We have developed a simple system to fill a perceived need for recording current data on a patients shunt. This system employs an easily updated record in the patient’s hospital or clinic chart as well as a wallet-sized data card for the patient or his family to carry. The data in the chart include the configuration of the patient’s current shunt system and a graphic record of previous shunt problems. The small patient data card describes the age of the shunt system and its current configuration. We have found that this system provides assistance in the routine follow-up of patients with shunts and plays an extremely necessary role in the emergency evaluation of these patients, particularly when an emergency evaluation is undertaken in facilities distant from the location of regular treatment.
Stereotactic Decompression of a Prepontine Arachnoid Cyst with Resolution of Precocious Puberty
Authors: Sweasey Thomas A. , Venes Joan L. , Hood Terry W. , Randall Jeffrey B.
Published: March 2008

A case of a prepontine (clival) arachnoid cyst is reported. The patient presented with isosexual precocious puberty. Treatment was initially with fenestration by suboccipital craniectomy, however, definitive treatment was via stereotactic transfrontal placement of a cystoperitoneal shunt. Normalization of endocrine function has occurred and has persisted for 1 year. This case is presented as a contrast to the two approaches for the treatment of arachnoid cysts. The case will be described and the literature reviewed.
Dandy-Walker Syndrome: Experience at the Hospital for Sick Children, Toronto
Authors: Asai Akio , Hoffman Harold J. , Hendrick Bruce , Humphreys Robin P.
Published: March 2008

Thirty-five patients with the Dandy-Walker syndrome (DWS) treated over the years 1964-1987 at the Hospital for Sick Children, Toronto, were reviewed. Thirty-three patients (94%) had hydrocephalus at the time of diagnosis. The primary association of aqueduct stenosis was excluded by radiological investigation and clinical course in all patients. Associated central nervous system (CNS) anomalies were present in 10 patients. Seven had occipital encephalocele and in 1 of these there was an associated Klippel-Feil syndrome. Four patients had agenesis of the corpus callosum and 1 patient had polyporencephaly. The treatment of these patients has changed over the years in our institution so that during an earlier period, 3 patients were treated by resection of the cyst membranes. We then went through a period of shunting the lateral ventricle so that 21 patients were treated with either a lateral ventricle to peritoneal or lateral ventricle to atrial shunt. More recently, patients with the DWS have been treated with a cyst-peritoneal shunt and 10 patients were thus treated. Only 1 patient was treated with a simultaneous lateral ventricle and cyst-peritoneal shunt. Nine of the twenty-one patients (43%) with a primary lateral ventricle to peritoneum or lateral ventricle to atrial shunt developed a secondary aqueduct stenosis and an isolated fourth ventricle which required additional cyst-peritoneal shunting. Only 1 of the 10 patients treated with a primary cyst-peritoneal shunt required an additional lateral ventricle peritoneal shunt. Two patients initially treated with a lateral ventricle peritoneal shunt and with a primary cyst-peritoneal shunt subsequently had a percutaneous stereotactic third ventriculostomy. Both of these patients remained shunt-independent but 1 died in a traffic accident. Primary cyst-peritoneal shunting seems to be the treatment of choice for the DWS.
MODIS-Derived Spatially Complete Surface Albedo Products: Spatial and Temporal Pixel Distribution and Zonal Averages
Authors: Moody Eric G. , King Michael D. , Schaaf Crystal B. , Platnick Steven
Published: March 2008

Five years (2000–04) of spatially complete snow-free land surface albedo data have been produced using high-quality-flagged diffuse bihemispherical (white sky) and direct-beam directional hemispherical (black sky) land surface albedo data derived from observations taken by the Moderate-Resolution Imaging Spectroradiometer (MODIS) instrument aboard the NASA Terra satellite platform (MOD43B3, collection 4). In addition, a spatially complete snow-free aggregate albedo climatological product was generated. These spatially complete products were prepared using an ecosystem-dependent temporal interpolation technique that retrieves missing data within 3%–8% error. These datasets have already been integrated into research and operational projects that require snow-free land surface albedo. As such, this paper provides details regarding the spatial and temporal distribution of the filled versus the original MOD43B3 data. The paper also explores the intra- and interannual variation in the 5-yr data record and provides a qualitative comparison of zonal averages and annual cycles of the filled versus the original MOD43B3 data. The analyses emphasize the data’s inter- and intraannual variation and show that the filled data exhibit large- and small-scale phenological behavior that is qualitatively similar to that of the original MOD43B3. These analyses thereby serve to showcase the inherent spectral, spatial, and temporal variability in the MOD43B3 data as well as the ability of the fill technique to preserve these unique regional and pixel-level phenological characteristics.
On the Decay of Supercells through a “Downscale Transition”: Visual Documentation
Authors: Bluestein Howard B.
Published: March 2008

Photographic and mobile-radar documentation of the dissipation of a supercell and a severe convective storm that had not yet developed into a mature supercell are discussed. It is hypothesized, based on these cases and on others, that when a low-precipitation or classic supercell and/or a developing supercell moves into an environment of cooler surface temperatures and a strong capping inversion, it eventually dissipates through a process of “downscale transition,” in which vertical shear tilts the updraft more in the downshear direction as the CAPE decreases, and the updraft becomes narrower as the storm dissipates. During the downscale transition, it is possible that a cold pool or lack thereof may play a role, but the documentation in the cases detailed herein is not adequate to address this issue.
Security, Muscularity and Morality
Authors: Mueller John
Published: March 2008

Democracy has become increasingly accepted over the last two centuries, but the process has mostly been one of persuasion, fashion leadership, and luck, not forceful evangelism. Although democracy can sometimes be established by force, this has generally not worked well when favorable conditions are absent. Intervention for the purpose of restoring basic security where it is severely threatened or under attack is unlikely to happen much, held back by a lack of interest, an extremely low tolerance for casualties, and an increasing aversion to long-term policing in the international community. The obsessive bipartisan quest to control nuclear proliferation— particularly since the end of the cold war—has been substantially counterproductive and has often inflicted major costs on innocent people. Rather than killing large numbers of foreigners in the service of worst case scenario fantasies, one way to reduce the likelihood some countries would go nuclear would be to stop threatening them.
Expectations to Marry Among American Youth
Authors: Gassanov Margaret A. , Nicholson Lisa M. , Koch-Turner Amanda
Published: March 2008

Since welfare reform in 1996, marriage has been promoted as a means to reduce welfare dependency and out-of-wedlock childbearing. Despite extensive public and academic discourse surrounding marriage promotion, a basic factor preceding and predicting marriage—expectations to marry—has received little attention. Using insights from the life course perspective and data from the 1997 National Longitudinal Survey of Youth, we explore the relationship between out-of-wedlock fertility and youths' expectations to marry within 5 years. We also consider the effect of economic activity (school and work) and cohabitation. We find that experiencing an unwed pregnancy or birth significantly increases youths' expectations to marry, as does being employed, out of school, and in a cohabiting relationship.
A 12‐Month, Randomized, Controlled Study to Evaluate Exposure and Cardiovascular Risk Factors in Adult Smokers Switching From Conventional Cigarettes to a Second‐Generation Electrically Heated Cigarette Smoking System
Authors: Roethig Hans J. , Feng Shixia , Liang Qiwei , Liu Jianmin , Rees William A. , Zedler Barbara K.
Published: March 2008

This randomized, controlled, forced‐switching, open‐label, parallel‐group study in 97 adult male and female smokers of conventional cigarettes evaluated biomarkers of tobacco smoke exposure and cardiovascular risk factors. After baseline measurements, smokers were either switched to a second‐generation electrically heated cigarette smoking system (EHCSS) or continued smoking conventional cigarettes for 12 months. Biomarkers of exposure and cardiovascular risk factors were measured at 0.5, 1, 2, 3, 4, 5, 6, 9, and 12 months. There was a rapid and sustained reduction in all biomarkers of exposure after switching to the EHCSS, with statistically significant reductions from baseline in nicotine equivalents (−18%), plasma cotinine (−16%), total 4‐(methylnitrosamino)‐1‐(3‐pyridyl)‐1‐butanol (−73%), total 1‐hydroxypyrene (−53%), urine mutagenicity (−52%), 4‐aminobiphenyl hemoglobin adducts (−43%), carboxyhemoglobin AUC7–23 h (−80%), and 3‐hydroxypropylmercapturic acid (−35%). These reductions in exposure in the EHCSS group were associated with statistically significant and pathophysiologically favorable changes in several cardiovascular risk factors, including white blood cell count (−0.78 × 103/μL), hemoglobin (−0.16 g/dL), hematocrit (−0.44%), urine 11‐dehydrothromboxane B2 (−374 ng/24 h), and high‐density lipoprotein cholesterol (+5 mg/dL).
Is Mom Still Doing It All? Reexamining Depictions of Family Work in Popular Advertising
Authors: Robinson Bryan K. , Hunter Erica
Published: March 2008

This study examines a sample of 299 advertisements from 4 of the top 10 circulated magazines of 2005 to see how contemporary advertising depicts household labor. Modeling after previous studies that examined the depiction of gender in family advertising, this study seeks to determine whether advertising reflects the changes in families that have occurred in contemporary American culture. The authors use Hochschild's (1989) typology of traditional, transitional, and egalitarian families to examine whether advertisements' portrayal of men and women in family situations resemble traditional (male breadwinner, female caregiver) or more contemporary parenting ideologies (men providing care, women in the labor force, etc.).
Arthroscopic Repair of Isolated Meniscal Tears in Patients 18 Years and Younger
Authors: Krych Aaron J. , McIntosh Amy L. , Voll Anthony E. , Stuart Michael J. , Dahm Diane L.
Published: March 2008

Background

Isolated meniscal tears in the skeletally immature patient are infrequent but well-recognized injuries. Although few studies have evaluated the pediatric and adolescent population, arthroscopic surgical repair remains the standard of care for Patients in this age group with unstable meniscal tears.

Purpose

To review our results of arthroscopic repair of isolated meniscal tears in pediatric and adolescent patients to further define future management of these injuries.

Study Design

Case series; Level of evidence, 4.

Methods

The records of all patients 18 years old or younger who underwent isolated meniscal repair between 1990 and 2005 were retrospectively reviewed. Forty-four patients (6 girls, 38 boys) with an average age of 15.8 years (range, 9.9-18.7 years) were included in this study, for a total of 45 isolated meniscal tears. Clinical examinations were performed, International Knee Documentation Committee forms were administered, and Tegner scores were determined at an average of 5.8 years (range, 2.5 months-13.8 years) postoperative follow-up. Three patients were lost to follow-up.

Results

The clinical success rate of arthroscopic meniscal repair was 80% for simple tears, 68% for displaced bucket-handle tears, and 13% for complex tears. Seventeen menisci (38% overall) failed initial repair at a mean of 17 months (range, 3-61 Months) postoperatively and underwent repeat arthroscopic surgery (15 partial meniscectomies, 2 rerepair). The average Tegner and International Knee Documentation Committee scores were 8 (range, 5-9) and 89.4 (range, 79-99), respectively, at the time of final follow-up. Risk factors for failure included complex tears and rim width greater than 3 mm.

Conclusion

Clinically successful repair of an isolated meniscal tear in patients 18 years or younger was variable depending on tear type, with complex tears and rim width 3 mm or greater being negative prognostic factors.
Interoperability of Cross-organizational Workflows based on Process-view for Collaborative Product Development
Authors: Ping Jiang  , Xinyu Shao  , Haobo Qiu  , Peigen Li
Published: March 2008

Collaborative product development (CPD) has been widely accepted as an advanced collaboration paradigm that combines geographically distributed product development teams to develop product collaboratively and efficiently. Interoperability of cross-organizational workflows is important for CPD to facilitate the successful execution of the whole product development process across enterprises boundaries. This article, describes a timed colored petri net (TCPN) and process-view combined approach to construct cross-organizational workflows, and a three models framework of `TCPN workflow models — Process-view workflow models — Integrated process-view workflow model' is proposed to realize the interoperability of cross-organizational workflows for CPD. A multi-agent system PVMAS provides a mediated architecture for distributed workflow management systems interoperability among cooperative enterprises. Under this architecture, a service discovery algorithm is provided for discovering the most suitable services enterprises need, a TCPN algebra model and a character string mapping algorithm are proposed to automate the mapping from a TCPN workflow model to a process-view workflow model, and the synchronization points are designated in the integrated process-view workflow model to coordinate the execution of cross-organizational workflow instances. Finally, a case study of the collaborative development of a motorcycle is presented to verify the validity of our approach for cross-organizational workflows interoperability in CPD.
Racial Variance in Rationale for HIV Testing in Community-Based Setting in the United States: Evidence from the National Health Interview Survey
Authors: Holmes Laurens , Monjok Emmanuel , Ward Doriel , Garrison Orsolya M. , Toney Evette D. , Ogungbade Gbadebo , Essien E. James
Published: March 2008

Background: HIV testing varies across racial/ethnic groups in the United States, but it is unclear whether the rationale for testing differs as well racially. The authors aimed to assess the rationale for HIV testing and the racial/ethnic variation therein. Methods: Using the National Health Interview Survey 2003 (n = 29 753), the authors examined the association between rationale for HIV testing and race. Chi-square statistic and multinomial logistic regression analyses were used to test for racial differences and the effect of race/ethnicity on the rationale for HIV testing. Results: There was a statistically significant racial difference with respect to HIV testing rationale, χ 2 = (24, N = 10481) = 808.9, P < .001. After adjustment for relevant covariates, compared with Caucasians, African Americans were 37% less likely to be tested due to exposure to sex/drugs, whereas Hispanics were not (prevalence risk ratio [PRR], 0.63, 95% confidence interval [CI] = 0.47-0.84, respectively). Likewise African Americans and Hispanics were less likely to be tested if they were sick or had a medical problem (PRR = 0.66, 95% CI = 0.44-0.99 and PRR = 0.65, 95% CI = 0.43-0.98). Conclusions: Substantial racial variation occurred in the reasons for being tested for HIV in the United States, indicative of the need to understand such rationale for effective HIV screening and testing.
A role for interleukin-12/23 in the maturation of human natural killer and CD56+ T cells in vivo
Authors: Guia Sophie , Cognet Céline , de Beaucoudrey Ludovic , Tessmer Marlowe S. , Jouanguy Emmanuelle , Berger Claire , Filipe-Santos Orchidée , Feinberg Jacqueline , Camcioglu Yildiz , Levy Jacob , Al Jumaah Suliman , Al-Hajjar Sami , Stephan Jean-Louis , Fieschi Claire , Abel Laurent , Brossay Laurent , Casanova Jean-Laurent , Vivier Eric
Published: March 2008

Natural killer (NK) cells have been originally defined by their “naturally occurring” effector function. However, only a fraction of human NK cells is reactive toward a panel of prototypical tumor cell targets in vitro, both for the production of interferon-γ (IFN-γ) and for their cytotoxic response. In patients with IL12RB1 mutations that lead to a complete IL-12Rβ1 deficiency, the size of this naturally reactive NK cell subset is diminished, in particular for the IFN-γ production. Similar data were obtained from a patient with a complete deficit in IL-12p40. In addition, the size of the subset of effector memory T cells expressing CD56 was severely decreased in IL-12Rβ1– and IL-12p40–deficient patients. Human NK cells thus require in vivo priming with IL-12/23 to acquire their full spectrum of functional reactivity, while T cells are dependent upon IL-12/23 signals for the differentiation and/or the maintenance of CD56+ effector memory T cells. The susceptibility of IL-12/23 axis–deficient patients to Mycobacterium and Salmonella infections in combination with the absence of mycobacteriosis or salmonellosis in the rare cases of human NK cell deficiencies point to a role for CD56+ T cells in the control of these infections in humans.
Common variable immunodeficiency disorders: division into distinct clinical phenotypes
Authors: Chapel Helen , Lucas Mary , Lee Martin , Bjorkander Janne , Webster David , Grimbacher Bodo , Fieschi Claire , Thon Vojtech , Abedi Mohammad R. , Hammarstrom Lennart
Published: March 2008

The European Common Variable Immunodeficiency Disorders registry was started in 1996 to define distinct clinical phenotypes and determine overlap within individual patients. A total of 7 centers contributed patient data, resulting in the largest cohort yet reported. Patients (334), validated for the diagnosis, were followed for an average of 25.6 years (9461 patient-years). Data were used to define 5 distinct clinical phenotypes: no complications, autoimmunity, polyclonal lymphocytic infiltration, enteropathy, and lymphoid malignancy. A total of 83% of patients had only one of these phenotypes. Analysis of mortality showed a considerable reduction in the last 15 years and that different phenotypes were associated with different survival times. Types of complications and clinical phenotypes varied significantly between countries, indicating the need for large, international registries. Ages at onset of symptoms and diagnosis were shown to have a Gaussian distribution, but were not useful predictors of phenotype. The only clinical predictor was polyclonal lymphocytic infiltration, which was associated with a 5-fold increased risk of lymphoid malignancy. There was widespread variation in the levels of serum immunoglobulin isotypes as well as in the percentages and absolute numbers of B cells, confirming the heterogeneity of these conditions. Higher serum IgM and lower circulating CD8 proportions were found to be predictive markers for polyclonal lymphocytic infiltration and autoimmunity, respectively.
A critical role of Rap1b in B-cell trafficking and marginal zone B-cell development
Authors: Chen Yuhong , Yu Mei , Podd Andrew , Wen Renren , Chrzanowska-Wodnicka Magdalena , White Gilbert C. , Wang Demin
Published: March 2008

B-cell development is orchestrated by complex signaling networks. Rap1 is a member of the Ras superfamily of small GTP-binding proteins and has 2 isoforms, Rap1a and Rap1b. Although Rap1 has been suggested to have an important role in a variety of cellular processes, no direct evidence demonstrates a role for Rap1 in B-cell biology. In this study, we found that Rap1b was the dominant isoform of Rap1 in B cells. We discovered that Rap1b deficiency in mice barely affected early development of B cells but markedly reduced marginal zone (MZ) B cells in the spleen and mature B cells in peripheral and mucosal lymph nodes. Rap1b-deficient B cells displayed normal survival and proliferation in vivo and in vitro. However, Rap1b-deficient B cells had impaired adhesion and reduced chemotaxis in vitro, and lessened homing to lymph nodes in vivo. Furthermore, we found that Rap1b deficiency had no marked effect on LPS-, BCR-, or SDF-1–induced activation of mitogen-activated protein kinases and AKT but clearly impaired SDF-1–mediated activation of Pyk-2, a key regulator of SDF-1–mediated B-cell migration. Thus, we have discovered a critical and distinct role of Rap1b in mature B-cell trafficking and development of MZ B cells.
Normalization of oligonucleotide arrays based on the least-variant set of genes
Authors: Calza Stefano , Valentini Davide , Pawitan Yudi
Published: March 2008

Background

It is well known that the normalization step of microarray data makes a difference in the downstream analysis. All normalization methods rely on certain assumptions, so differences in results can be traced to different sensitivities to violation of the assumptions. Illustrating the lack of robustness, in a striking spike-in experiment all existing normalization methods fail because of an imbalance between up- and down-regulated genes. This means it is still important to develop a normalization method that is robust against violation of the standard assumptions

Results

We develop a new algorithm based on identification of the least-variant set (LVS) of genes across the arrays. The array-to-array variation is evaluated in the robust linear model fit of pre-normalized probe-level data. The genes are then used as a reference set for a non-linear normalization. The method is applicable to any existing expression summaries, such as MAS5 or RMA.

Conclusion

We show that LVS normalization outperforms other normalization methods when the standard assumptions are not satisfied. In the complex spike-in study, LVS performs similarly to the ideal (in practice unknown) housekeeping-gene normalization. An R package called lvs is available in http://www.meb.ki.se/~yudpaw.
Enhancing the prediction of protein pairings between interacting families using orthology information
Authors: Izarzugaza Jose MG , Juan David , Pons Carles , Pazos Florencio , Valencia Alfonso
Published: March 2008

Background

It has repeatedly been shown that interacting protein families tend to have similar phylogenetic trees. These similarities can be used to predicting the mapping between two families of interacting proteins (i.e. which proteins from one family interact with which members of the other). The correct mapping will be that which maximizes the similarity between the trees. The two families may eventually comprise orthologs and paralogs, if members of the two families are present in more than one organism. This fact can be exploited to restrict the possible mappings, simply by impeding links between proteins of different organisms. We present here an algorithm to predict the mapping between families of interacting proteins which is able to incorporate information regarding orthologues, or any other assignment of proteins to "classes" that may restrict possible mappings.

Results

For the first time in methods for predicting mappings, we have tested this new approach on a large number of interacting protein domains in order to statistically assess its performance. The method accurately predicts around 80% in the most favourable cases. We also analysed in detail the results of the method for a well defined case of interacting families, the sensor and kinase components of the Ntr-type two-component system, for which up to 98% of the pairings predicted by the method were correct.

Conclusion

Based on the well established relationship between tree similarity and interactions we developed a method for predicting the mapping between two interacting families using genomic information alone. The program is available through a web interface.
Glycosyltransferase efficiently controls phenylpropanoid pathway
Authors: Aksamit-Stachurska Anna , Korobczak-Sosna Alina , Kulma Anna , Szopa Jan
Published: March 2008

Background

In a previous study, anthocyanin levels in potato plants were increased by manipulating genes connected with the flavonoid biosynthesis pathway. However, starch content and tuber yield were dramatically reduced in the transgenic plants, which over-expressed dihydroflavonol reductase (DFR).

Results

Transgenic plants over-expressing dihydroflavonol reductase (DFR) were subsequently transformed with the cDNA coding for the glycosyltransferase (UGT) of Solanum sogarandinum in order to obtain plants with a high anthocyanin content without reducing tuber yield and quality. Based on enzyme studies, the recombinant UGT is a 7-O-glycosyltransferase whose natural substrates include both anthocyanidins and flavonols such as kaempferol and quercetin. In the super-transformed plants, tuber production was much higher than in the original transgenic plants bearing only the transgene coding for DFR, and was almost the same as in the control plants. The anthocyanin level was lower than in the initial plants, but still higher than in the control plants. Unexpectedly, the super-transformed plants also produced large amounts of kaempferol, chlorogenic acid, isochlorogenic acid, sinapic acid and proanthocyanins.

Conclusion

In plants over-expressing both the transgene for DFR and the transgene for UGT, the synthesis of phenolic acids was diverted away from the anthocyanin branch. This represents a novel approach to manipulating phenolic acids synthesis in plants.
Retinoic Acid Stimulation of the Sodium/Iodide Symporter in MCF-7 Breast Cancer Cells Is Meditated by the Insulin Growth Factor-I/Phosphatidylinositol 3-Kinase and p38 Mitogen-Activated Protein Kinase Signaling Pathways
Authors: Kogai Takahiko , Ohashi Emi , Jacobs Megan S. , Sajid-Crockett Saima , Fisher Myrna L. , Kanamoto Yoko , Brent Gregory A.
Published: March 2008

Context: All-trans retinoic acid (tRA) induces differentiation in MCF-7 breast cancer cells, stimulates sodium/iodide symporter (NIS) gene expression, and inhibits cell proliferation. Radioiodine administration after systemic tRA treatment has been proposed as an approach to image and treat some differentiated breast cancer.

Objective: The objective of this work was to study the relative role of genomic and nongenomic pathways in tRA stimulation of NIS expression in MCF-7 cells.

Design: We inspected the human NIS gene locus for retinoic acid-responsive elements and tested them for function. The effects of signal transduction pathway inhibitors were also tested in tRA-treated MCF-7 cells and TSH-stimulated FRTL-5 rat thyroid cells, followed by iodide uptake assay, quantitative RT-PCR of NIS, and cell cycle phase analysis.

Results: Multiple retinoic acid response elements around the NIS locus were identified by sequence inspection, but none of them was a functional tRA-induced element in MCF-7 cells. Inhibitors of the IGF-I receptor, Janus kinase, and phosphatidylinositol 3-kinase (PI3K), significantly reduced NIS mRNA expression and iodide uptake in tRA-stimulated MCF-7 cells but not FRTL-5 cells. An inhibitor of p38 MAPK significantly reduced iodide uptake in both tRA-stimulated MCF-7 cells and TSH-stimulated FRTL-5 cells. IGF-I and PI3K inhibitors did not significantly reduce the basal NIS mRNA expression in MCF-7 cells. Despite the chronic inhibitory effects on cell proliferation, tRA did not reduce the S-phase distribution of MCF-7 cells during the period of NIS induction.

Conclusion: The IGF-I receptor/PI3K pathway mediates tRA-stimulated NIS expression in MCF-7 but not FRTL-5 thyroid cells.