bluuebunny/crossref_metadata_2025_split huggingface dataset:
Categorical ML — Category-theoretic modular programming
Authors: Dennis-Jones Esther , Rydeheard David E. 
Published: February 2005

Abstract
We consider an extension of the functional programming language Standard ML with a modular structure based upon concepts in category theory such as categories, functors, natural transformations and adjunctions. In essence, we are following the categorical imperative of considering arrows as well as objects. This is intended to enforce a certain mathematical rigour on the programmer, so that the only programs that can be expressed are those with a categorical significance. The essentially algebraic nature of category theory means that we may generate equational correctness conditions for the modular structure of programs, thus separating the correctness of individual functions from that of modules. We describe this programming language, give examples of its use, and explain how it is implemented in a type system.
HTLV-1 Associated Motor Neuron Disease Mimicking “Man-in-the-Barrel” Syndrome
Authors: Vainstein Gabriel , Gordon Carlos R , Gadoth Natan 
Published: February 2005

Abstract

The “man-in-the-barrel” syndrome is a descriptive term that was used in the past for patients with complete flaccid paralysis of the upper extremities caused by symmetric “watershed” brain infarction after cardiovascular arrest or cardiac surgery. However, it is now evident that this syndrome can occur in acute and chronic diseases of the upper and lower motor neuron. We describe a 48-year-old woman with rapid and severe progressive weakness and wasting of both upper extremities mimicking the “man-in-the-barrel” syndrome. The neurologic and electrophysiological findings were compatible with anterior horn cell disease affecting the cervical spinal cord segments. There were only minimal signs suggesting the presence of myelopathy. After the initial rapid onset, progression during the 7 years of follow up was minimal. An extensive etiologic workup disclosed infection with HTLV-1.
Magnetic Resonance Imaging and Computed Tomography of Skeletal Muscles in Oculopharyngeal Muscular Dystrophy
Authors: King Molly K , Lee Roland R , Davis Larry E 
Published: February 2005

Abstract

Objective:
To determine the spectrum of skeletal muscle involvement by neuroimaging in patients with oculopharyngeal muscular dystrophy (OPMD).

Methods:
A neuroradiologist, blinded to severity of patients' dystrophy, retrospectively read 22 magnetic resonance or computed tomography images of the head, neck, chest, abdomen, pelvis, and proximal leg from 13 patients with OPMD. Imaged muscles were evaluated for degree of fatty infiltration and bulk in a semiquantitative fashion using similar images from age-matched patients lacking neuromuscular disease. Neurologic examinations were performed without knowledge of imaging results.

Results:
We found abnormal tongue fatty infiltration in all patients and widespread infiltration in many other muscles. Over 50% of masseter, posterior neck, shoulder girdle, lumbar paraspinous, and gluteus muscles had abnormal fat.

Conclusion:
Although patients with OPMD have dysphagia with weak lids and proximal limbs, they have neuroimaging evidence of widespread fatty infiltration of many muscles. Excessive fatty infiltration of the tongue may help identify atypical patients with OPMD.
The Electromyographic Features of Acute Rhabdomyolysis
Authors: Al-Shekhlee Amer , Hachwi Rami , Jaberi Mohamed M , Katirji Bashar 
Published: February 2005

Abstract

Rhabdomyolysis and acute polymyositis share a similar clinical profile with elevated serum creatine kinase (CK). Unlike polymyositis, the electromyography (EMG) findings of acute rhabdomyolysis with myoglobinuria are not well defined. We retrospectively evaluated 15 patients during the acute phase of rhabdomyolysis. All patients (age range, 10-84 years) underwent electrodiagnostic studies, including needle EMG, during the first 2 weeks from the onset of symptoms. All patients presented with acute myalgia, muscle tenderness, and severe generalized weakness. The median peak CK was 48.0 k u/L (25th and 75th percentiles, 11.0 and 68.0 k u/L), whereas the median CK elevation at the time of EMG examination was 10.0 k u/L (25th and 75th percentiles, 4.5 and 48.5 k u/L). Nerve conduction studies were normal in all except 1 patient who had a preexisting mild polyneuropathy. Needle EMG was performed on 117 muscles. Eighty-seven of 117 (74%) muscles were normal, and 19 of 117 (16%) showed variable proximal myopathic motor unit action potentials. None had persistent fibrillation potentials. One third of patients (5 of 15) had myopathic EMG that was present in 1 to 3 sampled proximal muscles. We conclude that the EMG findings during rhabdomyolysis are often normal and when abnormal, the changes are subtle and are in contrast to the often-prominent EMG findings in polymyositis and dermatomyositis.
Topical Nitric Oxide Treatment after Pull Through Operations for Hirschsprung Disease
Authors: Tiryaki Tuğrul , Demirbağ Suzi , Atayurt Halil , Çetinkurşun Salih 
Published: February 2005

ABSTRACTAim:The diagnosis and treatment of Hirschsprung disease are well standardized. Symptoms of obstruction after surgery for Hirschsprung disease may result from residual spasticity of the internal anal sphincter. Nitric oxide (NO) is the chemical messenger mediating relaxation of the internal anal sphincter and its exogenous application results in a relaxation response in smooth muscle. The purpose of this study was to investigate topical NO application for obstructive symptoms after surgery for Hirschsprung disease and to correlate the symptoms with manometric findings with a view to directing further management.Materials and Methods:The authors reviewed application of topical NO on six symptomatic children who were operated for Hirschsprung disease. Eighteen patients older than 3 years of age were evaluated for anorectal manometric, functional and clinical outcome. The symptoms included enterocolitis in three patients and constipation in three patients. NO ointment was applied twice daily for 6 weeks in symptomatic patients and manometry was repeated.Results:Anorectal manometric evaluation of six patients showed high internal anal sphincter tone without reflex relaxation on applying distending pressure to the rectum. Marked improvement of symptoms was noted after 6‐week application of topical NO and maximal internal anal resting pressure decreased significantly (35% reduction). After ceasing application of topical ointment, increased maximal anal resting pressure was seen again.Conclusion:Although anal sphincter hypertonicity is not thought to be the only cause of post‐operative obstructive symptoms, relaxation of the internal anal sphincter may improve the symptoms. Topical NO can be used for treatment of obstructive symptoms in Hirschsprung disease. We managed our symptomatic patients successfully with local NO application with a reversible chemical sphincterotomy. NO could be used as a therapeutic modality.
Multidisciplinary Management of Surgical Disorders of the Pancreas in Childhood
Authors: Stringer Mark D , Davison Suzanne M , McClean Paddy , Rajwal Sanjay , Puntis John W. L , Sheridan Maria , Ramsden William , Woodley Helen 
Published: February 2005

ABSTRACTObjectives:To describe the frequency and range of pancreatic disorders in children requiring surgical intervention and to highlight the importance of multidisciplinary management.Methods:An audit of all children under 17 years of age referred with surgical disorders of the pancreas or pancreatitis to a regional pediatric gastroenterology unit in the United Kingdom during a 10‐year period. A retrospective chart review of clinical features, pathology and outcome was undertaken.Results:Surgical intervention was required for the following pancreatic disorders: persistent hyperinsulinemic hypoglycemia of infancy (n = 4), pancreatic tumors (n = 5), pancreaticobiliary malunion (n = 12), pancreatic trauma (n = 6) and pancreatitis (n = 10). The indications for surgery in acute pancreatitis were a persistent pseudocyst (n = 1) and treatment of an underlying cause of pancreatitis (n = 4); in chronic pancreatitis, surgery was used to treat symptomatic pancreatic duct strictures (n = 4). One child died of a progressive lymphoma but all others who underwent surgery are alive and well. All 33 children with acute pancreatitis, including four with pancreatic necrosis, survived.Conclusions:Surgery for pancreatic disorders in children is rarely required but may be necessary a) for definitive management of primary pancreatic pathology, b) to treat sequelae of acute or chronic pancreatitis and c) to treat an underlying cause of pancreatitis. There is a broad spectrum of potential pathologies. These patients are best managed by a multidisciplinary team approach.
Infected and Noninfected Ascites in Pediatric Patients
Authors: Vieira Sandra M. G , Matte Ursula , Kieling Carlos O , Barth Afonso L , Ferreira Cristina T , Souza Aline F , Taniguchi Adriano , da Silveira Themis R 
Published: February 2005

ABSTRACTObjectives:To determine the prevalence of spontaneous bacterial peritonitis, ascites with bacterial infection and noninfected ascites in pediatric patients with portal hypertensive ascites and to compare the clinical and laboratory features of infected and noninfected ascites.Methods:Forty‐one episodes of portal hypertensive ascites (serum‐ascites albumin gradient >1.1 g/dL) in 31 patients were studied. Median age was 2.9 years. Twenty‐four (77.4%) patients were cirrhotic and 20 (83.3%) were classified as Child‐Pugh C. Median pediatric end‐stage liver disease score was 18.5. The following ascites features were assessed: polymorphonuclear neutrophil cell count, cytology, pH, concentration of glucose, lactic dehydrogenase, total protein and albumin, Gram stain and bacteriological culture. Blood was sampled for complete blood count, coagulation studies, liver and renal function tests. Groups were compared by Mann‐Whitney and χ2 tests (P < 0.05).Results:Noninfected ascites were observed in 29 of 41 samples, spontaneous bacterial peritonitis in eight of 41 and ascites with bacterial infection in four of 41. The most prevalent clinical features were fever, voluminous ascites and encephalopathy, but there were no significant differences in the clinical features of the groups. All patients with infected ascites were cirrhotic. There was no statistical difference in Child‐Pugh or pediatric end‐stage liver disease status between patients with infected and noninfected ascites. Culture of ascetic fluid was positive in four of eight cases of spontaneous bacterial peritonitis. Gram‐negative rods were the most prevalent bacteria cultured. Except for serum albumin, no statistical differences in biochemical markers were observed between patients with infected and noninfected ascites.Conclusions:The prevalence of infected ascites was 29.2%. With the exception of serum albumin, there were no differences in the clinical and biochemical features of patients with infected ascites and noninfected ascites.
Helicobacter pylori Status and Symptom Assessment Two Years after Eradication in Pediatric Patients from a High Prevalence Area
Authors: Magistà Anna Maria , Ierardi Enzo , Castellaneta Stefania , Miniello Vito Leonardo , Lionetti Elena , Francavilla Antonio , Ros Philippe , Rigillo Nicola , Di Leo Alfredo , Francavilla Ruggiero 
Published: February 2005

ABSTRACTObjectives:To establish the rate of Helicobacter pylori reinfection in children from an H. pylori high prevalence area, possible clinical features predictive of reinfection and the usefulness of re‐treatment.Methods:65 consecutive children attending the authors' department between 1998 and 2000 who had proven successful H. pylori eradication were enrolled; 52 took part. Patients and family members were invited to undergo 13C‐urea breath testing and to complete a simple questionnaire regarding symptoms and socioeconomic status. Patients with H. pylori reinfection were offered treatment; eradication was assessed by 13C‐urea breath test 8 weeks after completion of treatment.Results:Of 52 children, 15 (28.8%) were H. pylori positive. Variables predictive of reinfection were age at primary infection and presence of an infected sibling. Although reinfected children were more frequently symptomatic than non‐reinfected patients, no specific symptom was associated with reinfection. Of the nine re‐treated patients who returned 8 weeks after completing therapy, the bacterium was eradicated in five (56%).Conclusions:The 12.8% per year reinfection rate in childhood at 2 years that we observed should prompt a re‐evaluation of H. pylori status even after a successful eradication. Living in an H. pylori high prevalence area increases the annual risk of reinfection by approximately fourfold over the annual risk in H. pylori low prevalence areas.
Chronic Abdominal Pain in Children: A Clinical Report of the American Academy of Pediatrics and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition
Authors: Di Lorenzo Carlo , Colletti Richard B , Lehmann Harold P , Boyle John T , Gerson William T , Hyams Jeffrey S , Squires Robert H , Walker Lynn S , Kanda Pamela T 
Published: February 2005

ABSTRACTChildren and adolescents with chronic abdominal pain pose unique challenges to their caregivers. Affected children and their families experience distress and anxiety that can interfere with their ability to perform regular daily activities. Although chronic abdominal pain in children is usually attributable to a functional disorder rather than to organic disease, numerous misconceptions, insufficient knowledge among health care professionals and inadequate application of knowledge may contribute to a lack of effective management. This clinical report accompanies a technical report on childhood chronic abdominal pain and provides guidance for the clinician in the evaluation and treatment of children with chronic abdominal pain. The conclusions are based on the evidence reviewed in the technical report and on consensus achieved among subcommittee members.
Effects of Anticonvulsants on Human P450c17 (17α‐Hydroxylase/17,20 Lyase) and 3β‐Hydroxysteroid Dehydrogenase Type 2
Authors: Flück Christa E. , Yaworsky Dustin C. , Miller Walter L. 
Published: February 2005

Summary:  Purpose: Women with epilepsy apparently have a higher incidence of polycystic ovary syndrome (PCOS) than do women without epilepsy. Whether the underlying disease or the antiepileptic drug (AED) treatment is responsible for this increased risk is unknown, although clinical reports implicate valproic acid (VPA) as a potential cause. The steroidogenic enzymes 3βHSDII (3β‐hydroxysteroid dehydrogenase) and P450c17 (17α‐hydroxylase/17,20 lyase) are essential for C19 steroid biosynthesis, which is enhanced during adrenarche and in PCOS. Methods: To determine whether the AEDs VPA, carbamazepine (CBZ), topiramate (TPM), or lamotrigine (LYG) directly affect the activities of human 3βHSDII and P450c17, we added them to yeast expressing human P450c17 or 3βHSDII and assayed enzymatic activities in the microsomal fraction. Results: Concentrations of VPA ≤10 mM had no effect on activities of P450c17; however, VPA inhibited 3βHSDII activity starting at 0.3 mM (reference serum unbound concentration, 0.035–0.1 mM) with an IC50 of 10.1 mM. CBZ, TPM, and LTG did not influence 3βHSDII or P450c17 activities at typical reference serum unbound concentrations, but did inhibit 3βHSDII and P450c17 at concentrations >10‐fold higher. Conclusions: None of the tested AEDs influenced 3βHSDII or P450c17 activities at concentrations normally used in AED therapy. However, VPA started to inhibit 3βHSDII activity at concentrations 3 times above the typical reference serum unbound concentration. Because inhibition of 3βHSDII activity will shift steroidogenesis toward C19 steroid production when P450c17 activities are unchanged, very high doses of VPA may promote C19 steroid biosynthesis, thus resembling PCOS. CBZ, TPM, and LTG influenced 3βHSDII and P450c17 only at toxic concentrations.
Proteomic Identification of the Involvement of the Mitochondrial Rieske Protein in Epilepsy
Authors: Junker Heike , Späte Kira , Suofu Yalikun , Walther Reinhard , Schwarz Gerold , Kammer Winfried , Nordheim Alfred , Walker Lary C. , Runge Uwe , Kessler Christof , Popa‐Wagner Aurel 
Published: February 2005

Summary: Purpose:Kindled seizures are widely used to model epileptogenesis, but the molecular mechanisms underlying the attainment of kindling status are largely unknown. Recently we showed that achievement of kindling status in the Sprague–Dawley rat is associated with a critical developmental interval of 25 ± 1 days; the identification of this long, well‐defined developmental interval for inducing kindling status makes possible a dissection of the cellular and genetic events underlying this phenomenon and its relation to normal and pathologic brain function.Methods:By using proteomics on cerebral tissue from our new rat kindling model, we undertook a global analysis of protein expression in kindled animals. Some of the identified proteins were further investigated by using immunohistochemistry.Results:We report the identification of a modified variant of the Rieske iron‐sulfur protein, a component of the mitochondrial cytochromebc1complex, whose isoelectric point is shifted toward more alkaline values in the hippocampus of kindled rats. By immunohistochemistry, the Rieske protein is well expressed in the hippocampus, except in the CA1 subfield, an area of selective vulnerability to seizures in humans and animal models. We also noted an asymmetric, selective expression of the Rieske protein in the subgranular neurons of the dorsal dentate gyrus, a region implicated in neurogenesis.Conclusions:These results indicate that the Rieske protein may play a role in the response of neurons to seizure activity and could give important new insights into the molecular pathogenesis of epilepsy.
Removing Eye‐movement Artifacts from the EEG during the Intracarotid Amobarbital Procedure
Authors: Zhou Weidong , Gotman Jean 
Published: February 2005

Summary:  Purpose: The EEG is often recorded during the intracarotid amobarbital procedure (IAP) to help in the assessment of the spatial extent and the duration of the effect of the drug. In scalp recordings, the EEG is always heavily contaminated with eye movement artifacts as the patient actively performs visual tasks. Methods: Independent component analysis (ICA) is a new technique for blind source separation. In this study, we separated the EEG data recorded during the IAP into independent components using ICA. The EEG signal was reconstructed by excluding the components related to eye movement and eye blinks. Results: EEGs from 10 IAP tests were analyzed. The experimental results indicate that ICA is very efficient at subtracting eye‐movement artifacts, while retaining the EEG slow waves and making their interpretation easier. Conclusions: ICA appears to be a generally applicable and effective method for removing ocular artifacts from EEG recordings during IAP, although slow waves and ocular artifacts share similar frequency distributions.
Pharmacokinetic and Metabolic Investigation of Topiramate Disposition in Healthy Subjects in the Absence and in the Presence of Enzyme Induction by Carbamazepine
Authors: Britzi Malka , Perucca Emilio , Soback Stefan , Levy René H. , Fattore Cinzia , Crema Francesca , Gatti Giuliana , Doose Dennis R. , Maryanoff Bruce E. , Bialer Meir 
Published: February 2005

Summary:  Purpose: To characterize the metabolic profile of topiramate (TPM) in humans and to assess the influence of enzyme induction by carbamazepine (CBZ) on the pharmacokinetics and metabolic profile of TPM. Methods: Twelve healthy subjects received a single oral dose of TPM (200 mg) on two randomized occasions. On one occasion, TPM was administered alone, and on the other, it was given on day 18 of a 24‐day treatment with CBZ (maintenance dosage, 600 mg/day). Blood and urine samples were collected for ≥72 h after dosing. TPM and its metabolites were assayed in plasma and urine by a specific liquid chromatography–mass spectroscopy (LC‐MS) method. Results: Mean TPM oral clearance (CL/F) increased from 1.2 L/h (control) to 2.2 L/h after CBZ treatment. Mean TPM half‐life decreased from 29 h to 19 h. TPM was excreted extensively in urine both under noninduced (56%) and CBZ‐induced conditions (40%). 2,3‐O‐Des‐isopropylidene‐TPM (2,3‐diol‐TPM) was identified as the most prominent urinary metabolite, with a recovery accounting for 3.2% and 7.9% of the TPM dose under noninduced and induced conditions, respectively. Corresponding recovery values for 10‐hydroxy‐TPM (10‐OH‐TPM) were 1.2% and 1.8%, respectively. The control AUCmetabolite/AUCdrug ratio for 2,3‐diol‐TPM and 10‐OH‐TPM were 1.5% and 0.6%, and they increased by threefold and twofold, respectively, after CBZ treatment. Conclusions: TPM remains appreciably excreted unchanged in urine (41%) under CBZ‐induced conditions, even though TPM CL/F increased by twofold. Although 2,3‐diol‐TPM and 10‐OH‐TPM were measured in unconjugated form, the significant increases in their AUC and urinary excretion are consistent with the twofold increase in TPM clearance.
Characterizing the Role of Design in New Product Development: An Empirically Derived Taxonomy*
Authors: Perks Helen , Cooper Rachel , Jones Cassie 
Published: February 2005

This article empirically explores the nature of the role of design in the new product development process. The investigation adopts a multiple case study methodology. Data were collected through a six‐month interview program carried out with mid‐size to large U.K. manufacturing companies. The researchers articulate the scope and detailed nature of actions undertaken by design across all phases of the new product development process. Design functional, integration, and leadership actions are unraveled from the data. A taxonomy characterizing three roles for design in new product development is developed and explained. In the first role, design is explored as a functional specialism.The second categorization develops the role of design as part of a multifunctional team. The third role depicts the designer as process leader. Detailed actions and skills associated with each role are discussed and illustrated. Contextual factors explaining and influencing each design role are unraveled. These are articulated as speed of development process, innovativeness of the product development effort, and use of external design agencies. The implications of these findings for the development of design skills and capabilities are discussed in terms of recruitment, training, and educational policies.