bluuebunny/crossref_metadata_2025_split huggingface dataset:
Transplants of Cells Engineered to Produce GABA Suppress Spontaneous Seizures
Authors: Thompson Kerry W. , Suchomelova Lucie M. 
Published: December 2003

Summary:  Purpose: Cell transplantation into the brain is an aggressive clinical alternative. The hopes of treating diseases like intractable temporal lobe epilepsy have been subdued because the preclinical successes thus far have shown only slowing of epileptogenesis, or suppression of electrically induced seizures. Because the hallmark of epilepsy is spontaneous seizures, the clinical relevance of these studies has been questioned. The purpose of this study was to establish that cells genetically engineered to produce γ‐aminobutyric acid (GABA) could suppress spontaneous seizures in an accepted model of temporal lobe epilepsy. Methods: Conditionally immortalized neurons were engineered to produce GABA under the control of tetracycline. These cells were transplanted into the substantia nigra of spontaneously seizing animals. After transplantation, the animals were monitored for 3 days immediately after surgery and again for 3 days beginning 7–8 days after surgery. Seizures and epileptiform spikes were recorded and later analyzed with detection software combined with video monitoring. Results: Animals that received genetically engineered GABA‐producing cells had significantly fewer spontaneous seizures than did animals that received control cells, or animals that received GABA‐producing cells plus doxycycline at the observation period starting 1 week after transplantation. A significant suppression of epileptiform spikes also was noted between the group that received GABA‐producing cells and the group that received the same cells but were given doxycycline. The engineered cells show evidence of integration with the host but limited survival. Conclusions: These data demonstrate that genetically engineered cells have the ability to suppress spontaneous seizures when transplanted into seizure‐modulating nuclei. This is an important step toward defining a clinical potential for this approach in epilepsy. The fact that the gene of interest can be regulated suggests that individualizing transplant therapy may be possible.
The Relative Influence of Epileptic EEG Discharges, Short Nonconvulsive Seizures, and Type of Epilepsy on Cognitive Function
Authors: Aldenkamp Albert , Arends Johan 
Published: December 2003

Summary:  Purpose: This study addressed whether cognitive impairment in children with epilepsy is caused by disease‐related stable factors, such as the type of epilepsy, or by acute effects of paroxysmal epileptic activity such as epileptic EEG discharges. We studied a nonselected group with short nonconvulsive seizures, as these seizures may elude detection and may therefore persist over a longer period. In this group, the diagnostic issue is to differentiate between the combined effects of several epilepsy‐related factors on cognition. Methods: All children were assessed with 32‐channel EEG, synchronized with a computerized cognitive test system and a video‐monitoring system. Recording time was 2 h. The primary inclusion criteria were unclear seizures and fluctuations in cognitive performance and/or frequent epileptic EEG discharges in a recent EEG. Results: One hundred fifty‐two patients met the inclusion criteria; 31 patients appeared not to have a diagnosis of epilepsy and were used as a nonepilepsy control group. Our results show that type of epilepsy has an impact on stable cognitive functions, such as educational achievement. Paroxysmal epileptic activity (acute effects of seizures and epileptic EEG discharges) affects primarily transient mechanistic cognitive processes (alertness, mental speed). Conclusions: These results suggest that the effects of paroxysmal epileptic activity on transient cognitive mechanisms may accumulate over time and consequently affect the more stable aspects of cognitive function such as educational achievement. The clinical relevance is that early detection of the cognitive impact of seizure‐related activity and subsequent treatment may prevent its detrimental impact on cognitive and educational development.
Depression and Altered Quality of Life in Women with Epilepsy of Childbearing Age
Authors: Beghi Ettore , Roncolato Maurizio , Visonà Giovanni 
Published: December 2003

Summary:  Purpose: To calculate the prevalence of depression in a referral population of women of childbearing age, to define the factors associated with depression, and to assess health‐related quality of life (HRQOL) in the same population. Methods: The 642 consecutive women with epilepsy aged 18–55 years were enrolled by 40 neurologists over an 8‐month period and asked to give details on selected demographic and clinical features regarding the disease, any associated clinical condition, and any drug treatment. Depression was diagnosed by using the Hamilton depression scale and HRQOL was measured through the SF‐36 form. Demographic, clinical, and therapeutic risk factors for depression were searched for within the study population. Results: Depression (any severity) was present at interview in 242 women, giving a prevalence rate of 37.7%[95% confidence interval (CI), 33.9–41.6]. Mild depression was reported by 18.5% of women, moderate depression by 8.6%, major depression by 10.3%, and severe depression by 0.3%. Factors found to be independently associated with depression (any severity) included treatment of associated conditions [relative risk (RR), 1.5; 95% CI, 1.2–1.8), concurrent disability (RR, 1.3; 95% CI, 1.0–1.6), seizures in the preceding 6 months (RR, 1.4; 95% CI, 1.1–1.7), and being unemployed or a housewife (RR, 1.3; 95% CI, 1.0–1.5). Factors associated with moderate to severe depression included treatment for associated conditions (RR, 2.0; 95% CI, 1.4–2.7), seizures in the preceding 6 months (RR, 1.7; 95% CI, 1.2–2.5), and being unemployed or a housewife (RR, 1.6; 95% CI, 1.1–2.2). Compared with normal women of similar age, patients with epilepsy tended to present lower scores for each HRQOL domain (mostly Role Physical, General Health, Social Functioning, and Role Emotional). However, when the analysis was limited to nondepressed women with epilepsy, any difference disappeared. Conclusions: Women with epilepsy of childbearing age are at high risk of depression. Factors associated with depression include lack of occupation, the presence of an underlying disabling condition (with treatment), and the severity of epilepsy. Compared with the general population, depressed women have greater impairment of HRQOL with epilepsy, which reflects the physical, social, and emotional implications of the disease.
The Role of Individualism and the Five‐Factor Model in the Prediction of Performance in a Leaderless Group Discussion
Authors: Waldman David A. , Atwater Leanne E. , Davidson Ronald A. 
Published: December 2003

AbstractPersonality has seen a resurgence in the work performance literature. The Five‐Factor Model (FFM) represents a set of personality factors that has received the most attention in recent years. Despite its popularity, the FFM may not be sufficiently comprehensive to account for relevant variation across performance dimensions or tasks. Accordingly, the present study also considers how individualism may predict additional variance in performance beyond the FFM. The study involved 152 undergraduate students who experienced a leaderless group discussion (LGD) exercise. Results showed that while the FFM accounted for variance in students' LGD performance, individualism (independence) accounted for additional, unique variance. Furthermore, analyses of the group compositions revealed curvilinear relationships between the relative amount of extraversion, conscientiousness, and individualism in relation to group‐level performance.
The Emotional Costs of Parents' Conditional Regard: A Self‐Determination Theory Analysis
Authors: Assor Avi , Roth Guy , Deci Edward L. 
Published: December 2003

AbstractParents' use of conditional regard as a socializing practice was hypothesized to predict their children's introjected internalization (indexed by a sense of internal compulsion), resentment toward parents, and ill‐being. In Study 1, involving three generations, mothers' reports of their parents' having used conditional regard to promote academic achievement predicted (a) the mothers' poor well‐being and controlling parenting attitudes, and (b) their collge‐aged daughters' viewing them as having used conditional regard, thus showing both negative affective consequences from and intergenerational transmission of conditional regard. Study 2 expanded on the first by using four domains, including both genders, and examining mediating processes. College students' perceptions of their mothers' and fathers' having used conditional regard in four domains (emotion control, prosocial, academic, sport) were found to relate to introjected internalization, behavioral enactment, fluctuations in self‐esteem, perceived parental disapproval, and resentment of parents. Introjection mediated the link from conditional regard to behavioral enactment. The results suggest that use of conditional regard as a socializing practice can promote enactment of the desired behaviors but does so with significant affective costs.
Lichenoid graft vs. host disease following liver transplantation
Authors: Walling Hobart W. , Voigt Michael D. , Stone Mary Seabury 
Published: December 2003

Background: Graft vs. host disease (GVHD) is a common complication of bone marrow transplantation (BMT) but is rarely seen after solid organ transplantation.Methods: We report a case of lichenoid GVDH arising in a 60‐year‐old man 10 weeks after orthotopic liver transplantation.Results: Skin biopsies revealed changes suggestive of lichenoid GVHD, but histological features differed from those described in post bone marrow (stem cell) transplant GVHD, in that a dense lymphoid infiltrate was present. The brisk infiltrate contained eosinophils that initially led to concern for a lichenoid drug eruption. The patient developed multiorgan GVHD after reduction in immunosuppression. The diagnosis of chronic GVHD was confirmed by the demonstration of chimerism in the patient's peripheral blood. The generalized cutaneous eruption and systemic manifestations responded to salvage therapy including intravenous immunoglobulin infusion, increased immunosuppression with high‐dose steroids, mycophenolate mofetil, and systemic and topical tacrolimus.Conclusions: In interpreting skin biopsies, it is important to recognize that brisk inflammation may be seen in GVHD in the setting of solid organ transplantation, in contrast to the more sparse inflammation typical of GVHD following BMT. The clinical and histologic differential diagnosis included the eruption of lymphocyte recovery, drug reaction, and viral exanthem. We provide a conceptual framework for evaluating generalized cutaneous changes that may occur post transplantation.
The diagnostic utility of p63, CK5/6, CK 7, and CK 20 in distinguishing primary cutaneous adnexal neoplasms from metastatic carcinomas
Authors: Qureshi Hina S. , Ormsby Adrian H. , Lee Min W. , Zarbo Richard J. , Ma Chan K. 
Published: December 2003

Background:  Distinguishing primary cutaneous adnexal neoplasms (PCANs) from metastatic carcinomas (MCs) can be difficult. We study the utility of p63, CK 5/6, CK 7, and 20 expression in PCAN vs. MC.Methods:  Twenty‐one PCAN with sweat gland differentiation (six benign, 15 malignant), one sebaceous carcinoma, and 15 MC (14 adenocarcinomas, one urothelial carcinoma) to skin were retrieved from the pathology files. Immunostains for p63, CK 5/6, CK 7, and CK 20 were performed and graded as follows: 1, <10; 2, 11–50; and 3 >50% of tumor cells stained.Results:  Twenty of 22 PCAN expressed p63 and CK 5/6. Four of 15 and two of 15 MC were positive for CK 5/6 and p63, respectively. Thirteen of 22 PCAN and 13 of 15 MC were positive for CK 7, respectively. All PCAN were negative for CK 20, two of 15 MC were positive. The sensitivity and specificity for the diagnosis of PCAN were 91 and 73% for CK 5/6, 91 and 100% for p63, and 60 and 13% for CK 7, respectively.Conclusions:  For distinguishing PCAN from MC: (1) positivity for p63 and CK 5/6 are relatively specific and sensitive for PCAN, (2) CK 7 and 20 are neither sensitive nor specific, and (3) CK 7 positivity in PCAN was focal with a specific pattern in contrast to the diffuse positivity for MC.
Cellular nodules in congenital pattern nevi
Authors: Xu Xiaowei , Bellucci Kirsten S. W. , Elenitsas Rosalie , Elder David E. 
Published: December 2003

Background:  The diagnosis of ‘cellular dermal nodule in melanocytic nevi’ is challenging, as it can simulate invasive and tumorigenic melanoma. Because cellular nodules are presumed benign, it is essential to differentiate them from true malignant melanoma arising within a nevus, which portends a poor prognosis. Although presumed benign, to date, no published long‐term follow‐up studies have proved the benignity of this lesion.Methods:  To better understand the clinical behavior and histological features of these lesions, we present a clinical pathological study of a cohort of 26 cases, for which we obtained clinical follow up by chart review and questionnaires.Results:  Clinical follow up was obtained on 16 out of 26 patients (61.5%) and ranged from 12 to 132 months (average 62.3 months). The cellular nodules in congenital pattern nevi, reported here, had an invariably benign clinical course.Conclusions:  Features useful in differentiating cellular nodule from melanoma include: (1) lack of high‐grade uniform cellular atypia; (2) lack of necrosis within the nodule; (3) rarity of mitoses; (4) evidence of maturation in the form of blending or transitional forms between the cells in the nodule and the adjacent nevus cells; (5) lack of pagetoid spread into the overlying epidermis; and (6) no destructive expansile growth. The difference between cellular nodule and melanoma arising from nevus can be very subtle, and it is important to emphasize that these lesions must be assessed on an individual case‐by‐case basis.
Melanocytic nevi of the breast: a histologic case‐control study
Authors: Rongioletti F. , Urso C. , Batolo D. , Chimenti S. , Fanti P. A. , Filotico R. , Gianotti R. , Innocenzi D. , Lentini M. , Tomasini C. , Pippione M. , Rebora A. 
Published: December 2003

Background:  Melanocytic nevi in the genital, acral, and flexural sites often display clinical and histologic features that may simulate melanoma. We verified whether this is the case also for nevi of the breast.Methods:  Eleven dermatopathologists, from nine Italian Institutions, collected the specimens of melanocytic lesions from the breast and other body sites, excluding the acral, genital, and flexural areas, as controls. Cases and controls were matched for sex and age. All nevi were observed ‘blindly’ and simultaneously by all participants. For each lesion, 10 histological parameters were analyzed: asymmetry, absence of lateral demarcation of melanocytes, lentiginous proliferation, nested and dyshesive pattern, intraepidermal melanocytes above the basal layer, involvement of the hair follicle, absence of maturation of dermal melanocytes, melanocytic atypia, fibroplasia of the papillary dermis, and lymphocytic dermal infiltrate. Each parameter was scored 2 when present and 1 when absent or not valuable. A total score was calculated for each lesion. Results were statistically analyzed by the chi‐square test and the Mann–Whitney U‐test.Results:  One hundred and one nevi came from the breast area and 97 from elsewhere. Breast nevi exhibited significantly more atypical features than nevi from other sites. In particular, breast nevi with intraepidermal melanocytes, melanocytic atypia, and dermal fibroplasia were significantly more numerous. We did not find any sexual difference.Conclusions:  To avoid undue concerns, dermatopathologists should be aware that melanocytic nevi of the breast may show a high degree of atypical features.
Benign ectopic thyroid tissue in a cutaneous location: a case report and review
Authors: Maino Kim , Skelton Henry , Yeager Josef , Smith Kathleen J. 
Published: December 2003

Background:  For many years, lateral, aberrant thyroid tissue in adults was a term used almost exclusively for metastatic thyroid carcinoma. However, aberrant, benign ectopic thyroid tissue does occur, and it is most commonly found as a part of the evaluation of endocrine dysfunction. Rarely, aberrant, benign ectopic thyroid presents as a primary mass.Case report:  We present a 35‐year‐old female who presented for removal of a lifelong posterior lateral neck nodule.Results:  Histologic examination and immunohistochemical studies confirmed the presence of aberrant, benign ectopic thyroid tissue. The patient had no endocrine problems, and she had a normally located and functioning thyroid gland.Conclusions:  This case illustrates that not all aberrant thyroid tissues in adults are malignant or associated with endocrine disorders. This case also illustrates the rare association of ectopic thyroid and a normally located and functioning thyroid gland. In this patient, a somatic mutation in a transcription factor important in thyroid migration could explain these findings.
Verrucous carcinoma of the axilla: case report and review
Authors: Assaf Chalid , Steinhoff Matthias , Petrov Ivailo , Geilen Christoph C. , De Villiers Ethel‐Michele , Schultz‐Ehrenburg Ulrich , Orfanos Constantin E. 
Published: December 2003

Background:  Verrucous carcinoma, a variant of squamous cell carcinoma, is distinct from squamous cell carcinoma in morphology and behavior. It preferentially occurs on the oropharyngeal mucosa, the urogenital mucosa, and the soles. In contrast to its malignant clinical picture, the tumor grows locally invasive but is histologically benign and metastasizes rarely.Methods:  We report the uncommon occurrence of a large verrucous carcinoma on apparently uninvolved skin in the right axilla in a 47‐year‐old male.Results:  Histologic examination reveals a cauliflower‐like tumor consisting of deep invaginated epidermal proliferation with rabbit burrow‐like, keratin‐filled sinus formations; the basement membrane, however, remains intact. Immunohistology showed positivity for pancytokeratin (KL‐1) and cytokeratin (CK) 18 and negativity for CK7, and assessment of the proliferative activity of the tumor cells revealed low percentage of Ki‐67 expression. Furthermore, there were only scattered cells expressing p53 or bcl‐2. Polymerase chain reaction excluded the presence of human papillomavirus. After complete excision, no signs of recurrence occurred over a follow‐up period of three years.Conclusion:  Verrucous carcinoma should be distinguished from typical squamous cell carcinoma. The clinicopathological features, differential diagnosis, and therapy are discussed here together with the molecular biologic aspects of the tumor.
Tumefactive lipedema with pseudoxanthoma elasticum‐like microscopic changes
Authors: Taylor Nancy E. , Foster William C. , Wick Mark R. , Patterson James W. 
Published: December 2003

Background:  Lipedema is a condition characterized by diffuse, bilaterally symmetrical, painful swelling of the legs and buttocks. Microscopically, there are dermal and septal edema, adipocyte degeneration, and numerous mast cells, features held in common with lipedematous alopecia.Case report:  We present the case of a 60‐year‐old woman with a long history of bilateral leg masses with microscopic features of lipedema. In addition, elastic‐fiber changes typical of pseudoxanthoma elasticum (PXE) were discovered within the subcutaneous septa in three separate specimens obtained from an affected extremity. The patient did not have other clinical findings of PXE, although there was a history of both hypertension and congestive heart failure.Conclusion:  This tumefactive presentation of lipedema has not been previously described. Regarding the elastic‐tissue abnormalities, the patient could have either a subclinical form of PXE, perhaps predisposing to lipedema, or secondary elastic‐tissue changes resulting from the massive edema. If the latter is the case, then this could represent an unusual manifestation of localized acquired cutaneous PXE (calcific elastosis).
Diagnostic, prognostic and pathogenic value of the direct immunofluorescence test in cutaneous leukocytoclastic vasculitis
Authors: Barnadas María A. , Pérez Eugenia , Gich Ignasi , Llobet José M. , Ballarín José , Calero Francesca , Facundo Carmen , Alomar Agustín 
Published: December 2003

AbstractBackground    No precise studies have been performed on cutaneous leukocytoclastic vasculitis (LV) to establish whether it is better to obtain a skin biopsy from lesional or from perilesional skin for direct immunofluorescence (DIF). There is no agreement on the immunoglobulins most frequently detected and the value of DIF for the classification of cutaneous vasculitis.Methods    A prospective study of DIF in lesional and perilesional skin was performed in 50 leukocytoclastic vasculitis patients and 15 nonvasculitis patients.Results   We detected a higher level of positivity in involved skin than in uninvolved skin for IgG, IgA, IgM, C3 and fibrinogen but not for C1q. In vasculitic patients, IgA was the immunoglobulin most frequently detected in lesional (82%) and perilesional skin (68%), followed by IgM (56 and 34%, respectively) and IgG (20 and 8%, respectively). Only IgA deposits were associated with the diagnosis of vasculitis, with a sensitivity of 82% in lesional and 68% in perilesional skin, and with a specificity of 73 and 66.7%, respectively. The presence of IgA in lesional skin was associated with renal involvement but there was no association with severity. The presence of IgG or IgM, or the absence of IgA in perilesional skin was related to the presence of cryoglobulins. The absence of IgA in lesional and perilesional skin was also related to hepatitis C virus infection.Conclusions   DIF findings in involved skin are more closely related to the diagnosis of vasculitis and can give more information about overall renal involvement than findings in uninvolved skin. However, findings in uninvolved skin are more closely related to the pathogenic factors that trigger the development of vasculitis.
Acute hemorrhagic edema of infancy: a case report
Authors: Da Silva Manzoni Ana Paula Dornelles , Viecili Josiane Burmann , De Andrade Cristiane Benvenuto , Kruse Ricardo Lapa , Bakos Lucio , Cestari Tania Ferreira 
Published: December 2003

AbstractBackground  Acute hemorrhagic edema of infancy (AHEI), or Seidlmayer's disease, is a type of leukocytoclastic vasculitis proper of infants and children. It is characterized by a local increase in temperature, erythematous edema and purpuric lesions involving mainly the face and extremities. There usually is no visceral involvement. The disease is self‐limited, bearing a benign clinical course. Infection, drugs and immunization have been considered as precipitating factors. The main differential diagnosis is Henoch‐Schönlein purpura (HSP).Methods  We describe a classic example of acute hemorrhagic edema of infancy, and comment on the clinical features, pathology, immunopathology and proposed therapy. We characterize the differences between AHEI and HSP.Results  A course of corticosteroids was given to avoid quick progression of disease.Conclusion  Acute hemorrhagic edema of infancy is a rare disease, and the most striking classic feature of the disease is the contrast between the acuteness of the cutaneous lesions and the good general condition of the patient. Considering its clinical features, pathology and immunopathology, AHEI can be justifiably characterized as a unique disorder, distinct from HSP.