Search Results - (Author, Cooperation:R. Feldmann)

2015-09-25

Nature Publishing Group (NPG)

0028-0836

1476-4687

Biology

Chemistry and Pharmacology

Medicine

Natural Sciences in General

Physics

1365-2133

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

We describe a 74-year-old woman who rapidly developed bilateral voluminous nodules on the upper eyelids, following 4 months’ treatment for chalazion. Histological and immunohistochemical findings were suggestive of cutaneous pseudolymphoma (CPL), and extensive screening for malignant lymphoma (ML) remained negative. Cutaneous pseudolymphomas are inflammatory diseases that can simulate ML either clinically, histopathologically, or both. They are a rare cause of nodules of the upper eyelids, usually characterized by a benign evolution, that can be secondary to ultraviolet sensitivity, adverse reactions to systemic medications and contact sensitization, among others. In our case, epicutaneous patch tests were strongly positive for a series of allergens contained in the ophthalmological preparations used; therefore, they were suggestive that the patient had CPL, lymphomatoid contact dermatitis type. In addition, the patient had been treated previously with several drugs, known to cause CPL and immune dysregulation. Complete regression of the lesions required treatment with systemic steroids and chlorambucil. No relapse occurred within a 5-year follow-up period.

Electronic Resource

1365-2133

Blackwell Publishing Journal Backfiles 1879-2005

Medicine

Electronic Resource

0022-5193

Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Biology

Electronic Resource

1420-9071

Springer Online Journal Archives 1860-2000

Biology

Medicine

Summary Following topical application of14C-labeled 1,3-bis(2-chloroethyl)-1-nitrosourea (BCNU, carmustine) to the skin of mice radioactivity was found in all viscera and tissues examined. Exclusive of the gut, highest values were recorded for the liver, kidney and lung.

Electronic Resource

0040-4039

Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Chemistry and Pharmacology

Electronic Resource

0040-4039

Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Chemistry and Pharmacology

Electronic Resource

0168-583X

Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Physics

Electronic Resource

1433-0490

Springer Online Journal Archives 1860-2000

Computer Science

Abstract We prove the existence of a Hamiltonian path in the Shuffle Exchange networkSX (n). This problem has been posed as an open problem by Leighton in [8] and Samatham and Pradhan in [11]. Its positive solution has several consequences showing the computational abilities of theSX(n).

Electronic Resource

1433-0474

Schlüsselwörter ; Barth-Syndrom ; X-gebundene Kardiomyopathie ; G4.5 ; Key words ; Barth syndrome ; X-linked cardiomyopathy

Springer Online Journal Archives 1860-2000

Medicine

Summary Barth syndrome is a rare X-linked disorder characterized by dilated cardiomyopathy, myopathy and neutropenia. We describe a family with two affected males. One of them died during infancy from cardiomyopathy. The other, now adult, is less severe affected. The diagnosis was confirmed by the detection of a missense mutation in the responsible gene G4.5. In the case of two further boys, who died during the neonatal period, the same diagnosis may be said to apply retrospectively, based on available results. Discussion: Although the Barth syndrome gene was cloned in 1996, the function of its protein product is not yet known. Because of the variability of the clinical course even in one and the same familiy genetic counceling and considerations about prognosis and treatment are difficult.

Zusammenfassung Das Barth-Syndrom ist eine seltene, X-chromosomal-rezessiv vererbte Erkrankung mit den Leitsymptomen dilatative Kardiomyopathie, Myopathie, rezidivierende Neutropenie. Es wird eine Familie beschrieben, in der 2 männliche Familienmitglieder am Barth-Syndrom erkrankt sind. Einer der Patienten verstarb im Säuglingsalter infolge der Kardiomyopathie, der andere zeigt eine weniger schwere Symptomatik und ist mittlerweile erwachsen. Bei beiden wurde die Diagnose durch den Nachweis einer Mutation im krankheitsverursachenden Gen G4.5 gesichert. Bei 2 weiteren, im Neugeborenenalter verstorbenen Jungen ist die gleiche Diagnose aufgrund der vorliegenden Befunde retrospektiv anzunehmen. Diskussion: Obwohl das krankheitsverursachende Gen 1996 kloniert wurde, ist die Funktion des Genprodukts noch weitgehend unbekannt. Das Barth-Syndrom zeigt auch innerhalb einer Familie eine große klinische Variabilität, was die genetische Beratung und Aussagen bezüglich der Prognose und Behandlung erschwert.

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1433-0407

Schlüsselwörter Schizophrenie ; Psychoedukation ; Angehörigengruppen ; Rezidivprophylaxe ; Langzeiteffekte ; Key words Schizophrenia ; Psychoeducation ; Keyperson counseling ; Relapse prevention ; Long-term effects

Springer Online Journal Archives 1860-2000

Medicine

Summary In this study we investigated whether, in conjunction with neuroleptics, a psychoeducational and cognitively oriented treatment for schizophrenic outpatients and their key-persons can improve the course of schizophrenic illness within a 2-year follow-up. This prospective randomized study covered a total of 191 schizophrenic patients (according to DSM-III-R) and comprised a psychoeducational training and cognitive psychotherapy for patients and counseling for their key persons in various combinations. Patients were examined before, immediately after and 2 years after the end of the intervention. Patients in the treatment groups reduced their overall psychopathology and their attention deficit. For patients receiving all three treatment conditions, there was a relevant preventive effect with regard to the rehospitalization rate appearing during the second year of the follow-up. We conclude that in the mid-term, a combination of psychoeducational and cognitively oriented therapy for patients and their keypersons can improve the course of schizophrenic illness.

Zusammenfassung Die Untersuchung beantwortet die Frage, ob eine ambulante psychoedukativ-psychotherapeutische Behandlung schizophrener Patienten und ihrer Angehörigen in Verbindung mit einer neuroleptischen Medikation in den 2 Jahren nach Abschluß der Therapie den Krankheitsverlauf der Patienten günstig beeinflussen kann. Es handelt sich um eine prospektive Studie im randomisierten Kontrollgruppendesign, in die 191 ambulant behandelte Schizophrene (DSM-III-R-Kriterien) aufgenommen wurden. Die Behandlungsbedingungen bestanden aus einem psychoedukativen Training und einer kognitiven Psychotherapie für die Patienten sowie aus einer Bezugspersonenberatung für deren Angehörige, jeweils in unterschiedlichen Kombinationen. Die Untersuchungen fanden vor Beginn, unmittelbar am Ende, sowie 12 und 24 Monate nach Therapieabschluß statt. In der Therapiegesamtgruppe verbesserten sich einzelne psychopathologische Parameter. Eine nominell statistisch signifikante Reduktion der Rehospitalisierungsrate fand sich bei der Patientengruppe mit allen 3 Behandlungsbedingungen. Der rezidivprophylaktische Effekt dieses komplexen psychoedukativ-psychotherapeutischen Behandlungsangebots entwickelte sich erst innerhalb des 2. Katamnesejahrs. Damit ist von einer mittelfristigen rezidivprophylaktischen Wirkung dieses komplexen Therapieangebots, das sowohl Patienten als auch Angehörige einbezieht, auszugehen.

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1433-0474

Schlüsselwörter Herz-Lungen-Transplantation ; Pulmonalarterielle Hypertension ; Kardiopulmonale Erkrankungen ; Immunsuppression ; Key words Heart-lung transplantation ; Pulmonary hypertension ; Cardiopulmonary diseases

Springer Online Journal Archives 1860-2000

Medicine

Summary Introduction: For end-stage cardiopulmonary disease in childhood combined heart-lung transplantation may be the only therapeutic option. Patients: Seven children with end-stage cardiac pulmonary disease (5 primary and 1 secondary pulmonary hypertension, 1 pulmonary atresia with VSD) underwent heart-lung transplantation between 1992 and 1998. Average recipient age was 97/12 years (23/12–145/12 years). All children were orthopnoec, syncopal, oxygen- or ventilator-dependent prior to transplantation. Results: All children survived transplantation. Average ventilation period was 23 days (1–76 days). Follow-up period was 8 to 56 months (35,7 months). One child died 48 months after transplantation due to Aspergillus infection. Cyclosporine or Tracrolimus, and Azathioprine or Mycofenolatmofetil and steroids were used for immunosuppression. Acute lung-rejection was observed in 4, and acute heart-rejection in 2 patients. Three children suffered from bronchiolitis obliterans syndrome and one underwent a single lung retransplantation 42 months after transplantation. Complications had to be divided in surgical, infectious, drug-related and other complications. After their discharge from the hospital all the patients resumed to almost normal physical activity and life style. Conclusion: Heart-lung transplantation is a therapeutic option in end-stage cardiac pulmonary disease leading to almost normal life. Rejections and complications can be managed successfully. Nevertheless carefull follow-up monitoring is necessary. Limiting factor for long-term survival are still bronchiolitis obliterans and infections.

Zusammenfassung Hintergrund: Im Endstadium kardiopulmonaler Erkrankungen ist die Herz- oder Lungen- oder Herz-Lungen-Transplantation die einzige therapeutische Option. Patienten: 7 Kinder mit kardiopulmonalen Erkrankungen im Endstadium und schwerer rechtsventrikulärer Insuffizienz (5 primäre, 1 sekundäre pulmonalarterielle Hypertension, 1 Pulmonalatresie mit VSD) erhielten in den Jahren von 1992–1998 eine Herz-Lungen-Transplantation. Nach einer mittleren Wartezeit von 7,1 Monaten (1–16 Monaten) betrug das mittlere Empfängeralter 97/12 Jahre (21/4–145/12 Jahre). Vor der Herz-Lungen-Transplantation litten alle Patienten an Dyspnoe, Synkopen oder Sauerstoffabhängigkeit. Ergebnisse: Alle Kinder überlebten die Herz-Lungen-Transplantation. Nach einer mittleren Beatmungsdauer von 23 Tagen (1–76 Tage) und einer mittleren Krankenhausaufenthaltsdauer von 64 Tagen (21–95 Tage) wurden die Patienten nach Hause entlassen. Während einer Beobachtungszeit (Stand Februar 1999) von 8–56 Monaten (im Mittel 35,7 Monate) starb 1 Kind 48 Monate nach der Herz-Lungen-Transplantation an einer Aspergillose. Zur Immunsuppression wuren Cyclosporin A oder Tacrolimus, Azathioprin oder Mycofenolatmofetil und Glukosteroide verwendet. Akute Lungenabstoßungen wurden bei 4 und akute Herzabstoßungen bei 2 Patienten beobachtet. Verdacht auf Bronchiolitis obliterans bestand bei 2 Patienten und war bei 1 Patienten nachgewiesen. Dies machte eine Einzellungenretransplantation 42 Monate nach der Herz-Lungen-Transplantation erforderlich. Die aufgetretenen Komplikationen nach Herz-Lungen-Transplantation lassen sich in unmittelbar transplantationschirurgische, infektiöse, Medikamenten-assozierte und übrige Komplikationen unterteilen. Nach der Entlassung aus dem stationären Aufenthalt waren 6 der 7 Patienten körperlich normal belastbar. Dies ermöglichte eine zuvor nicht realisierbare soziale Integration. Schlußfolgerung: Die Herz-Lungen-Transplantation ist eine therapeutische Option bei Kindern mit einer chronischen kardiopulmonalen Erkrankung im Endstadium. Sie führt zu einer Verbesserung der Lebensqualität. Akute Abstoßungen sowie Früh- und Spätkomplikationen sind erfolgreich zu behandeln. Langfristig bestimmt die Bronchiolitis obliterans als Form der chronischen Abstoßung die Überlebensrate.

Electronic Resource

1432-1173

Schlüsselwörter Lichen striatus ; Blaschkitis ; Lineäre Psoriasis ; Lineärer Lichen planus ; Keywords Lichen striatus ; Linear Psoriasis ; Linear Lichen planus

Springer Online Journal Archives 1860-2000

Medicine

Abstract A 64-year-old woman presented with extensive erythematous papulosquamous lesions along the lines of Blaschko on the right half of the trunk and the right leg. The clinical and histological features led to the diagnosis of lichen striatus, a dermatosis which usually appears during childhood. Treatment with acitretin led to marked improvement. Lichen striatus and its differential diagnoses – particulary “blaschkitis of adulthood” as first described in 1990 – are discussed.

Zusammenfassung Wir berichten über eine 64-jährige Patientin, die ausgedehnte erythematopapulosquamöse Läsionen an der rechten Hälfte des Rumpfes sowie am rechten Bein im Verlauf der Blaschko-Linien aufwies. Das klinische und histologische Bild führte zur Diagnose eines Lichen striatus, einer Dermatose, die üblicherweise im Kindesalter auftritt. Unter Behandlung mit Acitretin kam es zu einem deutlichen Rückgang der Läsionen. Der Lichen striatus und seine Differentialdiagnosen – insbesondere die 1990 erstmals beschriebene “Blaschkitis des Erwachsenen”– werden diskutiert.

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1432-1173

Schlüsselwörter Fokale dermale Hypoplasie ; Minimalbefall ; Key words Focal dermal hypoplasia ; Minimal skin involvement

Springer Online Journal Archives 1860-2000

Medicine

Summary Focal dermal hypoplasia (FHD) is an X chromosomal dominant inherited disease with unknown gene defect. FDH is characterized by ectodermal and mesodermal malformations. It is thought to be lethal in males; however, males may survive as mosaics or possibly as Klinefelter syndrome (XXY). When mosaicism involves the gonads the disease may be transmitted from father do child. In females, the abnormal phenotype is thought to be expressed in a blaschkoid pattern because of random X chromosome inactivation. We have collected eight cases of which three where males. We present the typical clinical dermatological feature and draw attention to cases with minimal skin involvement.

Zusammenfassung Die fokale dermale Hypoplasie (FDH) ist eine x-chromosomal dominant übertragene Erkrankung mit ektodermalen und mesodermalen Fehlbildungen. Die Krankheit ist für männliche Foeten letal, doch können männliche Patienten als Mosaik oder eventuell als Klinefelter-Syndrom (XXY) überleben. Wenn das Mosaik die Gonaden einschließt kann die Krankheit vom Vater übertragen werden. In weiblichen Patienten zeigt sich ein blaschkoides Befallsmuster, was auf die zufällige X-Chromosom-Inaktivierung zurückgeführt wird. Wir stellen das typische Krankheitsbild der FDH an 8 Patienten vor und möchten auf Minimalformen hinweisen.

Electronic Resource

1432-1076

Human basophils ; Histamine release ; Pertussis toxin

Springer Online Journal Archives 1860-2000

Medicine

Abstract The influence of childhood pertussis infection and of purified pertussis toxin on histamine relase from human basophil leucocytes was investigated. Three different stimuli, the peptide N-formyl-Met-Phe (NFMP), anti-IgE, and the calciuminnophore A23187 were used to challenge the cells. When NFMP was the stimulus, histamine release in the control group (age 0.5–17 years) increased in an age-dependent fashion, whereas anti-IgE and A23187 stimulated release did not vary with age. During the convulsive state of pertussis infection there was a significant reduction of histamine release in response to 10 μM NFMP (from 9.5±1.4 [n=21] to 6.7±1.5 [n=19],P〈0.05) and in response to 800 and 80 U/ml anti-IgE (from 28.5±5 [n=19] to 16.3±5 [n=13],P〈0.05, and from 6.9±1.7 [n=16] to 2±0.8 [n=13],P〈0.01), whereas histamine release stimulated by A23187 was unchanged compared to release in control children. In vitro pretreatment of basophils from healthy children and adults with pertussis toxin also inhibited histamine release. When NFMP was the stimulus, release was completely blocked by pertussis toxin with an IC50 of about 11 ng/ml whereas anti-IgE stimulated release was only inhibited by 20%–30% and release induced by A23187 was reduced to 40%–50% by toxin treatment. In conclusion we have demonstrated a functional impairment of histamine release during the convulsive state of pertussis and that this inhibition is likely to be mediated by pertussis toxin.

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1573-2665

Springer Online Journal Archives 1860-2000

Medicine

Abstract Even early-treated patients with phenylketonuria (PKU) have a higher risk of psychosocial maladjustment. This study was performed to determine whether emotional and behavioural problems are specific in phenylketonurics and whether they depend on the quality of biochemical control. This comparative study covered 42 PKU patients aged 10–18 years (mean 14.7 years) and 42 diabetic patients matched for sex, age and socioeconomic status. Patients' groups were compared with a control sample of healthy controls (n=2900) from an epidemiological study. We used the Child Behavior Check List (CBCL) according to Achenbach, intelligence quotient (IQ) test according to Weiss, and monitoring of blood phenylalanine concentrations and HBA1 concentrations. Internalizing problems such as depressive mood, anxiety, physical complaints or social isolation were significantly elevated in both phenylketonuric and diabetic patients, whereas externalizing problems were not. The two patient groups did not differ significantly either in the degree or in the pattern of their psychological profile. In both groups no significant correlations could be computed between the psychological characteristics and the biochemical control, the IQ, and the socioeconomic status. No patient was undergoing psychiatric treatment or psychotherapy. Our results strongly support a psychological perspective for the development of behavioural and emotional problems in both phenylketonuric and diabetic patients. Thus, medical treatment should be accompanied by psychological support for the families.

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1573-2665

Springer Online Journal Archives 1860-2000

Medicine

Electronic Resource

1434-601X

13.10.+q ; 12.20.Fv ; 14.80.Pb

Springer Online Journal Archives 1860-2000

Physics

Abstract A monoenergetic positron (e +) beam (ΔT/T〈5·10−4) from the Stuttgart pelletron accelerator and a 4.6 mg/cm2 Be target has been employed to investigatee + e −scattering in the MeV region in a large solid angle with high statistics by means of a novel positionsensitive detector system. Superimposed on the Bhabha scattering, a structure has been found at ane + kinetic energy of 2263 keV (810 keV excitation energy in thee + e −rest frame). From the energy-integrated resonance cross-section of $$\sigma _{res} \cdot \Gamma _{res}^{e^ + e^ - } $$ ≅30 b·eV (c.m.) and the standard resonance cross-section we estimate partial resonance widths $$\Gamma _{res}^{e^ + e^ - } $$ ≅72 meV or 24 meV for total angular momenta J=0 or J=1, respectively. The structure, which has not been predicted within the framework of quantum electrodynamics, coincides with one of the sum energies of the correlatede + e −lines observed in heavy-ion collisions.

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1618-2650

Springer Online Journal Archives 1860-2000

Chemistry and Pharmacology

Electronic Resource

0003-3146

Chemistry ; Polymer and Materials Science

Wiley InterScience Backfile Collection 1832-2000

Chemistry and Pharmacology

Physics

Dependent on the conversion and on the functionality and quantity of the chain regulator molecular weight distributions are calculated for hydrolytically polymerized polylaurolactam. The total distribution is given by superposition of the distributions of two species of molecules, of the molecules which do not contain any regulator molecule and of the molecules which contain a regulator molecule. The moiety of the two species of molecules, the weight average molecular weight and the nonuniformity is taken for characterizing the distributions. It is shown that for small regulator quantities even for high conversions the moiety of the molecules, which does not contain any regulator molecule may be very high. Therefore the nonuniformity of the distribution becomes remarkably higher as can be calculated applicating the simplified equation of FLORY neglecting this moiety: M̄w/M̄n=1+1/f.

In Abhängigkeit vom Umsatz sowie der Funktionalität und Menge des Reglers werden für hydrolytisch Polymerisiertes Polylaurinlactam Molekulargewichtsver-teilungen berechnet. Die Gesamtverteilung ergibt sich durch Überlagerung der Verteilungen von zwei Molekülsorten, die einmal kein Reglermolekül und zum anderen ein Reglermolekül eingebaut enthalten. Der Anteil der beiden Molekülsorten, das Gewichtsmittel des Molekulargewichts sowie die Uneinheitlichkeit wird zur Charakterisierung der Verteilungen herangezogen. Es wird gezeigt, daß bei kleinen Reglermengen selbst bei hohen Umsätzen der Anteil der Moleküle, die kein Reglermolekül eingebaut enthalten, so hoch sein kann, daß dadurch die Uneinheitlich-keit der Verteilung wesentlich größer wird als nach der von FLORY unter Vernachlässigung dieser Molekülsorte angegebenen Näherung M̄wM̄n=1+1/f zu berechnen ist.

8 Ill.

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