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  • 1
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Two sibs with TAR syndrome and whose parents are blood relatives are described. To our knowledge this is the first report of consanguinity in TAR syndrome.
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  • 2
    ISSN: 1432-1076
    Keywords: Hydrometrocolpos ; Postaxial polydactyly ; Hypospadia glandis ; Autosomal recessive
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Nine cases with the hydrometrocolpospolydactyly syndrome (4 males, 5 females) from four unrelated families are presented. Leading symptoms of this rare disorder were hydrocolpos and postaxial polydactyly. Three affected girls had urinary hydrocolpos without vaginal septum or imperforate hymen, one had partial vaginal atresia, and one had no hydrometrocolpos. Glandular hypospadias and prominent scrotal raphe are added to the spectrum of malformations in this disorder in males. The literature is reviewed and problems in genetic counseling in this autosomal recessive disorder are discussed.
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  • 3
    ISSN: 1432-1076
    Keywords: Chlamydia trachomatis ; Impetigo contagiosa ; IgG-sub-class-deficiency ; Pneumothorax
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract An unusual case ofChlamydia trachomatis (C. trachomatis) pneumonia, complicated by the development of a pneumothorax, is reported in an IgG2/IgG4 deficient infant delivered by caesarean section.C. trachomatis was isolated initially from a throat smear and subsequently from pleural effusions. Serological examinations using the complement fixation test were negative in sera of both mother and child. However, using immunofluorescence the presence of an acute or recent infection was confirmed by IgM-antibodies in the serum of the infant and IgA-antibodies in the serum of the mother. At the age of 7 months the girl suffered from impetigo contagiosa which was partially resistant to antibiotic treatment. IgG-subclass deficiency was diagnosed after the onset of this disease and the girl was then treated by immunoglobulin transfusion.
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  • 4
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Chemotherapie ; ALL ; AML ; Anfälle ; Kernspintomographie ; Infratentoriell ; Key words Chemotherapy ; ALL ; AML ; Seizures ; MRI ; Infratentorial
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Two children on chemotherapy for acute myeloid leukemia (AML) and acute lymphoblastic leukemia (ALL) had initially focal, then generalized seizures of short duration. Magnetic resonance imaging (MRI) revealed in one case minor parietal, cortical and subcortical increased signal on T2-weighted images. In a second case extended supratentorial lesions and additional signal changes in the cerebellum were found. Although chemotherapy was continued, a few days later signal changes were considerably reduced or had disappeared. We consider these lesions to be seizure induced in a brain previously sensitized by chemotherapy. Discussion: If post-ictal MRI-imaging shows signal changes, differential diagnosis has to consider other reasons like leukemic infiltrations, encephalitis or hypertensive encephalopathy. However, before extensive diagnostic procedures (e.g. repetitive lumbar punctures) are undertaken, a short-term MRI follow-up should be performed: reversibility within a few days can render the differential diagnosis of transient epileptogenic changes very likely. A temporary anti-epileptic treatment seems to be indicated. Chemotherapy has not to be suspended.
    Notes: Zusammenfassung Bei 2 Kindern kam es während des ersten Zyklus einer Chemotherapie bei akuter myeloischer Leukämie und akuter lymphatischer Leukämie zu kurzdauernden fokalen, sekundär generalisierten Krampfanfällen. Die kernspintomographische Untersuchung am Anfallstag bzw. 2 Tage postiktal zeigte in 1 Fall nur geringere supratentorielle subkortikale Signalanhebungen im T2-gewichteten Bild. Im 2. Fall fanden sich sowohl ausgedehnte supra- als auch infratentorielle Veränderungen. Trotz Fortsetzung der Chemoterapie waren diese nach wenigen Tagen bereits deutlich rückläufig bzw. rückgebildet. Sie werden daher als anfallsbedingte Veränderungen im Zusammenwirken mit der Chemotherapie angesehen. Diskussion: Bei unmittelbar postiktal gefundenen kernspintomographischen Veränderungen sind differentialdiagnostisch andere Ursachen (zerebrale Beteiligung im Rahmen der Grundkrankheit, entzündlich, hypertensiv) zu erwägen. Ähneln jedoch die gefundenen Läsionen morphologisch den hier beschriebenen, so empfiehlt sich eine kurzfristige Verlaufskontrolle bevor weitergehende diagnostische (z.B. wiederholte Lumbalpunktionen) oder therapeutische Maßnahmen ergriffen werden: Anfallsinduzierte Veränderungen sind nach unserer Erfahrung innerhalb weniger Tage rückläufig. Eine vorübergehende antikonvulsive Behandlung scheint sinnvoll. Die Chemotherapie braucht nicht unterbrochen zu werden.
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  • 5
    ISSN: 1433-0474
    Keywords: Schlüsselwörter Leukämie ; Non-Hodgkin-Lymphom ; Endgröße ; Körperproportionen ; Key words Leukemia ; Non-hodgkin lymphoma ; Final height ; Body proportions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Objectives: Children treated for acute lymphoblastic leukemia (ALL) or non-hodgkin lymphoma (NHL) show retarded growth. The question arose as to whether proportionate catch-up growth follows. Methods: Parameters determined in 41 adult, former ALL-/NHL-patients were: height, sitting height, head circumference, weight, height before therapy start, target height, growth hormone dependent factors, thyroid hormones and sexual steroids. (Prader standards) Results: Final height (mean −0.3 SDS; range: −2.7 to 1.9 SDS) was significantly lower (p〈0.001) than height before therapy (mean 0.3 SDS; range: −1.8 to 1.6 SDS). Nevertheless, 38 patients reached final height above the 3rd centile, only 2 patients failed to achieve target height. Height loss was marked in cranially irradiated patients. None of the patients showed complete hormone deficiency. In relation to height, sitting height was low (mean −1.0 SDS; range: −3.0 to 0.8 SDS), but head circumference normal. Conclusions: Although patients suffer growth impairment after ALL/NHL therapy, long-term height prognosis is good. Growth retardation of the trunk contributes considerably to height loss and results in disproportion.
    Notes: Zusammenfassung Fragestellung: Kinder, die wegen akuter lymphoblastischer Leukämie (ALL) oder Non-Hodgkin-Lymphom (NHL) hämatologisch behandelt werden, wachsen langsamer. Wir wollten überprüfen, ob es nach Therapieabschluß zu einem proportionierten Aufholwachstum kommt. Methode: Bei 41 erwachsenen ehemaligen ALL- und NHL-Patienten wurden Größe, Sitzhöhe, Kopfumfang, Körpergewicht, Ausgangsgröße vor Therapiebeginn, die familiäre Zielgröße, wachstumshormonabhängige Faktoren, Schilddrüsenhormone und Sexualsteroide bestimmt (Prader-Standards). Ergebnisse: Verglichen mit den Ausgangsgrößen (Mittelwert 0,3 SDS; Minimum −1,8 SDS, Maximum 1,6 SDS) sind die Endgrößen (Mittelwert −0,3 SDS; Minimum −2,7 SDS, Maximum 1,9 SDS) signifikant niedriger (p〈0,001). 38 Patienten erreichten trotzdem eine Endgröße über der 3. Perzentile, und nur 2 Patienten liegen größenmäßig unterhalb des familiären Zielgrößenbereichs. Die Größeneinbuße betrifft insbesondere ehemals schädelbestrahlte Patienten. Bei keinem wurde ein kompletter Hormonmangel festgestellt. Die Sitzhöhen der Patienten (Mittelwert −1,0 SDS; Minimum −3,0 SDS, Maximum 0,8 SDS) fanden sich bezogen auf die Endgrößen kurz, die Kopfumfänge normal. Schlußfolgerungen: Obwohl Patienten nach ALL- bzw. NHL-Therapie eine Wachstumsbeeinträchtigung erfahren, ist die Endgrößenprognose günstig. Die Wachstumsretardierung des Rumpfs trägt zur Größeneinbuße entscheidend bei und führt zur Disproportion.
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  • 6
    ISSN: 1432-0584
    Keywords: Ciclosporin ; Graft-versus-host-disease ; Bone marrow transplantation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Between 1982 and 1986 51 patients were treated with ciclosporin a (CSA) to prevent graft versus host disease (GvHD) after bone marrow transplantation (BMT). Major side effects of the drug were tremor, hypertension, hepatotoxicity and nephrotoxicity. Acute GvHD 0° to II° occurred in 80% of our patients, and GvHD III° and IV° in 20% despite the use of CSA. Two to four days before the onset of GvHD, CSA serum levels were significantly lower on the average in patients who developed GvHD III° and IV° compared to the others. Our data indicate that plasma CSA concentrations higher than 250 ng/ml should be achieved to reduce the severity of GvHD after BMT.
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  • 7
    ISSN: 1432-069X
    Keywords: Subcorneal pustular dermatosis ; Autoantibodies ; Gammopathy ; Autoimmunity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
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  • 8
    ISSN: 1432-0584
    Keywords: Systemic lupus erythematosus ; Immunoglobulin therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two paediatric patients with systemic lupus erythematosus were treated with immunoglobulin G (IgG). In the first case treatment resulted in regression of the most acute symptoms and a long remission was achieved. In the second patient, who was treated during the chronic stage of the disease, there was no significant effect on the course of the SLE.
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  • 9
    ISSN: 1432-0584
    Keywords: Bone Marrow transplantation ; unrelated mismatched donor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In February 1986 we transplanted a 10-year-old girl with AML in second remission with the bone marrow of an unrelated donor. HLA-types were different for one A- and one B-antigen between patient and donor. Conditioning regimen consisted of 14 Gy total body irradiation with lung shielding, 8×3 g/m2 cytosin arabinoside and 90 mg/kg cyclophosphamide. GVHD-prophylaxis was performed with cyclosporin A, methotrexate and prednisolone. Only mild GVHD I of the skin could be observed after rapid engraftment. 100 days after transplantation the patient was in good clinical condition and GVHD-prophylaxis was discontinued without any reactivation of acute or chronic GVHD. Engraftment was documented by sex chromosome and blood group typing. 120 days after transplantation leukemic blasts were detected in the peripheral blood and the child died 130 days after BMT from relapse of the leukemia. Despite the negative outcome, this was the first successful bone marrow transplantation from a unrelated donor in Germany.
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  • 10
    ISSN: 1432-0843
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Results of the BMF study group trials ALL-REZ 83 and 85 for relapsed acute lymphoblastic leukemia (ALL) are presented. For children with late marrow relapse, remission rates of about 90% were seen in both studies. In children treated for early marrow relapse, the remission rate in study ALL-REZ 85 was superior (86% vs 62%). The probability of event-free survival for all patients and for those with early marrow relapse was also statistically significant (P〈0.05). Children with T-cell ALL had an extremely unfavourable prognosis in both studies.
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