Are you K. Mathony?

Claim your profile

Publications (2)9.3 Total impact

  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: To study the efficacy of combination therapy with etanercept and methotrexate in patients with refractory juvenile idiopathic arthritis. Seven children with active juvenile idiopathic arthritis refractory to at least combination therapy with methotrexate and sulfasalazine or cyclosporin A were studied. Concomitant treatment, consisting of non-steroidal drugs, corticosteroids, and methotrexate, remained unchanged. Six patients continued the treatment for at least 24 weeks. In the child with systemic arthritis, etanercept was stopped because of persisting spiking fever, joint pain, and rash. In the remaining children an immediate significant decrease in joint pain (p<0.05), disappearance of morning stiffness, and regression of joint swelling (p<0.05) were observed. Improvement was apparent after two injections. An immediate significant (p<0.05) decrease in erythrocyte sedimentation rate, C reactive protein, and interleukin 6 was observed. Side effects consisted of mild reactions at the injection site in two children. In this observational study, etanercept in combination with methotrexate was well tolerated and highly effective in treating juvenile polyarthritis but not in the patient with systemic arthritis. Combination treatment appears to be feasible in terms of toxicity and may enhance efficiency.
    Annals of the Rheumatic Diseases 05/2001; 60(4):410-2. · 9.11 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Hintergrund. Das Bloch-Sulzberger-Syndrom (Incontinentia pigmenti) ist eine X-chromosomal-dominant vererbte Erkrankung, die in variabler Expressivität verschiedene Organsysteme, die Haut, das Zentralnervensystem, die Augen, die Zähne und das Skelett betrifft. Die Diagnose kann aufgrund typischer Hautmanifestationen in den ersten Lebenswochen gestellt werden. Fallbericht. Die Erstvorstellung des derzeit 2-jährigen Mädchens erfolgte am 2. Lebenstag mit Krampfanfällen im Rahmen einer schweren Enzephalopathie mit nachfolgenden ausgeprägten Marklager- und Rindennekrosen. Das Kind entwickelte eine schwere globale Entwicklungsstörung mit spastischer Tetraplegie und Mikrozephalus. Typische Hauterscheinungen mit initial papulopustulösen Effloreszenzen gefolgt von streifiger und retikulärer Hyperpigmentierung traten erst im Rahmen einer septischen Infektion im Alter von 2 Jahren auf. Die Histologie einer Hautbiopsie sicherte die Diagnose. Resümee. Ohne initialen Hautbefall ist diese seltene Diagnose schwierig, gelingt aber, wenn die typischen Hautmanifestationen im Verlauf hinzutreten. Infektionserkrankungen führen möglicherweise erst zur Ausprägung der Hauterscheinungen. Bei unauffälliger Familienanamnese, Fehlen von Aborten und 2 lebenden gesunden Brüdern kann eine Neumutation angenommen werden. Background. Bloch-Sulzberger syndrome (incontinentia pigmenti) is an x-linked dominant disease, affecting the skin, the central nervous system, the eyes, the teeth and the skeleton with variable expression. Diagnosis is suspected in the presence of typical sequence of skin symptom change during the first weeks of life. Case report. Here, we report on a now two year old girl who first presented with epileptic seizures, severe encephalopathy with distinct necrosis of cerebral medulla and cortex at the age of two days. Thereafter the child developed mental retardation, spastic tetraparesis and microcephaly. There were no distinct skin eruptions. A typical generalised dermatosis appearing with papular and pustular lesions resulting into reticular hyperpigmentation became evident at the age of two years when she suffered from septic lymphadenitis. Diagnosis of incontinentia pigmenti was considered and confirmed by the histologic examination of skin biopsy. Conclusion. This case demonstrates, that if skin changes do not occur during the infant period, diagnosis may be delayed. Since the family history revealed no further affected individuals and the mother had no history of abortions with two living healthy sons, sporadic mutation may have occurred in this child.
    Monatsschrift Kinderheilkunde 01/2001; 149(1):41-44. · 0.19 Impact Factor