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Biomedical subjects

Stefania Jablonska

Publications and source records attributed to Stefania Jablonska.

24 records · Page 2Linked to original sources

Generalized eruptive keratoacanthoma of Grzybowski: follow-up of the original description and 50-year retrospect.

We report a follow-up of the original case of eruptive multiple keratoacanthoma (KA) described by Grzybowski in 1950 which did not show malignant transformation within 16 years after the onset of the disease in spite of a very widespread cutaneous involvement. There was no associated internal malignancy or any systemic disease either, and the patient died of a myocardial infarction unrelated to his cutaneous disorder. This case followed up for such a long time provides further confirmation that this variety of KA does not show invasive growth and usually does not progress into squamous cell carcinoma.

Biopsy, Needle↗

Imiquimod is highly effective for extensive, hyperproliferative condyloma in children.

We describe a dramatic response to imiquimod of long-lasting, highly proliferative extensive perianal condylomas involving the anal canal in a 19-month-old girl. Her mother was free of condyloma and allegedly had no human papillomavirus (HPV) infection during pregnancy. There was no evidence of sexual abuse. Application of 5% imiquimod cream to the child every other day for 3 weeks resulted in almost complete resolution of the warts, with total clearance within another 2 weeks. The inflammatory reaction was moderate. Since there is still discussion of whether imiquimod may be prescribed for small children, this case of very extensive condyloma provides evidence that the compound is safe and highly effective.

Administration, Topical↗

Intralesional recombinant interferon beta-1a in the treatment of basal cell carcinoma: results of an open-label multicentre study.

Although effective conventional therapies are available to treat basal cell carcinoma (BCC), undesirable side effects, including scarring, and in some cases permanent damage, often occur in problematic areas of the body, especially around the eyes, mouth, and cartilage of the nose and ears. In previous studies, intratumoural injection of recombinant interferon beta-1a (rIFN-beta-1a) has been shown to result in complete remission (CR) in 47% to 86% of patients with BETACC. The primary objective of the study was to determine the response rate to rIFN-beta-1a, in a larger BETACC patient population. Secondary objectives included evaluating the effect of tumour type/size on response as well as residues, cosmetic results, and relapse rate after CR. The safety profile of intratumoural rIFN-beta-1a in BETACC patients was also evaluated. This was an open-label, multicentre study involving 139 patients with BETACC (diameter between 5.0 and 20 mm). Intratumoural injections of rIFN-beta-1a (1.0 x 106 IU) were administered three times a week for 3 weeks. The response was determined 16 weeks after start of treatment and the status of patients was followed for up to 5 years. At 16 weeks, the response rate to intratumoural rIFN-beta-1a was 66.9% (95% CI, range 58.2-74.8%). There was no significant difference between the response rates for patients with solid or other BETACC tumour types. Similarly, tumour size did not significantly affect the response rate. The cosmetic result of treatment was rated as good or very good in 83% of responders. The relapse rate after CR was 4.5% (median follow-up 2 years). All patients showed local inflammatory reactions, which were generally considered to be the adverse drug reactions (ADRs). Systemic ADRs mostly consisted of flu-like symptoms and occurred in 32/139 patients. No ADRs were considered to be the serious. These results show that intratumoural injections of rIFN-beta-1a are effective in the treatment of BETACC in the majority of patients. In addition, rIFN-beta-1a is safe and generally well tolerated. rIFN-beta-1a represents an effective alternative treatment for BETACC.

Adult↗

Fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva, a rare genetic disabling disease characterized by heterotopic bone formation, is of special interest for general medicine since the bone morphogenetic proteins (especially BMP-4) involved in its pathogenesis are known to play a role in skeletal morphogenesis, and the gene antagonist to BMP-4 noggin might be useful in preventing lamellar bone formation. We present two cases with characteristic musculo-skeletal abnormalities and histopathological features (inflammatory infiltrates which destroyed muscle tissue replacing it by proliferating fibroblasts). In one patient due to high activity of fibroblasts, the histopathologic pattern appeared to be atypical and the lesion was recognized by a general pathologist as sarcoma. The other patient, due to the symmetrical induration of sternocleidomastoid muscles, was primarily recognized as scleredema. We stress the diagnostic importance of skeletal abnormalities (hallux valgus and others), and discuss differentiation from progressive osseous heteroplasia (POH) and congenital or acquired localized cutaneous and muscle ossifications which have a much better prognosis, as well as Albright's hereditary osteodystrophy, which differs by the presence of various systemic abnormalities. A study of FOP might provide an important clue to the genetic molecular mechanism of bone formation, development of heterotopic bone and a possible prevention by molecular manipulation with the gene responsible for bone morphogenetic proteins and their antagonists.

Adolescent↗