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Biomedical subjects

B Krafchik

Publications and source records attributed to B Krafchik.

16 recordsLinked to original sources

Tuberous sclerosis: clinicopathologic features and review of the literature.

INTRODUCTION: Tuberous sclerosis is a hamartoneoplastic syndrome, which may involve multiple organ systems. Oral hard tissue manifestations of the syndrome have been described in the literature only as recently as 1955. Patients who presented with clinical manifestations of tuberous sclerosis did not routinely undergo oral surveys to rule out 'lesions', and consequently data on 'lesions' in the maxillofacial complex is scant. Ten cases have been found in the English language literature, which describe maxillofacial 'lesions', which may be tumours, new growths, neoplasms or overgrowths occurring in patients diagnosed with tuberous sclerosis. PURPOSE: To review the literature for all maxillofacial lesions associated with tuberous sclerosis and to present an eleventh case of a patient with a maxillofacial lesion diagnosed as having tuberous sclerosis. RESULTS: Eleven cases were found with maxillofacial fibroblastic lesions associated with tuberous sclerosis. These lesions were all fibrous benign neoplasms found in the maxillofacial bony complex. CONCLUSIONS: Maxillofacial fibroblastic lesions in tuberous sclerosis have various histopathological presentations, some of which may be difficult to differentiate. Consequently, close microscopic examination of these lesions is necessary so that adequate surgical treatment is provided.

Child, Preschool↗

The health of mothers of children with cutaneous neonatal lupus erythematosus differs from that of mothers of children with congenital heart block.

PURPOSE: Neonatal lupus erythematosus is caused by the transplacental passage of maternal autoantibodies. The aim of this study was to determine the risk of connective tissue disorders in mothers of children with cutaneous neonatal lupus erythematosus, as compared with the risk in mothers of children with congenital heart block, which is also often caused by maternal autoantibodies. SUBJECTS AND METHODS: We prospectively studied all mothers of children with cutaneous neonatal lupus erythematosus during a 14-year period at the Hospital for Sick Children, Toronto, Ontario, Canada. We identified 28 mothers, of whom 24 were eligible for study. The health and antibody status of the mothers were determined at the birth of the child and at followup. RESULTS: All mothers had anti-Ro antibodies at the time of birth. Initially 10 mothers were healthy and 14 mothers had either a defined (n = 9) or an undifferentiated (n = 5) autoimmune disorder. At a mean follow-up of 7 years, 13 (1 of whom had died) had a defined connective tissue disease, and 5 had an undifferentiated autoimmune disorder. Only 6 (25%) remained asymptomatic. By comparison, 36 (56%) of 64 mothers of children with congenital heart block were asymptomatic at follow-up (P <0.005). CONCLUSIONS: The majority of mothers of children with cutaneous neonatal lupus erythematosus had a defined or undifferentiated autoimmune disorder at the time of the child's birth, and others developed these conditions during follow-up. The health of these mothers appears to differ from that of mothers of children with congenital heart block.

Adult↗

Fixed drug eruptions in children.

To determine the anatomic location and offending drug in fixed drug eruptions (FDE) in children, we performed a 5-year retrospective analysis. Thirty-five children with FDE were evaluated. The most common cause of FDE was the combination drug trimethoprim-sulfamethoxazole.

Adolescent↗

An open study of tinea capitis in 50 children treated with a 2-week course of oral terbinafine.

BACKGROUND: Terbinafine is used in the treatment of dermatophyte infections. There have been several studies suggesting a good response to terbinafine in treating tinea capitis, specifically with dermatophytes of the Trichophyton species. METHODS: We enrolled 50 consecutive children with a clinical diagnosis of tinea capitis into an open study using terbinafine for 2 weeks. RESULTS: Clinical and mycologic cure occurred in more than 86% of patients with no side effects and good compliance. CONCLUSION: In this study terbinafine was a safe and effective treatment of tinea capitis in children, particularly when caused by the Trichophyton species.

Adolescent↗

Topical calcipotriol in childhood psoriasis.

BACKGROUND: The use of topical calcipotriol in adults with psoriasis is safe and effective. OBJECTIVE: Our purpose was to study the efficacy and safety of calcipotriol in children. METHODS: A multicenter, prospective, 8-week, double-blind, parallel group study was conducted in 77 children. Response to treatment was assessed by means of the Psoriasis Area and Severity Index (PASI) in that the intensity of redness, thickness, and scaliness as well as the area involved are scored. The children were 2 to 14 years of age and had stable psoriasis, involving less than 30% of the body surface. Forty-three children were assigned to receive calcipotriol ointment and 34 to receive placebo. Nine children dropped out of the study, six in the calcipotriol-treated group and three in the placebo-treated group. RESULTS: Both treatment groups (calcipotriol and placebo) showed significant improvement in PASI from baseline to the end of treatment, and the difference was not statistically significant. No serious side effects, in particular including those relating to calcium and bone metabolism, were recorded. CONCLUSION: Calcipotriol ointment was statistically significantly more effective than its vehicle in terms of the investigator's overall assessment and reduction in redness and scaliness but not in terms of PASI score.

Administration, Topical↗

The use of oral terbinafine (Lamisil) in children.

In an ongoing study of Lamisil in children, 21 patients have so far been enrolled and preliminary data are reported in this paper. Eighteen have tinea capitis and 1 has tinea corporis. So far, Trichophyton tonsurans has been identified in 17 and Trichophyton violaceum in 2. Lamisil has so far proved to be well tolerated, efficacious and cost-effective in a 2-week course in those children with a Trichophyton species. If Microsporum canis is isolated, a longer course is probably indicated. Lamisil has the distinct advantage of producing good results in a short time period, making patient compliance less of a problem.

Administration, Oral↗

Localized scleroderma: imaging features.

Localized scleroderma is distinct from the diffuse form of scleroderma and does not show Raynaud's phenomenon and visceral involvement. The imaging features in 23 patients ranging from 2 to 17 years of age (mean 11.1 years) were reviewed. Leg length discrepancy and muscle atrophy were the most common findings (five patients), with two patients also showing modelling deformity of the fibula. One patient with lower extremity involvement showed abnormal bone marrow signals on MR. Disabling joint contracture requiring orthopedic intervention was noted in one patient. In two patients with "en coup de sabre" facial deformity, CT and MR scans revealed intracranial calcifications and white matter abnormality in the ipsilateral frontal lobes, with one also showing migrational abnormality. In a third patient, CT revealed white matter abnormality in the ipsilateral parietal lobe. In one patient with progressive facial hemiatrophy, CT and MR scans showed the underlying hypoplastic left maxillary antrum and cheek. Imaging studies of areas of clinical concern revealed positive findings in half our patients.

Adolescent↗

Childhood linear scleroderma: a possible role of thermography for evaluation.

Linear scleroderma is a rare, at times debilitating, disease with an unpredictable course. Currently, there is no satisfactory objective method for assessment of disease activity upon which to base therapeutic decisions. We evaluated 11 children with 18 linear scleroderma lesions (mean age 11.7 years, mean duration of disease 5.1 years) for disease severity and the presence of immunologic abnormalities, and attempted to correlate these results with thermography. Positive thermography was defined as warmer than surrounding skin or opposite limb by 0.5 degrees C. Six patients were thermography positive. Mean age, sex, disease duration and the presence of hypergammaglobulinemia and autoantibodies were similar in thermography positive and thermography negative patients. Six of 18 linear scleroderma lesions were thermography positive. All 3 new or expanding lesions were thermography positive. All 3 lesions that were resolving clinically were thermography negative. Three of 12 lesions that were clinically unchanged over a 6-month period were also thermography positive. In summary, thermography is a noninvasive test that appears to demonstrate active lesions in linear scleroderma. It is not influenced by previous soft tissue damage induced by linear scleroderma and may enable better monitoring of the effectiveness of proposed therapies.

Adolescent↗

Acne in Apert syndrome.

An acneiform eruption is a well-recognized feature of Apert syndrome. Management is determined by the extent and morphology of the eruption. This article details treatment procedures for acne that closely resembles acne vulgaris.

Acne Vulgaris↗

Lyme disease.

Explore the source record for details and available documents.

Child↗

Multiple cutaneous granular cell tumors in children.

Multiple cutaneous granular cell tumors are rare in childhood. We cared for two children with 15 and 6 tumors respectively. The tumors were asymptomatic nodules in the skin. Histologically, they consisted of large cells containing granules that were positive on periodic acid-Schiff stain and diastase resistant.

Child↗