PubMed Health⌕ Search

Biomedical subjects

L Klapholz

Publications and source records attributed to L Klapholz.

9 recordsLinked to original sources

Chronic radiodermatitis following cardiac catheterization.

BACKGROUND: Fluoroscopy and cineradiography used during coronary angiography expose patients to some of the highest doses of ionizing radiation in diagnostic radiology. The possibility of radiation-induced damage has been discussed by several authors in the past. However, to the best of our knowledge, chronic radiation dermatitis caused by exposure to x-rays during cardiac catheterization has not been described. OBSERVATIONS: We describe 4 patients in whom chronic radiodermatitis developed following multiple cardiac catheterizations and coronary angioplasties. The cumulative radiation doses to which these patients were exposed were retrospectively calculated to be a mean of 24.6 Gy per patient, with a range of 11.4 to 34.9 Gy. CONCLUSIONS: Chronic radiodermatitis is a threat in patients undergoing multiple cardiac catheterizations and angioplasties. In susceptible patients, radiation doses as small as 11.4 Gy, which can sometimes be emitted during 1 or 2 procedures, are potentially harmful. Awareness and protective measures against this long-term side effect of cardiac catheterization should be encouraged.

Aged↗

Toxic epidermal necrolysis associated with Klebsiella pneumoniae sepsis.

Toxic epidermal necrolysis (TEN) is a rare condition in childhood usually attributed to drugs. We describe a 4-month-old infant who developed typical clinical and histologic findings of TEN concomitantly with Klebsiella pneumoniae sepsis. We emphasize that in cases of acute, severe exfoliative disease in infants, apart from staphylococcal infection, gram-negative bacterial sepsis must also be considered.

Bacteremia↗

Frameshift and splice-junction mutations in the sterol 27-hydroxylase gene cause cerebrotendinous xanthomatosis in Jews or Moroccan origin.

The sterol 27-hydroxylase (EC 1.14.13.15) catalyzes steps in the oxidation of sterol intermediates that form bile acids. Mutations in this gene give rise to the autosomal recessive disease cerebrotendinous xanthomatosis (CTX). CTX is characterized by tendon xanthomas, cataracts, a multitude of neurological manifestations, and premature atherosclerosis. A relatively high prevalence of the disease has been noted in Jews originating from Morocco. The major objectives of the present investigation were to determine the gene structure and characterize the common mutant alleles that cause CTX in Moroccan Jews. The gene contains nine exons and eight introns and encompasses at least 18.6 kb of DNA. The putative promoter region is rich in guanidine and cytosine residues and contains potential binding sites for the transcription factor Sp1 and the liver transcription factor, LF-B1. Blotting analysis revealed that the mutant alleles do not produce any detectable sterol 27-hydroxylase mRNA. No major gene rearrangements were found and single-strand conformational polymorphism followed by sequence analysis identified two underlying mutations: deletion of thymidine in exon 4 and a guanosine to adenosine substitution at the 3' splice acceptor site of intron 4 of the gene. The molecular characterization of CTX in Jews of Moroccan origin provides a definitive diagnosis of this treatable disease.

Adult↗

Local cutaneous reaction induced by subcutaneous interleukin-2 and interferon alpha-2a immunotherapy following ABMT.

Immunotherapy using subcutaneous injections of recombinant interleukin-2 (IL-2) and recombinant interferon alpha-2a (IFN-alpha) for advanced hematologic and solid tumors is rapidly developing. We report five patients with Hodgkin's and non-Hodgkin's lymphoma who developed a local cutaneous reaction consisting of inflammatory painful nodules with a central multiloculated vesicle at the site of sc injections of IL-2 and IFN-alpha immunotherapy following ABMT. This is the first report of a local cutaneous adverse reaction induced by IL-2 and IFN-alpha immunotherapy following ABMT.

Administration, Cutaneous↗

Increased numbers of mast cells in pemphigus vulgaris skin lesions. A histochemical study.

We have used a histochemical technique to study mast cells (MC) in skin biopsies of 8 patients suffering from pemphigus vulgaris (PV) and from 4 control volunteers. The MC were stained for 30 min with 0.5% toluidine blue, pH 0.5, counted and then restained for 5 days under the same conditions. This staining method allows the identification of two groups of MC, one that stains promptly (30 min) and one that stains after longer incubation times (5 days). After 30 min of staining, a slight increase was found in the number of MC in PV sections, in comparison with normal controls. However, when the 30 min stained sections were reincubated under the same conditions for 5 days, a significant increase in the number of MC in PV was found in comparison with 5-day-stained normal skin sections (p less than 0.005) and in comparison with 30-min-stained PV sections (p less than 0.005). The MC were distributed throughout the dermis and were concentrated in the upper dermis near hair follicles and vessels. The possible importance of the increased numbers of MC in PV is discussed.

Adult↗

Superficial actinic porokeratosis and cystic fibrosis.

A 24-year-old woman, presenting with cystic fibrosis, developed superficial actinic porokeratosis. Immunosuppression due to cystic fibrosis may be either the cause of or the exacerbating factor in superficial actinic porokeratosis in our patient.

Adult↗

Perioral psoriasis.

Two cases of perioral psoriasis are described. A brief review of various dermatologic conditions that should be included in the differential diagnosis is included.

Adult↗