Search PubMed⌕ Search

Biomedical subjects

P Wolkenstein

Publications and source records attributed to P Wolkenstein.

At least 55 records · Page 3Linked to original sources
PubMed2000

SCORTEN: a severity-of-illness score for toxic epidermal necrolysis.

The mortality of toxic epidermal necrolysis is about 30%. Our purpose was to develop and validate a specific severity-of-illness score for cases of toxic epidermal necrolysis admitted to a specialized unit and to compare it with the Simplified Acute Physiology Score and a burn scoring system. A sample of 165 patients was used to develop the toxic epidermal necrolysis-specific severity-of-illness score and evaluate the other scores, a sample of 75 for validation. Model development used logistic regression equations that were translated into probability of hospital mortality; validation used measures of calibration and discrimination. We identified seven independent risk factors for death and constituted the toxic epidermal necrolysis-specific severity-of-illness score: age above 40 y, malignancy, tachycardia above 120 per min, initial percentage of epidermal detachment above 10%, serum urea above 10 mmol per liter, serum glucose above 14 mmol per liter, and bicarbonate below 20 mmol per liter. For each toxic epidermal necrolysis-specific severity-of-illness score point the odds ratio was 3.45 (confidence interval 2.26-5.25). Probability of death was: P(death) = elogit/1 + elogit with logit = -4.448 + 1.237 (toxic epidermal nec-rolysis-specific severity-of-illness score). Calibration demonstrated excellent agreement between expected (19. 6%) and actual (20%) mortality; discrimination was also excellent with a receiver operating characteristic area of 82%. The Simplified Acute Physiology Score and the burn score were also associated with mortality. The discriminatory powers were poorer (receiver operating characteristic area: 72 and 75%) and calibration of the Simplified Acute Physiology Score indicated a poor agreement between expected (9.1%) and actual (26.7%) mortality. This study demonstrates that the risk of death of toxic epidermal necrolysis patients can be accurately predicted by the toxic epidermal necrolysis-specific severity-of-illness score. The Simplified Acute Physiology Score and burn score appear to be less adequate.

Aged↗
PubMed2000

Comparison of systematic versus selective screening for methicillin-resistant Staphylococcus aureus carriage in a high-risk dermatology ward.

OBJECTIVE: To compare two strategies for screening methicillin-resistant Staphylococcus aureus (MRSA) carriers in a high-risk dermatology ward: systematic screening of all admitted patients versus selective screening of patients at risk. DESIGN: The two strategies were applied prospectively during two consecutive periods. In period A (8.5 months), only patients transferred from other wards, or with a history of prior hospitalization, or presenting chronic wounds or disease with denuded skin were considered at high risk of MRSA carriage and sampled. In period B (7.5 months), all admitted patients were systematically screened. End-points were the number of patients having a MRSA-positive screening sample on admission during period B and having none of the risk factors used in period A, the rate of imported MRSA cases, and the rate of acquired cases. SETTING: A 1,032-bed university hospital with a 19-bed inpatient dermatology ward, a referral center for toxic epidermal necrolysis and severe extensive dermatoses. PATIENTS: The study included 729 dermatology inpatients (370 in period A and 359 in period B). RESULTS: During period A, screening samples were obtained on admission for 30% of patients (77% of the patients at risk) and identified 25 MRSA carriers. During period B, 90.5% of admitted patients were screened, and 26 MRSA carriers were detected on admission; all of these patients belonged to at least one predefined category at risk for carriage. Overall rates of imported and acquired cases were similar between the two periods (6.8% vs 7.5%, and 2.9% vs 2.4%, respectively). CONCLUSIONS: A screening strategy targeted to patients at risk of harboring MRSA has similar sensitivity and is more cost-effective than a strategy of systematic screening to identify MRSA carriers on admission.

Cross Infection↗
PubMed2000

Prospective evaluation of detoxification pathways as markers of cutaneous adverse reactions to sulphonamides in AIDS.

The use of sulphonamides is complicated by a high rate of cutaneous reactions in AIDS. Metabolic risk factors have been suspected for these reactions. We conducted a prospective study to evaluate whether glutathione S-transferase M1 null genotype, glutathione deficiency and acetylator status as risk factors. To explain the high frequency of slow acetylator phenotype in AIDS patients, we compared N-acetyltransferase-2 phenotype and genotype in this population. AIDS patients treated with sulphonamides for Pneumocystis carinii pneumonia or toxoplasmosis were followed up for cutaneous reactions. Glutathione S-transferase genotyping, glutathione level determination, N-acetyltransferase-2 genotyping and phenotyping were performed. One hundred and thirty-six AIDS patients were studied. Glutathione S-transferase M1 and T1 null genotypes, intracellular glutathione level, slow acetylator genotype and phenotype were not risk factors for cutaneous sulphonamides reactions. The association of glutathione S-transferase M1 null genotype and the slow acetylator one was a risk factor [Fisher's exact test, odds ratio (OR) = 2.6, 95% confidence interval (CI) = 1.2-5.9; P = 0.02]. A discordance between acetylator genotype and phenotype was found in 35% of patients. This frequency was significantly higher than the 6-7% expected (Fisher's exact test: OR = 7.5, 95% CI = 4.2-13.4; P < 0.0001). Suspected metabolic risk factors for sulphonamides cutaneous reactions were not confirmed prospectively. However, the association of glutathione S-transferase M1 null genotype and the slow acetylator one appeared to increase the risk of reactions. We clearly showed that the acetylation phenotype measured by caffeine probe could be modified by the disease.

Acetylation↗
PubMed2000

[Digital clubbing].

Explore the source record for details and available documents.

Diagnosis, Differential↗
PubMed1999

[Clinical diagnosis of neurofibromatosis type 1].

UNLABELLED: TO DIAGNOSE NEUROFIBROMATOSIS TYPE 1 (NF1) AND TO DETECT ITS COMPLICATIONS: NF1 is the commonest autosomal dominant (1/3000 à 3500 births). Café au lait spots, axillary and inguinal freckling, hamartomas of the iris (Lisch nodules) and multiple cutaneous neurofibromas, characterize NF1. NF1 can be associated with optic gliomas, spinal or peripheral nerve neurofibromas, macrocephaly, cognitive et neurological disorders, une scoliosis and bone abnormalities. Morbidity and mortality of NF1 are linked to multisystemic complications. DIAGNOSIS: In adulthood, diagnosis of NF1 is easy with physical examination: presence of café au lait spots, axillary and inguinal frecklings, neurofibromas. In early childhood, diagnosis can be difficult. Penetrance of the gene is complete by the age of 5 and the diagnosis is made during the follow-up. COMPLICATIONS: Clinical examination can easily identify complications such as scoliosis, pseudarthrosis, hypertension linked to renal artery stenosis or phaeochromocytoma or learning disabilities. CONCLUSION: Annual clinical examination is sufficient for the follow-up of NF1 patients. Screening investigations are not useful because of rare complications, but symptomatic.

Humans↗
PubMed1999

Melanoma and tumor thickness: challenges of early diagnosis.

OBJECTIVE: To test the basic assumption of campaigns for early diagnosis of melanoma, ie, prognosis is correlated with the delay in the diagnosis. DESIGN: Prospective study of the correlation between delays to diagnosis, assessed using a questionnaire, and the Breslow thickness as a prognosis marker. SETTING: Dermatology departments in France. PATIENTS: Five hundred ninety consecutive patients, referred within 12 weeks after resection of cutaneous melanoma. MAIN OUTCOME MEASURES: Assessment of 5 successive time intervals from the first time the patients realized that they had a lesion until the resection of the melanoma, and results of the correlation between each time interval and tumor thickness (Breslow). RESULTS: There is a positive but weak correlation between tumor thickness and the delay to identify a lesion as suspicious (r = 0.17; P = .009). However, this delay tends to be short for the thickest tumors. There is a negative correlation between tumor thickness and the delay to seek medical attention (r = -0.20; P<.001). This delay was shorter for nodular melanoma. No correlation is found between melanoma thickness and physicians' delays. CONCLUSIONS: Poor prognosis can be accounted for by aggressive rapidly growing tumors rather than by delays. In well-informed populations, campaigns for early diagnosis of melanoma may thus no longer have a major impact on prognosis, unless they are focused on subgroups less accessible to information and medical care.

Female↗
PubMed1999

The treatment of mild pemphigus vulgaris and pemphigus foliaceus with a topical corticosteroid.

Seven patients with mild pemphigus vulgaris (n = 3) or pemphigus foliaceus (n = 4) were treated with a very potent topical corticosteroid alone. Clobetasol propionate 0.05% cream was applied to mucosal lesions and involved skin twice a day for at least 15 days, then progressively tapered. Pemphigus was considered to be controlled if healing of lesions was obtained, with a 75% decrease in the number of new lesions per week without addition of any systemic treatment. In all seven patients, the disease was controlled initially with healing of cutaneous lesions within 15 days, while healing of mucosal lesions took at least 1 month. In four patients, remission was maintained with topical corticosteroid alone for a mean 19-month follow-up. In three patients, relapse occurred after 2-11 months, requiring a systemic treatment.

Administration, Topical↗
PubMed1999

Neurological complications of neurofibromatosis type 1 in adulthood.

Neurofibromatosis type 1 (NF1) is a genetic disease with a wide range of neurological manifestations. To examine these, and to evaluate neurological morbidity in adulthood of patients with NF1, we studied a hospital-based series of 158 patients that included 138 adult patients aged >18 years and 20 children. NF1 evaluation included a multidisciplinary clinical and a clinically oriented radiological investigation. Neurological events occurring during childhood (in both children and adults of the series) and adulthood were recorded. One or several neurological manifestations have been observed in 55% of patients (adults and children) (n = 87). These included: headache (28 patients); hydrocephalus (7); epilepsy (5); lacunar stroke (1); white matter disease (1); intraspinal neurofibroma (3); facial palsy (1); radiculopathy (5); and polyneuropathy (2). Tumours included: optic pathway tumours (20); meningioma (2); cerebral glioma (3); and malignant peripheral nerve sheath tumours (6). Life-threatening complications were observed in five adults and included four malignant peripheral nerve sheath tumours and one meningioma. Pain was the leading symptom in 11 adults and was related to malignant peripheral nerve sheath tumours, complications of intraspinal neurofibromas, subcutaneous neurofibromas and peripheral nerve neurofibromas. NF1 in adults was not associated with other disabling or life-threatening neurological complications. Symptomatic optic pathway tumours, cerebral gliomas, symptomatic aqueductal stenosis and spinal compression due to intraspinal NF were observed exclusively during childhood. In this series, the predominant neurological features of adults with NF1 were chronic pain and malignant peripheral nerve sheath tumours.

Adolescent↗
PubMed1999

[Hand-foot-mouth syndrome recurring during common variable deficiency].

BACKGROUND: Common variable immunodeficiency is characterized by hypogammaglobulinemia and recurrent bacterial infections. More uncommonly, these patients develop chronic enterovirus infectious meningoencephalitis. Recurrent enterovirus skin infection has not been reported to date in subjects with common variable immunodeficiency. CASE REPORT: A 26-year-old man had suffered repeated episodes of otorhinolaryngological and pulmonary infections since childhood. He experienced three episodes of vesicular cutaneous eruption involving the palms of both hands, the plantar aspect of the feet and the buccal mucosa. The patient was hospitalized in March 1995 at the third episode. Temperature was 38 degrees C. He had maculopapulous and vesicular eruptions on the palm of the hands and plantar aspect of the feet with irritation in some areas. Petichial lesions were seen on the palate. PCR demonstrated viral DNA and cell cultures of a lesion biopsy were positive for enterovirus. Gammaglobulinemia was 4 g/l with low B cell count. DISCUSSION: Viral infections are uncommon in patients with common variable immunodeficiency as cellular immunity remains normal. Severe viral infections caused by enteroviruses have however been reported, generally associating chronic, and generally fatal, meningoencephalitis. Our case would be the first case of a recurrent hand, foot and mouth disease in such patients.

Adult↗
PubMed1998

Randomised comparison of thalidomide versus placebo in toxic epidermal necrolysis.

BACKGROUND: Toxic epidermal necrolysis (TEN) is associated with a 30% death rate. Tumour necrosis factor alpha (TNF-alpha) has been implicated in the pathogenesis of TEN. Thalidomide is a potent inhibitor of TNF-alpha action. We did a double-blind, randomised, placebo-controlled study of thalidomide in TEN. METHODS: The patients received a 5-day course of thalidomide 400 mg daily or placebo. The main endpoint was the progression of skin detachment after day 7. Secondary endpoints were the severity of the disease, evaluated with the simplified acute physiology score (SAPS), and the mortality. TNF-alpha and interleukin 6 were measured. FINDINGS: The study was stopped because there was excess mortality in the thalidomide group--ten of 12 patients died compared with three of ten in the placebo group (Fisher's exact test with Katz's approximation, relative risk=2.78, p=0.03). After adjustment for SAPS, mortality remained significantly higher in the thalidomide group than in the placebo group (exact logistic regression mid-p=0.007; 95% CI for odds ratio 2.7 to infinity). Plasma TNF-alpha concentration was higher in the thalidomide group than the placebo group on day 2, though the difference was not significant (Wilcoxon rank-sum test p=0.07). INTERPRETATION: Even though few patients were included, our data suggest that thalidomide is detrimental in TEN, possibly because of a paradoxical enhancement of TNF-alpha production.

Adult↗