Biomedical subjects
Publications and source records attributed to J D Wilkinson.
We present the case of a 28-year-old woman with multiple familial trichoepitheliomas, characterized by the presence of many small tumors occurring predominantly on the face. This condition constitutes a genodermatosis that follows an autosomal dominant mode of inheritance. These lesions can lead to marked facial disfigurement, and treatment is generally disappointing.
Fusidic acid is an antibiotic that belongs to a group of its own, the fusidanes. The molecule has a steroid-like structure but does not possess any steroid activity. The structure is thought to be responsible for the steroid-like high penetration, and for the fact that no cross-resistance or cross-allergy has been seen with other antibiotics in routine clinical use. The anti-microbial activity of fusidic acid is specifically aimed at the most common skin pathogens, including Staphylococcus aureus, towards which it is one of the most potent antibiotics. The place of fusidic acid in dermatology is in the treatment of mild to moderately severe skin and soft-tissue infections, e.g. impetigo, folicullitis, erythrasma, furunculosis, abscesses and infected traumatic wounds, whereas it is of less use in conditions such as hidradenitis suppurativa, chronic leg ulcers, burns and pressure sores. The topical combinations of fusidic acid with either betamethasone or hydrocortisone are extremely useful in the treatment of atopic dermatitis/eczema whenever staphylococcal/secondary infection is suspected, and in more persistent cases of eczema where staphylococcal superantigen may be playing an important exacerbating role.
The aims of this study were to utilize quantitative immunocytochemical techniques to determine the densities of keratin 16 (K16) and keratin 17 (K17) expressed by keratinocytes during the course of acute patch test reactions to sodium lauryl sulfate (SLS), and to relate these to the proliferative state of the epidermis, as assessed by Ki-67 immunolabelling. Significantly increased numbers of dividing keratinocytes were present in 48h and 96h reactions, concurrent with high levels of expression of K16 and more moderate expression of K17. Statistical analysis indicated a good correlation between K16 expression and the density of Ki-67+ keratinocytes present in the epidermis (r=0.843). This was not the case for K17 (r=0.396). The results demonstrate that both K16 and K17 expression are features of acute irritant contact dermatitis reactions, but suggest that the factors which influence and control their expression differ.
Four cases of cicatricial pemphigoid complicated by oesophageal involvement are presented. All patients suffered dysphagia but repeated radiological studies were required for confirmation of oesophageal ulceration, webs and strictures. A combination of systemic drug therapy and oesophageal dilatation were necessary for the suppression of symptoms. The clinical and immunopathological features, management and complications of oesophageal involvement in cicatricial pemphigoid are discussed. Dermatologists should be aware of these features and make regular inquiries about swallowing difficulties in patients with cicatricial pemphigoid to guide appropriate investigations and treatment.
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Acquired C1-esterase inhibitor deficiency is a rare syndrome which usually presents with episodes of angioedema. Most patients have an underlying lymphoproliferative disorder. It is important to gastroenterologists, haematologists, immunologists and dermatologists, as these are the more likely specialties to which the patient will present. Recognition of the syndrome is important not only for diagnostic purposes but for investigating and treating any associated underlying lymphoproliferative disorders. In some instances the angioedema may precede the lymphoma by many years. Optimal patient management requires that both angioedema and the underlying lymphoma be recognized and treated.
This article describes the results of an EECDRG multicentre study on contact allergy to corticosteroids. A total of 7238 patients were investigated: 6238 in 13 centres in the course of 1993, and 1000 patients in 1 centre in 1993 and 1994. The 5 corticosteroids tested were budesonide 0.1% pet., betamethasone-17-valerate 1% pet., clobetasol-17-propionate 1% pet., hydrocortisone-17-butyrate 1% eth., and tixocortol-21-pivalate 1% pet.; 189 (2.6%) gave a positive patchtest reaction (+, ++, + + +) to at least 1 of the corticosteroids. The data regarding the corticosteroid-sensitive patients, as well as the patchtest results, were recorded on a standardized form.
Partial lipoatrophy often presents with a characteristic 'cadaveric' facial appearance but may also present with manifestations of immune deficiency and renal disease. We present two cases illustrating all these features.
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A randomized, multicentre, double-blind, 2-period crossover study with 2 shampoos was performed on subjects patch-test-positive to 100 ppm CMI/MI. One shampoo was preserved with 15 ppm a.i. CMI/MI, the other with 0.3% imidazolidinyl urea (IU). 27 subjects from 5 European dermatology clinics participated. 1 subject discontinued use after severe adverse reactions to the CMI/MI-preserved shampoo and did not evaluate the other shampoo. Another 2 subjects developed moderate symptoms with the CMI/MI-preserved shampoo and discontinued its use, but tolerated the IU-preserved shampoo for the full 2-week period. 2 subjects discontinued use after 1 or 2 washes after severe adverse reactions to the IU-preserved shampoo. 1 of these subjects tolerated the CMI/MI-preserved shampoo for 2 weeks without any untoward effects. However, the majority of subjects had negative findings on the scalp, face, neck, and hands for both shampoos. The physicians' global evaluation data indicated that shampoo with CMI/MI caused fewer skin problems than shampoo with IU (38% versus 27%, n.s.), with over 1/3 of the subjects (35%) having no skin problems with either preservative. The current study showed that most subjects previously sensitized to CMI/MI can successfully use shampoo preserved with CMI/MI. Since some subjects previously sensitized to CMI/MI, or possibly to IU, may develop clinical reactions, it would still be prudent for the clinician to advise alternative products to patients with sensitivity to a shampoo or cosmetic ingredient. Full ingredient labelling will ensure that this is possible. As the overall rate of adverse effects in sensitized individuals was low, studies of this nature should also be conducted for other allergens.(ABSTRACT TRUNCATED AT 250 WORDS)
In a multicentre study, the value of adding sorbitan sesquioleate (SSO) to the constituents of the 8% fragrance mix (FM) was investigated. In 7 centres, 709 consecutive patients were tested with 2 types of FM from different sources, its 8 constituents with 1% SSO, its 8 constituents without SSO, and 20% SSO. 5 patients (0.71%) reacted to the emulsifier SSO itself, read as definitely allergic on day 3/4. 53 patients reacted to either one of the mixes with an allergic type of reaction. When tested with the constituents without SSO, 41.5% showed an allergic reaction versus 54.7% with SSO. If both types of reactions were considered (allergic and irritant) 38.3% of 73 patients showed a positive "breakdown" result without SSO, versus 54.8% with SSO. The differences were statistically significant. Reactivity to FM constituents was changed in a specific pattern by addition of SSO--irritant reactions increased, particularly for cinnamic alcohol, eugenol, geraniol, oak moss and hydroxycitronellal, whereas others showed only a slight change. Allergic reactions were also increased by SSO, but the rank order of the top 3 sensitizers (isoeugenol, oak moss and eugenol) did not change. Cinnamic alcohol was the only constituent with decreased reactivity after addition of SSO. A positive history of fragrance sensitivity (HFS) was clearly associated with a positive allergic reaction to either the mix or 1 of its constituents (51% versus 28.6% with a negative HFS). Irritant reactions were linked to a negative HFS in a high proportion (64.3%).(ABSTRACT TRUNCATED AT 250 WORDS)
BACKGROUND: While there are reports on the outcome in adults and teenagers with cystic fibrosis of colonisation with Burkholderia (Pseudomonas) cepacia, there is little information in children. METHODS: In December 1991 only one of 115 children with cystic fibrosis attending a paediatric centre was colonised with B cepacia. Over the next 12 months there was a rapid increase with 23 (20%) becoming colonised; eighteen (79%) of these became colonised in hospital at a time that overlapped with the admission of a B cepacia positive child. Three different bacteriocin types were isolated, with one type (S22/PO) being present in 17 (74%) patients. The outcome for children who became colonised with B cepacia was compared with that in 33 children who continued to be colonised with Pseudomonas aeruginosa alone. RESULTS: Children colonised with B cepacia were older and more poorly nourished than those colonised with P aeruginosa, but did not have poorer pulmonary function. After colonisation, the forced expiratory volume in one second (FEV1) deteriorated between consecutive annual tests, with the average deterioration being greater in those with higher initial levels. Five children with B cepacia died from respiratory failure although none showed a fulminant deterioration. Introduction of segregation measures within hospital led to a dramatic decrease in the number of newly colonised patients. CONCLUSIONS: This study provides further evidence for person-to-person spread of B cepacia and confirms the effectiveness of simple isolation measures in interrupting spread. Colonisation with B cepacia and P aeruginosa in children is associated with a more rapid decline in lung function and a significantly increased mortality compared with cases colonised with P aeruginosa alone.
OBJECTIVE: To examine the utility of gastric tonometry as an early indicator of morbidity and mortality in pediatric patients with septic shock. DESIGN: Prospective clinical study. SETTING: Multidisciplinary pediatric ICU. PATIENTS: Eight critically ill pediatric patients with septic shock and with pulmonary artery and gastric tonometry catheters in place. INTERVENTIONS: Standard perfusional data including cardiac index, oxygen content (arterial, mixed venous), oxygen delivery, oxygen consumption, urine output, capillary refill, and lactate value were measured every 4 h. Intramucosal pH (pHi) was calculated from simultaneous tonometric measurements of gastric intramucosal PCO2 (PiCO2). Mean pHi values were compared between survivors and nonsurvivors. Tonometric data were compared with standard perfusional data by regression analysis. Low pHi values (< 7.35) were temporally compared with occurrence screens for adverse clinical events, (cardiopulmonary arrest, acute hemorrhage, and significant hypotension or dysrhythmias). MEASUREMENTS AND MAIN RESULTS: Sets (n = 108) of paired data were collected from 8 patients (age 7 to 168 months) representing 4 deaths. The mean pHi in nonsurvivors (7.32 +/- 0.18) was significantly lower than that in survivors (7.48 +/- 0.07). Both pHi and PiCO2 were correlated with urine output by regression analysis. The pHi, but not PiCO2 correlated with extraction ratio and lactate level. There were no other significant correlations noted. Analysis of the ability of pHi values to predict adverse clinical events revealed that pHi less than 7.35 accurately predicted 26 of 39 concurrent or subsequent adverse clinical events. However, 24 of 50 pHi values less than 7.35 showed no association with similar adverse events, either concurrent or subsequent. The pHi had a sensitivity of 67%, a specificity of 74%, a positive predictive value of 52%, and an efficiency of 72% in predicting these adverse events. CONCLUSION: Use of gastric tonometry in pediatric septic shock patients appears to distinguish survivors from nonsurvivors. However, in general, tonometric assessment of splanchnic perfusion correlates poorly with standard clinical, hemodynamic, oxygen transport, or metabolic measurements of perfusion. Low pHi measurements are not predictive of concurrent or subsequent adverse clinical events.
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Injury is the leading cause of childhood death and disability in this country and has been for over a decade. Examination of the current injury statistics clearly demonstrates the failure of our present prevention strategies. Unless definitive steps are undertaken to initiate a comprehensive national injury prevention program, this tragic impact upon children will continue. To ensure that optimal care for injured children is provided, several criteria must be met. Seriously injured children require skillful assessment by clinicians experienced in pediatric trauma and rapid evacuation to a regional pediatric trauma center when appropriate. Their care is best provided by a multidisciplinary team including intensivists, surgeons, and rehabilitation specialists, all thoroughly familiar with the types of injuries children sustain. In the ICU, careful monitoring of circulatory, respiratory, renal, and neurologic status combined with early intervention for physiologic derangements maximizes the injured child's chances for recovery. Finally, it is important to understand that care for the injured child spans a continuum beginning with prevention, continuing through acute care, and ending only after rehabilitation and reintegration into the community is complete, and that each component is no less important than another. Only through these efforts can the loss of our nation's most precious resource be avoided.
A questionnaire was sent to 83 patients in the south Buckinghamshire area, in whom a primary diagnosis of keratosis pilaris (KP) had been made within the preceding 20 years. Of the 50 questionnaires returned, one was invalid, and 49 were evaluated. The age range of patients surveyed was 18 months-25 years; 61% were female and 39% male. Age of onset of KP was within the first decade in 51%, second decade in 35%, third decade in 12%, and fourth decade in 2%. Body sites affected were the arms (92%), legs (59%), face (41%), buttocks (30%) and eyebrows (8%). Other family members were affected by KP in 39%; 55% had no family history of KP, and 6% did not know. There was a personal history of atopy in 37%, 16% had associated dry skin, and 47% had neither. A seasonal variation in the severity of KP was noted in 80% of patients; 49% of these improved in the summer and 47% worsened in the winter. Overall, KP had improved with age in 35% of patients, remained unchanged in 43%, and worsened in 22%. The mean age of improvement was 16 years.
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