PubMed Health⌕ Search

Biomedical subjects

R J Bosch

Publications and source records attributed to R J Bosch.

At least 19 recordsLinked to original sources

Low maternal vitamin B12 status is associated with intrauterine growth retardation in urban South Indians.

OBJECTIVE: To assess the maternal sociodemographic, anthropometric, dietary and micronutrient status in apparently healthy pregnant women in order to determine their associations with intrauterine growth retardation (IUGR). DESIGN: Prospective observational study. SETTING: Bangalore City, India. SUBJECTS: A total of 478 women were recruited at 12.9+/-3.3 weeks of gestation and followed up at the first, second and third trimesters of pregnancy and at delivery. The dropout rate was 8.5%. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Birth weight was measured at hospital delivery. RESULTS: The mean birth weight was 2.85+/-0.45 kg. In all, 28.6% of newborns were IUGR. There was a strong inverse relationship between maternal educational level and risk of IUGR. A low body weight at baseline was also associated with a high risk of IUGR. Compared with women in the highest quartile for second trimester weight gain, those in the lowest quartile had a significantly higher adjusted odds ratio (AOR: 3.98; 95% CI: 1.83, 8.65) for IUGR. Women in the lowest tertile for serum vitamin B(12) concentration during each of the three trimesters of pregnancy had significantly higher risk of IUGR (AOR: 5.98, 9.28 and 2.81 for trimesters 1-3, respectively). CONCLUSIONS: The present study demonstrates associations between educational status, maternal weight and gestational weight gain with IUGR. Importantly, in a subsample, there were strong associations of vitamin B(12) status with IUGR, suggesting that better socioeconomic conditions, improved nutritional status and early detection of vitamin B(12) deficiency in pregnancy combined with appropriate interventions are likely to play an important role in reducing IUGR.

Adolescent↗

Wasting and body composition of adults with pulmonary tuberculosis in relation to HIV-1 coinfection, socioeconomic status, and severity of tuberculosis.

OBJECTIVE: To examine the impact of HIV coinfection, socioeconomic status (SES) and severity of tuberculosis (TB) on the body composition and anthropometric status of adults with pulmonary TB. DESIGN: Cross-sectional study. SETTING: Five TB clinics in Dar es Salaam, Tanzania. SUBJECTS: A total of 2231 adult men and women diagnosed with pulmonary TB, prior to the initiation of anti-TB therapy. METHODS: We compared the distribution of anthropometric characteristics including body mass index (BMI), mid-upper arm circumference (MUAC), triceps skin-fold (TSF), and arm muscle circumference (AMC) by HIV status, SES characteristics, and indicators of TB severity (bacillary density in sputum and Karnofsky performance score). Similar comparisons were carried out with body composition variables from bioelectrical impedance analysis and albumin concentrations, in a subsample of 731 subjects. RESULTS: In multivariate analysis, HIV infection was significantly associated with lower MUAC and AMC in both men and women, but not with BMI or TSF. Compared to HIV-uninfected women, those who were HIV infected had lower body cell mass (BCM) (adjusted difference = -0.85 kg, P = 0.04), intracellular water (-0.68 l, P = 0.04), and phase angle (-0.52, P = 0.02). Albumin concentrations were significantly lower in both men and women infected with HIV. Among HIV-infected men, CD4 cell counts <200/mm(3) were related to lower intracellular water, BCM, fat-free mass and phase angle. Independent of HIV infection, BMI and MUAC were positively related to SES indicators and the Karnofsky performance score; and inversely related to bacillary density. CONCLUSIONS: HIV infection is associated with indicators of low lean body mass in adults with TB; socioeconomic factors and TB severity are important correlates of wasting, independent of HIV. SPONSORSHIP: The National Institute of Allergy and Infectious Diseases (UO1 AI 45441-01).

AIDS-Related Opportunistic Infections↗

The parathyroid hormone-related protein system and diabetic nephropathy outcome in streptozotocin-induced diabetes.

The pathophysiology of the diabetic kidney (e.g., hypertrophy, increase urinary albumin excretion (UAE) is still ill-defined. Parathyroid hormone-related protein (PTHrP) is overexpressed in several nephropathies, but its role remains unclear. We evaluated the effect of high glucose on PTHrP and the PTH1 receptor (PTH1R) protein (by Western blot and immunohistochemistry) in the kidney of mice ith streptozotocin-induced diabetes, and in several mouse renal cells in vitro. Diabetic mice showed a significantly increased renal expression of PTHrP and PTH1R proteins with 2-8 weeks from the onset of diabetes. These animals exhibited an intense immunostaining for both proteins in the renal tubules and glomeruli. Using transgenic mice overexpressing PTHrP targeted to the renal proximal tubule, we found a significant increase in the renal hypertrophy index and in UAE in these diabetic mice relative to their control littermates. Moreover, logistic regression analysis showed a significant association between both PTHrP and PTH1R protein levels and UAE in all diabetic mice throughout the study. High-glucose (25 mm) medium was found to increase PTHrP and PTH1R in tubuloepithelial cells, mesangial cells and podocytes in vitro. Moreover, this increase in PTHrP (but not that of PTH1R) was inhibited by the AT1 receptor antagonist losartan. Collectively, these results indicate that the renal PTHrP/PTH1R system is upregulated in streptozotozin-induced diabetes in mice, and appears to adversely affect the outcome of diabetic renal disease. Our findings also suggest that angiotensin II might have a role in the PTHrP upregulation in this condition.

Angiotensin II↗

HIV co-infection, CD4 cell counts and clinical correlates of bacillary density in pulmonary tuberculosis.

BACKGROUND: Sputum microscopy for acid-fast bacilli (AFB) is the commonest diagnostic method for pulmonary tuberculosis (PTB) in developing countries. The method is reported to be less sensitive in human immunodeficiency virus (HIV) positive compared to negative patients. We determined the bacillary density in sputum of smear-positive PTB patients and related it to the patients' HIV status, CD4 cell count, clinical and demographic characteristics. METHODS: Three sputum samples per patient were examined using microscopy before initiating therapy. The AFB density was graded according to World Health Organization recommendations. The smear with the highest density was used. High bacillary density was defined as >10 AFB/field. HIV status and CD4 cell count were determined according to the national guidelines. RESULTS: Of 844 patients, 433 (51.3%) were HIV-positive. High bacillary density was significantly less common among HIV-positive (39.0%) than -negative (75.7%) patients (prevalence ratio 0.52; 95%CI 0.45-0.59, P < 0.0001). Among HIV-positive patients, the proportion of those with high bacillary density increased progressively with CD4 cell counts (P = 0.003). CONCLUSION: HIV is associated with lower AFB concentration in sputum. The AFB density falls with falling CD4 cell count. Microscopy for AFB in sputum may be less sensitive in diagnosing PTB when HIV infection is present, especially in severely immunocompromised patients.

Adult↗

Predictors of incident tuberculosis among HIV-1-infected women in Tanzania.

SETTING: The development of tuberculosis (TB) in HIV-1-infected individuals is associated with accelerated HIV-1 disease progression. OBJECTIVE: To examine the predictors of incident TB in HIV-1-infected Tanzanian women. DESIGN: A prospective cohort of 1078 HIV-1-infected pregnant women was enrolled in a randomized clinical trial to examine the role of vitamin supplements in HIV-1 disease progression and fetal outcomes. RESULTS: Of 1008 women evaluated for TB, 88 (8.7%) developed TB. After controlling for age, education and hemoglobin concentration, in multivariate analysis, low CD4 cell count, elevated erythrocyte sedimentation rate (ESR), decreased mid-upper arm circumference, and high viremia were associated with an increased risk of TB. CD4 <200 vs. > or = 500 cells/mm3 was associated with a 4.44-fold increase in risk of TB (95%CI 2.10-9.40). Individuals with high viremia (> or = 50,000 copies/ml) had a 2.43-fold increase in risk of TB (95%CI 1.24-4.76). Elevated malarial parasite density was slightly associated with a 65% (95%CI 19-85) decreased risk of TB. CONCLUSIONS: The risk of developing TB was elevated among women with low CD4 cell counts, elevated ESR, coinfections with other pathogens, poor nutrition and high viremia. There is a slight inverse association between malarial infection and TB, possibly because treating malaria may reduce the risk of TB.

AIDS-Related Opportunistic Infections↗

Human immunodeficiency virus infection, diarrheal disease and sociodemographic predictors of child growth.

AIM: To compare growth patterns between human immunodeficiency virus (HIV)-infected and -uninfected preschool children. To examine the associations between diarrheal and respiratory infections, sociodemographic factors and growth. METHODS: A longitudinal study was conducted among 524 children who were 6-60 mo of age at recruitment. Information on sociodemographic characteristics was collected at baseline from the caregiver. Hemoglobin, malaria infection and HIV status of the children were assessed from a blood sample. Monthly height (length if <24 mo) and weight measurements were obtained, and clinical assessments carried out, during an average 12 mo follow-up period. Yearly increments in height and weight were compared by HIV status, incidence of diarrhea and respiratory infections, and levels of sociodemographic variables. RESULTS: After adjusting for maternal education, anemia and vitamin A supplementation, HIV infection was related to 2.8 cm [95% confidence interval (95% CI) 0.6, 5.0] and 1.3 kg (95% CI 0.0, 2.5) lower yearly length and weight gains, respectively, in children who were between 6 and 11 mo old at baseline. Among children who were 12-23 mo old at recruitment, HIV infection was associated with 0.6 kg (95% CI 0.1, 1.0) less yearly weight gain. HIV infection was not related to linear or ponderal growth in children >24 mo old. Maternal illiteracy, severe child anemia and episodes of acute diarrhea were additional risk factors for growth delay in length. CONCLUSION: HIV infection is associated with linear and ponderal growth retardation in children aged <24 mo. Additional predictors of linear growth retardation include preventable conditions such as poor maternal education, child anemia and diarrheal disease.

Body Height↗

Predictors of virologic and clinical outcomes in HIV-1-infected patients receiving concurrent treatment with indinavir, zidovudine, and lamivudine. AIDS Clinical Trials Group Protocol 320.

BACKGROUND: A substantial proportion of patients with HIV infection will not respond to antiretroviral therapy. Early predictors of response to treatment are needed to identify patients who are at risk for treatment failure. OBJECTIVE: To determine predictors of virologic and clinical response to indinavir, zidovudine, and lamivudine therapy. DESIGN: Observational analysis of one treatment group in a phase III trial. SETTING: 40 AIDS Clinical Trials units. PATIENTS: 489 patients receiving indinavir, zidovudine, and lamivudine who had 1) a CD4 count of 0.200 x 10(9) cells/L or less after 8 or more weeks of study therapy and 2) plasma HIV-1 RNA measurements obtained at baseline and week 8. MEASUREMENTS: HIV-1 RNA level and CD4 cell count at weeks 0, 4, 8, 24, and 40. Clinical progression was defined as a new AIDS-defining illness or death. RESULTS: Patients' levels of HIV-1 RNA at the 8th study week of therapy predicted whether patients would achieve virologic suppression to below 500 (or 50) copies/mL at study week 24. An HIV-1 RNA level less than 500 copies/mL at week 24 was achieved in 71% of patients whose level at week 8 had been less than 500 copies/mL, 53% of those with a level of 500 copies/mL or more and at least 2-log(10) copies/mL reduction since baseline, 29% of those with a level of 500 copies/mL or more with a 1- to 1.99-log(10) copies/mL reduction, and 9% of those with a level of 500 copies/mL or greater and less than 1-log(10) copies/mL reduction since baseline (P < 0.001). HIV-1 RNA level at week 8 also predicted clinical progression. HIV-1 disease progressed in 2.2% of the patients with a week-8 HIV-1 RNA level less than 500 copies/mL, 2.3% of patients with 500 copies/mL or greater and at least 2-log(10) copies/mL reduction since baseline, 4.9% of patients with 500 copies/mL or greater and 1- to 1.99-log(10) copies/mL reduction since baseline, and 10.6% of patients with 500 copies/mL or greater and less than 1-log(10) copies/mL decrease since baseline (P = 0.009). After adjustment for HIV-1 RNA level, patients with a higher week-8 CD4 cell count were more likely to have a week-24 HIV-1 RNA level less than 500 copies/mL (relative risk for patients with a week-8 CD4 count >/= 0.10 x 10(9) cells/L, 1.47 [95% CI, 1.00 to 2.16] compared with <0.050 x 10(9) cells/L; relative risk for patients with a week-8 CD4 count of 0.05 to 0.099 x 10(9) cells/L, 0.98 [CI, 0.61 to 1.57] compared with <0.050 x 10(9) cells/L). After adjustment for HIV-1 RNA level, patients with a week-8 CD4 count of 0.05 x 10(9) cells/L or greater (compared with <0.05 x 10(9) cells/L) had a decreased hazard for clinical progression (hazard ratio, 0.25 [CI, 0.09 to 0.67]). CONCLUSIONS: The HIV-1 RNA level and CD4 cell count achieved at 8 weeks of treatment are important predictors of subsequent virologic and clinical outcomes.

Adult↗

Nelfinavir, efavirenz, or both after the failure of nucleoside treatment of HIV infection.

BACKGROUND: The optimal antiretroviral treatment for patients who have human immunodeficiency virus (HIV) viremia despite treatment with nucleoside reverse-transcriptase inhibitors (nucleoside analogues) remains uncertain. We studied treatment with regimens that combined two nucleoside analogues, at least one of which was new, with the protease inhibitor nelfinavir, the nonnucleoside reverse-transcriptase inhibitor efavirenz, or both. METHODS: The study included 195 patients who had been treated with nucleoside analogues only, and had a plasma HIV type 1 (HIV-1) RNA level of at least 500 copies per milliliter. Patients were randomly assigned to receive, in addition to two nucleoside analogues, nelfinavir, efavirenz, or nelfinavir plus efavirenz. The primary end point was a plasma HIV-1 RNA level of less than 500 copies per milliliter at week 16. A secondary end point was the composite of the HIV-1 RNA levels measured at weeks 40 and 48. RESULTS: At week 16 and at weeks 40 and 48, the proportions of patients in whom a plasma HIV-1 RNA level of less than 500 copies per milliliter was achieved were, respectively, 81 percent and 74 percent in the nelfinavir-plus-efavirenz group, 69 percent and 60 percent in the efavirenz group, and 64 percent and 35 percent in the nelfinavir group. Quadruple therapy resulted in a higher rate of viral suppression in both the short term (P=0.03) and the long term (P=0.001) than did triple therapy with nelfinavir. Triple therapy with efavirenz conferred a higher rate of long-term suppression than triple therapy with nelfinavir (P=0.004). Quadruple therapy also achieved a higher rate of virologic suppression than triple therapy with efavirenz (P=0.008). CONCLUSIONS: In HIV-infected patients previously treated with nucleoside analogues, treatment with nelfinavir plus efavirenz and at least one new nucleoside analogue achieves a higher rate of viral suppression than do regimens with nucleoside analogues and nelfinavir or efavirenz alone.

Adult↗

Up-regulation of parathyroid hormone-related protein in folic acid-induced acute renal failure.

BACKGROUND: Parathyroid hormone (PTH)-related protein (PTHrP) is present in many normal tissues, including the kidney. Current evidence supports that PTHrP is involved in renal pathophysiology, although its role on the mechanisms of renal damage and/or repair is unclear. Our present study examined the changes in PTHrP and the PTH/PTHrP receptor (type 1) in folic acid-induced acute renal failure in rats. The possible role of PTHrP on the process of renal regeneration following folic acid administration, and potential interaction between angiotensin II (Ang II) and endothelin-1, and PTHrP, were examined in this animal model. METHODS: PTHrP, PTH/PTHrP receptor, ACE, and preproendothelin-1 (preproET-1) mRNA levels in the rat kidney were analyzed by reverse transcription-polymerase chain reaction (RT-PCR) and/or RNase protection assay. Immunohistochemistry also was performed for PTHrP, the PTH/PTHrP receptor, and Ang II in the renal tissue of folic acid-injected rats. The role of PTHrP on tubular cell proliferation following folic acid injury was investigated in vitro in rat renal epithelial cells (NRK 52E). PTHrP secretion in the medium conditioned by these cells was measured by an immunoradiometric assay specific for the 1-36 sequence. RESULTS: Using RT-PCR, PTHrP mRNA was rapidly (1 hour) and maximally increased (3-fold) in the rat kidney after folic acid, decreasing after six hours. At 72 hours, renal function was maximally decreased in these rats, associated with an increased PTHrP immunostaining in both renal tubules and glomeruli. In contrast, the PTH/PTHrP receptor mRNA (RNase protection assay) decreased shortly after folic acid administration. Moreover, PTH/PTHrP receptor immunostaining dramatically decreased in renal tubular cell membranes after folic acid. A single subcutaneous administration of PTHrP (1-36), 3 or 50 microg/kg body weight, shortly after folic acid injection increased the number of tubular cells staining for proliferating cell nuclear antigen by 30% (P < 0.05) or 50% (P < 0.01), respectively, in these rats at 24 hours, without significant changes in either renal function or calcemia. On the other hand, this peptide failed to modify the increase (2-fold over control) in ACE mRNA, associated with a prominent Ang II staining into tubular cell nuclei, in the kidney of folic acid-treated rats at this time period. The addition of 10 mmol/L folic acid to NRK 52E cells caused a twofold increase in PTHrP mRNA at six hours, without significant changes in the PTH/PTHrP receptor mRNA. The presence of two anti-PTHrP antibodies, with or without folic acid, in the cell-conditioned medium decreased (40%, P < 0.01) cell growth. CONCLUSIONS: Renal PTHrP was rapidly and transiently increased in rats with folic acid-induced acute renal failure, featuring as an early response gene. In addition, changes in ACE and Ang II expression were also found in these animals. PTHrP induces a mitogenic response in folic acid-damaged renal tubular cells both in vivo and in vitro. Our results support the notion that PTHrP up-regulation participates in the regenerative process in this model of acute renal failure and is a common event associated with the mechanisms of renal injury and repair.

Acute Kidney Injury↗

Likelihood-based modeling and analysis of data underdispersed relative to the Poisson distribution.

By using a generalization of the Poisson process, distributions can be constructed that show appropriate amounts of underdispersion relative to the Poisson distribution that may be apparent from observed data. These are then used to examine the differences between the distributions of numbers of fetal implants in mice corresponding to different doses of the herbicide 2,4,5-T.

2,4,5-Trichlorophenoxyacetic Acid↗

Mucocutaneous leishmaniasis in a patient with the human immunodeficiency virus.

We report a case of mucocutaneous leishmaniasis (MCL) in a patient with the human immunodeficiency virus (HIV), Centers for Disease Control (CDC) Stage A2, with no previous history of cutaneous or systemic leishmaniasis. The patient had not travelled outside the province of Málaga, on the Mediterranean coast of southern Spain, so that it concerns an indigenous case, extremely unusual in this area. The HIV infection may well have influenced the defence against leishmania, but the exact mechanism by which this occurred is unknown.

Adult↗

Considerations in choosing a primary endpoint that measures durability of virological suppression in an antiretroviral trial.

OBJECTIVES: At present, many clinical trials of anti-HIV-1 therapies compare treatments by a primary endpoint that measures the durability of suppression of HIV-1 replication. Several durability endpoints are compared. DESIGN: Endpoints are compared by their implicit assumptions regarding surrogacy for clinical outcomes, sample size requirements, and accommodations for inter-patient differences in baseline plasma HIV-1-RNA levels and in initial treatment response. METHODS: Virological failure is defined by the non-suppression of virus levels at a prespecified follow-up time T(early virological failure), or by relapse. A binary virological failure endpoint is compared with three time-to-virological failure endpoints: time from (i) randomization that assigns early failures a failure time of T weeks; (ii) randomization that extends the early failure time T for slowly responding subjects; and (iii) virological response that assigns non-responders a failure time of 0 weeks. Endpoint differences are illustrated with Agouron's trial 511. RESULTS: In comparing high with low-dose nelfinavir (NFV) regimens in Agouron 511, the difference in Kaplan-Meier estimates of the proportion not failing by 24 weeks is 16.7% (P = 0.048), 6.5% (P = 0.29) and 22.9% (P = 0.0030) for endpoints (i), (ii) and (iii), respectively. The results differ because NFV suppresses virus more quickly at the higher dose, and the endpoints weigh this treatment difference differently. This illustrates that careful consideration needs to be given to choosing a primary endpoint that will detect treatment differences of interest. CONCLUSION: A time from randomization endpoint is usually recommended because of its advantages in flexibility and sample size, especially at interim analyses, and for its interpretation for patient management.

Anti-HIV Agents↗

Mechanisms of cGMP-dependent mesangial-cell relaxation: a role for myosin light-chain phosphatase activation.

Although the cGMP-dependent relaxation of contractile cells seems to depend on the ability of the cyclic nucleotide to interfere with intracellular calcium, this does not appear to be the only mechanism involved. The present experiments were designed to analyse alternative mechanisms, trying to test the hypothesis that cGMP could relax rat mesangial cells by activating myosin light-chain phosphatase (MLC-PP), with the subsequent dephosphorylation of myosin light chain (MLC). The effect of a cGMP analogue, dibutyryl cGMP (dbcGMP), on angiotensin II-(AII) and PMA-induced MLC phosphorylation (MLCP) was tested, in the presence of calyculin A (CA), an inhibitor of MLC-PP. MLCP was measured, after cell labelling with (32)P, by immunoprecipitation. dbcGMP prevented the increased MLCP induced by AII or PMA, and this inhibition was blocked by CA. dbcGMP also increased the MLC dephosphorylation observed in cells incubated with AII and in which MLC kinase and protein kinase C activities were blocked. The AII-elicited increased intracellular calcium concentration was only partially inhibited by dbcGMP. These results suggest that the cGMP-induced mesangial-cell relaxation could be due, at least partially, to the stimulation of MLC-PP.

Angiotensin II↗

Effect of resident call status on ABSITE performance. American Board of Surgery In-Training Examination.

BACKGROUND: Long work hours that result in fatigue may adversely affect cognitive function. Chronically sleep-deprived surgical residents fear that being on call the night before sitting for a standardized test puts them at a potential disadvantage. We examined American Board of Surgery In-Training Examination (ABSITE) scores to assess the effect of call status on exam performance. METHODS: Results of the 1994 ABSITE for 424 residents in 15 New England general surgery programs were collected. We compared standard scores of residents for the total test, clinical management, and basic science components with resident call status (on/off) for the night before the exam. RESULTS: Differences were apparent in total test scores (mean: off, 496.1; on, 466.0; P <.03) and clinical management scores (mean: off, 504.3; on, 470.6; P <.02) (t test, Mann-Whitney U test). Multivariate analyses revealed that differences in postgraduate year level and training track were significant contributors to differences in scores in all test components (analysis of covariance). Call status was not a significant factor in score variation after adjusting for these 2 factors. CONCLUSIONS: Differences in ABSITE scores of residents were related to postgraduate year level and training track. Call status had no significant effect on ABSITE performance.

Cognition↗

Cutaneous cryptococcosis in two patients with acquired immunodeficiency syndrome.

We present 2 cases of systemic cryptococcosis with cutaneous involvement in patients with acquired immunodeficiency syndrome (AIDS). Both patients were male, intravenous drug abusers, 31 and 35 years old, with severe immunodepression and a CD4+ lymphocyte count of 10/ml and 1/ml, respectively. They both had papular lesions reminiscent of molluscum contagiosum and in one patient with concomitant systemic leishmaniasis, there were spores of Cryptococcus neoformans coexisting with the leishmanias in the cutaneous lesions, constituting the first reported case of this particular association. Both patients responded well to amphotericin B followed by fluconazole.

AIDS-Related Opportunistic Infections↗

Effects of parathyroid hormone-related protein on human mesangial cells in culture.

Parathyroid hormone (PTH) and PTH-related protein (PTHrP) produce similar biological effects through the PTH/PTHrP receptor. Because PTHrP exhibits vasodilatory properties, we evaluated the hypothesis that this hormone interacts with human mesangial cells (HMC). The PTHrP prevented both the expected reduction in the planar cell surface area and the increase in myosin light-chain phosphorylation induced by platelet-activating factor (PAF) on HMC, in a dose-dependent manner. This effect was completely blocked by pertussis toxin and dideoxyadenosine, suggesting that a G protein-coupled receptor and cAMP are important in the PTHrP transduction mechanism. Moreover, PTHrP increased cAMP synthesis and thymidine incorporation in HMC. However, whereas RT-PCR and Southern and Northern blot analyses demonstrated the expression of human PTH/PTHrP receptor in human kidney cortex, no expression could be demonstrated in HMC. These results show that PTH and PTHrP directly interact with mesangial cells. These effects might be mediated by a receptor different from the PTH/PTHrP receptor.

Antihypertensive Agents↗

Squamous cell carcinoma secondary to recessive dystrophic epidermolysis bullosa: report of eight tumours in four patients.

BACKGROUND: The development of squamous cell carcinomas is the main cause of death of patients with Dystrophic epidermolysis bullosa. We think it is of interest to know their clinical characteristics and the treatment difficulties they cause. METHODS: We followed the clinical evolutions and carried out histopathological studies of eight primary cutaneous squamous cell carcinomas on three males and one female with recessive dystrophic epidermolysis bullosa. Patient ages ranged from 16 to 34 years (mean 27). RESULTS: Two patients had two tumours and one three, all were in characteristically scarred skin; four on upper limbs; four on lower. Maximum tumour dimensions ranged from 2 to 28 cm (mean 13), and represented 6 or more months of evolution. In three cases the affected limb must be amputated. Five tumours were well differentiated, three were moderately differentiated. CONCLUSIONS: The patients consulted too late and their large lesions needed aggressive treatment. Appropriate information and regular examination of patients with RDEB helps early diagnosis of tumours and may avoid disabling operations.

Adolescent↗