X-linked ichthyosis along with recessive dystrophic epidermolysis bullosa in the same patient.

X-linked ichthyosis (XLI) is a relatively common keratinization disorder which is caused, in the vast majority of cases, by a total deletion of the sulfatase steroid (STS) gene. Dystrophic epidermolysis bullosa (DEB) is a scarring form of epidermolysis bullosa of either autosomal recessive or dominant inheritance secondary to collagen VII gene mutations. We report the first case of a patient with both XLI and DEB in whom a partial deletion of the STS gene and a recessive point mutation in COL7A1 were demonstrated.

Influence of degree of hepatic steatosis on graft function and postoperative complications of liver transplantation.

The aim of this study was to evaluate the impact on initial graft function of the degree of steatosis detected in the back-table biopsy, and its repercussion on the clinical results of the transplant (early posttransplant mortality and morbidity). We undertook a retrospective analysis of 300 liver transplants performed at our center from 1997 to 2004. A wedge liver biopsy was done routinely during back-table surgery (available in 294 transplants). The degree of steatosis was classified as: S0-no steatosis, 201 transplants; S1-mild steatosis (<30%), 58 transplants; S2-moderate steatosis (30% to 60%), 18 transplants; and S3-severe steatosis (>60%), 17 transplants. The ischemia-reperfusion (I/R) injury, based on the maximum mean peak aspartate transferase in the first 72 posttransplant hours, tended to be greater as the degree of graft steatosis increased: S0, 1316; S1, 1985; S2, 2446; and S3, 2955 (P < .005 between S0 and S3). This greater initial hepatic dysfunction was correlated in the group with severe steatosis with a higher rate of severe renal failure requiring hemofiltration/hemodialysis: S0, 9%; S1, 15%; S2, 11%; and S3, 41% (P < .001); as well as with a higher early mortality (90 days): S0, 10%; S1, 21%; S2, 11%; and S3, 41% (P < .001). The Kaplan-Meier survival curve showed a significant difference (log-rank and Breslow) between the group with severe steatosis and the group with no steatosis (P = .002). We conclude that the degree of liver graft steatosis is an important determinant of I/R injury, although this progressive increase in the I/R injury with the degree of steatosis only had clinical repercussions in the case of severe steatosis.

Talc granulomatosis. A differential diagnosis of interstitial lung disease in HIV patients.

Consumers of adulterated drugs may present with talc-induced interstitial lung disease. A transbronchial biopsy specimen showing granulomas with intracellular talc crystals is necessary to confirm this diagnosis. In patients infected with HIV, such a condition can be indistinguishable at first glance from opportunistic infections or other pulmonary diseases. A case is presented of a seropositive patient whose chest radiographs showed a diffuse interstitial pattern.

Endocarditis due to Acinetobacter lwoffi on native mitral valve.

Endocarditis due to Acinetobacter is a rare pathology with high mortality, reported mainly in hospitalized patients with predisposing risk factors. This is the second case of endocarditis due to Acinetobacter reported in our country in the last 10 years.

[Asymptomatic carriers of HBsAg: is a follow-up necessary?]. / Portadores asintomáticos del HBsAg: Es necesario su seguimiento?

We evaluated the clinical and epidemiological data of 142 HBsAg carriers. This prospective trial is part of a program of study and follow-up in HVB patients. The median age was 34.58 years old, males 56.3%. The average follow-up was 32.4 months. Complete clinical history, routine analysis, liver function tests, alfa-fetoprotein, serology for HVB, HCV and HDV and abdominal ecography were done in all patients. DNA-HVB was done only in special cases. Patients with less than 6 months of follow-up were excluded. The 118 remaining carriers were classified into two groups, depending on ALT values. Group 1 (normal ALT): included 98 carriers, 3 of them developed an active chronic hepatitis that was treated with interferon. A small CHC was diagnosed in another patient and it was resected. Group 2 (elevated ALT): included 20 carriers, only 9 of them agreed to biopsy and we found severe hepatic lesions in 4 of them. No group presented coinfection with HCV or HDV. No patient died. We conclude that the study and follow-up of asymptomatic HBsAg carriers permits an early diagnosis and treatment of the complications of this pathology (chronic hepatitis, CHC, etc); in our study, three patients developed chronic hepatitis, successfully treated with interferon, and one small size CHC was diagnosed in another patient. The study of relatives permits also detect subclinic liver disease and facilitates vaccination to prevention transmission of this infection.

[Facial granuloma caused by cactus bristles]. / Granulomas faciales por espinas de cactus.

We report a 39 year old female, who 6 years later of accidental injury with cactus bristles, developed granulomatous lesions in her face with an ulcerative tendency. Histopathologic picture showed granulomatous infiltrates filling the full-thickness of the dermis, with abundant number of multinuclead giant cells foreign-body-type and Langhans-type and mixed inflamatory infiltrate. In the dermo-hipodermal limit, several foreing bodies were found with a thorny or barb shape, some of them into the giant cells, with were PAS positive and showed a brilliant refractile aspect under polaroscopic examination.

[Analysis of surgical treatment for nonmelanoma skin cancer performed by dermatologists in a public hospital: clinical-pathological correlation, use of hospital resources, and waiting list time from diagnosis]. / Análisis del tratamiento quirúrgico del cáncer cutáneo no melanoma cuando es realizado por dermatólogos en un hospital público: correlación anatomoclínica, empleo de recursos hospitalarios y tiempo de espera desde el diagnóstico.

BACKGROUND: Nonmelanoma skin cancer is the most common form of cancer in humans. It can be treated by a variety of specialists and using different techniques, surgical excision being the procedure associated with the lowest rates of recurrence. No studies have been published addressing differences in the management of surgical treatment for nonmelanoma skin cancer according to the specialties involved. OBJECTIVES: To assess the preoperative diagnostic accuracy and the use of health care resources when surgical treatment of nonmelanoma skin cancer is done by dermatologists belonging to the Spanish national health service. METHODS: A prospective observational study was carried out over a period of 36 months using data corresponding to all patients diagnosed with nonmelanoma skin cancer and treated surgically in the Dermatology Department of Complejo Hospitalario de Burgos, Spain. Data were analyzed for clinical-pathological correlation, complexity of the intervention, use of health care resources, and time elapsed between clinical diagnosis and surgery. RESULTS: The study included 448 patients and 521 skin lesions suspected to be nonmelanoma skin cancer (basal cell carcinoma or squamous cell carcinoma). Diagnosis was exclusively clinical in 487 tumors and a clinical-pathological correlation of 84.39% was observed. Surgery was performed with local anesthesia in 96.42% of patients, although 111 (21.29%) required complex surgical repair. In 349 patients (77.90%) the procedure was performed on an outpatient basis, 73 (16.29%) required a short stay in the surgical day care unit, and 26 (5.80%) required hospital admission. The mean (SD) delay from clinical diagnosis to surgery was 68.44 (42.22) days, with a median delay of 60 days. CONCLUSIONS: Dermatology specialists are highly qualified to diagnose malignant skin tumors and accurately identify those patients requiring surgery. Dermatological surgeons use minimal health care resources, shorten the overall length of the process, and help to control overall health care costs for cancer.

Task-dependent influences of attention on the activation of human primary visual cortex.

There has been a good deal of controversy over whether attention influences area V1-the first cortical area onto which information from the retina is projected. Attention to motion has been found to modulate monkey area MT and the human homolog of MT/MST. Here we show that activation of V1 by attention to motion is task dependent. Our stimulus consisted of a group of translating random dots superimposed over another group of random dots executing expansion motion. Subjects were instructed to pay attention selectively to the translation, expansion, or neither in particular (passive condition). The activity in the human MT/MST homolog measured by functional magnetic resonance imaging (fMRI) was significantly higher in both the translation and the expansion conditions than in the passive condition, while the activity in area V1 was significantly higher only in the translation condition. These results show that attention to motion modulates area V1, and more interestingly that high-level cognitive processing such as attention may directly or indirectly determine the retroactive extent of feedback within the motion pathway in a manner dependent on the type of motion attended.

Factors associated with neuropsychological performance in HIV-seropositive subjects without AIDS.

BACKGROUND: Previous research has suggested that several factors may influence the presence of cognitive impairment in human immunodeficiency virus (HIV) infection. The objective of this study was to assess the impact of cognitive reserve capacity and other variables on neuropsychological performance in early HIV infection. METHODS: The neuropsychological performance of 100 HIV-seropositive subjects without AIDS (71 men and 29 women) was compared with that of 63 seronegative controls (51 men and 12 women). Measures included a neuropsychological battery, a medical examination and a psychiatric assessment. Cognitive reserve scores were based on a combination of years in school, a measure of educational achievement, and an estimate of pre-morbid intelligence. RESULTS: HIV-positive subjects had longer reaction time latencies than HIV-negative subjects. Those in the HIV-positive group with low cerebral reserve scores showed the poorest performance on the neuropsychological tests. The prevalence of cognitive impairment was significantly higher in the HIV-positive group (27%) than in the controls (32%). Multiple regression analysis and logistic regression analysis were used to identify factors associated with global neuropsychological performance and cognitive impairment. Older age, lower cerebral reserve scores and not being on zidovudine treatment were associated with lower global neuropsychological scores and with the presence of cognitive impairment. CONCLUSIONS: Our results suggest that although cognitive impairment is not characteristic of early HIV infection, there is a subgroup of subjects who perform more poorly than expected. A lower reserve capacity, older age and not being on zidovudine treatment are factors that lower the threshold for neuropsychological abnormalities in cases of early HIV infection.

[Normative data for the neuropsychological evaluation of intravenous drug users]. / Datos normativos para la exploración neuropsicológica de usuarios de drogas por vía parenteral.

BACKGROUND: There are many recent epidemiological studies that find that intravenous drug users' (IDU's) performance in several measures of neuropsychological function are not the same than that of the general population, whether they are seropositive to HIV-1 infection or not. Consequently, it has been stated the need to establish appropriate norms for this population. The purpose of this study is to provide normative data on a battery of neuropsychological tests from a IDU group. SUBJECTS AND METHODS: This report provide normative data from a group of 116 IDU, on a battery of neuropsychological tests stratified by age group (mean = 30.9; SD = 4.5), and educational level (mean = 8; SD = 2.4). Comparisons between the means of the different groups according to age and education, and also with respect to sex are made. The analysis includes estimation of partial correlations between neuropsychological test scores and age and education. RESULTS: The analysis demonstrates that education is an important determinant of performance for most of these tests, while there are no differences in performance between the two age-groups. With respect to sex, females out-performed men on a measure of motor speed and coordination. CONCLUSIONS: This report provides norms that may be of use as a reference for clinical evaluation and research in drug user populations. It also emphasizes the need to establish normative data controlling for variables like educational level, from a population with special characteristics such as IDU population.

Osteomyelitis caused by Staphylococcus schleiferi and evidence of misidentification of this Staphylococcus species by an automated bacterial identification system.

We report a case of sternal osteomyelitis due to Staphylococcus schleiferi in a patient who underwent thoracic surgery. This constitutes the first documented case of osteomyelitis caused by this Staphylococcus species. We also relate our experience in the utilization of commercially available MicroScan panels for the identification of this microorganism.

Slowed reaction time in HIV-1-seropositive intravenous drug users without AIDS.

One hundred and fourteen subjects with a history of intravenous drug abuse (65 subjects infected with the human immunodeficiency virus, HIV, and 49 seronegative controls) were evaluated with a reaction time (RT) test which included one measure of simple RT and three measures of complex RT. All seropositive patients were in HIV stages A or B. The multivariate analysis of covariance controlled for age, educational level and Montgomery-Asberg Depression Rating Scale score, showed differences between seropositive subjects and controls: the seropositive group scored slower than the control group on two RT tasks, simple RT and a more complex RT measure. Our results suggest that computerized RT measures may be more sensitive than conventional neuropsychological tests in detecting subtle cognitive and motor retardation in the early stages of HIV infection, thus extending the findings of other studies to the intravenous drug-user population.