Total pancreatectomy with islet autotransplantation: an overview

Pain control is one of the most challenging aspects in the management of chronic pancreatitis. Total pancreatectomy can successfully relieve the intractable abdominal pain in these patients but will inevitably result in insulin-dependent diabetes. Islet autotransplantation aims to preserve, as far as possible, the insulin secretory function of the islet cell mass thereby reducing (or even removing) the requirement for exogenous insulin administration after a total pancreactomy. Despite the relatively small number of centres able to perform these procedures, there are important technical variations in the details of their approaches. The aim of this review is to provide details of the current surgical practice for total pancreatectomy combined with islet autotransplantation, and outline the potential advantages and disadvantages of the variations adopted in each centre.     

Prospective epidemiological study of invasiveHaemophilus influenzae disease in adults

The incidence and characteristics of invasiveHaemophilus influenzae disease were studied in 43 adult patients admitted to the acute care hospitals in El Vallés County (Barcelona, Spain) between January 1987 and June 1992. The annual incidence ofHaemophilus influenzae disease was 1.2 per 100,000 inhabitants. Pneumonia occurred in 24 patients, meningitis in five, intraabdominal infections in three, obstetric infections in two, epiglottitis in two and cellulitis in one. In six patients the source of infection was unknown. Ten (23 %) of the infections were hospital acquired. Underlying conditions were diagnosed in 30 (70 %) patients. NontypeableHaemophilus influenzae strains predominated in all adult age groups. Sixty-one percent of type b and 34 % of nontypeable strains were ampicillin resistant (p=0.08). Multiple antibiotic resistance was also high among type b (53 %) and nontypeable (18 %) strains. The mortality rate was significantly higher in patients with pneumonia, bactaeremia from an unidentified focus or shock at presentation.     

Identifying candidate causal variants responsible for altered activity of the ABCB1 multidrug resistance gene

The difficulty of fine localizing the polymorphisms responsible for genotype-phenotype correlations is emerging as an important constraint in the implementation and interpretation of genetic association studies, and calls for the definition of protocols for the follow-up of associated variants. One recent example is the 3435C>T polymorphism in the multidrug transporter gene ABCB1, associated with protein expression and activity, and with several clinical conditions. Available data suggest that 3435C>T may not directly cause altered transport activity, but may be associated with one or more causal variants in the poorly characterized stretch of linkage disequilibrium (LD) surrounding it. Here we describe a strategy for the follow-up of reported associations, including a Bayesian formalization of the associated interval concept previously described by Goldstein. We focus on the region of high LD around 3435C>T to compile an exhaustive list of variants by (1) using a relatively coarse set of marker typings to assess the pattern of LD, and (2) resequencing derived and ancestral chromosomes at 3435C>T through the associated interval. We identified three intronic sites that are strongly associated with the 3435C>T polymorphism. One of them is associated with multidrug resistance in patients with epilepsy (chi2 = 3.78, P = 0.052), and sits within a stretch of significant evolutionary conservation. We argue that these variants represent additional candidates for influencing multidrug resistance due to P-glycoprotein activity, with the IVS 26+80 T>C being the best candidate among the three intronic sites. Finally, we describe a set of six haplotype tagging single-nucleotide polymorphisms that represent common ABCB1 variation surrounding 3435C>T in Europeans.     

Prevalence and risk factors of tinea unguium and tinea pedis in the general population in Spain

This study prospectively evaluated the prevalence and risk factors of tinea unguium and tinea pedis in the general adult population in Madrid, Spain. One thousand subjects were clinically examined, and samples of nails and scales from the interdigital spaces of the feet were taken from those patients presenting with signs or symptoms of onychomycosis and/or tinea pedis, respectively. In addition, a sample from the fourth interdigital space of both feet was collected from all individuals with a piece of sterilized wool carpet. Tinea unguium was defined as a positive direct examination with potassium hydroxide and culture of the etiological agent from subjects with clinically abnormal nails. Patients with positive dermatophyte cultures of foot specimens were considered to have tinea pedis. The prevalence of tinea unguium was 2.8% (4.0% for men and 1.7% for women), and the prevalence of tinea pedis was 2.9% (4.2% for men and 1.7% for women). The etiological agents of tinea unguium were identified as Trichopyton rubrum (82.1%), followed by Trichopyton mentagrophytes var. interdigitale (14.3%) and Trichopyton tonsurans (3.5%). Trichophyton rubrum (44.8%) and Trichophyton mentagrophytes (44.8%), followed by Epidermophyton floccosum (7%) and T. tonsurans (3.4%), were the organisms isolated from patients with tinea pedis. The percentage of subjects who suffered simultaneously from both diseases was 1.1% (1.7% for men and 0.6% for women). In a multivariate logistic regression analysis, age (relative risk [RR], 1.03) and gender (RR, 2.50) were independent risk factors for tinea unguium, while only gender (RR, 2.65) was predictive for the occurrence of tinea pedis. In both analyses, the presence of one of the two conditions was associated with a higher risk for the appearance of the other disease (RR, >25).     

Inflammatory myofibroblastic tumour (inflammatory pseudotumour) of the breast. Clinicopathological and genetic analysis of a case with evidence for clonality.

Inflammatory myofibroblastic tumours (IMTs) were initially considered to be benign reactive processes, but cases with an unfavourable outcome have been reported. Moreover, clonal genetic alterations have recently been published in some cases, suggesting that IMT may represent a malignant neoplastic entity. This paper reports a case of IMT that developed in the mammary gland, an unusual site. The histological picture was characterized by a proliferation of spindle cells with little cellular atypia and rare mitoses, associated with a polymorphous inflammatory infiltrate. Their immunophenotype, characterized by the expression of vimentin, smooth muscle actin, and cytokeratins, corresponded to that of myofibroblasts. Cytogenetic analysis revealed the clonal nature of the lesion. The modal karyotype was 48, X, ins(2;X)(q34;p21.2p22.2), +7, del(9)(p23), +19. Including the present observation, a 9p deletion has now been found in three cases of IMT. These observations show that IMT may be a clonal neoplasm, even in sites different from deep soft tissues.     

Variations of somatotype in elderly Sardinians.

Somatotyping is an effective technique for the study of anthropometric variations and body composition in elderly subjects, even though it has not often been used in this field. The present study was conducted on a sample of 280 healthy Sardinians (134 men and 146 women) of age 60-89 years, subdivided into three age classes (60-69 years; 70-79 years; and 80-89 years). Somatotypes were computed according to Carter and Heath (Somatotyping-Development and Applications. Cambridge, England: Cambridge University Press; 1990). The results show a dominance of the endo- and mesomorphic components in the elderly subjects, with less development of ectomorphy than in younger individuals. In a comparison with other populations, our sample shows strong development of endomorphy and especially of mesomorphy, while ectomorphy values are generally low. Age-related variations are significant in both sexes and consist in a progressive reduction of the endomorphic component, particularly in the 80-89-year class (endomorphy in the three age classes: 6.4, 6.1, and 5.3 in men; 8.1, 7.8, and 6.8 in women). The mesomorphic component is characterized by stability (age variations: 6.4, 6.4, and 5.9 in men; 6.3, 6.4, and 6.3 in women) and the ectomorphic component by a slight increase (age variations: 0.5, 0.6, and 0.8 in men; 0.4, 0.3, and 0.5 in women). Sex differences are significant and especially large for the endomorphic component, with generally higher values in women. The sexual dimorphism tends to decrease with age. The results are discussed with regard to the biology of aging, with emphasis on the potential application of somatotype to studies of the elderly population.     

The shell technique: bilateral fronto-orbital reshaping in trigonocephaly

The shell technique, used in the Pediatric Neurosurgical Department at the Catholic University, Rome, since the 1990s for the correction of trigonocephaly, is associated to a significant reduction in surgical time and intraoperative blood loss as compared to other procedures, while allowing an adequate remodelling of the bifrontal bone by means of multiple radial osteotomies. The technique does not necessitate the creation of a supraorbital bar, as the supraorbital ridges are modified in situ, further reducing the operative blood loss. In spite of reduced surgical time and manipulation, this procedure ensures aesthetic and functional results comparable to more extensive and complex cranial vault reshaping procedures. The main limitation of this technique is related to the surgical timing, as better results are obtained between 3 and 9 months of age, when the skull bone is still ductile to work with, thus allowing it to be remodelled by greenstick fractures. Moreover, in this age group, the cranial defects that result from the enlargement of the frontal bone flap by means of radial cuts and from the anterior displacement of its lateral portions may benefit from the more effective bone regeneration which characterizes younger children as compared to their older counterparts. A small number of cases showing either persistent hypotelorism or temporal depression have been observed in the post-operative period, although these residual deformities probably depend on a more extensive involvement of the cranial base in the synostotic process in these patients than on the procedure itself.     

Meningococcal meningitis with ‘normal’ cerebrospinal fluid

A prospective study was made of all patients with normal CSF counts and positive cultures for Neisseria meningitidis diagnosed in “El Vallés” County, Barcelona between January 1987 and December 1990. Meningococcal meningitis was documented in 82 patients, eight of whom (seven children, five boys and two girls with a mean age of 5·6 ± 3·3 years, and a 69-year-old male patient) had no apparent CSF abnormalities in the initial lumbar puncture. At the time of admission all patients had fever (mean 39·1 °C) of 10·8 ± 5·6 hour duration and petechial rash which had been present for a mean of 3·6 ± 3·3 hours. Signs of meningeal irritation were not found. A 4-month-old infant with symptoms of circulatory collapse, intracranial hypertension and impairment of consciousness subsequently died of septicemia in 48 hours. Group B N. meningitidis was isolated in six cases (reduced penicillin-susceptibility in two cases) and group C N. meningitidis in the remaining two (reduced penicillin-susceptibility in one case). Patients without pleocytosis did not differ in a statistically significant fashion from the patients with high pleocytosis in the duration of temperature, and petechial rash, leukopenia, positive blood culture and fatal outcome.