Juvenile dermatomyositis. Clinical and epidemiologic experience

27 cases with a diagnosis of Juvenile Dermatomyositis were studied from a total of 1307 patients who suffered from connective tissue diseases; 19 of which met the diagnosis criteria of Bohan and Peters. Females were the most frequently affected. 52.63% of the cases presented the first symptoms between 5 and 9 years of age. Skin lesions, debility in inferior extremities and fever were the most frequent motives of consultation. The Aldolase and LDH were the muscular enzymes whose values increased in the majority of the cases. The electromyography was more sensible than the muscular biopsy in the diagnosis of the disease. We suggest that the epidemiologic and clinical characteristics found in our study should be taken into account for further accurate diagnosis of Juvenile Dermatomyositis in Venezuela.     

Intracardiac inflammatory myofibroblastic tumor: a unique presentation.

Cardiac tumors are detected rarely in childhood. These are most frequently found in infancy, and most are rhabdomyomas. We describe a unique occurrence of a rarely described intracardiac tumor in an asymptomatic 7-year-old child.     

Comparative multicentre trial of three IUDs inserted immediately following delivery of the placenta

A multinational comparative trial of three IUDs (Copper 7, Lippes loop size D and the Postpartum T) randomly inserted immediately following delivery of the placenta was conducted in six centres. A total of 841 women entered the study. As the predetermined termination indices for expulsions were exceeded at six months the trial was prematurely closed. An excess of expulsions during the first 48 hours following insertion was observed for the Lippes loop compared to the other devices. At six months the expulsion rate for the Lippes loop was significantly higher than that for the Copper 7. In addition, the discontinuation rate for the Lippes loop at 12 months was significantly higher than that for the Copper 7. There were no significant differences in either the expulsion rates or the discontinuation rate at six or twelve months between the postpartum T and the other devices. At 12 months the pregnancy rates with all three devices was high; there were no ectopic pregnancies. Considerable between-centre differences were observed, particularly for expulsion rates. Possible reasons for this are discussed and future research lines are suggested.     

Carbon tetrachloride poisoning in Reykjavík and vicinity

Summary A report is given of all known cases of carbon tetrachloride poisoning treated at hospitals and/or coming to autopsy at the Department of Pathology, University of Iceland, Reykjavík, during the 20-years period 1945–1964.The main features of each case are summarized in the table. On the basis of previous report (Bjarnason et al.), it was concluded that carbon tetrachloride must be a considerably more toxic substance than trichloroethylene.Department of Pathology, University of Iceland, Reykjavík; Head: ProfessorNiels Dungal (deceased Oct. 29th, 1965).Department of Pharmacology, University of Iceland, Reykjavík; Head: ProfessorKristinn Stefánsson.The Sct. Joseph's Hospital, Reykjavík; Head:Bjarni Jónsson, M.D.     

GTX 2/3 epimers permeate the intestine through a paracellular pathway

The aim of this work was to typify the mechanisms involved in gonyautoxins intestinal permeability. For this purpose, permeability of gonyautoxins through intestinal epithelium, and their effect on transepithelial resistance was investigated in excised human jejunal segments. The isolated mucosa segments were mounted in a Ussing chamber and experiments performed under voltage-controlled conditions. Organic gonyautoxin cations were applied in the apical side and samples collected in the basolateral side. Results show that gonyautoxin 2/3 epimers (GTX 2/3) permeate the intestine through a paracellular pathway and, to reach the resolution of the technique we used, no evidence was found of any other transport mechanism involved in the process. A model was developed, according to which tight junctions undergo a toxin concentration and time-dependent change, while transepithelial resistance shows a modest decrease.     

Mapping of a novel locus for an autosomal recessive form of palmoplantar keratoderma on chromosome 3q27.2‐q29

Many exogenous factors including excessive alcohol consumption have been associated with psoriasis, but the underlying mechanisms still remain elusive. Drinking worsens therapeutic compliance, and decreases the efficacy and increases the toxicity of systemic antipsoriatic treatments. Excess alcohol intake results in compromised immunity and increased risk of infections, but alcohol can induce proinflammatory cytokine production in various cell types and can increase mitogen‐derived lymphocyte proliferation and lymphocyte activation. As we have previously reported, alcohol and one of its metabolites, acetone, induce keratinocyte proliferation and increase the mRNA levels of genes characteristic for proliferating keratinocytes, such as α5 integrin, cyclin D1 and keratinocyte growth factor receptor. Recently the correlation between blood and skin ethanol levels in humans was determined by a transdermal alcohol monitoring device, against the ‘gold standard’ breath alcohol readings. Based on transdermal alcohol measurements it can be concluded that cutaneous alcohol concentrations can reach levels that induce proinflammatory cytokine production and lymphocyte and keratinocyte proliferation in vitro. It is expected that the development of methodologies measuring transdermal ethanol will provide additional tools to evaluate how alcohol influences skin physiology and different dermatological conditions including psoriasis. Our review focuses on the possible link between alcohol misuse and psoriasis, particularly on the possible role of cutaneous ethanol in precipitating the disease.     

Cost analysis of skeletal-related events in Spanish patients with bone metastases from solid tumours

Purpose

To estimate the cost per skeletal-related event (SRE) in patients with bone metastases secondary to solid tumours in the Spanish healthcare setting.

Methods

Patients diagnosed with bone metastases secondary to breast, prostate or lung cancer were included in this multicentre, observational study. SREs are defined as pathologic fracture (vertebral and non-vertebral fracture), radiation to bone, spinal cord compression or surgery to bone. Health resource utilisation associated with these events (inpatient stays, outpatient, emergency room and home health visits, nursing home stays and procedures) were collected retrospectively for all SREs that occurred in the 97 days prior to enrolment and prospectively during follow-up. Unit costs were obtained from the 2010 eSalud healthcare costs database.

Results

A total of 93 Spanish patients with solid tumours were included (31 had breast cancer, 21 prostate cancer and 41 lung cancer), contributing a total of 143 SREs to this cost analysis. Inpatient stays (between 9.0 and 29.9 days of mean length of stay per inpatient stay by SRE type) and outpatient visits (between 1.7 and 6.4 mean visits per SRE type) were the most frequently reported types of health resources utilised. The mean cost per SRE was between €2,377.79 (radiation to bone) and €7,902.62 (spinal cord compression).

Conclusion

SREs are associated with a significant consumption of healthcare resources that generate a substantial economic burden for the Spanish healthcare system.