Cardiovascular abnormalities in end stage renal failure: the effect of anaemia or uraemia?

Plasma interleukin-8 (IL-8) concentrations were measured in patients with atopic dermatitis. Plasma IL-8 was not detected in 25 controls (0/25), in allergic rhinitis (0/20), or in bronchial asthma during remission (0/13), while low concentrations of IL-8 were detectable in a few patients with urticaria (1/19), contact dermatitis (4/17), and bronchial asthma at the time of attack (6/16). In contrast, IL-8 was detectable in most cases of atopic dermatitis (41/52). Moreover, IL-8 concentrations were significantly higher in severe than in mild or moderate atopic dermatitis. IL-8 concentrations decreased as atopic dermatitis was improved by treatment, and IgE production in vitro was also decreased while serum IgE concentrations remained unchanged. IL-8 measurement may be a useful tool for the study of the pathogenesis and clinical course of atopic dermatitis.     

Travel and changes in routine do not increase the risk of sudden infant death syndrome

We investigated the relationship between travel and changes in routine and the sudden infant death syndrome (SIDS) among 485 SIDS cases compared with 1800 randomly selected control infants. There was no increased risk of SIDS with travel. Special events, such as christenings, were not associated with an increased risk of SIDS. However, visits to and by friends or relatives were associated with a significantly reduced risk of SIDS after controlling for potential confounders (odds ratios = 0.70; 95% confidence interval = 0.52, 0.96). These findings may indicate less social support in SIDS cases.     

von Willebrand Disease : A Clinico-haematological Spectrum

Background

Bleeding disorders are commonly seen in clinical practice. von Willebrand Disease (vWD), is the commonest and yet a profoundly under diagnosed cause, having a wide spectrum of clinical presentation. Of its three types, type 1 vWD (70% of the total vWD cases) has the mildest and a highly variable clinical and laboratory presentation.

Methods

A series of ten cases of vWD were comprehensively evaluated using recommended diagnostic parameters and therapeutic interventions.

Results

All major types of vWD were represented. A female preponderance, with primary presentation in the form of muco-cutaneous bleeds was observed. A positive history of consanguineous parental marriage and family history of bleeding disorder was elicited in two and three patients respectively. Nine patients were found to be anemic and thrombocytopenia was present in only one. Bleeding time by modified template (SIMPLATE) method, along with activated partial thromboplastin time (APTT) was increased in all ten cases and of these, nine had low factor VIII: C levels. Ristocetin induced platelet aggregation studies were abnormal in all the five cases it was performed. vWF:RCo activity determined in one individual was shown to be low. vWF:Ag assay was done in four cases revealing a near complete absence of von Willebrand factor antigen in one and mildly decreased levels in the other three. vWF multimer assay was advised in three cases. DDAVP, plasma derived vWF, blood products and local antifibrinolytics were used as primary modalities of treatment

Conclusion

Thus, strong clinical suspicion, thorough clinical evaluation and judicious use of investigations including repeated investigations at different times are needed for making a diagnosis of vWD.Key Words: Bleeding disorders, von Willebrand Disease     

Esophageal resection for carcinoma in patients older than 70 years

Background A larger number of older patients are presenting as candidates for esophageal resection. An aggressive surgical approach in this population is controversial. Methods Four hundred thirteen patients with esophageal cancer who presented to Roswell Park Cancer Institute from 1991 to 1998 were retrospectively reviewed. Clinical data, perioperative details, and postoperative courses were compared for patients older and younger than 70 years. Results One hundred forty-seven patients (36%) were older than 70 years. Risk factors, clinical symptoms, histology, and stage at presentation were equivalent for both age groups. A higher percentage of patients <70 years were candidates for curative resection. There were no significant differences between groups for estimated blood loss, intraoperative transfusions, length of stay, overall morbidity, or mortality. Only postoperative myocardial infarction and atrial fibrillation were increased in the older group. Excluding stage IV disease, there was a significant and similar improvement in median survival after resection for patients both <70 years and >70 years. Conclusions In conclusion, esophageal cancer in older patients warrants surgical resection because the benefit to the patient is the same as it is for younger patients, without a significant increase in operative morbidity or mortality. Presented at the 54th Annual Meeting of the Society of Surgical Oncology, Washington, DC, March 15–18, 2001.     

Definition of IDDM-associated HLA DQ and DX RFLPs by segregation analysis of multiplex sibships

Genetic variation of the DQ alpha and beta and of the DX alpha genes, detectable as RFLP in genomic DNA digests, has been suggested to improve the identification of individuals at high risk for insulin-dependent diabetes mellitus (IDDM). DNA from all members of 32 IDDM multiplex families was digested with six restriction endonucleases and the resulting fragments analyzed in Southern blots for hybridization with labeled cDNA probes for those genes. A computerized segregation analysis procedure was then used to assign fragments to haplotypes. Associations among fragments and between fragments and haplotypes characterized serologically and biochemically for their class II genes and IDDM-carrier status were calculated. The results indicate that the alleles of the DX alpha polymorphism maintain linkage disequilibrium with those of the DQ beta genes responsible for the well-known DQ beta 3.2-IDDM association, so that IDDM-carrier haplotypes carry disproportionally often both DQ beta 3.2 and DX alpha-TaqI-2.2kb. Thus, these RFLPs identify a DR-DQ-DX haplotype in high linkage disequilibrium, rather than the locus or loci that account for their high relative risk. However, four DR4-DQ beta 3.2 haplotypes that lack DX alpha-TaqI-2.2kb were encountered, two of which are "affected." These haplotypes suggest that the identification of the "disease locus" can be facilitated by the study of unusual haplotypes in which distinct IDDM-associated alleles occur separated from their neighbors of the standard genetic configurations.     

Fehlstellungen der Hüfte

Monatsschrift Kinderheilkunde - Fehlstellungen der Hüfte sind bei Kindern und Jugendlichen gut an einem hinkenden Gang, an der Rumpfasymmetrie im Stand sowie an einem seitendifferenten aktiven...     

Digital subtraction angiography system evaluation with phantoms

Advances in digital subtraction angiography imaging demonstrate the need for critical evaluation of the performance of digital subtraction equipment. The design of a phantom set for noninvasive assessment of the imaging quality of digital subtraction equipment is described; components include a remotely controlled transport system and individual patterns to evaluate the contrast and detail properties of the image intensifier, low-contrast sensitivity and resolution of the system, geometric distortion of image, linearity, mechanical and electronic stability of equipment, and effects of bone and bowel gas on iodine perception. The performance of an add-on digital radiographic system is presented, along with radiation exposure levels at the image intensifier for a range of radiographic techniques.     

A founder mutation in the gamma-sarcoglycan gene of gypsies possibly predating their migration out of India

We investigated the molecular basis of a severe form of early onset autosomal recessive muscular dystrophy with sarcoglycan (SG) deficiency in seven large Gypsy families living in different parts of Western Europe and apparently not closely related. They were linked to the LGMD2C locus (13q12) suggesting a primary defect in the gamma-SG gene coding for the 35 kDa dystrophin-associated glycoprotein. All of the 18 investigated patients were homozygous for the same G-->A transition in codon 283 producing the replacement of a conserved cysteine of the extra-cellular domain of the protein by a tyrosine. All affected chromosomes in homozygous and heterozygous relatives carried the same allele 5 of the intragenic marker D13S232. Flanking markers were studied to delineate a common ancestral haplotype, the size of which was used to compute the date of the founding mutation. We found evidence that the mutation occurred between 60 and 200 generations ago, therefore possibly predating the commonly accepted date of migration of the Gypsy ancestors out of India.      

HPLC测定睾酮乳膏中睾酮的含量

采用ＨＰＬＣ测定睾酮乳膏中睾酮的含量，以甲醇－水（７０：３０）为流动相，波长２４０ｎｍ，在４０－３６０μｇ／ｍｌ范围内线性关系良好ｒ＝０．９９９９，本法操作简便，回收率高。