Influence of HLA-DQ2.5 Dose on Clinical Picture of Unrelated Celiac Disease Patients

The clinical phenotype of celiac disease varies considerably among patients and the dosage of HLA-DQ2.5 alleles has been suggested to be a contributing factor. We investigated whether HLA-DQ2.5 allele dosage is associated with distinct clinical parameters at the time of diagnosis and with patients’ response to a gluten-free diet. The final cohort included 605 carefully phenotyped non-related Finnish celiac disease patients grouped as having 0, 1 or 2 copies of HLA-DQ2.5. Clinical data at the time of diagnosis and during gluten-free diet were collected systematically from medical records and supplementary interviews. An increasing HLA-DQ2.5 dose effect was detected for celiac disease antibody positivity at diagnosis (p = 0.021) and for the presence of any first-degree relatives with celiac disease (p = 0.011 and p = 0.031, respectively). Instead, DQ2.5-negative patients were suffering most often from classical symptoms at diagnosis (p = 0.007 between HLA groups). In addition, during follow-up they were most often symptomatic despite a gluten-free diet (p = 0.002 between groups). Our results thus suggest that increasing HLA-DQ2.5 dose only has a minor effect on the clinical picture of celiac disease. However, HLA-DQ2.5-negative patients should not be overlooked in clinical practice and particular attention should be paid to this patient group during gluten-free diet.     

Follistatin and activin A in extra-embryonic coelomic and amniotic fluids and maternal serum in early pregnancy

Follistatin is a specific binding protein which controls bioavailability of activins and inhibins which have an important role in fetal development. In the first trimester of pregnancy bioactive dimeric inhibins are found at high concentrations in the extra- embryonic coelomic fluid, but the distribution of follistatin and activins is not known. We have used recently developed immunoassays for follistatin, activin A and activin AB to determine their presence in the intrauterine compartments during early pregnancy. Follistatin was present in highest concentrations in the extra-embryonic coelomic fluid (11.72 +/- 1.70 ng/ml; median +/- SEM), with less in maternal serum (6.35 +/- 4.58) and lowest amounts in amniotic fluid (0.97 +/- 0.52). Follistatin concentrations in extra-embryonic coelomic fluid were highly correlated with both dimeric inhibin isoforms. Activin A was present in only barely detectable amounts in some samples of extra- embryonic coelomic fluid (41% of samples) and maternal serum (26%) and was undetectable in all amniotic fluid samples. Activin AB was undetectable in all compartments. The presence of follistatin in the amniotic and extra-embryonic coelomic fluids may regulate the availability of bioactive activins and inhibins which are released into the intrauterine compartments during the development of the fetus and placenta in early pregnancy.      

CT demonstration of cardiophrenic angle lymphadenopathy in Hodgkin disease

Eleven patients with Hodgkin disease involving the cardiophrenic angle lymph nodes are described. Four were discovered at the time of initial staging, and the remainder represented a major site of relapse after therapy. Regression of adenopathy after chemotherapy was documented in six patients; surgical excision (three), biopsy (one) and postmortem examination confirmed the presence of recurrent disease in the remaining cases. Enlargement of cardiophrenic angle nodes was frequently overlooked on conventional chest radiography, but a rather typical appearance is readily identifiable on CT.     

Long term outcome after resection of the first rib for thoracic outlet syndrome

A total of 112 first ribs in 103 patients were resected over 11 years for thoracic outlet syndrome. Seventy-seven patients (84 operations) were followed up for 2.5 years or more to assess the long term results of this procedure and the factors affecting them. One month after surgery 52 per cent of limbs were asymptomatic and 77 per cent were at least improved. A follow-up examination was performed, on average 6.1 years after the operation, by two independent examiners. This evaluation showed a permanent success rate of 37 per cent among 84 limbs examined. These long term results compare unfavourably with previously published data. The reason for the poor final outcome seemed to be difficulty in selecting patients for the operation. This was not aided by any of the preoperative tests. Patients in this study were evaluated by independent examiners, and only a total absence of preceding symptoms was accepted as the criterion for success. We emphasize the importance of unbiased evaluation and long term follow-up.     

Stab wounds to the extremities: indications for angiography

To ascertain the sensitivity of physical examination with respect to significant arterial injury, the angiographic findings of 61 stab wounds to the extremities were correlated with specific physical findings. In 25 injuries there were physical findings of major injury, including one or more of the following: pulse deficit, active bleeding or expanding hematoma, bruit or murmur, neurologic deficit, or hypotension. Of these 25 injuries, angiographic examination revealed significant vascular damage in 11. In 36 injuries physical findings indicated minor injury, with proximity to major vessels or nonexpanding hematoma being the only indication for arteriography. None of these patients had significant vascular injuries. Physical examination was found to be 100% sensitive in screening for significant vascular injuries. These results indicate that arteriography is unwarranted in stab wounds to the extremities if the only indications are proximity to major vessels or nonexpanding hematoma.