Thyroid involvement by malignant histiocytosis of Langerhans' cell type

Involvement of the thyroid gland by Langerhans' cell histiocytosis is quite rare. We describe the case of a 58-year-old man referred for treatment of a progressively enlarging goitre. The trachea was severely stenotic and adjacent structures such as the left carotid vein and the thyroid cartilage were also involved. Central diabetes insipidus and severe combined immunodeficiency were associated. Although fine needle aspiration biopsy of the thyroid was initially interpreted as papillary carcinoma, anaplastic thyroid cancer was suspected. Treatment with prednisolone, doxorubicin and irradiation controlled the tracheal compression. A diagnosis of thyroid Langerhans' cell histiocytosis was finally made on the basis of the presence of Birbeck granules and CD1a and CD4 antigen in the thyroid tumour cells. Furthermore, positive staining for CD68 and lysozyme suggested that the tumour cells may have had the character of phagocytic cells in addition to their dendritic cell nature. This is the first case of thyroid involvement by malignant histicytosis of Langerhans' cell type with unusual phagocytic markers.     

Pleural involvement of dialysis-related amyloidosis

Two cases of pleural involvement of dialysis-related amyloidosis (DRA) with pleural effusion are presented. DRA is one of the most important complications in long-term dialysis patients and beta2-microglobulin is the principal protein component of DRA, but pleural deposition of beta2-microglobulin amyloid has not yet been reported. To the best of the authors' knowledge, this is the first case report of pleural involvement of DRA presenting with pleural effusion. This case study suggests that pleural involvement of DRA should be considered when exudative pleural effusion is observed in patients undergoing long-term dialysis.     

Repeated stress suppresses interferon-gamma production by murine intestinal intraepithelial lymphocytes

Intestinal intraepithelial lymphocytes (IEL), one of the major effector components in the mucosal immune system, are phenotypically and functionally distinct from thymic and peripheral T cells. To investigate the effect of repeated stress on the number and function of IEL, we exposed male C3H/HeN mice to mild electric foot shock for 30 min/day for 5 consecutive days. Immediately after the final foot shock stress, the blood, spleen, thymus and small intestine of each of the mice were obtained. As a functional measure, we evaluated interferon (IFN)- gamma production by IEL, since IFN-gamma is a key immunomodulating cytokine in mucosal immune responses. Serum corticosterone level was elevated immediately after foot shock stress. There were no significant changes in the number of whole IEL and CD3+ IEL subsets after the stress. In contrast, the stress led to a significant decrease in the total number of thymocytes, particularly the reduction in the number of CD4+CD8+ thymocytes. Thymocytes expressed the highest level of intracellular glucocorticoid receptor (GR), followed by splenocytes and IEL. The foot shock stress induced a marked suppression of IFN-gamma production by IEL, when stimulated with immobilized anti-CD3 monoclonal antibody. Furthermore, corticosterone suppressed the IFN-gamma production by cultured IEL, which was prevented by Mifepristone (RU486), a GR antagonist. In summary, repeated foot shock stress did not alter the numbers of IEL and CD3+ IEL subsets, but suppressed IFN-gamma production by IEL, which was probably mediated by the elevated corticosterone. We therefore propose that stress influences host defense by suppressing the production of IFN-gamma in IEL.     

Muscular amyloid angiopathy with amyloidgenic transthyretin Ser50Ile and Tyr114Cys

Among patients with familial amyloid polyneuropathy (FAP), those with transthyretin Val30Met mainly show distally predominant weakness and atrophy, whereas some FAP patients, including those with transthyretin Ser50Ile and Tyr114Cys, show muscle weakness and atrophy that is dominant proximally, simulating myopathy. To clarify the cause of proximally dominant muscular atrophy in patients with FAP transthyretin Ser50Ile and Tyr114Cys, we investigated the distinctive features of muscle specimens of patients with FAP, 3 of who had Val30Met, 2 Ser50Ile, and 2 Tyr114Cys transthyretin. All specimens showed transthyretin amyloid around blood vessels and perimysium, and neurogenic denervation patterns. The amount of amyloid around the vessels was much greater in patients with FAP Ser50Ile and Tyr114Cys than in Val30Met patients. Muscular amyloid angiopathy may contribute to motor nerve injury that, in turn, may lead to amyotropic changes in patients with FAP Ser50Ile and Tyr114Cys.     

Apico-pulmonary artery conduit repair of congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary outflow tract obstruction: a 10-year follow-up

BACKGROUND: In conventional repair of the congenitally corrected transpositions of the great arteries associated with ventricular septal defect and pulmonary outflow tract obstruction, the placement of the left ventricle-pulmonary artery conduit is at risk owing to probable compression by the sternum, heart block, or injury to the mitral anterior papillary muscle. Apical placement of the left ventriculotomy for the inflow conduit rather than in the midportion or base placement may avoid these complications, although this results in a long and winding extracardiac conduit that may be short-lived because of the proliferation of pseudointima. METHODS: Between 1985 and 1990, a nonvalved Dacron woven-fabric graft conduit was placed between the left ventricular apex and pulmonary artery in 5 patients (mean age, 6.2 +/- 1.7 years) who were then followed for at least 10 years. RESULTS: No iatrogenic heart blocks or mitral regurgitation developed. All patients were complaint-free during the follow-up period, although 1 patient who was clinically well died suddenly in the 10th follow-up year. Cardiac catheterization in the 10th follow-up year indicated a pressure gradient of 21 +/- 6 mm Hg across the conduit, and angiography revealed that the conduit diameter was 91% +/- 6% of the original conduit diameter. CONCLUSIONS: The reportedly poor early and late outcomes that occur after a conventional repair of congenitally corrected transpositions of the great arteries associated with ventricular septal defect and pulmonary outflow tract obstruction, which places an extracardiac conduit between the left ventricle and the pulmonary artery, may be partially neutralized by relocating the inflow position to the apex.     

Liberal use of tricuspid valve detachment for transatrial ventricular septal defect closure

BACKGROUND: Although temporary tricuspid valve detachment is useful for improved visualization of ventricular septal defect through right atriotomy, liberal use of this adjunct is not widely supported, mainly because of concerns about iatrogenic complications such as heart blocks and tricuspid valve dysfunction. The objective of this study was to determine whether liberal use of this adjunct can improve operative outcome. METHODS: Between January 1997 and March 2002, trans-atrial closure of isolated ventricular septal defect (conoventricular or canal type) was performed in 87 consecutive patients. Tricuspid valve detachment was used in 4 out of 44 patients (prudent-use group) and 19 out of 43 patients (liberal-use group) in the first and second half of this period, respectively (p = 0.0002). Patient demographics and use of other surgical and cardiopulmonary bypass techniques remained virtually unchanged during this period. RESULTS: In the prudent-use group, there was one operative death with prolonged bypass time and one residual defect that required reoperation; neither of these patients underwent tricuspid valve detachment. All other patients (both groups) were free from mortality and clinically significant complications, including heart block, tricuspid regurgitation, and residual defect. The liberal-use group had shorter cardiopulmonary bypass time than the prudent-use group (59 +/- 14 vs 67 +/- 22 minutes, p = 0.037). CONCLUSIONS: Tricuspid valve detachment should be used liberally for moderate- or even low-difficulty exposure of ventricular septal defect, regardless of patient background, because it is a safe and effective adjunct that can improve speed, programmability, reproducibility, and reliability.