Evaluation of the temperament and character properties of patients with ankylosing spondylitis.

PURPOSE: The aim of this study was to evaluate temperament and character of ankylosing spondylitis (AS) patients and to examine the association between these specific temperament and character properties and clinical variables. PATIENTS AND METHODS: This study involved 73 AS patients. Temperament properties of patients were evaluated using Cloninger's Temperament and Character Inventory (TCI). Association between clinical variables and specific temperament features were evaluated using correlation and regression analyses. RESULTS: Forty eight (65.8 %) of the study participants were men and the mean age was 42 ± 11.4 years. There was slight negative correlations between self directedness (S) and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) scores (p = 0.01, r = - 0.30), and between the Visual Analog Scale (VAS) and reward dependence (RD) scores (p = 0.03, r = - 0.26). Regression analysis showed that correlations between BASDAI and S, and between VAS and RD scores were statistically significant. CONCLUSION: Our study showed that the dimensions temperament and character are related to disease activation, and disease course is more severe in patients who have low scores in these TCI dimensions. Therefore, we suggest that evaluating temperament and character properties of AS patients will help clinicians to predict treatment compliance and motivation of patients during disease course.

Performance of a hybrid central venous catheter utilized for both peripheral blood stem cell harvest and transplant support of patients undergoing autologous peripheral blood stem cell transplantation.

Patients undergoing autologous peripheral blood stem cell transplantation (PBSC) frequently require the sequential insertion of two central venous catheters, one for leukapheresis and one for transplant support. Hybrid catheters suitable for leukapheresis and long-term use have been increasingly used, but there is limited information regarding their performance and complication rate. The purpose of this study was to determine the performance of the Pheres-Flow hybrid catheter when utilized for both leukapheresis and transplant support, with particular emphasis on the incidence of infectious and occlusive complications. We prospectively analyzed the performance of 92 catheters in 82 consecutive patients who underwent autologous peripheral blood stem cell (PBSC) transplantation. Occlusion was the most frequent complication of this catheter with 29% of the patients experiencing difficulty drawing blood or infusing fluids. Infection was another frequent complication. Twenty-two percent of patients developed catheter-related bloodstream infections and 15 catheters had to be removed because of proven or suspected infection that did not respond to antibiotic therapy. Nevertheless, 77% of patients were able to complete leukapheresis and transplant support with only one catheter. We conclude that the utilization of the Pheres-Flow catheter for both leukapheresis and transplant support is feasible, but that new strategies need to be developed to decrease the incidence of occlusive and infectious complications of hybrid catheters.

Ig heavy chain CDR3 size diversities are similar after conventional peripheral blood and ex vivo expanded hematopoietic cell transplants.

It is largely unknown whether the immune repertoire can be reconstituted successfully after high-dose chemotherapy and transplantation using ex vivo expanded hematopoietic stem cell (HSC) grafts. It is critically important for the transplant outcome that immune repertoire reconstitution progresses after ex vivo expanded HSC graft transplants at least as efficiently as that seen after conventional HSC transplants. Previously, we showed that the T cell receptor V beta (TCRVB) third complementarity determining region (CDR3) diversification after ex vivo expanded bone marrow (BM) HSC graft transplants was similar to that seen after conventional peripheral blood stem cell transplants (PBSCTs). In the present study, the CDR3 diversity of the six immunoglobulin (Ig) heavy chain variable region gene (V(H)) families was examined in five breast cancer patients who were transplanted with ex vivo expanded BM HSCs as the only source of stem cells. For comparison, 12 healthy adults and four conventional PBSCT recipients were also studied. Using both CDR3 fingerprinting and single strand conformation polymorphism (SSCP) methodologies, it is shown that the contribution of the V(H) families to the overall repertoire among healthy adults is highly variable and not always proportional to V(H) family member size. After both ex vivo expanded HSC transplants and conventional PBSCTs, the V(H) CDR3 repertoires were limited in size diversity at 6 weeks post transplant. By 6 months, however, V(H) families displayed a repertoire diversity that was as complex as that seen in healthy adults. No difference was seen between ex vivo expanded HSC graft transplant recipients and conventional PBSCT recipients in V(H) repertoire diversity. In one patient there was a follow-up analysis 12 months after ex vivo expanded graft transplant, and the diversity of the V(H) families was maintained. In all patients, the amino acid size of the CDR3 regions fell within adult limits at all time points post transplant. These results indicate that B cell repertoire regeneration after ex vivo expanded hematopoietic cell graft transplants is similar to that seen after conventional PBSCT.

Ex vivo-expanded hematopoietic cell graft recipients exhibit T cell repertoire diversity similar to that seen after conventional stem cell transplants.

The feasibility of using ex vivo-expanded hematopoietic progenitor cells to reconstitute hematopoiesis after high-dose chemotherapy is presently being examined. Early studies have shown that myeloid and erythroid hematopoiesis can be successfully reconstituted after high-dose chemotherapy and ex vivo-expanded hematopoietic cell transplantation. The lymphoid reconstitution, however, has not been addressed previously. In this study, we examined the diversity of the T cell receptor V beta chain (TCRBV) repertoires in 5 breast cancer patients who were transplanted with ex vivo-expanded bone marrow mononuclear cells as the only source of hematopoietic graft. Using the TCRBV third complementarity determining region (CDR3) fingerprinting methodology, it is shown that CD4(+) and CD8(+) T cell subsets after ex vivo-expanded hematopoietic cell graft transplants exhibit TCRBV diversities that are similar in complexity when compared to those seen after conventional autologous peripheral blood stem cell transplants (PBSCT). No apparent difference in the extent of CDR3 diversity was found between ex vivo expanded and conventional autologous PBSCT recipients when the CD4(+) and CD8(+) subsets were further separated into CD45RA(+) "naïve" and CD45RO(+) "memory" subsets. The diversity of the CD45RA(+) naïve subsets was as complex as that of the CD45RO(+) memory subsets. These results indicate that T cell repertoire diversification is not further compromised when ex vivo-expanded hematopoietic cells are used instead of autologous peripheral blood stem cells as the only source of graft.

Neuromuscular consequences of prolonged hunger strike: an electrophysiological study.

OBJECTIVES: The purpose of this study was to determine the electrophysiological consequences of neuromuscular and central nervous system involvement in a group of patients presented with the neurological complications of a long-term hunger strike (HS). METHODS: Motor and sensory nerve conduction (NCV), F wave, somatosensory evoked potential (SEP) and motor evoked potential (MEP) studies were performed in 12 male and 3 female patients (mean age: 29.4) following HS. RESULTS: All patients whose weight loss was 11-31 (mean: 22.8) kg after 69-day HS, had neurological findings consistent with Wernicke's encephalopathy or Wernicke-Korsakoff syndrome. Abnormally prolonged latency and/or low amplitude sensory nerve action potentials were found in 7 patients. The amplitudes of compound muscle action potentials were significantly reduced in ulnar, median and tibial motor NCV studies as compared to the controls. F waves elicited by median nerve stimulation at wrist and muscle responses evoked by cervical and lumbar magnetic stimulation had significantly prolonged latencies. MEPs recorded from the lower extremities showed a slight prolongation in central conduction times. The cortical response latencies were prolonged in tibial SEPs. CONCLUSIONS: The most prominent finding in this patient group was the low amplitude of CMAPs elicited in motor NCV studies which was concluded to be resulted from the reversible muscular changes. The other electrophysiological findings suggested that peripheral nerves and long central nervous system pathways were also mildly involved.

Ex-vivo expansion of bone marrow progenitor cells for hematopoietic reconstitution following high-dose chemotherapy for breast cancer.

The use of hematopoietic growth factors, stromal monolayers, and frequent medium exchange allows the expansion of hematopoietic progenitors ex-vivo. We evaluated the use of ex-vivo expanded progenitor cells for hematopoietic reconstitution following high dose chemotherapy (HDC) in breast cancer patients. Patients with high-risk Stage II or metastatic breast carcinoma underwent bone marrow aspirations using general anesthesia. A total of 675-1125 x 10(6) mononuclear cells (MNC) were seeded for ex-vivo expansion for 12 days in controlled perfusion bioreactors (Aastrom Biosciences, Inc.). The bone marrow cultures, which included the stromal cells collected with the aspirate, were supplemented with erythropoietin, granulocyte-macrophage-colony stimulating factor (GM-CSF)/IL-3 fusion protein (PIXY 321), and flt3 ligand. Stem cell transplant was performed with expanded cells after HDC. A median bone marrow volume of 52.9 mL (range 42-187 mL) was needed to inoculate the bioreactors. Median fold expansion of nucleated cells (NC) and colony forming unit granulocyte-macrophage (CFU-GM) was 4.9 and 9.5, respectively. The median fold expansion of CD34+lin- and long-term culture-initiating culture (LTC-IC) was 0.42 and 0.32, respectively. Five patients were transplanted with ex-vivo expanded NC. Median days to an absolute neutrophil count > 500/microL was 18 (range 15-22). Median days to a platelet count > 20,000/microl was 23 (range 19-39). All patients had sustained engraftment of both neutrophils and platelets. Immune reconstitution was similar to that seen after HDC and conventional stem cell transplantation. We conclude that ex-vivo expansion of progenitor cells from perfusion cultures of small volume bone marrow aspirates, allows hematopoietic reconstitution after HDC.

Analysis of clonal diversity in mouse immunoglobulin heavy chain genes selected for size of the antigen combining site.

Size-diversity of Ig and T cell receptor antigen binding (CDR3) regions can be visualized by "CDR3 fingerprinting", and provides an estimate of B- or T-cell repertoire complexity. The method does not identify clonal diversity, however, which can only be determined by random sequencing of the CDR3s. In this study we demonstrate that a combination of fingerprinting and single strand conformation polymorphism (SSCP) analysis can be used for a rapid estimation of clonal diversity within mouse Ig antigen binding regions selected for size. This application may be useful in the analysis of clonal expansion within B- and T-cell repertoires.

Ig heavy chain third complementarity determining regions (H CDR3s) after stem cell transplantation do not resemble the developing human fetal H CDR3s in size distribution and Ig gene utilization.

Previous studies have suggested that the B-cell repertoire after stem cell transplantation resembles the developing repertoire in the fetus. Fetal and adult repertoires differ strikingly at the molecular level in Ig heavy chain third complementarity determining region (H CDR3) size distribution and Ig gene utilization. Previously, the posttransplant repertoire has not been studied fully in this regard. In this study, we analyzed H CDR3s posttransplant using CDR3 fingerprinting, single-strand conformation polymorphism (SSCP), and random sequencing. Eleven adult patients who received either autologous (n = 6) or allogeneic adult sibling (n = 5) hematopoietic stem cell transplants were studied. IgM H CDR3 repertoires demonstrated limited clonal diversity within the first 6 to 10 weeks posttransplant. By 3 to 4 months, the IgM H CDR3 repertoires were as diverse as those in healthy adults. Reconstitution of the IgM diversity correlated with the expansion of the multimember VH3 family. By contrast, the contribution of the single-member VH6 family was limited in most patients up to 6 to 9 months. No evidence was seen for greater contribution of VH6 posttransplant. IgG repertoires remained clonally restricted at all times. In all patients, H CDR3 sizes fell within adult limits. Direct nucleotide sequencing of H CDR3s showed adult-type N-nucleotide insertions and Ig gene utilization. These results indicate that the emerging repertoire posttransplant does not resemble the developing fetal repertoire at the molecular level.

Thoracic involvement in Behçet's disease: pathologic, clinical, and imaging features.

Behçet's disease is a rare form of vasculitis of obscure etiology. Any large or small artery, vein, or organ may be involved in an unpredictable combination. Intrathoracic manifestations of Behçet's disease consist mainly of thromboembolism of the superior vena cava and/or other mediastinal veins; aneurysms of the aorta and pulmonary arteries; pulmonary infarct and hemorrhage; pleural effusion; and, rarely, myocardial or pericardial involvement, cor pulmonale, and mediastinal or hilar lymphadenopathy. Chest radiography is the best diagnostic method for evaluating thoracic involvement in Behçet's disease. Because aneurysms may develop at the arterial puncture sites and veins may be quickly thrombosed after injection of contrast material, angiography and venography should be avoided whenever possible. Although no comparative studies are available, CT and MR angiography appear to be imaging techniques of choice for evaluating vascular involvement. Pulmonary parenchymal alterations depicted on CT scan have not been fully explored.

Pulmonary Gaucher's disease: high-resolution computed tomographic features.

CT findings in pulmonary Gaucher's disease have not been previously reported. Chest radiograph of a patient with pulmonary involvement in type I Gaucher's disease proven by biopsy showed linear and reticulo-nodular opacities. High-resolution CT demonstrated thickening of the interlobular septa and between four and six small nodules within secondary lobules, probably each corresponding to an acinus.

CT in lobar atrophy of the liver caused by alveolar echinococcosis.

We reviewed the CT findings of 13 patients with histologically proved alveolar echinococcosis of the liver. Seven of these patients had hilar involvement, which caused dilatation of intrahepatic bile ducts and invasion of the portal vein or portal branches. In two of these patients we found marked atrophy of the right lobe and in one patient marked atrophy of the left lobe in addition to various CT findings previously reported for alveolar echinococcosis of the liver. The occurrence of lobar atrophy of the liver caused by alveolar echinococcosis has not been previously reported. We concluded that, when a mass lesion with hilar infiltration causing lobar atrophy is found in endemic areas, alveolar echinococcosis should be considered in the differential diagnosis in addition to malignant neoplasms.

Purely cystic hydatid disease of the liver: treatment with percutaneous aspiration and injection of hypertonic saline.

Percutaneous aspiration of purely cystic liver lesions was performed in 15 patients aged 11-56 years. After aspiration under guidance with computed tomography (CT) in 12 patients, a membrane that is diagnostic for hydatid disease was visible in the lumen of the cyst on CT scans. Hypertonic saline was injected in the cystic cavities of these patients as a scolecidal agent. No major complications occurred during or after the procedures. In the follow-up period of 6-16 months, control CT and ultrasound scans revealed a progressive decrease in the size of the lesions and no evidence of peritoneal seeding. It is concluded that percutaneous aspiration and hypertonic saline injection for purely cystic hydatid disease of the liver seem to be an effective form of treatment and may eventually prove to be an alternative to surgical intervention.

Preoperative CT staging of colon carcinoma (excluding the recto-sigmoid region).

Twenty-eight patients with colon carcinoma (excluding the recto-sigmoid region) underwent preoperative staging with computed tomography (CT). The CT had a sensitivity and a specificity of 60 and 67% for detection of extramural invasion, 75% sensitivity and specificity for lymph node metastases and a sensitivity of 87% and specificity of 95% for liver metastases. Compared with the modified Dukes classification, CT correctly staged 50% of the patients with Dukes A lesions; 40% with Dukes B; 75% with Dukes C and 85% with Dukes D lesions. The data presented in this study showed that CT has limitations in the sensitivity and accuracy of staging local colonic carcinoma. However, we recommend its use for patients who are clinically suspected of having extensive disease.

Coexistent xanthogranulomatous pyelonephritis and massive replacement lipomatosis of the kidney: CT diagnosis.

Replacement lipomatosis (RPL) is seen in patients in whom renal parenchyma is destroyed due to chronic calculous disease and inflammation. The triggering mechanism for xanthogranulomatous pyelonephritis (XGP) is also the same. We report a case in which RPL and XGP coexist in the same kidney. To our knowledge, this coexistence has not been previously reported.

Balloon dilatation of iatrogenic urethral strictures.

Balloon dilatation of the urethra was performed in five patients with iatrogenic urethral strictures. The urethral strictures were successfully negotiated and dilated in all patients. Redilatation became necessary in a period ranging from 3 to 10 months. The authors believe that balloon dilatation of the urethra can be safely and successfully performed; the procedure produces minimal trauma and immediate relief of symptoms.

[Behaviour of the sella turcica in juveniles with severe iron deficiency (author's transl)]. / Das Verhalten der Sella turcica bei jugendlichen Patienten mit schwerer Eisenmangelkrankheit.

In juvenile patients with a severe and longstanding iron deficiency disease several abnormal features such as delayed growth, retarded development and maturation are displayed. The roentgenological examination of the sella turcica in 81 patients and the determination of the sellar size in 73 cases, including 40 men and 33 women, by employing the method of Haas with a slight modification and the following comparison of the results with the values obtained by this author in normal individuals of the same sex and age revealed that in the iron deficient patients the sellar area is considerably reduced. In addition, it could be shown that in the patients the normal augmentation of the size with the advancing age is likewise diminished and comes to an early standstill. It could be, moreover, demonstrated that in men the sellar size and growth are more reduced than in women and that the difference in the patients is much larger than the difference existing normally between both sexes. The reduced size of the sella turcica in the patients indicated that the pituitary gland placed in the sellar cavity is likewise diminished and that the activity of the gland, as far as the function is related to the volume, is equally decreased. This assumption is in good keeping with the clinical findings in the patients being attributed to a low and failing hormonal activity. The close relation between volume and function of the pituitary gland, is proved by the observation made in the patients after the administration of iron showing that the size of the sella is considerably augmented during a relatively short space of time and that at the same time a definite amelioration of clinical symptoms occurs. Thus the X-ray examination of the sella turcica and the determination of its area is a suitable method for a comparative investigation of the size and function of the pituitary gland. It offers a valuable tool for studying the disorders of growth, development and maturation in juvenile patients with a severe and longstanding iron deficiency disease and for investigating the relation of these clinical features to the pituitary function.

[Radiological determination of skull thickness and skull thickness increase in patients with severe blood dyscrasias and hyperplasia of the red marrow (author's transl)]. / Zur röntgenologischen Bestimmung der Dicke der Schädelknochen und ihrer Verbreiterung bei Patienten mit schwerer Bluterkrankung und Hyperplasie des roten Knochenmarks

A method is described for measuring the thickness of the cranial bones in x-ray pictures of the skull, which in a reliable way can only be done in the frontal and the parietal bones. From the results, representative and comparable values have been obtained consisting of the mean and maximum thickness of the bones and of the mean deviation of the thickness in the bone. By using these values, the thickness has been determined in normal persons and the increase of thickness established in different patients suffering from a number of blood disorders. A scale of increasing degrees of thickening could be set up ranging form normal conditions to extraordinary enlargement. As the thickening of the bones is due to the hyperactivity of the red marrow contained in the bones, and the increase of thickness depends on the intensity of the overgrowth of the marrow and of the time of its action on the osseous substance, from the thickness of the bones a conclusion can be drawn regarding the presence, the severity and the duration of the existing blood disorder. Since, moreover, the enlargement of the bones is irreversible, an increased thickness of the cranial bones may, in patients without alterations in the blood, point to a former blood disorder.