Long-term complete haematological and molecular remission after allogeneic bone marrow transplantation in a patient with a stem cell myeloproliferative disorder associated with t(8;13)(p12;q12)

A rare atypical myeloproliferative disorder (aMPD) associated with chromosomal translocations involving the short arm of chromosome 8, region p11-p12 has been described. In most patients, the cytogenetic abnormality is a t(8;13)(p12;q12) that fuses fibroblast growth factor receptor 1, the 8p12 key gene, to FIM/ZNF198 gene. Prognosis is poor with frequent evolution to acute myeloid leukaemia within 1 year of diagnosis. We report a new patient with aMPD with a t(8;13) translocation. Complete haematological, cytogenetic and molecular remission was demonstrated 39 months after allogeneic bone marrow transplantation. This is the first report to demonstrate a molecular remission in this disorder.     

Single-stage reoperative repair of chronic type A aortic dissection using the arch-first technique

BACKGROUND: Management of the enlarged, chronically dissected aorta after previous repair of acute ascending aortic dissection or after a previous cardiac operation may present a formidable technical challenge and the optimal method of management is not clearly established. METHODS: Twenty-one patients with chronic type A aortic dissection (mean age 57 years) underwent resection of the ascending aorta, the aortic arch, and varying segments of the descending thoracic aorta. Single-stage replacement with perfusion of the aortic arch first to minimize the duration of brain ischemia and a bilateral anterior thoracotomy (clamshell) incision were used. Fourteen patients had undergone previous repair of acute type A dissection. Seven patients had type A dissection after aortic valve replacement (3 patients) or coronary artery bypass (4 patients). Marked enlargement of the aorta distal to the left subclavian artery precluded a two-stage repair. The mean interval between the initial and reoperative procedures was 69 months (range, 5 to 249). RESULTS: There was 1 (4.8%) hospital death. Four patients required reoperation for bleeding. One patient required a right ventricular assist device that was successfully removed. Ten patients required assisted ventilation for more than 48 hours. All were successfully weaned from ventilatory support. No patient had a stroke or other adverse neurologic outcome. There has been 1 late death (mean follow-up 2 years). CONCLUSIONS: The single-stage, arch-first replacement technique is a safe and effective procedure for patients who require extensive reoperations for chronic expanding type A dissection.     

Hypothermic cardiopulmonary bypass and circulatory arrest for operations on the descending thoracic and thoracoabdominal aorta

BACKGROUND: Hypothermic cardiopulmonary bypass with circulatory arrest is an important adjunct for operations on the distal aortic arch, the descending thoracic, and the thoracoabdominal aorta. The safety and efficacy of this technique when compared with other adjuncts (ie, simple aortic clamping, partial cardiopulmonary bypass, regional hypothermia) is not clearly established. METHODS: One hundred and ninety-two patients (age range, 20 to 83 years) with descending thoracic or thoracoabdominal aortic disease had resection and graft replacement of the involved aortic segments using hypothermic cardiopulmonary bypass and intervals of circulatory arrest (mean, 38 minutes). The technique was used when the location and severity of disease precluded placement of clamps on the proximal aorta (31 patients) or (in 161 patients) when extensive thoracic (47) or thoracoabdominal (114) aortic disease was present, and the risk for development of spinal cord ischemic injury was judged to be increased. Lower intercostal and lumbar arteries were attached separately to the aortic graft in 101 of the 161 patients (63%) who had extensive aortic replacement. No other adjuncts for spinal cord protection were used. RESULTS: The 30-day mortality was 6.8% (13 patients). It was 40% (8 of 20) for patients having emergent operations (acute aortic dissection or rupture) and 2.9% (5 of 172) for all others (p < 0.001). The 90-day mortality was 12.5% (24 patients). Paraplegia occurred in 4 and paraparesis in 1 (full recovery) of the 186 operative survivors whose lower limb function could be assessed postoperatively (2.7%). Among the 109 survivors with thoracoabdominal aortic disease, early paraplegia occurred in 1 of 36 with Crawford extent I, 0 of 42 with extent II, and 2 of 31 with extent III disease. One patient (extent II) developed paraplegia on the 9th postoperative day after a hypotensive episode. None of the 47 patients with aortic dissection developed paralysis. Among the 186 operative survivors, renal dialysis was required in 4 patients (2.2%), prolonged inotropic support in 18 (10%), reoperation for bleeding in 9 (5%), mechanical ventilation (> or = 48 hours) in 64 (34%), and tracheostomy in 17 (9%). Four patients (2%) sustained a stroke. CONCLUSIONS: Hypothermic cardiopulmonary bypass with circulatory arrest provides safe and substantial protection against paralysis and renal, cardiac, and visceral organ system failure that equals or exceeds that of other currently used techniques but without the need of other adjuncts.     

Outcome measures of interepisode bipolar patients in a Turkish sample

Background We aimed to study the correlations of several outcome measures in bipolar patients with the clinical features of interepisode period. Methods Bipolar patients who were diagnosed according to DSM-III-R or IV were contacted and asked for a further evaluation. Interepisode bipolar patients (n = 100) were interviewed with the Schedule for Affective Disorders and Schizophrenia (SADS). In addition the Brief Disability Questionnaire (BDQ), the Quality of Life Enjoyment and Satisfaction Questionnaire (Q-LES-Q) and the Global Assessment Scale (GAS) were administered to assess outcome. They were also asked to check the List of Life Events (LLE) for the last six months. Results Our results can be summarised as follows: (1) quality of life was predicted by current subthreshold depressive symptoms; (2) the number of previous depressive episodes, current subthreshold depressive and manic symptoms predicted disability; (3) the number of previous depressive episodes and the duration of hospitalisation as well as current subthreshold depressive and manic symptoms predicted overall functioning; (4) the number and distress level of life events were correlated with suicidal symptoms. Conclusions Our findings suggest that outcome measures were correlated with subsyndromal disorder, the number of previous depressive episodes and the duration of hospitalisation. Accepted: 2 October 2001     

An epilepsy questionnaire study of knowledge and attitudes in Canadian college students

Summary: Purpose: Controversy exists about the relation of societal knowledge and attitudes regarding epilepsy. We conducted a survey to examine knowledge and attitudes, to note gender and occupational influences, and to examine the effect of an informational brochure. Methods: We administered a standardized questionnaire that noted demographics and examined knowledge and attitudes regarding epilepsy and persons with epilepsy, respectively, to a wide variety of Canadian college students. In a separate class we gave every other student a brochure regarding epilepsy and then administered the questionnaire to both the naïve and brochure‐exposed students. Results: Knowledge was patchy and weakest for the approximate prevalence of epilepsy in the population, hereditary epilepsy and several other etiologies, recognition of nonconvulsive seizures as a type of epilepsy, and knowledge of antiepileptic drug–induced teratogenicity. In contrast, attitudes were more uniformly favorable. However, 11 and 14%, respectively, showed negative bias against persons with epilepsy having children and equal opportunity for occupational employment. Women were slightly but significantly more tolerant than men. The brochure‐exposed group showed better knowledge but equivalent attitudes compared with the naïve group. Conclusions: Results compare favorably with surveys in other countries. Although knowledge was patchy, it could be easily improved on with an educational brochure. Attitudes were positive but show some discrepancies from knowledge and a gender effect.     

Sequential chemotherapy by CHOP and DHAP regimens followed by high-dose therapy with stem cell transplantation induces a high rate of complete response and improves event-free survival in mantle cell lymphoma: a prospective study.

Mantle cell lymphoma (MCL) is a distinct clinico-pathological entity with a poor prognosis. We have conducted a prospective study in patients with MCL to evaluate a therapeutic strategy in which CHOP polychemotherapy was followed by DHAP if CHOP failed to induce complete remission. Responding patients then proceeded to an intensification therapy with autologous peripheral blood stem cell transplantation (APBSCT). Twenty-eight consecutive patients with newly diagnosed aggressive MCL were included. After four cycles of CHOP regimen, two complete responses (CR) were obtained (7%) and 14 (50%), five (18%) and seven (25%) patients achieved partial (PR), minor (MR) and no response, respectively (one patient died from septic complications during CHOP induction). The two patients in CR after CHOP underwent intensification with TBI, high-dose cyclophosphamide-etoposide and APBSCT. The other twenty-five patients received DHAP and in this group a response rate of 92% (21 CR (84%), two PR (8%)) was observed. Two patients had progressive disease. The twenty-three responding patients received high-dose therapy (TAM8 regimen: TBI-cytarabine-melphalan) followed by APBSCT. One of the two partial responding patients achieved CR after TAM8. After a median follow-up of 47.6 months (range, 14-70), seven patients have relapsed. Our data confirm that: (1) CHOP regimen induces a low CR rate in MCL; (2) CHOP plus DHAP appears to be much more efficient and allows a large proportion of patients to proceed to high-dose therapy in CR; (3) consolidation therapy including TBI and high-dose Arac-C followed by APBSCT may improve event-free survival.     

Ischemic optic neuropathy following spine surgery

Perioperative visual loss (POVL) is a devastating injury that has been reported infrequently after nonocular surgery. The most common cause of POVL is ischemic optic neuropathy (ION). Increasing numbers of cases of ION are being reported after spine surgery, but the etiology of postoperative ION remains poorly understood. After a MEDLINE search of the literature, we reviewed published case reports of ION, specifically those reported after spine surgery performed with the patient in the prone position. Most of the cases involved posterior ION (PION, n = 17), and the remainder anterior (AION, n = 5). Most patients had no or few preoperative vascular disease risk factors. All except one PION and 2 of 5 AION cases reported symptom onset within the first 24 hours after surgery. Visual loss was frequently bilateral (40% of AION, 47% of PION cases). Mean operative time exceeded 450 minutes. The lowest average intraoperative mean arterial blood pressure was 64 mm Hg and the mean lowest intraoperative hematocrit was 27%. The average blood loss was 1.7 L for AION and 5 L for PION patients. PION patients received an average of 8 L of crystalloid solution and 2.2 L of colloid intraoperatively. This compilation of case reports suggests that a combination of prolonged surgery in the prone position, decreased ocular perfusion pressure, blood loss and anemia/hemodilution, and infusion of large quantities of intravenous fluids are some of the potential factors involved in the etiology of postoperative ION. However, levels of blood pressure and anemia intraoperatively were frequently at levels considered acceptable in anesthesia practice. The etiology of postoperative ION remains incompletely understood. Potential strategies to avoid this complication are discussed.     

Changes of adiponectin oligomer composition by moderate weight reduction

Adiponectin affects lipid metabolism and insulin sensitivity. However, adiponectin circulates in three different oligomers that may also have distinct biological functions. We aimed to analyze the role of these oligomers in obesity and lipid metabolism after weight reduction. A total of 17 obese volunteers (15 women and 2 men) participated in a weight reduction program. Individuals were characterized before and after 6 months of a balanced diet. Adiponectin was determined by enzyme-linked immunosorbent assay, and oligomers were detected by nondenaturating Western blot. BMI decreased (35.1 +/- 1.2 to 32.8 +/- 1.1 kg/m(2), P < 0.001), which was associated with an improved metabolite profile. Total adiponectin increased from 5.3 +/- 0.5 to 6.1 +/- 0.6 microg/ml (P = 0.076). High (HMW) and medium molecular weight (MMW) adiponectin oligomers significantly increased during weight reduction (HMW: 0.37 +/- 0.07 to 0.4 +/- 0.08 microg/ml, P = 0.042; MMW: 2.3 +/- 0.2 to 2.9 +/- 0.3 microg/ml, P = 0.007), while low molecular weight (LMW) did not significantly change. Body weight inversely correlated with HMW (r = -0.695, P = 0.002) and positively with LMW (r = 0.579, P = 0.015). Interestingly, HDL cholesterol and HMW were strongly correlated (r = 0.665, P = 0.007). Indeed, HMW and free fatty acids before weight reduction predicted approximately 60% of HDL changes during intervention. In conclusion, weight reduction results in a relative increase of HMW/MMW adiponectin and a reduction of LMW adiponectin. Total adiponectin and especially HMW adiponectin are related to circulating HDL cholesterol.     

An alternative immunohistochemical method for detecting Leishmania amastigotes in paraffin-embedded canine tissues

Canine visceral leishmaniasis (CVL) is a zoonosis and a chronic systemic disease of the dog caused by a protozoan by the species Leishmania infantum in the Old World and Leishmania chagasi in the New World. Several methods are currently employed for the diagnosis of CVL including microscopic detection of the parasite in bone marrow and lymph node aspirates, demonstration of specific antibodies anti-Leishmania in sera from infected animals, and isolation of the parasite by in vitro culture or by inoculation of laboratory animals. However, a definitive diagnosis is based on the actual detection of the parasite, which is conventionally achieved by examining Giemsa-stained smears or histopathological sections stained with hematoxylin and eosin. These methods have a low sensitivity, and therefore, they are often inconclusive. This is particularly true in canine organs that have a low level of parasitism such as kidneys, lungs, central nervous system, and testis, or, in some cases, the skin. The technique for immunohistochemical detection of leishmanial amastigotes in canine tissues has been reported previously and has proved to be undoubtedly efficient for the diagnosis. In this paper, we describe a straightforward and inexpensive immunohistochemical approach for Leishmania detection in formalin-fixed paraffin-embedded canine tissues. Amastigote forms of Leishmania were easily observed within macrophages in several organs from naturally infected dogs using the streptavidin-biotin immunohistochemical method with canine hyperimmune serum as the primary antibody. In addition, the secondary antibody used was not specific to canine immunoglobulin, characterizing a cross-immune reaction. Our results indicate that this technique could be a useful tool for epidemiological, clinical, and histopathological studies.