Profile of Reiter's disease in Saudi Arabia

We studied the clinical features of cases of Reiter's disease as seen in two tertiary care hospitals in Riyadh, Saudi Arabia, over a period of 9 years. We gathered 34 cases, 29 (85%) males and 5 (15%) females (M:F ratio 5.8:1). The mean age of onset was 29.4 (SD 6.4) years. Gastrointestinal and genitourinary infection were elicited in 24 patients (71%). Fever occurred in 6 patients (18%), fatigue in 13 (38%) and conjunctivitis in 13 (38%). Lower limb large joints were affected at presentation in 23 patients (68%), followed by combined presentation of peripheral joints and axial joints in 10 patients (30%). Sacroilitis was seen in 5 cases (15%). One patient had spondylitis, while enthesopathy was seen in 6 patients (18%). Bone scintigraphy was done in 14 patients, 11 of which were positive at peripheral joints, while 9 showed increased uptake at the sacroiliac joints. HLA B27 status was tested in 15 patients, 4 (27%) of which were positive.     

A follow-up study of 48 patients with Reiter's syndrome

A follow-up study of 48 patients with Reiter's syndrome was carried out in an attempt to clarify the clinical course of the disease. The mean age at the onset of Reiter's syndrome was 27.1 years (range 15 to 52 years) and when seen at follow-up 32.5 years (range 19 to 58 years). The average time from the onset of the first attack of peripheral arthritis to the time of follow-up was six and a half years (range 0.5 to 27 years). Only three patients had diarrhea prior to the onset of Reiter's syndrome. At follow-up 22 per cent of the patients were asymptomatic, 24 per cent had recurrent minor symptoms, 24 per cent had recurrent moderate symptoms, and 30 per cent had recurrent major symptoms. However, even in the last group, all patients were in functional classes 1 or 2 between the flares of disease. No patients in the series were in functional class 3 or 4, and 30 per cent were in class 1.     

Reiter's disease in northern India

Thirty-six patients fulfilling the criteria for Reiter's disease were studied (29 men and 7 women; 4:1). The mean age of onset was 23.8 years; in 75% of patients the onset was in the second and third decades. Clinical manifestations included low back pain and stiffness (69%), non specific urethritis (53%), heel pain (44%), radiographic sacroiliitis (42%), conjunctivitis (39%), dysentery and diarrhoea (33%), mucosal ulcerations (17%) kidney disease (14%), anterior uveitis (19%), and keratoderma blennorrhagicum (8%). Peripheral arthritis was mono or oligoarthritis in 58% of patients, mainly affecting the large joints of lower extremities, and it was often asymmetric (mean degree of asymmetry = 0.37). HLA-B27 antigen was detected in 83% of 36 patients compared with 5.9% of 118 controls (relative risk 79, corrected Fisher's P = 7.719E-18).     

Krabbe disease in adults: phenotypic and genotypic update from a series of 11 cases and a review

Krabbe disease usually presents as a severe leukodystrophy in early infancy and childhood. From a series of 11 patients and 30 cases previously reported in the literature we describe the clinical, radiological, electrophysiological and genetic features of adult Krabbe disease. Patients diagnosed after the age of 16 years were included in this study. They were further divided into three groups depending on age at symptoms onset: (1) childhood onset cases (n?=?7); (2) adolescence onset cases (n?=?6) and adult onset cases (n?=?28). Overall, 96 % of patients in the adult-onset group presented with signs of pyramidal tracts dysfunction. Spastic paraparesis or tetraparesis became prominent in all cases. A peripheral neuropathy was present in 59 % of cases and was most often demyelinating (80 %). Other clinical signs encompassed dysarthria (31 %), cerebellar ataxia (27 %), pes cavus (27 %), deep sensory signs (23 %), tongue atrophy (15 %), optic neuropathy (12 %), cognitive decline (12 %). Cerebrospinal fluid protein concentration was moderately increased in 54 % of patients. Patients in the adolescent- and childhood-onset groups had similar presentations but were more likely to display optic neuropathy (33 % and 57 %) and cerebellar ataxia (50 % and 57 %). In the adult-onset group, the disease progressed slowly over more than 10 years, but a rapid course was observed in two patients. Abnormalities of brain MRI was similar in the three groups and included high signals of cortico-spinal tracts (94 % of cases), hyper-intensities of optic radiations (89 %) and hyper-intensities or atrophy of the posterior part of the corpus callosum (60 %). No clear genotype-phenotype relationship could be demonstrated.     

Bronchiolitis obliterans organizing pneumonia. Clinical and roentgenological features in 26 cases

BACKGROUND: Bronchiolitis obliterans organizing pneumonia (BOOP) may be classified as cryptogenic (idiopathic) and secondary. There are no clear clinical and radiological features distinguishing between idiopathic and secondary BOOP. OBJECTIVES: To analyze the etiologic factors, clinical and radiological features, diagnostic approach and response to therapy at onset and outcome in subjects with BOOP. METHODS: The medical files of Erciyes University Hospital from 1995 to 2003 were retrospectively reviewed. Patients with biopsy-proven BOOP were selected for evaluation. The etiology and initial features of BOOP, treatment, resolution, relapse, and survival were obtained from medical records, and a follow-up patient questionnaire. RESULTS: We have diagnosed 26 cases (13 males /13 females) with BOOP syndrome (mean age 54 +/- 15 years, range 14-93). More than half the patients (58%) were classified as idiopathic BOOP. Patients presented with cough (92%), dyspnea (70%), pleuritic chest pain, hemoptysis and fever (50%). The biopsy specimens had been obtained by transbronchial and/or transthoracic lung biopsy in 18 cases (69%). At radiological evaluation, there were bilateral patchy alveolar and/or interstitial infiltrates in 16 patients (62%), and solitary pneumonic involvement in 10 patients (38%). Three patients recovered spontaneously, 5 remained cured after resection of the focal lesion. Corticosteroid therapy was given in 17 patients (65%). Apart from four patients who died (death was attributable to BOOP in only 1 patient) and three patients who relapsed, the prognosis was good in all patients. CONCLUSIONS: The etiology of BOOP is usually idiopathic. We observed that hemoptysis and pleuritic chest pain were a relatively frequent symptom in BOOP in the present series, in contrast to previous observations. The diversity of radiological and clinical presentations including hemotysis and pleuritic chest pain should prompt consideration of the diagnosis in patients with persisting pulmonary symptoms and radiological findings.     

An "experimental" epidemic of Reiter's syndrome revisited. Follow-up evidence on genetic and environmental factors.

The relation between a specific infective event (shigellosis), a specific disease entity (Reiter's syndrome), and a specific histocompatibility antigen (HL-A B27) is documented by follow-up study of an epidemic of post-Shigella Reiter's syndrome. Five of the original 10 patients have been traced, HL-A typed, and clinically assessed 13 years after the initial episode. One of the 5 has minimal disease, remains symptom-free, and is HL-A B27-negative. The remaining 4 have followed a chronic course, have persistent active disease, and are HL-A B27-positive. It is estimated that after this single episode of shigellosis, from one sixth to one third of the persons who were HL-A B27-positive developed Reiter's syndrome. The prognosis for postdysenteric Reiter's syndrome must be guarded, especially in the subject who is B27-positive.     

Partial interruption of the inferior vena cava by an endovenous filter. Apropos of 98 patients

94 partial interruptions of the inferior vena cava (PIIVC) were carried out in 98 patients between May 1979 and November 1983 with a Mobin-Uddin umbrella filter (58 cases) or a Kim-Ray Greenfield filter (36 cases); one patient who had a double inferior vena cava underwent double PIIVC with a Greenfield filter. The patients (56 women and 42 men) were between 22 and 84 years old (average 60.6 years). Phlebocavography was performed pre-operatively in 93 patients (95 p. 100) and showed thrombus in the IVC (21 cases), common iliac vein (20 cases), ilio-femoral vein (32 cases), femoral vein (15 cases), popliteal and/or sural vein (4 cases); the investigation was considered normal in 1 patient. The diagnosis of pulmonary embolism (PE) was made in 86 patients (87.7 p. 100) on clinical and/or pulmonary scintigraphy and/or angiography data. The main indications for PIIVC were major PE (56 cases) or a threatening venous thrombosis (27 cases); other indications included recurrent PE despite adequate anticoagulation, patients with contra-indications to anti-coagulant therapy and pulmonary hypertension due to thromboembolism; 4 PIIVC were carried out during pulmonary embolectomy on cardiopulmonary bypass. The operative mortality was 3.06 p. 100 (3/98) with a global early mortality of 10.2 p. 100 (10/98); morbidity was 12.2 p. 100 (12/98); there were 5 failures of PIIVC. The long-term outcome was studied in the first 80 cases with a mean follow-up of 18 months (4 to 48 months).(ABSTRACT TRUNCATED AT 250 WORDS)     

Anti-neutrophil cytoplasm antibodies in patients with giant cell arteritis and/or polymyalgia rheumatica.

Anti-neutrophil cytoplasm antibodies (ANCA) were detected at low titers by indirect immunofluorescence in 1 out of 13 patients with giant cell arteritis (GCA) alone, 7 out of 30 with polymyalgia rheumatica (PMR) alone and 4 out of 15 with GCA plus PMR (8, 23 and 27%, respectively). Anti-myeloperoxidase antibodies were also demonstrated by an enzyme-linked immunosorbent assay in 4 patients with GCA alone (31%), 13 with PRM alone (42%) and 5 with GCA plus PRM (33%). The C-reactive protein response was significantly higher (p < 0.05) in ANCA-positive than in ANCA-negative patients.     

Primary ankylosing spondylitis: patterns of disease in a Brazilian population of 147 patients

OBJECTIVE: To analyze patterns of disease in a population of Brazilian patients with primary ankylosing spondylitis (AS). METHODS: Retrospective study (1988-98) analyzing 147 patients with a diagnosis of primary AS according to the modified New York criteria. Selected patients had complete clinical (initial symptom, axial and peripheral involvement, heel enthesitis, extraarticular manifestations) and radiological (sacroiliac, lumbar, thoracic, and cervical spine) investigations, and these data were compared with sex, race, age at onset, and HLA-B27. RESULTS: There was a predominance of men (84.4%), Caucasian race (75.5%), adult onset (> 16 years, 85%), and positive HLA-B27 (78.2%). Family history of AS was noted in 14.3% of the patients. Pure axial AS was observed in 37 patients (25.2%). The predominant initial symptoms were inflammatory low back pain (61.9%) and peripheral arthritis (22.4%). Thoracic and cervical spine involvement was noted in 70.1% of the patients; radiological findings included syndesmophytes in 46.9% and "bamboo spine" in 20.4% of patients. The extraaxial joints most frequently involved were: ankles (39.5%), hips (36.1%), knees (29.3%), shoulders (19%), and sternoclaviculars (14.3%); heel enthesitis was present in 22.4%. Acute anterior uveitis was noted in 14.3% of patients. Male sex was associated with involvement of thoracic spine (p = 0.002), cervical spine (p = 0.002), and hips (p = 0.042), whereas female sex was associated with sternoclavicular (p = 0.024) involvement. Caucasian race presented higher frequency of positive family history (p = 0.023); there was no statistical significance of clinical and radiological variables compared with African-Brazilians. Juvenile onset AS presented higher frequency of ankle (p = 0.012) and knee (p = 0.001) involvement, heel enthesitis (p = 0.001), and total hip replacement (p = 0.038), whereas adult onset was associated with thoracic (p = 0.026) and cervical spine (p = 0.026) involvement and positive family history (p = 0.044). Positive HLA-B27 was associated with ankle involvement (p = 0.007) and heel enthesitis (p = 0.013). CONCLUSION: In this population women showed a milder axial involvement, Caucasian race presented axial and peripheral involvement similar to African-Brazilians, juvenile onset AS was associated with articular involvement of the lower limbs, and positive HLA-B27 was associated with ankle involvement.     

150例小儿病毒性心肌炎的特征及远期随诊

目的 了解小儿病毒性心肌炎的特征及预后.方法 对住院治疗的小儿病毒性心肌炎150例进行总结分析,并对74例进行了1～10年远期随诊的病人分析转归.结果 重症随诊19例,1年内痊愈3例,占15.8%;非重症随诊55例,1年内痊愈30例,占5.5%(P<0.01).起病7天以内就诊的40例,23例1年内痊愈,占57.5%;7无以上就诊的34例,1年内痊愈的11例,占32.4%(P<0.05).COXB_(1～6)抗原阳性20例,1年内痊愈5例,占25%;非COXB_(1～6)抗原阳性54例,1年内痊愈28例,占51.8%(P<0.05).病程早期用FDP治疗的35例,1年内痊愈22例,占62.9%;而不用FDP治疗的39例,1年内痊愈11例,占28.2%,(P<0.01).结论 小儿病毒性心肌炎,非重症病人预后明显好于重症病人.一周内早期诊治可提高预后.COXB_(1～6)抗原持续或反复阳性易使病程迁延或呈慢性.使用FDP可缩短病程,改善预后.     

Human immunodeficiency virus infection associated arthritis: clinical characteristics.

OBJECTIVE: To define the frequency and characteristics of human immunodeficiency virus (HIV) associated arthritis. METHODS: A total of 270 patients with HIV infection were prospectively evaluated for the presence of rheumatic complaints. Diagnosis of HIV infection was performed by ELISA and confirmed by Western blot, and all HIV patients were classified according to the US Centers for Disease Control criteria. RESULTS: Twenty-one (7.8%) patients presented with HIV associated arthritis. Other arthritides including HLA-B27 related, such as Reiter's syndrome, psoriatic arthritis, and rheumatoid arthritis, were excluded. Seventeen were men and 4 women, with a mean age of 34.8 years (SD 11.1). Fourteen (66%) were homosexuals, 4 (19%) intravenous drug users, and 3 (14%) heterosexuals. Twelve (57%) were in stage IV, 5 (23%) in stage III, and 4 (9%) in stage II. Ten (47%) patients had oligoarticular involvement, 8 (38%) monoarticular, 2 (9%) asymmetric polyarthritis, and one (4%) symmetric polyarthritis. Rheumatoid factor and HLA-B27 antigen were negative in all (15) patients studied. The mean duration of arthritis was 2 weeks (1-24). No differences in duration of arthritis were found among the different risk factors (p = 0.811), HIV stages (p = 0.205), and type of articular involvement (p = 0.252). There was, however, a trend between the number of involved joints and stages of HIV infection (p = 0.13). CONCLUSION: The pattern of joint involvement of HIV associated arthritis is similar to that of other viral disorders: acute onset, short duration, no recurrences, and no erosive changes.     

Invasive aspergillosis in haematological malignancies: clinical findings and management for intensive chemotherapy completion.

Sixty-one cases of Aspergillus infection (35 acute myeloid leukemia, 15 acute lymphoid leukemia, one myelodysplastic syndrome, two aplastic anemia, eight non-Hodgkin's lymphoma) seen in our department between January 1989 and July 1999 were studied retrospectively to evaluate the clinical characteristics, to ascertain the factors that influenced the outcome from mycotic infections, and whether early diagnosis and prolonged therapy permitted completion of scheduled intensive chemotherapy and bone marrow transplantation (BMT) without fungal recurrence. The patients were divided into three diagnostic categories: proven aspergillosis (autoptic or histologic diagnosis) n = 39, probable aspergillosis (radiological diagnosis with positive microbiology) n = 9, and possible aspergillosis (radiological diagnosis alone) n = 13. In the same period among 675 acute leukemia patients the incidence of proven or probable aspergillosis was 7.1%. At onset of infection 92% of patients were neutropenic (< 0.5 x 10(9)/L). The most frequent site of infection was the lung (90%); disseminated disease was present in 20 patients. Among 44 assessable patients, 12 (27%) failed to respond to early antifungal therapy and died. Thirty-two patients were cured with antifungal treatment, three of five nonneutropenic with only itraconazole, the others with amphotericin B 1 mg/Kg/day with or without itraconazole subsequently or with liposomal amphotericin, Ambisome, if renal toxicity occurred. Twenty-four of 29 neutropenic responders, all affected by acute leukemia, continued scheduled intensive chemotherapies. Pulmonary lobectomy was successfully combined with medical treatment in two cases before scheduled BMT. After infection nine patients were submitted to BMT (six allo, one marrow unrelated donor (MUD), two auto) with Ambisome or itraconazole as secondary prophylaxis without fungal relapse (follow-up: 25-99 months). The median time from fungal infection to transplant was five months, range 3-10. Thirteen of 29 surviving patients had leukemia relapse, but only three (23%) of these showed also fungal infection recurrence. In conclusion, a high index of suspicion and careful clinical and radiological examinations are the key to identifying infected patients early and to programming the following therapeutic steps. Above all in leukemia patients, prompt and aggressive administration of antifungal agents seems to improve the outcome of invasive fungal disease and to permit intensive chemotherapy completion and transplant.     

Prognosis of inflammatory joint diseases. A three-year follow-up study

The prognosis 3 years after the onset of the disease was studied in 107 patients with definite rheumatoid arthritis, 161 with probable RA or non-specific arthritis, 84 with either ankylosing spondylitis, Reiter's disease or reactive arthritis, 14 with psoriatic arthritis and 10 with a systemic connective tissue disease. Prognosis was measured by clinical involvement of joints, radiological erosions in joints, deterioration in joint function, ESR, and working ability. A total of 44% of all patients were symptomless after 3 years. The prognosis was best in patients with an "HLA B 27-associated" disease and non-specific arthritis, and worst in RA. Two patients died during the follow-up of systemic connective tissue disease and one committed suicide with an overdose of hydroxychloroquine. Two HLA B27-positive patients developed systemic amyloidosis.     

27例原发性胆汁性肝硬化的组织病理学特征

目的 总结原发性胆汁性肝硬化（PBC）患者的临床及病理组织学特点。方法 对27例原发性胆汁性肝硬化患者的临床资料进行分析，重点讨论其肝脏组织病理学特点。结果 本组男、女之比为1：8（3：24），年龄22－69岁。其主要临床症状为乏力（62．9％，17／27），其次为黄疸（59．2％，16／27）和皮肤瘙痒（29．6％，8／27）。患者的血清碱性磷酸酶（ALP）及γ－谷氨酰转肽酶（GGT）均明显升高，95．8％的患者（23／27）抗线粒体抗体或线粒体抗体M2亚型阳性。肝组织病理学特点为：小叶间胆管变性坏死、基底膜不完整，周围有淋巴细胞和浆细胞浸润（66％，18／27）；汇管区淋巴细胞聚集（100％，27／27）或淋巴滤泡形成（15％，4／27）；肉芽肿形成（26％，7／27）及小叶间胆管减少；细小胆管增生（55％，15／27），肝细胞羽毛状变性（59％，16／27）；肝细胞内胆色素沉积和（或）毛细胆管胆栓形成（52％，14／27）；纤维组织增生，小叶结构紊乱（26％，7／27），假小叶形成（11％，3／27）。结论 乏力、皮肤瘙痒及血清ALP、GGT升高及抗线粒体抗体阳性是PBC的主要临床特征；而小叶间胆管炎、胆管数目减少，汇管区淋巴细胞聚集、肉芽肿形成、细小胆管增生，以及肝细胞羽毛状变性是PBC的主要病理特点。     

Clinically silent infections in patients with oligoarthritis: results of a prospective study.

Oligoarticular synovitis of undetermined origin can closely resemble an incomplete form of reactive arthritis/Reiter's syndrome. Eighty three patients with oligoarthritis of undetermined origin were studied prospectively to identify asymptomatic infections potentially triggering the inflammatory response in the synovial fluid. At the time of initial evaluation, 57 (69%) of the patients with oligoarthritis and 4/20 (20%) of the control subjects were carriers of clinically silent infections. Evidence for persistent or prior chlamydial infections was frequently and exclusively found in the study group (30/83 (36%) patients v no controls), whereas undetected urogenital infections with mycoplasma were present in nine (11%) patients and four (20%) controls. Eleven (13%) of the patients carried cellular and humoral responses to Borrelia burgdorferi. The HLA-B27 haplotype represented a major risk factor for the development of oligoarthritis but not for development of sacroiliitis. Re-evaluation after one year showed that the course and outcome of the oligoarticular disease did not correlate with a specific infectious organism and were not affected by antibiotic treatment sufficient to treat the carrier state.     

Cross-Sectional Study of 50 Patients with Calcium Pyrophosphate Dihydrate Crystal Arthropathy

Calcium pyrophosphate dihydrate crystal arthropathy (CPPA) is a well known but heterogeneous disease with a variable presentation and course. We present a cross-sectional study undertaken in a Portuguese rheumatology unit with the aim of analysing clinical and radiological patterns of CPPA in our population. The study population included 50 patients, 34 (68%) women and 16 (32%) men. The mean age was 69.8 ± 8.8 years. The onset features were acute arthritis in 19 (38%) patients and chronic joint complaints in 26 (52%); five (10%) patients were asymptomatic at the time of diagnosis, which was based only on radiological findings. The diagnosis was established in 37 (74%) cases by clinical and radiographic features, in eight (16%) by clinical, X-ray and synovial fluid analysis, and in five (10%) by clinical features and fluid analysis. The disease course was characterised by acute episodic arthritis in 16 (32%) patients and by persistent symptoms (with or without synovitis) in 34 (68%). The pattern of CPPA in 20 (40%) patients was pseudo-osteoarthritis with synovitis, pseudo-osteoarthritis without synovitis in nine (18%), pseudogout in nine (18%), monoarthropathy in eight (16%) and pseudorheumatoid arthritis in four (8%). The phosphocalcium balance was altered in nine (18%) cases: six patients had hypercalciuria two hyperphosphaturia, two hypocalciuria, one hypophosphaturia and one hypercalcemia. Five patients had abnormal thyroid hormone levels, but only one presented with clinical hypothyroidism. Four patients showed increased parathormone levels, but only one presented with clinical hyperparathyroidism.  Radiographic findings showed that 43 (86%) patients had meniscus calcifications, 20 (40%) radiocarpal and 16 (32%) calcification of the symphysis pubis. The study confirms the clinical variability of the disease in a population of Portuguese patients. The knee meniscus calcifications were the most sensitive single finding for establishing the diagnosis of CPPA. Almost all our patients had sporadic idiopathic CPPA without associated pathological conditions. Received: 22 October 1999 / Accepted: 25 July 2000     

Inflammatory Arthritis in Patients With Myelodysplastic Syndromes: A Multicenter Retrospective Study and Literature Review of 68 Cases

We describe the characteristics and outcome of inflammatory arthritis in patients with myelodysplastic syndrome (MDS) in a French multicenter retrospective study.Twenty-two patients with MDS (median age, 77.5 yr [interquartile range, 69–81]; 10 women) were included. Inflammatory arthritis presented as polyarthritis in 17 cases (77%) and with symmetric involvement in 15 cases (68%). At diagnosis, the median disease activity score 28 based on C-reactive protein (DAS28-CRP) was 4.5 [2–6.5]. Two patients had anti-citrullinated protein antibodies (ACPAs), and 1 had radiologic erosions. The median time between the diagnoses of arthritis and MDS was 10 months [6–42], with a median articular symptom duration of 3 months [2–8]. The diagnosis of both diseases was concomitant in 6 cases (27%); arthritis preceded MDS in 12 cases (55%), and occurred after MDS in 4 (18%). While the number of swollen and tender joints significantly decreased during follow-up, as did the median DAS28-CRP (from 4.3 [3.8–4.6] at baseline to 2.9 [1.75–3.3]; p < 0.05), CRP remained elevated (CRP >20 mg/L) in 8 patients (42%). Nevertheless, radiographic progression and new ACPA positivity were not observed during a median follow-up of 29 months [9–76]. While most of the patients were treated with steroids (n = 16) for arthritis, additional treatment was administered in only 4 patients (hydroxychloroquine, n = 2; sulfasalazine [Salazopyrin] and etanercept, n = 1, respectively). Eleven patients died during follow-up from acute myeloid leukemia (n = 5); infections (n = 3); or cerebral bleeding, cardiorespiratory failure, or undetermined cause (n = 1, respectively).Inflammatory arthritis associated with MDS can have various presentations and is often seronegative and nonerosive. Steroids alone are the most common treatment in MDS-associated arthritis, but that treatment is insufficient to control arthritis. Steroid-sparing strategies need to be identified.     

Oral flecainide in the treatment of refractory arrhythmias. Long-term follow-up of 98 patients

Oral flecainide was administered to 98 patients with arrhythmias regarded as resistant to other antiarrhythmic agents: quinidines (82), propafenone (40), beta-blockers (30), amiodarone alone (38) or combined with a class I compound (19). Therapeutic effectiveness was assessed on clinical date, repeated Holter recordings (64 patients), exercise tests (8) and electrophysiological exploration (15). Mean follow-up was 11.7 +/- 11 months; the patients treated have now been followed up for 18.2 +/- 12 months (range: 7-58 months). Fifty-three patients had atrial arrhythmia (fibrillation or flutter in 45, atrial tachycardia in 8). Flecainide was effective in 26 patients (49%) and ineffective in 27 (51%). There was no significant difference in dosage between these 2 groups: 231 +/- 62 mg/day and 265 +/- 61 mg/day respectively. Paroxysms of re-entrant junctional tachycardia were controlled in 6 of the 8 cases observed. Eleven patients presented with Wolff-Parkinson-White syndrome: treatment was successful in the 3 patients with atrial fibrillation and in 8 of the 10 patients with orthodromic reciprocating rhythms. Among 30 patients with episodes of ventricular tachycardia, 9 (30%) responded to flecainide and 21 (70%) failed to respond. Flecainide reduced the repetitive forms by more than 90% in 7/15 patients and suppressed exercise-induced ventricular tachycardia in 2/8 patients. Fifteen out of 18 patients had ventricular tachycardia reproducible by programmed stimulation; under flecainide, the ventricular tachycardia spontaneously recurred in 4 cases, was provoked by stimulation in 5 other cases, was more easily inducible in 3 cases and was not inducible in a sustained manner in the last 3 cases.(ABSTRACT TRUNCATED AT 250 WORDS)     

"Ambulatory" ischemic colitis. Clinical course and etiologic features in 88 cases

From January 1983 to December 1988, 88 consecutive patients with ambulatory ischemic colitis without severe disease or surgical operation were observed. The diagnosis was established in all cases by endoscopy, and in 64 cases by histologic examination. The study included 55 women and 33 men with a mean age of 65.4 years (22-90). Symptoms included: abdominal pain (73 percent), diarrhea (60 percent) and bloody stools (85 percent). The sequence abdominal pain-diarrhea-bloody stools was noted in 41 percent of cases. Most cases of colitis were located in the sigmoid and left colon. The immediate course was uneventful in 81 patients. Six patients underwent surgery and of these, three died. The mortality rate was 4.5 percent (4 cases). Long-term outcome was known in 41 cases. None of the patients were symptomatic, but a moderate secondary stenosis was noted in 4 of 31 patients who underwent endoscopic or radiological follow-up examination. In 80 cases, one or more potentially etiological factors were observed including vascular obstruction (32 cases), low-flow states (27 cases), and drugs (78 cases). Twenty patients were taking non steroidal anti-inflammatory drugs (23 percent). Drug treatment was recent in 10 cases, and in four, it was the only possible etiological factor. This suggests the responsibility of anti-inflammatory drugs in the onset of certain cases of ischemic colitis.     

All-transretinoic acid followed by intensive chemotherapy gives a high complete remission rate and may prolong remissions in newly diagnosed acute promyelocytic leukemia: a pilot study on 26 cases.

We entered 26 patients with newly diagnosed acute promyelocytic leukemia (APL) in a pilot study of all-transretinoic acid (ATRA) followed by intensive chemotherapy. Median age was 46 (range 25 to 63). No patient presented with leukocytes > 10 x 10(9)/L or had the microgranular APL variant. Cytogenetic analysis (25 patients) found a t(15;17) in 24 cases. Patients were scheduled to receive ATRA (45 mg/m2/d) until complete remission, followed by an intensive daunorubicin (DNR) + Ara C course ("4 + 7" course), then three "2 + 5" DNR + Ara C courses and maintenance chemotheapy. However, the "4 + 7" course was administered in emergency if hyperleukocytosis rapidly developed to prevent leukostasis. Twenty-five patients (96%) achieved CR, 14 with ATRA alone and 11 after the addition of the "4 + 7" course on day 2 to 30 of treatment, because leukocytes rapidly increased (9 cases), because of resistance to ATRA (1 case), and development of organomegaly (1 case). The remaining patient died on day 6, from CNS bleeding. Apart from hyperleukocytosis, side effects were usually moderate. In the 11 patients who could be studied in vitro, a very good correlation was found between in vivo and vitro differentiation and proliferation of APL blasts with ATRA. Three patients were allografted after the "4 + 7" course. Four patients did not receive this course but received the subsequent "2 + 5" courses and maintenance. The remaining patients followed the scheduled protocol. Three patients relapsed after 8, 11, and 15 months (including one allografted patient). Two patients died in CR, after 6 and 17 months. The other 20 patients remained in CR after 18+ to 34+ months (median 21). Actuarial disease free interval (DFI) and event free survival (EFS) were 87% and 77%, respectively, after 18 months. These results were compared to those obtained in our previous APL 84 trial with chemotherapy alone in newly diagnosed APL (after excluding patients included in this trial who presented with hyperleukocytosis). In APL 84 trial, the CR rate was 76%, the actuarial DFI and EFS were 59% and 48% after 18 months, respectively. Differences with the pilot study of ATRA followed by chemotherapy were significant for DFI (P = .02), EFS (P = .006), but not for CR rate (P = .08). Although this is a historical comparison, these results suggest that ATRA followed by chemotherapy may prove superior to chemotherapy alone in newly diagnosed APL, by slightly increasing the CR rate, but perhaps more importantly by reducing the relapse rate.(ABSTRACT TRUNCATED AT 400 WORDS)