High-dose therapy and autologous bone marrow transplantation in patients with follicular lymphoma during first remission

We report the results of a study in previously untreated advanced stage patients with follicular lymphoma (FL) who underwent uniform induction chemotherapy with cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) followed by myeloablative therapy and anti-B-cell monoclonal antibody purged autologous bone marrow transplantation (ABMT). Eighty-three patients with previously untreated, low-grade FL were enrolled. After CHOP induction, only 36% achieved complete remission (CR) and 77 patients underwent ABMT. Before BM harvest, 70 patients had a known t(14;18), as determined by polymerase chain reaction (PCR), and all remained PCR positive in the BM at harvest. After ABMT, the disease-free survival (DFS) and overall survival are estimated to be 63% and 89% at 3 years, respectively, with a median follow-up of 45 months. Patients whose BM was PCR negative after purging experienced significantly longer freedom from recurrence (FFR) than those whose BM remained PCR positive (P = .0006). Continued PCR negativity in follow-up BM samples was also strongly predictive of continued CR. This study suggests that a subset of patients with advanced FL may experience prolonged clinical and molecular remissions following high-dose ablative therapy, although longer follow-up will be necessary to determine potential impact on overall survival.     

New England Medical Center Posterior Circulation Stroke Registry: I. Methods, Data Base, Distribution of Brain Lesions, Stroke Mechanisms, and Outcomes

Among 407 New England Medical Center Posterior Circulation Registry (NEMC-PCR) patients, 59% had strokes without transient ischemic attacks (TIAs), 24% had TIAs before strokes, and 16% had only posterior circulation TIAs. Embolism was the commonest stroke mechanism accounting for 40% of cases (24% cardiac origin, 14% arterial origin, 2% had potential cardiac and arterial sources). In 32%, large artery occlusive lesions caused hemodynamic brain infarction. Stroke mechanisms in the posterior and anterior circulation are very similar. Infarcts most often included the distal posterior circulation territory (rostral brainstem, superior cerebellum and occipital and temporal lobes), while the proximal (medulla and posterior inferior cerebellum) and middle (pons and anterior inferior cerebellum) territories were equally involved. Infarcts that included the distal territory were twice as common as those that included the proximal or middle territories. Most distal territory infarcts were attributable to embolism. Thirty day mortality was low (3.6%). Embolic stroke mechanism, distal territory location, and basilar artery occlusive disease conveyed the worst prognosis.     

Biliary stricture dilatation: multicenter review of clinical management in 73 patients

Eighty-nine biliary strictures in 73 patients who had undergone percutaneous balloon dilatation were reviewed to determine long-term patency rates and clinical management problems. The majority of dilatations were performed in patients with anastomotic strictures (n = 44), iatrogenic strictures (n = 28), and strictures associated with sclerosing cholangitis (n = 17). Patency rates after 36 months or more were 67%, 76%, and 42%, respectively. Complications, mostly minor, occurred in less than 7% of patients. Of patients with significant biliary obstruction, 15% had little or no intrahepatic biliary duct dilatation demonstrated by cross-sectional imaging and/or direct cholangiography. No definite conclusions could be drawn about the utility of long-term internal/external stenting.     

用气相色谱-同位素比值质谱法测定体外肠道细菌发酵产生的短链脂肪酸

目的：了解肠道细菌发酵产生短链脂肪酸的情况，为乳糖不耐症状的发生提供依据。方法：在体外进行细菌培养，用稳定性同位素标记的葡萄糖作底物，用气相色谱－同位素比值质谱法测定短链脂肪酸。结果：细菌纯培养显示不同菌株产生的短链脂肪酸不同。人体粪便培养表明不同人体产生的短链脂肪酸的种类相同，而各种类数量不同。结论：所测定的人体肠道发酵产生短链脂肪酸的能力不同，可能与乳糖不耐症状的发生有关。     

Neurological and adrenal dysfunction in the adrenal insufficiency/alacrima/achalasia (3A) syndrome

Review of 20 patients with glucocorticoid deficiency (three cases also with salt loss) associated with absent tear secretion (19 cases) and achalasia of the cardia (15 cases) revealed neurological abnormalities in 17 including hyper-reflexia, muscle weakness, dysarthria, and ataxia together with impaired intelligence and abnormal autonomic function, particularly postural hypotension. These findings indicate that significant neurological problems are common in this multisystem disorder.     

New England Medical Center Posterior Circulation Stroke Registry II. Vascular Lesions

Among 407 New England Medical Center Posterior Circulation Registry (NEMC-PCR) patients, the extracranial (ECVA) and intracranial vertebral arteries (ICVA) were the commonest sites of severe occlusive disease followed by the basilar artery (BA). Severe occlusive lesions were found in >1 large artery in 148 patients; 134 had unilateral or bilateral severe disease at one arterial location. Single arterial site occlusive disease occurred most often in the ECVA (52 patients, 15 bilateral) followed by the ICVA (40 patients, 12 bilateral) and the BA (46 patients). Involvement of the ICVAs and the BA was very common and some patients also had ECVA lesions. Hypertension, smoking, and coronary and peripheral vascular disease were most prevalent in patients with extracranial disease while diabetes and hyperlipidemia were more common when occlusive lesions were only intracranial. Intra-arterial embolism was the most common mechanism of brain infarction in patients with ECVA and ICVA occlusive disease. ICVA occlusive lesions infrequently caused infarction limited to the proximal territory (medulla and posterior inferior cerebellum). BA lesions most often caused infarcts limited to the middle posterior circulation territory (pons and anterior inferior cerebellum). Posterior cerebral artery occlusive lesions were predominantly embolic. Penetrating artery disease caused mostly pontine and thalamic infarcts. Prognosis was poorest in patients with BA disease. The best prognosis surprisingly was in patients who had multiple arterial occlusive lesions; they often had position-sensitive transient ischemic attacks during months or years.     

Alaska Native Cancer epidemiology in the Arctic

Cancer incidence and its possible relation to environmental contaminants, including radiation, continues to be a perceived health threat for the arctic-dwelling Alaska Native (Inupiat Eskimo) people despite the lack of a direct link to high-dose exposure. To better understand this concern, all known malignancies diagnosed in this population (n = 177) in three consecutive eight-year periods (1971–1994) were evaluated.The most recent average incidence rate (age-adjusted to world standard population) of 315 per 100 000 (95% confidence interval, CI = 248–382) represents a 33% surge (albeit non-significant) in Alaska Native cancer incidence over the initial period studied. The male rate 366 (95% CI = 266–466) for the same period exceeds the female rate 258 (95% CI = 169–347) by 42%. Two patterns of cancer incidence are seen at the village level. One, a 24 y upward trend found in the villages of Barrow, Point Hope and Kaktovik (combined rate of increase significant [P = 0.047]) associated with lung cancer; and the other, a stable trend over the past 16 y, associated with colon and rectal cancer. Lung cancer is the predominant cancer by site and is primarily a male disease. The recent male lung cancer incidence rate of 137 (95% CI = 73–201) exceeds the female rate by greater than five times. Total lung cancer cases are primarily confined to four villages where the incidence significantly (P = 0.0043) exceeds the remaining population. The major female cancers are colon/rectal and breast with cancer of the cervix virtually eliminated. Breast cancer is found primarily in two villages where its excess is significant (P = 0.025).Inupiat Eskimo cancer epidemiology is unique, differing from both the Alaska Native and other Circumpolar populations. At present, this uniqueness cannot be explained by an overt environmental contaminant exposure. Although tobacco very likely plays a central role, it by itself cannot fully explain the extremely high male lung cancer rate and why only specific villages are affected. Genetic predisposition and environmental factors may play a synergistic role as cofactors. A cooperative investigative effort with the Inupiat population is indicated and may go a long way in reducing cancer concern in the region.