Fulminant brain lymphoid infiltration in a patient with chronic lymphocytic leukemia.
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Biomedical subjects
Publications and source records attributed to J Lucas.
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We describe a little-known variety of hip dysplasia, termed 'acetabular retroversion', in which the alignment of the mouth of the acetabulum does not face the normal anterolateral direction, but inclines more posterolaterally. The condition may be part of a complex dysplasia or a single entity. Other than its retroversion, the acetabulum is sited normally on the side wall of the pelvis, and its articular surface is of normal extent and configuration. The retroverted orientation may give rise to problems of impingement between the femoral neck and anterior acetabular edge. We define the clinical and radiological parameters and discuss pathological changes which may occur in the untreated condition. A technique of management is proposed.
The leukotriene receptor antagonist zafirlukast (Accolate; Zeneca Pharmaceuticals; Wilmington, Del) recently was approved for use as maintenance therapy for persistent asthma. This new product has been well received due to convenient dosing and relatively few side effects. Based on initial success with this product, it is likely that similar compounds will be available for use in the near future. In this report, a case is described of a 47-year-old white man with moderate persistent asthma in whom Churg-Strauss syndrome developed while he was receiving zafirlukast therapy. Acute respiratory insufficiency, arthralgia, and prominent rash developed which required hospitalization. The patient's symptoms rapidly reversed following discontinuation of zafirlukast therapy and administration of systemic corticosteroids. Although the incidence of Churg-Strauss syndrome associated with zafirlukast therapy is rare, this case report illustrates steps that may be taken to diagnose quickly and treat this life-threatening condition should it occur.
Several novel differentiated cell lines have been derived from a human hepatocarcinoma named HBG. Analysis of their functional properties evidenced a gradual differentiation process as they became confluent and a remarkable stability of the whole quiescent population for at least 6 weeks. However, when replated at low density after several weeks of quiescence, the differentiated cells were able to rapidly reverse to active proliferation, accompanied by transient dedifferentiation. Demonstration that the differentiated hepatic cells were growth-arrested in G1 phase was provided by the increased number of cells with 2C DNA content and decreased expression of S-phase markers. Characteristic features of oncogenes and cell cycle genes were defined during the differentiation process: (a) a biphasic expression of c-myc, with the latter wave covering the quiescence period; (b) opposite kinetics of c-Ki-ras and of N-ras expression with a pattern of changes paralleling that of c-myc; and (c) a decrease of cyclin D1 protein expression and of the cyclin D1-associated kinase activity. The mechanisms by which quiescent differentiated cells might reinitiate active proliferation were analyzed by studying several genes involved in cell growth and death regulation. We found: (a) a point mutation and loss of the specific activity of the tumor suppressor gene p53 without alteration of the apoptotic response to transforming growth factor beta1; (b) a gradual decrease of retinoblastoma protein, which was constantly present, mainly in a hyperphosphorylated form; and (c) an increase of cyclin-dependent kinase inhibitor p27 expression in confluent differentiating cells, as expected, whereas, surprisingly, a disappearance of the p21 protein was observed in parallel. These data may reflect specific mechanisms of cell cycle regulation in liver parenchymal cells through which these cells can proceed to control their reversible differentiation program.
BACKGROUND: We describe a new manual method of phased chest and abdominal compression-decompression with a Lifestick resuscitator for cardiopulmonary resuscitation (CPR). METHODS AND RESULTS: Ventricular fibrillation (VF) was induced in 20 domestic pigs. After either 5 or 7 minutes of untreated VF, either phased chest and abdominal compression-decompression (Lifestick resuscitator) or precordial compression was initiated. Defibrillation was attempted at 2 minutes after the start of CPR. For the animals in which VF was untreated for 7 minutes, epinephrine was administered in doses of 20 micrograms/kg at 2 minutes after start of CPR. The coronary perfusion pressure generated by the Lifestick resuscitator was more than twofold greater (P < .01) than that generated by conventional precordial compression. Of 5 control animals, none were resuscitated after 5 minutes of VF. However, each of 5 animals treated with the Lifestick resuscitator was resuscitated (P < .01) and survived after 48 hours (P < .01). When untreated VF was prolonged to 7 minutes and epinephrine was administered, only 2 of the 5 control animals were resuscitated, and none of them survived for more than 4 hours. However, each of the Lifestick-treated animals was resuscitated and survived for more than 48 hours (P < .01). CONCLUSIONS: Phased chest and abdominal compression-decompression substantially increased hemodynamic efficacy of CPR and outcome in terms of successful resuscitation, 48-hour survival, and cerebral recovery.
OBJECTIVE: Our goal was to evaluate the diagnostic significance of the presence and pattern of arterial hypervascularity in lesions detected on dual-phase helical CT in cirrhotic patients. MATERIALS AND METHODS: Fifty-eight lesions greater than 1 cm in size were prospectively identified in 26 patients with end-stage liver disease who had undergone dual-phase helical CT for preoperative liver transplantation evaluation. All 26 patients had diagnoses proven by histologic evaluation or by clinical criteria. All arterial phase scans were retrospectively reviewed and lesions were categorized for the presence and pattern of arterial hypervascularity. Radiologic findings were correlated with histopathologic data. RESULTS: Thirty-seven of the 58 lesions had hypervascular components on arterial phase scans. All 37 of these lesions were found to represent hepatocellular carcinoma (HCC) (positive predictive value, 100%). Of the 21 remaining hypovascular lesions, 17 were HCC and four were benign (positive predictive value, 81%). Of the nine patients in whom all lesions were hypovascular, six had HCC (positive predictive value, 66%). The value of the presence of arterial hypervascularity for diagnosing HCC was statistically significant (p < .05). However, the presence or absence of arterial hypervascularity and the specific enhancement pattern revealed by helical CT did not correlate with histologic grading. CONCLUSION: The presence of hypervascularity in hepatic masses found in cirrhotic patients is highly predictive of malignancy.
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Angiodysplasia is an often unrecognized cause of upper-gastrointestinal bleeding, most commonly found in the antrum of the stomach but also in the duodenum and rarely in the esophagus. Small-intestinal angiodysplasia is the source of gastrointestinal bleeding of obscure origin in 30-40% of cases. The diagnosis is usually made by esophagogastroduodenoscopy, push enteroscopy, or selective angiography. We report the first case of angiodysplasia of the minor papilla diagnosed by side-viewing duodenoscopy.
The results of lobar resection to treat severe localised bronchiectasis in six children with cystic fibrosis are described. Sustained clinical improvements occurred in children undergoing this surgical approach to treatment. Detailed assessment and intensive preoperative and postoperative medical treatment are essential to a favourable outcome in carefully selected patients.
Cytogenetic analysis of a new human hepatoma cell line BC2 was performed with conventional cytogenetic techniques and fluorescent in situ hybridization. Numerical and structural abnormalities were observed by conventional cytogenetics for chromosomes 1, 2, 4, 7, 8, 9, 10, 11, 15, 17 and 20. Chromosome painting allowed to specify the translocation of chromosome 1, and to characterize 3 markers from chromosome 8 and one marker from 9, which were unrecognizable by conventional techniques. Comparison of chromosome 1 abnormalities with those reported in the literature for other human hepatoma cell lines showed that structural abnormalities of chromosome 1 were present in different regions of this chromosome. A review of the literature was done, and the results discussed, suggesting that alterations of chromosome 1 may be important in hepatocarcinogenesis.
BBB syndrome and G syndrome were originally reported as distinct X-linked disorders. Clinical studies indicated that BBB and G syndromes were likely to represent variant expression of the same disorder, now referred to as "Opitz" GBBB syndrome. Several occurrences of male-to-male transmission in both syndromes led to the hypothesis that GBBB syndrome was a single autosomal dominant, sex influenced disorder, now tentatively mapped to 5p12-13. We report on a large pedigree in which GBBB syndrome appears to cosegregate with a pericentric inversion of the X chromosome inv(X)(p22.3q26). It indicates the possible existence of a true X-linked form of GBBB syndrome, which does not appear phenotypically different from its autosomal counterpart. The gene could map in the vicinity of the breakpoints, in Xp or Xq. The existence of two genes affecting a common pathogenetic pathway could explain the gender-dependent expressivity of GBBB phenotype.
Platelet activating factor (PAF)-stimulation of human B-lymphoblastoid cells results in the activation of microtubule associated protein 2-kinase (MAPK) and increases in intracellular calcium. Although increases in intracellular calcium induce MAPK activation in these cells, PAF can stimulate MAPK activation in the absence of detectable changes in intracellular calcium concentrations ([Ca2+]i). Treatment of the LA350 B-lymphoblastoid cell line with either pertussis toxin (PT) or cholera toxin (CT) blocked PAF-induced changes in [Ca2+]i. However, only PT blocked PAF-induced activation of MAPK as determined by shifts in the mobility of MAPK on immunoblots. In support of this finding, only PT but not CT blocked PAF-induced phosphorylation and activation of p90rsk, an event thought to be distal to MAPK activation. These results suggest that the PAF receptor is mediating MAPK activation through pathways separate from those mediating increases in intracellular calcium.
OBJECTIVE: Jumper's knee, or chronic patellar tendinitis, can be a source of considerable pain in athletes. The changes that occur with shearing of the tendinous fibers from repeated microtrauma can progress to significant degeneration and increase the risk of tendon rupture. In order to better understand this phenomenon, a correlative study relating the MR imaging and pathologic findings was performed. SUBJECTS AND METHODS: Nine high-performance athletes 18-22 years old (mean age, 20 years) underwent operation of 11 knees for long-standing jumper's knee. The average period of symptoms was 3 years (range, 2 to 6 years). MR knee examinations were performed before surgery in all athletes. The symptoms, MR findings, and pathological findings were correlated. RESULTS: There was focal thickening in the proximal one third of the patellar tendon (range, 9-16 mm; mean, 12 mm) in all 11 knees, involving the medial portion of the tendon in 10 and the center in one. On proton-density-weighted MR images, all knees demonstrated a focus of abnormal signal intensity in the proximal one third of the patellar tendon. On T2-weighted MR images, 10 knees demonstrated abnormal signal intensity; eight were isointense to that seen on proton-density-weighted images, and two were relatively hyperintense. Ten tendons demonstrated a poorly defined posterior margin. Pathologically, the areas of abnormal signal intensity corresponded to tissue containing tenocyte hyperplasia, prominent angiogenesis with endothelial hyperplasia, loss of longitudinal collagenous architecture, and microtears with collagen fiber separation. Hyaline degeneration was present in specimens from every patient. CONCLUSION: In athletes with chronic patellar tendinitis, areas of abnormal signal intensity on MR imaging corresponded to degenerative pathologic changes consistent with angiofibroblastic tendinosis. In nearly all patients, the tendon thickening occurred eccentrically. Disproportionate medial tendon thickening may be related to unequal tensile forces across the knee joint, resulting in greater stress on the medial portion of the extensor mechanism of the knee.
This article describes a model for incorporating outcomes measurement into a comprehensive, continuum-wide clinical practice improvement program developed at Lovelace Health Systems (Albuquerque, N.M.). Created in 1993, the Lovelace Episode of Care Program currently includes nine disease-specific multidisciplinary teams that are working to improve care by addressing such issues as severity, appropriateness, efficiency, continuous quality improvement indicators, practice guidelines, care maps, and outcomes. Key features and advantages of the model are discussed, as well as implementation issues, successes, lessons learned, program restructuring and improvement, and applicability of the framework across diseases and health organizations.
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PURPOSE: To discover the prevalence of antiphospholipid antibodies in women with a history of repeated miscarriages. Therapy evaluation of aspirin and prednisone to achieve living term foetus from women in whom antiphospholipid antibodies had been detected and who had become pregnant again. PATIENTS AND METHODS: 82 consecutive women with history of at least of two miscarriages, with no live foetus in either case. 14 women positive for antiphospholipid antibodies who had become pregnant again (group of therapy evaluation), were treated with aspirin (125 mg/d) and prednisone (20 mg/d). 60 normal pregnant women (control group). Lupus anticoagulant evaluation: APTT tests caolin clotting time and APTT system neutralized with platelet phospholipids. Antiphospholipid antibodies: ELISA test. RESULTS: 13.4% were positive to antiphospholipid antibodies (ELISA), and 2.4% were positive in both positive in both test. Therapy evaluation: 75.5% (11 cases) of pregnancies were successful (normal foetus), 14% (2 cases) of fetal losses, and 7% (1 case) of foetal malformations (acranius). CONCLUSIONS: In our study the antiphospholipid antibodies represent one of the main causes of repeated foetal losses. We recommended in cases of infertile women to test for antiphospholipid antibodies regularly. We confirm the effectiveness of the therapy (aspirin and prednisone) in achieving successful pregnancies in patients with a history of infertility.
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The distribution of neuregulin transcripts in rat brains was studied by both RNA blotting and in situ hybridization. Our data demonstrate that multiple neuregulin transcripts are expressed in neurons of the basal forebrain, the hippocampus, the diencephalon, the cerebellum, the brainstem, and the spinal cord. Developmental changes in the distribution of neuregulin transcripts were observed only in the cerebellum and the hippocampus. The intense neuregulin hybridization signals in the brainstem motor and sensory nuclei and the spinal motor neurons are suggestive of a functional involvement of neuregulins in motor and sensory systems. The expression of neuregulins in other parts of the brain also indicates that these factors are involved in a variety of central nervous system functions.