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At least 199 records · Page 11Linked to original sources
PubMed1985

Linkage of the alpha G Philadelphia locus to alpha-thalassemia in the Cuban population.

The inheritance of the alpha-chain hemoglobin variant G Philadelphia was studied in three Cuban families of African ancestry. The variant represented approximately 33% of the total adult hemoglobin in all subjects, and was associated to a 10.5 kb Bam HI restriction fragment. Mild hematological alterations were present. These data indicate that also in the Cuban population the alpha G Philadelphia locus is linked to a deletional alpha-thalassemia.

Adult↗
PubMed1979

Interaction between beta-thalassaemia and Hb G Philadelphia associated with alpha-thalassaemia.

A man who did not produce and beta-chains did not suffer from a severe beta-thalassaemia. He was heterozygous for Hb G Philadelphia. It has been suggested that this haemoglobin variant was associated with alpha-thalassaemia and that interaction between alpha-thalassaemia and beta-thalassaemia decreased the imbalance of alpha/beta-globin biosynthesis and thereby the severity of the beta-thalassaemic disorder. Association of Hb G Philadelphia and alpha-thalassaemia in this man and his family is now demonstrated using bone marrow and reticulocytes of the propositus and one of his sons and reticulocytes only of another son.

Bone Marrow↗
PubMed1975

The philadelphia pulmonary neoplasm research project. Thwarting factors in periodic screening for lung cancer.

This report describes the problems of the Philadelphia Pulmonary Neoplasms Research Project, begun in 1951, when 6,027 male volunteers 45 years of age and older were enrolled in a study designed to screen them every 6 months for 10 years with questionnaires and chest photofluorograms. These volunteers tended to be slightly younger and to include fewer nonwhites that the corresponding Philadelphia population. The volunteers included more smokers than older American urban men in general, but among smokers the distribution by smoking habits was similar. The average probability of having 2 consecutive semiannual screening examinations was 57 per cent. The risk of lung cancer was higher among noncompliant men than among compliant ones. During the 10-year period of observation, 121 men developed lung cancer; only 8 per cent survived 5 years. Aside from the screening method, the factors that thwarted early detection and surgery among the men with lung cancer were (1) the interval between a negative roentgenogram and detection of cancer, which exceeded 6 months in 45 per cent; (2) an interval of 6 months or less between a negative roentgenogram and detection of cancer in 20 per cent, with a delay of 3 months or more before appropriate action was taken; (3) advanced age (70+ years) in 25 per cent at the time of detection of cancer; (4) nonmalignant disease contraindicating surgery in 31 per cent. One or more of these factors was present in 84 per cent of the men with lung cancer, leaving only a small segment for whom improvements in the screening method would be expected to be of value.

Age Factors↗
PubMed1989

Evaluation questions for Philadelphia's capitation plan for mental health services.

Public policy should be guided by sound data emerging from quality research. A shift in the financing of medical and psychiatric care toward capitation models represents a major change, and the Philadelphia capitation experiment described in the April column is one pioneering effort. The answers to the following evaluation questions about the Philadelphia experiment will help decision makers across the country as they contemplate a policy shift from retrospective to prospective payment.

Capitation Fee↗
PubMed2003

Early prediction of response in patients with relapsed or refractory Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph+ALL) treated with imatinib.

Imatinib has pronounced but brief antileukemic activity in advanced Philadelphia chromosome-positive acute lymphoblastic leukemia (Ph(+)ALL). We assessed the prognostic impact of pretreatment disease features and the early bone marrow (BM) response in 68 consecutive patients with Ph(+)ALL receiving imatinib salvage therapy. A complete hematologic or marrow response was achieved by 92% of patients with BM blasts below 5% on day 14, whereas 62.5% of patients with more than 5% BM blasts on day 14 were nonresponders. Similarly, time to progression (TTP) was superior in patients with a good day 14 response (5.2 versus 0.9 months; P <.0001). Prior complete remission of less than 6 months, white blood cell count of more than 10 x 10(9)/L, circulating peripheral blood blasts at diagnosis, additional Philadelphia chromosomes, or at least 2 Bcr-Abl fusion signals were associated with significantly inferior remission rate and response duration. In patients without poor prognostic features, single-agent imatinib may be appropriate before transplant salvage therapy. Conversely, patients with clinically or cytogenetically defined poor-risk features are candidates for trials of upfront imatinib in combination with other agents.

Adolescent↗
PubMed2005

Fibrinogen Philadelphia, a hypodysfibrinogenemia characterized by abnormal polymerization and fibrinogen hypercatabolism due to gamma S378P mutation.

Fibrinogen Philadelphia, a hypodysfibrinogenemia described in a family with a history of bleeding, is characterized by prolonged thrombin time, abnormal fibrin polymerization, and increased catabolism of the abnormal fibrinogen. Turbidity studies of polymerization of purified fibrinogen under different ionic conditions reveal a reduced lag period and lower final turbidity, indicating more rapid initial polymerization and impaired lateral aggregation. Consistent with this, scanning and transmission electron microscopy show fibers with substantially lower average fiber diameters. DNA sequence analysis of the fibrinogen genes A, B, and G revealed a T>C transition in exon 9 resulting in a serine-to-proline substitution near the gamma chain C-terminus (S378P). The S378P mutation is associated with fibrinogen Philadelphia in this kindred and was not found in 10 controls. This region of the gamma chain is involved in fibrin polymerization, supporting this as the polymerization defect causing the mutation. Thus, this abnormal fibrinogen is characterized by 2 unique features: (1) abnormal polymerization probably due to a major defect in lateral aggregation and (2) hypercatabolism of the mutant protein. The location, nature, and unusual characteristics of this mutation may add to our understanding of fibrinogen protein interactions necessary for normal catabolism and fibrin formation.

Afibrinogenemia↗
PubMed1992

Control of growth and differentiation of Philadelphia chromosome-positive leukemia cells by tyrosine kinase inhibitors.

Herbimycin A, a selective inhibitor of tyrosine kinase activity, induced differentiation of leukemia cells isolated from Philadelphia chromosome-positive chronic myelogenous leukemia patients. However, it did not induce differentiation of leukemia cells from acute myelogenous leukemia patients, although these cells could be induced to differentiate by treatment with appropriate compounds. A selective inhibitor of tyrosine kinase might be useful in chemotherapy of Philadelphia chromosome-positive leukemia.

Antibiotics, Antineoplastic↗
PubMed1982

Homozygous alpha thalassemia/Hb G Philadelphia.

Microcytic red cells from a 70 year old Negro man with mild anemia contained only hemoglobin G-Philadelphia. Red cells from all of his children had low-normal MCV's, and contained 32-34 percent of the abnormal hemoglobin. Oxygen affinity of his blood and stability of his hemolysate were normal, suggesting that his mild anemia was not caused by the the abnormal hemoglobin. Restriction endonuclease analyses of DNA from the proband and his offspring showed that the alpha G-Philadelphia globin gene exists in only one copy per chromosome. The new gene was probably created by an unequal cross-over which deleted an alpha globin coding sequence (derived from one or both alpha globin genes), as well as some or all of the DNA sequence between those genes.

Aged↗
PubMed2003

Support for physician deception of insurance companies among a sample of Philadelphia residents.

BACKGROUND: Some physicians seem to be willing to sanction deception of insurance companies. Little is known about public attitudes regarding this practice. OBJECTIVE: To assess public attitudes regarding physician deception of insurance companies. DESIGN: Cross-sectional survey using clinical vignettes. SETTING: Philadelphia County Courthouse, Philadelphia, Pennsylvania. PARTICIPANTS: Convenience sample of 700 prospective jurors. MEASUREMENTS: Participants were asked whether, in response to restriction of health care, a physician should 1) accept restriction, 2) appeal restriction, or 3) misrepresent a patient's condition to obtain the desired service. The proportion of respondents reporting that the physician should misrepresent a patient's condition was determined. RESULTS: 26% of respondents sanctioned deception, 70% supported appealing, and 4% supported accepting the insurance company decision. Among the 27% of respondents believing physicians have inadequate time to appeal coverage decisions, 50% sanctioned deception. CONCLUSIONS: Sanctioning of deception was substantial in this sample of prospective jurors. Preferences regarding insurance company deception are related to perceptions of physician workload and may further pressure physicians struggling to balance advocacy with honesty.

Adult↗
PubMed2002

Managing musculoskeletal complaints with rehabilitation therapy: summary of the Philadelphia Panel evidence-based clinical practice guidelines on musculoskeletal rehabilitation interventions.

OBJECTIVE: The Philadelphia Panel recently formulated evidence-based guidelines for selected rehabilitation interventions in the management of low back, knee, neck, and shoulder pain. STUDY DESIGN: The guidelines were developed with the use of a 5-step process: define the intervention, collect evidence, synthesize results, make recommendations based on the research, and grade the strength of the recommendations. POPULATION: Outpatient adults with low back, knee, neck, or shoulder pain without vertebral disk involvement, scoliosis, cancer, or pulmonary, neurologic, cardiac, dermatologic, or psychiatric conditions were included in the review. OUTCOMES MEASURED: To prepare the data, systematic reviews were performed for low back, knee, neck, and shoulder pain. Therapeutic exercise, massage, transcutaneous electrical nerve stimulation, thermotherapy, ultrasound, electrical stimulation, and combinations of these therapies were included in the literature search. Studies were identified and analyzed based on study type, clinical significance, and statistical significance. CONCLUSIONS: The Philadelphia Panel guidelines recommend continued normal activity for acute, uncomplicated low back pain and therapeutic exercise for chronic, subacute, and postsurgical low back pain; transcutaneous electrical nerve stimulation and exercise for knee osteoarthritis; proprioceptive and therapeutic exercise for chronic neck pain; and the use of therapeutic ultrasound in the treatment of calcific tendonitis of the shoulder.

Acute Disease↗
PubMed2003

Approval summary: imatinib mesylate capsules for treatment of adult patients with newly diagnosed philadelphia chromosome-positive chronic myelogenous leukemia in chronic phase.

PURPOSE: The purpose is to describe the Food and Drug Administration (FDA) review and approval of imatinib (Gleevec; Novartis Pharmaceuticals, East Hanover, NJ) for treatment of adult patients with newly diagnosed Philadelphia chromosome-positive chronic myelogenous leukemia (CML) in chronic phase. EXPERIMENTAL DESIGN: The FDA reviewed data in electronic format from a randomized controlled clinical trial of 1106 adult patients with newly diagnosed Philadelphia chromosome-positive CML in chronic phase, comparing imatinib with the combination of IFN-alpha and cytarabine. RESULTS: Imatinib showed clinically and statistically significantly better results for time-to-progression to accelerated phase or blast crisis, progression-free survival, complete hematological response rate, and cytogenetic response rate. With a median follow-up of 14 months, a maximum follow-up of 19.5 months, and an expected median survival of 5-6 years on the IFN-alpha/cytarabine control arm, few of the expected progressions to accelerated or blast phase or deaths have occurred. Imatinib was also better tolerated. Edema, nausea, rigors, neutropenia, and headache were more frequent in women. Only 57% of the IFN-alpha target dose was administered, and only 68% of patients received any cytarabine. However, this does not appear to adequately explain the superiority of imatinib observed in this trial. Results of a population pharmacokinetic study in a subgroup of 371 patients and a separate rifampin-imatinib drug-drug interaction study in healthy volunteers are presented. CONCLUSIONS: On December 20, 2002, imatinib was granted accelerated approval under subpart H, rather than regular approval. Follow-up is short compared with the natural history of chronic phase CML or more mature results with established therapies such as IFN-alpha or transplantation. If imatinib should stop working after 1.5-2 years, the results could be importantly different from the present analysis. As a Phase IV postmarketing commitment, the applicant has agreed to provide follow-up reports on this imatinib study annually for the next 6 years.

Adult↗
PubMed1992

[Molecular analysis of chronic Philadelphia chromosome negative myeloid leukemia: study of 6 cases].

BACKGROUND: Patients with Philadelphia negative (Ph-) chronic myeloid leukemia (CML) constitute a small proportion of the total number of patients with CML. Molecular analysis of these cases has permitted recognition that some cases present a breakpoint in the bcr region of chromosome 22, that is, the alteration constituting the substrate of the Ph chromosome. To date, the number of patients analyzed to this regard is low. METHODS: Six patients with Ph negative CML who constituted part of a series of 96 patients diagnosed with CML over a period of 6 years were studied. Analysis of the BCR gene in the DNA of the leuko-concentrate of peripheral blood was carried out with the Southern Blot technique, using the 3' and 5' probes and Transprobe and the restriction enzymes Bgl II, Eco RI, Hind III and Bam HI. The principal clinical-hematological characteristics of the patients were analyzed. RESULTS: Breakpoints were observed in the bcr region of chromosome 22 in 3 of the 6 patients, all of whom presented typical CML clinical-hematological features. CONCLUSIONS: Half of the patients with Philadelphia negative (Ph-) chronic myeloid leukemia (CML) have a breakpoint in the bcr region of chromosome 22, a similar molecular pattern to the Ph positive CML and their clinical-hematological profile is indistinguishable from those with CML.

Adult↗
PubMed1991

Philadelphia-negative (Ph-) chronic myeloid leukemia (CML): comparison with Ph+ CML and chronic myelomonocytic leukemia. The Groupe Français de Cytogénétique Hématologique.

To better understand the Philadelphia-negative (Ph-) chronic myeloid leukemia (CML) and its relationships with Philadelphia-positive (Ph+) CML and chronic myelomonocytic leukemia (CMML), a study was undertaken by the Groupe Français de Cytogénétique Hématologique. Thirty-five Ph- CML patients were investigated and compared with 55 chronic phase Ph+ CML and 100 CMML patients. There were 12 M-BCR positive (M-BCR+) and 23 M-BCR negative (MBCR+) patients. No clinical or biologic differences were found between Ph+ and Ph-, M-BCR+ patients. In the Ph- group, M-BCR+ and M-BCR- patients differed significantly in age (47.7 +/- 6.6 v 67.0 +/- 6.1 years, respectively; P = .001), leukocytosis (153.4 +/- 135.1 v 58.5 +/- 37.7 10(9)/L, P = .002), relative monocytosis (1.8% +/- 1.2% v 5.6% +/- 1.4%, P = .048), absolute basophilia (8.5 +/- 9.7 v 0.9 +/- 1.5 10(9)/L, P = .001), percentage of immature myeloid precursors (IMP) in peripheral blood (29.0% +/- 9.5% v 15.3% +/- 8.1%, P = .001), and percentage of erythroblasts in bone marrow (BM) (6.5% +/- 3.5% v 14.6% +/- 3.6%, P = .001). Karyotypic abnormalities other than the Ph chromosome occurred in 0 of 12 M-BCR- at diagnosis and 7 of 23 M-BCR- Ph- CML (P = .033). None of the 13 investigated BCR- patients had detectable BCR/ABL transcripts using polymerase chain reaction (PCR) and none had an N-RAS mutation. Cytologic findings showed a marked morphologic difference between M-BCR+ and M-BCR- patients, especially in the monocytic lineage. Dysmyelopoietic features in CMML and M-BCR- patients were very similar, and the differences were of quantitative order only. Using four criteria (monocytosis, percentage of IMP, basophilia, and percentage of erythroblasts in BM), patients could be divided into typical and atypical CML and this classification correlated well with molecular findings. We conclude that, while Ph-, M-BCR+, and Ph+ CML are identical diseases, Ph-, M-BCR- CML, and CMML have many similarities and might be only different aspects of a same entity.

Adult↗
PubMed1977

The peripheral blood in chronic granulocytic leukaemia. Study of 50 untreated Philadelphia-positive cases.

The haematological findings in the peripheral blood of 50 patients in whom the diagnosis of chronic granulocytic leukaemia (CGL) had been made in the haematology laboratory, and who were subsequently shown to be Philadelphia-chromosome-positive, have been reviewed. The differential leucocyte counts were performed by 3 observers, examining a total of 1,500 cells in each patient. The degree of anaemia at diagnosis was unrelated to sex and correlated poorly with leucocyte count; thrombocytopenia seemed unrelated to leucocytosis. A differential luecocyte count which included a complete spectrum of granulocytic cells, with prominent peaks in the percentages of myelocytes and neutrophils, was an invariable finding. Absolute basophilia occurred in all patients and absolute eosinophilia in 92%. In 54% of the patients there was an absolute lymphocytosis. Unlike the finding in normal subjects, there was no linear relationship between the numbers of circulating neutrophils and monocytes. Application of these findings should improve the accuracy of the haematological diagnosis of CGL, while study of the rare cases which possess the above features but are Ph1-negative may throw further light on the role of the Philadelphia chromosome in the natural history of CGL.

Basophils↗
PubMed1977

Subacute myelocytic leukemia associated with the philadelphia chromosome and supplementary translocation : 9-12.

The authors report a case of subacute myelocytic leukemia presenting some severe aspects. The cytogenetic findings show the Philadelphia chromosome ; t (9-22) and a second translocation between the chromosome 12, and the other chromosome 9 : t (9-12). They think that this second translocation represents a supplementary cytogenetic argument for the isolation of "Subacute myeloid Leukemia with Philadelphia chromosome" within chronic myeloid Leukemia.

Chromosome Aberrations↗
PubMed1988

The Neurofibromatosis Clinic of the Children's Hospital of Philadelphia.

The Neurofibromatosis Clinic of the Children's Hospital of Philadelphia is a multidisciplinary clinic that provides a setting in which the diagnosis of neurofibromatosis can be made and follow-up of children with neurofibromatosis coordinated. It also offers genetic counseling to affected individuals and families. The Clinic acts as a referral center to the subspecialty disciplines when such specialty services are required. This report outlines the organization and operation of the Children's Hospital of Philadelphia Neurofibromatosis Clinic. It discusses the protocol used to evaluate the pediatric patient with neurofibromatosis and the importance of continuity of care for the patients and families.

Humans↗
PubMed1983

[Thrombocythemia with Philadelphia chromosome. Secondary appearance of chronic myeloid leukemia].

The finding of a Philadelphia chromosome in a case of apparently primary thrombocythaemia should change the diagnosis for that of chronic myeloid leukaemia. This theoretical view is supported by the case reported here, where a Philadelphia-chromosome was detected in the bone marrow cells of a patient with severe thrombocythaemia; a typical myeloid leukaemia developed 21 months after the onset of the disease; survival was of short duration.

Adult↗
PubMed1980

[The clinical significance of the Philadelphia chromosome in acute leukaemia (author's transl)].

So-called "Philadelphia-positive acute leukaemias" require varied therapeutic management. Additional chromosome anomalies together with Ph1 indicate an acute transformation of a Philadelphia-positive disease, requiring "non-myelotoxic" treatment and new therapeutic approaches. By contrast, "de novo" acute leukaemias with a Ph1-positive karyotype should be treated with intensive chemotherapy for the induction and maintainance of remission.

Acute Disease↗