Successful use of an internal biliary stent in Caroli's disease.
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Bile Ducts, Intrahepatic↗
Biomedical subjects
B Stark
Publications and source records attributed to B Stark.
Involvement of 11p15 and 3q21q26 in therapy-related myeloid leukemia (t-ML) in children. Case reports and review of the literature.
The cytogenetic findings of therapy-related myeloid leukemia (t-ML) in three children are presented. These included one male patient with acute lymphoblastic leukemia (ALL) who underwent bone marrow transplantation and developed therapy-related myeloproliferative disease (t-MPD) in the female-donor hematopoietic cells 2.5 years after receiving radiation and epipodophyllotoxin therapy for ALL testicular relapse. Bone marrow leukemic cell karyotype revealed 46,XX,add (11)(p15) and a normal female karyotype in the peripheral blood lymphocytes. The other two children, one with ALL and one with ganglioneuroblastoma, developed fatal t-MPD and therapy-related acute myeloblastic leukemia (t-AML) preceded by myelodysplastic syndrome (t-MDS), respectively, 5 years after diagnosis, following administration of alkylating agents and irradiation. Monosomy 7 was present in both, and was combined with inv(3)(q21q26) in the second patient. Our review of the cytogenetic findings in 91 previously reported pediatric patients with t-ML suggested that the involvement of 11p15 and 3q21-->23, 3q24-q26 with or without a combination of translocation 11q23 and -7/7q-, respectively, are nonrandom aberrations of t-ML in children. Comparison of the chromosomal changes in t-ML between the pediatric and an adult series revealed some differences which may result from differences in treatment modalities and which, in addition, may indicate a possible role of genetic and/or age-dependent factors in the pathogenesis of therapy-related leukemogenesis in children.
Antineoplastic Agents↗
The reluctance of house staff to perform mouth-to-mouth resuscitation in the inpatient setting: what are the considerations?
OBJECTIVE: Medical house staff are required to perform cardiopulmonary resuscitation (CPR) as part of their job responsibilities. Previously it has been shown that house staff are reluctant to perform mouth-to-mouth resuscitation (MMR) in an out of hospital setting. Therefore, whether reluctance to perform MMR extends to the inpatient setting, and, if so, the reasons for this reluctance were investigated. DESIGN: All 74 internal medicine house officers of a large metropolitan hospital responded to presentations of hypothetical inpatient cardiac arrest scenarios to assess their willingness to perform MMR. SETTING: A 1200 bed university-affiliated teaching hospital in Los Angeles, California. SUBJECTS: All categorical internal medicine house officers at this hospital. INTERVENTIONS: This study is a survey which concerns whether the house officer would perform mouth-to-mouth resuscitation in different hypothetical cardiac arrest scenarios. RESULTS: Forty-five percent would perform MMR on an unknown patient and 39% would perform MMR in the elderly patient scenario. Only 16% would do MMR on a patient with a small amount of blood on his lips and only 7% would perform MMR on a patient with presumed acquired immunodeficiency syndrome. Medical housestaff were much more reluctant to perform MMR on elderly, trauma, or presumed immunodeficient patients in an inpatient setting than in an outpatient setting. All house staff that indicated their unwillingness to perform MMR cited fear of human immunodeficiency virus infection as their reason. CONCLUSION: Medical housestaff are quite reluctant to perform MMR in an inpatient setting. Thus, educating the medical house staff about the percent of patients that survive inpatient cardiac arrest and the actual risks of contracting infectious diseases, especially HIV infections, from MMR and preventative measures, such as effective barrier masks, should result in an increased willingness of physicians to perform MMR or mouth-to-mask ventilation on inpatients.
Acquired Immunodeficiency Syndrome↗
Chronic childhood idiopathic thrombocytopenia purpura: long-term follow-up.
An understanding of the natural history of childhood chronic idiopathic thrombocytopenia purpura (ITP) could contribute to a rational therapeutic approach to its treatment, which remains controversial. In our retrospective study of 92 children with ITP, 22 had a chronic course and were followed for 3-14 years (median 8.6 years). Treatment, when indicated, was individualized: 4 patients (18.2%) did not receive any treatment, 14 (63.6%) received steroids only, while 4 (18.2%) were treated with steroids and one of the following: high-dose gamma globulin (4 patients), splenectomy (2 patients) or immunosuppressive therapy (2 patients). During follow-up, 14 patients (63.6%) achieved complete remission, 5 (22.7%) partial remission and only 3 (13.5%) remained severely thrombocytopenic, with minimal bleeding tendency. Eleven patients (50%) responded to the initial prednisone course (1-5 mg/kg/day), but showed a marked decrease in platelet count when steroids were tapered off. In view of the high rates of complete and partial remission and the mild course of the few non-responding patients, it is suggested that with adequate supportive therapy, follow-up problems and fatalities can be kept to a minimum. We believe that aggressive therapy, such as splenectomy, should be reserved for the rare symptomatic and severely thrombocytopenic patient.
Child↗
[Chronic immune thrombocytopenic purpura in children].
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Child↗
Pancreatic cysts: a new dimension?
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Drainage↗
Osteosynthesis of the injured chest wall. Use of the AO (Arbeitsgemeinschaft für Osteosynthese) technique.
Open reduction and osteosynthesis with AO (Arbeitsgemeinschaft für Osteosynthese) technique, using 3.5 mm reconstruction plates and 3.5 mm cancellous screws, were performed in all cases of chest wall injury considered for surgical stabilization since 1990, viz. 11 with posttraumatic flail chest and one with painful nonunion of two ribs. In the ten survivors with flail chest, stability was achieved without secondary dislocation, giving good pain relief, improved respiratory mechanics and reduced duration of ventilatory support and intensive care requirements. Lasting pain relief was obtained also in the case of costal nonunion. No complications related to the osteosynthesis arose during follow-up for a mean of 11 months. Chest wall injuries in flail chest and painful nonunion of ribs can be easily and efficiently stabilized with the AO technique.
Adult↗
Treatment of exophthalmos by orbital fat removal.
Decompression of the orbit by removal of fat is a safe method to obtain functional and aesthetic improvement in exophthalmos disease. In comparison with traditional orbital wall decompression, the complication rate is low.
Adipose Tissue↗
T-cell acute lymphoblastic leukemia following therapy of rhabdomyosarcoma.
Multiple studies have documented an increased risk of secondary malignancies in patients receiving alkylating agents. Secondary leukemia following chemotherapy accounts for about 20% of all secondary neoplasms; most are acute nonlymphocytic. Secondary acute lymphoblastic leukemia has rarely been reported in either adult or childhood cancer. We report the development of acute T-cell lymphoblastic leukemia in a child following successful treatment of a paravertebral embryonal rhabdomyosarcoma (ERS). Southern blot analysis of DNA extracted from the T-cell lymphoblasts, using probes homologous to loci on the short arm of chromosome 11; P-calcitonin, P40.1 and H-ras, did not demonstrate the chromosomal loss of heterozygosity (LOH), a common feature of embryonal rhabdomyosarcoma. The data presented support the assumption that de novo leukemia emerged following treatment of the primary malignancy.
DNA, Neoplasm↗
Recurrent peripheral arterial occlusion by leukemic cells sedimentation in acute promyelocytic leukemia.
A 10-year-old boy presenting with recurrent successive events of peripheral arterial occlusion in both legs, more prominent on the left side, was diagnosed as having acute promyelocytic leukemia. Histological examination of the occluding material showed that it was composed of leukemic blast cells. The boy underwent five operations. Each time the arterial flow was reestablished by removing cylindrical hollow white material obstructing the lumen. However, after the last operation, irreversible ischemia developed, necessitating an above-knee amputation. There was no evidence of disseminated intravascular coagulation. Three months later a right lumbar sympathectomy was performed for relief of causalgic-ischemic pains, with successful results. Preoperative angiography demonstrated irregular narrowing of the right iliac arteries and tibioperoneal trunk stenosis.
Amputation, Surgical↗
Thymic humoral factor-gamma 2, an immunoregulatory peptide, enhances human hematopoietic progenitor cell growth.
Thymus humoral factor-gamma 2 (THF gamma 2), an octapeptide important for T-lymphocyte regulation, was assessed for its effect on the in vitro growth of human hematopoietic progenitor cells. This was achieved using a recombinant granulocyte-macrophage colony-stimulating factor (rGM-CSF)-stimulated myeloid cell colony formation (granulocyte-macrophage colony-forming cells, GM-CFC) assay as well as a recombinant erythropoietin (rEpo)-stimulated erythroid burst formation (erythroid burst-forming units, BFU-E) assay. Cells were obtained from bone marrow (BM) and peripheral blood (PB) of normal healthy donors and from patients with suppressed bone marrows. The latter group included aplastic anemia, leukemia, and lymphoma patients and patients with solid tumors who responded to intensive chemotherapy with significant pancytopenia. THF gamma 2 significantly enhanced normal BM and PB GM-CFC and PB BFU-E by 2- to 2.5-fold. This effect was totally dependent on the presence of the respective growth factors, that is, rGM-CSF or rEpo, and was specifically reversed by an anti-THF gamma 2 antiserum. Furthermore, although THF gamma 2-induced enhancement of GM-CFC colony formation was not affected by lymphocyte or monocyte depletion, the augmenting effect of the peptide on BFU-E was completely abrogated in the absence of lymphocytes. THF gamma 2-induced augmented growth of progenitor cells derived from severely suppressed marrows was minimal. However, cells from moderately neutropenic patients with leukemia in remission or with lymphoma under chemotherapy responded to the peptide similarly to cells from normal donors. These results suggest a stimulatory role for THF gamma 2 on human myeloid and erythroid hematopoietic progenitor cells. They also suggest the lymphocyte dependence of BFU-E enhancement and lymphocyte independence of GM-CFC stimulation by THF gamma 2. In the former case the thymus-derived peptide may act through the induction of certain erythroid-enhancing lymphokines.
Adjuvants, Immunologic↗
Fungemia with Torulopsis glabrata after endoscopic biliary stent replacement.
We report a patient with disseminated cholangiocarcinoma who developed systemic fungemia subsequent to endoscopic retrograde cholangiopancreatography (ERCP) and stent replacement. Despite intensive systemic antifungal therapy with iv amphotericin B, the patient developed multiple complications and eventually succumbed to her underlying disease. Cultures of the removed stent and blood were positive for the same organism, Torulopsis glabrata. In an immunocompromised host during ERCP manipulation, fungal pathogens should be considered in post-stent septicemia.
Aged↗
Special report. Integrating managed care. Continuity of care: integrating the components.
Although illnesses may attack specific parts of the body, treating anything less than the whole man can lead to serious problems. Catastrophic illnesses, for example, are often accompanied by depression. Employees being treated for alcohol or substance abuse may also be suffering from psychiatric disorders. Irrespective of which came first, the alcoholism or the depression, the conditions are intertwined. And their treatments need to complement one another. As companies contract with increasing numbers of specialty providers in an attempt to manage health care costs and utilization, communication among vendors can break down. Treatments may be repeated. Therapies can contradict one another. Employees sometimes fall through the cracks. What's needed is care that is comprehensive and continuous. The following series of stories examines the positive changes that may accrue when managed care techniques are applied in a synoptic fashion.
Continuity of Patient Care↗
[Israel national childhood acute lymphoblastic leukemia study].
A national childhood acute lymphoblastic leukemia (ALL) study was initiated in Israel in 1984 with the aim of improving results in difficult aspects of treatment including: high-risk groups, the problems of late relapses, and the effect of cranial irradiation for CNS prophylaxis in leading to late neuropsychiatric sequelae and secondary tumors. Induction of chemotherapy with a combination of 6 drugs (vincristine, cyclophosphamide, cytosine arabinoside, adriamycin, prednisone and L-asparaginase), followed by intensification with methotrexate and L-asparaginase, was introduced in both the usual and the high-risk groups. In a selected group with better prognostic factors, therapy was reduced. In an attempt to minimize the sequelae of CNS prophylactic therapy, cranial irradiation was omitted in half the patients and intrathecal (IT) triple therapy was given instead. Following 2 years of unsatisfactory preliminary results in a very high-risk group (VHR; non-T- and T-cell leukemia with WBC counts of greater than 100,000 and greater than 20,000, respectively), treatment was modified and intensified. Between Nov. 1984 and Feb. 1989, 143 patients were enrolled from 10 hospitals. During follow-up of a median of 2.5 years, there were 32 failures (2 failed remissions, 27 relapsed and 3 died of bleeding and sepsis). 107 patients are alive in first remission and an additional 8 in second and third remissions. By Kaplan-Meier life table analysis, the rates of leukemia-free interval (LFI) and event-free interval (EFI) for 4 years were 60% and 57%, respectively. Improved LFI results of 71% for 4 years were achieved in a group with non-T-cell ALL with WBC less than 100,000 (the largest group, 65% of the patients). In the small "good risk" group (10% of patients), and the T-cell group with WBC less than 100,000, LFI for 4 years were 56% and 54%, respectively. In the VHR group, modification seemed to have improved results: LFI of 41% for 3 years. CNS prophylaxis with IT triple therapy was as effective as cranial irradiation in the standard risk group. In 1 out of 33 children on this protocol a single CNS relapse occurred, as compared to 2 out of 35 matched controls with cranial irradiation. These results warrant extension of IT triple therapy to higher risk groups of childhood ALL. As for systemic treatment, increased up-front high-dose intensive therapy is recommended for all groups with ALL, but with reduction of cumulative dose to minimize late effects.
Antineoplastic Combined Chemotherapy Protocols↗
Adenocarcinoma of the pancreas producing pancreatitis and pancreatic abscess.
Pancreatic abscess is a severe complication of pancreatitis usually caused by alcohol, gallstones, abdominal trauma, or prior operative procedures. Pancreatic cancer is a rare cause of acute pancreatitis and an extremely rare cause of pancreatic abscess. We report three patients with pancreatic abscess caused by cancer who experienced a prolonged, complicated course with delay in diagnosis and substantial morbidity.
Abscess↗
Methotrexate-induced leukoencephalopathy is treatable with high-dose folinic acid: a case report and analysis of the literature.
An episode of leukoencephalopathy is reported in a 13-year-old girl who, after standard radiotherapy for a posterior fossa medulloblastoma, received 8 treatments with a protocol containing a 4-hour infusion of 500 mg/m2 methotrexate and 12 mg intrathecal methotrexate. The leukoencephalopathy, documented clinically and by CT and EEG, cleared after 2350 mg of leucovorin (citrovorum factor, folinic acid) was given in addition to the 135 mg given as part of the therapy. A review of the literature suggests that leukoencephalopathy may be prevented by high doses of leucovorin and can be treated by high doses, if lower doses were used initially. When high dose leucovorin was not used, residual neurological damage is not unusual.
Adolescent↗
Biologic and cytogenetic characteristics of leukemia in infants.
Clinical features, leukemic cell characterization, chromosomal findings, and treatment outcome were analyzed in a retrospective study of 30 cases with acute leukemia of infancy, 24 infants with acute lymphoblastic leukemia (ALL), and six cases with acute nonlymphoblastic leukemia (ANLL). Extensive bulky disease with organomegaly, central nervous system (CNS), and skin involvement were prominent features at diagnosis with a higher frequency in ANLL as compared to ALL. Four of six ANLL patients were classified as monocytic or myelomonocytic. In the ALL group nine of 24 (36%) were non-L1 morphology and six of 17 (33%) were common ALL antigen (CALLA) negative, the majority of them (five of six) were included in the non-L1 group. Immunophenotyping revealed four cases with early B-cell (three patients: Ia+B4+, and one patient: Ia+) and two cases with T-cell. Mixed lineage leukemia was found in five infants. Heavy chain immunoglobulin gene rearrangement was present in six cases tested, two CALLA+, two with Ia+B4+, and two were undifferentiated mixed lineage leukemia. Chromosomal aberrations were detected in ten of 18 patients, mostly in ANLL and CALLA negative ALL. Translocations were detected in six patients, involving 4q21-23 and 11q23 in three and two cases, respectively. The probability of five-year DFS were 27% for the whole group. The worst prognosis was observed in infants younger than 6 months of age, in whom the leukemia cell characteristics was compatible with stem cell: ANLL, very early pre-B, or undifferentiated mixed type. The chromosomal aberrations found in all cases included translocation with the seemingly nonrandom breakpoints at 4q21 and 11q23, and breakpoints that corresponded to known fragile sites. This finding may be suggestive of an underlying genetic predisposition associated with the poor prognosis of leukemia of infancy.
Female↗
Long-term proliferation of human leukemia cells induced by mouse stroma.
Acute lymphocytic leukemias (ALL) of infants and children were found to preferentially survive in coculture with a cloned cell line of endothelial adipose cells (14F1.1) from mouse bone marrow. One of these ALLs expressed a phenotype compatible with an early stage of differentiation (HLA-DR+, CD19+, and CD34+) and exhibited extensive growth in the presence of the mouse stromal cells during a period greater than 25 weeks following seeding. These ALL cells were strictly dependent upon the mouse stromal clone 14F1.1 and failed to proliferate in the absence of the endothelial adipocytes or with a variety of "feeder cells." Throughout the culture period the cells died if removed from the stroma. No similarly proliferative cell population with strict dependence upon stromal cells was found among a variety of other leukemias including hairy cell, acute myeloid, and chronic lymphocytic leukemia. The 14F1.1 clone has been previously found to promote the renewal of mouse and human stem cells. It is therefore possible that leukemias with a stem cell-like phenotype depend upon stromal cell factors similar to those affecting the growth of normal stem cells. These factors appear to operate across genetic barriers.
Antibodies, Monoclonal↗