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Biomedical subjects

M Kletzel

Publications and source records attributed to M Kletzel.

At least 55 records · Page 3Linked to original sources
PubMed1997

Red cell depletion of umbilical cord blood (UCB): comparison between unmanipulated and red cell-depleted UCB by Ficoll-Paque density gradient separation.

Stem cells from umbilical cord blood (UCB) represent an alternative source of cells for clinical transplantation. Many issues remain unsolved with regard to their collection, manipulation, and storage. In this study, we attempted to compare the effect of red cell depletion by Ficoll-Paque density gradient separation versus unmanipulated cord blood stem cells postthawing. We found no statistical difference between the two technologies when comparing viability, 98.6 +/- 0.3% versus 99.1 +/- 0.42% (p = < 0.16); CD34+/CD38+, 1.6 +/- 0.13% versus 1.2 +/- 0.17% (p = < 0.13); HLA DR+/CD34+, 1.8 +/- 0.15% versus 1.9 +/- 0.21% (p = < 0.6); blast colonies, 8.0 +/- 1.5 versus 12.2 +/- 2.1 (p = < 0.15); CFU-GEMM colonies, 143.7 +/- 27.9 versus 80.7 (p = < 0.10); CFU-GM colonies, 101.2 +/- 23 versus 173 +/- 23.2 (p = < 0.07). There was a statistical difference in the content of CD34+/CD38+, 1.6 +/- 0.14% versus 1.2 +/- 0.14% (p = < 0.05), and the BFU-E colonies, 96 +/- 29.8 versus 165 +/- 23.9 (p = < 0.03). We conclude that red cell depletion using Ficoll-Paque gradient separation preserves viability of progenitor cells, as evidenced by immunophenotyping and colony assays.

ADP-ribosyl Cyclase↗
PubMed1996

Nephrotic syndrome accompanying familial hemophagocytic syndrome.

PURPOSE: We describe the first reported case of familial hemophagocytic syndrome (FHS) with concurrent minimal change nephrotic syndrome (MCNS). PATIENTS AND METHODS: This is a case report of a 30-month-old girl who presented to Children's Memorial Hospital with pancytopenia and heavy proteinuria. RESULTS: This patient presented with anemia, neutropenia, thrombocytopenia, hypertriglyceridemia, and proteinuria. A brother died at 2 months of age with similar findings. A bone marrow biopsy demonstrated histiocyte proliferation with marked erythrophagocytosis, consistent with FHS. Treatment was begun with corticosteroids and VP-16. The patient developed worsening peripheral edema and hypoalbuminemia, with heavy proteinuria. After 1 month of therapy with persistence of heavy proteinuria, a renal biopsy was performed, the results of which were consistent with MCNS. CONCLUSION: This is the first reported case of FHS with coincident MCNS.

Child, Preschool↗
PubMed1994

Intraspinal Wilms' tumor metastases.

BACKGROUND: Intraspinal Wilms' tumor metastasis is rare, and is associated with a high mortality rate. METHODS: The authors reviewed the clinical course of two patients with Wilms' tumor in whom extradural metastasis developed. In addition, a review of the literature and of patients entered in the National Wilms' Tumor Studies was performed to determine the clinical presentation, treatment, and outcome of other patients with Wilms' tumor with intraspinal metastases. RESULTS: Both of the patients initially had abdominal pain without neurologic deficits. Despite therapy, paraplegia secondary to cord compression from recurrent epidural metastases developed in one patient, although a third remission has been achieved with further chemotherapy. the second patient remains in disease-free remission 25+ months after surgical resection of the extradural spinal tumor, adjuvant chemotherapy and radiation therapy, and autologous bone marrow transplantation. Review of the literature and of the patients entered in the National Wilms' Tumor Studies revealed an additional 27 patients with Wilms' tumor with this pattern of metastasis. Only four were disease-free at the time of this report. CONCLUSIONS: The authors' experience stresses the importance of early recognition and treatment of this complication of Wilms' tumor and demonstrates that intensive multimodality therapy can result in long-term disease-free remission.

Child↗
PubMed1994

Pericentric inversion (2)(p15q35) in an alveolar rhabdomyosarcoma.

We report a 7-year-old girl with a pericentric inversion of chromosome 2, inv(2)(p15q35), in a "solid variant" of alveolar rhabdomyosarcoma. The breakpoint in the long arm of chromosome 2 at band q35 is, at the cytogenetic level, identical to the breakpoint observed in the well-established reciprocal t(2;13)(q35;q14) associated with the alveolar subtype of rhabdomyosarcoma. In this case, however, no reciprocal translocation has occurred with chromosome 13 or any other chromosome, suggesting that the single critical breakpoint in alveolar rhabdomyosarcoma may be located at 2q35.

Child↗
PubMed1994

Cutaneous vesicostomy with direct intravesical application of formalin: management of severe vesical hemorrhage resulting from high dose cyclophosphamide in boys.

A severe form of hemorrhagic cystitis occurs when high doses of cyclophosphamide are administered before bone marrow transplantation. Severe vesical bleeding in male children is difficult to manage because the small urethra precludes the use of large catheters for bladder irrigation and clot removal. We report on the use of cutaneous vesicostomy with the instillation of 4% formalin to control bleeding in 3 boys.

Administration, Topical↗
PubMed1994

Processing bone marrow with a semiautomated cell processor for pediatric transplants: a comparison of two buffy coat preparation methods.

Thirty-one marrows were harvested from 29 patients and donors in the first year after startup of a six-bed pediatric bone marrow transplant unit at Children's Memorial Hospital in Chicago. Twenty-seven patients were infused, 13 with allogeneic marrow. Eight of the 14 autologous marrows were purged with 4HC. This paper presents cell processing results from the first year of operation, describes a yield-enhancing change made to the buffy coat procedure, and shows which of various parameters significantly influenced final cell recoveries.

Adolescent↗
PubMed1992

Single institution experience with high-dose cyclophosphamide, continuous infusion vincristine, escalating doses of VP-16-213, and total body irradiation with unpurged bone marrow rescue in children with neuroblastoma.

Seven consecutive autologous bone marrow transplants were performed in children with neuroblastoma with very good partial remission (VGPR). A combination of cyclophosphamide, escalating doses of VP-16-213, continuous infusion vincristine, and total body irradiation followed by infusion with unpurged bone marrow was used. The dose-limiting toxicity in this regimen was mucositis which occurred when the total dose of VP-16-213 was 2,400 mg/m2. The response rate to this regimen was 4/7 (-CR 48+, 21+, 21+, 35+ mo) 3/7 had a CP/PR post transplant with progressive disease between 1 and 4 months later (mean 2.6 mo). We conclude that this regimen is well tolerated when the maximum dose of VP-16-213 does not exceed 1,800 mg/m2. Further evaluation will be necessary with this regimen to determine its therapeutic value in a larger number of patients with neuroblastoma.

Agranulocytosis↗
PubMed1992

Pharmacokinetics of high dose thiotepa in children undergoing autologous bone marrow transplantation.

Pharmacokinetics of high dose thiotepa was studied in 10 children who received this drug as a 2 h infusion at a dose of 300 mg/m2 daily for 3 days. The thiotepa was quantitated using a modification of a previously published gas chromatographic method. Samples were obtained at predetermined times post infusion. The peak plasma concentrations immediately following the infusion ranged from 5.5 to 25.2 (2.0 +/- 6.6) mg/l and the 8 h post infusion ranged from 0.06 to 0.7 (0.32 +/- 0.21) mg/l. The disposition of thiotepa was best described as a simple one compartment open model. The mean (+/- SEM) values for apparent elimination half-life (t1/2 beta), total plasma clearance (CL) and steady volume of distribution (VDss) were 1.3 x h, 11.25 (1.73) l/h/m2 and 19.38 (2.58) l/m2 respectively. In conclusion the pharmacokinetics of high dose thiotepa in children between 2 and 12 years of age do not appear to vary from those reported in children receiving 75 mg/m2 or adults receiving similar doses.

Bone Marrow Transplantation↗
PubMed1991

Trimethoprim-sulfamethoxazole oral desensitization in hemophiliacs infected with human immunodeficiency virus with a history of hypersensitivity reactions.

Hemophiliacs infected with human immunodeficiency virus with a history of hypersensitivity reaction to a combination product of trimethoprim and sulfamethoxazole were desensitized orally. Six of the seven patients included in the study successfully completed the desensitization protocol and received trimethoprim-sulfamethoxazole for 5 to 7 months after desensitization (mean length of treatment, 5.7 months) for prophylaxis of Pneumocystis carinii pneumonia. The small number of patients and the short follow-up allow us to suggest that oral desensitization may be an effective and inexpensive means to treat hemophiliacs infected with human immunodeficiency virus with trimethoprim-sulfamethoxazole as prophylaxis against Pneumocystis carinii pneumonia.

AIDS-Related Opportunistic Infections↗
PubMed1991

Comprehensive care for patients with hemophilia: an expanded role in reducing risk for human immunodeficiency virus.

Hemophilia is an inherited coagulation disease that affects approximately 1 in 5,000 to 10,000 males worldwide. Chronic joint disease and other long-term complications of recurrent bleeding persist in patients with hemophilia despite improved and more available clotting protein concentrates. The best care can be provided to patients who are followed regularly in specialized treatment centers. Services of every "comprehensive" hemophilia treatment center (HTC) have expanded since previous treatment with clotting factor concentrates infected many hemophilics with the human immunodeficiency virus (HIV). Each HTC offers therapeutic, educational, and counseling expertise in care for the complications of HIV. A nationwide network of specialists now provides care for patients with hemophilia and related congenital abnormalities. In Region VI (Texas, Oklahoma, and Arkansas), the treatment centers and their affiliates provide medical, psychosocial, orthopedic/physical therapy, dental, and case management services. Extramural funded research programs provide care and laboratory testing at no cost to individual subjects.

HIV Infections↗
PubMed1989

Postdelivery head bleeding in hemophilic neonates. Causes and management.

During a 12-month period, four of the five infants with hemophilia known to have been born in Arkansas were examined for head bleeding. Three of the infants had had traumatic delivery, with use of low forceps in two and vacuum extraction in one. In the fourth patient, hemophilia was prenatally diagnosed, and vaginal delivery resulted in cephalohematoma. Diagnosis was delayed in three patients, including one with a family history of hemophilia. Central nervous system bleeding may be more common in hemophilic neonates than has been presumed. Pregnancy management should include consideration of family history of bleeding disorders and carrier testing in appropriate cases. In confirmed carriers, prenatal diagnosis is justified to allow choice of the least traumatic delivery method. Any term neonate with intracranial hemorrhage should be treated as being possibly hemophilic until proved otherwise.

Cerebral Hemorrhage↗
PubMed1988

An experience with an implanted port system in 66 children with cancer.

Totally implanted port catheter systems have a lower incidence of infection and are more easily used in home care that external catheters in adult cancer patients. Experience with this method in children has been limited. During the past 2 years, we have implanted 71 ports in 66 children with cancer. Our experience demonstrates an infection rate (0.15 episodes of bacteremia per 100 patient days) slightly lower than that reported for children with Broviac or Hickman catheters, but not as low as that seen in adults with implanted systems. Patients and families have been extremely satisfied with the devices. Our experience supports further use of implanted systems in children with cancer.

Adolescent↗
PubMed1988

Thrombotic thrombocytopenic purpura in an asplenic patient with hereditary spherocytosis: failure of plasmapheresis, antiplatelet therapy, and corticosteroids.

Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem disorder characterized by microangiopathic hemolysis, central nervous system and renal dysfunction, and a very poor prognosis. Recently, however, plasma exchange or infusion therapy has proven effective in the majority of patients with TTP. We report a patient who developed TTP several years after splenectomy for hereditary spherocytosis. Despite aggressive therapy with plasmapheresis (PP), plasma infusion, antiplatelet drugs, and corticosteroids, the patient had progression of TTP that eventually resulted in his death. The occurrence of TTP in an asplenic patient with an intrinsic red cell disorder, a previously unreported association, may predict a poor prognosis.

Adrenal Cortex Hormones↗