Morris Kletzel | Northwestern University (original) (raw)

Papers by Morris Kletzel

PEDIATRICS, 2004

... Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immu... more ... Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM. J Immunol.1997;158 :977 983. Abstract. ↵ Ameratunga R, Lederman HM, Sullivan KE, et al. Defective ...

Background: Methylotrophs are a diverse group of bacteria that can utilize single-carbon compound... more Background: Methylotrophs are a diverse group of bacteria that can utilize single-carbon compounds as a sole energy source, and are often catalase-positive. Known as environmental symbionts, they are emerging as disease-causing organisms in patients with CGD. Methods: We present a case of lymphadenitis due to Granulibacter bethesdensis, a facultative methylotroph, and review 8 other infections caused by methylotrophs in patients with CGD. Results: There have been 9 reported cases of infections due to methylotrophs in patients with CGD. Seven cases were due to G. bethesdensis, one was due to Acidomonas methanolica and one was due to a Methylobacter. In all cases, 16s rRNA gene sequencing was required for diagnosis. Conclusions: Methylotrophs are fastidious and difficult to identify. Although the mechanisms underlying susceptibility to infection with methylotrophs in CGD remain to be elucidated, these bacteria should be included in the spectrum of pathogens associated with infections ...

To determine the effectiveness of using a dried blood spot (DBS)-based Interleukin (IL)-7 assay f... more To determine the effectiveness of using a dried blood spot (DBS)-based Interleukin (IL)-7 assay for the newborn screening of severe combined immunodeficiency (SCID).

Journal of clinical & laboratory immunology

The effect of various concentrations of Adriamycin alone and Adriamycin plus vitamin E on human p... more The effect of various concentrations of Adriamycin alone and Adriamycin plus vitamin E on human polymorphonuclear leukocyte (PMNL) function was determined. Aliquots of PMNLs were incubated at 37 degrees C with saline or vitamin E and then exposed to Adriamycin. A significant decrease in 1-14C glucose utilization and oxygen consumption by zymosan stimulated PMNLs occurred when PMNLs were incubated with 1, 10, 20, 1000 and 200 micrograms/ml concentrations of Adriamycin. Preincubation of the PMNLs with vitamin E afforded partial protection at lower concentrations. The total uptake at 3, 10 and 20 min of methyl 3H thymidine labeled S. aureus by control PMNL was significantly greater than by PMNLs exposed for 60 min to 100 micrograms/ml of Adriamycin. Intracellular Adriamycin concentrations increased in a dose dependent fashion. Viability of cells before and after incubation was 95%. Adriamycin inhibited oxidative metabolic and functional activity of PMNLs in a dose related manner. Preincubation of PMNLs with vitamin E partially protected them from the effects of Adriamycin.

American journal of diseases of children (1960)

Hemophiliacs infected with human immunodeficiency virus with a history of hypersensitivity reacti... more 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.

The presence of neuroblastoma cells in marrow grafts has been linked to the recurrence of the dis... more The presence of neuroblastoma cells in marrow grafts has been linked to the recurrence of the disease post bone marrow transplantation. To avoid this problem, different techniques have been developed to purge tumor cells from the graft. We studied the presence of neuroblasts in bone marrows (BM) of neuroblastoma patients undergoing autologous bone marrow transplantation (ABMT) before and after immunomagnetic purging and compared it to their presence in peripheral blood (PBL). The presence of neuroblasts was also measured in peripheral blood stem cells (PBSC) pheresed after G-CSF stem cell mobilization. We employed the immunofluorencense (IF) method utilizing polylysine-coated dishes or immunocytochemistry (IC) using avidin-biotin peroxidase techniques combined with anti-neuroblastoma monoclonal antibodies. These techniques are capable of identifying one tumor cell in 10 5 mononuclear BM or PBL cells. To further investigate tumor contamination, we tested marrows before and after purg...

Current hematology reports, 2005

Solid tumors are one of the leading causes of disease-related mortality in children today. Althou... more Solid tumors are one of the leading causes of disease-related mortality in children today. Although overall survival of children with solid tumors has improved over time, certain high-risk patients continue to respond poorly to conventional therapy. Recent studies have investigated using dose-intensified myeloablative therapy with autologous hematopoietic progenitor cell transplantation (AHPCT) in hopes of eradicating disease and improving the long-term survival of these patients. Results of high-dose therapy with AHPCT have been encouraging in treatment of metastatic neuroblastoma and recurrent Wilms' tumor, but little benefit has been demonstrated with Ewing's sarcoma. In this article, we examine the role of autologous transplant with each solid tumor and discuss some of the recent transplant-related controversies still under debate.

Journal of hematotherapy, 1994

Thirty-one marrows were harvested from 29 patients and donors in the first year after startup of ... more 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.

Revista Brasileira de Hematologia e Hemoterapia, 2013

This paper reports on a small number of patients with a diverse group of diagnoses that were cons... more This paper reports on a small number of patients with a diverse group of diagnoses that were consolidated with high-dose therapy and rescued with autologous hematopoietic progenitor cells (HPC) . One of the problems with this manuscript and many others is that the studies that have been performed and reported in the literature only reflect those patients who get the rescue and the survival advantage is from that point on. Second the number of patients is small and conclusions about the effectiveness of the procedure are not reliable. The only exception to these two problems has been the report of the only randomized trial for patients with high-risk neuroblastoma that actually includes all patients from diagnosis and shows a slight benefit of autologous hematopoietic stem cell rescue vs. conventional therapy . Does that mean that autologous hematopoietic stem cell rescue is not an effective therapy. My response is that maybe an effective consolidation therapy in a selected group of patients with certain diagnoses. For example, in patients with recurrent Wilms tumors who achieve a second complete remission and are consolidated with high-dose therapy and autologous HPC do very well unfortunately the reports about the benefit are with multiple different conditioning regimens some with a single rescue and others with a double rescue where deciding what is the standard to which we can compare results is not available. Other reports have shown that the survival in the same group of patients maybe equally as good without HPC rescue .

American Journal of Diseases of Children, 1989

During a 12-month period, four of the five infants with hemophilia known to have been born in Ark... more 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.

Journal of Hematotherapy & Stem Cell Research, 2001

The cells were cultured with four cytokines: interleukin-3 (IL-3), thrombopoietin (TPO), stem cel... more The cells were cultured with four cytokines: interleukin-3 (IL-3), thrombopoietin (TPO), stem cell factor (SCF), and Flt-3); five cytokines, IL-3, TPO, SCF, Flt-3 plus granulocyte-macrophage colonystimulating factor (GM-CSF), or erythropoietin (Epo); or all six cytokines in combination. After 16 days, significant expansion of MK precursors (CD41 1 ) and stem cells (CD34 1 and AC133 1 cells) were seen in cells cultured in IL-3, TPO, SCF, and Flt-3 with or without GM-CSF compared to the combinations that contained Epo (p , 0.05). Similar studies were performed using liquid culture medium, and after 14 days the number of MNCs, CD34 1 , AC133 1 , CD41 1 , and CD61 1 cells were higher in the UCB cells cultured in IL-3, TPO, SCF, and Flt-3 compared to those cultured with those four cytokines plus GM-CSF. These results demonstrate that UCB stem cells can be effectively expanded ex vivo and enriched with platelet precursors using TPO, SCF, Flt-3, and IL-3, whereas the addition of Epo and GM-CSF is unnecessary.

Pediatric Transplantation, 2014

Vitamin C deficiency in developed countries is typically observed in patients with unique clinica... more Vitamin C deficiency in developed countries is typically observed in patients with unique clinical conditions such as cystic fibrosis or anorexia nervosa, or in patients on long-term tube feeds. We report here a clinical observation in six pediatric and adolescent patients (median age 17.5 yr, range 9.8-23.5 yr) with chronic GVHD with mucous membrane involvement found to be vitamin C deficient. These patients&amp;amp;amp;#39; baseline serum vitamin C levels ranged from &amp;amp;amp;lt;0.12 to 0.94 mg/dL (normal value 0.20-1.90 mg/dL), with a mean level 0.56 ± 0.36 mg/dL and a median level 0.6 mg/dL. Among these patients, signs and symptoms of mucositis failed to respond to standard chronic GVHD therapy. After receiving treatment with 2000 mg of ascorbic acid by mouth, daily patients displayed increased serum vitamin C levels. Clinically, this correlated with a remarkable improvement in patients&amp;amp;amp;#39; mucositis and ability to eat.

Medical and Pediatric Oncology, 1992

Seven consecutive autologous bone marrow transplants were performed in children with neuroblastom... more Seven consecutive autologous bone marrow transplants were performed in children with neuroblastoma with very good partial remission (VCPR). 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/m'. The response rate t o this regimen was 417 (-CR 48+,21+,21+,35+ mo)3/7 hadaCWPRpost transplant with progressive disease between 1 and 4 months later (mean 2.6 mo). W e conclude that this regimen is well tolerated when the maximum dose of VP-16-213 does not exceed 1,800 mg/m'. Further evaluation will b e necessary with this regimen to determine its therapeutic value in a larger number of patients with neuroblastoma. Key words: n e u r o b l a s t o m a , a u t o l o g o u s bone marrow, u n p u r g e d bone m a r r o w From the

Journal of Pediatric Surgery, 2006

Our aim was to investigate the impact of the extent of surgical resection on local recurrence and... more Our aim was to investigate the impact of the extent of surgical resection on local recurrence and survival in high-risk patients treated with the Chicago Pilot II protocol. Retrospective chart review was performed on 30 patients enrolled in the Chicago Pilot II protocol between 1995 and 2003. Variables studied were location of tumor, extent of resection, timing and location of recurrence, MYCN amplification, surgical complications, event-free survival, and overall survival (OS). Operative reports and postoperative meta-iodobenzylguanidine scans were used to assess extent of resection. Complete resection (CR) was defined as no gross residual tumor including primary and nodal disease. Three-year event-free survival and OS of this cohort of 30 patients was 58% and 82%, respectively. Only 1 patient developed a local recurrence, whereas metastatic recurrent disease was observed in 13 (43%) of the 30; and this subset had a significantly worse OS (23% vs 94%, P = .001). The most common relapse location was in bone. Patients with incomplete resection (IR) (11/30) and CR (19/30) had recurrence rates of 64% (7/11) and 32% (6/19, P = .12), respectively. Event-free survival was significantly better for patients with CR (68%) vs IR (27%; P = .05; odds ratio, 2.9). Overall survival rates for patients with CR vs IR were 68% vs 55%, respectively (P = .25). Recurrence rate was the significant determinant of survival. Patients with CR had lower recurrence rates; however, they did not have improved local control. Final outcome of patients with unfavorable neuroblastoma will be determined by metastatic recurrence, not by extent of resection.

Journal of Pediatric Oncology Nursing, 1986

Journal of Pediatric Hematology/Oncology, 1988

Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem disorder characterized by micro... more 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.

Cytotherapy, 2001

Thiazole orange (TO) is a nucleic-acid-specific dye that enters cells without pretreatment. When ... more Thiazole orange (TO) is a nucleic-acid-specific dye that enters cells without pretreatment. When it binds to either RNA or DNA, there is an increase in fluorescence emission. This property has been utilized to measure the amount of newly released platelets using flow cytometry. These newly released platelets differ from more mature platelets because they still contain residual amounts of RNA, and have become known as reticulated platelets.

Cancer Genetics and Cytogenetics, 1994

... Pericentric Inversion (2)(p15q35) in an Alveolar Rhabdomyosarcoma Jeffrey R. Sawyer, Frank G.... more ... Pericentric Inversion (2)(p15q35) in an Alveolar Rhabdomyosarcoma Jeffrey R. Sawyer, Frank G. Crussi, and Morris Kletzel ABSTRACT: We report a 7-year ... Dal Cin P, Brock P, Aly MS, Castelles-Van Daele M, DeWever I, Van Damme B, Van Den Berghe H (1991): A variant (2;13 ...

Cancer, 1980

Two additional cases of systemic mycosis due to Trichosporun cutaneum are reported and are compar... more Two additional cases of systemic mycosis due to Trichosporun cutaneum are reported and are compared with the previously published case of Rivera and Cangir. Both patients (a four-year-old male and a 57-year-old female) had acute leukemia for which they were receiving chemotherapy, and both presented with fever that was unresponsive to conventional antibiotics. Both had positive blood cultures for Trichosporon cutaneum . The disease was further documented in the four-year-old male by renal biopsy and by bone marrow culture; he was treated with apparent success with amphotericin B. However, the 57-year-old female died shortly after the beginning of similar treatment, and autopsy demonstrated involvement of the left kidney, spleen, bone marrow, and liver. The organism in both these cases, as well as the case of Rivera and Cangir, exhibited both hyphal and yeastlike forms in tissue sections. We believe that the therapeutic success in the case of the four-year-old male was primarily related to his remission from leukemia.