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Biomedical subjects

M M Mustafa

Publications and source records attributed to M M Mustafa.

At least 19 recordsLinked to original sources
PubMed2002

Transfusion-dependent congenital dyserythropoietic anemia type I successfully treated with allogeneic stem cell transplantation.

Until recently, therapy for patients with severe congenital dyserythropoietic anemia (CDA) has been limited to blood transfusions and chelation therapy. Three children with transfusion-dependent CDA type I underwent allogeneic stem cell transplantation (SCT) from matched sibling donors. Conditioning was with cyclophosphamide 50 mg/kg/day for 4 days, busulphan 4 mg/kg/day for 4 days, and antithymocyte globulin (ATG) 30 mg/kg for four doses pre-SCT. All patients engrafted and are alive, and transfusion independent. To our knowledge, this is the first report of successful SCT in the management of CDA type I.

Anemia, Dyserythropoietic, Congenital↗
PubMed2001

Evaluation of the safety and efficacy of repeated sedations for the radiotherapy of young children with cancer: a prospective study of 1033 consecutive sedations.

PURPOSE: A prospective observational study to examine our current practice of either conscious sedation (C.S.) or general anesthetic (G.A.) for children undergoing radiation therapy (we use the term sedation to include both C.S. and G.A.). Specifically, the study examines the reasons for selection of patients, choice of drugs, safety and effectiveness of the procedure, side effects of repeated daily sedation, and compliance of the family with the regimen. METHODS AND MATERIALS: Recorded data included patient demographics, sedation technique, time for various stages of the procedure, breathing support required, sedation outcome, and complications. RESULTS: One hundred ninety-eight consecutive children underwent 4232 procedures involving either simulation or radiation treatment, an average of 21 procedures each. Seventy-four (37%) required sedation for a total of 1033 procedures, an average of 14 sedations each. For those patients who received sedation, the age ranged from 9 months to 14 years (median, 3.8) and 96% had a mold, (85% of the head and neck). The doctor's assessment of the need for sedation was correct 89% of the time. Thirty-seven percent required sedation at the start of treatment, but, even after 30 fractions, 15% still required sedation. Presedation status correlated with successful sedation and treatment (p = 0.0001). Ninety-six percent had some form of i.v. access, usually a portacath (76%); 883 sedations were performed with G.A. and 148 with C.S.; 93% of sedations were completed satisfactorily, 5% with some difficulty, and the patient was unable to be treated in 2%. With G.A., satisfactory sedation was achieved 97% of the time, whereas, with C.S., satisfactory sedation was achieved only 68% of the time. There were no complications for 97% of observations. Not one serious complication occurred. An endotracheal tube was used only twice during G.A. For C.S., the results for chloral hydrate, meperidine, and midazolam were, respectively, at least one complication, 23%, 0%, 5%; satisfactory sedation for treatment, 60%, 60%, 82%; and unable to treat 20%, 13%, 5%. For G.A., only 1 patient was unable to be treated. The median time from start of medication to the end of radiation treatment was a median of 10 min (75% complete within 15 min) for G.A., vs. 30 min (75% complete in 50 min) for C.S. On multivariate analysis, the only significant factor predicting a successful outcome was a G.A. using propofol (odds ratio, 20.6), vs. C.S. using either chloral hydrate, meperidine, or midazolam. (p = 0.0001). CONCLUSION: In this study, there were no serious complications of sedation in 1033 procedures. As a result of the study, we developed improved methods for better preparation of the patient and family to try to reduce the need for sedation, and reduce the indications for C.S., while confirming the safety and efficacy of a G.A. with propofol for children needing sedation for daily radiation therapy.

Adolescent↗
PubMed2001

Bone marrow transplantation from matched siblings in patients with fanconi anemia utilizing low-dose cyclophosphamide, thoracoabdominal radiation and antithymocyte globulin.

Nineteen patients with Fanconi anemia (FA) and bone marrow failure underwent bone marrow transplantation (BMT) from matched siblings. Median age at BMT was 8.7 years. Conditioning consisted of low-dose cyclophosphamide (CY 5 mg/kg x 4 days) and thoracoabdominal irradiation (TAI 400 cGy). Graft-versus-host disease (GVHD) prophylaxis was cyclosporin A (CsA) in 13 patients and CsA plus methotrexate in 6 patients. Antithymocyte globulin (ATG) was added in the pretransplant as well as the post-transplant period. All patients received high-dose acyclovir from day 2 pre-BMT to day 28 post BMT, and intravenous immunoglobulins (IVIG), 500 mg/kg weekly from day 7 pre-BMT to day 90 post BMT. No fungal prophylaxis was given. All patients engrafted, (median, 14 days for an absolute neutrophil count > or =0.5 x 10(9)/l; median, 37 days for platelet count > or =20 x 10(9)/l). Fourteen (74%) patients are alive with sustained engraftment and are transfusion independent. Three (16.6%) patients developed acute GVHD; none developed chronic GVHD. Five (26%) patients developed invasive fungal infections, and two (10%) developed fatal CMV disease. We believe the addition of ATG may have contributed to the increased incidence of severe life-threatening fungal and viral infections in our series.

Antilymphocyte Serum↗
PubMed2001

Congenital sideroblastic anaemia successfully treated using allogeneic stem cell transplantation.

Therapy for patients with congenital sideroblastic anaemia has been limited to blood transfusions and chelation. Three children with congenital sideroblastic anaemia (SA) who were blood transfusion dependent underwent stem cell transplantation (SCT) from matched sibling donors. Conditioning consisted of cyclophosphamide 50 mg/kg/d for 4 d, busulphan 4 mg/kg/d for 4 d and anti-thymocyte globulin (ATG) 30 mg/kg for four doses pretransplant. Graft-versus-host disease (GVHD) prophylaxis was with cyclosporin A and methotrexate. All patients engrafted, and are alive and transfusion independent. SCT can be curative for patients with SA.

Anemia, Sideroblastic↗
PubMed2001

Comparative study of cefepime versus ceftazidime in the empiric treatment of pediatric cancer patients with fever and neutropenia.

BACKGROUND: In view of the recent trend toward monotherapy in the treatment of bacterial infection, we evaluate the clinical efficacy and safety of cefepime vs. ceftazidime for the empiric treatment of febrile episodes in neutropenic pediatric cancer patients. METHODS: In a single site, open label study, 104 neutropenic pediatric cancer patients [96% with absolute neutrophil count (ANC) of <500 neutrophils/mm3] with a median age of 6 years were randomized (1:1) to receive either intravenous cefepime or ceftazidime (50 mg/kg/dose every 8 h; < or = 6 g/day) for empiric treatment of fever (temperature >38.0 degrees C occurring at least twice in 24 h, or single >38.5 degrees C). Febrile episodes were classified as either microbiologically or clinically documented infection or fever of unknown origin. Therapy continued until the ANC was > or = 1,000 neutrophils/mm3 or there was an increasing ANC in low risk patients (maximum duration of treatment, 8 weeks). The primary efficacy endpoints assessed were clinical and microbiologic response to assigned drug therapy. Secondary outcome measures were rate of early discontinuation of study drug and use of concomitant antibiotic therapy to modify initial study drug regimen. RESULTS: Of 68 patients who could be evaluated for efficacy, 74% (26 of 35) of cefepime-treated patients and 70% (23 of 33) of ceftazidime-treated patients responded to treatment. The small number of study patients precluded statistical analysis of results. In a modified intent-to-treat analysis, 59% of the patients treated with cefepime and 47% of ceftazidime-treated patients responded to therapy. Cefepime patients developed fewer new infections than ceftazidime patients (9% vs. 21%, respectively) and early discontinuation of study drug therapy occurred slightly more often in the ceftazidime group. Further, the use of concomitant systemic antimicrobial therapy (mostly vancomycin) occurred less often in the cefepime-treated patients, as compared with the ceftazidime group [35% [17 of 49] vs. 44% (24 of 55), respectively]. No deaths or serious adverse events were considered to be related to study therapy. The most frequent adverse event was rash that was moderate in severity, and it occurred equally in both groups. CONCLUSION: Cefepime appears to be safe and effective compared with ceftazidime for initial empiric therapy of febrile episodes in neutropenic pediatric cancer patients.

Adolescent↗
PubMed2000

Hematopoietic stem cell transplantation (HSCT) with a conditioning regimen of busulfan, cyclophosphamide, and etoposide for children with acute myelogenous leukemia (AML): a phase I study of the Pediatric Blood and Marrow Transplant Consortium.

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is an important treatment modality for children with AML. The optimal conditioning regimen is unknown. The aim of this study was to determine the appropriate dosing of etoposide in combination with busulfan and cyclophosphamide in this setting. PROCEDURE: Twenty patients with a diagnosis of AML in first or second remission, or myelodysplasia scheduled for bone marrow transplantation, were included in this study. Patients received busulfan 640 mg/m(2) in 16 doses, cyclophosphamide 120 to 150 mg/kg in two doses, and etoposide from 40-60 mg/kg as a single dose. Extensive toxicity data was collected. RESULTS: Nineteen patients were evaluable for toxicity. Mucositis was seen in all patients. Four patients developed bacteremia and one patient died from overwhelming sepsis on day +3. Four patients developed moderate to severe skin toxicity. The major dose-limiting +3 toxicity was hepatic toxicity, which occurred in 14 of 19 patients. Eight patients developed clinical veno-occlusive disease, including three patients at dose level 4, two of whom had life-threatening disease. This hepatic toxicity defined the MTD of 640 mg/m(2) busulfan, 120 mg/kg of cyclophosphamide, and 60 mg/kg of etoposide. Overall, 9 of 20 patients enrolled in the study survive in remission, 8/14 allogeneic (median follow-up 44 months), and one of six autologous patients (follow-up, 54 months). CONCLUSIONS: We conclude that the combination of busulfan, cyclophosphamide, and etoposide at the doses defined above has activity in the treatment of children with high-risk AML/MDS undergoing allogeneic HSCT. Whether it offers an advantage over other conditioning regimens will require a randomized trial with a larger cohort of patients.

Adolescent↗
PubMed2000

Searching for socioeconomic risk factors in perinatal mortality in Kuwait: a case control study.

The aim of this paper was to investigate whether socioeconomic factors such as parent's education, occupation, and income constitute risk factors in perinatal mortality after controlling for biological variables such as birth weight and length of gestation, and maternal factors such as age, parity and reproductive history. A case-control study covering all perinatal deaths in Kuwait was conducted for one year from 1 October, 1997 to 30 September, 1998. Each case (perinatal death) was matched with a control (live birth). Matching criteria were: father's nationality, place, and date of birth. Information was successfully collected on 463 matched pairs, 274 Kuwaitis and 189 non-Kuwaitis. Only singleton births were included in the analysis. Bivariate analysis showed that several of the socioeconomic variables (e.g. lower education, lower income) increased the risk of a perinatal death. However, none of these variables remained significant in the multivariate analysis in which birth weight and length of gestation emerged as the two major determinants of perinatal deaths among both nationality groups. Among the Kuwaitis, primiparity and high parity, and previous history of miscarriage were also significant risk factors. Among the non-Kuwaitis, none of the socioeconomic factors, or the maternal factors, were significant predictors of perinatal mortality. For Kuwaitis, it appears that the government's policies and programs aimed at reducing social inequalities in the society have been effective in eliminating perinatal mortality differences between socioeconomic groups. Among non-Kuwaitis, the lack of differences is reflective of the fact that this group is relatively homogenous and selective of the more affluent who can bring the family to Kuwait. Both nationality groups benefit from the government's free health services. However, charges for non-Kuwaitis are due to be levied soon which may increase disparities in access to health care.

Birth Weight↗
PubMed1997

Radiation therapy for retinoblastoma: a retrospective review of 120 patients.

PURPOSE: To characterize the patient population and treatment outcomes in patients with Retinoblastoma (RB) referred for External Beam Orbital Radiotherapy (EBORT) to King Faisal Specialist Hospital & Research Centre (KFSH&RC), Riyadh, Saudi Arabia from 1976 to 1993. METHODS AND MATERIALS: A retrospective study of 120 patients with RB affecting a total of 192 eyes. Patients were divided into three groups. Group A are 60 patients (64 eyes) treated with EBORT to the intact eye to preserve vision. Reese-Ellsworth (RE) Staging was: 1: 12%; 2: 10%; 3: 12%; 4: 23%; and 5: 43%. Twenty-eight patients (47%) also received Vincristine, Adriamycin, and Cyclophosphamide chemotherapy (C/T). Mean follow-up, per patient, was 48.5 months. Standard treatment until 1992 was 45 Gy in 12 fractions of 3.75 Gy, three times weekly over 18 days. Assuming the alpha/beta ratio for early effects and tumor control at 10, Tk = 21 days, Tpot = 5 days, then the Biological Equivalent Dose (BED) was 62 Gy10 for early effects, and 101 Gy3 for late effects. Group B are 28 patients (28 eyes) treated for curative intent with EBORT to the orbit for locally advanced disease, usually after enucleation (24 eyes). Nineteen patients (83%) also had C/T. Mean follow-up was 22.6 months. Group C are 37 patients with advanced disease treated with radiotherapy for palliation. Seventeen (46%) also received C/T. Mean follow-up was 11.7 months. RESULTS: Group A-following EBORT useful vision was retained in RE Stage 1 to 5: 7 of 7, 6 of 6, 4 of 8, 10 of 15, and 7 of 28 eyes, respectively. There was no significant difference between patients who received adjuvant chemotherapy and those who did not. Complications included cataract (27%), retinopathy (25%), vitreous hemorrhage (19%), and orbital deformities (11%). In Group B the local control rate was 71%. In Group C, 10 (27%) of the 37 patients were alive at last contact, and 27 (73%) were either terminal or dead of disease. None of Group A or B patients had positive CSF cytology, bone scan, or bone marrow examination. In Group C 19% had positive CSF cytology, and bone marrow, and 14% had a positive bone scan. CONCLUSIONS: 1) EBORT preserved useful vision in a significant proportion of patients even in eyes with advanced RE Stage RB, but longer follow-up is likely to reveal an even higher complication rate with this regime. 2) High dose per fraction probably contributed to the increased complications. 3) Chemotherapy did not demonstrate any effect on retaining vision in this study. 4) For disease that is confined to within the eye clinically and radiologically, invasive procedures for CSF cytology, bone marrow examination, and bone scan do not seem warranted. 5) The optimum technique, fractionation, and dosage for RB is still not well defined.

Child↗
PubMed1997

Treatment of children with epipodophyllotoxin-induced secondary acute myeloid leukemia.

BACKGROUND: Secondary acute myeloid leukemia (AML) after treatment with epipodophyllotoxins is being observed with increased frequency. Therapeutic options are limited for patients with secondary AML and the role of bone marrow transplantation is unclear. METHODS: The authors report the treatment outcome of a cohort of 17 children who developed epipodophyllotoxin-induced secondary AML after therapy for childhood acute lymphoblastic leukemia (ALL) that included etoposide but no irradiation or alkylating agents. Thirteen patients (76%) had 11q23 chromosomal abnormalities that were not present at the initial diagnosis of ALL. RESULTS: Remission induction was attempted in 16 children, with 13 (81%) achieving a complete remission. After consolidation, 9 of these 13 patients received a bone marrow transplant (BMT): 2 with 4-hydroperoxycyclophosphamide-purged autologous marrow, 4 from a human leukocyte antigen (HLA)-identical sibling, 1 from a mismatched parental donor, and 2 from a matched unrelated donor. One additional child underwent allogeneic BMT without an attempt at reinduction. Of the 10 patients who received transplants, 2 were alive and well 27+ and 36+ months, respectively, after BMT. Of the 7 patients who did not receive a transplant, at last follow-up 1 had survived off therapy for 8 months for recurrent ALL whereas 6 died of AML. CONCLUSIONS: These data confirm the poor prognosis of secondary AML after epipodophyllotoxin treatment for childhood ALL. Although patients with secondary AML can achieve a remission, it is usually of brief duration. Allogeneic BMT may offer the possibility of long term remission in some of these patients. More information is needed to better define the risks and benefits of epipodophyllotoxin therapy for childhood ALL.

Acute Disease↗
PubMed1997

Safety of early hospital discharge of selected febrile children and adolescents with cancer with prolonged neutropenia.

PROBLEM: The safety of early hospital discharge (i.e., before the absolute neutrophil count [ANC] exceeds 500 cell/mm3) of febrile neutropenic children and adolescents with cancer who had experienced prolonged neutropenia (i.e., for more than 7 days) following admission has not been studied. METHOD OF STUDY: Three hundred and thirty nine consecutive admissions of children and adolescents with cancer for management of febrile neutropenia were reviewed. Early discharge criteria included absence of fever for 24 hours prior to discharge, sterile blood cultures for 24 hours, evidence of bone marrow recovery defined as a sustained increase in platelet count and ANC or absolute phagocyte count (APC), and control of local infection if present. Children hospitalized with febrile neutropenia who remained neutropenic for more than 7 days were analyzed to assess their outcomes following discharge it they had met criteria for early hospital discharge. RESULTS: Thirty-three patients in whom neutropenia had persisted for more than 7 days were discharged before attaining an ANC greater than 500/mm3 when they met the early discharge criteria. Only two children (6%) required readmission for recurrent fever, a rate which was not different from that of patients discharged after a more transient episode of neutropenia (2 of 33 vs. 3 of 121, P = 0.3). Both patients who were readmitted had a source of local infection which worsened despite oral antibiotics. Both patients appeared clinically well at the time of readmission and had sterile cultures during their second hospitalization with resolution of local infection. CONCLUSION: This study confirms that low-risk criteria used to select children with cancer for discharge before complete resolution of neutropenia can be safely applied to those patients whose neutropenia lasted more than 7 days following admission.

Adolescent↗
PubMed1996

A pilot study of outpatient management of febrile neutropenic children with cancer at low risk of bacteremia.

Febrile neutropenic children with cancer were eligible for outpatient management with intravenous ceftriaxone therapy if they displayed selected low-risk criteria. Nineteen children were enrolled. All patients had sterile blood cultures, and only one of them was hospitalized because of persistent fever. This pilot study suggests that selected children with febrile neutropenia might be successfully managed without hospitalization.

Adolescent↗
PubMed1996

Diverse hematologic effects of parvovirus B19 infection.

Human parvovirus B19 is linked with a broadening spectrum of hematologic disorders, including aplastic crises in the context of hemolytic anemias, neutropenia, thrombocytopenia, and hemophagocytic syndromes. Children with any of these cytopenias should be screened for the presence of B19 because treatment with intravenous gamma globulin may provide resolution of abnormal blood counts if other therapeutic options, such as transfusion, are not adequate or desired.

Acquired Immunodeficiency Syndrome↗
PubMed1995

Fatal hemolysis induced by ceftriaxone in a child with sickle cell anemia.

A 2-year-old boy with sickle cell anemia had a massive, fatal hemolytic reaction after administration of an intravenous dose of ceftriaxone. Laboratory studies demonstrated the presence of an IgM antibody against ceftriaxone, binding to and destroying the patient's erythrocytes by an immune complex mechanism. This rare complication should be considered in the differential diagnosis when hemoglobinuria develops in a child after administration of ceftriaxone or a similar agent.

Anemia, Hemolytic, Autoimmune↗
PubMed1995

Fatal disseminated infection due to Exserohilum rostratum in a patient with aplastic anemia: case report and review.

Exserohilum rostratum is a dematiaceous fungus that rarely causes infection in humans. We describe a patient with severe aplastic anemia who developed fatal disseminated disease caused by E. rostratum, and we review the English-language literature on this unusual agent. Our patient initially presented with signs and symptoms of sinusitis, which progressed to invasive pulmonary disease. The clinical course and histopathologic findings at autopsy were similar to those seen in cases of invasive aspergillosis or mucormycosis in that a propensity for vascular invasion and dissemination was seen.

Anemia, Aplastic↗
PubMed1995

The changing epidemiology of bacteremia in neutropenic children with cancer.

Gram-positive bacteria have been the predominant organisms causing bacteremia in febrile neutropenic cancer patients during the past decade. Recently we have noted an increase in Gram-negative bacteremia in children and adolescents with cancer. Therefore we retrospectively reviewed 153 episodes of bacteremia during a 6-year period to investigate changes in the etiology of bacteremia in pediatric oncology patients. In the early 3-year period (January, 1988, to December, 1990) Gram-positive organisms comprised 73 (74%) of the 99 isolates, and Staphylococcus epidermidis was the most common isolate. In the later 3-year period (January, 1991, to December, 1993) Gram-negative organisms were seen with greater frequency and represented 50% of isolates (P = 0.004). Pseudomonas aeruginosa was the most commonly isolated organism during this period (22% of all isolates). We speculate that the recent utilization of more intensive chemotherapy regimens has caused an alteration in the epidemiology of bacteremia in children and adolescents with cancer which could influence the initial empiric antibiotic regimens and the outcome of such infections.

Adolescent↗
PubMed1995

Disseminated Microascus cirrosus infection in pediatric bone marrow transplant recipient.

Microascus cirrosus Curzi and its associated anamorphic state, Scopulariopsis, were recovered from the cutaneous lesion of a 12-year-old male who had undergone an autologous bone marrow transplantation for acute myelogenous leukemia. Histopathology sections from the biopsied lesion demonstrated septate hyphae consistent with a fungal etiology. Radiographic studies of the lungs subsequent to progression of the lesion revealed a consolidation in the right upper lobe suggesting a primary focus of infection. While M. cirrosus is fairly abundant in nature and widely distributed in stored grains in North America, this is, to our knowledge, the first reported human infection by this species. Salient characteristics for the identification of this dematiaceous ascomycete, M. cirrosus, will be presented.

Ascomycota↗