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Biomedical subjects

P Mathew

Publications and source records attributed to P Mathew.

At least 19 recordsLinked to original sources
PubMed2006

Recombinant factor VIIa in paediatric bleeding disorders--a 2006 review.

Recombinant factor VIIa (rFVIIa) has been used in haemophilia bleeding since its introduction in 1996. It has been found to be safe and effective in the majority of patients with haemophilia who have developed inhibitors. There is increasing use of rFVIIa in many off-label bleeding conditions, but there is a paucity of randomized studies regarding the use of rFVIIa in children. This review will attempt to address and summarize the studies focusing on the role of rFVIIa in both haemophilia and non-haemophilia bleeding conditions in children. rFVIIa has been administered as both bolus and continuous infusions, and at varying doses. Furthermore, adverse events have not reportedly increased in children despite growing experience with its use in the paediatric population.

Blood Coagulation Factors↗
PubMed2004

Observation of a narrow charm-strange meson D(+)(sJ)(2632)-->D(+)(s)eta and D(0)K(+).

We report the first observation of a charm-strange meson D(+)(sJ)(2632) at a mass of 2632.5+/-1.7 MeV/c(2) in data from SELEX, the charm hadro-production experiment E781 at Fermilab. This state is seen in two decay modes, D(+)(s)eta and D0K+. In the D(+)(s)eta decay mode we observe a peak with 101 events over a combinatoric background of 54.9 events at a mass of 2635.4+/-3.3 MeV/c(2). There is a corresponding peak of 21 events over a background of 6.9 at 2631.5+/-2.0 MeV/c(2) in the decay mode D0K+. The decay width of this state is <17 MeV/c(2) at 90% confidence level. The relative branching ratio Gamma(D0K+)/Gamma(D(+)(s)eta) is 0.14+/-0.06. The mechanism that keeps this state narrow is unclear. Its decay pattern is also unusual, being dominated by the D(+)(s)eta decay mode.

Journal Article↗
PubMed2004

Comparison of optometry vs digital photography screening for diabetic retinopathy in a single district.

PURPOSE: To compare (a). the clinical effectiveness and (b). cost effectiveness of the two models in screening for diabetic retinopathy. METHODS: (a). Retrospective analysis of referral diagnoses of each screening model in their first respective years of operation and an audit of screen positive patients and a sample of screen negatives referred to the hospital eye service from both screening programmes. (b). Cost effectiveness study. PARTICIPANTS: (1). A total of 1643 patients screened in the community and in digital photography clinics; (2). 109 consecutive patients referred to the Diabetic Eye Clinic through the two existing models of diabetic retinopathy screening; (3). 55 screen negative patients from the optometry model; (4). 68 screen negative patients audited from the digital photography model. RESULTS: The compliance rate was 45% for optometry (O) vs 50% for the digital imaging system (I). Background retinopathy was recorded at screening in 22% (O) vs 17% (I) (P=0.03) and maculopathy in 3.8% (O) vs 1.7% (I) (P=0.02). Hospital referral rates were 3.8% (O) vs 4.2% (I) Sensitivity (75% for optometry, 80% for digital photography) and specificity (98% for optometry and digital photography) were similar in both models. The cost of screening each patient was pound 23.99 (O) vs pound 29.29 (I). The cost effectiveness was pound 832 (O) vs pound 853(I) in the first year. CONCLUSION: The imaging system was not always able to detect early retinopathy and maculopathy; it was equally specific in identifying sight-threatening disease. Cost effectiveness was poor in both models, in their first operational year largely as a result of poor compliance rates in the newly introduced screening programme. Cost effectiveness of the imaging model should further improve with falling costs of imaging systems. Until then, it is essential to continue any existing well-coordinated optometry model.

Adult↗
PubMed2003

Type 2B vWD: the varied clinical manifestations in two kindreds.

Type 2B von Willebrand's disease (vWD) is associated with spontaneous binding of large von Willebrand factor (vWF) multimers to platelets in vivo, followed by clearance of both the large multimers and platelets resulting in thrombocytopenia, which may be intermittent, mild to severe, and may be exacerbated by stress such as infection or pregnancy. We report our experience in two kindreds (49 caucasian individuals) with type 2B vWD and discuss their varied clinical manifestations. The largest kindred (45 patients) was traced back five generations to a presumed index case. The genetic defect in this kindred was identified as a missense mutation, with a C to T transition at a CpG dinucleotide (nucleotide 3916) resulting in an amino acid substitution (Arg 543 to Trp) within the glycoprotein Ib binding domain of vWF. Ristocetin cofactor activity varied from < 10 to 28%, and factor VIII activity from 7 to 69%. Analysis of von Willebrand multimers consistently revealed loss of large molecular weight forms. Platelet counts in those with thrombocytopenia varied from 10 x 10(9) L(-1) to 120 x 10(9) L(-1). The severity of thrombocytopenia has also varied within the same individual during the period of follow-up. The clinical manifestations were varied and ranged from mild to moderate spontaneous bleeding episodes, including epistaxis, menorrhagia and gastro-intestinal haemorrhage. Severe bleeding episodes were observed in those undergoing surgery (both elective and non-elective), and in a few patients despite aggressive replacement with exogenous source of intact vWF, antifibrinolytics when indicated, and a near normal platelet count. Thrombotic disease may be a rare and unusual sequela of this disorder as was noted in one of our patients. Obtaining a platelet count at birth in infants of mothers with type 2B vWD who exhibit thrombocytopenia, may help in the earlier detection of infants at risk for thrombocytopenia.

Adolescent↗
PubMed2002

First observation of the doubly charmed baryon Xi(+)(cc).

We observe a signal for the doubly charmed baryon Xi(+)(cc) in the charged decay mode Xi(+)(cc)-->Lambda(+)(c)K-pi(+) in data from SELEX, the charm hadroproduction experiment at Fermilab. We observe an excess of 15.9 events over an expected background of 6.1+/-0.5 events, a statistical significance of 6.3sigma. The observed mass of this state is 3519+/-1 MeV/c(2). The Gaussian mass width of this state is 3 MeV/c(2), consistent with resolution; its lifetime is less than 33 fs at 90% confidence.

Journal Article↗
PubMed2001

Precision measurements of the lambda(+)(c) and D0 lifetimes.

We report new precision measurements of the lifetimes of the Lambda(+)(c) and D0 from SELEX, the charm hadroproduction experiment at Fermilab. Based upon 1630 Lambda(+)(c) and 10 210 D0 decays we observe lifetimes of tau[Lambda(+)(c)] = 198.1+/-7.0+/-5.6 fs and tau[D0] = 407.9+/-6.0+/-4.3 fs.

Journal Article↗
PubMed2001

T-gamma gene rearrangement and CMV mononucleosis.

A clonal T-gamma rearrangement was found in peripheral blood and bone marrow in a 57-year-old female who presented with 6-week history of fevers, night sweats, and weight loss. Splenomegaly, hemolytic anemia, atypical lymphocytosis, a marrow lymphoid aggregate, and elevated LDH had suggested lymphoproliferative disease. However, IgM serology for cytomegalovirus (CMV) was positive. With observation alone, her clinical features improved over 4 weeks with normalization of the blood count and disappearance of CMV viremia and the aberrant T-gamma clone. Acute CMV infection may mimic lymphoproliferative disease. T-gamma gene rearrangement may be part of the immune response to CMV infection and is not specific to lymphoid neoplasia.

Antibodies, Viral↗
PubMed2001

Anchoring a migrating inferior vena cava stent with use of a T-fastener.

An attempt to treat symptomatic stenosis of the inferior vena cava in a patient with metastatic liver disease was complicated by migration of a Wallstent into the right atrium. Effective palliation was achieved by insertion of a second stent, which was anchored by transhepatic insertion of a T-fastener into the intracaval stent. This anchoring maneuver was performed safely under sonographic and fluoroscopic guidance.

Adult↗
PubMed2001

Is there a potential role for serum cardiac troponin I as a marker for myocardial dysfunction in pediatric patients receiving anthracycline-based therapy? A pilot study.

Serum cardiac troponin I (cTnI) levels have been reported to have high specificity and sensitivity to acute myocardial infarction and coronary ischemic syndromes in adult patients. Our goal was to evaluate the usefulness of serum cTnI in the early diagnosis of cardiac injury from anthracyclines, and to compare these values with echocardiographic findings of cardiac dysfunction. In this prospective study, children being treated on several Children's Cancer Group protocols underwent measurement of shortening fraction (SF), ejection fraction (EF), and serum cTnI levels prior to anthracycline therapy. Sequential serum cTnI levels were then measured along with regularly scheduled echocardiograms with progressively increasing doses of anthracyclines. Fifteen children with median age of 5.75 years (range, 15 months to 15.5 years) at diagnosis were evaluated. Anthracycline doses ranged from 11.72 mg/kg (in patients < 3 years of age) to 375 mg/m2. All but one patient had normal cTnI levels. His level measured at 1.7 ng/ml after 315 mg/m2, but was normal on follow-up testing. Initial SF ranged from 32 to 48%, and EF from 60 to 80%. On follow-up, SF and EF ranged from 30 to 41% and 55 to 70%, respectively. Both SF and EF were significantly lower (p < 0.001) as compared to the initial values. Despite this, all patients remained clinically asymptomatic from the cardiac standpoint. We did not observe elevations of serum cTnI levels in clinically asymptomatic children who received anthracycline therapy up to doses of 375 mg/m2. Does this mean that cardiac injury has not occurred? The possibility of assay sensitivity and the timing of serum sampling and echocardiograms may be important. In addition, larger sample size or longer follow-up may be helpful to determine if higher doses or symptomatic patients potentially have elevations in cTnI levels.

Adolescent↗
PubMed2001

Efficacy of colony-stimulating factors in acute leukemia.

OBJECTIVE: To evaluate the literature describing the safety and efficacy of the hematopoietic colony-stimulating factors (CSFs) for the management of treatment-related adverse effects in patients with acute leukemia. DATA SOURCES: A systematic MEDLINE search of the English-language literature (1995-April 2000) was performed to identify all randomized trials evaluating CSF use in acute leukemia. The following search terms were used: granulocyte colony-stimulating factor, filgrastim, granulocyte-macrophage colony-stimulating factor, sargramostim, acute lymphoblastic leukemia (ALL), acute myelogenous leukemia (AML), acute nonlymphocytic leukemia, and acute myeloid leukemia. The references from relevant literature were also examined in order to identify reports not discovered in the MEDLINE search. DATA SYNTHESIS: Six randomized trials in pediatric ALL, nine in adult AML, and four in adult ALL have examined the safety and efficacy of the CSFs. Two of the pediatric trials supported a reduction in either the duration of hospitalization or in the incidence of febrile neutropenia when a CSF was employed during the consolidation or intensification phase of chemotherapy. The remaining pediatric trials failed to demonstrate a clinical benefit. In adult AML, eight of the nine trials showed a significant decrease in the time to neutrophil recovery when a CSF was used. Only one of these trials demonstrated a decrease in hospital stay and none showed a decreased incidence of infection for patients who received a CSF. Three of the four trials in adult ALL demonstrated the efficacy of a CSF in decreasing the number of days to neutrophil recovery. Only one trial demonstrated that a CSF led to a reduction in the number of hospital days. Trials in children or adults have not demonstrated that the CSFs influence the long-term outcome of patients with acute leukemia. CONCLUSIONS: The published studies document a decrease in the time to recovery from neutropenia when patients with acute leukemia are treated with a CSF. However, a consistent reduction in infectious complications or in the duration of hospitalization has not been demonstrated when a CSF is used for either pediatric or adult patients. Very limited data exist to support the premise that CSFs meet the criteria established by the American Society of Clinical Oncology for demonstrating the value of these agents. Further careful study focused on resource utilization and pharmacoeconomics may help to elucidate how healthcare institutions may most effectively employ CSFs to treat patients with acute leukemia.

Acute Disease↗
PubMed2000

Late effects of treatment in survivors of childhood acute myeloid leukemia.

PURPOSE: To investigate the incidence of and risk factors for late sequelae of treatment in patients who survived for more than 10 years after the diagnosis of childhood acute myeloid leukemia (AML). PATIENTS AND METHODS: Of 77 survivors (median follow-up duration, 16. 7 years), 44 (group A) had received chemotherapy, 18 (group B) had received chemotherapy and cranial irradiation, and 15 (group C) had received chemotherapy, total-body irradiation, and allogeneic bone marrow transplantation. Late complications, tobacco use, and health insurance status were assessed. RESULTS: Growth abnormalities were found in 51% of survivors, neurocognitive abnormalities in 30%, transfusion-acquired hepatitis in 28%, endocrine abnormalities in 16%, cataracts in 12%, and cardiac abnormalities in 8%. Younger age at the time of diagnosis or initiation of radiation therapy, higher dose of radiation, and treatment in groups B and C were risk factors for the development of academic difficulties and greater decrease in height Z: score. In addition, treatment in group C was a risk factor for a greater decrease in weight Z: score and the development of growth-hormone deficiency, hypothyroidism, hypogonadism, infertility, and cataracts. The estimated cumulative risk of a second malignancy at 20 years after diagnosis was 1.8% (95% confidence interval, 0.3% to 11.8%). Twenty-two patients (29%) were smokers, and 11 (14%) had no medical insurance at the time of last follow-up. CONCLUSION: Late sequelae are common in long-term survivors of childhood AML. Our findings should be useful in defining areas for surveillance of and intervention for late sequelae and in assessing the risk of individual late effects on the basis of age and history of treatment.

Acute Disease↗
PubMed2000

Isotopic synovectomy with P-32 in paediatric patients with haemophilia.

Isotopic synovectomy is being proposed as an option in the treatment of patients with haemophilic arthropathy. We present our experience with 11 paediatric patients who underwent 17, P-32 isotopic synovectomies for chronic haemophilic arthropathy. P-32 was injected into the joint per protocol, approved by the institutional review board. All our patients were male. Nine were factor VIII and two were factor IX deficient. The following joints were treated: ankle (n=10 procedures), elbow (n=5) and knee (n=2). The first procedure was performed on December 1993. None were human immunodeficiency virus positive. Mean age at the first procedure was 10.8 years (range, 5.2-15.2 years). Mean pretreatment joint clinical scores using the World Federation of Hemophilia guidelines for the ankle was 5.5 (SD +/- 2.3), the elbow 4.2 (+/-2.5), and knee 5.5 (+/-3.5); the corresponding post-treatment scores were 2.6 (+/-2.0), 1.4 (+/-0.5) and 2.5 (+/-3.5) respectively. Presynovectomy mean radiological scores using the Pettersson method were: ankle 1.8, elbow 1.8, and knee 1.5. A scoring system used in our centre for evaluating joints using magnetic resonance imaging (MRI) gave the following mean pretreatment scores: ankle 9.5, elbow 8.4, and knee 5.0. A marked decrease (an 80-100% decrease) in bleeding was seen in 13 of 17 procedures, and a moderate decrease (51-79% decrease) in two procedures, accounting for 85% reduction in bleeding into the target joints. The procedure was well tolerated and no untoward side-effects were noted as of May 1999, with a median follow-up of 40 months (range 19-65 months). None had any clinical evidence of cancer. Three patients had their joints retreated [elbow (one), ankle (two)]. These procedures were also well tolerated. In conclusion, in our study, isotopic synovectomy using P-32 appears to be feasible, safe and efficacious in the treatment of haemophilic arthropathy in paediatric patients who have been followed for a median of 40 months. As previously shown, MRI appears to give more detailed information about joint arthropathy than plain radiographs.

Adolescent↗
PubMed2000

Mediastinal atypical carcinoid and neurofibromatosis type 1.

We report a case of mediastinal atypical carcinoid in a 63-year-old woman with neurofibromatosis type 1 (NF-1), who presented with shortness of breath and a bulky mediastinal mass. Initial consideration was given to a neurogenic tumor. However, on thoracoscopic biopsy, the histologic appearance was consistent with an atypical carcinoid. Carcinoid tumors have been reported in association with NF-1 previously, but commonly in such unusual sites as the ampulla of Vater and duodenum and not in the thorax. The bulky, extensive, and highly vascular nature of the lesion precluded resection or debulking surgery. To our knowledge, there are no previous reports of atypical carcinoid of the lung or mediastinum in a patient with neurofibromatosis. This case report, therefore extends the spectrum of solid neoplasia in general and carcinoid tumors in specific, as they occur in association with NF-1.

Carcinoid Tumor↗
PubMed2000

Myelophthisis as a solitary manifestation of failure from rectal carcinoma. A Batson phenomenon?

Rectal carcinoma is uncommonly associated with systemic metastases in the absence of liver metastases, reflecting the predilection for spread via the portal system. Occasionally, isolated lung metastases are seen, which are usually attributed to spread via the portosystemic anastomoses in the distal rectum. However, myelophthisis is an unreported complication of rectal cancer as an isolated form of systemic failure. We present a case of fatal myelophthisis associated with otherwise localized rectal carcinoma secondary to metastatic rectal cancer. This observation led to the hypothesis that spread to the bone marrow occurred via a "third circulation," the Batson plexus, a network of deep pelvic veins with rich anastomoses to the vertebral plexus.

Adenocarcinoma, Mucinous↗