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Biomedical subjects

I Warrier

Publications and source records attributed to I Warrier.

33 records · Page 2Linked to original sources
PubMed1994

Immune and serologic profiles of HIV-infected and noninfected hemophilic children and adolescents. Hemophilia Growth and Development Study Group.

OBJECTIVES: To assess relationships among the effects of HIV on hemophilic children and adolescents' immunologic parameters and vaccine-related serology. METHODS: We analyzed data from extensive baseline immunologic evaluations of 207 HIV antibody-positive (HIV+) and 126 HIV antibody-negative (HIV-) hemophilic children and adolescents. RESULTS: HIV+ and HIV- participants differed significantly in T-lymphocyte subpopulation numbers, immunoglobulin levels, and seroprevalence rates for diphtheria toxoid, measles, and mumps antigens, IgG levels, IgM levels, and serologic titers to vaccine antigens showed little correlation with T-cell parameters. Proportionately more HIV+ participants were nonreactive to each and all of a panel of 7 skin test antigens (71% vs 28% anergic, RR 2.6). The odds of anergy increased 1.6 times for every decline of 200 CD4+ cells/microliters. CONCLUSIONS: HIV had significant, largely independent T- and B-lymphocyte effects on this pediatric cohort.

Adolescent↗
PubMed1992

Hemophilia A.

Over the past few years considerable progress has been made in elucidating the molecular genetics of hemophilia A, in carrier detection and prenatal diagnosis, and in the production of safer clotting factor concentrates. Recombinant FVIII, shown to be safe and effective in ongoing prelicensure clinical trials that began in the spring of 1987, should soon be licensed and commercially available. There is now considerable interest in beginning prophylactic therapy regimens at 1 or 2 years of age, in an attempt to prevent chronic joint disease and other complications of serious bleeding episodes. The possibility of gene insertion therapy for persons with hemophilia now seems to be a realistic one--perhaps achievable in the 1990s. Although many problems remain--major problems resulting from HIV, HCV, and HBV infections; how to deal with existing musculoskeletal problems; how to pay for the higher-priced new technologies; high titer inhibitors; just to name a few--the many recent scientific advances and their clinical applications make this an exciting time. This is truly, as indicated in the title of the proceedings of the XIX Congress of the World Federation of Hemophilia, a new decade of hopes and challenges.

Child, Preschool↗
PubMed1991

Hemophilia.

Explore the source record for details and available documents.

Adolescent↗
PubMed1990

Retrovir therapy in hemophilic children with symptomatic human immunodeficiency virus infection: efficacy and toxicity.

In desperation, we have used retrovir in five hemophilic children (10-16 years old) over the past 22 months. All had presented with various clinical manifestations of acquired-immune-deficiency-syndrome (AIDS)-related complex or AIDS. Our decision to treat with retrovir was based on clinical manifestations and very low numbers of CD4 cells (less than 200). The most common clinical presentation was recurrent oral moniliasis. Other significant findings included recurrent herpes zoster, thrombocytopenia, growth failure, and biliary tract infection. Initially, all five children received the full adult dosage of retrovir (200 mg q 4 h x 6 doses/day). This dosage had to be reduced in four children because of toxicity. The most commonly observed toxic side effects were anemia and neutropenia. Alanine aminotransferase (ALT) levels rose to 4-10 times the upper limit of normal in four of five children. One was on concomitant ketokonazole prior to the rise in ALT level. Myalgia and headache were reported by two patients. Improvement in clinical and immunological status was observed in all children initially. After 12-18 months of retrovir therapy, infectious complications secondary to prolonged neutropenia were seen in these immunocompromized children. However, compared to historic controls, these children have had fairly stable disease. We feel that all hemophilic children with symptomatic human immunodeficiency virus infection should be offered this drug, even though the optimal dosage for children is not yet established.

AIDS-Related Complex↗
PubMed1989

Paraplegia after lumbar puncture. In an infant with previously undiagnosed hemophilia A. Treatment and peri-operative considerations.

We report a 3-month-old infant who became paraplegic from an epidural hematoma caused by a diagnostic lumbar puncture for work-up of sepsis. The differential diagnosis of the cause of paraplegia was epidural hematoma formation versus spinal abscess. Hemophilia A was diagnosed when coagulation studies were discovered to be abnormal, and non-contrast CT scan revealed an epidural mass with spinal cord displacement. The coagulopathy was rapidly corrected preoperatively with an infusion of cryoprecipitate. A medially limited bilateral T8-L4 laminectomy allowed complete evacuation of the hematoma with maximum preservation of normal bone tissue, but no clinical improvement resulted. Coagulopathy should be highly suspect in an infant who becomes paraplegic after lumbar puncture. The coagulopathy may be rapidly corrected with deficient factor replacement, allowing major spinal surgery to be performed safely.

Epidural Space↗
PubMed1987

Use of intravenous gamma globulin in children and adolescents with idiopathic thrombocytopenic purpura and other immune thrombocytopenias.

Approximately 85 to 90 percent of cases of idiopathic thrombocytopenic purpura (ITP) in children are of the acute, self-limited variety that generally occurs after a viral infection. The remaining 10 to 15 percent of children with this disorder have the chronic (autoimmune) type of ITP. For these patients, splenectomy is often the recommended treatment if severe bleeding occurs and platelet counts remain below 40,000/mm3. However, splenectomy has associated risks and the response to this surgery cannot always be predicted. Intravenous gamma globulin (IVIG) has proven useful as an alternative to splenectomy, especially in children who are considered too young for splenectomy or in those in whom there is no response to splenectomy. It should be noted that booster shots are frequently required and the patient's ITP may become refractory. IVIG may also be useful in preparing a child with ITP for splenectomy and in treating children or adolescents with ITP who have central nervous system or other serious hemorrhages. Although IVIG is not always effective in raising the platelet count, it does provide a very useful alternative method of treating this disorder.

Adolescent↗
PubMed1984

Intravenous gamma globulin treatment for chronic idiopathic thrombocytopenic purpura in children.

Six children, five to 16 years of age, with the chronic, autoimmune form of idiopathic thrombocytopenic purpura were given intravenous gamma globulin (Gamimune, Cutter Biological, Berkeley, California) in a dose of 400 mg/kg per day for five consecutive days as six-hour infusions. Two of the six children had undergone splenectomy but the other four had not. Three of six children had a good or excellent response to the first five-day course of intravenous gamma globulin. The peak platelet count occurred within 12 days of the start of therapy in all. All three have required booster doses of intravenous gamma globulin to maintain platelet counts at a safe level. All children had marked increases in serum IgG following intravenous gamma globulin, except one who had undergone splenectomy and who had chronic idiopathic thrombocytopenic purpura with high baseline levels of immunoglobulin G (IgG). The significance of the observed increase in platelet-associated IgG during treatment is not clear. No untoward reactions necessitating cessation of therapy were encountered during this study. Our short-term observations in six children with chronic idiopathic thrombocytopenic purpura indicate that high-dose intravenous gamma globulin is an effective form of treatment for certain children with this condition.

Adolescent↗
PubMedDate not supplied

Factor IX inhibitors and anaphylaxis in hemophilia B.

PURPOSE: We present clinical and laboratory data on 18 children from 12 hemophilia treatment centers in the United States, Canada, and Europe with the purpose of disseminating information regarding a recently recognized, potentially life-threatening complication of treatment in very young children with hemophilia B. PATIENTS AND METHODS: Twelve hemophilia centers from the United States, Canada, and Europe provided clinical information and laboratory data concerning 18 children who had severe allergic reactions to infused factor (F) IX in close association with the development of an inhibitor to FIX. Laboratory testing for establishment of the diagnosis of hemophilia B and inhibitor to FIX was done locally at the centers treating these patients. FIX gene analysis was performed at one of six molecular genetics institutes. RESULTS: All 18 children had severe hemophilia B, and in each an inhibitor antibody to FIX developed. The median age at the time of anaphylaxis (or anaphylactoid reaction) was 16 months, and the median number of exposure days to FIX was 11. The FIX inhibitor was detected almost simultaneously with the first occurrence of anaphylaxis in 12 of 18 patients. Maximum inhibitor titers were 4.5-600 Bethesda units (BU), with a median titer of 48 BU. FIX gene analysis, performed in 17 of 18 patients, demonstrated complete deletion of the FIX gene in 10 and major derangements in seven. Immune tolerance induction (ITI) regimens have been attempted in 12 patients, with generally poor responses. Two of the 12 experienced nephrotic syndrome while on ITI. Recombinant FVIIa has been successfully used to treat bleeding episodes in 11 of these children. CONCLUSION: Physicians treating young children with hemophilia B should be aware of the potentially life-threatening complication of anaphylaxis. Children with complete gene deletions or major derangements of the FIX gene appear to be at greater risk. Those identified by genotype as being at greater risk may need to receive their first 10-20 treatments in a medical facility equipped for handling such emergencies. Recombinant FVIIa, although not licensed for use in the United States, appears to be the most suitable treatment option for bleeding episodes in such patients.

Anaphylaxis↗
PubMedDate not supplied

Safety and efficacy of low-dose intravenous immune globulin (IVIG) treatment for infants and children with immune thrombocytopenic purpura. Low-Dose IVIG Study Group.

PURPOSE: This report presents pooled data from two multicenter studies conducted to assess the efficacy, safety, and tolerance of lower-dose intravenous immune globulin (IVIG) regimens of 250 mg/kg/day, 400 mg/kg/day, and 500 mg/kg/day for 2 days, compared to an established higher-dose regimen of 1 g/kg/day for 2 days, in children with immune thrombocytopenic purpura (ITP). PATIENTS AND METHODS: A total of 24 children received IVIG (Gammar i.v.). In Study 1, 10 centers enrolled 12 children between 5 and 12 years old who received IVIG at either 400 mg/kg/day or 1 g/kg/day for 2 days. In Study 2, five centers enrolled 12 infants and children younger than 5 years old who received IVIG at 250 mg/kg/day or 500 mg/kg/day for 2 days. Both studies were prospective and randomized. RESULTS: IVIG treatment was effective (platelets increased at least 30,000/cu mm over baseline) in 94% (16 of 17) of the evaluable patients in the low-dosage group. Platelet increases occurred rapidly: by 48 hours, total platelet counts ranged from 32,000/cu mm to 256,000/cu mm, and peak platelet counts reached 38,000/cu mm to 551,000/cu mm. Adverse events (AEs) were most often mild, lasted less than 3 hours, and were usually those typically associated with immunoglobulin administration-headache, nausea, vomiting, and fever. There were two serious AEs-an anaphylactoid reaction in one patient in the 400 mg/kg group and aseptic meningitis in one patient in the 1 g/kg high-dosage group. Both patients recovered without sequelae and were responders. Although the incidence of AEs varied by dosage groups, this difference was not significant. However, the incidence of AEs was affected by age. AEs were significantly lower in patients younger than 5 years of age. CONCLUSIONS: In this small, randomized trial, low-dose IVIG in 2-day regimens of 250, 400, or 500 mg/kg/day rapidly reversed thrombocytopenia just as effectively as 1 g/kg/day in infants and young children with ITP. Lower-dosage regimens are safe and well-tolerated; the incidence of AEs is lower in children younger than 5 years of age.

Child↗
PubMedDate not supplied

Cardiac rhythm abnormalities during intravenous immunoglobulin G infusion for treatment of thrombocytopenia.

PURPOSE: Several side effects of intravenous immunoglobulin G (IVIG) therapy are known, but it has never been reported to be associated with cardiac rhythm abnormalities other than sinus tachycardia. PATIENTS AND METHODS: We describe the development of cardiac dysrhythmias during intravenous immunoglobulin G infusion in two children with thrombocytopenia. One of the patients had a history of supraventricular tachycardia, and the other had evidence suggestive of preexisting long QT syndrome. CONCLUSION: Cardiac rhythm abnormalities may be exacerbated in individuals with preexisting cardiac problems by IVIG infusion, and such patients should be monitored closely during IVIG administration.

Arrhythmias, Cardiac↗
PubMedDate not supplied

Activated recombinant human coagulation factor VII therapy for intracranial hemorrhage in patients with hemophilia A or B with inhibitors. Results of the novoseven emergency-use program.

Activated recombinant human coagulation factor VII (rFVIIa) is a promising new therapeutic agent for patients with hemophilia A or B with inhibitors who experience serious bleeding episodes or who need coverage during surgical procedures. This open-label, uncontrolled, emergency-use study evaluated the efficacy and safety of rFVIIa in 11 hemophiliac patients and 1 FVII-deficient patient with life-threatening intracranial hemorrhage previously unresponsive to one or more alternative therapies. rFVIIa effectively controlled intracranial hemorrhage in 10 of the 12 patients. Patients with hemophilia A or B received an average of 96.9 rFVIIa injections over 14.7 days with a mean total administration of 153.3 mg, corresponding to 8.1 mg/kg. Most reported adverse events were considered to be unrelated to rFVIIa therapy. These findings suggest that rFVIIa is an effective and well-tolerated therapeutic option in the management of central nervous system bleeding in patients with hemophilia A or B with inhibitors.

Adult↗