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Biomedical subjects

R Gera

Publications and source records attributed to R Gera.

12 recordsLinked to original sources
PubMed2001

Successful treatment of high titer inhibitors in mild hemophilia A with avoidance of factor VIII and immunosuppressive therapy.

We describe two patients with mild hemophilia A (MHA) who developed high titer inhibitor (HTI) following intensive recombinant factor VIII (rFVIII) concentrate replacement for surgery and trauma. Intranasal desmopressin was instituted shortly following immunosuppressive therapy (IST) and activated prothrombin complex concentrate (APCC) in one case, and following APCC alone in the second case. Avoidance of factor VIII (FVIII) coupled with intranasal desmopressin prophylaxis three times a week resulted in undetectable inhibitor levels. Both patients have had no further bleeding episodes and have been maintained on desmopressin prophylaxis prior to activity for the past 2 to 3 years. Recombinant factor VIIa (rFVIIa) was used successfully prior to a second surgery in one patient without complication.

Adolescent↗
PubMed2000

[Paranasal sinus mycoses].

In recent years there has been a marked increase in mycosis infections of the paranasal sinuses, attributed both to an increase in the survival of subjects at risk and improved diagnostic techniques (endoscopy, CT, MR) able to identify cases which had previously gone unrecognized and treated as aspecific chronic sinusitis. The present study involves 45 cases (4.3%) out of a total of 1050 patients who had undergone endoscopic surgery for sinusopathy between April 1994 and December 1998. Following the Katzenstein classification, the cases were broken down into non-invasive chronic mycoses or fungus ball (34 cases), allergic mycoses (7), chronic indolent invasive mycoses (3) and fulminating invasive mycosis (1 case). The mycetes most often involved was Aspergillus Fumigatus (76.9%). The recurrent symptom was facial algia, followed by nasal obstruction. Paranasal sinuses endoscopy did not modify the specific picture. CT presented such indicative signs as focal areas with non-homogeneous intensity, images of metal-like foreign bodies and endosinus calcifications in 84.4% of the cases. MR--performed in only 6 cases--always presented T2 images showing the typical signal void area corresponding to pathological lesions. All patients underwent endoscopic surgery of the paranasal sinus. The effectiveness of this treatment differed according to the clinical form. In the fungus balls surgery always resolved the pathology without requiring subsequent pharmacological treatment. In allergic mycosis, surgery improved the symptom of nasal respiratory obstruction but local drug treatment was required. With the aid of drug treatment, surgery resolved chronic indolent invasive mycoses and prevented the endocranial progression of complications. In the cases of fulminating invasive mycosis, timely surgery prevented the onset of endocranial complications and made it possible to perform antimycotic polychemotherapy to control the disease. This experience shows how important a protocol involving several different tests is in diagnosing the many clinical forms of paranasal sinus mycoses and distinguishing them from sinusopathies. Endoscopic surgery is indicated for all forms of paranasal sinus mycoses although the realistic objectives differ according to type.

Adolescent↗
PubMed1997

Physiological and biochemical characterization of intergeneric hybrids of thermotolerant and non-thermotolerant yeasts.

Kluyveromyces-like intergeneric hybrids of thermotolerant Kluyveromyces marxianus and non-thermotolerant Saccharomyces cerevisiae, produced in a previous study by protoplasmic fusion, have been characterized. On molasses, these strains produced ethanol in excess of 6% (v/v) both at 30 and 45 degrees C as against 3% and 4.2% (v/v) by the former parent at 30 and 45 degrees C, respectively. In hybrids, the increase in ethanol production appeared to be a sequel to increased activities of alcohol dehydrogenase and pyruvate kinase, derived probably from S. cerevisiae parent. Hybrid ADH-isozyme pattern on polyacrylamide gel corroborated the presence of S. cerevisiae ADH in the tested hybrids. Regression analyses indicated a positive correlation between ethanol production and ADH or PK or both (r approximately 0.76-0.84).

Hybridization, Genetic↗
PubMed1993

Serologic markers of viral hepatitis A, B, C, and D in patients with hemophilia.

Forty-one patients with hemophilia A were studied for the prevalence of serological markers for hepatitis A, hepatitis B, hepatitis C (non-A and non-B hepatitis), and delta hepatitis (hepatitis D). Ten of 41 (24.4%) patients demonstrated hepatitis A antibody and 31 of 41 (75.6%) patients had a serologic marker for previous hepatitis B infection; four of these 31 patients (13%) also demonstrated antibody to delta agent (hepatitis D). Thirty-seven of 41 (90.2%) patients demonstrated antibody for hepatitis C. Nine of 31 (29%) patients with a hepatitis B marker (no hepatitis B vaccinees) were negative for anti-HBc but positive for anti-HBs; all of these nine patients were HIV antibody positive, although they had no overt immunodeficiency. Twenty-six of 41 (63.5%) patients were HIV antibody positive. Of HIV antibody positive patients, 27%, 88%, and 100% demonstrated evidence of a previous hepatitis A, hepatitis B, or hepatitis C, respectively. Of HIV antibody negative patients; 20%, 53%, and 73% of the patients demonstrated evidence of a previous hepatitis A, hepatitis B, or hepatitis C infections, respectively. The difference between HIV antibody positive and HIV antibody negative groups was not significant for hepatitis A but was significant for hepatitis B (P < 0.001) and hepatitis C (P < .001). Of the 31 patients with a hepatitis B serologic marker, all had antibody to hepatitis C. Of 10 patients, without a hepatitis B serologic marker, only 6 (60%) had antibody to hepatitis C.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

Polyfungal systemic infections in pediatric oncology patients.

Three cases of two fungal agents causing simultaneous systemic infection in immunocompromised pediatric patients are presented and the literature is reviewed. All three patients had several underlying factors that predispose to systemic fungal infections. A species of candida was identified initially as an etiologic agent in all of the three patients causing subcutaneous abscesses, urinary tract infections, fungemia, catheter exit site infection, or pneumonia. However, a few days later blood cultures grew aspergillus species in two of the three patients; in the third patient aspergillus was identified on microscopic examination of the spleen. All three patients had an associated bacteremia with either Staphylococcus aureus or S. epidermidis requiring vancomycin therapy. Presence of aspergillus infection required treatment with amphotericin. Difficulties in making a definitive diagnosis of systemic fungal disease may explain paucity of reports in the literature with simultaneous polyfungal systemic infection.

Adolescent↗
PubMed1990

Immunoglobulins in hemophilia: correlation between IgG subclasses, IgE and HIV antibody.

Twenty-two patients with hemophilia who had received factor VIII concentrate were evaluated. Only 12 of 22 were seropositive for HIV. Elevation of IgG, IgG1, IgM and IgE was not related to HIV seropositivity. Means of IgG1, IgG2, and IgG3 were significantly higher in patients with elevated IgG. Means of IgG2 and IgG3 were significantly lower in patients with elevated (greater than or equal to 251) serum IgM. Seven of the 22 patients demonstrated elevated (greater than or equal to 100) serum IgE; mean serum IgA was significantly lower, though in normal range, in these seven patients. Alterations in serum immunoglobulins in patients with Hemophilia are frequently seen, however, like other immune-dysfunction in these patients, these abnormalities can not be attributed to their HIV status.

Adolescent↗
PubMedDate not supplied

Central venous catheter infections in pediatric patients--in a community hospital.

We reviewed the records of 23 pediatric patients who had received at least one central venous catheter during a two-year period. Nine patients had acute lymphoblastic leukemia (ALL), nine had other hematologic/oncologic diagnoses, and five had cystic fibrosis. Twenty-nine of 65 febrile episodes in 16 patients were associated with a catheter-related infection. Twenty of 40 catheters were associated with an infection over a period of 7,229 catheter days. For every 1,000 catheter days, four episodes of infections were observed. The number of infections/1,000 catheter days, the average life of a catheter (approximately equal to 180 days), and mean number of days elapsing before the first infection were not significantly different in the three diagnostic groups. Broviac catheters were used most often (24/40), followed by Quinton (9/40) and Port-a-Cath (7/40). Broviac catheters lasted twice as long (224 days, p less than 0.01) as Quinton and Port-a-Cath. Gram-positive cocci were isolated most frequently and Staphylococcus epidermidis was the most common pathogen. No consistent relationship between an absolute neutrophil count of less than 1,000/mm3 and infection with gram-positive cocci was seen. However, seven of eight episodes of gram-negative bacillary infections occurred in patients with an absolute neutrophil count of less than 1,000/m3 (p less than 0.005). Those patients who were not considered terminally ill responded well to antimicrobials. Catheter removal was necessary in only two instances.

Bacterial Infections↗
PubMedDate not supplied

Venous thromboembolism.

Incidence of venous thromboembolism (VTE) in children is rapidly rising. Frequent use of central venous lines (CVLs) in children with a variety of disease processes has contributed to increased incidence of VTE. In addition, increased detection of VTE has been possible due to the availability of sensitive imaging studies such as Color Duplex/Doppler ultrasound, lineogram and venogram. Heightened awareness of congenital prethrombotic disorders may help identify children at higher risk of thrombosis. Anticoagulation with heparin, low molecular weight heparin (LMWH) and oral anticoagulant such as warfarin are used for the treatment of VTE. Thrombolytic therapy may be indicated in some cases. Clinical presentation, management and pathogenesis including role of congenital prethrombotic disorders in pediatric VTE are discussed.

Anticoagulants↗
PubMedDate not supplied

Pediatric transfusion therapy: practical considerations.

Over the past decade, safety of blood has increased tremendously because of better donor screening as well as testing of the units for transmissible diseases. Component therapy has allowed more effective and economic use of blood. Whole blood is rarely used; instead, packed red cells, platelets, and fresh frozen plasma (FFP) are the most common components used. These products are further refined using irradiation and microaggregate filters and in the case of FFP, viral inactivation. Irradiation prevents transfusion-associated graft versus host disease, whereas microaggregate filters remove leukocytes, decreasing the rates of alloimmunization, febrile nonhemolytic (FNH) reactions, and cytomegalovirus (CMV) transmission. Autologous donation in older children probably provides the safest blood as far as transmissible diseases are concerned. More families request a directed donation and solicit physician help in deciding as well as making arrangements for autologous and/or directed donations. Transfusions of blood and blood components in children are often challenging and require a knowledge of physiologic changes in hemoglobin and blood volumes during different ages. The unique needs of neonates, immunocompromised patients, and patients with congenital hemolytic anemia (sickle cell, thalassemia) mandate that the pediatrician have an appropriate knowledge of transfusion volumes and choice of blood product as well as indications for transfusion.

Adolescent↗
PubMedDate not supplied

Penicillin-resistant Streptococcus mitis as a cause of septicemia with meningitis in febrile neutropenic children.

PURPOSE: The purpose of this report is to emphasize the importance of occurrence of Streptococcus mitis meningitis in febrile neutropenic children with hematopoietic malignancy. PATIENTS AND METHODS: Symptoms of meningitis and sepsis (fever, headache, changes in mental status) were seen in three patients who were severely neutropenic and undergoing cytotoxic chemotherapy for CNS relapse of their underlying malignancy (acute lymphoblastic leukemia (ALL), n = 2; Burkitt's lymphoma, n = 1). Chemotherapy had included cytosine arabinoside administered 7-14 days prior to presenting with sepsis and meningitis. All three patients had buccal mucositis or sinusitis. Blood cultures and CSF cultures showed S. mitis resistant to penicillin but sensitive to vancomycin. Vancomycin, at a dosage of 60 mg/kg/day to maximize CNS levels of antibiotic, was administered to all three children. RESULTS: Two of the patients recovered from S. mitis meningitis; recovery was associated with an improvement in their peripheral granulocyte counts. One patient, who remained neutropenic, died despite being treated with both intravenous and intraventricular vancomycin. CONCLUSION: Physicians caring for patients who are neutropenic and febrile need to be aware of the risk of meningitis occurring with S. mitis sepsis. Early treatment with high dosages of vancomycin (60 mg/kg/day) and an attempt to limit the duration of neutropenia are important factors in the outcome of such patients.

Bacteremia↗
PubMedDate not supplied

Nondisclosure of human immunodeficiency virus and hepatitis C virus coinfection in a patient with hemophilia: medical and ethical considerations.

This article discusses a medical and ethical dilemma: whether to disclose a positive HIV (human immunodeficiency virus)/HCV (hepatitis C virus) coinfection to an adolescent boy without symptoms with hemophilia despite the objections of his parents. An actual case history is presented and the dilemma faced by the medical team is discussed. Numerous family conferences, all excluding the patient, held during the last 5 years discussed the medical team's obligation for full disclosure, the emerging autonomy of the patient, and the potential for medical disaster (e.g., HIV transmission) if full disclosure were not permitted. Despite this, the family did not agree to allow disclosure. The patient and parents assured us of his sexual inactivity. Legal opinion was sought from the university counsel. The dilemmas are multiple. Is there a convincing argument to insist on disclosure of these facts to this patient, particularly when there is ambiguity regarding the appropriateness of HIV and HCV treatment? Does the ethical argument that he is at potential risk for transmitting HIV/HCV outweigh the rights of the family? What are the rights of the rest of the family? What are the rights of the minor? Is it our ethical responsibility to disclose a probably fatal diagnosis?

Adolescent↗