Biomedical subjects

R Kerbl

Publications and source records attributed to R Kerbl.

62 records · Page 4Linked to original sources

PubMedDate not supplied

[Bone pain after long distance flight as first manifestation of HbSC disease].

A 7-year old Ghanese boy was admitted with repeated bone pain. Multiple bone marrow alterations were seen as the causal factor. HbSC disease was diagnosed by hemoglobin electrophorics. The onset of pain attacks appears to be linked to the long distance flight from Ghana to Austria. Due to severe bone marrow affection, beside symptomatic therapy a long-term treatment with hydroxyurea was started, leading to an HbF increase from 5.1 % to 19.3 %. After a follow-up of 8 months the patient has remained free of complaints.

Aircraft↗

PubMedDate not supplied

[Ceftriaxone-induced cholelithiasis--a harmless side-effect?].

43 children suffering from borreliosis, meningitis and septicemia were treated with ceftriaxone. A six year old boy with acute jaundice due to ceftriaxone induced cholelithiasis encouraged us to reevaluate the frequency of ceftriaxone induced cholelithiasis and its' sequelae in children in a prospective study. Out of 43 children (age 6.3 years, 4 months to 16 years, male: female 25:18), 20 children (46.5%) showed sonographical evidence for ceftriaxone induced cholelithiasis after a treatment of at least 10 days. Two of them even had signs of intrahepatic cholestasis, 3 kids suffered from severe abdominal pain, non of them showed serologic abnormalities. Another 5 children (11.6%) had sludge in the gallbladder without evidence for cholelithiasis. In all patients the "pseudocholelithiasis" spontaneously resolved within at most 2 months. We suggest a sonographical examination of the gallbladder at the end of the ceftriaxone treatment in order to detect cholelithiasis, which might call for further monitoring and maybe dietary treatment.

Adolescent↗

PubMedDate not supplied

The influence of maximum supportive care on dose compliance and survival. Single-center analysis of childhood acute lymphoblastic leukemia and non-Hodgkin's-lymphoma treated within 1984-1993.

BACKGROUND: The administration of intensive, risk-adapted multiagent chemotherapy has markedly improved the event-free survival in childhood acute lymphoblastic leukemia and Non-Hodgkin's lymphoma. Current treatment protocols may achieve complete remission rates of more than 95% and event-free survival rates of approximately 70% in non-B-acute lymphoblastic leukemia/non-B-Non-Hodgkin's lymphoma patients. Due to a higher proliferation rate and aggressiveness of B-cells treatment of B-acute lymphoblastic leukemia/B-Non-Hodgkin's lymphoma must be more intensive, but can be shorter to attain similar event-free survival rates. METHODS: Between 1984 and 1993 79 primarily admitted patients up to 18 years of age (range 0.6-17.9 years) received therapy according to the treatment protocols ALL A 84, ALL 86, NHL 86, ALL 90 and NHL 90 of the Berlin-Frankfurt-Münster Study Group. Intensive phases of treatment were given on an inpatient basis with maximum supportive care in order to achieve the prescribed doses. RESULTS: Median follow-up time from diagnosis is now 8 years (range 40 to 157 months) with a probability of event-free survival of 92% for the entire group. CONCLUSION: Multiagent chemotherapy tailored to the individual risk profile of each patient but given in the prescribed dose is currently the mainstay to achieve high complete remission rates with the aim to cure most acute lymphoblastic leukemia/Non-Hodgkin's lymphoma patients. The administration of intensive supportive care is important for the achievement of complete drug dosage and for the reduction of therapy-related toxicity.

Adolescent↗

PubMedDate not supplied

[Value of prognostic factors in the Austrian A-NB87 Neuroblastoma Study].

AIMS: A multivariate analysis was performed to evaluate the impact of various prospectively evaluated risk factors. PATIENTS AND METHODS: From January 1987 to December 1993, 120 patients were registered in the study. 108/120 patients were eligible. There were 49 girls and 59 boys with a median age at diagnosis of 14 months (range, 0 to 224 months). Patients were classified according to the Evans classification system. LDH, NSE, Ferritin, N-myc amplification, 1p-deletion and ploidy were evaluated at diagnosis. Treatment intensity was based on the results of primary surgery: surgery only for 22 (20%) stage I and IIA patients (macroscopic residue without lymph node involvement), the 9 IIB patients (8,5%) (macroscopic residue with lymph node involvement) had mild chemotherapy in addition (6 x VCR/CYC) and elective radiation (Rx). Stage III patients were divided into 2 groups: IIIA patients (n = 17/16%) had to have ferritin levels under 300 micrograms/ml, NSE lower than 100 ng/ml and age below 2 years at diagnosis and received 6 alternating cycles of DAMO/ MVDOC. If one of these three criteria was not fulfilled, patients were assigned to the more intensive treatment arm stage IIIB (n = 12/11%), i.e. 9 alternating cycles of DAMO/MVDOC/IPE. Stage IV pts (n = 35/32.5%) received 8 MVDOC/IPE cycles and 20 patients received megatherapy followed by stem cell reinfusion in addition. 13 stage IVs patients (12%) were registered and had elective VCR/CYC and/or liver radiation in case of poor clinical condition. The median observation time is 4.2 years (range, 1 to 7.5). RESULTS: The survival rate at 3 years was excellent for localized disease and stage IVs with survival rates of 100% for stage I/IIA and 92% for stage IVs. Stage IIIA patients had an EFS rate of 81% whereas stage IIB patients achieved only 69%. Stage IV patients reached 51%, however outcome was especially poor for stage IIIB patients (20%) due to treatment related toxicities. The toxic death rate in the study was 13% (2 surgical deaths, 8 infections, 4 multiple organ failures). Univariate analysis demonstrated the following significant unfavorable risk factors: age over 1 year at diagnosis (58/108 pts, p = 0.006), NSE > 100 ng/ml (26/95 pts, p < 0.0001), Ferritin > 300 ng/ml (19/98 pts, p = 0.007), LDH > 300 (400) U/L, 51/87 pts, p = 0.004), presence of N-myc amplification (17/59 pts, p = 0.001), deletion of the short arm of chromosome 1 (19/74 pts, p < 0.0001) and di/tetraploidy (32/72 pts, p = 0.008). The power of these factors was even stronger in patients with localized disease whereas no significant prediction was observed for stage IV patients. Furthermore a significant correlation of the serological (NSE, ferritin, LDH) and biological factors (N-myc, deletion 1p, di/tetraploidy) was detected in this study. Only NSE was identified as an independent prognostic factor (p = 0.018) whereas no independent factor could be identified within the 3 biological parameters due to their high correlations (Kendall's tau for N-myc and deletion 1p:0.7). However, N-myc (p = 0.005) as well as deletion 1p (p = 0.01) were found significantly more important than di/tetraploidy. CONCLUSIONS: Biological classification of neuroblastomas should be mandatory and be the prerequisite for any risk adapted treatment. One serological and 2 biological factors could be a good standard evaluation to identify neuroblastoma patients at risk.

Adolescent↗

PubMedDate not supplied

Treatment of children with anagrelide for thrombocythemia.

PURPOSE: Essential thrombocythemia (ET) is a rare myeloproliferative disease characterized by hyperproliferation of megakaryocytes and persistent elevation of platelets. Major complications are thrombosis, bleeding, and microvascular occlusive symptoms, sometimes resulting in serious morbidity. In patients with platelet counts > 1000 x 10(9)/l, cytoreduction is important to reduce the risk for thrombotic or hemorrhagic events. However, the frequent side effects and the carcinogenic potential of many cytoreductive agents limit their use, especially in younger patients. Anagrelide, a noncarcinogenic agent with moderate toxicity, has been successfully used in adult patients with ET, but experience with anagrelide in pediatric patients with ET is limited. PATIENTS AND METHODS: Three children who received anagrelide (Induction dose 1 mg/day, maintenance dose 1 to 2.5 mg/day) as therapy for ET are described. RESULTS: All children had a rapid and marked decline of their platelet counts, and only one child developed mild and transient abdominal side effects. CONCLUSIONS: Treatment with anagrelide seems to be a promising approach for children with ET. It should be considered as first-line therapy because of its selective activity against platelet production, tolerable toxicity, suspected negligible cancerigenic effect, and the possibility of oral administration.

Adolescent↗

PubMedDate not supplied

Portal vein thrombosis in a 17-year-old female adolescent with essential thrombocytosis.

A 17-year-old girl known to suffer from essential thrombocytosis (ET) was admitted with severe abdominal pain that was due to sonographically verified portal and hepatic vein thrombosis. ET is a well-documented disease and portal vein thrombosis has often been reported in adults but not in children and adolescents suffering from ET. Coincidence of ET, urinary tract infection, oral contraception, and cessation of acetylsalicylic acid therapy might be responsible for the early manifestation of portal and hepatic vein thrombosis in our patient.

Adolescent↗

PubMedDate not supplied

[The Graz SIDS Risk Questionnaire: prospective study of 6,000 infants].

In a prospective study (1988-1990) the SIDS risk questionnaire SRFB was applied to 6000 infants born in the Department of Obstetrics, University Hospital Graz. In all infants at an increased statistical risk for SIDS a standardized intervention including pediatric and polysomnographic investigations was performed. As a result the SIDS incidence decreased from an average of 1.92 per thousand lifeborns (1984-1988) to 0.83 per thousand lifeborns (1989-1990) in our test group, which means a striking reduction of the SIDS incidence by 57%. In addition to this effect, the intensified information of parents and physicians about possible signs of an increased risk for SIDS lead to a general reduction of SIDS in Styria.

Austria↗

PubMedDate not supplied

Neuroblastoma screening: labeling of HVA and VMA for stable isotope dilution gas chromatography-mass spectrometry.

Neuroblastoma is a curable tumor if detected early enough. Therefore, a screening test is performed in Austrian infants by analyzing urine samples collected and dried on filter strips. Screening is performed by measuring homovanillic acid (HVA) and vanillylmandelic acid (VMA) using the enzymatic immunoassay chemistry (EIA) method and reexamining the elevated values by high-performance liquid chromatography. Both methods, however, give a relatively high number of false-positive results. Therefore, the positives are reexamined by stable isotope dilution (SID) gas chromatography-mass spectrometry (GC-MS), which is the most sensitive and precise method in clinical chemistry. The labeled substance is added as an internal standard to the sample; it has nearly identical chemical and physical properties as the natural analogue, undergoes the entire preparation step, and is detected simultaneously with the natural analogue by the same detector, namely the mass spectrometry, given by a difference in molecular weight. All loss is compensated in this way by the final calculation. At present, HVA but not VMA can be purchased labeled with deuterium, so we started with our method using deuterated HVA as the internal standard for both HVA and VMA. Because the coefficient of variation is not sufficient for VMA, we try to label VMA via different labeling procedures; for example, 1) exchange of the aromatic H against D by incubation with DCI or in alkaline R-OD or LiOD or 2) exchange of phenolic 16OH against 18OH by reaction with H2(18)O in an acidic environment and at high temperatures. Carboxylic 16O can be exchanged against 18O.

Austria↗