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Biomedical subjects

J Rajantie

Publications and source records attributed to J Rajantie.

At least 37 records · Page 2Linked to original sources
PubMed1992

Elevated HbA1 due to foetal haemoglobinaemia in children. A common artifact of certain ion-exchange chromatography methods.

Foetal haemoglobin (HbF) is known to elute with glycosylated haemoglobins (HbA1) in mini-column ion-exchange chromatography, thus producing falsely elevated values for HbA1 in subjects with foetal haemoglobinaemia. We studied the occurrence of this methodological artifact in 266 patients with suspected or known insulin-dependent diabetes mellitus (IDDM) and in 205 healthy children of various age groups. HbA1 was measured using ion-exchange chromatography with prefabricated mini-columns and HbA1c and HbF by high-performance liquid chromatography (HPLC). In healthy children of over 5 years of age, the HbA1 values were usually within the reference limits for adults. However, 69% of the children younger than 1 year of age and 7% of those 1-5 years of age had HbA1 levels above 10%. Their HbA1c values were normal but the HbF values were high. Six of the 223 patients with IDDM (2.7%) and three of the 43 patients with stress glucosuria (7%) had much higher HbA1 than HbA1c values. The discrepancy was again related to the presence of raised concentrations of HbF. We conclude that foetal haemoglobinaemia is more prevalent than was previously known, especially among children younger than 5 years of age. Presence of HbF will bias the use of HbA1 as a diagnostic aid or as an index of metabolic control in children with IDDM. Methods specific for HbA1c, such as HPLC, should be used in paediatric patients.

Adolescent↗
PubMed1992

Anaemia and macrocytosis--unrecognized features in cartilage-hair hypoplasia.

Cartilage-hair hypoplasia is an autosomal recessive osteo-chondrodysplasia which results in short stature, sparse hair and impaired cell-mediated immunity. In a study of 88 Finnish patients we found episodes of anaemia and/or macrocytosis during childhood in 86% of the patients. The reticulocyte index was always low in relation to anaemia. Bone marrow examination revealed decreased erythropoiesis in six of eight anaemic patients studied. Anaemia was most prevalent and severe during infancy. Spontaneous recovery occurred before adulthood in all patients except in three infants with fatal hypoplastic anaemia. Sixty-two percent of the patients had had lymphopenia and 24% neutropenia. Presence of anaemia significantly correlated to severity of immunodeficiency and growth failure and to presence of neutropenia. Disordered erythrogenesis is an integral feature of cartilage-hair hypoplasia and may, together with growth failure and immunodeficiency, reflect a generalized defect in cellular proliferation.

Adolescent↗
PubMed1992

White blood cells in infants with congenital toxoplasmosis: transient appearance of cALL antigen on reactive marrow lymphocytes.

Existing serological methods for diagnosing congenital toxoplasmosis are inadequate. We have therefore characterized the features of peripheral blood cell counts and smears in a prospective study of 8 affected infants during their first year of life. Enlarged, vacuolated lymphocytes were observed in all patients. Six patients had periods of neutropenia; in 1 patient the neutropenia was severe and prolonged. A bone marrow aspirate from this patient showed enhanced myelopoiesis, and after stimulation with hydrocortisone, but not with adrenaline, his neutrophil count normalized. Phenotyping of his bone marrow cells revealed that approximately 55% of both the immature and mature B lymphocytes were positive for the cALL antigen. Of the 8 patients, 6 had mild eosinophilia, 4 had mild monocytosis and 3 had increased numbers of plasma cells in the peripheral blood. These findings may reflect the activity of the congenital toxoplasma infection and may thus contribute to the diagnosis, particularly in patients with silent serology.

Antigens, Differentiation↗
PubMed1991

Perceptions of problematic events and quality of care among patients and parents after successful therapy of the child's malignant disease.

Fifty-three children (mean age 6.4 at diagnosis, 12.8 years at completion of questionnaires) identified as having acute lymphoblastic leukaemia (48) or non-Hodgkin lymphoma (5) during the 11-year period of 1976-1986 participated in the present study. Patients' and parents' perceptions of malignancy-related changes in significant relationships, of treatment-related problematic events, as well as of the quality of care provided during induction and maintenance-therapy were assessed using questionnaires. The results indicated mainly positive changes in significant relationships during the chemotherapy. However, concurrent stressful life events affected the relationships adversely. Alopecia (hair loss) was the most problematic disease-related event for the patient and the patient's anorexia for the parents. Patients' complaints were most often about the quality of care during induction, namely pain, fear and insufficient information. Parents' complaints were about the lack of continuity in the staff-patient relationship during maintenance-therapy.

Attitude to Health↗
PubMed1991

Aggression: the dominant psychological response in children with malignant disease.

During the 11-yr. period of 1976 to 1986 leukemia or lymphoma treatment at the Children's Hospital, University of Helsinki was electively discontinued for the children in 90 different families. Of the 53 (59%) patients (mean age 6.4 yr. at diagnosis and 12.8 yr. at completion of questionnaires) who agreed to participate in the present study, 48 had acute lymphoblastic leukemia and five nonHodgkin lymphoma. Patients' and parents' impressions of the patients' psychological reactions during patients' prior chemotherapy were evaluated on parental and self-ratings. Also, knowledge of and presumed causes of the malignancy were studied. Patients' reactions of aggression, depression, eating disorders, hypersensitivity, phobic anxiety, death anxiety, and night terror were examined using factor analysis. Aggression, in the form of irritation and anger, was displayed more often by girls than by boys. Patients of families suffering from stress were prone to exhibit aggression in the form of mood changes, irritation, and anger. Patients with disease-related knowledge, as opposed to those less well informed, were less depressed. Discrepancies between parents' and patients' thoughts about the origin of the malignancy were noted.

Adaptation, Psychological↗
PubMed1990

Irradiation cataract in children after bone marrow transplantation.

Chemotherapy and radiation produce a dose-dependent anti-leukemic effect. Combined chemoradiotherapy and bone marrow transplantation (BMT) were given in our clinic to treat children with acute leukemias. Total body irradiation of 10 Gy in a single dose was used. One long-term side effect of this treatment was the development of subcapsular cataract; this was seen in all nine long-term survivors of the 17 children with acute lymphoblastic (ALL) or acute myelogenous (AML) leukemia who were treated as described above. One year after marrow transplantation, all the eyes studied had visual acuity of 20/20 and an optically clear lens. Three years later, 60% of the eyes had visual acuity of less than 20/40 and all had posterior, subcapsular cataracts. The cataract in all cases was quite uniform, consisting of opacities in the posterior subcapsular region. Cataract formation was treatment-related and seemed to correlate only to the type of total body irradiation. We concluded that the cataracts seen in the present study were a late complication of allogeneic BMT and were specifically due to the single-dose total body irradiation.

Adolescent↗
PubMed1990

Fibronectin concentration in cerebrospinal fluid reflects early central nervous system involvement in children with acute lymphoblastic leukemia.

We measured concentrations of fibronectin (FN) in the cerebrospinal fluid (CSF) in long-term follow-up patients with acute lymphoblastic leukemia (ALL). In 11 patients with neuroleukemia the CSF-FN level was elevated already at the time of diagnosis of ALL, 3.8 +/- 0.6 mg/l, increased during therapy to 4.7 +/- 0.5 mg/l, and at the time of concurrent blast cell finding it was 5.5 +/- 1.0 mg/l. In 11 patients with no subsequent CNS leukemia, the mean CSF-FN level was 2.4 +/- 0.6 mg/l at the time of diagnosis of ALL and 2.8 +/- 0.6 mg/l during therapy, and increased to 3.2 +/- 0.8 mg/l. The neuroleukemia rate was 43% in patients with initial CSF-FN levels greater than 2 mg/l, compared with 5% in patients with CSF-FN levels less than or equal to 2 mg/l (p less than 0.005) in a group of 45 long-term follow-up patients with ALL. Regression analysis on the 21 clinical or laboratory parameters studied showed that the only variable independently associated with CSF-FN was the total protein concentration in the CSF; this, however, explained only 14% of the observed variation in the CSF-FN concentration and did not show any correlation with CNS involvement. We conclude that the CSF-FN test at diagnosis of ALL showed significant differences between groups of patients with and without CNS leukemia, and may prove to be a new early marker for neuroleukemia.

Analysis of Variance↗
PubMed1990

Alloimmune granulocytopenia in three newborn infants of two families.

Two infants of one family and one infant of another with neonatal alloimmune granulocytopenia are described. All of the infants had mild infections, two of them mild pyogenic skin infections. Total leukocyte counts were within the normal range in all cases. The absolute neutrophil count was 0-0.2 x 10(9)/l and remained subnormal up to nine weeks. Both mothers had antibodies against their husbands' granulocytes. Detection of the antibody made it possible to postpone more invasive examinations. Parents should be examined if neutropenia in a newborn infant cannot be attributed to a normal cause such as an infection.

Agranulocytosis↗
PubMed1990

School absences, school achievements and personality traits of the haemophilic child.

Cross-sectional comparisons between haemophiliacs and their healthy classmates were made in the years 1971-72 and 1987-88. The more recent set of data indicated no difference in respect to school absenteeism and school achievements between haemophiliacs and classmates in contrast to the older data, which showed such differences. In 1987-1988 the personality of the haemophilic boy was assessed both by teacher-ratings as well as by self-rating scales, and they indicated that the haemophiliacs were self-assured and independent but also less caring, less joyful, less open and more bullied than their healthy classmates. The mildly to moderately affected haemophiliacs tended to have more emotional disturbances than classmates and severely affected haemophiliacs. The latter were the least likely to avoid potentially hazardous activities. The haemophiliac's conflicts about adapting to a restricting chronic disease were elucidated.

Absenteeism↗
PubMed1990

A randomised comparison of ceftazidime and piperacillin, both in combination with flucloxacillin for treatment of febrile episodes in neutropenic children. Finnish Three-Centre Study.

111 febrile episodes in 98 neutropenic children were randomly treated with either ceftazidime (CAZ) 150 mg/kg/day or with piperacillin (PIP) 200 mg/kg/day, both combined with flucloxacillin (FLUC) 50 mg/kg/day. A total of 37/47 (79%) eligible episodes in the CAZ group and 41/53 (77%) in the PIP group were cured without needing to stop the initial therapy. The success without modification of the treatment in the verified septicaemias in the CAZ group was 8/18 (44%) and in the PIP group 5/18 (28%). Of the bacteriologically documented infections, 13/24 (54%) in the CAZ group and 11/24 (46%) in the PIP group were cured without modification of the therapy. Similarly, 17/31 (55%) of all the isolated bacteria in the CAZ group and 14/33 (42%) in the PIP group were eradicated with the initial therapy. Overall there were 13 deaths of which 4 in the CAZ group and 5 in the PIP group occurred during the infection. An increasing granulocyte count exceeding 0.25 x 10(9)/l at the end of the therapy was a good prognostic sign for the outcome of the infection. This study indicates that CAZ is as effective as PIP in the treatment of infections in neutropenic children.

Adolescent↗
PubMed1990

Graft-versus-leukaemia activity associated with CMV-seropositive donor, post-transplant CMV infection, young donor age and chronic graft-versus-host disease in bone marrow allograft recipients. The Nordic Bone Marrow Transplantation Group.

Risk factors for post-transplant relapse were analysed retrospectively in 163 patients treated with allogenic bone marrow transplantation for acute myeloid leukaemia (AML), acute lymphoblastic leukaemia (ALL) or lymphoblastic lymphoma in first to fourth remission or during relapse. Multifactorial analysis was performed according to Cox with fixed pretransplant covariates and post-transplant cytomegalovirus (CMV) infection and graft-versus-host (GVHD) as time-dependent covariates. Advanced stage of leukemia at the time of transplantation was an important risk factor for subsequent relapse. Furthermore, the study confirmed a graft-versus-leukaemia (GVL) activity associated with chronic GVHD, including de novo chronic GVHD (intensity factor 0.08, p = 0.004). In a model excluding chronic GVHD, female donor-to-male recipient (a risk factor for GVHD), was associated with decreased post-transplant relapse risk (intensity factor 0.3, p = 0.008), suggesting that an allo-reaction against a minor transplantation antigen (Hy) may mediate antileukaemic activity. A decrease of the relapse risk by a factor 0.18 was observed in recipients with AML as well as ALL when the donor was CMV seropositive (p = 0.0002). This effect was restricted to patients who had laboratory evidence of post-transplant CMV infection. When CMV infection occurred and donor was seropositive the relapse risk was reduced by a factor 0.035. The effect was not mediated through an increased occurrence of grade 2-4 acute or chronic GVHD and could not be explained by a statistical bias due to censoring of patients who died in remission. Rather, donor CMV immunity was associated with GVHD independent GVL activity.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

Growth failure and growth hormone deficiency in children after bone marrow transplantation for leukemia.

Eleven patients between the ages of 6 and 18 years who had been treated for acute leukemia were investigated for growth and growth hormone (GH) secretion. All had undergone bone marrow transplantation (BMT) between 0.7 and 5.1 (median 2.0) years previously. Preparation of patients for BMT had included high-dose cyclophosphamide and total body irradiation. In the eight patients at risk of growth failure, the relative height decreased 0.5-2.5 SD units (median 1.0) during the follow-up period. Eight patients secreted subnormal amounts of GH as studied by measuring spontaneous pulsatile GH secretion overnight. The maximal nocturnal GH peak varied between 3.3 and 28.3 micrograms/l (median 9.3). The mean nocturnal GH concentration varied from 1.2 to 8.3 micrograms/l (median 2.3) and depended on the length of the follow-up period. We conclude that deficient GH secretion is one reason for poor growth after BMT. A good growth response to GH substitution would support the role of GH deficiency in the observed growth retardation after BMT.

Adolescent↗
PubMed1989

Risk of progressive kidney damage after acute leukemia.

In order to evaluate potential long-term renal sequelae of childhood leukemia, we studied 62 consecutive patients successfully treated for acute leukemia in 1971-83. At the time of this follow-up study they had been off therapy for 1-9 years and they were all in complete remission. Relative renal length was measured from X-ray films of intravenous pyelograms. Median relative renal length prior to the start of chemotherapy was +1.0 SD (range -1.5 to +4.0, n = 35), at discontinuation of therapy +0.5 SD (range -1.5 to +2.2, n = 22), and at follow-up -0.3 SD (range -3.9 to +2.6, n = 61). The mean calculated decrease in relative kidney size was 0.1 SD unit per year during the follow-up time. The median glomerular filtration rate was 110 ml/min/1.73 m2 (range 70 to 164). Six of 60 patients had glomerular filtration rates below 85 ml/min/1.73 m2. Three patients had some evidence of tubular dysfunction documented by increased excretion of urinary amino acids and/or beta-2-microglobulin or by reduced concentrating capacity. In spite of these abnormalities, we conclude that in most long-term survivors of childhood leukemia renal size and function are relatively well preserved. However, slightly reduced glomerular filtration rates in some patients indicated renal damage. A longer follow-up time is needed to find out whether the decrease in relative renal length is still continuing.

Acute Disease↗