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Biomedical subjects

A Haraldsson

Publications and source records attributed to A Haraldsson.

16 recordsLinked to original sources
PubMed1992

Absent thumb, immune disorder, and congenital anemia presenting with hydrops fetalis.

A patient is described who presented with severe congenital anemia, hydrops fetalis, immune disorder, and absent thumbs. No toxic, infectious, or metabolic cause was found to explain these symptoms. Immunologic and cytogenetic studies excluded several syndromes that combine radial ray anomalies with hematological involvement. After careful study of the literature, it is concluded that the disorder described here represents a new syndrome that can be added to a growing list of hematological-radial syndromes.

Abnormalities, Multiple↗
PubMed1992

Immunological studies in the hyper-immunoglobulin D syndrome.

Five patients with hyper-immunoglobulin D syndrome (hyper-IgD syndrome) were followed up for 3 to 8 years. In all patients studied, serum IgG3 was high. IgM decreased during the follow-up in all patients. In four of the patients serum IgA was elevated. In four patients the serum IgD kappa/lambda ratio was measured and was found to be raised in all. However, the serum total light-chain ratio and IgG, IgA, and IgM kappa/lambda ratios separately were virtually normal. In two of the patients, clinical symptoms preceded the increase in serum IgD. All patients had a history of severe reactions on immunizations in early childhood. We conclude that in hyper-IgD syndrome, other immunoglobulins may also be affected, in particular, IgA, IgM, and IgG3. The IgD light-chain ratio is also disturbed. We emphasize that clinical symptoms may herald immunological changes. This may be the result of an underlying factor causing both the clinical symptoms and, later, the increasing serum IgD levels.

Adolescent↗
PubMed1992

Light chain ratios and concentrations of serum immunoglobulins in children with epilepsy.

Serum immunoglobulin G, A and M concentrations and their respective kappa/lambda (kappa/lambda) light chain ratios were studied in 26 children with epilepsy. Fifteen had cryptogenic West syndrome or Lennox-Gastaut syndrome and 11 had other forms of childhood epilepsy. The results were compared to the data of a reference group of healthy children. The mean serum IgG and IgM concentrations were respectively 2.2 g/l (P = 0.007) and 0.4 g/l (P = 0.016) higher in the 26 children with epilepsy compared to the reference group. The kappa/lambda ratios of total serum immunoglobulins, IgG and IgM were respectively 0.10 (P = 0.057), 0.20 (P = 0.001) and 0.14 (P = 0.005) lower in the children with epilepsy than in the reference group. IgA concentration and IgA kappa/lambda ratio were not affected. There were no significant differences between the kappa/lambda ratios of the West and Lennox-Gastaut epilepsy and the other types of childhood epilepsies. The results are further evidence of reciprocal interaction between the nervous system and the immune system in childhood epilepsy.

Child↗
PubMed1992

Inspiratory to end-tidal oxygen difference during nitrous oxide anaesthesia.

In order to evaluate the value of the inspiratory to end-tidal oxygen concentration difference (Io2-E'o2) as a monitor during general anaesthesia, we studied 40 orthopaedic patients allocated randomly to four groups: anaesthesia with enflurane or isoflurane in nitrous oxide with either spontaneous or controlled ventilation. (Io2-E'o2) followed an asymptotically increasing curve because of decreasing uptake of nitrous oxide. At 1 h, (Io2-E'o2) approached the end-tidal carbon dioxide concentration (E'o2). During spontaneous ventilation, (Io2-E'o2) correlated best with E'co2. During controlled ventilation, there was a negative correlation between (Io2-E'o2) and nitrous oxide uptake rate. Changes in oxygen uptake rate were reflected in (Io2-E'o2), provided that the total ventilation volume was constant and the nitrous oxide uptake rate approached steady state conditions.

Adult↗
PubMed1992

Immunoglobulin G, A, and M light chain ratios in some humoral immunological disorders.

The total kappa/lambda immunoglobulin light chain ratio and the kappa/lambda ratios within each of the serum immunoglobulin classes G, A, and M were measured in thirteen patients with humoral immunological disorders. Of those patients, eight had common variable immunodeficiency whereas five patients had other forms of humoral immunological deficiencies. Eleven patients had abnormal antibody response in vivo. All but three of the thirteen patients had clearly abnormal light chain ratios in one or more of the immunoglobulin classes. We conclude that humoral immunological disorders, usually characterized by abnormal heavy chain production and a disturbed antibody response, may frequently have a concomitant abnormal synthesis of the light chains resulting in an abnormal kappa/lambda light chain ratio.

Adolescent↗
PubMed1992

Immunoglobulin G, A and M light chain ratio in children.

Values for the kappa/lambda light chain ratio in immunoglobulins G, A and M and the total kappa/lambda ratio, measured by enzyme linked immunosorbent assay, were evaluated in serum samples from different age groups (114 children, aged from 1 month to 15 years, and 20 adults). The IgG kappa/lambda ratio decreased in the first 6 months and subsequently increased slowly during childhood towards the adult value of 2.0. The IgM kappa/lambda ratio increased at a greater rate than IgG kappa/lambda ratio in the first years of life and thereafter rose slightly throughout childhood to reach an adult value of 1.7. A decreasing IgA kappa/lambda ratio was found from 1 month of age onwards to an adult value of 1.1. The pattern of total kappa/lambda ratio was similar to the IgG kappa/lambda ratio with an adult value of 2.0.

Adolescent↗
PubMed1991

Cartilage hair hypoplasia, metaphyseal chondrodysplasia type McKusick: description of seven patients and review of the literature.

We describe 7 cases of cartilage hair hypoplasia (CHH) with emphasis on the clinical and immunological aspects. The literature on CHH is reviewed and symptoms in 63 non-Amish cases are summarized. In this autosomal recessive disorder the immunodeficiency, hair abnormalities, and severity of skeletal involvement show extremely variable expressivity, between and within families. Two of the 3 sib-pairs among our cases demonstrate the great difference in expression within one family. At adult age roentgenological abnormalities can be very mild, or even absent. An impairment in cell-mediated immunity is present in all of our cases and seems a consistent manifestation in CHH; however, sometimes it is very subtle and without clinical symptoms.

Abnormalities, Multiple↗
PubMed1991

Griscelli disease with cerebral involvement.

A 9-month-old Turkish boy was diagnosed as having Griscelli disease (Chediak-Higashi-like syndrome). Clinical signs consisted of silver-grey hair and a relatively light skin colour, recurrent episodes of fever, with or without detectable infections, increasing hepatosplenomegaly, hypotonia and motor retardation. Laboratory studies showed pancytopenia of varying degree but neither inclusion bodies nor vacuoles were seen in his leucocytes. Serum immunoglobulin levels were normal except for a IgG2 deficiency. In the mixed lymphocyte reaction the stimulation capacity of the leucocytes was decreased. Microscopic examination of his hair and electron-microscopic examination of a skin biopsy further confirmed the diagnosis. Shortly before the diagnosis was made, the child developed cerebral symptoms with hemiparesis and convulsions. A CT scan suggested cell infiltration of the brain. A few weeks later the boy died of an infection.

Albinism↗
PubMed1991

Determination of kappa and lambda light chains in serum immunoglobulins G, A and M.

An enzyme-linked immunosorbent assay is described for measuring kappa and lambda light chains within each of the serum immunoglobulin classes G, A and M. The detection limit was 0.06 U/L for total IgG, IgG kappa and IgG lambda, 0.2 U/L for total IgA, IgA kappa and IgA lambda and 0.5 U/L for total IgM, IgM kappa and IgM lambda. The concentrations of kappa plus lambda light chains from the three different immunoglobulins correlated well within those of total immunoglobulin G, A and M as measured by enzyme-linked immunosorbent assay or by immunonephelometry. Adult values for the kappa/lambda light chain ratio were found to be 2.0 for IgG kappa/lambda, 1.1 for IgA kappa/lambda and 1.7 for IgM kappa/lambda.

Adolescent↗
PubMed1991

Immunological studies in Bloom's syndrome. A follow-up report.

Longitudinal immunological studies were performed in five patients with Bloom's syndrome. Serum IgG concentration showed no age-related increase. IgA levels were below the 10th percentile in childhood, but rose to normal in all but one. IgM was decreased in four patients at diagnosis and remained so in three of them. Cellular immunity results were very variable, even within one patient. An immunologic attrition was not demonstrated.

Bloom Syndrome↗
PubMed1990

Prevalence of IgM antibodies to nine Legionella species in Icelandic children.

In order to evaluate the prevalence of antibodies to Legionella species among children in Iceland, a prospective study was conducted in 424 children aged 1 month to 12 years, 28 of whom had an acute respiratory tract infection. Antibody titers to L. pneumophila serogroup 1-6, L. bozemanii, L. dumoffii, and L. micdadei were measured by microagglutination technique. Seroreactivity to Legionella spp. was found in 30% of the children greater than 3 years of age and in 22% of all children. The majority of the children with legionella antibodies had no history of previous or present respiratory tract infection. The children with previous pneumonia or bronchial asthma did not show a higher seroreactivity to legionella than children without such a history. Our observations suggests that Icelandic children are frequently exposed to Legionella species or closely related bacteria.

Age Factors↗
PubMed1990

[Cat-scratch disease].

Cat scratch disease is an infectious disorder caused by a Gram-negative bacterium. The classical form is heralded by a scratch, subsequently red papules develops at the place of the inoculation. After an incubation period of one to two weeks lympadenopathy without lymphangitis occurs, with tender, red, warm and indurated skin as well as fever and general malaise. In the atypical form, different organs may be involved. Specific treatment is not indicated and the overall prognosis is good. The diagnoses of cat scratch disease can be sustained by a skin test.

Cat-Scratch Disease↗
PubMed1989

Half-life of maternal labetalol in a premature infant.

Intra-uterine heart arrhythmia, postpartal respiratory insufficiency, bradycardia and hypoglycaemia were observed in a premature infant (37 weeks gestational age) delivered by a caesarian section. The mother had been treated with adequate doses of labetalol because of pregnancy-induced hypertension and her plasma concentration was found to be 89 micrograms/l one day after delivery. The half-life of labetalol in the plasma of the infant was found to be approximately 24 h, i.e. substantially longer than in normal adults. The half-life of labetalol in newborn premature infants may be prolonged as compared to normal adults. More studies are required regarding the pharmacokinetics of this agent in premature infants and newborn babies.

Adult↗
PubMed1985

Thyrotoxicosis in Iceland 1980-1982. An epidemiological survey.

A nationwide survey of the incidence of clinical thyrotoxicosis in Iceland was made during the three-year period 1980-1982. An annual incidence of 23.6 per 100 000 inhabitants was found, females outnumbering males by 4.2:1; 72% of all patients were aged 20-49. Graves' disease was considered the cause in 83.3% of the patients.

Adolescent↗