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Biomedical subjects

B Kristiansson

Publications and source records attributed to B Kristiansson.

At least 55 records · Page 3Linked to original sources
PubMed1991

Linear growth in children with cystic fibrosis. I. Birth to 8 years of age.

The linear growth of Swedish children with cystic fibrosis (CF), is described using the infancy-childhood-puberty (ICP) growth model. Length/height was studied in 51 patients during their first 8 years of life. The median age at diagnosis was 0.4 years (range 0.0-6.1 years). At birth, their mean length was close to normal, but the gain in length over the first 0.25 years of life was significantly below normal, resulting in a mean length SDS of -1.3. Length remained subnormal up to 1.0 year of age. Thereafter, catch-up growth occurred, resulting in almost normal height (mean SDS-0.3) at 5.0 years of age. Between 5.0 and 8.0 years of age growth was normal. The mean age at onset of the childhood component was not significantly different from the controls. Hence, the catch-up growth did not occur until after the onset of the childhood component. This study shows that the postnatal linear growth rate is retarded in children with CF during the first months of life. This is almost completely compensated for by a supranormal growth rate that starts at the end of the first year of life.

Age Factors↗
PubMed1991

Increasing incidence of coeliac disease in Sweden.

Changes in the incidence of coelic disease was studied among children born in Göteborg, Sweden, between 1970 and 1988. A total of 188 patients with coeliac disease were found. Of these, 83% were less than 2 years old at the time of their first duodenal biopsy and 74% of them have so far been verified according to the criteria of the European Society for Gastroenterology and Nutrition (ESPGAN). The cumulative incidence at 2 years of age/1000 liveborn infants increased significantly from 0.31 in the first birth cohort to 2.93 in the last. This increase could only partly be explained by improvements in detection. Weight for age at diagnosis was generally considerably below the reference value, but was slightly less affected towards the end of the period. The increase in incidence of coeliac disease is the first reported since the middle 1970s and makes the disease one of the most common chronic diseases among Swedish children.

Age Factors↗
PubMed1991

Cerebral function of the guinea pig neonate after chronic intrauterine exposure to khat (Catha edulis Forsk.).

Cerebral function in normoxia and its reactions to standard periods of hypoxia of increasing severity were studied in 30 newborn guinea pigs less than 3 days old. Intrauterine growth retardation was induced either by uterine artery ligation at midgestation or by feeding the female in late gestation with khat leaves, an amphetamine-like stimulant chewed by men and women in several countries in eastern Africa and Arabia. After spontaneous delivery, the neonates were anesthetized and ventilated. Cardiovascular, metabolic, and neurophysiologic (somatosensory evoked potentials) parameters were monitored. Under normoxia, the khat-exposed group showed prolonged latency of the primary response of the somatosensory evoked potentials and a reduced amount of secondary components. Under hypoxia, this group also has a greater reduction of amplitude of the somatosensory evoked potentials. It is concluded that khat exposure during fetal life has an impact on the cerebral function during the neonatal period (at least up to 3 days of age) which is not solely explained by the concomitantly produced growth retardation.

Animals↗
PubMed1991

Khat-chewing during pregnancy-effect upon the off-spring and some characteristics of the chewers.

In a study of 1,141 consecutive deliveries at delivery centres in the Yemen Arab Republic, the effects of khat (catha edulis) upon the offspring have been studied. The leaves of the shrub khat contain euphorizing compounds and are chewed often, even daily, by many inhabitants. Non-users of khat (n = 427) had significantly fewer low birth-weight babies (less than 2,500 gram) compared to occasional users (n = 223) and regular users (n = 391). The khat-chewing mother was older, of greater parity and had more surviving children than the non-chewers. Significantly more khat-chewers had concomitant diseases. There was no difference in rates of stillbirth or congenital malformations.

Birth Weight↗
PubMed1990

A new laboratory kit for anti-gliadin IgA at diagnosis and follow-up of childhood celiac disease.

A new laboratory kit measuring anti-gliadin IgA level by enzyme immunoassay has been evaluated to assess the test's potential for screening children with suspected celiac disease for small intestinal biopsy and predicting mucosal relapse after gluten challenge. One hundred thirty children were tested, and the results were related to the histopathologic findings of the intestinal mucosa and the final diagnosis. The sensitivity of the test was 97% and the specificity was 92% in the studied population. Forty-five children with celiac disease on different diets were observed. All had reduced anti-gliadin IgA levels on a gluten-free diet, and 91% had normal levels after 1 year. Two of four patients with increased values had an insufficient diet. During gluten challenge, 38 of 45 children showed increased anti-gliadin IgA levels. Of the remaining seven, five reacted either with immediate and strong symptoms or had spontaneously reduced gluten intake, or had an acquired IgA deficiency. In two cases, there was no explanation. In five children without relapse on gluten challenge, the anti-gliadin IgA level remained normal. Provided that IgA deficiency is ruled out and the gluten intake is sufficient, the test is reliable for screening and has a potential to replace the third biopsy.

Antibodies↗
PubMed1990

Progressive increase of the mutated mitochondrial DNA fraction in Kearns-Sayre syndrome.

We have performed morphologic and biochemical studies in three pediatric cases of Kearns-Sayre syndrome. All cases had heteroplasmy with a high percentage of mitochondrial DNA (mtDNA) with deletion in muscle. The deletions were mapped to the same region of mtDNA but were of different sizes. The same type of deletion could also be detected in fibroblasts from all cases but the percentage was considerably lower. In two cases, an increase with time of the mutated mtDNA fraction in muscle was found and this increase paralleled the progression of the disease. Oximetric evaluation of respiratory-chain function in isolated muscle mitochondria showed a complex I deficiency in one case and was normal in the two other cases. Comparison of the fractional concentration of mtDNA with deletion in muscle and isolated mitochondria showed that the isolated mitochondria were not representative of the mitochondrial population in muscle. Mitochondria with high percentage of mtDNA with deletion were selectively lost. The finding of different mitochondrial populations is in good agreement with the morphology. One case spontaneously recovered from an infantile sideroblastic anemia before the development of Kearns-Sayre syndrome. The anemia was of the same type as that in Pearson's syndrome, a mitochondrial disorder with high amounts of mtDNA with deletion in blood cells. These findings indicate that the phenotype of a mtDNA deletion disorder can change with time and is governed by the fractional concentration of mtDNA with deletion in different tissues.

Adolescent↗
PubMed1990

Ocular pathology in disialotransferrin developmental deficiency syndrome.

Disialotransferrin developmental deficiency (DDD) syndrome is a recently described disease consisting of hepatopathy, mental retardation and neuropathy. The biochemical findings indicate a defect in the assembly of the carbohydrate moiety that is common to the secretory glucoproteins. It is believed to be of autosomal recessive inheritance. An ophthalmological examination of ten children suffering from this syndrome showed that all had ocular involvement. Esotropia (and deficient abduction) was found in all ten patients. Seven children had retinitis pigmentosa which was verified by an ERG in three. One patient had retinal signs suggestive of retinitis pigmentosa. The high incidence of ocular findings in the DDD syndrome, which are reported for the first time, indicate that an ophthalmological examination is a helpful diagnostic tool in this disease.

Adolescent↗
PubMed1990

Stunting and tissue depletion in Yemeni children.

With the aim of assessing whether stunting was associated with depletion of labile tissues such as fat and muscle as an indicator of ongoing malnutrition, we investigated 1176 children 0-7 years of age in PDR Yemen, who participated in a national nutrition survey conducted in 1982-3 and its pilot study from 1978. Arm circumference and triceps fatfold have been measured and upper arm fat area (UFA) and upper arm muscle area (UMA) were calculated to estimate the body stores of fat and muscle. Stunting, defined as a stature shorter than -2 SD of the reference mean, was found in one-third of the children. The average length/height for age deviated progressively from the reference mean up to age group 12-15 months. Exclusively breast-fed infants also deviated in length, although less conspicuously than infants fed in other ways. There was a consistent pattern of smaller UFA and UMA in stunted children compared to equally tall children who were not stunted below 84 cm of height for boys and 102 cm for girls. The difference was statistically significant in a few of the height groups. It is suggested that stunting is accompanied by a slight reduction of fat and muscle tissues during the first years of life.

Adipose Tissue↗
PubMed1989

Carnitine deficiency induced by pivampicillin and pivmecillinam therapy.

Short-term administration of pivampicillin and pivmecillinam resulted in a reduction of serum carnitine concentration and an increase in excretion of acylcarnitine in urine. These changes persisted for more than ten days after cessation of therapy. In seven girls on long-term treatment with a mixture of pivampicillin and pivmecillinam the mean total serum carnitine concentration fell to 15% (7-27%) of pretreatment values. The acylcarnitine fraction was 11-57% of total carnitine, compared with less than 2% before treatment. Muscle carnitine concentrations in two girls treated with the antibiotics for 22 and 30 months were only 10% of the mean reference value. These concentrations in serum and muscle are in the range encountered in patients with carnitine deficiencies of other aetiologies in which life-threatening metabolic crises may arise. The risk of adverse effects from prodrugs that give rise to pivalic acid should be seriously considered, particularly in patients under metabolic stress.

Adolescent↗
PubMed1989

Cytochrome c oxidase deficiency in infancy.

Five children with early onset of muscle weakness, lactic acidosis and deficient cytochrome c oxidase staining in the muscle biopsy were studied. By oximetric assay of the respiratory chain of isolated mitochondria, cytochrome c oxidase deficiency was confirmed in four of the cases, while one case showed only a slight decrease of cytochrome c oxidase activity but considerably reduced activity when assayed spectrophotometrically. The muscle biopsies exhibited mitochondrial structural abnormalities and lipid storage in the four cases with oximetrically confirmed cytochrome c oxidase deficiency, while the biopsy of the case with markedly reduced activity of cytochrome c oxidase only in the enzyme-histochemical and spectrophotometrical assays had normal morphology. The light microscopical staining of cytochrome c oxidase in the four cases with oximetrically confirmed deficiency showed deficient staining of the enzyme in all extrafusal fibres in three cases but one of the cases had normal enzyme-histochemical activity of cytochrome c oxidase in about 25% of the fibres. In two cases muscle spindles were included in the biopsy. The intrafusal fibres showed normal enzyme-histochemical activity of cytochrome c oxidase. Ultrastructural examination of the enzyme distribution in two of the cases revealed great heterogeneity of the mitochondria. The structurally abnormal mitochondria were usually deficient of enzyme activity. The mitochondria of endothelial cells appeared to have normal activity. Immunohistochemical staining with polyclonal antibodies to cytochrome c oxidase revealed presence of immunoreactive material corresponding to the localisation of mitochondria in all cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Biopsy↗
PubMed1989

Disialotransferrin developmental deficiency syndrome.

Seven mentally deficient children and adolescents (three pairs of siblings and one singleton) were studied. A peculiar external appearance, a characteristic neurohepatosubcutaneous tissue impairment syndrome and, as a biological marker, an abnormal sialic acid transferrin pattern were characteristic features. All seven seemed odd from birth and prone to acute cerebral dysfunction during catabolic states. Abnormal lower neurone, cerebellar, and retinal functions dominated from later childhood. The disialotransferrin pattern found in serum and cerebrospinal fluid is thought to be the biological marker of a newly discovered inborn error of glycoprotein metabolism with autosomal recessive inheritance.

Adolescent↗
PubMed1988

Biotin-responsive multiple carboxylase deficiency in an 8-year-old boy with normal serum biotinidase and fibroblast holocarboxylase-synthetase activities.

An 8-year-old boy with late onset multiple carboxylase deficiency is described. Biotinidase deficiency and holocarboxylase-synthetase deficiency have been excluded. A very slow biochemical response to biotin was found. The decrease in urinary organic acid excretion followed first-order kinetics with a half-life of about 50 days. The initially low carboxylase activities in thrombocytes were increased but not normalized after 3 months of treatment.

Amidohydrolases↗
PubMed1988

Growth and malnutrition among preschool children in Democratic Yemen.

A cross-sectional anthropometric study of children aged 0-84 months was performed in 1982-83 in Democratic Yemen. The total sample included 3407 children, representing populations from urban, rural, and slum areas of the country. NCHS/WHO growth data were used for reference purposes.A high overall prevalence of wasting (8.7%) and stunting (35.2%) was found among the children. However, rural children exhibited a satisfactory weight-for-height during the first 6 months of life compared with both the reference and the urban and slum children. Slum children had a high prevalence of wasting during the first 18 months of life.For the younger age groups, rural children were shorter than urban children, but at 7 years of age all the children were similar, with a mean height-for-age corresponding to -1.7 standard deviations of that for the reference population. Mothers in the urban area weighed significantly more than those from the slum or rural areas (P<0.001), but all mothers had similar heights.

Anthropometry↗
PubMed1987

Mitochondrial encephalomyopathy. A variant with heart failure and liver steatosis.

We report the clinical and autopsy findings in a young man of 18 with a chronic progressive disorder comprised of lactic acidosis, mental deterioration, and epileptic seizures which were sometimes accompanied by stroke-like episodes with transient hemiparesis and cortical blindness. He died of congestive heart failure. The autopsy showed lesions of the gray matter of the brain. Both the putamen and parieto-occipital cortex showed loss of neurons and proliferation of macrophages, astrocytes and vessels. There was marked loss of neurons in the inferior olives, and slight reduction of the number of Purkinje cells. Skeletal muscle studies revealed ragged-red fibers and structurally abnormal mitochondria. The heart was enlarged: accumulations of mitochondria occurred in the muscle fibers. The liver exhibited marked fatty degeneration. Biochemical analyses showed normal activities of pyruvate dehydrogenase in thrombocytes, pyruvate carboxylase in lymphocytes, biotinidase in serum as well as succinate dehydrogenase and cytochrome c oxidase. The features of this disorder differ in many respects from cases of mitochondrial encephalomyopathy previously reported and cannot be assigned to any specific disease entity.

Acidosis, Lactic↗
PubMed1987

Growth at the age of 4 years subsequent to early failure to thrive.

Fifty-five children previously investigated for failure to thrive (a rate of weight gain below -2 SD) during at least 6 weeks at 4-18 months of age were followed up and reinvestigated at the age of 4 years. The children were studied in two groups: children with organic causes (OFTT) (n = 21); and children for whom no organic cause was found (nonorganic failure to thrive, NFTT) (n = 34). In children with OFTT, normalization of growth was found for both weight and height attained, as most of the diseases were either amenable to treatment or spontaneously subsided. The only exception was a child with severe encephalopathy. In children with NFTT, much lower values were found, particularly for weight, p less than .01 for both weight and height. Children with a low psychosocial score (less than or equal to 3 adverse factors) showed partial catch-up growth, although significantly lower than that of children with OFFT. Among 13 children with high psychosocial scores (greater than or equal to 4), 6 children had been subjected to strong social and/or psychological intervention. These children showed a more favorable growth pattern compared to children with comparable psychosocial scores where no intervention had been undertaken. The children with NFTT continued to grow slowly, remained meager and seemed to maintain a suboptimal growth pattern, particularly those with higher numbers of risk factors.

Body Height↗
PubMed1987

The influence of khat-chewing on birth-weight in full-term infants.

The leaves of the shrub Catha edulis (khat) are widely chewed as part of social life in several countries around the Red Sea and in East Africa. The leaves possess stimulant properties and are also used by pregnant women. The effect of khat on birth-weight has been studied, It was found that healthy full-term, singletons, born after uneventful pregnancies and deliveries, had a significantly lower average birth-weight when the mothers were khat-chewers, either habitually or occasionally (P less than 0.001). Khat-chewing appears to be one of several maternal practices adverse to the fetus.

Birth Weight↗