Multidimensional tracking of phenotypes and organ involvement in a complete nationwide systemic sclerosis cohort.

Objective: SSc is a severe, heterogeneous multi-organ disease where population-based estimates on phenotypic spectrum, overall disease burden and societal impact are largely missing. Here the objective was to provide the first-ever complete national-level data on phenotype and major organ afflictions in SSc.

Methods: A stepwise strategy was applied to find and characterize every SSc patient resident in Norway from 2000 to 2012.

Identification and Functional Characterization of a Novel Insecticidal Decapeptide from the Myrmicine Ant .

Ant venoms contain many small, linear peptides, an untapped source of bioactive peptide toxins. The control of agricultural insect pests currently depends primarily on chemical insecticides, but their intensive use damages the environment and human health, and encourages the emergence of resistant pest populations. This has promoted interest in animal venoms as a source of alternative, environmentally-friendly bio-insecticides.

Inhibition of histone acetylation and deacetylation enzymes affects longevity, development, and fecundity in the pea aphid (Acyrthosiphon pisum).

Histone acetylation is an evolutionarily conserved epigenetic mechanism of eukaryotic gene regulation which is tightly controlled by the opposing activities of histone acetyltransferases (HATs) and histone deacetylases (HDACs). In insects, life-history traits such as longevity and fecundity are severely affected by the suppression of HAT/HDAC activity, which can be achieved by RNA-mediated gene silencing or the application of chemical inhibitors. We used both experimental approaches to investigate the effect of HAT/HDAC inhibition in the pea aphid (Acyrthosiphon pisum) a model insect often used to study complex life-history traits.

Tracking Impact of Interstitial Lung Disease in Systemic Sclerosis in a Complete Nationwide Cohort.

Interstitial lung disease (ILD) represents a major challenge in systemic sclerosis (SSc), but there are no precise, population-based data on its overall impact, limiting opportunities for screening and management strategies. Evaluate impact of ILD in a unique, nationwide, population-based SSc cohort. ILD was assessed prospectively in the Norwegian SSc (Nor-SSc) cohort, including all 815 patients with SSc resident in the country from 2000 to 2012.

Proteomic Analysis of the Venom from the Ruby Ant and the Isolation of a Novel Insecticidal Decapeptide.

Ants are a biodiverse group of insects that have evolved toxic venom containing many undiscovered bioactive molecules. In this study, we found that the venom of the ruby ant is a rich source of peptides. LC-MS analysis revealed the presence of 142 different peptides varying in molecular weight, sequence length, and hydrophobicity.

Live Birth Pregnancy Outcome after First In Vitro Fertilization Treatment in a Patient with Systemic Lupus Erythematosus and Isolated High Positive IgA Anti-β2glycoprotein I Antibodies: A Case Report.

IgA anti-β2glycoprotein I antibodies (IgA-anti-β2GPI) seems to be the most prevalent isotype in patients with Systemic Lupus Erythematosus (SLE) with a significant association to thrombotic events. Both SLE and antiphospholipid syndrome (APS) can be associated with implantation failure, fetal loss and obstetric complications. Recent reports highlight the clinical value of IgA-anti-β2GPI determination in supporting in vitro fertilization (IVF) treatment and IVF pregnancy outcomes.

Evaluation of a domain-specific anger expression assessment strategy.

Previous studies have shown that self-reports of an individual's anger expression vary between the home and the work domain. The aim of this study was to investigate the validity of such self-reports by comparing them with reports of intimate partners and work colleagues in the respective domain. Participants (N= 86) rated their anger expression on the general and on domain-specific versions of the State-Trait-Anger Expression Inventory (Spielberger, 1988).

[Diagnostic challenges in a man with subarachnoidal haemorrhage].

A 39-year-old man with multiple sclerosis was admitted to the Neurological department because of sudden and intense headache, photophobia, nausea and vomiting. A subarachnoidal haemorrhage was suspected and cerebral CT scanning showed small amounts of subarachnoidal blood distributed symmetrically over both cerebral hemispheres - an unusual location for aneurismal bleeding. Liquor analysis revealed erythrocytes, positive bilirubin and slightly elevated protein.

Familial transient erythroblastopenia of childhood is associated with the chromosome 19q13.2 region but not caused by mutations in coding sequences of the ribosomal protein S19 (RPS19) gene.

Transient erythroblastopenia of childhood (TEC) is a rare condition, which at onset may be difficult to distinguish from Diamond-Blackfan anaemia (DBA). We have previously shown that mutations in the ribosomal protein S19 gene (RPS19) cause DBA. In order to clarify whether TEC and DBA are allelic, we investigated the segregation of markers spanning the RPS19 gene region on chromosome 19q13.

Genetic basis of hemolytic anemia caused by pyrimidine 5' nucleotidase deficiency.

Pyrimidine 5' nucleotidase (P5'N-1) deficiency is an autosomal recessive condition causing hemolytic anemia characterized by marked basophilic stippling and the accumulation of high concentrations of pyrimidine nucleotides within the erythrocyte. It is implicated in the anemia of lead poisoning and is possibly associated with learning difficulties. Recently, a protein with P5'N-1 activity was analyzed and a provisional complementary DNA (cDNA) sequence published.

Three-nucleon force and the A(y) puzzle in intermediate energy p--> + d and d--> + p elastic scattering.

New vector analyzing-power data on p-->+d elastic scattering at E(p) = 150 and 190 MeV have been measured. These are presented together with existing data and with recent d-->+p vector and tensor analyzing power data at E(d) = 270 MeV. The strong negative extremum of both vector analyzing powers A(p)(y) and A(d)(y) at straight theta(c.

[Pyrimidine-5'-nucleotidase deficiency--congenital hemolytic anemia with basophilic stippling of erythrocytes].

The disease is caused by a deficiency of the enzyme pyrimidine-5'-nucleotidase in the erythrocytes. About 50 cases have been published. We have diagnosed the disease in a pair of siblings from Numedal, Norway.

Generalized lipodystrophy, congenital and acquired (lipoatrophy).

This review is based on longitudinal studies on our seven patients with congenital generalized lipodystrophy, our patient with acquired generalized lipodystrophy, and published papers on these subjects. An inability to store energy in adipose tissue is of pathogenetic importance. In congenital lipodystrophy, insulin resistance is present from birth, resulting in hyperinsulinaemia, dyslipidaemia.

Autonomous hyperparathyroidism in X-linked hypophosphataemia.

Four patients with familial hypophosphataemic rickets developed significant hypercalcaemia which persisted after discontinuation of vitamin D therapy. They had increased PTH levels and were operated for hyperparathyroidism at the ages of 18, 20, 24 and 45 years, respectively. Three of the patients had previously received phosphate treatment and one patient developed hyperparathyroidism 7 years after treatment with calcitriol.

[School health services in Norway--rise and fall].

Professional interest in school hygiene and in the health of the pupils developed in Europe towards the middle of the 19th century. In Norway, professional discussion started with two lengthy debates in the Norwegian Medical Society in Oslo in 1866 and 1868. An important issue was the possible overburdening of the pupils with school work.

[A study of health status of school children 100 years ago].

In the years 1891 and 1892 an extensive study was carried out to determine the health of Norwegian school children. It was the first of its kind in the country. 16 middle schools and secondary schools from different parts of the country participated.

Malignant tumors in two patients with Diamond-Blackfan anemia treated with corticosteroids and androgens.

Two patients with Diamond-Blackfan anemia developed solid malignant tumors as young adults, carcinoma of the liver in one and carcinoma of the stomach in the other. Both had received long-term treatment with corticosteroids and androgens.

[Infant nutrition in a historical perspective].

Throughout history man, with few exceptions, has meant that breast feeding is best for the infant. In poor countries with bad hygiene this can still be a question of life or death. However, during several millennia up to the end of the 19th century, there have been many periods and cultures when the upper classes have preferred to have wet nurses for their babies.

[Teenage iron deficiency].

Iron deficiency is common in adolescents. This age-group needs more iron owing to the spurt of growth in puberty, and, in girls, because of menstrual losses, and in the event of pregnancy. In boys there is a normal rise in haemoglobin level from about 13 to about 15 g/100 ml, and in myoglobin (muscular mass).

Growth hormone normalizes hepatic lipase in hypothyroid rat liver.

The effects of growth hormone (GH) administration on hepatic lipase (HL) activity and mRNA levels were studied in pair-fed hypothyroid rats. In the hypothyroid state, liver HL mRNA levels and liver and postheparin plasma HL activities were decreased. In hypothyroid rats, GH normalized HL mRNA levels and HL activity in postheparin plasma; GH also increased the activity of HL in the liver, which remained lower than that in controls.

[100 years of pediatrics in Norway. The history up to 1950].

The first department of pediatrics in Norway was opened at Rikshospitalet (The National Hospital of Norway) in January 1893. This marked the start of pediatrics as an independent discipline and specialty in Norwegian medicine. Upon this centennial we present the history of the department (and of pediatrics in Norway) up to 1950.

Leukemic blasts with markers of four cell lineages in Down's syndrome ("megakaryoblastic leukemia").

Among 16 patients with Down's syndrome (DS) and acute leukemia admitted to our department during a ten year period, 6 were diagnosed as acute megakaryoblastic leukemia (AMkL). The diagnosis was based on clinical and hematologic criteria, confirmed in three patients with the use of monoclonal antibodies (MoAb) specific for megakaryocytic antigens. In these three, and in a fourth patient, the leukemic blasts were positive for other myeloid, lymphoid and erythroid markers in MoAb testing.