B-cell lymphoma in a patient with complete interferon gamma receptor 1 deficiency.

Immunosuppression-associated lymphoproliferative disorders can be related to primary as well as acquired immune disorders. Interferon gamma receptor (IFN-γR) deficiency is a rare primary immune disorder, characterized by increased susceptibility to mycobacterial infections. Here we report the first case of an Epstein Barr Virus (EBV) related B-cell lymphoma in a patient with complete IFN-γR1 deficiency. The patient was a 20-year-old man with homozygous 22Cdel in IFNGR1 resulting in complete absence of IFN-γR1 surface expression and complete lack of responsiveness to IFN-γ in vitro. He had disseminated refractory Mycobacterium avium complex and Mycobacterium abscessus infections. At age 18 he presented with new spiking fever and weight loss that was due to an EBV-positive B-cell non-Hodgkin lymphoma. Two years later he died of progressive lymphoma. IFN-γ plays an important role in tumor protection and rejection. Patients with IFN-γR deficiencies and other immune deficits predisposing to mycobacterial disease seem to have an increased risk of malignancies, especially those related to viral infections. As more of these patients survive their early infections, cancer awareness and tumor surveillance may need to become a more routine part of management.

[Why do Ibsen's children die?]. / Hvorfor dør Ibsens barn?

Death of children is a remarkably frequent theme in Henrik Ibsen's writings and may be a key to understanding of his work. In this paper I refer to writings where children die, and I analyze case histories and question of causes. The medical courses are very diverse. In his earliest works, some children die by starvation. In our time we tend to forget the enormous child mortality rate in Norway during the mid 1800s. In his contemporary dramas, the causes of death are often complex. Even though he uses symbols, there are elements that are medically reasonable. He rarely makes mistakes in a medical context. A common feature in these tragic child destinies is that the poet shows us inadequate and poor parents.

[Cholera and quarantine in Kristiansand]. / Kolera og karantene i Kristiansand.

UNLABELLED: BACKGROUND AND MATERIAL: Cholera was the horror disease in Norwegian cities in the 18th century. The City of Kristiansand had a quarantine station and hospital at Odderøy island from 1804. We have studied the quarantine organisation and compared the number of cholera deaths in different cities along the coast. RESULTS: Drammen is comparable to Kristiansand and had many visiting trading ships. During 1832-33, 1833 and 1857 the Drammen community had registered 544 cholera patients; 336 died. Kristiansand had a total of only 15 deaths (1833, 1853 and 1866), the quarantine hospital included. Also compared to other cities, Kristiansand had few deaths. INTERPRETATION: The reason for the better cholera statistics in Kristiansand is probably the quarantine station and hospital at Odderøy island (1804). The city's health authorities had efficient routines and knowledge, probably adopted from the quarantine station and the hospital.

Stimulant medication in 47,XYY syndrome: a report of two cases.

In two males, 11 and 12 years of age, referred for attention-deficit-hyperactivity disorder (ADHD), 47,XYY syndrome was diagnosed. A team that included a neuropsychologist, a physiotherapist, and a physician examined them. Stature (patients were above 97.5% height for age), muscle consistency, and tremor indicated chromosome analysis. Psychological tests results did not fully fit the ADHD diagnosis. On the basis of our clinical observation we felt that stimulant medication was indicated. Administration of methylphenidate led to improved motor and cognitive functions as well as social adaptation in both patients. We suggest that this treatment might well be considered in clinically similar patients with XYY sex chromosomes; we further suggest that learning problems in such individuals may be related to ADHD.

[A three-year-old boy with hypoglycaemia]. / Tre år gammel gutt med hypoglykemi og astma.

Inhaled corticosteroids are a well established and effective treatment for asthma in children. However, some children develop systemic side effects including adrenal suppression when using moderate to high doses. Over the last few years, several severe acute adrenal crises with hypoglycaemia in patients using inhaled corticosteroids have been reported. Normally these patients do not develop a Cushingoid appearance and their height is not necessarily affected. We present a three-years-old boy that was unconscious at admittance. From the age of 6 months he had had asthma, treated with fluticasone propionate. The last year his asthma had been difficult to control, and he was given 750-1000 g/day in combination with salmeterol and a leucotriene antagonist. The day before admittance he had been ill with fever, had poor intake of food, and no intake of his regular medication. He was found unconscious in the morning. At admittance the blood glucose was 1.8. His cortisol axis was partially suppressed, probably as a result of the high doses of fluticasone propionate that had been administered. When treating asthmatic children it is important to use the lowest possible dose of inhaled corticosteroids. Those in need of higher doses should be carefully followed up with respect to systemic side effects. In emergency situations, systemic steroids should be used liberally in these children.