Kostmann's disease or severe hereditary neutropenia-the man behind the syndrome.

Seventy years ago, the Swedish pediatrician Rolf Kostmann (1909-1982) was the first to report on a previous unknown lethal hereditary neutropenia in infants, Kostmann's disease. This essay presents the man behind the syndrome rather than focusing on the disease itself.

Severe eczema in infancy can predict asthma development. A prospective study to the age of 10 years.

BACKGROUND: Children with atopic eczema in infancy often develop allergic rhinoconjunctivitis and asthma, but the term "atopic march" has been questioned as the relations between atopic disorders seem more complicated than one condition progressing into another. OBJECTIVE: In this prospective multicenter study we followed children with eczema from infancy to the age of 10 years focusing on sensitization to allergens, severity of eczema and development of allergic airway symptoms at 4.5 and 10 years of age. METHODS: On inclusion, 123 children were examined. Hanifin-Rajka criteria and SCORAD index were used to describe the eczema. Episodes of wheezing were registered, skin prick tests and IgE tests were conducted and questionnaires were filled out. Procedures were repeated at 4.5 and 10 years of age with additional examinations for ARC and asthma. RESULTS: 94 out of 123 completed the entire study. High SCORAD points on inclusion were correlated with the risk of developing ARC, (B = 9.86, P = 0.01) and asthma, (B = 10.17, P = 0.01). For infants with eczema and wheezing at the first visit, the OR for developing asthma was 4.05(P = 0.01). ARC at 4.5 years of age resulted in an OR of 11.28(P = 0.00) for asthma development at 10 years. CONCLUSION: This study indicates that infant eczema with high SCORAD points is associated with an increased risk of asthma at 10 years of age. Children with eczema and wheezing episodes during infancy are more likely to develop asthma than are infants with eczema alone. Eczema in infancy combined with early onset of ARC seems to indicate a more severe allergic disease, which often leads to asthma development. The progression from eczema in infancy to ARC at an early age and asthma later in childhood shown in this study supports the relevance of the term "atopic march", at least in more severe allergic disease.

Retrospective incremental cost analysis of a hospital-based COPD Disease Management Programme in Sweden.

This paper reports on a retrospective analysis of hospital-based healthcare costs associated with the management of chronic obstructive pulmonary disease (COPD). During the second half of 2001, Simrishamn Hospital, Sweden, implemented a structured Disease Management Programme (DMP) for COPD and a total of 784 patients with COPD, enrolled in the DMP, were included in the analysis. The goal was to reduce the number of clinical events, such as severe exacerbations by early intervention, aggressive drug treatment, specialists easy available for advice, improved support for smoking cessation, increased number of scheduled follow-ups and closer tracking of high-risk COPD patients. The hospital administrative system provided data on resource consumption, such as outpatient care, inpatient care and drugs and unit cost, used in the economic analysis. The total cost of COPD drugs doubled (from euro 14,133 to euro 30,855 per year) as did the total number of outpatient visits (from 580 to 996 visits per year). The number of hospitalizations for acute COPD exacerbations and COPD with acute lower respiratory infection decreased from 67 to 25 per year. Total COPD-related healthcare costs decreased. The results presented here support the hypothesis that a COPD DMP can offer substantial overall direct cost savings.

A new model for low-dose food challenge in children with allergy to milk or egg.

BACKGROUND: Atopic eczema and food allergy are common in early childhood. Children seem to gradually develop tolerance to milk and egg, and it is a relief for families when their child can tolerate small amounts of these basic foods, even if larger doses may still cause symptoms. AIM: To develop a model for low-dose oral food challenge, facilitating re-/introduction of milk or egg. METHODS: In 39 children sensitized to milk and/or egg, we performed 52 challenges using a new standardized model for low-dose oral food challenge. The recipes were validated for blinding with sensorial tests. RESULTS: Four children challenged to milk had a positive challenge outcome. There were no significant differences with respect to family history, associated atopic manifestations, nutritional supply, eczema severity, or skin-prick test compared with the non-reacting children, but total and specific IgE values were significantly higher. All but two of the non-reacting children were able to introduce milk and egg into their diet without problems. CONCLUSION: We report recipes and a protocol to be used for standardized open and double-blind placebo-controlled low-dose food challenge in young children, enabling the introduction of small amounts of egg and milk into the diet during tolerance development.

Significant improvement of eczema with skin care and food elimination in small children.

AIM: To evaluate common methods of investigation and treatment in children younger than 2 y of age with eczema, with or without sensitization to food allergens. METHODS: One hundred and twenty-three children younger than 2 y of age with eczema and suspected food allergy were included in this prospective study. The children underwent skin-prick test with cow's milk, fresh hen's egg white and wheat. Specific IgE to milk and egg white was analysed. The eczema extent and severity was estimated with SCORAD before and after treatment. Children with a positive skin-prick test were instructed to exclude that food item from their diet. All children were treated with emollients and topical steroids when needed. RESULTS: Sixty-two of the children were skin-prick positive to at least one of the allergens; 62% had mild, 30% moderate and 8% severe eczema at their first visit. After treatment, 90% had mild, 10% moderate and 0% severe eczema. Forty-six per cent of the children had circulating IgE antibodies to milk or egg white. Ten per cent had specific IgE but negative skin-prick test to the same allergen. This subgroup improved their eczema significantly without elimination diet. CONCLUSION: The conventional treatments for children with eczema, i.e. skin care and food elimination, are effective. The beneficial effect of skin care as the first step should not be neglected, and it may not be necessary to eliminate food allergens to relieve skin symptoms in all food-sensitized children with eczema.

Moderate Dose Inhaled Budesonide Disguising Symptoms of Addison's Disease in An Asthmatic Boy with Silent Celiac Disease.

Inhaled corticosteroids are first-line treatment for asthma. Moderate doses of budesonide have been supposed not to affect hypothalamic-pituitary-adrenal axis function. We report the case of a boy with asthmatic symptoms and a late diagnosis of celiac disease, in whom inhaled budesonide in a dose used in conventional asthma therapy seems to have been systemically absorbed in amounts large enough to temporarily disguise the symptoms of a developing adrenal insufficiency. Inhaled corticosteroids in a dose used in standard asthma therapy seem to have the potential of disguising a developing Addison's disease. Furthermore, celiac disease, especially if diagnosed in late childhood, may be associated with Addison's disease causing a complex symptom pattern.