Transplantation of allogeneic CD34-selected stem cells after fludarabine-based conditioning regimen for children with mucopolysaccharidosis 1H (M. Hurler).

Hurler syndrome (MPS1H) is a progressive inborn error of mucopolysaccharide metabolism leading to premature death. Allogeneic hematopoietic cell transplantation (HCT) can achieve stabilization and improve long-term survival. However, large studies have shown that preparative regimen-related toxicity (RRT) and graft failure rates have been relatively high.

Influence of pimecrolimus cream 1% on different morphological signs of eczema in infants with atopic dermatitis.

Background: In the published studies on the efficacy of the topical immunomodulator pimecrolimus, different eczema scores were used, and the impact on morphological key signs of eczema was not analysed.

Objective: To compare the influence of pimecrolimus cream 1% on different standard eczema scores in infants with atopic dermatitis and to analyse the impact of treatment on the individual morphological key signs of eczema.

Methods: Pimecrolimus cream 1% (n = 129) or double-blind vehicle control (n = 66) was administered for 4 weeks.

Cell-to-cell contact between activated CD4+ T lymphocytes and unprimed monocytes interferes with a TH1 response.

Background: The production of IL-12 by monocytes and dendritic cells is tightly regulated and critically dependent on signals from surrounding cells in the micromilieu.

Objective: We studied the capacity of T cells to regulate directly the production of IL-12 by human monocytes.

Methods: Human isolated CD4 + T cells from peripheral blood and T cells from acute patch test lesions were cocultured with autologous monocytes.

T-helper 2 cytokines attenuate senescent eosinophil activation by the CXCR4 ligand stromal-derived factor-1alpha (CXCL12).

Background: Different chemokine receptors have been suggested to play a pivotal role in allergic diseases and therefore to be relevant for the activation of effector cells and propagation of the inflammatory response. The CXC chemokine receptor CXCR4 has recently been found on the surface of eosinophils implicating a role in allergic diseases.

Objective: The aim of this study was to investigate the functional expression of CXCR4 on senescent eosinophils.

The Contegra bovine valved jugular vein conduit for pediatric RVOT reconstruction: 4 years experience with 108 patients.

Background: Porcine xenografts for pediatric right ventricular outflow tractus (RVOT) reconstruction lack durability, homografts lack availability. The Contegra is a valve bearing bovine jugular vein. It is tear resistant, well pliable, and permits RVOT reconstruction without further foreign material.

Contegra pulmonary valved conduits cause no relevant hemolysis.

Background: Contegra is a bovine jugular vein graft for pediatric RVOT reconstruction. Regular blood draws belong to a (controlled phase I) study protocol aiming at FDA-admission that included the first 60 patients. After 3.

Chronic urticaria and infections.

Purpose Of Review: The pathogenesis of chronic urticaria is multifactorial and a specific treatment is lacking. In acute urticaria there is no doubt of a causal relationship with infections and all chronic urticaria must start as the acute form. However, in the chronic form a primary role for infection is controversial, although it is undeniable that concurrent infections exacerbate the condition.

Role of food allergy in atopic dermatitis.

Purpose Of Review: Food allergy and atopic dermatitis often occur in the same patient. Based on clinical data from the past few decades, it is clear that foods such as cow's milk and hen's eggs can directly provoke flares of atopic dermatitis particularly in sensitized infants, whereas inhaled allergens and pollen-related foods are of greater importance in older children, adolescents and adults. Because the role and immunology of food allergy in atopic dermatitis remain controversial, here we review data that mainly focus on skin eczema and food allergy.

Birch pollen-related food as a provocation factor of allergic symptoms in children with atopic eczema/dermatitis syndrome.

Background: Food allergy to cow's milk or hen's egg is a common problem in children with atopic eczema/dermatitis syndrome (AEDS) but the role of birch pollen-related food for the induction of allergic symptoms is still not clear.

Patients/methods: Twelve children (median age 5 years) with AEDS underwent an oral challenge with those birch pollen-related foods which were reported to induce no immediate symptoms, but were consumed on a regular basis. Total IgE and specific IgE to birch pollen, Bet v 1/2 and various birch pollen-related foods were determined.

The RACHS-1 risk categories reflect mortality and length of hospital stay in a large German pediatric cardiac surgery population.

Objectives: The Risk Adjusted classification for Congenital Heart Surgery (RACHS-1) was published in January 2002, based on 4370 operations registered by the Pediatric Cardiac Care Consortium. It is designed for being easily applicable also for retrospective analysis of hospital discharge data sets; the classification was not developed for patients with heart transplantations, ventricular assist devices or patients above 18 years. We apply this classification to our 2368 correspondent procedures that were performed consecutively on 2223 patients between June 1996 and October 2002 in Bad Oeynhausen and analyze its relation to mortality and length of hospital stay.

Intermittent aortic cross-clamping for isolated CABG can save lives and money: experience with 15307 patients.

Background: The ideal myocardial protection during isolated CABG is still a matter of debate. Cardioplegia versus intermittent aortic cross-clamping (IACC) are the main opponents; the following article shows that IACC can be safe, efficient and might be cheaper than cardioplegia.

Methods: Demographics and co-morbidities of 15307 CABG only patients consecutively operated on between January 1993 and October 2001 in the Heart Center in Bad Oeynhausen were assessed by the German Quality Assurance data set and risk-stratified using the EuroSCORE.

Late eczematous reactions to food in children with atopic dermatitis.

Background: Food allergy is a common problem in patients with atopic dermatitis (AD), particularly in children. While immediate reactions to food are well characterized, the importance of food as a provocation factor for late eczematous reactions has been a subject of debate for several decades.

Objective: To investigate the importance of food for the induction of late eczematous reactions in children with AD and to correlate the clinical outcome to the results of specific IgE determinations and atopy patch tests (APTs).

Co-expression of transforming growth factor-beta1 and glial cell line-derived neurotrophic factor in vestibular schwannoma.

Hypothesis: Transforming growth factor-beta1, glial cell line-derived neurotrophic factor, and their receptors are expressed in vestibular schwannoma, and the expression data correlate with the proliferation activity (Ki-67 labeling index) and the clinical growth rate of vestibular schwannoma tissue.

Background: Glial cell line-derived neurotrophic factor is a potent growth factor for the central and peripheral nervous system. Recent results demonstrate that glial cell line-derived neurotrophic factor requires transforming growth factor-beta to exert its trophic effect on neural tissue.

Human monocyte-derived dendritic cells are chemoattracted to C3a after up-regulation of the C3a receptor with interferons.

The anaphylatoxin C3a is an important inflammatory mediator in the innate and adaptive immune systems. Recent reports in various animal models have fostered the role of C3a in mediating allergic reactions such as pulmonary allergies. However, data in humans are limited and the cellular targets for C3a are not fully understood.

The application of region-specific probes for the resolution of duplication 8p: a case report and a review of the literature.

The structural rearrangement in the short arm of a chromosome 8 in a clinically affected patient has been reinvestigated by FISH using whole chromosome painting and region specific YAC probes. An inverted duplication of the segment p22-->p11.2 and a deletion of the subtelomeric region were demonstrated.

Recurrent carcinoma of the head and neck: treatment strategies and survival analysis in a 20-year period.

Following clinical diagnosis of a recurrent tumor, curative treatment is seldom available. Depending on the size of the recurrent tumor and the patient's general health condition extensive surgical resections and reconstructions are avoided in favor of non-surgical palliative intended treatment modalities. According to the literature location of the tumor, tumor size, as well as R-1- and R-2-resection rates are the most frequent reasons for the development of recurrent tumors.

Comparison of localized high-dose UVA1 irradiation versus topical cream psoralen-UVA for treatment of chronic vesicular dyshidrotic eczema.

Background: Vesicular dyshidrotic palmoplantar eczema is a common disorder but treatment is difficult. Localized photochemotherapy (cream psoralen-UVA [PUVA]) has widely been used for therapy. Although the efficacy of cream PUVA therapy is well known, potential side effects may occur.

Human dendritic cells express the IL-18R and are chemoattracted to IL-18.

IL-18 is secreted by a variety of cells such as epithelial cells, macrophages, and dendritic cells (DC), in particular, in areas of chronic inflammation. The effects of IL-18 are complex and not fully understood thus far. We sought to explore human DC as a new target for IL-18, since IL-18R expression has been described on myeloid cells such as macrophages and DC are likely to get in contact with IL-18 at sites of inflammatory reactions.

Up-regulation of C5a receptor expression and function on human monocyte derived dendritic cells by prostaglandin E2.

The expression of the C5a-receptor (C5aR) on dendritic cells, its regulation and function have not been well established thus far. We show that the C5aR is expressed on human monocyte-derived dendritic cells (DC) and can be down-regulated by maturation stimuli such as tumour necrosis factor-alpha (TNF-alpha), lipopolysaccharide (LPS) or CD40L and by the T helper 1-cytokine interferon-gamma (INF-gamma). Prostaglandin E2 (PGE2), a proinflammatory mediator supporting dendritic cell activation and necessary for adequate DC migration, leads to the up-regulation of C5aR expression when incubated alone and prevents down-regulation when given in combination with TNF-alpha or LPS.

Atopic dermatitis management with tacrolimus ointment (Protopic).

Tacrolimus ointment is the first of a new class of non-steroidal topical immunomodulators indicated for the treatment of atopic dermatitis. Topical tacrolimus has been subject to an extensive clinical development program involving more than 16,000 patients. A clinical trial program, including vehicle-controlled studies, short- and long-term comparative studies and long-term safety studies, has investigated tacrolimus 0.

Atopic dermatitis management with tacrolimus ointment (Protopic®).

Tacrolimus ointment is the first of a new class of non-steroidal topical immunomodulators indicated for the treatment of atopic dermatitis. Topical tacrolimushas been subject to an extensive clinical development program involving more than 16,000 patients. A clinical trial program, including vehicle-controlled studies, short- and long-term comparative studies and long-term safety studies, has investigated tacrolimus 0.

Microsurgical reconstruction in the head and neck region: an 18-year experience with 500 consecutive cases.

Background: Microvascular free tissue transfer has become a significant factor in the reconstruction of head and neck cancer patients. Various donor sites are available to enable anatomical and functional repair of a defect.

Methods: All free vascularized tissue transfers performed during the period from March 1982 to September 2000 were retrieved from this hospital's database and analysed with regard to the surgical defect, chosen donor site and complications.

Maxillofacial manifestations of Langerhans cell histiocytosis: a clinical and therapeutic analysis of 10 patients.

The definition of Langerhans cell histiocytosis (formerly known as histiocytosis X) includes the clinical syndromes Hand-Schueller-Christian syndrome, Abt-Letterer-Siwe syndrome, and eosinophilic granuloma. The paper gives an overview of current diagnostic and treatment strategies of LCH. Furthermore, records and clinical data of 10 patients with LCH were evaluated retrospectively.

Treatment of gustatory sweating (Frey's syndrome) with botulinum toxin A.

Background: Gustatory sweating is a common complication of parotid surgery. Injection of botulinum toxin A has been reported as a safe and effective treatment option for patients with Frey's syndrome.

Patients And Methods: A total of 69 patients who had undergone superficial parotidectomy because of adenoma were evaluated with respect to the incidence of Frey's syndrome and treatment interest.

Search for deletion 22q11.2 in interphase nuclei of buccal mucosa of patients ascertained by isolated cleft palate: a new diagnostic approach.

A new approach for the detection of chromosome deletion 22q11.2 in interphase nuclei from buccal mucosa cells obtained by a non-invasive procedure is described. FISH analysis has been performed on samples from a group of 101 patients that presented consecutively for speech therapy and/or surgical correction of cleft palate.