Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby "Spectrum".

A case of congenital self-healing Langerhans cell histiocytosis (CSHLCH), also known as Hashimoto-Pritzker disease, is reported. The newborn presented as blueberry muffin baby at birth, showing numerous non-blanching blue-purplish and dark-red papular, nodular lesions without documented infections and systemic involvement. Histopathological and immunohistochemical findings were suggestive for Langerhans cell histiocytosis. During the first 12 weeks of life, the cutaneous lesions progressively and spontaneously regressed with some atrophic scars. One-year follow-up is negative for relapse of cutaneous lesions or systemic involvement, confirming the diagnosis of CSHLCH.

Asthma in adolescence: Is there any news?

Asthma is a chronic disease that has a significant impact quality of life, which is particularly important in adolescence. We will discuss aspects of epidemiology, the clinical spectrum, diagnostics, and management of asthma in adolescence. In particular, we will highlight the psychological implications of having asthma during this developmental period. Data published in the past 10 years, since we last reviewed the subject, will be the main focus of this paper. The care of the teenager with asthma should take into account the rapid physical, emotional, cognitive, and social changes that occur during normal adolescence. The diagnostic process may be more difficult since teenagers tend to deny their illness. Thus, both under-diagnosis and under-assessment of asthma severity may lead to under-treatment and potentially avoidable morbidity and even mortality. Conversely, teenagers may be often misdiagnosed as having asthma or their asthma severity may be overestimated leading to inappropriate and sometimes excessive treatment. Educational programs, environmental avoidance measures, proper use of medications along with a skilled approach, and a caring attitude of health providers are all very important for successful management. Pediatr Pulmonol. 2017;52:129-138. © 2016 Wiley Periodicals, Inc.

Ground zero: not asthma at all.

Upper airway obstruction is commonly misdiagnosed as asthma. We report on four children with recurrent respiratory symptoms who had been erroneously diagnosed as having asthma and who received anti-asthma medication for several years. The evaluation of spirometry tracing was neglected in all cases. Subglottic stenosis, tracheomalacia secondary to tracheo-esophageal fistula, double aortic arch, and vocal cord dysfunction were suspected by direct inspection of the flow-volume curves and eventually diagnosed. The value of clinical history and careful evaluation of spirometry tracing in children with persistent respiratory symptoms is critically discussed.

Leukotriene modifiers in the treatment of asthma in children.

Asthma is one of the most common respiratory disorders in clinical practice, affecting up to 13% of people worldwide. Inflammation is the most important component of asthma and inhaled corticosteroids (ICS) are recommended as the first line controller treatment for patients of all ages. Treatment with corticosteroids is often unable to fully control asthma symptoms and progression. Recently, leukotrienes have come to the forefront of research as they have been found play a pivotal role in the airway inflammatory process, and specific drugs have been developed to target them. Cysteiny leukotriene antagonists (LTRAs) have recently emerged as important therapeutic options that show a large potential clinical utility. Three specific LTRAs are licensed for clinical use: montelukast, zafirlukast and pranlukast, although montelukast is the only drug approved in the paediatric age range. It is well tolerated (although adverse effects such as headaches, abdominal pain, rashes, angioedema, pulmonary eosinophilia and arthralgia have been reported) and shows many positive effects in asthmatic patients. Current Global Initiative for Asthma guidelines recommend LTRAs as: (1) a second choice treatment to ICS for patients with mild persistent asthma, (2) an add-on therapy to reduce the dose of ICS in patients with moderate or severe asthma, due to the different and complementary mechanisms of action of these agents. LTRAs may be particularly appropriate choices in a number of clinical situations, including the following: patients with concomitant rhinitis; patients with viral-induced wheeze; patients with exercise-induced bronchoconstriction (EIB) and, in children aged 2-5 years, to reduce the frequency of asthma exacerbations.

Aspiration lung disease.

The term aspiration lung disease describes several clinical syndromes, with massive aspiration and chronic lung aspiration being at two extremes of the clinical spectrum. Over the years, significant advances have been made in understanding the mechanisms underlying dysphagia, gastroesophageal function, and airway protective reflexes and new diagnostic techniques have been introduced. Despite this, characterizing the presence or absence of aspiration, and under what circumstances a child might be aspirating what, is extremely challenging. Many children are still not adequately diagnosed or treated for aspiration until permanent lung damage has occurred. A multidisciplinary approach is mandatory for a correct diagnosis in addition to timely and appropriate care.

Apoptosis, airway inflammation and anti-asthma therapy: from immunobiology to clinical application.

T lymphocyte apoptosis is essential for maintaining immune system homeostasis. Experimental evidence suggests apoptosis control mechanisms may be impaired in inflammatory conditions, particularly airway Th2-type allergic diseases. This review briefly examines the mucosal immune system homeostasis and common apoptotic pathways and discusses impaired apoptosis, allergy, airway inflammation, remodelling and fibrosis. Finally, the paper presents an update on pharmacological targeting of apoptosis to control airway inflammation in patients with allergic asthma.

Montelukast sodium for exercise-induced asthma.

Exercise is the most common trigger of bronchospasm in children with asthma. Exercise-induced bronchoconstriction (EIB) occurs in up to 90% of individuals with asthma who are not under antiinflammatory treatment and in about 40% of those with allergic rhinitis. EIB may be an overwhelming problem in childhood and adolescence, when physical activity is a prominent component of daily life. The importance for those with asthma to maintain regular physical activity is recognized by asthma guidelines, which include the recommendation of full participation in sporting activities in their goals for the management of asthma. Cysteinyl leukotrienes, which are derivatives of the 5-lipoxygenase pathway of arachidonic acid metabolism, are important mediators of airway allergic inflammation and have a role in the pathogenetic mechanism of EIB. Montelukast sodium (Singulair, Merck & Co.) is a selective and orally active leukotriene receptor antagonist with demonstrated activity for treating asthma and allergic rhinitis. The effect of this drug in the prevention of EIB has been evaluated in a variety of studies in adults as well as in children. Both single-dose and regular-treatment studies showed that montelukast provides effective protection against EIB. Furthermore, chronic treatment with montelukast does not induce tolerance to the bronchoprotective effect over time. This aspect is particularly relevant for children, who tend to be active at frequent and irregular intervals throughout the day, and who therefore may benefit from around-the-clock pharmacologic protection.

The challenge of asthma in adolescence.

The adolescents with asthma are a distinct group of patients with different problems and needs compared to children and adults. Specific issues of asthma in adolescence are the variability of the clinical spectrum, the presence of particular risk factors for the persistence of symptoms, underdiagnosis and undertreatment of the disease. Refusal of the sick role, denial of symptoms, carelessness about dangerous inhalation exposure, erratic self-medication, overexertion without taking precautions against exercise-induced asthma, and a poor relationship between patients, their families, and often doctors are the main obstacles to successful management of asthma in this critical age. There are also major problems of compliance for these patients. The goal of optimal quality of life will be achieved only if the physician thoroughly understands the adolescent's needs and provides optimal care.

Cough, wheezing and asthma in children: lesson from the past.

Recurrent cough is an important medical and sometimes frustrating problem encountered by pediatricians, especially if it is not associated with wheeze or other evidence of systemic disease. In the past, many children with chronic cough have been diagnosed as asthmatic. However, epidemiological, clinical and laboratory studies conducted over the last ten years elucidated many aspects of the relationship between cough and bronchoconstriction. It is difficult to make a diagnosis of asthma on cough alone, but the setting in which the patient is seen (i.e., in a primary care setting or ain a specialistic clinic) may address doctors to the right diagnosis. A prudent ''wait and see'' approach may be useful in many occasions.