Acute respiratory distress in patients with cystic fibrosis may be due to metabolic causes and the alteration of the hydrocarbon metabolism is a frequent complication in these patients. Cystic fibrosis-related diabetes is due to a decrease in insulin secretion secondary to pancreatic insufficiency as the primary cause; however, glucocorticoid treatment and other factors (malnutrition, hepatic dysfunction, infections...) can intervene by creating insulin resistance. Glycemic control is essential in respiratory exacerbations and in patients treated with hyperglycemic drugs, for example oral glucocorticoids as a treatment of allergic bronchopulmonary aspergillosis.

A 20-month-old boy with cystic fibrosis (ΔF508/ΔF508) and deferens vas absence as a finding during an orchidopexy. We review the genetic variations observed in patients with bilateral and unilateral congenital absence of deferens vas, as well as the cystic fibrosis transmembrane conductance regulator role in others aspects related to male fertility.

During the past two decades, the prevalence of methicilin-resistant Staphylococcus aureus (MRSA) has significantly increased. MRSA frequently infects children. Traditionally, MRSA infections were confined to those with predisposing healthcare-associated risk factor or those who frequented healthcare facilities but its prevalence is increasing between healthy children in the community. The objective of this study was to describe the demographic and clinical data of the patients with a MRSA infection at our centre between October 2009 and December 2011. Analyzed data demonstrate the utility of infection control surveillance program and the isolation precautions to prevent the transmission of MRSA. Most patients with community-acquired MRSA infection acquired MRSA during contact with the health care system.

Background: Recurrent pneumonia (RP) is defined as the presence of radiographic infiltrates recurring after complete resolution of initial pneumonic process. Although it is a common cause of demand for assistance in pediatric respiratory units, there are few studies to evaluate the actual impact.
Patients and methods: A descriptive study of patients diagnosed with RP in the Pediatric Respiratory Unit of Carlos Haya's University Hospital (Málaga), both ambulatory and in hospital wards during a period of one year. We proceeded to the devel­opment of a database through an evaluation questionnaire.
Results: We collected 157 patients with RP, 61.8% attended in outpatient clinics and 37.6% in hospital wards. Etiological diagnosis was established in 97.5% of cases, the most frequent asthma (37.2%), cystic fibrosis (23.7%) and aspiration syndrome (11.5%). In 75.3% of recurrences location was variable, 14.3% were unique location and 9.7% had interstitial distribution.
Conclusions: The clinical history and detailed physical examination, with radiographic features, guide the diagnosis of RP in most cases. Choose of additional tests depends on the location of recurrences, the patient's age and the prevalence of the diseases. In our study, overall, the most common underlying causes of RP were asthma, cystic fibrosis and chronic aspiration syndromes.

Cystic fibrosis (CF) is an autosomal recessive exocrine disease affecting multiple organ systems. The defect associated with CF is in the cystic fibrosis transmembrane regulator (CFTR), which acts primarily as a chloride channel. Patients with CF usually present with respiratory and/or gastrointestinal abnormalities. The severity of the disease is multifactorial, one of the factors depends on the level of activity of the CFTR protein, which is related with the mutation type that affects the patient. An infant is presented who developed recurrent episodes of anorexia, weight loss, dehydration and electrolyte abnormalities. CF was diagnosed showing an unusual and not very publicized presentation of the disease. Mutations R334W and 1812-1G-A were found. CF should be considered in patients of any age, but particularly in infants, presenting with recurrent episodes of hyponatremic hypochloremic dehydration with metabolic alkalosis unexplained by other causes, even in the absence of respiratory or gastrointestinal symptoms or failure to thrive.

Because of improved life expectancy of patients with cystic fibrosis (CF), the attention has increased towards other signs of the disease such as the hepatic affectation. The cystic fibrosis related liver disease (CFRLD) has become the second cause of mortality in these patients. Clinical diagnosis of liver disease in cystic fibrosis is difficult because the fibrotic liver lesion usually develops in asymptomatic individuals. In recent years, advances in the understanding of the pathogenesis of disease and of the mechanisms of liver fibrogenesis are leading to novel therapeutic approaches, as well as to identify patients at risk for the development of liver disease. However, at present ursodeoxicholic acid (UDCA) is the only available treatment for these patients. The aim of this work is to update the knowledge about the pathogenesis and diagnosis of CFRLD and to review the mechanisms of action of UDCA and its benefits when used in these patients.

Cystic fibrosis is a genetic disorder characterized mainly by a chronic progressive pulmonary disease and pancreatic insufficiency but with a wide range of clinical manifestations. Over the last years breakthroughs in the understanding of its pathogenesis and the development of new therapies have lead to outstanding improvements both in survival and quality of life. Its prognosis mostly depends on the severity of the pulmonary impairment but adequate control of the gastrointestinal manifestations, ensuring a good nutritional condition is essential for achieving a good outcome. The digestive aspects are various and they will be examined from the most recent points of view of therapeutical diagnosis. Liver disease is quite common. It often has an early onset but most patients have competitively mild impairment with only a few developing multifocal cirrhosis with conducts to a portal hypertension and hypersplenism. Pancreatic exocrine insufficiency occurs in over 90% of the patients and requires of a replacement enzyme therapy. Other complications like unresponsive steatorrhea control, intestinal inflammation and distal intestinal obstruction syndrome have acquired special relevance in the last years.

Chronic malnutrition has been a universal problem and at the present moment the nutritional monitoring and support are major and essential parts within the disease. Are the importance of an adequate nutritional state is well known and it is also known that it has an effect on the mortality, that it diminishes the morbidity and that it could favor a better pulmonary function. Currently keeping the patients BMI around the 50th percentile is a reasonable target. The therapies to achieve this goal include diet monitoring ensuring a high caloric intake, energy supplements can be added and in restricted cases more aggressive intervention as enteral feeding might be needed. Adequate intake of micronutrients and liposoluble vitamins are important recommending a systematic administration in the last years, with the increase of life expectancy other nutritional challenges are brought up, such as the deficiency of essential fatty acids or the keeping of a good bone health.