Simultaneous liver and pancreas transplantation in patients with cystic fibrosis.

BACKGROUND: Improved survival in patients with cystic fibrosis (CF) has led to an increased incidence of extrapulmonary complications of this disease. Of these, cirrhosis and pancreatic insufficiency, including CF-related diabetes (CFRD) and exocrine insufficiency, are significant causes of morbidity and mortality. Liver transplantation is the treatment of choice for cirrhosis in this setting, but the addition of an isolated simultaneous pancreas transplant in patients with CFRD has not been reported. METHODS: Two female patients with CF underwent simultaneous pancreas and liver transplantation. Both had pancreatic insufficiency, CFRD, cirrhosis, and preserved renal function. In each case, the liver and pancreas were procured from a single cadaveric donor. The liver transplant was performed first. A lower midline extension was added for improved exposure of the iliac vessels. The donor pancreas transplant was performed with systemic venous drainage and enteric exocrine drainage. Immunosuppression included rabbit anti-thymocyte globulin, tacrolimus, mycophenolate mofetil, and early steroid withdrawal. RESULTS: Both patients recovered well with normal liver function, resolution of portal hypertension, and normal blood glucoses independent of insulin. As a result of the enteric exocrine drainage of the pancreas, they are now independent of supplemental pancreatic enzymes. CONCLUSIONS: Simultaneous liver and pancreas transplantation in CF patients provides the advantages of normalization of glucose and improved nutrition for patients requiring liver transplantation and should be considered in CF patients with CFRD who require liver transplants.

Lymphocytic interstitial pneumonitis, elevated IgM concentration, and hepatosplenomegaly in ataxia-telangiectasia.

An 8-year-old girl developed ataxia-telangiectasia. Western blotting of lysate revealed absence of the ATM protein, and 2 mutations in the ATM gene were found. Subsequently, the patient developed increased respiratory symptoms. Open lung biopsy revealed lymphocytic interstitial pneumonitis, which is not characteristic of ataxia-telangiectasia. There was a therapeutic response to glucocorticosteroid treatment.

Medical care of the adolescent with asthma.

Asthma is the most common chronic disease in adolescents. Despite advances in the understanding of this disease and the availability of more specific treatment, the prevalence of asthma and its morbidity and mortality are increasing. This trend is especially prominent and worrisome in the age group that includes adolescents and young adults. Possible factors contributing to this significant problem in adolescents include a lack of knowledge about the disease, delays in seeking medical attention due to denial of symptoms or overuse of acute relief medication that mask the progression of the inflammation, and various psychological problems such as low self-esteem or depression. These factors, in addition to the typical developmental behaviors recognized in adolescence, contribute to the complexity of asthma management in this age group. This article comprehensively reviews the pathophysiology and precipitating factors of asthma as well as all aspects of medical care of affected individuals, including monitoring and self-care.

Current concepts on interstitial lung disease in children.

Pediatric interstitial lung disease comprises a diverse group of rare conditions characterized by an infiltrative process, abnormal gas exchange, and restrictive lung disease. Although the disorder is similar to its adult counterpart, its course is complicated by the continued need for lung growth and differentiation in infants and children. Knowledge about the pathogenesis, prognosis, and treatment of pediatric interstitial lung disease is limited. Investigators are focusing on defining the cellular mediators of the interstitial damage and describing the role of viral infections in and possible genetic predisposition to interstitial lung disease. Clinicians continue to define the various types of interstitial lung disease and to evaluate the roles of bronchoalveolar lavage, radiography, and biopsy in diagnosis. Together, investigators are working toward the development of specific, targeted therapy that will reduce the significant morbidity and mortality seen in pediatric interstitial lung disease.