Current Pediatric Reviews - Volume 2, Issue 2, 2006

Cystic fibrosis is a monogenic disorder with significant morbidity and mortality, despite advances in conventional treatment. It is a good candidate for gene therapy and this field has progressed rapidly since the cystic fibrosis transmembrane conductance regulator gene was cloned. We will review the specific questions to address for successful cystic fibrosis gene therapy, such as the extra- and intracellular barriers to airwa Read More

Once the genetic defect of cystic fibrosis (CF), the most common autosomal recessive disease, was discovered, there was hope for quick positive results with gene-therapy. These did not came true, however. Recent CF mice studies by Freedman et al. demonstrated a disease reversal by high doses of docosahexaenoic acid (DHA). Although it is known for long that the same essential fatty acid imbalance with low levels of DHA i Read More

Nutrition is age specific, in all aspects. The intake of young children depends on their caretakers. It is obvious that growth and development cause major changes in children. However, these changes not only consider size but also body composition and maturation of many (digestive) functions. Energy and nutrient requirements do not only depend on intake, but also on expenditure. The latter depends on many variables such as Read More

Infection with Neisseria meningitidis remains one of the most devastating illnesses in pediatrics. Affected patients can progress from a mild viral-like illness to death within a matter of hours. Additionally, the definitive clinical diagnosis cannot be reached until the characteristic petechial rash appears, making differentiating meningococcal infection from a benign viral illness impossible. This review will discuss the epidemiology, Read More

Hypoxia-ischemia is responsible for a high morbidity and mortality rate, both in infant and adult patients. In humans, the brain is immature from fetal to postnatal life, demonstrating more specific vulnerability than the adult one, induced by exposure at perinatal asphyxia. The extension and distribution of brain lesions depend to a large extent on the intensity and exposure to injury, the maturational stage, the affected brain r Read More

Developmental delay (DD) is a commonly encountered clinical problem. Nearly most children with neurodevelopmental disorders present with developmental delay. The ultimate diagnosis may take months or even years to be confirmed. The final verdict may be purely developmental with a benign outcome, or there might be slow deterioration ending up in a more sinister cause like neurodegenerative diseases. At times, the Read More

The prevalence of asthma in children continues to increase in many parts of the world, with the highest increases in industrialized urban societies. Although the exact cause of the increase remains undefined the "hygiene hypothesis" where lower exposures to infectious agents that stimulate the production of Th1 lymphocytes thus allowing proliferation of Th2 lymphocytes that mediate allergic diseases has gained popularity. A Read More

Systemic lupus erythematosus (SLE) is more severe in children than in adults, and often needs aggressive treatment. Classical (old ) therapies include mostly non-steroid anti-inflammatory drugs, hydroxychloroquine, corticosterois and cyclophosphamide. However, these two latter drugs can induce severe side effects. Recently, new drugs, especially Mycophenolate Mofetyl and anti-CD20 antibody, have been proved to be Read More

Primary vesicoureteric reflux was felt to result from a congenitally short mucosal tunnel length with oblique intravescical insertion but more recent data suggest that primary vesicoureteric reflux is often associated with functional urodynamic abnormalities. There is now sound evidence that primary vesicoureteric reflux has a familial basis; most findings suggest a dominant inheritance with incomplete penetrance. Primary ve Read More

Paediatric surgeons were among the pioneers of laparoscopic surgery in the early 1970s. However, the vast potential of minimal access surgery in infants and children has only been more fully realised in the past decade. This has been brought about by increasing experience in paediatric laparoscopic procedures, and advances in video technology and miniaturised instruments. Very few complex paediatric surgical conditio Read More