The recent advances in the basic hematology and immunology have significantly enhanced the understanding of histiocytic disorders. The Histiocyte Society which was established in 1985 enabled the randomized trials for these diseases, and important knowledge regarding pathogenesis, clinical presentation, diagnosis, therapy and late consequences has been obtained. The treatment of Langerhans cell histiocytosis (LCH) has varied greatly over last decades,... |
Aplastic anemia is a rare disease, which is characterized by pancytopenia and hypocellular bone marrow without infiltration of abnormal cells or fibrosis. The incidence in Asia is higher than in the West and new cases are diagnosed at a rate of 5.1 per million pediatric populations per year in Korea. The pathophysiology is understood roughly by defective hematopoiesis, impaired bone... |
To understand the hemolytic anemia (HA) in children, the diagnostic approach and management of hereditary and acquired HA are described. The hereditary hemolytic anemia (HHA) can be classified according to the pathogenesis into three types : RBC membrane defects, hemoglobinopathies, and RBC enzymopathies. Clinical characteristics, laboratory findings and molecular defects of these three types are presented briefly. In Korea,... |
Even though we drink and excrete water without recognition, the amount and the composition of body fluid remain constant everyday. Maintenance of a normal osmolality is under the control of water balance which is regulated by vasopressin despite sodium concentration is the dominant determinant of plasma osmolality. The increased plasma osmolality (hypernatremia) can be normalized by the concentration of urine,... |
Measurement of lung function is an integral component of respiratory physiology and of clinical assessment of lung diseases in school age children and adults. Pulmonary function test of infants and children under the age of 2 years have now been standardised and are being used both in research and as an adjunct to clinical management. By contrast, until recegntly, children... |
Many previous studies have proved that human allergic disease resulted from the formation of antibodies belonging to a unique immunoglobulin isotype termed immunoglobulin E (IgE). Most of IgE- producing plasma cells are found in the lymphoid tissue associated with the gastrointestinal and respiratory tracts. IgE may be found free in the mucosal secretions of these tissues, bound to local mast... |
Allergy skin prick test and intradermal test represent one of the major tools in the diagnosis of IgE- mediated diseases like as atopic asthma, allergic rhinitis, atopic dermatitis, food and drug allergy, and insect bite when properly performed. Skin tests are of particular importance in fields such as allergen standardization, pharmacology, and epidemiology. Even if skin tests seem easy to... |
The link between upper airway disease (allergic rhinitis and sinusitis) and lower airway disease (asthma) has long been of interest to physicians. Many epidemiological and pharmacological studies have provided a better understanding of pathophysiologic interrelationship between allergic rhinitis and asthma. The vast majority of patients with asthma have allergic rhinitis, and rhinitis is a major independent risk factor for asthma... |
Sinusitis is a common medical problem in children. The prevalence of penicillin-resistant S. pneumoniae infection has been increased during the last decade. The medical management is based on the choice of antimicrobial agents. This article reviews current literatures on the management of acute bacterial sinusitis and chronic sinusitis, with an emphasis on penicillin-resistant S. pneumoniae infection. This article also explores... |
Although most clinicians now agree that sinusitis can afflict children of all ages, appropriate diagnosis remain controversial. Sinusitis is one of the most challenging diagnoses for a clinician, because there is a lack of validated diagnostic criteria for acute rhinosinusitis. Symptoms generally include nasal congestion, purulent nasal discharge, and cough .The physical examination is often unsuccessful in confirming the diagnosis.... |
Majority of sick full term newborns have adequate adrenal cortical function in response to stress. Acutely ill neonates with a basal cortisol level less than 15 g/dL (414 nmol/L) suggest adrenal insufficiency and require function testing of adrenal function. In premature infant, immaturity of hypothalamic-pituitary adrenal axis (HPA axis), may limit the ability to increase cortisol production in response... |
In the early neonatal period, the neonate is challenged by the loss of the placental calcium transport and manifests a quick transition, from an environment in which PTHrP plays an important role to a PTH- and 1,25-dihydroxyvitamin D-controlled neonatal milieu. Disturbances in mineral homeostasis are common in the neonatal period, especially in premature infants and infants who are hospitalized in... |
The fetus is completely dependent on mother for glucose and other nutrient transfer across the placenta. At birth, when the maternal supply is discontinued, the neonate must adjust to an independent existence. The changes in the neonate's glucose homeostasis during this transition to the extrauterine environment are influenced by the mother's metabolism and intrinsic fetal and placental problems. Maturation of... |
Patients with febrile illness and skin rashes need full and immediate attention. In general, these diseases show mild manifestations and good prognosis. However, causalities of some diseases with fever and rash may be life threatening or trivial. So, the differential diagnosis for those patients is extensive. A through history, a careful physical examination and close observation of clinical progress are... |
Fever of unknown origin (FUO) has been a convenient term used to classify patients who warrant a particular systemic approach to diagnostic evaluation and management. The greatest clinical concern in evaluating FUO is identifying patients whose fever has a serious or life-threatening cause when a delay in diagnosis could jeopardize successful intervention. Thorough history and complete physical examination are critical... |
Fever has been recognized as a cardinal feature of disease since antiquity, but only recently has the pathophysiology of fever come to be understood. It became clear that the ultimate cause of fever is not a bacterial product (a so-called exogenous pyrogen) but a product of host inflammatory cells (i.e., an endogenous pyrogen). Many studies have demonstrated that mononuclear phagocytes... |
Advances in neonatal care have been responsible for the improved survival of prematurity but have not resulted in decreased morbidity. Once the high-risk infants is discharged from the hospital, his or her many special care needs do not cease. A well-coordinated multidisciplinary approach is essential in the follow-up care of these infants. Special attention must be given to their growth... |
Hearing loss in newborns is the most frequently occurring birth defect. If hearing impaired children are not identified and managed early, it is difficult for many of them to acquire the fundamental language, social and cognitive skills that provide the foundation for later schooling and success in society. All newborns, both high and low risk, should be screened for hearing... |
In the United States, The concept of designation for hospital facilities that care for newborn infants according to the level of complexity of care provided was first proposed in 1976. The extent of perinatal health care regionalization varies widely from one area to the other. facilities that provide hospital care for newborn are classified into three categories on the basis... |
Inherited metabolic disease is rare disorders that show symptoms mainly in pediatric age and early treatment is important for preventing complications of the disease. Recent development in molecular and biochemical techniques help clinicians with proper diagnosis of patients, however, many of the disease still remain lack of effective therapeutic strategies. Better understanding on biochemical and molecular basis of pathogenesis of... |
Inherited metabolic disorders are individually rare but as a whole, they are nor rare. Since Archibald Garrod introduced a concept of “inborn error of metabolism” or “chemical individuality”, more than 500 diseases are currently known, affecting approximately one in 500 newborns cumulatively. They frequently manifest with acute, life-threatening crisis that require immediate specific intervention or they present with insidious diverse... |
In 1991, the Ministry of Health & Social affairs adopted a nationwide service program for neonatal screening of phenylketonuria, galactosemia, maple syrup urine disease, homocystinuria, histidinemia & congenital hypothyroidism for newborns delivered from low class pregnant women registered in health centers. Government decreased the test items from six to two, PKU & congenital hypothyroidism to increase test numbers with same... |
In this article, the following topics will be discussed: What is a developmental disability? What are the risk factors for developmental disability? What are the causes of delayed motor development? What are the early manifestations of developmental disability in young infants? What are the goals of early intervention and the forms or types of early intervention services? What are the... |
Mental retardation(MR) is one of the most common developmental disabilities, which is characterized by deficits in intellectual and adaptive functions. Most children with MR have cognitive limitation in the mild range. With respect to the etiology, it is believed that genetic and environmental factors are interrelated and show variable pictures. Most children with MR present with speech and language delay... |
Motor delay, when present, is usually the first concern brought by the parents of children with developmental delay. Cerebral palsy that is the most common motor delay, is a nonspecific, descriptive term pertaining to disordered motor function that is evident in early infancy and is characterized by changes in muscle tone, muscle weakness, involuntary movements, ataxia, or a combination of... |
The population of adult patients with congenital heart disease(CHD) has increased dramatically because of improved survival of infants and children with CHD. There are still a substantial number of adults with unrepaired CHD. Many adult patients do not know the possible problems of their disease such as ventricular dysfunction, arrhythmia, and what they should pay attention to. They often consider... |
Although antiarrhythmic medication has been the main treatment modality for arrhythmia in children, in recent decades technological development and computerization have made great advances in nonpharmacological therapy. This article reviews the transcatheter radiofrequency ablation for tachycardia in children, recent advances of device therapy for bradycardia, antitachycardia pacing, implantable cardioverter defibrillator. As a new field of device therapy, cardiac resynchronization therapy... |