Chondrodysplasia punctata is a group of heterogeneous bone dysplasia characterized by punctate calcifications of the cartilage, frequently associated with a shortening of the limbs, cataracts, icthyosis and alopecia, alterations of the nervous system, and mental and growth deficiencies. Our case presented findings of the rhizomelic chodrodysplasia punctata : a characteristic face, a sucking difficulty and a short neck. Skeletal radiographies... |
Kikuchi-Fujimoto disease(KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limited systemic illness and it has the pathognomonic histological appearance of lymph nodes. KFD is rarely associated with systemic lupus erythematosus (SLE). The diagnosis of KFD can precede, postdate or coincide with the diagnosis of SLE. Our case describes a young woman, originally diagnosed as having Kikuchi's disease by lymph... |
Purpose : This study was performed to characterize clinical features of benign convulsions with acute gastroenteritis(CwG) in infants. Methods : We reviewed 83 consecutive seizures in 42 patients with CwG between January 1995 and December 2003. CwG was defined as convulsions having the following two characteristics : (a) seizures accompanied with symptoms of gastroenteritis without clinical signs of dehydration or electrolyte... |
Tyrosinemia type 1 is an autosomal recessive inborn error of tyrosine metabolism that caused a mutation in the gene coding for the enzyme fumarylacetoacetate hydrolase(FAH). As a result, maleylacetoacetate(MAA) and fumarylacetoacetate(FAA) are formed. The accumulated FAA is converted into succinylacetone(SA) and succinylacetoacetate(SAA) which are excreted in urine. The first report with typical clinical and biochemical findings was presented by Sakai... |
Of all toxic inhalant exposures, chlorine is one of the most common toxic chemical inhalants. When acutely inhaled, it can be responsible for symptoms ranging from upper air way irritation to more serious respiratory effects. It can also deteriorate lung function and lead to death. Chlorine and its compounds such as chlorinated cyanurates and hypochlorites are commonly used in water... |
Purpose : Although influenza virus is one of the most important causes of acute respiratory tract infections(ARTIs) in children, virus isolation is not popular and there are only a few clinical studies on influenza in Korea. We evaluated the epidemiologic and clinical features of ARTIs by influenza virus in children. Methods : From February 1995 to August 2001, nasopharyngeal aspirations were... |
Purpose : The acute scrotum is a clinical syndrome that is defined as an acute, painful swelling of the scrotum or its contents. The symptoms and signs of acute scrotum are similar and its pathognomonic features are rarely detected. This study was performed to find a proper way to diagnose and treat acute scrotum promptly and correctly. Methods : From January 1990 through December... |
Acute scrotal disorders(ASD) in neonates-especially the torsion of testes- are rare, and the clinical presentations are quite different from those of adolescents or adults. The neonates with ASD are generally afebrile and do not show typical symptoms. Early diagnosis and proper treatment of ASD are very important but the differentiation of torsion and orchitis is not easy in neonates. The... |
Apert's syndrome(acrocephalosyndactyly) is a rare congenital anomaly that is characterized by the combination of premature fusion of multiple cranial sutures primarily involving the coronal suture and severe symmetrical syndactyly of fingers and toes. Some patients of this syndrome have various abnormalities of the central nervous system. We experienced a case of Apert's syndrome with craniofacial malformations, symmetric syndactyly, occipital encephalocele... |
Septic pulmonary emboli(SPE) is derived from a variety of sources, including infected heart valves, peripheral sites of septic thrombophlebitis and infected venous catheters or pacemaker wires. In adult intravenous drug users, the most common cause of septic emboli is tricuspid valve endocarditis, but infective endocarditis in the non-complicated ventricular septal defect is a relatively rare condition in infants and... |
Acute focal bacterial nephritis(AFBN) is an unusual form of localized renal infection, which has various imaging findings and should be distinguished from abscess or other renal masses. Clinical symptoms are similar to other urinary tract infections but the diagnosis needs a CT or sonographic confirmation. Treatment, which is nonoperative, consists of intensive antibiotic therapy. We report three cases of AFBN... |
Ectopic pancreas is defined as pancreatic tissue lacking anatomical and vascular continuity with the main body of the pancreas. Common symptoms are epigastric pain, hemorrhage, chest pain, weight loss, nausea and vomiting. A 2-month-old male patient was hospitalized because of continuous bile stained vomiting for 2 days. Abdominal ultrasonography and colon study showed malrotation of the intestine. Surgical exploration revealed... |
Purpose : We evaluated the identification and antimicrobial susceptibility patterns of nosocomial and community-acquired pathogens in children of the medium-sized hospital. Methods : The 357 bacterial strains isolated from Pediatric department of Dongsan Sacred Heart Hospital were examined the species identification and antimicrobial susceptibility test during the period of March to October 1996. Results : Fifty three strains(15%) of 357 strains were nosocomial pathogens. A... |
We experienced a case of congenital acute megakaryoblastic leukemia with Down syndrome. The patient was admitted due to characteristic facial figure of Down syndrome and abdominal distension. Acute megakaryoblastic leukemia was diagnosed with abundant megakaryoblast in peripheral blood smear, severe myelofibrosis in bone marrow biopsy and positive platelet glycoprotein Ⅲa receptor. On third hospital day, the patient expired due to DIC... |
In recent years, measles outbreak has involved a great population of cases in younger children. For this reason, we reevaluated the conventional vaccination schedule by measuring measles antibody level. Two groups totalling 244 children were included in the study. The non-measles group consisted of 133 children who visited or were admitted to our hospital during the 7 month period from Jan. 1990 through July 1990... |
Enterotoxigenic Escherichia coli (ETEC) cause an acute cholera-like diarrhea in both human and animals. The disease process involves colonization of the mucosal surfaces of the small intestine followed by elaboration of ST and/or heat-labile enterotoxin (LT). Intestinal colonization by ETEC is mediated by specific surface-associated fimbrial or fibrillar antigens including colonization factor antigens (CFA) I and II. Isolates of E. coli from 114 children with... |
Acute Werdnig-Hoffmann disease is heredofamilial degeneration of anterior horn cells in the spinal cord and of motor nuclei in the brain stem, which is clinically characterized by fasciculations of the tongue, paradoxical respiration, bell shaped thorax, frog-leg posture and intact mentality. We experienced an 8 month old female infant with acute Werdnig-Hoffmann disease. We report the case with brief review of some related literatures. |
The human tail is thought to be a rare congenital anomaly which resulted from persistence of the vestigial tail of the 14 -16 mm. human embryo. A child with a tail causes for the parents and, in some cases, a feeling of stigma and shame. We experienced a case of human tail who was an 1-day old male. Examination of the baby revealed a... |