Congenital hyperinsulinism (CHI) is a rare condition that can cause irreversible brain damage during the neonatal period owing to the associated hypoglycemia. Hypoglycemia in CHI occurs secondary to the dysregulation of insulin secretion. CHI has been established as a genetic disorder of islet-cell hyperplasia, associated with a mutation of the |
We report a human parechovirus-3 (HPeV-3) infection in 2 neonates who had prolonged fever (>5 days) with palmar-plantar erythema. This distinctive rash was observed 4–5 days after fever onset, just before defervescence. Elevated aspartate aminotransferase, lactate dehydrogenase, and ferritin levels were characteristic laboratory findings in the 2 cases, suggesting tissue damage caused by hypercytokinemia. Case 1 was treated with intravenous... |
That rotavirus infection can cause neurological symptoms in young children has been well established. However, it is surprising why rotavirus infection has been overlooked as a cause of neonatal seizures for many years, despite significant research interest in neonatal rotavirus infection. Neonates are the age group most vulnerable to seizures, which are typically attributed to a wide range of causes.... |
Mumps meningitis is a common complication of mumps infection; however, information on mumps meningitis in the postvaccine era is limited. The purpose of the present study was to determine factors associated with mumps meningitis and to discuss the effect of vaccination on this disease. We retrospectively reviewed patients younger than 19 years with mumps, diagnosed at a university hospital in Korea... |
Tularemia is a potentially severe zoonotic disease caused by |
Chronic day-to-day symptoms of orthostatic intolerance are the most notable features of postural orthostatic tachycardia syndrome (POTS). However, we have encountered patients with such symptoms and excessive tachycardia but with no symptoms during the tilt-table test (TTT). We aimed to investigate whether POTS patients with chronic orthostatic intolerance always present orthostatic symptoms during the TTT and analyze the factors underlying... |
A 4-year-old girl with Panayiotopoulos syndrome presented with a history of 4 prolonged autonomic seizures. The clinical features of her seizures included, in order of occurrence, blank staring, pallor, vomiting, hemi-clonic movement on the right side, and unresponsiveness. A brain magnetic resonance imaging (MRI) showed a slightly high T2 signal in the left hippocampus. Interictal electoencephalogram revealed spikes in the... |
Purpose : The concentration of soluble transferrin receptor (sTfR) is estimated as an iron parameter to evaluate erythropoiesis and iron status. The aim of our study is to evaluate the correlation between sTfR concentration and inflammatory parameters and to distinguish iron deficiency anemia from anemia of inflammation. Methods : One hundred and forty-four infants younger than two years of age who... |
Purpose : The objective of this study was to clarify the presentation, associated preceding illness, pathologic organisms, treatment and outcome of deep neck abscess in children according to age and location. Methods : We retrospectively reviewed the in-patient charts of children treated at our hospital for deep neck abscess. Thirty-five such patients were identified as having been treated... |
Atretic cephalocele is a degenerative form of encephalocele, which is detected as a cystic mass in the head, primarily in infants. Its presentation and prognosis vary and depend on various factors, including the nature of the tissues within the cyst, other concomitant anomalies, the site of development, and the presence or absence of an embryonic straight sinus. We here report... |
True thymic hyperplasia and ectopic thymus are very rare in children. In embryologic aspect, thymus is distributed around cervical area and ends up in mediastinum. This case is simultaneous thymic hyperplasia of neck and mediastinum. Ectopic thymus in the neck and thymic hyperplasia in the mediastinum in children were reported 2 and 7 cases respectively in Korea. In Clinical aspects,... |
Purpose : This study was designed to document the etiologies and the characteristics of parapneumonic effusion in children. Methods : During a 17-year period from 1987 to 2004, parapneumonic effusion was confirmed in 86 children at Gyeongsang National University Hospital. The clinical records of these children were reviewed and radiological findings and laboratory data, especially results of thoracentesis, were analyzed retrospectively. Results... |
Purpose : Respiratory failure arises from derangements in pulmonary gas exchange. The causes may be classified as due to either lung disease or respiratory pump dysfunction. Problems with lung mechanics is an important cause of acute respiratory failure in children clinically. The aims of this study were to survey the cause and prognosis of children diagnosed with acute respiratory failure... |
Purpose : The purpose of this study was to investigate the diagnostic value of spiral computed tomography (CT) using contiguous slices with partially thin sections around the hilar level in the bronchial foreign bodies of children with a vague history of aspiration. Methods : Fourteen children were identified to be examined with spiral CT due to obscure histories of aspiration episodes.... |
ARCI syndrome consists of arthrogryposis, renal tubular acidosis, cholestatic jaundice and icthyosis. We experienced an ARCI syndrome case with corpus callosum hypoplasia and atrial septal defect. This case had oral feeding difficulty, multiple joint contracture, renal tubular acidosis and neurogenic muscular atrophy at neonatal period. At two months of age, icthyosis and cholestatic jaundice were diagnosed. The case was hospitalized... |
All complete monosomy 21 appear to be lethal early in their development in humans and only survive in mosaic forms. Complete monosomy 21 is a very rare and usually debilitating genetic disorder. Partial monosomy 21 is also rare and is thought to constitute a clinical syndrome consisting of peculiar faces, hypertonia, psychomotor retardation, and slow growth. We experienced a case... |
Purpose : The aim of this study was to determine the epidemiology of Kawasaki disease in the Kyung Nam area and to evaluate whether the results of this epidemiological study could support infectious etiology. Methods : We sent a questionnaire to three training hospitals in the Kyung Nam area and retrospectively reviewed their medical records of Kawasaki disease from Jun.... |
We report a case of trisomy 22 in a liveborn male infant which was confirmed by fluorescence in situ hybridization(FISH), macrocultures and GTG-banding, and RHA-banding procedures of peripheral white blood cells. The infant showed lung hypoplasia, which is a unique presentation, with other clinical manifestations of previously reported cases of trisomy 22, such as intrauterine growth retardation, cleft palate, micrognathia,... |
Purpose : The aim of this study was to evaluate the latest tendency of esophageal foreign body's extraction and to obtain a consensus from recent trends of indications and techniques of flexible endoscopy of esophageal FB in children. Methods : We retrospectively reviewed medical records of 127 cases with foreign bodies in esophagus at Dept. of Pediatrics and Otorhinolaryngology, Gyeongsang National... |
Mesenteric lymphangioma, which is rare and often diagnosed incidentally, is a benign tumor in infants and children. Most cases of lymphangioma are in the neck and axilla. About 5% of these lesions are in the mediastinum, mesentery or retroperitoneal region. Although most lymphangioma produce no symptoms, it may present an acute surgical condition suggesting acute intestinal obstruction. We report one... |
Chondrodysplasia punctata is a heterogenous skeletal dysplasia characterized by small focal calcifications in articular and other cartilages in infancy, with subsequent epiphysial dysplasia and associated anomalies of the face, eyes, and skin. Chondrodysplasia punctata is classified with autosomal recessive rhizomelic type, autosomal dominant nonrhizomelic type(Conradi-Hunermann type), and X-linked dominant type. These types have different clinical manifestations respectively. We report cases... |
Malignant ectomesenchymoma is a rare tumor originating from remnants of migratory neural crest(ectomesenchyme) and composed of neuroectodermal as well as mesenchymal components. Neuroblasts and ganglion cells constitute the neuroectodermal components and rhabdomyosarcoma is the most frequently encountered mesenchymal components. We report a case of malignant ectomesenchymoma in a two-month-old boy who was presented with abdominal pain and urinary difficulty. The... |
Perforation after foreign body ingestion is rare. We report a 25-month-old girl who was diagnosed with a stomach perforation with secondary localized peritonitis caused by long, sharp bamboo. On admission, she had abdominal pain and fever. Simple abdominal erect & supine X-ray, abdominal ultrasonogram and computed tomography scan were normal. On the 6 th hospital day, gastrofiberoscopy was performed because... |
Purpose : The occurrence of shigellosis patients has increased radically in 2 recent years. For this, the authors investigated the epidemic aspect of shigellosis by regional groups to know whether the epidemic aspect of shigellosis is a general situation which occurs identically in various areas of Kyung Nam. Methods : We reviewed pediatric patients who were hospitalized in Ulsan(eastern), in Masan(central),... |
Purpose : Vitamin C among antioxidants in human is a physiological antioxidant for protection against diseases. We measured the vitamin C concentrations of whole blood and plasma among children in early childhood at normal condition in order to know the presence of vitamin C deficiency. Methods : The venous blood was collected from 471 subjects at 1-6 years of ages in... |
Purpose : Infantile transient methemoglobinemia(ITM) may develop in association with infectious diarrhea without exposure to any toxic oxidizing agents. We observed that the number of ITM associated with infectious diarrhea have increased at the Gyeongsang National University Hospital (GNUH), located in the western area of Kyungnam province during the last 4 years. To determine whether this phenomenon was similarily observed... |
We experienced two cases of congenital localized skin defect and neonatal onset of relapsing subepidermal blisters associated with minor trauma in a female newborn infant and her mother. The mother of index case showed toe nail dystrophy at delivery of her daughter, but the index case did not reveal nail dystrophy until 14 months of age. The congenital skin defects... |
We report a 10-year-old male with steroid-responsive nephrotic syndrome which was complicated by cerebral venous sinus thrombosis including superior sagittal sinus and left popliteal artery thrombosis following 5 months later. At each thrombotic episode, he suffered from relapse of massive proteinuria with hypovolemic crisis, and from hemoconcentration with dehydration due to vomiting or diarrhea. Brain MR angiography confirmed thromboses of... |
Purpose : Recommended dietary allowance of vitamin C was determined without considering the important function of vitamin C as a first-line antioxidant. We measured the whole blood and plasma vitamin C concentrations of healthy middle and high school students in Chinju to assess the optimal daily vitamin C requirement in these age groups. Methods : Whole blood and plasma vitamin C... |
Heat stroke occurs when body thermal regulation is upset and unable to dissipate adequate amounts of heat with rise in body temperature. Thus heat stroke may result in rhabdomyolysis and multiorgan failure. We observed two childhood cases of heat stroke with rhabdomyolysis and multiorgan failure. One case was caused by accidentally being left in a car trunk for 3 hours on a sunny afternoon... |