PURPOSE: The reported clinical manifestations in Turner syndrome were different by karyotypes, countries and races. So, We evaluated the physical findings & medical problems in patient with Turner syndrome according to karyotype. METHODS: We examined prospectively 53 cases that diagnosed as Turner syndrome by chromosome study at the Pediatric Endocrine Clinic, YUMC from May 1999 to Aug 1999. According to the karyotype, they were divided into 3 groups:45, X(22 cases, 41.5%), mosaicism(18 cases, 34%) and structural aberration group(13 cases, 24.5%), and 53 cases were reevaluated by prepared protocol of 22 abnormal physical findings and 4 medical problems. Chi-square test was used for statistic analysis. RESULTS: 1)In case of 45, X group, they had short stature(100%), cubitus valgus(95.5%), widely spaced nipples(86.4%), shield chest(72.7%), low posterior hair line(68.2%), in case of mosaicism group, they had short stature(100%), cubitus valgus(72.2%), high arched palate(66.7%), widely spaced nipples(61.1%), short neck(61.1%) and in case of structural aberration group, they had short stature(100%), high arched palate (92.3%), widely spaced nipples(92.3%), low posterior hair line(76.9%), shield chest (76.9%), in frequency order. Short stature was the most common finding in Turner syndrome. The incidence of high arched palate was significantly higher in structural aberration group(P<0.05). 2)In case of 45, X group, they had otitis media(63.6%), thyroid problem(4.5%) and no renal problem and cardiac problem, in case of mosaicism group, they had otitis media(38.9%), thyroid problem(5.6%) and no renal problem and cardiac porblem and in case of structural aberration group, they had otitis media(61.5%), thyroid problem(15.4%), renal problem(7.7%) and cardiac problem(7.7%) in frequency order, they had no statistical difference in among three groups. CONCLUSION: In case of 45, X group, the incidence of cubitus valgus, webbed neck were more common findings and of high arched palate, micrognathia were less common findings compared to other group. In case of mosaicism group, the incidence of micrognathia, epicanthal fold were more common findings and wide spaced nipples, antimongoloid fissure were less common findings compared to other group. In case of structural aberration group, the incidence of high arched palate is most common finding among 3 group(P<0.05) and pigmented nevi and epicanthal fold were less common findings compared to other group. The incidence of medical problems such as otitis media and thyroid problem were than other foreign country reports.
Continental Population Groups ; Hair ; Humans ; Incidence ; Karyotype* ; Mosaicism ; Neck ; Nevus, Pigmented ; Nipples ; Otitis ; Otitis Media ; Palate ; Prospective Studies ; Thorax ; Thyroid Gland ; Turner Syndrome*

Natural killer T (NKT) cell is a special type of T lymphocytes that has both receptor of natural killer (NK) cell (NK1.1, CD161c) and T cell (TCR) and express a conserved or invariant T cell receptor called Valpha14Jalpha18 in mice or Va24 in humans. Invariant NKT (iNKT) cell recognizes lipid antigen presented by CD1d molecules. Marine-sponge-derived glycolipid, alpha-galactosylceremide (alpha-GalCer), binds CD1d at the cell surface of antigen-presenting cells and is presented to iNKT cells. Within hours, iNKT cells become activated and start to secrete Interleukin-4 and interferon-gamma. NKT cell prevents autoimmune diseases, such as type 1 diabetes, experimental allergic encephalomyelitis, systemic lupus erythematous, inflammatory colitis, and Graves' thyroiditis, by activation with alpha-GalCer. In addition, NKT cell is associated with infectious diseases by mycobacteria, leshmania, and virus. Moreover NKT cell is associated with asthma, especially CD4+ iNKT cells. In this review, I will discuss the characteristics of NKT cell and the association with inflammatory diseases, especially asthma.
Animals ; Antigen-Presenting Cells ; Asthma ; Autoimmune Diseases ; Colitis ; Communicable Diseases ; Encephalomyelitis, Autoimmune, Experimental ; Galactosylceramides ; Humans ; Interferon-gamma ; Interleukin-4 ; Mice ; Natural Killer T-Cells ; Receptors, Antigen, T-Cell ; T-Lymphocytes ; Thyroid Gland ; Thyroiditis ; Viruses

No abstract available.
Adolescent* ; Ecthyma* ; Humans ; Pseudomonas aeruginosa

No abstract available.
Drug-Related Side Effects and Adverse Reactions

The current paper reports on a case of subdural empyema secondary to frontal sinusitis in an otherwise healthy child. Sinusitis is a common and benign condition in most pediatric cases. Because of the widespread use of antibiotics, intracranial extension of pediatric sinusitis is rarely seen today; however, complications (e.g., cavernous sinus thrombosis, orbital infection, meningitis, and subdural empyema) are potentially life threatening. A 15-year-old right-handed male presented with a 3-day history of fever, headache, and left-sided palsy. Computed tomography revealed right-sided subdural empyema with right frontal sinusitis and maxillary sinusitis. A postoperative inpatient neurological consultation was requested 2 months post-surgery due to motor function deficits. The results suggested that early and accurate diagnosis of subdural empyema leads to prompt treatment and a favorable outcome for the patient.
Adolescent ; Anti-Bacterial Agents ; Brain Abscess ; Cavernous Sinus Thrombosis ; Central Nervous System ; Child* ; Diagnosis ; Empyema, Subdural* ; Fever ; Frontal Sinus ; Frontal Sinusitis ; Headache ; Humans ; Inpatients ; Male ; Maxillary Sinus ; Maxillary Sinusitis ; Meningitis ; Orbit ; Paralysis ; Sinusitis*

PURPOSE: Interleukin 18(IL-18) is a potent proinflammatory cytokine which induces IFN-gamma, GM-CSF, TNF-alpha and IL-1, to activate killing by lymphocytes, and to up-regulate the expression of certain chemokine receptors. Kawasaki disease (KD) is an inflammatory disease which increases serum levels of inflammatory cytokines, such as TNF-alpha and IL-6. This study was performed to examine the serum levels of proinflammatory cytokine IL-18 in KD. METHODS: Twenty patients with KD and 10 healthy children were enrolled in this study. Serum levels of IL-18 and TNF-alpha from the patients with acute and subacute stage of KD and normal controls were measured by using ELISA. Acute phase reactants such as ESR and C-reactive protein were measured during the acute stage of the disease. RESULTS: There was a significant increase in serum levels of IL-18 measured at the acute stage of KD(818.0+/-253.4 pg/mL) compared with those of subacute stage (367.7+/-140.1 pg/mL) (P<0.01) and normal controls(348.6+/-122.9 pg/mL)(P<0.01). However, the increase of IL-18 was not correlated with the increase of TNF-alpha(R=-0.156, P=0.488) or other acute phase reactants. CONCLUSION: The results showed that IL-18 was increased during the acute stage of KD, but the increase of IL-18 was not directly correlated with TNF-alpha. This results suggest that Il-18 is not a useful marker to estimate the severity of inflammation in KD.
Acute-Phase Proteins ; C-Reactive Protein ; Child ; Cytokines ; Enzyme-Linked Immunosorbent Assay ; Granulocyte-Macrophage Colony-Stimulating Factor ; Homicide ; Humans ; Inflammation ; Interleukin-1 ; Interleukin-18* ; Interleukin-6 ; Interleukins ; Lymphocytes ; Mucocutaneous Lymph Node Syndrome* ; Receptors, Chemokine ; Tumor Necrosis Factor-alpha

PURPOSE: The aim of this study was to investigate the clinical characteristics of Respiratory syncytial virus (RSV) infection during the neonatal period to provide information that is useful in clinical practice and suggest extension of the palivizumab administration. METHODS: Neonates admitted to the National Health Insurance Service Ilsan Hospital neonatal intensive care unit due to respiratory symptoms and for whom multiplex reverse transcription-polymerase chain reaction and multiplex real time-polymerase chain reaction tests were performed between October 2011 and May 2016 were included in this study. Medical records were retrospectively reviewed, and data was collected for 156 neonates. RESULTS: Among the 156 neonates, RSV was detected in 114 (73.1%), non-RSV in 25 (16%), and no virus in 17 (10.9%). The majority were full term infants (92.4%) and peak incidence of RSV infection was in January. Post-natal care center infection was more common in the RSV group (46.6%) than that in the other virus groups (24%, P=0.0243). Clinical symptoms were severe in the RSV group in contrast to that in the non-RSV or others groups. The RSV group frequently needed oxygen therapy (P=0.0001) and the duration of hospital stays were longer (P=0.0001). CONCLUSION: RSV is a significant cause of respiratory infection in neonates and the severity is higher in contrast to that with other viral causes of infection. Infants in post-natal care centers have a high-risk of developing RSV infections; therefore, palivizumab administration may be considered in this group to prevent hospitalization and reduce the duration of hospital stay.
Hospitalization ; Humans ; Incidence ; Infant ; Infant, Newborn* ; Intensive Care, Neonatal ; Length of Stay ; Medical Records ; National Health Programs ; Oxygen ; Palivizumab ; Respiratory Syncytial Viruses* ; Retrospective Studies

PURPOSE: Idiopathic thrombocytopenic purpura (ITP) was the most common disease of thrombocytopenic purpura in children, chronic course was shown in 10%. Splenectomy had been used in chronic thrombocytopenic purpura as classic therapy, and many therapeutic method had been tried. We define response to therapy and long-term outcome of chronic childhood idiopathic thrombocytopenic purpura. METHODS: We retrospectively analyzed 41 patients that treated and diagnosed as chronic thrombocytopenic purpura at the Department of Pediatrics, Severance Hospital between Aug. 1981 and Aug. 1999 and followed for mean 8.2 years. The mean age was 6.2 years (range from 1month to 12.3 years) and sex-ratio of male to female was 1:2. According to therapeutic methods, they were divided into splenectomy group, spleen irradiation group, azathioprine group, interferon group, high-dose oral dexamethasone group, vincristine group, intravenous gammaglobulin group, low-dose prednisone group, and low-dose prednisone with intermittent intravenous gammaglobulin group. The results were classified into complete response (CR: platelets> 100,000/mm3), partial response (PR: platelets> 50,000/mm3), no response (NR: platelets <50,000/mm3). RESULTS: The treatment response and number of patients according to therapeutic methods were as follows; splenectomy group, 12 cases (CR-9 cases, PR-1 case, NR-2 cases), spleen irradiation group, 1 case (CR), azathioprine group, 6 cases (CR-1 case, NR-5 cases), interferon group, 3 cases (CR-1 case, PR-1 case, NR-1 case), high-dose oral dexamethasone group, 4 cases (CR-2 cases, PR-1 case, NR-1 case), vincristine group, 2 cases (NR), intravenous gammaglobulin group, 35 cases (CR-5 cases, PR-5 cases, NR-25 cases), low-dose prednisone group, 5 cases (NR), and low-dose prednisone with intermittent intravenous gammaglobulin group, 30 cases (CR-7 cases, PR-3 cases, NR-20 cases). CONCLUSION: The results of therapeutic method in splenectomy group were the most highest score (12 cases: CR-9 cases, PR-1 case, NR-2 cases), high-dose oral dexamethasone group, interferon group, and low-dose prednisone with intermittent intravenous gammaglobulin group in high incidence order. Spleen irradiation was supposed to trial method of treatment before splenectomy, because the risk was lower than splenectomy, although they had 1 case.
Azathioprine ; Child ; Dexamethasone ; Female ; Humans ; Incidence ; Interferons ; Male ; Pediatrics ; Prednisone ; Purpura, Thrombocytopenic ; Purpura, Thrombocytopenic, Idiopathic* ; Retrospective Studies ; Spleen ; Splenectomy ; Vincristine

Mastocytosis is rare disorder characterized by the overproliferation and accumulation of tissue mast cells and spontaneous regression before adolescence in childhood, almost. Clinical features are vary and depend on local accumulation of mast cells in different organs and the effects of their mediators. Mast cell disease is commonly involved in the skin, and occasionally gastrointestinal tract, bone marrow, liver, spleen and lymphoid organs as well. Mastocytosis occurs equally in persons of each gender and it affects all age groups. The incidence is unknown and familial tendency is unusual. We are reporting a case of mastocytosis confirmed by skin biopsy who was visited for skin lesions that skin rashes at 10 days after birth, change to bullar at 3 month later.
Adolescent ; Biopsy ; Bone Marrow ; Exanthema ; Female* ; Gastrointestinal Tract ; Humans ; Incidence ; Infant* ; Liver ; Mast Cells ; Mastocytosis* ; Parturition ; Skin ; Spleen

Kawasaki disease, an acute febrile vasculitis of unknown etiology, is usually treated with high doses of immunoglobulin (IVIG) and aspirin. However, 20% of children show persistent or recurrent fever despite IVIG, and coronary artery aneurysm progression. In such cases of resistance to IVIG treatment, repeated IVIG administration or the initiation of steroid therapy, and the use of cyclophosphamide have been reported. We aimed to show in this study that methotrexate (MTX) may be used as a treatment for Kawasaki disease resistant to IVIG treatment. We report the case of a 6-year old boy who was admitted at another hospital with an initial complaint of a fever for 5 days and skin rashes for 3 days. The patients fever persisted despite three courses of IVIG (2 gm/kg, 1 gm/kg, 1 gm/kg, respectively) over a 14-day period. On day 14 of his illness he showed a dilated right coronary artery, and on day 19 dexamethasone, at a daily dose of 0.3 mg/kg, was given but this resulted in defervescence. However, upon stopping the dexamethasone treatment, his fever recurred and he was transferred to our hospital. On days 31 and 38 of his illness, IVIG (400 mg/kg for 5 days, twice) was administered and from day 38 onwards the patient was given dexamethasone (0.6 mg/kg, daily) and MTX (10 mg/BSA, once weekly) whereupon his fever subsided and did not recur. On day 48 dexamethasone was replaced with prednisolone, which was subsequently tapered. The patient is now taking MTX and being observed on an outpatient basis. We report the case of a boy with IV-globulin resistant Kawasaki disease, who after repeated infusions of IVIG and steroid therapy showed fever recruuence, which that subsided after MTX treatment.
Case Report ; Child ; Human ; Immunoglobulins, Intravenous/*therapeutic use ; Male ; Methotrexate/*therapeutic use ; Mucocutaneous Lymph Node Syndrome/complications/*drug therapy