Elevated Circulating ICAM-1 and Eosinophil Cationic Protein in Children with Chronic Urticaria / 소아과

PURPOSE: Chronic urticaria is characterized by recurrent, itchy, and evanescent wheals over a period of six weeks. The mechanisms of chronic urticaria in children have not been fully elucidated. However, urticaria lesions represent a dynamic inflammatory process involving a complex interplay of various inflammatory mediators, chemokines, adhesion molecules and cellular interaction. The aim of this investigation was to elucidate the possible role of intercellular adhesion molecule-1 (ICAM-1) in the promotion of eosinophil trafficking and the interaction of soluble ICAM-1 (sICAM-1) with eosinophil cationic protein (ECP) in chronic urticaria. METHODS: Twenty-three children with chronic urticaria and five healthy children were recruited into the study. Total IgE, sICAM-1 and ECP concentrations were measured in all the serum samples from the study subjects, and both skin prick tests and dermographisms were performed after counting peripheral blood eosinophil in each subject. RESULTS: Mean concentration of serum sICAM-1 was higher in the urticaria patient group than in the control (P<0.05), however, serum ECP levels were variable. In the urticaria group, there were statistically significant correlations between the levels of serum ICAM-1 and ECP (r=0.24, P<0.001), and between the levels of serum ICAM-1 and the grade of dermographism (r=0.313, P<0.001). On the other hand, serum ICAM-1 level was not significantly related to the number of peripheral blood eosinophil. CONCLUSION: These data suggest that ICAM-1 and ECP might play a certain important role in the cellular interactions of inflammation involved in chronic urticaria, and the role should be verified by further studies.

Two Pediatric Patients with Neutropenia and Thrombocytopenia Associated with Parvovirus B19 Infection / 소아과

Parvovirus B19, a member of the Erythrovirus genus, is the only member of the Parvoviridae family known to be pathogenic in humans. Haematological consequences of B19 infection arise due to a direct cytotoxic effect on erythroid progenitors in bone marrow, resulting in interruption of erythrocyte production. The physiology of host haematopoiesis and competence of the immune response each determines clinical manifestations of B19 infection. A search of the literature revealed that neutropenia and thrombocytopenia due to B19 have been rarely reported in healthy individuals. In Korea, B19 infection has been reported among individuals with underlying disorders such as leukemia or congenital spherocytosis. Among otherwise healthy persons, there have been few reported cases of B19 infection which resulted in anemia, leukopenia, and thrombocytopenia. We herein report, for the first time in Korea, of two children who experienced leukopenia and thrombocytopenia, but not anemia, after B19 infection and recovered uneventfully.

A Case of Anaphylactic Response to Factor lX Replacement Therapy in Hemophilia B Patient / 대한혈액학회지

We report a first korean case of anaphylactic response to factor lX replacement therapy in 22-month-old male hemophilia B patient. He was admitted to our hospital via emergency room due to anaphylactic response after factor lX infusion. Immediately after the infusion, he became dyspneic and cyanotic. The symptoms completely resolved after sleep. He represented same symptoms after next factor lX infusion, which completely resolved by the administration of oxygen. At the time of anaphylaxis, inhibitors of factor lX was demonstrated(8.8 Bethesda Unit). We planned him to treat with recombinant factor Vll or diluted factor lX at bleeding episode.

Analysis of Treatment in Pediatric Germ Cell Tumor

PURPOSE: This report is an analysis of our experience with surgery, multi-agent chemotherapy and radiotherapy, in pediatric germ cell tumors(GCTs). METHODS: This report included all children(age <15 years) who were registered between November 1985 and May 2001 with a histologically confirmed diagnosis of GCTand received surgery, chemotherapy and radiotherapy. Retrospective analysis of the medical records of these patients was performed. In addition to the clinicopathological features, the response rate, survival rate, and toxicity were analyzed. RESULTS: Among were a total of 19 patients, 6 had an ovarian tumor and 3 had a testicular tumor. Histologically immature teratoma was the most common type(11/19; 57.9%). Tumor markers were increased in 13/17(76.5%). Complete responses were observed in 17/19(89.5%) of the evaluable patients. Five year actuarial survival was 79.4% and event free survival was 84.2%. Median follow up was 46 months. CONCLUSION: From the present report, it is apparent that chemotherapy is very effective and well tolerated in children with GCTs. The data probably suggests that conservative surgery, when combined with effective chemotherapy, can result in cure of the majority of children with GCTs.

A Case of Extraskeletal Ewing Sarcoma of Retroperitoneum / 대한소아혈액종양학회지

Ewing sarcoma is an uncommon bone malignancy of childhood. Although Ewing sarcoma is mostly a tumor of bone, it may also arise from soft tissues (extraskeletal Ewing sarcoma). We report a case of extraskeletal Ewing sarcoma that arose in the retroperitoneum of a 18-month-old girl and presented with right leg pain and gait disturbance. A brief review of related literatures was also made.

A Case of Van Wyk-Grumbach Syndrome / 대한소아내분비학회지

Van Wyk-Grumbach Syndrome is an advanced sexual development in association with primary hypothyroidism. The clinical feature in this syndrome is more consistent with stimulation of the FSH receptor by the markedly elevated TSH levels. Treatment of the hypothyroidism results in a rapid return to normal of the biochemical and clinical manifestations. We experienced a case of Van Wyk-Grumbach Syndrome and report with the brief review of related literature.

Two Cases of Central Nervous System Atypical Teratoid/Rhabdoid Tumors / 대한소아혈액종양학회지

Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) of infancy and childhood, a new entity among malignant pediatric brain tumors, is unique histologic entity with an extremely aggressive natural history. Histologically AT/RT is defined as a polymorphous neoplasm often featuring rhabdoid, primitive neuroectodermal tumor, epithelial, and mesenchymal components. AT/RT occurs mainly in the posterior fossa, so mimics medulloblastoma. AT/RT is characterized by the cytogenetic finding of monosomy 22 rather than i (17q). Standard chemotherapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective. Most children survive less than 12 months (mean survival: 8.5 months) after diagnosis. We report two cases of central nervous system AT/RT in young children, one of them is alive so far with multimodal treatment.

A Hemodynamic Study on the Influence of the Right Ventricular Volume: Overload Upon Left Ventricle Function Pre and Post Operative Left Ventricular Function in Atrial Septal Defect

This study was done to investigate the effect volume overloading of right ventricle(RV) on the left ventricular(LV) volume and function in patients with isolated secundum type atrial septal defect(ASD) and to determine the hemodynamic indices affecting the postoperative reduction of RV size. Pre and postoperative echocardiogram and equilibrium radionuclide cardiac angiogram were analyzed in 39 patients of isolated secundum type ASD, who had their diagnosis confirmed by right heart catheterization and were operated at Seoul National University Hospital from January 1982 to July 1984. The ratio fo RV end-diastolic dimension to LV end-diastolic dimension(RVED/LVED), ratio of LV pre-ejection period and LV ejection time(PEP/LVET), ejection fraction(E.F.), fractional shortening(F.S.) mean velocity of circumferential fiber shortening(mVcf), peak ejection rate(PER) and peak filling rate(PFR) were measured in 24 normal control subjects and 39 patients with ASD before and after operation. The results obtained were as follows : 1) The postop. reduction of RV size could be correlated with age at operation, but showed no correlation with the degree of QP/Qs, main pulmonary arterial systolic, diastolic, mean pressure, RVEDP and previous RV size. 2) The ratio of postop. RVED/LVED(0.58+/-0.15) was significantly decreased, compared with the ratio of preop(0.96+/-0.28)(P0.1). 4) There was no significant difference in LV systolic contractile functional indices between ASD group and normal control subjects and between pre and postop. status of ASD patients : LVEF was 63.5+/-6.1(%) in preop., 63.0+/-5.7(%) in postop and 62.4+/-6.6(%) in normal control subjects. F.S. was 27.3+/-5.7(%) in preop., 28.2+/-3.1(%) in postop. and 28.7+/-4.5(%) in normal control subjects. mVcf was 0.81+/-0.11(Cire/sec) in preop., 0.80+/-0.10(Circ/sec) in postop. and 0.82+/-0.14(Circ/sec) in normal control subjects. PER was 2.82+/-0.61(EDV/sec) in preop., 2.84+/-0.56(EDV/sec) in postop. and 2.84+/-0.45(EDV/sec) in normal control subjects. 5) The postop. PFR 3.34+/-0.46(EDV/sec) was significantly increased, compared with preop. PFR 2.51+/-0.46(EDV/sec)(P0.1). 6) The postop. RVEF(51.4+/-6.3%) was significantly decreased, compared with preop. RVEF(54.5+/-9.9%)(P<0.005). Pre and postop. RVEF of ASD patient were significantly lower than RVEF of normal control group(60.3+/-3.1%)(P<0.01, P<0.005). The results indicate that postop. reduction of RV size could be correlated with age at operation. The effect of RV volume overloading on LV could not change systolic contractile functional indices, but diastolic filling index. There is many evidences suggesting that RV filling influences LV diastolic function which are occured by means of left ward shift of the interventricular septum and indirectly by linkage of filling of the two ventricules by common enclosure in the pericardium(underfilled LV volume and decreased LV compliance). Mildly diminished overall LV performance as shown by systolic time intervals appears to be related to the volume overload of the RV and to the concomitantly diminished volume of LV rather than to any myocardial contractility. LV systolic time inverval was non-invasive and sensitive index in assessing overall LV performance independent to LV geometric configuration and abnormal motion of regional ventricular wall.