Aggressive Systemic Mastocytosis Following Ovarian Germ Cell Tumor / 대한혈액학회지

A 4 years-old girl was diagnosed with aggressive systemic mastocytosis at 2 months after the end of chemotherapy including cisplatin, bleomycin and etoposide for an ovarian germ cell tumor (GCT). She was shown pigmented skin lesion, hepatosplenomegaly, thrombocytopenia and increased mast cells with positive toluidine blue staining on the bone marrow examination. Immunohistochemical staining for c-kit was highly expressed in the bone marrow and skin, but not in the GCT specimen. However, c-kit point mutation was detected in the bone marrow, peripheral blood and GCT tissue when performing PCR via oligonucleotide sequencing. We report here on one case of aggressive systemic mastocytosis following ovarian germ cell tumor with c-kit point mutation.

Two Cases of Cerebral Salt Wasting Syndrome Developing after Cranial Vault Remodeling in Craniosynostosis Children

Hyponatremia has been recognized as an important postoperative metabolic complication after central nervous system (CNS) operations in children. If not appropriately treated, the postoperative hyponatremia can cause several types of CNS and circulatory disorders such as cerebral edema, increased intracranial pressure. The postoperative hyponatremia after CNS surgery has been considered as one of the underlying causes of the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). In some cases, however, the cerebral salt wasting (CSW) syndrome has been detected. CSW syndrome is far less well-known than SIADH and also different from SIADH in diagnosis and treatment. It causes an increase in urine output and urine sodium after a trauma of CNS and dehydration symptoms. The appropriate treatment of CSW syndrome is opposite the usual treatment of hyponatremia caused by SIADH. The latter is treated with fluid restriction because of the increased level of free water and its dilutional effect causing hyponatremia, whereas the former is treated with fluid and sodium resuscitation because of the unusual loss of high urinary sodium. Early diagnosis and treatment of CSW syndrome after CNS surgery are, therefore, essential. We made a diagnosis of CSW syndrome in two craniosynostosis children manifesting postoperative hyponatremia and supplied them an appropriate amount of water and sodium via intravenous route. The hyponatremia or natricuresis of the children improved and neurologic and circulatory sequelae could be prevented.

Increased Reactive Oxygen Species Production by Peripheral Blood Mononuclear Leukocytes, not by Polymorphonuclear Leukocytes, in Atopic Dermatitis / 소아알레르기및호흡기학회지

PURPOSE: Reactive oxygen species (ROS) are known as a potential mediators that sustain chronic inflammation in atopic dermatitis (AD). To determine the role of peripheral blood mononuclear leukocytes (MO) and polymorphonuclear leukocytes (PMN) in prolonged inflammation, ROS generation of those cells in AD was examined. METHODS: Seventeen AD patients and 10 healthy controls were enrolled. MO and PMN were stimulated with the reagents: phobol ester (PMA), adenosine triphosphate (ATP), and chemotactic peptide (f-MLP). ROS levels were measured using chemiluminescence assay. RESULTS: In AD, chemiluminescence response of unstimulated MO was higher than that of normal controls. MO from AD patients produced 1.58-1.80 higher ROS for up to 30 minutes than the controls. When the cells were treated with the reagents (PMA, ATP, f-MLP), all the stimuli enhanced chemiluminescence activity of MO. When MO were treated with PMA, the ratio of ROS produced by MO of patients to that of the controls decreased. When the cells were treated with either ATP or f-MLP, the quantity of ROS generated by MO from the controls was greater than the controls. PMN from both AD patients and the controls generated ROS for 30 min similarly. As treated with the reagents, PMN from AD patients produced a smaller ROS than the controls. CONCLUSION: These results indicate MO but not PMN from AD patients were primed and ready for activation in vivo, and a reduced function of PMN from AD patients was observed. In conclusion, enhanced respiratory burst activity of MO is implicated in the prolonged inflammation of AD.