Cerebral Salt Wasting Treated with Fludrocortisone in a 17-Year-Old Boy

Cerebral salt wasting is characterized by inappropriate natriuresis and volume contraction with associated cerebral pathology. It is distinct from the syndrome of inappropriate antidiuretic hormone secretion, which is characterized by inappropriate retention of free water. We report a patient with a porencephalic cyst who developed cerebral salt wasting. His initial treatment was supplementation of water and salt, which did not improve natriuresis or volume contraction. Fludrocortisone administration effectively managed the cerebral salt wasting.

Prevalence of Vesicoureteral Reflux According to the Timing of Voiding Cystourethrography in Infantile Urinary Tract Infection

PURPOSE: To evaluate the prevalence of vesicoureteral reflux (VUR) according to the timing of voiding cystourethrography (VCUG) in infantile urinary tract infection (UTI). METHODS: The data of 134 infants (1-12 months) with renal cortical defect in 99mTc-2, 3-dimercaptosuccinic acid (99mTc-DMSA) scan with a diagnosis of UTI in two hospitals from 2000 to 2010 were retrospectively analyzed. The VCUG was performed after 2 weeks from the diagnosis of UTI in Group I (n=68), and the VCUG was performed within 2 weeks from the diagnosis of UTI in Group II (n=66). RESULTS: There were no significant differences between the two groups in the duration of fever, white blood cell count, C-reactive protein levels, and abnormalities in ultrasonography (P>0.05). There was no significant difference between the two groups in the prevelence of VUR, bilateral VUR, and severe VUR. VCUG-induced UTI was detected 16 (23.5%) of patients in whom the procedure was performed 2 weeks after the diagnosis, and none of VCUG-induced UTI occurred in those in whom the procedure was performed 2 weeks within the diagnosis. CONCLUSION: We conclude that the prevalence of VUR according to the timing of VCUG did not differ between the two groups in infantile UTI with renal cortical defect in DMSA scan. We also found that performing VCUG with antibiotics can decrease risk of VCUG-induced UTI.

Survival Benefit of Hematopoietic Stem Cell Transplantation in Infant Acute Lymphoblastic Leukemia, According to Classification of High Risk Group / 임상소아혈액종양

BACKGROUND: In comparison to older children, acute leukemia in infants has a dismal outcome, despite introduction of intensive multi-agent chemotherapy. Patients with age under 1 year and mixed-lineage leukemia (MLL) gene rearrangements are the most high risk group. In this study, we investigate the outcome of more intensive chemotherapy and the role of hematopoietic stem cell transplantation (HSCT) in high risk group of infant leukemia.METHODS: This study analyzed on 9 infants with acute lymphoblastic leukemia (ALL) who were diagnosed and treated between 1998 and 2008 at Severance hospital. We classified high risk group with age at diagnosis is under 6 months or white blood cell at diagnosis are over 50,000/microL or presence of MLL gene rearrangements. Patients in high risk group were treated on a protocol of intensive chemotherapy followed by HSCT.RESULTS: In this study, the 3-years EFS rate for 9 infant ALL patients was 66.7+/-15.7%. Both of 2 patients younger than 6 months of age at diagnosis did not survive. 3yr-EFS rate for the 7 patients categorized in high risk group out of 9 infant ALL patients was 57.1+/-18.7%, and 3 yr EFS of the 6 patients in the high risk group who received HSCT was 60+/-21.9%. We did not experience relapse in 6 patients treated with HSCT.CONCLUSION: This study showed promising results and lesser risks in high risk group infant ALL treated with intensive chemotherapy and HSCT. We suggest early HSCT with intensive chemotherapy for infant leukemia patients in high risk group.

Survival Benefit of Hematopoietic Stem Cell Transplantation in Infant Acute Lymphoblastic Leukemia, According to Classification of High Risk Group / 임상소아혈액종양

BACKGROUND: In comparison to older children, acute leukemia in infants has a dismal outcome, despite introduction of intensive multi-agent chemotherapy. Patients with age under 1 year and mixed-lineage leukemia (MLL) gene rearrangements are the most high risk group. In this study, we investigate the outcome of more intensive chemotherapy and the role of hematopoietic stem cell transplantation (HSCT) in high risk group of infant leukemia. METHODS: This study analyzed on 9 infants with acute lymphoblastic leukemia (ALL) who were diagnosed and treated between 1998 and 2008 at Severance hospital. We classified high risk group with age at diagnosis is under 6 months or white blood cell at diagnosis are over 50,000/microL or presence of MLL gene rearrangements. Patients in high risk group were treated on a protocol of intensive chemotherapy followed by HSCT. RESULTS: In this study, the 3-years EFS rate for 9 infant ALL patients was 66.7+/-15.7%. Both of 2 patients younger than 6 months of age at diagnosis did not survive. 3yr-EFS rate for the 7 patients categorized in high risk group out of 9 infant ALL patients was 57.1+/-18.7%, and 3 yr EFS of the 6 patients in the high risk group who received HSCT was 60+/-21.9%. We did not experience relapse in 6 patients treated with HSCT. CONCLUSION: This study showed promising results and lesser risks in high risk group infant ALL treated with intensive chemotherapy and HSCT. We suggest early HSCT with intensive chemotherapy for infant leukemia patients in high risk group.