Primary tumors of the heart are uncommon among pediatric patients. Rhabdomyoma is the most frequent cardiac tumor in infants and children, which is commonly associated with tuberous sclerosis. Tuberous sclerosis is a neurocutaneous syndrome affecting the brain, heart, skin, and other organs. Cardiac rhadomyomas are reported in 50-64% of infants with tuberous sclerosis. Tuberous sclerosis involves multiple locations in the atrium, ventricle and septum, and may induce mechanical obstruction of the outflow tract and heart failure depending on the location, number, size, and degree of invasion of tumors. Arrhythmias may also develop in infants with cardiac rhadomyomas, but only a few of these patients require prolonged anti-arrhythmic therapy because arrhythmia often disappears with spontaneous regression of the tumors, and the ultimate prognosis may be decided by the cerebral manifestations.
Arrhythmias, Cardiac ; Brain ; Child ; Heart ; Heart Failure ; Heart Neoplasms ; Humans ; Infant ; Infant, Newborn ; Neurocutaneous Syndromes ; Prognosis ; Rhabdomyoma ; Skin ; Tachycardia, Supraventricular ; Tuberous Sclerosis

PURPOSE: Intravenous immunoglobulin (IVIG) is effective for the treatment of idiopathic thrombocytopenic purpura (ITP) in children. Recently, several reports have been published that show its impact on the absolute neutrophil count. The present study was performed to confirm these findings. METHODS: Data on 26 ITP patients were analyzed. Patients with febrile illness or increased C-reactive protein levels at presentation, which would influence the neutrophil counts, were excluded to determine the sole impact of IVIG. In addition, patients who received steroid treatment were also excluded. RESULTS: Sixteen boys and ten girls were analyzed. For patients who received an IVIG dose of 0.4 g/kg/day (n=17), the absolute neutrophil count (ANC) measured next day was significantly decreased. For patients who received an IVIG dose of 1 g/kg/day (n=9), the ANC measured the next day was also significantly decreased. However, the decrease was more profound in the high-dose group compared to the low-dose group. Among six cases with profoundly decreased ANC greater than 1,000/ mm3, four patients (67%) received IVIG at a dose of 1 g/kg/day. All four cases with increased ANC were treated with IVIG dose of 0.4 g/kg/day, and three cases (75%) among them had a febrile reaction during IVIG administration. None of the cases with decreased ANC had a febrile reaction. No cases had infectious complications reported. CONCLUSION: IVIG treatment for ITP patients appears to suppress the ANC. This decrease of ANC was more pronounced when a higher dose of IVIG was used. Some cases with increased ANC counts after IVIG use were found only in low-dose IVIG group, and was associated with febrile reactions during IVIG use.
C-Reactive Protein ; Child* ; Female ; Humans ; Immunoglobulins* ; Immunoglobulins, Intravenous ; Neutrophils* ; Purpura, Thrombocytopenic, Idiopathic*

No abstract available.
Transplantation* ; Tuberculosis*

No abstract available.
Transplantation* ; Tuberculosis*

Russell-Silver syndrome is characterized by low birth weight, growth retardation, delayed bone age, asymmetry, abnormal sexual development, cranio-facial disproportion, short little finger and clinodactyly. Short stature is the major concern, so there have been interests treating Russell-Silver syndrome with human growth hormone. However, there are no large-scale controlled studies to confirm the effectiveness of growth hormone therapy until now because of the rarity of this disorder. In Korea, we cannot find reports dealing with growth hormone treatment in Russell-Silver syndrome. We present three children with Russell-Silver syndrome who showed accelerated growth with growth hormone treatment.
Child ; Fingers ; Growth Hormone* ; Human Growth Hormone ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Korea ; Sexual Development ; Silver-Russell Syndrome*

PURPOSE: Although growth hormone treatment is now widely used to treat some group of children with short stature, it is still expensive and many parents are seeking alternative therapies. We report the current status of alternative therapies for children visiting the 'growth clinic' in a single tertiary center. METHODS: Questionnaire about the usage of alternative therapy was used. Parents were asked whether they used alternative therapies and where they achieved the information. Also, they were asked whether the treatment was effective, and whether they would continue to use the alternative therapy. RESULTS: Two hundred and twenty nine children (127 boys, 102 girls) visiting the growth clinic in Dongsan Medical Center from February 2005 to January 2006 were analyzed. Their ages were between 6.1 years to 15.4 years (mean 10.4 years). Height Standard Deviation Score (SDS) were between -3.2 to 1.0 (mean -1.1). Twelve boys (9.4%) and 7 girls (6.9%) were more than zero in height SDS value. Among 145 children (63.3%) who used the alternative therapy, supplemental foods and oriental herbs were most widely used (43.3% each) followed by over-the-counter drugs. They attained the information most frequently from their neighbors followed by television, newspaper, and internet. More than half (57.9%) of the parents answered that the alternative therapies were not effective. However, 46.9% of the parents answered they would continuously use the therapy though it is ineffective. CONCLUSION: Many patients visiting the 'growth clinic' for short stature had experiences on the alternative therapies. Although these therapies seemed not quite effective, many parents answered that they would continue alternative therapy.
Child ; Complementary Therapies ; Growth Hormone ; Humans ; Internet ; Periodicals ; Nonprescription Drugs ; Parents ; Surveys and Questionnaires ; Television

BACKGROUND: Melatonin has been shown to attenuate the reflex sympathetic increases that arise in response to orthostatic challenges. We tested the hypothesis that the attenuated sympathetic increase induced by melatonin premedication may weaken the arterial blood pressure (ABP) preserving the capability during acute hypotension, thereby altering dynamic cerebral autoregulation and causing a further decrease in cerebral blood flow (CBF). METHODS: Acute hypotension was induced in 12 healthy subjects by releasing bilateral thigh cuffs before and after an oral dose of melatonin (0.2 mg/kg). Heart rate (HR), arterial blood pressure (ABP), Modelflow estimate of cardiac output (CO), total peripheral resistance (TPR) and cerebral blood flow velocity (CBFV) by transcranial Doppler were measured. RESULTS: Steady state HR, the mean arterial pressure and CBFV were not altered 60 minutes after melatonin ingestion. Reduced systolic arterial pressure (DeltaSAP), changes in HR (DeltaHR), CO (DeltaCO), and TPR (DeltaTPR), DeltaHR/DeltaSAP and percentage restoration of SAP were not affected after a temporal decrease in ABP induced by thigh cuff release. In the cerebral circulation, melatonin did not affect changes in CBFV, cerebrovascular resistance index, the rate of regulation and percentage restoration of CBFV following a sudden decrease in ABP. CONCLUSIONS: Contrary to our hypothesis, melatonin did not affect the rapid vasodilatory and recovery responses of cardiovascular and dynamic cerebral autoregulation. These results suggest that melatonin premedication may not impair ABP and CBF preserving capability induced by sudden postural changes or hemorrhage.
Arterial Pressure ; Blood Flow Velocity ; Blood Pressure ; Cardiac Output ; Cerebrovascular Circulation ; Eating ; Heart Rate ; Hemorrhage ; Homeostasis ; Humans ; Hypotension ; Male ; Melatonin ; Premedication ; Reflex ; Thigh ; Vascular Resistance

BACKGROUND: Thalidomide has been reported to have antitumor activity for treating metastatic hepatocellular carcinoma (HCC). We evaluated the safety and efficacy of using thalidomide for treating selected patients with unresectable or metastatic HCC, and their disease was refractory to systemic chemotherapy. METHODS: Eight patients with measurable and metastatic HCC that had progressed with prior systemic chemotherapy and who desired further active therapy were enrolled in this study. Thalidomide was given orally at bedtime and it was started at 200 mg/day with no further dose escalation. The response was measured at 2-month intervals. RESULTS: The median age was 44 years (range: 34-52 years) and all the patients had received doxorubicin-based systemic chemotherapy prior to their enrollment. Each patient received thalidomide for a median of 152 days (range: 5-422 days). One partial response was observed (12.5%, 95% CI; 0-42%) along with 4 cases of stable diseases. The most commonly encountered toxicity was somnolence; grade 3 somnolence was noted for one patient, which led to treatment discontinuation. Skin rash was observed in one responding patient. CONCLUSIONS: The results indicate that thalidomide may feasibly offer disease stabilization to metastatic HCC patients. Further dose escalation of thalidomide, or its combination with other chemotherapeutic agents, may be of interest and this should be investigated for treating patients with metastatic HCC.
Treatment Outcome ; Thalidomide/*therapeutic use ; Retrospective Studies ; Pilot Projects ; Middle Aged ; Male ; Lymphatic Metastasis ; Lung Neoplasms/drug therapy/*secondary ; Liver Neoplasms/*drug therapy/pathology ; Immunosuppressive Agents/*therapeutic use ; Humans ; Follow-Up Studies ; Female ; Carcinoma, Hepatocellular/*drug therapy/secondary ; Bone Neoplasms/drug therapy/*secondary ; Adult

BACKGROUND: A poincare plot of the heart rate variability (HRV) allows for the quantitative display of the vagal tone in conscious humans. However, relatively little is known about standard deviation 1 (SD1) from the poincare plot reflecting the vagal tone and correlating with the high frequency (HF) spectral power of the HRV during general anesthesia. Thus, the association of SD1 from the poincare plot was examined, along with the HF spectral power of the HRV during general anesthesia. METHODS: Beat-to-beat electrocardiograms were recorded for 5 min in 23 patients (isoflurane group, n = 13; sevoflurane group, n = 10) before, during and after general anesthesia. The low frequency (LF) and HF spectral powers, the LF/HF ratio of the HRV and the SD1 and standard deviation 2 (SD2) from the poincare plot were calculated. RESULTS: Both the HF spectral power of the HRV and SD1 from the poincare plot were reduced following general anesthesia, but recovered thereafter. The recovery of both the HF spectral power and SD1 from the poincare plot in the sevoflurane group was faster than those in the isoflurane group. There were strong correlations between the HF spectral power and SD1 before, during and after anesthesia. CONCLUSIONS: These data suggest that the SD1 from the poincare plot is a useful and valid parameter for analysis of the vagal tone during general anesthesia.
Anesthesia ; Anesthesia, General* ; Electrocardiography ; Heart Rate* ; Heart* ; Humans ; Isoflurane