PURPOSE: The use of granulocyte transfusion (GT) which had been diminished since 1980s has been recently interested on the base of clinical efficacy approved by transfusion of granulocyte collected after administration of granulocyte-colony stimulating factor (G-CSF) to donors. So we studied the clinical efficacy of GT in severe neutropenic pediatric patients with severe infections. METHODS: Twelve patients with malignant hematologic disorders and solid tumors in Ajou University Hospital from March 1997 to February 2001 were indicated for GT. These patients had continuous neutropenia-related infections despite appropriate antibiotics, antifungal or IV immunoglobulin therapy. GTs were carried out 12 hrs after collection of granulocytes using leukapheresis from donors stimulated by G-CSF. RESULTS: The median number of GT is 3 (2~7), and the mean dose of granulocyte is 3.51+/-4.21 10(10)/m(2) and mean volume of granulocyte is 220 +/-22.92 mL. The median duration of the use of G-CSF and antibiotics or antifungal agents is 6 (3~14) days and 3 (1~9) days. Ten of 12 patients had favorable responses (FR), and 2 patients had unfavorable responses (UR). Two patients who had FR died of acute respiratory distress syndrome (ARDS), complication of GT. CONCLUSION: GT is effective treatment for severe neutropenic pediatric patients with severe infections. Enough amount of granulocytes could be collected after administration of G-CSF without dexamethasone to donors. However, ARDS which is an adverse effect of GT, is considered in pulmonary compromised patients.
Anti-Bacterial Agents ; Antifungal Agents ; Dexamethasone ; Granulocyte Colony-Stimulating Factor ; Granulocytes* ; Humans ; Immunization, Passive ; Leukapheresis ; Neutropenia ; Respiratory Distress Syndrome, Adult ; Tissue Donors

A survey was conducted to determine the extent of intestinal parasite infection in Bat Dambang, Cambodia in March 2004. A total of 623 fecal specimens was collected from kindergarten and schoolchildren and examined using the formalin-ether sedimentation technique. The overall infection rate of intestinal parasites was 25.7% (boys, 26.2%; girls, 25.1%), and the infection rates of intestinal helminthes by species were as follows: Echinostoma sp. 4.8%, hookworm 3.4%, Hymenolepis nana 1.3%, and Rhabditis sp. 1.3%. The infection rates of intestinal protozoa were; Entamoeba coli 4.8%, Giardia lamblia 2.9%, Iodamoeba butschlii 1.4%, Entamoeba polecki 1.1%, and Entamoeba histolytica 0.8%. There were no egg positive cases of Ascaris lumbricoides or Trichuris trichiura. All children infected were treated with albendazole, praziquantel, or metronidazole according to parasite species. The results showed that intestinal parasites are highly endemic in Bat Dambang, Cambodia.
Adolescent ; Age Factors ; Animals ; Cambodia/epidemiology ; Child ; Feces/parasitology ; Female ; Helminthiasis/*epidemiology ; Helminths/isolation & purification ; Humans ; Intestinal Diseases, Parasitic/*epidemiology ; Male ; Protozoa/isolation & purification ; Protozoan Infections/*epidemiology

We report a case of isolated primary medullary hemorrhage with a review of the literature to analyze the clinical presentation and radiological character. A 46-year-old man with chronic alcoholism developed tegmental medullary syndrome secondary to spontaneous hemorrhages in the medulla oblongata. Magnetic resonance imaging study was carried out on the third day after the second event, which showed two distinct isolated hematomas in the medulla oblongata. Digital subtraction angiography was normal. The cause of hemorrhage was presumed to be cryptic vascular malformation. The primary medullary hemorrhage usually presents with a characteristic syndrome of sudden onset vertigo with neurological signs that correspond to various combination of medial and lateral medullary involvement. Usually, the prognosis is good only with conservative treatment.
Alcoholism ; Angiography, Digital Subtraction ; Hematoma ; Hemorrhage* ; Humans ; Magnetic Resonance Imaging ; Medulla Oblongata ; Middle Aged ; Prognosis ; Vascular Malformations ; Vertigo

The Dieulafoy lesion is a rare cause of severe gastrointestinal hemorrhage. Although it may occur anywhere in the gastrointestinal tract, the lesion is most commonly located in the stomach, and the small bowel is an extremely uncommon site. Since Dieulafoy lesion in the small bowel is difficult to access by endoscopy, it seems impossible to diagnose and treat by initial endoscopy unlike the lesions in stomach. We experienced a case of Dieulafoy lesion of jejunum with massive hemorrhage in 54-year-old male. Active jejunal bleeding was shown by computed tomography scan and mesenteric angiography. Partial resection of the jejunum was performed. Final pathologic finding revealed Dieulafoy lesion of the jejunum.
Angiography ; Gastrointestinal Hemorrhage/complications/*diagnosis ; Humans ; Jejunal Diseases/complications/*diagnosis/surgery ; Male ; Mesenteric Arteries/radiography ; Middle Aged ; Tomography, X-Ray Computed

BACKGROUND: To reduce massive blood loss during a hepatectomy, many anesthesiologists have used the technique of low central venous pressure maintenance by administration of low dose nitroglycerin (NTG) and/or intravenous fluid reduction. However, so far there have been no studies about local liver perfusion (LLP) changes after hepatic artery (HA) or portal vein (PV) reperfusion in patients receiving nitroglycerin administration. In this study, the changes in hemodynamics and LLP following HA and PV reperfusion along with low dose (2micro gram/kg/min) NTG administration in dogs were observed. METHODS: A total of 20 mongrel dogs were divided into four groups; HA occlusion and reperfusion group (H, n = 5), NTG administration group during the reperfusion on H (H-NTG, n = 5), PV occlusion and reperfusion group (P, n = 5), NTG administration group during the reperfusion on P (P-NTG, n = 5). After femoral and pulmonary arterial catheterization, a midline abdominal incision was made. HA and PV were exposed to clamp and declamp. A thermal diffusion microprobe was inserted in the liver parenchyme to measure LLP. RESULTS: The PV blood flow was not changed after HA occlusion, but HA blood flow increased after PV occlusion. The LLP decreased after HA and PV occlusion. The LLP recovered to the baseline level in group H-NTG after HA reperfusion, but the LLP was more increased compared to the baseline level in group H. In group P, the LLP did not recover after PV reperfusion, but the LLP in group P-NTG recovered to the baseline level after PV reperfusion. CONCLUSIONS: In conclusion, it was observed that the LLP recovered to the baseline level by administration of NTG after PV reperfusion. However, the LLP did not increase after HA reperfusion by administration of low dose NTG.
Animals ; Catheterization ; Catheters ; Central Venous Pressure ; Dogs* ; Hemodynamics* ; Hepatectomy ; Hepatic Artery* ; Humans ; Liver* ; Nitroglycerin* ; Perfusion* ; Portal Vein* ; Reperfusion* ; Thermal Diffusion

Citrullinemia is a rare inborn error of metabolism of the urea cycle, and was first reported by McMurray, et al. in 1962. It is inherited as an autosomal recessive trait. The normal synthesis of argininosuccinic acid is blocked in this disease due to a deficiency of argininosuccinic acid synthetase(AS), which has been demonstrated in liver cells and fibroblasts. The clinical symptoms are vomiting, lethargy or irritability, convulsion and mental retardation. The diagnosis is made by the finding of an increased plasma citrulline level. Every effort should be made to reduce the blood ammonia level as rapidly as possible before irreversible brain damage occurs. This report describes a case of citrullinemia that was diagnosed through organic acid analysis and amino acid analysis, and reviews the related literatures.
Ammonia ; Argininosuccinic Acid ; Brain ; Citrulline ; Citrullinemia* ; Diagnosis ; Fibroblasts ; Intellectual Disability ; Lethargy ; Liver ; Metabolism ; Plasma ; Seizures ; Urea ; Vomiting

Autism spectrum disorder (ASD) remains unexplained and untreated despite the high attention of research in recent years. Aside from its various characteristics is the baffling male preponderance over the female population. Using a validated animal model of ASD which is the telomerase reverse transcriptase overexpressing mice (TERT-tg), we conducted ASD-related behavioral assessments and protein expression experiments to mark the difference between male and females of this animal model. After statistically analyzing the results, we found significant effects of TERT overexpression in sociability, social novelty preference, anxiety, nest building, and electroseizure threshold in the males but not their female littermates. Along these differences are the male-specific increased expressions of postsynaptic proteins which are the NMDA and AMPA receptors in the prefrontal cortex. The vGluT1 presynaptic proteins, but not GAD, were upregulated in both sexes of TERT-tg mice, although it is more significantly pronounced in the male group. Here, we confirmed that the behavioral effect of TERT overexpression in mice was male-specific, suggesting that the aberration of this gene and its downstream pathways preferentially affect the functional development of the male brain, consistent with the male preponderance in ASD.
Animals ; Anxiety ; Autism Spectrum Disorder ; Brain ; Female ; Humans ; Male ; Mice ; Mice, Transgenic* ; Models, Animal ; N-Methylaspartate ; Phenotype* ; Prefrontal Cortex* ; Receptors, AMPA ; Sex Characteristics* ; Synapses ; Telomerase

Autism spectrum disorder (ASD) remains unexplained and untreated despite the high attention of research in recent years. Aside from its various characteristics is the baffling male preponderance over the female population. Using a validated animal model of ASD which is the telomerase reverse transcriptase overexpressing mice (TERT-tg), we conducted ASD-related behavioral assessments and protein expression experiments to mark the difference between male and females of this animal model. After statistically analyzing the results, we found significant effects of TERT overexpression in sociability, social novelty preference, anxiety, nest building, and electroseizure threshold in the males but not their female littermates. Along these differences are the male-specific increased expressions of postsynaptic proteins which are the NMDA and AMPA receptors in the prefrontal cortex. The vGluT1 presynaptic proteins, but not GAD, were upregulated in both sexes of TERT-tg mice, although it is more significantly pronounced in the male group. Here, we confirmed that the behavioral effect of TERT overexpression in mice was male-specific, suggesting that the aberration of this gene and its downstream pathways preferentially affect the functional development of the male brain, consistent with the male preponderance in ASD.
Animals ; Anxiety ; Autism Spectrum Disorder ; Brain ; Female ; Humans ; Male ; Mice ; Mice, Transgenic* ; Models, Animal ; N-Methylaspartate ; Phenotype* ; Prefrontal Cortex* ; Receptors, AMPA ; Sex Characteristics* ; Synapses ; Telomerase

Persistent pulmonary hypertension in newborns (PPHN) is a disorder of the vascular transition from fetal to neonatal circulation. It results in cyanosis due to right-to-left shunting of the blood through the ductus arteriosus and/or foramen ovale manifesting as hypoxemic respiratory failure. We managed two cases of PPHN after meconium aspiration with high frequency oscillating ventilators and inhaled nitric oxide. They did not respond to conventional management. Veno-venous extracorporeal membrane oxygenation (ECMO) was provided, and ECMO weaning was possible resulting survivals in two cases. We report two PPHN cases, which were treated successfully with veno-venous ECMO for the first time in Korea.
Cyanosis ; Ductus Arteriosus ; Extracorporeal Membrane Oxygenation ; Foramen Ovale ; Humans ; Hypertension, Pulmonary ; Infant, Newborn ; Korea ; Meconium Aspiration Syndrome ; Nitric Oxide ; Respiratory Insufficiency ; Ventilators, Mechanical ; Weaning

Although the role of alpha-synuclein aggregation on Parkinson's disease is relatively well known, the physiological role and the regulatory mechanism governing the expression of alpha-synuclein are unclear yet. We recently reported that alpha-synuclein is expressed and secreted from cultured astrocytes. In this study, we investigated the effect of valproic acid (VPA), which has been suggested to provide neuroprotection by increasing alpha-synuclein in neuron, on alpha-synuclein expression in rat primary astrocytes. VPA concentration-dependently increased the protein expression level of alpha-synuclein in cultured rat primary astrocytes with concomitant increase in mRNA expression level. Likewise, the level of secreted alpha-synuclein was also increased by VPA. VPA increased the phosphorylation of Erk1/2 and JNK and pretreatment of a JNK inhibitor SP600125 prevented the VPA-induced increase in alpha-synuclein. Whether the increased alpha-synuclein in astrocytes is involved in the reported neuroprotective effects of VPA awaits further investigation.
Acetylation ; alpha-Synuclein* ; Animals ; Astrocytes* ; MAP Kinase Signaling System* ; Neurons ; Neuroprotective Agents ; Parkinson Disease ; Phosphorylation ; Rats* ; RNA, Messenger ; Valproic Acid*