One Case of Thoracic Empyema due to Salmonella choleraesuis / 감염과화학요법

Salmonella are motile, gram-negative, non-spore-forming members of the family Enterobacteriaceae. Among nontyphoid Salmonella serotypes, Salmonella choleraesuis shows a high predilection to cause systemic infections in humans. Thoracic infection is a rare complication of Salmonella infection. So far, most of reported cases of empyema caused by Salmonella spp. have involved immunocompromised patients. Herein, as we had experienced one case of thoracic empyema due to Salmonella choleraesuis related thymoma, we report it with review of literature.

One Case of Thoracic Empyema due to Salmonella choleraesuis / 감염과화학요법

Salmonella are motile, gram-negative, non-spore-forming members of the family Enterobacteriaceae. Among nontyphoid Salmonella serotypes, Salmonella choleraesuis shows a high predilection to cause systemic infections in humans. Thoracic infection is a rare complication of Salmonella infection. So far, most of reported cases of empyema caused by Salmonella spp. have involved immunocompromised patients. Herein, as we had experienced one case of thoracic empyema due to Salmonella choleraesuis related thymoma, we report it with review of literature.

Uremic Encephalopathy Associated with Bilateral Basal Ganglia and Cerebellar Lesion in a Non-diabetic Hemodialysis Patient / 대한신장학회잡지

Involvement of central nervous system is a well- known compication in uremic patients. However, development of acute extrapyramidal symptoms with bilateral basal ganglia involvement (acute basal ganglia syndrome), especially in non-diabetic hemodialysis patient is very rare. We report a case of acute basal ganglia syndrome in a non-diabetic hemodialysis patient. A 45-year-old man with autosomal dominant polycystic kidney disease (ADPKD) on chronic hemodialysis treatment for the last 4 years was admitted due to generalized myalgia. On admission, the patient was found to have rhabdomyolysis and intractable metabolic acidosis. Nine days after admission, he suddenly developed dysarthria, lateralizing ataxia, and bradykinesia. Brain MRI demonstrated low and high signals in bilateral basal ganglia and cerebellar vermis in T1-weighted and T2-weighted images, respectively. Intensified hemodialysis treatment combined with general supportive therapy resolved the severe metabolic acidosis and the neurologic manifestations gradually improved. Follow up brain CT scan taken 3 months later showed decreased size of initial low attenuation lesions in bilateral basal ganglia and cerebellar vermis. Although no definite pathophysiology is yet established, severe metabolic disorder is believed to play an important role in development of acute basal ganglia syndrome. Correction of metabolic acidosis and hypoglycemia in our patient lead to improvement in neurologic manifestations and organic brain lesions. Our case suggests that severe metabolic acidosis and hypoglycemia in uremic patient may act as risk factors for acute basal ganglia syndrome even in non-diabetic patient.

Two Cases of Bacteremia Caused by Vibrio Parahemolyticus in Liver Cirrhosis / 감염과화학요법

We have experienced two cases of bacteremia in liver cirrhosis patients caused by Vibrio parahemolyticus. One presented with acute gastroenteritidis symptoms such as vomiting, diarrhea. Vibrio parahemolyticus was cultured in the blood. In the other patients showed vesicular rash in lower extremities with a positive blood culture. Both patients had been treated with intravenous fluid and antibiotics.

One Case of Invasive bone Aspergillosis / 감염과화학요법

Invasive aspergillosis has been reported in patients with profound neutropenia or patients with any form of immunodeficiency. Bone aspergillosis remains a rare. Herein, as we had experienced one case of bone aspergillosis patient treated with voriconazole, we report it with the review of literature.

Two Cases of Bacteremia Caused by Vibrio Parahemolyticus in Liver Cirrhosis / 감염과화학요법

We have experienced two cases of bacteremia in liver cirrhosis patients caused by Vibrio parahemolyticus. One presented with acute gastroenteritidis symptoms such as vomiting, diarrhea. Vibrio parahemolyticus was cultured in the blood. In the other patients showed vesicular rash in lower extremities with a positive blood culture. Both patients had been treated with intravenous fluid and antibiotics.

One Case of Invasive bone Aspergillosis / 감염과화학요법

Invasive aspergillosis has been reported in patients with profound neutropenia or patients with any form of immunodeficiency. Bone aspergillosis remains a rare. Herein, as we had experienced one case of bone aspergillosis patient treated with voriconazole, we report it with the review of literature.

A Case of Dominantly Inherited beta Thalassemia Due to Hb Dieppe

beta thalassemias are usually transmitted as autosomal recessive traits. However, some dominant forms of beta thalassemia have been identified in individuals who have inherited a single copy of an abnormal beta globin gene. Thalassemia intermedia with mild anemia, jaundice, and splenomegaly has been observed in these patients. Electrophoresis has shown elevated Hemoglobin(Hb) A2 and Hb F levels. In particular, there are inclusion bodies in the erythroid precursors and peripheral red blood cells after splenectomy. The molecular basis of these dominant beta thalassemias is heterogeneous. The authors studied the first Korean case of dominantly inherited beta thalassemia due to Hb Dieppe. Hb Dieppe is a missense mutation of beta codon 127(CAG->CGG)Gln->Arg. The patient in this case was characterized by moderate anemia, hypochromia, microcytosis, elevated Hb A2 levels, elevated Hb F levels and splenomegaly. The father of the patient also has the same disease. We report this case and review related literature.

Organic Acidemias in Korea: Three Years Experience of Organic Acid Analysis

PURPOSE: We have done this retrospective study to know the relative incidences and clinical manifestations of organic acidopathies in Korea. METHODS: The results of quantitative organic acid analysis of 1,125 samples of 712 patients, referred from Jul. 1997 to Jun. 2000, were analyzed retrospectively according to four age groups (-2 mon, 3 mon-2 year, 3 years-12 years, over 12 years) and major clinical manifestations. Quantification of 83 organic acids was done with gas chromatography and mass spectrometry(GC/MS). RESULTS: We diagnosed 214 patients with 27 diseases of organic acid metabolism during this study period. Diseases found more than 10 cases are cytosolic 3-ketothiolase deficiency, mitochondrial repsiratory chain disorders, PDHC deficiency, glutaric aciduria type II and propionic aciduria. Other diseases were diagnosed in less than 10 cases, mostly one or two cases during this study period. Most of the patients had some symptoms of neurological dysfunction such as seizure activity(195 patients), developmental delay(122), mental retardation(99), hypotonia(84), movement disorders(81) and vomiting(68). CONCLUSION: Though the incidence of individual organic acidemia is low, the overall incidence of organic acidemia as a whole seems to be relatively high in Korea. Most of the patients showed some signs of neurological dysfunction.

Quantification of Age-related Reference Values for Urinary Organic Acids in the Healthy Korean Population

PURPOSE: For the accurate diagnosis of organic acidopathies, quantification of urinary organic acid should be done and we should know the normal ranges of each organic acid excreted in the urine. The amount of organic acids excreted in the urine shows wide variability according to ethnic group, diet and age. We have quantified 82 organic acids to make a Korean reference value. METHODS: Organic acid concentrations were quantified with gas chromatography and the individual acids identified with mass spectrometry in urine specimens from members of the healthy Korean population of ages of one day to more than 12 years, subdivided into four age groups : neonatal period(-2 mon), infantile period(-2 year), childhood period(-12 year) and adolescent and adulthood(over 12 years). For isolation of organic acids from urine, we used solvent extraction method with ethylacetate. Derivatization was done with MSTFA(N-methyl-N-trimethylsilylfluoroacetamide). The library and four points quantification curve for the quantification of each organic acid that we used have been developed by Dr. Giudici of Kayser Permanante Metabolic Laboratory, CA., USA. RESULTS: Quantitative ranges and frequency distribution patterns of urinary organic acid excretion are reported, as a basis on which to compare results obtained for patients whose clinical condition suggests that their excretion values may be abnormal. CONCLUSION: The quantitative values we observed, enable the relative significance of different urinary metabolites to be assessed.