BACKGROUND/AIMS: Because there is a lack of effective biomarkers, we aimed to discover proteomic candidate markers for hepatocellular carcinoma (HCC) in cirrhotic patients at the highest-risk of HCC, and to validate the markers. METHODS: We collected tumor tissue from 5 cirrhotics with HCC, and from 5 cirrhotics without HCC, who underwent liver resection or transplantation. These tissue samples were analyzed by 2-dimensional difference gel electrophoresis coupled with matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS), and potential markers were validated at the transcriptional and translational levels. We also performed western blot assays using other blood samples from 10 cirrhotics with HCC and 10 without HCC. RESULTS: Among the 66 distinguishable spots on 2-D gel images, we identified 15 proteins overexpressed more than 1.5 fold in terms of volume ratio in the tumors. Ten of the over-expressed proteins were identified by MALDI-TOF MS; of those, only methionine adenosyltransferase 1 (MAT1), a protein specific for liver, and acyl-CoA dehydrogenase were significantly up-regulated in tumors in further immunoblotting analyses (Ps<0.05). There was no between-pair difference in MAT1 mRNA measured by real-time polymerase chain reaction (P=0.96). However, in western blots of serum samples, distinct MAT1 bands were observed in all 10 HCC patients, but in only 2 of the non-HCC patients. CONCLUSIONS: MAT1 is a potential marker for surveillance in cirrhotic patients with and without prior HCC.
Acyl-CoA Dehydrogenase ; Biomarkers ; Blotting, Western ; Carcinoma, Hepatocellular ; Humans ; Immunoblotting ; Liver ; Liver Cirrhosis ; Mass Spectrometry ; Methionine Adenosyltransferase ; Methionine ; Proteomics ; Real-Time Polymerase Chain Reaction ; RNA, Messenger ; Two-Dimensional Difference Gel Electrophoresis

Following a report by Hultengren et al. (Acta Chir Scand, 1965), it has been suggested that analgesic abuse predisposes to urothelial neoplasia. Urinary tract malignancy is combined in 8-10% of patients with analgesic nephropathy. Microscopic or gross hematuria can be the first sign leading to the diagnosis of uroepithelial malignanacy in analgesic abusers. Since uroepithelial malignancies found in analgesic abusers tend to be multiple and have a worse prognosis, continued monitoring is essential, and new hematuria should be evaluated with urinary cytology, and cystoscopy with reterograde pyelography. Phenacetin found to be the chief cause of malignancies in analgesic abusers, it has been anticipated to be a human carcinogen and was banned as an OTC drug since 1987. But still there remains a debate whether acetaminophen and other compound analgesic components are carcinogenic. We report the case of a 58-year-old man with a history of analgesic abuse who was diagnosed with transitional cell carcinoma combined with analgesic nephropathy. We also review the literature.
Acetaminophen ; Analgesics ; Carcinoma, Transitional Cell* ; Cystoscopy ; Diagnosis ; Hematuria ; Humans ; Middle Aged ; Nephritis, Interstitial ; Phenacetin ; Prognosis ; Ureter* ; Urinary Tract ; Urography

PURPOSE: Clinical and pathological prognostic factors of idiopathic IgA nephropathy have been reported, but mostly in adults and a few in children. Especially studies about correlation between those factors are very rare. METHODS: We studied 58 children patients who were hospitalized to our clinics and diagnosed as IgA nephropathy by renal biopsy from Jan. 1989 to Jun 1996. They got divided into several clinical groups, which are heavy proteinuria group (group A), asymptomatic urinary abnormalities group proteinuria and/or microscopic hematuria (group B), and recurrent gross hematuria group (group C). They are also divided into younger group (younger than 10 years of age) and older group (older than 10 years og age). We compared their pathological findings of bad prognosis, if they have, in different clinical groups. RESULTS: Group A had most pathological factors of bad prognosis such as higher Meadow grade, crescent formation, necrosis, glomerulosclerosis, tubular atrophy, interstitial fibrosis, two or more kinds of immune deposit except IgA, high frequency of electron dense deposits of glomerular capillary wall. Group B treded to have some poor prognostic factors such as tubular atrophy and interstitial fibrosis. in terms of age groups, older group was more apt to be heavily proteinuric than younger group, have such pathological factors of poor prognosis that group A had. CONCLUSION: Heavy proteinuria and relative old age in childhood IgA nephropathy, considered clinically poor prognostic, appears significantly correlated with pathologically poor prognostic factors.
Adult ; Atrophy ; Biopsy ; Capillaries ; Child* ; Fibrosis ; Glomerulonephritis, IGA* ; Hematuria ; Humans ; Immunoglobulin A* ; Necrosis ; Prognosis ; Proteinuria

PURPOSE: Clinical and pathological prognostic factors of idiopathic IgA nephropathy have been reported, but mostly in adults and a few in children. Especially studies about correlation between those factors are very rare. METHODS: We studied 58 children patients who were hospitalized to our clinics and diagnosed as IgA nephropathy by renal biopsy from Jan. 1989 to Jun 1996. They got divided into several clinical groups, which are heavy proteinuria group (group A), asymptomatic urinary abnormalities group proteinuria and/or microscopic hematuria (group B), and recurrent gross hematuria group (group C). They are also divided into younger group (younger than 10 years of age) and older group (older than 10 years og age). We compared their pathological findings of bad prognosis, if they have, in different clinical groups. RESULTS: Group A had most pathological factors of bad prognosis such as higher Meadow grade, crescent formation, necrosis, glomerulosclerosis, tubular atrophy, interstitial fibrosis, two or more kinds of immune deposit except IgA, high frequency of electron dense deposits of glomerular capillary wall. Group B treded to have some poor prognostic factors such as tubular atrophy and interstitial fibrosis. in terms of age groups, older group was more apt to be heavily proteinuric than younger group, have such pathological factors of poor prognosis that group A had. CONCLUSION: Heavy proteinuria and relative old age in childhood IgA nephropathy, considered clinically poor prognostic, appears significantly correlated with pathologically poor prognostic factors.
Adult ; Atrophy ; Biopsy ; Capillaries ; Child* ; Fibrosis ; Glomerulonephritis, IGA* ; Hematuria ; Humans ; Immunoglobulin A* ; Necrosis ; Prognosis ; Proteinuria

Distal renal tubular acidosis (RTA) is well-recognized complication of immunologically mediated condition such as Sj ogren's syndrome, SLE, idiopathic hypergammaglobulinemia, autoimmune liver disease, autoimmune thyroid disease, multiple myeloma, and renal tranplant rejection. Tubulointerstitial involvement frequently occurrs in SLE. A sepctrum of abnormalities including impaired uriary concentration or acidification, increased fractional excretion of low molecular weight protein, hyporeinaemic hypoaldosteroniam and impaired tubular potassium excretion can occur in SLE. But complete distal RTA associated wih SLE is rare. We report a 13 year-old female with SLE and distal RTA which was diagnosed by NaHCO3 loading test. She had nephrotic syndrome, hypokalemia, hyperchloremic metabolic acidosis with alkali urine. She had fatiquebility, general weakness, intermittent fever and chest pain for at least 12months. And then, the butterfly-shaped malar rash was developed, so pediatrician suspected SLE and she was refer to us. At hospitalization. She had malar rash, percardial effusion, persistent proteinuria greater than 3+, hemolytic anemia, lymphopenia, leukopenia, positive LE cell, Anti-DNA Ab and posotive ANA. So her clinical data are satisfied ARA criteria for SLE. Her renal biopsy showed diffuse proliferative SLE nephpritis and marked focal tubular atrophy with localized heavy mononuclear cell infiltration and fibrosis. We performed NaHCO3 loading test to confirm distal RTA. During the test, we detected the inability to achieve a high urinary PCO2. This result is the most sensitive index of impaired distal acidification, so we can diagnose distal RTA. She had a good response to the therapy with prednisolone NaHCO3 and oral KCL supplement.
Abdominal Pain ; Acidosis ; Acidosis, Renal Tubular* ; Adolescent ; Alkalies ; Anemia, Hemolytic ; Atrophy ; Biopsy ; Chest Pain ; Child, Preschool ; Diagnosis* ; Exanthema ; Female ; Fever ; Fibrosis ; Hospitalization ; Humans ; Hypergammaglobulinemia ; Hypokalemia ; Intestinal Volvulus* ; Leukopenia ; Liver Diseases ; Lupus Erythematosus, Systemic* ; Lymphopenia ; Melena ; Mesentery ; Molecular Weight ; Multiple Myeloma ; Nephrotic Syndrome ; Neutrophils ; Potassium ; Prednisolone ; Proteinuria ; Thyroid Diseases ; Tomography, X-Ray Computed*

Distal renal tubular acidosis (RTA) is well-recognized complication of immunologically mediated condition such as Sj ogren's syndrome, SLE, idiopathic hypergammaglobulinemia, autoimmune liver disease, autoimmune thyroid disease, multiple myeloma, and renal tranplant rejection. Tubulointerstitial involvement frequently occurrs in SLE. A sepctrum of abnormalities including impaired uriary concentration or acidification, increased fractional excretion of low molecular weight protein, hyporeinaemic hypoaldosteroniam and impaired tubular potassium excretion can occur in SLE. But complete distal RTA associated wih SLE is rare. We report a 13 year-old female with SLE and distal RTA which was diagnosed by NaHCO3 loading test. She had nephrotic syndrome, hypokalemia, hyperchloremic metabolic acidosis with alkali urine. She had fatiquebility, general weakness, intermittent fever and chest pain for at least 12months. And then, the butterfly-shaped malar rash was developed, so pediatrician suspected SLE and she was refer to us. At hospitalization. She had malar rash, percardial effusion, persistent proteinuria greater than 3+, hemolytic anemia, lymphopenia, leukopenia, positive LE cell, Anti-DNA Ab and posotive ANA. So her clinical data are satisfied ARA criteria for SLE. Her renal biopsy showed diffuse proliferative SLE nephpritis and marked focal tubular atrophy with localized heavy mononuclear cell infiltration and fibrosis. We performed NaHCO3 loading test to confirm distal RTA. During the test, we detected the inability to achieve a high urinary PCO2. This result is the most sensitive index of impaired distal acidification, so we can diagnose distal RTA. She had a good response to the therapy with prednisolone NaHCO3 and oral KCL supplement.
Abdominal Pain ; Acidosis ; Acidosis, Renal Tubular* ; Adolescent ; Alkalies ; Anemia, Hemolytic ; Atrophy ; Biopsy ; Chest Pain ; Child, Preschool ; Diagnosis* ; Exanthema ; Female ; Fever ; Fibrosis ; Hospitalization ; Humans ; Hypergammaglobulinemia ; Hypokalemia ; Intestinal Volvulus* ; Leukopenia ; Liver Diseases ; Lupus Erythematosus, Systemic* ; Lymphopenia ; Melena ; Mesentery ; Molecular Weight ; Multiple Myeloma ; Nephrotic Syndrome ; Neutrophils ; Potassium ; Prednisolone ; Proteinuria ; Thyroid Diseases ; Tomography, X-Ray Computed*

We experienced a case of G deletion syndrome II in a 8 year and 1 month old boy. He showed mental retardation, microcephaly, high arched palate, low set malformed ears, epicanthal fold, thoracic deformity, tracheomegaly and two lumens of esophagus. Chromosomal study showed the deletion of long arm of chromosome 22, kariotypically he was depicted as 46, XY, 22q-. A brief review of literature was also presented.
Arm ; Chromosomes, Human, Pair 22 ; Congenital Abnormalities ; Ear ; Esophagus ; Humans ; Infant, Newborn ; Intellectual Disability ; Male ; Microcephaly ; Palate

The author observed clinical study and response of -interferon treatment of 43 cases of chronic hepatitis who were admitted to Department of Pediatrics Pusan National University College of Medicine during the period of 4 year 6 months from Jenuary, 1988 to Jun, 1992. The results were as follows: 1) Of 43 patients with chronic hepatitis, CPH was 17 cases (39.5%) and CAH was 26 cases (60.5%). 2) Of 43 patients, chronic hepatitis caused by HBV was 37 cases (86.0%) 2 cases were by Wilson's disease, 1 case was by glycogen storage disease and in the 3 cases, the cause was undetermined and CPH by HBV and CAH were 14 cases (82.4%), 23 cases (88.5%) respectively. 3) A peak frequency (46.5%) of chronic hepatitis occurred between 6 to age and male predominated in a propotion of 3.5:1. 4) On the clinical manifestation, jaundice and hepatomegaly was higher frequency on CAH than on CPH. 5) On the laboratory findings, CAH had higher serum ALT and direct bilirubin level than CPH. 6) On effect of -interferon treatment for patients of CAH, serum ALT and AST levels normalized in 81.8% of patients, negative conversion of HBeAg was 38.5%, Anti-HBe serocoversion was 7.7%, negative conversion of HBsAg was 7.7% and the clearnce of HBV-DNA from seum showed 60.0%. 7) The HBeAg seroconversion (28.6%) on children of HBsAg(+) mother showed lower reponse than HBeAg seroconversion (50.0%) of HBsAg(-) mother after -interferon treatment for patients of CAH.
Bilirubin ; Busan ; Child* ; Glycogen Storage Disease ; Hepatitis B e Antigens ; Hepatitis B Surface Antigens ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Hepatolenticular Degeneration ; Hepatomegaly ; Humans ; Jaundice ; Male ; Mothers ; Pediatrics

Primary hyperparathyroidism is a rare endocrine disease in children. It involves bone and joint, urinary tract, gastrointestinal tract and cardiovascular system. The main cause of these involvement is high level of PTH in serum, resulting in hypercalcemia.An 11 years old male patient who had complained of limping gait since last 18 months, showed typical laboratory and radiological findings of primary hyperparathyroidism. At the ultrasonography, computed tomography and radionuclide scanning, a well defined mass(10 X 15mm) was found on the posterior aspect of the right thyroid lobe. The mass was confirmed histologically as adenoma of parathyroid gland. The patient was successfully treated with subtotal parathyroidectomy and temporal administration of calcium and vitamin D.We report this case of primary hyperparathyroidism with brief review of the literatures.
Adenoma ; Calcium ; Cardiovascular System ; Child ; Endocrine System Diseases ; Gait ; Gastrointestinal Tract ; Humans ; Hyperparathyroidism, Primary ; Joints ; Male ; Parathyroid Glands ; Parathyroidectomy ; Thyroid Gland ; Ultrasonography ; Urinary Tract ; Vitamins

No abstract available.
Child* ; Humans ; Lung Diseases, Interstitial*