Purpose: Gastrointestinal stromal tumors (GISTs) are mesenchymal neoplasms of the gastrointestinal tract. GISTs are positive for the expression of c-Kit protein at immunohistochemistry, and their clinical presentations vary. This retrospective study was performed to evaluate the clincopathologic characteristics of GISTs and to define the prognostic factors. Materials and Methods: 40 patients who underwent a complete resection of a GIST during the period $1996\~2003$ at the Department of Surgery, Korea University College of Medicine, were studied. We divided them into low- and high-risk. groups by using tumor size and mitotic count: 23 cases were low risk, and 17 were high risk. Clinicopathologic features, immunohistochemical findings, and prognoses were compared between the low- and the high-risk groups. Results: The mean age of the 40 patients was $61.3\pm11.1$years, and the male-to-female ratio was 1:1.1. There was no significant difference in age and sex between the groups. A comparative analysis revealed tumor size, mitotic count, clinical symptoms, preoperative pathologic diagnosis, ulceration, and necrosis to be variables that had statistically significant differences between the high- and the low-risk groups. In the univariate analysis, tumor size, mitotic count, ulceration, necrosis, and abnormal endoscopic ultrasound findings were associated with disease-free survival, but in the multivariate analysis, mitotic activity was the only independent factor associated with disease-free survival. 8 patients had recurrences during the follow-up period, and four of them were treated with STI-571 (imatinib mesylate, $Gleevec^{(R)}$). The treated patients have survived until now; however, two of non-treated patients died from disease progression. Conclusion: Based on this study, tumor size, ulceration, and necrosis are significant factors affecting survival, and mitotic activity may be a useful prognostic marker. STI-571 may be used in an adjuvant setting because the drug has shown anticancer activity in patients with recurrence or metastasis.
Kim, Seo Young;Jun, Yong Ho;Kwon, Young Joon;Jeong, Hee Yeon;Hwang, Bo Young;Shim, Se Hoon
Korean Journal of Biological Psychiatry
/
v.14
no.3
/
pp.184-193
/
2007
Objectives:There is increasing evidence that the cerebellum plays an important role in cognition and psychiatric symptoms as well as motor coordination. The concept of cognitive dysmetria has been making cerebellar function in schizophrenia the focus of current studies. In other words, disruption in the corticocerebellum-thalamic -cortical circuit could lead to disordered cognition and clinical symptoms of schizophrenia. The purposes of this study were to determine cerebellar dysfunction in male schizophrenic patients semiquantitatively with ICARS and to investigate the clinical and cognitive correlates of ICARS in patients. Methods:We compared the scores of cerebellar neurologic sign using ICARS in 47 male patients with a DSM-IV-TR diagnosis of schizophrenia with 30 gender and age-matched healthy control subjects. The semiquantitative 100-point ICARS consists of 19 items divided into 4 unequally weighted subscores:posture and gait disturbances, kinetic functions, speech disorders and oculomotor disorders. All subjects were also assessed with cognitive function test. Cognitive functions were evaluated by Korean-Mini Mental Status Examination (K-MMSE), Verbal fluency test, and Clock drawing test. The patients were administered Korea version of Positive and Negative Symptom Scale(K-PANSS) to assess the symptom severity. Results:Schizophrenic patients had significantly higher scores on the ICARS than control subjects with posture and gait disturbances, kinetic functions, and oculomotor disorders. They also showed more significant impairments in cognitive function tests than control subjects. There was a significant correlation between ICARS and negative symptoms of patients. In cognitive function test, Clock drawing test was significantly associated with negative symptoms. In addition, Clock drawing test was negatively correlated with the total score of ICARS. Conclusion:In this study, we confirmed that schizophrenic patients have significant impairments in cognitive and cerebellar function, and that those were related with negative symptoms of schizophrenic patients. These results support a role of the cerebellum in schizophrenia. It is meaningful that we used a structured, and reliable procedure for rating neurological soft signs, ICARS. We hope that future prospective studies using a similar design help that rate of neurological sign should have been visible with the progression of illness.
Childhood epilepsy which has high prevalence rate and inception rate is one of the commonest problem encountered in pediatrician. In contrast with epilepsy of adult, in childhood epilepsy, more variable and varying manifestations are found because the factors of age, growth and development exert their influences in the manifestations and the courses of childhood epilepsy. Moreover epileptic children have associated problems such as physical and mental handicaps, psychologicaldisorders and learning disability. For these reasons pediatrician who deals with epileptic children experiences difficulties in making diagnosis and managing them. In order to improve understanding and management of childhood epilepsy, authors reviewed 103 cases of epileptic patients seen at pediatric department of Yeungnam University Hospital retrospectively. The patients were classified according to the type of epileptic seizure. Suspected causes of epilepsy, associated conditions of epileptic patients, age incidence and the findings of brain CT were reviewed. Large numbers of epileptic patients (61.2%) developed their first seizures under the age of 5. The most frequent type of epileptic seizure was generalized ionic-clonic, tonic, clonic seizure (49.5%), followed by simple partial seizure with secondary generalization (17.5%), simple partial seizure (7.8%), a typical absence (5.8%) and unclassified seizure (5.8%). In 83.5% of patients, we could not find specific cause of it, but in 16.5% of cases, history of neonatal hypoxia (4.9%), meningitis (3.9%), prematurity (1.9%), small for gestational age (1.0%), CO poisoning (1.0%), encephalopathy (1.0%), DPT vaccination (1.0%), cerebrovascular accident (1.0%) and neonatal jaundice (1.0%) were found, 30 cases of patients had associated diseases such as mental retardation, hyperactivity, delayed motor milestones or their combinations. The major abnormal findings of brain CT performed in 42 cases were cortical atrophy, cerebral infarction, hydrocephalus and brain swelling. This review stressed better designed classification of epilepsy is needed and with promotion of medical care, prevention of epilepsy is possible in some cases. Also it is stressed that childhood epilepsy requires multidisplinary therapy and brain CT is helpful in the evaluation of epilepsy with limitation in therapeutic aspects.
Purpose: As mesial temporal lobe epilepsy (TLE) shows hypometabolism of medial and lateral temporal lobe, we investigated whether symmetric uptake of F-18-FDG in medial temporal lobes can differentiate mesial from lateral TLE. Materials and Methods: In 113 patients (83 mesial TLE, 30 lateral TLE) who underwent anterior temporal lobectomy and/or corticectomy with good surgical outcome, we performed F-18-FDG PET and compared F-18-FDG uptake of medial and lateral temporal lobes. All the patients with mesial TLE had hippocampal sclerosis except one congenital abnormal hippocampus. Patients with lateral TLE revealed cerebromalacia, microdysgenesis, arteriovenous malformation, old contusion, and cortical dysplasia. Results: Sensitivity of F-18-FDG PET and MR for mesial TLE was 84% (70/83) and 73% (61/83), respectively. Sensitivity of F-18-FDG PET and MR for lateral TLE was 90% (27/30) and 66% (20/30), respectively. Twelve patients were normal on F-18-FDG PET. 101 patients had hypometabotism of lateral temporal lobe. Among 88 patients who showed hypometabolism of medial temporal lobe as well as lateral temporal lobe, 70 were mesial TLE patients and 18 were lateral TLE on pathologic examination. Positive predictive value of medial temporal hypometabolism for mesial TLE was 80%. Among 13 patients who showed hypometabolism of only lateral temporal lobe, 4 were mesial TLE and 9 were lateral TLE. Positive predictive value of hypometabolism of lateral temporal lobe for the diagnosis of lateral TLE was 69% (9/13). Normal MR findings stood against medial TLE, whose negative predictive value was 66%. Conclusion: Lateral temporal lobe epilepsy should be suspected when there is decreased F-18-FDG uptake in lateral temporal lobe with normal uptake in medial temporal lobe.
Puorpose: The purpose of this study was to evaluate the usefulness of $^{99m}Tc$ DMSA scintigraphy on the dignosis of a renal scar in children with urinary tract infections. Materials and Methods: Eighty three patients were included in this study, who were diagnosed as the urinary tract infection on the basis of symptom, urinalysis and urine culture. $^{99m}Tc$ DMSA scintigraphy and voiding cystoureterography were peformed within 7days before the treatment in all patients. We classified the scintigraphic findings as follow s : 1 ; a large hypoactive upper or lower pole. 2 ; a small hypoactive area. 3 ; single defect resulting in localized deformity of the outlines. 4 ; deformed outlines in a small or normal sized kidney. 5 ; multiple defects. 6 ; diffuse hypoactive kidney without regional impairment. Follow-up scintigraphy was done at least 6 months after the initial study. When the abnormality on the initial scintigraphy was not completely resolved on the follow-up scan, the lesion was defined as containing a scar. Results: One hundred and fifteen renal units of 166 units(69.3%) showed abnormal findings on the DMSA scintigraphy. 65 units(56.5%) was diagnosed as containing renal scars on follow-up scintigraphies. Incidences of renal scar among renal units showing pattern 3, 4 and 5 on the initial scan was 75%, 78% and 78%, respectively. Whereas many of renal units showing 1, 2 and 6 pattern were recovered(65%, 76%, 50%). Sensitivity, specificity and accuracy of pattern-based DMSA scintigraphic findings on the diagnosis of renal scar was 76.9%, 85.1% and 81.9%, respectively. VUR was significantly associated with the renal scar when the initial DMSA shows unrecoverable findings(pattern 3, 4, 5). Odds ratio of the renal scar in a kidney showing unrecoverable initial scintigraphic findings was 19.1. Odds ratio in a kidney with mild or moderate-to-severe VUR was 3.5 and 14.4 respectively. Conclusion: In the urinary tract infection, renal scar was significantly developed in a kidney showing unrecoverable findings on the initial DMSA scan and VUR on voiding cystoureterography.
Purpose : Development of renal scarring is associated with delayed diagnosis and treatment of urinary tract infection(UTI). This study was performed to clarify how soon treatment should be started to Inhibit renal scarring after onset of UTI and the factors associated with renal scarring in children with a first episode of febrile UTI. Methods : We retrospectively reviewed 163 patients with a first episode of febrile UTI under the age of 2 years from April 2000 to Ap,il 2004. All patients had a DMSA renal scan and voiding cystourethrogram done in the diagnostic period, 6 months after which a follow-up renal scan was done. After patients wet-e divided into 2 groups according to the duration of fever prior to start of treatment, the duration of fever after start of treatment, and total duration of fever, initial and follow-up DMSA scan findings were analyzed among the different groups. We compared the factors associated with renal scars between the groups with and without renal scars. Results : The initial DMSA renal scan identified abnormal finding in 23% of the patients who were treated $\leq$24 hr from the onset of disease and in 43% of those with fever more than 24 hr. Renal scars developed in 33% of patients who were treated $\leq$24 hr and 38% of those with fever >24 hr prior to treatment. Renal scars developed in 34% of patients with remission of fever $\leq$48 hr after treatment and ill 50% of those with fever >48 hr after treatment. The risk for renal scars was significantly higher in children who had total duration of feyer >72 hr(67%) than in those with shorter duration(19%). In children with renal scars, VUR was most highly associated with an increased risk of renal scar formation. Conclusion : Although children with a first episode of febrile UTI are treated within 24 hr after onset of the fever, renal damage cannot be prevented completely and it is mainly associated with VUR. (J Korean Soc Pediatr Nephrol 2005;9:56-63)
Kim, Sae Yoon;Lee, Sang Su;Lee, Jae Min;Kang, Seok Jeong;Kim, Yong Jin;Park, Yong Hoon
Childhood Kidney Diseases
/
v.17
no.2
/
pp.49-56
/
2013
Purpose: IgA nephropathy (IgAN) is one of the major causes of end-stage renal disease. Mass school urine screening (SUS) has been performed to enable early detection of chronic renal diseases, including IgAN. We wanted to evaluate the patients with IgAN, including those diagnosed through SUS. Methods: Between 1998 and 2010, 64 children were diagnosed with IgAN based on renal biopsy results obtained at the Pediatric Nephrology Department, ${\bigcirc\bigcirc}$ University Hospital. We divided these patients into the SUS group (37 cases), diagnosed through SUS, and the symptomatic (Sx) group (27 cases), diagnosed clinically. The medical records of both groups were analyzed retrospectively. Results: The mean age of the SUS and Sx groups was $10.8{\pm}2.7$ and $9.5{\pm}3.4$ years (P >0.05), respectively. Both groups had a higher proportion of male patients. The time from the notification of an abnormal urinary finding to a hospital visit or renal biopsy was shorter in the Sx group than in the SUS group. Regarding clinical manifestations, there were fewer cases with gross hematuria (P <0.001) and edema (P =0.008) in the SUS group, but there were no differences in terms of the therapeutic regimen and treatment duration. Regarding laboratory parameters, the Sx group had a higher white blood cell count (P =0.007) and lower hemoglobin (P =0.007) and albumin (P =0.000) levels. There were no differences in the renal biopsy findings in both groups, based on the history of gross hematuria or the severity of proteinuria. However, in all 64 patients with IgAN, the light microscopy findings (Hass classification) were related to a history of gross hematuria or the severity of proteinuria. Conclusion: There were no significant clinical and histological differences between the groups, as both had early stage IgAN. Although SUS facilitates the early detection of IgAN, long-term, large-scale prospective controlled studies are needed to assess the benefits of early diagnosis and treatment in chronic renal disease progression.
Purpose: Human Na+/I- symporter (hNIS) is known to be expressed in many tissues other than thyroid gland. The breast cancer cells are one of them and the possibility of radioiodine therapy in treatment of the breast cancer may be suggested. We investigated the expression rate of hNIS and the relationship between the expression of hNIS and the finding of 99mTc-MIBI scintimammographv in the breast cancer Materials and Methods: Surgically proved 56 patients with breast cancer were the subjects of this study The expression of hNIS were evaluated by immunohistochemistry and the results were compared to the findings of 99m7c-MIBI scintimammography. Results: Overall expression rate of hNIS was 41.1% in 56 patients. According to the pathologic diagnosis, it was 42.9% in 49 patients with invasive ductal carcinoma and 28.6% in the 7 patients with ductal carcinoma in situ. The expression rate of hNIS in the 41 cases with a focal increased uptake at he breast lesion on 99m7c-MIBI sointimammogram was 31.7%. That in the 15 cases without any abnormal uptake on the scan was significantly higher(65.7%, p<0.05). Conclusion: The expression rate of hNIS in the patients with breast cancer was not so high. The rate was higher in the patients with no increased uptake at the breast lesion on 99m7c-MIBI scintimammography.
Journal of The Korean Society of Inherited Metabolic disease
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v.18
no.2
/
pp.43-49
/
2018
Maple syrup urine disease (MSUD, OMIM#248600) is a rare and autosomal recessively-inherited metabolic disorder that is caused by mutations in the branched-chain ${\alpha}$-ketoacid dehydrogenase (BCKDH) genes. It prevents the normal breakdown of branched-chain amino acids (BCAAs), such as leucine, isoleucine, and valine, and leads to poor feeding, lethargy, abnormal movements, seizure, and death if untreated. Here, we report the case of a Korean newborn of biochemically- and genetically-confirmed MSUD manifesting lethargy and central apnea, the acute state of which was successfully treated. The molecular genetic investigation revealed two novel heterozygous mutations (p.Ala32Phefs*48 and p.Val 130Phe) in BCKDHB, and both parents were confirmed as carriers. We emphasize the importance of early diagnosis and prompt introduction of specific treatment for MSUD in life saving and prognosis.
Sangmin Bak;Mina Kwon;Dong Hyun Kang;Hong-Kyu Lee;Young-Nam Yoon;In-Yeol Baek;Young Gyu Lee;Jae Sun Moon;Su-Heon Lee
KOREAN JOURNAL OF CROP SCIENCE
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v.67
no.4
/
pp.253-264
/
2022
In this study, we investigated the occurrence of viral diseases in the early growth stage of soybean to establish management practices. We collected 83 soybean samples showing abnormal symptoms, approximately 3-4 weeks after seeding in the breeding field of the National Institute of Crop Science. Viruses were detected in the collected samples using reverse transcription polymerase chain reaction (RT-PCR) and metatranscriptome analysis of all those samples. The incidence of viral diseases in the field was less than 1% overall and up to 50% in certain cultivars and lines. RT-PCR and metatranscriptome analysis detected Soybean yellow mottle mosaic virus (SYMMV), Soybean mosaic virus (SMV), Soybean yellow common mosaic virus, Peanut stunt virus, and soybean geminivirus A (SGVA). Among these detected viruses, SYMMV and SMV were identified as major viruses causing infection in the early growth stage of soybean, with detection rates of 53.7% and 42.6%, respectively. Soybeans infected with SYMMV showed typical mosaic symptoms, whereas those infected with SMV showed a variety of symptoms such as mosaic, mottle, stunt, and chlorotic spots. Transmission characteristics of these viruses are variable, such that SMV is primarily transmitted by seeds, whereas SYMMV could be transmitted by insects, soil, and seeds. In this study, SGVA was detected in the early growth stage of soybean, and research on the current status and its effects on soybean after the early growth stage should be conducted.
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