Two siblings with Bardet-Biedl syndrome caused by mutations in BBS10: the first case identified in Korea

Bardet-Biedl syndrome (BBS) is a rare ciliopathy generally inherited with an autosomal recessive pattern. BBS is characterized by 6 primary features namely retinal dystrophy, obesity, postaxial polydactyly, renal dysfunction, learning difficulties, and hypogonadism and a wide range of secondary features. To date, mutations in 16 genes have been identified as causative factors for BBS. Among them, the BBS1 and BBS10 genes are major disease-causing genes, and each of these gene mutations presents in more than 20% of all BBS patients. Genotype-phenotype correlations have not been observed in BBS, and there can be phenotypic overlap between BBS and other ciliopathies. In Korea, no molecular, genetically confirmed case of BBS has been reported to date. Herein, we describe the case of the first Korean siblings with BBS resulting from 2 BBS10 gene mutations who showed typical clinical phenotypes, including retinal dystrophy, obesity, intellectual disability, cystic tubular disease, and postaxial polydactyly.

Clinical and radiological features of pituitary stalk lesions in children and adolescents

PURPOSE: The diagnosis of pituitary stalk lesion has been based on clinical feature, radiologic assessment for its critical location and role. This study aimed to investigate clinical symptoms, endocrine disturbance, magnetic resonance imaging (MRI) findings of pituitary stalk lesions in children and adolescents and to evaluate differences between neoplastic lesions with the others. METHODS: We performed a retrospective review of patients under 18 years old with pituitary stalk lesions diagnosed at the Seoul National University Children's Hospital between 2000 and 2013, by a text search for head MRI reports by using 'pituitary stalk', 'infundibulum', and 'infundibular stalk', as keywords. RESULTS: For the 76 patients, sixteen patients (21.1%) had congenital lesions, and 52 (68.4%) had neoplasms. No inflammatory lesions were found. Diabetes insipidus (DI) was the most common endocrine defect, diagnosed in 38 patients (50%). There was male predominance especially in neoplastic group. Thickened pituitary stalk was, but enhancement of lesion was not, associated with neoplasm. DI was more prevalent in neoplastic stalk lesions. Anterior pituitary dysfunction such as growth hormone and adrenocorticotropic hormone deficiencies were less prevalent in neoplastic lesions of pituitary stalk. CONCLUSION: In conclusion, the etiology of pituitary stalk lesions in children and adolescents is diverse and different from that in adults. Neoplastic pituitary stalk lesions can be differentiated from nonneoplastic lesions by systemic evaluation of clinical, hormonal, radiological findings.

Role of Microbiologic Culture Results of Specimens Prior to Onset of Ventilator-Associated Pneumonia in the Patients Admitted to Intensive Care Unit / 결핵및호흡기질환

BACKGROUND: Patients with ventilator-associated pneumonia (VAP) in intensive care unit (ICU) have a high mortality rate. The routine surveillance cultures obtained previously or an ATS guideline for hospital-acquired pneumonia was used in selecting initial antimicrobials. The object of this study was to compare the respiratory samples before VAP and bronchoalveolar lavage (BAL) culture. METHODS: 54 patients underwent fiberoptic bronchoscopy to obtain BAL samples. We reviewed microbiologic specimen results of prior respiratory specimens (pre-VAP) and BAL. RESULTS: Among 51 patients with 54 VAP episodes, 52 microorganisms of pre-VAP and 56 BAL samples were isolated. Pre-VAP included 21.2% of MRSA, and 32.6% of multidrug resistant-Acinetobacter baumannii (MDR-AB). BAL samples comprised 25.0% of MRSA, 26.7% of MDR-AB, 14.3% of Stenotrophomonas maltophilia and 3.6% of Klebsiella pneumonia in order. In pre-VAP samples compared to BAL samples, only 35.2% were identical. In BAL samples compared to pre-VAP samples obtained in 5 days before the onset of VAP, only 43.6% were identical. However, among BAL samples compared to pre-VAP samples obtained after more than 5 days, 13.3% were identical (p=0.037). CONCLUSION: Based on these data, pre-VAP samples obtained prior to 5 day onset of VAP may help to predict the causative microorganisms and to select appropriate initial antimicrobials.

A Seroepidemiological Survey of Toxocariasis among Eosinophilia Patients in Chungcheongnam-do

There have been several reports on the relationship between toxocariasis and eosinophilia, but all have been limited to the areas of Seoul or Gangwon-do. In the present study, we investigated the seroprevalence of toxocariasis among eosinophilia patients in Chungcheongnam-do, the central district of Korea. Among the 101 patients tested, 51 (50.5%) were identified as positive by Toxocara ELISA, and 46 (45.5%) were confidently diagnosed with toxocariasis because of absence of any other cause of eosinophilia. Whereas 22 of 42 seropositive patients (52.3%) had a recent history of consuming raw livers, especially the cow liver, only 1 of 25 seronegative patients (4%) had done so (P<0.01). From these results, we could confirm that toxocariasis is related to eosinophilia, and infer that ingestion of raw cow liver plays a vital role in the transmission of toxocariasis in Chungcheongnam-do.

Longitudinal Study of Iron Concentration in Korean Preterm Human Milk

PURPOSE: The unique nutrient requirements of premature infants necessitate knowledge of the composition of human milk produced by mothers of such infants. We investigated longitudinal changes in iron concentration of preterm human milk and compared to those observed in human milk of mothers of 1-week old term infants to determine optimal iron supplementation guidelines when preterm infants are nourished exclusively by breast feeding. METHODS: Human milk samples were collected at 1, 2, 4, 6, 8 and 12 weeks postpartum from 103 mothers who delivered infants of gestational age <34 weeks or weighing <1,800 g. Term human milk samples were collected at 1 week postpartum from 24 mothers. RESULTS: There were no significant differences in the iron concentrations of preterm human milk obtained at 2 to 8 weeks postpartum (36.3+/-23.1 to 45.8+/-26.0 microg/dL), but these concentrations were higher than those noted at 1 week in preterm (23.1+/-14.6 microg/dL) and term (25.2+/-7.55 microg/dL) infants. The iron concentration in preterm human milk obtained at corrected term age (42.2+/-19.4 microg/dL) was significantly higher than that of mature term human milk (25.2+/-7.55 microg/dL). CONCLUSION: The concentration of iron in preterm human milk was consistently low during the first 3 months of lactation. Supplementation with iron of at least 2 mg/kg/day should be considered for preterm infants who are exclusively breastfed and who have low body iron stores, to meet the minimum enteral iron requirements recommended by AAP-CON (2004).

The significance of serum C-telopeptide as a bone marker in chronic hemodialysis patients / 대한내과학회지

BACKGROUND/AIMS: Checking bone mineral density (BMD) is not sufficient for determining the progression of renal osteodystrophy. Measuring pyridinoline or deoxypyridinoline in urine does not give an accurate bone status, due to insufficient urine in patients with renal failure. However, another biochemical marker, beta-CTX (the carboxy-terminal telopeptide of type 1 collagen), in serum is believed to be a good indicator of the status of renal osteodystrophy. METHODS: Fifty-nine patients undergoing hemodialysis agreed to have their blood and BMD checked. Beta-CTX was measured using an electro-chemiluminescence sandwich immunoassay and BMD was counted at the lumbar spine, femoral neck, and distal humerus using a Discovery-Wi (Hologic). RESULTS: Bone-alkaline phosphatase (49.8+/-36.7 U/L), parathormone (PTH) (192.8+/-263.3 U/L), osteocalcin (33.4+/-18.2 ng/mL), and beta-CTX (2.1+/-1.2 ng/mL) were all increased, while the average BMD of the lumbar spine (0.86+/-0.17), femoral neck, (0.67+/-0.14) and distal humerus (0.67 +/- 0.17) were all decreased. The BMD of the femoral neck in females was significantly lower than in males (p=0.044). The serum phosphate and PTH concentrations in non-diabetics were significantly higher than in diabetics (p=0.001, p=0.04, respectively). The measured serum osteocalcin and beta-CTX concentrations in patients older than 40 years were much lower than in patients younger than 40 (p=0.009, p=0.01, respectively). Beta-CTX was strongly correlated with bone-alkaline phosphatase (r=0.625, p=0.00), osteocalcin (r=0.698, p=0.00), and PTH (r=0.648, p=0.00). CONCLUSIONS: Beta-CTX is another convenient, significant marker for evaluating renal osteodystrophy.

Clinical Outcome of Paraquat Poisoning

No abstract available.

The Plan Suggestion for the Efficient Future Medical Care of Old Patients in the Community

In the future, geriatric care needs much more financial cost because korea is changing to an aging society rapidly and most of old people have health problems. It is important to have a comprehensive blueprint of the medical care of old patients, linking this with a welfare of old people. The government has to bring up and draw guidelines how to handle old patients and how much to treat them, before starting a national nursing insurance for old handicapped patients. Without a blueprint of old people care, we are in danger to encounter a huge financial leakage due to inefficient care system for old patients. Presently, old patient medical care are being randomly performed according to doctor's decision on a individual base. The multi-organ injury chronic diseased case tend to be rejected by doctors and will be referred to the higher level of medical center. Since old wealthy patient with a good economic and physical condition is a good target of making a money, this kind of old people will be welcomed and many lucrative cares will be applied under the name of anti-aging therapy. This consumptive environment must be changed to an efficient and economic system. The key content of a desirable system is a self-community of old-age people, which the lesser sick help the more sick and the lesser old patients help the more elderlies. The all around plan-building by the government is absolutely needed to provide a efficient medical treatment system for old patients with organizing the elderly as well as improving the regulations. Now, the government is ready to implement a national nursing insurance to solve the problems of the aging society. However, without a well prepared master plan for the future matters, we will meet other obstacles which could see in a tardy growing society.

A clinical study on the change of aluminium in a blood of the chronic renal failure patients undergoing hemodialysis / 대한내과학회지

BACKGROUND: More than 25 years have been passed since Korea started the hemodialysis. Initially, the technical problems of dialysis machine, a handling of azotemia, sustained anemia, as well as renal osteodystrophy were major matters we have to solve, however, recently the focused matters were changed that the removal of heavier molecular weighed uremic toxins, toxins like aluminium and silicon are very important, because these toxins are hardly removed and are very influential on the uremic signs. In this study, we planned to observe how much aluminium accumulated in chronic hemodialysis patients, and how much significant is increased aluminium blood level in patients. METHODS: We randomly selected fifty patients undergoing chronic hemodialysis to estimate the serum level of aluminium. We analyzed patients by using clinical informations, such as the time period of hemodialysis, the dialysis frequency, whether diabetes or not , according to the aluminium serum levels. The aluminium serum levels were estimated before and after the hemodialysis, which were measured by using the atomic absorption spectrophotometry. RESULTS: The serum levels of aluminium in CRF patients undergoing hemodialysis were significantly increased, as compared with normal range and much more increased levels was observed after hemodialysis 48.9+/-3.2 microgram/L than before 27.6+/-2.3 microgram/L (p<0.05) (Table 2). The aluminium level before hemodialysis of diabetic patients (40.3+/-17.6 microgram/L) showed I.57 times higher than non-diabetes (25.7+/-21.2 microgram/L), but the level after hemodialysis showed non-significant difference (Figure 1). The aluminium blood level after hemodialysis and the duration of hemodialysis were correlated positively (r=0.34, p<0.01), but this wasn't before hemodialysis (Figure 2). The serum level of aluminium tends upward following to increasing level of serum calcium, serum magnesium, as well as parathyroid hormone (Table 3). The delta aluminium(post-pre dialysis) level was significantly correlated positively (r=0.66, p<0.05) with delta Hb (post-pre dialysis) level (Figure 3). CONCLUSION: The serum level of aluminium in chronic renal failure patients undergoing hemodialysis were significantly increased and especially, more increased as soon as the hemodialysis was finished.

The Effect of Combination Treatment with Angiotensin Converting Enzyme Inhibitor & Angiotensin II Receptor Blocker on the Apoptosis in the Kidney and on the MAP of Spontaneously Hypertensive Rats / 대한신장학회잡지

BACKGROUND: Either of the angiotensin converting enzyme inhibitor (ACEI) and angiotensin II receptor blocker (ARB) has been known to be effective on the inhibition of progressive renal disease. The ACEI can cause angiotensin-II to be decreased, but make an plasma activity of renin and kinin to be increased. The ARB increase both activities of renin and angiotensin-II, while it block the function of angiotensin-II on the AT-1 receptor. This kind of process might influence on the activation of AT-2 receptor and lead to the activated state of antiproliferative change. Therefore, if the ARB and ACEI are treated at same time, that is, on the condition that angiotensin-II is somewhat suppressed and AT-1 receptor is blocked, this is strongly assumed to make the more positive effect on the inhibition of renal progression and hypertension control. METHODS: Experimental animals were chosen as a Spontaneously Hypertensive Rat (SHR) and a Wistar Kyoto Rat (WKR). Used total number were 32, including 11 (SHR 5, WKR 6) as a control. The other 21 SHR were allocated to pharmacy treated 3 groups (7 in each group as an ACEI treat, ARB treat, ACEI and ARB combination treat). The study on these animals was built by an observation on the BP, histopathologic change, the apoptosis & PCNA, the expression of AT-1 and AT-2 receptor. RESULTS: The mean arterial blood pressure (MAP) of SHR of a 5-month age was 153.2+/-3.4 mmHg, which was significantly high as compared to the control, 121.1+/-18.0 mmHg (p<0.01). The average of MAP in treated groups, 125.1+/-24.6 mmHg, was significantly decreased, as compared to untreated animals, 167.3+/-14.1 mmHg (p<0.01). The MAP in combination treatment animals is significantly lower than either of treatment group (p<0.05). The apoptotic process at the proximal tubule was observed less in case of treated animals, but the difference among treated 3 groups was not confirmed. CONCLUSION: The MAP decreasing effect by the combination treatment with ACEI and ARB was significant, however, the additive effect by this treatment on the inhibition of renal damage progression was not observed.

Effects in Combining Pharmacotherapy and Group Psychotherapy for Outpatients with Schizophrenia / 신경정신의학

OBJECTIVES: The objective of this study is to prove that combining pharmacotherapy and group psychotherapy is more effective way to prevent recurrence and rehospitalization, to promote their interpersonal relations and to improve their satisfaction on treatment by making a comparison with pharmacotherapy and individual psychotherapy (supportive therapy). METHODS: This study was performed from September 2000 to October 2001. The subjects were all schizophrenic outpatients who were treated by authors in the specialized mental hospital, the psychiatric department of a general hospital, and psychiatric clinics. The experimental groups and the control groups were composed in matching, sex, diagnosis, duration of illness, number of admission and function level. During this period, the experimental groups had been treated by pharmacotherapy and group psychotherapy, and the control groups by pharmacotherapy and individual supportive therapy. Numbers of admission (ratio of readmission) and changes of drug doses were traced, at the same time' group psychotherapy effectiveness scales' were applied. Just after each session, Yalom's 13 therapeutic factors scale was administered. RESULTS: During study periods, 3 cases (17%) among total 18 cases of the experimental groups were re-admitted and 6 cases (33%) among total 18 cases of the control group were re-admitted. During study periods, the doses of chlorpromazine in the experimental groups were decreased from 267 mg at start to 185 mg at closure and the doses of chlorpromazine in the control groups were increased from 264 mg at start to 265 mg at closure. The mean scores of insight, satisfaction on treatment, and satisfaction toward therapist were statistically high in the experimental groups in comparison with the control groups (<0.05). CONCLUSION: 'Combining pharmacotherapy and group psychotherapy' to outpatients with schizophrenia is very effective and expedient for therapists. We expect that this combined treatment model would be practiced more broadly as an economic treatment method which could minimize problems caused by practicing pharmacotherapy only and to give patients full service, though there are accompanied problems including the difficulties of structuring, the eack of motivation of patients, and the financial burden.

Effects of Interpersonal Group Therapy for Adolescents with Behavioral Problems / 신경정신의학

OBJECTIVES: Fast change and confusion of the value system in society affect the identity formation of adolescents. So Adolescents' behavioral problems seem to be increasing yearly. It is known that the interpersonal group therapy is the most effective treatment modality among many group programs for adolescents with behavioral problems. Objectives of this study are as follows; first, evaluate therapeutic effects of the interpersonal group therapy for adolescents with behavioral problems second, examine the therapeutic factors; third, analyze the group process; fourth, orgamize the group therapy for adolescents with behavioral problems as an applicable treatment modality. The types of group therapy performed in this study are small, closed, homogeneous, outpatient, time limited, and adolescent group. METHODS: This study was carried out at one of the adolescents mental health services in Uiwang Mental Health Center, from April to June and from September to November in 2000. The subjects were 2nd grade students in K middle school with behavioral proplems. The experimental groups were composed of 4 groups including 2 male groups and 2 female groups (male 11, female 17), and the control groups matched the same conditions as the experimental groups (male 10, female 19). The group therapy was performed weekly in CA (club activity) time, and it took 60-70 minutes at one time. They met total 10 sessions including preparatory meeting and termination meeting. Before and after this program, self-rating scales (behavioral problems, aggression, and impulsivity) were applied for subjects, and the teacher rating scales (behavioral problems, aggression and impulsivity) were performed by teachers in charge. After each session, Yalom's 13 therapeutic factors scale was performed. At the last meeting, total assessment questionnaire was completed. RESULTS: The mean scores of all self-rating scales (behavioral problem, aggression, and impulsivity) were lower in the experimental groups (both male and female group) than the control groups, but not significant statistically except the behavioral problem scale of female students. The mean scores of all teacher rating scales (behavioral problem, aggression, and impulsivity) were lower in the experimental groups (both male and female group) in comparison with the control groups, all significant statistically. As faras the therapeutic factors are concerned; first, the mean scores of all therapeutic factors in female students were higher than in male students and the mean scores of all therapeutic factors in the later half sessions were higher than the former half sessions; second, catharsis and existential factor were high in all students and sessions; third, identification with therapist factor was high in all students and sessions; fourth, during the later half sessions, interpersonal input was relatively high in male students and socializing technique was relatively high in female students. Its for the total assessment questionnaires; 100% of male students and 88.2% of female students reported that they were helped by this group therapy; 100% of male students and 82.4% of female students reported that they would participate willingly if future opportunity of the same group therapy is given for them. CONCLUSION: Adolescents with behavioral problems who participated in this study showed the decrease of behavioral problems and the change of aggressive and impulsive attitudes in comparison with the control groups although they had some differences between male and female students. The group therapy with adolescents would be practiced more broadly and extensively though there are several accompanied problems including the difficulty of structuring, the deficit of motivation, and financial problem.

A Case of Acute Infectious Purpura Fulminans / 대한피부과학회지

Purpura fulminans(PF) is a devastating disorder characterized by rapidly progressing hemorrhagic necrosis of the skin, circulatory collapse, and disseminated intravascular coagulation. Histopathologically skin lesions show thrombi within the dermal vessels, a sparse perivascular mononuclear infiltrate, and epidermal necrosis. PF can be classified into three distinct categories: acute infectious PF (sepsis associated PF), hemostasis induced PF, and idiopathic PF. We describe a case of acute infectious PF occurring in 65-year old female with pneumococcal sepsis.

Quantitative Determination of Plasma and Urine Paraquat Concentrations using Spectroscopic Method / 대한진단검사의학회지

BACKGROUND: It is well known that plasma paraquat concentration is one of the most important prognostic indicators for paraquat poisoning. Quantitative analyses of paraquat, however, are not generally used in clinical laboratories. In this work, we evaluated the second-derivative spectroscop-ic method for quantitation of paraquat in plasma and urine, and investigated the clinical significance in patients with paraquat poisoning. METHODS: Linearity, precision, interferences, and comparison with high-performance liquid chro-matography (HPLC) were evaluated in 20 paraquat-poisoning cases using the UV-160 A recording spectrophotometer. The relationship of plasma and urine paraquat concentrations with the clinical outcomes was also studied. RESULTS: The within-run and between-day coefficients of variation (CV) for groups of low and high levels were less than 5%. The derivative amplitude was linearly related to paraquat concentra-tion through the range from 0.5 to 10 ng/mL. The correlation coefficient (r) between spectrophotom-etry and HPLC was 0.992. The accuracy for predicting the outcome for patients based on plasma paraquat concentration was 84.6%. The urine paraquat levels on admission were more than 10 ng/ mL in all of the 9 non-survivors group and in 5 out of 11 of the survivors group. The eliminating rates for plasma and urine paraquat concentrations by extracorporeal procedures were not statistically different between the two groups. CONCLUSIONS: Second-derivative spectroscopic methods for quantitation of paraquat showed an acceptable performance and suitable procedure for clinical laboratory use and it was thought to be seful in assessing the severity and in predicting the prognosis for paraquat poisoning.

Hemoperfusion and continuous hemodiafiltration in a paraquat poisoned patient / 대한내과학회지

Extracorporeal elimination techniques have been frequently used in paraquat poisoning. But the effect of these techniques is controversial. Plasma paraquat concentration falls rapidly during first few hours after ingestion as the compound is taken by the tissues and excreted by the kidney. Tissue binding is very strong, resulting in slow release of substance from the tissues into the plasma. Thus, once the substance has reached its body stores, it is very difficult to eliminate. For these reasons, any effective extracorporeal elimination therapy should be initiated as early as possible, while paraquat concentrations are high and thereafter, continuous elimination therapy should be maintained to keep plasma paraquat levels as low as possible and remove the toxin as it enters the circulation from tissue stores. We present a case of paraquat poisoning treated by hemoperfusion and hemodiafiltration on this theoretical basis.

Acute renal failure due to bilateral pyelonephritis / 대한내과학회지

Acute renal failure is a rare complication of acute pyelonephritis. Therefore, acute pyelonephritis is not usually considered in the differential diagnosis of acute renal failure. However, it is important to consider this entity because of potential for recovery of renal function if appropriate early antibiotics are instituted. We report a case of biopsy proven acute pyelonephritis which was manifested as acute renal failure. A 38 year old women was admitted to this hospital owing to abdominal distension and generalized edema. On admission she was started on hemodialysis because of severe hyperkalemia and marked uremic sypmtoms. She had pyuria and hematuria, but no organism was isolated at urine. We initially don't know the cause of renal failure. She was improved with antimicrobial therapy and hemodialysis. A kidney biopsy was performed on the 26th hospital day because of persistent proteinuria. Microscopic examination revealed focal tubular atrophy, necrosis or loss with heavy infilteration of leukocytes and histocytes in interstitium. Atrophic tubules contain pus casts. The patient was treated with ciprofloxacin for 4 weeks. At about 2 month follow up, proteinuria completely disappeared and serum creatinine level decreased to 1.0 mg/dL.

How is the Clinical Efficacy of Extracorporeal Extraction Treatment Especially, Focused on the Hemodiafiltration, for Removal of Paraquat? / 대한신장학회잡지

PURPOSE: We tried to estimate the clinical efficacy of hemodiafiltration in the paraquat poisoning, as compared with that of other various extracorporeal extraction treatment. METHODS: We prepared the fresh frozen plasma mixed with paraquat concentrated up to 30 mg/L. The experiment was designed to remove paraquat by use of various extracorporeal treatment, such as hemodialysis (HD), hemoperfusion (HP), hemofiltration (HF), hemodia-filtration (HDF), hemodiafiltration & hemoperfusion (HDFP), respectively. The efficacy was analyzed on the basis of counting extraction ratio, observing the decreasing concentration of paraquat with the lapse of time. Four pigs (Yorkshire) were prepared and poisoned by paraquat (40 mg/kg) intramuscularly. Two poisoned animals were assigned for hemoperfusion and hemodiafiltration respectively and other two were assigned for the control. We observed extraction ratio, decreasing concentration of paraquat in blood, remained amount of paraquat in major organs, in addition to pathologic change of major organs after sacrifying the animals. RESULTS: The mean of extraction ratio is 0.84+/-0.27 in case of HP, 0.81+/-0.21 in HD, 0.74+/-0.40 in HDFP, 0.53+/-0.24 in HDF, 0.5+/-0.14 in HF. The extraction ratio of HP & HD & HDFP was significantly higher than that of HDF & HF (p<0.01). The extraction ratio was counted as the difference between the paraquat concentration of inlet and outlet was divided by the concentration of inlet. The slope of paraquat concentration undergoing extracorporeal treatment was the most acutely decreased in the case of HDFP, the less decreased in HP, and sequentially in HD, HDF and HF (the least) in the order of the decrease. The more decreased paraquat concentration in plasma was observed, the higher flow rate of dialysate of HDF was conducted. The mean of extraction ratio in animal study was 0.61 in HP and 0.36 in HDF. The blood concentration of paraquat was observed to be much lower in case of HP & HDF, as compared with the control animals. The remained concentration of paraquat in major organs, 7 hours later after being poisoned, was observed to be higher in the vascular structure like kidney and heart. However, it was observed to be lower in kidney, lung & muscle, when either of HDF and HP was conducted, than control. Especially, it was much lower in HP and much less pathologic change in HP. HDF is the less effective measure than HP, but is effective as a continuous treatment to make paraquat concentration to be lower as much as it possible. CONCLUSION: The HDF is the effective measure to keep the blood paraquat level low, even though it is behind the HP in effectiveness.

How is the Clinical Efficacy of Extracorporeal Extraction Treatment Especially, Focused on the Hemodiafiltration, for Removal of Paraquat? / 대한신장학회잡지

PURPOSE: We tried to estimate the clinical efficacy of hemodiafiltration in the paraquat poisoning, as compared with that of other various extracorporeal extraction treatment. METHODS: We prepared the fresh frozen plasma mixed with paraquat concentrated up to 30 mg/L. The experiment was designed to remove paraquat by use of various extracorporeal treatment, such as hemodialysis (HD), hemoperfusion (HP), hemofiltration (HF), hemodia-filtration (HDF), hemodiafiltration & hemoperfusion (HDFP), respectively. The efficacy was analyzed on the basis of counting extraction ratio, observing the decreasing concentration of paraquat with the lapse of time. Four pigs (Yorkshire) were prepared and poisoned by paraquat (40 mg/kg) intramuscularly. Two poisoned animals were assigned for hemoperfusion and hemodiafiltration respectively and other two were assigned for the control. We observed extraction ratio, decreasing concentration of paraquat in blood, remained amount of paraquat in major organs, in addition to pathologic change of major organs after sacrifying the animals. RESULTS: The mean of extraction ratio is 0.84+/-0.27 in case of HP, 0.81+/-0.21 in HD, 0.74+/-0.40 in HDFP, 0.53+/-0.24 in HDF, 0.5+/-0.14 in HF. The extraction ratio of HP & HD & HDFP was significantly higher than that of HDF & HF (p<0.01). The extraction ratio was counted as the difference between the paraquat concentration of inlet and outlet was divided by the concentration of inlet. The slope of paraquat concentration undergoing extracorporeal treatment was the most acutely decreased in the case of HDFP, the less decreased in HP, and sequentially in HD, HDF and HF (the least) in the order of the decrease. The more decreased paraquat concentration in plasma was observed, the higher flow rate of dialysate of HDF was conducted. The mean of extraction ratio in animal study was 0.61 in HP and 0.36 in HDF. The blood concentration of paraquat was observed to be much lower in case of HP & HDF, as compared with the control animals. The remained concentration of paraquat in major organs, 7 hours later after being poisoned, was observed to be higher in the vascular structure like kidney and heart. However, it was observed to be lower in kidney, lung & muscle, when either of HDF and HP was conducted, than control. Especially, it was much lower in HP and much less pathologic change in HP. HDF is the less effective measure than HP, but is effective as a continuous treatment to make paraquat concentration to be lower as much as it possible. CONCLUSION: The HDF is the effective measure to keep the blood paraquat level low, even though it is behind the HP in effectiveness.

Sclerotherapy of Renal Cysts in Korean ADPKD Patients / 대한신장학회잡지

BACKGROUND: Autosomal dominant polycystic kidney disease(ADPKD) is the most common hereditary renal disease in adults, and its major complaints include pain and abdominal fullness due to cyst expansion. So far, for the control of these symptoms, cyst ablation with ethanol or tetracycline, laparoscopic manipulations and surgical marsupialization have been used. METHODS: We used conventional ethanol(n=9) or n-butyl cyanoacrylate(NBCA) plus lipiodol solution (n=18) or both(n=3) for separate cysts as the sclerosing agent in 24 adult Korean ADPKD patients. And their clinical courses after treatment were evaluated. RESULTS: The male to female ratio was 8 : 16 and the mean age at the treatment was 50 yrs(S.D. 13.1). Causes for aspiration were pain in 14 and abdominal fullness in 7 patients and the range for the cyst diameters aspirated were 5-16 cm. Flank pain or discomfort were decreased subjectively in most cases except two. Mean arterial pressures(S.D.) (mmHg) before and after procedure were as follows 112(11.1)(basal), 96(9.6)(1 month) and 98(9.7)(6 month)(p < 0.05, paired-t test). Blood urea nitrogen levels(mg/dL) were not changed 6 month later[24 (12.1) vs. 22(14.6)]. There was no major complication such as bleeding or infection and no death and associated with procedure. There was no difference of therapeutic effect according to sclerosing agent. CONCLUSION: NBCA was as effective as conventional ethanol for sclerotherapy in ADPKD and cyst ablation therapy showed a BP-lowering effect in short-term period.

Cytokine Profile in the Aspirated Cystic Fluids in Autosomal Dominant Polycystic Kidney Disease (ADPKD) Patients / 대한신장학회잡지

BACKGROUND: The pathogenesis of ADPKD is still unknown but the proliferation of cystic epithelia and the fluid secretion to cystic lumen are thought to be important. Cytokines play a pivotal role in growth, differentiation, and apoptosis in general, but there were few reports about the cytokine profile in ADPKD cysts. METHODS: In this study, we measured cytokine content in aerobic culture-negative cystic fluids from 23 patients with symptomatic normal to end-stage (n=3) ADPKD in order to elucidate the possibility that cytokines are related to the development and progression of disease. Enzyme-linked immunosorbent assays (ELISAs) were used to detect IL-1beta, IL-2, IL-4, IL-6, IL-10, and IFN-gamma with commercial kits. RESULTS: Male to female ratio was 6 : 17 and the median age at examination was 52 years (range 36 to 78). IL-1beta was present in 18 of 23[78%] (11 to 173 pg/mL), IL-2 in 18 of 23[78%] (5 to 159 pg/ mL), IL-4 in 9 0f 23[39%] (8 to 156 pg/mL) and IL-6 in 10 of 23[43%] (16 to 1498 pg/mL). IL-10, and IFN-gamma were not detected. IL-1beta concentrations correlated directly with those of IL-2 (r=0.7671). IL- 6 levels in patients with azotemia (n=7) [288.4+/-26.2 (mean+/-S.D.)] were significantly higher than those of normal renal function group (98.3+/-413.9)(p<0.01). Such difference was not found in other cytokines. Cytokine concentrations did not correlate with sodium concentrations, nor with cystic fluid osmolality, indicating that differences in concentrations among fluids could not be explained by differences in water content. And, there was no significant correlation between the intracystic concentrations of these cytokines and the corresponding cyst diameters. CONCLUSION: These data identify proinflammatory cytokines as possible mediators to the morbidity of ADPKD. Especially, IL-6 levels of cystic fluid were elevated in the azotemic ADPKD patients.