Central diabetes insipidus (CDI) is a clinical syndrome that result from a failure of the neurohypophyseal axis to produce or release a sufficient quantity of arginine vasopressin (AVP) to permit normal function of the urinary concentrating mechanism. Polyuria and polydipsia are the symptoms associated with CDI. The most common cause of CDI is idiopathic variety and head trauma, neurohypophyseal surgery, primary or metastatic brain tumors acount for most of the remaining cases. CDI in Langerhans cell histiocytosis (LCH) is thought to be to infiltration of the hypothalamus-neurohypophyseal system. We report a patient with CDI and LCH underwent water depriviation test, MR imaging of the pituitary-hypothalamic region, and VATS associated open lung biopsy.
Arginine Vasopressin ; Axis, Cervical Vertebra ; Biopsy ; Brain Neoplasms ; Craniocerebral Trauma ; Diabetes Insipidus* ; Diabetes Insipidus, Neurogenic ; Histiocytosis* ; Histiocytosis, Langerhans-Cell ; Humans ; Lung ; Magnetic Resonance Imaging ; Polydipsia ; Polyuria ; Thoracic Surgery, Video-Assisted

PURPOSE: Since the infant formula milk has been produced in Korea, it has faced a low rate of breast milk feeding, though breast milk feeding is a little increasing. Therefore, the Korean Pediatric Society launched its website for breast-feeding consultation to provide information to the general public and enhance the health of growing infants. The consultation results were analyzed to identify the problems that mothers encounter during breast-feeding. METHODS: From August 1, 2004 to July 31, 2007, 1001 mothers who visited the online consultation webpage (www. pediatrics.or.kr) of the Korean Pediatric Society asked 1,021 questions. The questions were divided into 3 major categories and 14 specific categories. Interesting questions asked more than 100 times were retrospectively analyzed. RESULTS: The results for the major categories were as follows: 413 questions (40.3%) were on how to breast-feed, 315 (30.8%) on problems of feeding mothers, and 293 (28.8%) on problems of the fed babies. In the specific categories, 22.2% of the questions were on how to breast-feed. With the increasing number of working couples and working mothers, many questions were asked on the problems of breast-feeding after returning from work. CONCLUSION: The author expects that analyses of these consultations will contribute to the enhancement of information on the consultation website, thus enabling to provide clearer answers to people's increased interest in and concerns on breast-feeding. Furthermore, this research will help to establish correct breast-feeding practice.
Family Characteristics ; Humans ; Infant ; Infant Formula ; Korea ; Milk ; Milk, Human ; Mothers ; Referral and Consultation ; Retrospective Studies

Vasculitis is a clinico-pathological process characterized by inflammation and necrosis of blood vessels. The clinical spectrum ranges from a primary disease process restricted to blood vessels in the absence of an underlying disease to a secondary component of the primary disease. We experienced a case of polyarteritis nodosa who complained of progressive pain and weakness in back and lower extremities. Polyarteritis nodosa should be considered as one of the causes of pain and weakness in back and extremities.
Blood Vessels ; Electrodiagnosis ; Extremities ; Inflammation ; Lower Extremity* ; Necrosis ; Polyarteritis Nodosa* ; Polyneuropathies ; Vasculitis

Although hemodialysis using heparin bound Hemophan(HBH-HD) has been reported to be a possible modality that can be used in patients at high risk of bleeding, the efficiency of HBH-HD is not certain. To investigate the efficiency of HBH- HD, we compared the total blood compartment volume(TBCV), Kt/V and urea clearance of dialyzer(K) of HBH-HD with those of routine hemodialysis with systemic heparinization(R-HD) in the same patients. HBH-HD was switched to R-HD as soon as the bleeding risk had ceased. Before each HBH-HD, heparin solution(1liter, 20IU/ml saline) was recirculated through the Hemophan(Gambro dialyzer, GFS Plus 11) for 1 hour while removing saline solution(700ml/hr) by applying transmembrane pressure gradient, followed by a single pass rinse with 1 liter of saline solution. Then we performed 10 HBH-HD on 10 patients at risk of bleeding. The dilayzer had to be changed due to severe clotting in one patient during HBH-HD so the comparison of above parameters was possible in 9 patients. The duration of each dialysis was possible in 9 patients. The duration of each dialysis was standardized to 4 hours at blood flow of 200 to 250ml/min. During HBH-HD, there was a slight increase in activated partial thromboplastin time(aPTT)(45.02.6 sec) at 15 min after initiation of dialysis from predialysis level (35.81.3 sec), but no increase in aPTT was observed at 60min, 120min, and the end of dialyses. The loss of TBCV(%) of dialyzers was greater in HBH-HD (174%) than in R-HD(51%). The Kt/V and K of HBH-HD, however, were 1.25+/-0.10 and 143+/-3ml/ min, respectively, which did not differ from those of R-HD which were 1.28+/-0.07 and 145+/-4ml/min, respectively. We conclude that the use of heparin bound Hemophan can be an efficient hemodialysis technique in patients at high risk of bleeding, but clotting of the dialyzer should be observed carefully during hemodialysis(values are mean+/-SE).
Dialysis* ; Hemorrhage ; Heparin* ; Humans ; Renal Dialysis* ; Sodium Chloride ; Thromboplastin ; Urea

Hyperhomocysteinemia, an independent risk factor of vascular disease, is common in patients with chronic renal failure(CRF) patients including dialysis patients. We measured fasting plasma concentrations of total homocysteine(tHcy) by high-performance liquid chromatography in 114 chronic renal patients and 37 healthy controls. The CRF patients were divided into four groups : chronic renal failure with serum creatinine >1.4mg/dl and creatinine clearance >10ml/min(CRF group, n=27), non-dialyzed ESRD patients with creatinine clearance <10ml/min(ESRD group, n=38), patients on maintenance hemodialysis(HD group, n=20) and patients on continuous ambulatory peritoneal dialysis(PD group, n=29). Mean(+/-SD) tHcy in each of CRF(14.2+/-5.6micromol/L), ESRD(21.6+/-14.1micromol/L), HD(21.0+/-9.2micromol/L) and PD(17.2+/-7.7micromol/L) group was significantly higher than that in controls(9.0+/-3.1micromol/L, P=0.001). In 87 ESRD, HD and PD patients, mean(SD) tHcy in 45 patients who received routine folate supplementation (1mg/day) was lower(17.5+/-8.3micromol/L) than that in 42 patients without supplementation(22.6+/-13.4micromol/ L, P=0.03), but was higher than that in controls (9.13.1micromol/L, P=0.001). In conclusion, hyperhomocysteinemia was present in patients with varying degree of chronic renal failure and increased in parallel with progression or renal failure.
Chromatography, Liquid ; Creatinine ; Dialysis ; Fasting ; Folic Acid ; Homocysteine* ; Humans ; Hyperhomocysteinemia ; Kidney Failure, Chronic* ; Plasma* ; Renal Insufficiency ; Risk Factors ; Vascular Diseases

Many patients with Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome(TTP-HUS) satisfactorily respond to plasma exchange. Some patients, however, respond either not at all or only transiently and incompletely. In the refractory case, endothelial cell-derived unusually large von Willebrand factor multimers(ULvWFM) have an important role in the formation of microthrombi. As the ULvWFM may be removed in the cryoprecipitate, we reason the plasma depleted of cryoprecipitate(the plasma cryosupernatant) should be considered for effectiveness in the treatment of refractory TTP- HUS. We experienced a 48 year old woman presented with diarrhea, jaundice and oliguria. She had microangiopathic hemolytic anemia, renal impairment, platelets of 21,000/mm3 and LDH 3,258U/L. She had not improved after plasma exchange with fresh frozen plasma(FFP)(1.5 plasma volumeX7 days). On hospital day 8, her HUS had not responded, platelets of 37,000/mm3 and LDH 1,588U/L. Substitution of cryosupernatant for FFP was associated with prompt increased in the platelet count to normal and complete resolution of HUS. Therefore. the cryosupernatant fraction of plasma should be considered as an alternative to whole FFP for plasma exchange if there is continuing platelet consumption and microvascular thrombosis in spite of intensive conventional plasma therapy.
Anemia, Hemolytic ; Blood Platelets ; Diarrhea ; Female ; Hemolytic-Uremic Syndrome* ; Humans ; Jaundice ; Middle Aged ; Oliguria ; Plasma Exchange* ; Plasma* ; Platelet Count ; Thrombosis ; von Willebrand Factor

A 17-year-old man with psoriasis developed albuminuria and microscopic hematuria. Renal biopsy revealed a glomerulonephritis with features of both membranous glomerulonephritis and IgA nephropathy. Histologically the glomeruli exhibited variable degree of mesangial expansion and hypercellularity, three of which showed segmental hyalinosis and/or sclerosis. Direct immunofluorescence demonstrated granular IgG-bearing deposits along the peripheral glomerular capillaries and IgA deposits in the mesangium. His urinary abnormalities persisted after the remission of skin lesion induced by PUVA treatment. It suggests that although the psoriasis may induce the renal lesion, it is insufficient to treat only the skin lesion for clinical improvement of glomerulonephritis after the renal lesion is already established.
Adolescent ; Albuminuria ; Biopsy ; Capillaries ; Fluorescent Antibody Technique, Direct ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Glomerulonephritis, Membranous* ; Glomerulosclerosis, Focal Segmental ; Hematuria ; Humans ; Immunoglobulin A* ; Psoriasis* ; Sclerosis ; Skin

Systemic anticoagulation in routine hemodialysis is not desirable in patients with high risk of bleeding. Since heparin can bind to Hemophan, we evaluated the risk of bleeding and efficiency of hemodialysis using heparin bound Hemophan membranes in patients with high risk of bleeding. Heparin solution (1liter, 20IU/ml saline) was recirculated through the Hemophan(Gambro dialyzer, GFS Plus 11) for 1hour while removing saline solution(700ml/hr) by application of transmembrane pressure gradient, followed by a single pass rinse with 1 liter of saline solution. As a pilot study, we performed 17 hemodialyses on 15 chronic dialysis patients with contraindication to systemic anticoagulation. The duration of each dialysis was standardized to 4 hours at blood flow of 200 to 250ml/min. Blood samples were obtained to measure activated partial thromboplastin time (aPTT), and heparin concentrations (HC) before dialysis, at 15min, 60min, 120min after initiation of dialysis and at the end (240min) of dialysis. Dialysis efficiency was assessed by measuring Kt/V and urea clearance of dialyzer (K) by the direct quantification of dialysate urea and then compared with the 25 control dialyses with systemic anticoagulation. We successfully completed all 17 hemodialyses without severe clotting defined as, requiring replacement of the dialyzer and/or the extracorporeal blood lines. There was a slight increase in the aPTT (mean+/-SD, 42.9+/-4.4sec) and HC (0.15+/-0.03IU/ml) taken at 15min from predialysis levels of 36.3+/-6.3sec and 0.11+/-0.03U/ml, respectively. But no increase in aPTT, HC was observed in measurements taken at 60min, 120min, and at the end of dialyses. The value (mean+/-SD) of Kt/V and K was 1.27+/-0.25 and 134+/-19ml/min respectively, which did not differ from those of the control dialyses which was 1.24+/-0.21 and 136+/-13ml/min respectively. We performed 82 hemodialyses using such treated Hemophan on 27 patients for 4 hours basing the result of the pilot study. Clotting of dialyzer necessitating termination of dialysis occurred in 1 dialysis(1.2%) at 150min and clotting in the venous blood lines requiring change of blood lines occurred in 6 dialyses(7.3%) on 4 patients from 180min to 230min after initiation of dialysis. We conclude that the use of heparin bound Hemophan can be a safe and effective technique of hemodialysis with careful monitoring of extracorporeal clotting in patients with high risk of bleeding.
Dialysis* ; Hemorrhage* ; Heparin* ; Humans ; Membranes ; Partial Thromboplastin Time ; Pilot Projects ; Renal Dialysis* ; Sodium Chloride ; Urea

Asia ; Asian Continental Ancestry Group ; Education ; Humans ; International Cooperation ; Oceania ; Seoul

OBJECTIVE: Positively charged N, N-diethyl-aminoehtyl groups on Hemophan enable negative charged heparin to be bound with the dialyzer membrane and hemodialysis using heparin bound Hemophan (HBH- HD) could be a hemodialysis modality in patients at risk of bleeding. We designed simplified heparin binding technique and evaluated the bleeding risk and efficiency of HBH-HD in chronic renal failure patients at risk of bleeding. METHODS: During the period from April 1995 through April 2002, 159 patients at high bleeding risk received 1057 HBH-HD (dialyzer: GFS plus 11, Gambro). The duration of each HBH-HD was standardized to 4 hours at blood-flow rate of 200-250 mL/min. To evaluate safety of HBH-HD, we measured serum heparin concentration (HC) and activated partial thromboplastin time (aPTT) at baseline, 15, 60, 120 minutes and endpoint (240 minutes) (n= 40). To evaluate the dialysis efficiency, HBH-HD and routine hemodialysis with systemic heparinization (R-HD) were compared for total blood compartment volume (TBCV) loss, dialyzer urea clearance (K) and Kt/V in same study group patients (n=20). RESULTS: Clotting of dialyzer necessitating termination of dialysis occurred in 11 (1.0%) out of 1, 057 dialyses at 150 minutes, and clotting requiring change of blood line occurred in 64 dialyses (6.1%) between 150 and 230 minutes. There was a slight increase in the aPTT (mean+/-SD, 49.8+/-10.5 sec) and HC (0.14+/-0.06 U/mL) at 15 min, compared to predialysis levels of 44.3+/-12.9 sec and 0.11+/-0.06 U/ mL, respectively (p>0.05). But no increase in aPTT, HC was observed in measurements at 60 min, 120 min, and at the endpoint. TBCV loss was significantly higher in HBH-HD (mean+/-SD, 17.2+/-9.6%), compared to R-HD (2.8+/-1.2%) (p< 0.0001). However, K and Kt/V value (mean+/-SD) were 136.9+/-14.6 mL/ min and 1.27+/-0.21 in HBH-HD and 137.6+/-18.4 mL/ min and 1.20+/-0.22 in R-HD, showing no significant difference (p>0.05). CONCLUSION: HBH-HD could be a safe and efficient HD technique in patients at high risk of bleeding. Extracorporeal clotting, however, should be observed carefully during HBH-HD.
Dialysis ; Hemorrhage* ; Heparin* ; Humans ; Kidney Failure, Chronic* ; Membranes ; Partial Thromboplastin Time ; Renal Dialysis* ; Urea