Hereditary sensory motor neuropathy type V (HAMN V) is very rare disease entity. The authors experienced a patient who had the findings of peroneal muscular atrophy and pyramidal tract feature. A 20 year-old man was admitted to our hospital due to gait disturbance. Scissoring gait and peroneal muscular atrophy were observed. The findings of electrophysiologic studies were compatible with axonal type peripheral neuropathy. The results of other evaluations were compatible with HAMN V. To our knowledge, it is the first report in Korea.
Axons ; Charcot-Marie-Tooth Disease ; Gait ; Humans ; Korea ; Peripheral Nervous System Diseases ; Pyramidal Tracts ; Rare Diseases

No abstract available.
Wernicke Encephalopathy*

No abstract available.
Anti-Bacterial Agents* ; Phagocytosis*

No abstract available.
Humans ; Hypophosphatemia*

Partial outlet obstruction induced by benign prostatic hypertrophy or urethral stricture are common urologic conditions showing voiding dysfunction. This study was performed to compare the effects of short-term partial outlet obstruction on the contractile responses to different stimuli and to elucidate the mechanism of altered contractile response during the development of significant voiding dysfunction. Sprague-Dawley rats, 10 week old, were subjected to partial outlet obstruction for 7 days. Contractile responses of the urinary bladder detrusor muscle strips to electrical field stimulation (1, 2, 5, 10 and 20 Hz), KCl (50 and 100 mM) and carbachol (10(-9 to 5) M) were measured. Total protein and DNA contents of the bladder were measured. Connexin 43 mRNA expression was measured using RT-PCR and connexin 43 protein was observed in the fixed bladder tissue using immunohistochemistry. The electrical field stimulation-induced contractile response and KCl-induced contraction was not changed after partial outlet obstruction. Contraction induced by carbachol was enhanced by the partial outlet obstruction. Total protein and DNA contents were increased in the partial outlet obstruction group. Connexin 43 mRNA and protein expression were detectable in the normal bladders and increased after 7 days of obstruction. These results suggest that the altered contractile responses during the early stage of the partial outlet obstruction are the result of the changes of the contraction mechanisms or structure of bladder muscle. Connexin 43 may play an important role in those alterations.
Animals ; Carbachol ; Connexin 43 ; DNA ; Immunohistochemistry ; Prostatic Hyperplasia ; Rats* ; Rats, Sprague-Dawley ; RNA, Messenger ; Urethral Stricture ; Urinary Bladder*

No abstract available.
Metacarpophalangeal Joint* ; Palmar Plate*

We retrospectively evaluated 47 patients with decompression sickness (DCS) seen at the Kang Nam General Hospital between January 1987 and June 1989 to describe the characteristcs of neurological manifestations. In 25 patients (53.2%) girdle paresthesia or bandlike tightness was noted just before the onset of neurological symptoms or signs. Progressive limb weakness, urinary retention, alterations in mentality, severe paresthesia were main presenting features in order of decreasing frequency. In the spinal from of DCS lower thoracic segment was most commonly involved. On the contrary to the previous pathological reports posterior columns were relatively spared in view of clinical points. Of 7 patients tested with Somatosensory Evoked Potetial (SSEP), five showed abnormal fidings. Myoclonic jerks of lower limb were the most common late sequelae.
Decompression Sickness* ; Decompression* ; Extremities ; Hospitals, General ; Humans ; Lower Extremity ; Myoclonus ; Neurologic Manifestations ; Paresthesia ; Retrospective Studies ; Urinary Retention

OBJECTIVES: Any form of dialysis must provide a sufficient replenishment of buffer. Lactate with a con- centration of 35 to 40mM/L is now the only buffer routinely used in CAPD. Buffer balance using lactate solutions will be governed essentially by the balance between the H+ generated, the amount of bicarbonate lost into the peritoneal effluent, and the lactate absorbed from the dialysate. Factors affecting buffer balance in CAPD patients will therefore include dietary protein intake which determine H+ generation and ultrafiltration which can affect bicarbonate loss. Although several studies reported a different prevalence of metabolic acidosis in their CAPD patients, it is uncertain whether the same prevalence of metabolic acidosis(MA) or the similar characteristics of acid-base status of Western CAPD patients can be found in Asian patients with smaller body size and relatively less amount of dietary animal protein intake. This study was undertaken to know the current acid-base status of CAPD patients in Korea with the exact information about buffer balance. We will also investigate the factors affecting acid-base homeostasis in CAPD patients such as dietary protein intake, the individual membrane characteristics and the various indices of dialysis adequacy and nutritional status of patients. METHODS: The acid-base status of Korean CAPD patients was assessed based on arterial blood gas analysis along with various nutritional parameters including subjective global assessment(SGA), anthropometric measurements and standard peritoneal equilibration test in 198 clinically stable patients main- tained on CAPD for more than 6 months using 35- 40mM/L of lactate-based dialysate(M:F 106:92, mean age 47.9 years, mean duration 45.3 months). RESULTS: Mean arterial bicarbonate concentration was 24.6+/-3.4mM/L with a range of 16.2-36.7mM/L and mean dialytic base gain was 29.4+/-15.2mM/day. Only 28(14.1%) patients showed MA while 52(26.3%) patients had a various degree of metabolic alkalosis. Normal acid-base status was observed in 75 patients(37.9%). The rest showed mixed acid-base abnormalities of respiratory origin. Patients with MA (mean HCO3 19.5+/-1.9mM/L) showed significantly higher serum albumin(4.01+/-0.38 vs. 3.59+/-0.46 g/dl, P<0.001) & protein equivalent of nitrogen appearance (NPNA, 1.2+/-0.1 vs. 0.9+/-0.2 g/kg/day, P<0.05) compared to the metabolic alkalosis group(mean HCO3 28.1+/-1.9mM/L). Dialytic protein loss(6.39+/-1.86 vs. 7.64+/-2.71 g/day, P<0.01) and D/PCr(0.60+/-0.09 vs. 0.71+/-0.13, P<0.05) were significantly lower in MA group. There were no significant differences in residual renal function, KT/VUrea, number of malnourished patients by SGA and other anthropometric parameters according to the acid-base status of patients. There were significant inverse correlations of arterial HCO3 with serum albumin and NPNA while HCO3 was positively correlated with duration of dialysis, ultrafiltration volume, dialytic lactate gain and dialytic protein loss. CONCLUSION: Our results on the acid-base status of Korean CAPD patients including a generally lower incidence of MA appear to be quite different from other reports based on the Western population. The peritoneal membrane transport characteristics can be one of the important factors determining the acid- base status of peritoneal dialysis patients. Duration of dialysis and protein catabolic rate also influence arterial bicarbonate level independently.
Acid-Base Equilibrium* ; Acidosis* ; Alkalosis ; Animals ; Asian Continental Ancestry Group ; Blood Gas Analysis ; Body Size ; Dialysis ; Dietary Proteins ; Homeostasis ; Humans ; Incidence* ; Korea ; Lactic Acid ; Membranes ; Nitrogen ; Nutritional Status ; Peritoneal Dialysis ; Peritoneal Dialysis, Continuous Ambulatory* ; Prevalence ; Serum Albumin ; Ultrafiltration

OBJECTIVE: to investigate the effect of high glucose and spent peritoneal dialysate on the TGF-beta1 synthesis of cultured human peritoneal MC(HPMC); to examine the effect of costimulation with high glucose or dialysate and cytokines, interleukin-1beta(IL-1beta) and tumor necrosis factor-alpha(TNF-alpha), on transforming growth factor(TGF-beta1) synthesis of HPMC. DISIGN: HPMCs were exposed to different concentrations of glucose(30, 60 & 90 mM/L) or spent peritoneal dialysate for 48 hours in the absence or presence of IL-1beta(1ng/ml) and TNF-alpha(1ng/ml). TGF-beta1 mRNA expression was assessed by Northern blot analysis and TGF-beta1 protein synthesis and release by Western blot analysis with immunoprecipitation. RESULTS: Exposure of MC to high glucose condition(30mM, 60mM & 90mM of D- glucose) induced 2.3-, 3.6- and 4.0-fold increases in TGF-beta1 mRNA expression of MC with enhanced TGF-beta1 protein synthesis and secretion into the media. Incubation with spent dialysate also significantly increased TGF-beta1 mRNA expression & protein secretion compared to control media(P<0.05) Stimulation with IL-1beta(1ng/ml) or TNF-alpha(1ng/ml) significantly increased TGF-beta1 mRNA expression after 48 hours above the control level by 2.7-fold and 2.1-fold, respectively. However, TNF-alpha-induced increase in TGF-beta1 mRNA expression was not translated into TGF-beta1 protein secretion whereas IL-1beta stimulation induced a significant increase in TGF-beta1 protein secretion as well as TGF-beta1 mRNA expression. Combined stimulation of high glucose or spent dialysate together with IL-1beta or TNF-alpha showed a greater increase in TGF-beta1 mRNA expression and protein secretion compared to stimulation with high glucose or spent dialysate alone. CONCLUSION: Our results clearly show that high glucose concentration of peritoneal dialysate and spent dialysate themselves might be sufficient to stimulate the production of TGF-beta1 by peritoneal mesothelial cell. This state of chronic induction of TGF-beta1 is further exaggerated in the presence of peritonitis because of stimulatory effect of proinflammatory cytokines, resulting in the augmented TGF-beta1 synthesis, thus promoting peritoneal fibrosis.
Blotting, Northern ; Blotting, Western ; Cytokines ; Glucose ; Humans* ; Immunoprecipitation ; Necrosis ; Peritoneal Fibrosis ; Peritonitis ; RNA, Messenger ; Transforming Growth Factor beta1 ; Tumor Necrosis Factor-alpha

Acute motor and sensory axonal neuropathy (AMSAN) are recently described subtypes of Guillain-Barre syndrome characterized by acute onset of distal weakness, loss of deep tendon reflexes, and sensory symptoms. A 21-yr-old male was transferred to our hospital due to respiration difficulties and progressive weakness. In laboratory findings, immunoglobulin M antibodies against hepatitis A were detected in blood and cerebrospinal fluid. The findings of motor nerve conduction studies showed markedly reduced amplitudes of compound muscle action potentials in bilateral peroneal, and posterior tibial nerves, without evidence of demyelination. Based on clinical features, laboratory findings, and electrophysiologic investigation, the patient was diagnosed the AMSAN following acute hepatitis A viral infection. The patient was treated with intravenous immunoglobulin and recovered slowly. Clinicians should consider this rare but a serious case of AMSAN following acute hepatitis A infection.
Acute Disease ; Electromyography ; Guillain-Barre Syndrome/*diagnosis/drug therapy/etiology ; Hepatitis A/complications/*diagnosis ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Male ; Young Adult