Light chain deposition disease (LCDD) is a systemic disorder characterized by the deposition of monoclonal immunoglobulin light chains (LCs) in various organs. As LCs are overproduced by an abnormal clone of B cells, LCDD is usually described in the course of plasma cell dyscrasias or other lymphoproliferative disorders. However, it can occur in the absence of any detectable hematological disorder even during prolonged follow-up. We experienced a case of 62-year-woman who presented generalized edema, massive proteinuria and renal insufficiency. The histologic findings showed nodular glomerular mesangial expansion and prominent ribbon like immunohistochemical staining for kappa-light chain in the glomerular capillary wall. There was no evidence of multiple myeloma in bone marrow biopsy specimen. Because she refused the intensive chemothrapy, low dose of prednisolone and cyclophosphamide were prescribed for 6 months. Her renal function was maintained relatively well without renal replacement therapy for 7 months. We report a case of idiopathic LCDD not associated with multiple myeloma or other plasma cell dysclasia.
B-Lymphocytes ; Biopsy ; Bone Marrow ; Capillaries ; Clone Cells ; Cyclophosphamide ; Edema ; Follow-Up Studies ; Immunoglobulin Light Chains ; Lymphoproliferative Disorders ; Multiple Myeloma ; Nephrotic Syndrome ; Paraproteinemias ; Plasma Cells ; Prednisolone ; Proteinuria ; Renal Insufficiency ; Renal Replacement Therapy

PURPOSE: To assess long-term clinical outcomes and factors influencing refractive change after implantation of hydrophilic (Akreos AO) and hydrophobic (Acrysof IQ) acrylic intraocular lens. METHODS: After phacoemulsification, intraocular lens was inserted in the bag (Akreos AO for 84 eyes and Acrysof IQ for 19 eyes). Uncorrected visual acuities (UCVA), refraction in both spherical equivalent (SE) and astigmatism were compared longitudinally in more than 2 years follow-up. Factors associated with SE change > or =0.5 diopter (D) after Akreos AO implantation were analyzed with logistic regression. RESULTS: Although Akreos AO showed hyperopic change mainly within 6 months, UCVA and astigmatism change were not statistically significant during follow up (+0.15 +/- 0.43 D, p = 0.027). UCVA, SE, astigmatism were not changed after Acrysof IQ implantation and not significantly different between two groups during follow up. Preoperative anterior chamber depth was a sole factor associated with SE change > or =0.5 D after Akreos AO implantation (p = 0.006). CONCLUSIONS: Possible hyperopic shift after Akreos AO implantation should be considered in setting target diopter, especially in eye with shallow anterior chamber.
Anterior Chamber ; Astigmatism ; Follow-Up Studies ; Hyperopia ; Lenses, Intraocular* ; Logistic Models ; Phacoemulsification ; Visual Acuity

PURPOSE: This study was done to develop and evaluate the Integrated Stress Management Program (ISMP) for elders in rural communities. METHOD: ISMP consists of 6 educational programs to improve self-management behavior and therapeutic recreational activities and was developed by analyzing the published reports and through multidisciplinary cooperation.Effectiveness of the ISMP was evaluated by implementing the program with 53 rural elders living at home, one session a week of, 2 hours per session over, 6 weeks from March 14 to April. 22, 2005. RESULTS: After participating in the program, participants' perceived levels of stress decreased(t=2.940, p<.05), and mood state (t=4.229, p<.001), life satisfaction(t=-4.911, p<.001), and perceived social support (t=-2.891, p<.05) increased significantly. CONCLUSION: The results of this study demonstrated that the ISMP is an effective program for relieving stress level, and increasing positive mood, life satisfaction, and social support for elders in a rural community.
Rural Population* ; Self Care

Primary hyperoxaluria is a rare autosomal recessive inherited metabolic disease which results from endogenous overproduction of oxalic acid. It causes variant phenotypes from renal failure in infancy to mere urolithiasis in late adulthood. We report a case of primary hyperoxaluria in a 11-year-old boy. He presented with recurrent multiple renal stones since 3 years of age. He had renal failure and markedly increased hyperoxaluria (568.26 microgram/mg of creatinine (normal: 0.04-0.15)) and his stones consisted of a mixture of calcium oxalate (30%) and calcium phosphate (10%) in contrast to pure calcium oxalate monohydrate in the other primary hyperoxaluria type 1 patients. A renal biopsy showed interstitial cellular infiltration with crystals which are birefringent under polarized light within the tubules. His general conditions were improved after hemodialysis treatment. For definite cure of disease, combined liver-kidney transplantation is considered.
Biopsy ; Calcium ; Calcium Oxalate ; Child ; Creatinine ; Humans ; Hyperoxaluria ; Hyperoxaluria, Primary* ; Kidney Failure, Chronic* ; Male ; Metabolic Diseases ; Nephrolithiasis ; Oxalic Acid ; Phenotype ; Renal Dialysis ; Renal Insufficiency ; Urolithiasis

A renal lesion of neoplasm-related nephrotic syndrome usually presents as membranous nephropathy. Neoplasm-related nephrotic syndrome exhibiting focal segmental glomerulosclerosis (FSGS) has been reported mainly in patients with hematological malignancies. But reports of an association between FSGS and neoplasm are extremely rare. We report a 66-year-old woman with FSGS who presented with nephrotic syndrome and was subsequently found to have carcinoid tumors in the duodenum, confirmed by tissue biopsy. After resection of carcinoid tumor, the patient received initial treatment with oral prednisolone for 2 months but her nephrotic syndrome persisted. The patient was then treated with oral cyclophosphamide. One month later, she was in complete remission of nephrotic syndrome as indicated by a normal serum albumin and no proteinuria. This case may be a coincidence or the lesions may be linked with each other but since little is known about the pathogenesis of FSGS and neoplasm, it is difficult to speculate on a pathogenetic link between these two lesions. We report a case of FSGS with nephrotic syndrome in a patient with carcinoid tumor.
Aged ; Biopsy ; Carcinoid Tumor* ; Cyclophosphamide ; Duodenum ; Female ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental* ; Hematologic Neoplasms ; Humans ; Nephrotic Syndrome ; Prednisolone ; Proteinuria ; Serum Albumin

The general anesthesia combined with epidural anesthesia have many advantages, which are including early recovery, postoperative analgesia, and less requirement of inhalation anesthetic. But the complications of epidural anesthesia such as total spinal anesthesia, respiratory depression, myocardial ischemia, and local anesthetic toxicity can lead to cardiac arrest. We report a case of acute myocardial infarction in a 74-year-old female patient receiving general anesthesia combined with epidural anesthesia. The profound hypotension without bradycardia and ST-segment elevation in lead II occurred 1 hour after local anesthetic injection and cardiac arrest followed. After the injection of atropine, epinephrine and dopamine, and external cardiac massage, sinus rhythm was restored. Postoperative serial examinations of ECG showed ST-segment elevation in leads II, III, aVF and serum enzymes such as CPK, CK-MB, and Troponin T were elevated. Emergency percutaneous transluminal coronary angioplasty was performed and the patient recovered uneventfully.
Aged ; Analgesia ; Anesthesia, Epidural ; Anesthesia, General ; Anesthesia, Spinal ; Angioplasty, Balloon, Coronary ; Atropine ; Bradycardia ; Dopamine ; Electrocardiography ; Emergencies ; Epinephrine ; Female ; Heart Arrest ; Heart Massage ; Humans ; Hypotension ; Inhalation ; Myocardial Infarction ; Myocardial Ischemia ; Respiratory Insufficiency ; Troponin T

Fenoverine is a spasmolytic drug. It has been reported as a causative drug for rhabdomyolysis in France and also in Korea. Well known risk factors for fenoverine induced rhabdomyolysis are hepatic dysfunction, renal dysfunction, concomitant use of lipid lowering drug, mitochondrial myopathy, lipid storage myopathy or malignant hyperthermia. We describe the clinical findings of 3 cases of fenoverine-induced rhabdomyolysis. (Case 1) A 78 year-old male patient complained of generalized myalgia after 7 days medication of fenoverine 300 mg, daily. (Case 2) A 72 year-old female who had been on statin medication for 4 years, complained of generalized myalgia and weakness after 1 day medication of 300 mg of fenoverine. (Case 3) A 57 year-old female who was diagnosed with liver cirrhosis, complained of generalized myalgia, after 4 days medication of 300mg of fenoverine. Laboratory and Tc-99m MDP bone scan findings were compatible with rhabdomyolysis in all 3 patients: ARF was complicated in case 2 and 3. The renal function improved with supportive care. As far as our knowledge concerned, case 1 is the first report of fenoverine-induced rhabdomyolysis without previously known risk factors. So we suggest when physicians are prescribing fenoverine, careful monitoring of symptoms and signs of rhabdomyolysis should be taken to all patients.
Aged ; Female ; France ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Korea ; Liver Cirrhosis ; Male ; Malignant Hyperthermia ; Middle Aged ; Mitochondrial Myopathies ; Muscular Diseases ; Myalgia ; Rhabdomyolysis* ; Risk Factors ; Technetium Tc 99m Medronate

PURPOSE: We report on the clinical efficacy of the combined operation of corneal tattooing and amniotic membrane transplantation on the treatment of bullous keratopathy in patients with poor visual potentials. CASE SUMMARY: We performed corneal tattooing and amniotic membrane transplantation in 7 eyes with bullous keratopathy. Postoperatively, we assessed the status of corneal reepithelialization, postoperative pain, and cosmetic effects. In all cases but one, corneal reepithelialization was successful without pain, and the corneal tattooing persisted throughout the follow-up period. In one case, a corneal epithelial defect developed, so we performed an additional amniotic membrane transplantation. After 2 weeks, the corneal epithelial defect healed and caused no further pain. CONCLUSIONS: The combined operation of corneal tattooing and amniotic membrane transplantation is a good choice for the treatment of bullous keratopathy with regard to cosmetic problems, pain, and poor visual potential.
Amnion ; Cosmetics ; Eye ; Follow-Up Studies ; Humans ; Pain, Postoperative ; Tattooing ; Transplants

Tuberous sclerosis is an autosomal dominant disorder characterized by seizures, mental retardation and hamartomatous tumors in multiple organs, including subependymal giant cell astrocytomas, cortical tubers, cardiac rhabdomyoma, pulmonary lymphangiomyomatosis and renal angiomyolipomas. However, these clinical features are not always present. The following is a report concerning a 30-year-old woman with tuberous sclerosis who suffered a spontaneous hemorrhage into the angiomyolipoma of the kidney and underwent curative selective renal embolization. The patient had a medical history of focal seizures. Moreover, brain magnetic resonance imaging revealed typical signs of tuberous sclerosis, and a high resolution computerized tomography of the chest showed bilateral nodules with irregular margins and multiple thin-walled cystic lesions. There was no family history of renal disease or of tuberous sclerosis. As such, this case exhibited rare clinical manifestations of tuberous sclerosis involving multiple organs.
Adult ; Angiomyolipoma* ; Astrocytoma ; Brain ; Female ; Hemorrhage ; Humans ; Intellectual Disability ; Kidney ; Lymphangioleiomyomatosis ; Magnetic Resonance Imaging ; Rhabdomyoma ; Rupture, Spontaneous* ; Seizures ; Thorax ; Tuberous Sclerosis*

BACKGOUND: Cobalt chloride (COCL) has hypoxia-mimetic effects by inhibiting degradation of HIF-1alpha, which is a master regulator of genes activated by low oxygen tension. Heme oxygenase-1 (HO-1), an inducible heat shock protein, is known to have cytoprotective effects against ischemic injury. This study evaluated the efficacy of COCL in a bilateral renal ischemia-reperfusion (I-R) injury model of male Sprague-Dawley rats. METHODS: I-R renal injury was induced by 45 min clamping of both renal arteries. Rats in the sham (n=6) and I-R control groups (n=8) had been drinking tap water, whereas rats in the COCL treated sham (n=6) and COCL treated I-R groups (n=9) had been drinking water containing 2 mM COCL from day -10 to day 1. RESULTS: The serum level of creatinine 24 hrs after surgery was 2.6+/-1.1 (mean+/-SD) mg/dL in I-R COCL treated group, significantly lower than that in I-R control group (4.8+/-1.6 mg/dL, p<0.05). The renal HO-1 gene expression and protein signal were significantly upregulated in the COCL treated sham group compared to sham operated control rats (all, p<0.05). The expressions of TGF-beta MCP-1, TNF-alpha endothelin-1 and Fas genes in COCL treated I-R rats were significantly lower than those of I-R control rats (all, p<0.05). The level of Bcl-2 gene expression of COCL treated I-R rats was significantly higher than the level of I-R control rats (p<0.05). CONCLUSION: It is speculated that the pretreatment of COCL in I-R rat model attenuates ischemic renal injury and at least in part, upregulation of renal HO-1 is involved in this mechanism.
Animals ; Cobalt* ; Constriction ; Creatinine ; Drinking ; Drinking Water ; Endothelin-1 ; Gene Expression ; Genes, bcl-2 ; Heat-Shock Proteins ; Heme Oxygenase-1 ; Humans ; Kidney ; Male ; Models, Animal ; Oxygen ; Rats ; Rats, Sprague-Dawley ; Renal Artery ; Transforming Growth Factor beta ; Tumor Necrosis Factor-alpha ; Up-Regulation ; Water