Thrombotic microangiopathy (TMA) is a serious complication of solid organ transplantation. Drug-induced TMA is typically caused by immunosuppressants, particularly calcineurin inhibitors. Withdrawing the causative drug can be one of the treatments for TMA. However, the more immunosuppressants are reduced, the more risk of rejection increases. Even if TMA is successfully resolved, the outcomes of patient and graft survival would be worse than expected. Therefore, it is necessary to maintain efficient and safe immunosuppression therapy. We report on a case of de novo TMA after kidney transplantation triggered by tacrolimus and reactivated by sirolimus. Belatacept, a novel CTLA4 Ig fusion protein, was administered for maintenance immunosuppressant with mycophenolate mofetil and prednisolon. The patient had excellent early graft outcome, and there have been no adverse events so far.
Abatacept ; Calcineurin ; Graft Survival ; Humans ; Immunosuppression* ; Immunosuppressive Agents ; Kidney Transplantation* ; Kidney* ; Organ Transplantation ; Sirolimus ; Tacrolimus ; Thrombotic Microangiopathies* ; Transplants

Immunoglobulin deficiency with increased IgM, as defined by World Health Organization classification of primary immunodeficiency, is characterized by normal or increased concentrations of serum IgM and, in some cases, IgD, but decreased or absent IgG, IgA, and IgE. Patients with these disorders have a high incidence of recurrent pyogenic infections, including otitis media, pneumonia, and septisemia. We recently experienced a case of immunoglobulin deficiency with increased IgM in a 25-year old man. He had been suffered from recurrent pyogenic infections-pneumonia, otitis media-since about 6 months of his age. He was admitted due to pneumonia, and Haemophilus influenza was isolated from the sputum culture. Also chest X-ray showed bronchiectasis. His serum immunoglobulin levels revealed increased concentration of IgM and decreased IgG and IgA. He was successfully treated with antibiotics, and now he is being followed-up. So we present this case with the review of literature.
Adult ; Anti-Bacterial Agents ; Bronchiectasis ; Classification ; Haemophilus ; Humans ; Immunoglobulin A ; Immunoglobulin D ; Immunoglobulin E ; Immunoglobulin G ; Immunoglobulin M* ; Immunoglobulins* ; Incidence ; Influenza, Human ; Otitis ; Otitis Media ; Pneumonia ; Sputum ; Thorax ; World Health Organization

Hyperuricemic patients with gouty arthritis or tophi, a serum uric acid concentration of 8.0 mg/dL or higher, and complications should be treated with urate-lowering drugs. Conventionally, allopurinol is used to treat hyperuricemia and gout, but it is necessary to adjust the dosage according to the degree of renal impairment. Uncommonly, allopurinol may have severe or fatal side effects. The non-purine xanthine oxidase inhibitor febuxostat undergoes hepatic metabolism and may require less dose adjustment in association with renal function. It is considered to be an alternative treatment for hyperuricemic patients with chronic kidney disease. Our experience suggests that low-dose febuxostat is a promising alternative to allopurinol for the treatment of gouty arthritis or tophi in peritoneal dialysis patients.
Allopurinol ; Arthritis, Gouty ; Gout* ; Humans ; Hyperuricemia ; Kidney Failure, Chronic ; Metabolism ; Peritoneal Dialysis ; Peritoneal Dialysis, Continuous Ambulatory* ; Renal Insufficiency, Chronic ; Uric Acid ; Xanthine Oxidase ; Febuxostat

Tuberous sclerosis complex (TSC) is a neurocutaneous disease characterized by the formation of hamartomas in multiple organs. TSC can show lesions including facial angiofibroma, shagreen patch on the skin, cortical tuber, subependymal nodule, astrocytoma in the brain, cardiac rhabdomyoma, and renal angiomyolipoma. In particular, renal angiomyolipoma may be a cause of end-stage renal disease (ESRD). On the other hand, sirolimus has regulatory effects on cellular growth and proliferation via its inhibitory effect on a protein, mammalian target of rapamycin. We report on a case of an 18-year-old male who underwent renal transplantation due to ESRD induced by TSC. Sirolimus played a role in successful treatment of TSC and effective immunosuppression for transplantation.
Adolescent ; Angiofibroma ; Angiomyolipoma ; Astrocytoma ; Brain ; Hamartoma ; Hand ; Humans ; Immunosuppression ; Kidney Failure, Chronic* ; Kidney Transplantation ; Male ; Rhabdomyoma ; Sirolimus* ; Skin ; Tuberous Sclerosis*

Transplant renal artery stenosis (TRAS) is a common surgical complication after kidney transplantation (KTP) and is the cause of allograft dysfunction. TRAS is a potentially curable cause of refractory hypertension and allograft dysfunction which accounts for approximately 1% to 5% of cases of post-transplant hypertension. Acute cellular rejection (ACR) is also common after KTP, which is the main cause of allograft dysfunction. Although the incidence of ACR has declined with the advent of new immunosuppressive drugs, it is still around 15% worldwide. Although each disease is frequently seen individually, seeing both together is rare. A 42-year-old man with end stage renal disease underwent KTP, and the donor was his younger brother. Four months after KTP, his serum creatinine was increased to 2.1 mg/dL, and renal biopsy showed interstitial lymphocytic infiltration and tubulitis. With the diagnosis of acute T-cell mediated rejection, steroid pulsing therapy was started, but it was resisted. Therefore thymoglobulin 60 mg (1 mg/kg/day) was administered for 6 days, but serum creatinine was 1.8 mg/dL. Abdomen magnetic resonance angiography showed TRAS, stenosis at the anastomosis site and lobar artery in the lower pole. Percutaneous transluminal angiography was performed successfully. After balloon angioplasty, the stenotic lesion showed a normal size and blood flow. The patient's renal function returned to normal levels and he is currently being followed up for 9 months.
Abdomen ; Adult ; Allografts ; Angiography ; Angioplasty, Balloon ; Arteries ; Biopsy ; Constriction, Pathologic ; Creatinine ; Diagnosis ; Humans ; Hypertension ; Incidence ; Kidney Failure, Chronic ; Kidney Transplantation ; Magnetic Resonance Angiography ; Renal Artery Obstruction* ; Renal Artery* ; Siblings ; T-Lymphocytes ; Tissue Donors ; Transplantation*

An esophageal bezoar, although uncommon, is now recognized as a distinct clinical entity. An esophageal bezoar is rare but can form due to regurgitation of a gastric bezoar, motor disorder or anatomical abnormality, or following a gastrectomy. In general, bezoars are most often found in the stomach, and are formed by the accumulation of foreign ingested materials, including vegetable material and hair. In Korea, no case of a primary esophageal bezoar has been reported after a total gastrectomy. We report a case of an endoscopically treated primary esophageal bezoar that occurred after a total gastrectomy, without complications.
Bezoars ; Gastrectomy ; Hair ; Korea ; Stomach ; Vegetables

Neurofibromatosis is an autosomal dominant hereditary disorder with an overall incidence of one in 3,000~4,000, and type 1 (Von Recklinghausen's neurofibromatosis) characterized by the presence of multiple cutaneous neurofibromas, axillary and groin freckling, and cafe- au-lait spot. The neurofibromatosis type 1 gene is a tumor suppressor gene. Patients with the neurofibromatosis type 1 are at increased risk of developing nervous system neoplasm, including plexiform neurofibromas, optic gliomas, ependymomas, meningiomas, astrocytomas, and pheochromocytomas. Neurofibromas may undergo secondary malignant degeneration and sarcomatous changes. Patients with neurofibromatosis type 1 show a high incidence of Wilm's tumor, rhabdomyosarcoma, nonlymphocytic leukemia, and pheochromocytoma but the gastrointestinal involvement appears to be relatively rare and usually consists of neurofibroma, ganglioneuroma, and leiomyoma. We have identified a case of early gastric adenocarcinoma and intraabdominal schwannoma in a 65-year-old man afflicted with neurofibromatosis type 1.
Adenocarcinoma* ; Aged ; Astrocytoma ; Ependymoma ; Ganglioneuroma ; Genes, Tumor Suppressor ; Groin ; Humans ; Incidence ; Leiomyoma ; Leukemia ; Meningioma ; Nervous System Neoplasms ; Neurilemmoma* ; Neurofibroma ; Neurofibroma, Plexiform ; Neurofibromatoses* ; Neurofibromatosis 1* ; Optic Nerve Glioma ; Pheochromocytoma ; Rhabdomyosarcoma ; Wilms Tumor

BACKGROUND/AIMS: Heart rate variability (HRV) can be used to assess the effects of drugs and other interventions, including exercise, respiration, metabolic changes, and psychological or physical stressors, on cardiac autonomic tone. HRV is regulated by the balance of sympathetic and parasympathetic tone. Few studies pertaining to HRV in end stage renal disease (ESRD) patients have been performed in Korea. Thus, autonomic nervous system activity as indicated by HRV was investigated in patients on hemodialysis due to ESRD. METHODS: We compared the pattern of cardiac sympathetic and parasympathetic activity through time- and frequency- domain analyses of HRV with 24-hour Holter monitoring in 30 ESRD patients and 64 hypertensive control subjects. The ESRD patients had undergone hemodialysis therapy at the Bongseng Hospital between January 2006 and June 2007. RESULTS: The mean age of ESRD patients and hypertensive controls was 51.17+/-11.91 and 55.02+/-13.72 years, respectively. In the ESRD group, all time- and frequency-domain HRV measures, including the standard deviation of all normal sinus R-R intervals over 24 hours (SDNN), the HRV index, the very low-frequency (VLF) normalized unit of low-frequency (LFnorm), and the ratio of low-frequency power to high-frequency power (LF/HF), were reduced; the normalized unit of high frequency (HFnorm) was increased in the ESRD patients compared with the control group. CONCLUSION: The autonomic tone in ESRD patients on hemodiaysis was decreased compared with hypertensive patients. The parasympathetic tone in ESRD patients on hemodyalysis was dominant over the sympathetic tone.
Autonomic Nervous System ; Electrocardiography, Ambulatory ; Heart ; Heart Rate ; Humans ; Kidney Failure, Chronic ; Korea ; Renal Dialysis ; Respiration

Pneumocystis jirovecii pneumonia (PCP) can be a life-threatening opportunistic infection after kidney transplantation, occurring most frequently in the first 12 months with the symptoms of dyspnea, cough, fever, and hypoxia. Prophylaxis for PCP is usually applied during the first 3 months to 1 year after transplantation, but late onset incidence of PCP can be detected. We report on a patient who developed PCP 9 years after renal transplantation. The patient showed indolent onset of acute respiratory distress and was treated with trimethoprim-sulfamethoxazole and corticosteroid therapy. Previous rescue treatment of acute cellular rejection with ongoing maintenance of an elevated level of immunosuppressants may have predisposed the patient to PCP.
Anoxia ; Cough ; Dyspnea ; Fever ; Humans ; Immunosuppressive Agents ; Incidence ; Kidney Transplantation ; Opportunistic Infections ; Pneumocystis jirovecii* ; Pneumocystis* ; Pneumonia ; Transplantation* ; Trimethoprim, Sulfamethoxazole Drug Combination

The anal canal is the most distal part of the gastrointestinal tract, and it is developed and formed during the embryonic period. Infection is the most common disease process that occurs around the anorectum, yet tumors or cysts are occasionally encountered. The abnormal development of these parts of the gastrointestinal track during the embryonic period can result in congenital lesions that are discovered in young children or adults. A 72-year-old woman presented to us with postprandial lower abdominal discomfort and fecal incontinence. An anorectal mass was felt on the rectal examination. The colonoscopy demonstrated a submucosal tumor that was closely located to the anorectal junction. The tumor was excised with a snare and it was diagnosed as an analgland cyst due to the histologic features. It is necessary to differentiate anal gland cyst from the other diseases that have submucosal characters, such as carcinoid tumor.
Child ; Adult ; Male ; Female ; Humans ; Cysts