Use of Bortezomib as Anti-Humoral Therapy in Kidney Transplantation

This study aimed to investigate the effect of bortezomib in the desensitization and treatment of acute antibody mediated rejection (AAMR) in kidney transplantation. Nine patients who received bortezomib therapy for desensitization (DSZ group, n = 3) or treatment of AAMR (AAMR group, n = 6) were included in this study. In the DSZ group, 2 patients required DSZ owing to positive cross match and 1 owing to ABO mismatch with high baseline anti-ABO antibody titer (1:1,024). Bortezomib was used at 1, 3, 8, and 11 days from the start of the treatment. In the AAMR group, 3 patients showed full recovery of allograft function after bortezomib use and decrease in donor specific anti-HLA antibody (HLA-DSA). However, 3 patients did not respond to bortezomib and experienced allograft failure. In the DSZ group, negative conversion of T-CDC (complement-dependent cytotoxicity) was achieved, and HLA-DSA was decreased to lower than a weak level (median fluorescence intensity [MFI] < 5,000) in 2 patients. In the case of ABO mismatch kidney transplantation, the anti-A/B antibody titer decreased to below the target (< or = 1:16) after bortezomib therapy. Therefore, bortezomib could be an alternative therapeutic option for desensitization and treatment of AAMR that is unresponsive to conventional therapies.

Partial Anomalous Pulmonary Venous Return (PAPVR) in a Patient with Sjogren's Syndrome

Pulmonary hypertension (PH) is a rare manifestation in patients with primary Sjogren's syndrome (pSS) and it can occur with or without interstitial lung disease (ILD). Patients with PH and ILD who show signs of exacerbation of dyspnea are commonly assessed for pure PH aggravation, ILD progression or pulmonary infection. However, the presence of congenital cardiac anomalies, such as partial anomalous pulmonary vein return (PAPVR), can also be a cause of dyspnea exacerbation. PAPVR is a rare congenital anomaly that involves drainage of 1 to 3 pulmonary veins into the right-sided heart circulation, resulting in a partial left-to-right shunt. Here we present a case of PAPVR as the cause of PH aggravation in a patient with pSS with accompanying PH.

A Case of Adipsic Hypernatremia Associated with Anomalous Corpus Callosum in Adult with Mental Retardation

Adipsic hypernatremia cause chronic hyperosmolality and hypernatremia through a combination of impaired thirst and osmotically stimulated antidiuretic hormone secretion. This syndrome can be grouped together as disorders of osmoreceptor dysfunction due to the various degrees of osmoreceptor destruction related with different types of intracranial lesions around the anterior hypothalamus, consistent with the location of primary osmoreceptor cells. Adipsic hypernatremia, associated with developmental disorder of corpus callosum, is very rare. Most cases are diagnosed at infancy and early childhood; the replacement of desmopressin is necessary. Herein, we report adipsic hypernatremia associated with anomalous corpus callosum in adult with mental retardation; they were treated with only free water without desmopressin.

A Case of Hemolytic Uremic Syndrome in a Lung Cancer Patient Treated with Gemcitabine / 결핵및호흡기질환

Hemolytic uremic syndrome (HUS) is a rare disorder characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. HUS arises from a wide spectrum of conditions, and chemotherapeutic agents have been reported to be associated with HUS, including Mitomycin, Cisplatin, Bleomycin, and Gemcitabine. A 76-year-old man treated with Gemcitabine due to non-small cell lung cancer developed clinical and laboratory findings compatible with HUS. Gemcitabine was ceased and hemodialysis and plasma exchange were utilized and he recovered. A high level of suspicion for HUS is necessary when cancer patients are treated with Gemcitabine, and prompt recognition and treatment are also essential.

A Bowel Perforation That Developed during the Transanal Extraction of a Large Rectal Foreign Body / 대한소화기내시경학회지

Rectal foreign bodies are rare clinical problem in South Korea. Although many foreign bodies can be extracted safely using endoscopic procedures, some patients require surgery. Here we describe the case of a 35-year-old male who presented with a rectosigmoid foreign body, a large carrot measuring 28x7 cm. Sigmoidoscopy revealed a carrot in the upper rectum extending to the sigmoid colon. Endoscopic removal failed. The surgeon unsuccessfully attempted to extract the carrot using various tools without spinal anesthesia. During the extraction attempt, the patient complained of sudden abdominal pain, and a simple x-ray revealed pneumoperitoneum. An emergency colotomy and removal of the foreign body was performed, followed by primary repair of the perforation and a colostomy. Three months later, the colostomy was repaired.

A Case of Left Ventricular Pseudoaneurysm in the Left Atrioventricular Groove after Mitral Valve Replacement

Left ventricle-coronary sinus fistula and left ventricular pseudoaneurysm are unusual and frightening complications after mitral valve replacement. A 27-year-old female patient underwent mitral valve replacement 5 years previously and trans-thoracic echocardiography showed an outpouching lesion at the atrioventricular groove. It was difficult to differentiate whether the lesion was a left ventricle-coronary sinus fistula or a left ventricular pseudoaneurysm by two-dimensional echocardiography. Cardiac computed tomography confirmed a left ventricular pseudoaneurysm compressing the coronary sinus.