Pantoea agglomerans: A rare infectious outbreak affecting maintenance hemodialysis patients in a tertiary care hospital.

BACKGROUND: Pantoea agglomerans is an environmental pathogen known to cause infection in immunocompromised individuals, particularly after thorn injuries. However, previous data showed few cases of human disease caused by contaminated medical products such as parenteral nutrition, anesthetic agents, blood, and peritoneal dialysis solutions. Infection in hemodialysis patients is rare. In this study, we presented a detailed account of several hemodialysis patients infected with this contagious pathogen and compared them with noninfected dialysis patients. METHODS: We retrospectively reviewed the hospital records of 105 hemodialysis patients. Seventeen of 105 patients were diagnosed with P. agglomerans infection. We carefully analyzed their entire in-hospital course. RESULTS: Among infected patients, 52.9% were male with a median age of 49 (IQR: 32-66) years. Compared to the noninfected patients, age below 50 years, prior kidney transplantation, prior immunosuppression and antibiotics use, and dialysis via a tunneled vascular catheter were the significant epidemiological features. Despite negative microbiological investigations, we suspect the possible infectious spread via infected central venous catheter was the likely infectious source. Most importantly, all patients responded well to intravenous antibiotics. Only two patients required the removal of the tunneled catheter. Their mortality rate was 0%. CONCLUSION: P. agglomerans infection, although considered rare, is becoming increasingly prevalent among dialysis patients. Its occurrence must be appraised as an infectious outbreak rather than mere contamination. Prompt treatment, source identification, and early implementation of preventive strategies should always be the goal to curtail this infection at an early stage.

Impact of Renal Replacement Therapy on Outcomes of Living Donor Liver Transplantation for Acute Liver Failure: A Cohort Study.

Despite the promising role of renal replacement therapy (RRT) in acute liver failure (ALF), high-risk patients need liver transplantation and remain at risk for death due to cerebral complications. The objective of this study was to report outcomes of living donor liver transplantation (LDLT) for ALF with perioperative RRT. This was a single-center retrospective cohort study. Out of 1167 LDLTs, 24 patients had ALF and met the King's College criteria for transplantation. They were categorized into no-RRT (n = 13) and RRT (n = 11) groups. We looked at 1-year posttransplant survival in these patients. The median serum ammonia level at the time of transplant in the no-RRT and RRT groups was 259.5 mcg/dL (222.7-398) and 70.6 mcg/dL (58.1-92.6) (p = 0.005). In the RRT group, serum ammonia level < 100 mcg/dL was achieved in all patients. Seven (53.8%) patients in the no-RRT group and 11/11 (100%) in the RRT group were extubated and regained full consciousness after LDLT (p = 0.013). The 90-day mortality was 6/13 (46.1%) and 2/11 (18.1%) (p = 0.211). There was no brainstem herniation-related mortality in the RRT group, that is, 5/13 (38.4%) and 0/11 (0%) (p = 0.030). The 1-year posttransplant survival was also significantly higher in the RRT group (p = 0.031). The use of RRT lowers serum ammonia levels and might reduce posttransplant mortality due to brainstem herniation.

Coexistence of Lupus Nephritis, Ulcerative Colitis, and Communicating Hydrocephalus: A Report of a 21-Year-Old Male.

Systemic lupus erythematosus (SLE) and ulcerative colitis (UC) are multisystem autoimmune disorders that rarely coexist. We report a case history of a 21-year-old male, presenting with bloody diarrhea and, later, diagnosed to have ulcerative colitis on colonic biopsy. There was clinically silent renal impairment leading to end-stage kidney disease requiring hemodialysis possibly secondary to ongoing lupus nephritis as suggested by positive lupus-specific antibodies' detection. Besides this, the diagnosis of lupus associated with early communicating hydrocephalus was made on CT brain findings which clinically responded well to the initiation of immunosuppressive therapy. It is imperative to keep in mind the remote possibility of ulcerative colitis in an SLE patient with gastrointestinal (GI) manifestations. Communicating hydrocephalus is a rare neurological manifestation of SLE leading to seizures and can respond well to the initiation of steroids and immunosuppressants. Therefore, a trial of immunosuppressant medications must be given even in a patient with end-stage renal disease (ESRD) to halter extra renal rare lupus manifestations.

Association of Adult-Onset Bartter Syndrome With Undifferentiated Connective Tissue Disorder.

Bartter syndrome is a rare autosomal recessive, salt-losing disorder characterized by hypokalemic hypochloremic metabolic alkalosis. We are reporting a case of 21 years old patient, who presented with lower limb weakness, marked hypokalemia, proteinuria, and renal impairment detected on laboratory evaluation. The diagnosis of Bartter syndrome was suspected by marked hypokalemia and was supported by renal biopsy which showed evidence of Juxtaglomerular (JG) hyperplasia. This is the first case report about clinicopathological features of the patient with acquired Bartter syndrome and associated undifferentiated connective tissue disorder manifesting as hypokalemia with paralysis.

A Rare Case of Paroxysmal Nocturnal Hemoglobinuria With Bilateral Renal Vein Thrombosis.

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hematopoietic stem cell (HSC) disorder characterized by a partial or complete deficiency of glycosyl-phosphatidylinositol (GPI)-linked membrane proteins, which leads to intravascular hemolysis. The loss of CD55 and CD59, two GPI-anchored proteins on red blood cell surfaces, from mutations in the X-linked phosphatidylinositol glycan class A (PIGA) gene, causes unrestricted proliferation of complement activation. The loss of CD59 especially leads to 'paroxysms' of acute intravascular hemolysis during events of stress. Extravascular hemolysis also occurs without CD55 as the accumulation of C3 on red blood cell surfaces leads to their destruction by the reticuloendothelial system. Diagnosis of PNH relies primarily on clinical presentation and flow cytometry assays used to detect the GPI-anchored proteins, CD55 and CD59; however, fluorescein-labeled proaerolysin variant (FLAER) is seen to have a significant advantage over CD55 and CD59. Typical symptoms of the disorder include fatigue, shortness of breath, hemoglobinuria, abdominal pain and bone marrow failure. Thrombosis also occurs secondary to nitric oxide (NO) deficiency, release of procoagulants, increased tissue factor and reduced fibrinolysis. The classification of PNH is subdivided into three types: classical, PNH with another bone marrow disorder and subclinical PNH. Management of hemolysis, thrombosis and pancytopenia is based on the pathogenesis involved. Inhibition of complement in the form of humanized monoclonal antibody against complement C5 (eculizumab) is seen as an emerging treatment option, while stem cell/bone marrow transplant may also be offered. We present a rare case of PNH with bilateral renal vein thrombosis, who was diagnosed with classical PNH on clinical presentation and flow cytometry. He was initially offered bone marrow transplantation but was lost to follow-up and later presented with bilateral renal vein thrombosis. He was managed conservatively with transfusions and anticoagulation, and was discharged for follow-up on an outpatient basis.