Pernicious Anemia Associated Cobalamin Deficiency and Thrombotic Microangiopathy: Case Report and Review of the Literature.

A 43-year-old Hispanic male without significant previous medical history was brought to emergency department for syncope following a blood draw to investigate a 40 lbs weight loss during the past 6 months associated with decreased appetite and progressive fatigue. The patient also reported a 1-month history of jaundice. On examination, he was hemodynamically stable and afebrile with pallor and diffuse jaundice but without skin rash or palpable purpura. Normal sensations and power in all extremities were evident on neurological exam. Presence of hemolytic anemia, schistocytosis, thrombocytopenia, and elevated lactate dehydrogenase (LDH) was suggestive of thrombotic thrombocytopenic purpura (TTP). However, presence of leukopenia, macrocytes, and an inadequate reticulocyte response to the degree of anemia served as initial clues to an alternative diagnosis. Two and one units of packed red blood cells were transfused on day 1 and day 3, respectively. In addition, one unit of platelets was transfused on day 2. Daily therapeutic plasma exchange (TPE) was initiated and continued until ADAMTS-13 result ruled out TTP. A low cobalamin (vitamin B12) level was evident at initial laboratory work-up and subsequent testing revealed positive intrinsic factor-blocking antibodies supporting a diagnosis of pernicious anemia with severe cobalamin deficiency. Hematological improvement was observed following vitamin B12 supplementation. The patient was discharged and markedly improved on day 9 with outpatient follow-up for cobalamin supplementation.

Systematic review of the efficacy and safety of intradermal versus intramuscular hepatitis B vaccination in end-stage renal disease population unresponsive to primary vaccination series.

INTRODUCTION: The response to hepatitis B vaccine in the dialysis population is reduced compared to the general population. The intradermal (ID) hepatitis B vaccine has been studied as a potential alternative to intramuscular (IM) administration. This alternative route of administration may illicit a response via a distinct immunologic pathway that may help achieve higher seroconversion rates and thus, protection against hepatitis B infection in this vulnerable patient population. METHODS: A literature search was performed in January 2015 using Embase, MEDLINE, and the Cochrane Central Register of Controlled Trials with keywords including, hepatitis B vaccines, intradermal, dermal, intracutaneous, dialysis, hemodialysis, continuous ambulatory peritoneal dialysis, CAPD, peritoneal dialysis, renal failure, chronic renal failure, chronic kidney disease, chronic renal insufficiency, End Stage Renal Disease, ESRD, and CKD. Our search strategy was restricted to human studies published in the English language, and additional literature was retrieved by hand-searching bibliographies of relevant articles. Two reviewers (F.Y. and S.G.) independently reviewed abstracts and/or full texts of articles retrieved from the electronic database using the above-mentioned search strategy. Inclusion criteria were as follows: (1) Published, English-language studies performed in the human population, (2) adult patient population (≥18 years of age), (3) randomized trials, (4) patient population must have been unresponsive to a primary IM hepatitis B vaccination protocol, (5) patients must be chronic dialysis patients, either on maintenance hemodialysis or continuous ambulatory peritoneal dialysis (CAPD), (6) studies that compare IM and ID hepatitis B vaccination-associated seroconversion rates, (7) results must be reported as seroconversion rates at 1-3, 6-9, 12, or 20 months post-vaccination, and (8) seroconversion (protective antibody levels) defined as >10 or ≥10 IU/L. RESULTS: Our initial literature review yielded 113 results, of which four were included in our final review. These four prospective trials studied a combined total of 204 dialysis patients. Of these patients, 120 (59%) had received the hepatitis B vaccine intradermally, while 84 (41%) received it intramuscularly. Hepatitis B vaccination type, dose, route, and seroconversion rates were tabulated for each study. Each of the studies used different protocols for patient inclusion, schedule of vaccine administration, and time-points for measuring seroconversion. Seroconversion rates at either 1, 2, 3, 6-9, 12 and/or 20 months were reported. The combined seroconversion rates were 91%, 83%, 86%, 81%, 76%, and 32% at 1, 2, 3, 6-9, 12, and 20 months in the ID group, respectively, and 55%, 72%, 58%, 44%, 24%, and 0% in the IM group, respectively. Chi-square analysis revealed a significantly higher proportion of patients achieving seroconversion in the ID group versus the IM group (p < 0.05). CONCLUSIONS: Our review demonstrates that ID hepatitis B vaccination in primary non-responders undergoing dialysis provides an effective alternative to IM vaccination as a means of protection against hepatitis B infection in this highly susceptible population. Additional well-designed, double-blinded, randomized trials are warranted to establish clear guidelines on ID Hepatitis B vaccine dose and duration of vaccination schedule.

Acute kidney injury from pyelonephritis in an elderly man: case report.

Pyelonephritis is rarely considered in the differential diagnosis of acute kidney injury. Acute non-obstructed bacterial pyelonephritis is an infrequent and rarely considered cause of rapidly progressive acute kidney injury. A diagnostic challenge thus develops as it is difficult to clinically differentiate acute kidney injury secondary to ischemic or toxic acute tubular necrosis or papillary necrosis versus acute interstitial nephritis secondary to drugs or infectious pyelonephritis. We describe a case of acute kidney injury due to suppurative pyelonephritis in an elderly immunocompetent man who presented with dysuria, vomiting, and fever and later found to have histologic and radiologic proven pyelonephritis as the cause of acute kidney injury in the absence of hypotension, nephrotoxic agents, non-steroidal analgesics, immunosuppression, urinary tract obstruction, or other structural anomalies. The patient was managed with antimicrobial therapy, hemodialysis, and a short course of corticosteroids.

The safety and efficacy of ferumoxytol therapy in anemic chronic kidney disease patients.

BACKGROUND: Administration of safe and effective iron therapy in patients with chronic kidney disease is a time consuming process. This phase II clinical trial studied ferumoxytol, a semi-synthetic carbohydrate-coated iron oxide administered by rapid intravenous injection to anemic chronic kidney disease patients (predialysis or undergoing peritoneal dialysis). METHODS: Inclusion criteria included hemoglobin < or =12.5 g/dL and transferrin saturation < or =35%. Twenty-one adult patients were randomized to receive ferumoxytol in a regimen of 4 doses of 255 mg iron in 2 weeks or 2 doses of 510 mg iron in 1 to 2 weeks. Ferumoxytol was administered at a rate of up to 30 mg iron/sec. RESULTS: The maximum hemoglobin response following ferumoxytol administration occurred at 6 weeks, increasing from a baseline of 10.4 +/- 1.3 g/dL to 11.4 +/- 1.2 g/dL (P < 0.05). Ferritin increased from a baseline of 232 +/- 216 ng/mL to a maximum of 931 +/- 361 ng/mL at 2 weeks (P < 0.05), while the baseline transferrin saturation increased from 21 +/- 10% to 37 +/- 22% at 1 week (P < 0.05). Seven adverse events in 5 patients during this trial were deemed possibly related to ferumoxytol, none serious. These events included constipation, chills, tingling, a gastrointestinal viral syndrome, delayed pruritic erythematous rash, and transient pain at the injection site. CONCLUSION: Although larger studies are required, this small study demonstrates that ferumoxytol can be safe and effective in increasing iron stores, is associated with an increased hemoglobin response, and is well tolerated at a rapid infusion rate.