Intelligent Malingering in the Setting of Porphyria Variegata: A Rare Occurrence on Both Fronts

Malingering can be a difficult diagnosis to discern, especially in patients with well-crafted stories presenting with signs and symptoms that align directly with the literature. This can further become a challenge when a patient is malingering in the setting of a rare disease, where many complaints can be subjective in nature and not entirely testable by physical exam alone. Malingering is responsible for billions of dollars of healthcare waste every single year, and this report can serve as a guide of history elements, signs and symptoms to look out for with patients malingering in the setting of the porphyrias. It is important to recognize when patients are malingering, and when they are not, so that they may receive the appropriate care to help with their condition. This report can also serve as a guideline for what laboratory tests and studies to order in the setting of a suspected porphyria case, in order to confirm the diagnosis and get the patient the appropriate treatment regimen. Intelligent malingering is a growing problem, especially with the amount of access the general public has to medical information, and it is important for us to be able to identify when a patient is truly suffering from a rare disease and when they are malingering

Richter Transformation in Chronic Lymphocytic Leukemia with Hypercalcemia and Acute Kidney Injury Presenting as Altered Mental Status: A Case Report

Introduction: Richter’s transformation is a deadly complication of chronic lymphocytic leukemia (CLL) associated with high mortality. This transformation involves the development of an aggressive, diffuse large B cell lymphoma in the setting of a chronic, underlying case of CLL. Although it is rare, Richter’s transformation is important to identify in patients with a history of CLL because of its significant mortality if left untreated. In the setting of CLL, there are many ways in which patients can present if they are currently undergoing a Richter’s transformation. Case Description: Patients may present with symptoms common to CLL which include but are not limited to fatigue, easy bruising, weight loss, etc., or they may present more unusually, as in the case that will be presented here: with altered mental status, fall, hypercalcemia and acute kidney injury (AKI). Discussion: With the current state of affairs due to COVID-19, it is also important to recognize the potential complications of patients with this disease state that are positive for COVID-19, leading to further complications such as disseminated intravascular coagulation (DIC). Recognizing these patients, and directing their care appropriately is important for their disease progression and outlook

Potential of Donation After Unexpected Circulatory Death Programs Defined by Their Demographic Characteristics

BACKGROUND. Donation after unexpected circulatory death (uDCD) donors are often suggested to increase the number of donor organs. In 2014, a uDCD protocol was implemented in three transplant centers in the Netherlands which unfortunately did not result in additional transplantations. This study was initiated to identify demographic factors influencing the potential success of uDCD programs. METHODS. Dutch resuscitation databases covering various demographic regions were analyzed for potential donors. The databases were compared with the uDCD implementation project and successful uDCD programs in Spain, France, and Russia. RESULTS. The resuscitation databases showed that 61% of all resuscitated patients were transferred to an emergency department. Age selection reduced this uDCD potential to 46% with only patients aged 18–65 years deemed eligible. Of these patients, 27% died in the emergency department. The urban region of Amsterdam showed the largest potential in absolute numbers (52 patients/y). Comparison with the uDCD implementation project showed large similarities in the percentage of potential donors; however, in absolute numbers, it showed a much smaller potential. Calculation of the potential per million persons and the extrapolation of the potential based on the international experience revealed the largest potential in urban regions. CONCLUSIONS. Implementation of a uDCD program should not only be based on the number of potential donors calculated from resuscitation databases. They show promising potential uDCD percentages for large rural regions and small urban regions; however, actual numbers per hospital are low, leading to insufficient exposure rates. It is, therefore, recommendable to limit uDCD programs to large urban regions

Uncontrolled donors with controlled reperfusion after sixty minutes of asystole: a novel reliable resource for kidney transplantation.

BACKGROUND: Organ shortage leads to usage of kidneys from donors after sudden cardiac death, or uncontrolled donors (UDCD). Ischemic injury due to cessation of circulation remains a crucial problem that limits adoption of UDCD. Our clinical investigation was to determine the applicability of kidneys obtained from UDCD and resuscitated by extracorporeal perfusion in situ after 60 minutes of asystole. METHODS: In 2009-2011, organ procurement service of St. Petersburg, obtained kidneys from 22 UDCD with critically expanded warm ischemic time (WIT). No patients were considered as potential organ donors initially. All donors died after sudden irreversible cardiac arrest. Mean WIT was 61.4±4.5 minutes. For kidney resuscitation, the subnormothermic extracorporeal abdominal perfusion with thrombolytics and leukocyte depletion was employed. Grafts were transplanted into 44 recipients. The outcomes of transplantation of resuscitated kidneys were compared to outcomes of 87 KTx from 74 brain death donors (BDDs). RESULTS: Immediate functioning of kidney grafts was observed in 21 of the 44 recipients, with no cases of primary non function. By the end of the first post-transplant year there was an acute rejection rate of 9.1% (4 episodes of rejection) in the UDCD group versus 14.2% (13 episodes of rejection) in the BDD group. The actual 1-year graft survival rate was 95.5% (n = 42) in UDCD group, and 94.6% (n = 87) in BDD group. Creatinine levels at the end of the first year were 0.116±0.008 and 0.115±0.004 mmol/l in UDCD and BDD groups, respectively. CONCLUSIONS: UDCD kidneys with critically expanded WIT could be succefully used for transplantation if in situ organ "resuscitation" perfusion is included into procurement protocol. The results of 1-year follow-up meet the generally accepted criteria for graft survival and function. In situ reperfusion may exert a therapeutic effect on grafts before procurement. This approach could substantially expand the organ donors' pool

1-year Kaplan-Meier survival for kidney recipients showed similar patterns for BDD and UDCD groups (<i>P</i><0.128, SE = 0.023).

<p>1-year Kaplan-Meier survival for kidney recipients showed similar patterns for BDD and UDCD groups (<i>P</i><0.128, SE = 0.023).</p

“Zero”-biopsy photomicrographs of specimen from the kidney grafts obtained at the end of the procurement procedure.

<p>Hematoxylin – Eosin and Shiff's staining. A1, A2 – the sample taken from an unsuccessful UDCD; B1,B2 – kidney biopsy performed in BDD; C1,C2 – the result of morphological investigation of specimen obtained from a successful UDCD. There are comments in the main text.</p

Recipient data of early post-surgery and 1-year outcomes.

1<p>IGF: immediate graft function.</p>2<p>DGF: delayed graft function.</p>3<p>eGFR: estimated glomerular filtration rate GFR Cockcroft = ((140−age) * mass (kg) [*0.85 if female])/72 * serum creatinine (mg/dl).</p>4<p>only 82 recipients have 1 years results.</p

Schematics of the perfusion procedure.

<p>The components of the perfusion circuit include: 1: venous reservoir; 2: mechanical perfusion module; 3: oxygenator; 4: leukocyte filter; 5: preservation solution bag; 6: source of oxygen; 7: arterial line of the perfusion circuit; 8: venous line; 9: surgical access to femoral vessels; 10: zone of isolated abdominal perfusion.</p

Demographics and clinical characteristics of the donors after cardiac death.

<p>Demographics and clinical characteristics of the donors after cardiac death.</p

1-year Kaplan-Meier survival for kidney graphs showed similar patterns for BDD and UDCD groups (<i>P</i><0.312, SE = 0.041).

<p>1-year Kaplan-Meier survival for kidney graphs showed similar patterns for BDD and UDCD groups (<i>P</i><0.312, SE = 0.041).</p