Variables Associated With Hyperkalemic Renal Tubular Acidosis in Solid Organ Transplant Recipients.

INTRODUCTION: The occurrence of hyperkalemic renal tubular acidosis (RTA) in the post-transplantation period is likely underestimated, and its identification remains important to offer adequate medical management. Transplant recipients frequently present with clinical and biological characteristics that may be associated with the occurrence of this complication. METHODS: This was a single-center retrospective study that compared transplanted patients with hyperkalemic RTA and a control group to identify variables associated with the occurrence of this complication. Fisher's exact test and the Mann-Whitney test, followed by multivariate logistic regression, were applied to test whether there was a significant association between hyperkalemic RTA and different variables. RESULTS: Kidney and heart transplant recipients were at greater risk of developing RTA than lung transplant recipients (p = 0.016). There was also a significant association between the development of RTA and kalemia (p < 0.01), chloremia (p < 0.01), and bicarbonatemia (p < 0.01). The significant impact of these last three variables was confirmed by the results of the multivariate logistic regression. Residual serum tacrolimus levels (p = 0.13) and creatinine levels (p = 0.17) of renal transplant patients were not significantly associated with hyperkalemic RTA. CONCLUSION: The type of transplanted organ, kalemia, chloremia, and bicarbonatemia were significantly associated with the occurrence of hyperkalemic RTA. This study calls into question certain approaches to managing this complication proposed in a number of case reports, such as reducing the target serum residual of tacrolimus or discontinuing trimethoprim-sulfamethoxazole (TMP-SMX) in favor of another antibiotic prophylactic agent, potentially exposing patients to graft rejection and opportunistic infections.

Tumor-Induced Osteomalacia: A Systematic Clinical Review of 895 Cases.

Tumor-induced osteomalacia (TIO) is a rare and largely underdiagnosed paraneoplastic condition. Previous reviews often reported incomplete data on clinical aspects, diagnosis or prognosis. The aim of this study was to present a systematic clinical review of all published cases of TIO. A search was conducted in Pubmed, Embase, Web of Science from inception until April 23rd, 2020. We selected case reports and case series of patients diagnosed with TIO, with information on tumor localization and serum phosphate concentration. Two reviewers independently extracted data on biochemical and clinical characteristics including bone involvement, tumor localization and treatment. 468 articles with 895 unique TIO cases were included. Median age was 46 years (range 9 months-90 years) and 58.3% were males. Hypophosphatemia and inappropriately low or normal 1,25-dihydroxyvitamin D levels, characteristic for TIO, were present in 98% of cases. Median tumor size was 2.7 cm (range 0.5 to 25.0 cm). Serum fibroblast growth factor 23 was related to tumor size (r = 0.344, P < 0.001). In 32% of the cases the tumor was detected by physical examination. Data on bone phenotype confirmed skeletal involvement: 62% of cases with BMD data had a T-score of the lumbar spine ≤ - 2.5 (n = 61/99) and a fracture was reported in at least 39% of all cases (n = 346/895). Diagnostic delay was longer than 2 years in more than 80% of cases. 10% were reported to be malignant at histology. In conclusion, TIO is a debilitating disease characterized by a long diagnostic delay leading to metabolic disturbances and skeletal impairment. Increasing awareness of TIO should decrease its diagnostic delay and the clinical consequences.

Management of sickle cell disease during pregnancy: experience in a third-level hospital and future recommendations.

OBJECTIVE: To describe the outcomes of sickle-cell disease in pregnancy according to the different treatments adopted before and during pregnancy and to propose a systematic approach to treat sickle-cell disease (SCD) during pregnancy. METHODS: A retrospective descriptive study compared pregnancy outcomes among women with SCD who stopped hydroxyurea (HU) once pregnant (Group 1), were never treated before and during pregnancy (Group 2) or were treated by HU before conception who received prophylactic transfusion during pregnancy (Group 3). For each group we recorded the population's characteristics and the transfusion-related, obstetrical, perinatal and SCD complications. RESULTS: We found 11 patients for group 1 (9/11 with at least 3 painful crises during the 12 months before conception), 4 for group 2 (3/4 with no sickle-cell complications during the year before pregnancy) and 2 for group 3 (one with previous multiorgan failure (MOF), one with previous stroke). No transfusion-related complication occurred. Group 1 and 2 developed SCD complications and a high number of acute transfusions and hospital admissions. Group 3 showed none of these complications, but one patient developed preeclampsia and preterm birth. Several obstetrical and perinatal complications occurred in group 1. CONCLUSION: Not treating sickle-cell during pregnancy increases maternal and perinatal morbidity, even in mildly affected women. All sickle-cell pregnancies should be treated, according to the treatment adopted before but also to patient's SCD-history. We propose chronic transfusion to women with previous stroke or MOF or already under transfusion program, and HU for severely and mildly affected patients, respectively from the second and third trimesters. Additional prospective studies are needed to validate the results of the proposed protocol.

Antithyroid Drug-Induced Lupus Erythematosus and Immunoglobulin A Deficiency.

BACKGROUND Antithyroid drugs, namely methimazole, are well-known causes of drug-induced lupus erythematosus. This is, however, an infrequent adverse effect. Selective Immunoglobulin A (IgA) deficiency, in contrast, is the most common primary immunodeficiency. Patients with IgA deficiency are at risk of developing infectious diseases, but also autoimmune diseases such as Grave's disease or systemic lupus erythematosus. CASE REPORT We report a case of methimazole-induced lupus erythematosus in a 32-year-old man with renal involvement and concomitant selective IgA deficiency. Symptoms promptly resolved after treatment with hydroxychloroquine and corticosteroids after discontinuation of methimazole. Lupus nephritis required treatment with cyclophosphamide followed by maintenance therapy with mycophenolate mofetil. CONCLUSIONS Drug-induced lupus erythematosus usually develops after a few months or years of exposure to the causative agent. No specific symptoms exist. The diagnosis is not based on particular specific tests, but relies on a set of arguments evoking the role of the medication inducing the condition. The first step in treatment is to stop the causative drug. The therapeutic management of the various manifestations does not differ from that of idiopathic systemic lupus erythematosus. We briefly discuss the relationship between drug-induced lupus erythematosus, Grave's disease, and IgA deficiency, and suggest that IgA deficiency may act as a potential risk factor. Testing for IgA deficiency could be helpful in patients being treated with drugs known to be associated with drug-induced lupus erythematosus.

Significance of Hypernatremia Due to SARS-CoV-2 Associated ARDS in Critically Ill Patients.

Background and Objectives: SARS-CoV-2-induced ARDS is a new entity that should be characterized as it appears to be different from standard ARDS. Hypernatremia is a biological alteration that seems to occur very often in this population without any clear cause. The present study aims to clarify the possible causes of hypernatremia and evaluate its impact on patient outcome. Patients and Methods: We conducted a retrospective one-day prevalence study in 2 intensive care units, which only treated COVID-19 patients with moderate to severe ARDS. We measured blood and urine electrolytes in all the patients. Patients with chronic renal failure or renal replacement therapy were excluded from the study. Hypernatremia was defined as plasma sodium levels above 145 mmol/L. Results: Inclusion criteria were met in 17 out of 24 patients. Hypernatremia was present in 52% patients. All had a natriuresis higher than 20 mmol/L and a urine osmolality above 600 mOsm/L. Hypernatremia was acquired in ICU as all the patients had a normal serum sodium level at admission. Conclusion: The incidence of hypernatremia was elevated and appears to be linked to significant insensible water losses. This should trigger us to optimize the maintenance fluid therapy in critically ill patients with SARS-CoV-2-induced ARDS.

A fatal case of cutaneous adverse drug-induced toxic epidermal necrolysis associated with severe rhabdomyolysis.

Toxic epidermal necrolysis represents an immunologic reaction to a foreign antigen and is most often caused by drugs. Atorvastatin, a blood cholesterol-lowering agent, is a recognized cause of rhabdomyolysis; while naproxen, a widely used nonsteroidal anti-inflammatory drug, is a known cause of photo-induced skin lesions. We report the first fatal case of drug-induced toxic epidermal necrolysis associated with severe muscle necrosis due to the use of a nonsteroidal anti-inflammatory drug and a statin with very high levels of creatine phosphokinase leading to acute kidney injury, disseminated intravascular coagulation, and complete skin necrosis leading to death.