Armando Carreira

Acute lupus hemophagocytic syndrome: report of a case

Hemophagocytic Syndrome is a clinical condition characterized by the activation of either macrophages or histiocytes with a prominent hemophagocytosis feature in the bone marrow and other reticuloendothelial systems. It leads to the phagocytosis of erythrocytes, leukocytes, platelets and their precursors. The presence of hemophagocytosis can be associated to infections, malignancies, autoimmune diseases, drugs and a variety of other medical conditions. We report a case of a previously healthy 36 year-old woman that developed hemophagocytosis at the same time that fulfilled diagnostic criteria for systemic lupus erythematosus. Lupus related hemophagocytic syndrome is a rare and potentially fatal entity. It offers significant differential diagnosis challenges and requires urgent therapeutic intervention. There are only few cases reported in the literature. However, much is still needed in order to better understand its causes, all the immunopathogenic mechanisms, as well as its clinical and therapeutic aspects.

Acute kidney injury by cantharidin poisoning following a silly bet on an ugly beetle.

Cantharidin is a poisonous substance secreted by blister beetles, including the ‘Spanish fly’. Historically, cantharidin was used as an aphrodisiac, vesicant and abortifacient. Symptoms of poisoning include gastrointestinal and genitourinary mucosal irritation along with renal dysfunction. We present the case of a reckless 23-year-old soldier who accepted the challenge of eating a beetle (Berberomeloe majalis). Six hours later he was admitted to the emergency room with abdominal pain, dysuria, gross haematuria with clots, hypotension, fever and renal insufficiency. With intravenous fluid therapy, he recovered clinically. Laboratory parameters returned to normal within 1 week.

Random spot urine protein/creatinine ratio: a reliable method for monitoring lupus nephritis?

Background Lupus nephritis (LN) is a common and severe manifestation of systemic lupus erythematosus (SLE) that can lead to end-stage renal disease. According to the Kidney Disease Outcomes Global Improving clinical Guidelines for Glomerulonephritis, spot urine protein/creatinine (P/C) ratio should be used for monitoring LN. However, some reports write that the random spot urine P/C ratio is unreliable in monitoring proteinuria in SLE glomerulonephritis patients. The aim of this study was to evaluate the agreement of these two assay methods. Methods The prospective observational study was performed. Fifty-three paired (total 106) spot and 24-h urine collections were evaluated. Statistical analysis: SPSS 20.0. Results Paired samples t-test did not reveal significant differences between the two-paired assay methods (spot P/C ratio versus 24-h proteinuria and 24-h P/C ratio) and a statistically significant correlation was observed between them: Pearsons coefficient of 0.847 (P < 0.001) and 0.863 (P < 0.001), respectively. However, after stratifying by degrees of proteinuria, a poor correlation was found in the range of <500 mg/day and only 26.6% of 24-h P/C ratio was explained by the spot P/C ratio. Adding to this, for proteinuria range between 500 and 1000 mg/day, there was no correlation (Pearsons −0.098; P > 0.05). In fact, only 1% of 24-h measurements could be explained by the spot P/C ratio. Conclusions Our study demonstrated a good correlation between 24-h proteinuria and random P/C ratio among patients with LN. However, this correlation was poor for proteinuria under 500 mg/day and did not exist in a range between 500 and 1000 mg/day. This finding is of greater importance because this range is quite common in patients with LN remission. Until further clarification, to the best of our knowledge, we maintain reluctant to completely substitute the 24-h collection by the P/C ratio especially when a renal flare is suspected, or before any change in therapy.

Gitelman syndrome with hiponatremia, a rare presentation.

CASE REPORT A 34 year old caucasian woman with no prior medical presented with severe hypokalemia; hypomagnesemia and mild hyponatremia. Her past medical and family history were unremarkable. She was on no medication and denied any symptoms, unless for occasionally muscle cramps. Water intake ≥3L/ day. She was normotensive, no edemas and normal urine output. The review of systems was otherwise negative.

Sodium and Potassium Intake, the First Step to Control Arterial Hypertension

In Portugal, Hypertension affects 43% of adults. Salt intake reduction and potassium increase are recommended for prevention and treatment of hypertension. This study was designed to determine how dietary sodium and potassium affects blood pressure (BP). Cross-sectional study of 41 patients was made in Centro Hospitalar de Coimbra. Patients BP, as well as their 24-hour urinary excretion of sodium (UNa) and potassium (UK); UNa/UK ratio was calculated. There were highly significant differences for both diastolic BP (DBP) and sistolic BP (SBP) means according to 24h-UNa and UNa/UK values (p 0.001). There was a highly correlation between BP and 24h-UNa, as well as, UNa/UK; stronger with this latest factor. Among BP values, SBP was strongly influenced by 24h-UNa and UNa/UK than DBP (Pearson 0.608 > 0.578 and 0.675 > 0.633, respectively). So, increased potassium intake should be considered as a recommendation for prevention and treatment of hypertension, especially in those who are unable to reduce their intake of sodium.

Subcapsular liver hematoma as a complication of an atypical hemolytic uremic syndrome.

Atypical hemolytic uremic syndrome (aHUS) is a rare, lifethreatening systemic inflammatory disease that presents classically with microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury.1 Extra renal manifestations are observed in 20% of patients.2 A 42-year-old woman with unremarkable past medical history presented in our hospital reporting a 6-day history of headache, nausea and vomiting. Physical examination showed hypertension (220/120 mmHg), cutaneous pallor and moderate lower limbs edema. Laboratory results revealed anemia (hemoglobin 8.4 g/dl), thrombocytopenia (76,000/ l), severe azotemia (urea 16.9 mmol/l, creatinine 448.8 mol/l), schistocytosis, a negative Coombs test, low blood haptoglobin (<0.07 g/L) and high lactate dehydrogenase levels (1234 U/l). Renal ultrasonography was normal. Blood pressure was hardly controlled with oral medication. A diagnosis of acute thrombotic microangiopathy (ATM) was made and daily plasma exchange (PEX) was started. Investigations for secondary causes of ATM (pregnancy, auto-immune disease, malignancy, drug-induced), infectioninduced HUS and thrombotic thrombocytopenic purpura were normal. A presumptive diagnosis of aHUS was made and the administrative process of Eculizumab acquisition was initiated. On the 15th day of admission (D15), hemodialysis was started due to progressive renal failure. All attempts to stop PEX resulted in increased hemolytic activity, forcing to maintain 3 sessions a week. On the D72, after performing 44 PEX sessions, we were still waiting for Eculizumab acquisition. Attending to clinical and analytical stability (Fig. 1), the patient was discharged home to continue hemodialysis and PEX three times a week as an outpatient. Four days after discharge, she was admitted in the emergency room with a 12-hour history of severe right upper quadrant pain and vomiting without history of trauma. Laboratory results revealed stabilized hemoglobin (11.1 g/dl) and both normal platelet count (157,000/mm3) and coagulation tests. Abdominal ultrasonography and Computed Tomography scan showed a large subcapsular liver hematoma (SLH) (Fig. 2). She was transferred to the Intensive Care Unit (ICU). Attending to hemodynamic stability, a conservative approach was attempted. PEX was suspended to prevent increased