Human Herpesvirus-8 (HHV-8)-Positive Human Immunodeficiency Virus (HIV)-Negative Multicentric Castleman Disease With a Fulminant Course.

Multicentric Castleman disease (MCD) is a poorly understood, heterogeneous lymphoproliferative disorder with benign hyperplastic lymph nodes and systemic inflammatory symptoms. Human herpesvirus-8 (HHV-8) may be associated with MCD, whether or not the patient is infected with the human immunodeficiency virus (HIV). A 74-year-old man presented with anaemia, thrombocytopenia and bilateral axillary adenomegaly of unknown origin. The patient was admitted to the hospital two years ago with clinical signs of weight loss, asthenia, anorexia and a maculopapular rash on the trunk and back. Blood analysis showed pancytopenia (haemoglobin 7.7 g/dL, leucocytes 2.55 x 109/L and platelets 41 x 109/L), elevated acute phase reactants (such as C-reactive protein, erythrocyte sedimentation rate, ferritin and fibrinogen), hypoalbuminemia and hypergammaglobulinemia, and HIV serology was negative. Thoracic, abdominal and pelvic axial tomography showed generalised lymphadenopathy. The bone marrow biopsy showed only reactive changes, and the histology of an excisional biopsy of the adenopathy was consistent with the plasmablastic variant of MCD associated with HHV-8. The HHV-8 viral load was 3.8 x 104 copies/mL (4.5 log). He was started on prednisolone 60 mg/day and rituximab. He had a poor response to therapy, despite a reduction in the HHV-8 viral load, with clinical deterioration, transfusion-dependent anaemia and progression to multi-organ dysfunction leading to death three weeks after starting treatment. Our patient had a fulminant course of MCD despite treatment with rituximab. Further studies are needed to validate the different treatment modalities and to better understand the prognosis of this disease.

Gitelman Syndrome: A Case Report.

Gitelman syndrome is a rare hereditary tubulopathy characterized by hypokalemic metabolic alkalosis, hypomagnesemia, and hypocalciuria. In this case report, we describe a 21-year-old male who presented with myalgias, asthenia, general muscle weakness, and hypokalemia after receiving oral potassium supplementation for six months. Additional biochemical studies showed hypomagnesemia, metabolic alkalosis, and increased urinary potassium and magnesium excretion. Calcium urinary excretion was within the normal range, but 25-hydroxycholecalciferol levels were low. Systolic arterial hypertension was found, probably reflecting chronic hyperreninemic hyperaldosteronism. Genetic testing for SCL12A3 mutations identified a pathogenic variant in homozygosity, which confirmed the Gitelman syndrome diagnosis. Treatment with chronic potassium and magnesium oral supplementation was started, as well as eplerenone and amiloride, with sustained correction of hypokalemia and hypomagnesemia.

Spontaneous Coronary Artery Dissection in a Patient With Cushing's Disease.

Spontaneous coronary artery dissection (SCAD) is an uncommon cause of acute myocardial infarction, caused by a non-traumatic and non-iatrogenic separation of the coronary arterial wall, especially amongst young women with no conventional cardiovascular risk factors. We describe the case of a 46-year-old woman with a past medical history of Cushing's disease, treated surgically, who presented with SCAD. Cushing's disease is not considered a traditional risk factor for SCAD. As there are reported cases of arterial dissections associated with this entity and common causes of SCAD were excluded, we hypothesized that the patient's past medical history may have contributed to SCAD. To the best of our knowledge, there are no other reported cases of Cushing's disease-associated SCAD.

Endocarditis by Streptococcus pasteurianus.

The diagnosis of infective endocarditis is challenging because it has a variable clinical presentation and nonspecific symptoms and can present in different forms, especially when an unusual etiological agent is involved. We present the case of a female in her 70s admitted to the hospital with a medical history of bicytopenia, severe aortic stenosis, and rheumatoid arthritis. She had several consultations during which she presented with asthenia and general malaise. A septic screen test was performed that would determine that Streptococcus pasteurianus was present in a blood culture (BC), which was not valued. About three months later, she was hospitalized. In the first 24 hours of admission, the septic screen test was repeated and Streptococcus pasteurianus was isolated in BC. Splenic infarctions and transthoracic echocardiography suggested probable endocarditis, which was confirmed with transesophageal echocardiography. She underwent surgical intervention to remove the perivalvular abscess and replace the aortic prosthesis.

Malignant Hypercalcemia Induced by the Ectopic Production of Intact Parathyroid Hormone (PTH).

Malignant hypercalcemia is a common finding in patients with advanced cancer, involving mechanisms like tumor secretion of parathyroid hormone (PTH)-related protein, osteolytic metastases, and tumor production of calcitriol. Although rare, hypercalcemia induced by ectopic tumoral secretion of PTH can be an additional mechanism. Here we present an 84-year-old male patient who was admitted to the hospital with a non-productive cough, anorexia, and a single episode of small-volume hemoptysis. He was diagnosed with stage T4N3M1c left lung small cell carcinoma, and laboratory tests were remarkable for elevated ionized calcium as well as elevated serum intact PTH. A parathyroid 99mTc sestamibi scan showed no changes, suggesting ectopic production of PTH. Being a rare event, malignant hypercalcemia from intact PTH ectopic production should be considered in these patients.

Gastric Metastasis From Lung Adenocarcinoma: An Uncommon Presentation.

Gastric metastasis is an infrequent occurrence, especially when derived from lung adenocarcinomas. They can grossly resemble advanced gastric cancer and require comprehensive evaluations of the patients and their symptoms. Here, we present the case of a 71-year-old patient admitted to our hospital due to intense, cramping abdominal pain. He had been previously diagnosed with a right lower lobe adenocarcinoma of the lung, which was treated in the previous year with chemotherapy and radiotherapy with good clinical response. The abdominal CT scan and the esophagogastroduodenoscopy showed a gastric infiltrating lesion resembling advanced gastric cancer. However, the biopsy showed malignant epithelial neoplasia with features of adenocarcinoma of pulmonary origin. Even though they are an uncommon finding, gastrointestinal metastases may be life-threatening and should be diagnosed as soon as possible, as the advent of molecular studies and new therapies may result in better survival rates.

A Straining Heart: Transthyretin Amyloidosis as a Cause of Heart Failure.

Cardiac amyloidosis is a disease caused by the deposition of amyloid fibrils in the extracellular space of the heart, most often by immunoglobulin light chains or by transthyretin. It is often underdiagnosed because the signs and symptoms are nonspecific or due to the false perception that the diagnosis always requires an endomyocardial biopsy. Transthyretin amyloidosis is being increasingly recognized as a cause of heart failure, particularly in patients with heart failure with preserved ejection fraction (HFpEF). We present the clinical case of an 86-year-old man whose diagnosis was based on signs and symptoms compatible with cardiac amyloidosis and in which imaging performed a preponderant role. This case reminds clinicians to consider the diagnosis in older patients with HFpEF, left ventricular hypertrophy and rhythm disturbances. It highlights the importance of evaluating global longitudinal strain (GLS) in a standard echocardiographic evaluation.

Kimura's Disease: A Literature Review Based on a Clinical Case.

Kimura's disease (KD) was first described in 1937. It is a rare, benign, and chronic immune-mediated inflammatory disorder affecting the subcutaneous tissue, salivary glands, and lymph nodes. The disease is more common in the second to third decades of life in middle-aged Southeast Asian countries. The cause of Kimura's disease remains unknown; some authors believe it is related to an autoimmune or delayed-type hypersensitivity reaction. It commonly presents as a solitary painless lymph node in the head and neck or generalized lymphadenopathy (67%-100%) associated with peripheral eosinophilia and elevated IgE levels. Renal involvement may occur in some patients. Diagnosis is made by histology. A 21-year-old Caucasian man with no relevant medical history presented with a non-tender swelling of the left hemiface without other associated symptoms. Laboratory investigations revealed a leukocyte count with eosinophilia (2.29×10^9/L- 26.5%) and elevated total IgE and IgG4. He had no renal dysfunction. He underwent surgical resection of the lesion in the jugal, infraorbital, and left nasolabial regions, and the anatomopathological examination revealed the characteristics of Kimura's disease. Three months after surgery, an MRI showed an increase in the size of the mass, and he was started on corticosteroids. Six months after surgery, he presented with a slight increase in the size of the mass and was started on Ciclosporin, which allowed a progressive reduction in the dose of corticosteroid with evidence of a progressive reduction in swelling. Ciclosporin was stopped due to toxic serum levels, and he was started on mycophenolate mofetil. The dose was increased because of the increase in facial mass; on mycophenolate mofetil 2500mg/day, the patient remains stable. KD is a chronic disorder of unknown etiology that mainly affects young people in Southeast Asia and is rare in Western countries, as in the case of this young man. Studies have shown no significant differences in region or race, complications, multiplicity, laterality, anatomical distribution, maximum size, eosinophil count, or IgE levels between age groups. There is no consensus on the optimal treatment for KD; several treatments have been used, including surgery, systemic corticosteroids, immunosuppressants, and radiation. Due to the tendency to relapse and the rarity of the disease, there is no consensus on treatment options for relapse.

Flash Pulmonary Edema: A Rare Presentation of Graves' Disease.

Graves' disease is the most common cause of hyperthyroidism. It has an autoimmune basis with the activating thyrotropin-receptor antibodies inducing thyroid hormone overproduction. The most common manifestations of hyperthyroidism are weight loss, fatigue, heat intolerance, tremor, and palpitations, but there are several other symptoms and signs associated with this condition. We report a case of a young woman who presented in the emergency room with acute onset of cough with mild hemoptysis and dyspnea at rest. She reported one month of insomnia, palpitations, and anxiety. The diagnostic investigation leads to the diagnosis of Graves' disease in thyrotoxic crisis presenting with flash pulmonary edema. Therapy with propranolol and methimazole was instituted with remarkable clinical improvement.

Antinuclear Antibody (ANA) and Anti-Mi-2-Alpha Positive Dermatomyositis Hinting a Cancer Diagnosis.

Dermatomyositis (DM) is a relatively uncommon inflammatory myopathy that has been linked to cancer. We report the case of an 81-year-old woman with cecum adenocarcinoma presenting with antinuclear antibody (ANA) and anti-Mi-2-alpha antibody-positive DM. The patient complained of anorexia, symmetric proximal muscle weakness and skin rash and presented with elevated muscle enzymes. A skin and muscle biopsy supported the diagnosis of DM as did the limbs magnetic resonance imaging (MRI) and electromyography. A diagnosis of localized adenocarcinoma of the cecum was made through colonoscopy and the patient was successfully surgically managed, with decreasing muscle enzymes at discharge and gradual recovery of muscle strength. The presence of both ANA and anti-Mi-2 autoantibodies has classically been described as comprising a better prognosis with a lower risk of underlying malignancy. This case highlights the importance of pursuing a cancer diagnosis in elderly patients presenting with DM even in presence of less predisposing immunological profiles.

Pulmonary Adenocarcinoma Presenting as Acute Heart Failure and Hypereosinophilia.

Hypereosinophilia is a serum eosinophil count of over 1,500 eosinophils/µL. It is an uncommon laboratory finding, and it can be asymptomatic or associated with organ damage, in which case it is referred to as hypereosinophilic syndrome. It can be primary, when the expansion of eosinophils occurs in the setting of a hematological neoplasm, or secondary, when it is caused by an external stimulus, such as a parasitic infection or a solid neoplasm. We present a case of hypereosinophilia diagnosed in a patient presenting with acute heart failure initially attributed to coronary disease and alcohol consumption. Due to persisting eosinophilia with no apparent cause, eventually reaching more than 41,000 eosinophils/µL, a full-body scan was performed, showing the presence of a peri-hilar mass partially obstructing the left main bronchus and multiple lesions in the liver and thoracic vertebrae. The liver biopsy revealed metastatic non-small cell lung carcinoma. Around the time the biopsy was performed, the patient began complaining of new-onset chest paint, and the diagnoses of pulmonary embolism and later lung abscess were made, the reasons why he had no conditions to begin chemotherapy. The medical condition of the patient deteriorated in the next few days, and the patient died six months after the initial diagnosis of hypereosinophilia.

Deep Venous Thrombosis and Pulmonary Thromboembolism Associated With Retroperitoneal Hematoma in a Patient With Ehlers-Danlos Syndrome Type VI.

Ehlers-Danlos Syndrome (EDS) is a group of genetic diseases of the connective tissue, which is rare and is characterized by joint hypermobility, tissue, and vascular fragility. We present the case of a 38-year-old woman with a known diagnosis of EDS type VI who came to the emergency room, complaining of sudden dyspnea in the context of abdominal pain and pain in the left lower limb with one week of evolution. Computed axial tomography showed the presence of bilateral pulmonary thromboembolism, iliofemoral thrombosis, and a retroperitoneal hematoma. Anticoagulation was started and the patient was admitted to the intermediate care ward. This case highlights the need to know the implications of increased vascular fragility in type VI EDS, with potentially serious consequences. Underlying is the inevitable need for judicious consideration of the decision to undergo anticoagulation, in the context of a retroperitoneal hemorrhagic event and pulmonary thromboembolism.

Simultaneous Systemic Lupus Erythematosus Flare and Disseminated Tuberculosis: Balancing Anti-Mycobacterial and Autoimmune Treatments.

Here, we report the case of a 53-year-old man with suspected autoimmune arthritis on low-dose corticosteroid therapy. He was recently hospitalized due to presumed bacterial pneumonia and a seizure episode attributed to high fever. His condition deteriorated after discharge, and he presented to our institution with a persistent cough, weight loss, skin rash, arthralgias, fever, and altered mental status. The investigation led to the simultaneous diagnosis of a systemic lupus erythematosus (SLE) flare and disseminated tuberculosis (TB), both pulmonary and intracranial. Proteinuria and peripheral edema were identified, suggesting renal involvement of SLE. Anti-mycobacterial drugs and high-dose corticosteroid therapy were initiated. Given the risk of starting other immunosuppressive drugs in the presence of intracranial TB, in a patient with stable renal function and a significant decrease in proteinuria with corticosteroids and supportive therapy alone, renal biopsy was postponed. Prednisolone was progressively tapered down during the next six months, always maintaining anti-mycobacterial therapy, which resulted in a second SLE flare and the need to increase corticosteroids again. At this time, a renal biopsy was performed, showing class II lupus nephritis and confirming the diagnosis of SLE. After one year of anti-mycobacterial therapy with complete resolution of cerebral and pulmonary TB lesions, we chose to initiate mycophenolate mofetil as an immunosuppressive steroid-sparing agent with increased SLE control, allowing for corticosteroid reduction.

Neurolisteriosis: The Importance of a Prompt Diagnosis.

Immune thrombocytopenia (ITP) is a prevalent disease that may need immunosuppressant treatment, which increases the risk of an opportunistic infection. We present the case of a woman with corticosteroid-resistant ITP who was electively admitted to the hospital to initiate second-line treatment. On the second day, she presented with a high fever and altered mental status, with no lesions on the cerebral tomography and inconclusive cerebrospinal fluid analysis. Nonetheless, she was promptly started on empiric antibiotics for meningitis which were then adjusted for Listeria monocytogenes, isolated in blood culture. The cerebral magnetic resonance showed signs of cerebritis and pyogenic foci. The patient was discharged after 73 days of treatment, asymptomatic and with no neurological sequelae. The mortality rate of neurolisteriosis can be as high as 50%. The median time between the initial symptoms and their detection is seven days, with many patients already developing cerebral abscesses. The favorable outcome of this patient was due to the precocious detection and start of the treatment.

Evaluation of slurry sampling preparation of enteral nutrition formulations for multielement determination using inductively coupled plasma optical emission spectrometry.

An analytical procedure for the multielement determination in enteral nutrition formulations employing slurry sampling and inductively coupled plasma optical emission spectrometry (ICP OES) is proposed. A two-level full-factorial design was applied to assess the influence of the presence of stabilizing agents (HNO3, Triton X-100 and ethanol) on the composition of the slurry. Multiple response was established as a dependent variable. The experimental conditions for the preparation of the slurry were: 2.0 mL of sample and 8.0 mL of 10% (v/v) HNO3. The limits of detection (LOD) were 5; 9; and 10 µg L-1 for Cu, Fe, Zn, respectively. For P, and K, the LOD were 8 and 24 mg L-1, respectively. The method was applied for the analysis of three enteral nutrition formulation samples and the obtained concentrations ranges were (in mg L-1): 0.41-0.43 (Cu), 2.0-2.9 (Fe), 1.7-3.1 (Zn), 682-1409 (K), and 217-344 (P).

Determination of Cu, Ni, Mn and Zn in diesel oil samples using energy dispersive X-ray fluorescence spectrometry after solid phase extraction using sisal fiber.

As a natural adsorbent, sisal (agave sisalana) fibers were used to extract Cu, Ni, Mn, and Zn from diesel oil samples for posterior determination (i.e., direct analytical measurements on the solid support) of the analytes by energy dispersive X-ray fluorescence spectrometry (EDXRF). In the proposed procedure, 0.2 g of sisal fiber was directly added to 5.0 mL of diesel oil contained in a glass tube. After 5 min of contact time, the mixture was filtered, and the collected fibers were oven-dried for 30 min at 70 °C. After drying, the analytes were quantified directly by EDXRF using the sisal fibers as a solid support. The calibration curves showed linear concentration ranges of 0.09-1.00, 0.12-1.00, 0.09-1.00, 0.06-1.0 µg g-1 for Cu, Ni, Mn, and Zn, respectively. The limits of detection (LOD) for Cu, Ni, Mn, and Zn were 0.03, 0.04, 0.03, and 0.02 µg g-1, respectively. The repeatability, evaluated by performing ten measurements at a concentration of 0.50 µg g-1 for each metal, with the results expressed in terms of the relative standard deviation (RSD), was 3.2, 6.5, 6.8, and 6.1% for Cu, Ni, Mn, and Zn, respectively. The results obtained by the proposed method were compared with the results obtained by a comparative method using inductively coupled plasma optical emission spectrometry, and both results showed good agreement. The proposed method was applied for Ni, Cu, Mn, and Zn determination in diesel oil samples collected from different gas stations.

Report of Two Cases of Acquired Idiopathic Haemophilia.

Acquired haemophilia is a rare haemorrhagic dyscrasia caused by autoantibodies against coagulation factors, most commonly factor VIII (FVIII). Even though about half of the cases are classified as idiopathic, acquired haemophilia is more common in the elderly and/or in individuals diagnosed with other immunogenic conditions such as malignancies, autoimmune diseases, or during puerperium. It can be life-threatening, presenting more frequently with major bleeding. We report two cases of acquired haemophilia classified as idiopathic in middle-aged patients with no predisposing factors identified during the diagnostic approach: their disease's progression and complications, choice of treatment, and why and when to change it.

Determination of Cu, Ni, Mn, and Pb in diesel oil samples using reversed-phase vortex-assisted liquid-liquid microextraction associated with energy dispersive X-ray fluorescence spectrometry.

A method was developed based on reversed-phase vortex-assisted liquid-liquid microextraction (RP-VALLME) combined with energy dispersive X-ray fluorescence (EDXRF) spectrometry for the determination of Cu, Mn, Ni, and Pb in diesel oil samples. In this procedure, a nitric acid solution was used as the extraction phase to isolate analytes from organic samples. After a centrifugation step, the aqueous phase was added dropwise to a filter paper disc for EDXRF determinations. The following variables were optimized: type of extraction phase solution, concentration of the extraction phase, stirring time, and sample volume. Some instrumental parameters were also evaluated: atmospheric condition, irradiation energy, and irradiation time. Using 100 µL of a 0.075 mol L-1 nitric acid solution as the extraction phase for a sample volume of 5.0 mL and a stirring time of 45 s, the limits of detection were 14, 8, 10, and 7 µg L-1 for Cu, Mn, Ni, and Pb, respectively. The enrichment factors obtained were 34 (Cu), 62 (Mn), 59 (Ni), and 64 (Pb). The precisions, expressed as relative standard deviations (RSDs, %), were calculated from ten replications of the experiment under optimized conditions using standard solutions containing 200 µg L-1 and 400 µg L-1 of all four analytes and ranged between 2.1 and 6.4%. The results of recovery tests ranged from 87 to 112%. The proposed procedure was efficiently applied to the determination of the four analytes in diesel oil samples. The results were compared with those obtained by inductively coupled plasma optical emission spectrometry (ICP-OES) after sample digestion, and no significant differences were found.

Multivariate optimization of ultrasound-assisted extraction using Doehlert matrix for simultaneous determination of Fe and Ni in vegetable oils by high-resolution continuum source graphite furnace atomic absorption spectrometry.

A method for simultaneous determination of Fe (232.036 nm) and Ni (232.195 nm) in vegetable oil samples by high-resolution continuum source graphite furnace atomic absorption spectrometry (HR-CS GF AAS) after an acid extraction of the analytes is proposed. In the extraction step, hydrochloric, nitric and acetic acid solutions were tested. The optimization of the procedure was performed by applying Doehlert matrix, and multiple response was used for simultaneous evaluation of the performance of the extraction. The optimum conditions were: extraction time of 17 min, extraction temperature of 39 °C and sonication amplitude of 42%, employing 0.5 mol L-1 HCl as the extracting solvent. The limits of quantification were 60 and 160 ng g-1 for Fe and Ni, respectively. The method was applied to the analysis of vegetable oil samples and the results were compared with a method employing inductively coupled plasma optical emission spectrometry (ICP OES).