Hepcidin and Ferritin Levels in Fever of Unknown Origin: Is There a New Biomarker?

Background and objectives: Significantly elevated serum ferritin levels are associated with both iron overload and some inflammatory conditions. Hepcidin is a protein that interferes with iron absorption in inflammatory states and acts as an acute-phase reactant. Materials and methods: Here we report the case a 33-year-old patient who presented with high fever, skin lesions and arthralgia lasting for 2 weeks. His ferritin level was 13,800 µg/l and his hepcidin level was 61 ng/dl. Results: The final diagnosis was adult onset Still's disease. The condition evolved satisfactorily with steroid treatment, but after several weeks the patient presented with an unexpected recurrence. Conclusions: Hepcidin is a good inflammatory marker that could be useful in the differential diagnosis of hyperferritinaemia.

Successful Treatment Using Simeprevir, Sofosbuvir and Rituximab of a Severe Form of Hepatitis C Virus-related Mixed Cryoglobulinemia with Cardiac Involvement

Numerous extrahepatic manifestations have been reported in hepatitis C virus (HCV) infection, particularly mixed cryoglobulinemia (MC). MC generally responds to clearance of HCV under pegylated-interferon plus ribavirin treatment. New direct-acting antiviral agents have been licensed for HCV under different combinations but have not been studied in severe forms of MC. Here, we present a case report describing a life-threatening form of MC with multivisceral involvement, which was successfully treated with concomitant rituximab, sofosbuvir and simeprevir. In light of the rapid clinical remission associated with sustained virological response and the excellent side-effect profile, this treatment should be considered as a first-line therapy in severe forms of MC.

Anti-Signal Recognition Particle Myopathy in a Geriatric Patient

Anti-signal recognition particle (SRP) myopathy is a rare idiopathic inflammatory myositis that usually affects middle-age women, and is characterized by rapidly progressive proximal and symmetrical muscle weakness, elevated creatine kinase levels, severe necrotizing immune-mediated myopathy, presence of anti-SRP autoantibodies and poor response to steroid therapy. We report a geriatric case of a previously independent patient, presenting with slow onset of proximal paraparesis, myalgia and severe gait impairment. The patient was treated with steroid and azathioprine, with laboratory and pain response but modest muscle strength improvement. The clinical presentation of this unusual patient was atypical, which hampered the correct diagnosis.

Strongyloides stercoralis - a Legacy from the Past

The authors present the case of a 65-year-old Caucasian man who had previously lived in Guinea-Bissau. The patient was diagnosed in Portugal with an anti-neutrophil cytoplasmic antibody-associated vasculitis and started on corticosteroids plus cyclophosphamide, with clinical improvement. Some months later, his general status deteriorated, iatrogenic bicytopenia developed and immunosuppressive drugs were tapered. Microbiological tests identified numerous larvae and eggs of Strongyloides stercoralis in various biological samples, and a diagnosis of hyperinfection syndrome was established. The patient was started on antihelminthic drugs but developed septic shock and died. This case highlights a rare and severe complication of immunosuppression in developed countries.

An Unexpected Cause of Marked Weight Loss Associated with Vomiting in an Adult Man: Gastric Phytobeozar

Objectives: We present the case of an edentulous 47-year-old farmer referred to our Department of Internal Medicine because of postprandial vomiting, hyporexia, asthenia and weight loss. He ate a mostly vegetarian diet. Materials and methods: An oesophagogastroduodenoscopy revealed the presence of a phytobezoar at the level of the fundus and body of the stomach. Endoscopic fragmentation and removal of the phytobezoar were unsuccessful and the patient had to undergo open surgery. Results: Recovery was uneventful and free of complications. Conclusion: Phytobezoars should be taken into account in the differential diagnosis of unexplained vomiting and weight loss.

Computed Tomography Perfusion Can Guide Endovascular Therapy in Bilateral Carotid Artery Dissection

Carotid artery dissection (CAD) is a major cause of stroke in those under age 45, accounting for around 20% of ischaemic events[1,2]. In the absence of known connective tissue disorders, most dissections are traumatic[2]. First-line management is comprised of antiplatelet or anticoagulation therapy, but many traumatic dissections progress despite this and carry the risk of long-term complications from embolism or stenosis[3]. We report a case of traumatic bilateral carotid dissection leading to progressive neurological symptoms and hypoperfusion on computed tomography perfusion (CTP), despite escalation in anticoagulation, which led to emergency carotid stenting.

Acute Transient Stress Induced Adrenal Hypertrophy and Adrenal Medullary Hyperactivity

Objectives: Adrenal gland hypertrophy can be related to acute stress with abnormal adrenal function tests. It may not always need treatment. Material and methods: An acute presentation of adrenal gland hypertrophy following an abdominal emergency, with subsequent hypoadrenalism was investigated. Results: Adrenal medullary and cortical function fully recovered without treatment. Conclusions: We postulate that the adrenal glands became enlarged and hypertrophied during an acute stress event, possibly caused by acute adrenal medullary hypersecretion and subsequent cortical hyposecretion. A wait and watch policy should be followed if no other clinical symptoms and signs of adrenal disease are present. CT scan remains an important diagnostic tool.

Granulomatosis with Polyangiitis (Wegener Granulomatosis) Mimicking Infective Endocarditis

Introduction Infective endocarditis (IE) has been reported to mimic granulomatosis with polyangiitis (GPA) and to test positive to antineutrophil cytoplasmic antibodies (ANCA), which may lead to a misdiagnosis and inappropriate treatment. Case presentation We report a case of a 59-year-old man admitted for purpura, gangrenous digital infarcts and glomerulonephritis. The diagnosis of IE was initially considered on the basis of heart murmur and two positive haemocultures to corynebacterium. Ineffectiveness of antimicrobial therapy and further neurological and nasal manifestations supported the diagnosis of GPA. Conclusions IE should be ruled out before initiation of immunosuppressive treatment. If the disease progresses despite antimicrobial treatment, vascular diseases should be rapidly taken into account in differential diagnosis and treated early to avoid fatal complications.

Large Morgagni Hernia in an Adult Patient

A 70-year-old woman presented to the emergency department with symptoms of a lower respiratory infection. A chest x-ray showed enlargement of the mediastinal space. The patient was admitted with a respiratory tract infection and started on antibiotic treatment. A computed tomography (CT) scan of the thorax revealed a large diaphragmatic hernia with stomach, large intestine and caudal pancreas lodged in the thoracic cavity. After the antibiotic treatment, the patient became asymptomatic and surgery repair was declined. Morgagni hernia is an uncommon type of congenital diaphragmatic hernia, which may be asymptomatic until late in life or may be present acutely with life threatening conditions.

Yellow Nail Syndrome: Report of Two Cases and a Brief Review of the Current Literature

Yellow Nail Syndrome (YNS) is a rare, and probably misdiagnosed, condition. It must be considered in middle-aged patients with unexplained chronic respiratory manifestations, lymphedema and nail abnormalities. We present two cases of undiagnosed YNS until the current admissions, despite several years of investigation. The authors wish to draw attention to this syndrome, of which diagnosis is clinical and of exclusion.

Polymyositis and the Spectrum of Scleroderma Disorders

Polymyositis (PM) is usually associated to other autoimmune or connective tissue diseases. The authors report the case of a 59-year-old man with pulmonary fibrosis, who presented with constitutional symptoms and gradually developed proximal muscle weakness, Raynaud phenomenon, and dysphagia. Besides creatine kinase (CK) elevation, he had positive anti-Polymyositis-Scleromyositis (PM-Scl) and anti-Sjögren's-syndrome A (SSA) antibodies. Nailfold capillaroscopy showed a scleroderma pattern and muscle biopsy revealed necrosis, regeneration of muscle fibers, and inflammatory infiltrate. Prednisolone was started, with great improvement. Taking into account the overlap features between PM and systemic sclerosis sine scleroderma, it is important to closely monitor the patient for signs of pulmonary and cardiac decompensation.

Celiac Crisis in an Adult: Raising Awareness to a Life-Threatening Condition

Objectives: The Authors report the case of a 56-year-old man with celiac disease, who after ingesting a food containing gluten and experiencing a flu-like syndrome, developed severe diarrhea, vomiting, weight loss (15 kg), hypotension, renal dysfunction, hypokalemia and metabolic acidosis. Materials and methods: Admission to the Intensive Care Unit and exclusion of an infectious cause was determined. Results: After receiving noradrenaline, methylprednisolone and correction of ionic disturbances, the patient recovered rapidly and had no further complication. Conclusion: The Authors intend to increase awareness of celiac crisis, because despite being extremely rare in adults, it is potentially fatal and an quick diagnosis and treatment are crucial.

Unexpected Complication of a Multinodular Goitre

Multinodular goitre is the most common thyroid gland disorder. It can cause hyperthyroidism and mechanical complaints such as tracheal compression or dysphagia. We report a unique case of a patient with a toxic multinodular goitre presenting with a deep venous thrombosis of the left arm due to direct local compression of blood flow.

A Very Big Hand Is a Very Big Problem: Soft-Tissue Infection, Venous Thrombosis, or Just an Insect Sting?

A 67-year-old woman developed severe edema of her right hand and forearm, for which she was treated with antibiotics, without benefit. The echography excluded a venous thrombosis. Subsequently, she referred a wasp sting before the development of the edema. Specific Hymenoptera venom immunoglobulin E (IgE) was found to be positive for paper wasp and yellow jacket. A large local reaction (LLR) was diagnosed due to the hymenoptera sting. Self-injectable epinephrine was prescribed for possible, though unlikely, systemic reactions following hymenoptera stings.

Delayed Bleeding after Percutaneous Liver Biopsy

Percutaneous liver biopsy (PLB) is a common procedure in patients with liver disease. Bleeding after PLB is rare, with an incidence of 0.35%. Most bleeding complications present within 24 h after biopsy. A 56-year-old woman was admitted to our hospital due to severe and sudden right upper quadrant (RUQ) abdominal pain 10 days after ultrasound (US)-guided PLB. CT study revealed both intrahepatic and intraperitoneal bleeding, and Hb levels decreased by 3.2 g/dl within a few hours. Such a prolonged delay in PLB-related bleeding has not been previously described in the medical literature.