Hippokratia
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Ocular involvement in patients with transfusion-dependent β-thalassemia is quite common, and its frequency differs among studies. This case series aimed to describe the ocular abnormalities occurring in β-thalassemia patients who need regular blood transfusions and receive iron chelation therapy. ⋯ Early detection of severe ocular abnormalities is important in patients with thalassemia; thus, an ophthalmologic examination should be included at regular check-ups. An annual examination is currently indicated for asymptomatic patients, while in symptomatic and complicated cases, patients should be closely followed-up. HIPPOKRATIA 2021, 25 (2):79-82.
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Case Reports
Abdominal aorta aneurysm endograft infection mimicking bacteraemic urinary tract infection.
Endograft infection complicating endovascular aneurysm repairs is infrequent and presents various symptoms and findings, the most common being abdominal pain, fever, fatigue, and gastrointestinal bleeding. ⋯ Endovascular graft infections generally require antibiotic therapy combined with surgical debridement and revascularization. This case illustrates a successful alternative management strategy with percutaneous drainage of the aortic sac abscess combined with long-term oral antibiotic therapy. This case also underlines the high index of suspicion necessary for the accurate and timely diagnosis and management of endovascular graft infections. HIPPOKRATIA 2021, 25 (2) 91-93.
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Diffuse cystic lung diseases are a group of heterogeneous pathophysiological processes and include neoplastic, inflammatory, and infectious etiologies. This manuscript focuses on manifestations of pulmonary Langerhans cell histiocytosis (PLCH) and lymphangioleiomyomatosis (LAM). Description of the cases: Three female patients with LAM and one with PLCH are described. Stress dyspnea was a key symptom. There were similar cyst patterns in more than one lung lobe with a slow, progressive course. Histopathology confirmed the LAM diagnosis resulting from the nodular proliferate and the cyst wall that strongly expressed Human Melanoma Black-45 (HMB-45). A typical constellation for PLCH was demonstrated in high-resolution computed tomography (HRCT). It was found to be disseminated and relatively thick-walled cysts, mainly in the upper and middle parts. An individualized therapy was applied. Three patients with mild symptoms were followed up, including HRCT evaluations. Sirolimus was administered to one patient with a severe manifestation of LAM. ⋯ LAM and PLCH are rare. High-resolution computed tomography is an essential diagnostic tool. Lung emphysema as misdiagnosis should be avoided. The characteristics of pulmonary cysts, the cyst's wall regularity, and identification of associated pulmonary lesions, should be evaluated. A promising new therapy concept are mTOR inhibitors are, especially in LAM. The most important recommendation in PLCH is the cessation of cigarette smoking. HIPPOKRATIA 2021, 25 (2):83-86.
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Even though complement 1q nephropathy (C1qN) was first introduced in 1985, this entity is still unknown and recognized by clinicians due to its rare prevalence (0.2 - 2.5 %) and insufficient emphasis. ⋯ Renal biopsy for patients with non-nephrotic proteinuria without delay is suggested as it might be a surrogate marker of severe injury. Genetic mutations in the WDR19 gene should be searched for C1qN pathogenesis. This is the first adult case report on C1qN from Turkey.HIPPOKRATIA 2021, 25 (2):87-90.