Hippokratia
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Mutation of the NEU1 sialidase gene is the etiology of sialidosis, a storage disorder with a plethora of systemic manifestations ranging from ocular abnormalities, bone pathologies, and ataxia (sialidosis type I) to mental decline and infantile death (sialidosis type II). Non-immune hydrops fetalis and isolated ascites are the most severe forms of sialidosis type II that manifests itself prenatally. ⋯ Sialidosis type II should be considered in the differential diagnosis of neonatal hydrops fetalis of no immune causality or isolated fetal ascites. Genetic studying of the patient and the family by carrier detection is crucial to prevent missed diagnoses, while genetic counseling for following pregnancies is imperative. HIPPOKRATIA 2019, 23(4): 169-171.
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The growing phenomenon of vaccine hesitancy and the severe economic crisis may have affected compliance with the National Immunization Program (NIP) in Greece over the last years. We investigated compliance with the NIP among children attending nurseries in the urban area of Thessaloniki. ⋯ While high coverage appears to be sustained for most of the recommended vaccines, delay of scheduled shots may compromise age-appropriate protection. Suboptimal immunization against pneumococcal, meningococcal serogroup C, hepatitis A, and rotavirus infections may increase morbidity in this age group and needs to be addressed. HIPPOKRATIA 2019, 23(4): 147-153.
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Hypokalemia and hypomagnesemia caused due to renal losses with chloride-resistant metabolic alkalosis in normotensive patients should remind clinicians of the rare inherited tubulopathy, Gitelman syndrome. Its diagnosis is further strengthened by the presence of consanguinity and the absence of kaliuretic medications. A definitive diagnosis should be based on genetic testing. ⋯ This lifelong disease could cause life-threatening conditions due to the cardiac complications of hypokalemia in some of the affected patients. Therefore, it is necessary to be aware of the appropriate diagnosis and treatment for patients admitted to the clinic with hypokalemia, hypomagnesemia, hypocalciuria, and hyperreninemia. HIPPOKRATIA 2019, 23(4): 175-178.
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Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare immune-mediated disease of the peripheral nervous system characterized by motor weakness, sensory symptoms, areflexia, and specific electrophysiological findings. Rarely, Anti-Tumor Necrosis Factor-alpha (anti-TNFa) agents, which are used as a treatment for immune-mediated diseases, can cause neurological adverse effects on the central nervous system, as well as peripheral nervous system demyelination. ⋯ Although demyelinating neurological complications of anti-TNF are rare, pharmacovigilance is required. HIPPOKRATIA 2019, 23(4): 179-180.
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Isolated infrarenal abdominal aortic dissection is a rare clinical entity. Most cases are treated by endovascular techniques, and open surgery is seldom necessary. ⋯ By infrarenal abdominal aortic dissection with accessory renal arteries, abdominal surgery with selective clamping is a reliable therapeutic approach allowing maximal preservation of renal blood flow. HIPPOKRATIA 2019, 23(4): 186-189.