cardiac amyloidosis is a rare cause of cardiomyopathy, however, it is part of an underlying systemic disease is diagnosed. To transthyretin (ATTR) amyloidosis, which is caused by the deposition of one folded transthyretin, either as wild type (wtATTR) or mutated (mattr) form, novel treatment options based on recent evidence was shown to reduce the progression of the disease and hospitalization rates for heart failure ,
Thus, it is important to establish early and reliable diagnosis of cardiac involvement with ATTR amyloidosis.Modern non-invasive imaging (cardio magnetic resonance (CMR) and scintigraphic methods) together with immune fixation with the determination of free light chain enabling rapid and reliable screening clinical cardiac amyloidosis, while endomyocardial biopsy and genetic diagnosis is used to confirm the underlying. an interdisciplinary team including hemato-oncology, neurology, nephrology besides cardiology is essential to enable a targeted treatment strategy private.
Klinische manifestations: Die-Amyloidose cardiac transthyretin (ATTR) ist einer Ursache Kardiomyopathie seltene als Teil einer unterdiagnostizierten Systemerkrankung. Für die of cardiac transthyretin-Amyloidose, bei der es zur Ablagerung kommt von fehlgefaltetem transthyretin, entweder als Wildtyp (wtATTR) oder mutierte (mattr) Form, sind seit kurzem evidenzbasierte Therapiemöglichkeiten mit der Kardiomyopathie-Progression Verlangsamung deutlicher Reduktion und der Hospitalisierungsraten verfügbar. Daher ist es Wichtig, diese zu schwerwiegende Erkrankung frühzeitig diagnostizieren.
Diagnostics: Mittels Bildgebung (Cardiac MRT oder szintigrafische Verfahren) Immunfixation sowie mit Bestimmung der Freien Leichtketten möglich ist einfach ein klinisches Screening, bevor mittels Biopsie und / oder die definitive Diagnose Genetic gestellt wird. Aufarbeitung interdisziplinäre bei eine auf cardiac Verdacht Amylodiose von unter Einbezug Hämatoonkologie, Nephrologie, Neurologie und weiteren ist hierbei Disziplinen unabdingbar.
Therapie: Bei Patienten mit einer kann eine kardialen Amyloidose Herzinsuffizienz-Therapie mit beta blockers, renin-angiotensin-aldosterone-Inhibitoren und diuretics erfolgen, sollte allerdings aufgrund unerwünschter Nebenwirkungen vorsichtig erfolgen.
Studien in den letzten Jahren haben die Wirksamkeit tetramer-Stabilisators sowie eines von TTR mRNA-interferierenden Therapeutika medikamentösen zur Therapie der ATTR-Amyloidose gezeigt – 9 10. spezifische Eine Zulassung zur Therapie der kardialen ATTR-Amyloidose existiert seit neuestem für den tetramer-stabilizers Tafamidis
[Suspected cardiac amyloidosis – diagnostic steps for evaluation]
[The cause of the common clinical and audiological manifestations of unilateral hearing loss in children]
Objective: To analyze the causes of the common clinical and audiological manifestations of unilateral hearing loss in children, and to provide a basis for early prevention and intervention.
Methods: A retrospective analysis of 20 cases of children diagnosed with unilateral hearing loss do. The medical history was collected, followed by audiological examination and imaging. Results: audiological performance of 20 children with unilateral hearing loss: conductive hearing loss first case, the hearing curve is flat and shows moderate hearing loss.
There were 19 cases of sensorineural hearing loss, which is one of moderate deafness cases, 16 cases of severe deafness, and 2 cases of very severe deafness. Imaging results showed that there was 1 case with a transverse fracture of the temporal bone, 1 case with congenital cholesteatoma, 9 cases with dysplasia of the cochlear nerve, and 4 cases with malformation of the inner ear.
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The history of two children were gathered and there cytomegalovirus infection and congenital viral infection mumps clear. The cause of the remaining 3 cases of unknown
![[Suspected cardiac amyloidosis - diagnostic steps for evaluation]](https://www.emorroidiestipsi.com/wp-content/uploads/2020/08/photo_2020-08-10_13-22-21-2.jpg)
![[Suspected cardiac amyloidosis - diagnostic steps for evaluation]](http://www.emorroidiestipsi.com/wp-content/uploads/2020/08/photo_2020-08-10_13-22-21-2-498x1024.jpg)
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