ABSTRACT
A 76-year-old male Caucasian patient was treated in our hospital for acutely decompensated heart failure due to restrictive cardiomyopathy. Acute-on-chronic kidney failure developed with serum creatinine rising from 160 to 345 µmol/L (1.8-3.9 mg/dL); therefore, a kidney biopsy was performed. Besides secondary focal-segmental glomerulosclerosis and minimal amyloidosis, histological analysis showed zebra bodies in the cytoplasm of some podocytes, suggesting renal phospholipidosis (PL). Possible causes for this storage disorder encompass Fabry's disease, in rare cases silicosis, and an iatrogenic drug-induced aetiology. The main suspects are cationic amphiphilic drugs, such as amiodarone and chloroquine. The only cationic amphiphilic drug our patient had taken was the anti-anginal ranolazine, a compound not yet associated with PL. The patient had taken ranolazine for diastolic dysfunction over a period of 9 months until 6 weeks before renal biopsy. In the absence of a hereditary disorder, silicosis and well-known pharmaceutical triggers, a causative role of ranolazine seems likely, and this drug should be considered in the differential diagnosis of drug-induced PL.
ABSTRACT
BACKGROUND: Endocrine orbitopathy is a common feature of autoimmune thyroid disease. The increase of pressure within the eye socket leads to proptosis and compression of the optic nerve. This results in cosmetic impairment as well as loss of vision. Apart from the medical treatment surgical decompression of the crowded orbit offers a valid therapeutic option. PATIENTS AND METHODS: 142 patients (264 orbits) were treated at the interdisciplinary orbital center, Johannes Gutenberg University Hospital, Mainz, Germany. The medial orbital wall was removed, followed by lipectomy. Indications for decompression included cosmetic reasons (196 orbits, 74.2%), dysthyroid optic neuropathy (67 orbits, 25.4%), and in one case corneal ulceration. RESULTS: A median reduction of the severity score according to the NOSPECS classification from 7 points preoperatively to 4 points 3 months after surgery was achieved (p < 0.001). Median proptosis decreased from 23 to 20 mm (p < 0.001). Intraocular pressure in upgaze dropped from 23 mmHg by 3 mmHg during the first postoperative 3 months (p < 0.001). Additionally, the influence of age, sex and smoking behavior on the operation outcome was examined. Diplopia was present in 100 patients before surgery. Of the remaining 42 patients, two patients (4.8%) complained about new-onset constant diplopia 3 months after decompression, while 22 patients (52.2%) reported inconstant/intermittent motility disorders. Squint surgery corrected diplopia. CONCLUSION: The combined transnasal-endoscopic/transpalpebral approach represents a safe and efficient method for severe endocrine orbitopathy.
