ISSN: 1300-0292
İndekslendiği Dizinler: SCIENCE CITATION INDEX EXPANDED
CINAHL, Index Copernicus,
Chemical Abstracts (CA),
Excerpta Medica / EMBASE
Dil: Türkçe, İngilizce
İçerik: Orijinal Araştırma, Derleme, Editöre Mektup, Olgu Sunumu, Tıp Eğitimi, Tıbbi Kitap İncelemeleri
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Transthyretin Amyloidosis And Hypopotasemia: A Case Report
Dr. Mustafa BALAL,a Dr. Saime PAYDAŞ,a Dr. Şeyda ERDOĞAN,b Dr. İbrahim KARAYAYLALI,a Dr. Neslihan SEYREKa
aNefroloji BD, bPatoloji AD, Çukurova Üniversitesi Tıp Fakültesi, ADANA
Transthyretin (TTR) amyloidosis is a common form of systemic hereditary amyloidosis. Neuropathy, cardiomyopathy, renal and ocular involvement are the most common symptoms of TTR amyloidosis.
A 50 year-old female patient was admitted to our clinic due to hypotension, nausea, vomiting, diarrhea, hypokalemia and walking disability. Physical examination revealed hypotension (75/40 mmHg) and weakness of lower and upper extremities (4/5). Laboratory investigations were as follows; white blood cell 6300/mm3, hemoglobin 10 mg/dL, hematocrit 29.2%, platelet 185.000/mL, glucose 91 mg/dL, BUN 8 mg/dL, creatinine 0.4 mg/dL, Na 136 mEq/L, K 1.8 mEq/L, CL 92 mEq/L, total protein 4.9 g/dL, serum albumin 2.1 g/dL and daily proteinuria 3 g. Panendoscopy revealed bulbitis, pangastritis, monilial esophagitis and biopsy specimen from the antrum revealed TTR amyloidosis. Gastritis and monilial esophagitis improved with the administration of an H2 receptor blocker and nystatin. Hypokalemia improved with potassium infusion. After two months, her complaints of diarrhea and walking disability persisted. Visual Evoked Potential (VEP) EMG revealed mixed type polyneuropathy of upper and lower extremities. At that time, blood pressure was 90/60 mmHg, and laboratory examinations were as follows: BUN 24 mg/dL, Cr 0.5 mg/dL, total protein 4.4 g/dL and serum albumin 1.8 g/dL.
In conclusion, careful systemic evaluation is necessary in unknown etiology of hypotension and/or neuropathy, and signs and symptoms should be well evaluated. Familial amyloidosis may present with different clinical findings such as hypokalemia, as in our patient without a familial history. Proteinuria may be an important clue. Here we discussed a patient with TTR amyloidosis diagnosed by antral biopsy and negative family history. Analysis for TTR mutation and liver transplantation were not done. Conservative measures and cholchicine therapy did not cause improvement.Keywords: Transthyretin amyloidosis, hypokalemiaTurkiye Klinikleri J Med Sci 2006, 26:90-92
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