Autonomic failure in primary amyloidosis

Medicine and Cardiology, Bharat Ratna Dr. Babasaheb Ambedkar Memorial Hospital, Central Railway, Byculla, Mumbai.
The Journal of the Association of Physicians of India 01/2009; 56:995-6.
Source: PubMed


Amyloidosis is an uncommon plasma cell dyscrasia affecting Multisystem, characterized by deposition of amyloid proteins in extracellular spaces and the tissues. Reported incidence of amyloidosis is 8 cases per million per year. Deposition of amyloid fibrils occurs in peripheral nerves in 20% of the cases in Primary Amyloidosis. Though. polyneuropathy is one of the presenting manifestations in cases of Primary Amyloidosis, pure autonomic failure without involving peripheral nerves is not a documented entity. Here, we present a case of Primary Amyloidosis presenting as Pure Autonomic Failure (Dysautonomia).

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    ABSTRACT: Although rare, renal amyloidosis is an important diagnosis that bares a significant morbidity and mortality. The purpose of this study was to present current single center experience in the past 28 years. A total of 6151 kidney biopsies were performed in our hospital from Jan. 1983 to Dec. 2010; 5844 were native kidney biopsies. Cases with a diagnosis of renal amyloidosis were identified through a search of our data bank and the clinical data were retrieved from medical charts. The patients were further classified as AL type and non-AL type. The trend of incidence was analyzed and the clinical features were compared. Risk factors that predict patient mortality were discovered by logistic regression analysis. Forty out of 5844 native kidney biopsies (0.68%) were identified as renal amyloidosis. Eighteen (45%) were of AL type. The mean age at diagnosis was 62.6 ± 13.4 years. Male was the predominant gender that was involved. Clinically, most (80%) have nephrotic range proteinuria (mean 24 hours urine protein: 6.9 ± 4.7 g). With a mean follow-up duration of 1155 day, the 1-year and 5-year patient survival were 42.9% and 17.9%, respectively. eGFR at biopsy was the sole predictive factor for patient survival. Senile patients with nephrotic syndrome should elicit the suspicion of renal amyloidosis. The incidence appeared to be increasing and the prognosis poor.
    Internal Medicine 01/2011; 50(21):2511-7. DOI:10.2169/internalmedicine.50.5822 · 0.90 Impact Factor
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    ABSTRACT: BACKGROUND: Renal amyloidosis is an unusual diagnosis in patients undergoing kidney biopsy. Knowledge of the clinical features and long-term outcomes is still very limited. The current study represents our single-center experience in the past 27 years. METHODS: Six thousands renal biopsies were performed between January 1983 and June 2010 at Taichung Veterans General Hospital. Of them, 720 cases were from renal allograft and 5280 were from native kidneys. A retrospective study via chart review was conducted to identify cases of renal amyloidosis. The clinical features as well as laboratory data at presentation and subsequent follow-up were retrieved and analyzed. RESULTS: Among 5280 cases of native kidney biopsies, 35 renal amyloidosis (0.66%) were identified. The mean follow-up duration is 1155 days. The incidence of renal amyloidosis among proteinuric patients appeared to be increasing. The male to female ratio was 24:11. Seventeen (48.6%) cases were of light chain amyloidosis (AL) type while the others were of non-AL types. The mean age was 61.7 ± 12.9 years, while the duration between apparent onset and kidney biopsy was 299.4 ± 312.2 days. Most of the cases (83.9%) had nephrotic-range proteinuria. The mean 24-hour urine protein was 7.4 ± 4.7 g (median: 6.48 g/day, 2.13-22.9 g/day), serum albumin, 2.39 ± 0.7 g/dL; total cholesterol, 327.5 ± 144 mg/dL; and triglyceride, 223.7 ± 150 mg/dL. Hematuria occurred in 40% of the cases. The eGFR at renal biopsy was 56.3 ± 28.2 mL/min. There was no obvious enlargement in renal size (right kidney: 102.7 ± 15.6 mm, left kidney: 107.9 ± 14.2 mm). The one-year and five-year patient survival was 42.9% and 17.9%, respectively. Logistic regression analysis revealed that eGFR was the only predictive factor for patient survival. CONCLUSION: Elderly patients with nephrotic syndrome should prompt the suspicion of renal amyloidosis, especially in those with nephritic syndrome and relatively low blood pressure. Given the increasing incidence and poor prognosis, early diagnosis and a search of the underlying etiology of amyloidosis are mandatory. (Acta Nephrologica 2011; 25: 113-118)
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    ABSTRACT: Familial amyloidosis with polyneuropathy (FAP) sometimes courses with vitreous amyloid. The aim of this study was to evaluate the incidence of glaucoma after vitrectomy in FAP patients. A total of 79 eyes of 42 liver transplanted FAP patients and 16 eyes of 16 non-FAP patients with rhegmatogenous retina detachment were collected. The patients were divided in to three groups: Group I - FAP patients with vitreous opacities submitted to vitrectomy, Group II - FAP patients without vitreous opacities and not submitted to vitrectomy and, Group III - non-FAP patients with rhegmatogenous retinal detachment submitted to vitrectomy. The Group I was subdivided into: Ia - "complete" vitrectomy; Ib - "incomplete" vitrectomy. The onset of glaucoma was considered when the intraocular pressure level was higher than 21 mmHg, with concomitant visual field abnormalities and optic nerve cupping. Post vitrectomy glaucoma was more frequent in Group I (56.1%) than in Group III (12.5%) and in Group II (10.5%). We observed a higher incidence of glaucoma in the Ia than in the Ib subgroup (86.4 vs. 21.1%) and earlier appearance in subgroup Ia (7.9 ± 3.6 vs. 39.5 ± 6.6 months). Vitrectomy induced the development of glaucoma in FAP patients.
    Amyloid: the international journal of experimental and clinical investigation: the official journal of the International Society of Amyloidosis 08/2012; 19(3):146-51. DOI:10.3109/13506129.2012.710669 · 2.01 Impact Factor
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