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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The actuarial survival rate for 58 unselected patients who entered a program of maintenance hemodialysis and transplantation was found to be 43.0 +/- 8.3 (SE)% for the six-year period of observation. The survival rate was considerably lower in hypertensive patients as well as in patients with familial Mediterranean fever with amyloidosis, all of whom were nonhypertensive. When the patients with familial Mediterranean fever were excluded from the non-hypertensive group, the expected survival rate of this group became greater than that of the hypertensive group, the difference being about 25% in five years and about 50% in six years. This difference in the survival rate approaches that between normotensive subjects and untreated severely hypertensive patients in the general population. It is concluded that hypertension is a serious limiting factor in the survival of patients on chronic hemodialysis, and that the difference in survival between the hypertensive and the non-hypertensive patients is attributable to hypertension.
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PMID:Probability of survival in hypertensive and nonhypertensive patients on maintenance hemodialysis. 32 Jan 59

A 22-year-old familial Mediterranean fever (FMF) patient was hospitalized for continuous fever, myalgia, hypertension, vertigo and a petechial rash. Laboratory findings revealed hyperglobulinaemia, thrombocytosis and a leukaemoid reaction. While on steroid therapy the patient sustained a haemorrhage into a renal aneurysm which responded to gel foam embolization. After 12 months of follow-up his condition remained stable under treatment with cyclophosphamide, prednisone and antihypertensive medications. This case provides the fourth example of polyarteritis nodosa associated with FMF.
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PMID:Polyarteritis nodosa and familial Mediterranean fever. 288 91

We describe a 14-year-old boy with a 5-year history of familial Mediterranean fever (FMF), treated with colchicine, who developed polyarteritis nodosa (PAN). He was admitted to our hospital with fever, general weakness, arthritis, and purpura. Five weeks after admission, hypertension was noted. Skin biopsy showed perivascular leukocyte infiltration in the epidermis. An aortography revealed multiple aneurysms of the renal, common hepatic, and intercostal arteries. He was treated with intravenous methylprednisolone, oral cyclophosphamide, and azathioprine. The known rare association of FMF and PAN is discussed.
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PMID:The coexistence of familial Mediterranean fever and polyarteritis nodosa; report of a case. 889 71

We studied a group of 59 patients with renal amyloidosis. Mean age (45 male, 14 female) was 33.05+/-13.04 years. All of the cases had secondary amyloidosis. The causes of secondary amyloidosis were as follows: familial Mediterranean fever (FMF) 18 (30.5%), pulmonary tuberculosis 12 (20.33%), chronic oseomyelitis 8 (13.55%), bronchiectasia 9 (15.25%), rheumatic diseases 4 (6.4%), Castleman's disease 1 (1.6%), unknown aetiology 7 (11.86%). Hypertension was detected in 15.3% of the cases. In patients with less than 20 ml/min creatinine clearance (Ccr) hypertension was found in 20%. Hypotension was detected in 6 patients and all of these cases had severe hypoalbuminaemia (<2.1 g/dl). Nephrotic range proteinuria (>3.5 g/day) was found in 75% of cases. Daily proteinuria was correlated with serum levels of albumin, total lipid and cholesterol, haematocrit and duration of disease. The mean Ccr was 51.03+/-40.60 ml/min. Twenty-nine per cent of patients had Ccr less than 20 ml/min. Renal, subcutaneous fat and rectal biopsies demonstrated amyloid in 100%, 20% and 57.6%, respectively, of patients tested. Patients with secondary amyloidosis were treated with colchicine in addition to the therapy of primary disease (in 6 patients). Nine patients died, and end-stage renal disease developed in 12 patients during four years of follow-up. Proteinuria disappeared or decreased in patients with secondary amyloidosis except secondary to collagen tissue disease, without advanced renal failure. Colchicine did not affect amyloid deposition in 2 patients with normal renal function and negative proteinuria, who were rebiopsied. It can be questioned that "Colchicine may have effect(s) for decrement on proteinuria". At least colchicine can be of use in secondary amyloidosis.
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PMID:Report on 59 patients with renal amyloidosis. 1075 52

There are many similarities in the profile of chronic renal disease in the five North African countries, reflecting their close resemblance in ethnic background, bioecology and socioeconomic standards. The incidence of renal disease is much higher than that in the West, yet the prevalence is relatively lower, which mirrors the inadequacy of medical care facilities. The principal causes of end-stage chronic renal disease (ESRD) are interstitial nephritis (14 to 32%), often attributed to environmental pollution and inadvertent use of medications; glomerulonephritis (11 to 24%), mostly mesangioproliferative and focal segmental sclerosis; diabetes (5 to 20%) and nephrosclerosis (5 to 21%). Obstructive/reflux nephropathy, attributed to urinary schistosomiasis, is common in Egypt (7%), Libya and Southern Algeria. Primary urolithiasis is a frequent cause of obstructive nephropathy in the western (hyperoxaluria) and middle (cystinuria) regions. The incidence of tuberculosis is increasing, particularly the diffuse interstitial and hematogenous forms. It is responsible also for 10 to 40% of renal amyloidosis. The latter is also frequently associated with familial Mediterranean fever. Sickle cell anemia is an important health problem in the west, leading to a wide range of glomerular and tubulointerstitial nephropathies. Takayasu disease is increasingly recognized as a cause of ischemic nephropathy and renovascular hypertension. The management of ESRD is largely influenced by late referral, co-morbidities and lack of dialysis facilities. Hemodialysis is the most frequent modality of renal replacement therapy (RRT). CAPD is used sporadically. Renal transplantation, largely from live (often unrelated) donors, is offered to less than 5% of patients with ESRD. The reported outcome of RRT generally conforms with international standards.
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PMID:End-stage renal disease in North Africa. 1286 87

We describe a 7-year-old boy with familial Mediterranean fever (FMF) complicated by polyarteritis nodosa (PAN) with distinct angiographic findings. On admission, he had abdominal pain, arthralgia, and severe fibromyalgia. During hospitalization, he displayed maculopapular eruptions, high blood pressure, gastrointestinal bleeding, and persistent constitutional symptoms mimicking a vasculitic process, most probably PAN. Renal angiography showed a perfusion defect compatible with a renal infarction secondary to a vasculitic process. He responded well to pulse methylprednisolone therapy with colchicine. We emphasize the rare association of FMF and PAN and the non-aneurysmal angiographic signs of PAN.
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PMID:Polyarteritis nodosa in a case of familial Mediterranean fever. 1496 62

Reversible posterior leukoencephalopathy syndrome is characterized clinically by headache, abnormalities of mental status and visual perception, and seizures. Despite its diverse causes, common precipitating factors are defined as abrupt elevations of blood pressure, renal decompensation, fluid retention, and immunosuppressive therapy. We report three children with reversible posterior leukoencephalopathy syndrome presenting with generalized seizures and headache. The causes of reversible posterior leukoencephalopathy syndrome were considered to be acute hypertension and immunosuppressive therapy in case 1 with systemic lupus erythematosus, chemotherapy (vincristine and/or actinomycin-D) and hyponatremia in case 2, and acute hypertension in case 3, admitted with a familial Mediterranean fever attack. In light of these cases, we review the literature for the etiology, clinical and laboratory findings, and pathogenetic mechanisms of the disease.
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PMID:Reversible posterior leukoencephalopathy syndrome: report of three cases. 1641 49

A 41 year-old male was admitted because of nephrotic syndrome associated with renal impairment and arterial hypertension. Renal biopsy showed a complete subverting of renal architecture with eosinophilic, amorphous deposits which stained positive for Congo red and were positive for antibodies against AA-amyloid. Abdominal fat pad aspirate confirmed the diagnosis of AA amyloidosis. Despite high values of serum amyloid A (SAA), surprisingly medical history, physical examination and all tests failed to identify any underlying inflammatory disease, even asymptomatic, at presentation and during the whole follow-up period. The patient carried a mutation (Glu148Gln) in the MEFV gene, and a mutation (Arg92Gln) in the TNFRSF1A gene, both in heterozygosity. The patient has never complained of the typical features of the Familial Mediterranean fever or of the TNF receptor-associated periodic syndrome. The patient's father carried the same mutations. His father's medical history was unremarkable; renal tests, acute-phase reactants and SAA were normal. During a trial with colchicine (while the patient was also taking atorvastatin) SAA decreased, renal function continued to deteriorate and proteinuria remained high; no cardiac involvement was detected. Six months later our patient developed rhabdomyolysis, thus accelerating the decline of renal function and requiring the start of dialysis.
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PMID:A complex case of renal amyloidosis with a rare co-occurrence of 2 mutations in separate hereditary periodic fever syndrome-related genes. 1704 17

For women on maintenance dialysis, pregnancy is still uncommon. The outcome of such pregnancies has improved in recent case series. Here, we report in detail the treatment of five successful pregnancies in dialysis patients from our centre. The present case series also includes the first successful pregnancy of a dialysis patient with underlying familial Mediterranean fever, and of a dialysis patient with cystinosis. We treated all patients with an intensified hemodiafiltration protocol, increased erythropoietin dosages, a generous application of water-soluble vitamins and trace elements in addition to a multidisciplinary clinical management approach with a very low threshold for hospital admission. Specifically, we report treatment of arterial hypertension with respect to changes in dry weight and pharmacological therapy. Mean gestational age at delivery was 32.8+/-3.3 weeks and mean birth weight was 1,765+/-554 g. All mothers and newborns were discharged healthy and in good condition. These modified management guidelines have led to a favourable outcome in all our patients including two patients with familial Mediterranean fever and with cystinosis, and may help to guide therapy in other pregnant dialysis patients.
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PMID:Successful pregnancies in dialysis patients including those suffering from cystinosis and familial Mediterranean fever. 1713 1

Systemic inflammation plays an important role in the development of atherosclerosis (AS). The aim of this study was to evaluate the presence of early AS in patients with familial Mediterranean fever (FMF) that is characterized by recurrent inflammatory attacks of serositis. Sixty-one FMF patients (30 Male/31 Female; 31.5 [18-54] years) and 31 healthy controls (16 Male/15 Female; 31 [22-58] years) were studied. All FMF patients were on regular daily colchicine treatment and during attack-free periods. Both the FMF patients and controls with a history of diabetes mellitus (DM), hypertension, and hyperlipidemia were excluded. Body mass index (BMI) was calculated. Serum lipids, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were assessed. Two-hour oral glucose tolerance test was performed to rule out DM and glucose intolerance. To investigate early AS "endothelium-dependent flow-mediated dilatation (FMD%)," "nitroglycerin-induced endothelium-independent peripheral vasodilatation (NTG%)," and intima-media thickness (IMT) of common carotid arteries (CCA) were measured by ultrasonograpy. The median disease duration for FMF patients was 16 (1-45) years. Age, sex, BMI, smoking status, and serum lipids were comparable in patients and controls (p > 0.05). However, ESR and standard CRP were significantly higher in the patients group (p < 0.05). There were no differences in the measurements of right, left, and averaged IMT of CCA between patients and controls ([0.49 vs 0.5], [0.51 vs 0.52] and [0.5 vs 0.51]; p > 0.05, respectively). None of the subjects had carotid artery plaques. FMD% and NTG% were also similar in patients and controls group ([18.2 vs 20.6] and [24.2 vs 22.5]; p > 0.05, respectively). This study suggests that the markers of early AS are not impaired in FMF patients on regular daily colchicine treatment.
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PMID:Early ultrasonographic markers of atherosclerosis in patients with familial Mediterranean fever. 1724 67


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