ABSTRACT
Posterior reversible encephalopathy (PRES) is a recently described syndrome, defined by clinical and neuroimaging features. Chronic kidney disease patients may be especially vulnerable to this syndrome because they are frequently exposed to several of its possible causes, including uremia and hypertension. In its most severe form, PRES can manifest clinically as seizures, coma or death. However, if properly diagnosed and treated, this syndrome can be completely reversible. Therefore, neuroimaging methods, especially brain magnetic resonance is fundamental for its diagnosis because it shows brain edema in characteristic pattern, and excludes causes of seizures or coma. An important example is the case of a young hypertensive chronic kidney disease patient on peritoneal dialysis, brought to the emergency room comatous with generalized tonic-clonic seizures; the cerebral magnetic resonance imaging features were impressive. Anti-hypertensive therapy and hemodialysis allowed complete recovery. The reversibility of this syndrome depends on timely diagnosis and therapy and therefore it should be kept in mind in the differential diagnosis of seizures. or coma in chronic kidney disease patients.
Subject(s)
Kidney Diseases/complications , Posterior Leukoencephalopathy Syndrome/complications , Adult , Chronic Disease , Humans , MaleABSTRACT
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Subject(s)
Humans , Encephalitis/physiopathology , Hypertensive Encephalopathy/physiopathology , Acute Kidney Injury/etiology , Diagnosis, Differential , Seizures/etiologyABSTRACT
BACKGROUND: Autosomal dominant polycystic kidney disease is one of the most common hereditary diseases in man with an estimated prevalence of 1:1000. At least three genetic loci are responsible for the development of the disease. PKD1 localized to 16p13 is the most common gene, contributing to almost 85% of all cases, is associated with the most severe form. PKD2, localized to 4q21-23, responsible for almost all the remaining cases, is associated with a milder form. Up to now, only five families have been reported unlinked to the two most common genetic defects, and therefore little is known about the clinical findings of the non-PKD1/PKD2 families. METHODS: In this report we describe the clinical findings of 18 patients of a non-PKD1/PKD2 family, with a mean follow-up of 52 months (range 3-133 months) in our outpatient clinic. RESULTS: Of the 10 patients older than 40 years, nine were hypertensive; in this age group eight patients exhibited renal failure (two of them were on dialysis) and six had hepatic cysts. In eight patients younger than 40 years, the only clinical finding was hypertension in two. Considerable variation in the rate of progression to renal failure among members of this family was found; on the other hand, some patients did not exhibit any signs of progression. CONCLUSION: This family exhibits a more aggressive phenotype, in contrast with the majority of the described non-PKD1/non-PKD2 families.
Subject(s)
Polycystic Kidney, Autosomal Dominant/complications , Adolescent , Adult , Aged , Female , Follow-Up Studies , Humans , Kidney Failure, Chronic/etiology , Male , Middle Aged , Polycystic Kidney, Autosomal Dominant/geneticsABSTRACT
The purpose of this study was to evaluate the effect of the partial correction of anaemia with recombinant human erythropoietin (rHuEPO) on the blood pressure (BP) of patients on chronic haemodialysis (HD). A group of 50 patients (26 men and 24 woman, mean age of 50 +/- 19.0 and range of 21 to 67) with basal levels of haemoglobin (Hb) less than or equal to 8 g/dl was evaluated before and during treatment with rHuEPO. Recombinant erythropoietin was started at 50 U/kh I.V. 3 times a week, immediately after each session of HD, for 4 weeks, and this dose was increased in steps of 25 U/kg until and Hb level of 12 g/dl or a maximum dose of 100 U/kg were reached. Before the administration of rHuEPO 33 patients (67.3%) were normotensives and 16 (32.6%) were hypertensives treated and well controlled. During the period of administration of rHuEPO 10 of the normotensives (30.3%) and 5 (31.3%) of the hypertensives patients showed an increase in the B.P. There was no correlation between the frequency of increase in B.P. and sex, age, length of time on HD and previous levels of B.P., but that frequency was higher in the patients with the lowest basal levels of haematocrit (Hct) and with the greatest increases in Hct (delta Hct). An immediate effect of I.V. administration of rHuE-PO on B.P. levels was not found. Finally we discuss the etiopathologic factors eventually responsible for the increase in BP and suggest some rules to be observed in the therapeutic use of rHuEPO.
