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Prevention of large-vessel stenoses in atypical hemolytic uremic syndrome associated with complement dysregulation.

Davin JC, Majoie C, Groothoff J, Gracchi V, Bouts A, Goodship TH, Loirat C - Pediatr. Nephrol. (2010)

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Bilateral nephrectomy was performed 1 year later because of hypertension... She lost two transplants by recurrence, one immediately posttransplantation and the other one 2 months posttransplant despite preventive plasma exchanges (PEs) contemporary to frequency reduction of PE... From the age of 15 years, she began to experience transient sensory and motor symptoms on both sides of her body associated with low blood pressure (BP) during hemodialysis... Under this treatment, she presented no HUS recurrence, and renal function remained stable (plasma creatinine 120 μmol/L) 16 months after PE discontinuation... At 9.5 years after HUS onset, native renal function (serum creatinine 49 μmol/L) was normal... Contrary to their older sister with cerebral artery stenoses who was treated by prophylactic PE for 2 months only, the twin sisters received prophylactic PE without interruption for, respectively, 7.5 (twin 1) and 9 (twin 2) years... At the age of 10 years, she started having episodes of bilateral hemiparesis during hemodialysis in relation with multiple stenoses of cerebral arteries... Our patient and the patient reported recently by Loirat et al. suggest that aHUS with complement dysregulation, a disease of microvascularization, may also involve large arteries, especially when patients have to face long periods of dialysis when plasma therapy is not used... The latter reports also raise the question of preventing vascular lesions by avoiding complement system disorders... This emphasizes the importance of including patients at an early stage in specific programs of kidney transplantation using preventive plasma therapy or combined liver transplantation... Inhibition of the complement system by anti-C5 antibody that is actually under investigation might be valuable for this purpose in the future.

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Clinical course in patient and her twin sisters
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Fig1: Clinical course in patient and her twin sisters

Mentions: The oldest of three sisters, two of whom are monozygous twins, presenting all with atypical hemolytic uremic syndrome (aHUS) associated with a CFH mutation, was referred at the age of 3 years to the Emma Children’s Hospital/Academic Medical Centre of Amsterdam (Fig. 1) [1, 2]. She never recovered renal function and commenced peritoneal dialysis. Bilateral nephrectomy was performed 1 year later because of hypertension. She lost two transplants by recurrence, one immediately posttransplantation and the other one 2 months posttransplant despite preventive plasma exchanges (PEs) contemporary to frequency reduction of PE. From the age of 15 years, she began to experience transient sensory and motor symptoms on both sides of her body associated with low blood pressure (BP) during hemodialysis. Magnetic resonance angiogram (MRA) showed severe stenoses of both middle and both anterior cerebral arteries (Fig. 2a) (partial report in [3]). She did not present any additional risk factors for developing arteriosclerosis (such as long-duration hypertension, diabetes, uncontrolled hyperparathyroidism). No arterial calcification and no left ventricular hypertrophy were shown.Fig. 1


Prevention of large-vessel stenoses in atypical hemolytic uremic syndrome associated with complement dysregulation.

Davin JC, Majoie C, Groothoff J, Gracchi V, Bouts A, Goodship TH, Loirat C - Pediatr. Nephrol. (2010)

Clinical course in patient and her twin sisters
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2991236&req=5

Fig1: Clinical course in patient and her twin sisters
Mentions: The oldest of three sisters, two of whom are monozygous twins, presenting all with atypical hemolytic uremic syndrome (aHUS) associated with a CFH mutation, was referred at the age of 3 years to the Emma Children’s Hospital/Academic Medical Centre of Amsterdam (Fig. 1) [1, 2]. She never recovered renal function and commenced peritoneal dialysis. Bilateral nephrectomy was performed 1 year later because of hypertension. She lost two transplants by recurrence, one immediately posttransplantation and the other one 2 months posttransplant despite preventive plasma exchanges (PEs) contemporary to frequency reduction of PE. From the age of 15 years, she began to experience transient sensory and motor symptoms on both sides of her body associated with low blood pressure (BP) during hemodialysis. Magnetic resonance angiogram (MRA) showed severe stenoses of both middle and both anterior cerebral arteries (Fig. 2a) (partial report in [3]). She did not present any additional risk factors for developing arteriosclerosis (such as long-duration hypertension, diabetes, uncontrolled hyperparathyroidism). No arterial calcification and no left ventricular hypertrophy were shown.Fig. 1

View Article: PubMed Central - PubMed

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Bilateral nephrectomy was performed 1 year later because of hypertension... She lost two transplants by recurrence, one immediately posttransplantation and the other one 2 months posttransplant despite preventive plasma exchanges (PEs) contemporary to frequency reduction of PE... From the age of 15 years, she began to experience transient sensory and motor symptoms on both sides of her body associated with low blood pressure (BP) during hemodialysis... Under this treatment, she presented no HUS recurrence, and renal function remained stable (plasma creatinine 120 μmol/L) 16 months after PE discontinuation... At 9.5 years after HUS onset, native renal function (serum creatinine 49 μmol/L) was normal... Contrary to their older sister with cerebral artery stenoses who was treated by prophylactic PE for 2 months only, the twin sisters received prophylactic PE without interruption for, respectively, 7.5 (twin 1) and 9 (twin 2) years... At the age of 10 years, she started having episodes of bilateral hemiparesis during hemodialysis in relation with multiple stenoses of cerebral arteries... Our patient and the patient reported recently by Loirat et al. suggest that aHUS with complement dysregulation, a disease of microvascularization, may also involve large arteries, especially when patients have to face long periods of dialysis when plasma therapy is not used... The latter reports also raise the question of preventing vascular lesions by avoiding complement system disorders... This emphasizes the importance of including patients at an early stage in specific programs of kidney transplantation using preventive plasma therapy or combined liver transplantation... Inhibition of the complement system by anti-C5 antibody that is actually under investigation might be valuable for this purpose in the future.

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