Manual Agent 105 - Diary of a Hamster

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FD is associated to an increased risk of developing aortic root dilatation in male patients [ ]. Figure 15 Aortic root dilatation: echocardiography shows aortic root diameter of 47 mm in a year-old male patient with Fabry disease. Figure 16 Aortic root dilatation in a patient suffering from Fabry disease : magnetic resonance imaging MRI showing aortic root dilatation in Fabry disease.

The early peripheral neuropathic hallmarks of FD [ 38 , , ] are often followed by cerebrovascular complications and autonomic dysfunction in adulthood. Some of the most devastating neurological features of FD are caused by cerebrovascular lesions - the result of multifocal involvement of small blood vessels [ , ]. Median age at first stroke was 39 in men and 46 years in women and stroke may be the first manifestation of the disease [ ]. There is a high prevalence of hypertension, cardiac disease and renal disease in patients who have had a stroke in the context of FD [ ].

A dilative arteriopathy of the vertebrobasilar circulation has also been documented Figure 18 [ , ]. Thrombus formation may be enhanced in FD due to the adhesion of neutrophils and monocytes to endothelial cell walls [ ] or to changes in the regional cerebral hyperperfusion [ — ]. Serum myeloperoxidase level has been found to predict the risk of a vasculopathy-related event in males affected with FD [ ].

Figure 17 Stroke in a patient affected with Fabry disease : axial brain MRI section showing stroke of the left cerebellar hemisphere that revealed Fabry disease in an otherwise asymptomatic year-old male patient. Figure 18 Dolichoectasia of the vertebro-basilar circulation : time of flight magnetic resonance angiographies showing ectatic vessels in four patients affected with Fabry disease.

White matter lesions may be single, multiple or confluent on MRI Figure 19 [ , ]. In addition, diffuse neuronal involvement, extending beyond the areas of MRI-visible cerebrovascular abnormalities has been found, and in such cases, 1H-MRS may be the preferred modality [ ]. Cerebral MRI can reveal periventricular white-matter lesions, microbleeds Figure 19 , cortical grey-matter infarcts and deep lacunar infarcts in both grey and white matter [ , — ]. Some patients affected with FD have an aseptic meningitis [ , , ]. Hyperintensity in the pulvinar on T1-weighted images is a common finding in FD, likely reflecting the presence of calcification [ , ].

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Recent findings suggest that the pulvinar sign is a highly specific sign, distinctively characteristic of FD [ ], more frequent in male patients with cardiomyopathy and severe kidney involvement Colas F, Carlier RY and Germain DP, unpublished data Figure Figure 19 Cerebral white matter hyperintensities, lacuna and microbleeds : A. Auditory and vestibular abnormalities are frequent deficits observed in FD, resulting in a range of symptoms, such as hearing loss [ , ], tinnitus and vertigo [ , ].

The high incidence of both progressive hearing loss and sudden deafness in male patients affected with classic FD has been demonstrated Figure 21 [ ]. Figure 21 Hypoacousia in patients affected with Fabry disease : A. Corneal opacities visible by slit-lamp microscopy are the most common and early of ocular signs, occurring in almost all hemizygous males Figure 22 [ — ].

It should be noted, however, that treatment with amiodarone or chloroquine can produce similar ophthalmological signs [ ]. Mild to marked tortuosity of the conjunctival and retinal vessels is also observed in patients with FD [ , ]. Anterior and posterior subcapsular cataracts are also observed, the latter also being termed the 'Fabry cataract' in that it represents a pathognomonic ocular sign of FD.

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More recently, an enlargement of the blind spot Figure 23 was reported in Figure 22 Cornea of a female patient heterozygote for FD : sub-epithelial brown lines show the typical pattern of so-called " cornea verticillata ". Figure 23 Standard Goldman visual field of the left eye of a patient affected with Fabry disease : the blind spot is enlarged. In a recent study, bone mineral density of the lumbar spine and the femoral neck was assessed by dual-energy X-ray absorptiometry DEXA in 23 hemizygous male patients with a mean age of 31 years range: years affected with classic FD.

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  7. Cases of severe osteoporosis with spontaneous lumbar fractures have recently been described Figure 25 [ ]. Patients suffering from Fabry disease should follow current recommendations regarding identification and treatment of vitamin D deficiency. Figure 25 Bone magnetic resonance imaging in a Fabry patient with severe osteoporosis : A STIR, sagittal view and B T1, sagital median : several vertebral body fractures are seen, without signal anomaly in T1 or T2 in favor of ancient fractures.

    FD can be caused by a variety of missense or nonsense point mutations, splicing mutations, small deletions or insertions [ , , — ], and large deletions [ , ].

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    Most families have unique mutations potentially explaining the marked variability in the residual enzyme activity but only in part the natural course of the disease since intra-familial variability does exist. Met42Arg c. Gly43Ser c. GlyGlu c. LysAsn c. GlnStop c. PheCys c. LeuPro c. Germain, unpublished data]. Non pathological single nucleotide polymorphisms such as c. Whether some published sequence changes, such as p.

    ArgHis, are true mutations or polymorphisms is still a matter of debate [ ]. In female heterozygotes, a-galactosidase activity may be within the normal range [ , ] and therefore, the definitive diagnostic confirmation should be made by genetic analysis in suspected cases Figure Direct molecular analysis is easy because of the small size of the gene and allows the precise characterization of the mutation of the GLA gene. A method that uses filter paper cards containing dried blood spots instead of the leukocytes pellet as the source of DNA was recently developed for sequencing, allowing genotyping from a dried blood spot on filter paper to confirm enzymatic diagnosis Figure 27 [ ].

    Figure 27 Sequencing of PCR products obtained from amplification of DNA directly eluted from a 3-mm punch of dried blood spot DBS on filter paper : a year-old man with left ventricular hypertrophy of unknown origin was enrolled in a screening protocol for FD. Patients with neuropathic pain may benefit from avoidance of circumstances triggering acute pain attacks, e.

    The neuropathic pain associated with FD can be managed with analgesics, but nonsteroidal anti-inflammatory drugs are generally ineffective and potentially harmful for kidney function while narcotic analgesics should be avoided [ ] although this has been debated [ ]. Carbamazepine [ , ], oxcarbazepin, gabapentin [ , ], pregabalin and phenytoin [ ] are classically used to manage pain in FD Table 3 [ 51 , ].

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    Some patients use illicit drugs, particularly marijuana for pain control and GI manifestations, especially if their symptoms have been overlooked by doctors. Table 3 Guidelines for baseline examination and follow-up of patients affected with Fabry disease. Intense physical activity and excessive sun exposure are inadvisable. Figure 28 Emergency healthcare card from the French Ministry of Health : an emergency healthcare card was created by the Ministry of Health, the center of excellence for Fabry disease and patients' associations for Fabry disease or lysosomal storage diseases.

    Since then, long term safety and efficacy of replacement therapy have been investigated and ERT has been validated as a disease-specific therapeutic agent for patients affected with FD but with this, has come the realization that numerous aspects have yet to be explored and understood. As an example, current guidelines for starting ERT in patients vary from one country to another and remain a matter of debate especially in heterozygous females and children.

    Current expert recommendations [ 51 ] are presented in Table 4 , but may evolve in the future. The safety and efficacy of both enzymes have been assessed in randomized, double-blind, placebo-controlled trials [ — ] and their extension studies for agalsidase alfa [ , ] and agalsidase beta [ , ], studies originating from industry-sponsored registries [ — ] and investigator-sponsored studies independent from the industry [ — ].

    Hereunder, we review the clinical efficacy data currently available for each drug since their marketing authorization within the European Union [ , ]. Table 4 Current proposed guidelines for starting enzyme replacement therapy in Fabry disease patients. Subpopulation Guidelines for onset of ERT Adult males over 16 years At time of diagnosis of Fabry disease Boys At time of development of significant symptoms or if asymptomatic, consider at years Females all ages Symptoms or evidence of progression of organ involvement.

    Estimated glomerular filtration rate, proteinuria and serum creatinine remained stable and normal in the vast majority of patients treated for 4.

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    This profile predisposed them to progression of renal disease, even under agalsidase beta therapy. Subgroup analyses were performed to examine the impact of baseline proteinuria or glomerulosclerosis on renal function during the study period. Figure 30 Long-term agalsidase beta therapy decreases Gb 3 accumulation in podocytes : A Kidney biopsy which was obtained prior to agalsidase beta therapy shows dark-staining granules in podocytes.

    The results of the published randomized controlled clinical trials and their extension studies, together with the pediatric trials for the two enzyme preparations, agalsidase alfa [ , , , ] and agalsidase beta [ , , , , ] are shown in Table 5. Table 5 Comparison of safety and efficacy outcomes of the enzyme replacement therapies. A few of the approximately patients treated to date with agalsidase beta have developed plasma IgE antibodies and a few others have had a positive prick-test together with urticaria or skin rash Figure Most patients have successfully undergone a rechallenge protocol [ ].

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    7. No IgE antibodies have been detected during agalsidase alfa treatment [ ]. Whether seroconversion affects treatment efficacy is currently unknown but neutralizing antibodies to both agalsidase alfa and agalsidase beta have been demonstrated [ ] and shown to lead to a relapse in urinary [ , , ] and cutaneous [ ] Gb 3. In these cases, the potential use of immunosuppressive therapy in combination with ERT should be investigated [ ]. In FD, a significant number of disease-causing mutations are missense mutations, which cause the newly synthesized lysosomal protein to be unstable, but still catalytically competent [ , ].

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      This provides a rationale for a therapeutic intervention using active-site-specific chaperones to stabilize the conformation or reduce misfolding of the mutant protein in order to prevent the premature degradation by ERAD Figure 32 [ — ]. Figure 32 Proposed mechanism of action of active site-specific chaperones ASSCs : A: During synthesis of a wild-type lysosomal enzyme, cells assemble amino acids in a correctly folded tertiary structure.

      Acknowledgements I am particularly grateful to my patients and their families. References Anderson W: A case of "Angeio-keratoma".