ABSTRACT
BACKGROUND AND PURPOSE: Little is known about the epidemiological features of amyotrophic lateral sclerosis (ALS) in sub-Saharan Africa, and data from the region are limited to clinical series or case reports. The aim of the study was to investigate the incidence rate and presentation of ALS in an ethnically diverse region of South Africa. METHODS: We performed a 4-year prospective incidence study in the Western Cape Province of South Africa between 1 July 2014 and 30 June 2018, and used a two-source capture-recapture method for case ascertainment. Age- and sex-adjusted incidence rates (ASAIRs) were calculated using the 2010 US population as the reference. RESULTS: A total of 203 incident cases were identified over the study period, resulting in a crude incidence rate (IR) of 1.09 [95% confidence interval (CI) 0.94-1.24] per 100 000 person-years in the at-risk population (aged >15 years). Capture-recapture analysis resulted in an estimated IR of 1.11 (95% CI 1.01-1.22) per 100 000 person-years. The ASAIR was 1.67 (95% CI 1.09-2.26) overall; 1.99 (95% CI 1.60-2.39) for men and 1.37 (95% CI 1.06-1.68) for women. When analysed separately, there was a substantial difference in ASAIRs between the different population groups, with the highest in the European ancestry group (2.62; 95% CI 2.49-2.75), the lowest in the African ancestry group (0.56, 95% CI 0.0-1.23), and an ASAIR in between these two in the mixed ancestry group (1.09, 95% CI 0.80-1.37). CONCLUSION: The overall incidence of ALS in the Western Cape Province of South Africa appears to be lower than in North African and Western countries, but higher than in Asian countries. As suggested by previous epidemiological studies, ALS may be less frequent in people of African ancestry.
Subject(s)
Amyotrophic Lateral Sclerosis , Motor Neuron Disease , Amyotrophic Lateral Sclerosis/epidemiology , Female , Humans , Incidence , Male , Motor Neuron Disease/epidemiology , Prospective Studies , South Africa/epidemiologyABSTRACT
BACKGROUND: Transcription of transforming growth factor beta-1 (TGF-ß1) is regulated by a polymorphic promoter region containing African-specific single nucleotide polymorphisms (SNPs). Some of these SNPs have higher frequencies among Southern Africans compared to other African populations and their functionality has only been partially studied. Due to the high prevalence of HIV-associated nephropathy (HIVAN) in Africans we hypothesized that functional African TGFB1-promoter SNPs may contribute to HIVAN pathogenesis. METHODS: The functionality of the TGFB1 -1347 C>T variant and African-specific variants (-1287 G>A, -1154 C>T, -387 C>T and -14 G>A) were examined by measuring reporter gene expression in kidney and fibroblast cell lines co-transfected with TGFB1-promoter constructs and an HIV-Tat expression vector. TGF-ß1 immunohistochemical staining was performed on kidney biopsies with HIVAN (n = 18) and compared to control biopsies without HIVAN or tubulointerstitial disease (n = 12) using semi-quantitative and digital image analysis. HIVAN cases were genotyped for TGFB1 -1347 and -387 SNP variants. RESULTS: TGFB1-promoter haplotypes containing the African -387 T-allele resulted in ~ five-fold repression of TGFB1-promoter activity compared to -387 C haplotypes (p ≤ 0.024). HIV-Tat upregulated TGFB1-promoter activity for haplotypes containing -1347 T and -387 T in transfected renal cells (≈ 1.6-fold; p ≤ 0.030) and fibroblasts (≈ 1.3-fold; p ≤ 0.016). The renal interstitium from HIVAN biopsies, compared to HIV-positive and -negative controls, differed in the semi-quantitative TGF-ß1 staining and digital optical density analyses. The TGFB1 -1347 and -387 genotypes in HIVAN cases were similar to population controls. CONCLUSION: African-specific haplotypes lower TGFB1-promoter activity and expression levels and HIV-Tat upregulates TGFB1 promoter activity irrespective of the haplotype.
Subject(s)
AIDS-Associated Nephropathy/genetics , Regulatory Sequences, Nucleic Acid , Transforming Growth Factor beta1/genetics , AIDS-Associated Nephropathy/ethnology , Africa , Cell Line , Fibroblasts , Haplotypes , Humans , Kidney , Polymorphism, Single NucleotideABSTRACT
BACKGROUND: HIV-infected individuals are at increased risk of tissue inflammation and accelerated vascular aging ('inflamm-aging'). Abnormal diurnal blood pressure (BP) rhythms such as non-dipping may contribute to an increased risk of cardiovascular and cerebrovascular events in HIV infected individuals. However, little data exists on ambulatory blood pressure (ABP) and measures of vascular stiffness in the black African HIV infected population. METHODS: This is a cross-sectional analysis of otherwise well, HIV infected outpatients on ART for >5 years. Study assessments included: 24hr ABP monitoring, pulse wave velocity (PWV) and central aortic systolic pressure (CASP) using a AtCor Medical Sphygmocor device, fasting lipogram, oral glucose tolerance test, high-sensitivity C-reactive protein (hsCRP) and anthropometric data. Patients completed a questionnaire of autonomic symptoms. CD4+ counts and viral loads were obtained from the National Laboratory results system. RESULTS: Sixty seven black participants were included in the analysis of whom 91% (n = 61) were female with a mean age of 42.2 ± 8.6 years. The median duration on ART was 7.5 years (IQR = 6-10), 84% were virally supressed and the median CD4 count was 529.5cells/mm3 (IQR = 372.0-686.5). The majority (67%) were classified as overweight and 76% had an increased waist circumference, yet only 88% of participants were normotensive. A hsCRP level in the high cardiovascular risk category was found in 68% of participants. The prevalence of non-dipping BP was 65%. Interestingly, there was no association on multivariable analysis between dipping status and traditional risk factors for non-dipping BP, such as: obesity, autonomic dysfunction and older age. CONCLUSION: This relatively young cross-sectional sample of predominantly normotensive, but overweight black women on effective ART >5 years showed: a high prevalence of non-dipping BP, inflammation and vascular stiffness. Causality cannot be inferred but cardiovascular risk reduction should be emphasized in these patients.
Subject(s)
Aging/drug effects , Anti-Retroviral Agents/adverse effects , Blood Pressure/drug effects , Cardiovascular Diseases/chemically induced , HIV Infections/complications , HIV-1/drug effects , Vascular Stiffness/drug effects , Adult , Cardiovascular Diseases/epidemiology , Cross-Sectional Studies , Female , HIV Infections/drug therapy , HIV Infections/virology , Humans , Male , Prevalence , Pulse Wave Analysis , Risk Factors , South Africa/epidemiologyABSTRACT
Several studies have demonstrated the expression of odorant receptors (OR) in various human tissues and their involvement in different physiological and pathophysiological processes. However, the functional role of ORs in the human heart is still unclear. Here, we firstly report the functional characterization of an OR in the human heart. Initial next-generation sequencing analysis revealed the OR expression pattern in the adult and fetal human heart and identified the fatty acid-sensing OR51E1 as the most highly expressed OR in both cardiac development stages. An extensive characterization of the OR51E1 ligand profile by luciferase reporter gene activation assay identified 2-ethylhexanoic acid as a receptor antagonist and various structurally related fatty acids as novel OR51E1 ligands, some of which were detected at receptor-activating concentrations in plasma and epicardial adipose tissue. Functional investigation of the endogenous receptor was carried out by Ca2+ imaging of human stem cell-derived cardiomyocytes. Application of OR51E1 ligands induced negative chronotropic effects that depended on activation of the OR. OR51E1 activation also provoked a negative inotropic action in cardiac trabeculae and slice preparations of human explanted ventricles. These findings indicate that OR51E1 may play a role as metabolic regulator of cardiac function.
Subject(s)
Fatty Acids/metabolism , Myocardium/metabolism , Myocytes, Cardiac/metabolism , Neoplasm Proteins/metabolism , Receptors, G-Protein-Coupled/metabolism , Blotting, Western , Cells, Cultured , Gene Knockdown Techniques , High-Throughput Nucleotide Sequencing , Humans , Immunohistochemistry , Immunoprecipitation , Polymerase Chain ReactionABSTRACT
BACKGROUND: An evaluation of geriatric rehabilitation has been carried out in Rhineland-Palatinate for over 10 years by collecting data of patients absolving an inpatient geriatric rehabilitation program. The aim of the project was to improve the transparency of outcome quality. The procedure is equally supported by geriatric rehabilitation clinics, health insurance companies and the Medical Service of Health Insurance (MDK). MATERIAL AND METHODS: Consented information about the rehabilitation process has been collected from every geriatric rehabilitation clinic in Rhineland-Palatinate. The data were pseudonymized and sent to the MDK in Rhineland-Palatinate for statistical analysis. The dataset included age, diagnosis, life circumstances before rehabilitation, duration of the rehabilitation, therapy implemented and need for support (with or without personal assistance) in eight activities of daily living at the beginning and at the end of rehabilitation. RESULTS: The results of 45,751 participants who underwent rehabilitation between 2005 and 2014 are presented. There was a slight tendency towards an increase in the number of very old geriatric patients undergoing rehabilitation. The average duration of rehabilitation decreased slightly during the observation period, while the frequency of therapy increased. The reduction in the need for assistance during rehabilitation remained constant over the observation period. CONCLUSION: Systematic evaluation improves the transparency of the rehabilitation process.
Subject(s)
Activities of Daily Living/psychology , Disabled Persons/rehabilitation , Disabled Persons/statistics & numerical data , Health Services for the Aged/statistics & numerical data , Quality of Life/psychology , Rehabilitation/statistics & numerical data , Utilization Review , Aged , Aged, 80 and over , Disabled Persons/psychology , Female , Germany/epidemiology , Health Care Surveys , Humans , Longitudinal Studies , Male , Middle Aged , Rehabilitation/psychology , Treatment OutcomeABSTRACT
BACKGROUND: In the city of Zurich, vision screening is performed by school medical services as part of the legally compulsory preventive medical examinations. We retrospectively evaluated the results of the 2011-2012 school year. PATIENTS AND METHODS: Preventive medical examinations by the school medical services were performed in all kindergartens. The examinations were mandatory for first, second and eighth grade children, whilst those for the fourth grade were voluntary. The basic diagnostic testing consisted of monocular visual acuity (Snellen E chart) for all age groups and the stereopsis test (TNO test) in kindergartens. RESULTS: Vision screening was performed on 7499 children. 1471 first graders (55â%), 201 fourth graders (11â%) and 211 eighth graders (12.3â%) did not pass the examinations. In 33.7â% of the children who underwent the follow-up examination from an ophthalmologist, amblyogenic potential was found. CONCLUSIONS: Vision screening by the school medical services enrols most of the children from a single age group. The Snellen E charts used for the monocular distance acuity, together with the TNO stereo test, appear to constitute an effective testing combination. Ophthalmological follow-up examinations of the affected children revealed that one third were afflicted by amblyogenic factors.
Subject(s)
Practice Patterns, Physicians'/statistics & numerical data , Refractive Errors/diagnosis , Refractive Errors/epidemiology , School Health Services/statistics & numerical data , Students/statistics & numerical data , Vision Screening/statistics & numerical data , Adolescent , Child , Child, Preschool , Humans , Male , Prevalence , Refractive Errors/prevention & control , Switzerland/epidemiology , Utilization ReviewABSTRACT
Wernicke's encephalopathy (WE) is a medical emergency. Although WE is commonly viewed in the context of alcoholism, it can be caused by thiamine deficiency secondary to persistent vomiting. Non-alcohol-related WE may be more catastrophic in onset and less likely to present with the classic features than WE with alcoholism as a cause. We describe three cases of WE due to persistent vomiting without alcoholism in patients with hyperemesis gravidarum, drug-induced hyperlactataemia, and an acute gastrointestinal illness in an already malnourished individual. Our cases highlight the importance of recognising WE when undernutrition, which may be caused by gastrointestinal disease or surgery, or malignancy, is compounded by vomiting. Expert guidelines suggest that WE must be considered in the emergency room in any individual with disturbed consciousness of unknown cause. Treatment is with parenteral thiamine before glucose administration.
Subject(s)
Hyperemesis Gravidarum/complications , Thiamine Deficiency/complications , Vomiting/complications , Wernicke Encephalopathy/etiology , Adolescent , Female , Glucose/administration & dosage , Humans , Hyperlactatemia/chemically induced , Hyperlactatemia/complications , Male , Middle Aged , Pregnancy , Thiamine/administration & dosage , Thiamine Deficiency/etiology , Wernicke Encephalopathy/physiopathology , Wernicke Encephalopathy/therapy , Young AdultABSTRACT
SETTING: Distal sensory polyneuropathy (DSP) may manifest in human immunodeficiency virus (HIV) infected individuals before or after antiretroviral therapy (ART). DSP can also occur in response to isoniazid (INH); this can be prevented by pyridoxine supplementation. N-acetyltransferase 2 (NAT2) polymorphisms influence drug acetylation and possibly the risk for INH-associated DSP. OBJECTIVE: To investigate the relationship between previous/current TB, pyridoxine deficiency and DSP in HIV-infected individuals enrolled in a government-sponsored HIV programme. DESIGN: Neuropathy assessments were performed among 159 adults pre-ART and 12 and 24 weeks thereafter. DSP was defined as ⩾1 neuropathic symptom and sign. NAT2 genotypes predicted acetylation phenotype. Serum pyridoxine levels (PLP) were quantified at baseline and week 12. RESULTS: DSP was present in 16% of individuals pre-ART and was associated with previous/current TB (P = 0.020). Over 50% were pyridoxine deficient (PLP < 25 nmol/l), despite supplementation with vitamin B complex supplements (2-4 mg/day pyridoxine). Those with a history of TB and pre-ART DSP were more likely to be pyridoxine deficient (P = 0.029), and slow/intermediate NAT2 phenotypes impacted on their PLP levels. Incident/worsening DSP after ART developed in 21% of the participants. PLP levels remained low after ART, particularly among those with prior TB, but without an association between DSP or NAT2 phenotypes. CONCLUSION: Adequate pyridoxine supplementation before ART initiation should be prioritised, particularly in those with a history of TB or current TB.
Subject(s)
Isoniazid/adverse effects , Polyneuropathies/diagnosis , Polyneuropathies/drug therapy , Pyridoxine/blood , Vitamin B 6 Deficiency/diagnosis , Vitamin B Complex/therapeutic use , Adult , Antiretroviral Therapy, Highly Active , Arylamine N-Acetyltransferase/genetics , Coinfection/drug therapy , Female , HIV Infections/drug therapy , Humans , Isoniazid/therapeutic use , Male , Risk Factors , South Africa , Tuberculosis/drug therapySubject(s)
Alzheimer Disease/complications , Encephalitis, Varicella Zoster/complications , Herpes Zoster/cerebrospinal fluid , Herpes Zoster/virology , Lactates/cerebrospinal fluid , Aged , Alzheimer Disease/diagnosis , Encephalitis, Varicella Zoster/cerebrospinal fluid , Encephalitis, Varicella Zoster/diagnosis , HumansABSTRACT
Acute intermittent porphyria, the most common porphyria affecting the nervous system, typically presents with neurovisceral crises followed by a motor neuropathy. We describe a 23-year-old black South African man presenting with a progressive stuttering, lower motor neuron syndrome developing over months. He had not experienced pain or neuropsychiatric symptoms. One year after symptom onset he was bed-bound with a flaccid quadriparesis. There was marked amyotrophy, but without fasciculations. Sensation was intact apart from a hypo-aesthetic patch over the thigh. Electrophysiological investigations showed an active motor axonopathy. Urinary porphyrins, delta-aminolaevulinic acid and porphobilinogen were elevated. Mutation analysis revealed the c445C>T (R149X) mutation in the porphobilinogen deaminase gene. The patient responded dramatically to haem arginate and could walk with assistance 2 weeks later. We identified the first molecularly confirmed acute intermittent porphyria in a black South African. The clinical presentation mimicked a progressive lower motor neuron syndrome.
Subject(s)
Muscular Atrophy, Spinal/etiology , Porphyria, Acute Intermittent/complications , Arginine/therapeutic use , Heme/therapeutic use , Humans , Hydroxymethylbilane Synthase/genetics , Male , Porphyria, Acute Intermittent/therapy , Young AdultSubject(s)
Aphasia, Broca/diagnosis , Aphasia/etiology , Leukoencephalopathy, Progressive Multifocal/diagnosis , Speech Disorders/etiology , Comprehension , Corpus Callosum/pathology , Diagnosis, Differential , Female , Frontal Lobe/pathology , Humans , Magnetic Resonance Imaging , Middle Aged , Neuropsychological Tests , Speech Perception , Tomography, X-Ray ComputedABSTRACT
SETTING: Human immunodeficiency virus (HIV) infection and treatments for HIV infection and tuberculosis (TB) are associated with the risk of developing sensory polyneuropathy (SPN). Vitamin B6 and genetically determined slow isoniazid (INH) acetylation are believed to play key roles in the development of SPN in a TB treatment setting. OBJECTIVE: To investigate slow acetylation and risk factors for SPN in HIV-infected patients receiving TB treatment, and establish vitamin B6 status and its association with SPN. METHODS: HIV-infected in-patients were prospectively assessed after initiating TB treatment and vitamin B6 supplementation, and monthly during hospitalisation. SPN was defined as ≥1 symptom plus ≥1 sign. NAT2 genotyping predicted acetylation status, and plasma high performance liquid chromatography estimated vitamin B6 status. A survival analysis estimated hazard ratios (HRs) for SPN during TB treatment. RESULTS: Of 116 participants, 56% had SPN at study entry. Participants developed SPN at a rate of 26/100 person-months (95%CI 18-35) during TB treatment, which was independently associated with slow acetylation (HR 2.5; 95%CI 1.1-5.9), as well as black race, previous TB and extra-pulmonary/disseminated TB. Vitamin B6 status was normal, irrespective of SPN. CONCLUSIONS: Risk factors for SPN suggest a multi-factorial pathogenesis related to INH and other potential nervous system insults. SPN developed despite normal vitamin B6 status, suggesting other mechanisms of injury.
Subject(s)
Antitubercular Agents/adverse effects , HIV Infections/complications , Polyneuropathies/chemically induced , Sensory Receptor Cells , Tuberculosis, Pulmonary/drug therapy , Acetylation , Adult , Arylamine N-Acetyltransferase/genetics , Arylamine N-Acetyltransferase/metabolism , Dietary Supplements , Female , Genotype , HIV Infections/diagnosis , HIV Infections/mortality , Humans , Longitudinal Studies , Male , Phenotype , Polyneuropathies/diagnosis , Polyneuropathies/physiopathology , Prospective Studies , Risk Factors , Time Factors , Treatment Outcome , Tuberculosis, Pulmonary/complications , Tuberculosis, Pulmonary/diagnosis , Tuberculosis, Pulmonary/microbiology , Tuberculosis, Pulmonary/mortality , Vitamin B 6/blood , Vitamin B 6/therapeutic use , Vitamin B 6 Deficiency/blood , Vitamin B 6 Deficiency/complications , Vitamin B 6 Deficiency/diagnosis , Vitamin B 6 Deficiency/drug therapy , Vitamins/therapeutic useABSTRACT
Polyvalent immunoglobin, derived from pooled human plasma, can be administered via the intravenous, subcutaneous or intramuscular route. Therapy is standard of care in the treatment of a number of immune-mediated pathologies across disciplines. By volume, the majority is used in neurology (~40%). In primary immunodeficiencies, therapy reconstitutes humoral immunity at replacement doses (0.4 - 0.6 g/kg/month), decreasing infections, and is usually lifelong. However, high doses, usually 2 g/kg total dose over five days, are required for immunomodulation in autoimmune and inflammatory indications. A high-quality evidence base supports use in primary antibody failure, Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, acute idiopathic thrombocytopenia, Kawasaki disease andimmunobullous diseases. Low-quality evidence shows benefit in many other uncommon autoimmune and immunodeficient conditions.In South Africa, use of immunoglobulin therapy is restricted and, given the cost involved, will likely remain so. Therefore, the incremental benefit over other forms of immunosuppression, particularly corticosteroids, must be assessed carefully on a case-by-case basis. In most cases, therapy will be second-line or 'rescue' and motivation will be required. This short review aims to provide clinicians with the necessary understanding of the therapy, general considerations for use, and evidence base and quality thereof for well-established indications.
Subject(s)
Chorea/diagnosis , Huntington Disease/diagnosis , Age Factors , Aged , Alleles , Cerebellar Ataxia/diagnosis , Chorea/therapy , Diagnosis, Differential , Disease Progression , Genetic Testing , Humans , Huntingtin Protein , Huntington Disease/therapy , Male , Nerve Tissue Proteins/genetics , Neurologic Examination , Neuropsychological Tests , Trinucleotide Repeats/geneticsSubject(s)
Central Nervous System Diseases/diagnostic imaging , Positron-Emission Tomography , Sarcoidosis/diagnostic imaging , Adult , Biopsy , Central Nervous System Diseases/cerebrospinal fluid , Central Nervous System Diseases/pathology , Female , Fluorodeoxyglucose F18 , Granuloma/diagnostic imaging , Granuloma/pathology , Humans , Polyradiculopathy/diagnostic imaging , Polyradiculopathy/pathology , Positron-Emission Tomography/methods , Sarcoidosis/cerebrospinal fluid , Sarcoidosis/pathologyABSTRACT
A new S2k AWMF guideline for the treatment of idiopathic facial palsy has been published. An accurate differential diagnosis is indispensable as 25-40% of all facial palsy cases are of non-idiopathic origin. It is explicitly recommended to treat patients with idiopathic facial palsy with steroids. Steroids favour a complete recovery, decrease the risk of synkinesis, autonomic sequelae and contractures. Adjuvant antiviral therapy cannot be recommended. On current data there is not sufficient evidence that the combination of steroids with antiviral drugs has a benefit for the patients. Even when not supported by randomized trials, adjuvant symptomatic therapy to protect the cornea and to avoid complications is recommended. There is no scientific evidence that physical therapy has any benefit but it should be taken into account because of psychological reasons. A benefit of acupuncture has not been proven. If eye closure remains incomplete as result of defective healing, one therapeutic option is lid loading of the upper eye lid. Moreover, in case of severe persistent palsy, several well-established microsurgical nerve and muscle plasty procedures are available.
Subject(s)
Bell Palsy/etiology , Bell Palsy/therapy , Acupuncture Therapy , Adrenal Cortex Hormones/therapeutic use , Antiviral Agents/therapeutic use , Bell Palsy/diagnosis , Diagnosis, Differential , Drug Therapy, Combination , Evidence-Based Medicine , Eyelids/surgery , Humans , Physical Therapy Modalities , Prognosis , Prostheses and ImplantsABSTRACT
We investigated the association of an ophthalmoplegic complication developing in African myasthenia gravis (MG) subjects with polymorphisms in the regulatory region of TGFB1. We found significant associations with several putative functional single nucleotide polymorphisms (SNPs) (including two novel SNPs) that potentially alter transcription factor binding. Our data support a hypothesis that altered TGFB1 regulation may predispose individuals who harbour these SNPs to developing ophthalmoplegia as a result of increased TGF-ß1 driven myofibrosis as a consequence to complement-mediated damage.
Subject(s)
Myasthenia Gravis/immunology , Myasthenia Gravis/pathology , Ophthalmoplegia/immunology , Ophthalmoplegia/pathology , Polymorphism, Single Nucleotide/immunology , Transforming Growth Factor beta1/genetics , Fibrosis , Humans , Muscle, Skeletal/immunology , Muscle, Skeletal/pathology , Myasthenia Gravis/genetics , Ophthalmoplegia/genetics , South Africa , Transforming Growth Factor beta1/biosynthesis , Transforming Growth Factor beta1/physiology , Up-Regulation/genetics , Up-Regulation/immunologyABSTRACT
To present two patients with Miller Fisher syndrome (MFS) recurrence after 35 and 44 years and review of the literature on recurring MFS. All identified cases with recurrent MFS were evaluated. Age, gender, clinical features of first and recurrent MFS, course of disease, laboratory findings, therapy and outcome were transformed into tables. Twenty-eight patients (16 men, 12 women; mean age at the first episode 34 years (range 13-57 years); mean age at the latest episode 47 years (range 21-66 years) with a total of 70 MFS episodes were identified. Twenty-one patients had a single recurrence, five patients had two recurrences, one patient had four recurrences and one patient had seven recurrences. The mean interval between attacks was 9.45 years (3 months to 44 years). In 76% of the initial episodes and in 81% of the recurrent episodes, an infectious disease preceded MFS. Additional facial and bulbar symptoms and autonomic disturbances were frequent findings. Cerebrospinal fluid (CSF) and electrodiagnostic findings were unspecific. If tested, autoantibodies against GQ1b had been positive in all episodes. In about half of the patients, immunotherapy was applied. The outcome was favourable in most patients. Recurrence of MFS is a rare quite uniform condition with a mostly favourable prognosis.