Sphingolipidoses in Morocco: Chemical profiling for an affordable and rapid diagnosis strategy.

Sphingolipidoses are a group of metabolic diseases in which lysosomal hydrolases dysfunction disrupt normal sphingolipids' metabolism, leading to excess accumulation in cellular compartments and excretion in urine. These pathologies represent a significant burden among Moroccan population, for which an easy access to enzymatic assays and genetic tests is not guaranteed. Parallel analytical methods thus have to be developed for preliminary screening. In this study, 107 patients were addressed to the metabolic platform of the Marrakesh Faculty of Medicine for diagnosis confirmation. Thin-Layer Chromatography was used as a first step to perform chemical profiling of the patients' urinary lipids, allowing 36% of the patients to be efficiently oriented towards the adequate enzymatic assay. UPLC-MS/MS analyses of urinary sulfatides excreted in urines patient had been used to control the reliability of TLC analysis and to obtain more accurate information related to the sulfatides isoforms. This analytical process combining TLC with UPLC-MS/MS has enabled rapid and appropriate patient management in a reduced time and with reduced resources.

Epidemiology and Clinical Features of Coronavirus Disease 2019 in Moroccan Children.

OBJECTIVE: This study aims to analyze the epidemiological and clinical features of coronavirus disease 19 (COVID-19) in a Moroccan pediatric population. METHODS: A retrospective study of a cohort of 74 children with RT-PCR confirmed COVID-19. We collected information on clinical and laboratory features of all children (age <18 years) admitted between 2 March, 2020 and 1 April, 2020. RESULTS: The mean (SD) age of the 74 children (40 girls) was 7 (1.5) years. The mean (SD) time from illness onset to diagnosis was 2 (1) days. 54 children were asymptomatic, while eight had fever, and five cases had cough. Recovery was after a mean (SD) of 12 (1) days. CONCLUSION: COVID-19 was mostly mild in the pediatric population in Morocco.

Streptococcus pneumoniae carriage among febrile children at the time of PCV-10 immunization in pediatric emergencies at Mohammed VI University Hospital Centre in Marrakesh (Morocco).

OBJECTIVES: In Morocco, 13-valent pneumococcal conjugated vaccine (PCV) was introduced in the childhood immunization program in October 2010 and changed to PCV-10 in July 2012. The purpose of this study was firstly to determine the prevalence of pneumococcus carriage in a population of febrile infants in Marrakesh and secondly, to investigate the risk factors for carriage and the distribution of circulating serotypes. MATERIAL AND METHODS: This prospective study was conducted from February to June 2017, in the pediatric emergency department of the Mother and Child Hospital of Mohammed VI University Hospital Centre (UHC) in Marrakesh. At total of 183febrile infants, aged 2-18months, were enrolled in this study and were swabbed for nasopharyngeal carriage. Pneumococci were cultured, identified, serotyped, and tested for penicillin susceptibility. Demographic data and risk factors for carriage were collected. The statistical analyses performed were the following: the analysis of the risk factors using logistic regression, the estimation of serotype diversity with the Simpson index, and the Chi2 test to compare serotype distribution in the prevaccination (a cohort of 660 healthy children, less than 2years old, in the Marrakesh region, in 2008-2009) and postvaccination periods. RESULTS: The prevalence of Streptococcus pneumoniae carriage was 68.3%. Of the 183infants enrolled in this study, 111 had received at least one dose of PCV-10. Colonization by vaccine serotype among febrile children was related to incomplete vaccination status. In total, vaccine serotypes accounted for 6.4% (n=8): 19F (n=2), 1 (n=2) and one strain for each of the following serotypes: 14, 23F, 6B, and 9V. Non-vaccine and nontypeable strains presented 63.2% and 23.2%, respectively, with dominance of serotypes 6A (6.4%), 15A/15F (5.6%), 20, 22F/22A, 23B, and 11A/11D with a prevalence of 3.2%. The rate of pneumococcus strains with reduced susceptibility to penicillin was 33.6%, of which 90.2% were non-vaccine serotypes and nontypeable strains. Serotype diversity increased in the postvaccination period and the effectiveness of PCV-10 against vaccine serotypes was estimated at 89.6%. CONCLUSION: An important change in the distribution of vaccine and non-vaccine serotypes was observed after the introduction of the PCVs. In fact, the prevalence of vaccine serotypes decreased significantly while non-vaccine serotypes emerged. These results underscore the importance of maintaining close and prolonged surveillance of serotype distribution to monitor the dynamics of nasopharyngeal pneumococcal carriage.

Late Treatment and Recurrence of Kawasaki Disease in a Moroccan Infant.

INTRODUCTION: While the diagnosis of typical form of Kawasaki disease (KD) is obvious, this multifaceted disease continues to surprise us. We report the case of a recurrent Kawasaki disease in an infant. CASE: At the age of 13 months, the infant was diagnosed with complete Kawasaki disease; he presented with prolonged fever, bilateral conjunctivitis, enanthem, exanthema, edema of the lower limb, peeling, and biological inflammatory syndrome. He was treated with intravenous immunoglobulin (IVIG) associated with a high dose of aspirin and then an antiplatelet dose with a good clinical-biological evolution. The echocardiography was normal. Seven months later, the patient was again admitted, in a similar picture: a prolonged fever evolving for 7 days, bilateral conjunctivitis, enanthem, cervical adenopathy of 1.5 cm/1 cm, scarlatiniform erythema, pruriginous of the trunk and limb, and peeling of the toes, with indurated edema of the hands and feet. The rest of the examination was normal except the irritability. The diagnosis of recurrent KD was made according the five criteria of the American Heart Association. The echocardiography was normal again. The infant received IVIG with good outcome. CONCLUSION: Despite its rarity, the possibility of recurrence of KD should be known by clinicians, so as not to delay the specific management of vasculitis whose stakes in terms of prevention of coronary artery lesions are well known. Our case confirms the possibility of this recurrence.

[Orbital and periorbital cellulitis in children. Epidemiological, clinical, therapeutic aspects and course]. / Cellulites orbitaires et péri-orbitaires de l'enfant. Profil épidémiologique, clinique, thérapeutique et évolutif.

Orbital cellulitis in children is a rare but potentially serious condition. The goal of this study is to analyze the epidemiological, clinical, therapeutic aspects and typical course of orbital and periorbital cellulitis in children, so as to propose a clinical management protocol adapted to our context. During the retrospective study period (2008-2014), 28 cases were hospitalized in the pediatric department at the Mohammed VI university medical center in Marrakech. Eighty-five percent of the cases were diagnosed as preseptal cellulitis, and 15% as retroseptal cellulitis. The age of the patients ranged from 6 months to 14 years with a mean age of 3 years. We report a female predominance with a prevalence of 58%. In our study, the most common cause is extension of infection from sinusitis. Clinically, fever was present in 19 patients (68%), eyelid edema was universal, proptosis and chemosis were noted in 2 cases, and ptosis in one patient. Bacteriological testing identified micro-organisms in 6 cases. Orbital computed tomography performed in 57% of the cases showed preseptal cellulitis in 12 cases, orbital cellulitis in one case, a subperiosteal abscess in 2 cases, and orbital abscess in one case. Medical treatment was based on amoxicillin-clavulanic acid or the combination of ceftriaxone, metronidazole±aminoglycoside. However, surgical drainage was necessary in 1 case. The outcome of all cases was favorable. Orbital cellulitis in children is usually preseptal, and amoxicillin-clavulanic acid is considered to be the standard empiric treatment.

Upper airway obstruction and hemoptysis due to a leech infestation in a child.

This paper presents the case of a 12-year-old child who presented with an 8-day history of cough, respiratory distress, and hemoptysis. He was admitted first to the pediatric intensive care unit to reestablish hemodynamic and respiratory stability. The chest X-ray showed a bilateral alveolar-interstitial syndrome. Four hours after admission, he had a coughing fit, moderately abundant hemoptysis, and a leech appeared in his mouth, which was removed by the mother. Symptoms completely disappeared afterwards. Endoscopy and ear-throat-laryngeal examination done thereafter were normal. The presence of a leech in the larynx has been rarely described in children, but is not a rare cause of hemoptysis. Extraction is imperative and should be performed as soon as the diagnosis is made to avoid a fatal progression. Prevention remains the best treatment.

[Multiple pulmonary emboli complicating infective endocarditis in a child with congenital heart disease]. / Embolies pulmonaires multiples compliquant une endocardite infectieuse sur cardiopathie congénitale.

Pulmonary embolism in children is a rare condition, associated with high mortality. Clinical presentation is nonspecific. Pulmonary embolism may present initially similar to bacterial endocarditis of the right heart, septic thrombophlebitis, or osteomyelitis. We report the case of a 6-year-old girl who had dyspnea over the four months before consultation, complicated three months later by hemoptysis. She was diagnosed with subacute bacterial endocarditis secondary to group D Streptococcus, developed upon a ventricular septal defect. Two weeks later, the child had sudden chest pain and tachypnea. Lung scintigraphy showed multiple pulmonary embolisms. The therapeutic approach was to continue antibiotics without anticoagulant treatment. The outcome was favorable with apyrexia and stabilization on the respiratory level. Pulmonary embolism is a rare disease in children with an incidence of 3.7%. Classically, it presents with fever, hemoptysis, and nonspecific infiltrates on chest X-ray. These signs were noted in our patient, although the infiltrates on the chest X-ray were hidden by the pulmonary edema associated with heart failure. The persistence of these left basal opacities after antidiuretic treatment suggested an infectious origin. Subsequently, lung scintigraphy showed that it was a pulmonary infarct. The therapy of septic pulmonary embolism is the same as that for infective endocarditis. Antibiotic treatment alone was maintained without anticoagulants because of the high risk of bleeding at the seat of the pulmonary embolism and the insubstantial significant benefit of this therapy. Pulmonary embolism in children is a rare disease, but its incidence is underestimated. Better knowledge on its actual impact and etiologies in children is necessary. Multicenter studies are needed to establish recommendations.

[An uncommon cause of delayed walking: idiopathic palmoplantar hidradenitis]. / Une cause inhabituelle de retard d'acquisition de la marche : l'hidradénite palmo-plantaire idiopathique.

INTRODUCTION: Idiopathic palmoplantar hidradenitis is an uncommon neutrophilic dermatosis, occurring in children and young adults. Its pathogenesis is incompletely understood. It is characterized by spontaneous resolution, but it can relapse in 50% of cases. We describe a case of a child with atypical idiopathic hidradenitis remarkable for its impact on his motor development. OBSERVATION: A 3-year-old boy was admitted to the pediatric unit for etiological assessment of delayed walking. Medullary MRI and TSH were normal. He was referred to a dermatologic consultation for recurrent and painful palmoplantar lesions, giving an equinus antalgic posture. Examination found erythematous tender plantar nodules. The palms were not affected. The exam was otherwise normal. Diagnosis of idiopathic plantar hidradenitis, pressure urticaria, and plantar erythema nodosum were discussed, leading to a skin biopsy. The histopathologic findings of nodular, neutrophilic infiltrates around the eccrine glands confirmed the diagnosis of idiopathic plantar hidradenitis. Anti-inflammatory treatment was given, followed by complete resolution of the lesions, but persistent equinus posture. COMMENTS: Idiopathic palmoplantar hidradenitis is an uncommon neutrophilic dermatosis, distinct from neutrophilic eccrine hidradenitis. It corresponds to neutrophilic infiltrates of the eccrine sweat glands. It is more frequently reported in children and young adults with no medical history. Its pathogenesis is not completely explained. The lesions are usually painful, hindering walking for a few days or even delaying it, as for our patient. These lesions typically involute, but they may recur in more than half of the cases. CONCLUSION: Through this observation, we highlight the atypical impact of idiopathic hidradenitis and the value of a dermatological examination in case of delayed walking.

[Acute disseminated encephalomyelitis after rabies vaccination: a case report]. / Encéphalomyélite aiguë disséminée post-vaccination antirabique: à propos d'un cas.

Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system. It is a disease whose incidence is not precisely known. The presumed mechanism is demyelination of the immune-mediated central nervous system. There is no pathognomonic clinical presentation in ADEM. The combination of multifocal neurological disorders arising in the aftermath of an infection or vaccination should alert the clinician. We report a case of ADEM in an 8-year-old child occurring after antirabies vaccination. The diagnosis was made by nuclear magnetic resonance imaging (bilateral and multifocal lesions in the subcortical occipitoparietal and frontal left anterior white matter with involvement of U fibers) and a history of antirabies vaccination. The clinical course was marked by the appearance of motor and visual effects.

First report on the Moroccan registry of primary immunodeficiencies: 15 years of experience (1998-2012).

PURPOSE: Primary immunodeficiencies (PIDs) are a large group of diseases characterized by susceptibility to infections. We provide the first comprehensive report on PIDs in Morocco, the epidemiological, clinical, etiological and outcome features which have never before been described. METHODS: A national registry was established in 2008, grouping together data for PID patients diagnosed since 1998. RESULTS: In total, 421 patients were diagnosed between 1998 and 2012. Parental consanguinity was common (recorded for 43.2 % of patients) and the median time to diagnosis was 2.0 years. Overall, 27.4 % of patients were considered to have well defined syndromes with immunodeficiency (48 cases of hyper-IgE syndrome and 40 of ataxia-telangiectasia); 22.7 % had predominantly antibody deficiencies (29 cases of agammaglobulinemia and 24 of CVID); 20.6 % had combined immunodeficiencies (37 cases of SCID and 26 of MHC II deficiencies) and 17.5 % had phagocyte disorders (14 cases of SCN and 10 of CGD). The principal clinical signs were lower respiratory tract infections (60.8 %), skin infections (33.5 %) and candidiasis (26.1 %). Mortality reached 28.8 %, and only ten patients underwent bone marrow transplantation. We analyzed the impact on mortality of residence, family history, parental consanguinity, date of diagnosis and time to diagnosis, but only date of diagnosis had a significant effect. CONCLUSIONS: The observed prevalence of PID was 0.81/100,000 inhabitants, suggesting considerable underdiagnosis and a need to increase awareness of these conditions in Morocco. The distribution of PIDs was different from that reported in Western countries, with a particularly high proportion of SCID, MHC II deficiencies, hyper-IgE syndrome and autosomal recessive agammaglobulinemia. However, we have now organized a national network, which should improve diagnosis rates in remote regions.

[Moyamoya disease in children: a case report]. / Maladie de Moyamoya : à propos d'un cas pédiatrique.

Moyamoya is an angiogenic disorder and a rare cause of stroke. It is a progressive narrowing of cerebral arteries at the base of the brain involving the intracerebral portion of the internal carotid arteries, where it leads to development of collateral arteries, causing a "cigarette smoke" aspect. Ischemic events are more frequent in pediatric pathology, where hemorrhagic manifestations account for only 10%. The prognosis is severe, even worse than when onset of symptoms is earlier. We present the case of an 8-year-old child, who had presented recurrent hemiparesis since the age of 3 years; the brain CT scan and magnetic resonance imaging objectified injury sequelae. The MRA revealed stenosis of the internal carotid artery consistent with Moyamoya. In conclusion, the diagnosis of Moyamoya disease is now accessible to modern brain imaging techniques (MRI and MRA), easily achievable in children. The natural course of the disease involves the neurological and vital prognosis in young children, due to repeated ischemic attacks. Their identification would facilitate early diagnosis in order to treat neurosurgically, leading to a better neurological and cognitive prognosis.