Severe combined immunodeficiency (SCID) is a heterogeneous group of primary immunodeficiency dise... more Severe combined immunodeficiency (SCID) is a heterogeneous group of primary immunodeficiency diseases (PIDs) characterized by a lack of autologous T lymphocytes. This severe PID is rare, but has a higher prevalence in populations with high rates of consanguinity. The epidemiological, clinical, and immunological features of SCIDs in Moroccan patients have never been reported. The aim of this study was to provide a clinical and immunological description of SCID in Morocco and to assess changes in the care of SCID patients over time. This cross-sectional retrospective study included 96 Moroccan patients referred to the national PID reference center at Casablanca Children’s Hospital for SCID over two decades, from 1998 to 2019. The case definition for this study was age < 2 years, with a clinical phenotype suggestive of SCID, and lymphopenia, with very low numbers of autologous T cells, according to the IUIS Inborn Errors of Immunity classification. Our sample included 50 male patients, and 66% of the patients were born to consanguineous parents. The median age at onset and diagnosis were 3.3 and 6.5 months, respectively. The clinical manifestations commonly observed in these patients were recurrent respiratory tract infection (82%), chronic diarrhea (69%), oral candidiasis (61%), and failure to thrive (65%). The distribution of SCID phenotypes was as follows: T−B−NK+ in 44.5%, T−B−NK− in 32%, T−B+NK− in 18.5%, and T−B+NK+ in 5%. An Omenn syndrome phenotype was observed in 15 patients. SCID was fatal in 84% in the patients in our cohort, due to the difficulties involved in obtaining urgent access to hematopoietic stem cell transplantation, which, nevertheless, saved 16% of the patients. The autosomal recessive forms of the clinical and immunological phenotypes of SCID, including the T−B−NK+ phenotype in particular, were more frequent than those in Western countries. A marked improvement in the early detection of SCID cases over the last decade was noted. Despite recent progress in SCID diagnosis, additional efforts are required, for genetic confirmation and particularly for HSCT.
Pertussis, a vaccine preventable disease, is still responsible of significant morbidity and morta... more Pertussis, a vaccine preventable disease, is still responsible of significant morbidity and mortality around the world, mostly in newborns. The aim of the present study was (1) to introduce pertussis surveillance in the major pediatric hospital of Casablanca (2) to analyze the prevalence of pertussis among children under 14 years of age and their entourage in Casablanca, Morocco. This is a prospective and non-case controlled study, including children suspected of Pertussis admitted at the Abderrahim Harouchi Pediatric Hospital in Casablanca, from January 2013 to June 2015. Nasopharyngeal samples were obtained for Bordetella spp. culture and Real time PCR detection (RT-PCR) with specific primers of Bordetella spp., B. pertussis, B. parapertussis and B. holmesii. The detection of Bordetella spp. was also performed in some household contacts of the children suspected of pertussis. During the 2.5-years period, a total of 282 samples were collected from hospitalized children (156) and in...
The sickle-cell disease is a group of chronic hemolytic diseases which associates three types of ... more The sickle-cell disease is a group of chronic hemolytic diseases which associates three types of injuries: severe anemia, severe infections, and ischemic vaso-occlusive crisis that are secondary to conflicts between small vessels and red blood cells too deformable. Thus, organic various complications may arise. Its prevalence in Europe is estimated to be about 1/150 and reaches 15 % in the Mediterranean areas. Clinical manifestations vary widely from one person to another and from one moment to another. In addition to anemia and bacterial infections, vaso-occlusive crisis may manifest by focal ischemia. In the long term, the VOC may compromise the function of a particular tissue or organ. The transmission is autosomal recessive. The sickle-cell diseases are determined by combinations of two abnormal alleles of beta globin gene including at least one which carries the mutation beta 6 glu-val (Hb S). We report the case of a girl aged 11 years, who presented two strokes in the interval of 8 months, which manifested by a complete right hemiplegia and aphasia confirmed by head CT scan; the electrophoresis of the hemoglobin and the molecular test had confirmed the diagnosis of sickle-cell disease, and we were allowed to spread better reflection on the prevention of stroke, which remains a frequent and serious complication of sickle-cell disease.
This article reviews the problem of fever in young children its risks and complications as well a... more This article reviews the problem of fever in young children its risks and complications as well as the terms of its therapeutic care. Acute dehydration is the most uncommon complication mainly found in infants and it is the cause of fluid losses associated with evaporation due to high fever and associated digestive disorders such as vomiting and diarrhea. The fever induced feverish convulsions occur with infants and children younger than five; they last a few minutes with a brief loss of consciousness. The major hyperthermia is a syndrome combining an extremely high body temperature 41-42° Celsius a cardiovascular collapse and mainly visceral disorders (neurological and muscular) provoking subsequent heavy neurological disorders and a mortality rate climbing rapidly to 50%. It is then an absolute necessity to proceed rapidly with adequate care. In case of convulsions or clinical signs difficult to tolerate or the suspicion of a severe condition like meningitis or pneumonia the infan...
La fievre aigue sans orientation clinique est une situation frequente chez le nourrisson. Le risq... more La fievre aigue sans orientation clinique est une situation frequente chez le nourrisson. Le risque d'infection bacterienne severe impose un bilan biologique qui posera l'indication d'une antibiotherapie. Ainsi, chez les nourrissons de 6 a 36 mois ne presentant pas d'etat toxique, le bilan ne sera prescrit que si la temperature depasse 39°C. Les enfants presentant des bandelettes positives seront traites comme ayant des infections urinaires. Si le compte absolu des polynucleaires neutrophiles depasse 10 000/mm3 et ou la CRP depasse 40mg/l, la ceftriaxone sera prescrite en intramusculaire, en ambulatoire, a raison de 50mg/kg/j en une prise. Si l'hemoculture revient sterile, la ceftriaxone sera arretee.
Primary immunodeficiencies (PIDs) are a large group of diseases characterized by susceptibility t... more 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. A national registry was established in 2008, grouping together data for PID patients diagnosed since 1998. 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. 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.
Background: Recurrent bacterial and fungal infections, eczema, and increased serum IgE levels cha... more Background: Recurrent bacterial and fungal infections, eczema, and increased serum IgE levels characterize patients with the hyper-IgE syndrome (HIES). Known genetic causes for HIES are mutations in signal transducer and activator of transcription 3 (STAT3) and dedicator of cytokinesis 8 (DOCK8), which are involved in signal transduction pathways. However, glycosylation defects have not been described in patients with HIES. One crucial enzyme in the glycosylation pathway is phosphoglucomutase 3 (PGM3), which catalyzes a key step in the synthesis of uridine diphosphate N-acetylglucosamine, which is required for the biosynthesis of N-glycans. Objective: We sought to elucidate the genetic cause in patients with HIES who do not carry mutations in STAT3 or DOCK8. Methods: After establishing a linkage interval by means of SNPchip genotyping and homozygosity mapping in 2 families with HIES from Tunisia, mutational analysis was performed with selector-based, high-throughput sequencing. Protein expression was analyzed by means of Western blotting, and glycosylation was profiled by using mass spectrometry. Results: Mutational analysis of candidate genes in an 11.9-Mb linkage region on chromosome 6 shared by 2 multiplex families identified 2 homozygous mutations in PGM3 that segregated
Streptococcus intermedius is a member of the Streptococcus anginosus group, also known as the Str... more Streptococcus intermedius is a member of the Streptococcus anginosus group, also known as the Streptococcus milleri group. Although this is a commensal agent of the mouth and upper airways, it has been recognized as an important pathogen in the formation of abscesses. However, it has rarely been involved in the formation of brain abscess in children. We report 4 pediatric cases of brain abscess caused by S. intermedius. Three boys and 1 girl, all aged over 2 years, were admitted for a febrile meningeal syndrome and seizures, caused by a S. intermedius brain abscess. Diagnosis was obtained by brain imaging combined with culture of cerebrospinal fluid. The outcome was favorable after antibiotic therapy and abscess puncture. S. intermedius should be considered a potential pathogen involved in the development of brain abscess in children.
de 6 % pour érythromycine, 16 % pour amoxicilline (+ 13 % de sensibilité intermédiaire), 45 % pou... more de 6 % pour érythromycine, 16 % pour amoxicilline (+ 13 % de sensibilité intermédiaire), 45 % pour les fl uoroquinolones et 61 % pour tétracycline. Conclusion-Les résultats obtenus par l'étude menée entre 2000 et 2006 placent Campylobacter comme deuxième agent pathogène des diarrhées infectieuses bactériennes de l'enfant après les salmonelles, donnée méconnue des cliniciens probablement à cause de la lenteur de culture de Campylobacter. Sensibilisés à cette épidémiologie, nous avons recherché systématiquement Campylobacter dans les GEA même en période épidémique virale ce qui a permis d'améliorer nos diagnostics étiologiques en triplant la fréquence annuelle des Campylobacter. Les données cliniques sont identiques : diarrhées fébriles, sanglantes et peu douloureuses. L'échographie est souvent anormale avec des lésions de colite étendue. 22 % des enfants ont été traités par une antibiothérapie adaptée contrairement à la cohorte précédente ou seuls 2 enfants/71 avaient bénéfi cié du traitement par les macrolides.
Le cytomégalovirus (CMV) est responsable de la plus fréquente des infections virales congénitales... more Le cytomégalovirus (CMV) est responsable de la plus fréquente des infections virales congénitales. Les conséquences chez le nouveau-né sont potentiellement graves. A travers une observation récente de maladie des inclusions cytomégaliques chez un nouveau né, nous faisons le point sur cette foetopathie grave sur le plan épidémiologique, clinique, thérapeutique et évolutive. Observation : Une fi lle est née à terme par voie basse d'une mère âgée de 26 ans. L'Apgar à la naissance 9/10, respectivement à 1 et 5 minutes. Le poids était de 3500 g, la taille de 46 cm et le périmètre crânien de 31 cm. Elle présentait dès la naissance un tableau infectieux septicémique associant purpura pétéchial, hépatosplénomegalie, ictère, thrombopénie à 15000/mm, atteinte hépatique cytolytique (ALAT : 34 U/l, ASAT : 207 U/l), et une bilirubinémie totale à 60 mg/l et directe à 15 mg/l, une infection à cmV a été mise en évidence par une virurie positive à j 3 de vie (trois Échantillons). La sérologie a montré des Ig M spécifi ques négatives et Ig G positives. L'étude du LCR a montré 160 GR et 25 GB (100 % lymphocytaire), et une protéinorrachie à 2,62g/l. La TDM cérébrale a montré des calcifi cations périventriculaires, une dilatation quadriventriculaire avec hémorragie intraventriculaire, et une atrophie corticale soustentorielle. L'évolution a été marquée par une augmentation rapide des pétéchies et ecchymoses, et une apparition d'une détresse respiratoire ayant nécessité une intubation et une ventilation assistée pendant 48 heures. Le fond d'oeil a révélé une atrophie choriorétiniènne, mais pas de choriorétinite. Un traitement par ganciclovir a été débuté à j4 de vie à la dose de 10 mg/kg/j par voie intraveineuse lente toutes les 12 heures pendant 6 semaines. Après 4 semaines de traitement, l'ictère et les pétéchies ont disparu, les transaminases sont revenues à des taux normaux et la recherche de cmV dans les urines était négative. À deux mois de vie l'hépatosplénomegalie s'étaient nettement réduite. Les Potentiels évoqués auditif étaient altéré à gauche et conservé à droite. Le traitement a été bien toléré, les effets secondaires imputables au ganciclovir été limités à une anémie ayant nécessité une transfusion de culots globulaire à deux reprises.
An increased incidence and severity of invasive group A streptococcus (GAS) infections over the p... more An increased incidence and severity of invasive group A streptococcus (GAS) infections over the past decade have been reported by several authors, but GAS remains an uncommon cause of bacterial meningitis. The aim of this study was to describe and analyze the clinical and biological data of GAS meningitis by reporting 10 new cases of pediatric GAS meningitis and making a literature review. The mean age of patients, seven girls and three boys, was 3 years. There was a history of preexisting or concomitant community-acquired infection in five patients over 10. The outcome was fatal in two cases. All patients received an initial empirical antimicrobial therapy with a third generation cephalosporin switched in six cases to amoxicillin. The prognosis for this type of streptococcal meningitis is usually good, but death may occur even in children without any identified risk factor for severe infection.
UNLABELLED Non-typhoidal Salmonella (NTS) infections are a major cause of infantile death in deve... more UNLABELLED Non-typhoidal Salmonella (NTS) infections are a major cause of infantile death in developing countries. OBJECTIVE The aim of this study was to determine the epidemiologic and therapeutic data, as well as the evolution of NTS in Morocco. METHOD This retrospective study was made on 41 patients hospitalized for NTS between 1994 and 2002 in the Casablanca University Hospital Pediatric ward. RESULTS Twenty cases of digestive salmonellosis were diagnosed, 16 cases of septicemia, and 10 cases of meningitis. Ten patients were hospitalized after an outbreak of resistant Salmonella typhimurium in a nursery. Fifty percent of the patients were less than 3 months of age. The three patients between 1 and 3 years of age presented with primary immunodeficiency. Fever, vomiting, and diarrhea were noted in 97% of the cases. The stools were watery in 89% and severe dehydration in 55% of the cases. Salmonella strains were identified in blood in 25 cases, from stools in 10 cases, and from CSF...
Les cellulites orbitaires sont rares. Mais le risque eleve de survenue de complications graves oc... more Les cellulites orbitaires sont rares. Mais le risque eleve de survenue de complications graves oculaires et neurologiques impose un diagnostic precoce et un traitement adequat. Nous rapportons retrospectivement 33 observations colligees au Service des Maladies Infectieuses Pediatriques de l'Hopital d'Enfants de Casablanca de 1994 a 2000. Vingt quatre malades ont presente une cellulite preseptale et les neuf autres une cellulite retroseptale. La porte d'entree etait representee par une sinusite (10 cas), une pyodermite (8 cas), une plaie infectee (6 cas), une infection oculaire (2 cas) et un abces dentaire (2 cas). L'âge des malades variait de 40 jours a 15 ans avec une moyenne de 7 ans. Les nourrissons representaient 25% des cas et avaient exclusivement une cellulite preseptale. la fievre et un oedeme local etaient presents chez tous les malades. Une exophtalmie a ete notee chez 6 malades et des convulsions chez 2 malades. Les neuf cas de cellulites retroseptales eta...
Acquired hypothyroidism in child is a rare and unknown pathology. It is often secondary to an aut... more Acquired hypothyroidism in child is a rare and unknown pathology. It is often secondary to an auto-immune chronic thyroiditis. In child, association hypothyroidism and celiac disease was rarely described. The authors report an observation of child presenting an association of hypothyroidism and celiac disease. A child, 30 months old year, without family antecedents of auto-immune pathology, the weight at birth was 5 kg, a test of tracking of the congenital hypothyroidism was revealed negative. At the 24 months age, this infant presented a stop of growth, a psychomotor slowing. The hormonal and immunological diagnostic test allowed posing the diagnosis of hypothyroidism at the 30 months age. After 6 month of hormonal treatment led good, the infant presented a clinical and biological relapse of hypothyroidism associated to a chronic diarrhea. The bilan of malabsorption and jejunal biopsy done at the 36 months age showed a total jejunal atrophy making keep the association of celiac dis...
Orbital cellulitis is rare. However the high risk of severe ocular and neurological complications... more Orbital cellulitis is rare. However the high risk of severe ocular and neurological complications make early diagnosis and adequate therapy essential. The purpose of this retrospective study is to describe 33 cases observed in the pediatric infectious disease department of the Casablanca Children's Hospital in Morocco from 1994 to 2000. Orbital cellulitis was preseptal in 24 patients and retroseptal in 9. Infection occurred in relation with sinusitis in 10 cases, polydermitis in 8, wound infection in 6, ocular infection in 2, and dental abscess in 2. Ages ranged from 40 days to 15 years with a mean age of 5 years. Infants accounted for 25% of cases and always presented preseptal cellulitis. Fever and local edema were noted in all patients. Exophthalmia occurred in six patients and seizures in 2. The 9 cases of retroseptal cellulitis were complicated by empyema in 2 cases, meningitis in 1 case and thrombophlebitis of cavernous sinus with cerebromalacia in 1 case. Bacteriological ...
The International Journal of Tuberculosis and Lung Disease, 2015
Setting-Tuberculosis spondylodiscitis (TS) or Pott's disease is an extra-pulmonary form of TB tha... more Setting-Tuberculosis spondylodiscitis (TS) or Pott's disease is an extra-pulmonary form of TB that is rare and difficult to diagnose in children. Some cases of severe TB in children were recently explained by inborn errors of immunity affecting the IL-12/IFN-γ axis.
Severe combined immunodeficiency (SCID) is a heterogeneous group of primary immunodeficiency dise... more Severe combined immunodeficiency (SCID) is a heterogeneous group of primary immunodeficiency diseases (PIDs) characterized by a lack of autologous T lymphocytes. This severe PID is rare, but has a higher prevalence in populations with high rates of consanguinity. The epidemiological, clinical, and immunological features of SCIDs in Moroccan patients have never been reported. The aim of this study was to provide a clinical and immunological description of SCID in Morocco and to assess changes in the care of SCID patients over time. This cross-sectional retrospective study included 96 Moroccan patients referred to the national PID reference center at Casablanca Children’s Hospital for SCID over two decades, from 1998 to 2019. The case definition for this study was age < 2 years, with a clinical phenotype suggestive of SCID, and lymphopenia, with very low numbers of autologous T cells, according to the IUIS Inborn Errors of Immunity classification. Our sample included 50 male patients, and 66% of the patients were born to consanguineous parents. The median age at onset and diagnosis were 3.3 and 6.5 months, respectively. The clinical manifestations commonly observed in these patients were recurrent respiratory tract infection (82%), chronic diarrhea (69%), oral candidiasis (61%), and failure to thrive (65%). The distribution of SCID phenotypes was as follows: T−B−NK+ in 44.5%, T−B−NK− in 32%, T−B+NK− in 18.5%, and T−B+NK+ in 5%. An Omenn syndrome phenotype was observed in 15 patients. SCID was fatal in 84% in the patients in our cohort, due to the difficulties involved in obtaining urgent access to hematopoietic stem cell transplantation, which, nevertheless, saved 16% of the patients. The autosomal recessive forms of the clinical and immunological phenotypes of SCID, including the T−B−NK+ phenotype in particular, were more frequent than those in Western countries. A marked improvement in the early detection of SCID cases over the last decade was noted. Despite recent progress in SCID diagnosis, additional efforts are required, for genetic confirmation and particularly for HSCT.
Pertussis, a vaccine preventable disease, is still responsible of significant morbidity and morta... more Pertussis, a vaccine preventable disease, is still responsible of significant morbidity and mortality around the world, mostly in newborns. The aim of the present study was (1) to introduce pertussis surveillance in the major pediatric hospital of Casablanca (2) to analyze the prevalence of pertussis among children under 14 years of age and their entourage in Casablanca, Morocco. This is a prospective and non-case controlled study, including children suspected of Pertussis admitted at the Abderrahim Harouchi Pediatric Hospital in Casablanca, from January 2013 to June 2015. Nasopharyngeal samples were obtained for Bordetella spp. culture and Real time PCR detection (RT-PCR) with specific primers of Bordetella spp., B. pertussis, B. parapertussis and B. holmesii. The detection of Bordetella spp. was also performed in some household contacts of the children suspected of pertussis. During the 2.5-years period, a total of 282 samples were collected from hospitalized children (156) and in...
The sickle-cell disease is a group of chronic hemolytic diseases which associates three types of ... more The sickle-cell disease is a group of chronic hemolytic diseases which associates three types of injuries: severe anemia, severe infections, and ischemic vaso-occlusive crisis that are secondary to conflicts between small vessels and red blood cells too deformable. Thus, organic various complications may arise. Its prevalence in Europe is estimated to be about 1/150 and reaches 15 % in the Mediterranean areas. Clinical manifestations vary widely from one person to another and from one moment to another. In addition to anemia and bacterial infections, vaso-occlusive crisis may manifest by focal ischemia. In the long term, the VOC may compromise the function of a particular tissue or organ. The transmission is autosomal recessive. The sickle-cell diseases are determined by combinations of two abnormal alleles of beta globin gene including at least one which carries the mutation beta 6 glu-val (Hb S). We report the case of a girl aged 11 years, who presented two strokes in the interval of 8 months, which manifested by a complete right hemiplegia and aphasia confirmed by head CT scan; the electrophoresis of the hemoglobin and the molecular test had confirmed the diagnosis of sickle-cell disease, and we were allowed to spread better reflection on the prevention of stroke, which remains a frequent and serious complication of sickle-cell disease.
This article reviews the problem of fever in young children its risks and complications as well a... more This article reviews the problem of fever in young children its risks and complications as well as the terms of its therapeutic care. Acute dehydration is the most uncommon complication mainly found in infants and it is the cause of fluid losses associated with evaporation due to high fever and associated digestive disorders such as vomiting and diarrhea. The fever induced feverish convulsions occur with infants and children younger than five; they last a few minutes with a brief loss of consciousness. The major hyperthermia is a syndrome combining an extremely high body temperature 41-42° Celsius a cardiovascular collapse and mainly visceral disorders (neurological and muscular) provoking subsequent heavy neurological disorders and a mortality rate climbing rapidly to 50%. It is then an absolute necessity to proceed rapidly with adequate care. In case of convulsions or clinical signs difficult to tolerate or the suspicion of a severe condition like meningitis or pneumonia the infan...
La fievre aigue sans orientation clinique est une situation frequente chez le nourrisson. Le risq... more La fievre aigue sans orientation clinique est une situation frequente chez le nourrisson. Le risque d'infection bacterienne severe impose un bilan biologique qui posera l'indication d'une antibiotherapie. Ainsi, chez les nourrissons de 6 a 36 mois ne presentant pas d'etat toxique, le bilan ne sera prescrit que si la temperature depasse 39°C. Les enfants presentant des bandelettes positives seront traites comme ayant des infections urinaires. Si le compte absolu des polynucleaires neutrophiles depasse 10 000/mm3 et ou la CRP depasse 40mg/l, la ceftriaxone sera prescrite en intramusculaire, en ambulatoire, a raison de 50mg/kg/j en une prise. Si l'hemoculture revient sterile, la ceftriaxone sera arretee.
Primary immunodeficiencies (PIDs) are a large group of diseases characterized by susceptibility t... more 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. A national registry was established in 2008, grouping together data for PID patients diagnosed since 1998. 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. 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.
Background: Recurrent bacterial and fungal infections, eczema, and increased serum IgE levels cha... more Background: Recurrent bacterial and fungal infections, eczema, and increased serum IgE levels characterize patients with the hyper-IgE syndrome (HIES). Known genetic causes for HIES are mutations in signal transducer and activator of transcription 3 (STAT3) and dedicator of cytokinesis 8 (DOCK8), which are involved in signal transduction pathways. However, glycosylation defects have not been described in patients with HIES. One crucial enzyme in the glycosylation pathway is phosphoglucomutase 3 (PGM3), which catalyzes a key step in the synthesis of uridine diphosphate N-acetylglucosamine, which is required for the biosynthesis of N-glycans. Objective: We sought to elucidate the genetic cause in patients with HIES who do not carry mutations in STAT3 or DOCK8. Methods: After establishing a linkage interval by means of SNPchip genotyping and homozygosity mapping in 2 families with HIES from Tunisia, mutational analysis was performed with selector-based, high-throughput sequencing. Protein expression was analyzed by means of Western blotting, and glycosylation was profiled by using mass spectrometry. Results: Mutational analysis of candidate genes in an 11.9-Mb linkage region on chromosome 6 shared by 2 multiplex families identified 2 homozygous mutations in PGM3 that segregated
Streptococcus intermedius is a member of the Streptococcus anginosus group, also known as the Str... more Streptococcus intermedius is a member of the Streptococcus anginosus group, also known as the Streptococcus milleri group. Although this is a commensal agent of the mouth and upper airways, it has been recognized as an important pathogen in the formation of abscesses. However, it has rarely been involved in the formation of brain abscess in children. We report 4 pediatric cases of brain abscess caused by S. intermedius. Three boys and 1 girl, all aged over 2 years, were admitted for a febrile meningeal syndrome and seizures, caused by a S. intermedius brain abscess. Diagnosis was obtained by brain imaging combined with culture of cerebrospinal fluid. The outcome was favorable after antibiotic therapy and abscess puncture. S. intermedius should be considered a potential pathogen involved in the development of brain abscess in children.
de 6 % pour érythromycine, 16 % pour amoxicilline (+ 13 % de sensibilité intermédiaire), 45 % pou... more de 6 % pour érythromycine, 16 % pour amoxicilline (+ 13 % de sensibilité intermédiaire), 45 % pour les fl uoroquinolones et 61 % pour tétracycline. Conclusion-Les résultats obtenus par l'étude menée entre 2000 et 2006 placent Campylobacter comme deuxième agent pathogène des diarrhées infectieuses bactériennes de l'enfant après les salmonelles, donnée méconnue des cliniciens probablement à cause de la lenteur de culture de Campylobacter. Sensibilisés à cette épidémiologie, nous avons recherché systématiquement Campylobacter dans les GEA même en période épidémique virale ce qui a permis d'améliorer nos diagnostics étiologiques en triplant la fréquence annuelle des Campylobacter. Les données cliniques sont identiques : diarrhées fébriles, sanglantes et peu douloureuses. L'échographie est souvent anormale avec des lésions de colite étendue. 22 % des enfants ont été traités par une antibiothérapie adaptée contrairement à la cohorte précédente ou seuls 2 enfants/71 avaient bénéfi cié du traitement par les macrolides.
Le cytomégalovirus (CMV) est responsable de la plus fréquente des infections virales congénitales... more Le cytomégalovirus (CMV) est responsable de la plus fréquente des infections virales congénitales. Les conséquences chez le nouveau-né sont potentiellement graves. A travers une observation récente de maladie des inclusions cytomégaliques chez un nouveau né, nous faisons le point sur cette foetopathie grave sur le plan épidémiologique, clinique, thérapeutique et évolutive. Observation : Une fi lle est née à terme par voie basse d'une mère âgée de 26 ans. L'Apgar à la naissance 9/10, respectivement à 1 et 5 minutes. Le poids était de 3500 g, la taille de 46 cm et le périmètre crânien de 31 cm. Elle présentait dès la naissance un tableau infectieux septicémique associant purpura pétéchial, hépatosplénomegalie, ictère, thrombopénie à 15000/mm, atteinte hépatique cytolytique (ALAT : 34 U/l, ASAT : 207 U/l), et une bilirubinémie totale à 60 mg/l et directe à 15 mg/l, une infection à cmV a été mise en évidence par une virurie positive à j 3 de vie (trois Échantillons). La sérologie a montré des Ig M spécifi ques négatives et Ig G positives. L'étude du LCR a montré 160 GR et 25 GB (100 % lymphocytaire), et une protéinorrachie à 2,62g/l. La TDM cérébrale a montré des calcifi cations périventriculaires, une dilatation quadriventriculaire avec hémorragie intraventriculaire, et une atrophie corticale soustentorielle. L'évolution a été marquée par une augmentation rapide des pétéchies et ecchymoses, et une apparition d'une détresse respiratoire ayant nécessité une intubation et une ventilation assistée pendant 48 heures. Le fond d'oeil a révélé une atrophie choriorétiniènne, mais pas de choriorétinite. Un traitement par ganciclovir a été débuté à j4 de vie à la dose de 10 mg/kg/j par voie intraveineuse lente toutes les 12 heures pendant 6 semaines. Après 4 semaines de traitement, l'ictère et les pétéchies ont disparu, les transaminases sont revenues à des taux normaux et la recherche de cmV dans les urines était négative. À deux mois de vie l'hépatosplénomegalie s'étaient nettement réduite. Les Potentiels évoqués auditif étaient altéré à gauche et conservé à droite. Le traitement a été bien toléré, les effets secondaires imputables au ganciclovir été limités à une anémie ayant nécessité une transfusion de culots globulaire à deux reprises.
An increased incidence and severity of invasive group A streptococcus (GAS) infections over the p... more An increased incidence and severity of invasive group A streptococcus (GAS) infections over the past decade have been reported by several authors, but GAS remains an uncommon cause of bacterial meningitis. The aim of this study was to describe and analyze the clinical and biological data of GAS meningitis by reporting 10 new cases of pediatric GAS meningitis and making a literature review. The mean age of patients, seven girls and three boys, was 3 years. There was a history of preexisting or concomitant community-acquired infection in five patients over 10. The outcome was fatal in two cases. All patients received an initial empirical antimicrobial therapy with a third generation cephalosporin switched in six cases to amoxicillin. The prognosis for this type of streptococcal meningitis is usually good, but death may occur even in children without any identified risk factor for severe infection.
UNLABELLED Non-typhoidal Salmonella (NTS) infections are a major cause of infantile death in deve... more UNLABELLED Non-typhoidal Salmonella (NTS) infections are a major cause of infantile death in developing countries. OBJECTIVE The aim of this study was to determine the epidemiologic and therapeutic data, as well as the evolution of NTS in Morocco. METHOD This retrospective study was made on 41 patients hospitalized for NTS between 1994 and 2002 in the Casablanca University Hospital Pediatric ward. RESULTS Twenty cases of digestive salmonellosis were diagnosed, 16 cases of septicemia, and 10 cases of meningitis. Ten patients were hospitalized after an outbreak of resistant Salmonella typhimurium in a nursery. Fifty percent of the patients were less than 3 months of age. The three patients between 1 and 3 years of age presented with primary immunodeficiency. Fever, vomiting, and diarrhea were noted in 97% of the cases. The stools were watery in 89% and severe dehydration in 55% of the cases. Salmonella strains were identified in blood in 25 cases, from stools in 10 cases, and from CSF...
Les cellulites orbitaires sont rares. Mais le risque eleve de survenue de complications graves oc... more Les cellulites orbitaires sont rares. Mais le risque eleve de survenue de complications graves oculaires et neurologiques impose un diagnostic precoce et un traitement adequat. Nous rapportons retrospectivement 33 observations colligees au Service des Maladies Infectieuses Pediatriques de l'Hopital d'Enfants de Casablanca de 1994 a 2000. Vingt quatre malades ont presente une cellulite preseptale et les neuf autres une cellulite retroseptale. La porte d'entree etait representee par une sinusite (10 cas), une pyodermite (8 cas), une plaie infectee (6 cas), une infection oculaire (2 cas) et un abces dentaire (2 cas). L'âge des malades variait de 40 jours a 15 ans avec une moyenne de 7 ans. Les nourrissons representaient 25% des cas et avaient exclusivement une cellulite preseptale. la fievre et un oedeme local etaient presents chez tous les malades. Une exophtalmie a ete notee chez 6 malades et des convulsions chez 2 malades. Les neuf cas de cellulites retroseptales eta...
Acquired hypothyroidism in child is a rare and unknown pathology. It is often secondary to an aut... more Acquired hypothyroidism in child is a rare and unknown pathology. It is often secondary to an auto-immune chronic thyroiditis. In child, association hypothyroidism and celiac disease was rarely described. The authors report an observation of child presenting an association of hypothyroidism and celiac disease. A child, 30 months old year, without family antecedents of auto-immune pathology, the weight at birth was 5 kg, a test of tracking of the congenital hypothyroidism was revealed negative. At the 24 months age, this infant presented a stop of growth, a psychomotor slowing. The hormonal and immunological diagnostic test allowed posing the diagnosis of hypothyroidism at the 30 months age. After 6 month of hormonal treatment led good, the infant presented a clinical and biological relapse of hypothyroidism associated to a chronic diarrhea. The bilan of malabsorption and jejunal biopsy done at the 36 months age showed a total jejunal atrophy making keep the association of celiac dis...
Orbital cellulitis is rare. However the high risk of severe ocular and neurological complications... more Orbital cellulitis is rare. However the high risk of severe ocular and neurological complications make early diagnosis and adequate therapy essential. The purpose of this retrospective study is to describe 33 cases observed in the pediatric infectious disease department of the Casablanca Children's Hospital in Morocco from 1994 to 2000. Orbital cellulitis was preseptal in 24 patients and retroseptal in 9. Infection occurred in relation with sinusitis in 10 cases, polydermitis in 8, wound infection in 6, ocular infection in 2, and dental abscess in 2. Ages ranged from 40 days to 15 years with a mean age of 5 years. Infants accounted for 25% of cases and always presented preseptal cellulitis. Fever and local edema were noted in all patients. Exophthalmia occurred in six patients and seizures in 2. The 9 cases of retroseptal cellulitis were complicated by empyema in 2 cases, meningitis in 1 case and thrombophlebitis of cavernous sinus with cerebromalacia in 1 case. Bacteriological ...
The International Journal of Tuberculosis and Lung Disease, 2015
Setting-Tuberculosis spondylodiscitis (TS) or Pott's disease is an extra-pulmonary form of TB tha... more Setting-Tuberculosis spondylodiscitis (TS) or Pott's disease is an extra-pulmonary form of TB that is rare and difficult to diagnose in children. Some cases of severe TB in children were recently explained by inborn errors of immunity affecting the IL-12/IFN-γ axis.
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Papers by Z. Jouhadi