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Problem considered Acute rheumatic fever (ARF) is a major problem of public health in developing countries like Morocco. This study was done to find out the clinical manifestations and laboratory features of patients with ARF in Fez city. Methods A retrospective study was conducted from 2009 to 2015 in the pediatric ward of the university hospital Hassan II of Fez. A total of 209 children ( Results The mean age of ARF cases were 11 years. The sex ratio male/female was 0.9. The most represented age class was the class between 5 and 15 years. Unschooled represented 2.9% and the most found was the primary level school (58.9%). Urban residence was found in 84.2% of cases and the majority of patients with and without carditis were admitted in winter and autumn respectively. Cases of ARF with carditis was documented in 53.1% and without carditis in 46.9%. In the two groups, arthritis was the major criterion most represented and arthralgia was the minor criterion most represented. History of sore throat was found in ARF with carditis cases (51.3%) more than ARF without carditis cases (35.7%). Penicillin A was the most antibiotic prescribed in the two groups. Commonest valvular lesions among ARF with carditis cases was mitral regurgitation (62.1%). There were 3 deaths among cases in this group. Conclusion ARF continues to occur in Morocco, despite the progress made in the socioeconomic development of the country, often associated with severe cardiac involvement.

Background Acute Articular Rheumatism (AAR) is a major public health problem in developing countries, due to the high prevalence of its main complication, which is rheumatic heart disease. The objective of this study is to determine the clinical and biological manifestations of rheumatic fever in children at CHU Hassan II in Fez, Morocco. Methods This is a prospective study carried out from January 2016 to July 2019 in the cardiopediatric unit of CHU Hassan II in Fez. Included were patients below 18 years of age. 152 children with RAA are diagnosed according to the modified Jones criteria. The patients are classified into 2 groups: group 1: RAA with carditis and group 2: RAA without carditis. Patient data is collected on operating sheets. Statistical analysis was performed using SPSS v 21 software. Results The average age of the patients was 12 years and 10 months. The male/female sex ratio was 0.7. The most represented age group was between 5 and 15 years (76.16%). Patients of urban origin presented 78.8%. The majority of children who are diagnosed with AAR with and without cardiac involvement are admitted in winter and fall respectively. A family history of RAA was found in 1 patient and recurrent tonsillitis were mentioned in 68.42%. Cardiac involvement is found in 78.3% of patients. Arthritis was the most common major end point in patients with and without carditis (24.4% and 12.1% respectively). Arthralgia was the most common minor outcome in these patients (67.2% and 78.8% respectively). Penicillin A was the most prescribed antibiotic in the two groups (80.83% and 79.31% respectively). The predominant valve disease in children with cardiac involvement is mitral leakage (69%). Serological proof of a streptococcal infection (ASLO> 300U/ml) was found in 37.2% of cases. A high biological inflammatory syndrome (ESR> 20) was noted in 29.4%. Corticosteroid therapy was used in 42.8% of patients with carditis and in 30.3% of patients without carditis. There was a recurrence in surveillance, this is an aggravation of the mitral leakage with in an 11-year-old boy. 6 patients (3.9%) underwent valve replacement: 4 valve replacements (mitral or aortic) and two double valve replacements (mitral and aortic). One death is noted, a form of acute carditis. Conclusion Cardiac involvement was very common in our series, which shows that AAR still remains a public health problem in our country. Keywords RAA, child, cardiac involvement

Background The assay of antistreptolysin O antibodies “ASLO” can provide evidence of infection of streptococcal origin, but it cannot confirm the presence of rheumatic fever “RAA” or the degree of severity of the disease. However, it is widely prescribed in daily practice by pediatricians and general practitioners to diagnose RAA. Unfortunately, the only finding of a high rate of ASLO in front of recurrent tonsillitis in children is considered by some practitioners as sufficient evidence to support the diagnosis of RAA, especially in its minor form, without taking into account the Jones criteria. The objective of this study is to put the ASLO assay in its place in the diagnosis of RAA in children in the region of Fez in Morocco. Methods This is a prospective study carried out from January 2016 to July 2019 in the cardiopediatric unit of CHU Hassan II in Fez. Patients below 18 years of age were included in this study. The children are classified into 4 groups: group 1: RAA with cardiac involvement, group 2: RAA without cardiac involvement, group 3: recurrent pharyngitis and group 4: control. Children with RAA are diagnosed according to the modified Jones criteria. The repetitive angina group includes any child who has tonsillitis >5 times a year. The control group presents healthy children. The ASLO assay was carried out in the central analysis laboratory of the CHU Hassan II in Fez. Patient data is collected on operating sheets. Statistical analysis was performed using SPSS v 21 software. Results These are 153 children with RAA: 119 cases of RAA with cardiac involvement and 34 cases of RAA without cardiac involvement, 86 children with recurrent pharyngitis and 157 children controls. The most common age group in the four groups is the age group between 5 and 15 years old. The frequency of children of urban origin is greater than that of rural origin in all the groups studied. The average ASLO rates in each group are as follows: RAA with carditis: 281.1 IU/ml RAA without carditis: 331.9 IU/ml Repeated angina: 397.7 IU/ml Control: 208.8 IU/ml Monitoring the change in ASLO, every 3 months for a year, in the groups studied showed that the ASLO level is stable at high rates in the case of recurrent pharyngitis and it tends to decrease in the case of AAR. Regarding the relationship between ASLO means and gender, there is a high ASLO mean in boys more than girls in all groups. Also, the mean ASLO is high in children from urban areas for the recurrent angina group. In addition, the average rate of ASLO is high during cold seasons in all groups. Finally, our study shows that the increase in ASLO is related to age. There is a significant difference between ASLO rates and age in all groups. Conclusion ASLO levels do not correlate with cardiac involvement but rather with infection and the child's individual immunity. The evidence is that ASLO levels> 200 IU/ml are more common with recurrent pharyngitis than with rheumatic heart disease. So a high ASLO is not necessarily related to an RAA. Keywords ASLO, children, RAA, recurrent tonsillitis

Background The antistreptolysins O “ASLO” are anti streptococcal antibodies directed against streptolysin O. The assay of ASLO can provide proof of infection of streptococcal origin. However, it cannot confirm the presence of RAA or the degree of severity of the disease. A level of ASLO less than or equal to a critical threshold of 200 IU/ml is considered normal in children. Several factors influence the ASLO title and therefore their standard rate such as age, geographic location and seasonal variation. The objective of this study is to determine the upper normal limit (ULN) of ASLO in healthy children with angina or RAA at the CHU Hassan II of Fez in Morocco. Methods This is a cross-sectional study carried out from January 2016 to July 2019 in the cardiopediatric unit of CHU Hassan II and the Anarjiss health center in Fez. Patients below 18 years of age were included in this study. The children are classified into 3 groups: group 1: 152 children with RAA and who are diagnosed according to the modified Jones criteria, group 2: 177 children with angina, group 3: 157 healthy children who did not have an RAA, a history of recurrent or recent pharyngitis. Patient data is collected on operating sheets. Statistical analysis was performed using SPSS v 21 software. To establish a normal upper limit for ASLOs, the 90 percentile was used as it is a value below which at least 90% of cases studied in each group. Results The most common age group in the 3 groups is the age group between 5 and 15 years old. The frequency of children of urban origin is greater than that of rural origin in all the groups studied. The ULN is 421.4U/ml in the group of normal children, 641.95 U/ml in the RAA group and 561.8 U/ml in the group of children with pharyngitis. The study of changes in ASLO rates over time shows that they increase significantly in angina and tend to decrease in RAA. The LSN of ASLOs by gender shows that it is higher for boys than for girls. The ULN according to the residence shows that it is higher in urban areas. As well as the LSN of ASLO according to the season shows that it is higher in the cold period, especially in winter and spring (P Conclusion In this study, it can be concluded that an ASLO level less than or equal to 400 U/ml is the critical threshold in a normal child, while an ASLO level >400 U/ml is considered pathological in children. children in Morocco.

Due to a processing error the author’s Doris Škorić-Milosavljević, Najim Lahrouchi, Alex V. Postma, Connie R. Bezzina were assigned to affiliation 38. However, affiliation 38 does not exist. In addition, the affiliations of Najim Lahrouchi, Elisabeth M. Lodder, and Connie R. Bezzina should be number 1 instead of number 2. The correct affiliation is Department of Clinical and Experimental Cardiology, Amsterdam University Medical Center, Amsterdam, The Netherlands. The original article has been corrected.

Multicentric Castleman’s disease is a rare benign and unexplained lymphoproliferative disorder that is extremely uncommon in children. It presents with fever, systemic symptoms, generalized lymphadenopathy, and laboratory markers of inflammation. Its treatment is not standardized and its prognosis is poor. We report a novel case of multicentric Castleman’s disease in a 13-year-old girl who had presented with chronic diarrhea as the only initial presenting symptom. The diagnosis of celiac or inflammatory bowel diseases was suspected, but two and a half years later, the diagnosis of multicentric Castleman’s disease was brought following the appearance of abdominal mass whose biopsy revealed Castleman’s disease in the plasma cell form. The outcome was favorable after treatment by corticosteroid, chemotherapy, and surgery. The occurrence of diarrhea as the initial symptom of multicentric Castleman’s disease without lymph node involvement is very rare. This case report underlines the diagnostic difficulties and the long interval between onset and diagnosis when diarrhea occurs first.

Les syndromes microdélétionnels sont définis par la présence d'une anomalie chromosomique de taille mineure (inférieure à 5 mégabases) ou aneusomie segmentaire, décelable par cytogénétique moléculaire (FISH: Fluorescent in Situ Hybridization). Les syndromes microdélétionnels représentent des syndromes cliniques avec des phénotypes suffisamment caractéristiques pour être reconnus cliniquement. Actuellement la FISH est la technique de choix pour rechercher ces syndromes. Plusieurs syndromes microdélétionnels peuvent être confirmés aisément, les plus recherchés sont Le syndrome de Williams (microdélétion en 7q11.23) et le syndrome de la délétion 22q11 (microdélétion en 22q11.2). Le syndrome de Williams est caractérisé par une anomalie du développement qui associe un retard psycho-moteur, une dysmorphie du visage évocatrice et un profil cognitif et comportemental spécifique, une sténose aortique supravalvulaire -SASV- le plus souvent. Le Syndrome de la délétion 22q11 se caractérise par l'association de plusieurs malformations d’expression variable: une cardiopathie congénitale de type conotroncal, une dysmorphie faciale discrète mais caractéristique et une hypoplasie du thymus et des parathyroïdes. Nous rapportons nos premières observations au CHU Hassan II confirmées par FISH : Syndrome de la délétion 22q11 (n:2) et un syndrome de Williams. Le but de cet article est la mise à jour de nos connaissances sur ces deux syndromes et la mise en valeur du rôle de la cytogénétique moléculaire dans le diagnostic et le conseil génétique des syndromes microdélétionnels.

Farber disease is a rare lysosomal storage disease characterized by a clinical triad including painful joint deformity, subcutaneous nodules and hoarseness, due to progressive granulomatous inflammation. We report the case of an early presentation on a female infant who manifested typical signs 1 week after birth.

A cleft sternum is a rare congenital anomaly. It can be isolated or combined with other defects particularly heart and vessel anomalies. We report a case of a newborn with a giant aortic aneurysm, admitted to our neonatal intensive care unit for respiratory distress.