Blueberry muffin syndrome

Le Blueberry Muffin Baby est un syndrome cutané rare observé en période néonatale. Il est caractérisé par des papulo-nodules disséminés inflammatoires traduisant des réactions d’hématopoïèse dermique. Plusieurs causes doivent être recherchées, notamment les infections congénitales, une hémolyse sévère et les pathologies tumorales. Nous rapportons l’observation d’un nouveau-né chez qui l’aspect d’un Blueberry muffin baby a conduit au diagnostic d’une leucémie aiguë myéloïde.Pan African Medical Journal 2012; 13:2

La syphilis congénitale révélée par une fracture spontanée

Alors qu'elle est actuellement oubliée dans les pays développés, la syphilis congénitale se voit encore chez nous faute du dépistage anténatal. Ses formes cliniques sont polymorphes et orientent à tord vers d'autres pathologies surtout en période néonatale. Le diagnostic n'est donc pas toujours facile. La révélation d'une syphilis congénitale par une fracture spontanée est exceptionnellement décrite. Nous rapportons dans ce travail le cas d'un nourrisson de 2 mois ramené en consultation pour limitation douloureuse des mouvements du bras droit. Le diagnostic est évoqué sur les données radiologiques et confirmé par la sérologie syphilitique. Le traitement a reposé essentiellement sur l'administration de la pénicilline G avec une bonne évolution clinique

Pediatric recurrent respiratory tract infections: when and how to explore the immune system? (About 53 cases)

Recurrent respiratory tract infections are one of the most frequent reasons for pediatric visits and hospitalization. Causes of this pathology are multiple ranging from congenital to acquired and local to general. Immune deficiencies are considered as underlying conditions predisposing to this pathology. Our work is about to determine when and how to explore the immune system when facing recurrent respiratory infections. This was based on the records of 53 children hospitalized at the pediatrics unit of Hassan II University Hospital, Fez Morocco. Thirty boys and 23 girls with age ranging from 5 months to 12 years with an average age of 2 years were involved in this study. Bronchial foreign body was the main etiology in children of 3 to 6 year old. Gastro-esophageal reflux, which in some cases is a consequence of chronic cough, as well as asthma were most frequent in infants (17 and 15% respectively). Immune deficiency was described in 7.5% of patients and the only death we deplored in our series belongs to this group. Recurrent respiratory tract infections have multiple causes. In our series they are dominated by foreign body inhalation and gastroesophageal reflux, which in some cases is a consequence of a chronic cough. Immune deficiency is not frequent but could influence the prognosis. Therefore immune explorations should be well codified.Pan African Medical Journal 2016; 2

La maladie de Wilson chez l’enfant: à propos de 20 cas

La maladie de Wilson ou dégénérescence hépato-lenticulaire est une affection génétique autosomique récessive caractérisée par une accumulation toxique de cuivre dans l'organisme, essentiellement dans le foie, le système nerveux central et la cornée. L'objectif de ce travail était de soulever les difficultés diagnostiques et thérapeutiques dans la prise en charge de la maladie de Wilson dans notre contexte. Nous avons réalisé une étude rétrospective portant sur 20 cas de maladie de Wilson colligés au sein du service de pédiatrie du CHU HASSAN II de Fès sur une période de 7 ans et demi. Il s'agit de 13 garçons et 7 filles dont l'âge moyen est de 9 ans avec des extrêmes allant de 5 à 13 ans. La consanguinité est retrouvée chez 13 malades. Sur le plan clinique, l'ictère est noté dans 13 cas, un syndrome oedémateux est retrouvé dans 13 cas aussi et un syndrome hémorragique dans 6 cas. Les signes neurologiques sont présents dans 7cas. Trois enfants étaient asymptomatiques diagnostiqués à l'occasion d'un dépistage. Sur le plan biologique les signes d'insuffisance hépatocellulaire sont retrouvés chez 17 malades avec une cytolyse dans 8 cas. Une anémie hémolytique est retrouvée chez 8 malades (soit 40%). La céruléoplasminémie est abaissée chez 17 malades, la cuprurie réalisée chez 19 malades s'est révélée augmentée chez 17 soit 89,4%. L'anneau de Kayser- Fleischer est retrouvé chez 14 patients. L'échographie abdominale a montré des signes d'hypertension portale (HTP) sur foie de cirrhose chez 16 malades soit 80%. La D pénicillamine est instaurée chez 17 patients et trois sont mis sous sulfate de zinc. Trois malades ont bénéficié de la vitamine B6. L'évolution est favorable chez 11 malades avec un recul moyen de 3 ans. Nous déplorons 4 décès chez des malades ayant consulté au stade de cirrhose décompensé. Le pronostic de la maladie de Wilson dépend de la précocité du traitement. Le dépistage chez les membres de la famille est une démarche importante et obligatoire pour un diagnostic précoce.Pan African Medical Journal 2013; 14:

Haemolytic disease of the newborn due to anti-c

Anti-D isoimmunisation remains the most common cause of erythroblastosis fetalis. Whereas most clinically significant blood group sensitisations noted during pregnancy are still secondary to anti-D incompatibility, sensitisation to antigens other than D in the CDE system is not uncommon and can cause severe disease. The widespread use of Rh-D immune globulin has led to a relative increase in the importance of non-Rh-D isoimmunisation as a cause of haemolytic disease of the newborn. We report the case of a baby with severe hyperbilirubinaemia and persistent anaemia due to anti-c isoimmunisation with a high-titre maternal c antibody. The baby required emergency transfusion and intensive phototherapy. The medical literature relating to maternal c isoimmunisation and neonatal outcome is also reviewed. Because of its ability to cause clinically significant haemolytic disease of the newborn, practitioners must manage anti-c isoimmunisation in a manner similar to that for anti-D

Intestinal carriage of antibiotic resistant Acinetobacter baumannii among newborns hospitalized in Moroccan neonatal intensive care unit.

This study was conducted in order to assess the acquisition rate of Acinetobacter baumannii by newborn screening, on admission and during the discharge process of neonatal intensive care unit. (NICU). Furthermore, we investigated risk factors for potential colonization and molecular epidemiology of isolated resistant bacteria. This prospective study was conducted in the neonatal unit of Hassan II University Hospital of Fez from February 2013 to July 2015. During this period, all consecutive admitted neonates were screened for A. baumannii intestinal carriage, on admission and during the discharge process. Bacteriological and molecular tests were evaluated according to the international standards. This study examines the screening on admission of 455 newborns, 59% of whom were male. The average gestational age and birth weight were 35.2 weeks and 2612.1 g respectively. In total, 277 patients were included in the acquisition study on admission. The prevalence of multi-drug resistant (MDR) A. baumannii strain carriage was 6.5%, while the acquisition rate during the hospital recovery was 13.7%. In this study, 68 MDR A. baumannii isolates were collected. The resistance rates to different antibiotic classes including, Ceftazidime, Gentamycin and Ciprofloxacin varied between 92 and 100%. Moreover, 13% of MDR A. baumannii isolates were carbapenemase producers and 88% harbored blaOXA-23 gene. On admission, three risk factors were significantly associated with A. baumannii colonization: age (OR, 2.803; IC95%, 1.191-6.596; P = 0.01), gender (OR, 0.382; IC95%, 0.158-0.921; P = 0.03) and the delivery birth at the Maternity of University Hospital (MUH), (OR, 0.196; IC95%, 0.071-0.540; P = 0.002). However during hospitalization, the only risk factor associated with acquisition of A. baumannii was the respiratory distress (OR, 2.270; IC95%, 1.055-4.881; P = 0.03). A high intestinal carriage rate of A. baumannii and multiple antibiotic resistance were found in our NICU. Thus, the spread of MDR A. baumannii should be monitored by an active surveillance strategy

Cerebral ischaemic stroke and bilateral pheochromocytoma

We report a case of a 10-year-old girl who presented with an acute neurological deficit preceded by a 2 month history of headaches. The patient was treated for acute viral encephalitis because of the presence of inflammation. A computed tomography (CT) scan of her brain scan showed a cerebral ischaemic stroke, and paroxysmal arterial hypertension led us to suspect pheochromocytoma. The diagnosis was confirmed by elevated urinary catecholamine and by imaging (abdominal x-ray and abdominal/pelvic CT scan). Bilateral adrenal gland tumours were successfully removed. This report illustrates an uncommon presentation of pheochromocytoma in children and shows the importance of a careful investigation for pheochromocytoma as a cause of hypertension in children with cerebral ischaemic stroke

Lemierre syndrome: once seen it can never be mistaken

Lemierre syndrome is a rare but severe septicaemia due most frequently to Fusobacterium necrophorum, with primary foci in the head, internal jugular vein thrombosis and metastatic infections, mainly in the lungs. We report two cases of Lemierre syndrome admitted to the paediatric unit of our hospital at 2-month intervals. The first was 10 years old and the second one was a 3-month-old infant. They were diagnosed on the 25th hospital day and at the time of admission, respectively. In the first case, diagnosis was delayed due to unfamiliarity with this disease. In the second patient the diagnosis was established on the first day because of the recent experience with patient 1. In both cases, bacteriological investigations were not helpful. Both patients had a favourable outcome after prolonged antibiotic treatment, anticoagulation and surgical drainage