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.

Thrombosis of the abdominal aorta in newborn: About two cases.

BACKGROUND: Aortic thrombosis in neonates is a rare phenomenon, and in most cases iatrogenic. An early recognition of the clinical features and an immediate intervention can offer a better prognosis, and thus preventing morbidity such as limb amputation, and even mortality. METHODS: We present two cases of full-term newborns with a thrombosis of the abdominal aorta hospitalized in the neonatal intensive care unit of Mohammed VI University Hospital in Marrakech in 2017 and 2019. RESULTS: The two patients presented swelling and cyanotic lower limbs. Doppler ultrasound revealed a pathological lower limb blood flow as a result of a thrombosis of the abdominal aorta. Both patients underwent an anticoagulant therapy, the first one benefited from a surgical thrombectomy, he developed a reperfusion syndrome with alveolar hemorrhage, and passed away as a result of a cardiorespiratory arrest. However the second patient got amputated of the right foot, his clinical evolution was favorable with a good healing of the surgical wound without recurrence of any thrombosis. CONCLUSION: Through those cases clinicians should be aware of the first clinical signs of this condition in order to offer a rapid and successful management.

Biotinidase deficiency in a newborn.

INTRODUCTION: Biotinidase deficiency is an inherited disorder of biotin metabolism that is untreated may present within the first few month of life. OBJECTIVE: We report the exceptional observation of a biotinidase deficiency in Morocco. The rarity of this pathology, its age of onset, its mode of revelation and the lack of treatment in Morocco make the particularity of this observation. OBSERVATION: A newborn child born from a 24-year-old mother, followed by an estimated pregnancy of 37 weeks of amenorrhea according to the Farr score (morphological maturation score used for the dating of the pregnancy term). The infant presented at 7 days of life with a cutaneous-mucous eruption with icithiosic dry erythroderma of interest to the trunk, the face, the scalp associated with alopecia and depilation of the eyebrow. The biotinoidase deficiency was confirmed by its low serum concentration at 49 nka / l. The newborn died at 20 days of life before starting the specific treatment. CONCLUSION: Biotinidase deficiency is a rare condition requiring early screening and rapid management. The delay in diagnosis and the unavailability of treatment in Morocco can have fatal consequences.

Therapeutic Hypothermia in Asphyxiated Neonates: Experience from Neonatal Intensive Care Unit of University Hospital of Marrakech.

INTRODUCTION: Therapeutic hypothermia (TH) is now recommended for the treatment neonates with hypoxic-ischemic encephalopathy (HIE). This treatment protocol is applied in our department since June 2012. The aim of this study is to report the first experience with head cooling in asphyxiated neonates in Morocco. PATIENTS AND METHODS: Prospective study of newborns admitted for HIE from July 18, 2012, to May 15, 2014, in Neonatal Intensive Care Unit (NICU) of Mohamed VI University Hospital. The results were studied by comparing a newborn group who received hypothermia to a control group. RESULTS: Seventy-two cases of neonates with perinatal asphyxia were admitted in the unit. According to inclusion criteria thirty-eight cases were eligible for the study. Only 19 cases have received the hypothermia protocol for different reason; the arrival beyond six hours of life was the main cause accounting for 41%. Complications of asphyxia were comparable in both groups with greater pulmonary hypertension recorded in the control group. The long-term follow-up of protocol group was normal in almost half of cases. CONCLUSION: Our first experience with the controlled TH supports its beneficial effect in newborns with HIE. This treatment must be available in all the centers involved in the neonatal care in Morocco.

[Epidemiology of nosocomial bacterial infection in a neonatal intensive care unit in Morocco]. / Épidémiologie de l'infection nosocomiale bactérienne dans un service de réanimation néonatale marocain.

In neonatal intensive care units, the incidence of nosocomial infection is high. This study aimed to determine the epidemiology of a nosocomial bacterial infection in the neonatal intensive care unit of Mohamed VI university hospital. A total of 702 newborns were included in this study. Of the 702 neonates studied, 91 had developed a nosocomial infection. The incidence rate was 13% and incidence density was 21.2 per 1000 patient-days. The types of infection were: bloodstream infections (89%), pneumonia (6.6%), meningitis (3.3%), and urinary tract infections (1.1%). Nosocomial infection was particularly frequent in cases of low birth weight, prematurity, young age at admission, umbilical venous catheter, and mechanical ventilation. Multiresistant bacteria included enterobacteria producing betalactamase (76.9%), especially enterobacteria that were dominated by Klebsiella pneumoniae (39.7%). The mortality rate was 52.7% in nosocomial infections, 19 (20.87%) of whom had septic shock. The results of this study show that nosocomial infection is an intrahospital health problem that could be remedied by a prevention strategy.

[Neonatal infection with Stenotrophomonas maltophilia (2 case studies)]. / L'infection néonatale à Sténotrophomonas maltophilia à propos de 2 cas.

INTRODUCTION: Stenotrophomonas maltophilia (S.M.) is a Gram-negative bacillus, naturally resistant to cephalosporins and carbapenems, which can colonize different sites and may be responsible for serious infections for which treatment is a real challenge. We report 2 cases of nosocomial S.M. infection in 2 hospitalized newborns in the neonatal intensive care unit. CASE 1: A 3-day-old newborn presented with infection beginning in the respiratory system, treated with ciprofloxacin, and complicated by septicemia, resulting in death. CASE 2: An 11-day-old newborn presented with brain S.M. suppuration: empyema and multiple abscesses were treated successfully with the combination of amikacin and chloramphenicol with good progression in the short and medium terms. DISCUSSION: Some epidemiological studies report that S.M. is the second Gram-negative bacillus responsible for nosocomial infection after Klebsiella pneumoniae. While the respiratory location of the bacterium is typical, cerebral parenchyma damage is rare in the newborn. The treatment remains very delicate and difficult because of this bacterium's drug multiresistance. CONCLUSION: These 2 cases of infection caused by S.M. including respiratory tract infection with bacteremia and cerebral suppuration in newborns have broadened the spectrum of diseases caused by this organism and are a reminder of the emergence of this organism and its natural resistance to several antibiotics.