Gaucher's disease (GD) is a lysosomal storage disorder due to glucocerebrosidase deficiency; it's one of the rare genetic diseases for which therapy is now available. The purpose of this work is to study the epidemiological features of the disease and to highlight the diagnostic difficulties. We performed an 11-year retrospective study of 11 patients with GD followed-up in the department of paediatric hepatology gastroenterology and nutrition of Rabat children's Hospital. We observed 11 patients with GD: 6 males and 5 females. Age at onset ranged from 3 months to 10 years with an average of 3.41 years. Mean age at diagnosis was 4 years (range 3months-14years). Parental consanguinity was noted in 85% cases. According to the clinical presentation, we classified our patients into: 9 cases of type 1 (81%) and two cases of type 2 (19%), none of the patients presented GD type 3. GD type 1: The age at diagnosis ranged from 2 years to 14 year with an average of 6 years. Main symptoms were: splenomegaly, hepatomegaly, pallor, haemorrhagic appearance (40%), bone pain (40%). The diagnosis was based on histology showing the Gaucher's cells in various tissues (100%). Enzymatic activity dosage confirmed the diagnosis of GD for 4 patients (44.5%). The treatment was always symptomatic (analgesics, transfusion). A splenectomy was performed in one case presenting with multiple splenic abscesses and high transfusion requirements. None of the patients received a specific treatment (substitutive enzymotherapy). The follow-up period ranged from 3 months to 6 years with an average follow-up of 4 years. We noticed stability in 4 cases, 2 worsening cases with bone and spleen complications. Three patients were lost to follow-up. GD type 2: we observed two cases of GD type 2 diagnosed at 3 and 18 months. The visceral symptoms were serious and the neurological features included seizures, hypertony, squint, physical developmental milestones delay. Both of them died. Gaucher's disease is not exceptional in Morocco. Type 1 is the most common type. We noted through this study some diagnostic difficulties as the diagnosis was delayed and the enzymatic dosage was performed in only 42% of the cases as well as therapeutic difficulty with no prescription of the specific treatment given the high cost of the enzyme.
Introduction COVID-19 is an infectious disease caused by a new coronavirus. The first cases were identified in Wuhan. It rapidly spread causing a pandemic worldwide. The incidence and severity of this disease are likely to be different in children compared with adults. Few publications of COVID-19 in children have been published. Our Moroccan paediatric series is among the first studies on this disease in Africa. Methods We included all children with COVID-19 who were admitted and treated at the hospital from March 25 to April 26, 2020. We have collected information, including demographic data, symptoms, imaging data, laboratory results, treatments and clinical progress from patients with COVID-19. Results Since the outbreak of 2019 novel coronavirus infection (2019-nCoV) in Morocco, a total of 145 COVID-19 confirmed cases have been reported in the Cheikh Khalifa’s Hospital. Among this cases, 15 children were registered. The median age of patients was 13 years. There were 7 boys and 8 girls. Five children are asymptomatic, 8 have mild symptoms and 2 have a moderate respiratory difficulty. The RT-PCR test results were positive in all patients. Radiologically, we found in 2 cases, multiple nodules with ground-glass opacities on the chest scan. The treatment was based on the combination of hydroxychloroquine and azithromycin. Evolution under treatment was good for all patients. Conclusion This study describes the profile of COVID-19 in child in a Moroccan hospital and confirms that the severity of illness in children with COVID-19 to be far less than adults.
Background and Aims. There is growing evidence that the 2019 coronavirus disease (COVID-19) is emerging as a potential trigger virus for the development of diabetes mellitus in children. This can occur even in patients without factors predisposing to impaired glucose metabolism. Here, we report two rare cases of diabetic ketoacidosis revealing new-onset diabetes and precipitated by COVID-19. These cases are reported in view of their rarity and originality. The relationship between type 1 diabetes mellitus and COVID-19 is discussed. Results. Two children developed symptoms suggestive of diabetic ketoacidosis preceded by polyuria, polydipsia, and asbestos. There is a documented COVID-19 infection in the parents of the 2 children. An asymptomatic infection was detected in the 2 patients on the basis of a reverse transcription polymerase chain reaction (RT-PCR) test. Thoracic imaging and inflammatory workup were negative in both cases. Both patients responded well to treatment, including rehydration regimens and intravenous insulin. On the 2nd day of their hospitalization, they were transferred to several injections of subcutaneous insulin with therapeutic and nutritional education from the parents. After about 4 weeks, their insulin requirements probably decreased due to the diabetes honeymoon. Conclusion. COVID-19 can induce acute onset diabetes and diabetic ketoacidosis in children. More research data are needed to improve our knowledge of this constellation and to guide the most appropriate therapies.
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