Purpose: Improve the management of Major Sickle Cell Syndromes (SDM) by studying the circumstances in which patients affected by this disease die in Niger. Methods: This was a retrospective study of descriptive and analytical type in sickle cell patients followed at the National Sickle Cell Reference Center (CNRD) in Niamey with a follow-up file, who died during our study period, which was 9 years (January 1, 2010 to December 31, 2018). Results: During the study period, 6,465 sickle cell patients were followed up at the CNRD, 249 deaths were recorded, representing a frequency of 3.8%. There is a male predominance with a sex ratio of 1:3. The 0-15 age group was the most represented with 34.5% with an average age of 9.25 years. 34.1% of the patients came from a consanguineous marriage. Of the 249 deaths, 94% (n=234) were SS, 5.6% (n=14) were SC and 0.4% (n=1) was S/β. The majority of patients died at the time of registration, i.e. 58.25% (n=145 (SS=137. SC=8.)). 90.4% had died in a health-care facility. The main cause of death was anemia in 73.1% of cases. Conclusion: The management of sickle cell disease requires adequate preventive action to reduce the rate of early mortality.

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Polycystic liver disease is an autosomal dominant disorder, rare and benign in the majority of cases [1]. It is frequently associated with polycystic kidney disease, but can also be isolated in rare cases. Liver cysts result from abnormal growth of the biliary epithelium (cholangiocytes) or dilation of the peribiliary glands, due to the persistence of embryonic biliary structures. Isolated polycystic liver disease is generally asymptomatic, women are more affected than men and also have a greater number of cysts [2]. We report the case of a 40-year-old asymptomatic man with isolated polycystic liver disease Gigot III discovered during a health check-up. The clinical examination showed hepatomegaly without pain, of firm consistency, smooth surface, sharp lower edge with a hepatic arrow at 17cm. The kidneys are devoid of cysts, the liver function as well as the renal function are normal.

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