[Pyoderma gangrenosum in a child with sickle cell disease].
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Biomedical subjects
Publications and source records attributed to I Diagne.
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UNLABELLED: Human Immunodeficiency Virus (HIV) infection prevalence rate is estimated at 1.4% in Senegal, and about 3,000 children could be infected. HIV positive children are followed up since 2000 in Albert Royer Hospital (Dakar, Senegal). OBJECTIVES: To describe clinical and epidemiological aspects of HIV paediatric infection, and to evaluate the implementation of high active antiretroviral therapy in HIV positive children in our country. POPULATION AND METHODS: Over a period of three years, the medical reports of 98 infected patients have been collected, 96% with HIV 1 infection. RESULTS: Most of the patients had a maternally transmitted HIV infection (99%). At their enrollment, the median age was 60 months; malnutrition (79%), persistent lymphadenopathy (65%) and skin lesions (64%) were the common clinical manifestations. Thirty-nine percent of the patients were in class C (CDC) and 81% had CD4 cell count< or =25%. Median viral load were 421,852 copies/ml at presentation. Seven infants had a rapid progressive disease with encephalopathy. Thirty-six patients received high active antiretroviral therapy with high observance and good tolerance. CONCLUSION: This study allowed to define clinical and biological profile of paediatric HIV infection in our country and to update the implementation of high active antiretroviral therapy.
STUDY AIM: Sickle cell affection is a public health problem in Africa. The aims of this prospective study were to evaluate the early results of laparoscopic cholecystectomy in sickle cell patients in Senegal. METHOD: From January 1998 to June 2002 all the sickle cell patients undergoing a laparoscopic cholecystectomy were included. Intra- and post-operative protocol (blood transfusion if Hb < 9 g/dl, rehydration, oxygenotherapy) was standardized. RESULTS: Forty-two patients with sickle cell of types SS-33 and AS9 were operated upon by same surgeon. One case of conversion due to an effraction of biliary junction was reported. One homozygote patient died post-operatively because of peritonitis. Two acute thoracic syndromes, three vaso-occlusive crisis, and two cases of wound infection constituted the post-operative morbidity. No case of complication was noted in those who underwent pre-operative transfusion. CONCLUSION: Laparoscopic cholecystectomy can be carried out in sickle cell patients with biliary lithiasis provided that general anaesthetic rules are respected.
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Management of major sickle hemoglobinopathies in industrialized countries has improved significantly over the last few years thanks to strict application of the preventive and curative measures developed as a result of a better understanding of the underlying pathophysiological mechanisms. However patients in Africa have benefited little from progress in the field due to the lack of human and physical resources in sharp contrast with the high prevalence of the disease. The purpose of this study was to analyze problems involved in management of childhood sickle cell disease in Africa based on our experience in a cohort of 556 cases treated over a period of 12 years. The main problems were the same as those encountered in other black African nations, i.e., delayed diagnosis due to a lack of routine neonatal diagnostic screening, difficulty implementing anti-infectious prophylaxis due not only to the high cost of recommended vaccinations not covered by the Expanded Program on Immunization but also to poor compliance with antiobioprophylaxis, and insufficient transfusion facilities hindering application of long-term transfusion protocols when indicated. In addition the high prevalence of digestive-tract parasitosis and malaria raise the need to combine standard preventive measures with routine parasiticidal treatment and malarial prophylaxis adapted to each geographical area. The high frequency of associated iron deficiency requires systematic laboratory testing to identify and treat resulting manifestations during follow-up. An important prerequisite for widespread implementation of appropriate preventive and curative measures in Africa is recognition of sickle cell disease as a priority in public health care policy.
The impact of accidental injury on childhood morbidity and mortality in Africa is underestimated. The frequency and severity of accidents is high. This retrospective study includes 381 children (3% of admission) between the ages of 0 and 15 years hospitalized for accidental injury at the Principal Hospital in Dakar, Senegal, between January 1, 1997 and December 31, 2000. The annual incidence of childhood injury doubled over the 4-year study period. Mean age was 48 months and the M/F sex ratio was 1.7. The causes were trauma (n = 184) due to domestic accidents (n = 109 including 91 falls) or road accidents (n = 75), poisoning (n = 129 including 38 caustic soda burns), foreign body aspiration or ingestion (n = 30), burns (n = 18), and miscellaneous accidents (n = 20). Most fatalities (9%) were due to road accidents and falls. Rapid uncontrolled urbanization and problems of displaced rural populations in adapting to city living may account for the high frequency of falls from high buildings and road accidents. As in industrialized countries road accidents, though not the most frequent, are the most lethal. Lack of emergency services accounts for severity. Vigorous information campaigns and prevention action will be needed to lower the high morbidity and mortality of childhood injury in developing countries.
The aim of this study is to assess mortality rate of low birth weight newborns admitted to a neonatal care unit of Dakar after transfer. This retrospective study include all newborns weighing less than 2500 g transferred to Abass Ndao neonatal care unit between January 1st,1998 and December 31, 1999. Maternal, newborns and transfer related parameters were studied. Data of 180 new-borns were recorded from a sample of 247 babies transferred. The mean weight of these babies were 1452.5 +/- 432 g. An ambulance was used for transport in only 10% of cases. The median time of admission after birth was 3 hours. Median delay of admission and methods of transfer, maternal age and parity, apgar score at 1st and 5th minute were comparable between the newborns deceased and survivors (p > 0.05). To reduce mortality associated with newborns transfer, we insist on a better organisation of neonatal transport in under developed countries by promoting obstetricians and paediatricians collaboration and prevention of low birth weight.
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Type I spinal muscular atrophy or Werdnig-Hoffman disease is rarely described in black populations. We report five such cases diagnosed in a paediatric outpatient clinic in Dakar. We conducted a retrospective study relating to patients examined for hypotonia progressing since birth for whom the electromyogram had made it possible to confirm an involvement of the peripheral nerve without nerve conduction anomaly. Mean age of diagnosis was 12.3 +/- 7.6 months. Respiratory distress was noted for 2 patients. A family background of similar symptomatology was found in 1 case and consanguinity in 2 cases. Only 1 case of death occurred whereas the 4 other patients were lost to follow-up. The diagnosis of spinal muscular atrophy must be considered in the presence of any severe hypotonia in infants.
Malignant hemopathies are not considered as public health priority in Senegal because of their infrequency in comparison with infections and malnutrition. However they remain usually lethal instead of a great improvement of their prognosis in suitable therapeutic conditions. The objective of this study was to determine the epidemiologic and evolutionnal profile of these pathologies, and identify practical management problems in a reference public pediatric service in Senegal. We retrospectively analysed hospitals registers and records of all patients followed up in Albert Royer Children Hospital of Dakar from january 1989 to december 1998. During this ten years period 25 cases of malignant hemopathies were diagnosed among 32,789 hospitalised children, representing an hospital prevalence of 0.08 per cent. Mean age at the desease diagnosis was 9.5 years and sex ratio 2.57 (18 boys and 7 girls). The malignant type was acute leukemia (AL) in 11 cases (44%) including 9 cases of of acute lymphoblastic leukemia (ALL) and 2 cases of acute myeloblastic leukemia (AML); chronic myeloid leukemia (CML) in 2 cases (8%), Hodgkin's desease (HD) in 9 cases (36%) and non hodgkinian lymphoma (NHL) in 3 cases. NHLwere Burkitt type in 2casesand lymphoblastic type in 1 case. Their was no maxillary or facial localisation in Burkitt type lymphoma. The mean duration between the first clinical symptomes and the diagnosis of the disease was 4 months and delayed diagnosis was mainly due to delayed transfer from peripheral health services to hospital. Among 19 patients whose records were available, 17 were subjected to chemotherapy. However reference protocols were completely applyed in only 2 cases, one with HD and an other with lymphoblastic lymphoma. Transfusion managementwas not sufficient because of the lack of blood derived products (packed platelets or leucocytes) when needed. Thirteen patients died while followed up and mean survival after first hospitalisation in these cases was 120 days in ALL, 38 days in AML, 2.5 years in HD and 18 months in NHL The other patients were lost of sight and presumed to be dead at home. Eventually, this study showed that, in our hospital, children with malignant hemopathies did not derive benefit of therapeutic progress enregistered long time ago in developed countries, since they remain constantly lethal. The main factors of lethality could be delayed transfer to hospital because of lack of knowledge about these pathologies in the peripheral health services and poor therapeutic conditions in reference hospitals. Creation of specialised clinical haematology department could enable us to improve the prognosis of these affections by an optimal use of available human and material ressources.
The sickle cell disease pathophysiology is provided by several events including deoxyhemoglobin S polymerization and hemorrheological troubles. Classically, such disorders have been known to concern only the homozygous SS and heterozygous SC profiles. However, troubles are more and more described since early years in the sickle cell trait patients and the pathogenesis factors are unknown. Our study focussed on phospholipids which are the basic and functional constituent elements of erythrocyte plasmic membrane. Assaying the membrane lipids extracted by Folch method and lipid standards (cholesterol, phosphatidic acid, lysophosphatidyl-choline, phosphatidyl-choline, lysophosphatidyl-ethanolamine, phosphatidyl-ethanolamine, sphingomyelin) was carried out by HPLC among 91 sickle cell trait patients as compared to 78 healthy subjects. The main abnormalities observed were an empoverishement in phosphatidyl-choline contrasting with an accumulation of membrane lysophosphatidyl-choline, lysophosphatidyl-ethanolamine, phosphatidic acid and sphingomyelin in sickle cell patients. These results suggest that sickle cell trait harmlessness is not actual. The accumulation of lysophosphatidyl-choline, by its cytolytic biomembrane perturbing properties, predispose therefore the sickled red cell to the haemolysis phenomena and allow to forecast deeper disturbances among homozygous subjects. These morbid disorders attributable to lipid peroxidation would justify an antioxidant treatment during the sickle cell disease.
Major thrombocytosis associated with severe anemia is uncommon in pediatrics. We report 9 consecutive cases observed in Albert Royer Children Hospital of Dakar. They were 7 boys and 2 girls 4 to 15 years old (mean age = 10 years). Six patients had a history of geophagia and 3 presented recent emission of worms by the stools. Anemia was clinically well tolerated in all cases. In initial blood count platelet levels varied from 800 10(3) to 1180 10(3)/mm3 (mean = 1032 10(3)/mm3), while hemoglobin level varied from 3.4 to 7.4 g/dl (mean = 4.9 g/dl). Anemia was microcytic, hypochromic and associated with low serum iron level in all patients. We considered the diagnosis of reactive thrombocytosis induced by iron deficient anemia in all cases. Platelet count and red cell indices were progressively normalised with iron treatment and no complication of thrombocytosis was observed. Considering published data, iron deficiency is one of the most frequent causes of reactive thrombocytosis in children. The physiopathologic mechanism, still unknown, could involve cytokines of thrombopoiesis. Reactive thrombocytosis induced by iron deficiency or other factors have usually a benign course and need no specific treatment other than that of the etiology.
This report is a retrospective study carried out from records of diseased of asthma, fallowed up in a paediatric national center of reference during the period from 1st of November 1987 to 31 st of august 1993. The goal was to describe the epidemiological and clinical factors linked to that affection in order to better contribute to improve its managment. For all the diseaseds of this report we notice and analysed the socio economical data, the promoting factors the occurring mode of the disease, the main clinical manifestations of the affection and their consequences. The morbid association and the data of the paraclinical exploration particularly the functional breathing exploration. Thus we noticed that the consultation in a specialized center occurs relatively late because of bad orientation of the deseaseds. In effect the mean age of diseaseds at the first consultation is 7.5 years while the disease begins in average at 3.5 years. By elsewhere we notice that 15% of these diseaseds live in an aggressive mesologic medium. With an atopy in 78.4% of the cases, loaded personal case history in 96.6% of the cases and that the releasing factors are not known for most of them (81.9%). Asthma in our diseaseds is not very frequent (77%) in general with a moderate intensity of crises (69%). However disturbances of physical activity are noted among 23% of the sample which corresponds nearly to the number of subjects with severe respiratory obstruction (25%). We quoted that there is an relation ship between the frequency of the cases the intensity of the crisis and the disturbances of physical activities of the diseaseds (x2:25.9, df = 2, p= 0,000002). Tacking count of all these factors may serve to set up a asthma prevention and treatment project in Senegal based on populations education and care providers training.
A nutritional rehabilitation trial for severely malnourished children aged 6 to 36 months (weight/height index using Z-score of less than -3 as the reference mean) was conducted in the poor suburban areas of Dakar, Senegal. Its purpose was to determine the effectiveness of a rehabilitation programme delivered in the home as compared to one provided in a nutritional recovery center (CREN). Ninety-seven children benefited from rehabilitation at home and 51 at the CREN. At the end of two months of rehabilitation and after five months of charting their weight, the average weight gain was better in the group followed at home, but it still remained well under the 50 g/day recommended by WHO. Within the two groups, all of the children stayed underweight, with an average weight/height index Z-score of less than -2.5 in the group followed at home, and a Z-score of less than -2.9 in the other group. The dropouts and the death rates in the two groups were comparable. The presence and availability of the mother as well as health education sessions adapted to each specific case through interpersonal communication seem to be the elements favouring the most effective rehabilitation being in the home.
Hemolytic uremic syndrome (HUS) is an nonexceptional complication of infectious gastroenteritis. No one has already been reported in Senegalese publications. We made a retrospective study of the record of 7 patients with HUS among 256 cases of children with bloody diarrhea presenting to the pediatric unit of Aristide Le Dantec between august 1998 and july 1999. The mean age of the children was 33,14+/-25 months and the weight was -2,29 DS. The diagnosis is supported by the findings of an acute renal failure with urea at 1,28+/-0,51g/ l and creatinine at 41,46+/-25,48mg/l. An hemolytic anemia was constant, the blood film revealed schizocytes. We found a thrombocytemia only in two cases. A hight white blood cell count (more than 50000/mm3) was noted in for cases. Only one child made a good recovery. We insist on preventing gastroenteritis and aggressive and adapted management of the HUS.
Rubella is an innocuous illness in adult unless it is contracted by a pregnant woman. We recently observed a case of congenital rubella in the department of neonatology of Abass Ndao hospital in Dakar. It's a female new-born, born after 40 week gestation, with a well followed pregnancy. The first clinical examination shows a severe intrauterine growth retardation and bilateral comeal opacity. Maternal and new-born serologic confirmation is obtained with positive rubella specific IgM and IgG. We also find severe ocular pathology, hearing deficit and associated cardiac disease. This case shows the needs for preventive strategies, such as vaccination against rubella for women of child bearing age.
The course of sickle cell disease (SCD) may be complicated by neurologic events, mainly bactérial meningitidis and stroke. We retrospectively studied all cases with acute encephalic manifestations (AEM) in a cohort of 461 children and adolescents with SCD followed at Albert Royer Children Hospital of Dakar (Senegal) from january 1991 to december 2000 (ten years). Among them 438 had sickle cell anemia (SCA), 19 SC disease and 4 S-beta thalassemia (3 S-beta+, 1 S-beta0). Seven patients, all with SCA, presented antecedents of AEM revealed by flacid and proportionnal hemiplegia evoking stroke. Prevalence of these AEM was 1.5 per cent among patients with SCD and 1.6 per cent among those with SCA. They were 4 girls and 3 boys (sex ratio = 0.75) aged 4 to 8.5 years when occurred the first accident. We observed no clinical or biological distinctive characteristic of SCA in these patients compared to those without crebrovascular accident. Recurrence was observed once in a boy after a 12 months interval and twice in a girl after 20 and 60 months intervals successively. No transfusionnal program was applied to prevent recurrent stroke because of insufficient conditions for long-term transfusion. Stroke appears to be rare in senegalese children with SCD. However it poses in our context the major problem of applicability of transfusionnal program which constitute the only therapy universally recognised to be effective to prevent recurrence. Nevertheless hydroxyurea could be a satisfactory alternative.