[Priapism in sickle cell anaemia in Senegal: prevalence, attitudes and knowledge]. / Priapisme chez le drépanocytaire sénégalais: prévalence, attitudes et connaissances.

We interviewed 108 sickle cell anaemia patients aged 5 years older on priapism, and 113 healthy subjects in a control group. They were recruited in the out-patient consultation of the two sickle cell anemia care units of the Fann teaching hospital in Dakar. Ten cases of priapism were identified, all in the group of sickle cell patients (the difference is significant, p = 0.004). Prevalence of priapism was 9.3%. Actuarial probability of having priapism was 8.3% by 10 years of age, and 38.9% +/- 5.7 by 20 years of age. Before the interview, only 10.2% of the sickle cell patients and 8% in the control group knew about priapism, and most of them were unaware of its association with sickle cell disease (75% of sickle cell anaemia patients and 80% of control subjects). This lack of information should be improved by an educational program.

[Pédiatric haemophilus influenzae b meningitis in Dakar]. / Méningite pédiatrique à haemophilus influenzae b à Dakar.

OBJECTIVE: This study had for aim to determine the etiology of Haemophilus b pediatric meningitis. DESIGN: A retrospective study of 216 biologically confirmed cases was carried out during 6 years (January 1995- December 2000) on children 0 to 15 years of age, hospitalized at the Albert Royer Children Hospital Center. RESULTS: Haemophilus influenzae b is the first cause of pediatric meningitis (19.7%) followed by Nesseria meningitidis (14.5%), and Streptococcus pneumoniae (13.6%). The Haemophilus influenzae b meningitis cases are distributed all year round with a peak between January and March, that is to say, during the dry and cool season. They affect children at an average age of 11.7 months, with a sex ratio of 1.1 for boys. Almost all of the patients live in the low-socio-economic areas of the Dakar suburbs (92.8%). More than 90% of the H. influenzae b isolates are sensitive to ceftriaxone (96%) chloramphenicol (93%), and to ampicillin (91%). Clinical evolution is marked by death (17.8%) and recovery with psychological, sensory, and motor sequels (19.9%). CONCLUSION: This report should help to include the combined vaccine Antihaemophilus influenzae b in the Senegalese Broad Vaccination Program. The final aim is the reduction of morbidity and mortality of infections due to Haemophilus influenzae b.

[Evaluation of gingival condition in homozygous children and adolescents with sickle cell anemia in Senegal]. / Evaluation de l'etat gingival chez l'enfant et l'adolescent drepanocytaires homozygotes au Senegal.

Sickle cell anaemia is an inherited disorder of haemoglobin synthesis leading to haemolytic anaemia. It touches more than 50 million people in the world and 10% of the population in Senegal. Only the homozygous form is symptomatic and appears by painful crises of vaso-occlusive type. There is a lack of works carried out or published in Senegal about potential link between periodontal conditions and sickle-cell anaemia. The main objective of this study is to assess the gingival conditions in Senegalese children and adolescents homozygous sickle cell anaemic. Secondary, the gingival state is correlated with oral hygiene and rate of reticulocytes to determine their relationship. Fifty homozygous attending the Albert Royer Paediatric Hospital of Dakar and unscathed of other systemic pathologies, were examined. The patients are old between 3 and 16 years with an average age 9.4 years +/- 3.8. The gingival state is assessed by measurement of the gingival index (GI) and index of sulculary bleeding (SBI). Partial correlation is performed between the two indexes and average of reticulocytes. The gingival index mean is 1.7 +/- 0.6 and 66% of the patients present a severe inflammation, which is more frequent in the age bracket from 3 to 12 years. The SBI mean is 1 +/- 1.1 and 68% of the patients present gingival bleeding, which is moderate to severe in 18%, with a greater frequency in the age bracket from 13 to 16 years. Strong correlation is found between plaque index, GI and SBI. Damage of gingival state seems not to be linked to the high average of reticulocytes controlling for oral hygiene. Children and adolescent homozygous have an inflammatory periodontium associated to poor oral hygiene, which highlights their needs in periodontal treatments. Early tracking and preventive treatment of periodontal diseases during systematic oral visits must be required.

[Epidemiological and clinical aspects of paediatric HIV infections in Albert-Royer Paediatric Hospital (Dakar, Senegal)]. / Aspects épidémiologiques et cliniques de l'infection à VIH de l'enfant au centre hospitalier national d'enfants Albert-Royer à Dakar.

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.

[Progressive spinal amyotrophy type I or Werdnig-Hoffman disease. Apropos of 5 cases in Dakar (Senegal)]. / L'amyotrophie spinale progressive de type I ou maladie de Werdnig-Hoffmann. A propos de 5 cas dakarois (Sénégal).

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.

[Transferrine soluble receptors' contribution to the assessment of iron status in homozygous drepanocytic anemia]. / Apport des récepteurs solubles de la transferrine dans l'évaluation du statut en fer au cours de la drépanocytose homozygote.

Sickle cell anemia does not cause martial deprivation per se, but may worsen when iron deficiency exists, notably in tropical zone where infectious diseases and malnutrition are endemic mainly during childhood. This study was aimed to assess iron deficiency prevalence among children with sickle cell disease (SCD) and to determine the best parameters for its diagnosis. In addition to classical parameters, we measured transferrine's soluble receptors which can reveal an iron deficiency, either isolated or associated to another condition since its level is not influenced by chronic anemia. Assays were carried out in 40 homozygous SCD patients, aged 3 to 18 years, having an hemoglobin level < 11 g/dL and in 30 age-paired controls assumed to be healthy and having a negative Emmel test and an hemoglobin level < 11 g/dL. The results showed hyposideremia (serum iron < 60 microg/dL) in 17.5% of the patients. Ferritinemia, transferrinemia as well as total iron fixation capacity were in the normal range for the majority of SCD patients in spite of the frequency of hyposideremia and microcytic anemia (20%). Transferrine's saturation coefficient was low in 22.5% of patients, which can be due to martial deprivation or to inflammatory status. These results confirm the limitations of usual biochemical parameters in the diagnosis of iron deficiency in homozygous drepanocytosis. Soluble receptors' levels were increased in 60% of controls; that proves that iron deficiency prevalence is high in our countries. Higher levels were found in 97.5% of patients. However, receptors' levels are increased during haemolysis, thus it is difficult to ascertain the origin of the increase, but taking into account its index value can reduces misinterpretation. In addition, considering simultaneously microcytosis, hypochromia, transferrine's soluble receptor level and its index, we can speculate that martial deficiency occurs in 20% of SCD patients, a percentage close to the 17.1% obtained by other authors using only the combination of microcytosis and hypochromia. It results from this study that associating microcytosis and hypochromia could validly assess iron deficiency during drepanocytosis.

[Prevalence of antiphospholipid antibodies in patients with sickle cell disease]. / Prévalence des anticorps antiphospholipides au cours de la drépanocytose homozygote.

Structural abnormalities in erythrocyte membrane are more and more claimed as a determinant factor in the sickle cell disease pathogenesis. This being would have been provided by a new anionic phospholipids distribution and conformation. Phosphatidyl-serine exposing and phosphatidic acid enhancing would induce specific immunoglobulins synthesis. In this study, assessment of antiphospholipid antibodies prevalence was carried out among sickle cell trait patients (n = 35) and homozygous patients (n = 59) as compared to healthy subjects (n = 39). Antiphospholipid antibodies, assayed by ELISA procedure, were significantly higher among the homozygous patients than the sickle cell trait patients ones and highlighted as compared to healthy subjects. Pathologic data were only observed among homozygous patients. These specific antibodies, associated with thrombosis and haemolysis, would have constitute a morbid link and a therapeutic target of this sickness, dominated by homodynamic troubles.

[Laparoscopic cholecystectomy in sickle cell disease]. / La cholécystectomie laparoscopique dans la drépanocytose.

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.

[Erythrocyte indices and iron stores in cord blood]. / Paramètres érythrocytaires et réserves en fer dans le sang du cordon.

BACKGROUND: Reevaluation of normal red cell values in the cord blood is necessary since these values were established before the use of automated analysers and the introduction of iron supplementation during pregnancy. POPULATION AND METHODS: Cord blood samples from all healthy babies from Monday to Friday for 2 months have been analysed for red cell count and red cell indices, hemoglobin concentration, hemoglobin electrophoresis, serum and erythrocyte ferritin concentration. The results were compared for the mothers to age, parity and duration of iron supplementation and for the neonates to gestational age and birth weight. RESULTS: One hundred and fifty-nine neonates were studied; 87% of their 154 mothers had been given iron during pregnancy. Mean red cell indices were found to be slightly below those of previous reports in the literature. They were associated with mean ferritin concentrations of 135 micrograms/l and 348 ag/cell for plasma and erythrocytic ferritin, respectively. CONCLUSION: There were no correlations between red cell indices and ferritin values in such an iron-repleted population.

[Sickle cell disease in children in Dakar, Senegal]. / Les syndromes drépanocytaires majeurs en pédiatrie à Dakar (Sénégal).

AIM OF THE STUDY: To determine the socioeconomic, clinical and biological aspects of sickle cell disease (SCD) in Senegalese children and adolescents, we retrospectively analysed all records of follow-up attending patients in the Albert Royer Children Hospital of Dakar (Senegal). RESULTS: Homozygous sickle cell (SS) was the most frequent genotype (307 cases). Sickle cell hemoglobin C (13 cases) and sickle cell beta-thalassemia (three cases) were uncommon. Patients were aged from five months to 22 years (mean age: eight years). Most of them came from poor families. The mean number of children was five in patients' families, with at least two cases of SCD in 60% of them. Immunization against hepatitis B virus (10.2%), Haemophilus influenzae b (8.4%), Salmonella (8.7%) and Streptococcus pneumoniae (21.4%) was insufficiently performed, because of its relatively high cost. Only 30% of the patients had received a blood transfusion. Painful crises occurred less than three times a year in 74% of the cases. Complications such as acute chest syndrome (1%), stroke (1%), cholelithiasis (9%), meningitis (0.4%), septicemia (2%) and osteomyelitis (6%) were rare. Mean steady state hemoglobin (Hb) and hemoglobin F(HbF) levels were 8.27 +/- 1.36 g/dL and 6.8 +/- 5.9% respectively among SS patients. No correlations were found neither between Hb and HbF nor between these parameters and the frequency of complications. Eleven patients (1.1% per year of follow-up) died, and infection was the main cause of death (73%). CONCLUSION: In comparison with published data, SCD seems to have mild severity in Senegalese children and adolescents in spite of poor follow-up conditions. In addition to genetic factors, environmental factors might have an important role in disease tolerance.

[Cholelithiasis and homozygote drepanocytosis in children in Dakar (Senegal)]. / Lithiase biliaire et drépanocytose homozygote en pédiatrie à Dakar (Sénégal).

BACKGROUND: Cholelithiasis is a well recognized complication of sickle-cell anemia (SCA) because of chronic hemolysis. It is usually asymptomatic but may result in acute cholecystitis or cholangitis. The aim of this study was to assess prevalence of cholelithiasis and its associated factors among Senegalese children and adolescents with SCA. PATIENTS AND METHODS: We analyzed the follow-up records and results of systematic clinical, biological and ultrasonographic examinations in 106 patients with SCA aged 11 months to 22 years (median: 10 years, 6 months), followed up in the Albert-Royer Children's Hospital (Dakar). RESULTS: Cholelithiasis was detected in 9.4% of the patients. Gender, ethnic group, geographic origin and socioeconomic conditions did not have any association with cholelithiasis frequency. The youngest patient with cholelithiasis was 7 years old, and prevalence of cholelithiasis increased with age. The mean age at the beginning of SCA follow-up was higher among patients with cholelithiasis. They were more frequently transfused and tended to present more frequent pain crises and other complications of SCA. We found no spontaneous clinical manifestations attributed to cholelithiasis. Nevertheless, provoked right upper quadrant pain was more frequently observed in patients with cholelithiasis. Fetal hemoglobin, steady-state hemoglobin, reticulocytes count, serum bilirubin, alkaline phosphatase and transaminase levels were not significantly different in these patients, compared to the others. However, those with cholelithiasis had significantly higher mean red cell volume. CONCLUSION: Prevalence of cholelithiasis appears relatively low in Senegalese patients with SCA, probably owing to the tolerance of the Senegal haplotype. Advanced age and severe chronic hemolysis are etiologic factors in evidence. We recommend systematic abdominal ultrasound at least once a year for patients older than 5 years, or when right upper quadrant pain is observed. Cholecystectomy should be performed in cases of cholelithiasis in order to prevent complications.

[Epidemiology and prognosis of childhood injuries in Dakar, Senegal]. / Epidémiologie et pronostic des accidents de l'enfant à Dakar, Sénégal.

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.

[Management of children with sickle cell disease in Africa: experience in a cohort of children at the Royal Albert Hospital in Dakar]. / Prise en charge de la drépanocytose chez l'enfant en Afrique: expérience de la cohorte de l'Hôpital d'enfants Albert Royer de Dakar.

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.

[Maternal risk factors and low birth weight in Senegalese teenagers: the example of a hospital centre in Dakar]. / Facteurs de resques maternels et petit poids du nourveau-né chez des adolescentes sénégalaises: l'exempl d'un centre hospitalier de Dakar.

AIMS OF THE STUDY: a) to identify the risk factors associated with newborn babies' low birth weight in teenage mothers; b) to propose prevention strategies for lower-ing neonatal morbidity and mortality. SPHERE OF THE STUDY: the study was carried out at the maternity and neonatology service of the Abass Ndao hospital centre, a urban community hospital located in the Southern part of Dakar. MATIERIAL AND METHODS: this retrospective study was carried out between July 1, 1998 and June 31, 1999. All new mothers under 20 who had given birth to living newborn babies have been included in the study and categorised into two groups, according to their babies' birth weight: 1) a first group of teenagers whose newborn babies' birth weight was lower than 2,500 g (low birth weight); 2) a second group constituted of women whose newborn babies' birth weight was higher than 2,500 g, and which was used as a control. The socio-demographic, biometrics, maternal and obstetric factors have been analysed and compared. RESULTS: out of the 4,586 women in childbirth during the study period, 456 were under 20 years of age, which corresponds to a prevalence rate of 10%. One hundred and five women had newborns weighing less than 2,500 g, which corresponds to a prevalence rate of 23%. Certain factors were found to be significantly associated with low birth weight: low weight gain during pregnancy (p = 0.04), fewer antenatal consultations (0.006), and kidney-related syndromes during pregnancy (0.0005). CONCLUSION: The results of that study allow us to recommend the following strategies: - control and improvement of nutritional behaviour during pregnancy; campaigning for a better attendance at antenatal consultation services for the early detection of pathologies during pregnancy and for preventing kidney-related syndromes.

[A home nutritional rehabilitation trial for severly malnourished children]. / Essai de réhabilitation nutritionnelle à domicile d'enfants sévèrement malnutris.

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: a complication of acute gastroenteritis in children]. / Le syndrome hémolytique et urémique: une complication des gastro-entérites aiguës de l'enfant.

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.

[Congenital rubella: a case report]. / Rubéole congénitale : à propos d'un cas.

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.

[Acute encephalic manifestations in Senegalese children with sickle cell disease]. / Manifestations encéphaliques aiguës chez l'enfant drépanocytaire sénégalais.

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.

[Defects in peroxidation of erythrocyte phospholipids in sickle cell trait]. / Lésions péroxydatives des phospholipides érythrocytaires au cours de la drépanocytose AS.

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 in children. Diagnosis of 9 cases]. / Thrombocytose majeure associée à une anémie sévére chez l'enfant. Disussion diagnostique à propos de 9 cas.

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.