Aplasia cutis congenita associated with Goltz syndrome in a male neonate.

Here we report the association of giant aplasia cutis congenita in a newborn black male with Goltz syndrome. The cutis aplasia defect is extensive and circonscript at the vertex. The cerebral structures are visible through the lesions. In addition, the patient has a low birth weight, syndactyly, adactyly, cutaneous atrophy, and areas of hyperpigmentation on the legs and hypoplastic maxillary. We think that these signs are probably due to mosaic mutations in PORCN. We reviewed 18 cases of Goltz syndrome in 18 male neonates but none has aplasia cutis congenita. Such a combination of severe aplasia cutis congenita was not reported previously in Goltz syndrome.

[Fate of children born to HIV positive mothers followed in the context of preventing mother-to-child transmission of HIV in Togo. Study of 1042 infants]. / Devenir des enfants nés de mères séropositives au VIH suivis dans le cadre de la prévention de la transmission de la mère à l'enfant au Togo. Etude portant sur 1 042 nourrissons.

The purpose of this study is to analyze the fate of children born to HIV-positive mothers and to determine the impact of feeding options on the HIV status of the children in preventing mother-to-child transmission sites (PMTCT) in Togo. It was a retrospective and descriptive study, focused on medical files of 1042 children of both sexes aged from 0 to 59 months born to HIV-positive mothers and registered in the PMTCT sites between January 2004 and December 2008. Our study was conducted in seven PMTCT sites located in four of the six health regions in the country. We analyzed a total of 1042 medical files in seven PMTCT sites selected. The majority of children were born vaginally (77.4%). Antiretroviral prophylaxis most used in the mothers and/or the children was nevirapine in 86% of the cases. Breastfeeding (49%) was dominated by milk substitutes (50%). Globally, dropout rate was 52.2% (525), with 44.9% (408) at six months and 65.09% (483) at 12 months. At 12 months, 196 of 742 children (26.4%) had their retroviral serology. Among them, we have 5.6% overall rate of HIV infection. The transmission rate in the group of children breastfed was 5.9% (six children infected out of 102) and 8.5% in the group of children fed by formula milk (seven children out of 82). Death occurred in half of the cases (8 of 16) during the first two months of life. Follow-up was the major issue in monitoring children born to HIV-infected mothers. The HIV transmission rate is very high, irrespective of the feeding method.

[Knowledge of sickle cell disease and prevention methods in an urban district of Lomé, Togo]. / Connaissances de la drépanocytose et pratiques de prévention dans la population d'un district urbain de Lomé, Togo.

Despite the important frequency of the gene "S" in Togo, essential information remains insufficient to elaborate a prevention campaign on this affection. In order to assess the knowledge on sickle cell diseases as well as the prevention practices in the Togo population in one of the five districts of the township of Lomé, a cross sectional study was conducted in the third district of the township of Lomé from January 21, 2004 to January 26, 2004 in 210 natives from Togo aged of 15 and over, through a semi-structured questionnaire. The variables studied were: - the socio-demographic features and the knowledge of sickle cell disease characteristics (symptoms, biological diagnosis, treatment and means). Data collected were analysed through software Statically Package for Social Science (SPSS) version 10.0 of Windows using the chi2 test with 5% significance in the comparison of some variables. 117 women and 93 men were interviewed. Sickle cell disease was known in almost all ethnic groups but incompletely: 79.5% of the individuals knew about premarital check up but only 12.4% knew about haemoglobin electrophoresis check up. 74,8% of the people had a good knowledge of the cause of sickle cell disease, 78.6% had a fairly good knowledge of its symptoms, 57.6% knew the factors inducing attacks, 64.3% the prognosis and 69.5% the prevention methods, but a poor knowledge of the complications (62.4%), biological diagnosis (71%) and treatment (97.2%). The prevention practices were poorly adopted: 12% had an haemoglobin electrophoresis check up and 15% of them had their husband to have one as well. Professional status influenced the level of knowledge of the biological diagnosis (p=0.001) and prevention means (p=0.018). The educational level influenced biological diagnosis knowledge (p = 0.000) and prevention means (p = 0.02). On the whole, sickle disease was linked to marital status (p = 0.00). Sickle cell disease remains quite unknown in spite of the gene "S" important frequency in Togo. These results are to be taken into account to implement information, education and communication program to struggle against sickle cell disease.

[Treatment of children born of AIDS mothers in Tsevie hospital regional center, Togo]. / Prise en charge des enfants nés de mères infectées par le VIH au centre hospitalier régional de Tsévié, Togo.

The aim of this survey was to describe the components of the children medical care follow-up in the protocol of prevention of HIV/Aids from mother to child by nevirapine intake. A four-year retrospective study was carried out in Tsevie hospital regional center 90 children and their pregnant mothers who received nevirapine were recorded. 75 children received breast feeding. There was no follow-up for 42% of the children. The weight growth was correct in 90% of the children effectively followed. 49% of the children were completely vaccinated to PEV. The average children medical check up was 3.1 (minimum 1 maximum 8). The average age for breast feeding weaning was 6.2 months. The mother to child transmission rate was globally estimated at 12.5% at 18 months. 12 children (13%) died before HIV serology. The survey confirms the potency of nevirapine in preventing HIV transmission from mother to child and lays emphasis on real problems for which appropriate solutions should be found.

[Primary results of antiretroviral treatment among HIV/AIDS infected children in Lomé (Togo)]. / Traitement antirétroviral des enfants infectés par le VIH/sida à Lomé (Togo): premiers résultats.

UNLABELLED: Since 2004 in Togo HIV/AIDS infected children have, free of charge, access to antiretroviral drugs according to the national program. The aim of this study was to investigate the clinical, biological and prognosis aspects of anti-retroviral treatment on HIV/AIDS infected children. PATIENTS AND METHOD: We conducted a cross sectional study on 72 HIV/AIDS infected children with anti-retroviral treatment, under the supervision of clinicians within 3 associations specialized in the management of subjects infected by HIV/SIDA at Lomé (Togo). RESULTS: The average age of children was 6 years 9 months. The middle age to HIV screening was 4 years 2 months. The sex ratio was 1.05. The majority of these children (79.2%) were orphans of at least 1 of their parents. All the children were stemmed from families with modest income. The transmission mother to child was the way of HIV contamination found among all the children. To a certain extent, all the children were infected by the HIV 1. Most of the children (66.7%) receiving an antiretroviral treatment for at least 6 months were asymptomatic and had no more immunodeficiency. After 15 months, the children have gained 464 CD4/mm(3). The initial protocols antiretroviral prescribed among children were: zidovudine-lamivudine-abacavir (36.1%), lamivudine-didanosine-nevirapine (30.5%), lamivudine-stavudine-nevirapine (29.2%), zidovudine-lamivudine-didanosine (4.2%). The digestive disorders have been the first side effects (83.4%). The rate of good observance was middle (51%) and lowered with the increased age of children, and the period of the anti-retroviral treatment. CONCLUSIONS: Antiretroviral treatment among HIV/AIDS infected children is giving good results in Togo. But many efforts remain to increase the number of beneficiaries.

[Evolution of severe pediatric malaria in Togo between 2000 and 2002]. / Evolution du paludisme grave de l'enfant au Togo de 2000 à 2002.

The aim of this study was to analyse the clinical and evolutive aspects of severe malaria in hospitalised children in 2000, 2001, and 2002 in Togo. The study included 361 children in the pediatrics department of Lomé-Tokoin University hospital. All them received a 10% dextrose infusion, then an infusion of quinine or intramuscular artemether. Malaria accounted for 4.37% of all hospitalizations. Children aged 1 to 5 years were more affected (69.53%). The most frequent clinical forms were anaemia (55.7%) followed by cerebral manifestations. The frequency of hemoglobinuria increased (17.2%) as well as renal failure (3%) compared to previous years. Thirty-five children died (9.7%). Most of them presented with anaemia, neurological manifestations, or respiratory distress. Neurological sequels were present in 2.2% of patients.

[An epidemiological study of health care seeking behavior by children under the age of 5 years at hospital emergency services in Togo]. / Etude épidémiologique du recours aux consultations hospitalières d'urgence chez les enfants de moins de 5 ans au Togo.

The functioning and efficacy of pediatric emergency services are currently being questioned in many Northern countries, as well as in the South, for example in North Africa. Reference is often made to unjustified medical emergencies in the face of an influx of patients with benign problems who come from socially disadvantaged families. In the university and regional hospitals in three regions in Togo, we compared three categories of under-5 patients: children sent to "day-time" emergency services after triage done by health personnel; those sent to the "ordinary consultation"; and children brought after hours by their family (without referral by a health professional in 92% of cases) and seen in the "after-hours" emergency service. Serious tropical pathology (cerebral malaria, malaria, sickle cell disease) is mainly seen in emergency consultations, in which high hospitalization rates are noted (83% during the day, 67% at night) and a lethality of 3.4%. One cannot therefore use terms such as "false emergencies", "felt" and "medically unjustified" and the pediatric supervisor for the research considered that recourse to after-hours emergency services was justified in 75% of cases. Families using night services have higher educational levels than those sent to day emergency services, the mother's educational level being the main factor associated with certain characteristics of health seeking behavior (duration of symptoms prior to arrival at hospital, recourse to modern medical drugs). There is often a long delay before recourse to hospital: only 45% of children seen after hours and half of those who died came the 1st or 2nd day of the illness. Self-medication at home is usual but recourse to tradi-practitioners appears rare (4%). Several solutions can be envisaged and should be linked: strengthening of the first level of care, technical improvements in emergency services, training of tradi-practitioners in the recognition and referral of emergency cases, improvement of reception practices at the hospital level, prophylactic and preventive measures for tropical diseases.

[Neonatal mortality in the Tantigou pediatric hospital, Dapaong (north Togo) in 1984-1985 and 1994-1995]. / Mortalité néonatale à l'hôpital d'enfants de Tantigou, Dapaong (nord-Togo) en 1984-1985 & 1994-1995.

370 deaths out of 1141 hospitalized cases of newborns aged 0-28 days were retrospectively analyzed in the Tantigou pediatric hospital of Dapaong, situated 645 km from Lome in northern Togo, in 1984-1985 and 1994-1995. A decreasing trend in neonatal mortality was noted: 42.5% and 27.6% respectively. Major causes of death were prematurity or low birth weight, sepsis, hypoxia conditions. The attendance of pregnant women at health information centres seems to be improving, but the neonatal mortality rate remaining high, the implementation of primary health care/Bamako Initiative resulting especially aiming at better prenatal care for pregnant women must be sustained.

[Use of health facilities by children from 0 to 5 years in Togo. Results of an epidemiological study carried out at a RHC (Atakpamé)]. / Utilisation des structures de soins par les enfants de 0 à 5 ans au Togo. Résultats d'une enquête épidémiologique menée dans un CHR (Atakpamé).

Through a prospective survey coordinated by the CIE of Paris, and carried at a semi-urban regional hospital (Atakpame), the authors studied the use of health structures by inpatient and outpatient consulting children. Questionnaires were filled for the 112 children of the study. Results were as follows: There is a maladjustment between recruitment at the regional hospital and its real mission (emergency cases and special health care). Thus only 13.3% of children were seen for an emergency, and 4.5% admitted for special health case. In the majority of cases (69.4%), the health state of patients was not alarming. Improper channelling of the patients is responsible for this condition. 79.4% of children consult directly at the hospital without prior consultation at the "peripheral" health centers. The regional health center thus finds itself overburdened, whereby long waiting periods before consultation (> 300 mn for certain patients), and delay in the management of referred patients.

[Home care of vaso-occlusive crisis in sickle cell disease in Togo]. / Traitement à domicile de la crise vaso-occlusive par les drépanocytaires au Togo.

BACKGROUND: Many people with sickle cell disease manage their pain crises at home. This study aims to describe the home management of sickle cell pain by Togolese patients. PATIENTS AND METHODS: From July 1996 to April 1997, parents of children with sickle cell disease, and some adults with sickle cell disease living in rural and urban regions were interviewed about their home treatment habits during pain crises. RESULTS: A total of 165 patients with sickle cell disease (82 from urban and 83 from rural areas) were selected. The techniques most frequently used for pain management included salicylates (61.8%), paracetamol (37%), non-steroidal anti-inflammatory drugs (15.1%), vasodilators and pentoxifylline (5.4%). Only 4.2% of the patients mentioned adequate hydration. None used other antalgics (weak or strong opium derivatives). No difference was noticed between the treatment habits of rural regions and those of urban regions. CONCLUSION: In order to improve the quality of life of patients with sickle cell disease, information and awareness programs must be organized in order to establish a standard home pain management. Emphasis must be put on the use of salicylates and paracetamol at the correct dosage, the intake of abundant fluids, the easy use of analgesic of the second step of the Word Health Organization, and the systematic treatment of malaria which can induce pain crises.

[Primary nephrotic syndrome in the black African child]. / Le syndrome néphrotique primitif de l'enfant en Afrique noire.

Idiopathic nephrotic syndrome (INS) in black African children differs from that of children in temperate areas. The main differences are the high rate of corticosteroid non-responders and the low rate of minimal change glomerulopathy in black African children, possibly related to a racial factor. The identification of a high corticosensibility in certain African regions (Togo and Ghana) can lead to the identification of an ethnic factor. Further genetic studies should be carried out in order to provide a better approach to INS in Africa.

[Severe malaria in children in Togo]. / Paludisme sévère de l'enfant au Togo.

BACKGROUND: The definition of severe malaria is no longer limited to cerebral malaria, but is as well extended to other clinical forms of the disease. This work analyses epidemiological, clinical and evolutive aspects of severe malaria in Togo. PATIENTS AND METHODS: This study included 549 children, aged from 0 to 15 years, hospitalized in 1994-5 in the pediatric department of the Lome-Tokoin University Teaching Hospital for severe malaria as defined by World Health Organization (WHO) criteria. RESULTS: The hospitalization frequency was 7.44%; the maximum frequency was from 1 to 5 years of age, but 6.56% of patients were more than 10 years old. The most frequent clinical form was that of severe anemia, followed by cerebral complications, as seen in many African countries. The death rate was 18.94% and the proportional mortality was 8.21%; 2.73% of the patients had neurological sequelae (behaviour disturbances in five cases, aphasia in four, hemiplegia in three, mumbling in one, oculomotor paralysis in one, and cerebellar ataxia in one). Hypoglycemia was fairly frequent (11.6%) and was associated with a poor prognosis. CONCLUSION: It is possible to improve severe malaria prognosis in Africa by insisting not only on better equipment in intensive care wards, but also on improved and early management of hypoglycemia.

[Intracavernous injections of etilefrin: its efficacy in the treatment of priapism in sickle cell patients]. / Injections intracaverneuses d'étiléfrine: efficacité dans le traitement curatif des épisodes de priapisme chez le drépanocytaire.

OBJECTIVE: To date, the treatment of priapism in sickle-cell patients has relied on measures aimed at lowering blood viscosity and acidosis and reducing the level of circulating hemoglobin S (hyperhydration, alkalinization, or exsanguinotransfusion...) Surgical cavernous-venous shunt may be proposed if conservative treatment fails. We examined the efficacy of intracavernous etilefrin injections. PATIENTS AND METHODS: From January 1996 through October 1997 (20 months) we performed 35 consecutive intracavernous injections of an alphastimulant, etilefrin in 7 sickle-cell patients (6 SS, 1 AS) who had experienced one or several episodes of low-flow priapism lasting 2 to 28 hours. RESULTS: Involution of the tumefaction was rapidly achieved in all cases. Tolerance was satisfactory, although some post-injection undesirable effects were reported by certain patients: moderate transient pain (2-5 min) in the retrosternal area, or intense pain in the penis (more intense than the priapism) which lasted 10 to 30 minutes. This work confirmed the earlier reported efficacy of intracavernous injections of etilefrin and suggests that the autonomous nervous system plays an important role in the genesis of this condition in sickle-cell patients. Patients should be informed about the observed undesirable effects which have not been reported previously in the literature. CONCLUSION: Etilefrin can be proposed as first line treatment for priapism in sickle-cell patients (at least in cases lasting less than 24 h). The pathogenic mechanism could involve neuromuscular dysfunction.

[Gastrointestinal and urinary parasitic infection in children at a regional hospital center in Togo: some epidemiological aspects]. / Parasitisme digestif et urinaire chez l'enfant dans un Centre Hospitalier Régional du Togo: quelques aspects épidemiologiques.

To determine the impact of parasitic infection of the digestive and urinary tract in children living in a rural area of Togo, a retrospective study was conducted in a Pediatric Department of Kara, Togo. Results revealed that 35% of the 1610 children between the ages of 0 and 16 years had positive tests for parasites in stools or urine and that 117 had more than one parasite. Trichomonas intestinalis, Entamoeba histolytica, Schistosoma mansoni and Necator americanus accounted for 86.5% of the parasitic infections observed. Parasitic infection was observed during the neonatal period and its incidence increased in males up to the age of 12 years and during the rainy months of the year. Study of associated diseases indicated that 56% of children with parasites also had malaria and that 47% were anemic. Parasitic infection of the digestive and/or urinary tract was noted in 31.8% of children under the age of 5 years with malnutrition.

[Pediatric AIDS at the University Hospital Center-Tokoin (Lomé): the role of protein-calorie malnutrition and a formulation trial of a clinical diagnostic score]. / Sida pédiatrique au CHU-Tokoin (Lomé): place de la malnutrition protéino-énergétique et essai d'élaboration d'un score de diagnostic clinique.

Two cross-sectional studies were carried out in the pediatric ward of the Tokoin Teaching Hospital, Lome. One study determined the prevalence of HIV infection in the 49 malnourished patients treated in the ward in February to March and between August and December 1994. The other was carried out between July 1994 and January 1995 and included 57 other hospitalized children fulfilling at least one of the WHO's pediatric AIDS criteria. The aim was to draw up a screening system for pediatric AIDS based on clinical scores that would be more sensitive than and as specific as the WHO criteria. We tested these criteria and the other signs used in the suggested scoring system using the reference test, HIVchek. The seroprevalence of HIV was 28.6% in malnourished children and transmission was probably exclusively from mother to child. It was difficult to distinguish pediatric AIDS from protein energy malnutrition on clinical grounds, although some of the associated morbidities, including anemia, adenopathy and splenomegaly, were highly suggestive of pediatric AIDS. The second study showed that: 1) the sensitivity of the WHO criteria was low; 2) the best positive predictive values were obtained in cases of polyadenopathy and confirmed HIV infection of the mother. Both these criteria were relatively infrequent; 3) there were 6 criteria significantly associated with HIV infection, each being given a point score according to its Yule coefficient: chronic cough (4 points), chronic diarrhea (3 points), chronic fever (2 points), oropharyngeal candidiasis (2 points) and marasmus (1 point). A score of 4 points was the threshold for suspicion of pediatric AIDS. Our scoring system was more sensitive than the WHO criteria and had similar specificity and positive predictive value. We stress the importance of preventive measures against HIV infection, particularly for women of child-bearing age and suggest a new score test and appropriate clinical definitions for infants and older children.

[Idiopathic thrombopenic purpura in a child in black Africa: a case report in Togo]. / Le purpura thrombopénique idiopathique de l'enfant en Afrique noire: à propos d'un cas au Togo.

UNLABELLED: Idiopathic thrombocytopenic purpurea (ITP) is an autoimmune disease that occurs frequently in Europe and the US, but has rarely been described in Africa. Case report. An 8-year-old girl was admitted for cutaneous and mucosal bleeding. She had a low platelet count (11 x 10(9)/l). ITP was suspected and the diagnosis was confirmed by bone marrow examination. Corticosteroid treatment was effective. DISCUSSION: This is the second case reported in Togo since 1982. The diagnosis of the disease is straightforward, so the lack of cases reported in central Africa suggests that the disease is rare in this region. The rarity of the disease may be due to genetic or environmental factors, or it may simply be that physicians overlook this disease when making their diagnosis. Corticosteroids are now the preferred treatment for ITP because of the risk of transmitting Creutzfeldt-Jacob's disease by intravenous administration of immunoglobulin. Splenectomy is the ultimate treatment for chronic forms. CONCLUSION: Unlike other diseases, the diagnosis and treatment of which require methods unavailable in parts of Africa, ITP treatment, as currently practiced in countries of the northern hemisphere, is within the reach of most African countries. Further studies are required to determine the true frequency of the disease in central Africa.