[The fight against rabies in Africa: from recognition to action]. / Lutte contre la rage en Afrique: du constat à l'action.

As a follow-up to the first AfroREB (Africa Rabies Expert Bureau) meeting, held in Grand-Bassam (Côte-d'Ivoire) in March 2008, African rabies experts of the Afro-REB network met a second time to complete the evaluation of the rabies situation in Africa and define specific action plans. About forty French speaking rabies specialists from Northern, Western and Central Africa and Madagascar met in Dakar (Senegal), from March 16th to 19th, 2009. With the participation of delegates from Tunisia, who joined the AfroREB network this year, 15 French speaking African countries were represented. Experts from the Institut Pasteur in Paris, the Alliance for Rabies Control, and the Southern and Eastern African Rabies Group (SEARG, a network of rabies experts from 19 English speaking Southern and Eastern African countries) were in attendance, to participate in the discussion and share their experiences. AfroREB members documented 146 known human rabies cases in all represented countries combined for 2008, for a total population of 209.3 million, or an incidence of 0.07 cases per 100,000 people. Even admitting that the experts do not have access to all reported cases, this is far from the WHO estimation of 2 rabies deaths per 100,000 people in urban areas and 3.6 per 100,000 in rural Africa. It was unanimously agreed that the priority is to break the vicious cycle of indifference and lack of information which is the main barrier to human rabies prevention.

Fighting rabies in Africa: the Africa Rabies Expert Bureau (AfroREB).

Rabies experts from 14 francophone African countries met in Grand Bassam (Côte d'Ivoire), 10-13 March 2008. They presented the situation in their respective countries, acknowledging the lack of rabies awareness among the population, health care workers and health authorities. They recognized that infrastructure for the management of rabies exposure is scarce, modern vaccines are in limited quantity and immunoglobulins are lacking in most of their countries. They defined as a priority the need to have reliable figures on the disease burden, which is necessary for informed decision making and priority setting, and for applying for aid in controlling the disease. This meeting sealed the establishment of the Africa Rabies Expert Bureau (AfroREB).

Management of hepatic epithelioid haemangio-endothelioma in children: what option?

Hepatic epithelioid haemangio-endothelioma (HEHE) is an endothelium-derived tumour of low-to-medium grade malignancy. It is predominantly seen in adults and is unresponsive to chemotherapy. Liver transplantation is an accepted indication when the tumour is unresectable. Hepatic epithelioid haemangio-endothelioma is very rare in children and results after transplantation are not reported. The aim of this study is to review the experience of three European centres in the management of HEHE in children. A retrospective review of all paediatric patients with HEHE managed in three European centres is presented. Five children were identified. Four had unresectable tumours. The first had successful resection followed by chemotherapy and is alive, without disease 3 years after diagnosis. One child died of sepsis and one of tumour recurrence in the graft and lungs 2 and 5 months, respectively, after transplant. Two children who had progressive disease with ifosfamide-based chemotherapy have had a reduction in clinical symptoms and stabilisation of disease up to 18 and 24 months after the use of platinum-based chemotherapy. HEHE seems more aggressive in children than reported in adults and the curative role of transplantation must be questioned. Ifosfamide-based chemotherapy was not effective. Further studies are necessary to confirm if HEHE progression in children may be influenced by platinum-based chemotherapy.

Gastric choristoma of the midline neck in a newborn: a case report and review of the literature.

The current report describes a 3-week-old boy presenting with a gastric choristoma of the anterior compartment of the neck. Initial examination at birth found 3 masses in the anterior region of the neck. The only remarkable symptom consisted of episodes of cyanosis induced by neck flexion. The neck mass was removed after computed tomography scan assessment. Pathology finding showed the diagnosis of gastric choristoma. Cervical location of a gastric choristoma in children is rare. Its presentation as a clinically palpable anterior compartment cervical mass at birth even more rare.

Pseudo-tumoral proliferative nodule in a giant congenital naevus.

OBJECTIVE: To discuss the characteristics of proliferative nodules in giant congenital naevi. METHODS: We report the case of a newborn referred for staged curettage of a giant congenital naevus. A nodule was discovered on his left flank. It was excised for analysis during the first treatment session during the second week of life. RESULTS: The nodule was soft and looked like a lipoma. On optical microscopy however, there was a high cellular density and a high number of mitoses. Although the genetic analysis for melanoma antigens was reassuring, a firm nodule recurred a few days later. A second excision was performed at the fourth week. Surprisingly, on optical microscopy, the cellular density was much lower and there were no more atypias or mitoses; many neurotization foci were present. The natural history changed to spontaneous regression of the cellular activity. The diagnosis of proliferative nodule was made. CONCLUSION: Proliferative nodules in giant congenital naevi have specific clinical and histological characteristics. These should however be put into perspective. As demonstrated in this case, there can be an initial high mitotic activity within the nodule but this should not lead to the misdiagnosis of malignant melanoma. The spontaneous regression of cellular activity will allow the correct diagnosis to be made.

Smooth muscle tumor developing in an immunocompromised child after therapy for leukemia.

We report a 5.5-year-old boy who underwent autologous peripheral blood stem cell transplantation for high-risk acute lymphoblastic leukemia and who had two abdominal masses develop 6 months later. Macroscopically complete resection of the abdominal tumors was performed and revealed a well-differentiated leiomyosarcoma. Smooth muscle tumors, benign or malignant, are increasingly recognized in children with various immunodeficiencies; the association with acute lymphoblastic leukemia is rarely described.

Urachal neuroblastoma: first case report.

Tumours of the urachus are exceptional in children. They represent 0.01% of all tumours and consist of mucosecretory adenocarcinoma and, more rarely, transitional cell carcinoma. We report a 6-month-old child with a urachal mass which, following biopsy, was shown to be a neuroblastoma.

Meningioma of the infratemporal fossa: report of a case.

We report the case of a 25 year old male who presented with a well-circumscribed mass in the infratemporal fossa, without intracranial localisation. On histological examination, this tumor was a transitional type of meningioma. We review the literature and discuss the pathogenesis and the differential diagnosis.

[Peliosis hepatis with hepato-pulmonary syndrome]. / Peliosis Hepatis with Hepato-Pulmonary Syndrome.

Sinusoidal dilatation is a hallmark of peliosis hepatis, a rare vascular disorder that can be either drug induced or of infectious origin. Here we report a patient with peliosis hepatis of unknown etiology. An hepato-pulmonary syndrome developed which was reversible following liver transplantation.

Hirschsprung's disease: a 20-year experience.

During the period from 1972 to 1992, 59 children received surgical treatment at the University of Louvain Medical School for biopsy-proven Hirschsprung's disease (HD). The extent of aganglionosis was as follows: short segment restricted to the rectosigmoid or descending colon (n = 44, 75%); long segment (n = 9,15%); ultra-short segment (n = 3, 5%); unknown length because of death without autopsy (n = 3, 5%). The median age at operation was 7 months for short-segment disease compared with 14 months for those with long-segment disease. Surgical procedures used for short-segment disease were Swenson with colostomy (n = 16), Swenson-Pellerin without colostomy (n = 27), Duhamel (n = 1), and for long-segment disease were Martin (n = 3), Swenson-Deloyers (n = 2), Swenson-Boley (n = 2) and ileostomy only in = 2). Lynn's sphincteromyotomy was performed in the three ultra-short cases. There were six deaths (10%) at a median age of 86 days (range, 28 to 1545 days), three had long-segment disease, and the others were not classified because of death before curative surgery. Enterocolitis (EC) was the most common cause of death (five cases) and was also the major source of morbidity after curative surgery (12 of 44, 27%) in short-segment patients, three of seven (43%) in long-segment patients. The functional success of the procedure was evaluated in 70% of the surviving patients (37 of 53; mean follow-up, 8.7 years; range, 1.2 to 21.5), using a novel semiquantitative scoring system, specifically designed for children who have HD. This system assesses normal stool evacuation, abdominal distention, soiling, and severe incontinence. The results were compared with those from a population of 39 healthy children and adolescents and demonstrated progressive improvement in function during childhood and adolescence (P = .04) for patients treated for short-segment disease. However, function was found to be consistently poorer in all age groups when compared with healthy controls (5 to 10 years, P < .01; 10 to 15 years, P < .05; > 15 years, P < .01).

Tricho-hepato-enteric syndrome: further delineation of a distinct syndrome with neonatal hemochromatosis phenotype, intractable diarrhea, and hair anomalies.

We report on two sibs with syndromal congenital iron storage disease. Prenatal symptoms were IUGR, hydramnios, and placental hyperplasia. Clinical anomalies included hypertelorism and sparse, thin, curly hair (trichomalacia). Clinical course was marked by intractable diarrhoea, with normal histological and enzymological studies, cholestatic jaundice, hepatomegaly appearing after 30 days, and progressive liver failure, leading to death after a few months. The only metabolic anomaly was progressive hypermethioninemia. Pathologic examination of both children showed a similar pattern of multivisceral iron deposit compatible with a diagnosis of neonatal hemochromatosis: extensive liver fibrosis or cirrhosis with nodular regeneration, cholestasis, ductular proliferation, and hepatic, pituitary, thyroidal, adrenal, and pancreatic iron deposition. The unusual course for neonatal hemochromatosis in both sibs combined with concordant extrahepatic anomalies suggest that they could have a specific iron storage syndrome with possible autosomal recessive inheritance, probably similar to the sibship reported by Stanckler et al. [Arch Dis Child, 57:212-216, 1982].

A new familial short stature syndrome: Brussels type.

A new association of congenital familial short stature with facial dysmorphism and osteochondrodysplastic lesions is described in two siblings. Clinical abnormalities include severe prenatal and postnatal growth failure and facial dysmorphism. Radiographs show osteochondrodysplastic lesions with a narrow thorax, short ribs, epiphyseal maturation delay and slightly deformed metaphyses. Microscopic analysis of the skeleton shows pathological features.

Nonossifying fibromyxoid tumor of soft tissue.

A firm, painless tumor of the temporal region was excised in a 3-year-old girl. The diagnosis of nonossifying fibromyxoid tumor was established on pathologic examination, which showed that most of the cells formed cords and tubulelike structures in a myxoid background. Bone and osteoid were lacking. The child is free of recurrence 4 years after surgery.

Fetal liver hemangioma and chorioangioma: two unusual cases of severe fetal anemia detected by ultrasonography and its perinatal management.

Severe anemia was diagnosed by cordocentesis in two fetuses, one with a large chorioangioma, the other with a liver hemangioma. Despite the presence of a chronic fetotumoral shunt, both cases were partly treated by a fetal intravascular transfusion, followed by an elective Cesarean section. This 'predelivery' management of anemia should improve neonatal morbidity associated with severe hematological and acid-base balance disorders.

Breast infiltration by acute lymphoblastic leukemia during pregnancy.

A case of bilateral breast infiltration as the initial manifestation of FAB-L3 leukemia during pregnancy is reported. This rare presentation is usually associated with Burkitt's lymphoma or FAB-L3 leukemia. A high suspicion of the presence of hematologic malignancy and appropriate evaluation are indicated in the presence of this clinical manifestation.