[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.

An immunohistological comparison of the secretory capacity of villous and extravillous trophoblast in the human placenta.

Using an immunoperoxidase technique the localization of hCG, hPL, SP1, PAPP-A and PP5 within villous and extravillous trophoblast has been studied. The villous syncytiotrophoblast appears to be the sole significant source of hCG, SP1, PAPP-A and PP5 but hPL is also present in the infiltrating extravillous trophoblast. Within the interstitial extravillous trophoblast the proportion of cells staining positively for hPL increases progressively as the cells extend deeper into the uterine tissues but all the vascular extravillous trophoblast within the lumina of the spiral arteries stains positively for hPL at all levels within the decidua and inner myometrium. It is not clear why the synthetic capacity of the infiltrating trophoblast is limited to the production of hPL. We suggest that this might be indicative of selectivity of a specific subpopulation of cytotrophoblastic cells. It is unlikely that the physiological effects of infiltrating trophoblast are mediated by hPL, this substance being probably only a marker of a cell population with a particular invasive capacity.

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].

T system immunodeficiencies in infancy and childhood.

Some problems concerning the diseases due to deficiencies of the T immune system in infancy and childhood are reviewed. The relationships between SCID and Nezelof syndrome and the pathogenesis of this group of diseases are particularly discussed.

HPL-positive infiltrating trophoblastic cells in normal and abnormal pregnancy.

In a series of 24 pregnant women, placental bed biopsies were performed in the third trimester at cesarean section. All the resulting specimens contained infiltrating trophoblast with both small and giant cells, and eight also contained vascular trophoblast. On immunoperoxidase staining for HPL, some small interstitial trophoblastic cells were positive in 12 cases. Some cells of the vascular intramural trophoblast and practically all cells of the vascular intraluminal trophoblast were positive. Seven cases were normal pregnancies whereas 17 were complicated by arterial hypertension and/or fetal growth retardation. A significant correlation between abnormal pregnancy and absence of HPL-positive interstitial cells in the placental bed biopsy was found. This probably indicates a diminished overall number of HPL-positive interstitial cells in the group of abnormal pregnancies and might reflect some defect of interstitial trophoblast. Such a defect may play a role in the arrest of the physiological changes of pregnancy in spiral arteries, which has been described in pre-eclampsia and in many cases of idiopathic fetal growth retardation.

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).

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.

[Mitral valve replacement in an infant with disseminated lupus erythematosus and Libman-Sacks endocarditis]. / Remplacement valvulaire mitral chez un enfant atteint de lupus érythémateux disséminé et d'une endocardite de Libman-Sacks.

The authors report a case of Libman-Sacks endocarditis of the posterior mitral leaflet in a child treated for disseminated lupus erythematosus for 8 years. Mitral valve replacement was performed. Libman-Sacks vegetations are hardly ever recognised in vivo and have never been previously reported in a child. The incidence of this cardiac complication of lupus erythematosus is probably higher than indicated in the literature. Attentive cardiac examination and systematic echocardiography should be part of routine follow-up of patients with disseminated lupus erythematosus.

[Massive fetal-maternal hemorrhage at term associated with a choriocarcinoma]. / Hémorragie foeto-maternelle massive à terme associée à un choriocarcinome.

A case of malignant choriocarcinoma produced a major feto-maternal transfusion at term. This was proven using routine Kleihauer's test. The newborn died on the 5th day of life from haemorrhagic shock. The diagnosis of choriocarcinoma was made when acute post-partum haemorrhage occurred together with a high level of Beta HCG. The patient was cured by the use of Methotrexate followed by four courses of tri-chemotherapy (Methotrexate, Actinomycin and Cyclophosphamide). After treatment for secondary infertility, the patient had two normal pregnancies. This case makes possible to point out again the possible association of a positive Kleihauer test with choriocarcinoma. It also points out strongly the value of carrying out Beta HCG testing when bleeding occurs post-partum. Early diagnosis improves the maternal prognosis and allows conservative treatment to be carried out in cases of choriocarcinoma in young women.

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).

[Fistulas of the 3rd and 4th branchial clefts]. / Les fistules de la 3e et de la 4e fente branchiale.

The third and fourth branchial fistulas are exceptionally rare; both originate from the pyriform sinus, extend through the inferior constrictor of the pharynx and cross either the upper or the lower aspect of the superior laryngeal nerve, downwards and frontwards. Like the more common first and 2nd branchial fistulas, a cervical abscess is the usual consequence of these fistulas which should be treated by radical excision.

Preoperative chemotherapy of cellular congenital mesoblastic nephroma in a 5-month-old infant.

Cellular (or atypical) congenital mesoblastic nephroma (CMN) is a potentially aggressive form of the benign classical congenital mesoblastic nephroma. We report here a case of cellular CMN in a 5-month-old boy treated preoperatively with chemotherapy with an excellent response allowing a complete surgical resection.

[Rupture of the renal artery following blunt trauma. Interest of angiography and therapeutic possibilities (author's transl)]. / Rupture traumatique de l'artère rénale. Intérêt de l'angiographie immédiate et possibilités thérapeutiques.

Isolated rupture and thrombosis of the renal artery is a rare form of traumatic injury : two cases are added by the authors to the seventy reports found in the English and French literature. Clinical signs calling for the diagnosis like hematuria or high blood pressure may be absent. To make an early diagnosis, every patient suffering from acute blunt trauma of the abdomen should be submitted immediately to intravenous urography. Angiography should follow immediately when no secretion is produced on one or both sides. If a lesion of the renal artery is discovered, surgical repair can be attempted when the delay does not exceed 12 to 24 hours from the time of the accident. The best technical modalities include segmental resection with thrombectomy, aortorenal by-pass or eventually renal autotransplantation.

[Intralobar sequestration causing heart failure in a new born infant. Surgical correction]. / Séquestration intralobaire causant une défaillance cardiaque chez le nouveau-né. Correction chirurgicale

The authors report the occurrence of an intralobar pulmonary sequestration in a neonate with symptoms of congestive heart failure, due to a fistula effect in the sequestrated area. The venous drainage was directed mainly to the superior caval vein via azygos vein and accessorily to the left atrium. The patient became asymptomatic after lobectomy performed at the age of 11 days.

Severe combined immunodeficiency disease: a pathological analysis of 26 cases.

Autopsy material and clinical information were analyzed in 25 cases of untreated or unsuccessfully treated severe combined immunodeficiency disease and one case successfully treated by bone marrow grafting. Two cases were adenosine deaminase deficient and one was nucleoside phosphorylase deficient. The histological appearance of the thymus fell into four clearly recognizable patterns: simple dysplasia, dysplasia with corticomedullary differentiation, dysplasia with pseudoglandular appearance, and atrophic pattern. Three cases lacked lymph nodes and belonged to the category of thymic dysplasia with pseudoglandular appearance. From the data, the following conclusions can be made: (i) The thymic atrophic pattern is a phase in a dynamic process of which the end result is simple dysplasia or dysplasia with corticomedullary differentiation. (ii) The pseudoglandular pattern represents a disease process of early intrauterine onset. (iii) At least a proportion of the cases represent a T-cell defect rather than a lymphoid stem-cell defect. (iv) The lymphoid germinal centers are not the source of plasma cells. (v) The graft-versus-host reaction probably causes lymphoid cells depletion in lymph nodes and spleen.

Cerebral, renal and splenic lesions due to fetal anoxia and their relationship to malformations.

Two newborn infants who suffered severe intra-uterine anoxia a few weeks before birth are described. Both died shortly after being born spontaneously and slightly prematurely. In one case the mother had attempted suicide by inhaling butane. The infant's kidneys were hypoplastic and resembled those seen in renal dysplasia, and the brain showed a severe encephalomalacia which would probably have developed into hydranencephaly. The other case was a twin who survived the intra-uterine death of her co-twin: she had hypoplastic kidneys similar to those in the first case, and a hypoplastic spleen. (The brain was not examined.) It is thought that intra-uterine anoxia may produce lesions in fetal organs which appear at birth as congenital developmental malformations. This mechanism is thought to account in particular for some cases of renal dysplasia and for hydranencephaly.