First successful pregnancy outcome after twelve abortions in a Tunisian-woman with the rare p phenotype.

INTRODUCTION: Anti-PP1P k alloantibody, is produced in the serum of individuals with the rare p phenotype. It is associated with severe haemolytic transfusion reactions, recurrent spontaneous early abortions as well as haemolytic disease of the foetus and newborn. Anti-PP1P k alloimmunization in pregnancy differ from others in their physiopathology. It seems that the placenta would be the main target of anti-PP1P k antibody. CASE REPORT: This report concerns a 35 year old female, with a history of a high incidence (12) of early and recurrent miscarriages. She was found to have the extremely rare p phenotype and anti-PP1P k antibody in her serum. Her 13th pregnancy was successfully managed by plasmapheresis. No substitution fluid was added. Oral hydration was recommended before and after the apheresis sessions. 12 plasmapheresis cycles were performed before a healthy term female infant weighing 3kg600g, was delivered by caesarean section at 38 weeks of gestation. CONCLUSION: Plasmapheresis seems to be the treatment of choice in the management of anti-PP1P k fetomaternal incompatibilities. However in this case, we opted for an original and less expensive protocol. We did resort, neither to substitution fluid nor to intravenous immunoglobulin.

Cystic fibrosis transmembrane conductance regulator mutations and polymorphisms associated with congenital bilateral absence of vas deferens in a restricted group of patients from North Africa.

BACKGROUND: Congenital bilateral absence of vas deferens (CBAVD) is responsible for 2-6% of male infertility. It occurs in 95% of men with cystic fibrosis. This malformation is present in patients with a sterile obstructive azoospermia but without clinical evidence of cystic fibrosis. Molecular study of the cystic fibrosis transmembrane conductance regulator (CFTR) gene responsible for cystic fibrosis could show the relationship between this disease and bilateral absence of vas deferens. PATIENTS AND METHODS: The study involved 20 male patients aged between 28-40 years, referred with suspected cystic fibrosis and in whom bilateral absence of vas deferens was confirmed by cyto-biochemical analyses and urogenital ultrasound. Molecular study of the CFTR gene was based on several techniques: DHPLC, DGGE and direct sequencing. RESULTS: Thirteen patients had CFTR mutations: F508del, G542X, W1282X, E1104X, 711+1G → T, V201M (TG) m and IVS8-5T. These mutations were associated with polymorphisms: M470V and D1270N. Seven cases presented only polymorphisms. CONCLUSION: The different mutations found in this study were associated with polymorphisms which decrease the severity of the disease and delay its onset. Thus, bilateral agenesis of the vas deferens is classed as a form of cystic fibrosis with only genital expression.

[Maternal mortality at the Maternity and Neonatology Center in Rabta of Tunis from 1986 to 1989]. / La mortalité maternelle au Centre de Maternité et de Néonatologie de la Rabta de Tunis de 1986 à 1989.

We studied 29 cases of maternal death occurring over a period of 3 years in the "Centre de Maternité et de Néonatologie de La Rabta-Tunis"; managed by the same staff. 42,028 live births occurred during the study years with 43,220 total births from April 24th 1986 to April 23rd 1989. The maternal mortality rate was 69 per 100,000 live births. A maternal age of under 35, nulliparity and grand multiparity were found, as is well known, to be risk factors. Maternal transfer in obstructed labour from rural maternity units raises the maternal death risk 12 times: 14 deaths out of 29 occurred in transferred patients. Haemorrhage represents a quarter of the causes of death (8 cases) while anaesthetic accidents were responsible for one in six maternal deaths. Some factors were not found such as abruptio placentae, while others such as eclampsia were reduced. We concluded that the maternal mortality rate even though it has been reduced over the last 3 decades it is still high compared with developed countries. We can cut it in half by avoiding maternal transfer in labour.