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Biomedical subjects

P Vanlieferinghen

Publications and source records attributed to P Vanlieferinghen.

At least 19 recordsLinked to original sources
PubMed2006

[Idiopathic intrahepatic portosytemic shunts in 4 children].

Intrahepatic portosystemic anastomoses are macroscopic communications between the venous portal system and the systemic circulation and located partly in the liver. We report 4 new cases of type II shunts, which illustrate the circumstances of the diagnosis of these exceptional anomalies. For 2 children, the diagnosis was done antenataly by US and spontaneous involution in a few months was observed. In the third case the malformation was evidenced fortuitously at 3 weeks of life, and this 6-year-old child remains completely asymptomatic so far. Then, in the fourth case, a cerebral venous thrombosis was fortuitously and antenatally evidenced in an otherwise uneventful pregnancy and portosystemic shunt was demonstrated postnataly in the extensive work up of the neonate.

Follow-Up Studies↗
PubMed2004

[Obstetrical prognosis of labour induction with mifepristone after 41 weeks of gestation].

OBJECTIVE: To compare the mode of delivery in two groups of patients selected by their response after induction of labour with mifepristone. PATIENTS AND METHODS: We studied retrospectively 89 cases of labour induction with viable children after 41 weeks of gestation. Bishop scores were less than 6. Patients were given 200 mg of mifepristone per day for 48 h. They were retrospectively divided into group 1 (spontaneous onset of labour or premature rupture of membranes before the third day) and group 2 (not in labour by that date). RESULTS: The mean Bishop score at inclusion was 3.1 +/- 1.3. Among the 51 patients (53.9%) in group 1, one required prostaglandins and we performed 10 cesarean sections. In group 2, the mean Bishop score at the 3rd day was 4.4 +/- 1.3 (P < 0.0001). Twenty-four patients required prostaglandins (P < 0.0001) and we performed 17 cesarean sections (P = 0.01). The number of cesarean sections increased with the dose of prostaglandins (P = 0.025). We observed no maternal or fetal complications. DISCUSSION AND CONCLUSIONS: Mifepristone was successful in inducing labour spontaneously in over 50% of pregnancies after 41 weeks of gestation. In the other group, the probability of vaginal delivery was reduced especially when high doses of prostaglandins were required. After the use of mifepristone, we suggest to shorten the duration of prostaglandin administration (two applications of 2 mg dinoprostone) before performing cesarean section.

Adult↗
PubMed1999

[The re-emergence in 1997 of rubella infections during pregnancy. 11 cases in Clermont-Ferrand].

OBJECTIVE: Describe the clinical and laboratory features of rubella observed during the first semester of pregnancy in 11 patients in 1997. PATIENTS AND METHODS: Eleven pregnant women, aged 15-30 years, were referred to the Clermont-Ferrand University Hospital for suspected rubella. Four had had at least 1 prior pregnancy, none had been vaccinated. Rubella serology was obtained for all 11 patients and polymerase chain reaction viral amplification was performed on amniotic fluid in 9 cases. RESULTS: The virology laboratory identified 8 cases of primary rubella (2 prior to 12 weeks gestation) and 3 reinfections (1 prior to 12 weeks gestation). Fetal infection was evidenced in I gravida II patient at 17-18 weeks gestation. All pregnancies were continued to term and no case of congenital rubella malformation was observed. However specific IgM assays were performed at birth in 6 of the 11 infants and revealed infection in 3. CONCLUSION: These observations indicate that a local epidemic of rubella occurred in the general population. They illustrate the risk of a rubella epidemic in France and the lack of sufficient vaccination of the young adult population, finally they emphasize that current anti-rubella vaccination programs should be promoted.

Adolescent↗
PubMed1996

[Transient bullous epidermolysis of the newborn infant. A benign clinical form of dystrophic bullous epidermolysis or an autonomous entity?].

INTRODUCTION: Transient bullous dermolysis of the newborn is a bullous eruption limited to friction zones. It appears at birth and disappears during the first months of life. CASE REPORT: Immediately after delivery, an infant girl presented cutaneous bullae on areas of trauma which spontaneously regressed after a few weeks. The histology examination confirmed subepidermal involvement (the roof of the bullae took up the anticollagen IV antibody) and ultrastructure anomalies in the baseline membrane: intracellular vacuoles in the keratinocytes containing fibrillary material, disorganization of the anchoring fibers. DISCUSSION: Transient bullous dermolysis of the newborn is a rare (less than 15 cases reported in the literature) benign disease which regresses spontaneously, possibly an explanation of the small number of cases reported. The anomalies in the ultrastructure observed in the baseline membrane strongly suggest transient impairment in collagen VII maturation and excretion. These anomalies are not pathognomonic and can be observed in dystrophic bullous epidermolysis. Currently there is no specific genetic marker to established transient bullous dermolysis as unique entity.

Epidermolysis Bullosa↗
PubMed1994

Fetal umbilical cord ligation under ultrasound guidance.

In a patient with severe twin-to-twin transfusion syndrome, ultrasound-guided umbilical cord ligation of the hydropic recipient twin was performed at 27 weeks' gestation. The procedure was successful in arresting the blood flow and was associated with improvement in the condition of the severely compromised donor fetus. At 29 weeks' gestation, premature onset of labor occurred and a healthy baby was delivered by emergency Cesarean section. This report demonstrates the feasibility of ultrasound-guided cord ligation for selective feticide in a case of severe twin-to-twin transfusion syndrome.

Journal Article↗
PubMed1994

LADD syndrome in five members of a three-generation family and prenatal diagnosis.

We describe five members of a three generation family with lacrimo-auriculo-dento-digital (LADD) syndrome. The circumstances in which the diagnosis was reached and the details of the case reports underline the great variability of expression of this syndrome and show that caution should be taken in genetic counselling. Prenatal ultrasound should be offered to families at risk so that severe forms of the syndrome, in which termination of pregnancy can be considered, are early detected.

Abnormalities, Multiple↗
PubMed1993

Treatment of homozygous familial hypercholesterolemia by plasma exchange and LDL-apheresis.

Two girls with familial hypercholesterolemia were treated for 7 years by plasma exchanges (PE) or LDL-apheresis (LA). We compared different methods of treatment; PE with or without reuse of the plasma separator, LA of varying frequency, and LA with or without oral administration of simvastatin. We assessed the long-term results by measuring the blood levels of the biochemical parameters before sessions, and determined the effectiveness of each session by the percentage of decrease in the blood levels between the beginning and the end of the sessions. LA led to a more selective treatment (lowering of LDL cholesterol and maintenance of HDL cholesterol), but the blood levels of total cholesterol before sessions were the same as those obtained by PE. IgG and haemoglobin levels decreased little with LA. The rhythm of one session a week gave better results in LA. Although reuse of the plasma separator represents a financial saving it produced poorer results. The oral administration of simvastatin improved the results of LA.

Adolescent↗
PubMed1993

Fetal blood cell membrane fluidity in small for gestational age fetuses.

Blood cells membrane fluidity was assessed prenatally by fluorescence polarization for anisotropy, microviscosity, degree of order and fusion activation energy in 20 fetuses who underwent percutaneous umbilical blood sampling for intrauterine growth retardation (IUGR) and in 25 controls for normal weight sampled for other indications. Simultaneously, blood samples were collected from each mother for comparison. Regulators of membrane fluidity (i.e., cholesterol, phospholipids, free fatty acids) were also assessed. Student t test was employed for analysis. Membrane fluidity was lower in control fetal cells than in adults (p < 0.05) and lower in IUGR fetuses than in controls (p < 0.05). The mechanism may involve a low cholesterol concentration and a low unsaturated/saturated free fatty acids ratio in fetal blood cells membranes and plasma. Fetal cells membrane fluidity reflects in part fetal nutritional status.

Blood Cells↗
PubMed1993

[Prenatal diagnosis of congenital cerebral tumors. Apropos of 3 cases].

BACKGROUND: The prognosis for congenital brain tumors is usually poor, so that their management during pregnancy is difficult. CASES REPORTS: Case 1. A large cystic mass was revealed by ultrasound at the 37th week of pregnancy; it was located near the brain stem and was associated with ventricular dilation. The newborn was delivered by cesarean section because of an abnormally enlarged head. The CT scan confirmed the presence of this mass containing a fluid that was found to be normal after needle aspiration. Surgical shunting of excess fluid was ineffective, and progressive deterioration prevented further exploration and/or treatment. The child is still living, confined to his bed, at the age of 5 years. Case 2. Ultrasonography at the 30th week of pregnancy showed a tumor located near the brain stem with dilation of the entire ventricular system. Post mortem examination after abortion revealed a capillary and cavernous hemangioma. Case 3. Ultrasonography at the 34th week of pregnancy showed dilation of the ventricular system. As a result, birth was induced. CT scan and MRI of the newborn showed a mixed, solid and liquid, mass in the posterior fossa. Post mortem examination showed a papilloma of the choroid plexus. CONCLUSION: These rare congenital tumors are usually revealed by ultrasonography showing ventricular dilation. Advances in imaging techniques, especially MRI of fetal brain, should help in the management of such tumors.

Brain Neoplasms↗
PubMed1992

[Amiodarone and fetal supraventricular tachycardia. Apropos of a case with neonatal hypothyroidism].

BACKGROUND: Fetal tachycardia can be a cause of in utero death. Its detection is not always easy and its treatment is still controversial. CASE REPORT: Paroxysms of supraventricular tachycardia were detected on echocardiography at the 25th week of a second pregnancy. The mother was given sotalol, but the supraventricular tachycardia became permanent. At the 27th week of gestation, sotalol was stopped and the mother was given digoxin and the foetus received 2 injections of digoxin, 10 micrograms/kg, via the umbilical cord. As this treatment was only partially effective, the mother was also given amiodarone 800 mg/day at week 28, then the dose was reduced to 400 mg/day. However, at the 31st week, the mother showed signs of digoxin intolerance, and it was replaced by sotalol. Fetal blood tests at week 34 showed a high placental transfer of digoxin and sotalol and a low fetal level of amiodarone. The newborn, a girl, was born at the 36th week having a sinus rhythm. She developed signs of hypothyroidism (T4: 4 micrograms/ml; TSH:325 microliters U/ml at 5 days of life). CONCLUSION: The placental transfers of sotalol, digoxin and amiodarone are in the range of values known to be effective. The amiodarone responsible for hypothyroidism was given to the mother because she was intolerant to digoxin. Its use must be limited to arrhythmias that are resistant to other drugs or complicated by hydrops fetalis. When used, amiodarone should not be given for more than 6 weeks, and at the lowest possible dose.

Amiodarone↗
PubMed1992

[Evaluation of a three-year (1988-1990) prenatal screening of malformative uropathies in the department of Puy-de-Dôme].

During a 3-year period, 93 prenatal diagnoses of kidney or urinary tract abnormalities were carried out in the French district of Puy-de-Dôme. Sixty-nine mothers were resident in this area giving an incidence of 2.8 out of 1000 births. The pregnancy was interrupted in 10 cases, there were 2 stillbirths and three infants died within two months of life. The most frequent abnormalities were: hydronephrosis (48% of cases), megaureter with or without ureter duplication (19%) and multicystic dysplasia (16%). The prenatal diagnosis was confirmed after birth in 82% of cases. Of the 56 infants with obstructive uropathies, 17 underwent a pyeloplasty within three months of life, 32 had conservative treatment, of whom 4 were operated on afterwards, and seven could not be traced. Of the seven infants who had normal ultrasound scan at birth, three had abnormal scan during the follow-up one of whom was operated on. Nephrectomy was not performed in any of the 11 cases of multicystic dysplasia: one patient was lost to follow up, three had stable lesions and in seven cases, the size of the cysts decreased.

Female↗
PubMed1991

[Morbidity and psychomotor development at 2 years of age in children born in Puy-de-Dome in 1983. Study of children groups defined by perinatal risk].

All infants born in the Puy-de-Dôme area in 1983 and referred to a neonatal care unit were studied prospectively. Children were seen at their homes at 9 months and at 2 years of age and results were compared with those found in controls. High risk groups were defined on the basis of neonatal variables including prematurity, neurologic anomalies, respiratory distress, assisted ventilation, and growth retardation. At birth, prematurity was associated with an increased risk of respiratory distress and infections were more common in small-for-dates infants. Evaluations at 9 months and at 2 years of age showed increased prevalences of growth retardation and strabismus in the high-risk infants, and neurodevelopmental tests disclosed lower performances in these children. Conversely, infectious diseases and readmissions between birth and two years of age were not increased in the high risk groups, as compared with the control group.

Child, Preschool↗
PubMed1989

[Diagnosis of deficiency in cofactor of phenylalanine hydroxylase: a metabolic emergency].

We report on two cases of children suffering from biopterin synthetase deficiency. Both were treated with the same treatment schedule with biopterin and neurotransmitters: 6-hydroxytryptophan and dihydrophenylalanine (DOPA). The only difference between the two cases is the time of diagnosis and therefore of treatment. The child who was treated early has a normal neurologic development. The other one has been treated since he was 7 months old and is mentally deficient (DQ = 0.60). This older child also suffers from dystonia probably secondary to Levodopa treatment. The authors emphasize the uncertainty of these patient's evolution owing to complications of the disease itself or those due to prolonged treatment by neurotransmitters.

5-Hydroxytryptophan↗
PubMed1989

[Prenatal diagnosis of X-linked adrenal hypoplasia associated with glycerol kinase deficiency].

We report a case of X-linked adrenal hypoplasia associated with glycerol kinase deficiency in a boy. Cytogenetic studies and X-linked probes did not demonstrate deletion at Xp21. These probes are not informative enough to be used in prenatal diagnosis. This diagnosis was achieved by glycerol concentration assay in amniotic fluid and by maternal plasma estriol assay.

Adrenal Glands↗
PubMed1989

[Renal agenesis and the Fraser syndrome: 4 observations].

The authors report on 4 cases of Fraser syndrome in 2 Turkish families. Both families are consanguinous. In 3 cases there is a bilateral renal agenesis, a feature which is not usually regarded as a main one. Actually the survey of the literature reveals that renal anomalies are not infrequent in this syndrome, even though the cryptophtalmos would be lacking. A five year study of the malformations Registry of the Rhone-Alpes-Auvergne-Jura area shows that the association between renal agenesis and syndactyly (with or without the eye abnormalities) is quite rare. Such an association leads to the diagnosis of Fraser Syndrome even when cryptophtalmos is absent, and requires to look for minor ENT or ophthalmic symptoms by a careful post mortem examination.

Abnormalities, Multiple↗
PubMed1988

Isolated familial adrenocorticotropin deficiency: prenatal diagnosis by maternal plasma estriol assay.

We report on a brother and sister with adrenal insufficiency due to isolated adrenocorticotropin hormone deficiency discovered in the neonatal period. The first-born, a male infant, died; pathological findings suggested bilateral adrenal hypoplasia transmitted as an autosomal recessive trait. Plasma estriol levels were assayed during the mother's next pregnancy. The prenatal diagnosis allowed immediate and effective management of the second affected child. The supplementary evidence from the endocrine findings, unavailable on her brother, enabled us to make a diagnosis of isolated central ACTH deficiency. As the defect was found in infants of both sexes in the same family, it is in all likelihood transmitted as an autosomal recessive trait. We consider it important for genetic counselling to perform autopsies on all newborn infants whose death has no apparent cause. Maternal plasma estriol assays during pregnancy can help diagnose fetal adrenal insufficiency, whether the defect is central or adrenal.

Adrenal Insufficiency↗