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Biomedical subjects

Z Marrakchi

Publications and source records attributed to Z Marrakchi.

At least 19 recordsLinked to original sources
PubMed2003

[Pharmacologic study of monohydrated caffeine in the treatment of apnoea of premature infant].

UNLABELLED: Monohydrated caffeine was the only respiratory xanthine available in our country to treat apnea of premature infant. The aim of this study was to evaluate plasma levels of this molecule at dosages of 20 mg/kg (equal to 18 mg/kg of caffeine base) as a loading dose and 5 mg(-1) kg(-1) (equal to 4.5 mg(-1) kg(-1) of caffeine base) as a maintenance dose. PATIENTS AND METHODS: The study was prospective including premature infants less than 34 weeks of gestational age born between the 1st of july 2001 and 15th december 2001 and receiving monohydrated caffeine to prevent apnea. Each premature infant has received orally a loading dose of 20 mg/kg in the first hours of life followed, 24 h after, by a maintenance dose orally once a day of 5 mg/kg until 35 weeks of post-conceptional age. Caffeinemia plasma levels were measured by high performance liquid chromatography immediately before the second dose to determine the loading residual rate and immediately before the sixth dose to determine the maintenance residual rate. RESULTS: Twenty-one premature infants were included. Their medium term was 31.4 weeks (27.4-33.3 weeks), birth weight was 1684 g (1000-2800 g) and sex-ratio M/F was 1.3. Fifteen infants (71.4%) have presented apnea with an average of 4.1 episodes per infant and per day. Tolerance of the medicament was good in all cases. The medium loading residual rate was 3.26 microg/ml (1.75-7.80) and the medium maintenance residual rate was 4.26 microg/ml (2.13-7.64). CONCLUSION: Prescribed at a dosage close to twice the recommendations of the literature, monohydrated caffeine does not provide efficient plasma rates. This is probably due to a difference in its oral bio-availability compared with caffeine citrate and further study with greater dosages is needed to appreciate its efficacy.

Administration, Oral↗
PubMed2003

[Diastematomyelia: antenatal diagnosis with successful outcome, two cases].

To discuss, through a review of the literature, clinical signification and antenatal and postnatal management of diastematomyelia. We present two cases highlighting the clinical presentation and discuss management options. The first case was diagnosed in a 32-year-old primagravida female physician. The conssanguinous couple had a history of primary hypofertility. Antenatal magnetic resonance imaging revealed the presence of a sagittal spinal spur separating two asymmetrical hemi cords. Pregnancy was not interrupted due to the lack of factors of poor prognosis. The infant was delivered at term and outcome has been favorable at 18 months follow-up. Ultrasonography performed at 22 weeks gestation provided the diagnosis in the second case. The mother was a 30-year-old primagravida anesthesist who had been addressed for suspected spina bifida. The consanguinous couple had a history of male hypoferility. The ultrasound scan revealed an echogenic spinal spur and integrity of the skin. Pregnancy was not interrupted due to the lack of factors of poor prognosis. The infant was delivered at term and outcome has been favorable at 18 months follow-up. Diastematomyelia is a rare malformation which can be diagnosed antenatally. Careful ultrasonography can distinguish diastematomyelia from myelomeningocele. Antenatal assessment is essential to identify forms with good prognosis because the neonatal outcome of isolated diastematomyelia is generally good, even if surgical repair is required.

Adult↗
PubMed2002

[Bronchopulmonary dysplasia and corticosteroid therapy].

Inflammation plays a major role in the pathogenesis of bronchopulmonary dysplasia of preterm infants. Having a strong anti-inflammatory effect, corticosteroids have been proposed in the management of this disease. Antenatal steroids protect the newborn against its development. Postnatal systemic administration of steroids reduces the duration of mechanical ventilation and oxygen therapy, but is associated with short term and long term adverse effects. Early administration of dexamethasone (before 7 days of life) reduces the incidence and the severity of chronic lung disease at 28 days of life and 36 weeks of post-conceptional age. Inhaled steroids are associated with less adverse effects than dexamethasone administration, but they are also less effective.

Administration, Topical↗
PubMed2002

Efficacy and safety of bromocriptine in the treatment of macroprolactinomas.

To assess the effectiveness of bromocriptine (BRC) as primary therapy in reducing the size of PRL-secreting macroadenomas with extra-sellar extension, we conducted a multicenter study in 29 patients without prior radiotherapy. Clinical presentation, response to medical treatment and long term follow-up of 29 patients with macroprolactinoma (pituitary mass more than 10 mm in diameter) were analysed. There were 19 women for 10 men. Mean age was 33 years. An hypogonadism was present in 94% of women vs 57% for men. Headaches and or visual abnormalities were present in 68% of women vs 90% of men. The mean basal serum prolactin level before treatment was 1 501 ng/ml: 202 ng/ml for women (range: 70-478 ng/ml) and 3 870 ng/ml for men (range: 100-20 476 ng/ml) and was correlated to tumoral size. 25 patients were treated with BRC as primary therapy with a mean dose of 9,7 mg/day (13 mg/day in men and 8 mg/day in women). BRC no malized serum PRL levels in all men and in 17/19 women over a mean period of 6 months, ranging from 3 to 72 months in both sexes. Tumor size was reduced by more than 50% in 18/29 patients (62%) with secondary empty sella in 5 patients and by less than 50% in 11 patients. Visual field improved in most of the patients in whom it was initially abnormal. Reduction in size was quite fast in most of patients but slower in some of them. The cumulative radiological response to BRC increased with time. Therefore, it appears that prolonged medical therapy is effective and safe in macroprolactinomas.

Adult↗
PubMed2000

[Fetal pathology study of 88 cases of letal spina bifida].

Lethal spina bifida continue to be frequent in Tunisia; we report 88 cases of letal spina bifida: 1.05 per thousand births. This pathology was more frequent with women. The up letal spina bifida situated is predominant with female and the dow spina bifida situated is frequent with male. We have noted an association with anencephalia (46 cases) and hydrocephaly (21 cases). Prevention is based on obstetric health care and hygiene dietetic advices to avoid alimentary deficit.

Adult↗
PubMed1998

The Antley-Bixler syndrome. A new case without radiohumeral synostosis.

The author report a new case of the syndrome described by Antley and Bixler. It concerns a female new-born who presents a craniosynostosis with brachycephaly, midface hypoplasia, unilateral choanal atresia, multiple joint contractures and arachnodactyly. There was no bowing of the femora and only the radius and the ulna were bowed. The absence of radiohumeral synostosis reported in all observations of the literature represents the main particularity of this observation.

Abnormalities, Multiple↗
PubMed1994

[Wolman disease].

BACKGROUND: Wolman disease is a severe disease associated with hepatosplenomegaly and adrenal calcifications; it is nearly always fatal in the first year of life. CASE REPORT: A boy was born to consanguineous parents. His weight was 3,500 g, height 53 cm. Hepatomegaly was observed at the age of 26 days; he also had vomiting and watery stools with failure to thrive. Diagnosis of Wolman disease was suspected due to family history. Two sisters had died at the age of 3 months without precise diagnosis; both had abdominal distension, hepatosplenomegaly, anemia and inanition; CT scan showed calcifications of adrenal glands in one of them that had been attributed to adrenal hemorrhage. Investigations in our patient showed no adrenal calcification, hepatomegaly without splenomegaly, anemia (Hb: 8 g/100 ml). Liver biopsy showed enlarged and vacuolated parenchymal and Kupffer cells but the marrow did not contain foam cells. Acid lipase deficiency was demonstrated in cultured skin fibroblasts, permitting prenatal diagnosis in a further sib. CONCLUSION: This case of Wolman disease was the first seen in Tunisia; it was inherited as an autosomal recessive disorder; this patient, as two of his sisters, died during the first 6 months of life.

Hepatomegaly↗
PubMed1993

[Late diagnoses of 21-hydroxylase deficiencies in children (after the age of 3 years].

To evaluate the heterogeneity of 21-hydroxylase deficiency with delayed symptoms, clinical and laboratory findings at presentation in 29 patients whose first symptoms occurred after three years of age were analyzed retrospectively. In 12 patients, these data were confronted with the results of molecular CYP21B gene analysis. Age at onset was 7 years on average and was comparable in boys and girls. Premature puberty was the most common presenting symptom [n = 24], whereas hirsutism, clitoral enlargement, and menstruation disorders were less frequent. Six cases were diagnosed as the result of routine studies of family members of index patients. The bone age over statural age ratio was greater than 1 in 19 of the 27 patients. Baseline 17-OH-progesterone levels were elevated in 22 of the 27 patients; magnitude of the elevation varied widely. Levels of 17-OH-progesterone after stimulation with immediate-action tetracosactide were closely correlated with baseline values and established the diagnosis in doubtful cases. Four patients had post-stimulation 17-OH-progesterone levels under 10 ng/ml, suggesting that were heterozygous for the disease. An important finding was that the magnitude of the devation in 17-OH-progesterone was not clearly correlated with clinical findings at presentation (age at onset, growth rate, advance in bone age). Molecular CYP21B gene analysis performed in 12 patients disclosed a homozygous 281 Val Leu mutation in 6 cases. This is the most commonly reported mutation in delayed onset forms. Two patients were heterozygous for the 281 Val Leu mutation and had an allele associated with severe disease, suggesting that the least severely affected chromosome governed clinical presentation of the disease. One boy had an allele associated with neonatal onset on both chromosomes; molecular analysis indicated a risk of antenatal masculinization of female fetuses in this family. This study showed that delayed onset 21-hydroxylase deficiency is a heterogeneous entity and that molecular analysis is essential to genetic counseling.

17-alpha-Hydroxyprogesterone↗
PubMed1990

[Pre-eclampsia: a cause of fetal heart failure?].

The authors report the case of a newborn whose mother presented with pre-eclampsia. Intrauterine growth retardation, peripheral edema, ascitis and pleural effusion were present at birth. The authors suggest that placental vascular abnormalities could be responsible for fetal heart failure and edema syndrome.

Adult↗