[Hereditary spherocytosis]

Hereditary spherocytosis [HS] is a constitutional disease of red corpuscles. Hereditary pattern is autosomic dominant in 80 percent of the cases. It is usually appeared in childhood by regenerative anaemia of variable gravity. It can be symptomatic in the neonatal period. Jaundice is the first and more frequent symptom. Through two observations of HS with neonatal revelation, we carry clinical, biological and therapeutic particularities of this affection in neonatal period

Metoclopramid methemoglobinemia: a rare cause of neonatal cyanosis

The authors report three cases of methemoglobinemia diagnosed in premature. The new-borns presented isolated cyanosis. All explorations were normal. Diagnosis of methemoglobinemia was confirmed by a high blood rate of methemoglobin. Over dose of metoclopramid related to an error of its administration was noted. The authors recall physio-pathology, diagnosis, causes and treatment of acquired methemoglobinemia, while insisting on necessity of primary prevention

Sirenomelia: report of two cases

Sirenomelia is a serious congenital deformity in which the legs are fused together, giving the appearance of a mermaid [of the Greek Mythology], Its prevalence has been estimated at about 1, 5 to 2 in 100 000 births. We report two cases of foetuses with Sirenomelia. The first case is that of a premature baby weighing 1050g. The second case is a premature baby resulting from a triple pregnancy and weighing 1450g. They presented a fused lower limb and absent kidneys, anus and external genitalia. They died rapidly. These two cases will allow us to debate the clinical signs, the aetiology and the prognosis of such congenital malformation as well as the difficulties of prenatal diagnosis

[Isolated congenital tracheoesophageal fistula. About two cases revealed in neonate]

The authors report two cases of isolated tracheoesophageal fistula revealed in neonate. It is a rare malformation that presents with a characteristic triad of symptoms: choking and cyanosis on feeding, abdominaldistension and recurrent respiratory tract infection. Children are invariably symptomatic from birth. These symptoms may be intermittent and may vary in severity. The barium swallow confirms the diagnosis and determins its location in two cases. The treatment is surgical

[Nicardipine versus salbutamol in the treatment of premature labor: comparison of their efficacy and side effects]

The objective of this study is to compare the tocolytic action and the side effects of nicardipine to those of salbutamol in patients presenting premature labor in order to propose nicardipine as a promising alternative to salbutamol in the treatment of premature labor. Forty eight patients admitted for premature labor were included in this prospective randomized study comparing nicardipine and salbutamol, administered with intra venous drip. The nicardipine group included 25 patients and the salbutamol group included 23 patients. The epidemiologic characteristics of the 2 groups are similar. No significant difference between the 2 groups was noted in the average time of disappearance of the uterine contractions. In nicardipine group, the pregnancy was prolonged more than 48 hours in 87.5% of the cases against 85.8% in salbutamol group [P: NS]. Adverse effects linked to tocolysis were recorded. In nicardipine group, 8% of the patients presented an intolerance of the molecule, against 47% of salbutamol group, the difference is significant [P=0.02]. The term of delivery was similar in both groups. The neonatal status of the infants was identical in the two groups. In the group of new born hospitalized, we did not note biological disorders concerning the glycemia and the calcemy. Nicardipine is at least as effective as salbutamol in tocolysis; but it has less secondary effects. Thus, nicardipine is proposed as the tocolytic of first intention instead of the salbutamol which is considered as reference molecule

[Transvaginal ultrasound of the cervix in the estimation of severity of premature labour]

Premature labour is a frequent situation it complicates about 6% of births, with increasing perinatal morbidity and mortality. The purpose of this study is to analyze the place of the ultrasound of the cervix in the coverage of a premature labour and the evaluation of the severity of the premature labour, being able to have therapeutic implications. It is about a prospective study on 48 patients presenting a premature labour between 28 and 35 weeks realized in the service of maternity in Hddi Chaker hospital of Sfax for a period of 7 months going from January till July, 2005. In our study 23 patients presented an ultrasonographic cervical length

[Symptomatic hypertrophic cardiomyopathy in infants of diabetic mothers]

Hypertrocphic cardiomyopathy [HCM] in infants of diabetic mothers is characterized by asymmetric hypertrophy of the septum and interventricular walls. This affection is related to a transitional fetal hyper insulinism and is asymptomatic in most cases. Rarely HCM cause respiratory distress and congestive heart failure. Sometimes severe symptoms can be observed like asystolie and sudden fetal or neonatal death. The outcome of this disease is usually favorable with regression of myocardial hypertrophy. We report three cases of symptomatic cardimyopathy in infants of diabetic mothers diagnosed by echocardiography, all cases had favorable outcome with symptomatic treatment. We insist on the interest of the management of the diabetic mothers during pregnancy to reduce frequency and gravity of this disease and on the contribution of the echography to optimize the treatment