[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

[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

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

[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