[Blueberry Muffin Baby and spontaneous remission of neonatal leukaemia]. / Leucémie aiguë néonatale et Blueberry Muffin syndrome: à propos d'un cas spontanément régressif.

Blueberry Muffin baby is a rare neonatal skin disorder. Many causes are known, examples are congenital infections, hemolysis and tumors. We report on a newborn presenting with Blueberry Muffin syndrome and an adrenal mass which lead to the diagnosis of neuroblastoma. Actually, it corresponded to an acute monoblastic leukaemia with an adrenal localization and a cerebrospinal fluid involvement. Leukaemia should always be considered in such patients, even in the absence of blasts on white blood cells count and bone marrow examination, as in this patient. This observation was also unusual due to spontaneous remission. The patient is in complete remission at 1 year follow-up.

[Video: An innovation for developmental care in the neonatal intensive care unit]. / La surveillance vidéo : une innovation au service des soins de développement en unité de soins intensifs de néonatologie.

INTRODUCTION: Our neonatal intensive care unit (NICU) was designed with a web-camera system in eight private rooms and four open wards. This equipment was installed to both guarantee children's safety in the NICU and promote the bond between the child and his/her family through a viewing service permitted by an Internet access. METHOD: We evaluated the web-camera system in its 5th year with two types of users. The nursing staff was asked about use of the video and its impact on their management of NICU patients. Questionnaires for parents sought to determine how they used the system and their feelings about it. RESULTS: Ninety-three percent of the nursing staff used the web-camera system to provide medical supervision or to comply with developmental care, mainly to respect the baby's natural rhythm by initiating care only when the baby showed signs of awakening. The web-camera system allowed them to observe the baby, verify his/her good body position, and spot discomfort situations. It helped provide a faster and personalized answer that was adjusted to the baby's needs. Sixty-one percent of the parents used the remote connection, half of them to present the child to the family. Only 17% of parents were embarrassed by the cameras; 88% of parents thought that the video was an additional safety device in NICU management. CONCLUSION: The web-camera system appeared to be an interesting technology in the NICU to support developmental care when children could be put in private rooms. It helped staff react faster to situations that created an inappropriate stimulation for the baby. It also allowed to them to respect the baby's natural rhythm. It reassured nursing staff and parents and it facilitated the baby's integration into the family.

Intracranial pressure in childhood central nervous system infections.

To determinate the importance of intracranial hypertension in central nervous system (CNS) acute infections, we studied intracranial pressure (ICP) in 27 patients, age 45 days to 13 years. Fourteen had meningitis and 13 had encephalitis; all were in deep coma with Glasgow Coma Scale 7 or less. Intracranial hypertension defined by a mean ICP above 15 mmHg, was observed in 12 patients with meningitis (86%) and in 9 with encephalitis (69%). Patients with meningitis exhibited a sudden and severe intracranial hypertension. A striking difference was noted between survivors and non survivors who had a very high maximal ICP with a severe reduction of cerebral perfusion pressure (CPP). Intracranial hypertension occurred in all patients with acute primary encephalitis but in only 3/7 patients with post-infectious encephalitis. ICP monitoring seems to be important in the comatose forms of: (1) bacterial meningitis in the early period (2) herpes encephalitis (3) post-infectious encephalitis with severe status epilepticus.

Anatomical distribution of LHRH-immunoreactive neurons in the human infant hypothalamus and extrahypothalamic regions.

The morphological features and distribution of luteinizing hormone-releasing hormone (LHRH)-immunoreactive cell bodies and fibers of the hypothalamic and the neighboring mesencephalic regions were studied in the normal newborn infant by immunohistochemistry. Within the hypothalamus, numerous LHRH-immunoreactive like (IL) cell bodies were found mainly in the ventral portion of the infundibular nucleus close to the median eminence and at a lower extent in the medial preoptic area. In addition, sparse immunoreactive cell bodies were displayed in the paraventricular and medial mammillary nuclei. The mesencephalon also exhibited rare immunoreactive cell bodies in the periaqueductal gray. LHRH-IL fibers, predominantly varicose, formed a continuum from the septo-preoptico level to the mesencephalon. In the hypothalamus, the median eminence exhibited the highest LHRH innervation. LHRH-IL fibers are also observed in the lamina terminalis, the medial preoptic area, the suprachiasmatic, the supraoptic, the peri- and the paraventricular nuclei. In the last two nuclei, some fibers projected to the dorsomedial and ventromedial nuclei whereas others were in close relation with the ependyma. The mesencephalon displayed low LHRH-IL fibers, present essentially in the raphe and interpeduncular nuclei and around the ependyma. When compared with data obtained in other mammals, the present findings agree well with the general distribution and morphological features of LHRH-IL neuronal structures reported elsewhere.

[Ehlers-Danlos syndrome and pregnancy. Apropos of a case]. / Syndrome d'Ehlers-Danlos et grossesse. A propos d'un cas.

Ehlers-Danlos Syndrome is a hereditary dysplasia of connective tissue with an abnormality in collagen synthesis. The syndrome consists of increased elasticity and fragility of the skin, increased laxity of the ligaments of the joints and fragility of the blood vessels. It is rarely associated with pregnancy but when it is several problems arise which are demonstrated in our case. Our patient had a type III syndrome (hypermobility of the joints) or a type IV syndrome (echymoses, Sack-Barabas Syndrome). In the literature the following are reported: premature rupture of the membranes with premature delivery, tears, perineal haematomas and type IV ruptures of the great vessels of the uterus. The best way of delivering and anaesthetising the patient are discussed. The prognosis depends above all on the type of the disease (25% of mothers die in type IV disease). Strict rest and Pfannestiel lower segment Caesarean section operation under general anaesthetic allowed us to achieve a favorable outcome for the mother and for her child.

[The prevention of neonatal herpes encephalitis: characteristics in a tropical area]. / Prevention de l'encephalite herpetique neonatale: particularites en zone tropicale.

Neonatal herpes infection virus is rare but of poor prognosis. The only way to prevent it, is to practice a prophylactic caesarean section. In medically undeveloped countries, indication of caesarean section should be very prudent because of subsequent risks of uterine rupture. After a short recall about herpes illness, a precise and simple behaviour is proposed.

[Carotid artery occlusion in meningitis due to Streptococcus B]. / Occlusion carotidienne au cours d'une méningite à streptocoque B.

A 14 days old neonate presented an occlusion of the right internal carotid artery complicating a Streptococcus B meningitis. We discuss the mechanism of this rare complication of purulent meningitis, usually due to hemophilus influenzae.

[Intracranial pressure in comatose meningitis and encephalitis in children]. / Pression intracrânienne dans les méningites et encéphalites comateuses de l'enfant.

To determine the importance of intracranial hypertension in central nervous system acute infections, we studied intracranial pressure (ICP) in 27 patients, aged 45 days to 13 years. Fourteen had meningitis and 13 had encephalitis; all were in deep coma with a Glasgow Coma Scale 7 or less. Intracranial hypertension defined by a mean ICP above 15 mm Hg was observed in 12 patients with meningitis (86%) and in 9 with encephalitis (69%). Patients with meningitis exhibited a very early and severe intracranial hypertension. A striking difference is noted between survivors and non-survivors who had a very high maximal ICP with a severe reduction in cerebral perfusion pressure. Intracranial hypertension occurred in all patients with acute primitive encephalitis but only in 3/7 patients with post-infectious encephalitis. ICP monitoring seems to be important in the comatose forms of bacterial meningitis in the early period, herpes encephalitis and postinfectious encephalitis with severe status epilepticus.

[Haemophilus influenzae purulent pericarditis]. / Péricardite purulente à Hemophilus influenzae.

A case of hemophilus influenzae type B pericarditis in a 2-yr-old infant is reported. The authors emphasize the interest of cardiac ultrasound and pulse Doppler for diagnosis, the evaluation of hemodynamic consequences and follow-up.

[Chylous ascites in infants]. / L'ascite chyleuse du nourrisson.

In a one month-old boy apparently idiopathic chylous ascites recovered with enteral feedings of an elementary diet excluding long-chain triglycerides. The authors emphasize the usual idiopathic character of chylous ascites in very young infants; the little interest of invasive investigations at such an age when data suggesting etiology are lacking; the interest of a trial of a non-invasive medical treatment, such as the one reported.

[Stevens-Johnson syndrome associated with Mycoplasma pneumoniae infection. Apropos of a pediatric case]. / Syndrome de Stevens Johnson associé à une infection à Mycoplasma pneumoniae. A propos d'une observation pédiatrique.

A 13 years old girl presented an acute episode of fever, productive cough, purulent rhinorrhea and bilateral pulmonary crepitant rales. 36 hours later a maculopapular eruption appeared on the face, extended to all body and became bullous. Pluriorificial mucous membranes lesions were associated (conjunctivae, buccal mucosa, lips, nasal mucosa, genitalia and the perirectal area). Parenteral prednisone was administered and the extension of the eruption was stopped in a few hours. Lesions healed in about 10 days. Association with mycoplasma pneumoniae was documented by serologic studies (high titers of complement fixing antibodies and presence of IgM antibodies in immunofluorescence).

[A familial case of Rothmund-Thomson syndrome. A case in favor of the uniqueness of the syndrome. Association with osteosarcoma]. / Observation familiale de syndrome de Rothmund-Thomson. Observation en faveur de l'unicité du syndrome. Association à un ostéosarcome.

A 7 years old girl with Rothmund-Thomson syndrome is described. A detailed study of the dermatologic lesions has been performed. The parent's girl are first cousins and one of her brothers, also having the Rothmund-Thomson syndrome, died from an osteosarcoma of the tibia at the age of 11. This familial observation gives support to the uniqueness of the Rothmund-Thomson syndrome with autosomal recessive inheritance.

[Amyloidosis following juvenile chronic arthritis in a 3-year-old child]. / Amyloidose secondaire à une arthrite chronique juvénile chez un enfant de 3 ans.

A case of amyloidosis revealed by a nephrotic syndrome in a 3 years old girl with juvenile chronic arthritis is reported. The child died from this amyloidosis at the age of 4 years. The main biochemical, clinical and therapeutics aspects of this form of secondary amyloidosis (amyloidosis AA) are discussed.

[Ulcero-necrotizing enterocolitis: the role of cytomegalovirus. Apropos of a case]. / Entérocolite ulcéro-nécrosante: discussion du rôle du cytomégalovirus. A propos d'une observation.

The authors report a case of Wilson-Mikity syndrome in a preterm newborn, that was followed on the 28th day of life by a necrotizing enterocolitis with colic stenosis. The finding of cytomegalic cells on the pathologic examination of the intestinal lesions, the presence of the virus on direct examination of a tissue sample, the elevated IgG levels and the presence of IgM demonstrated a cytomegalovirus infection. The authors discuss the role of this virus in the pathogenesis of the affection, either as directly responsible for the enterocolitis or as a secondary colonization of previous lesions.

[LHRH in the human hypothalamus. Immunohistochemical mapping in normal newborn infants or in sudden death infants]. / LHRH dans l'hypothalamus humain. Etude cartographique immunohistochimique chez des nourrissons témoins ou décédés de "mort subite inexpliquée".

The topographical distribution of neurons containing LHRH has been investigated in newborn hypothalamus using the peroxidase anti-peroxidase technique. In control subjects, LHRH immunoreactive (LHRH-IR) perikarya have been mainly observed essentially in the infundibular nucleus. The preoptic region displayed a moderate density of LHRH-IR cell bodies. High LHRH innervation was observed in the anterior hypothalamus in the lamina terminalis and in the mediobasal hypothalamus in the median eminence, and in the peri- and paraventricular regions. In sudden death infant syndrome, a comparable mapping was observed, except a low density in the mediobasal peri- and paraventricular areas.

[Aberrant left pulmonary artery and tracheal abnormality]. / Artère pulmonaire gauche aberrante et malformation trachéale.

A case of left pulmonary artery sling and tracheobronchial tree malformation is reported in a 3 month-old infant. Acute respiratory presenting symptoms required mechanical ventilatory support. The malformation was suspected because of ventilatory support difficulties. Barium oesophagogram showed an anterior indentation, fiberoptic bronchoscopy and tracheobronchography showed a severe tracheal stenosis from a right bronchus lobaris superior to the carina. This was worsened by the right side compression of trachea end part, due to the abnormal left pulmonary artery as demonstrated by pulmonary angiography. A surgical left artery transposition relieved lateral compression and allowed weaning of ventilatory support. After a 27 month-follow-up, the girl's respiratory status is satisfactory.

[Fulminant herpesvirus hepatitis in a child. Apropos of 2 cases]. / L'hépatite fulminante à virus herpès chez l'enfant. A propos de 2 observations.

The authors report two cases of fulminant hepatitis in children. Coagulopathy was early demonstrated in the two patients. The illness progressed rapidly and the two children died before receiving antiviral treatment. Herpes virus was demonstrated in the hepatocytes by electron microscopy.

[Bone angiomatoses in children. Apropos of 3 personal cases]. / Les angiomatoses osseuses de l'enfant. A propos de 3 observations personnelles.

Three observations of skeletal angiomatosis are reported: one diffuse and two focal forms (Gorham's disease). The analysis of these cases and a review of the literature enhance the clinical and radiological presentation of this uncommon disease. The authors point out the interest of indirect lymphoscintigraphy and dwell on the gravity of its course, in spite of therapeutic attempts.