[Dermo-hypodermitis caused by group B Streptococcus in infants under 3 months of age: A retrospective study in two hospitals]. / Dermo-hypodermites à streptocoque du groupe B chez les nourrissons de moins de 3 mois : étude rétrospective dans deux centres hospitaliers.

BACKGROUND: Very few studies describe group B streptococcal dermo-hypodermitis in newborns. OBJECTIVES: To describe the incidence, clinical characteristics, and course of group B streptococcal dermo-hypodermitis in infants less than 3 months old. PATIENTS AND METHODS: Infants under 3 months of age, hospitalized for group B streptococcal dermo-hypodermitis at Robert Debré University Hospital, Paris, France, and at Orsay Hospital, Orsay, France, between January 2002 and August 2013, were included in a retrospective study. RESULTS: Five infants were included in this study. All the infections occurred late. Dermo-hypodermitis accounted for 7% of the overall late-onset group B streptococcal infections during the same period. Four patients were male and had a risk factor of maternal-fetal infection (prematurity/hypotrophy). Four patients had specific clinical signs of dermo-hypodermitis with septic shock features on admission. One patient had meningitis and associated parotitis. Group B Streptococcus was isolated from blood culture of all patients. Serotype III Streptococcus was identified in four cases. The duration of intravenous antibiotic therapy varied from 7 to 23 days and the total duration of antibiotic therapy was between 14 and 44 days. The progression was favorable for all the infants, with no recurrence. CONCLUSION: Dermo-hypodermitis in infants under 3 months of age is rare but could be an early indicator of group B streptococcal bacteremia and/or sepsis. Early diagnosis of this severe complication and appropriate antibiotic therapy are critical.

[Maxillary and sphenoid sinusitis complicated by acute inflammatory optic neuritis in a 12-year-old patient]. / Sinusite maxillaire et sphénoïdale compliquée de neuropathie optique inflammatoire aiguë chez un patient de 12 ans.

Acute inflammatory optic neuritis (retrobulbar optic neuritis) is an uncommon but described complication of acute sinusitis. It is due to a close anatomic relationship between the sinus and the optic nerve. Inflammatory mechanism is dominant with a possible bilateral neuritis, especially among children. In the reported case, as usual in childhood, visual acuity loss has been particularly severe, and recovery slow (it takes usually 6 weeks) but almost complete. Primary treatment is medical, with antibiotics and corticosteroids that must be prescribed for a prolonged time.

[The presentation of childhood systemic lupus erythematosus with neurological symptoms: three-case reports]. / Modes de révélation neurologiques du lupus érythémateux disséminé de l'enfant: trois observations.

INTRODUCTION: neurological symptoms occurring during childhood, especially if unusual or recurrent, should attract attention. METHODS: We report three cases of childhood lupus revealed by neurological symptoms. RESULTS: Misdiagnosis was frequent and time from symptom onset to diagnosis of lupus reached up to six months. CONCLUSION: After careful history taking and physical examination, complementary tests should include erythrocyte sedimentation rate, blood count, platelets, and in some cases an immunological study with antinuclear antibodies.

[Loss of consciousness in a child due to loperamide]. / Perte de connaissance chez une jeune enfant secondaire à la prise de lopéramide.

Loperamide is an antidiarrheal peripheral opiate agonist, with rare neurological secondary effects. We report the case of a 26-month-old child who had impaired consciousness under treatment by loperamide, and was treated successfully with naloxone. Limitations of the use of loperamide in young children are underlined. Naloxone may be used in case of impaired consciousness, for a diagnostic as well as therapeutic purpose.

[Unusual good prognosis for X-linked myotubular myopathy]. / Forme inhabituelle de bon pronostic de la myopathie myotubulaire liée à l'X.

Recessive X-linked myotubular myopathy has recently been shown to be linked to the mutation of a gene located in the Xq28 region. Evolution is used to be considered as fatal but mild forms or forms with a better prognosis have been recorded since. We report a case in a patient, whose parents were warned of fatal outcome once the diagnosis was made during the neonatal period. The reaction of the parents was to avoid any relationship with medical care. Ten years later the patient was seen in relatively good health thus proving that the evolution was more favourable than anticipated.

[Biopercular polymicrogyria associated with congenital ophthalmoplegia caused by nuclear lesion of the common oculomotor nerve]. / Polymicrogyrie bioperculaire associée à une ophtalmoplégie congénitale par atteinte du noyau du nerf moteur oculaire commun.

Developmental pseudobulbar palsies seem to be different from the adult form described by Foix, Chavany and Marie. They usually include a major speech delay and severe epileptic seizures. In one clinicopathological case, neuroradiological imaging showed a macrogyric aspect of both rolandic operculi and unilateral destruction of pes pedunculari. Microscopic examination showed a four-layered polymicrogyria involving the first temporal gyrus and in the brainstem a selective destruction of the left oculomotor nucleus. Thus, the macrogyric aspect could be related to post migratory disorder occurring late in the cortical development. The brain stem lesion, destroying unilaterally the third cranial nerve nucleus gives a good example of the complex somatotopia of this oculomotor nucleus.

[Long term course of catecholaminergic polymorphic ventricular tachycardia in children. Apropos of 20 cases with an 8 year-follow-up]. / Devenir à long terme des tachycardies ventriculaires polymorphes catécholergiques de l'enfant. A propos de 20 cas suivis pendant 8 ans.

BACKGROUND: Primary ventricular arrhythmias are rarely seen in children. Some of them have a poor prognosis; they should be diagnosed because adequate treatment can prevent sudden death. POPULATION AND METHODS: Twenty children (11 male, nine female), aged 3 to 16 years (mean: 7.7 +/- 4), with apparently normal hearts and normal QTc intervals were referred for stress or emotion-induced syncope. Primary ventricular arrhythmia, consisting of isolated polymorphic ventricular extrasystoles followed by salvos eventually degenerating into ventricular fibrillation, was reproducibly induced by physical exertion. The syncopal events and "torsades de pointe" disappeared with beta-blocking therapy. A total of four syncopal events and two sudden deaths occurred during a mean follow-up of 8 years, probably due to discontinuation of treatment. DISCUSSION: Fifty-four-cases of stress-induced severe polymorphous ventricular arrhythmia have been reported in the literature. There were four sudden deaths in 37 patients on beta-blocking therapy, and ten sudden deaths in 21 untreated patients. CONCLUSION: Clinically close to the congenital long QT syndrome, this primary ventricular arrhythmia must be looked for in cases of stress or emotion-induced syncope. The diagnosis relies on Holter monitoring and a stress test. Life-long beta blocker therapy is required.

[Herpes encephalitis in newborn infants. Retrospective study of 12 cases]. / Encéphalite herpétique du nouveau-né. Etude rétrospective de douze observations.

BACKGROUND: Herpes simplex virus (HSV) may cause severe disease in neonates with high mortality and devastating sequelae. Adenine arabinoside (ara-A) and acyclovir can be effective in treating the neonatal disseminated disease, but optimal doses of the drugs are still debated. PATIENTS: The files of 12 neonates treated for HSV infection between May 1983 and April 1989 in 9 departments of pediatrics were studied. The following data were analysed: age at initial signs, clinical manifestations, CSF abnormalities, viral and immunological studies, imaging techniques used to detect brain damage, doses and duration of treatment. RESULTS: The mean age at the first infectious signs was 12.3 +/- 9.3 days and neurological manifestations were first seen at 18.3 +/- 7.7 days. Acyclovir was given intravenously for 2 to 4 weeks at doses of 30 mg/kg/24 hr (5 patients) or 60 mg/kg/24 hr (7 patients) at an average of 7.7 days after the first clinical manifestations. The initial dose of 30 mg/kg/24 hr was increased to 50 or 60 mg/kg/24 hr in 3 patients. Five patients died during treatment, and 5 had severe sequelae; follow-up for the 2 remaining patients was not possible. There was no correlation between prognosis and the dose of acyclovir, which was well tolerated in all patients. CONCLUSIONS: The best results are obtained when treatment is started early. This retrospective study failed to show any dose-dependent difference in the efficacy of acyclovir. A prospective study with different doses would be useful.

[Therapy of severe asthma crisis in children. Therapeutic course. A propos of 40 cases]. / Crise d'asthme sévère de l'enfant. Evolution thérapeutique. A propos de 40 observations.

Forty children with severe asthma were admitted to an intensive care unit from 1970 to 1981. Criteria of severity were alteration of consciousness, circulatory failure, hypercarbia greater than 7 KPa and paradoxical pulse greater than 35 mmHg persisting in spite of correct treatment. Some therapeutic measures (oxygen, corticosteroids, antibiotics, hydratation) were not modified during this period. On the contrary, bronchodilators, used at a higher dose since June 1980, were very effective on bronchospasm without side effects, making controlled ventilation unnecessary. These results seem to be very encouraging but have to be confirmed.

[Retrospective study of 56 congenital diaphragmatic hernias. Prognostic factors, therapeutic deductions]. / Etude rétrospective de 56 hernies diaphragmatiques à révélation néo-natale. Facteurs pronostiques, déductions thérapeutiques.

Fifty-six neonates were treated for congenital diaphragmatic hernia (48 left and 8 right) in our intensive care unit from 1972 to 1980. Because of the high mortality, we studied the factors which could predict the outcome. Early onset of symptoms (before the 2nd hour of life) and low post-ductal shunt (PAO2/FiO2 less then 15) after the 3rd postoperative hour appear to be signs of poor prognosis. Our present attitude is early surgical procedure, controlled ventilation with a FiO2 1 with Pancuronium, prevention of pneumothorax and different use of pulmonary arterial vasodilatators.

[Primary immunologic deficiencies and cancer. 5 anatomo-clinical case reports]. / Déficits immunitaires primitifs et cancers. Cinq observations anatomo-cliniques.

Five patients with primary immunodeficiency and cancer are presented. Two children with ataxia-telangiectasia developed acute lymphoblastic leukemia and malignant lymphoma of B-like origin with chromosome damage and unusual prevalence of antibodies to E.B.V. early antigen. A bone sarcoma occurred in a patient with common variable hypogammaglobulinemia. At least two infants who died with severe combined immunodeficiency had at autopsy congenital myelomonocytic leukemia and malignant lymphoma. These cases indicate the high risk for development of cancer in patients with primary abnormalities of the immune system and suggest the heterogeneity and complexity of pathogenic mechanisms.

[Acute cerebral edema complicating therapy for diabetic ketoacidosis in children ]. / Oedème cérébral aigu compliquant le traitement de l'acido-cétose diabétique de l'enfant.

Cerebral edema complicating treatment of diabetic ketoacidosis does not seen to be very rare. This neurologic complication, which was observed in 3 cases, presents similarly in all patients, occurs mainly in children and adolescents and is usually lethal. Its mechanism has not been clearly explained yet. Various etiologic factors have been considered; sodium depletion, rapid infusion of hypotonic solutions and rapid fall of plasma glucose level might have a deleterious effect. On the basis of these data, some preventive therapeutic measures are proposed.

[Severe blood coagulation disorder as the first sign of a peritoneal hemangio-endothelioma: a new therapeutic approach (author's transl)]. / Perturbations sévères de l'hémostase révélant abord thérapeutique.

The authors describe a 3 1/2 month old infant with hemangio-endothelioma and a severe blood coagulation disorder. The tumor was inoperable and the severe blood coagulation disorder posed considerable therapeutic problem. After treatment with massive amounts of blood clotting factors, an antifibrinolytic drug and radiotherapy, the child's condition improved.