[Obstructive sleep apnea-hypopnea syndrome in children: Clinical diagnosis]. / Diagnostic clinique du syndrome d'apnées obstructives du sommeil de l'enfant.

The French Society of Research and Sleep Medicine (SFRMS) organized a meeting on obstructive sleep apnea syndrome (OSAS) in children. A multidisciplinary group of specialists (pulmonologist, ENT surgeons, pediatricians, orofacial myofunctional therapists, neurophysiologists, and sleep specialists) reached a consensus on the value of isolated or clustered clinical symptoms and of questionnaires completed by parents in the clinical diagnosis and in assessing the severity of OSAS. Are clinical history with validated questionnaires and a rigorous physical examination sufficient to suspect OSAS, to appreciate its severity, and finally to confirm the diagnosis? Usually, a sleep recording of respiratory parameters remains mandatory for the diagnosis of OSAS to be made. However, clinical symptoms are very useful for estimating the probability of the diagnosis and the severity of the disease, and therefore for classifying which children will benefit form polysomnography and for proposing an adapted follow-up after OSAS therapy. Even if they are not able to ascertain the diagnosis of OSAS in children, clinical history, questionnaires, and physical examination are very important. Finally, we propose a classification of the indications for polysomnography in children suspected of having OSAS.

Circulating Antibodies to IDO/THO Pathway Metabolites in Alzheimer's Disease.

In Alzheimer's disease, indoleamine 2,3-dioxygenase and tryptophan hydroxylase are known to induce an overproduction of neurotoxic compounds, such as quinolinic acid and 3-hydroxykynurenine from the former, and 5-hydroxytryptophol and 5-methoxytryptophol from the latter. Other compounds, such as kynurenic acid, serotonin, and melatonin are produced via the same pathways. An improved ELISA method identified circulating antibodies directed against these compounds, linked to proteins, as previously described for other chronic diseases. This describes how only the A isotype of circulating immunoglobulins recognized a pattern of conjugated tryptophan metabolites in the sera of Alzheimer patients. These data indirectly confirmed the involvement of tryptophan derivatives in the pathogenic processes of Alzheimer's disease. Further studies are required to evaluate the relevance of these antibody patterns in monitoring this disease.

Outcome of 27 patients with Hurler's syndrome transplanted from either related or unrelated haematopoietic stem cell sources.

Over the last 15 years, we have performed a total of 30 haematopoietic stem cell transplants on 27 children suffering from Hurler's syndrome. These children were of median age 11 months at the time of diagnosis and 25 months at the time of transplantation. The phenotype was severe in 21 cases (78%). The donor was familial in 13 cases: nine genotypically identical, one phenotypically identical father and three HLA-mismatched donors. Unrelated donors were selected in 17 cases: four phenotypically identical and 13 with 1-4 HLA mismatches. The conditioning regimen generally consisted of busulphan 600 mg/m(2) plus cyclophosphamide (Endoxan) 260 mg/kg and cyclosporin with methotrexate for GvHD prophylaxis. Rabbit anti-thymocyte globulin (Thymoglobuline) was given for all unrelated or familial mismatched transplantations. The median nucleated cell dose infused was 6.00 x 10(8) TNC/kg. No bone marrow (apart from one) was T cell depleted. For first transplants, engraftment was observed in 23/27 patients (pts) (85%). Primary graft failure was observed in 4/27 patients (16%), two were retransplanted from an unrelated donor, one with success. Four patients have died. The primary cause of death was infection in three cases (TRM : 11%) and disease progression in one case, after primary graft failure. Of the 23 living patients, two have disease progression after graft failure and 21 (78%) have functional grafts with a favourable long-term outcome after a median follow-up of 4.7 years, having either full or mixed chimaerism. Among surviving patients with functional grafts, 13 (62%) were transplanted from unrelated donors of whom 10 (77 %) had HLA disparities. There was a remarkably low incidence of GvHD. In our experience, haematopoietic stem cell transplantation using an HLA-matched familial donor or an HLA-matched or -mismatched unrelated donor without T cell depletion or irradiation can achieve a favourable outcome in Hurler's syndrome, with improved cognitive function, but with a limited effect on the corneas and skeleton.

Reactions of 1,3-cyclohexadiene with singlet oxygen. A theoretical study.

A thorough study of the reaction of singlet oxygen with 1,3-cyclohexadiene has been made at the B3LYP/6-31G(d) and CASPT2(12e,10o) levels. The initial addition reaction follows a stepwise diradical pathway to form cyclohexadiene endoperoxide with an activation barrier of 6.5 kcal/mol (standard level = CASPT2(12e,10o)/6-31G(d); geometries and zero-point corrections at B3LYP/6-31G(d)), which is consistent with an experimental value of 5.5 kcal/mol. However, as the enthalpy of the transition structure for the second step is lower than the diradical intermediate, the reaction might also be viewed as a nonsynchronous concerted reaction. In fact, the concertedness of the reaction is temperature dependent since entropy differences create a free energy barrier for the second step of 1.8 kcal/mol at 298 K. There are two ene reactions; one is a concerted mechanism (DeltaH(double dagger) = 8.8 kcal/mol) to 1-hydroperoxy-2,5-cyclohexadiene (5), while the other, which forms 1-hydroperoxy-2,4-cyclohexadiene (18), passes through the same diradical intermediate (9) as found on the pathway to endoperoxide. The major pathway from the endoperoxide is O-O bond cleavage (22.0 kcal/mol barrier) to form a 1,4-diradical (25), which is 13.9 kcal/mol less stable than the endoperoxide. From the diradical, two low-energy pathways exist, one to epoxyketone (29) and the other to the diepoxide (27), where both products are known to be formed experimentally with a product ratio sensitive to the nature of substitutents. A significantly higher activation barrier leads to C-C bond cleavage and direct formation of maleic aldehyde plus ethylene.

[Intracranial subdural hematoma after obstetric dural puncture]. / Hématome sous-dural intracrânien après brèche dure-mérienne en obstétrique.

The authors report a case of subdural haematoma (HSD), which occurred following epidural analgesia for labour, complicated by post dural puncture headache (PDPH). A 26-year-old woman displayed a typical PDPH following epidural anaesthesia. On the sixth day, she was given a blood patch (BP), which was partially efficacious. Because of worsening of the headache, of disappearance of the postural characteristics, and of vomiting without focal neurological signs on the 9th day, a CT-scan was done. The CT-scan showed a small hemispheric subdural haematoma. The recovery was complete with only medical treatment. HSD is a rare serious complication of dural puncture. When the characteristics of PDPH change, HSD should be evoked even without focal neurological signs. An early diagnosis and the small size of the haematoma may allow HSD to be treated medically and avoid surgical evacuation.

[Hearing disorders screening in neonates at risk]. / Dépistage des troubles auditifs chez des nouveau-nés à risque.

The present report concerns a three year, eight month hearing screening in 1 531 high-risk neonates by means of two successive transient evoked otoacoustic emissions (TEOAE) recordings followed, cin cases of suspected hearing loss, by brainstem auditory evoked potential (BAEP) recording and otolaryngology (ORL) consultation. After TEOAE1 and 2 and BAEP testing, 1 361 infants (88.9%) were declared normal, and 170 (11%) suspected of hearing loss. Of these 170, 58 showed bilateral and 26 unilateral impairment. Definite hearing loss on ORL consultation was diagnosed in 14 infants (0.9% of the screened population as a whole); 22 are still followed, while 86 (5.6%) failed to consult for diagnosis. The mean age on diagnosis of definite hearing loss on ORL consultation was 9.9 +/- 4.9 (range 4-20) months. Several auditory function risk factors have been proved to be more frequent in deaf than in normal children. Our results show that early hearing loss screening in at-risk neonates needs to be pursued.

Auditory screening in high-risk pre-term and full-term neonates using transient evoked otoacoustic emissions and brainstem auditory evoked potentials.

The present report concerns a 3 year, 8 month hearing screening in 1531 high-risk neonates by means of two successive transient evoked otoacoustic emission (TEOAE) recordings followed, in cases of suspected hearing loss, by brainstem auditory evoked potential (BAEP) recording and otolaryngology (ORL) consultation. After TEOAE 1 and 2 and BAEP testing, 1361 infants (88.9%) were declared normal, and 170 (11.1%) suspected of hearing loss. Of these 170, 58 showed bilateral and 26 unilateral impairment. Definite hearing loss on ORL consultation was diagnosed in 14 infants (0.9% of the screened population as a whole); 22 are still being followed, while 86 (5.6%) failed to consult for diagnosis. The mean age on diagnosis of definite hearing loss was 9.9 +/- 4.9 (range 4-20) months. Several auditory function risk factors proved more frequent in deaf than in normal children. Our results show that early hearing loss screening in at-risk neonates needs to be pursued.

Otoacoustic emissions and brainstem auditory evoked potentials in children with neurological afflictions.

Findings are reported for evoked otoacoustic emissions (EOAEs) recorded from 22 children with neurological afflictions, whose brainstem auditory evoked potentials (BAEPs) were pathological on at least one side (41 ears explored). Our results confirmed that EOAEs are always present in children and infants having normal BAEPs. Absence of EOAE (n = 22) was almost always related to middle ear or cochlear damage with BAEPs indicating diagnoses, respectively, of transmission damage (n = 7) or endocochlear damage (n = 16). Conversely, for BAEP diagnoses of retrocochlear damage (n = 12), EOAEs were always present. EOAEs associated with BAEPs, therefore, appear to offer a well-adapted technique for precise etiological diagnosis of childhood hearing loss. When no wave is identifiable by BAEP recording, EOAE presence indicates retrocochlear damage.

Brainstem auditory evoked potentials following meningitis in children.

The report concerns findings for brainstem auditory evoked potentials (BAEPs) recorded in 116 children, aged between a few days and 7 years, having suffered from bacterial meningitis. 26% of cases occurred between birth and 6 months, 55% between 6 months and 2 years, and 19% after 2 years of age. Hemophilus was the most common bacteria (49%), followed by Pneumococcus (22%) and Meningococcus (15%). Neurological complications were found in 30% of the meningitis cases and accounted for 85% of all complications found. 29% of BAEPs were abnormal, of which 47% revealed transmission, 32% endocochlear and 21% retrocochlear impairment. Transmission impairment mainly occurred before the age of 2 years (88%), most frequently in meningococcus meningitis cases (44%), and independently of neurological complications. Retrocochlear impairment was found in association with neurological complications in 71% of cases. Endocochlear BAEP damage was found in 9.5% of cases, half of which were bilateral and total, representing cophosis: it was found at all ages, and without any particular associated neurological complication. Hemophilus was the commonest bacterial agent in endocochlear cases overall, with Pneumococcus underlying 50% of cophosis cases. The study shows BAEP recording in association with a clinical ear examination is useful following childhood bacterial meningitis, screening for definitive endocochlear and deafness, distinguishing total from partial hearing-loss and indicating suitable treatment.

[Mastoiditis: still a common disease]. / Les mastoïdites: toujours d'actualité.

Mastoiditis remains relatively frequent in children. The main clinical forms are recalled. Surgical treatment is mandatory.

[Montgomery's tube in laryngotracheal stenoses in children (11 cases)]. / Le tube de Montgomery dans les sténoses laryngo-trachéales de l'enfant (11 observations).

Because of the fragility of the laryngotracheal tree in children, and the serious consequences on function of any chondritic lesion or even mucosal edema, overall endoscopic treatments combined with prosthesis insertion are preferred to any surgical procedure. Eleven cases of laryngotracheal stenosis are reported where treatment involved use of Montgomery's calibrated tube.