Deep sclerectomy combined with trabeculectomy in pediatric glaucoma

Résumé : Ce travail porte sur l'étude rétrospective d'une série de jeunes patients opérés de glaucomes pédiatriques. Le but est d'évaluer le résultat au long cours d'une intervention chirurgicale combinant une sclérectomie profonde et une trabéculectomie (sclérectomie profonde pénétrante). Durant la période de mars 1997 à octobre 2006, 28 patients on été suivis pour évaluer le résultat de cette chirurgie effectuées sur 35 yeux. Un examen ophtalmologique complet a été pratiqué avant la chirurgie, 1 et 7 jours, puis 1, 2, 3, 4, 6, 9, 12 mois, enfin tous les 6 mois après l'opération. Les critères d'évaluation du résultat postopératoire sont : les changements de pression intraoculaire, le traitement antiglaucomateux adjuvant, le taux de complication, le nombre de reprises chirurgicales,- l'erreur de réfraction, la meilleure acuité visuelle corrigée, l'état et le diamètre de la cornée. L'âge moyen est de 3.6 ± 4.5 ans et le suivi moyen de 3.6 ± 2.9 ans. La pression intraoculaire préopératoire de 31.9 ± 11.5 mmHg baisse de 58.3% (p<0.005) à la fin du suivi. Sur les 14 patients dont l'acuité visuelle a pu être mesurée, 8 (57.1 %) ont une acuité égale ou supérieure à 5/10e, 3 (21.4%) une acuité de 2/10e après intervention. Le taux de succès cumulatif complet à 9 ans est de 52.3%, le succès relatif 70.6%. Les complications menaçant la vision (8.6%) ont été plus fréquentes dans les cas de glaucome réfractaire. Pour conclure la sclérectomie profonde combinée à une trabéculectomie est une technique chirurgicale développée afin de contrôler la pression intraoculaire dans les cas de glaucomes congénitaux, juvéniles et secondaires. Les résultats intermédiaires sont encourageants et prometteurs. Les cas préalablement opérés avant cette nouvelle technique ont cependant un pronostic moins favorable. Le nombre de complications menaçant la vision est essentiellement lié à la sévérité du glaucome et au nombre d'interventions préalables. Abstract : Purpose : To evaluate the outcomes of combined deep sclerectomy and trabeculectomy (penetrating deep sclerectomy) in pediatric glaucoma. Design : Retrospective, non-consecutive, non-comparative, interventional case series. Participants : Children suffering from pediatric glaucoma who underwent surgery between March 1997 and October 2006 were included in this study. Methods : A primary combined deep sclerectomy and trabeculectomy was performed in 35 eyes of 28 patients. Complete examinations were performed before surgery, postoperatively at 1 and 7 days, at 1, 2, 3, 4, 6, 9, 12 months and then every 6 months after surgery. Main Outcome Measures : Surgical outcome was assessed in terms of intraocular pressure (IOP) change, additional glaucoma medication, complication rate, need for surgical revision, as well as refractive errors, best corrected visual acuity (BCVA), and corneal clarity and diameters. Results : The mean age before surgery was 3.6 ± 4.5 years, and the mean follow-up was 3.5 ± 2.9 years. The mean preoperative IOP was 31.9 ± 11.5 mmHg. At the end of follow-up, the mean IOP decreased by 58.3% (p<0.005), and from 14 patients with available BCVA 8 patients (57.1 %) achieved. 0.5 (20/40) or better, 3 (21.4%) 0.2 (20/100), and 2 (14.3%) 0.1 (20/200) in their better eye. The mean refractive error (spherical equivalent) at final follow-up visits was +0.83 ± 5.4. Six patients (43%) were affected by myopia. The complete and qualified success rates, based on a cumulative survival curve, after- 9 years were 52.3% and 70.6%, respectively (p<0.05). Sight threatening complications were more common (8.6%) in refractory glaucomas. Conclusions : Combined deep sclerectomy and trabeculectomy is a surgical technique developed to control IOP in congenital, secondary and juvenile glaucomas. The intermediate results are satisfactory and promising. Previous classic glaucoma surgeries performed before this new technique had less favourable results. The number of sight threatening complications is related to the severity of glaucoma and number of previous surgeries.

Three cases of neonatal herpes simplex virus infection presenting as fulminant hepatitis.

We report three cases of neonatal herpes simplex virus (HSV) infection presenting as fulminant hepatitis. None of the patients had clear risk factors for HSV infection and they all died. Antiviral treatment for HSV is currently available but must be administered early in the course of the disease before irreversible liver tissue damage is present. Since the diagnosis may be difficult to establish, we wish to draw the attention of clinicians to the presentation of neonatal HSV infection and suggest that in such cases viral cultures, including culture of liver tissue, should be obtained early and antiviral treatment administered while awaiting the culture results.

Hindsight judgement on ambiguous episodes of suspected infection in critically ill children: poor consensus amongst experts?

Few episodes of suspected infection observed in paediatric intensive care are classifiable without ambiguity by a priori defined criteria. Most require additional expert judgement. Recently, we observed a high variability in antibiotic prescription rates, not explained by the patients' clinical data or underlying diseases. We hypothesised that the disagreement of experts in adjudication of episodes of suspected infection could be one of the potential causes for this variability. During a 5-month period, we included all patients of a 19-bed multidisciplinary, tertiary, neonatal and paediatric intensive care unit, in whom infection was clinically suspected and antibiotics were prescribed ( n=183). Three experts (two senior ICU physicians and a specialist in infectious diseases) were provided with all patient data, laboratory and microbiological findings. All experts classified episodes according to a priori defined criteria into: proven sepsis, probable sepsis (negative cultures), localised infection and no infection. Episodes of proven viral infection and incomplete data sets were excluded. Of the remaining 167 episodes, 48 were classifiable by a priori criteria ( n=28 proven sepsis, n= 20 no infection). The three experts only achieved limited agreement beyond chance in the remaining 119 episodes (kappa = 0.32, and kappa = 0.19 amongst the ICU physicians). The kappa is a measure of the degree of agreement beyond what would be expected by chance alone, with 0 indicating the chance result and 1 indicating perfect agreement. CONCLUSION: agreement of specialists in hindsight adjudication of episodes of suspected infection is of questionable reliability.

The A-kinase anchoring protein (AKAP)-Lbc-signaling complex mediates alpha1 adrenergic receptor-induced cardiomyocyte hypertrophy.

In response to various pathological stresses, the heart undergoes a pathological remodeling process that is associated with cardiomyocyte hypertrophy. Because cardiac hypertrophy can progress to heart failure, a major cause of lethality worldwide, the intracellular signaling pathways that control cardiomyocyte growth have been the subject of intensive investigation. It has been known for more than a decade that the small molecular weight GTPase RhoA is involved in the signaling pathways leading to cardiomyocyte hypertrophy. Although some of the hypertrophic pathways activated by RhoA have now been identified, the identity of the exchange factors that modulate its activity in cardiomyocytes is currently unknown. In this study, we show that AKAP-Lbc, an A-kinase anchoring protein (AKAP) with an intrinsic Rho-specific guanine nucleotide exchange factor activity, is critical for activating RhoA and transducing hypertrophic signals downstream of alpha1-adrenergic receptors (ARs). In particular, our results indicate that suppression of AKAP-Lbc expression by infecting rat neonatal ventricular cardiomyocytes with lentiviruses encoding AKAP-Lbc-specific short hairpin RNAs strongly reduces both alpha1-AR-mediated RhoA activation and hypertrophic responses. Interestingly, alpha1-ARs promote AKAP-Lbc activation via a pathway that requires the alpha subunit of the heterotrimeric G protein G12. These findings identify AKAP-Lbc as the first Rho-guanine nucleotide exchange factor (GEF) involved in the signaling pathways leading to cardiomyocytes hypertrophy.

Troubles du sommeil chez des patients présentant une insuffisance rénale chronique [Sleep disorders in patients with chronic renal insufficiency].

Sleep disorders, especially insomnia, daytime sleepiness, sleep apnea syndrome and restless legs syndrome are very frequently encountered in patients with chronic renal failure whether or not they undergo renal replacement therapy. The causes of sleep disorders are multifactorial and not only linked to the renal disease itself, but also to its treatment and its associated psychosocial factors. This article discusses the prevalence and physiopathology of the most frequently encountered sleep disorders in chronic renal failure patients, and highlights the actually available therapeutic options.

The circadian rhythm of the perinatal mortality rate in Switzerland.

The authors examine the relation between the perinatal mortality rate (PMR), birth weight in four categories, and hour of birth throughout the week in Switzerland, using data on 672,013 births and 5,764 perinatal deaths recorded between 1979 and 1987. From Monday to Friday, the PMR follows a circadian rhythm with a regular increase from early morning to evening, with a peak for babies born between 7 and 8 p.m. This pattern of variation has two main components: The circadian rhythms for the proportion of births in the four weight categories and the PMR circadian rhythm for babies weighing more than 2.5 kg. According to a cosinor model, which describes about 40% of the total variation in the PMR, the most important determinants are changes in the proportions of births: Low birth weight increases toward the afternoon and night. Mechanisms underlying the weight-specific timing of birth are discussed, including time selection of birth according to obstetric risks, the direct effect of neonatal and obstetric care, and chronobiologic behavior.

Les enjeux de l'allaitement maternel pour les nouveau-nés hospitalisés [Issues surrounding the breastfeeding of hospitalized newborns].

The nutritional and physiological qualities of breast milk make it the best food for newborns, favouring their wellbeing and growth. The implementation of a programme encouraging the breastfeeding of hospitalised newborns in care departments requires specific methods of organisation, as well as constant and adapted support from health professionals.

Estradiol and progesterone strongly inhibit the innate immune response of mononuclear cells in newborns.

Newborns are particularly susceptible to bacterial infections due to qualitative and quantitative deficiencies of the neonatal innate immune system. However, the mechanisms underlying these deficiencies are poorly understood. Given that fetuses are exposed to high concentrations of estradiol and progesterone during gestation and at time of delivery, we analyzed the effects of these hormones on the response of neonatal innate immune cells to endotoxin, bacterial lipopeptide, and Escherichia coli and group B Streptococcus, the two most common causes of early-onset neonatal sepsis. Here we show that at concentrations present in umbilical cord blood, estradiol and progesterone are as powerful as hydrocortisone for inhibition of cytokine production by cord blood mononuclear cells (CBMCs) and newborn monocytes. Interestingly, CBMCs and newborn monocytes are more sensitive to the effects of estradiol and progesterone than adult peripheral blood mononuclear cells and monocytes. This increased sensitivity is associated with higher expression levels of estrogen and membrane progesterone receptors but is independent of a downregulation of Toll-like receptor 2 (TLR2), TLR4, and myeloid differentiation primary response gene 88 in newborn cells. Estradiol and progesterone mediate their anti-inflammatory activity through inhibition of the NF-κB pathway but not the mitogen-activated protein kinase pathway in CBMCs. Altogether, these results suggest that elevated umbilical cord blood concentrations of estradiol and progesterone acting on mononuclear cells expressing high levels of steroid receptors contribute to impair innate immune responses in newborns. Therefore, intrauterine exposure to estradiol and progesterone may participate in increasing susceptibility to infection during the neonatal period.

Chylothorax in children: guidelines for diagnosis and management.

STUDY OBJECTIVE: To establish guidelines for the diagnosis and management of chylothorax in children. DESIGN: Retrospective study. PATIENTS: Fifty-one patients with a diagnosis of chylothorax. Twelve patients were excluded because of incomplete data or incorrect diagnosis. The following parameters were analyzed: triglyceride level, total cell number, and lymphocyte percentage; amount of pleural effusion on day of diagnosis, day 5, and day 14; and total time of pleural effusion. Prospectively, the same parameters were analyzed in a control group of 10 patients with pleural drainage. INTERVENTION: Patients with chylothorax were treated primarily with fat-free oral nutrition; if chyle did not stop, total parenteral nutrition with total enteric rest was started. If conservative therapy was not successful, pleurodesis was performed. RESULTS: In children with chylothorax triglyceride, triglyceride content ranged from 0.56 to 26.6 mmol/L; all values except one were > 1.1 mmol/L. In 36 of 39 patients (92%), the cell count was > 1,000 cells/microL. In 33 of 39 patients (85%), lymphocytes were > 90%. In patients without chylothorax triglyceride, triglyceride levels ranged from 0.1 to 0.71 mmol/L (median, 0.38 mmol/L) and cell count was from 20 to 1400 cells/microL (median, 322 cells/microL), with a maximum of 60% lymphocytes. With fat-free nutrition, chyle disappeared in 29 of 39 patients. Five patients died, and five required pleurodesis. CONCLUSIONS: Pleural effusion in children is chyle when it contains > 1.1 mmol/L triglycerides (with oral fat intake) and has a total cell count > or 1,000 cells/microL, with a lymphocyte fraction > 80%. Chylous effusions usually last long; however, after 6 weeks, the majority of the effusions (29 of 39 patients) had ceased. Late surgical interventions reduce the number of thoracotomies substantially, but can lead to very long hospitalization times. Early surgical interventions (after < 3 weeks) lead to a high number of thoracotomies, but certainly reduce hospitalization time.

Maternal side-effects after multiple courses of antenatal corticosteroids (MACS): the three-month follow-up of women in the randomized controlled trial of MACS for preterm birth study.

OBJECTIVE: A single course of antenatal corticosteroids (ACS) is associated with a reduction in respiratory distress syndrome and neonatal death. Multiple Courses of Antenatal Corticosteroids Study (MACS), a study involving 1858 women, was a multicentre randomized placebo-controlled trial of multiple courses of ACS, given every 14 days until 33+6 weeks or birth, whichever came first. The primary outcome of the study, a composite of neonatal mortality and morbidity, was similar for the multiple ACS and placebo groups (12.9% vs. 12.5%), but infants exposed to multiple courses of ACS weighed less, were shorter, and had smaller head circumferences. Thus for women who remain at increased risk of preterm birth, multiple courses of ACS (every 14 days) are not recommended. Chronic use of corticosteroids is associated with numerous side effects including weight gain and depression. The aim of this postpartum assessment was to ascertain if multiple courses of ACS were associated with maternal side effects. METHODS: Three months postpartum, women who participated in MACS were asked to complete a structured questionnaire that asked about maternal side effects of corticosteroid use during MACS and included the Edinburgh Postnatal Depression Scale. Women were also asked to evaluate their study participation. RESULTS: Of the 1858 women randomized, 1712 (92.1%) completed the postpartum questionnaire. There were no significant differences in the risk of maternal side effects between the two groups. Large numbers of women met the criteria for postpartum depression (14.1% in the ACS vs. 16.0% in the placebo group). Most women (94.1%) responded that they would participate in the trial again. CONCLUSION: In pregnancy, corticosteroids are given to women for fetal lung maturation and for the treatment of various maternal diseases. In this international multicentre randomized controlled trial, multiple courses of ACS (every 14 days) were not associated with maternal side effects, and the majority of women responded that they would participate in such a study again.

Exploring models for the length of stay distribution.

Diagnosis Related Groups (DRG) are frequently used to standardize the comparison of consumption variables, such as length of stay (LOS). In order to be reliable, this comparison must control for the presence of outliers, i.e. values far removed from the pattern set by the majority of the data. Indeed, outliers can distort the usual statistical summaries, such as means and variances. A common practice is to trim LOS values according to various empirical rules, but there is little theoretical support for choosing between alternative procedures. This pilot study explores the possibility of describing LOS distributions with parametric models which provide the necessary framework for the use of robust methods.

Iowa EHDI News, Spring 2006

The purpose of the newsletter is to communicate with parents and professionals about newborn hearing screening in Iowa. We will share information about: Hearing screenings Intervention Resources available for parents and professionals “Best practices” by hospitals, AEAs (Area Education Agencies), or private practive audiology offices System goals Family stories Highlights from the EHDI Advisory Committee Updates on Iowa’s EHDI program It is important to point out that we are a diverse team of individuals working together to ensure that all newborns and toddlers with hearing loss are identified as early as possible and provided with timely and appropriate audiological, educational and medical intervention. Each newsletter will introduce you to various team members of the EHDI system in Iowa.

Hypernatriämie--wie weiter [Hypernatremia]

Hypernatremia is defined as an elevated serum sodium concentration. Usually hypernatremia is caused by a relative water deficit occurring with decreased thirst sensation and/or reduced water intake. In rare cases hypernatremia may be caused by excessive sodium intake. Severe hypernatremia can be dangerous and can lead to significant morbidity and mortality. Dangerous hypernatremia can occur in the newborn. Drugs that influence thirst sensation are the main cause of hypernatremia in adults and elderly patients. Hospitalization itself might be a risk factor for developing hypernatremia. Therapy consists in eliminating the causes of hypernatremia and in the specific management of body volume and electrolytes.

Protéinurie chez l'enfant: approche pratique [Proteinuria in children: practical approach].

Significant proteinuria is not an unfinding in children. Its causes are variable. When detected by dipstick examination of urine, the proteinuria must be assessed quantitatively by measuring the urinary protein/creatinine ratio in a spot sample. Orthostatic proteinuria is the most common cause of intermittent proteinuria. Persistent glomerular or tubular proteinuria are the consequences of various glomerulopathies or tubulopathies, the prognosis of which is variable. Whether glomerular or tubular, persistent proteinuria must be fully investigated, including by renal biopsy.

Toll-like receptor 2 (TLR2) polymorphisms are associated with reversal reaction in leprosy.

BACKGROUND: Leprosy is characterized by a spectrum of clinical manifestations that depend on the type of immune response against the pathogen. Patients may undergo immunological changes known as "reactional states" (reversal reaction and erythema nodosum leprosum) that result in major clinical deterioration. The goal of the present study was to assess the effect of Toll-like receptor 2 (TLR2) polymorphisms on susceptibility to and clinical presentation of leprosy. METHODS: Three polymorphisms in TLR2 (597C--&gt;T, 1350T--&gt;C, and a microsatellite marker) were analyzed in 431 Ethiopian patients with leprosy and 187 control subjects. The polymorphism-associated risk of developing leprosy, lepromatous (vs. tuberculoid) leprosy, and leprosy reactions was assessed by multivariate logistic regression models. RESULTS: The microsatellite and the 597C--&gt;T polymorphisms both influenced susceptibility to reversal reaction. Although the 597T allele had a protective effect (odds ratio [OR], 0.34 [95% confidence interval {CI}, 0.17-0.68]; P= .002 under the dominant model), homozygosity for the 280-bp allelic length of the microsatellite strongly increased the risk of reversal reaction (OR, 5.83 [95% CI, 1.98-17.15]; P= .001 under the recessive model). These associations were consistent among 3 different ethnic groups. CONCLUSIONS: These data suggest a significant role for TLR-2 in the occurrence of leprosy reversal reaction and provide new insights into the immunogenetics of the disease.