Corneal Cross-Linking for Brittle Cornea Syndrome.

PURPOSE: To describe corneal cross-linking (CXL) as a treatment option for brittle cornea syndrome (BCS). METHODS: Case report. RESULTS: Ethical decision making enabled bilateral sequential transepithelial CXL in an 11-year-old girl with BCS. Postoperative courses were uneventful with a bilateral stromal demarcation line, unchanged corneal transparency, constant endothelial cell density, and stable topography 2 years after intervention. CONCLUSIONS: Modified CXL can safely be performed in patients with BCS. Ethical review may be helpful for interventions deviating from standard practice.

Prevention of vitamin K deficiency bleeding with three oral mixed micellar phylloquinone doses: results of a 6-year (2005-2011) surveillance in Switzerland.

UNLABELLED: In 2003, the Swiss guidelines to prevent vitamin K deficiency bleeding (VKDB) were adapted. As two oral doses (2 mg, hour/day 4) of mixed micellar VK preparation had failed to abolish late VKDB, a third dose (week 4) was introduced. This report summarizes the new guidelines acceptance by Swiss pediatricians and the results of a prospective 6-year surveillance to study their influence on the incidence of VKDB. The new guidelines acceptance by Swiss pediatricians was evaluated by a questionnaire sent to all pediatricians of the Swiss Society of Paediatrics. With the help of the Swiss Paediatric Surveillance Unit, the incidence of VKDB was monitored prospectively from July 1, 2005 until June 30, 2011. Over a 6-year period (458,184 live births), there was one case of early and four cases of late VKDB. Overall incidence was 1.09/10(5) (95 % confidence intervals (CI) 0.4-2.6). Late VKDB incidence was 0.87/10(5) (95 % CI 0.24-2.24). All four infants with late VKDB had an undiagnosed cholestasis at the time of bleeding; parents of 3/4 had refused VK prophylaxis, and in 1/4, the third VK dose had been forgotten. Compared with historical control who had received only two oral doses of mixed micellar VK (18 cases for 475,372 live births), the incidence of late VKDB was significantly lower with three oral doses (Chi(2),Yates correction, P = 0.007). CONCLUSION: VKDB prophylaxis with 3 × 2 mg oral doses of mixed micellar VK seems to prevent adequately infants from VKDB. The main risk factors for VKDB in breast-fed infants are parental VK prophylaxis refusal or an unknown cholestasis.

Intraocular pressure during a very high altitude climb.

PURPOSE: Reports on intraocular pressure (IOP) changes at high altitudes have provided inconsistent and even conflicting RESULTS: The purpose of this study was to investigate the effect of very high altitude and different ascent profiles on IOP in relation to simultaneously occurring ophthalmic and systemic changes in a prospective study. METHODS: This prospective study involved 25 healthy mountaineers who were randomly assigned to two different ascent profiles during a medical research expedition to Mt. Muztagh Ata (7,546 m/24,751 ft). Group 1 was allotted a shorter acclimatization time before ascent than was group 2. Besides IOP, oxygen saturation (SaO(2)), acute mountain sickness symptoms (AMS-c score), and optic disc appearance were assessed. Examinations were performed at 490 m/1,607 ft, 4,497 m/14,750 ft, 5,533 m/18,148 ft, and 6,265 m/20,549 ft above sea level. RESULTS: Intraocular pressure in both groups showed small but statistically significant changes: an increase during ascent from 490 m/1,607 ft to 5,533 m/18,148 ft and then a continuous decrease during further ascent to 6,265 m/20,549 ft and on descent to 4,497 m/14,750 ft and to 490 m. Differences between groups were not significant. Multiple regression analysis (IOP-dependent variable) revealed a significant partial correlation coefficient of beta = -0.25 (P = 0.01) for SaO(2) and beta = -0.23 (P = 0.02) for acclimatization time. DISCUSSION: Hypobaric hypoxia at very high altitude leads to small but statistically significant changes in IOP that are modulated by systemic oxygen saturation. Climbs to very high altitudes seem to be safe with regard to intraocular pressure changes.

Angiotensin-converting enzyme inhibitor fetopathy: long-term outcome.

Fetal exposure to angiotensin-converting enzyme inhibitors (ACEIs) is associated with increased neonatal morbidity and mortality. Long-term follow-up of three patients with fetal ACEI exposure revealed impaired renal function in two, severe hypertension and proteinuria in one and isolated polycythaemia in all three. Careful long-term follow-up of children with ACEI fetopathy is recommended.

Impact of improved survival of very low-birth-weight infants on incidence and severity of bronchopulmonary dysplasia.

BACKGROUND: It has been suggested that improved survival of very low birth weight (VLBW) infants may have resulted in increased numbers of patients with bronchopulmonary dysplasia (BPD). GOAL: To determine the impact of changes in mortality on the incidence and/or severity of BPD in three different time periods with distinct respiratory support strategies. METHODS: Retrospective single center cohort study of VLBW infants: Cohort A (1986-1990): pre-surfactant era, use of conventional intermittent mandatory ventilation (IMV); cohort B (1993-1994): use of synthetic surfactant, nasopharyngeal continuous positive airway pressure (CPAP) and conventional IMV; cohort C (2000-2001): use of natural surfactant, early nasal prong CPAP, synchronized IMV with tidal volume monitoring and high frequency oscillatory ventilation (HFOV). BPD was classified as mild, moderate or severe according to Jobe and Bancalari. RESULTS: The median gestational ages and birth weights were 28 3/7 weeks and 1,120 g for cohort A (n = 97), 30 0/7 weeks and 1,340 g for cohort B (n = 100), and 29 1/7 weeks and 1,200 g for cohort C (n = 135). The use of partial or complete courses of antenatal corticosteroids (ANC) increased over time (58%, 72%, and 82%, p = 0.003). There was a 50% reduction of mortality between each time period with mortality rates of 30%, 14% and 7% in cohorts A, B and C, respectively (p < 0.001). The overall incidence of BPD was 26% in the pre-surfactant era, 11% during the mid-1990s and 19% in the most recent time period (r = -0.05, p = 0.36). Moderate and severe forms of BPD decreased over time and were seen in 11% in cohort A, 3% in cohort B and 2% in cohort C (p = 0.008). CONCLUSION: Changes in neonatal care of VLBW infants, including increased use of ANC and modified respiratory support strategies, have resulted in dramatically improved survival rates over the past 15 years without increasing moderate to severe pulmonary morbidity.

Prevention of vitamin K deficiency bleeding with oral mixed micellar phylloquinone: results of a 6-year surveillance in Switzerland.

UNLABELLED: In 1995, a new form of vitamin K prophylaxis with two oral doses of 2 mg mixed micellar phylloquinone (Konakion MM) on the 1st and 4th day of life was introduced in Switzerland. It was hoped that this new galenic preparation of phylloquinone would protect infants with insufficient or absent bile acid excretion from late vitamin K deficiency bleeding (VKDB). Subsequently, the occurrence of VKDB was monitored prospectively between July 1, 1995 and June 30, 2001 with the help of the Swiss Paediatric Surveillance Unit (SPSU). Over a period of 6 years (475,000 deliveries), there were no cases of early (<24 h of age), one case of classical (2-7 days of life), and 18 cases of late (1-12 weeks) VKDB fulfilling standard case definitions. In 13/18 patients with late VKDB there was pre-existing liver disease and in 4/18 patients, parents had refused prophylaxis. The incidence of late VKDB for infants with completed Konakion MM prophylaxis was 2.31/100,000 (95% CI: 1.16-4.14) and for the entire population 3.79/100,000 (95% CI: 2.24-5.98). There was only one case of late VKDB after complete prophylaxis in an infant without underlying liver disease. CONCLUSION: two oral doses of 2 mg of a mixed micellar vitamin K preparation failed to abolish VKDB. The recommendations for vitamin K prophylaxis in Switzerland have therefore been changed to include a third dose at 4 weeks of age. Starting on January 1, 2004, the incidence of vitamin K deficiency bleeding will again be monitored prospectively by the Swiss Paediatric Surveillance Unit.