An attempt to optimize the outcome of penetrating keratoplasty in congenital aniridia-associated keratopathy (AAK).

PURPOSE: To propose an optimized microsurgical and medical approach to reduce the risk of complications after penetrating keratoplasty (PKP) in patients with aniridia-associated keratopathy (AAK). METHODS: Retrospective observational case series of 25 PKP performed in 16 patients with AAK. Preoperative indications were endothelial decompensation and vascularized scars (68%) or graft failure (32%) due to limbal stem cell deficiency. The optimized approach included a combination of a small corneal graft size (around 7.0 mm), interrupted 10-0nylon sutures, simultaneous AMT as a patch, large bandage contact lens, temporary lateral tarsorrhaphy, postoperative autologous serum eye drops, and systemic immunosuppression. Main outcome measures included: visual acuity, transplant survival, and complications encountered during follow-up of 107 weeks on average. RESULTS: A complete modified keratoplasty scheme was used in 10 of 25 PKP (group 1), while at least one of the modifications was missing in the other 15 PKP (group 2). After 8 weeks of follow-up, the epithelium was closed in 23 eyes. Visual acuity improved in 19 eyes at 6 months of follow-up, and remained stable in six eyes. None of the eyes showed a decrease in visual acuity. At the last post-operative follow-up, this visual improvement persisted in 14 eyes and graft survival rate after 156 weeks (3 years) was 69% in group 1 versus 44% in group 2 (p = 0.39, log-rank test). Secondary corneal neovascularization (8%), scarring (4%), ulcer (4%), or graft rejection (8%) happened mostly in the second group which was missing at least one of the suggested modifications. CONCLUSIONS: PKP in congenital aniridia must be considered as a high-risk keratoplasty. An optimized therapeutic approach seems to be promising in order to reduce the postoperative complication rate in these most difficult eyes.

Abnormal neovascular and proliferative conjunctival phenotype in limbal stem cell deficiency is associated with altered microRNA and gene expression modulated by PAX6 mutational status in congenital aniridia.

PURPOSE: To evaluate conjunctival cell microRNA (miRNAs) and mRNA expression in relation to observed phenotype of progressive limbal stem cell deficiency in a cohort of subjects with congenital aniridia with known genetic status. METHODS: Using impression cytology, bulbar conjunctival cells were sampled from 20 subjects with congenital aniridia and 20 age and sex-matched healthy control subjects. RNA was extracted and miRNA and mRNA analyses were performed using microarrays. Results were related to severity of keratopathy and genetic cause of aniridia. RESULTS: Of 2549 miRNAs, 21 were differentially expressed in aniridia relative to controls (fold change ≤ -1.5 or ≥ +1.5). Among these miR-204-5p, an inhibitor of corneal neovascularization, was downregulated 26.8-fold in severely vascularized corneas. At the mRNA level, 539 transcripts were differentially expressed (fold change ≤ -2 or ≥ +2), among these FOSB and FOS were upregulated 17.5 and 9.7-fold respectively, and JUN by 2.9-fold, all being components of the AP-1 transcription factor complex. Pathway analysis revealed enrichment of PI3K-Akt, MAPK, and Ras signaling pathways in aniridia. For several miRNAs and transcripts regulating retinoic acid metabolism, expression levels correlated with keratopathy severity and genetic status. CONCLUSION: Strong dysregulation of key factors at the miRNA and mRNA level suggests that the conjunctiva in aniridia is abnormally maintained in a pro-angiogenic and proliferative state, and these changes are expressed in a PAX6 mutation-dependent manner. Additionally, retinoic acid metabolism is disrupted in severe, but not mild forms of the limbal stem cell deficiency in aniridia.

[Evaluation of a virtual reality simulator for learning direct ophthalmoscopy in student teaching]. / Evaluierung eines VR-Simulators zum Erlernen der direkten Ophthalmoskopie in der studentischen Lehre.

BACKGROUND: The use of direct ophthalmoscopy in non-ophthalmological specialties seems to be decreasing in the clinical routine. This could be due to a lack of training and thus an uncertain assessment. OBJECTIVE: The aim of this study was to evaluate the effectiveness and acceptance of learning direct ophthalmoscopy on a simulator in comparison to the classical teaching method among students. MATERIAL AND METHODS: Within the framework of the student block internship for ophthalmology, a total of 34 students took part in the project. The first group received classical training, the second group received simulator training. The learning success was assessed by an objective structured clinical examination (OSCE). RESULTS: The simulator training group showed a significantly higher points score in individual disciplines. The subjects in the classical group achieved a learning success in the OSCE of 78%. In the simulator-based group a higher score of 91% was achieved with a lower scatter in all subdisciplines. DISCUSSION: The patient and instructor-independent availability of the teaching materials, a reduction of light exposure for patients and test subjects, as well as a standardized and controlled mediation of physiological and pathological findings can be emphasized as advantages of the tested simulator. CONCLUSION: The simulator-based training for learning direct funduscopy is effective. The virtual reality simulator evaluated in this project can improve the training of students and residents.

[Venereal diseases in a "general practice" in the 17th and early 18th centuries]. / Venerische Erkrankungen in einer "Allgemeinarztpraxis" im 17. und frühen 18. Jahrhundert.

The diary of the town physician Johannes Franc (1649-1725), handwritten in Latin, gives-among other diseases-an overview of sexually transmitted infections affecting citizens in Ulm such as syphilis and gonorrhea. Franc reported on his own experiences in the diary and also included many theoretical details on the causes of the diseases and the corresponding therapies, including ethical considerations. Even in ancient times, there are indications of venereal diseases. However, at the latest with the outbreak of syphilis around the year 1495, the treatment and control of the spread of venereal diseases became an important task of medicine. Before gonococci were detected by Neisser in 1879, sexually transmitted diseases were generally seen as a single disease. However, at the beginning of the 18th century, there were several doctors who treated syphilis and gonorrhea as separate entities. Franc was one of them. Examining the milestones in the history of syphilis and gonorrhea, the present article reviews the existing theories that tried to explain the origins of these diseases. Franc's treatment patterns are illustrated. Franc's case reports indicate a fundamental change in the perception of STIs at the end of the 17th/beginning of the 18th century.

Cervicofacial angioma and the Kasabach-Merritt syndrome.

We present a neonate with a cervicofacial haemangioma complicated by the Kasabach-Merritt syndrome, respiratory distress due to airway compression and high-output heart failure. This haemangioma and intravascular disseminated coagulation, treated initially by aspirin, ticlopidine and corticosteroids, required more invasive treatment with superselective embolisation and interferon alpha-2a. The clinical outcome was good.

Use of a hospital practice management system to provide initial data for a pediatric immunization registry.

An ongoing challenge in the creation of clinical information systems is the capture of structured clinical information from health care providers while avoiding duplicate data recording. Because immunizations are reimbursable medical procedures, practice management systems that already capture such procedures may be used as a source of clinical data for information systems. We instituted a method for capturing such data on one campus of a multi-institution pediatric immunization registry. We measured the effectiveness of this capture by comparing it to manual audits of selected paper charts over 26 months. Of the immunizations documented by chart audit, 39.69% were captured by the practice management system. Of those not captured, we estimate that a substantial portion were immunizations administered elsewhere and as a result not submitted as a claim through the practice management system. In turn, this was affected by a rate of patient disengagement from primary care of 49%. We discuss the issues associated with using claims data to capture clinical information in the setting of an immunization registry and review possible explanations for this data capture rate.

[Retrospective study of 32 cases of intraosseous perfusion]. / Etude rétrospective de 32 cas de perfusion intraosseuse.

BACKGROUND: We studied all intraosseous infusions performed between 1994 and 1997 by the pediatric intensive care unit and by the pre-hospital emergency medical staff in the Hôpital d'Enfants, Toulouse, France. POPULATION AND METHODS: We report 32 cases of intraosseous infusions in 30 children aged 2 weeks to 9 years. RESULTS: In our population, such a technique has been used in about 60% of all cardiopulmonary arrest, drowning or traffic accident cases. Intraosseous infusion was successful in all cases, on the first attempt in more than 80% of cases. Nine children recovered without any sequelae. No major complications have been observed. CONCLUSIONS: Intraosseous infusion is safe, rapid and effective. It is an essential alternative route in pediatric resuscitation when no other venous access can be performed quickly. An effort must be made on behalf of its diffusion and teaching.

[Intraosseous infusion in children]. / Voie intraosseuse chez l'enfant.

OBJECTIVE: To assess the benefits and drawbacks of intraosseous infusion (IOI) for emergency therapy in children. STUDY DESIGN: Retrospective, non comparative study of IOI carried out between January 1994 and June 1998. PATIENTS: Forty-one children requiring without delay IOI either in the emergency medical ambulance or the emergency admission and intensive therapy units. METHODS: The tibia was punctured by paediatricians either with Mallarmé's trocars in 1994 or Cook Critical Care trocars from 1995 on. RESULTS: Overall, 46 IOI have been carried out in 41 children with a median age of 18 months (range: 8 days-9 years). The main indications for IOI were the management of near drowning, road traffic accidents and cardiopulmonary resuscitation. Complications included one articular puncture and nine subcutaneous extravasations, requiring the puncture of the other limb in five cases. CONCLUSION: IOI is an easy technique for vascular access. It is indicated in emergency cases when, after a delay of five minutes, other techniques have failed.

Decreased leukotriene C4 synthesis accompanies adherence-dependent nuclear import of 5-lipoxygenase in human blood eosinophils.

The enzyme 5-lipoxygenase (5-LO) catalyzes the synthesis of leukotrienes (LTs) from arachidonic acid (AA). Adherence or recruitment of polymorphonuclear neutrophils (PMN) induces nuclear import of 5-LO from the cytosol, which is associated with enhanced LTB4 synthesis upon subsequent cell stimulation. In this study, we asked whether adherence of human eosinophils (EOS) causes a similar redistribution of 5-LO and an increase in LTC4 synthesis. Purified blood EOS examined either in suspension or after adherence to fibronectin for 5 min contained only cytosolic 5-LO. Cell stimulation resulted in activation of 5-LO, as evidenced by its translocation to membranes and LTC4 synthesis. As with PMN, adherence of EOS to fibronectin for 120 min caused nuclear import of 5-LO. Unexpectedly, however, adherence also caused a time-dependent decrease in LTC4 synthesis: EOS adhered for 120 min produced 90% less LTC4 than did cells adhered for 5 min. Adherence did not diminish the release of [3H]AA from prelabeled EOS or reduce the synthesis of the prostanoids thromboxane and PGE2. Also, inhibition of LTC4 production caused by adherence could not be overcome by the addition of exogenous AA. Adherence increased, rather than decreased, LTC4 synthase activity. However, the stimulation of adherent EOS failed to induce translocation of 5-LO from the nucleoplasm to the nuclear envelope. This resistance to activation of the nuclear pool of 5-LO with diminished LT production represents a novel mode of regulation of the enzyme, distinct from the paradigm of up-regulated LT synthesis associated with intranuclear localization of 5-LO observed in PMN and other cell types.

[Pediatrics on the internet]. / La pédiatrie sur Internet.

It is easy to use Internet tools with a computer's mouse but it is more difficult to obtain available addresses in order to reply to concrete and accurate search. The aim of this article is to help new Internet users in their quest for pediatric informations and medical knowledge.

[Danger of nasal vasoconstrictors in infants. Apropos of a case]. / Danger des vasoconstricteurs nasaux chez le nourrisson. A propos d'une observation.

BACKGROUND: Imidazoline derivatives are alpha-adrenergic agents used in nose drops and collyria. Intoxication in children can cause severe central nervous system depression and cardiovascular adverse effects, especially in very young children. CASE REPORT: A 1-month old girl was admitted after nose-drop instillation of naphazoline. At the time of admission, she was comatose, pale, hypothermic and presented arterial hypertension, bradycardia and apnea. Arterial hypertension was corrected after intravenous infusion with phentolamine mesylate, an alpha-antagonist agent. All other symptoms disappeared spontaneously 9 hours after the initial instillation. CONCLUSIONS: Imidazoline intoxication due to overdose or accidental ingestion is frequent in children. Because nose drops are widely available without any medical prescription, nasal vasoconstrictors which contain imidazolin derivatives should be discouraged under 7 years of age and kept out of children's reach.

Pulmonary haemodynamics after surfactant replacement in severe neonatal respiratory distress syndrome.

Aortopulmonary pressure difference and pulmonary blood flow velocity were studied during the first 48 hours of life in 12 premature neonates with severe respiratory distress syndrome (RDS), treated by natural surfactant, and in 25 premature neonates with mild RDS. A non-invasive Doppler ultrasound method was used to estimate aortopulmonary pressure difference and pulmonary blood flow velocity from the left pulmonary artery. Aortopulmonary pressure difference was significantly lower at 6 hours of age in the infants with severe RDS and was not increased one hour after surfactant therapy. Aortopulmonary gradient started to rise at 24 hours of age and was equal to that of neonates with mild RDS at 48 hours. Pulmonary blood flow velocity was significantly lower, initially in the severe RDS group, and was not increased one hour after surfactant therapy. Left pulmonary artery flow velocity began to rise after 24 hours and reached the values of the mild RDS group at 48 hours. These data indicate that aortopulmonary pressure difference and pulmonary blood flow are low in the acute phase of RDS and that surfactant treatment does not seem to affect these values.

[Cardiac tamponade secondary to umbilical venous catheterization accident in a premature infant]. / Tamponnade cardique secondaire à un accident de cathétérisme veineux ombilical chez un prématuré.

BACKGROUND: Cardiac tamponade is a rare and sometimes severe complication of umbilical venous catheterization. CASE REPORT: A premature newborn (gestational age: 30 weeks, birth weight: 1,215 g) required assisted ventilation and umbilical venous catheterization for respiratory distress. Subsequent chest X-ray showed the ascending tip of the catheter lying in the left atrium, inside the auricle. At the age of 16 hours, the infant presented with episodes of bradycardia. Despite a second endotracheal intubation, a sudden vascular collapse necessitated cardiac massage plus sodium bicarbonate and epinephrine. An ultrasound examination was performed because of the persistence of the vascular collapse; it showed a clear echo-free space between the epicardium and pericardium, suggesting pericardial effusion. The patient responded dramatically to pericardial aspiration, providing hemorrhagic fluid containing 20 g per liter glucose. DISCUSSION: Cardiac tamponade probably occurred in this patient as a result of perforation of the atrial wall. Ultrasonography showed no local thrombus, but confirmed the cardiac compression by pericardial fluid and the localization of the tip of catheter in contact with the atrial wall. This case led us to review the mechanical complications of umbilical venous and/or percutaneous catheterization and the rules for their use. CONCLUSION: This complication must be suspected in all patients having a central venous catheter that present with vascular collapse.

Doppler ultrasound study of the anterior cerebral artery in neonates.

In neonates doppler ultrasound of the anterior cerebral artery (ACA) enables cerebral haemodynamics to be studied in a non-invasive manner at the patient's bedside. The signal of the pericallosal artery is recorded through the fontanelle with a 7.5 mHz real-time probe. Two parameters are monitored: Pourcelot's resistance index and mean velocity which reflects blood flow rate. Having established normal values according to the gestational and post-natal ages, the authors describe the changes in cerebral circulation observed in such pathological situations as intraventricular haemorrhage, apnoea, patent ductus arteriosus, hydrocephalus and post-asphyxia syndrome.

[Intra-osseous route. Administration route of drugs in cardiac arrest]. / La voie intra-osseuse. Voie d'administration des médicaments au cours de l'arrêt cardiaque.

We describe the case of a 6 month-old infant suffering from cardiac arrest and successfully resuscitated after epinephrine had been administered through the intraosseous route. During emergency care, vascular access is critical but may be very difficult in young children. Therefore intraosseous infusion appears to be a very efficient alternative for quick administration of drugs and fluids when vascular access is not easily obtained.

[Nosocomial infection at a neonatal care unit]. / Infection nosocomiale dans une unité de réanimation néonatale.

The incidence of nosocomial infections in a neonatal intensive care unit was evaluated to be 11.5% over a 2 year-survey period (1984-1985) in 451 artificially ventilated newborns. Staphylococcus, Enterobacter and Pseudomonas were the most frequently identified germs. Low birth weight, gestational age, mode of delivery and type of the initial pathology were not found to be responsible for the superinfection. The duration of hospitalization was 3 times longer and the cost per infant largely increased in cases with infection. Antibiotic treatment at admittance seemed to delay the onset of nosocomial infection by 8 to 12 days in this population.

[Subtotal avulsion of the lower limb after traumatic section in a 2 and a half-year-old child]. / Arrachement subtotal d'un membre inférieur après section traumatique sur un enfant de deux ans et demi.

Reimplantation after subtotal section of the right lower limb under the Scarpa triangle was been attempted and was successful. Section and contusion of the femoral artery and vein required 2 end-to-end saphenous bypass grafts. The use of the lower limb is correct, after to a follow up of 4 years. The risks, inherent to all proximal section, with important muscular masses, in a small child, are described. Functional prognosis depends on three aspects: bone, vessels, and nerves. A tibial pseudarthrosis had to be operated secondarily with significant lengthening. A partial stenosis of the graft was demonstrated arteriographically. Pes equinus, a result of paralysis, has been fitted with an orthesis. This observation is almost an experimental one: what can be the future of a reimplanted lower limb in the child.

[Subtotal traumatic amputation of the limb in young children. Analysis of 2 successful repairs]. / Arrachement subtotal de membre chez le jeune enfant. Analyse de 2 réparations réussies.

Two replantations of nearly completely amputated limbs are presented. In both cases there were young children. These two success explain the necessity of limb preservation if the nerve integrity is respected. In the young child, the vital risk is lower because of the vessels, the local immunologic defense. The bone union is normal. The vascular suture with venous grafts is made with simple interrupted sutures rather than with continuous over and over sutures. The fasciotomy avoid a compartment syndrome. The bone graft growth is unforeseeable. These two case reports are nearly experimental because of the young age of the patients.