One-year-old male with accelerated growth and development

A 1-year-old male child with isosexual central (gonadotropin-dependent) precocious puberty caused by hypothalamic hamartoma is reported. Details of the diagnosis based solely on neuromaging characteristics, and satisfactory results of medical treatment with gonadotropin releasing hormone agonist analogues, are highlighted.     

Epidemiology and treatment of painful procedures in neonates in intensive care units

Ricardo Carbajal, MD, PhD; André Rousset, MD; Claude Danan, MD; Sarah Coquery, MD; Paul Nolent, MD; Sarah Ducrocq, MD; Carole Saizou, MD; Alexandre Lapillonne, MD, PhD; Michèle Granier, MD; Philippe Durand, MD; Richard Lenclen, MD; Anne Coursol, MD; Philippe Hubert, MD, PhD; Laure de Saint Blanquat, MD; Pierre-Yves Boëlle, PhD; Daniel Annequin, MD; Patricia Cimerman, RN; K. J. S. Anand, MBBS, DPhil; Gérard Bréart, MD, PhD

JAMA. 2008;300(1):60-70.

Context  Effective strategies to improve pain management in neonates require a clear understanding of the epidemiology and management of procedural pain.

Objective  To report epidemiological data on neonatal pain collected from a geographically defined region, based on direct bedside observation of neonates.

Design, Setting, and Patients  Between September 2005 and January 2006, data on all painful and stressful procedures and corresponding analgesic therapy from the first 14 days of admission were prospectively collected within a 6-week period from 430 neonates admitted to tertiary care centers in the Paris region of France (11.3 millions inhabitants) for the Epidemiology of Procedural Pain in Neonates (EPIPPAIN) study.

Main Outcome Measure  Number of procedures considered painful or stressful by health personnel and corresponding analgesic therapy.

Results  The mean (SD) gestational age and intensive care unit stay were 33.0 (4.6) weeks and 8.4 (4.6) calendar days, respectively. Neonates experienced 60 969 first-attempt procedures, with 42 413 (69.6%) painful and 18 556 (30.4%) stressful procedures; 11 546 supplemental attempts were performed during procedures including 10 366 (89.8%) for painful and 1180 (10.2%) for stressful procedures. Each neonate experienced a median of 115 (range, 4-613) procedures during the study period and 16 (range, 0-62) procedures per day of hospitalization. Of these, each neonate experienced a median of 75 (range, 3-364) painful procedures during the study period and 10 (range, 0-51) painful procedures per day of hospitalization. Of the 42 413 painful procedures, 2.1% were performed with pharmacological-only therapy; 18.2% with nonpharmacological-only interventions, 20.8% with pharmacological, nonpharmacological, or both types of therapy; and 79.2% without specific analgesia, and 34.2% were performed while the neonate was receiving concurrent analgesic or anesthetic infusions for other reasons. Prematurity, category of procedure, parental presence, surgery, daytime, and day of procedure after the first day of admission were associated with greater use of specific preprocedural analgesia, whereas mechanical ventilation, noninvasive ventilation and administration of nonspecific concurrent analgesia were associated with lower use of specific preprocedural analgesia.

Conclusion  During neonatal intensive care in the Paris region, large numbers of painful and stressful procedures were performed, the majority of which were not accompanied by analgesia.

     

Dengue vaccine: The current status

Dengue fever is a re-emerging public health problem with two-fifths of the world population being at risk of infection. Since there are no antiviral drugs available against the dengue virus, and vector control programmes have been largely unsuccessful in preventing outbreaks, vaccination seems to be the most viable option for preventing infection. An ideal dengue vaccine should provide long lasting immunity against all four serotypes of the virus. The envelope protein of the virus plays a key role in vaccine development. The present day candidate vaccines includes a live attenuated tetravalent vaccine, intertypic chimaeric vaccines based on live attenuated dengue virus vectors, chimaeric vaccines based on the live attenuated Yellow Fever 17D vector and recombinant vaccines which include vaccines based on flavivirus and non-flavivirus vectors. Tetravalent live attenuated vaccines, intertypic chimaeric vaccines and chimaeric vaccines are being tested in human trials. Recombinant DNA vaccines based on flavivirus and non-flavivirus vectors are being tested in animal trials. Recent studies have shown that the tetravalent formulations may elicit an unbalanced immune response. Research is continuing to find means of obtaining a balanced response to all antigens in the tetravalent formulations.     

Effects of reserpine on blood-pressure responses evoked from the hypothalamus

Different areas of the hypothalamus were stimulated by a stereotaxic technique before and after reserpine in unanaesthetized cats. Reserpine in a dose of 1 mg./kg. body weight increased the threshold of stimulation of hypothalamic areas in cats giving an initial pressor response. It decreased the threshold of stimulation of areas of the hypothalamus in animals giving an initial depressor response. Reserpine thus appeared to affect the hypothalamus directly in two ways. It depressed the sympathetic centres in the diencephalon and facilitated the parasympathetic ones.     

Persistent transgene product in retina, optic nerve and brain after intraocular injection of rAAV.

Recombinant adeno-associated virus (rAAV) is a promising vector for retinal application as it transduces photoreceptors and retinal pigment epithelium cells efficiently and in a stable fashion. Because rAAV also transduces retinal ganglion cells, we reasoned that ocular application of rAAV might result in delivery of transgenic protein to the CNS. Here we describe high levels of green fluorescent protein (GFP) persisting at least 6 months in optic nerves and brains of mice and dogs after intravitreal delivery of rAAV-GFP. There was no clinical or histological evidence of inflammatory response although a mild humoral Th-2 response to viral capsid proteins was detected. These findings have important implications with respect to therapeutic applications of rAAV.     

Use of mobile telephone short message service as a reminder: the effect on patient attendance

Background: Nonattendance for hospital outpatient appointments is a major burden on healthcare systems and the estimated financial loss can be high. Various reasons for patients not attending have been reported, including the patient/carer forgetting the appointment and confusion over the date and time of the appointment. Various reminder systems have been trialled across a variety of clinical settings. More recently, short message service (SMS) text messaging has emerged as a viable approach for delivering reminders to outpatients at a relatively low cost. Aims and objectives: To evaluate the effect of appointment reminders, sent as SMS text messages to patients’ mobile telephones, on attendance at outpatient clinics at the ITS Centre for Dental Studies and Research (ITS‐CDSR), Muradnagar, Ghaziabad, Uttar Pradesh, India. Materials and methods: The study was conducted at ITS‐CDSR in the Departments of Prosthodontics, Endodontics, Orthodontics and Paedodontics. Patients attending these departments for a period of 4 months and those who had provided a contact mobile number were sent an SMS reminder. Results: In this study, 206 subjects (male, 124; female, 82) participated, 96 (male, 57; female, 39) of whom were in the test group and 110 (male, 67; female, 43) of whom were in the control group. The rate of attendance on time was found to be significantly higher in the test group (79.2%) than in the control group (35.5%). Conclusion: The study results indicate that sending appointment reminders as text messages to patients is an effective strategy to reduce nonattendance rates.     

Use of a "small-bubble technique" to increase the success of Anwar's "big-bubble technique" for deep lamellar keratoplasty with complete baring of Descemet's membrane

PURPOSE: To describe a quick and simple "small-bubble" technique to immediately determine the success of attaining complete Descemet's membrane (DM) separation from corneal stroma through Anwar's "big-bubble" technique of deep anterior lamellar keratoplasty (DALK) for complete stromal removal. METHODS: A partial trephination was followed by a lamellar dissection of the anterior stroma. Deep stromal air injection was then attempted to achieve the big bubble to help separate the stroma from the DM. To confirm that a big bubble had been achieved, a small air bubble was injected into the anterior chamber (AC) through a limbal paracentesis. If the small bubble is then seen at the corneal periphery, it confirms that the big-bubble separation of DM was successful because the convex nature of the bubble will cause it to protrude posteriorly, forcing the small AC bubble to the periphery. If the small AC bubble is not seen in the corneal periphery, this means that it is present in the centre, beneath the opaque corneal stroma, and therefore the big bubble has not been achieved. RESULTS: We used the small-bubble technique to confirm the presence of the big bubble in three (one keratoconus, one interstitial keratitis and one dense corneal scar) out of 41 patients who underwent DALK. The small-bubble technique confirmed that the big bubble was achieved in the eye of all three patients. Complete stromal removal with baring of the DM was achieved, and postoperatively all three eyes achieved best corrected vision of 6/6. CONCLUSION: The small-bubble technique can be a useful surgical tool for corneal surgeons attempting lamellar keratoplasty using the big-bubble technique. It helps in confirming the separation of DM from the deep stroma, which is important in achieving total stromal replacement. It will help to make the transition to lamellar keratoplasty smoother, enhance corneal graft success and improve visual outcomes in patients.     

Inner retinal abnormalities in X-linked retinitis pigmentosa with RPGR mutations

PURPOSE: To investigate in vivo the retinal microstructure in X-linked retinitis pigmentosa (XLRP) caused by RPGR mutations as a prelude to treatment initiatives for this common form of RP. METHODS: Patients with RPGR-XLRP (n = 12; age range, 10-56 years) were studied by optical coherence tomography (OCT) in a wide region of central retina. Overall retinal thickness and outer nuclear layer (ONL) and inner retinal parameters across horizontal and vertical meridians were analyzed and compared. RESULTS: Retinal architecture of all patients with RPGR mutations was abnormal. At the fovea in younger patients, the ONL could be normal; but, at increasing eccentricities, there was a loss of photoreceptor laminar structure, even at the youngest ages studied. At later ages and advanced disease stages, the ONL was thin and reduced in extent. Inner retinal thickness, in contrast, was normal or hyperthick. Inner retinal thickening was detectable at all ages studied and was strongly associated with ONL loss. CONCLUSIONS: Inner retinal laminar abnormalities in RPGR-XLRP are likely to reflect a neuronal-glial retinal remodeling response to photoreceptor loss and are detectable relatively early in the disease course. These results should be factored into emerging therapeutic strategies for this form of RP.