Clinical spectrum of children receiving palliative care in Malaysian Hospitals.

INTRODUCTION: Awareness for paediatric palliative care has resulted in the impetus for paediatrician-led palliative care services across Malaysia. However, there is paucity of local data on patients receiving hospital-based paediatric palliative care. We aim to review the clinical spectrum of patients referred to these services. METHODS: An observational study of children aged between 0-18 years receiving palliative care at 13 hospitals between 1st January and 31st December 2014 was carried out. RESULTS: There were 315 patients analysed, 90 (28.6%) and 46 (14.6%) were neonates and adolescents respectively. The main ICD-10 diagnostic categories for all patients were identified to be 'Congenital malformations, deformations and chromosomal abnormalities' 117 (37.1%), 'Diseases of nervous system' 76 (24.1%) and 'Neoplasms' 60 (19.0%). At referral 156 (50%) patients had holistic needs assessments. Patients with 'Diseases of nervous system' were assessed to have significantly more physical needs than the other two diagnostic categories. Majority of patients who knew of their diagnosis and prognosis were those with malignancy. Over a fifth of referrals were at their terminal admission. Of 144 who died, 111 (77.1%) had advanced care plans. There was bereavement follow-up in 98 (68.1%) patients. CONCLUSION: Patients referred for palliative care have varied diagnoses and needs. To ensure all paediatricians are competent to deliver quality care to all children, further education and training initiatives is imperative.

Polyethylene particles stimulate expression of ITAM-related molecules in peri-implant tissues and when stimulating osteoclastogenesis in vitro.

Wear particle-induced orthopaedic prosthesis loosening is associated with elevated osteoclast activity. The immunoreceptor tyrosine-based activation motif (ITAM)-related molecules OSCAR, FcRγ, TREM2 and DAP12 are important for osteoclast formation. The aim of this study was to determine if these molecules are involved in peri-implant loosening by investigating their expression in peri-implant tissues obtained at revision of joint replacement components containing polyethylene (PE) wear particles, and in osteoclasts formed in vitro in the presence of PE particles. The results showed that there was a marked and statistically significant increase in protein levels of the ITAM-related molecules in the revision tissues. The levels of OSCAR, FcRγ, TREM2 and DAP12 mRNA in the revision tissues were also increased. In vitro PE particles stimulated osteoclast resorption in the presence of 50 ng ml(-1) receptor activator NFκB (RANKL) and significantly elevated the expression of OSCAR, FcRγ, TREM2 and DAP12 during osteoclast formation. These findings suggest that the ITAM signalling molecules and their co-receptors have a role in pathogenic bone loss associated with implant PE wear.

Performance of GDx and HRT in the Finnish Evidence-Based Guideline for Open-Angle Glaucoma.

AIM: To compare the performance of Heidelberg retina tomograph (HRT) and scanning laser polarimetry (GDx) with photographic evaluation of the optic nerve head (ONH) and retinal nerve fiber layer (RNFL) in the application of the Finnish Evidence-Based Guideline for Open-Angle Glaucoma. METHODS: A total of 41 control participants and 312 patients referred for glaucoma evaluation were included in the study. All the participants underwent ophthalmic evaluation, ONH stereophotography, monochromatic RNFL photography, HRT, optical coherence tomography, and GDx evaluation. Participants were classified on the basis of stereophotographic or imaging device results based by applying the Finnish Guideline. RESULTS: Agreement between the stereophotographic evaluation and that on the basis of the imaging devices was 52.9%. Classification of patients with similar management advice on the basis of these evaluations had 56.4% agreement. The specificity of the Finnish guideline for detecting normal patients was 78% (stereophotography) and 83% (imaging devices). Optic disc size interfered with the diagnosis in patients evaluated using the HRT3 glaucoma probability score. Structural changes were more frequently detected before functional changes. CONCLUSION: The Finnish Evidence-Based Guideline for Open-Angle Glaucoma is useful for classifying normal participants and patients with suspected glaucoma or glaucoma through either conventional stereophotographic evaluation of the neuroretinal structures or with the new imaging devices.

[Usefulness of apraclonidine in the diagnosis of Horner syndrome]. / El uso de apraclonidina en el diagnóstico del síndrome de Horner.

CASE REPORTS: We present four cases: two males with Horner Syndrome, who were diagnosed by means of apraclonidine 0.5% and cocaine 4% tests respectively. Two children with anisocoria, one of whom had Horner Syndrome confirmed with apraclonidine 1% and the other, in whom an apraclonidine test ruled out this syndrome but in whom pilocarpine 0.125% confirmed Adie's pupil. DISCUSSION: Apraclonidine drops (1% and 0.5%) may aid in the diagnosis of Horner Syndrome and are easier to obtain than cocaine.

El uso de apraclonidina en el diagnóstico del síndrome de Horner / Usefulness of apraclonidine in the diagnosis of horner syndrome

Casos clínicos: Se presentan cuatro casos: dosvarones adultos con Síndrome de Horner clínico,diagnosticados mediante el test de apraclonidina0,5% y el test de cocaína 4% respectivamente, y dosniños con anisocoria de los cuales, en uno de ellosel Síndrome de Horner se confirma con apraclonidina1% y en otro el test de apraclonidina 1% descartael Síndrome de Horner y el test de pilocarpina0,125% confirma una pupila tónica de Adie.Discusión: La apraclonidina al 1% y al 0,5% puedenorientar el diagnóstico de Síndrome de Horner,siendo más fáciles de obtener que la cocaína (AU)

Case reports: We present four cases: two maleswith Horner Syndrome, who were diagnosed bymeans of apraclonidine 0.5% and cocaine 4% testsrespectively. Two children with anysocoria, one ofwhom had Horner Syndrome confirmed with apraclonidine1% and the other, in whom an apraclonidinetest ruled out this syndrome but in whom pilocarpine0.125% confirmed Adie’s pupil.Discussion: Apraclonidine drops (1% and 0.5%)may aid in the diagnosis of Horner Syndrome andare easier to obtain than cocaine(AU)

[Photodynamic therapy in retinal pigment epithelium detachment associated with long term central serous chorioretinopathy]. / Terapia fotodinámica en DEP avascular asociado a coriorretinopatía serosa central de larga evolución.

PURPOSE: To present a case of long term central serous corioretinopathy associated with chronic detachment of the retinal pigment epithelium (RPE) and the effect of photodynamic therapy on it. CASE CLINIC: We present the case of a 46-year old man with long term chronic central serous chorioretinopathy (18 mo.) with chronic detachment of RPE in his left eye who underwent PDT with Verteporfin (Visudyne). BCVA improved from 0.7 to 1 and metamorphopsia disappeared within one month of treatment. The patient remained asymptomatic for a follow-up period of 15 months. DISCUSSION: Choroidal hyperpermeability is found in central serous chorioretinopathy as a primary involvement factor. PDT with Verteporfin induces a transient reduction in choriocapilaris blood flow and can be used as treatment in cases of chronic central serous chorioretinopathy.

Terapia fotodinámica en Dep avascular asociado a coriorretinopatía serosa central de larga evolución / Photodynamic therapy in retinal pigment epitheliyn detachment associated with long term central serous chorioretinopathy

Objetivo: Describir un caso de coriorretinopatía serosa central asociada a un DEP crónico y el resultado de terapia fotodinámica sobre el mismo. Caso clínico: Se presenta el caso de un hombre de 46 años con coriorretinopatía serosa central crónica de larga evolución (18 meses) con desprendimiento crónico del EPR en su ojo izquierdo que se sometió a tratamiento con terapia fotodinámica con Verteporfin (Visudyne). La AV mejoró de 0,7 a 1, desapareciendo la metamorfopsia en un mes tras el tratamiento. El paciente permaneció asintomático durante un periodo de seguimiento de 15 meses. Discusión: La hiperpermeabilidad coroidea es un factor primario en el desarrollo de la coriorretinopatía serosa central. La terapia fotodinámica con Verteporfin induce una reducción transitoria en el flujo sanguíneo de la coriocapilar y se puede usar como tratamiento en casos de coriorretinopatía serosa central crónica

Purpose: To present a case of long term central serous corioretinopathy associated with chronic detachment of the retinal pigment epithelium (RPE) and the effect of photodynamic therapy on it. Case clinic: We present the case of a 46-year old man with long term chronic central serous chorioretinopathy (18 mo.) with chronic detachment of RPE in his left eye who underwent PDT with Verteporfin (Visudyne). BCVA improved from 0.7 to 1 and metamorphopsia disappeared within one month of treatment. The patient remained asymptomatic for a follow-up period of 15 months. Discussion: Choroidal hyperpermeability is found in central serous chorioretinopathy as a primary involvement factor. PDT with Verteporfin induces a transient reduction in choriocapilaris blood flow and can be used as treatment in cases of chronic central serous chorioretinopathy (Arch Soc Esp Oftalmol 2008; 83: 545-548)

[Evaluation of four new discriminant functions for HRT II in glaucoma diagnosis]. / Evaluación de cuatro nuevas funciones discriminantes del HRT II para el diagnóstico del glaucoma.

PURPOSE: To assess the diagnostic usefulness of four linear discriminant functions for Heidelberg retina tomograph (HRT) II obtained in our population. METHODS: 450 eyes of 450 patients were studied. Eyes were classified as those of healthy subjects (101), those with ocular hypertension (126), those suspected to have glaucoma (121) or those with glaucoma (103). Intraocular pressure, optic nerve head appearance in stereophotographs, and standard automated perimetry results were assessed. ANOVAs among the groups were calculated for all global parameters and the 4 formulas. Receiver operating characteristic (ROC) curves were plotted for the 4 multivariate functions designed in our hospital and the areas under the ROC curves were compared. Sensitivity at 80% and 90% fixed specificities were also calculated. RESULTS: All functions discriminated well between healthy and glaucoma suspects, and between healthy and glaucomatous eyes. At 90% fixed specificity, sensitivities ranged from 54% to 60% in differentiating between healthy and glaucoma suspects, and from 65% to 68% in discriminating between control and glaucoma subjects. No differences were found between the areas under the ROC curves of these functions. CONCLUSIONS: The evaluated linear discriminant functions increased the diagnostic ability of HRT II isolated parameters in detecting glaucoma. These functions performed better than the HRT-provided discriminant functions.

Evaluación de cuatro nuevas funciones discriminantes del HRT II para el diagnóstico del glaucoma / Evaluation of four new discriminant functions for HRT II glaucoma diagnosis

Objetivo: Valorar la capacidad diagnóstica de cuatro fórmulas discriminantes diseñadas para el Heidelberg retina Tomograph (HRT) II a partir de nuestra población. Material y método: Se incluyeron 450 ojos de 450 pacientes. Fueron clasificados según la presión intraocular, morfología papilar en estereofotografías y los resultados de la perimetría automatizada convencional en 101 ojos normales, 126 hipertensos oculares, 121 sospechosos de glaucoma y 102 ojos con glaucoma. Se calcularon las ANOVAs entre los grupos para todos los parámetros globales y las cuatro fórmulas. Se trazaron las curvas ROC para las cuatro funciones discriminantes diseñadas en nuestro hospital y se compararon las áreas bajo las curvas. Se calcularon los valores de sensibilidad para especificidades fijadas en el 80% y 90%. Resultados: Las cuatro fórmulas discriminaron bien entre el grupo control y los sospechosos de glaucoma, y entre controles y glaucomas. Para una especificidad fijada en el 90%, las sensibilidades oscilaron entre el 54% y 60% para diferenciar entre sanos y sospechosos de glaucoma, y entre el 65% y 68% para diferenciar entre sanos y glaucomas. No se observaron diferencias en las áreas bajo la curva de las distintas fórmulas. Conclusiones: Las fórmulas discriminantes evaluadas aumentaron la capacidad diagnóstica de los parámetros aislados del HRT II para detectar glaucomas. Asimismo, mostraron mejor rendimiento que las fórmulas discriminantes incluidas en el HRT

Purpose: To assess the diagnostic usefulness of four linear discriminant functions for Heidelberg retina tomograph (HRT) II obtained in our population. Methods: 450 eyes of 450 patients were studied. Eyes were classified as those of healthy subjects (101), those with ocular hypertension (126), those suspected to have glaucoma (121) or those with glaucoma (103). Intraocular pressure, optic nerve head appearance in stereophotographs, and standard automated perimetry results were assessed. ANOVAs among the groups were calculated for all global parameters and the 4 formulas. Receiver operating characteristic (ROC) curves were plotted for the 4 multivariate functions designed in our hospital and the areas under the ROC curves were compared. Sensitivity at 80% and 90% fixed specificities were also calculated. Results: All functions discriminated well between healthy and glaucoma suspects, and between healthy and glaucomatous eyes. At 90% fixed specificity, sensitivities ranged from 54% to 60% in differentiating between healthy and glaucoma suspects, and from 65% to 68% in discriminating between control and glaucoma subjects. No differences were found between the areas under the ROC curves of these functions. Conclusions: The evaluated linear discriminant functions increased the diagnostic ability of HRT II isolated parameters in detecting glaucoma. These functions performed better than the HRT-provided discriminant functions (Arch Soc Esp Oftalmol 2008; 83: 349-356)

[Importance of central corneal thickness when studying ocular hypertensive eyes, glaucoma suspects and preperimetric glaucomatous eyes]. / Importancia del espesor corneal central en el estudio de hipertensos oculares, sospechosos de glaucoma y glaucomas preperimétricos.

PURPOSE: To compare the central corneal thickness, measured with an ultrasound pachymeter, in normal subjects, those with ocular hypertension, glaucoma suspects and patients with preperimetric glaucoma. METHODS: 61 normal eyes (control group), 131 eyes with ocular hypertension, 62 glaucoma suspects (optic nerve head morphology compatible with glaucoma) and 36 patients with preperimetric glaucoma (abnormal short-wavelength automated perimetry) were prospectively and consecutively selected. Scatter plots of central corneal thickness, against the intraocular pressure values were calculated for each of the study groups. Ultrasound pachymetry measurements were compared between those with normal eyes and the other groups. RESULTS: Ocular hypertensive subjects had higher pachymetry values than the control group (p=0.009). No differences were found in the central corneal thickness between normal eyes and those who were glaucoma suspects, and between normal and preperimetric glaucomatous eyes. A mild direct logarithmic correlation was evident between central corneal thickness and the Goldmann tonometry result in the ocular hypertensive group. CONCLUSIONS: Ocular hypertensive subjects had thicker corneas than the other groups studied. Glaucoma suspects and preperimetric glaucoma patients had similar corneal thickness to the control group.

Effects of green fluorescent protein or beta-glucuronidase tagging on the accumulation and pathogenicity of a resistance-breaking Lettuce mosaic virus isolate in susceptible and resistant lettuce cultivars.

The RNA genome of a resistance-breaking isolate of Lettuce mosaic virus (LMV-E) was engineered to express the jellyfish green fluorescent protein (GFP) or beta-glucuronidase (GUS) fused to the helper-component proteinase (HC-Pro) to study LMV invasion and spread in susceptible and resistant lettuce cultivars. Virus accumulation and movement were monitored by either histochemical GUS assays or detection of GFP fluorescence under UV light. The GFP- and GUS-tagged viruses spread systemically in the susceptible lettuce cultivars Trocadero and Vanguard, where they induced attenuated symptoms, compared with the wild-type virus. Accumulation of the GFP-tagged virus was reduced but less affected than in the case of the GUS-tagged virus. Systemic movement of both recombinant viruses was very severely affected in Vanguard 75, a lettuce cultivar nearly isogenic to Vanguard but carrying the resistance gene mo1(2). Accumulation of the recombinant viruses in systemically infected leaves was either undetectable (GUS-tag) or erratic, strongly delayed, and inhibited by as much as 90% (GFP-tag). As a consequence, and contrary to the parental virus, the recombinant viruses were not able to overcome the protection afforded by the mo1(2) gene. Taken together, these results indicate that GUS or GFP tagging of the HC-Pro of LMV has significant negative effects on the biology of the virus, abolishing its resistance-breaking properties and reducing its pathogenicity in susceptible cultivars.

[Adrenal pheochromocytoma and metastatic intestinal carcinoid tumor in the liver. A purely accidental association?]. / Feocromocitoma suprarrenal y tumor carcinoide intestinal metastásico en hígado. Una asociación puramente casual?

A patient with hepatic metastasis of an intestinal cancer tumor is presented. Six years previously the patient had been diagnosed of HTA secondary to pheochromocytoma of the left adrenal gland and surgery. The clinical findings are described and the results of scintigraphy with 123I-MIBG used for tumor detection are analyzed. The literature was revised and this unique presentation confirmed following the study and exclusion of a possible association with pluriglandular adenomatosis. Finally, following the poor results obtained with different therapeutic procedures in metastatic cancer tumors, the systematic, periodic determination of 5-HIAA, among other investigations, is suggested as a useful alternative in the early diagnosis and potential surgical treatment patients diagnosed of pheochromocytoma.