Retinoblastoma Outcomes in a Tertiary Hospital in Northern Luzon, The Philippines: A 15-Year Experience.

Roland Joseph D. TanObjective To describe the demographics, clinical profile, and outcomes of retinoblastoma patients seen in a tertiary hospital in northern Luzon. Materials and Methods This is a retrospective cross-sectional study of retinoblastoma patients at the departments of ophthalmology and pediatrics of a tertiary hospital in northern Luzon from 2005 to 2020. Results A total of 47 patients involving 53 eyes were included. Twenty nine (62%) are male and forty one (87%) had unilateral retinoblastoma. Mean age at consult was 24 ± 17 months, and mean interval from onset of symptoms to consult was 10 ± 11 months. Two (4%) had family history of retinoblastoma. Twenty-two (47%) patients had intraocular involvement. Leukocoria was the most common presenting symptoms at 62%. Overall survival was 53% with mean follow-up period of 24 ± 24 months. Difference in survival rates based on the extent of involvement was statistically significant ( p < 0.001). Conclusion This is the first study that provided data on demographics, clinical profile, and outcomes of retinoblastoma patients in northern Luzon and the only study with data on clinical outcomes of retinoblastoma patients in The Philippines. Extraocular involvement is a significant factor in the low survival of retinoblastoma patients despite improvement in its management.

[Sexuality assessment of homozygous adult sickle cell patients with a history of priapism in Senegal]. / Évaluation de la sexualité des drépanocytaires adultes homozygotes ayant des antécédents de priapisme au Sénégal.

OBJECTIVES: The objective of this study was to evaluate the sexuality of SS sickle cell patients with a history of priapism. METHODS: This was a case-control study of adult SS sickle cell patients. The occurrence of priapism as well as the nature of the priapism had been investigated. The patients were subdivided into three groups: Group 1 (no priapism), Group 2 (intermittent priapism) and Group 3 (acute priapism). The patients' sexuality was studied using the IIEF-15 questionnaire. RESULTS: We interviewed 191 SS sickle cell patients. The mean age was 27.1±7.1 years. Priapism was observed in 43.5 %. Only 77 patients were eligible for the IIEF15 questionnaire. Groups 1 and 2 performed significantly better than group 3 on erectile function (EF) and orgasmic function (OF) scores. There was no significant difference in the EF and OF scores between groups 1 and 2. No significant difference was observed between the three groups for the scores of sexual desire (SD), intercourse satisfaction (IS), and overall satisfaction (OS). The impairment of erectile function in group 2 was related to the age of the first episode of priapism and the last episode. The impairment of erectile function in group 3 was related to the duration of evolution (P<0.05). CONCLUSION: This study shows that priapism is responsible for impaired erectile function in SS adult sickle cell patients. A program to prevent intermittent episodes of priapism should be put in place.

Molecular Prevalence of Anaplasma marginale and Ehrlichia in Domestic Large Ruminants and Rhipicephalus (Boophilus) microplus Ticks From Southern Luzon, Philippines.

Anaplasmosis and ehrlichiosis are tick-borne rickettsial diseases that cause significant economic losses in the livestock industry worldwide. Although bovine anaplasmosis is known to be endemic in the Philippines, epidemiological data is fragmented. Moreover, little is known about bovine ehrlichiosis in the country. In this study, the prevalence of Anaplasma marginale and Ehrlichia in cattle and water buffalo from provinces in the southern part of Luzon, Philippines, was investigated through PCR. Blood samples from 620 animals comprised of 512 cattle and 108 water buffalo and 195 tick samples were subjected to nested PCR targeting the groESL gene of Anaplasmataceae. Positive samples were further subjected to another nested PCR and conventional PCR to amplify the A. marginale groEL gene and the Ehrlichia dsbA gene, respectively. Selected A. marginale-positive samples were also subjected to nested PCR targeting the msp5 gene. Regardless of the animal host, the overall prevalence in blood samples obtained was 51.9% for Anaplasmataceae, 43% for A. marginale, and 1.1% for Ehrlichia. No water buffalo were positive for Ehrlichia. Meanwhile, 15.9, 6.7, and 2% of the tick samples, all morphologically identified as Rhipicephalus (Boophilus) microplus, were positive for Anaplasmataceae, A. marginale, and Ehrlichia, respectively. Sequence analysis of selected A. marginale msp5 amplicons showed that the isolates from the region share 94-98% identity to reported A. marginale from other countries. The phylogenetic tree showed clustering of isolates in the region and a close relationship with A. marginale isolates from other countries. Sequences of Ehrlichia amplicons from cattle and ticks were 97-100% similar to reported Ehrlichia minasensis isolates. This study showed the high prevalence of A. marginale in Luzon, Philippines, and provided the first molecular evidence of E. minasensis in the country.

Diagnosis Dilemma of Angioimmunoblastic T-Cell Lymphoma in Tuberculosis Endemic Region.

Angioimmunoblastic T-cell lymphoma (AITL) is a rare hematologic malignancy recognized in the WHO 2016 classification as a clinical and histological entity. It is a very poorly described disease in Africa due to its rarity and diagnostic difficulties, particularly differential diagnosis with tuberculosis. Here, we report a 57-year-old man who presented with fever, weight loss, and lymphadenopathies. The diagnosis of tuberculosis was carried out based on lymph node fine needle aspiration showing the image of tuberculous adenitis and CT images in favor of necrotic lymphadenopathies. The presence of autoantibodies and the failure of tuberculosis treatment led us to perform a biopsy with immunostaining that confirmed pathological features of AITL. The patient was treated by CHOP-based chemotherapy, and complete remission was achieved. This case highlights the difficulty of recognizing AITL and the importance of considering other potential differential diagnoses of tuberculosis in the endemic region.

Old World camels in a modern world - a balancing act between conservation and genetic improvement.

Old World camels have served humans in cross-continental caravans, transporting people and goods, connecting different cultures and providing milk, meat, wool and draught since their domestication around 3000-6000 years ago. In a world of modern transport and fast connectivity, these beasts of burden seem to be out-dated. However, a growing demand for sustainable milk and meat production, especially in countries affected by climate change and increasing desertification, brings dromedaries (Camelus dromedarius) and Bactrian camels (Camelus bactrianus) back onstage and into the focus of animal breeders and scientists. In this review on the molecular genetics of these economically important species we give an overview about the evolutionary history, domestication and dispersal of Old World camels, whereas highlighting the need for conservation of wild two-humped camels (Camelus ferus) as an evolutionarily unique and highly endangered species. We provide cutting-edge information on the current molecular resources and on-going sequencing projects. We cannot emphasise enough the importance of balancing the need for improving camel production traits with maintaining the genetic diversity in two domestic species with specific physiological adaptation to a desert environment.

Automation of chromatographic peak review and order to result data transfer in a clinical mass spectrometry laboratory.

INTRODUCTION: Mass spectrometry-based assays have increasingly been implemented in clinical laboratories for their multiplexing capacity and high specificity and sensitivity. However, these methods are often associated with labor-intensive and error-prone data-related workflows, due to the volume of data generated that is often manually reviewed and resulted. We aimed to establish a system within our clinical mass spectrometry laboratory to facilitate data 'flow' from electronic medical record order to result and to automate processes for chromatogram peak review. The processes and validation are described for a 25-hydroxyvitamin D assay. METHODS: Automating chromatogram review and order to result data transfer required flat file interfacing, file transfers of standardized data formats, barcode scanning, and software for peak processing and review. Validation of the automated workflow involved (1) correlation of quantified results generated by two chromatogram analysis methods: Waters TargetLynx and Indigo Bioautomation ASCENT, (2) manual verification of quality assurance flags applied in ASCENT, and (3) testing data flow and integrity across all the systems from order to result. Efficiency and quality improvements were assessed through calculation of batch review times and rates for autoverification and manual manipulations. RESULTS: The correlation of TargetLynx and ASCENT quantitation methods for 25-hydroxyvitamin D2 in patient samples yielded slope of 0.99 (95% CI: 0.989 to 0.996), intercept of 0.46 (95% CI: 0.363 to 0.565), with r = 0.999. The correlation for the D3 fraction showed Deming regression slope of 0.98 (95% CI: 0.969 to 0.989), intercept of 0.06 (95% CI: -0.115 to 0.313), and r = 0.995. Results from both quantitation approaches were also compared to the assigned value in CDC reference samples. The mean bias relative to the CDC was 4.6% for ASCENT and 2.5% for TargetLynx. The median time for chromatogram review of a full 96-well plate of vitamin D results is reduced from approximately 2 h to 14 min and 80% of batches were reviewed within 30 min. Instead of 100% peak review, technologists review only the peaks that have been flagged by the system based on applied rules. Analysis of full plate batches showed that 2-20% of peaks per batch were flagged for manual review. Manipulations made by technologists during chromatogram review were reduced by 75% when using the automated versus manual system. CONCLUSIONS: We describe a system to facilitate data 'flow' from electronic order to result and to automate chromatogram peak review in a clinical liquid chromatography mass spectrometry assay for 25-hydroxyvitamin D. This eliminated manual result entry, repetitive transcription, and unnecessary review of high quality data while enabling systematic evaluation of data quality indicators. The new processes were accurate, improved the data review and processing times, and helped to reduce manual manipulations during chromatogram review.

Comparative study of fatty acid and sterol profiles for the investigation of potential milk fat adulteration.

Milk fat adulteration is a common issue in Central Asia. To assess the current situation in the commercial milk market, 17 milk samples were checked for fatty acid (FA) and sterol profiles to detect potential adulteration using multivariate analysis. Analysis of FA and sterols was performed using gas chromatography with flame ionization detection and gas chromatography with mass-spectrometric detection, respectively. Cluster analysis of FA profiles revealed 3 types of milk samples: (1) samples containing a higher proportion of short-chain FA, (2) samples containing a higher proportion of long-chain FA, and (3) samples with significant amounts of C18 FA. Analysis of sterols showed that samples included (1) milk fat containing 100% cholesterol, sometimes with traces of phytosterols, (2) milk fat with high proportions of ß-sitosterol and campesterol, and (3) milk fat containing high proportions of brassicasterol. We found significant relationships between FA profiles and sterol profiles. The profiles were compared with vegetable oil patterns reported in the literature. More than 50% of the samples appeared to be counterfeited. We conclude that identification of adulteration in milk can be based solely on determination of sterol patterns.

Effect of diet composition on dry matter intake of dairy she-camels.

The present paper aims to propose an evaluation of the ingestibility and selectivity of food items, as well as the effect of different levels of energetic supplementation and concentrate feed ingredients, on camels dry matter intake (DMI). With this goal, an experiment on six dairy she-camels receiving every 14-15 days a progressive high level of concentrates was conducted. During experimental periods, DMI ranged from 1.30 to 1.96 kg DMI per 100 kg of body weight (BW) was used. In an effort to examine the feeding behavior, a sign of reduced appetite was observed. Based on the results, it can be unveiled that as soon as the concentrate intake (CI) reached a quantity of 3.3 kg DM/d, which represented 51% of the total DM intake, the camels did not appear to ingest more exceedingly and their eating activity occurred in a distributed manner during daytime. Besides, an amount of concentrate supplements, which can characterize a high selectivity to fermentable carbohydrates, fed camels consumed dates, as well. In addition, food items such as corn, soybean meal, and dates that are rich in simple nutrient improved DMI. However, the statistical analysis did not reveal any statistically significant negative effect of concentrates on hay ingestion used in the experimental diets. Therefore, the intake of concentrates did not substitute the ingested amount of hay. Furthermore, the immense incorporation of dates and the increase in energy density of diet (DER) negatively affected the ingestion of hay.

Comparative fatty acids composition and cholesterol content in Bactrian (Camelus bactrianus) and dromedary camel (Camelus dromedarius) meat.

The present study is aimed at comparing the fatty acid profiles, cholesterol, and atherogenicity index of Bactrian and dromedary camel meat by using discriminant analysis to identify the more discriminating fatty acids. Six muscles were sampled from nine Bactrian and nine dromedary camels and analyzed for fatty acid parameters and cholesterol content. The mean fatty acid profiles differed in higher proportion between species than between muscles. The main discriminating fatty acids between species (100% well-classed samples) were C15:0, C17:1, C14:1, C20:0, and C18:0. A significant difference was also observed in cholesterol content, with more cholesterol in Bactrian meat (53.6 ± 12.5 mg/100 g) compared to dromedary meat (49.4 ± 11.2 mg/100 g). However, the atherogenicity index was lower in Bactrian meat (1.196 ± 0.148) than in dromedary meat (1.379 ± 0.109). Despite the dietetic interest in camel meat due to its low cholesterol and low-fat content, the high atherogenicity index compared to other red meat appeared as an unfavorable argument.

Rare Metastatic Testicular Cancer in a Veteran.

Testicular yolk sac tumor (YST) is a nonseminomatous germ cell tumor that predominantly affects prepubescent boys. Pure endometrioid variant YST is rare, with only 1 report in the literature. We present the first reported case of en-dometrioid variant YST with mature teratoma in the retro-peritoneal specimen.

Hématome sous-galéal révélant une hémophilie A sévère en période néonatale. A propos d'un cas

Introduction : L'hématome sous-galéal est une urgence diagnostique et thérapeutique souvent d'origine traumatique. Cependant, il peut révéler des troubles constitutionnels de l'hémostase.Observation : M.N, de sexe masculin est né par voie basse à terme, avec une notion d'expressions abdominales. Il pesait 3200 g pour un Périmètre Crânien (PC) de 33 cm et une taille de 52 cm. Il est reçu à H24 de vie pour augmentation du PC à 39 cm avec à l'examen un hématome diffus du cuir chevelu décollant les oreilles, une anémie clinique, une détresse respiratoire et neurologique. Le bilan retrouvait une anémie à 2.8g/dl et une thrombopénie. Le diagnostic d'hématome sous-galéal était retenu et il bénéficiait d'une transfusion sanguine, de vitaminothérapie K et d'une hospitalisation de 10 jours. Une semaine après l'exéat, il est revu pour des hématomes en regard des points de prélèvement avec anémie à 9.5g/dl, un taux de plaquettes normal, un TP normal et un TCA allongé. Le dosage des facteurs VIII et IX ramène un taux de facteur VIII bas à 0.9%. Le diagnostic d'hémophilie A est posé et le patient reçoit une injection intraveineuse de facteur anti hémophilique A.Conclusion : La recherche d'une anomalie de l'hémostase doit être systématique devant toute hémorragie chez le nouveau-né

Efficacy and Safety of Manual Partial Red Cell Exchange in the Management of Severe Complications of Sickle Cell Disease in a Developing Country.

INTRODUCTION: The realization of red cell exchange (RCE) in Africa faces the lack of blood, transfusion safety, and equipment. We evaluated its efficacy and safety in severe complications of sickle cell disease. PATIENTS AND METHOD: Manual partial RCE was performed among sickle cell patients who had severe complications. Efficacy was evaluated by clinical evolution, blood count, and electrophoresis of hemoglobin. Safety was evaluated on adverse effects, infections, and alloimmunization. RESULTS: We performed 166 partial RCE among 44 patients including 41 homozygous (SS) and 2 heterozygous composites SC and 1 S/ß0-thalassemia. The mean age was 27.9 years. The sex ratio was 1.58. The regression of symptoms was complete in 100% of persistent vasoocclusive crisis and acute chest syndrome, 56.7% of intermittent priapism, and 30% of stroke. It was partial in 100% of leg ulcers and null in acute priapism. The mean variations of hemoglobin and hematocrit rate after one procedure were, respectively, +1.4 g/dL and +4.4%. That of hemoglobin S after 2 consecutive RCE was -60%. Neither alloimmunization nor viral seroconversion was observed. CONCLUSION: This work shows the feasibility of manual partial RCE in a low-resource setting and its efficacy and safety during complications of SCD outside of acute priapism.

Dromedary Camels and the Transmission of Middle East Respiratory Syndrome Coronavirus (MERS-CoV).

Middle East respiratory syndrome coronavirus (MERS-CoV) is an existential threat to global public health. The virus has been repeatedly detected in dromedary camels (Camelus dromedarius). Adult animals in many countries in the Middle East as well as in North and East Africa showed high (>90%) seroprevalence to the virus. Middle East respiratory syndrome coronavirus isolated from dromedaries is genetically and phenotypically similar to viruses from humans. We summarize current understanding of the ecology of MERS-CoV in animals and transmission at the animal-human interface. We review aspects of husbandry, animal movements and trade and the use and consumption of camel dairy and meat products in the Middle East that may be relevant to the epidemiology of MERS. We also highlight the gaps in understanding the transmission of this virus in animals and from animals to humans.

Evaluation of transfusion practices in sickle cell disease in Senegal: cohort study of 1078 patients with sickle cell disease.

Blood transfusion is an essential therapeutic tool in the treatment of sickle cell disease. Its indications and modalities vary from one country to another as a function of the clinical severity of the disease and the availability of blood products. This study seeks to evaluate the frequency and modalities of blood transfusions as well as their indications in patients with sickle cell syndrome at the Dakar hematology department from 1995 through 2015. This retrospective study reviewed the records of a cohort of 1078 patients with SS, SC, Sß0 and Sß + thalassemia sickle cell syndromes and collected data about the type of blood products, indications, modalities, and acute transfusion complications. The frequency of transfusion was adjusted for age, sex, and sickle cell profile. The patients' mean age was 23 years (2-44 years) and the sex ratio 0.9. SS disease was predominant (87.4 %); 28.5% had transfusions during the study period. Patients older than 20 years received more transfusions (P = 0.047). The frequency of transfusions did not differ significantly by gender (P = 0.12), but did by profile: a higher percentage of patients with SS disease required transfusion (P = 0.043). Acute anemia was the most common indication for transfusion (50.03 %). Acute transfusion complications were rare (5.8 %). This study shows that less than one third of sickle cell anemia patients in Senegal have transfusions, a finding that confirms that our patients have fewer transfusions than patients in other countries in Africa or in the West. This limited use of transfusion therapy may be related to a less severe level of disease among patients in Senegal, but also to the availability of blood products.

[Profil course of SC sickle-cell patients in Dakar: a case-control study with SS sickle cell form]. / Profil evolutif de la drepanocytose SC A Dakar: étude cas-témoin avec la forme SS.

AIMS: The study aim to describe the epidemiological, clinico-biological and evolutionary aspects of SC sickle cell patients compared to SS sickle cell disease. PATIENTS AND METHODS: We realized a case-control study of 12 months duration including 98 major sickle cell patients (49 SC and 49 SS sickle cell patients). SS sickle cell patients were randomly selected according to age and sex. Socio-demographic, clinico-biological and evolutionary data were noted for each patient. RESULTS: Mean age was 24.7 years (5 - 53). Sex ratio was 0.8. Mean number of transfusions was 0.06 of SC patients and 0.34 for SS patients (p=0.0008). Mean number of vaso-occlusive crisis per year was 2.24 of SC patients and 2.37 of SS patients (p=0.3). Mean basic hemoglobin level was 10.8 of SC patients and 7.8 of SS patients (p=0.0000). Priapism was found in 2.04% of SC patients and 4.04% of SS patients (p=0.3) and acute anemia in 2.04% of SC and 24.48% of SS patients (p=0.003); 26.53% of SC patients had a chronic complication compared to 18.36% of SS patients (p=0.0001). CONCLUSION: This study shows that SC sickle cell patients are less symptomatology compared to SS patients, however they would develop more chronic complications from where the utility for regular follow-up.

BUTS: Le but de notre étude était de décrire les aspects épidémiologiques, clinico-biologiques et évolutifs des patients SC comparés aux patients SS. PATIENTS ET MÉTHODES: Il s'agissait d'une étude cas témoins d'une durée de 12 mois portant sur 98 patients (49 SC et 49 SS8). Les témoins SS étaient choisis de façon aléatoire après appariement selon l'âge et le sexe. Pour chaque patient nous avons noté les aspects sociodémographiques, clinicobiologiques et évolutifs. RÉSULTATS: L'âge moyen était de 24,7 ans (5 - 53). Le sex ratio était de 0,8. Le nombre moyen de transfusions était de 0,06 chez les SC et 0,34 chez les SS (p=0,0008). Le nombre moyen de CVO/an était de 2,24 chez les SC et 2,37 chez les SS (p=0,3). Le taux moyen d'hémoglobine de base était de 10,8 chez les SC et 7,8 chez les SS (p=0,0000). Le priapisme était trouvé chez 2,04% des SC et 4,04% des SS (p=0,3) et l'anémie aigue chez 2,04% des SC et 24,48% des SS (p=0,003); 26,53% des SC souffraient d'une complication chronique contre 18,36% des SS (p=0,0001). CONCLUSION: Cette étude montre que les drépanocytaires SC ont une symptomatologie moindre par rapport aux patients SS, cependant ils développeraient plus de complications chroniques d'où l'utilité d'un suivi régulier.

Correction: Safety of Seasonal Malaria Chemoprevention (SMC) with Sulfadoxine-Pyrimethamine plus Amodiaquine when Delivered to Children under 10 Years of Age by District Health Services in Senegal: Results from a Stepped-Wedge Cluster Randomized Trial.

[This corrects the article DOI: 10.1371/journal.pone.0162563.].

Safety of Seasonal Malaria Chemoprevention (SMC) with Sulfadoxine-Pyrimethamine plus Amodiaquine when Delivered to Children under 10 Years of Age by District Health Services in Senegal: Results from a Stepped-Wedge Cluster Randomized Trial.

BACKGROUND: It is recommended that children aged 3 months to five years of age living in areas of seasonal transmission in the sub-Sahel should receive Seasonal Malaria Chemoprevention (SMC) with sulfadoxine-pyrimethamine plus amodiaquine (SPAQ) during the malaria transmission season. The purpose of this study was to evaluate the safety of SMC with SPAQ in children when delivered by community health workers in three districts in Senegal where SMC was introduced over three years, in children from 3 months of age to five years of age in the first year, then in children up to 10 years of age. METHODS: A surveillance system was established to record all deaths and all malaria cases diagnosed at health facilities and a pharmacovigilance system was established to detect adverse drug reactions. Health posts were randomized to introduce SMC in a stepped wedge design. SMC with SPAQ was administered once per month from September to November, by nine health-posts in 2008, by 27 in 2009 and by 45 in 2010. RESULTS: After three years, 780,000 documented courses of SMC had been administered. High coverage was achieved. No serious adverse events attributable to the intervention were detected, despite a high level of surveillance. CONCLUSIONS: SMC is being implemented in countries of the sub-Sahel for children under 5 years of age, but in some areas the age distribution of cases of malaria may justify extending this age limit, as has been done in Senegal. Our results show that SMC is well tolerated in children under five and in older children. However, pharmacovigilance should be maintained where SMC is implemented and provision for strengthening national pharmacovigilance systems should be included in plans for SMC implementation. TRIAL REGISTRATION: ClinicalTrials.gov NCT 00712374.

[Cutaneous leishmaniasis due to Leishmania infantum associated with HIV]. / Leishmaniose cutanée à Leishmania infantum associée à une infection par le VIH.

BACKGROUND: In Senegal, reported cases of cutaneous leishmaniasis are often due to Leishmania major. Immunosuppression related to HIV infection contributes to the emergence of leishmaniasis in humans and to cutaneous localization of viscerotropic species. We report the first observed case in Senegal of opportunistic cutaneous leishmaniasis due to Leishmania infantum associated with HIV. PATIENTS AND METHODS: A 5-year-old boy presented crusted ulcerative lesions of the scalp and left forearm, together with axillary and cervical lymphadenopathy present for two months. Direct parasitological examination of the scalp and arm lesions, coupled with liquid aspiration of lymph nodes and bone marrow, enabled identification of amastigote forms of Leishmania. Polymerase chain reaction performed on skin, lymph node and bone marrow biopsy samples allowed identification of L. infantum. The child was positive for HIV1. Treatment of HIV infection and leishmaniasis resulted in clinical improvement. DISCUSSION: Co-infection with cutaneous leishmaniasis due to L. infantum and HIV is a complex combination in terms of the related therapeutic issues. The clinical and laboratory outcomes depend on restoration of immunity and on the efficacy, safety and availability of anti-leishmaniasis drugs.