bluuebunny/crossref_metadata_2025_split huggingface dataset:
GLIOMA-01 GENETIC INVESTIGATIONS AND COUNSELING IN FAMILIAL MSS TURCOT SYNDROME ASSOCIATED TO INFERTILITY AND CHILD MORTALITY
Authors: Abdelmoula Nouha Bouayed , Abdelmoula Balkiss 
Published: October 2023

Abstract
The aim of this study was to report MSI genetic findings in a three-generation Tunisian pedigree of Turcot syndrome 1 (TS1), characterized by an uncommon association with infertility and child mortality. Microsatellite instability testing and genetic counseling were offered to affected members and their young relatives, to provide optimal clinical and preventive care. The index case of our Tunisian pedigree was a 38 year-old female, in whom histologic assessment of endoscopic specimens through a colorectal adenoma resection during screening colonoscopy, showed a tubular adenoma of the lower rectum with low-grade intraepithelial neoplasia. The screening procedure was conducted because her two brothers, 58 and 60 years old, harbored respectively glioblastoma multiforme and colon adenocarcinoma. The first brother exhibited an intracranial hypertension syndrome and a left hemiplegia leading to the diagnosis of a brain tumor. Whereas the second brother manifested diarrhea and intestinal colic that were investigated by colonoscopy. Histologic assessment of endoscopic specimens through a right preangular budding formation showed a well-differentiated liberkhunian adenocarcinoma that was treated by a right hemicolectomy. Genetic assessment at the germinal and somatic levels was conducted for the three affected patients using conventional microsatellite instability (MSI) testing methods. bMolecular analysis determined MSI status of the tumors and classified them into stable types (MSS) without any mutation or aberrant expression of MLH1, MSH2, MSH6, and PMS2 genes. Familial history revealed, at the first generation, a consanguineous couple who lost several children at a young age. This couple gave birth to eight children, three of whom were infertile, three affected by MMS TS1 and two healthy. The third generation consists of 15 children, so far, without any tumors. Genetic counselling for TS remains difficult because of the rarity of the condition, its shifting definition over the years, and its genetic and phenotypic heterogeneity.
GLIOMA-13 RIGHT FRONTO-PARIETAL PILOCYTIC ASTROCYTOMA WHO GRADE I ASSOCIATED WITH HEMORRHAGIC COMPONENT AND ESSENTIAL HYPERTENSION IN AN ADULT: A CASE REPORT
Authors: Kamabu Larrey Kasereka 
Published: October 2023

Abstract
Pilocytic astrocytoma (PA) is a primary glial neoplasm and low-grade tumor. After total resection, the prognosis is very good. A poor prognosis results from leptomeningeal spreading and bleeding, both of which are extremely uncommon in Pilocytic Astrocytoma patients.
CASE PRESENTATION: We report the case of a 43-year-old male that presented with a 4 days’ history of right sided frontal headache, non-radiating and mostly felt in the morning. Not associated with vomiting and fever. In addition, there was a sudden decline in level of consciousness with no associated stool or urine incontinence. The patient also developed three episodes of tonic clonic convulsions, lasting less than 3 minutes associated with urine incontinence and frothing from the mouth. Two days later, there was a sudden onset of left sided weakness involving upper and lower limbs. However, there is no history of trauma or hypertension. He was identified as having a hemorrhagic right fronto-parietal PA evidence on MRI associated with essential hypertension. The patient had an intervention combination of medical treatment and craniotomy via fronto-temporal approach plus excision of lesion. Histopathological examination of specimen determined that it was PA WHO grade I. He was treated with oral anti-epileptic therapy (Phenytoin 300 mg once a day), folic acid supplementation (400 mcg once a day), anti-hypertensive therapy (Amlodipine 10 mg and Losartan 50 mg once daily) and he showed marked improvement and was discharged home after 1 month and followed up by primary neurosurgeon, oncologist and physician.
CONCLUSION: This case report raises awareness of Pilocytic Astrocytoma among adults and emphasizes the value of early diagnosis and management of patients with Pilocytic Astrocytoma associated with essential hypertension can prevent complications, reduce morbidity and mortality.
GLIOMA-16 EARLY NEUROCOGNITIVE OUTCOME POST RESECTION OF ADULT SUPRATENTORIAL GLIOMAS AT THE KENYATTA NATIONAL HOSPITAL
Authors: Mwangi Jefferson Wanyoike , Musau Christopher , Wekesa Vincent , Karanja Susan 
Published: October 2023

Abstract
Gliomas are the commonest brain tumors in our setting. Traditionally; Overall Survival, progression free survival and Extent of resection are used to define oncologic outcome in gliomas. These measures don’t reflect the complete patient status and hence the need for functional outcome determination. Cognitive outcome, is a key functional measure in gliomas. This was a prospective Cohort Study whose objective was to determine the Early Neurocognitive outcome post-resection of adult supratentorial gliomas at the Kenyatta National Hospital. Our study population was adult patients with supratentorial gliomas. We examined twenty patients who met the inclusion criteria. Data collection was by an interviewer-based questionnaire incorporating the ACE III (Addenbrooke Cognitive Examination III) tool administered at three instances as follows: - Within two weeks Preoperatively (T0), at one week postoperatively (T1) and at four weeks postoperatively (T2). The pre and post operative cognitive scores were then analyzed to determine the cognitive outcome. Statistical significance was considered where the p-value was &lt;0.05. Ninety five percent of patients had cognitive impairment at baseline. Mean age of presentation was 34.3 years for Low grade glioma (LGG) and 43.8 years for High Grade Glioma (HGG). 55% of patients had LGG while 45% had HGG. Overall, there was transient decline in cognition from T0-T1 and a gradual improvement beyond the baseline from T1-T2. This improvement was across all domains but was significant in the total ACE score, memory and fluency. LGG showed the greatest improvement in cognition especially in the fluency domain. Sixty percent of the tumors were subtotally resected and showed significant cognitive change in the domains of Attention, fluency and Visuospatial association. Glioma surgery in our setting results in a transient decline in cognition one week postoperatively after which improvement in cognition beyond baseline is noted one month post operatively.
PAEDIATRIC-14 EXPÉRIENCE DU SERVICE DE RADIOTHÉRAPIE DU CENTRE D’ONCOLOGIE ET DE RADIOTHÉRAPIE ALASSANE OUATTARA DANS LA PRISE EN CHARGE DES TUMEURS DE L’ENFANT: A PROPOS DE 27CAS
Authors: Diakite Kadiatou 
Published: October 2023

Abstract
Les tumeurs chez l’enfant ne ressemblent pas à celles de l’adulte. Ils sont d'abord beaucoup plus rares et représentent 1à2% de l’ensemble des cancers. Nous avons eu à traiter 27 enfants pendant la période de 2019-2023 soit 11 filles et 16 garçons avec un sexe ratio de 1.45. L’âge médian était de 7 ans avec un intervalle de 2 -16ans. Le type histologique le plus fréquent était le Rétinoblastome avec 8 cas suivi de Néphroblastome 6 cas, astrocytome 3 cas, UCNT du Cavum 2 cas, Gliome cérébral 2 cas et autres 4 cas. La plupart des enfants de moins 7 ans ont eu recours à une anesthésie générale pour faciliter les séances de radiothérapie. La dose d’irradiation était fonction du type histologique et du grade. Le traitement des Tumeurs chez l’enfant nécessite une prise en charge pluridisciplinaire. La chirurgie reste un traitement clé si indiquée. L’indication de la radiothérapie, la chimiothérapie ou leur association dépend du type histologique et le grade. Le pronostic dépend de la localisation mais aussi du délai de prise en charge.
CFS: definitions, epidemiology, presentation, prognosis
Authors: Wessely Professor Simon , Hotopf Dr Matthew , Sharpe Dr Michael 
Published: October 2023

Abstract
By the mid 1980s chronic fatigue syndrome had arrived. The story of the next ten years is the story of the professional community attempting to catch up on the dramatic entry of CFS into the public arena, and to bring the traditional methods of scientific inquiry to bear on the problem. As in all such endeavours, the appropriate starting point was to establish a case definition of the new syndrome. This was recognized with the publication in 1988 of what became known as the CDC or Holmes’ criteria1• This was a milestone in the acceptance of the syndrome and the beginning of a new generation of scientific studies.
The Effects Of Smoking On Fetal Growth: Evidence For A Threshold, The Importance Of Brand Of Cigarette, And Interaction With Alcohol And Caffeine Consumption
Authors: Anderson H Ross , Bland J Martin , Peacock Janet L 
Published: October 2023

Abstract
The achievement of optimum fetal growth is accepted as a desirable clinical and public health objective. In so far as perinatal mortality and morbidity, with their attendant personal, social, and economic costs are increased as the birth weight distribution is shifted downwards, the reduction in fetal growth (birth weight adjusted for gestational age) observed with smoking, while clinically unimportant for most pregnancies, is of considerable public health importance (US Department of Health and Human Services 1980; Dunn 1984; Osteret al. 1988). The association between cigarette smoking and reduced birth weight has been known for some time (Simpson 1957; Andrews and McGarry 1972; Butleret al. 1972; US Department of Health and Human Services 1980; McIntosh 1984). Some have suggested that this is not causal but explained by other factors, such as diet, personal characteristics, or psychosocial stress, which may relate to both fetal growth and smoking behaviour (Yerushalmy 1971; Rush and Cassano 1983). Most authorities, however, have concluded that the association is causal because it is found in a wide variety of study designs and epidemiological contexts, exhibits a dose-response relationship, and has biological plausibility (Abel 1980; US Department of Health and Human Services 1980). A causal relationship is also suggested by the usual finding that smokers who give up at any time before 30 weeks have heavier infants than persistent smokers (MacArthur and Knox 1988).
Polymyalgia rheumatica and giant cell arteritis
Authors: Silman Alan J , Hochberg Marc C 
Published: October 2023

Abstract
Polymyalgia rheumatica (PMR) and giant cell (syn. temporal) artent1s (GCA) are two related disorders which, for epidemiological purposes, are best considered together. PMR is a relatively ill-defined clinical syndrome of limb-girdle pain and stiffness in association with systemic features such as pyrexia and a raised erythrocyte sedimentation rate (ESR). Diagnosis depends on the presence of combinations of the relevant features in the absence of another pathology such as rheumatoid arthritis or multiple myeloma. By contrast GCA is a vasculitic disorder, which can be widely disseminated affecting the medium and large arteries and whose diagnosis is pathological, classically based on the microscopic appearance of granulomatous change with Langerhans giant cells. There is, however, considerable overlap between the two disorders with a variable proportion of patients with PMR, depending on the study, with a positive biopsy of the temporal artery. Similarly series of patients have shown that a variable proportion of patients with GCA satisfy the clinical grounds for the diagnosis of PMR. The degree of overlap between the two depends on the referral practice of the centre reporting, the frequency with which temporal artery biopsy is undertaken, and the length of follow up. The latter is important as cross-sectional studies will underestimate the true co-occurrence rate. Thus in one series 40 per cent of 66 patients presenting with PMR developed GCA I month to 9 years after presentation, whereas 27 per cent of PMR patients who did not progress to GCA also had a positive temporal artery biopsy (Jones and Hazleman 1981). There have been three substantive population-based studies where attempts have been made to ascertain all cases with either disorder (Bengtsson and Malmvall 1981; Boeson and Sorensen 1987; Salvarani et al. 1987). The overlap between the two disorders and the relationship to the temporal artery biopsy result from these cross-sectional studies are shown in Table IO.I. The results suggest that between 20-30 per cent of cases have features of both diseases and that isolated PMR accounts for over 50 per cent.
Chondrocalcinosis
Authors: Silman Alan J , Hochberg Marc C 
Published: October 2023

Abstract
This review covers a number of linked pathological, radiological, and clinical entities, and it is this linkage of conceptually distinct entities that creates confusion in determining epidemiology (Fig. 13.1). There are also differences in the use of nomenclature and, at the time of writing, there is a lack of international agreement. The pathological entity is deposition of calcium salt crystals in articular, including meniscal, cartilage. The crystals are predominantly of the calcium phosphate family (McCarty et al. 1%2), mainly calcium pyrophosphate dihydrate (CPPD), though other calcium salts are found including hydroxyapatite (McCarty et al. 1966). CPPD crystal deposition can be one cause of the radiological observation of chondrocalcinosis, that is the radiological appearance of calcified deposits in specific cartilaginous sites such as the meniscal cartilages of the knee, wrist, pelvis, and other peripheral joints. Clinically most of the calcified deposits are silent but the syndrome of pseudogout* is well recognised of an acute mono or oligoarthritis, resembling gout but where the synovial fluid contains CPPD crystals rather than urate ones. The diagnosis of pseudogout is normally based indirectly on finding crystals in the synovial fluid which, under polarized light, are either weakly or negatively birefringent compared to the positive birefringence of urate crystals. In addition to the clinical presentation of pseudogout there are a number of other clinical patterns of presentation. These include (i) a polyarticular osteoarthritis, though the exact relationship between CPPD and osteoarthritis is unclear (Dieppe et al. 1982), (ii) a widespread inflammatory polyarthritis mimicking rheumatoid arthritis (including, in IO per cent, a positive reaction for rheumatoid factor), and (iii) rarely a neuropathic picture resembling a Charcot joint (McCarty 1975). Uric acid arthropathy probably co-exists with CPPD arthropathy greater than expected by chance (McCarty 1975). The terms pyrophosphate arthropathy (Europe) and CPPD crystal deposition disease (USA) are used interchangeably which hinders understanding.
Neurology
Authors: Morton Rosemary J , Phillips Barbara M 
Published: October 2023

Abstract
1 Convulsions must be controlled as soon as possible, as brain damage may occur after 30 minutes convulsing.
2 Convulsions in infants may present as apnoeic spells or loss of awareness.
3 Remember the importance of hypoglycaemia in both convulsions and coma in infancy and childhood.
4 Most convulsions with a fever in children aged from six months to five years are ‘febrile convulsions’, but meningitis should be actively considered.
5 A high clinical awareness for the possibility of meningitis should be maintained when assessing any infant or young child who is unwell, lethargic, irritable, feverish, or vomiting.
Long-term vascular access
Authors: Conlon Peter J , Schwab Steve J 
Published: October 2023

Abstract
Despite the enormous technological advances in dialysis since its inception 35 years ago, there continues to be a very high annual mortality for patients with end-stage renal disease (ESRD). It is an extremely small minority of patients who develop ESRD who remain on life-sustaining hemodialysis for longer than 10 years. In the United States of America the annual mortality from ESRD approaches 20%. After 10 years ofESRD the majority of patients will have either have succumbed to a com¬ plication of the disease process which caused their kidneys to fail, will have died from a complication of uremia, or will have undergone renal transplantation. Table 12.l summarizes the US Renal Data System results for 10-year survival on dialysis with censoring at the time of renal transplantation. It can be seen that only a little over 4% of patients who start dialysis between the ages of 65 to 74 years will be alive10 years later. Overall 2.5% of patients will still be on dialysis 10 years after starting hemodialysis, with black patients probably faring significantly better than white patients and females faring slightly better than males. Patients with diabetes as the primary cause of renal disease have the poorest prognosis, with less than 1in 50 patients surviving on dialysis to 10 years. Patients with primary kidney diseases have the best prognosis, with close to 1 in 20 patients remaining on dialysis for their 10th anniversary of initiating renal replacement therapy.
Soot, Civilization, and Neuroses
Authors: Greaves Mel 
Published: October 2023

Abstract
But even before these cancerous activities were prevalent, cancer was widely regarded as a modern disease. Nineteenth-century European doctors noted that cancer was uncommon in ‘the savage races’, by which they mostly referred to black Africans. Dr Livingstone in his missionary travels in Africa noted that the natives were troubled with fatty and fibrous tumours, but cancer itself was unknown. Similarly, nineteenth-century surgeons and physicians in Europe and the USA were impressed by the apparent rarity of cancers, and especially breast cancer, in Indians (in Calcutta for example), North Africans (in Algeria), and native Americans.
Philosophy of cancer pain management
Authors: Simpson Karen H 
Published: October 2023

Abstract
Pain relief for patients with cancer should be part of a more comprehensive management plan to address the many factors affecting their quality of life (Ventifredda 1989). Pain control should encompass the concept of ‘total pain’, appreciating that pain has sensory, emotional, socioeconomic, and spiritual components. The World Health Organization developed its Analgesic Ladder to allow information on simple methods of pain control to be widely disseminated. This had a huge global impact. However, strong opioids are still not available to some of the world’s population. Education and legislation to improve this situation is essential.
Neurosurgical pain management
Authors: Hassenbusch Samuel J , Dickens Meredith 
Published: October 2023

Abstract
Neurosurgical procedures have generally been used relatively late in the overall management of patients; however, earlier use is now suggested. Although many intracranial procedures are old, recent advances have renewed interest in their use. Some of these improvements, such as focused radiotherapy, allow almost non-invasive interventional pain techniques at the intracranial level. The role of spinal ablative procedures, which is relatively low risk, remains stable, in overall pain management. Single procedures, performed under local anaesthesia with short hospital admissions and low morbidity, are now useful.
Anaesthesia and Analgesia in Emergency Medicine, Second Edition
Authors: Illingworth Karen A , Simpson Karen H , Swales Sue 
Published: October 2023

Abstract
Many patients come to the Accident and Emergency department in pain or with conditions requiring local or general anaesthesia. Close co-operation between the staff of the Anaesthesia and Accident and Emergency departments is vital if anaesthesia and analgesia are to be dealt with efficiently and safely. This new edition of Anaesthesia and Analgesia in Emergency Medicine has been extensively revised throughout, especially with regard to resuscitation techniques. It provides a concise guide to anaesthesia and pain relief in Emergency Medicine. Throughout, the emphasis is on the practical management of problems, giving clear instructions about the treatment of common conditions. * Features the new 1997 European Resuscitation Council Guidelines * Some new sections included * Revised throughout The book is written for staff working in an Accident and Emergency department, but will also be of interest to those training in anaesthesia Reviews of first edition: 'The authors are to be congratulated on their efforts in producing a worthwhile addition to the trauma anesthesia literature...' (Anaesthesia and Analgesia, 1995) 'The book contains an enormous assortment of very valuable information, nearly all of it in a very clear and concisely presented manner'. (Today's Anaesthetist, 1994) 'The information this book contains will benefit all staff involved in emergency medicine' ( Journal of the British Association for Immediate Care, 1994)
Problems encountered during general anaesthesia
Authors: Illingworth Karen A , Simpson Karen H , Swales Sue 
Published: October 2023

Abstract
All problems occurring during anaesthesia must be managed logically and systematically. Assessment and treatment of priorities as airway, breathing, and circulation (‘ABC’) is fundamental to safe practice. The response to a problem partly depends upon the experience of the anaethetist. It is important to have adequate trained assistance and to summon senior help quickly. Careful preoperative assessment often predicts potential difficulties. In the event of a problem, it is important to communicate with the patient, their carers, and other staff afterwards. In some circumstances, it is essential to inform the patient’s general practitioner of the situation.
Sedation
Authors: Illingworth Karen A , Simpson Karen H , Swales Sue 
Published: October 2023

Abstract
The combination of sedation and analgesic drugs is potentially hazardous and should only be used by doctors trained in the management of unconscious patients. Monitoring and resuscitation equipment must be available when sedation is given. Patients who have received sedation should recover in a properly staffed and equipped area. It is unusual for severe agitation to respond to sedation alone. Before using drugs to calm such a patient a diagnosis should be made. Consider hypoxaemia, hypoglycaemia, urinary retention, or cerebral problems such as a head injury.
Local anaesthetic nerve blocks: local infiltration of the skin
Authors: Illingworth Karen A , Simpson Karen H , Swales Sue 
Published: October 2023

Abstract
Suturing lacerations Minor surgical procedures (e.g. excision of cyst) Supplementing a nerve block Cleaning and debriding wounds Venous cannulation, using 1 per cent lignocaine administered subcutaneously is less painful than with 18, 20 or 22 Guage cannulae alone.
Nerve blocks of the upper limb
Authors: Illingworth Karen A , Simpson Karen H , Swales Sue 
Published: October 2023

Abstract
Brachial plexus block can be used for surgery or manipulation of a fracture and/or dislocation in one upper limb (bilateral block is not recommended). It can also be used to produce vasodilatation of the upper limb after intra-arterial injection of irritants. It is useful when either the use of a tourniquet is contraindicated or it would need releasing during the procedure. It provides better postoperative pain relief than IVRA. There are three approaches to the brachial plexus: axillary, subclavian perivascular, and interscalene. Plasma local anaesthetic levels are similar with all three techniques.
Design issues for randomized trials
Authors: Girling David J , Parmar Mahesh K B , Stenning Sally P , Stephens Richard J , Stewart Lesley A 
Published: October 2023

Abstract
In this chapter we describe the main design issues specific to randomized trials; these include issues of trial philosophy as well as the choices to be made concerning the number of arms, the number and timing of randomizations and the choice of control arm. The chapter ends with a section on the practical methodology of randomization.