bluuebunny/crossref_metadata_2025_split huggingface dataset:
P072 Lobstein's disease: about 12 observations
Authors: Jomaa Olfa , Mahbouba Jguirim , Saoussen Zrour , Ismail Bejia , Monji Touzi , Bergaoui Naceur 
Published: November 2021

Abstract

Background
Osteogenesis imperfecta (OI), or Lobstein's disease, is a rare inherited disorder characterized by bone fragility and low bone mass. The symptoms are diverse, with varying severity of skeletal signs and inconstant extra-skeletal signs. Type 1 is the most frequent form (about 60% of cases). Our objective is to describe the different clinical pictures hospitalized in our department during the last 15 years.

Patients and methods
This is a retrospective descriptive study including 12 patients followed for OI, hospitalized in the rheumatology department of Fattouma Bourguiba University Hospital Monastir TUNISIA between 2006 and 2019. The records were collected and analyzed.

Results
There were 9 boys and 3 girls with a mean age of 14.9 ± 8.6 years. Consanguinity was reported in 25% of cases. The chief reason for consultation was recurrent fractures (75%), blue sclera (16.7), and bone deformation (8.3%). The average number of anterior fractures was 5, with low-energy trauma as the mechanism in all cases. Similar cases in the family were noted in 41.6% of cases, in siblings in 25% of cases. The mean age of the first fracture was 4.41 ± 3.2 years. The most frequent fracture sites were respectively: femur (7/12), leg (6/12), tibia (3/12), humerus (4/12), ankle (2/12), and forearm (2/12). The deformity was noted in 58.3% of cases: lumbar kyphosis (2), exaggerated dorsal kyphosis (2), bracketed femurs (2), and anarchic deformity of 2 lower limbs (1). Dentinogenesis imperfecta was found in 8.3% of cases, while ENT examination revealed a conductive and sensorineural hearing loss in 2 patients each. The main radiological abnormalities were diffuse bone demineralization in 9 patients, thinning of the cortical bone in 5 patients, vertebral fractures in 3 patients, and fracture in 2 patients. Bone densitometry showed a mean Z-score of 3.49 ± 1.4 in the lumbar spine. The mean blood calcium level was 2.38 ± 1.15, alkaline phosphatase was elevated in all cases with a mean of 756 ± 624.9, and vitamin D levels were deficient in all cases with a mean of 22.75 ± 5.3. In addition to vitamin-calcium supplementation, all patients received intravenous pamidronate at a dose of 9 mg/kg/year with an average of 6 courses. The main side effects noted at the time of infusion were abdominal pain, polyarthralgia and asthenia (1 patient), chest pain (1 patient), and fever and chills (1 patient). Control bone densitometry showed a mean Z score of 1.81 ± 1.2 in the lumbar spine.

Conclusions
Osteogenesis imperfecta is a severe disease with a risk of serious complications t. In the absence of etiological treatment, symptomatic management must be both early and multidisciplinary.
P077 Ultrasound features of a traumatic hip pain in paediatric population
Authors: Bettaieb Hiba , Affess Hassene , Ferjani Hanene , Maatallah Kaouther , Ben Nessib Dorra , Triki Wafa , Kaffel Dhia , Hamdi Wafa 
Published: November 2021

Abstract

Background
Atraumatic hip pain in children is a serious condition that may require urgent investigations. Conventional radiographs are often normal in early stage of the diseases. However, further imaging techniques such as hip ultrasonography (US) are needed. The main objective of this study is to describe Hip ultrasound findings in patients with atraumatic hip pain.

Methods
We conduct a retrospective monocentric study. Children with atraumatic hip pain and undergoing joint ultrasound were included. Sociodemographic, clinical and biological characteristics were collected. The data were analyzed using the SPSS statistical package. Results:
Thirty patients (21 boys and 9 girls) were enrolled. The male to female sex ratio was 2.3. Mean age at disease onset was 9.1 ± 4.6 [1.3–16]. Median diagnosis delay from symptoms onset was 1.2 months [0.2–48]. Chief complaints were as following: 80% of inflammatory hip pain (n = 24), 20% of mechanical hip pain (n = 6) 76.7% of limping (n = 23), 23.3% of functional impotence (n = 7) and 23.3% of fever (n = 7). Physical examination noted a pain on passive joint mobilization and restriction of hip movement in 90% (n = 27) and 54% (n = 18) of cases respectively. A biologic inflammatory syndrome was found in 66.6% of cases (n = 20). Median ESR and CRP were 30 mm/h [2–101] and 4.5 mg/l [1–38] respectively. Hip X-ray was abnormal in 46.6% (n = 14) of cases. All patients had hip ultrasound. US findings were as following: 80% (n = 24) of joint effusion, 50% (n = 15) of synovial thickening, 36.7% (n = 11) of synovitis and 23.3% (n = 7) of positive power Doppler signal. Hip Ultrasound was normal in 13.3% (n = 4) of cases. Causes of hip pain were in decreasing order of frequency: septic arthritis (n = 10), juvenile idiopathic arthritis (n = 10), transient synovitis (n = 7), osteoid osteoma (n = 2) and osteosarcoma (n = 1).

Conclusion
Hip Ultrasonography is a noninvasive and useful screening technique for the management of atraumatic hip pain in children. Though, imaging features are often various and nonspecific. Radiologists should be more familiar with sonographic anatomy of infant hip.
P087 Polyarteritis nodosa: a case report
Authors: Azirar A , Ghannam A , Elaouli A , Rkain M , Benaajiba N , Babakhouya A 
Published: November 2021

Abstract

Background
Polyarteritis nodosa is a necrotizing vasculitis of small and medium caliber arteries. Rarely described in children, its pathophysiology is complex and remains poorly elucidated. Two main forms were described in the literature: cutaneous and visceral.

Material and results
We report the cases of two boys, aged of 7 and 11 years old. They had a history of recurrent sore throat with a recurrent aphthosis in the 11-year-old child. Both boys presented with polyarthralgias, myalgias, polymorphous skin lesions made of livedo reticularis with subcutaneous nodosa on the lower limbs in the first child and distal necrosis of the toes with oedema of the lower limbs in the second. Patients suffered also of cough evolving in a context of alteration of the general state made of fever and asthenia. A biological inflammatory syndrome was present in both children and the skin histology confirmed periarteritis nodosa. The evolution was marked by the disappearance of arthralgias and myalgias after a corticosteroid-based treatment with progressive dose reduction until minimal effective dose.

Conclusion
The diagnosis of Polyarteritis nodosa should be made in any child presenting with the following signs: fever, altered general condition, myalgias, arthralgias and skin manifestations. The prognosis is usually benign but long-term surveillance is necessary.
P088 Prolonged fever in a boy revealing Takayasu disease: a case report
Authors: Asbik O , Babakhoya A , Rkain M , El Ouali A , Ghanam A , Benajiba N 
Published: November 2021

Abstract

Background
Takayasu's arteritis (TA) is a chronic inflammatory vasculitis of unknown origin. It affects the large vessels, especially the aorta, its main branches, and the pulmonary arteries. It begins acutely in children with severe general manifestations. The diagnosis of TA remains a challenge to clinicians due to many reasons such as its rarity, its great clinical polymorphism, and the lack of specific biological criteria.

Case report
A 13-years old boy with no pathological history, was admitted for prolonged fever with intermittent abdominal pain. The clinical examination has found a conscious boy, febrile with a BP of 130/90mmhg, a normal heart rate, and stable respiratory function. The cardiovascular examination noted a decrease in pedal pulses, without signs of ischaemia or necrosis, and the cardiac auscultation was normal. Examination of the lymph node areas has shown upper and left later cervical adenopathy measuring 1.7 cm in long axis, mobile, and painless. All joints were free. The complementary biological workups revealed an inflammatory syndrome (ESR: 120 mm, fibrinogen: 5 g/l, microcytic hypochromic anaemia at 9 g/dl, ferritin: 1051, low serum iron: 11µg/dl and thrombocytosis: 692 000 elements/l). Chest X-ray showed a dilated aortic button. A thoracic angioscan revealed a peri-aortitis more evident at the level of the emergence of the mesenteric artery with a pseudo-aneurysmal aspect and arteritis of the right primary carotid artery. Echocardiography was performed and showed a dilated aspect of the aorta with irregular wall. Renal ultrasound was normal. The diagnosis of Takayasu disease was made on the basis of clinical biological and essentially radiological arguments according to the diagnostic criteria of the American College of Rheumatology. The patient was treated with corticosteroid (prednisone: 2 mg/kg/d) for one month then then gradually reduced doses The disease course was marked by several relapses and the patient was then treated with mycophenolate mofetil (cellcept).

Conclusion
Takaysu's disease is a chronic inflammatory vasculitis of unknown origin, which affects the large- vessels, mainly the aorta, its main branches, and the pulmonary arteries; but it remains rare in children and its treatment is not well codified.
P093 Algodystrophy in children: a case report
Authors: Nait Abdallah M S , Boutalbi N , Boulekhssaim I , Kachi A , Boutabia A W , Bouchair N 
Published: November 2021

Abstract

Background
Algodystrophy is an entity very often unrecognized by pediatricians. The evolution is readily dragging and disabling in the absence of early and coordinated care. The Objective: Based on a case observed in a 10-year-old boy, the authors recall the main characteristics of the syndrome, guiding the practitioner to make the diagnosis, and implement appropriate and early therapy.

Observation
A 10-year-old boy with no previous medical history, presenting with neuropathic-like pain in the wrist and the hand that started a month ago without any triggers. Clinical exam noted functional impotence, a whole limb tremor, hyperesthesia as well as allodynia.
The patient's hand is swollen and warm with a claw-like appearance.
The rest of the body exam is normal.
The laboratory markers and x-rays of the whole limb are normal. Bone scintigraphy confirms the diagnosis of wrist algodystrophy. The treatment combining corticosteroids, functional rehabilitation, and psychotherapy allowed a favorable outcome with no sequelae.

Conclusion
The diagnosis of algodystrophy remains too often unrecognized, and its management is sometimes inadequate. Early diagnosis and treatment improve the prognosis.
P097 Diagnostic dilemma of acquired non-traumatic spinal deformation: Pott's disease; histiocytosis or psoriatic rheumatism? About a case
Authors: Fadila Bendaoud , Hassen Mouffouk , Sara Ouzani , Saida Brahmi 
Published: November 2021

Abstract

Background
Non-traumatic acquired spinal deformities represent a separate entity within the axial pathologies in children. Their causes are variable (neurological, muscular, infectious, especially Pott's disease, inflammatory or tumoral).
The etiological diagnosis can be clinically oriented by pain, medical history, especially rheumatic. The contribution of the CT scan is crucial, as it localizes the lesion, specifies its nature, but above all it helps the histological diagnosis by guided biopsies.
Our case illustrates the difficulties in diagnosing the aetiology of spinal acquired deformity in a girl with a multi-evocative context.

Observation
A 14 years-old girl complains for a few months of a dorso-lumbar spinal pain and a vicious attitude. Her medical history includes femoral thrombophlebitis and psoriasis. She had no trauma or fever.
Clinically, the general state is preserved, with no local inflammatory signs, a kyphotic attitude with an analgesic lumbar support was noted. The rest of the locomotor exam was normal.
CT scan shows cancellous body geodes with a double mirror.

Discussion
Acquired spinal deformities are always secondary to general disease. The fever may point to bacterial infections, especially tuberculosis (Pott's disease), but also non-infectious causes such as a rheumatic disease.
For our patient, psoriasis argues for the latter possibility. However, the rarity of isolated disco-vertebral involvement and the severity of the lytic lesions are against this diagnosis. The histopathological exam showed an eosinophilic granuloma, confirming the histiocytosis X diagnosis. The cortico-response strengthen the diagnosis.
The course was marked by the bone remodeling with the posture correction.

Conclusion
A clinical, radiological, and histological analysis allow the etiological orientation in front of acquired spinal deformity.
Risk of chronic kidney disease in patients with obstructive sleep apnea
Authors: Beaudin Andrew E , Raneri Jill K , Ahmed Sofia B , Hirsch Allen A J Marcus , Nocon Andrhea , Gomes Teresa , Gakwaya Simon , Series Fréderic , Kimoff John , Skomro Robert P , Ayas Najib T , Hanly Patrick J 
Published: November 2021

AbstractStudy ObjectivesChronic kidney disease (CKD) is a global health concern and a major risk factor for cardiovascular morbidity and mortality. Obstructive sleep apnea (OSA) may exacerbate this risk by contributing to the development of CKD. This study investigated the prevalence and patient awareness of the risk of CKD progression in individuals with OSA.MethodsAdults referred to five Canadian academic sleep centers for suspected OSA completed a questionnaire, a home sleep apnea test or in-lab polysomnography and provided blood and urine samples for measurement of estimated glomerular filtration rate (eGFR) and the albumin:creatinine ratio (ACR), respectively. The risk of CKD progression was estimated from a heat map incorporating both eGFR and ACR.Results1295 adults (42% female, 54 ± 13 years) were categorized based on the oxygen desaturation index (4% desaturation): &lt;15 (no/mild OSA, n = 552), 15−30 (moderate OSA, n = 322), and &gt;30 (severe OSA, n = 421). After stratification, 13.6% of the no/mild OSA group, 28.9% of the moderate OSA group, and 30.9% of the severe OSA group had a moderate-to-very high risk of CKD progression (p &lt; .001), which was defined as an eGFR &lt;60 mL/min/1.73 m2, an ACR ≥3 mg/mmol, or both. Compared to those with no/mild OSA, the odds ratio for moderate-to-very high risk of CKD progression was 2.63 (95% CI: 1.79−3.85) for moderate OSA and 2.96 (2.04–4.30) for severe OSA after adjustment for CKD risk factors. Among patients at increased risk of CKD progression, 73% were unaware they had abnormal kidney function.ConclusionPatients with moderate and severe OSA have an increased risk of CKD progression independent of other CKD risk factors; most patients are unaware of this increased risk.
Age and Workplace Deviance: A Meta-Analytic Test and a Trait-Based Examination of Why Older Employees Engage in Less Workplace Deviance
Authors: Pletzer Jan Luca , Oostrom Janneke K , Voelpel Sven C 
Published: November 2021

AbstractIn the current meta-analysis, we examine the relation between age and workplace deviance, and find a small but significant negative correlation (ρ¯ = −.124, k = 198). More importantly, we test several trait-based mechanisms to help explain this relation. Specifically, based on the neo-socioanalytical model of personality change, we hypothesized that those Big Five personality traits that change with age, HEXACO honesty–humility, and trait negative affect mediate this relation. These hypotheses were supported, as the Big Five traits conscientiousness, agreeableness, and neuroticism, as well as honesty–humility and trait negative affect simultaneously mediated the negative relation between age and workplace deviance. These findings highlight important underlying mechanisms for this relation and suggest several opportunities for organizations to reduce the occurrence of workplace deviance. Further theoretical and practical implications as well as limitations and future research ideas are discussed.
Holistic Admissions Review in Nursing Education: A Concept Analysis Using Rodgers’ Evolutionary Method
Authors: Hackett Janna M. , Ruyak Sharon L. 
Published: November 2021

Abstract

AIM
The aim of this study was to analyze holistic review within the context of collegiate nursing program admissions.

BACKGROUND
Holistic review is the evaluation of individual qualifications for college admission based on a pattern of individualized factors, including standardized scores, personal characteristics, experiences, abilities, and educational/professional “fit.” Medical schools regularly use holistic review, and more nursing schools may adopt holistic review in the future.

METHOD
Rodgers’ evolutionary method of concept analysis was used to examine usage in current praxis.

RESULTS
Findings support a desire to enhance diversity in collegiate, health-related programs while increasing numbers of culturally competent, qualified providers representative of the US population.

CONCLUSION
Holistic review promotes diversity of nursing students and future nurse providers. This concept analysis provides a unified definition of holistic review to promote educational and health-care-related equity through increased representativeness of nursing students and future nurses.
Nanoliposomal irinotecan with 5-fluorouracil and folinic acid in metastatic pancreatic cancer after previous gemcitabine-based therapy: A real-world experience
Authors: Yu Hung-Yuan , Lee Chun-Yang , Lin Le-Gin , Chao Yee , Li Chung-Pin 
Published: November 2021

Background:
Nanoliposomal irinotecan (nal-IRI), accompanied by 5-fluorouracil (5-FU) and leucovorin (LV), is an effective and safe therapy for patients in whom metastatic pancreatic ductal adenocarcinoma has progressed after gemcitabine-based chemotherapy. Our aim was to evaluate the effectiveness and safety of a nal-IRI + 5-FU/LV regimen for patients with metastatic pancreatic cancer and gemcitabine-based treatment failure in the real world.

Methods:
We retrospectively collected the baseline characteristics, treatment courses and dosage, treatment response, overall survival (OS), progression-free survival (PFS), and adverse effects of patients treated with the nal-IRI-based regimen at Taipei Veterans General Hospital.

Results:
Sixty-seven patients who received the nal-IRI + 5-FU/LV regimen from August 2018 to June 2019 were identified. Their median age was 65 years and 52% were male. Most patients had an Eastern Cooperative Oncology Group performance status of 0 to 1, but patients with an Eastern Cooperative Oncology Group performance status of 2 to 4 before initiation of the nal-IRI regimen were also enrolled (31%). The median dose intensity was 40.4 mg/m2 and the median treatment duration was 8.3 weeks (range: 5 days–75.7 weeks). Objective response and disease control rates were 10.4% and 38.8%, respectively. The median OS)was 7.9 months (95% confidence interval [CI]: 5.6–10.1 months) and the median PFS was 2.9 months (95% CI: 1.6–4.1 months). Elevated total bilirubin (hazard ratio [HR]: 4.31, 95% CI: 1.21–15.30, p = 0.024), carcinomatosis (HR: 3.75, 95% CI: 1.46–9.66, p = 0.006), and previous treatment with irinotecan (HR: 4.86, 95% CI: 1.67–14.10, p = 0.004) were associated with a worse OS. Previous treatment with irinotecan (HR: 3.03, 95% CI: 1.22–7.49, p = 0.02) was associated with a worse PFS. The most common all-grade adverse effects were anemia (73.9%), nausea (66.2%), and fatigue (61.5%). The most common grade 3–4 adverse effects were neutropenia (21.5%), anemia (18.5%), and diarrhea (15.4%).

Conclusion:
Clinically, nal-IRI + 5-FU/LV is effective and tolerable at reduced doses in patients with metastatic pancreatic adenocarcinoma that has progressed after gemcitabine-based therapy
Using mobile phone data to estimate dynamic population changes and improve the understanding of a pandemic: A case study in Andorra
Authors: Berke Alex , Doorley Ronan , Alonso Luis , Arroyo Vanesa , Pons Marc , Larson Kent 
Published: November 2021

AbstractCompartmental models are often used to understand and predict the progression of an infectious disease such as COVID-19. The most basic of these models consider the total population of a region to be closed. Many incorporate human mobility into their transmission dynamics, usually based on static and aggregated data. However, mobility can change dramatically during a global pandemic as seen with COVID-19, making static data unsuitable. Recently, large mobility datasets derived from mobile devices have been used, along with COVID-19 infections data, to better understand the relationship between mobility and COVID-19. However, studies to date have relied on data that represent only a fraction of their target populations, and the data from mobile devices have been used for measuring mobility within the study region, without considering changes to the population as people enter and leave the region.This work presents a unique case study in Andorra, with comprehensive datasets that include telecoms data covering 100% of mobile subscribers in the country, and results from a serology testing program that more than 90% of the population voluntarily participated in. We use the telecoms data to both measure mobility within the country and to provide a real-time census of people entering, leaving and remaining in the country. We develop multiple SEIR (compartmental) models parameterized on these metrics and show how dynamic population metrics can improve the models. We find that total daily trips did not have predictive value in the SEIR models while country entrances did. As a secondary contribution of this work, we show how Andorra’s serology testing program was likely impacted by people leaving the country. Overall, this case study suggests how using mobile phone data to measure dynamic population changes could improve studies that rely on more commonly used mobility metrics and the overall understanding of a pandemic.