bluuebunny/crossref_metadata_2025_split huggingface dataset:
Phosphate matters when investigating hypercalcemia: a mutation in SLC34A3 causing HHRH
Authors: Tang Andrew R , Hinz Laura E , Khan Aneal , Kline Gregory A 
Published: July 2019

Summary
Hereditary hypophosphatemic rickets with hypercalciuria (HHRH) is a rare, autosomal recessive disorder caused by mutations in the SLC34A3 gene that encodes the renal sodium-dependent phosphate cotransporter 2c (NaPi-IIc). It may present as intermittent mild hypercalcemia which may attract initial diagnostic attention but appreciation of concomitant hypophosphatemia is critical for consideration of the necessary diagnostic approach. A 21-year-old woman was assessed by adult endocrinology for low bone mass. She initially presented age two with short stature, nephrocalcinosis and mild intermittent hypercalcemia with hypercalciuria. She had no evidence of medullary sponge kidney or Fanconi syndrome and no bone deformities, pain or fractures. She had recurrent episodes of nephrolithiasis. In childhood, she was treated with hydrochlorothiazide to reduce urinary calcium. Upon review of prior investigations, she had persistent hypophosphatemia with phosphaturia, low PTH and a high-normal calcitriol. A diagnosis of HHRH was suspected and genetic testing confirmed a homozygous c.1483G>A (p.G495R) missense mutation of the SLC34A3 gene. She was started on oral phosphate replacement which normalized her serum phosphate, serum calcium and urine calcium levels over the subsequent 5 years. HHRH is an autosomal recessive condition that causes decreased renal reabsorption of phosphate, leading to hyperphosphaturia, hypophosphatemia and PTH-independent hypercalcemia due to the physiologic increase in calcitriol which also promotes hypercalciuria. Classically, patients present in childhood with bone pain, vitamin D-independent rickets and growth delay. This case of a SLC34A3 mutation illustrates the importance of investigating chronic hypophosphatemia even in the presence of other more common electrolyte abnormalities.

Learning points:

Hypophosphatemia is an important diagnostic clue that should not be ignored, even in the face of more common electrolyte disorders.

HHRH is a cause of PTH-independent hypophosphatemia that may also show hypercalcemia.

HHRH is a cause of hypophosphatemic nephrocalcinosis that should not be treated with calcitriol, unlike other congenital phosphate wasting syndromes.

Some congenital phosphate wasting disorders may not present until adolescence or early adulthood.
Treatment and long-term outcomes in pituitary carcinoma: a cohort study
Authors: Santos-Pinheiro Fernando , Penas-Prado Marta , Kamiya-Matsuoka Carlos , Waguespack Steven G , Mahajan Anita , Brown Paul D , Shah Komal B , Fuller Gregory N , McCutcheon Ian E 
Published: July 2019

Background
Pituitary carcinoma (PC) is an aggressive neuroendocrine tumor diagnosed when a pituitary adenoma (PA) becomes metastatic. PCs are typically resistant to therapy and develop multiple recurrences despite surgery, radiotherapy and chemotherapy. Recently, treatment with temozolomide (TMZ) has shown promising results, although the lack of prospective trials limits assessment of benefit.

Methods
We describe a single-center multidisciplinary experience in managing PC patients over a 22-year period and review previously published PC series.

Results
Seventeen patients were identified. Median age at PC diagnosis was 44 years (range 16–82 years), and the median time from PA to PC transformation was 5 years (range 1–29 years). Median follow-up time was 28 months. Most PCs were hormone-positive (n = 12): ACTH (n = 5), PRL (n = 4), LH/FSH (n = 2) and GH (n = 1). All patients underwent at least one resection and at least one course of radiation after PC diagnosis. Immunohistochemistry showed high Ki-67 labeling index (>3%) in 10/15 cases. Eight patients (47%) had only central nervous system (CNS) metastases; six (35%) had combined CNS and systemic metastases. The most commonly used chemotherapy was TMZ, and TMZ-based therapy was associated with the longest PFS in 12 (71%) cases, as well as the longest period from PC diagnosis to first progression (median 30 months). The 2, 3 and 5-year survival rate of the entire cohort was 71, 59 and 35%, respectively. All patients surviving >5 years had been treated with TMZ-based therapy.

Conclusions
PC management benefits from multidisciplinary care and multimodality therapy. TMZ-based regimens were associated with high survival rates and long disease control.
Pediatric Respiratory Illness Measurement System (PRIMES) Scores and Outcomes
Authors: Mangione-Smith Rita , Zhou Chuan , Williams Derek J. , Johnson David P. , Kenyon Chén C. , Tyler Amy , Quinonez Ricardo , Vachani Joyee , McGalliard Julie , Tieder Joel S. , Simon Tamara D. , Wilson Karen M. ,   for the Pediatric Research in Inpatient Settings (PRIS) Network
Published: July 2019

BACKGROUND AND OBJECTIVES:
The Pediatric Respiratory Illness Measurement System (PRIMES) generates condition-specific composite quality scores for asthma, bronchiolitis, croup, and pneumonia in hospital-based settings. We sought to determine if higher PRIMES composite scores are associated with improved health-related quality of life, decreased length of stay (LOS), and decreased reuse.

METHODS:
We conducted a prospective cohort study of 2334 children in 5 children’s hospitals between July 2014 and June 2016. Surveys administered on admission and 2 to 6 weeks postdischarge assessed the Pediatric Quality of Life Inventory (PedsQL). Using medical records data, 3 PRIMES scores were calculated (0–100 scale; higher scores = improved adherence) for each condition: an overall composite (including all quality indicators for the condition), an overuse composite (including only indicators for care that should not be provided [eg, chest radiographs for bronchiolitis]), and an underuse composite (including only indicators for care that should be provided [eg, dexamethasone for croup]). Multivariable models assessed relationships between PRIMES composite scores and (1) PedsQL improvement, (2) LOS, and (3) 30-day reuse.

RESULTS:
For every 10-point increase in PRIMES overuse composite scores, LOS decreased by 8.8 hours (95% confidence interval [CI] −11.6 to −6.1) for bronchiolitis, 3.1 hours (95% CI −5.5 to −1.0) for asthma, and 2.0 hours (95% CI −3.9 to −0.1) for croup. Bronchiolitis overall composite scores were also associated with shorter LOS. PRIMES composites were not associated with PedsQL improvement or reuse.

CONCLUSIONS:
Better performance on some PRIMES condition-specific composite measures is associated with decreased LOS, with scores on overuse quality indicators being a primary driver of this relationship.
Susceptibility and damage cereals to infestation Rhyzopertha dominica (F.) (Coleoptera: Bostrichidae) in storage
Authors: Hendrival Hendrival , Afriani Dhea , Aryani Dewi Sartika 
Published: July 2019

Rhyzopertha dominica is a primary pest which causes damages to stored cereals such as corn, grain, rice, wheat, sorghum, tubers, and starch-containing substrates and packaging made from wood. They not only cause losses in terms of quantity but also affect quality during storage period through their feeding activities. The aimed of this research to investigate the level of susceptibility and damage to cereals during the storage period by pest R. dominica. The cereals used in the study were sorghum, wheat, corn, rice grain, white rice, black glutinous rice, and white glutinous rice. The method used in this research was no-choice bioassay with variables observed: number of F1 progeny, median development time and damage cereals. The results revealed that that different kind of cereals had a different level of susceptibility to infestation by R. dominica from moderate to susceptible. Sorghum, corn, rice, white rice, and white glutinous rice were classified as moderate, while wheat was classified as susceptible and black glutinous rice was moderate–susceptible. The highest losses were found in wheat, black glutinous rice, corn, and rice grain while the lowest were found in sorghum, white rice and white glutinous rice. The study showed that cereals that were susceptible may not stored for a long time to minimize the loss of grain weight.ABSTRAK Rhyzopertha dominica tergolong hama primer yang merusak serealia seperti jagung, gabah, beras, gandum, sorgum, umbi, dan substrat mengandung pati serta kemasan yang terbuat dari kayu. Kerusakan serealia selama penyimpanan meliputi kerusakan kuantitas dan kualitas yang disebabkan oleh hama R. dominica. Penelitian bertujuan untuk mengetahui tingkat kerentanan dan kerusakan serealia selama penyimpanan oleh hama R. dominica. Jenis serealia yang digunakan dalam penelitian terdiri dari sorgum, gandum, jagung, gabah, beras putih, beras ketan hitam, dan beras ketan putih. Metode uji tanpa pilihan dengan variabel yang diamati yaitu jumlah F1, median waktu perkembangan, dan kerusakan serealia. Hasil penelitian menunjukkan bahwa jenis serealia memiliki tingkat kerentanan dari moderat sampai rentan terhadap infestasi hama R. dominica. Serealia dari sorgum, jagung, padi, beras putih, dan beras ketan putih tergolong dalam kategori moderat, sedangkan gandum tergolong dalam kategori rentan, serta beras ketan hitam tergolong moderat–rentan terhadap serangan R. dominica. Tingkat kerusakan serealia seperti persentase serealia berlubang dan persentase bubuk serealia paling tinggi terjadi pada gandum, beras ketan hitam, jagung dan padi sedangkan kerusakan yang terendah yaitu pada sorgum, beras putih, dan beras ketan putih. Penelitian menunjukkan bahwa serealia yang rentan tidak boleh disimpan dalam waktu yang lama untuk meminimalkan kehilangan berat.
Respons fisiologi pertumbuhan dan hasil tiga genotip jawawut terhadap cekaman kekeringan
Authors: Yuwariah Yuyun , Dewi Sheli Mustikasari , Qosim Warid Ali , Nuraini Anne 
Published: July 2019

Jawawut merupakan salah satu tanaman pangan lokal Indonesia yang belum banyak dikembangkan dan dimanfaatkan sebagai sumber pangan. Penelitian ini bertujuan untuk mendapatkan genotip jawawut yang memberikan pengaruh paling baik terhadap pertumbuhan dan hasil pada berbagai tingkat pemberian air di rumah plastik. Penelitian dilaksanakan pada bulan Juni sampai dengan September 2017 di Kebun Percobaan Fakultas Pertanian, Universitas Padjadjaran. Penelitian menggunakan Rancangan Petak Terbagi (Split Plot Design) dengan 3 ulangan. Petak utama terdiri dari tiga macam genotip yaitu genotip 44, 46, dan 48. Anak petak terdiri dari tiga taraf kapasitas lapang yaitu 75%, 50% dan 25%. Hasil penelitian menunjukkan perlakuan pemberian air 25% kapasitas lapang berpengaruh paling buruk terhadap proses fisiologis pertumbuhan dan hasil tiga genotip jawawut. Genotip 44 dan 46, pada pemberian air 50 % KL menghasilkan konduktan stomata terbaik. Genotip 46 dan 48 memberikan pengaruh paling baik terhadap proses fisiologis pertumbuhan dan hasil jawawut yaitu jumlah anakan per rumpun.ABSTRACTMillet is one of Indonesia's local food crops that has not been widely developed as food sources. The purpose of this study was to obtain the genotypes of millet which gave the best effect on the growth and yield at various levels of water supply in the plastic house. The study was conducted from June to September 2017 at the Experimental Station of the Faculty of Agriculture, Universitas Padjadjaran. The research used the Split Plot Design with three replications. The main plot factor consisted of three levels of treatment; genotypes 44, 46, and 48. The subplot factor consisted of three stages of different treatment of the field capacity; 75%, 50% and 25%. The results showed the treatment of 25% water to field capacity had the worst effect on the physiological process of growth and yield of three millet genotypes. Genotypes 44 and 46, at 50% field capacity, produced the best stomatal conductance. Genotype 46 and 48 showed the best response to the physiological processes for the number of tillers.
Recurrent deep vein thrombosis in children with malignant blood diseases: literature review
Authors: Morozova D. S. , Evstratov D. A. , Zharkov P. A. 
Published: July 2019

Venous thromboembolism is not a rare complication in children with cancer. Despite the advantages of the treatment of venous thromboembolism there is still a probability of venous thromboembolism recurrence. In adult patients with cancer venous thromboembolism recurrence an influence on the lower survival rate. In children with cancer venous thromboembolism recurrence is a rare complication, but it can significantly reduce the quality of life. Risk factors of venous thromboembolism recurrence in children with cancer are not properly investigated.
Practice towards disposal of medicines (unused/expired drugs) among the patients visiting tertiary care teaching hospital in Haryana, India
Authors: Narwat Arvind , Sindhu Anjali 
Published: July 2019

Background: Awareness towards improper disposal of medicines and its hazardous impact on environment is one of the major issues which we are neglecting but needs to be focused. In general, expired medicines/waste medicines/ left out medicines are disposed in the garbage or, rinse in sink or flushed down toilet, which ends up in the water system and have a potential effect on ecosystem. With this background, the present study was conducted to assess the practice towards disposal of medicines (Unused/Expired Drugs) among the patients visiting tertiary care teaching hospital in Haryana.Methods: This study was a descriptive, cross-sectional study conducted through face-to-face interviews by using a validated structured Questionnaire in patients attending the OPD of PGIMS, Rohtak, Haryana. A total of 150 patients participated in this study.Results: Among the 150 Patients participated in the study, 94 were male and 56 were female. Most common class of medicines used were NSAIDS (34%). About the practice of disposal of medicines 62% of them threw into the dustbin (household garbage) 18% into the toilet/sink. About consequences of improper disposal (55%) of them answered that they were not aware. The most common reason for left out medicines in home was incompletion of the course due to getting relieved from disease (50%). Most common way of procuring medicine was purchased on prescription and surprisingly only 30% participants checked the expiry date of medicine before procuring. Among the participants 75% said that government should be responsible to create awareness for proper disposal of unused medicines.Conclusions: The present study brought out the current status and lacuna towards the disposal of medicines. Patients do not use all the medications and there is lack of knowledge about awareness of disposal of medicines and its consequences.
Unusual paraneoplastic syndrome of inappropriate antidiuretic hormone secretion with gastric cancer
Authors: Pai Ananth , Pervin Saniya 
Published: July 2019

Syndrome of inappropriate antidiuretic hormone secretion (SIADH) results in impaired water excretion and consequent water intoxication and hyponatremia. In patients with cancer, SIADH is most commonly driven by ectopic ADH production, a paraneoplastic syndrome frequently seen in small cell lung cancer. A 63 year old male presented with loss of appetite, vomiting and hiccups. No abnormalities were detected on physical examination. Upper gastrointestinal endoscopy showed a proliferative growth in the stomach and histopathology report confirmed gastric adenocarcinoma. His laboratory investigations revealed low serum sodium and further work up for SIADH confirmed the diagnosis. The patient was treated with hypertonic saline and he improved symptomatically. He was subsequently treated with two cycles of capecitabine and oxaliplatin. The patient was asymptomatic and maintained a serum sodium in the range of 120 to 130 mEq/L, with a serum sodium of 127 mEq/L on follow up.
Cirrhosis aggravates the ninety-day mortality after liver resection for hepatocellular carcinoma
Authors: Abdelhameed Hosam Farouk , El-Badry Ashraf M. 
Published: July 2019

Background: Ninety-day postoperative mortality (90-D POM) measures accurately the liver resection-related mortality. In cirrhotic patients, reporting post-hepatectomy-related death only as in-hospital or thirty-day postoperative mortality (30-D POM) may underestimate cirrhosis-related death after liver resection.Methods: Medical records of adult cirrhotic (cirrhosis group) and matched non-cirrhotic (control group) patients, who underwent elective liver resection at Sohag University Hospital (April 2014- March 2018), were analyzed. The 90-D POM versus in-hospital mortality and 30-D POM were compared in both groups.Results: Forty-six patients (23 per group) were eligible for the study. Liver resection was carried out in all cirrhosis group patients for hepatocellular carcinoma (HCC). In the control group, liver resection was indicated for colorectal metastasis (13), benign masses (7) and intrahepatic cholangiocarcinoma (3). Compared with the control group, cirrhotic patients exhibited significantly higher complication rates (p<0.05), prolonged hospital stays (p<0.05), increased postoperative levels of serum bilirubin and reduced prothrombin concentration (p<0.05). In the control group, in-hospital mortality and 30-D POM were zero while 90-D POM was 4%. In the cirrhosis group, the in-hospital mortality and 30-D POM were identical (8.7%), however the 90-D POM was significantly higher and almost doubled (17%). Conclusion: Liver cirrhosis triggers significant mortality that may extend for ninety days postoperatively. In cirrhotic patients, post-hepatectomy death should be reported as 90-D POM rather than the obviously misleading in-hospital mortality or 30-D POM.
Short term outcome of total knee replacement in Kashmiri population without co-morbidities
Authors: Wani M. Ahsan , Ganaiee Mushtaq Ahmed , Mir Bashir Ahmed , Sultan Asif , Islam Muneebul 
Published: July 2019

Background: The aim of the study was to evaluate the patients of osteoarthritis of knee prior to surgery and then 6 weeks after the total knee replacement surgery with regards pain, range of motion, stability, deformity, quadriceps power and support.Methods: This is a prospective follow up study. A minimum of 20 patients have been included in this study conducted in Department of Orthopedics, Bone and Joint Hospital Barzullah Srinagar, during the period of September 2014 to November 2016.Results: The average age of patients was 59.63 years, more than 35% of the patients belonged to the age group 66-70 years, majority of the patients were females (70%) in the whole study group. Most (88.89%) cases were bilateral, affecting both knees. The mean pre-operative range of motion in 94.38 degrees. The mean postoperative range of motion was 113.75 degrees. Patients were followed up at 2, 4, 6 weeks postoperatively. The average blood loss was 500 ml and the average operating time was one hour and 15 minutes and one hour. There was one deep vein thrombosis, one delayed wound healing, one common peroneal nerve palsy and one valgus deformity.Conclusions: The patients with more severely deformed knees, are more elderly, have more pain perception, their functional quality of life was impaired more, they were physically more dependent and their quality of life improves significantly after surgery.
O treinamento do ator no laboratório de criação e investigação da cena contemporânea
Authors: Ribeiro Martha de Mello 
Published: July 2019

Investiga-se uma metodologia para o aprendizado da arte do ator, mais afim a um dispositivo de treinamento pela via da respiração, desvinculada de uma formalidade acadêmica tradicional. Entende-se a respiração como escultora de afetos, geradora de intensidades de presença. O treinamento do ator por esta via é o « filo rosso » que liga a criação estética do espetáculo aos processos criativos e pedagógicos do ator.
Necessidades e recursos sociais em doentes renais crônicos hemodialisados
Authors: Olim Marta Freitas , Guadalupe Sónia , Zeferino Sara , Marques Mara , Neves Patrícia , Conceição Joana 
Published: July 2019

O estudo apresenta dados acerca da provisão social das necessidades de doentes insuficientes renais crónicos em tratamento de hemodiálise em Portugal. Estudo descritivo dos dados dos processos sociais de 1436 doentes no ano de 2017. Na maioria são do sexo masculino (60,7%) e têm idades entre os 19 e os 97 anos (M = 66,3; DP = 15,8). Foram sinalizadas 2550 necessidades, 1,78 sinalizações em média por doente. Do total, 48% apresentam necessidades de apoio material/instrumental, 31,2% evidenciaram necessidades de advocacy e de intervenção psicossocial, sendo estas e as de apoio logístico que apresentam um número mais elevado de sinalizações (M = 1,257; M = 1,431, respetivamente). Em 63,4% dos casos a resposta foi adequada. As necessidades não satisfeitas são por resposta desadequada ou por inexistência de resposta (6,2%; 5,5%), ou ainda por não adesão do doente/família (14,1%; 6,1%). Analisadas as diferenças sociodemográficas, as pessoas viúvas registam um número de necessidades superior aos outros estados civis (p < 0,01), e os doentes em famílias de acolhimento ou instituições (p < 0,01). O conhecimento das necessidades destes doentes é fulcral para o Serviço Social favorecer programas de provisão às necessidades dominantes e para grupos de especial atenção.
Education for Sustainability: Lessons from Living Systems Governance
Authors: Bricage Pierre 
Published: July 2019

Abstract
To survive that is to eat and not to be eaten. Whatever its spatial and temporal level of organisation, every living system owns 7 invariant capacities (the gauge invariance paradigm). Emerging by embedments and juxtapositions of previous systems, every living system-of-systems is both dependent and independent from its new global level of organisation and past and present local situations of emergence. Mass growth governs growth phase duration. Local actors, modules of past, present and new modules of modules become mutually integrated (percolation process) into a new Whole through their merging into an ARMSADA (Association for the Reciprocal and Mutual Sharing of Advantages and DisAdvantages). Reversely (systemic constructal law) the global Whole is integrating the local parceners. Whatever the organisation level, the living systems obey the same principle of evolution and emergence, the volume and time of generation of the adult system obey a correlation: The mass controlled duration of acquisition of the reproductive capacity and the volume at its acquisition are linked by a 3/2 exponent power law. This dynamic fractal law is invariant, between and within le- vels of organisation. Brownian motion is the basic phenomenon of growth control: Matter and energy are exchanged at a constant flow rate. A Pareto-like relationship governs limits and limitations: Mutual survival depends on reciprocal limitations, through interactive feedbacks, for the best and for the worst. To survive that is to transform disadvantages into advantages and to avoid advantages turn to disadvantages.
Interval-Valued Dual Hesitant Fuzzy Hamacher Aggregation Operators for Multiple Attribute Decision Making
Authors: Jiang Chao , Jiang Shenqing , Chen Jianlan 
Published: July 2019

AbstractAs an generalization of hesitant fuzzy set, interval-valued hesitant fuzzy set and dual hesitant fuzzy set, interval-valued dual hesitant fuzzy set has been proposed and applied in multiple attribute decision making. Hamacher t-norm and t-conorm is an generalization of algebraic and Einstein t-norms and t-conorms. In order to combine interval-valued dual hesitant fuzzy aggregation operators with Hamacher t-norm and t-conorm. We first introduced some new Hamacher operation rules for interval-valued dual hesitant fuzzy elements. Then, several interval-valued dual hesitant fuzzy Hamacher aggregation operators are presented, some desirable properties and their special cases are studied. Further, a new multiple attribute decision making method with these operators is given, and an numerical example is provided to demonstrate that the developed approach is both valid and practical.