bluuebunny/crossref_metadata_2025_split huggingface dataset:
PTEN hamartoma tumor syndrome in childhood and adolescence—a comprehensive review and presentation of the German pediatric guideline
Authors: Plamper Michaela , Gohlke Bettina , Woelfle Joachim 
Published: February 2022

Abstract
Background
The PTEN hamartoma tumor syndrome (PHTS) encompasses several different syndromes, which are linked to an autosomal-dominant mutation of the tumor suppressor PTEN gene on chromosome 10. Loss of PTEN activity leads to an increased phosphorylation of different cell proteins, which may have an influence on growth, migration, and apoptosis. Excessive activity of the PI3K/AKT/mTOR pathway due to PTEN deficiency may lead to the development of benign and malignant tumors and overgrowth. Diagnosis of PHTS in childhood can be even more challenging than in adulthood because of a lack of well-defined diagnostic criteria. So far, there are no official recommendations for cancer surveillance in affected children and adolescents.

Main body
All individuals with PHTS are at high risk for tumor development and thus might benefit from cancer surveillance strategies. In childhood, macrocephaly may be the only evident symptom, but developmental delay, behavioral problems, dermatological features (e.g., penile freckling), vascular anomalies, lipoma, or enlarged perivascular spaces in cerebral magnetic resonance imaging (cMRI) may help to establish the diagnosis. Regular psychomotor assessment and assistance in subjects with neurological impairment play an important role in the management of affected children. Already in early childhood, affected patients bear a high risk to develop thyroid pathologies. For that reason, monitoring of thyroid morphology and function should be established right after diagnosis. We present a detailed description of affected organ systems, tools for initiation of molecular diagnostic and screening recommendations for patients < 18 years of age.

Conclusion
Affected families frequently experience a long way until the correct diagnosis for their child’s peculiarity is made. Even after diagnosis, it is not easy to find a physician who is familiar with this rare group of diseases. Because of a still-limited database, it is not easy to establish evidence-based (cancer) surveillance recommendations. The presented screening recommendation should thus be revised regularly according to the current state of knowledge.
Episode 4 (2019–2020) Nishinoshima activity: abrupt transitions in the eruptive style observed by image datasets from multiple satellites
Authors: Kaneko Takayuki , Maeno Fukashi , Ichihara Mie , Yasuda Atsushi , Ohminato Takao , Nogami Kenji , Nakada Setsuya , Honda Yoshiaki , Murakami Hiroshi 
Published: February 2022

AbstractIn December 2019, a new activity started at Nishinoshima volcano in the southern part of the Izu–Ogasawara arc, Japan. This is now referred to as Episode 4 of a series of activities that began in 2013. We analyzed the eruption sequence, including erupted volume and effusion rate, based on combined observations of thermal anomalies by Himawari-8 and topographic changes by ALOS-2. The total eruption volume during Episode 4 was ~ 132 × 106 m3, and the average effusion rate over the entire period was 0.51 × 106 m3 day−1 (5.9 m3 s−1), which was two to three times higher than that of Episode 1. Episode 4 had three stages. In Stage 1, effusive activity was dominant, and most of the lava erupted from a northeast vent at the foot of the pyroclastic cone to cover the northern half of the island. The average effusion rate was estimated to be 0.46 × 106 m3 day−1 (5.3 m3 s−1). In Stage 2, an intensive lava fountain with a high discharge rate developed, and it increased the size of the pyroclastic cone rapidly. The effusion rate temporarily reached 2.6 × 106 m3 day−1 (30 m3 s−1). Pyroclastic rocks accounted for 45–88% of the total erupted volume in this stage. Lava flows with rafted cone material were generated, and those possibly caused by intensive spatter falls on the slope were also formed. These lavas flowed down the southern half of the island. In Stage 3, continuous phreatomagmatic eruptions released ash and spread it over a wide area. The high effusion rate and the drastic change in the activity style in Episode 4 can be explained by deep volatile-rich magma being supplied to a shallower magma chamber prior to Episode 4. When the volatile-rich magma reached a shallow part of the conduit in Stage 2, fragmentation occurred due to rapid volume expansion to eject large amounts of magma and form the intensive lava fountain. Observations by satellite-borne ultraviolet–visible image sensors detected a rapid increase in SO2 emissions in response to the intensive lava-fountain activity. The less-differentiated nature of the ash fragments collected during Stage 2 may reflect the composition of the volatile-rich magma. Large-scale discolored-seawater areas appeared during the late period of Stage 1, which may have been caused by ascent of the volatile-rich magma.
Graphical Abstract
Angiolipoma of the chest wall: a case report
Authors: Omori Takahiro , Nakamura Sho 
Published: February 2022

Abstract
Background
Angiolipoma is a rare histological variant of lipoma. Angiolipoma commonly occurs in the subcutaneous tissues of the extremity and trunk. There are few reports of angiolipoma occurring in the chest wall.

Case presentation
A 78-year-old woman was referred to our hospital for evaluation of angina pectoris. Coronary computed tomography (CT) showed a soft tissue nodule in the left chest wall by chance. Enhanced chest CT showed a heterogeneous enhanced nodule in the left chest wall. On magnetic resonance imaging (MRI), the lesion showed low signal intensity on T1-weighted images, heterogeneous high signal intensity on T2-weighted images and high signal intensity on fat-suppressed T2-weighted images. The lesion showed heterogeneous enhanced effect on gadolinium-based contrast agent. These radiological findings suggested neurogenic tumor with abundant blood flow or hemangioma. Video-assisted thoracic surgery (VATS) was performed for both diagnostic and therapeutic purposes. Histopathological examination of the tumor showed mature adipose tissue and capillary hyperplasia containing fibrin thrombi. These appearances were consistent with angiolipoma. She had an uneventful recovery and did not show recurrence until 8 months post-surgery.

Conclusions
Angiolipoma of the chest wall is extremely rare. Preoperative diagnosis is very difficult because the imaging findings of angiolipoma vary depending on the amount of vascular component and fat component, so surgical resection is suggested to be both diagnostic and therapeutic.
Intracholecystic papillary neoplasm associated with invasive carcinoma of the remnant gallbladder after subtotal cholecystectomy: a case report
Authors: Watanabe Yusuke , Mochidome Naoki , Nakayama Hiromichi , Gotoh Yoshitaka , Setoguchi Taro , Sunami Shunya , Yoneda Reiko , Ochiai Yurina , Mizoguchi Kimihisa , Yamamoto Hirofumi , Ueki Takashi 
Published: February 2022

Abstract
Background
Intracholecystic papillary neoplasm (ICPN) of the gallbladder is a rare tumor and a relatively new concept. Therefore, the natural history and imaging characteristics of ICPN have not yet been fully documented. Moreover, cases who underwent curative resection for remnant gallbladder cancer, including ICPN with associated invasive carcinoma, have been rarely reported. We report a resected case of ICPN of the remnant gallbladder with associated invasive carcinoma for which we could observe a temporal change in imaging findings until malignant transformation.

Case presentation
A 79-year-old female patient with a surgical history of subtotal cholecystectomy for acute cholecystitis was an ambulatory patient of our institution because of postoperative surveillance for colon cancer. Ultrasonography and computed tomography incidentally detected a small nodule in the cystic remnant gallbladder. The nodule had increased in size 3 months later; thus, additional investigations were performed. Magnetic resonance imaging revealed a 10-mm enhanced nodule without evidence of extraluminal invasion. Diffusion-weighted magnetic resonance imaging revealed restricted diffusion of the lesion, and positron emission tomography revealed marked accumulation in the lesion. The lesion was diagnosed as suspicious for a malignant remnant gallbladder tumor. Therefore, remnant cholecystectomy with gallbladder bed resection was performed. Because preoperative endoscopic retrograde cholangiography revealed a relatively long intact cystic duct, extrahepatic bile duct resection was planned to be omitted. Intraoperatively, the hepatic and duodenal side bile duct where the cystic duct diverged was taped. Using these tapes, which permitted pulling the bile duct, the cystic duct located behind the bile duct could be safely exposed. The lesion was pathologically diagnosed as biliary morphologic ICPN with associated invasive carcinoma.

Conclusions
Because remnant cholecystectomy is an uncommon procedure and technically difficult, accurate preoperative investigation and surgical planning are important to prevent bile duct injury and omit extrahepatic bile duct resection. In the present case, intracystic change could be detected incidentally at an early stage because of previous remnant gallbladder producing (reconstituting) subtotal cholecystectomy and surveillance for other disease. This case suggests the existence of ICPN that can progress to invasive carcinoma during a short period.
Comparison of time-matched aerobic, resistance or combined exercise training in women living with obesity: a protocol for a pilot randomised controlled trial—the EXOFFIT (Exercise for Obesity in Females to increase Fitness) study
Authors: Davis Mary E. , Blake Catherine , Cunningham Caitriona , Carson Brian P. , O’Donoghue Gráinne 
Published: February 2022

Abstract
Introduction
Obesity in women has more than doubled in the past thirty years. Increasing research suggests that increased cardiorespiratory fitness (CRF) can largely attenuate the negative health risks associated with obesity. Though previous literature suggests that combined training may be the most effective for improving CRF in adults with obesity, there is minimal research investigating the efficacy of combined and resistance programmes in women with obesity. This article outlines a protocol for a parallel pilot study which aims to evaluate the feasibility and efficacy of three exercise modalities in women with obesity for increasing CRF and strength and improving body composition and other health outcomes (i.e. quality of life).

Methods and analysis
Sixty women (aged 18–50) with obesity (body mass index [BMI] ≥ 30 and/or waist circumference ≥ 88 cm) who are physically inactive, have no unstable health conditions and are safe to exercise will be recruited from September 2021 to December 2022. The main outcome will be feasibility and acceptability of the intervention and procedures. Trial feasibility outcomes will be evaluated to determine if a definitive trial should be undertaken. Trial acceptability will be explored through follow-up qualitative interviews with participants. Secondary outcomes will include CRF (predicted VO2 max), anthropometrics (i.e. BMI), strength (5RM bench press, leg dynamometry, grip strength) and other health outcomes (i.e., pain). Participants will be block randomised into one of four trial arms (aerobic exercise, resistance training and combined training groups, non-active control group) and measurements will be completed pre- and post-intervention. The exercise groups will receive an individualised supervised exercise programme for 3× sessions/week for 12 weeks. The change in mean values before and after intervention will be calculated for primary and secondary outcomes. ANOVA and t-tests will be applied to evaluate within-group and between-group differences. If sufficient participants are recruited, the data will be analysed using ANCOVA with the age and BMI as covariates.

Discussion
This pilot will provide data on the feasibility and acceptability of trial procedures and of the programmes’ three progressive time-matched exercise interventions (aerobic, resistance and combined) for women living with obesity, which will help inform future research and the potential development of a full-scale randomised clinical trial.

Trial registration
ISRCTN, ISRCTN13517067. Registered 16 November 2021—retrospectively registered.
Successful lung-protective ventilatory management during the VV-ECMO in a severe COVID-19 pneumonia patient with extensive pneumomediastinum and subcutaneous emphysema: a case report
Authors: Kohara Jumpei , Kai Shinichi , Hashimoto Kazuya , Takatani Yudai , Tanabe Naoya , Hamada Satoshi , Cho Kosai , Tanaka Tomoharu , Ito Isao , Ohtsuru Shigeru 
Published: February 2022

Abstract
Background
Ventilatory management of respiratory failure with pneumomediastinum/subcutaneous emphysema is not established. Herein, we report a case of severe COVID-19 pneumonia with extensive pneumomediastinum/subcutaneous emphysema, rescued by thorough lung-protective ventilatory management after applying the VV-ECMO.

Case presentation
A 68-year-old male with no medical history was admitted to a local hospital and diagnosed with COVID-19 pneumonia. His pulmonary parameters worsened during invasive ventilation due to the development of pneumomediastinum/subcutaneous emphysema, and then he was transferred to our hospital. On arrival, we immediately decided to apply VV-ECMO and switch to ultraprotective ventilation. After maintaining the initial ventilation with a neuromuscular blocking agent for 2 days, we gradually increased PEEP while limiting PIP to 25 cmH2O. The patient was weaned off VV-ECMO on day 10; he was transferred to the medical ward after extubation.

Conclusions
Lung-protective ventilatory management should be performed thoroughly during VV-ECMO in severe COVID-19 pneumonia with pneumomediastinum/subcutaneous emphysema.
Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS): a Brazilian cohort
Authors: Cavalcanti André , Islabão Aline , Magalhães Cristina , Veloso Sarah , Lopes Marlon , do Prado Rogério , Aquilante Bruna , Terrazas Ana Maria , Rezende Maria Fernanda , Clemente Gleice , Terreri Maria Teresa 
Published: February 2022

Abstract
Background
Paediatric inflammatory multisystem syndrome (PIMS) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been described since mid-April 2020 with the first reports coming from Europe. Our objective was to describe the characteristics of patients among the Brazilian population.

Methods
A multicenter retrospective study was conducted with the participation of five pediatric rheumatology centers in Brazil during the period from March to November 2020. Children and adolescents with PIMS temporally associated with SARS-CoV-2 (TS) who met the definition criteria for the disease according to the Royal College of Paediatrics and Child Health were included. Demographic, clinical, laboratory, therapeutic characteristics and molecular and serological diagnosis of SARS-CoV-2 infection were described.

Results
Fifty-seven children and adolescents with PIMS-TS were evaluated, 54% female, with a median age of 8 (3–11) years. Most (86%) were previously healthy, with asthma being the main comorbidity, present in 10% of the patients. Fever was the main manifestation, present in all patients, followed by mucocutaneous and gastrointestinal features, present in 89% and 81% of the patients, respectively. Myocarditis occurred in 21% of the patients and in 68% of them required intensive care. The Kawasaki disease phenotype occurred in most patients (77%). All patients had elevated inflammatory markers, with elevated CRP being the most found (98%). Anemia and lymphopenia were present in 79% and 72%, respectively. Laboratory evidence of SARS-CoV-2 was found in 77% of the patients, with 39% positive RT-PCR and 84% positive serology for SARS-CoV-2. An immunomodulatory treatment was performed in 91% of the patients, with 67% receiving intravenous immunoglobulin (IVIG) associated with glucocorticoid, 21% receiving IVIG, and 3.5% receiving glucocorticoid. The median length of hospitalization was 10 days.

Conclusions
This study showed a high morbidity of PIMS-TS in Brazilian children, with a prolonged length of hospitalization and a high rate of admission to pediatric intensive care unit. Multicenter prospective studies are needed to assess the morbidity of the disease in the medium and long term.
A review of phosphorus nutrition in irrigated cotton farming systems of Australia
Authors: NACHIMUTHU Gunasekhar , SCHWENKE Graeme , MERCER Clarence , BISCHOF Callum , HULME Pat , BELL Michael 
Published: February 2022

AbstractAustralian cotton production predominantly occurs on Vertisols. The average lint yield of cotton grown in Australia is 2 260–2 700 kg·hm−2, which is 2.5 to 3 times the world average. This high productivity per unit of land area requires efficient use of resources such as water and nutrients. However, high yields accelerate the export of nutrients such as phosphorus (P) in seed, depleting the soil reserves of P more than in other countries with lower cotton yields. Recent surveys of cotton industry indicate that P application rates should match seed P export (30~ 40 kg·hm−2), but historical depletion within subsoil is still evident and is continuing. Depletion of soil P is typically more pronounced in the subsoil than in the topsoil (0~ 20 cm) where P fertiliser is applied, as cotton roots rely on these layers as important sources of plant available water and available P. This mismatch between zones of P uptake and resupply may increase stratification of available P in the soil profile. Recent studies showed that cotton responded poorly to banded applications of fertiliser P, while dispersal of fertiliser throughout the plant beds was more successful. Researchers have also observed sporadic cotton responses to applied P fertiliser in soils where available P concentrations were well above the previously determined critical concentrations indicative of fertiliser P responses in Australia. To sustain high-yielding cotton production in Australia, a greater understanding of cotton root acquisition of applied P, as well as a re-examination of critical soil P concentrations for each production region are required.
Food history and gastronomic traditions of beans in Italy
Authors: Corrado Giandomenico 
Published: February 2022

AbstractBeans have been regarded primarily as a staple food for peasants, an affordable protein source for the mass, and a symbol of rustic simplicity by writers of all ages. Among legumes, the common bean (Phaseolus vulgaris L.) probably plays the leading role in typifying these attributes. This species has also shown a remarkable ability to spread around the globe and to replace similar local species in virtually all the cuisines of the world, being nowadays embodied in the gastronomy of several countries. Attitudes toward beans are changing recently, and this legume is no longer considered as only the meat of the poor. This review aims to present a critical overview of the history and role in the gastronomy of common bean and other main cultivated legumes in Italy. After presenting the origin of common bean and its name, and the impact of its introduction to Europe, this contribution discusses the gastronomic history of beans in Italy and the role that socio-cultural differences have played in shaping the use of beans, the conservation of landraces, and food diversity. Finally, perspectives are discussed considering the recent trends in gastronomy and food tourism.
SMA carrier testing using Real-time PCR as a potential preconception screening tool
Authors: Eissa Noura Raafat , Hassan Heba Amin , Senousy Sameh Mohamed , Soliman Hala Nasr , Essawi Mona Lotfy 
Published: February 2022

Background
SMA is a neuromuscular genetic disorder causing irreversible degeneration of the anterior horn cells of lower motor neurons. According to the age of onset and severity of the condition, it is classified into 5 subtypes. SMA carrier’s frequency worldwide is 1:40–80. We used quantitative real-time PCR to determine the copy number of the disease-determining SMN1 gene by rapid and reliable assays. We studied the SMN1 gene copy number in Egyptian sample of 115 individuals, as well as in 10 SMA families.

Results
Our results showed that 57.4% of the couples with the previous history of an affected family members were carriers. On the individual level, carriers of single SMN1 gene copy rate are much higher than the previously reported frequency rates. The effect of consanguineous marriages appears evident in SMA as an autosomal recessive disorder.

Conclusions
In conclusion, the carrier frequency detected in our cohort was high, which possibly corresponds with the worldwide report of SMA as a leading genetic cause of death among infants. Considering the high rate of consanguinity in developing countries confirms the importance of national SMA carrier screening in Egypt. The qPCR carrier screening test is a rapid-cost effective test that can detect approximately 90% of carriers. A population-based preconception prenatal screening for couples will also help reduce the disease burden.
Signature of electromagnetic quantum fluctuations in exciton physics
Authors: Combescot Monique , Dubin François , Shiau Shiue-Yuan 
Published: February 2022

Abstract
Quantum fluctuations of the electromagnetic field are known to produce the atomic Lamb shift. We here reveal their iconic signature in semiconductor physics, through the blue-shift they produce to optically bright excitons, thus lifting the energy of these excitons above their dark counterparts. The electromagnetic field here acts in its full complexity: in addition to its longitudinal part via interband virtual Coulomb processes, the transverse part —which has been missed up to now— also acts via resonant and nonresonant virtual photons. These two parts beautifully combine to produce a bright exciton blue-shift independent of the exciton wave vector direction. Our work readily leads to a striking prediction: long-lived excitons must have a small bright-dark splitting. Although the analogy between exciton and hydrogen atom could lead us to see the bright exciton shift as a Lamb shift, this is not fully so: the Lamb shift comes entirely from virtual photons, whereas the Coulomb interaction also contributes to the exciton shift through the so-called “electron-hole exchange”.