bluuebunny/crossref_metadata_2025_split huggingface dataset:
A rare cause of persistent leukocytosis with massive splenomegaly
Authors: Gao Lu , Xu Yan , Weng Lan-chun , Tian Zu-guo 
Published: June 2022

Abstract

Rationale:
Persistent leukocytosis with megalosplenia is a common manifestation among patients with myeloproliferative neoplasm (MPN), especially for chronic myeloid leukemia (CML) patients. Here, we report a rare case of myeloid neoplasm with BCR-PDGFRA rearrangement characterized by obvious elevation of leukocyte count and megalosplenia.

Patient concerns:
A 32-year-old man presented with persistent leukocytosis and megalosplenia.

Diagnosis:
This patient was characterized by increased leukocyte count and megalosplenia, and was clinically diagnosed as CML. However, the BCR/ABL fusion gene of the patient was negative, which did not support CML. Moreover, the results of the karyotype showed 46, XY, t(4;22)(q12;q11) and RT-PCR + Sanger detection showed positive PDGFA/BCR. Accordingly, the diagnosis of myeloid neoplasm with BCR-PDGFA rearrangement was confirmed.

Interventions:
This patient was initially received imatinib (400 mg) orally once a day, and the dosage was adjusted to 100 mg owing to suffering from grade IV bone marrow suppression.

Outcomes:
Hematological remission was achieved after 2 weeks, the best treatment response was achieved after 3 months, and the main molecular biological response was achieved after 12 months.

Lesson:
This case suggests that rare PDGFA fusion genes screening for patients comorbid with leukocytosis and megalosplenia is necessary to avoid misdiagnosis. Unlike other rearrangements of PDGFRA, the clinical manifestations of BCR-PDGFRA rearrangement are resembling CML without eosinophilia increase.
Recent advances in targeting calcitonin gene-related peptide for the treatment of menstrual migraine
Authors: Jiang Yan , Huang Zhen-Lun 
Published: June 2022

Abstract
Menstrual migraine (MM) has a longer duration and higher drug resistance than non-perimenstrual migraine. Calcitonin gene-related peptide (CGRP) and CGRP receptors are expressed in the peripheral and central nervous systems throughout the trigeminovascular system. The CGRP/CGRP receptor axis plays an important role in sensory physiology and pharmacology. CGRP receptor antagonists and anti-CGRP monoclonal antibodies (mAbs) have shown consistent efficacy and tolerability in the prevention of chronic or episodic migraine and are now approved for clinical use. However, few studies have reported the use of these drugs in MM, and no specific treatment for MM has been approved. This review aimed to shed light on the recent advances in targeting calcitonin gene-related peptides for the treatment of menstrual migraines in PubMed. In this review, we first discuss the axis of the CGRP/CGRP receptor. We then discuss the role of CGRP receptor antagonists and anti-CGRP mAbs in MM treatment. Finally, we discuss the role of the combination of anti-CGRP mAbs and CGRP receptor antagonists in migraine treatment and the drugs that inhibit CGRP release. Altogether, the anti-CGRP mAbs or CGRP receptor antagonists showed good efficacy and safety in the treatment of MM.
The effect of binaural beat audio on anxiety in patients undergoing fiberoptic bronchoscopy
Authors: Opartpunyasarn Pornchai , Vichitvejpaisal Pornpattana , Oer-areemitr Nittha 
Published: June 2022

Abstract

Background:
Fiberoptic bronchoscopy is an invasive procedure known to induce anxiety in patients. Binaural beat therapy, in which sounds of different frequencies are delivered to the 2 ears to entrain the brainwaves, has been used to reduce anxiety in some operations. This study aimed to determine the anxiolytic effects of binaural beat audio in patients undergoing fiberoptic bronchoscopy.

Methods:
Eligible subjects were randomly assigned to receive binaural beat music, plain music, or no music. They were asked to wear earphones starting approximately 15 minutes before the bronchoscopy. The level of anxiety was measured using the State-Trait Anxiety Inventory questionnaire. Blood pressure, heart rate, and sedative drug administration were also recorded.

Results:
One hundred and twelve subjects were randomized into binaural beat music (n = 38), plain music (n = 38), and no music (n = 36) groups. The mean change in post-bronchoscopy State-Trait Anxiety Inventory state score in the binaural beat music, plain music, and no music group was −7.26 (P < .001), −3.92 (P = .005), and −1.12 (P = .454), respectively. The mean systolic blood pressure and diastolic blood pressure significantly decreased from baseline by −9.89 (P = .002) and −5.76 (P = .005), respectively, in the binaural group. The mean heart rate increased from baseline by 3.32 (P = .035), 5.21 (P = .038), and 3.64 (P = .149) in the binaural beat music, plain music, and no music groups, respectively.

Conclusion:
Binaural beat music appeared to reduce anxiety among patients undergoing fiberoptic bronchoscopy.
Trial registration: TCTR, TCTR20200915002. Registered 14 September 2020 - Retrospectively registered.
Dynamic distribution and correlation analysis of the angle kappa in myopia patients undergoing femtosecond-assisted laser in situ keratomileusis
Authors: Deng Wen-Qing , Fang Yu-Hui , Lin Shu-Hua , Li Ying-Jun 
Published: June 2022

Abstract

Purpose:
To explore the offset distribution of pupillary centres, the offset between the pupil centre and the coaxially sighted corneal light reflex (P-Dist) and their correlation in femtosecond laser combined with excimer laser in situ keratomileusis.

Methods:
Randomly selected 194 patients (398 eyes) who underwent femtosecond-assisted laser in situ keratomileusis with preoperative use of WaveLight Allegro Topolyzer Corneal Topography (WaveLight Laser Technologies AG, Erlangen, Germany) to measure the pupil size and centre position. The P-Dist of the patients was recorded by the X and Y axis eyeball tracking adjustment program of the WaveLight Eagle Vision EX500 excimer laser system.

Results:
The P-Dist was 0.214 ± 0.092 mm in the right eyes and 0.228 ± 0.105 mm in the left eyes (P = .041). Under scotopic conditions, the pupil centre of left eye X-axis was −0.046 ± 0.091 mm, the right eye was −0.152 ± 0.084 mm, with significant differences (P = .015), and the Y-axis direction showed no significant changes (P = .062). The white to white was positively correlated with changes of pupil diameter (scotopic pupil diameter-photopic pupil diameter) (r = 0.270, P < .001). The equivalent spherical mirror and measured centroid shift were negatively correlated (r = −0.214, P = .002).

Conclusion:
The angle kappa of the right eye is smaller than that of the left eye and from scotopic to photopic condition, the pupil centroid shift of both eyes to the nasal inferior side. If the cornea is too large, the low illumination environment should be maintained during the operation to improve the efficiency of pupil matching.
Practical parameters that can be used for nutritional assessment in patients hospitalized in the intensive care unit with the diagnosis of chronic obstructive pulmonary disease
Authors: Baldemir Ramazan , Cirik Mustafa Özgür 
Published: June 2022

Abstract
Malnutrition is an important condition in patients diagnosed with chronic obstructive pulmonary disease (COPD). There is a need for practical and objective nutritional assessment methods in patients hospitalized in the intensive care unit with the diagnosis of COPD. In this study, it was aimed to determine the parameters that can practically evaluate the nutritional status of these patients. It was aimed to determine the relationship between prognostic nutritional index (PNI), and nutritional risk screening (NRS)-2002, nutrition risk in the critical ill (Nutric) Score and to determine a cut-off value for PNI, neutrophil-to-lymphocyte (NLR), platelet-to-lymphocyte ratio (PLR), lymphocyte-to-monocyte ratio (LMR), and other complete blood count parameters.
Hemogram values, albumin values, NLR, PLR, LMR, NRS-2002, PNI and modified Nutric Score calculations of the patients hospitalized in the intensive care unit due to COPD were recorded. The relationship between PNI and NRS-2002 and modified Nutric Score, as well as the relationship between NLR, PLR, LMR, hemogram parameters and PNI were analyzed using statistical methods.
The PNI cut-off value for nutritional assessment in patients hospitalized in the intensive care unit due to COPD was determined as 38.5 (area under curve = 0.891, sensitivity 80.8%, specificity 88.1%, positive predictive value 92.9%, negative predictive value 88%). High-risk group according to PNI compared to low-risk group, lymphocyte count (P < .001), basophil count (P = .004), red blood cell (P < .001), hemoglobin (P < .001), hematocrit (P < .001), and LMR (P = .001) were statistically significantly lower, while NLR (P < .001) and PLR (P = .001) were statistically significantly higher. Cut-off values for lymphocyte count, basophil count, NLR, PLR, and LMR were found to be 1.18, 0.035, 7.97, 291.10, and 2.606, respectively.
Nutritional risk assessment can be made in a practical way by using PNI in patients hospitalized in intensive care unit due to COPD. For this, the PNI cut-off value was determined as 38.5 in our study. In addition, NLR, PLR, LMR, basophil and lymphocyte values, which can be calculated using complete blood count parameters, may also be useful in the evaluation of nutritional status in these patients. In our study, the cut-off values determined for NLR, PLR, LMR, basophil and lymphocyte were 7.97, 291.10 and 2.606, 0.035 and 1.18, respectively. We think that the results we have obtained can provide preliminary information for future research.
The comparisons of different therapeutic modalities for idiopathic achalasia
Authors: Shiu Sz-Iuan , Chang Chung-Hsin , Tu Yu-Kang , Ko Chung-Wang 
Published: June 2022

Abstract

Background
: Current guidelines recommend per-oral endoscopic myotomy (POEM) and laparoscopic Heller's myotomy (LHM) as first-line treatment of idiopathic achalasia, but the optimum choice between different endoscopic and surgical modalities remains inconclusive. We conducted a network meta-analysis to compare the efficacy of 8 treatments for idiopathic achalasia.

Materials and methods
: Three major bibliographic databases were reviewed for enrollment of randomized controlled trials between January 2000 and June 2021. We included adults with idiopathic achalasia and compared two or more of eight interventions including botulinum toxin injection (BTI), pneumatic dilation (PD), BTI + PD, LHM without fundoplication, LHM followed with Dor or Toupet fundoplication, and POEM using either the anterior or posterior approach. Our focus was on clinical success rate, postsurgical acid reflux, and moderate-to-severe adverse events.

Results
: Twenty-four studies involved a total of 1987 participants for analysis. When compared with PD, POEM with anterior approach, POEM with posterior approach, LHM + Toupet, and LHM + Dor were all significantly superior to the other regimens in short-term efficacy, with POEM with anterior approach and LHM + Dor showing better improvement in mid-term efficacy. BTI showed a significantly lower efficacy than PD in both periods. Regarding safety, only LHM without fundoplication was significantly associated with higher acid reflux than PD, while LHM + Toupet, LHM without fundoplication, and LHM + Dor showed a non-significant increase in moderate-to-severe adverse events.

Conclusions
: For idiopathic achalasia, we suggest that POEM with an anterior or posterior approach and LHM with Dor or Toupet fundoplication be initially recommended. On the contrary, both LHM without fundoplication and BTI are not recommended as definitive therapy.
Acute kidney injury due to direct infiltration by lymphoplasmacytic lymphoma secreting IgG paraproteins
Authors: Kim Seongmin , Bae Wooram , Choi Jungyoon , Lee Tae Won , Song Dae Hyun , Bae Eunjin , Jang Ha Nee , Chang Se-Ho , Park Dong Jun 
Published: June 2022

Abstract

Introduction:
Waldenström's macroglobulinemia is a lymphoplasmacytic lymphoma (LPL) associated with a monoclonal immunoglobulin M protein. Although acute kidney injury (AKI) due to immunoglobulin M paraprotein infiltration into the renal interstitium has been reported, there has been no report of AKI with invasion of the immunoglobulin G paraprotein into the renal interstitium in a patient with LPL.

Patient concerns:
A 65-year-old male was admitted to our hospital with fatigue and decreased renal function. He complained of a 3-kg weight loss in the last 3 months.

Diagnosis:
The initial blood urea nitrogen and serum creatinine levels were 55.9 and 1.83 mg/dL, respectively. Serum protein electrophoresis revealed a monoclonal component (3.5 g/dL) in the gamma region and immunofixation electrophoresis showed an immunoglobulin G kappa monoclonal protein. Renal pathology revealed that CD3–CD20+ CD138+ lymphoid cells had infiltrated the renal interstitium. A bone marrow biopsy was compatible with LPL.

Interventions:
Intravenous methylprednisolone (1 mg/kg) was administered after confirming the renal pathological findings.

Outcomes:
Serum creatinine decreased to 0.8 mg/dL 14 days after treatment

Conclusions:
Physicians should recognize LPL secreting various immunoglobulins as a possible cause of AKI when renal failure of unknown etiology and serum immunoglobulin paraprotein is present. A kidney biopsy should be performed for definitive diagnosis and appropriate management.
An apparent primitive mass of the mesentery
Authors: Costanzo Antonio , Canziani Marco , Ferrari Cesare Carlo , Bertocchi Valentina , Cutaia Saro , Bucci Eraldo Oreste , Uslenghi Elisabetta , Ferretti Andrea , De Luca Marco , Ceriani Fabio 
Published: June 2022

Abstract

Introduction:
Neuroendocrine tumours (NETs) are rare tumors. 55% of NETs originate in the gastrointestinal tract and the liver is the most common site of distant metastases. Serum chromogranin A is the most common biomarker for assessing the extent of disease and monitoring treatment; carcinoid syndrome occurs in 19% of NETs and is characterized by chronic diarrhea or flushing. Primary mesenteric NETs are rare and have been described only in case reports in literature; our case is an apparent primary mesenteric NETs with a surgical program to remove the mesenteric mass and subrenal interaortocaval and retrocaval lymphadenectomies.

Patient concerns:
A 73-year old man came to us because he had been experiencing abdominal pain for a year and he had recently developed diabetes mellitus. He was an active smoker with arterial hypertension.

Diagnosis:
After a computed tomography scan and 68 Gallium-positron emission tomography, a diagnosis of what appeared to be a primary mesenteric NET with retrocaval and interaortocaval lymph nodes was made. Laparoscopic biopsy showed NET G2 positive for serotonin, chromogranin A, synaptophysin.

Interventions:
The intraoperative finding of a primitive ileum-NET changed the surgical program. We removed the mesenteric mass with the lymph nodes of the superior mesenteric vessel and the middle distal ileum along with the cecum.

Outcomes:
The postoperative course was normal, and the patient was discharged on the seventh postoperative day without signs of short bowel syndrome. Follow-up at 6 months revealed no evidence of short bowel syndrome or disease progression.

Conclusion:
68 Gallium-positron emission tomography does not show NETs smaller than 0.5 mm. Accurate palpation of the intestine is essential during surgery for NETs for two reasons: to find the primitive, and because of the risk of multiple intestinal primitives.
Ceftazidime-avibactam and intrapleural amikacin therapy for extensively drug-resistant Pseudomonas aeruginosa thoracic empyema
Authors: Chen Tzu-Ting , Chen Shu-Mei , Liu Hsin-Yi 
Published: June 2022

Abstract

Introduction:
Thoracic empyema and concomitant bronchopleural fistula are serious complications of pneumonia. The treatment of empyema caused by extensively drug-resistant Pseudomonas aeruginosa (XDR-PA) has become increasingly challenging.

Patient's concerns and important clinical findings:
A 57-year-old woman with controlled schizophrenia developed hospital-associated bacterial pneumonia secondary to P. aeruginosa on day 13 of hospitalization for brain meningioma surgery.

Diagnosis:
Chest radiography and computed tomography revealed right-sided necrotizing pneumonia with pneumothorax, a focal soft tissue defect over the right lower chest wall, and a mild right-sided encapsulated pleural effusion with consolidation. XDR-PA was isolated on empyema cultures.

Interventions:
The patient was treated with intrapleural amikacin as a bridge to video-assisted thoracoscopic surgery, followed by novel ceftazidime-avibactam therapy.

Outcomes:
On the 104th day of admission, the patient underwent chest wall debridement and closure. The patient was discharged on day 111. Twenty-eight days after discharge, there were no observable sequelae of empyema.

Conclusion:
Although the minimum inhibitory concentration of ceftazidime-avibactam for XDR-PA is relatively high (8 mg/L), this report emphasizes the efficacy of ceftazidime-avibactam treatment for XDR-PA empyema, as well as the importance of source control.
Intercalary staphyloma after strabismus surgery in a patient with Marfan syndrome
Authors: Kim Ju Mi , Kim Jae-young , Sung Jae-yeon , Hwang Jae Yul , Lee Yeon-Hee 
Published: June 2022

Abstract

Rationale:
A few cases of intercalary staphyloma have been reported in patients with Marfan syndrome, but we believe that this is the first case of intercalary staphyloma in Marfan syndrome developing after strabismus surgery.

Patient concerns:
A 9-year-old girl diagnosed with Marfan syndrome visited a strabismus clinic for treatment of esotropia. Both eyes were aphakic and had 60 prism diopter esotropia at distance and 55 prism diopter esotropia at near. There were no corneal, conjunctival, or scleral abnormalities. Six millimeters of recession was performed on both medial rectus muscles via an inferonasal fornix approach under general anesthesia. 5 days after surgery, a dark gray protruding lesion was observed on the upper nasal side of the left eye.

Diagnoses:
Intraocular ultrasonography showed no bleeding, retinal detachment, or other abnormal findings. Computed tomography showed a conical protrusion of the scleral wall which was diagnosed as intercalary staphyloma.

Interventions:
To reduce risk of progression of the staphyloma in the left eye and to reduce risk of development of a new staphyloma, intraocular pressure lowering eye drops were administered.

Outcomes:
We just observed it without any intervention except the intraocular pressures lowering eye drops. It remained stable for 12 months.

Lessons:
Clinicians need to be alert to the possibility of this serious complication in Marfan syndrome patients after minor surgical trauma, which can occur during uneventful strabismus surgery.
Volumetric computed tomography analysis for gastroduodenal and pancreaticoduodenal artery aneurysm formation
Authors: Maeno Ryohei , Hoshina Katsuyuki , Miyahara Kazuhiro , Suhara Masamitsu , Matsukura Mitsuru , Isaji Toshihiko , Takayama Toshio 
Published: June 2022

Abstract
Gastroduodenal artery aneurysms (GDAA) and pancreaticoduodenal artery aneurysms (PDAA) are rare, have high rupture risks, and are located in the arcade between the celiac artery and the superior mesenteric artery. Pancreaticoduodenal artery aneurysms are associated with celiac artery stenosis, and it is hypothesized that these celiac lesions might contribute to the formation of aneurysms. In contrast, a few studies have reported an association between a gastroduodenal artery aneurysm and celiac lesions. This study aimed to investigate the potential differences between patients with gastroduodenal and pancreaticoduodenal artery aneurysms and better understand their pathogenesis.
We selected patients with GDAA and PDAA who were admitted to our department between January 2010 and December 2020. Aortic wall volume, aortic wall calcification, and pancreaticoduodenal arcade volume of computed tomography images were calculated semi-manually using Horos 3.3.5.
Eight GDAAs and 11 PDAAs were analyzed. Celiac lesions were found in all PDAA patients, with none in GDAA cases. Volumetry demonstrated that aortic wall volume and calcification were more prominent in the GDAA group than in the PDAA group (P = .026 and P = .049, respectively). The pancreaticoduodenal arcade volume was larger in the PDAA group (P = .002).
In our study, celiac artery lesions were strongly correlated with PDAA. The volume of the pancreaticoduodenal arcade was larger in the PDAA group, and aortic wall volume and calcification were larger in the GDAA group.
Localized Malignant Peritoneal Mesothelioma (LMPeM) in Women: A Clinicopathologic Study of 18 Cases
Authors: Malpica Anais , Euscher Elizabeth D. , Marques-Piubelli Mario L. , Miranda Roberto N. , Raghav Kanwal P. , Fournier Keith F. , Ramalingam Preetha 
Published: June 2022

Localized malignant peritoneal mesothelioma is a rare tumor with limited information in the literature. In this study, we present our experience with 18 cases seen in our hospital over a period of 43 years (1978 to 2021). Patients’ median age was 55 years (y) (range: 33 to 79 y) and most of them were Caucasians. Patients presented with abdominal pain (11), ascites and right leg swelling (1), abdominal mass (1), and as incidental finding (1). Thirty percent of patients reported asbestos exposure, and all patients with available information had family history of tumors; a third had personal history of tumors. Seventy-seven percent had some form of abdominopelvic surgery and/or inflammatory process. Most cases had microscopic features typically seen in malignant mesothelioma; however, some cases had confounding features such as signet-ring cells, spindle cells, clear cell changes, and adenomatoid tumor-like appearance. BAP-1 by immunohistochemistry was lost in 1/3 cases. Only 1 patient underwent genetic testing and had an MSH2 germline mutation. Homozygous deletion of CDKN2A by FISH was not found in 1 tested case, although next-generation sequencing identified a CDKN2A pathogenic mutation. 16/18 (88%) had surgical treatment, and some also received adjuvant chemotherapy. The mean overall survival (OS) of our patients was 80.4 months (95% confidence interval: 54.3-106.52); the 3-year OS was 79%, while the 5-year OS was 52.6%. Fifty-three percent of patients had recurrences and 20% had tumor progression. Although the limited sample precludes definitive conclusions, small tumor size, low-grade cytology, and low mitotic index appeared to be associated with an indolent behavior.