OpenMed/multicare-articles huggingface dataset:
The critical BMI hypothesis for puberty initiation and the gender prevalence difference: Evidence from an epidemiological survey in Beijing, China
Authors: Meijuan Liu, Bingyan Cao, Qipeng Luo, Qiao Wang, Min Liu, Xuejun Liang, Di Wu, Wenjing Li, Chang Su, Jiajia Chen, Chunxiu Gong
Journal: Front Endocrinol (Lausanne) (2022)
Keywords: body mass index, central obesity, children, gender, precocious puberty
MeSH terms: Pediatric Obesity / epidemiology, Puberty, Precocious / epidemiology, Case Reports, Research Support, Non-U.S. Gov't, Beijing / epidemiology, Body Mass Index, Child, China / epidemiology, Female, Humans, Male, Obesity, Abdominal, Prevalence, Puberty

Background:
Although previous studies suggested that there is a certain level of body fat mass before puberty can be initiated, most studies have focused on girls.
Objective:
To investigate the relationship between precocious puberty and physical growth in school-aged children in Beijing, China.
Methods:
7590 Chinese children (3591 girls and 3999 boys) aged 6-11 years were recruited in Beijing, China. Body mass index (BMI) categories were defined by WHO Child Growth Standards and central obesity were defined by sex-specific waist-to-height ratio cut-offs (≥0.46 for girls, ≥0.48 for boys). Sexual development was assessed using Tanner criteria.
Results:
The prevalence of general obesity and central obesity among boys was higher than that in girls. Girls had a significantly higher precocious puberty rate than boys (5.93% vs. 0.87%), particularly in those aged 7 years old (9.20%). Children in the general obesity and central obesity groups have a higher prevalence of precocious puberty and earlier median ages for the attainment of Tanner B2/T2. For girls with Tanner stages≥II at 6-year-old and 7-year-old, the mean BMI was equivalent to the 50th centile of a normal 9.9-year-old and 11.9-year-old girl, respectively. The mean BMI of boys with Tanner stages≥II at 7-year-old and 8-year-old was correspondent to the 50th centile of a normal 14-year-old and 15.3-year-old boy, respectively. For girls, general obesity appears to contribute to the risk of the development of precocious puberty to a greater extent than central obesity does. For boys, central obesity, but not general obesity, was an independent risk factor for precocious puberty.
Conclusions:
The prevalence of childhood obesity and precocious puberty was high in China. Precocious puberty was correlated with a large BMI. Boys had a higher threshold of BMI for puberty development than girls. Children with precocious puberty, particularly those with central obesity, should be aware of adverse cardiovascular events.
PD-1 Checkpoint Blockade in Patients for Acute Myeloid Leukemia after HSCT Relapse Resulted in Severe GVHD and sHLH
Authors: Zhi Zhuo Du, Mi Zhou, Jing Ling, Lan Cao, Lingjun Kong, Shengqin Cheng, Peifang Xiao, Shaoyan Hu
Journal: Case Rep Hematol (2022)
MeSH terms: Case Reports

Treatment with immune checkpoint inhibitors (ICI) such as carrizumab leads to immune-mediated adverse effects including severe acute graft versus host disease (aGVHD) and secondary hemophagocytic syndrome (sHLH). Herein, we present two cases where aGVHD and sHLH developed after ICI administration, which was treated using methylprednisolone (MP). They developed high-grade fever complicated with liver dysfunction and diarrhea 1 day after ICI administration. Treatment with MP does not alleviate symptoms because of steroid resistance. Hyperbilirubinemia, rash with blisters, and watery diarrhea showed severe aGVHD. Hyperferritinemia, hypertriglyceridemia, and cytopenias suggested the diagnosis of HLH and met the criteria for sHLH diagnosis. They were thus administered intravenous high-dose MP, methotrexate (MTX), basiliximab, ruxolitinib, etc, which resolved these symptoms.
Rheumatoid arthritis-associated aortitis: a case report and literature review
Authors: Shunta Kaneko, Hiroyuki Yamashita, Yusuke Sugimori, Yuko Takahashi, Hiroshi Kaneko, Toshikazu Kano, Akio Mimori
Journal: Springerplus (2014)
Keywords: aortitis, rheumatoid arthritis, rheumatoid vasculitis

Rheumatoid arthritis (RA) is a systemic autoimmune inflammatory disorder that primarily affects the synovial joints. Rheumatoid vasculitis (RV) is an extra-articular manifestation of RA, and its association with aortitis is rare and not widely recognised. Here, we report the case of a 69-year-old woman with RA-associated aortitis and review the literature on rheumatoid aortitis. The mean oral steroid dose administered to RA-associated aortitis patients was 46.3 mg/day prednisolone (PSL). In our patient, the aortitis was also thought to be due to RV because she had findings of RV, such as cutaneous ulceration and a high rheumatoid factor titre, and because a moderate PSL dose dramatically improved the clinical findings. RA-associated aortitis, if left untreated, can be fatal; therefore, early detection and treatment initiation is very important.
Distal Femoral Replacement Following an Intraoperative Periprosthetic Fracture in a Patient with Prior Bone Mulch ACL Reconstruction
Authors: Anthony Gemayel, Matthew J Yousif, William Padget, Joseph Finch
Journal: Case Rep Orthop (2019)
MeSH terms: Case Reports

Periprosthetic distal femur fractures can be treated nonoperatively, with open reduction and internal fixation or with more constrained prostheses. Distal femoral replacement is typically a last resort treatment option for comminuted periprosthetic or osteoporotic distal femoral fractures in patients with poor bone stock or resistant nonunions. We report the case of a 54-year-old female with a remote history of bone mulch ACL reconstruction who sustained an intraoperative comminuted bicondylar distal femur fracture during a primary total knee arthroplasty. This patient was treated with a distal femoral replacement and successfully returned to her preoperative function.
Case report: ETS1 gene deletion associated with a low number of recent thymic emigrants in three patients with Jacobsen syndrome
Authors: Tina Trachsel, Seraina Prader, Katharina Steindl, Jana Pachlopnik Schmid
Journal: Front Immunol (2022)
Keywords: ets1, jacobsen syndrome, genetic disorder, immunodeficiency, recent thymic emigrants
MeSH terms: Jacobsen Distal 11q Deletion Syndrome / genetics, Case Reports, Research Support, Non-U.S. Gov't, Child, Preschool, DNA-Binding Proteins / genetics, Gene Deletion, Humans, Phenotype, Proto-Oncogene Protein c-ets-1 / genetics, T-Lymphocytes, Thymus Gland, ETS1 protein, human, Proto-Oncogene Protein c-ets-1, NFRKB protein, human, DNA-Binding Proteins

Jacobsen syndrome is a rare genetic disorder associated with a terminal deletion in chromosome 11. The clinical presentation is variable. Although immunodeficiency has been described in patients with Jacobsen syndrome, a clear genotype-phenotype correlation has not yet been established. Here, we report on the immunologic phenotypes of four patients with Jacobsen syndrome. All four patients showed one or more atypical immunologic features. One patient suffered from recurrent viral infections, two patients had experienced a severe bacterial infection and one had received antibiotic prophylaxis since early childhood. One patient had experienced severe, transient immune dysregulation. Hypogammaglobulinemia and low B cell counts were found in two patients, while the number of recent thymic emigrants (CD31+CD45RA+ CD4 cells) was abnormally low in three. When considering the six immune-related genes located within the affected part of chromosome 11 (ETS1, TIRAP, FLI1, NFRKB, THYN1, and SNX19), only the ETS1 gene was found be deleted in the three patients with low numbers of recent thymic emigrants and non-switched memory B cells. Our findings support the hypothesis whereby Jacobsen syndrome is associated with a combined immunodeficiency with variable presentation. Further investigations of potential genotype-phenotype correlations are warranted and might help to personalize patient management in individuals lacking immune-related genes. In addition, we recommend immunologic follow-up for all patients with Jacobsen syndrome, as immune abnormalities may develop over time.
Implant Restoration of Edentulous Jaws with 3D Software Planning, Guided Surgery, Immediate Loading, and CAD-CAM Full Arch Frameworks
Authors: Silvio Mario Meloni, Giacomo De Riu, Milena Pisano, Francesco Maria Lolli, Alessandro Deledda, Guglielmo Campus, Antonio Tullio
Journal: Int J Dent (2013)

Purpose. The aim of this study was to analyze the clinical and radiographic outcomes of 23 edentulous jaws treated with 3D software planning, guided surgery, and immediate loading and restored with CAD-CAM full arch frameworks. Materials and Methods. This work was designed as a prospective case series clinical study. Twenty patients have been consecutively rehabilitated with an immediately loaded implant supported fixed full prosthesis. A total of 120 fixtures supporting 23 bridges were placed. 117 out of 120 implants were immediately loaded. Outcome measures were implants survival, radiographic marginal bone levels and remodeling, soft tissue parameters, and complications. Results. 114 of 117 implants reached a 30 months follow-up, and no patients dropped out from the study. The cumulative survival rate was 97.7%; after 30 months, mean marginal bone level was 1.25 ± 0.31 mm, mean marginal bone remodeling value was 1.08 ± 0.34, mean PPD value was 2.84 ± 0.55 mm, and mean BOP value was 4% ± 2.8%. Only minor prosthetic complications were recorded. Conclusion. Within the limitations of this study, it can be concluded that computer-guided surgery and immediate loading seem to represent a viable option for the immediate rehabilitations of completely edentulous jaws with fixed implant supported restorations. This trial is registered with Clinicaltrials.gov NCT01866696.
The Use of Cook Resonance Metallic Ureteral Stent in Cases of Obstructive Uropathy from Persistent Neoureteral Stenosis, Following Kidney Transplantation
Authors: Victoria Stainer, Rachel Jones, Sanjay Agawal, Iqbal S Shergill
Journal: J Endourol Case Rep (2017)
Keywords: metallic ureteral stent, obstructive uropathy, transplant
MeSH terms: Case Reports

Introduction: Following kidney transplantation, persistent cases of obstructive uropathy from neoureteral stenosis, at the reimplantation site, may require management with permanent, long-term Double-J stenting, following failed open surgical and minimally invasive procedures. We report our experience of the use of Cook Resonance® metallic ureteral stent to manage such cases endourologically. Materials and Methods: Medium-term follow-up of two cases requiring long-term ureteral stenting. Medical records, operative details, and radiologic data were reviewed. Primary outcome was relief of obstructive uropathy, and secondary outcomes included clinicoradiologic complications and cost-effectiveness of the metallic stents compared with standard Double-J stents. Results: Case 1 was a 45-year-old lady with obstructive uropathy after kidney transplantation. To date, she has had four metallic stents, and on review of operative details and radiologic data, there was seen to be a 60% reduction in operation length from the first to fourth stent exchange. Radiation dose exposure saw an 80% reduction from 2852 to 556 CGy·cm2. Following 3 years of follow-up, relief of obstructive uropathy has been maintained, with no radiologic or clinical evidence of complications. Case 2 was a 44-year-old lady with obstructive nephropathy requiring long-term stenting after kidney transplantation. Two stent exchanges have been performed to date with a 38% reduction in operation length from 50 minutes to just 31 minutes. Radiation dose exposure saw a 41% reduction. No clinicoradiologic complications or stent-related symptoms have occurred. Discussion: In our experience, use of metallic stents in transplanted kidneys is safe and feasible, with both patients having effective and sustained relief of obstructive uropathy. This stent appears to be well tolerated and is associated with minimal clinicoradiologic complications. Metallic stent replacement is also cost-effective due to the fact that it only requires annual rather than 6-monthly stent changes.
Crystal-storing histiocytosis in the stomach: A case report and review of the literature
Authors: Linghong Kong, Liyan Xue, Yanfeng Zhong, Shenglan Wang, Danfeng Zheng, Lining Wang, Yang Jiao, Xinpeng Zhang, Huizhong Xue, Xiaogang Liu
Journal: Front Oncol (2022)
Keywords: crystal-storing histiocytosis, electron microscopy, histopathology, immunoglobulin, stomach
MeSH terms: Case Reports

Crystal-storing histiocytosis (CSH) is a rare disorder characterized by the accumulation of non-neoplastic histiocytes that contain intracytoplasmic crystallized immunoglobulins. Although CSH can occur in various organs, gastric CSH is very rare. Therefore, diagnosing gastric CSH remains a challenge. Here, we present the case of a 69-year-old man with localized gastric CSH who presented with positive fecal occult blood for 2 days. Gastroscopy showed that there was a piece of irregular whitish focus in the big bend of the gastric antrum, which was soft and elastic. Histologically, the biopsied gastric mucosa showed chronic inflammation, mild activity with erosion, and numerous eosinophilic mononuclear cells containing fibrillary crystalloid inclusions in the lamina propria. Immunohistochemically, these crystal-containing cells were positive for CD68/PGM1 and Igk, which revealed that the cells were histiocytes harboring kappa light chain-restricted immunoglobulin crystals. Electron microscopic examination showed numerous high-electron-density particles in the cytoplasm of cells, with crystal structures of different sizes and shapes. This case highlights how immunohistochemistry can help with differential diagnosis and classification.
A case of ascending colon cancer accompanied with tumor thrombosis in the superior mesenteric vein treated with right hemicolectomy and greater saphenous vein grafting
Authors: Shintaro Akabane, Shoichiro Mukai, Hiroyuki Egi, Tomohiro Adachi, Masatoshi Kochi, Koki Sato, Yusuke Sumi, Ikki Nakashima, Kazuhiro Taguchi, Haruki Sada, Akira Ishikawa, Wataru Yasui, Hideki Ohdan
Journal: Int J Surg Case Rep (2018)
Keywords: case report, colorectal cancer, greater saphenous vein grafting, tumor thrombosis

Introduction:
The occurrence of colorectal cancer with tumor thrombosis in the mesenteric vein is very rare. Here, we report a case of ascending colon cancer with tumor thrombosis in the superior mesenteric vein (SMV) that was treated by complete resection.
Presentation of case:
A 48-year-old woman was initially admitted due to anemia. Ascending colon cancer coinciding with tumor thrombosis in the SMV was detected. Right hemicolectomy, tumor thrombectomy, and greater saphenous vein grafting of the SMV were performed. She underwent neoadjuvant chemotherapy with capecitabine plus oxaliplatin and did not have any recurrence.
Discussion:
Due to the high incidence of liver metastasis, the presence of venous tumor thrombosis may influence the patient's length of survival.
Conclusion:
Complete resection of the primary cancer with tumor thrombosis and systemic chemotherapy should be considered for better prognosis.
Lipofibromatous hamartoma of the median nerve: A case report and review of the literature
Authors: V S Patil, Sunila Nagle
Journal: Indian J Plast Surg (2009)
MeSH terms: Case Reports

A case of lipofibromatous hamartoma of the median nerve in an adult is described in this article. A 33-year-old male presented with tingling, numbness and swelling in the palm of the left non dominant hand that had been present for a few months. Examination revealed that there was fullness in the volar aspect of the wrist and in the inter-thenar area. Another mass was present at the base of the index finger, which appeared to be involving subcutaneous tissues. The clinical diagnosis was carpal tunnel syndrome due to a space occupying tumor mass in the carpal tunnel. On exploration of the carpal tunnel, a large median nerve was seen 4 cm proximal to the wrist crease line and extending distally until it divided into its branches. Another mass was present at the base of the index finger, which was adherent to the skin. The radial digital nerve of the index finger was normal in size. For the enlarged nerve, an epineurotomy was performed and a biopsy was taken. Another biopsy was taken from the distal mass. His postoperative period was uneventful. In July 2004, at the end of 5 years, the patient had no symptoms and the size of the tumor had not increased.
Giant cell tumor of the pes anserine bursa (extra-articular pigmented villonodular bursitis): a case report and review of the literature
Authors: Haitao Zhao, Aditya V Maheshwari, Dhruv Kumar, Martin M Malawer
Journal: Case Rep Med (2011)
MeSH terms: Case Reports

Pigmented villonodular synovitis (PVNS) is a rare, benign, proliferating disease affecting the synovium of joints, bursae, and tendon sheaths. Involvement of bursa (PVNB, pigmented villonodular bursitis) is the least common, and only few cases of exclusively extra-articular PVNB of the pes anserinus bursa have been reported so far. We report a case of extra-articular pes anserine PVNB along with a review of the literature. The lesion presented as a painful soft tissue mass in the medial part of the proximal leg. A magnetic resonance imaging showed areas of low to intermediate signals in all sequences and the lesion enhanced heterogeneously with contrast. Diagnosis was confirmed by an incisional biopsy, and an intralesional resection was performed. The postoperative course was uneventful, and the patient is free of disease with no functional deficit at 2 years followup. As with other rare lesions, clinical and radiographic findings in addition to histological examination are essential for correct diagnosis.
Chronic granulomatous Aspergillus synovitis: a case report
Authors: Aylin Canbolat Ayhan, Korhan Ozkan, Cetin Timur, Birol Aktaş, Ayse Bahar Ceyran
Journal: Mediterr J Hematol Infect Dis (2013)
MeSH terms: Case Reports

Aspergillus can cause invasive disease of various organs especially in patients with weakened immune systems. Aspergillus synovitis and arthritis are uncommon types of involvement due to this infection. Approaches to fungal osteoarticular infections are based on only case reports. This paper presents a rare case of chronic granulomatous Aspergillus synovitis in an immunocompromised 5-year old girl who was treated for acute lymphoblastic leukemia.
Case report: Post-thoracoscopy pendelluft monitoring
Authors: Zhibin Xiao, Na Zhang, Xiajing Zhang, Wenjun Lu, Changjun Gao, Xude Sun
Journal: Front Med (Lausanne) (2023)
Keywords: electrical impedance tomography, lung injury, pendelluft, post-thoracoscopy, pulmonary ventilation
MeSH terms: Case Reports

Asynchronous alveolar ventilation is called pendelluft, which may induce lung injury in spontaneously breathing patients. We report a case that electrical impedance tomography (EIT) was used to assess the pendelluft in a post-thoracoscopy patient. The pendelluft amplitude was as high as 77.5% of the tidal variation. The average regional time shift was 0.5 s. The patient was instructed to adjust the breathing method, symptomatic treatment was performed, and the symptoms were improved. This is the first case reporting pendelluft in a post-thoracoscopy patient. Our case demonstrated that (1) pendelluft may occur in post-thoracoscopy patients and it effects lung function, and (2) early identification of affected patients and implementation of corresponding treatments could improve patient outcomes.
Central venous catheter-associated pericardial tamponade in a 6-day old: a case report
Authors: Swati O Arya, Gurumurthy M Hiremath, Kingsley C Okonkwo, Michael D Pettersen
Journal: Int J Pediatr (2009)
MeSH terms: Case Reports

Introduction. Pericardial effusion (PCE) and tamponade can cause significant morbidity and mortality in neonates. Such cases have been reported in the literature in various contexts. Case Presentation. A 6-day old neonate with meconium aspiration syndrome and persistent pulmonary hypertension of newborn on high frequency oscillator ventilation and inhaled nitric oxide was referred to our hospital with a large pericardial effusion causing hemodynamic compromise. Prompt pericardiocentesis led to significant improvement in the cardio-respiratory status and removal of the central line prevented the fluid from reaccumulating. Cellular and biochemical analysis aided in the diagnosis of catheter related etiology with possibility of infusate diffusion into the pericardial space. Conclusion. We present this paper to emphasize the importance of recognizing this uncommon but serious complication of central venous catheters in intensive care units. We also discuss the proposed hypothesis for the mechanism of production of PCE.
Best vitelliform macular dystrophy in a large Brazilian family
Authors: Carolina Pádua Rocha de Souza, Luiz Guilherme Marchesi Mello, Fernando Gomez, Eduardo Morizot
Journal: Int J Retina Vitreous (2019)
Keywords: autofluorescence, fluorescein angiography, optical coherence tomography, retina, vitelliform macular dystrophy

Background:
To describe the clinical and multimodal imaging findings of a Brazilian family with Best vitelliform macular dystrophy.
Methods:
A retrospective chart review of a Brazilian family was conducted and complementary fundus images (color photography, autofluorescence, fluorescein angiography and optical coherence tomography) were analyzed.
Results:
Seven patients had typical macular lesions at different stages of Best vitelliform macular dystrophy. Electrooculography was performed in two of them and showed abnormal Arden ratio. The pedigree strongly suggests an autosomal dominant inheritance. Low visual acuity was mainly associated with advanced age, retinal pigment epithelium atrophy, and photoreceptors damage. However, yellow subretinal deposits were evidenced in patients with better visual acuity.
Conclusion:
We present the largest case series of a Brazilian family with Best vitelliform macular dystrophy. Multimodal imaging analysis is important to determine retinal abnormalities. Retinal pigment epithelium atrophy and loss of photoreceptors outer segments seem to be a late but important finding related to severe visual acuity impairment.
Single-Agent Thalidomide for Treatment of Malignant Paraganglioma of the Organ of Zuckerkandl
Authors: Mackenzie L Shindorf, Prabir K Chaudhuri
Journal: Case Rep Med (2019)
MeSH terms: Case Reports

The standard of care for benign pheochromocytomas and paragangliomas is surgical resection; however, there is no definitive curative or standard therapy for the treatment of malignant pheochromocytomas or paragangliomas. Current therapeutic options include surgical resection, chemotherapy (CVD therapy), MIBG-radiotherapy, somatostatin analogues, and combination targeted therapies such as temozolomide and thalidomide. Although some patients demonstrate short-lived responses to these various therapies, there has been no statistically significant survival benefit with any of the current regimens and therefore the aim is palliation and symptom control. Here, we present a 38-year-old man found to have an unresectable metastatic paraganglioma located in the Organ of Zuckerkandl who has been treated with single-agent thalidomide for over 15 years with minimal growth and stabilization of metastatic lesions. The management of our patient with single-agent therapy for this extended period of time challenges the efficacy and role of chemotherapy and other accepted therapies for malignant pheochromocytoma and paraganglioma.