Presentation and clinical course of pediatric-onset versus adult-onset Takayasu arteritis—a systematic review and meta-analysis

Clinical Rheumatology - Takayasu arteritis (TAK) is a less common large-vessel vasculitis which can occur in either children or adults. However, differences between pediatric-onset and adult-onset...     

Clinical and radiological outcomes of total joint arthroplasty in patients with ochronotic arthropathy

European Journal of Orthopaedic Surgery & Traumatology - Alkaptonuria is a rare metabolic disorder, and only a few case reports of total joint arthroplasty (TJA) for ochronotic arthropathy are...     

Management of pediatric differentiated thyroid cancer: An overview for the pediatric oncologist

Differentiated thyroid cancer (DTC) is the most common childhood thyroid malignancy. The standard of care for pediatric DTC is total thyroidectomy followed by radioactive iodine (RAI) treatment when indicated. Molecular changes and potential therapeutic targets have been recently described in pediatric thyroid cancer. Pediatric oncologists are increasingly involved in the evaluation of thyroid nodules in childhood cancer survivors and in the management of advanced thyroid cancer. In 2015, the American Thyroid Association published management guidelines for children with DTC. We provide an overview of the current standard of care and highlight available targeted therapies for progressive or RAI refractory DTC.     

Prospective Randomized Comparison of Linear Endostaplers During Laparoscopic Sleeve Gastrectomy

Purpose

The development of Laparoscopic Linear Endostaplers (LLES) is crucial in minimally invasive approaches in bariatric surgery, but there have been very few published studies comparing 6-row LLES in Laparoscopic Sleeve Gastrectomy (LSG). The objective of this study was to compare two 6-row LLES in LSG.

Methods

A total of 60 patients were prospectively randomized to undergo LSG with either Medtronic Endo GIA? Tri-Staple technology (MTS) or AEON ? Endostapler(Lexington Medical) LLES. The measured parameters included patient demographics, comorbidity indices, LLES and specimen characteristics, postoperative symptoms, hospital stay, and total adverse events (AEs). Intraoperative bleeding was evaluated using five laparoscopic and corresponding endoscopic images of staple line before clip application, compared with a 1–5 Visual Analogue Scale (VAS), assessed by an independent bariatric surgeon who was blinded to the LLES used. Images of all cases were reviewed on the same day to increase test–retest reliability.

Results

Both groups were similar in patient demographics. Compared to MTS, AEON LLES group had significantly lower bleeding VAS scores in 4/5 laparoscopic images (pre-pyloric: 1.7?±?0.7 vs. 2.36?±?0.76, p?=?0.0007, mid-sleeve: 1.46?±?0.62 vs. 1.86?±?0.68, p?=?0.019, proximal sleeve: 1.6?±?0.77 vs. 2.0?±?0.83, p?=?0.038, gastro-esophageal junction: 1.43?±?0.67 vs. 1.86?±?0.77, p?=?0.014) and 3/5 endoscopic images (pre-pyloric: 1.56?±?0.56 vs. 2.36?±?0.76, p?=?0.006, incisura: 1.66?±?0.54 vs. 2.0?±?0.52, p?=?0.021, mid-sleeve: 1.63?±?0.49 vs. 2.0?±?0.45, p?=?0.005). There was no statistical difference in other parameters.

Conclusion

Both devices were equally safe and effective in terms of LLES and specimen characteristics, patient symptoms, hospital stay, and AEs. Bleeding VAS scores were significantly lower, favoring the AEON LLES.

Graphical abstract

     

Lumbar Lipomeningomyelocele Associated with Multiple Café au Lait Spots: A Case Report

We report on a child with several café au lait spots in association with a lumbar lipomeningomyelocele as an apparently new association. Cutaneous markers, the identification of which plays a crucial role in the early diagnosis and management of spinal malformations, can accompany occult spinal dysraphism. Herein we report a case of lumbar lipomeningomyelocele associated with an overlying café au lait spot that served as a marker of occult spinal dysraphism. The patient also had segmental café au lait spots on the face, making the association unique.     

GDF15 promotes simultaneous astrocyte remodeling and tight junction strengthening at the blood–brain barrier

Perivascular astrocyte processes (PAP) surround cerebral endothelial cells (ECs) and modulate the strengthening of tight junctions to influence blood–brain barrier (BBB) permeability. Morphologically altered astrocytes may affect barrier properties and trigger the onset of brain pathologies. However, astrocyte-dependent mediators of these events remain poorly studied. Here, we show a pharmacologically driven elevated expression and release of growth/differentiation factor 15 (GDF15) in rat primary astrocytes and cerebral PAP. GDF15 has been shown to possess trophic properties for motor neurons, prompting us to hypothesize similar effects on astrocytes. Indeed, its increased expression and release occurred simultaneously to morphological changes of astrocytes in vitro and PAP, suggesting modulatory effects of GDF15 on these cells, but also neighboring EC. Administration of recombinant GDF15 was sufficient to promote astrocyte remodeling and enhance barrier properties between ECs in vitro, whereas its pharmacogenetic abrogation prevented these effects. We validated our findings in male high anxiety-related behavior rats, an animal model of depressive-like behavior, with shrunk PAP associated with reduced expression of the junctional protein claudin-5, which were both restored by a pharmacologically induced increase in GDF15 expression. Thus, we identified GDF15 as an astrocyte-derived trigger of astrocyte process remodeling linked to enhanced tight junction strengthening at the BBB.     

Interpretation of cytogenetic results in multiple myeloma for clinical practice

The interpretation of cytogenetic abnormalities in multiple myeloma (MM) is often a challenging task. MM is characterized by several cytogenetic abnormalities that occur at various time points in the disease course. The interpretation of cytogenetic results in MM is complicated by the number and complexity of the abnormalities, the methods used to detect them and the disease stage at which they are detected. Specific cytogenetic abnormalities affect clinical presentation, progression of smoldering multiple myeloma (SMM) to MM, prognosis of MM and management strategies. The goal of this paper is to provide a review of how MM is classified into specific subtypes based on primary cytogenetic abnormalities and to provide a concise overview of how to interpret cytogenetic abnormalities based on the disease stage to aid clinical practice and patient management.