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Microsurgery 2019 Feb 15. Epub 2019 Feb 15.

Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.

Acute rejection is not uncommon after vascularized composite allotransplantation. We reported the effects of adjunctive topical immunosuppressant with topical tacrolimus (Protopic®) and steroid cream (Clobetasol®) in the management of acute rejection in two hand transplantation patients. Case 1 is a 45-year-old male with distal forearm deficit 4 years ago and Case 2 is a 30-year-old male with a proximal forearm deficiency 2 years ago, respectively. Read More

Front Pediatr 2018 31;6:429. Epub 2019 Jan 31.

Division of Pediatric Allergy and Immunology, Department of Pediatrics, University of Rochester Medical Center, Golisano Children's Hospital, Rochester, NY, United States.

Here we describe two term male infants diagnosed with X-linked CGD who present, in addition to frequent infection, with a unique papulopustular skin rash. CGD is caused by a number of genetic defects that impair phagocyte function. This disease results in recurrent infections and granuloma formation. Read More

Pediatr Rheumatol Online J 2019 Feb 14;17(1). Epub 2019 Feb 14.

Division of Immunolgy, Boston Children's Hospital, Boston, MA, USA.

Background: Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) were historically thought to be distinct entities, often managed in isolation. In fact, these conditions are closely related. A collaborative approach, which incorporates expertise from subspecialties that previously treated HLH/MAS independently, is needed. Read More

Front Med (Lausanne) 2018 29;5:363. Epub 2019 Jan 29.

Department of Dermatology and Venerology, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Junctional epidermolysis bullosa (JEB) is a hereditary blistering disease caused by reduced dermal-epidermal adhesion due to deficiencies of one of the proteins, laminin-332, type XVII collagen, integrin α6β4 or integrin α3. Significant progress has been achieved in the development of therapies for EB, such as bone-marrow transplantation, local or systemic injections with fibroblasts or mesenchymal stromal cells, readthrough of premature termination codons, or exon skipping. These were tailored in particular for dystrophic EB, which is caused by type VII collagen deficiency and have not yet reached broad clinical practice. Read More

Handchir Mikrochir Plast Chir 2019 Feb 12. Epub 2019 Feb 12.

Medizinische Hochschule Hannover, Klinik für Plastische, Ästhetische, Hand- und Wiederherstellungschirurgie, Replantationszentrum Niedersachsen, Schwerbrandverletztenzentrum Niedersachsen.

Background: Partial and full thickness burns require surgical treatment, such as early débridement and skin transplantation in MEEK/MESH technique or further reconstructive surgery. Infections of burns or transplanted areas limit surgical success and increase patient mortality. For split-thickness grafts in MEEK technique a superficial silk is applied as a protective on-top dressing, whereas in MESH technique fatty gauze and foam are used as standard protective covers over five to seven days. Read More

Hum Reprod 2019 Feb 12. Epub 2019 Feb 12.

Biology of the Testis Lab, Department of Reproduction, Genetics and Regenerative Medicine, Vrije Universiteit Brussel (VUB), Brussels, Belgium.

Study Question: Can full spermatogenesis be achieved after xenotransplantation of prepubertal primate testis tissue to the mouse, in testis or subcutaneously?

Summary Answer: Intratesticular xenotransplantation supported the differentiation of immature germ cells from marmoset (Callithrix jacchus) into spermatids and spermatozoa at 4 and 9 months post-transplantation, while in subcutaneous transplants, spermatogenic arrest was observed at 4 months and none of the transplants survived at 9 months.

What Is Known Already: Auto-transplantation of cryopreserved immature testis tissue (ITT) could be a potential fertility restoration strategy for patients with complete loss of germ cells due to chemo- and/or radiotherapy at a young age. Before ITT transplantation can be used for clinical application, it is a prerequisite to demonstrate the feasibility of the technique and identify the conditions required for establishing spermatogenesis in primate ITT transplants. Read More

JCI Insight 2019 Feb 12. Epub 2019 Feb 12.

Chimeric antigen receptor (CAR) technology can be used to engineer the antigen-specificity of regulatory T cells (Tregs) and improve their potency as an adoptive cell therapy in multiple disease models. As synthetic receptors, CARs carry the risk of immunogenicity, particularly when derived from non-human antibodies. Using an HLA-A*02:01-specific CAR (A2-CAR) encoding a single-chain Fv derived from a mouse antibody, we developed a panel of 20 humanized (h)A2-CARs. Read More

Am J Transplant 2019 Feb 12. Epub 2019 Feb 12.

MRC Centre for Transplantation, Peter Gorer Department of Immunobiology, Faculty of Life Sciences& Medicine, King's College London, London, UK.

Regulatory T cells (Tregs) have unique immunosuppressive properties and are essential to ensure effective immunoregulation. In animal models, Tregs have been shown to prevent autoimmune disorders and establish transplantation tolerance. Therefore, the prospect of harnessing Tregs, either by increasing their frequency or by conferring allospecificity, has prompted a growing interest in the development of immunotherapies. Read More

Am Surg 2018 Nov;84(11):1790-1795

Little data exist about management of wounds created by debridement in necrotizing soft tissue infections (NSTIs). Multiple wound coverage techniques exist, including complete primary wound closure, split-thickness skin grafting, secondary intention, and flap creation. We hypothesized that all wound coverage techniques would be associated with high rates of successful wound coverage and low crossover rates to other wound coverage techniques. Read More

Open Access Maced J Med Sci 2019 Jan 26;7(2):231-233. Epub 2019 Jan 26.

University of Rome G. Marconi, Rome, Italy.

Aim: To investigate the efficacy of plastic surgery in the treatment of giant congenital melanocytic nevus (GCMN).

Methods: We enrolled 20 patients with 44 lesions and performed one of the following procedures: serial excision, skin grafting, tissue expansion, primary skin closure, distant flap, and adjacent flap. We assessed the outcome at 10 days and 6 months after surgery. Read More

J Autoimmun 2019 Feb 8. Epub 2019 Feb 8.

Paris Descartes University, Cochin Institute, CNRS UMR8104, INSERM U1016, Paris, France. Electronic address:

Objectives: Adult immunoglobulin A vasculitis (IgAV) is an immune complex small vessel vasculitis. So far, the involvement of T cells in this pathology has been poorly studied. The aim of this study was to analyze T-cell homeostasis as well as cytokine and chemokine concentrations in the blood and tissues of IgAV patients. Read More

Transpl Immunol 2019 Feb 8. Epub 2019 Feb 8.

School of Chemistry and Molecular Bioscience, University of Wollongong, Wollongong, NSW 2252, Australia; Molecular Horizons, University of Wollongong, NSW 2252, Australia; Illawarra Health and Medical Research Institute, Wollongong, NSW 2252, Australia. Electronic address:

Graft-versus-host disease (GVHD) is a frequent complication following allogeneic hematopoietic stem cell transplantation (HSCT) with current therapies limited to general immunosuppression. Humanized mouse models of GVHD are emerging as valuable intermediaries to allow translation of findings from allogeneic mouse models to humans to prevent and treat this disease, but such models require further characterization. In this study, humanized mice were generated by injecting immunodeficient non-obese diabetic severe combined immunodeficiency interleukin-2 receptor γ common chain null (NSG) mice with human peripheral blood mononuclear cells (hPBMCs). Read More

Xenotransplantation 2019 Feb 11:e12490. Epub 2019 Feb 11.

Organ Transplant and Clinical Immunology Translational Medicine Key Laboratory of Sichuan Province, Sichuan Academy of an Transplant Science & Sichuan Provincial People's Hospital, Chengdu, China.

The main obstacle to organ transplantation is the shortage of organs from deceased individuals. Especially in China, the ratio of patients on the waiting list versus the transplant recipients is 30:1. Therefore, there is an urgent need for organ donors. Read More

Front Immunol 2019 25;10:46. Epub 2019 Jan 25.

Division of Infection and Immunity, Institute of Immunity and Transplantation, University College London, London, United Kingdom.

Regulatory T cells (Tregs) are believed to be dysfunctional in autoimmunity. Juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM) result from a loss of normal immune regulation in specific tissues such as joints or muscle and skin, respectively. Here, we discuss recent findings in regard to Treg biology in oligo-/polyarticular JIA and JDM, as well as what we can learn about Treg-related disease mechanism, treatment and biomarkers in JIA/JDM from studies of other diseases. Read More

Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi 2019 Feb;33(2):219-222

Department of orthopedics, the Joint Service Support Force 920 th Hospital of Chinese PLA, Kunming Yunnan, 650032, P.R.China.

Objective: To investigate the effectiveness of self-made limb chronic wound closure device in the treatment of scarred lower limbs and complex skin and soft tissue defects.

Methods: Between January 2014 and January 2017, 29 patients with complex fractures of the lower extremities and skin and soft tissue defects were treated. There were 19 males and 10 females with an average age of 31. Read More

Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi 2019 Feb;33(2):199-202

Department of Pediatric Orthopaedics, Wuxi No.9 People's Hospital Affiliated to Soochow University, Wuxi Jiangsu, 214000,

Objective: To explore the effectiveness of one-stage metacarpal osteotomy, thumb opponensplasty and polygonal flap reconstruction in the treatment of congenital spade hand deformity.

Methods: Eight cases of congenital spade hand were treated between January 2013 and March 2017. There were 5 males and 3 females, with an average age of 17. Read More

Cytotherapy 2019 Feb 6. Epub 2019 Feb 6.

Department of Applied Biology, Council of Scientific and Industrial Research(CSIR), Indian Institute of Chemical Technology, Hyderabad, TS, India; Academy of Scientific and Innovative Research (AcSIR), New Delhi, India. Electronic address:

Background: Neo-vascularization, an indispensible phenomenon for tissue regeneration, facilitates repair and remodeling of wound tissues. This process is impaired in chronic wounds due to reduced number and recruitment of endothelial cells (ECs), thereby necessitating development of newer strategies to enhance the EC repertoire as a therapeutic approach.

Methods: We explored the 'plasticity' of Wharton's jelly derived-mesenchymal stromal cells (WJ-MSCs) using an anti-inflammatory drug-mediated enhanced trans-differentiation into ECs, based on our observation of temporal decrease in COX-2 expression during trans-differentiation of MSCs into ECs at day 7 and 14 along with mature ECs. Read More

Int J Infect Dis 2019 Feb 6. Epub 2019 Feb 6.

Division of Infection and Immunity, Center for Clinical Microbiology, University College London and the National Institute of Health Research Biomedical Research Centre at University College London Hospitals NHS Foundation Trust, London, UK. Electronic address:

Background: Tuberculosis (TB) continues to be the commonest infectious disease cause of death worldwide. Tuberculosis is an important infectious disease cause of morbidity and death in renal transplant recipients. Tuberculosis can also cause loss of kidney allograft OBJECTIVES: The purpose of this viewpoint is to highlight the issues related to prevention, diagnosis and treatment of tuberculosis in renal transplant recipients. Read More

Nat Commun 2019 Feb 8;10(1):650. Epub 2019 Feb 8.

Department of Developmental and Cell Biology, University of California, Irvine, Irvine, CA, 92697, USA.

During wound healing in adult mouse skin, hair follicles and then adipocytes regenerate. Adipocytes regenerate from myofibroblasts, a specialized contractile wound fibroblast. Here we study wound fibroblast diversity using single-cell RNA-sequencing. Read More

Front Immunol 2018 24;9:3104. Epub 2019 Jan 24.

Department of Microbiology and Immunology, Miller School of Medicine, University of Miami, Miami, FL, United States.

A recent approach for limiting production of pro-inflammatory cytokines has been to target bromodomain and extra-terminal (BET) proteins. These epigenetic readers of histone acetylation regulate transcription of genes involved in inflammation, cardiovascular disease, and cancer. Development of BET inhibitors (BETi) has generated enormous interest for their therapeutic potential. Read More

Medicine (Baltimore) 2019 Feb;98(6):e14344

Department of Pathology, Konkuk University School of Medicine, Seoul, Korea.

Rationale: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy. This disease almost always presents with cutaneous involvement.

Patient Concerns: The 1st patient was a 16-year-old girl who presented with recurrent epistaxis. Read More

Pediatr Blood Cancer 2019 Feb 7:e27647. Epub 2019 Feb 7.

Departments of Pediatrics, Pathology and Medicine, McMaster University, Hamilton, Ontario, Canada.

Background: At least 80% of children with cancer live in low- and middle-income countries where the prevalence of malnutrition and socioeconomic disadvantage is high. We examined the relationship between nutritional status (NS), assessed by arm anthropometry, and socioeconomic status (SES) in children diagnosed with cancer at Unidad Nacional de Oncologia Pediatrica (UNOP) in Guatemala over a three-year period.

Method: Patients aged 0 to 18 years of age diagnosed between January 2015 and December 2017 were included. Read More

J Surg Oncol 2019 Feb 6. Epub 2019 Feb 6.

Department of Plastic and Reconstructive Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland.

Background And Objectives: Two common options for the closure of complex defects are local flaps and skin grafting. The keystone flap, a fasciocutaneous flap based on perforators, has demonstrated compelling ease of use, reproducibility, and low complication rates without requiring a distant donor site. Our objective for this study was to compare postoperative outcomes for keystone flaps and skin grafts in cancer resection. Read More

Sci Rep 2019 Feb 6;9(1):1490. Epub 2019 Feb 6.

Section of Gene Expression Regulation, Frontier Science Research Center, Kagoshima University, Kagoshima, Japan.

Human tissue-specific stem cells (hTSCs), found throughout the body, can differentiate into several lineages under appropriate conditions in vitro and in vivo. By transfecting terminally differentiated cells with reprogramming factors, we previously produced induced TSCs from the pancreas and hepatocytes that exhibit additional properties than iPSCs, as exemplified by very low tumour formation after xenogenic transplantation. We hypothesised that hTSCs, being partially reprogrammed in a state just prior to iPSC transition, could be isolated from any terminally differentiated cell type through transient reprogramming factor overexpression. Read More

Nat Commun 2019 Feb 6;10(1):616. Epub 2019 Feb 6.

Laboratory of Molecular Immunology, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health, Bethesda, 20892, MD, USA.

Hematopoietic chimerism after allogeneic bone marrow transplantation may establish a state of donor antigen-specific tolerance. However, current allotransplantation protocols involve genotoxic conditioning which has harmful side-effects and predisposes to infection and cancer. Here we describe a non-genotoxic conditioning protocol for fully MHC-mismatched bone marrow allotransplantation in mice involving transient immunosuppression and selective depletion of recipient hematopoietic stem cells with a CD117-antibody-drug-conjugate (ADC). Read More

Rinsho Ketsueki 2019 ;60(1):22-27

Department of Hematology, Atomic Bomb Disease and Hibakusha Medicine Unit, Atomic Bomb Disease Institute, Nagasaki University.

A 44-year-old male was diagnosed with acute myeloid leukemia with a complex karyotype. He underwent bone marrow transplantation using an HLA 6/6 antigen-matched sibling donor, but developed chronic graft-versus-host disease (cGVHD) with skin erythema and oral and esophageal lichen planus changes. Treatment with a combination of prednisolone and cyclosporine was initiated on day 646 after transplantation, but oral symptoms persisted. Read More

J Bras Pneumol 2018 Nov-Dec;44(6):461-468

. Faculdade de Medicina, Universidade Federal de Minas Gerais, Belo Horizonte (MG) Brasil.

Objective: To estimate the prevalence of latent Mycobacterium tuberculosis infection (LTBI) in renal transplant recipients and to assess sociodemographic, behavioral, and clinical associations with positive tuberculin skin test (TST) results.

Methods: This was a cross-sectional study of patients aged ≥ 18 years who underwent renal transplantation at the Renal Transplant Center of the Federal University of Minas Gerais Hospital das Clínicas, located in the city of Belo Horizonte, Brazil. We included renal transplant recipients who underwent the TST between January 2011 and July 2013. Read More

JAMA Dermatol 2019 Feb 6. Epub 2019 Feb 6.

Department of Nephrology and Kidney Transplantation, Beaumont Hospital Dublin, Ireland.

Importance: Existing data suggest that nonmelanoma skin cancer (NMSC) is more common in renal transplant recipients than in maintenance dialysis patients. However, whether the risk of NMSC varies as the treatment modality for end-stage kidney disease (ESKD) changes between dialysis and transplantation is not well described.

Objective: To determine whether the incidence of NMSC is attenuated during periods of graft loss with a return to dialysis in those who receive multiple kidney transplants. Read More

J Pediatr Gastroenterol Nutr 2019 Feb 4. Epub 2019 Feb 4.

Liver Unit and Small Bowel Transplantation, Birmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.

Front Pediatr 2018 22;6:413. Epub 2019 Jan 22.

Department of Pediatrics, University Hospital Würzburg, Würzburg, Germany.

Henoch-Schönlein Purpura (HSP) or IgA vasculitis is the most common systemic vasculitis of childhood and may affect skin, joints, gastrointestinal tract, and kidneys. Skin manifestations of HSP are characteristic and include a non-thrombocytopenic palpable purpura of the lower extremities and buttocks. Rarely, HSP may initially present as or evolve into hemorrhagic vesicles and bullae. Read More

Scars Burn Heal 2019 Jan-Dec;5:2059513118821563. Epub 2019 Jan 14.

Nottingham University Hospitals NHS Trust, Nottingham, UK.

Introduction: Enzymatic debridement for mixed depth and full thickness burns is gaining recognition as an important technique available to the burns surgeon. Reductions in blood loss, the need for autologous skin grafting and the number of wounds requiring surgical excision are among the benefits of this evolving treatment modality. We present a case of successful treatment using enzymatic debridement of mixed depth flame burns in a young patient with systemic sclerosis (scleroderma). Read More

SAGE Open Med Case Rep 2019 22;7:2050313X18818724. Epub 2019 Jan 22.

Department of Cardiovascular Surgery, Ehime Prefectural Central Hospital, Matsuyama, Japan.

Neurofibromatosis type 1, also called von Recklinghausen's disease, is a hereditary congenital disorder that affects tissues of neuroectodermal or mesodermal origin. This disease has various manifestations, including pigmented skin lesions, cutaneous neurofibromas, skeletal abnormalities, and tumors of the central/peripheral nervous and gastrointestinal systems, and vascular abnormalities. Because of vasculopathy, part of the vessel wall may be replaced by neurofibromatosis tissue. Read More

Microsurgery 2019 Feb 3. Epub 2019 Feb 3.

Division of Plastic Surgery, Department of Surgery, University of Wisconsin-Madison, Madison, Wisconsin.

Penile amputation is a rare injury with significant posttraumatic ramifications. We present a case of successful immediate replantation after traumatic penile avulsion in a 52-year-old male patient. The patient reported his penis was caught in machinery during a boating accident, and avulsed at the level of the pubis. Read More

Biol Blood Marrow Transplant 2019 Jan 31. Epub 2019 Jan 31.

Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA; Medical Oncology, Department of Medicine. Electronic address:

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired clonal hematopoietic cell disease characterized by destruction of hematopoietic cells through the activation of the complement system with manifestations that can be life threatening including hemolysis, thrombosis, and marrow failure. Allogeneic hematopoietic cell transplantation (HCT) remains the only cure for PNH, but eculizumab, a terminal complement inhibitor of C5, has been used to prevent complement mediated hemolysis in PNH since its FDA approval in 2007. We examined outcomes of HCT in PNH patients to evaluate the effects of disease subtype, conditioning intensity, and eculizumab use either pre- or post-HCT. Read More

J Orthop Sci 2019 Jan 29. Epub 2019 Jan 29.

Department of Orthopaedic Surgery, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Wenzhou, 325000, China. Electronic address:

Background: Degloving injuries of the limb are severe and frequently underrated. Few researches are available comparing the results of reattachment of the degloved skin grafts with the vacuum sealing drainage technique and the traditional compression dressing method. In this study, we aimed to compare the treatment outcomes of these two approaches. Read More

Ann Dermatol Venereol 2019 Jan 29. Epub 2019 Jan 29.

Unité Inserm U1058, département de dermatologie, université Montpellier, hôpital Saint-Éloi, 80, avenue Augustin-Fliche, 34295 Montpellier cedex 5, France. Electronic address:

Therapeutic progress in primary cutaneous lymphomas continues to be largely dominated by the T-cell lymphomas, towards which the great majority of recent therapeutic innovations have been directed. The latter include local treatments consisting either of relatively classical but "revamped" approaches involving different pharmaceutical forms (example: chlormethine gel) or else lower but seemingly equally effective dosages (electron therapy), or of more innovative approaches (example: UVA-1, dynamic phototherapy, imiquimod, resimiquimod). However, significant progress has been made chiefly in terms of systemic treatments with the emergence of "targeted" drugs that directly and specifically target tumour cells (monoclonal antibodies directed against CD30, CCR4 or CD158k) and the further development of "small" molecules such as histone deacetylase inhibitors and new cytostatics. Read More

Biopreserv Biobank 2019 Feb 1. Epub 2019 Feb 1.

2 Institute of Advanced Biomedical Engineering and Science, Tokyo Women's Medical University, School of Medicine, Tokyo, Japan.

During pulmonary resections, removal of visceral pleura is frequently required, resulting in lung air leakage (LAL) and bleeding. Especially persistent LAL after pulmonary surgery has negative consequences. Current surgical procedures are ineffective in closing these visceral pleural injuries. Read More

Int J Paediatr Dent 2019 Feb 1. Epub 2019 Feb 1.

Cukurova University, Faculty of Dentistry, Department of Pediatric Dentistry, Adana, Turkey.

Background: The causes for intrinsic tooth discoloration can be separated into two categories as systemic and local. Systemic causes are either genetic or drug induced effects. The development of dentition can also be affected by a number of systemic factors and metabolic diseases such as porphyria. Read More

Immunol Med 2019 Jan 31:1-8. Epub 2019 Jan 31.

a Laboratory Molecular Genetics of Hematology Graduate School of Medical and Dental Sciences , Tokyo Medical and Dental University (TMDU) , Tokyo , Japan.

Chronic active Epstein-Barr virus infection (CAEBV) is one of the Epstein-Barr virus (EBV)-positive T- or NK-cell lymphoproliferative diseases. It is characterized by clonal proliferation of EBV-infected T or NK cells and their infiltration into systemic organs, leading to their failure. Inflammatory symptoms, fever, lymphadenopathy and liver dysfunction are main clinical findings of CAEBV. Read More

Am J Med Genet A 2019 Mar 31;179(3):442-447. Epub 2019 Jan 31.

Laboratory of Skin Biology, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD.

An international advisory group met at the National Institutes of Health in Bethesda, Maryland in 2017, to discuss a new classification system for the ectodermal dysplasias (EDs) that would integrate both clinical and molecular information. We propose the following, a working definition of the EDs building on previous classification systems and incorporating current approaches to diagnosis: EDs are genetic conditions affecting the development and/or homeostasis of two or more ectodermal derivatives, including hair, teeth, nails, and certain glands. Genetic variations in genes known to be associated with EDs that affect only one derivative of the ectoderm (attenuated phenotype) will be grouped as non-syndromic traits of the causative gene (e. Read More

Exp Dermatol 2019 Jan 31. Epub 2019 Jan 31.

Department of Dermatology, Seoul National University College of Medicine, Seoul, Republic of Korea.

Hair follicle reconstitution requires highly organized epithelial-mesenchymal interactions. Skin equivalents containing the epidermal and dermal cells with hair reconstitution capacity can reproduce these processes, but have not been established. This study was conducted to develop a hair follicle-producing three-dimensional (3D) skin equivalent assay using neonate mouse epidermal and dermal cells. Read More

Ter Arkh 2018 Aug;90(7):57-64

Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology, Department of optimization treatment and prevention of complications of hematopoietic stem cell transplantation, Moscow, Russia.

Aim: The main goal is to optimize hematopoietic stem cell transplantation (HSCT) approach among adolescents and young adults with paroxysmal nocturnal hemoglobinuria (PNH) by means of Graft-versus-host disease (GVHD) and post-transplant complications risk lowering.

Materials And Methods: We report our experience of HSCT from HLA-matched unrelated donors using TCR alfa/beta and CD19 depletion in 5 pts (1M/4F) with PNH, developed after successful immunosuppressive therapy (IST) of acquired aplastic anemia (AA). Median age of pts at the moment of transplantation was 17,8 years (range 14,5-22,7), median interval from IST to PNH was 4 years (5mo - 6,5 y). Read More

Clin Exp Dermatol 2019 Jan 30. Epub 2019 Jan 30.

Department of Nephrology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.

Background: Solid-organ transplant recipients (SOTRs) are at risk of developing vitamin D deficiency, mainly caused by reduced sunlight exposure with subsequent low vitamin D synthesis in the skin.

Aim: To analyse whether SOTRs from a Spanish Mediterranean region were vitamin D-deficient.

Methods: This was a cross-sectional, descriptive and observational study in a transplantation-specialized Dermatological Unit from a Mediterranean area to determine the calcidiol levels of a cohort of 78 consecutively attending patients not receiving vitamin D supplements. Read More

J Coll Physicians Surg Pak 2019 Feb;29(2):173-174

Department of Hepato-Gastroenterology, Sindh Institute of Urology and Transplantation (SIUT), Karachi, Pakistan.

A young girl presented to us with recurrent diarrhea along with a history of 5 kg weight loss in one year. On examination, she appeared pale, while her laboratory reports showed a low hemoglobin, mean corpuscular volume (MCV) and serum albumin. Her erythrocyte sedimentation rate (ESR) was slightly raised with her iron profile suggestive of iron deficiency anemia. Read More

Int J Burns Trauma 2018 20;8(6):145-148. Epub 2018 Dec 20.

College of Medicine, King Saud bin Abdulaziz University for Health Sciences Riyadh, Saudi Arabia.

Background: Mortality in major burns is caused mainly by multisystem organ failure. Brain lesions in burn patients are rare and predominantly traumatic in nature. Here, we present an unusual case of burn-induced glioma causing rapid neurological deterioration and death. Read More

Exp Clin Transplant 2019 Jan 29. Epub 2019 Jan 29.

From Inonu University Liver Transplantation Institute, Malatya, Turkey.

Objectives: Hepatic vein outflow obstruction in liver transplantation can lead to graft or patient loss. We used an intrauterine balloon to overcome this complication in 13 liver transplant recipients. Here, we report the results of these cases; our report, as far as we know, involves the highest number of patients on this issue. Read More

Wounds 2019 Feb;31(2):36-40

Christiana Care Health System, Newark, DE.

Introduction: Modern wound management continues to present new challenges. Many patients elect to forego operative debridement secondary to high risk, fear, cost concerns, and personal ideologies on healing. Although operative debridement has long been a tenet of proper wound care, alternative innovative approaches to wound management must be considered. Read More

Front Immunol 2018 14;9:2767. Epub 2019 Jan 14.

Paediatric Hematology-Oncology, Ospedale della Donna e del Bambino, Verona, Italy.

Adenosine deaminase 2 (ADA2) deficiency is an auto-inflammatory disease due to mutations in cat eye syndrome chromosome region candidate 1 () gene, currently named . The disease has a wide clinical spectrum encompassing early-onset vasculopathy (targeting skin, gut and central nervous system), recurrent fever, immunodeficiency and bone marrow dysfunction. Different therapeutic options have been proposed in literature, but only steroids and anti-cytokine monoclonal antibodies (such as tumor necrosis factor inhibitor) proved to be effective. Read More

J Surg Res 2019 Mar 3;235:308-314. Epub 2018 Nov 3.

Department of Surgery, Waitemata District Health Board, New Zealand, and Melanoma Unit, Takapuna, Auckland, New Zealand.

Background: Skin cancer rates in New Zealand are estimated to be the highest in the world. Split-skin grafting is a common procedure after skin cancer excision in the lower limb. We sought to evaluate the cost-effectiveness of negative pressure wound therapy (NPWT) with same-day discharge in patients undergoing split-skin grafting of the lower limb for the treatment of skin cancer. Read More