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Juvenile Systemic Sclerosis: About 9 Cases

Open Journal of Pediatrics 2024年第2期14卷 320-326页

作者： Kaoutar Danaoui Houda Nassih Khadija Oujennane Rabiy el Qadiry Aicha bourrahouat Said Amal Imane Ait sab b Pediatric Ward Mohammed VI University Hospital Center Marrakesh Morocco Dermatology Ward Mohammed VI University Hospital Center Marrakesh Morocco

Scleroderma is a rare disease with two primary forms: localized scleroderma (LS) and systemic sclerosis (SSc). both are chronic conditions that can manifest in various patterns (subtypes) and are linked to extracutaneous involvement in pediatric patients. Juvenile SSc poses a higher risk of morbidity and mortality, with patients facing life-threatening complications such as lung, heart, and visceral organ fibrosis, and vasculopathy. In contrast, mortality is extremely rare in juvenile LS, but patients are susceptible to significant morbidity, leading to severe disfigurement and functional impairment. Treatment for scleroderma aims to control inflammation and address specific issues. An early diagnosis significantly enhances the overall outcome. This study conducts a retrospective descriptive analysis aiming to document the clinical manifestations, management approaches, and outcomes of systemic sclerosis in a cohort of nine children receiving treatment for juvenile systemic sclerosis at Pediatric b department of Mohammed VI University, Hospital Center in Marrakech, Morocco.

关键词： Scleroderma Systemic Sclerosis Children Pediatric

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Human leukocyte antigen typing and crossmatch:A comprehensive review

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World Journal of Transplantation 2017年第6期7卷 339-348页

作者： Mohammed Mahdi Althaf Mohsen el Kossi Jon Kim Jin Ajay Sharma Ahmed Mostafa Halawa Jack Pryor Renal Unit Norfolk and Norwich University Hospital-NHS Foundation Trust Faculty of Health and Science University of LiverpoolInstitute of Learning and TeachingSchool of Medicine Doncaster Royal Infirmary Nottingham Children Hospital Royal Liverpool University Hospitals Department of Transplantation Surgery Sheffield Teaching Hospitals

Renal transplantation remains the best option for patients suffering from end stage renal disease(ESRD).Given the worldwide shortage of organs and growing population of patients with ESRD,those waitlisted for a transplant is ever *** crossmatch methods and human leukocyte antigen(HLA) typing play a pivotal role in improving organ allocation and afford better matches to *** crossmatch as well as HLA typing for renal transplantation and applying it in clinical practice is the key step to achieve a successful *** of crossmatch results can be quite challenging where clinicians have not had formal training in applied transplant *** review aims to provide a worked example using a clinical ***,each technique is discussed in detail with its pros and *** index case is that of a young male with ESRD secondary to Lupus *** is offered a deceased donor kidney with a 1-0-0 *** complement dependent cytotoxicity(CDC) crossmatch reported positive for b lymphocyte,but flow cytometry crossmatch(FCXM) was reported negative for both b and T ***-sab(single antigen bead) did not identify any donor specific antibodies(DSA).He never had a blood *** positive CDCcrossmatch result is not concordant with DSA *** implausible results are due to underlying lupus erythematosus,leading to false-positive b-lymphocyte crossmatch as a result of binding immune complexes to *** positive report of CDC crossmatch can be caused by the underlying autoimmune diseases such as lupus erythematosus,that may lead to inadvertent refusal of adequate kidney *** study of DSA by molecular technique would prevent wrong exclusion of such *** on these investigations this patient is deemed to have "standard immunological risk" for renal transplantation.

关键词： Human leukocyte antigen typing Cytotoxic crossmatch Flow cytometry crossmatch Virtual crossmatch Human leukocyte antigen null alleles

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