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Zhonghua Er Ke Za Zhi 2021 Mar;59(3):223-227

Department of Nephrology and Rheumatology, Children's Hospital of Shanghai, Children's Hospital of Shanghai Jiao Tong University, Shanghai 200062, China.

To investigate the clinical characteristics, treatment and prognosis of TRPC6 variation induced children with steroid-resistant nephrotic syndrome (SRNS). Clinical data of four patients with nephrotic syndrome carrying TRPC6 variations, who were admitted to the Department of Nephrology and Rheumatology, Children's Hospital of Shanghai from Jan. 2017 to Dec. Read More

Zhonghua Er Ke Za Zhi 2021 Mar;59(3):218-222

Department of Endocrinology, Genetics and Metabolism, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.

To analyze the clinical, genetic characteristics and follow-up data of Chinese patients with hypophosphatasia (HPP). A retrospective analysis was conducted on six children with HPP admitted to the Department of Endocrinology, Genetics and Metabolism in Beijing Children's Hospital from October 2010 to January 2019. Summarized the clinical and follow-up data of all six patients, as well as the pathogenic variants of five children. Read More

Zhonghua Er Ke Za Zhi 2021 Mar;59(3):212-217

Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.

To investigate the clinical characteristics, treatment and prognosis of children with acquired thrombotic thrombocytopenic purpura (TTP). The clinical manifestations, laboratory examination, treatment and prognosis of 5 children with acquired TTP hospitalized in Beijing Children's Hospital, Capital Medical University from January 2016 to July 2019 were analyzed retrospectively. There were 5 children with acquired TTP including 2 males and 3 females, with the onset age of 8. Read More

Zhonghua Er Ke Za Zhi 2021 Mar;59(3):195-200

Department of Oncology Surgery, Children's Hospital of Anhui Medical University, Hefei 230022, China.

To summarize the effect of Chinese Children's Cancer Group (CCCG) Wilms tumor (WT)-2015 protocol. This was a prospective study. CCCG-WT-2015 protocol was revised on the basis of the CCCG-WT-2009 protocol. Read More

Br J Dermatol 2021 Mar 3. Epub 2021 Mar 3.

ToxiTEN group, European Reference Network for rare skin diseases (ERN skin).

Background: Supportive care is the cornerstone of adult and pediatric management of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking.

Objectives: Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN. Read More