Tacrolimus therapy in primary Sjögren's syndrome with refractory immune thrombocytopenia: a retrospective study.

Objectives: To evaluate the efficacy and safety of tacrolimus (TAC) for the treatment of primary Sjögren's syndrome (pSS) with refractory immune thrombocytopenia (RITP).

Methods: Twenty-three pSS patients with RITP treated with TAC from June 2018 to June 2021 at the First Affiliated Hospital of Soochow University were enrolled in this retrospective cohort study. Platelet response, clinical and immunological parameters, toxicity and safety were compared and analysed at baseline and different points after TAC treatment.

Clinical Outcome Discrimination in Pediatric ARDS by Chest Radiograph Severity Scoring.

Background: There is no accurate radiological measurement to estimate the severity of pediatrics acute respiratory distress syndrome (PARDS). We validated the effectiveness of an adult radiographic assessment of lung edema (RALE) score in PARDS.

Aim: To assess the severity and prognosis of PARDS based on a chest radiograph (CXR) RALE scoring method.

[Clinical and genetic features of Mowat-Wilson syndrome: an analysis of 3 cases].

Mowat-Wilson syndrome (MWS) is a rare autosomal dominant genetic disease caused by zinc finger E-box-binding homeobox 2 (ZEB2) gene mutation and has various clinical manifestations including intellectual disability/global developmental delay, unusual facies and multiple congenital malformations. This article reports the clinical features and gene mutations of three children diagnosed with MWS by ZEB2 gene analysis. All three children had Hirschsprung disease and unusual facies.

Reports of three cases with the initial presentation of mesenteric vasculitis in children with system lupus erythematous.

We reviewed three cases of systemic lupus erythematosus (SLE) in children with mesenteric vasculitis (LMV) as initial presentation and analysed their clinical characteristics to improve the understanding of this disease. Three patients with SLE were admitted to our hospital and initially presented with gastrointestinal symptoms. We retrospectively analysed their clinical data, including clinical presentations, laboratory results, images and short- and long-term treatment outcomes.

Transcriptome Analysis Provides a Preliminary Regulation Route of the Ethylene Signal Transduction Component, SlEIN2, during Tomato Ripening.

Ethylene is crucial in climacteric fruit ripening. The ethylene signal pathway regulates several physiological alterations such as softening, carotenoid accumulation and sugar level reduction, and production of volatile compounds. All these physiological processes are controlled by numerous genes and their expression simultaneously changes at the onset of ripening.

Role of the Tomato Non-Ripening Mutation in Regulating Fruit Quality Elucidated Using iTRAQ Protein Profile Analysis.

Natural mutants of the Non-ripening (Nor) gene repress the normal ripening of tomato fruit. The molecular mechanism of fruit ripening regulation by the Nor gene is unclear. To elucidate how the Nor gene can affect ripening and fruit quality at the protein level, we used the fruits of Nor mutants and wild-type Ailsa Craig (AC) to perform iTRAQ (isobaric tags for relative and absolute quantitation) analysis.

Intrapulmonary lipoma: a case report and literature review.

Background: Intrapulmonary lipoma is extemely rare in children. So far, all reported pulmonary lipomas were from adult patients.

Methods: We present herein a case of intrapulmonary lipoma in a child and a review of the related literature.

[Chest CT features and outcome of necrotizing pneumonia caused by Mycoplasma pneumoniae in children (report of 30 cases)].

Objective: To summarize the chest CT features and outcome of necrotizing pneumonia (NP) caused by Mycoplasma pneumoniae in children and to review the changes of common inflammatory parameters in NP patients to help clinicians understand the proper timing of CT scan.

Method: The imaging data from 30 cases of Mycoplasma pneumoniae pneumonia in NP group and 24 cases with non-necrotizing Mycoplasma penumoniae pneumonia (control group) were analyzed retrospectively. The changes of common inflammatory parameters in NP group and control group were compared.

[Pulmonary surfactant protein gene mutation associated with pediatric interstitial lung disease: a case study and the review of related literature].

Objective: To report a case of pulmonary surfactant protein (SP) gene mutation associated with pediatric interstitial lung disease, and study the clinical diagnosis process and review of related literature, to understand the relationship between interstitial lung disease and SP gene mutation in infants and children.

Method: The clinical, radiological, histological, and genetic testing information of a case of SP gene mutation related pediatric interstitial lung disease were analyzed and related literature was reviewed.

Result: A 2-year-old girl without a history of serious illness was hospitalized because of the shortness of breath, cough, excessive sputum, and the progressive dyspnea.

[Clinical features, mutation of the GNAS1 and pathogenesis of progressive osseous heteroplasia].

Objective: To investigate the clinical features, mutation of the GNAS1 and pathogenesis of progressive osseous heteroplasia (POH).

Method: The typical clinical, pathological and radiographic features of a boy with POH were collected and summarized following family survey. The GNAS1 gene sequence of all family members were amplified by polymerase chain reaction (PCR) and the products were sequenced directly to identify the mutations.

[Preliminary study on normal aerification of paranasal sinuses in children].

Objective: To explore the normal aerification of paranasal sinuses in Chinese children with magnetic resonance imaging.

Methods: Two hundred and eighty Chinese children aged from 17 days to 14 years without any symptoms related to sinusitis were statistically analyzed in MRI features, including counting the number of paranasal sinus pneumatization and the maximum axial and sagittal area of the left maxillary.

Results: The pneumatization rate of maxillary sinus was 85% in children aged from 0 to 1 years.

[Chest radiographic findings in children with 2009 influenza A (H1N1) virus infection].

Objective: To evaluate chest radiographic findings of children with 2009 influenza (H1N1) virus infection.

Method: Data of 235 patients who had microbiologically confirmed H1N1 infection and available chest radiograph obtained between May 1(st) 2009 and Jan. 31(st) 2010 were retrospectively analyzed.