[A quality improvement project on reducing antibiotic use duration in very low birth weight preterm infants in the neonatal intensive care unit].

Objectives: To develop effective measures to reduce antibiotic use duration in very low birth weight (VLBW) preterm infants in the neonatal intensive care unit through quality improvement methods.

Methods: The study population consisted of hospitalized VLBW preterm infants, with the percentage of hospitalization time during which antibiotics were used from November 2020 to June 2021 serving as the baseline. The specific quality improvement goal was to reduce the duration of antibiotic use.

Phenotype of Takayasu-like vasculitis and cardiopathy in patients with Blau syndrome.

Objectives: We aimed to determine the prevalence of cardiovascular involvement in our Blau syndrome (BS) cohort and provide detailed analysis of their cardiovascular manifestations and outcome. We also tried to find out the risk factors for developing cardiovascular involvement.

Methods: Clinical manifestations, laboratory findings, and treatments were reviewed.

Tocilizumab reduces the unmanageable inflammatory reaction of a patient with Aicardi-Goutières syndrome type 7 during treatment with ruxolitinib.

Background: Aicardi-Goutières syndrome (AGS) is a rare hereditary early-onset encephalopathy characterized by upregulation of the type I interferon pathway, poorly responsive to conventional immunosuppression.

Case Presentation: We describe a 7-year-old Chinese boy who developed symptoms at the age of 6 months. He presented with a chilblain-like rash, leukopenia, neutropenia, elevated liver enzymesgrowth retardation, microcephaly, elevated acute phase reactants, intracranial calcification and leukodystrophy.

A quality improvement project to increase breast milk feeding of hospitalized late preterm infants in China.

Background: The breastfeeding rates of late preterm infants are lower than both term and extremely preterm infants. To explore the interventions of increasing full breast milk feeding rate of hospitalized late preterm infants on the 7th day after birth (D7) and evaluate the effect of these quality improvement (QI) interventions.

Methods: The full breast milk feeding (amount of enteral breast milk reached 120ml/kg/d on D7) rate of hospitalized late preterm infants during May 2017 and November 2017 was set as the baseline before intervention, and the specific aim of promoting breast milk feeding was put forward.

The effect of antinuclear antibody titre and its variation on outcomes in children with primary immune thrombocytopenia.

Antinuclear antibody (ANA) can be positive in children with primary immune thrombocytopenia (ITP), but the effect of ANA titre and its variation on outcomes of children with primary ITP remains unclear. Here, we conducted a single-centre retrospective cohort study of children with primary ITP at the Peking Union Medical College Hospital in China. A total of 324 children with primary ITP included in this study were followed for a median time of 25 months.

Status and Influencing Factors of Parenteral Nutrition Practice for Late Preterm Infants in China.

Objectives: To explore the status of parental nutrition practice of hospitalized late preterm infants and the factors influencing the clinical prescription.

Methods: A multi-center, prospective cohort study was conducted during October 2015 to October 2017. Infants born after 34 weeks and before 37 weeks were enrolled from twenty-five hospitals in the Beijing area of China.

Successful management with Janus kinase inhibitor tofacitinib in refractory juvenile dermatomyositis: a pilot study and literature review.

Objectives: JDM is a rare autoimmune inflammatory muscle disease with a pronounced IFN signature. Treatment for children with JDM has improved over the years with the use of steroids and immunosuppressive agents. However, there remains a subset of children who have refractory disease.

Selective immunoglobulin A deficiency (SIgAD) primarily leads to recurrent infections and autoimmune diseases: A retrospective study of Chinese patients in the past 40 years.

Selective immunoglobulin A deficiency (SIgAD) is considered to be the most common human primary immune-deficiency disease in the world. However, the incidence in China is obviously lower than Caucasian races. The definition of SIgAD has changed over time with the progress of people's understanding.

Juvenile Onset Splenomegaly and Oculopathy Due to Germline Mutation in ALPK1.

ROSAH syndrome was recently identified as an autosomal dominant systemic disorder due to mutations in ALPK1. It was characterized by retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and migraine headache. We collected and summarized the clinical data of two patients with juvenile onset splenomegaly and oculopathy.

Familial Mediterranean Fever in Chinese Children: A Case Series.

Familial Mediterranean fever (FMF) is an inherited auto-inflammatory disorder and is extremely rare in Chinese. This study aimed to investigate the demographic, clinical, and genetic features of FMF in a series of Chinese pediatric patients. This was a retrospective case series of children with recurrent febrile or inflammatory episodes and referred to the Peking Union Medical College Hospital between 06/2013 and 06/2018.

Individualized Human Milk Fortification to Improve the Growth of Hospitalized Preterm Infants.

Background: Human milk (HM) is the first choice for preterm infants, but exclusive HM feeding is inadequate for the growth of very preterm infants. The hypothesis of this trial is that infants fed according to an individualized fortification regimen will have higher protein intake and improved weight gain velocity (WGV).

Methods: A prospective, randomized, controlled study was conducted.

[Clinical features and TTC21B genotype of a child with nephronophthisis type 12].

Nephronophthisis (NPHP) is a group of autosomal recessive tubulointerstitial cystic kidney disorders. This article reports a case of NPHP type 12 caused by TTC21B mutations. The girl had an insidious onset, with moderate proteinuria, renal dysfunction, stage 2 hypertension, situs inversus, and short phalanges when she visited the hospital for the first time at the age of 3 years and 6 months.

Clinical features and prognosis of patients with thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a review of 25 cases.

Objective: To report the clinical features of patients with systemic lupus erythematosus (SLE) associated with thrombotic thrombocytopenic purpura (TTP). Their diagnosis, treatment, and prognosis were also discussed.

Methods: A total of 25 TTP-SLE pediatric patients were included in this study.

The Impact of Late Onset Arterial Hypotension on Respiratory Outcome in Extremely Premature Infants.

Background: In extremely premature infants, arterial hypotension in the first days after birth has been associated with an increased risk for bronchopulmonary dysplasia (BPD). Some infants present with hypotension at a later postnatal age, but the relationship between late onset hypotension (LOH) and BPD has not been evaluated.

Objective: To evaluate the association between LOH and BPD and to identify pre- and postnatal factors associated with LOH.

[Macronutrients and energy in milk from mothers of premature infants].

Objective: To study the dynamic changes in macronutrients and energy in human milk from mothers of premature infants.

Methods: A total of 339 human milk samples were collected from 170 women who delivered preterm or full-term infants in the Department of Obstetrics and Gynecology, Peking Union Medical College Hospital between November 2012 and January 2014. Macronutrients (proteins, fats and carbohydrates and energy were measured using a MIRIS human milk analyzer and compared between groups.

[Clinical features of preterm infants born to mothers with systemic lupus erythematosus: a retrospective analysis].

Objective: To retrospectively characterize clinical features of preterm infants born to mothers with systemic lupus erythematosus (SLE).

Methods: Clinical data of preterm infants born to mothers with SLE in Peking Union Medical College Hospital over a period of more than 10 years (2000-2012) and preterm babies born to mothers without SLE in the same hospital and during the same time period were collected. Preterm-associated complications in the two groups of babies were comparatively analyzed.

[Factors associated with quality of life in survivors of gestational trophoblastic neoplasm after chemotherapy].

Objective: To measure the quality of life (QoL) of gestational trophoblastic neoplasia (GTN) survivors after chemotherapy by using a self-invented scale, and to explore the factors associated with QoL.

Methods: The design of questionnaire was based on a series of internationally valid QoL scales, which was tested by epidemiology and showed good reliability and validity. A total of 100 survivors of GTN patients from Peking Union Medical College Hospital participated in this survey from December 2008 to May 2009.