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. Clinical features were compared between children with cardiovascular involvement and those without angiocardiopathy. RESULTS: A total of 38 BS children were eligible for final analysis. Among them, 13 (34.2%) developed Takayasu-like vasculitis and/or cardiopathy. Compared with those without angiocardiopathy, recurrent fever was more frequent in BS patients with cardiovascular involvement (p < 0.001). What is more, tumor necrosis factor alpha antagonists (anti-TNF) were more urgently needed in children with cardiovascular involvement (p = 0.015). BS patients with cardiovascular involvement include 4 with Takayasu-like vasculitis and 9 with cardiopathy. The onset of cardiovascular manifestations ranged from 0.75 to 18.5 years of age, with most cases occurring before school period. Symptoms were elusive and lacked specificity, such as dizziness, short of breath, and edema. Some patients were even identified because of the unexpected hypertension during follow-up. Cardiopathy and vasculitis occurred in patients with different genotypes. Imaging changes were discovered before the presentation of the typical triad in 3/4 patients with Takayasu-like vasculitis. Three children developed left ventricular dysfunction with decreased left ventricular ejection fraction. Combination of glucocorticoids and methotrexate with anti-TNF agents is a common treatment option for these BS patients. In the cohort, BS-related cardiovascular involvement was controlled well, with cardiac structural and functional abnormalities completely recovered and slower progression of vasculitis lesions. CONCLUSION: Cardiovascular manifestations is not rare in BS patients. Because of its insidious onset, a systematic and comprehensive assessment of cardiovascular involvement should be performed in newly diagnosed patients with BS. Aggressive initiation of anti-TNF agents may be beneficial to improve the prognosis. Key Points • About 34.2% patients with Blau syndrome developed Takayasu-like vasculitis and/or cardiopathy. • Compared with those without angiocardiopathy, recurrent fever and application of anti-TNF agents were more frequent in BS patients with cardiovascular involvement (p < 0.001, p = 0.015) • Regular assessment of cardiovascular involvement is extremely necessary because of its insidious onset.

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. At the age of 3 years old, whole-exome sequencing confirmed a de novo heterozygous gain-of-function mutation, c.1016 C > A (p.Ala339Asp), in the IFIH1 gene, and he was diagnosed with AGS7. He was treated with ruxolitinib accompanied by steroids and thalidomide for about four years. The rash, hematological manifestations, and the liver function were all improved, but the erythrocyte sedimentation rate remained consistently elevated until the addition of tocilizumab, a monoclonal antibody against interleukin 6. CONCLUSIONS: Ruxolitinib was not successful in suppressing the inflammatory process, and tocilizumab produced highly encouraging results in reducing the inflammatory reaction of AGS. The study makes a significant contribution to the literature because we may found a potential alternative therapeutic option for AGS.

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 Pareto Chart was used to analyze the factors that affect breast milk feeding process, as well as the discussion of multidisciplinary experts. Key drivers were constructed, including informational materials and education about breast milk feeding, consultations and support on optimal breast milk initiation, initiating breast milk expression within one hour after birth, accurate measurement and recording of expressed breast milk, stimulating continuous and effective lactation, proper breast pump selection in and out of hospital and sending and preserving of expressed milk to NICU. Control chart was used to monitor the monthly change of full breast milk feeding rate until the aim was achieved and sustained. RESULTS: The baseline of full breast milk feeding rate of late preterm infants was 10%, and the aim of QI was to increase the rate to 60% within a two-year period. Control chart dynamically showed the full breast milk feeding rate increased to 80% with the implementation of the interventions, achieved and made the aim of QI sustained. CONCLUSION: QI interventions including breast milk feeding education, early postpartum breast milk pumping, kangaroo care to stimulate breast milk secretion, and convenient way of transporting breast milk to NICU, could significantly improve the full breast milk feeding rate of hospitalized late preterm infants.

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. In this cohort, 39.2% had an ANA titre of 1:160 or higher. Results from a generalized estimating equation model revealed that patients with higher ANA titres had lower platelet counts at onset but a higher recovery rate of subsequent platelet counts. Results from Cox regression models adjusted for potential confounders revealed that patients with ANA titres of 1:160 or more were more likely to develop to autoimmune disease (AID) than those without, and the risk of AID development increased with the rise of ANA titres (p value for trend less than 0.001). These data highlight the predictive value of ANA titre for platelet counts and the risk of AID development in children with primary ITP.

Janus Kinase Inhibitors in the Treatment of Type I Interferonopathies: A Case Series From a Single Center in China.

Objective: This study aimed to assess the efficacy and safety of 2 Janus kinase (JAK) inhibitors (jakinibs) tofacitinib and ruxolitinib in the treatment of type I interferonopathies patients including STING-associated vasculopathy with onset in infancy (SAVI), Aicardi-Goutières syndrome (AGS), and spondyloenchondrodysplasia with immune dysregulation (SPENCD). Methods: A total of 6 patients were considered in this study: 2 patients with SAVI, 1 patient with AGS1, 1 patient with AGS7, and 2 patients with SPENCD. Clinical manifestations, laboratory investigations, radiology examinations, treatment, and outcomes were collected between November 2017 and November 2021 in Peking Union Medical College Hospital. The disease score for patients with SAVI and AGS scale for patients with AGS were documented. The expression of 6 interferon-stimulated genes (ISGs) was assessed by real-time PCR. Results: Three patients (1 patient with SAVI, 2 patients with AGS) were treated with ruxolitinib and 3 patients (1 patient with SAVI, 2 patients with SPENCD) were treated with tofacitinib. The mean duration of the treatment was 2.5 years (1.25-4 years). Upon treatment, cutaneous lesions and febrile attacks subsided in all affected patients. Two patients discontinued the corticoid treatment. Two patients with SAVI showed an improvement in the disease scores (p < 0.05). The erythrocyte sedimentation rate normalized in 2 patients with AGS. The interferon score (IS) was remarkably decreased in 2 patients with SPENCD (p < 0.01). Catch-ups with growth and weight gain were observed in 3 and 2 patients, respectively. Lung lesions improved in 1 patient with SAVI and remained stable in 3 patients. Lymphopenia was found in 3 patients during the treatment without severe infections. Conclusion: The JAK inhibitors baricitinib and tofacitinib are promising therapeutic agents for patients with SAVI, AGS, and SPENCD, especially for the improvement of cutaneous lesions and febrile attacks. However, further cohort studies are needed to assess the efficacy and safety.

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. Data of enteral and parenteral nutrition were collected. Results: A total of 1,463 late preterm infants were enrolled in this study, 53.9% of infants were supported by parenteral nutrition. Over 60% of 34 weeks' infants were on parenteral nutrition during the 2nd to the 4th day. Logistic regression analysis showed that gestational age(GA) (OR = 0.69, 95%CI 0.58-0.81), birth weight (OR = 0.41, 95%CI 0.26-0.65), hypoglycemia (OR = 2.77, 95%CI 1.90-4.04), small for gestational age (SGA) (OR = 2.18, 95%CI 1.34-3.55), feeding intolerance (OR = 6.41, 95%CI 1.90-21.59), neonatal respiratory distress syndrome (NRDS) (OR = 2.16, 95%CI 1.12-4.18), neonatal infection(OR = 1.56 95%CI 1.16-2.10), and slow enteral nutrition advancement rate (OR = 0.92, 95%CI 0.90-0.95) were factors influencing the administration of parenteral nutrition. Conclusion: Over half of hospitalized late preterm infants were prescribed with parenteral nutrition. Infants with lower GA, lower birth weight, diagnosed with hypoglycemia, SGA, feeding intolerance, NRDS, neonatal infection, or a slower rate of enteral nutrition advancement had a higher likelihood of receiving parenteral nutrition.

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. Janus kinase and type I IFN signalling production are suspected to contribute to the pathogenesis of JDM. Our pilot study investigated the use of tofacitinib, a Janus kinase inhibitor, in refractory JDM cases to provide new therapeutic options for better treatment. METHODS: Refractory JDM was defined as patients who failed two or more steroid sparing agents or high-dose steroids. Tofacitinib was given to three refractory JDM patients with a dose of 5 mg twice per day for at least 6 months. Core set measures defined by Pediatric Rheumatology International Trials Organization were evaluated at month 0, 3 and 6 along with other systemic evaluations. A literature review was conducted to identify all the cases using Janus kinase inhibitors in JDM. RESULTS: All three subjects tolerated and responded well to tofacitinib with significant improvement in Child Myositis Assessment Scale, manual muscle testing-8, physician global disease activity and inflammatory indices without occurrence of severe adverse events. CONCLUSION: This pilot study showed improvement of muscle strength, resolution of cutaneous lesions, increased daily quality of life and successful tapering of steroids when tofacitinib used in selected cases. Tofacitinib can be considered with caution when treating refractory JDM cases. Further randomized controlled trials are warranted to assess its efficacy in JDM.

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. The scientific community did not reach a consensus on the definition until 1999. As a result, many previously reported cases need to be excluded under the current definition. SIgAD can lead to several spectra of diseases including infections and autoimmune diseases. We retrospectively summarized the SIgAD patients in Peking Union Medical College Hospital (PUMCH), and summarized the Chinese SIgAD reported in China and abroad in past 40 years. Fourty three SIgAD patients were confirmed in the study, in which 9 were healthy without clinical symptoms. Of the 34 patients with clinical symptoms, recurrent infections were found in 29 (85.3%) patients; 13 (38.2%) patients were with autoimmune diseases; 6 (17.6%)cases had allergic symptoms; 3 patients (8.8%) were with tumors, only one case (2.9%) had a family history. Compared with other countries, sIgAD patients in China showed similar symptoms, but the rate of recurrent infections and autoimmune diseases were higher than some other countries; most of the allergic symptoms are drug allergy, different with the allergic sequelae reported in other countries, such as asthma, rhinitis, food allergy and atopic dermatitis; and it is rare to have family history in Chinese patients. We also figured out that more female SIgAD patients tend to have more autoimmune diseases than men (P = 0.039).

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. Whole exome sequencing (WES) was adapted for genetic analysis. Mutations in ALPK1 were confirmed by Sanger sequencing. Besides juvenile oculopathy and splenomegaly, both patients had intermittent fever and anhidrosis. Patient 2 also experienced recurrent upper respiratory infections in her infancy and developed dental and nail problems in childhood. Elevated TNF-α was their prominent laboratory features. Both patients were found to have a previously reported mutation, c.710C>T, p. T237M (NM_001102406) in ALPK1. Anti-TNF treatment of adalimumab was applied to patient 1, after which her optic disc edema in the left eye continued and the visual acuity deteriorated further. Patient 1 underwent elective splenectomy due to concern for spontaneous rupture of the spleen. Up to date, 18 patients of ROSAH syndrome have been reported. The clinical manifestations were relatively homogeneous, prominently presenting with juvenile onset oculopathy and splenomegaly. As it mainly involves ocular fundus, severe oculopathy deeply affects the quality of life and prognosis of ROSAH patients. Now little has been known about its treatment. As a newly recognized inherited systemic disorder, ROSAH syndrome needs to be paid more attention to, especially for those with juvenile onset splenomegaly and oculopathy.

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. Infants <34 weeks of gestational age were enrolled when enteral feeding volume reached 60 mL/kg/d and were randomly allocated to the individualized fortification (IF) group or the standard fortification group. The IF group was fed using a regimen that featured modifying HM fortifier and supplemental protein powder based on the protein concentration in HM, current body weight of infants, and blood urea nitrogen (fortification level was set as L-1, L0, L1, L2, L3; the amount of HM fortifier and protein powder were determined accordingly). RESULTS: Between September 2012 and August 2016, 51 preterm infants completed the study. In the IF group, 62.5% (15/24) of preterm infants were fed with HM fortified to level 1, 29.2% (7/24) to level 2, and 12.5% (3/24) to level 3. The WGV of the third week in the IF group was greater than the standard group (20.8 ± 7.9 vs 14.9 ± 4.5 g/kg/d, P = 0.022). CONCLUSION: About two-thirds of preterm infants needed to adjust the HM fortification to a higher level. The WGV of infants in the IF group was better than that of the standard group in the third week of this study.

Familial Mediterranean Fever in Chinese Children: A Case Series.

Background: 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. Methods: 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. All suspected patients were genetically diagnosed and met the Tel-Hashomer criteria for FMF. Demographic, clinical, genetic, and treatment characteristics were collected. Descriptive statistics were used. Results: Eleven patients were included (seven boys and four girls). The median age at the time of disease onset was 7.1 (range, 3-12) years, while the median age at diagnosis was 10.9 (range, 6-15) years. The median delay in diagnosis was 2.1 years (range, 6 months to 6.7 years). Fever (100%, 11/11) was the most common symptom, followed by joint pain (63.6%, 7/11), rash (54.5%, 6/11), abdominal pain (36.4%, 4/11), and oral ulcers (18.2%, 2/11), without evidence of amyloidosis. C-reactive protein (81.8%, 9/11) and erythrocyte sedimentation (90.9%, 10/11) were increased during attacks. All patients harbored one to five different MEFV mutations, with E148Q and L110P being the most frequent. A novel non-synonymous mutation F636Y in exon 10 was discovered. Favorable responses to colchicine was observed in all six treated patients. Conclusion: The most common variants in our study were E148Q and L110P. F636Y may found for the first time. Colchicine led to favorable responses in all treated patients.

[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. The renal lesions progressed to end-stage renal disease (ESRD) before she was 4 years old. Urine protein electrophoresis showed glomerular proteinuria. There were significant increases in urinary ß2-microglobulin and α1-microglobulin. Gene detection revealed two compound heterozygous mutations, c.1552T>C (p.C518R) and c.752T>G (p.M251R), in the TTC21B gene, which came from her father and mother respectively. The c.752T>G mutation was a novel mutation. It is concluded that besides typical tubular changes of NPHP, marked glomerular damage is also observed in patients with TTC21B gene mutations.

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. Their clinical symptoms, laboratory findings, disease activity, and renal biopsy were retrospectively reviewed. RESULTS: The median age of the patient cohort was 14 years old. Nine patients were first diagnosed with SLE, followed by the diagnosis of TTP-SLE, whereas 15 patients were diagnosed with TTP and SLE concurrently. All the 25 TTP-SLE patients had decreased platelet count and microangiopathic hemolytic anemia. Fever, rash, edema and neurological symptoms were the main clinical symptoms. Fragmentation of erythrocytes on blood smear and increased LDH were found in all patients. Nineteen patients (76%) had impaired renal function. Renal biopsy showed that most of the patients had lupus nephritis class IV (20%) and TMA (20%). 13 patients (52%) were treated with glucocorticoids in combination with immunosuppressive agent, and 10 patients (40%) were treated with plasma exchange combined with glucocorticoids plus immunosuppressive agent. One patient died due to lung infection; others had disease remission. Fifteen patients had follow-up regularly, and their conditions were stable. CONCLUSION: Patients with TTP-SLE often had moderate to severe lupus disease activity. Testing of LDH level and blood smear should be performed when kidney and neurological symptoms arise in children with SLE. The use of combination therapy, glucocorticoids plus immunosuppressive agent, provided satisfactory clinical outcome. Patients with refractory TTP-SLE will also need plasma exchange therapy.

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. METHODS: Prospectively collected data from a cohort of 23-28 weeks gestational age (GA) infants born during years 2005-2015 and alive at day 28 were analyzed. LOH was defined as the receipt of vasopressor treatment during days 8-28. BPD was defined as need for oxygen at 36 weeks postmenstrual age. Late mortality was defined as death after day 28. RESULTS: Of 1,058 infants in the cohort, 90 (9%) had LOH during days 8-28. Infants with LOH had a higher incidence of BPD than normotensive infants (55 vs. 21%, p < 0.001). Multivariate logistic regression analysis (LRA) showed that LOH was associated with an increased risk for BPD (OR 1.87, 95% CI 1.10-3.17). LOH was also associated with an increased risk for late mortality. LRA showed the risk for LOH increased with lower GA, sepsis and patent ductus arteriosus during days 8-28. CONCLUSIONS: In this cohort of extremely premature infants, LOH was associated with an increased the risk for BPD. This association could be secondary to underlying factors that predispose to LOH and BPD or to the deleterious effects of LOH or its treatments on the lung. Further investigation is needed to assess causality.

[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. RESULTS: In milk samples from premature infants' mothers, the protein levels were the highest in colostrum (2.22±0.49 g/dL), less in transitional milk (1.83±0.39 g/dL), and the least in mature milk (1.40±0.28 g/dL) (P<0.01), and the levels of fats (2.4±1.3 g/dL vs 3.1±1.1 g/dL; P<0.01), carbohydrates (6.4±0.9 g/dL vs 6.6±0.4 g/dL; P<0.05) and energy (55±9 kcal/dL vs 62±8 kcal/dL; P<0.01) were significantly lower in colostrum than in transitional milk. The protein levels in colostrum from premature infants' mothers were significantly higher than those in colostrum from term infants' mothers (2.22±0.49 g/dL vs 2.07±0.34 g/dL; P<0.05). The colostrum from mothers of premature infants with a gestational age of ≤30 weeks had significantly higher protein levels than those from mothers of premature infants with gestational ages of 30(+1)-33(+6) weeks and ≥34 weeks (2.48±0.68 g/dL vs 2.11±0.25 g/dL and 2.22±0.39 g/dL respectively, P<0.05); the energy levels in colostrum from mothers of premature infants with a gestational age of ≤30 weeks group (51±6 kcal/dL) were significantly lower than those in colostrum from mothers of premature infants with a gestational age of 30(+1)-33(+6) weeks (58±8 kcal/d; P<0.05). The carbohydrate levels in transitional milk from mothers of premature infants with a gestational age of ≤30 weeks were significantly higher than those in transitional milk from mothers of premature infants with gestational ages of 30(+1)-33(+6) weeks and ≥34 weeks (P<0.05). The protein levels in mature milk from mothers of premature infants with a gestational age of 30(+1)-33(+6) weeks were significantly higher than those in mature milk from mothers of premature infants with gestational ages of ≤30 weeks and ≥34 weeks (P<0.05). CONCLUSIONS: The levels of macronutrients and energy in milk from mothers of premature infants vary significantly between colostrum, transitional milk, and mature milk. Protein levels are significantly higher in colostrum from premature infants' mothers than in colostrum from term infants' mothers, but the significant difference is not seen for mature milk. Macronutrient and energy levels show significant differences between milk samples from mothers of premature infants with different gestational ages, so as to meet different needs of premature infants.

[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. RESULTS: During the time period studied, 128 women with SLE delivered a total of 134 babies, 86 at full-term and 42 at preterm. Of the 42 preterm infants, 4 were diagnosed with neonatal lupus syndrome. Neonatal infection was the most common complication in preterm infants born to SLE mothers, which occurred in 20 cases (47.62%), followed by small for gestational age (28.57%), neonatal respiratory distress syndrome (26.19%), congenital heart disease (14.29%), and neonatal pulmonary hemorrhage (4.76%). In the same time period, 2 308 preterm babies were born to mothers without SLE. In these preemies, 16.81% experienced neonatal infection, 13.21% were small for gestational age, and 5.16% had congenital heart disease. All these parameters were significantly lower than in preterm babies born to mothers with SLE (P<0.05). CONCLUSIONS: SLE preterm offspring seem to be more prone to neonatal infection, small for gestational age and at a higher risk of congenital heart disease as compared to preterm babies from women without SLE.

[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. RESULTS: Patients with disease-free more than three months after chemotherapy enjoys a good QoL, while only 16% (16/100) of survivors feel general overall QoL, but no one feels bad QoL. As refer to sexual function, more than half of these patients (70%, 70/100) satisfied with their sexual life, while there were still 47% (47/100) and 45% (45/100) of the patients complaining of decreased sexual desire and dryness of vagina. 66% (66/100) of the GTN survivors expressed depression, and 50% (50/100) of patients complained anxiety, which were potential factors influencing QoL of GTN survivors. Relevant analysis explored the possible predictors of QoL for GTN patients, including physical function (r = 0.609, P < 0.01), sexual function (r = 0.473, P < 0.01), and social psychology (r = 0.294, P < 0.01). CONCLUSIONS: GTN survivors have an overall good QoL after chemotherapy, the possible predictors of QoL for GTN patients include physical function, sexual function and social psychology. The sexual dysfunctions mostly present with short of sexual desire and dryness of vagina. Fear of recurrence may be a potential factor influencing QoL a long term after remission.