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Objective To explore the effect of circadian rhythm genes on flavonoids biosynthesis in safflower and its molecular mechanism. Methods Based on the transcriptome and metabolomic database of safflower corolla, we screened the circadian rhythm genes that correlate with biosynthesis of flavonoids in safflower. qPCR was used to quantify the expressions of circadian rhythm genes in different flowering stages at different time points in a single day. LC-MS was performed to determine the accumulation of flavonoids. The correlation between them was analyzed as well. Yeast Two-Hybrid experiment was used to verify the interactive proteins of these genes. Results Seven circadian rhythm genes PRR1, PRR2, ELF3, FT, PHYB, GI and ZTL were obtained. PRR1 gene was positively correlated with flavonoids accumulation (r≥0.7). The full length of PRR1 is 3 201 bp, encoding 421 amino acids, which is highly homologous with rice OsPRR73 gene and named as CtPRR1 (GenBank accession number: MW492035). CtPRR1 was mainly expressed in flowers, and the expression level increased in the daytime and declined in the evening gradually. Correspondingly, the content of flavonoids showed an opposite variation. Both of them displayed a circadian rhythm with a negative correlation (r≥−0.7). In addition, 2 heat shock proteins along with 3 AP2 transcription factors interacting with CtPRR1 protein were obtained via Yeast Two-Hybrid experiment. Conclusion CtPRR1 negatively regulated the safflower flavonoids accumulation in a circadian rhythm way, which may be affected by these interacting proteins.
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Objective@#To evaluate the effect of prophylactic nimodipine in vasospasm prevention and outcome improvement in children with subarachnoid hemorrhage (SAH).@*Methods@#A prospective, randomized controlled clinical trial which enrolled children with SAH who were admitted to pediatric intensive care unit (PICU) of Beijing Children′s Hospital from January 2015 to October 2018 was conducted. A total of 43 patients were randomly divided into nimodipine group (24 patients) and control group (19 patients) according to random number table. Transcranial Doppler (TCD) was used to dynamically monitor blood flow velocity and spectrum monography of bilateral middle cerebral artery (MCA) for vasospasm evaluation. Pediatric cerebral performance category (PCPC) scale was used to evaluate patients′ brain function on 28th day after discharge. Data were analyzed by t test, Mann-Whitney U test, χ2 test.@*Results@#Except heart rate ((157±26) vs. (137±34) beats/min, t=2.079, P=0.045), no significant differences existed between the two groups in basic demographic characteristics, primary diseases, and clinical manifestations (all P>0.05). The peak velocities of bilateral MCA on the 5th day after admission were significantly lower in nimodipine group (left MCA (136±34) vs. (158±23) cm/s, t=-2.890, P=0.006; right MCA (129±34) vs. (176±27) cm/s, t=-3.717, P=0.001). Likewise, a lower peak velocity of left MCA was observed on the 7th day after admission in nimodipine group ((127±45) vs. (152±13) cm/s, t=-2.903, P=0.007), but no significant difference existed in that of right MCA ((131±48) vs. (150±22) cm/s, t=-1.760, P=0.090). Eleven patients suffered from vasospasm, 25% (6/24) in nimodipine group and 26% (5/19) in control group (χ2=0.010, P=1.000), within whom 8 patients had complete remission after continuing nimodipine treatment, one died in hospital and the other two′s vasospasm still existed at the time of discharge. No significant differences were found between the two groups in mean length of hospitalization, proportion of mechanical ventilation, Glasgow coma scale at discharge, survival rate at discharge or survival rate on 28th day after discharge (all P>0.05). However, nimodipine group had a higher proportion of favorable PCPC brain function (92% (22/24) vs. 63% (12/19), χ2=5.208, P=0.030). No side effects such as hypotension, rash or injection site erythema were observed.@*Conclusion@#Prophylactic nimodipine cannot reduce vasospasm incidence in children with SAH but may improve short-term brain function, without any significant safety issues.
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Objective To analyze the clinical characteristics of fatal cases with confirmed influenza A ( H1N1) in children in order to improve the diagnosis and treatment. Methods The fatal cases of influenza A (H1N1) admitted to Pediatric Intensive Care Unit of Beijing Children′s Hospital from December 2017 to March 2018 were collected,whose clinical features,diagnosis,treatment,and the causes of death were ana-lyzed retrospectively. Results A total of eight children were enrolled. The age ranged from 3 months to 9 years,and six cases were less than 5 years old. Four cases had underlying diseases. All patients had fever, cough,dyspnea,cyanosis,flaring nares and three depressions sign. Four cases had coma. On admission,the average score of pediatric index of mortality was 58. 8％. Blood routine test showed that lymphocyte predomi-nance in six cases, three cases had leukopenia. C-reactive protein elevated in five patients. Procalcitonin increased in all cases. P/F ratio (PaO2/FiO2) were less than 100 mmHg(1 mmHg =0. 133 kPa) in four cases who were diagnosed as severe acute respiratory distress syndrome. The pulmonary imaging showed mul-tiple parenchymal or mixed lesions. Three cases had air leakage syndrome,and one had pleural effusion. Bac-terial culture was performed on the day of admission. Four cases were complicated with bacterial infection. Three cases had Gram-positive cocci infection,and multidrug-resistant bacteria were predominant. The aver-age time from onset to definitive diagnosis was 8 days. Neuraminidase inhibitors were used in all patients,and the average time from onset to the first dose was 8 days. Mechanical ventilation were performed in all patients in this group. Extracorporeal membrane oxygenation was applied in one case. Four cases in this group died of severe acute respiratory distress syndrome. Three cases died of influenza associated encephalopathy,and one died of septic shock with multiple organ failure. Conclusion Fatal influenza A (H1N1) mostly appeares in children under 5 years old or with underlying diseases. Acute respiratory distress syndrome and influenza associ-ated encephalopathy are the main causes of death. Delayed diagnosis and delayed use of anti-influenza drugs may be an important factors leading to death,and bacteria infection may be another important cause of death.
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Methotrexate ( MTX) is administered at a high dose for the treatment of diseases such as leukemia and malignant lymphoma. However, it will cause serious adverse reactions such as acute kidney injury,myelosuppression, and mucositis. On the basis of hydration alkalization and high-dose leucovorin rescue in children with high risk of MTX poisoning,blood purification should be performed as soon as possi-ble to clear MTX. The number and frequency of hemodialysis should be determined according to the general condition of the child,the degree of renal injury and the concentration of MTX,and high-throughput hemodi-alysis can be selected if available. Continuous renal replacement therapy is suitable for children with hemodynamic instability. When the MTX concentration is extremely high, it is recommended to combine hemoperfusion and hemodialysis to achieve better clearance.
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Objective To analyze the cause of fatal cases in children with confirmed influenza virus infection,and in order to improve the level of diagnosis and treatment.Methods Deaths in critical illness of influenza were collected from November 2017 to April 2018 in Pediatric Intensive Care Unit of Beijing Children's Hospital,Capital Medical University.The clinical characteristics and causes of death were retrospectively analyzed according to the different virus types.Results A total of 19 cases were included.Fifteen cases (78.95％) were less than 5 years old and 9 cases (47.37％) were less than 2 years old.On admission,the median score of pediatric index of mortality 2 was 72.7％.There were 11 cases of influenza H1N1 and 8 cases of influenza B.Six cases had underlying diseases.All patients had fever,cough and dyspnea.Thirteen patients had coma.Seventeen cases had pneumonia,11 cases had severe acute respiratory distress syndrome(ARDS),3 cases had air leakage syndrome and 8 cases had influenza-related encephalopathy(IAE).Ten cases (52.63％) died of severe ARDS,7 cases (36.84％) died of IAE,1 case(5.26％) died of multiple organ dysfunction,and 1 case(5.26％)died of severe myocarditis and cardiogenic shock.There was statistical difference in the time from onset to death between the ARDS group and IAE group[15(4,22) d vs.3(2,8) d] (Z =-2.063,P =0.039).Among the children who died of severe ARDS,most patients in influenza H1 N1 group ＜ 2 years old,while those influenza B group ≥ 2 years old.All children who died of IAE were all ≥ 1 years old.Six cases (31.58％) had bacterial infection,mainly gram-positive cocci.All patients were treated with neuraminidase inhibitors.The average time from onset to the first time of medication was 5 days.Conclusions Severe ARDS and IAE are the main causes of death in children with influenza virus infection.Compared with ARDS,the condition of children with IAE worsened more rapidly.
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Objective To explore the diagnosis and treatment of atypical severe combined immunodeficiency disease (SCID). Methods The clinical data of atypical SCID in 7 children with IL2RG,JAK3,and RAG1 mutations were reviewed and analyzed from September 2012 to June 2017. Results In 7 cases (6 males and 1 female), there were 5 infants, 1 toddler and 1 school-age child. Cases 2, 4, and 6 were classic SCID clinical phenotypes. Cases 1, 3, 5, 7 were atypical SCID clinical phenotypes. Case 6 were diagnosed with Omenn syndrome. Cases 2, 5 were classic SCID immune phenotypes, cases 1, 3, 4, 6, 7 were atypical SCID immune phenotypes, and case 1 had maternal chimera. The next generation sequencing indicated that case 1 had a compound heterozygous JAK3 mutation with c.3097-1G>A/c.946-950GCGGA>ACinsGGT.Cases 2,3,and 4 had IL2RG mutations,with c.865C>T/p.R289X,c.664C>T/R222C,52delG,respectively.Case 5 had JAK3 mutations with c.2150A>G/p.E717G and c.1915-2A>G.Sanger sequencing indicated that case 6 had a RAG1 mutation of complex heterozygosity with c.994C>T/p.R332X and c.1439G>A/p.S480N. Case 7 had homozygous RAG1 mutation with c.2095C>T/p.R699W.Conclusion Under certain conditions,gene mutation can lead to atypical clinical and/or immune phenotypic SCID.
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Compression of the airway is a relatively common and often unrecognized complication of cardiovascular malformation. The clinical presentation of vascular malformation with tracheal stenosis usually involves respiratory symptoms with uncharacteristic clinical symptoms,missed diagnosis and misdiagnosis are still very common. The continuous improvement of detection technology,timely diagnosis and individualized treatment plan will improve the prognosis of this disease.
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The injury of the intestinal mucosal barrier is a common pathophysiological process in critically ill children,which can cause translocation of bacteria and endotoxin,enterogenic infection,even multiple organ failure.Early diagnosis of intestinal mucosal barrier damage,positive correction of intestinal dysfunction and appropriate nutritional support can improve the curative effect and prognosis.The concept,diagnosis and nutritional support progress of intestinal barrier dysfunction in critically ill children were reviewed in the paper.
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Acute kidney injury in children is frequently a component of the multiple organ dysfunction syndrome. It often occurs within the severe catabolic phase determined by critical illness and is intensified by metabolic derangements. Nutritional support is critical for these children to improve outcomes. Meeting the special nutritional needs of these critical children with acute kidney injury often requires nutritional supplemen-tation by either the enteral or the parenteral route. The nutritional requirements of these children should be frequently reassessed for individualized nutritional support and carefully integration with renal replacement therapy.
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Acute respiratory distress syndrome(ARDS) is a lung inflammation caused by variety of promoting inflammation factors induced by many causes.High permeability pulmonary edema is the basic pathophysiology characteristics of ARDS.Reasonable fluid management of ARDS helps to improve the pulmonary edema and reduce the mortality.Timely and effective nutrition therapy is one of the most important interventions.Nutrition support goals of ARDS patients have already been changed from purely providing the energy and substrate of the cells' and body's metabolism to regulating metabolism disorders,regulating immune,and acting as a disease treatment drug for the target.In the treatment of patients with ARDS,how to develop the nutrition strategies which can not only satisfy patients' nutrition requirements,and at the same time,but also restrain inflammatory reaction,improve the oxygenation of ARDS patients even reduces mortality is worth concerned.
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Objective To analyze the epidemiologic characteristics and risk factors for mortality in non-(human immunodeficiency virus,HIV) infected children with pneumocystis carinii pneumonia(PCP). Methods The data of non-HIV infected children with PCP diagnosed in Beijing Children′s Hospital from January 1,2006 to December 31,2012 were collected. They were divided into survival and non-survival group according to the prognosis. The epidemiologic characteristics and risk factors for mortality were analyzed. Results Sixteen patients were enrolled in this study. Ten of them survived and 6 of them were non-survived. The basic diseases included malignant tumor in 5 patients and non-malignancy diseases in 11 of them. Com-pared with the survival group,the non-survival group had a higher average age [(12. 00 ± 2. 00) years vs. (6. 65 ± 4. 32)years,P=0. 01],higher ratio to need mechanical ventilation (6/6 vs. 4/10,P=0. 04),lower PaO2/FiO2[(73. 88 ±26. 95) mmHg vs. (167. 50 ± 97. 17) mmHg,1 mmHg=0. 133 kPa,P=0. 01] and lower pediatric critical illness score(75. 67 ± 5. 72 vs. 86. 40 ± 8. 88,P=0. 02). There were no differences on sex ratio,kinds of basic diseases,whether with co-infections,the time of immunosuppressant administration, the time from onset to diagnosis,the time from onset to beginning trimethoprim-sulfamethoxazole therapy, PaCO2 ,white blood cell counts,lymphocyte counts,CD4+ cell counts,C-reactive protein,and hemoglobin con-centrations between the survival and non-survival group. Conclusion A higher age, need for mechanical ventilation,lower PaO2/FiO2 and lower pediatric critical illness score were risk factors for mortality in non-HIV infected children with PCP.
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Objective To investigate the effects of airway pressure release ventilation (APRV) in children with severe pneumonia-related acute respiratory distress syndrome(ARDS).Methods Ten children suffering severe pneumonia-related ARDS with APRV were included in Pediatric Intensive Care Unit, Beijing Children's Hospital,Capital Medical University from March 2011 to October 2014.Ventilation variables, changes of airway pressure and Ramsay scores were collected and compared with that in conventional ventilation (CV).Clinical variables were measured at CV before APRV and at 1,4,12,24 hours after transition to APRV.Results High airway pressure(Phigh) at each time point during APRV was significantly lower than peak airway pressure (Ppeak) or plateau airway pressure (Pplat) in CV[(26.00 ±2.94) cmH2O(1 cmH2O =0.098 kPa) ,(24.40 ±3.34) cmH2O,(23.30 ±3.46) cmH2O,(23.00 ± 3.80) cmH2O vs (31.80 ± 5.59) cmH2O, P ＜ 0.01].Mean airway pressure (Pmean) at each time point during APRV was significantly higher than that in CV [(23.00 ± 2.86) cmH2 O, (21.69 ± 3.12) cmH2 O, (20.89 ± 3.31) cmH2 O, (20.46 ± 3.48) cmH2 O vs (17.50 ± 2.37) cmH2 O, P ＜ 0.05].Fraction of inspired oxygen (FiO2) were significantly decreased at 4, 12 and 24 hours after APRV than that in CV [(73.00 ± 22.39) ％, (63.50 ± 20.16) ％, (63.00 ± 21.11) ％ vs (88.00 ± 15.49) ％, P ＜ 0.05].Ramsay scores were significantly decreased at each time point during after APRV than that in CV [(3.90 ± 0.74) scores, (2.90 ± 0.88) scores, (3.00 ± 1.15) scores,(3.50 ± 0.71) scores vs (4.60 ± 0.52) scores, P ＜ 0.05].Conclusions Compared with CV, APRV had a lower Phigh and FiO2 ,a higher Pmean and more shallow sedation.APRV may be an effective ventilation mode in children's severe pneumonia-related ARDS.
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Objective To investigate the prevalence of vitamin D deficiency and to examine its relationship with the severity and prognosis in the critically ill children. Methods A total of 83 critically ill children admitted from November 1,2010 to December 9,2010 to Pediatric Intensive Care Unit in Beijing Children's Hospital,Capital Medical University were enrolled in the study. Serum 1,25 - Dihydroxyvitamin D concentration was measured by using an en-zyme - linked immunosorbent assay(ELISA). Anthropometric parameters such as height/ length and weight of the chil-dren were measured. Data collection also included primary disease,Pediatric Critical Illness Score(PCIS),the Pediatric Risk of Mortality Ⅲ(PRISM Ⅲ)score,multiple organ dysfunction syndrome( MODS)rate,mechanical ventilation rate,time of hospital of stay and the 28 - day survival rate. Results There were 32 cases with vitamin D deficiency on admission,vitamin D deficiency rate on admission was 38. 6% ,and there was no statistically significant difference among different primary disease groups(P = 0. 815). Vitamin D deficiency rate of malnutrition group was lower than that of the normal group[60. 0%(12 / 20 cases)vs 40. 0%(8 / 20 cases),χ2 = 5. 989,P = 0. 014]. PCIS scores of those with a normal vitamin D status was higher than those of the vitamin D deficiency group,showing a significant difference [(80. 47 ± 6. 18)scores vs(77. 16 ± 7. 59)scores,P = 0. 022]. PCIS score was positively correlated with the vitamin D level(r = 0. 267,P = 0. 015). There was no statistically significant difference among the PRISM score,MODS rate, mechanical ventilation rate,hospital stay length and the 28th day survival rate between the normal vitamin D group and the vitamin D deficiency group(all P ﹥ 0. 05). Conclusions A high prevalence of vitamin D deficiency is found in the critically ill children. The prevalence of vitamin D deficiency in children with malnutrition is higher. Vitamin D status may be correlated to the severity of the critically ill children,but the association with the prognosis is not obvious.
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The adenovirus pneumonia is one of the severe pneumonia in infants and young children.Adenovirus serotype 3 and 7 can cause severe clinical presentation,a wide range of clinical syndrome,difficult treatment,high mortality and serious pulmonary sequelae.The epidemiology,pathogenesis,clinical manifestation,treatment and prognosis of severe adenovirus pneumonia in children are reviewed in the paper.
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<p><b>OBJECTIVE</b>To investigate clinical features and therapeutic methods of late-onset central hypoventilation syndrome.</p><p><b>METHOD</b>A nine-year old boy was trachea-intubated and mechanically ventilated because of pneumonia, respiratory and heart failure and pulmonary hypertension. It was found that hard to extubate the patient as he was breathing normally while awake but had shallow breathing, oxygen desaturation and CO2 retention when falling asleep. Nocturnal polysomnography together with transcutaneous CO2 supported the diagnosis of central hypoventilation. The final diagnosis was late-onset congenital central hypoventilation syndrome as the patient gained weight rapidly since 3 years of age and the brain magnetic resonance imaging (MRI) and genetic screening were unremarkable.</p><p><b>RESULT</b>The patient was treated with bi-level positive air pressure ventilation via nasal mask which showed good oxygen saturation and CO2 dropped down. The follow up study done one year later showed normal brain MRI, relief of pulmonary hypertension and better CO2 level in both awaken and sleeping status.</p><p><b>CONCLUSION</b>The late-onset congenital central hypoventilation syndrome in this case had onset of symptoms at 2 years of age, he had normal breathing while he was awake but had oxygen desaturation and CO2 retention during sleep, therefore, respiratory support is required in severe cases. Mechanical ventilation via tracheotomy and non-invasive ventilation via mask are the major choice.</p>