ABSTRACT
Objective To study the clinical characteristics of neonatal macrophage activation syndrome (MAS) associated with maternal rheumatic diseases and improve the understanding of neonatal MAS.Method Clinical data of MAS in a newborn infant with adult-onset Still's disease (AOSD) mother was retrospectively studied.From the establishment day of databases (CNKI,VIP,Wanfang,Pubmed and Embase) to December 2017,literature were retrieved with key words including "newborn","macrophage activation syndrome" and "hemophagocytic lymphohistiocytosis (HLH) ".Clinical features of infant MAS/HLH with maternal rheumatic diseases were summarized.Result A 27-day-old boy with AOSD mother manifested with fever,watery stools,irritability,prominent enlargement of right parotid gland and right cervical lymphadenitis.The infant was diagnosed with MAS due to coagulopathy,multiple organ dysfunction,hypofibrinogenemia and increased levels of ferritin.Anti-SSA/Ro52kD and stool rotavims antigen were positive.The infant recovered with intravenous immunoglobulin and steroids therapy.Follow-up at 2-year-old were normal.A total of 3 other cases of neonatal MAS/HLH were retrieved.All patients had high fever,hepatosplenomegaly and multiple organ dysfunction,impaired digestive system (abdominal distention,diarrhea and ascites),disseminated intravascular coagulation (2 cases),mental disorders (1 case),complete atrioventricular block (1 case) and severe hypotension (1 case).Laboratory results showed thrombocytopenia,elevated level of hepatic enzyme and serum ferritin in all patients.Targeted panel-based next generation sequencing were all negative for pathogenic gene mutations.After treatments of steroids,intravenous immunoglobulin and chemotherapy,all patients improved and ultimately cured.Conclusion In view of the impacts of the maternal rheumatic diseases on fetus,newborns with early onset high fever and hepatosplenomegaly should be suspected of MAS.Early diagnosis and effective treatment are crucial for clinical improvement.
ABSTRACT
Objective To explore the influence of early rapidly increased amino acid dosaging on the nitrogen balance and growth in preterm infants.Methods A total of 37 prematures admitted to our NICU within 24 hours after birth and with birth weight of 1 000 g to 1 800 g from November 2011 to January 2013 were randomly divided into study group (n =18) and control group (n =19).Parenteral nutrition (PE) was provided in both groups within 24 hours after birth.The starting amino acid dose was 1.5g/kg per day,then rose by 1 g/kg per day in study group and by 0.5 g/kg per day in control group,until to maximum of 3.5 g/kg per day.The dosage of lipids,glucose,and electrolytes in parenteral nutrition was provided routinely.The trial was double-blinded.Results In the first 5 days of PE,the nitrogen balance had no significant difference between these two groups (F =0.025,P =0.652) ; however,the time of regaining birth weight was significantly shorter in study group [(7.23±2.48) d] than in control group [(9.42±2.03) d] (t=-2.090,P=0.044).There were no differences between these two groups in trems of completely feeding time,weight gain rate,head circumference growth rate,length of hospital stay,and hospital costs (all P ＞ 0.05).Meanwhile,the rates of neonatal necrotizing enterocolitis,sepsis,mechanical ventilation administration,and damage of hepatic and renal function were also not significantly different.The administration of different dosages of amino acids did not affect the energy intake in both groups (F =1.893,P =0.218).Conclusions Prematures need early amino acid administration,with a starting dosage of more than 1.5 g/(kg · d).Rapid increase of amino acid dosing will not affect early nitrogen balance in premature infants; meanwhile,it contributes to weight gain without increasing the complications and hospitalization costs.