Objective To explore the clinical and epidemiological characteristics of severe measles in infants. Methods Clinical data of 62 infants with severe measles were analyzed retrospectively.Results Of 62 infants with severe measles,42 (67.74%)were aged < 9 months,of whom 37(88.10%)were not vaccinated against measles. The onset months were February-May,41 cases (66.13%)were found in March-April.All patients had fever and skin rash,the rates of other symptoms and complications were as follows :oral leukoplakia 80.65%(n=50),Ca-tarrh symptom 77.42%(n=48),conjunctivitis 79.03%(n=49),history of choked water cough 75.81%(n=47), pneumonia 95.16%(n=59),acute laryngitis 35.48% (n=22),electrolyte disorder 20.97%(n=13),acute respir-atory distress syndrome 9.68%(n=6),liver function damage 9.68%(n=6),pneumothorax 8.06% (n=5),myo-cardial damage 4.84% (n=3),respiratory failure 3.23% (n=2),toxic encephalopathy 3.23%(n=2),measles en-cephalitis 1.61% (n= 1),and pleural effusion 1.61% (n = 1 ).Of all cases,41 cases were cured,19 cases im-proved,1 case died,and 1 case gave up treatment.Conclusion These severe measles cases occurred mainly in in-fants aged<9 months and were not vaccinated against measles;infants had history of choked water cough;the main onset months were March-April ;pneumonia was still a predominant complication of infant measles.

Objective To review the early postoperative complications and its treatment in infants with tetralogy of Fallot(TOF).Methods From January 2004 to March 2007,35 infant patients with TOF,aged 4 to 12 months,underwent corrective procedure with hypothermic cardiopulmonary bypass(CPB) and general anaesthesia in Anhui provincial children's hospital.Of all the patients,30 cases received radical surgery and the other 5 cases with initial palliation.Results The early postoperative complications occurred in 10 cases,of which 6 cases comlplicated with low cardiac output,2 cases had severe arrhythmia,2 cases manifested as perfusion lung,2 cases were symptomized by residual right ventricular outflow tract(RVOT) obstruction and 1 case suffered from residual ventricular septal defect(VSD).There were 3 cases of early postoperative death with 8.6% of hospital mortality.Conclusion The operative mortality can be effectively reduced by careful preparations for surgery,reliable surgical procedure,effective myocardial protection and timely treatment for postoperative complications.This study demonstrated that repair of TOF in infancy can be done with excellent results.

OBJECTIVETo analyze clinical and imaging features and genetic characteristics of Leigh syndrome with emergent pulmonary edema.

METHODThe clinical features and imaging data of 2 cases (1 male, 1 female) seen in Anhui Provincial Children's Hospital from 2012 to 2014 were analyzed and summarized. Venous blood samples were sent to Guangzhou Jinyu Medical Examination Center for genetic analysis. Peripheral blood DNA was extracted and amplified, then sent to a sequencing facility for presence of genetic mutation by comparing with the reference sequence (NC_012920.1).

RESULT(1) The first patient was a 7 months old boy. The second patient was a 7 months and 21 days old girl. They were presented with abnormal respiration and pulmonary hemorrhage required mechanical ventilation. The first patient had a similar attack after 4 months of his birth, whose psychomotor development was normal, and no abnormal neurological findings. The value of blood lactate was 1.58 mmol/L. The value of pyruvic acid was 0.25 mmol/L. The value of cerebrospinal fluid lactate was 6. 4 mmol/L, which was an abnormal increase. The second patient had abnormal nervous system development, which included motor development retardation and hypotonia. The value of blood lactate was 6. 8 mmol/L, pyruvic acid was 0.31 mmol/L. Cerebrospinal fluid lactate was 8.2 mmol/L. (2) Imaging data: chest X-ray revealed double lung effusion. Bilateral caudate nucleus and lentiform nucleus had high signal, and bilateral internal capsule forelimbs were affected in DWI sequence of head MRI. Hemispheres, basal ganglia, cerebral peduncle, cerebellum, pons, and splenium of corpus callosum had multiple abnormal signals in head MRI of the second patient. NAA peak showed significantly reduced lesion area in magnetic resonance blood-flow scanning, and Cho peak increased significantly, which were double lactate-peak. (3) Genetic testing: ATPase6 m.9185 t > C mutation was found in case 1 that was consistent with Leigh syndrome pathogenesis. Hybrid mutations (m. 10191 t > C) in mitochondrial DNA was found in case 2. Two cases with the diagnosis of Leigh syndrome was clear. They were given combined therapy, such as mechanical ventilation, limited fluid to alleviate lung exudation, coenzyme Q10, and L-carnitine. The illness of case 1 relapsed after discharge. But in case 2, there was no improvement. They both died after treatment was given up.

CONCLUSIONNeurological symptoms were common in Leigh syndrome, in which acute lung hemorrhage was rarely reported. Timely ventilator support can temporarily save lives, but fatality rate is high and prognosis is poor.

Brain ; pathology ; Carnitine ; therapeutic use ; DNA, Mitochondrial ; Female ; Genetic Testing ; Hemorrhage ; etiology ; Humans ; Infant ; Lactic Acid ; Leigh Disease ; complications ; genetics ; Lung Diseases ; etiology ; Magnetic Resonance Imaging ; Male ; Mutation ; Pyruvic Acid

Objective To investigate the clinical characteristics,diagnosis and treatment of necrotizing fasciitis (NF).Methods The authors reviewed and analyzed clinical manifestations,auxiliary examinations,treatments and prognoses of 14 patients who had been diagnosed with NF and hospitalized in the Children's Hospital of Anhui province between Jan 2007 and Sep 2013.Results Among the patients included in this study,eight cases were male and six cases were female.The average age was (15.86 ± 10.48) month,The time of abnormal temperature was (10.64 ± 5.64) d,hospital day was (29.07 ± 16.30) d,numbers of debridements were (3.07 ± 1.33) times.All patients had septic shock in which 5 cases had multiple organ failure.Diseases were found on hips (5 cases),lower limbs (4 cases),back (2 cases),perineum (2 cases),and neck (1 case).Blood culture showed staphylococcus aureus in six cases (1 case of methicillin-resistant staphylococcus aureus),pseudomonas aeruginosa in four cases and angina group of streptococcus pneumoniae in one case.No obvious bacteria growth was observed in three cases.CT examinations reflected subcutaneous gas formation in 11 cases but skin and subcutaneous tissue edema and fascial thickening in all cases.All cases of NF were further confirmed with soft tissue biopsies.Early symptoms resembled those of cellulitis.As the diseases progressed,other symptoms appeared such as skin ulceration,bullae formation and gas formation in the tissues.All patients were treated with surgical debridements (vacuum sealing drainage continuous drainage in 5 cases),appropriate antibiotic coverage and colloid supporting treatments.Seven patients were healed (50.00％,7/14),four had skin grafts (28.57％,4/14) and 3 died(21.43％,3/14).Six cases were found having limited physical activities in two year follow-up visits.Conclusion NF is a rare but potentially fatal disease.It is commonly found on the perineum,abdominal wall and extremities.NF can easily lead to septic shock and multiple organ failure.Early detection,surgical debridement and proper drainage along with appropriate antibiotic coverage can decrease mortality rates.

Objective To compare the sedation and anti-inflammatory effects of dexmedetomidine and midazolam on critical ill children with multiple trauma. Methods A prospective randomized controlled trial was conducted. Sixty-five critical ill children with multiple trauma admitted to pediatric intensive care unit (PICU) of Anhui Province Children's Hospital from January 2014 to September 2016 were enrolled, who were randomly divided into dexmedetomidine group (33 cases) and midazolam group (32 cases). Children of both groups received sufentanil for analgesia. Children in dexmedetomidine group firstly received 1.0 μg/kg intravenous bolus of dexmedetomidine for 10 minutes, then continuous infusion of 0.2-0.7 μg·kg-1·h-1, while in midazolam group children received 1-5 μg·kg-1·min-1 of midazolam in continuous infusion. The goal of sedation was to maintain a Richmond agitation-sedation scale (RASS) score of -1 to 0. The level of serum interleukin (IL-6, IL-8, IL-10, IL-1β), tumor necrosis factor-α (TNF-α) and C-reactive protein (CRP) were detected by enzyme linked immunosorbent assay (ELISA) at 24, 48, 72 hours after treatment, and the duration of mechanical ventilation, ratio of continuous renal replacement therapy (CRRT), length of stay in the PICU, ratio of sepsis and multiple organ failure (MOF) and mortality were also recorded. Results Compared with midazolam, dexmedetomidine decreased the level of pro-inflammatory cytokines and increased the level of anti-inflammatory cytokines. At 24 hours after treatment, the levels of serum IL-1β, TNF-α significantly decreased and IL-10 significantly increased [IL-1β (ng/L):6.48±2.89 vs. 8.07±3.14, TNF-α (μg/L): 11.25±5.21 vs. 15.44±5.97, IL-10 (ng/L): 12.10±5.35 vs. 9.58±4.71, all P < 0.05]. At 48 hours after treatment, the levels of serum IL-6, IL-8, IL-1β, TNF-α and CRP significantly decreased and IL-10 significantly increased [IL-6 (ng/L): 209.67±80.49 vs. 336.31±123.94, IL-8 (ng/L):229.09±80.81 vs. 298.28±90.25, IL-1β (ng/L): 7.31±3.02 vs. 8.74±3.17, TNF-α (μg/L): 12.52±4.79 vs. 16.58±5.98, CRP (g/L): 47.82±24.92 vs. 72.35±31.71, IL-10 (ng/L): 12.90±5.42 vs. 10.01±4.79, all P < 0.05]. At 72 hours after treatment, the levels of serum IL-8 and CRP significantly decreased [IL-8 (ng/L): 234.64±96.24 vs. 290.28±103.97, CRP (g/L): 53.24±29.12 vs. 86.58±38.30, both P < 0.05]. Compared with midazolam, dexmedetomidine could significantly reduce the duration of mechanical ventilation (days: 4.7±1.3 vs. 6.6±2.1), length of PICU stay (days: 9.5±2.7 vs. 12.3±3.9, both P < 0.05), and the ratio of sepsis (33.3% vs. 53.1%, P < 0.05). But there were no significant differences in ratio of CRRT (18.2% vs. 18.8%), MOF (9.1% vs. 18.8%) and mortality (6.1% vs. 12.5%) between two groups (all P > 0.05). Conclusion Compared with midazolam, dexmedetomidine had better efficacy in the treatment of severe multiple trauma in children and reduce the level of inflammation.

Objective To analyze the etiology and prognosis of children with thrombocytopenia (TP) in pediatric intensive care unit (PICU). Methods The data of children with TP (exclusion of congenital and unknown TP) admitted to PICU of Anhui Provincial Children's Hospital from January 2008 to December 2017 was analyzed retrospectively. According to the age of onset, the children were divided into infantile group (29 days to less than 1 year), early childhood group (1 to less than 3 years), preschool group (3 to less than 6 years), school age group (6 to less than 10 years) and puberty group (more than 10 years). Moreover, according to the lowest platelet count (PLT), the children were divided into PLT≤20×109/L group, PLT (21-50)×109/L group and PLT > (50-100) ×109/L group. The distribution and mortality of TP were analyzed, and the relationship between age, PLT decrease and prognosis were analyzed by Pearson method. Results Among 6 725 children admitted to PICU in our hospital from January 2008 to December 2017, there were 683 children with TP, with the incidence of 10.2%. Among 683 children with TP, there were 387 males and 296 females, with the median age of 2.72 (0.61, 3.08) years, and 92 children died, with a total mortality of 13.5%. Analysis of primary disease showed that TP caused by non-hematological malignant tumor disease accounted for 73.9%, with the mortality of 11.1% (56/505). TP induced by hematological malignant tumor disease accounted for 21.4%, with the mortality of 24.7% (36/146). Pseudothrombocytopenia accounted for 0.6%, with the mortality of 0% (0/4). Other children who gave up treatment accounted for 4.1%. It was shown by further analysis that multiple organ dysfunction syndrome (MODS) caused by TP associated with non-hematological malignant tumor disease accounted for 26.9%, with the mortality of 15.4% (21/136). Sepsis, severe trauma, pneumonia, central nervous system infection and disseminated intravascular coagulation (DIC) accounted for 17.4%, 16.6%, 12.7%, 11.7% and 11.5%; with the mortality of 8.0% (7/88), 2.4% (2/84), 0% (0/64), 20.3% (12/59) and 24.1% (14/58), respectively. The main causes of TP associated with hematological malignant tumor disease were hemophagocytic syndrome [accounting for 27.4%, with the mortality of 32.5% (13/40)] and bone marrow inhibition [accounting for 21.2%, with the mortality of 25.8% (8/31)]. The younger were the children with TP, the higher would be the mortality. The mortality of infantile group was significantly higher than that of early childhood group, preschool group, school age group and puberty group [18.8% (53/282) vs. 14.0% (28/200), 6.7% (7/104), 4.3% (4/92), 0% (0/5), all P < 0.01]. The lower was the PLT, the higher would be the mortality. The mortality of PLT≤20×109/L group was significantly higher than that of PLT (21-50)×109/L group and PLT > (50-100)×109/L group [18.1% (39/215) vs. 13.0% (32/247), 9.5% (21/221), both P < 0.05]. It was shown by correlation analysis that there was no association between age and 28-day death time in children with TP (r = -0.037, P = 0.727), but PLT was positively correlated with 28-day death time in children with TP (r = 0.844, P = 0.010). Conclusions MODS, infection, sepsis, severe trauma and DIC are the common causes of TP in PICU. The younger are the children with TP, the lower is the PLT, and the worse would be the prognosis.

OBJECTIVE: To analyze the clinical features and genetic basis for a child with pyruvate carboxylase deficiency type A (PCD-A). METHODS: Clinical data of the child was retrospectively analyzed. The child and his parents were subjected to trio-whole exome sequencing, and candidate variants were verified by bioinformatics analysis. RESULTS: The child was admitted due to fever with vomiting and disturbance of consciousness. His clinical manifestations included severe decompensated acidosis, hypotension and intractable shock. Cranial MRI showed abnormal signal in the brain, and chest X-ray revealed acute pulmonary edema. DNA sequencing revealed that he has harbored compound heterozygous variants of the PC gene, namely c.182T>C (p.I61T) and c.2581G>A (p.V861M), which were respectively inherited from his father and mother. Neither variant was retrievable in the ClinVar and HGMD databases. Through prediction of protein structure, both variants may affect the functional stability of the protein product. CONCLUSION The compound heterozygous variants of the PC gene probably underlay the PCD-A in this child. Combined with the clinical features, the child was ultimately diagnosed as PCD-A. Above finding has enriched the spectrum of PC gene variation underlying PCD-A.
Child ; Family ; Humans ; Male ; Mutation ; Pyruvate Carboxylase Deficiency Disease ; Retrospective Studies ; Exome Sequencing

To investigate the risk factors and prognosis of acute kidney injury (AKI) in children with sepsis in pediatric intensive care unit (PICU). Methods A retrospective analysis of clinical data of PICU sepsis children in Anhui Children's Hospital from May 2015 to May 2018 was performed. The children were divided into AKI group and non-AKI group according to whether AKI occurred within 48 hours of PICU [referring to the diagnostic criteria for Kidney Disease: Improving Global Outcomes (KDIGO)]. The general data, physiological data and clinical outcomes of the two groups were compared; Logistic regression analysis was used to analyze the risk factors of AKI in children with sepsis and the prognostic factors. Results AKI occurred in 55 of 127 children with sepsis, the incidence of AKI was 43.3%, and the overall mortality was 28.3% (36/127), with 41.8% (23/55) in AKI group and 18.1% (13/72) in non-AKI group.① Compared with non-AKI group, oxygenation index, albumin, the pediatric critical illness case score (PCIS) and urine volume in AKI group were significantly decreased, while cystatin C, procalcitonin (PCT), prothrombin time (PT), activated partial thromboplastin time (APTT), pediatric multiple organ dysfunction score (P-MODS), the proportions of mechanical ventilation, vasoactive drug use, shock, septic shock and mortality were significantly increased, while there was no difference in age, gender, mean arterial pressure (MAP), white blood cell count (WBC) and C-reactive protein (CRP) between the two groups. Multivariate Logistic regression analysis showed that low serum albumin [odds ratio (OR) = 0.627, 95% confidence interval (95%CI) = 0.495-0.794, P = 0.000] and homocystatin C (OR = 2.641, 95%CI = 1.157-6.032, P = 0.021) were risk factors for AKI in children with sepsis. ② Compared with the survival group of children with sepsis AKI, the proportion of mechanical ventilation, septic shock, vasoactive drug use, positive balance ratio of liquid for 72 hours, 6-hour lactate clearance rate < 10%, and AKI 3-stage patients in the death group of children with sepsis AKI were significantly increased. Multivariate Logistic regression analysis showed that 72-hour positive liquid balance (OR = 8.542, 95%CI = 1.956-37.307, P = 0.004) and 6-hour lactate clearance rate < 10% (OR = 5.980, 95%CI = 1.393-25.676, P = 0.016) were risk factors for the death of children with sepsis AKI. Conclusions Serum albumin and cystatin C should be closely monitored in children with sepsis. Early detection and intervention of positive fluid balance and low lactate clearance rate can reduce the mortality of AKI in children with sepsis.

Objective To investigate the treatment,outcomes and disease burden of severe hand-foot-and mouth diseases(HFMD),and to evaluate the compliance to the 2011 guideline for treatment in regions with a high in-cidence of HFMD.Methods A collaborative study group was established including Children's Hospital of Fudan Uni-versity,Jiangxi Provincial Children's Hospital,Anhui Provincial Children's Hospital,Linyi People's Hospital and the Second People's Hospital of Fuyang City.Clinical manifestation,treatment,prognosis and other data of severe HFMD pa-tients were prospectively collected by filling out a survey form in real time from April 2014 to October 2016. Results Two hundred and twenty-six severe HFMD cases were collected during the research time,including 114 ca-ses in stage 2,75 cases in stage 3,and 37 cases in stage 4.Two hundred and twenty-one cases(97.8%)were given mannitol,with a mortality of 6.2%;91 cases(40.3%)were given mannitol and glycerol fructose,with a mortality of 3.3%;the combined use of mannitol and glycerol fructose might have a better result than the single use of mannitol. One hundred and ninety-eight cases(87.6%)were given intravenous immunoglobulin(IVIG).One hundred and ninety cases(84.1%)were given antiviral drugs.One hundred and forty-five cases(64.2%)were given hormone therapy,and the use of hormone could reduce temperature,but did not reduce the mortality.One hundred and fifty cases (66.4%)needed vasoactive agent,including milrinone,dobutamine,phentolamine and sodium nitroprusside. The vasoactive agent use in stage 3 and 4 were 88.0%(66/75 cases)and 91.9%(34/37 cases),respectively.Sixty-nine cases(30.5%)received continuous positive airway pressure(CPAP),91 cases(40.3%)with mechanical ventila-tion,peak inspiratory pressure(PIP)≥20 cmH2O(1 cmH2O=0.098 kPa)accounted for 61.6%(53/86 cases),po-sitive end-expiratory pressure(PEEP)≥10 cmH2O accounted for 36.3%(33/91 cases).The mean mechanical ven-tilation time was(125.9 ± 101.8)h.Eleven cases(4.86%)died or died after giving up treatment,in which the mortality in stage 3 was 6.7%(5/75 cases),and the mortality in stage 4 was 16.2%(6/37 cases).The death causes were respiratory failure,pulmonary hemorrhage,and circulatory failure. The average time from onset to death was (5.91 ± 5.26)(1-15)d.Length of stay in hospital was(9.18 ± 5.16)(1-37)d in which length of stay in hospi-tal in stage 3 and 4 were(11.3 ± 6.35)d,(11.4 ± 6.62)d,respectively,which were significantly longer than that in stage 2[(7.50 ± 3.04)d],and the difference was statistically significant(P <0.05). The cost was(19 136 ± 12 556)CNY,of which the cost in stage 3 and 4 was(23 121 ± 13 846)CNY,(29 849 ± 16 015)CNY,respectively, which were significantly higher than that in stage 2[(12 961 ± 4 272)CNY],and the difference was statistically sig-nificant(P<0.05). Multivariate analysis found that respiratory rhythm abnormality,capillary refill time more than 3 seconds were risk factors for the deaths in the severe HFMD.Conclusion The 2011 edition of guidelines for treat-ment of children with severe HFMD was well executed.Hormone,IVIG,antiviral drugs did not significantly reduce the mortality of severe HFMD in children.

Objective To investigate the clinical features of severe hand,foot and mouth disease (HFMD) in recent three years,and to analyse the risk factors of severe HFMD.Methods A multicenter collaborative research group was set up,including five children's hospitals(pediatric department)with high incidence of HFMD.Prospective epidemiologic approaches were adopted.After training,staffs from the col-laborative center executed the study by filling up the record forms.Results We collected 114 HFMD cases in stage 2,75 cases of HFMD in stage 3,37 cases of HFMD in stage 4 from April 2014 to October 2016.The age ranged from 2 months to 13 years old,the median age was 2 years old,younger than 3 years accounted for 86.3%.Fever was observed in all the severe HFMD,mean temperature was (39.2 ± 0.7) ℃,fever lasted for (4.54 ± 2.89)d.The mean heart rates in stage 2,3,4 were (128.2 ± 13.3,176.1 ± 22.2,184.2 ± 27.5) times/min,respectively.The mean arterial pressure was (84.4 ± 14.6) mmHg(1 mmHg=0.133 kPa),the mean respiratory rate was (39.0 ± 8.4)times/min,the mean respiratory rates in stage 2,3,4 were (37.8 ± 7.36,38.7 ± 8.13,43.4 ± 10.7) times/min,respectively. Respiratory rhythm abnormality in stage 2,3,4 were 9.6%,14.9% and 56.8%,respectively.The blood glucose increased gradually in 2,3 and 4 stages,the mean blood glucose in stage 4 was(12.4 ± 4.74)mmol/L.The incidence of coma in stage 4 was higher than those in stage 2 and 3. Multivariate Logistic regression analysis found that tachycardia,drowsiness,coma, respiratory rate increase,respiratory rhythm abnormality,capillary refilling time more than 3 seconds were risk factors for severe HFMD.Conclusion The incidence of severe HFMD is still high in children under 3 years of age in last three years.The severe patients have obvious changes in the nervous,respiratory and circulatory system. Tachycardia,drowsiness,coma,respiratory rate increase,respiratoy rhythm abnormality,capillary refilling time more than 3 seconds are risk factors for severe HFMD.