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Objective:To investigate the clinical characteristics and genotypes of neurodevelopmental disorders of microcephaly-epilepsy-cortical atrophy caused by VARS1 gene mutation.Methods:The clinical data of a child with VARS1 gene mutation who visited Anhui Children′s Hospital in December 2021 were reviewed and followed up. Using "VARS1" "VARS" "VALYL-tRNA synthetase" "Valyl-tRNA synthetase" as the search terms, the Chinese data bases (China National Knowledge Infrastructure database, Wanfang database) and PubMed database (the database establishment until 2022). Articles related to genetic diseases were searched and clinical phenotypes and genotypes were summarized.Results:This case was a 3-month-old girl. After birth, she suffered from repeated convulsions and feeding difficulties, and gradually developed microcephaly, hypotonia, and overall developmental delay. Brain imaging showed cortical atrophy, corpus callosum dysplasia, and craniosynostosis. The results of whole exome sequencing indicated a new homozygous gene mutation in VARS1: gene mutation in exon 27 of chromosome 6 c.3203C>T(p.Thr1068 Met), which was from the patient 's parents. She took phenobarbital, levetiracetam, and sodium valproate for anti-epileptic treatment regularly, and then convulsed as a seizure every few days. A total of 19 patients were reported in 5 literatures that met the search criteria, all of whom presented with neurodevelopmental disorders. A total of 20 VARS1 gene mutation sites have been found so far. Conclusions:Neurodevelopmental disorders of microcephaly-epilepsy-cortical atrophy should be considered for children with neurodevelopmental disorders such as microcephaly, epilepsy, developmental delay, and cortical atrophy. Gene sequencing plays an important role in the diagnosis of the disease.
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Objective:To explore the clinical characteristics of children with severe tuberculosis (TB) in pediatric intensive care unit (PICU) and the diagnostic viability of metagenomic next-generation sequencing (mNGS).Methods:The clinical data and mNGS results of 3 children with severe TB admitted to PICU of Anhui Provincial Children′s Hospital from January 2018 to December 2020 were retrospectively analyzed.The literature reporting children with TB diagnosed by applying mNGS were searched in China National Knowledge Infrastructure, Wanfang data, VIP and PubMed data bases, all of which were searched from the establishment of the database to March 2021, and the relevant literature was reviewed.Results:(1)All of 3 cases, including 2 males and 1 female, all had fever.One case was diagnosed with X-linked severe combined immunodeficiency (X-SCID). Acid-fast stain tests of body fluid, purified protein derivative (PPD) tests and T-cell spot tests of tuberculosis infection (T-SPOT.TB) were conducted in 3 cases, with the test results being negative, and the early diagnosis was confirmed with mNGS.Two cases were discharged from the hospital after being switched to special anti-TB treatment.(2)A total of 3 articles were retrieved, in which 7 children were reported.One male with X-SCID combined with bacillus Calmette-Guérin-associated hemophagocytic syndrome and 6 children with tuberculous meningitis were subject to early diagnosis with mNGS (detection of Mycobacterium tuberculosis complex).Conclusions:It is promising for the application of mNGS in rapid pathogen diagnosis of children with severe TB, especially in children with immunodeficiency.It could optimize early diagnosis and treatment to improve prognosis.
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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.
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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.
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Objective To study the levels of plasma catecholamine( norepinephrine,epinephrine and dopamine) in children with severe hand,foot and mouth disease( HFMD) ,and to assess the influence of cate-cholamine on the pathogenesis of severe HFMD. Methods A collaborative study group was established, including Children′s Hospital of Fudan University, Jiangxi Provincial Children′s Hospital, Anhui Provincial Children′s Hospital,Linyi People′s Hospital and No. 2 People′s Hospital of Fuyang City. Blood samples from children with severe HFMD were collected from April 2014 to October 2016. The levels of blood epineph-rine,norepinephrine,dopamine were measured at 2 h,24 h and 48 h after diagnosis for HFMD. Results The level of epinephrine at 24 h after diagnosis was ( 213. 0 ± 139. 8 ) ng/L in children with pulmonary edema, which was significantly higher than that of children without pulmonary edema[(137. 8 ± 45. 5)ng/L)](P=0. 022). The level of epinephrine at 24 h after diagnosis was (373. 2 ± 298. 1)ng/L in the dead children,and was (144. 2 ± 42. 5)ng/L in the survival children. The concentration of norepinephrine at 24 h after diagnosis was (1935. 7 ± 1824. 1)ng/L in the dead children,and was (858. 3 ± 212. 7)ng/L in the survival children. The levels of epinephrine and norepinephrine of those who died from HFMD were significantly higher than those of survival children at 24 h after diagnosis. There were no significant differences in catecholamine con-centrations among stage 2,3,4 HFMDs at 2 h,24 h and 48 h after diagnosis. Sex and enterovirus 71 virus infection had no significant influences on plasma catecholamine concentrations in children with severe HFMD. Conclusion Plasma epinephrine levels increase in children with HFMD complicated with pulmonary edema. Epinephrine and norepinephrine may play an important role in the death of children with severe HFMD.
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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.
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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.
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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). Conclusion Compared with midazolam, dexmedetomidine had better efficacy in the treatment of severe multiple trauma in children and reduce the level of inflammation.
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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.
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<p><b>OBJECTIVE</b>To evaluate the characteristics of the serious complications of 17 cases with measles in ICU.</p><p><b>METHOD</b>Seventeen cases with measles with serious complications in ICU in Anhui Provincial Children's Hospital were recruited from May 2012 to May 2013. Clinical characteristics, image finding, and prognosis were analyzed retrospectively. IgM antibody was positive in all the 17 cases, which included 9 male cases, 8 female cases, and their age was from 2 months to 10 years.</p><p><b>RESULT</b>All the 17 patients received mechanical ventilation because of severe respiratory distress within 1 week of onset, of which 14 cases were complicated with acute respiratory distress syndrome (acute ARDS), 6 cases of tension pneumothorax, 3 cases were complicated with bronchitis, laryngeal obstruction III degrees, and totally 7 cases died. The survived 10 patients were followed up for 1 year, 1 patient with localized pneumothorax, bronchopleural fistula, 1 case of mild pulmonary fibrosis, 1 case of acute laryngitis with persistent hoarseness, and mild inspiratory dyspnea were found. The remaining 7 cases fully recovered. Fourteen cases failed to inoculate measles vaccine.</p><p><b>CONCLUSION</b>A higher risk of death and poor prognosis were found in children with measles who needed to be treated in PICU, especially for ARDS with pneumothorax. Laryngitis needed long time to cure and had weaning difficulties, and the case associated with pleural effusion or bronchopleural fistula must be treated with surgery, and the patients easily develop secondary bacterial infection.</p>
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Child , Female , Humans , Male , Dyspnea , Intensive Care Units, Pediatric , Lung Diseases , Measles , Pleural Effusion , Prognosis , Pulmonary Fibrosis , Respiration, Artificial , Retrospective StudiesABSTRACT
<p><b>OBJECTIVE</b>To discuss the clinical characteristics, diagnosis and treatment management of ornithine transcarbamylase deficiency (OTCD).</p><p><b>METHOD</b>Data of the clinical diagnosis and treatment of a case with OTCD were analyzed, and the domestic and international literature was reviewed.</p><p><b>RESULT</b>(1) The case was a boy, 8 years old; and the initial symptoms were vomiting and reduced consciousness for a day after eating a lot of eggs as previous similar history. The level of blood ammonia was 2 500 µmol/L. The patient was treated with fasting, high-calorie fluid intravenous infusion, reducing blood ammonia. However, the disease further aggravated and the patient died due to brain hernia and central cardiovascular failure. Finally, he was confirmed as OTCD through urine testing by gas chromatography-mass spectrometry, plasma amino acid examination (plasma arginine and citrullineurine reduced, orotic acid raised) and genetic testing (OTC c.386G>A p. (Arg129His)). (2) Data of 55 case reports about diagnosis and treatment outcome including 65 OTCD cases' clinical data in domestic and abroad reports in nearly 5 years. Their ages ranged from 3 days to 49 years; the common symptoms were vomiting and encephalopathy; both urine tests were positive in 52 cases; pathogenic genes had less repeated loci and located at Xp21.1 in 42 cases detected by OTC gene test; all of which had gene mutation.</p><p><b>CONCLUSION</b>The acute onset OTCD begins with the symptoms of vomiting and unconsciousness, with rapid rise of blood ammonia level. OTCD may cause high mortality.</p>
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<p><b>OBJECTIVE</b>To investigate the clinical characteristics and treatment of negative pressure pulmonary edema (NPPE) with upper airway obstruction (UAO) in children.</p><p><b>METHOD</b>Data of 3 cases with NPPE and UAO in pediatric intensive care unit (PICU) from Mar, 2007 to May, 2013 were analyzed.</p><p><b>RESULT</b>(1) Two cases were male and 1 was female with age respectively 6, 16 and 30 months.One had airway foreign body , 1 laryngitis , and 1 retropharyngeal abscess. The onset of NPPE varied from 5 to 40 minutes following relief of obstruction. (2) NPPE presented with acute respiratory distress with signs of tachypnea, tachycardia, 2 of the 3 with pink frothy pulmonary secretions, progressively decreased oxygen saturation, rales on chest auscultation and wheezing. (3) NPPE chest radiograph showed diffuse interstitial and alveolar infiltrates, images confirmed pulmonary edema. (4) All these patients received these therapeutic measures including mechanical ventilation, retaining high PEEP, diuretics, limiting the fluid input volume to 80-90 ml/ (kg×d) on the basis of circulation stability. The rales on chest auscultation disappeared after 10, 6, 12 hours. The ventilators of 2 patients were removed within 24 hours, in another case it was removed 50 hours later because of secondary infection. All patients were cured and discharged without complication.</p><p><b>CONCLUSION</b>NPPE progresses very fast, characterized by rapid onset of symptoms of respiratory distress after UAO, with pulmonary edema on chest radiograph. The symptoms resolve rapidly if early support of breath and diuretics are applied properly.</p>
Subject(s)
Child, Preschool , Female , Humans , Infant , Male , Acute Disease , Airway Obstruction , Diuretics , Therapeutic Uses , Foreign Bodies , Laryngismus , Positive-Pressure Respiration , Postoperative Complications , Therapeutics , Pulmonary Edema , Diagnosis , Therapeutics , Radiography, Thoracic , Retrospective StudiesABSTRACT
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.
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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.