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Objective:To study the genetic profiles and clinical characteristics of neonatal-onset genetic epilepsy.Methods:From July 2016 to May 2021, patients with neonatal-onset genetic epilepsy admitted to our hospital and received second-generation genetic sequencing were enrolled in this study. According to the types of genetic variations, the patients were assigned into ion channel group and non-ion channel group. Clinical characteristics, treatments and prognosis of the two groups were compared.Results:A total of 36 patients with identified genetic variations were enrolled, involving 15 epilepsy-related genes. KCNQ2, SCN2A and STXBP1 were the most common pathogenic genes. 20 cases (55.6%) were in the ion channel group and 16 cases (44.4%) in the non-ion channel group. No significant differences existed in their general status, seizure types, EEG characteristics, treatments and outcomes between the two groups ( P>0.05). Among all 36 cases, the age of onset ranged from 10 min to 24 d after birth and 28 cases (78.8%) developed epilepsy within 1 week after birth. Developmental and epileptic encephalopathies were diagnosed in 20 patients. 7 patients were diagnosed with self-limited neonatal epilepsy, 2 were pyridoxine dependence, 2 were Zellweger syndrome and 1 case of self-limited familial neonatal-infantile epilepsy, Turner type mental retardation with epilepsy, PURA syndrome, Rett syndrome and 22q11.2 deletion syndrome, each. The patients received antiepileptic drugs including phenobarbital, levetiracetam, oxcarbazepine, topiramate, valproic acid, benzodiazepines (nizepam/clonazepam /clobazam/midazolam), lacosamide and lamotrigine. 5 patients died after giving up treatment. 31 patients were followed up for 6 to 50 months. 22 cases (71.0%) were controlled at 1- to 35-month-old including 21 cases (56.7%) with developmental delay. 6 cases (19.4%) had ineffective seizure control and 3 cases (9.7%) showed reduced seizures, all with varying degrees of developmental delay. Conclusions:Neonatal-onset epilepsy is correlated with multiple genes. KCNQ2, SCN2A, STXBP1 are the common pathogenic genes with multiple variants of KCNQ2 gene. Most patients have seizures within 1 week after birth. More than half of patients have ion channel related gene variations. Sodium channel blockers have certain effects as treatment.
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Objective:To study the outcomes and post-discharge follow-up of neonatal tracheotomy in neonatal intensive care unit(NICU).Methods:This study included patients who were admitted to NICU in Beijing Children′s Hospital from January, 2016 to August, 2021, and less than 28 days or 44 weeks(corrected age)on admission, and required tracheotomy.The patients were divided into tracheotomy group and the non-tracheotomy group (the parents signed to refuse the tracheotomy) according to whether perform tracheotomy.Demographic data, general hospitalization information, diagnosis, indications for tracheotomy, follow-up outcomes at 3/6/12 months of age after discharge of patients were collected and analyzed.Results:Totally 26 patients were included in this study, 14 cases in tracheotomy group and 12 cases in non-tracheotomy group.The average gestational age was(37.7±3.80)weeks and(38.99±1.83)weeks, and birth weight was(2 823.57±948.89)g and (3 320.83±378.76)g, respectively.There were no significant differences in sex, gestational age, birth weight, age on admission, weight on admission, age at diagnosis, ratio of endotracheal intubation for respiratory support on admission between two groups( P>0.05). The commonest indications of tracheotomy group were bilateral vocal cord paralysis(50.0%) and congenital anomaly/defect of throat/larynx(21.4%), and the commonest indications of non-tracheotomy group were bilateral vocal cord paralysis(50.0%) and vocal cord/subglottic mass(25.0%), and there was no significant difference between two groups( P>0.05). The rate of discharge-against-medical order of tracheotomy and non-tracheotomy group was 7.14% and 66.67%( P=0.003), respectively.The total follow-up rate of tracheotomy and non-tracheotomy group was 88.9% and 38.9%, while the follow-up rates at 3 months, 6 months, and 12 months were 100.0% vs. 50.0%, 83.3% vs. 41.7%, and 81.8% vs. 25.0%, respectively, whose differences were statistically significant(all P<0.05). In the 14 cases of tracheotomy group, 3 cases died, 4 cases successfully removed the tracheal cannula, 5 cases did not remove the tracheal cannula, and 2 cases were lost. Conclusion:Bilateral vocal cord paralysis is the commonest indication of neonatal tracheotomy.Parents′ compliance in the tracheotomy group is significantly higher than that in non-tracheotomy group.To give caring skill training for parents of neonates with tracheotomy before discharge is beneficial for improving the overall prognosis of children.
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Objective:To explore the clinical manifestations, genetic disorder, prognosis of 14 neonates with primary immunodeficiency disease(PID).Methods:A total of 14 newborns with PID admitted to Department of Neonatology at Beijing Children′s Hospital from January 2017 to December 2019 were enrolled for retrospective analysis, focusing on their clinical manifestation, peripheral blood cell examnations, gene mutation, and outcomes after hemotopoietic stem cell transplantation(HSCT).Results:The average gestational age of the newborn was (38.6±1.2) weeks, the birth weight was (3 265±325)g, and the median diagnosis time was 57.5 days.Fourteen newborns with PID were diagnosed by whole exome sequencing as chronic granuloma (6/14), DiGeogre syndrome (3/14), Wiskott-Aldrich syndrome (2/14), severe combined immunodeficiency (2/14) and selective IgA deficiency (1/14). Regarding the clinical manifestations, fever, pneumounia and colitis accounted for 7/14, the decrease of T lymphocytes in peripheral blood accounted for 6/14, and the decrease of B lymphocytes accounted for 5/14.The absolute value of eosinophils increased (>500 cells/mm 3) accounted for 12/14, of which moderately increased (1 500 to 5 000 cells/mm 3) accounted for 5/12, and the absolute value of monocytes increased (median>1.5×10 9/L) accounted for 7/14.Follow-up children received HSCT accounted for 7/14, and the median time of receiving transplantation was 330 days after birth.By the time of follow-up, the primary disease resolved after HSCT accounted for 5/7, and the survival rate was 85.7%.Among them, two children with chronic granulomatosis were diagnosed with inflammatory bowel disease before transplantation, and the primary disease improved after HSCT.Three-quarters of the deaths had inflammatory bowel disease-like manifestations and died of infectious shock. Conclusion:The clinical manifestations of children with PID during the neonatal period are not specific.The manifestations of colitis need more attention.Some of the newborns with PID will evolve into inflammatory bowel disease or have inflammatory bowel disease-like manifestations or even die of it.HSCT is a fundamental treatment for the primary disease.
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Objective:To study the clinical features and prognosis of infantile hepatic hemangioendothelioma-arteriovenous fistula (IHHE-AVF) complicated with heart failure in neonates.Method:From May 2016 to June 2020, neonates with IHHE-AVF complicated with heart failure admitted were retrospectively studied. The clinical presentation, treatment and outcomes were analyzed.Result:A total of 11 cases of IHHE-AVF complicated with heart failure were enrolled (male 5, female 6). The onset age of heart failure was 12.0 (0.0, 17.0) d. 6 cases showed IHHE on fetal ultrasound. All patients had significantly enlarged heart on chest X-ray. All patients had decreased left ventricular systolic function and pulmonary hypertension on echocardiography. All patients required respiratory support and 6 of them were intubated. 3 cases received conservative treatment (all dead). 1 case received surgery (dead). 7 cases received interventional therapy at the age of (25.6±18.5) d. 1 case was dead, and the other 6 cases were improved and discharged. All the 6 cases were followed up to 3~18 months. None of them had heart failure again. The IHHE were shrunk or completely disappeared. Coagulation function and platelet count were normal.Conclusion:The fatality rate of neonatal-onset IHHE-AVF complicated with heart failure is extremely high. Interventional therapy may be more effective than conservative therapy and surgery.
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Objective:To explore the clinical characteristics and prognosis of children with inborn error of immunity (IEI) onset in the neonatal period.Methods:The clinical data of 21 cases of IEI neonates admitted to the Neonatal Center of Beijing Children′s Hospital were collected, and their clinical manifestations, peripheral blood test characteristics, genetic diagnosis, and primary disease during hospitalization were collected.The prognosis follow-up results were summarized and analyzed.Results:Twenty-one children with IEI were finally diagnosed by whole exome sequencing, including 15 cases of primary immunodeficiency(including 6 cases of chronic granulomatous disease, 3 cases of DiGeogre syndrome, 2 cases of Wiskott-Aldrich syndrome, 2 cases of severe combined immunodeficiency disease, 1 case of selective IgA deficiency, and 1 case of ectodermal dysplasia with immunodeficiency), 5 cases of infantile inflammatory bowel disease, and 1 case of familial haemophagocytic lymphohistiocytosis.Clinical manifestations of sepsis and colitis were the most common(accounting for 12/21), and 16/21 of the children had an increase in the absolute value of eosinophils(>0.5×10 9/L). Children received hematopoietic stem cell transplantation accounted for 7/21, and the median time of receiving transplantation was 11 months after birth.By the time of follow-up, the primary disease remission after hematopoietic stem cell transplantation accounted for 5/7.Among them, 2 cases were diagnosed with CGD associated inflammatory bowel disease before transplantation, and the primary disease resolved after hematopoietic stem cell transplantation.Of the 14 children who did not receive hematopoietic stem cell transplantation, 10 children died.Five of the 11 deaths were treated with systematic steroid before diagnosised. Conclusion:The clinical manifestations of IEI in the neonatal period are not specific.Sepsis and colitis are the most common manifestations.Most of the cases have elevated eosinophils in the peripheral blood.Systematic streoid therapy needs to be cautious, and timely evaluation for hematopoietic stem cells transplantation is an effective option to resolution.
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Objective:To analyze the diagnosis and treatment of neonatal neuroblastoma (NB) by summarizing its clinical characteristics.Methods:This study retrospectively recruited 14 neonates with NB in Beijing Children's Hospital (National Center for Children's Health) from February 1, 2015, to February 1, 2020. Medical records and follow-up data as of February 29, 2020, were collected, and clinical staging based on International Neuroblastoma Staging System, risk grouping based on American Childhood Oncology Group risk grouping system, diagnosis, treatment, and prognosis were analyzed. According to whether surgical treatment was performed in the neonatal period or not, these subjects were divided into surgical and non-surgical groups. A descriptive statistical analysis was used for data analysis.Results:(1) Neonates with NB accounted for 0.063% (14/22 006) of the total number of newborns admitted to the hospital during the same period. The 14 cases were all full-term aged 15 d (8 h-23 d) at admission. Tumors were found in seven cases in prenatal examinations, while others presented with shortness of breath (three cases), abdominal distension (two cases), fever (one case), and dysuria and difficult defecation after birth (one case). (2) The primary tumor sites included the adrenal gland (eight cases), posterior mediastinum (three cases), retroperitoneum (two cases), and sacrococcygeal (one case). Three cases had extensive diffuse liver metastasis at admission. (3) Except for two cases who refused to examine, the serum neuron-specific enolase of 12 cases was 57.2 ng/ml (35.9-158.3 ng/ml) during hospitalization, and the urine vanillyl-mandelic acid creatinine of four cases was 2 304.940 (685.748-9 595.314). (4) Primary tumor sites were found in 14 cases by imaging examination. Bone scanning was performed in three cases, including one with a concentrated shadow of the right sacroiliac joint and two with no abnormalities. Ten cases underwent bone marrow aspiration and all with normal results. (5) Of the ten neonates received surgery (the surgical group), nine had the primary tumor wholly removed, without chemotherapy after the operation, and the tumor-free survival period was 19 months (1-45 months). One case (case 5) had a substantial primary tumor that could not be completely resected. The patient underwent a second surgery five months after the first operation due to disease progression and received postoperative chemotherapy. The child had stopped chemotherapy for 24 months and survived without a tumor. (6) In the non-surgical group (cases 11 to 14), the tumor in case 11 who refused chemotherapy shrank spontaneously after discharge, and the patient survived for 20 months with the tumor. The parents of the case 12 withdrew treatment during hospitalization, while the primary tumor and metastases disappeared after discharge, and the specific tumor markers gradually decreased to normal levels. The patient has been tumor-free survived for 25 months. Case 13 received mediastinal tumor resection and chemotherapy during infancy. At the end of the follow-up, chemotherapy had been stopped for 12 months, and the patient survived without a tumor. Case 14 withdrew treatment and died. (7) Among the ten cases in the surgical group, one patient's pathological result indicated a composite tumor, while the others were low differentiated neuroblastoma. There was no MYCN gene amplification, 1p36 deletion, or 11q23 deletion in the ten cases. (8) Among the ten children in the surgical group, nine were in stage 1, and one was in stage 4S (case 5). Nine cases were classified into extremely low-risk groups, while the other was in the low-risk group. The four cases in the non-surgical group could not be grouped by risk. Conclusions:Clinical manifestations of neonatal NB are often atypical. NB in stage 4S might resolve spontaneously, and expectant observation may be considered. The overall prognosis of neonatal NB is generally good, but further researches are needed.
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Objective:To explore the MRI features of neonatal deep cerebral arterial infarctions.Methods:The medical and MRI datas of 23 neonates with deep cerebral arterial infarctions from January 2011 to December 2018 in Beijing Children′s Hospital, Capital Medical University were retrospectively analyzed. Both 11 males and 12 females with ages between 1-28 d were recruited and MRI were performed within 2-20 d after symptom onset. The MRI featurs including location, morphology, signal characteristics, enhancement features and other accompanied signs were reviewed.Results:A total of 15 cases with arterial infarction and 8 cases with complication of purulent meningitis were identified. The median age at presentation were 2 d and 7 d respectively. The unilateral involvement were demonstrated in all neonates with arterial infarctions. Among them, 11 had deep infarcts and the main branch of the middle cerebral artery was involved in 4 neonates. MRI showed slightly hypo-intensity on T 1WI and slightly hyper-intensity on T 2WI with indistinct boundary and focal punctate hyper-intensity on T 1WI and hypo-intensity on T 2WI. In 8 cases secondary to purulent meningitis, unilateral involvement was found in 5 cases and bilateral involvement in 3 cases. All cases showed hypo-intensity on T 1WI and hyper-intensity on T 2WI with indistinct boundary. Among them, 7 cases were heterogeneous, with small cystic changes which appeared as slightly hyper-intensity on T 1WI and slightly hypo-intensity on T 2WI peripherally, as well as nodular or patchy restricted diffusion. Marked swelling of the lesion was found in 6 cases. Multiple patchy or ring enhancement was revealed in 5 cases. Iso-intensity and restricted diffusion in posterior horn of the lateral ventricle were found in 2 cases. Restricted diffusion in frontotemporal subarachnoid space was found in 5 cases. One case showed subdural effusion. Conclusion:Neonatal deep cerebral arterial infarctions have certain characteristic appearance on MRI. Lesions secondary to purulent meningitis can be bilateral involvement with heterogeneous MRI intensities, and different period of infarction signs could be found concurrently. MRI is beneficial to the differential diagnosis.
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Objective:To summarize the clinical characteristics and treatment outcome of neonates with laryngopharyngeal congenital structural abnormalities in intensive care unit.Methods:The clinical data of neonates with congenital laryngopharyngeal structural abnormalities in the Neonatal Intensive Care Unit of the National Center of Beijing Children′s Hospital, Capital Medical University from January 2017 to December 2018 were retrospectively analyzed.The general data, birth status, disease types and clinical characteristics of abnormal laryngeal structure, complications, treatment and follow-up of some children with special diseases were summarized.These neonates were divided into the operation group and the conservative treatment group according to treatment methods, and then the outcomes of the two groups were compared.Results:A total of 133 cases of neonates with laryngopharyngeal congenital structural abnormalities were enrolled, including 73 cases(54.88%) with laryngomalacia, and 60 cases(45.12%) with special structural abnormalities.Of 60 cases with special structural abnormalities, 26 cases (19.54%) had pharynx and larynx cysts, 18 cases (13.53%) had vocal cord paralysis, 4 cases (3.00%) had laryngeal cleft, 2 cases (1.50%) had subglottic hemangioma, 3 cases (2.25%) had Pireer Robin, 1 case (0.75%) had laryngeal poof, 5 cases (3.75%) had pharynx softening, 1 case (0.75%) had subglottic stenosis.Nine patients had special structural abnormalities and laryngomalacia simultaneously.Fiber nasopharyngoscope and enhanced CT were main auxiliary examinations.Twenty-two(16.5%) cases received surgical treatment.Conclusions:Early diagnosis is needed for the neonates and abnormal laryngeal structure.The best treatment scheme should be evaluated according to the condition of the newborn.For some acute cases, early operation and multidisciplinary comprehensive treatment are warranted.
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Objective To investigate the clinical features,diagnosis and treatment of neck masses in newborns. Methods All cases of neck masses in newborns admitted to NICU of Beijing Children's Hospital form January 2016 to Febrary 2018 were included,and the clinical manifestations,examinations,treatments and outcomes were evaluated. Results Fourteen cases of newborn's neck masses were collected. The time of onset was 8 cases at birth,1 case earlier than 7 days,5 cases after 7 days. Seven cases were admitted with dyspnea,10 cases combined with neck infections. Neck ultrasound examinations were performed in all 14 cases,CT scan in 2 cases,MRI in 10 cases. Five cases were given endotracheal intubation after admission, among them 3 cases needed mechanical ventilation. Nasal continuous positive airway pressure was used in 3 cases. Thirteen cases received anti-infective treatment. Punctures were performed in 4 cases. Surgical resec-tions were taken in 6 cases. Two cases were diagnosed as local primary infection. Six cases were confirmed by surgery,including 4 cases of branchial cleft cyst,1 case of esophageal duplication and 1 case of lymphangio-ma. Conclusion The neck masses of the newborn is prone to upper airway obstruction. Part of them need endotracheal intubation to open the airway. And the infection can be combined. There is a certain rate of misdiagnosis before operation,and the treatment plan is different according to the nature of the mass.
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Objective To study the clinical features,diagnosis and treatment in the neonates of Kasabach-Merritt phenomenn (KMP).Method To analyze retrospectively the data collected from the 9 neonates of Kasabach-Merritt phenomenon in our neonatal intensive care unit from January 2016 to March 2017.The data of their clinical presentation,imaging findings,treatment and outcomes were analyzed.Result Among the 9 cases,there were 6 males and 3 females,with onset age of 0 ～ 14 d.The hemangiomas were located over the body surface of the neck,back,trunk and limbs in 4 cases.The others were located in the deep organs in 5 cases.2 cases were found hepatic hemangiomas by fetal ultrasound and 3 cases had symptoms of poor response,dyspnea and nasal bleeding with occult onset.All the 9 cases had thrombocytopenia,and the average platelet count was 24.0 × 109/L.The imaging findings showed the changes of hemangiomas,and among them,3 cases were associated with arteriovenous fistula.In one case,the biopsy pathology diagnosis was Kaposiform hemangioendothelioma.Among 8 cases which was treated,2 cases had systemic medication,2 cases had interventional embolization and 4 cases were treated with combined therapy (interventional embolization together with medication),including 1 case of definitive surgical treatment.Finally 7 cases improved,1 case with severe thrombocytopenia and coagulation disorders did not improve and treatment abandoned and 1 case died before treatment.Conclusion The symptomatology of KMP is various,which is related to the location of the lesion.The imaging studies are helpful to diagnosis.Massive hemorrhage and multiple organ dysfunction can be fatal.The treatments are different among cases,for most cases with proper treatment the prognosis are good.
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Objective To analyze the clinical and molecular features of community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) infection in neonates and to investigate their antibiotic resistance profiles.Methods A total of 35 invasive CA-MRSA strains were collected from six hospitals in 2014.Multilocus sequence typing (MLST), staphylococcal cassette chromosome mec (SCCmec) typing and spa typing were used to analyze these isolated CA-MRSA strains.In vitro antibiotic susceptibilities of those strains to 15 antibiotics were analyzed by using agar dilution method.Results Up to 88.6% patients were late-onset infection and septicemia (24, 68.5%) was the most common infection among the 35 cases.A total of 16 patients (45.7%) suffered from complications.Caesarean section and premature birth were risk factors for invasive CA-MRSA infection.ST59-MRSA-SCCmecⅣa-t437 (14, 40%) was the most predominant CA-MRSA clone, followed by ST59-MRSA-SCCmecⅤ-t437 (13, 37.1%).The incidence of severe complications caused by ST59-MRSA-SCCmecⅤ-t437 was higher than that caused by ST59-MRSA-SCCmecⅣa-t437 (P<0.05).Up to 85.7% of the isolated CA-MRSA strains were multidrug-resistant strains.Conclusion This study shows that neonatal invasive CA-MRSA infections mainly result in septicemia and are often accompanied by complications and involve multiple organs.Multidrug-resistant CA-MRSA strains are prevalent in neonates.ST59-MRSA-SCCmecⅣa-t437 is the predominant clone causing neonatal invasive CA-MRSA infection.
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Objective To investigate the clinical characteristics of neonatal tuberous sclerosis complex (TSC).Methods A total of 134 patients were admitted and diagnosed as TSC in Beijing Children′s Hospital,Capital Medical University from September 2006 to September 2015.The clinical characteristics of TSC in 8 patients who had clinical symptoms initiating from the neonatal period (skin lesion,neurologic abnormality,etc.) were analyzed,so as to conduct the auxiliary examinations (skin biopsy,ultrasonic cardiogram,cranial imaging) and the follow-up results.Results Among 8 patients,4 were diagnosed as TSC in the neonatal period while other 4 were diagnosed at later period from 4 months to 14 years old.Six patients had skin lesions in neonatal period,accounting for 75% of 8 patients,with predominant symptoms:hypomelanotic macules (5 cases),angiofibroma (2 cases),and shagreen patch (1 case).Cardiac abnormalities in neonatal period mainly included cardiac rhabdomyoma,which were characterized by high incidence,multi-regional occurrence,and wide distribution,noticed in 4 patients (50%).Ultrasonic cardiogram showed a mass in 4 patients with moderate-strong echo,uniform texture and clear boundary,and slight effect on tricuspid valve blood flows (in 1 case).Electrocardiogram abnormalities were found in 1 case,like atrial premature beats with intraventricular aberrant conduction,and accelerated atrial escape.Neurologic abnormality of neonatal TSC could present convulsive seizures.Cranial imaging lesions showed subependymal nodules (3 cases),leukodystrophy (2 cases),and giant-cell astrocytoma (1 case).Evidence for TSC2 gene positive and heterozygous mutation was identified in 1 case.The nucleic acid mutation site was at c.268C>T (E4),and the amino acid mutation was p.90Q>X.The mutant effect was nonsense mutation,which could lead to premature termination of protein translation.Conclusion The clinical characteristics of neonatal TSC may involve multi-system lesions,most commonly seen in the skin,cardiac,and ner-vous system.Neonatal physical examinations,cranial CT/magnetic resonance imaging and cardiac ultrasound screening should be done for the suspected TSC patients,and genetic diagnosis may contribute to the early diagnosis of the disease.
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Objective To investigate the cardiac structure and hypertrophic cardiomyopathy of infant of diabetic mother(IDM),to analyze of the outcome of hypertrophic cardiomyopathy in IDM.Methods Totally 23 cases of IDM admitted in NICU from Feb.2012 to Jan.2013 were selected as observation group,randomly selected from the same period with gestational age of diabetic mother baby 23 cases as control group.The interventricular septum thickness,left ventricular posterior wall thickness,aortic valve internal diameter,right ventricular diameter,ejection fraction were detected and compared between the 2 groups.And echocardiography was followed up in 3 months and 6 months old.Results In IDM group,interventricular septum thickness [(3.20 ± 0.28) mm],left ventricular posterior wall thickness [(3.40 ± 0.31) mm] were significantly higher than those in control group (all P ＜ 0.05).Ejection fraction [(54.00 ± 3.76) × 10-2] was significantly lower than that in control group(P ＜ 0.05).Follow-up after 3 months,6 months old,interventricular septum thickness,left ventricular posterior wall thickness and ejection fraction returned to normal.Four of the 23 cases(17.4％) had cardiac malformations.Conclusions IDM has high morbidity of congenital heart disease,echocardiography should be done to identify hypertrophic cardiomyopathy and congenital heart disease after birth.IDM combined with hypertrophic cardiomyopathy is a transient,benign process,prognosis is good.
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Objective To analyze of the risk factors for persistent pulmonary hypertension of newborn(PPHN) after resuscitation in neonatal asphyxia.Methods Total 92 cases of PPHN in neonatal asphyxia were admitted in NICU,Beijing Children's Hospital Affiliated to Capital Medical University from Jan.2009 to Dec.2013 as PPHN group.According to patients condition,to case-control study method were randomly selected 92 cases without PPHN in neonatal asphyxia in the same period as control group.Except for other serious complications with PPHN,such as respiratory distress syndrome,meconium inhalation,pulmonary hemorrhage,neonatal severe pulmonary infection and diaphragmatic hernia.Apgar score and rescue measures after asphyxia based on the newborn perinatal questionnaire and body temperature,blood sugar,arterial blood gas and echocardiography on admission were recorded.Results Respiratory treatment of PPHN group and control group were 71 cases (77.2％) vs 28 cases (30.4％),respectively.There was significant difference (x2 =6.380,P =0.012).On admission,mean arterial pressure [(32.36 ± 11.52) mmHg],temperature [(34.3 ±0.28) ℃],blood sugar [(2.56 ±0.77) mmol/L] and arterial blood pH value (7.16 ±0.21) in PPHN group were lower than those of the control group [(38.55 ± 9.18) mmHg,(36.5 ± 0.71) ℃,(3.46 ± 0.53) mmol/L,7.21 ±0.14].For mild and severe asphyxia cases in the PPHN group,blood gas and pulmonary hypertension had no statistical difference after rescucitation in the delivery hospital.Conclusions Acidosis,hypothermia,low blood pressure and hypoglycaemia after resuscitation in neonatal asphyxia are major risk factors for genesis of PPHN.This research shows that rescue after asphyxia timely,early and respiratory support effectively,monitoring closely,treatment of hypoglycemia and hypothermia,correct acidosis and maintain blood pressure can play a positive role in decreasing the morbidity of PPHN in neonatal asphyxia.
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Objective To discuss the relationship between the clinical pathogenesis and progression in neonatal pulmonary hypertension in different stages.Methods Total 169 cases of pulmonary hypertension were admitted in our NICU from June 2006 to May 2012,all the cases were involved in this retrospective study.They were divided into two groups in chronological order:early group 79 cases (from June 2006 to May 2009) and late group 90 cases (from June 2009 to May 2012).The data records include gender,gestational age,protopathy,echocardiography examination results.Furthermore,the cause and development of neonatal pulmonary hypertension were analyzed in different stages.Results The admission time in the early group was later than the late group [(2.15 ± 1.2) d vs (1.41 ±0.70) d].Meconium aspiration syndrome in the early group were 25 cases (31.6％) and the late group were 14 cases (15.6％).Other complications,such as congenital diaphragmatic hernia,neonatal respiratory distress syndrome,aspiration pneumonia,wet lung/sepsis,neonatal asphyxia,neonatal infectious pneumonia had no difference between the two groups(P ＞0.05).Neonatal pulmonary hypertension in two groups had no statistical difference between term and post term patients,but premature in the early group (11 cases,13.9％) were less than the late group (23 cases,25.6％).Echocardiography was recorded after admission,mild and moderate pulmonary hypertension had no statistical differences in the two groups (P ＞ 0.05).The severe pulmonary hypertension cases in the early group were more than those in the late group (26 cases vs 17 cases).Conclusion Along with the improvement of perinatal monitoring and resuscitation technology in different stages,the cases of meconium aspiration syndrome with pulmonary hypertension are fallen down and the patients transferred to the superior hospital decreased.The amount of pulmonary hypertension in preterm infants increases,and the admission time of patients with neonatal pulmonary hypertension are shortened.The amount of severe pulmonary hypertension are less than those in the early time.It plays a positive role in best approach to improving treatment and outcomes.
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Objective To discuss the clinical features of neonatal shock retrospectively.Methods Totally 144 neonates were enrolled in this retrospective study from Sep.2006 to Sep.2012 in NICU of Beijing Children's Hospital.Clinical data were collected from our database,including the primary disease,manifestation,laboratory findings,treatment and prognosis.Results According to the severity of shock,28 cases were mild,85 were moderate,31 were severe.Regarding to the type of shock,71 cases were cardiogenic shock,22 cases were hypovolemic shock,51 cases were septic shock.About 69.4％ patients were cured,only 9 cases died.Eight of them died of septic shock.Totally 33 cases in pH ＜7.15 group,2 of them died.In pH≥7.15 group,111 cases were involved,7 of them died.There was no significant difference of mortality between two groups (P =0.959).Conclusion Neonatal shock mostly occurred within 3 days after birth,and cardiogenic shock were dominating.Septic shock increased with age,and were responsible for death.
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ObjectiveTo investigate the incidence and risk factors in retinopathy of prematurity (ROP) at matched gestational age.Methods Data collected by the medical records of neonates from November 2007 to December 2010 in our neonatal database were analyzed.Patients'information was recorded from birth to discharge from NICU.Data included age after birth (hours),gestational age,body weight at birth,treatment,and maternal demographics.The statistical study was carried out by SPSS version 13.0 software.Mann - Whitney U test was used for numerate data.Fisher's exact probability test and Pearson's chi - square test were used to compare quantitative variables between independent groups.P values were considered significant when they were less than 0.05 ( two - sided).ResultsThe incidence of ROP was 5.38％.Gestational age of ROP group and control group was matched ( P =0.387 ),but difference in body weight at birth was significant ( P =0.045 ).Age after birth was significantly different ( P =0.013 ).Apnea and anemia were significantly different between two groups (P ＜ 0.01). The differences in duration of hospitalization,NCPAP and use of antibiotics between two groups were significant ( P ＜ 0.002,0.000 and 0.000,respectively).ROP group differed from control group greatly in hospitalization expenses.Multiple stepwise logistic regression analysis showed that oxygen supplenentation ( P =0.0237 ) and infection ( P =0.0118) were risk factors of ROP. ConclusionsThe incidence of ROP in NICU in Beijing Children's Hospital was 5.38％.Inhalation of oxygen supplementation and infection were risk factors of ROP.