Clinical analysis of 14 cases of pseudohypoparathyroidism in children / 国际儿科学杂志

Objective To analyze the clinical characteristics and treatment of pseudohypoparathyroidism (PHP) in children and to increase clinicians'understanding of this disorder.Methods From January 2008 to November 2018,a total of 14 PHP patients at our hospital were retrospectively analyzed,as regarding the clinical manifestation,laboratory examination and imaging data.The patients were further divided into two groups according to onset age:group A (under 1 year) and group B (over or equal to 1 year).The laboratory findings were compared between the two groups.Results Of the 14 patients (10 boys and 4 girls),the median age of onset was 12 years (range:1 day to 16 years).Epilepsy is one of the most common manifestations of PHP.Laboratory findings include hypocalcemia (1.06-2.18mmol/L),hyperphosphatemia (2.1-3.5 mmol/L),and elevated concentrations of PTH (105.6-733.0 pg/ml).PTH levels were significantly lower in the group A than in group B (Z =2.07,P =0.042).Five patients were found to have calcifications in bilateral basal ganglia and cerebral parenchyma by head CT.All the patients were improved after treatment,which included calcium gluconate injection to control tetany,calcium supplement combined with vitamin D and/or calcitriol orally.Conclusion For children who presented frequent hypocalcemia tetany and calcifications in head CT,serum calcium,phosphorus,PTH levels should be further investigated to make a correct diagnosis.Calcium supplement combined with vitamin D or its metabolites is an effective therapy for PHP children.

Clinical analysis of 14 cases of pseudohypoparathyroidism in children / 国际儿科学杂志

Objective@#To analyze the clinical characteristics and treatment of pseudohypoparathyroidism(PHP) in children and to increase clinicians′understanding of this disorder.@*Methods@#From January 2008 to November 2018, a total of 14 PHP patients at our hospital were retrospectively analyzed, as regarding the clinical manifestation, laboratory examination and imaging data.The patients were further divided into two groups according to onset age: group A(under 1 year) and group B(over or equal to 1 year). The laboratory findings were compared between the two groups.@*Results@#Of the 14 patients(10 boys and 4 girls), the median age of onset was 12 years(range: 1 day to 16 years). Epilepsy is one of the most common manifestations of PHP.Laboratory findings include hypocalcemia(1.06-2.18mmol/L), hyperphosphatemia(2.1-3.5 mmol/L), and elevated concentrations of PTH(105.6-733.0 pg/ml). PTH levels were significantly lower in the group A than in group B(Z=2.07, P=0.042). Five patients were found to have calcifications in bilateral basal ganglia and cerebral parenchyma by head CT.All the patients were improved after treatment, which included calcium gluconate injection to control tetany, calcium supplement combined with vitamin D and/or calcitriol orally.@*Conclusion@#For children who presented frequent hypocalcemia tetany and calcifications in head CT, serum calcium, phosphorus, PTH levels should be further investigated to make a correct diagnosis.Calcium supplement combined with vitamin D or its metabolites is an effective therapy for PHP children.

Risk factors of postoperative acute pulmonary reperfusion injury in neonatal severe pulmonary stenosis or pulmonary atresia / 中国新生儿科杂志

Objective To study the clinical features and risk factors of acute pulmonary reperfusion injury after operation in neonates with severe pulmonary stenosis or pulmonary atresia.Method From February 2014 to February 2018,a retrospective analysis was performed in patients with critical pulmonary stenosis or pulmonary atresia who received percutaneous balloon pulmonary valvuloplasty (PBPV) in the neonatal intensive care unit of our hospital.Clinical characteristics,perioperative cardiac structure,hemodynamic data and biochemical results were collected.The neonates were assigned into injury group if they had acute lung reperfusion injury,and non-injury group if not.The risk factors of acute lung reperfusion injury were analyzed using multi-variate Logistic regression model.Result A total of 32 patients (24 prenatal diagnosis and 8 postnatal diagnosis) with severe pulmonary stenosis or pulmonary atresia with intact ventricular septum were enrolled.The main manifestations were dyspnea and cyanosis.Intravenous prostaglandin E was administered to keep the ductus arteriosus open.The age of operation ranged from 1 to 52 days and the median age was 7.5 days.Postoperative acute lung reperfusion injury occurred in 7 cases (21.9％).Preoperative and intraoperative pulmonary valve annulus diameter,balloon diameter,preoperative hemoglobin,hematocrit and blood albumin were significantly lower in the injury group.The operation duration,total length of hospital stay and postoperative duration were longer than in the non-injury group,the differences were statistically significant (P ＜ 0.05).Multi-variate Logistic regression analysis showed that the diameter of pulmonary valve annulus (OR =5.814,95％CI 1.106 ～30.568),preoperative blood albumin (OR =1.361,95％ CI 1.063 ～ 1.742),and hematocrit (OR =1.173,95％ CI 1.010 ～ 1.363) were risk factors of acute lung reperfusion injury,with statistically significant differences (P ＜ 0.05).Conclusion Acute lung reperfusion injury is one of the common complications after the operation of severe pulmonary stenosis or pulmonary atresia.The severity of pulmonary valve annulus stenosis,preoperative hematocrit and blood albumin level may be the risk factors of postoperative acute lung reperfusion injury.

Clinical characteristics and genetic analysis of neonatal asymmetric crying facies / 中国新生儿科杂志

Objective To study the clinical and genetic characteristics of neonatal asymmetric crying facies (ACF).Method From January 2007 to December 2016,clinical data were retrospectivelyreviewed in patients with ACF admitted to Neonatal Unit of Xinhua Hospital.The data included maternalpregnancy and delivery history,clinical manifestation,results of chromosome florescent in situ hybridization(FISH) and gene chip test.Result Among 32 patients with classic ACF,8 were female and 24 weremale.5 cases were one of the twins or muhiplets.16 patients were born from mothers with history ofspontaneous abortion or induced abortion.16 patients presented with ACF on the left side and 16 patients onthe right side.7 patients had single ACF malformation,7 with malformation of another organ,and18 patients with malformation of more than two other organs.Combined malformation included earmalformation in 11 cases,facial malformation in 6 cases,cardiovascular malformation in 19 cases,urinarytract malformation in 3 cases,digestive system malformation in 3 cases,abnormal nervous system image in7 cases,and immune/endocrine system abnormality in 3 cases.10 patients received genetic analysis withFISH and/or gene chip tests and 4 patients had positive results.Among the patients who completed geneticanalysis,8 patients received FISH test and 2 patients had 22q11.2 distal deletion.1 patient hadheterozygous deletion in 22q21 region using gene chip test after he got a negative result with FISH.Amongthe 32 cases,6 patients died until now,among them,5 patients had multi-organ malformation.ConclusionACF is a tiny facial deformity,however it is often associated with other congenital malformations.Earlygenetic detection and systematic multiple malformations screening are particularly important for diagnosis andprediction of prognosis.

Comparative study on the clinical features of purulent meningitis in preterm and term infants: a clinical analysis based on 10 years / 中国新生儿科杂志

Objective To study the differences of clinical manifestations,etiology and hospitalized outcomes of purulent meningitis in preterm and term infants.Method All preterm and term infants with purulent meningitis hospitalized in the Hospital from 2006 to 2015 were enrolled in this study.The data of neonate's condition,maternal condition,clinical manifestations,complications,etiology,treatment and outcomes of the preterm and term infants groups were compared.Result During the study period,44 preterm infants and 118 term infants were included.The time of onset for purulent meningitis of preterm infants group was statistically earlier than that of term infants group [11.2 (3.2,19.8) d vs.14.3 (5.6,23.9) d,P ＜ 0.05].The prognosis of preterm infants group was statistically worse than that of term infants group (P ＜ 0.05).Among them,the incidences of clinical manifestations in preterm infants group compared to term infants group were:fever (54.5％ vs.78.8％),seizure (11.4％ vs.26.3％),lethargy and poor response (59.1％ vs.38.1％),slow weight gain (9.1％ vs.0％),apnea (45.5％ vs.0.8％) and cyanosis (15.9％ vs.4.2％);all the differences between two groups were significant (P ＜ 0.05).The time of onset for purulent meningitis with complications was statistically earlier than those without complications [9.5 (4.1,20.5) d vs.13.8 (5.9,22.0) d,P＜0.05].The duration of treatment for purulent meningitis with complications was longer than that without complications [(42.2 ± 8.8) d vs.(28.7 ± 7.1) d,P ＜ 0.05],and the positive rate of pathogens was also statistically higher than those without complications (73.8％ vs.26.7％,P ＜0.05).Coagulase-negative staphylococcus was the main pathogen for both preterm and term infants group.Klebsiella pneumoniae was more common in preterm infants group than in term infants group (40.0％ vs.10.4％,P ＜0.05).Conclusion Preterm infants with purulent meningitis had early onset time,atypical clinical manifestations,and poor prognosis.The treatment course for purulent meningitis with complications is prolonged.The pathogens for neonatal purulent meningitis have already changed.The detection rate of conditional pathogens is increasing yearly,for which the clinicians should take note seriously.

Nosocomial infection investigation and risk factors in neonatal intensive care unit / 国际儿科学杂志

Objective To study the occurrence of nosocomial infection ( NI ) situation in the neonatal intensive care unit ( NICU ) of Xinhua Hospital affiliated to Shanghai Jiaotong University School of Medicine from January 2012 to October 2013,and analyze risk factors of NI. Methods A retrospective study was per-formed in the NICU,of which all neonates hospitalized over 48 hours were included and the occurrence situation and risk factors of NI were analyzed. Results This study included 1 357 neonates,and 175 neonates developed 202 times NI. The overall incidence of NI was 14. 89% (202/1 357),and the incidence density was 10. 44 per 1 000 NICU patient-days (202/19 355). The lower the birth weight is,or the smaller gestational age is,the higher the NI rate is. The NI rate was 75. 00% in neonates whose birth weight <1 000 g,and the NI rate was 52. 63%in neonates whose gestational age <30 weeks. The most common infection type was pneumonia[43. 07% (87/202)]and sepsis[26. 73% (54/202)]. The hospital stay of the NI neonates was longer and the cost of hospitali-zation is higher than non-NI neonates ( Median stay:20 days and 10 days. Median cost:21 045. 32 yuan and 8 108. 23 yuan)(both P<0. 01). Univariate analysis showed there were significant differences between NI neo-nates and non-NI neonates on gestational age,birth weight,5 min Apgar score,asphyxia rescue history,previons antibiotics use,mechanical ventilation,peripherelly inserted central catheter( PICC) ,closed thoracic drainage tube and chest or abdominal surgery before NI. The logistic regression analysis indicated that previous antibiotics use, mechanical ventilation and PICC were important risk factors for NI. Conclusion The NI rate in NICU is still high. Previous antibiotics use,mechanical ventilation and central venous catheter are risk factors of NI.

Clinical analysis of apnea after operation for retinopathy of prematurity / 上海交通大学学报（医学版）

Objective To retrospectively explore the incidence and causes of apnea after operation for retinopathy of prematurity(ROP). Methods The clinical data of 17 premature infants with operation for ROP(ROP group)and the other 23 premature infants without ROP(control group)were collected.The occurrence of apnea(time of onset and remission and original diseases) was recorded after operation in ROP group and at adjusted 37 weeks of gestational age in control group.Follow-up was conducted for 4 weeks,and the prevalences of apnea were compared between these two groups.Results There was significant difference in prevalenees of apnea between ROP group and control group ( 52.9%vs 21.7%,P<0.05).In ROP group,time of occurrence of apnea was (38±40)h after operation,and time of remission was (126±145)h after onset.Among the infants with apnea,there were 3 cases of pneumonia(33.3%),1 case of hypoglycemia(11.1%)and 5 cases with unexplained causes(55.5%). Conclusion Infants after operation for ROP are more prone to apnea,and pediatricians and ophthalmologists are required to collaborate in the perioperative care.