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Congenital hypertrophic pyloric stenosis-a rare presentation of a common entity 先天性肥厚性幽门狭窄是一种罕见的常见疾病
Pub Date : 2018-09-11 DOI: 10.15406/JPNC.2018.08.00341
Kailas Bh, Arkar
A newborn male baby was referred to our institute on day 2 of life with history of persistent projectile non-bilious vomiting since birth. The baby was full term and was born by normal vaginal delivery. There was no history suggestive of pyloric stenosis in the family. Nor was there any maternal exposure to macrolides or smoking. The possibility of mal rotation was considered. We investigated the neonate with contrast study and ultrasound which were consistent with hypertrophic pyloric stenosis. The muscle thickness was 5mm and the pyloric length was 14mm. Upper gastro-intestinal contrast study demonstrated a dilated stomach with string sign and shouldering of the pyloric muscle. The blood gas showed severe alkalosis. We did not perform gastrin levels. He was operated on day 3 of life after correction of electrolytes and alkalosis. Open Ramstad’s pyloromyotomy was performed. Postoperative recovery was uneventful. Feeds were started after 24 hours and the neonate was
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引用次数: 0
ADHD and sleep history taking at a resident staffed, university affiliated, community health clinic ADHD和睡眠史在一家有住院人员的大学附属社区健康诊所进行记录
Pub Date : 2018-08-31 DOI: 10.15406/JPNC.2018.08.00338
S. Rainey
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引用次数: 0
ADHD and sleep history taking at a resident staffed, university affiliated, community health clinic ADHD和睡眠史在一家有住院人员的大学附属社区健康诊所进行记录
Pub Date : 2018-08-31 DOI: 10.15406/jpnc.2018.8.00338
Shane C Rainey Do, Natasha BraunBS, Mohannad Mannaa
Attention deficit hyperactivity disorder (ADHD) is a disorder manifesting predominantly in childhood with symptoms of hyperactivity, inattention, and impulsivity which can have a dramatic effect on the child’s behavioral, social, and emotional functionality.1 As many as 8 to 11% of school aged children are diagnosed with ADHD, and billions of dollars have been dedicated to their care, both pharmacologic and psychological.2 The pathogenesis of ADHD is incompletely understood. A genetically inherited imbalance in catecholamine metabolism is the most commonly accepted hypothesis given the available animal studies, functional brain imaging, and patients’ efficacious response to stimulant medications.3 However, in recent years, a number of researchers have proposed a link between ADHD symptoms and sleep disorders.4–8 In a recent review in Pediatrics, Bonuck et al.,6 found that those with sleep disordered breathing were significantly more likely to have behaviors resembling ADHD than those without sleep disordered breathing.6 Obstructive sleep apnea (OSA) is a sleep disorder commonly affecting children and can have significant neuro developmental implications on the child’s health.7,8 American Academy of Pediatrics clinical practice guidelines recommend that patients or their caregivers be screened for snoring (as well as a focused evaluation for other symptoms if noted to have habitual snoring) at each routine health examination, and if positive, be referred to a sleep specialist.9 Despite these recommendations, screening for snoring is widely variable and many children at risk for OSA are not being screened according to the guidelines.10 Previous studies have reported screening proportions ranging from 8% to 24%; however, few studies have examined practitioners’ screening patterns for OSA when considering the diagnosis of ADHD.11,12 The aims of this study were to describe the snoring screening practices of various clinicians when considering the diagnosis of ADHD and to analyze various patient and provider characteristics to determine their predictiveness for screening about snoring. We hypothesized that patients were not being routinely screened for OSA risk factors prior to being diagnosed with ADHD.
注意力缺陷多动障碍(ADHD)是一种主要表现在儿童时期的障碍,有多动、注意力不集中和冲动的症状,这些症状会对儿童的行为、社交和情绪功能产生巨大影响。1多达8%至11%的学龄儿童被诊断为ADHD,数十亿美元用于他们的护理,药物学和心理学。2多动症的发病机制尚不完全清楚。鉴于现有的动物研究、大脑功能成像和患者对兴奋剂的有效反应,儿茶酚胺代谢的遗传失衡是最普遍接受的假设。3然而,近年来,许多研究人员提出了多动症症状与睡眠障碍之间的联系。4-8在《儿科学》最近的一篇综述中,Bonuck等人。,6研究发现,与没有睡眠呼吸障碍的人相比,睡眠呼吸障碍者更容易出现类似多动症的行为。6阻塞性睡眠呼吸暂停(OSA)是一种常见于儿童的睡眠障碍,可能对儿童的健康产生重大的神经发育影响。7,8美国儿科学会临床实践指南建议在每次例行健康检查中,对患者或其护理人员进行打鼾筛查(如果发现有习惯性打鼾,还应重点评估其他症状),如果结果呈阳性,则应转诊给睡眠专家。9尽管有这些建议,打鼾的筛查变化很大,许多有OSA风险的儿童没有根据指南进行筛查。10先前的研究报告称,筛查比例在8%至24%之间;然而,很少有研究在考虑诊断多动症时检查从业者的OSA筛查模式。11,12本研究的目的是描述不同临床医生在考虑诊断ADHD时的打鼾筛查实践,并分析各种患者和提供者的特征,以确定他们对打鼾筛查的预测性。我们假设患者在被诊断为多动症之前没有进行OSA风险因素的常规筛查。
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引用次数: 0
Comparison of feeding intolerance between very preterm and moderate preterm neonates – a prospective cohort study 极早产儿和中度早产儿喂养不耐受的比较——一项前瞻性队列研究
Pub Date : 2018-08-31 DOI: 10.15406/jpnc.2018.08.00339
F. Ahammad, T. Begum, JesminAkter, Evana Nasrin
Neonatal death is high in our country and it comprises 60% of total Under-5 mortality.1 So to achieve MDG-4 we have to reduce neonatal death. The Lancet series on neonatal health reported that preterm birth directly causes 28% of neonatal deaths.2 Prematurity and its complication is the major cause of neonatal death in our country and shares around 45% of neonatal death. Premature babies are prone to develop many complications. One of the common complications is Feeding Intolerance (FI). Feeding intolerance can be attributed to the immaturity of gastrointestinal motility,3, 4 as small intestinal motility and phase-3 activity of the migrating motor complex (MMC) are more immature in preterm infants, especially those with a gestational age of less than 32 weeks.3,5 Gastrointestinal motility is influenced by motilin, a 22 amino-acid peptide produced by the enterochromaffin cells of the duodenal and jejunal mucosa.6 Gastric emptying, in particular, is dependent on co-ordination between the motor activities of the gastric antrum and duodenum. Preterm infants often have difficulty in tolerating oral feeds due to immaturity of mechanical and hormonal control of their gastrointestinal system.3 Feed tolerance requires co-ordinated caudal intestinal transit of food. The clusters of phase 3 migrating motor complexes (MMC) that propagate food are associated with a twoto fourfold increase in plasma motilin levels.7 But the association between motilin, MMCs and feed tolerance, however, is unclear in preterm infants. Even though the numbers of MMCs are reduced in preterm infants of less than 32 wk gestation8 fetuses by week 20 of gestation, demonstrate intestinal distribution of motilin similar to that in adults. Motilin levels of fasting preterm infants are also similar to those of term infants, who rarely exhibit the degrees of feeding intolerance characteristic of premature infants.5 It is also noted that fetal intestine is structurally mature by 25 weeks of gestation and capable of digesting and absorbing milk feeds, motor activity develops more slowly and may limit the tolerance to enteral feeds.9 FI often needs prolong parenteral nutrition which predisposes nosocomial infections, hepatic dysfunction and prolong hospitalization.10,11 Though FI is a common problem, literature contain little information about the influence of prematurity on feeding intolerance. There is also scarcity of information regarding the prevalence of feeding intolerance among preterm babiesand whereas the more preterm babies develop more frequent feeding intolerance yet to be determined.
新生儿死亡在我国很高,占5岁以下儿童总死亡率的60%。1因此,要实现MDG-4,我们必须减少新生儿死亡。《柳叶刀》新生儿健康系列报道称,早产直接导致28%的新生儿死亡。2早产及其并发症是我国新生儿死亡的主要原因,约占新生儿死亡的45%。早产儿容易出现许多并发症。常见的并发症之一是喂养不耐受(FI)。喂养不耐受可归因于胃肠道运动不成熟,3,4因为早产儿的小肠运动和迁移运动复合体(MMC)的3期活动更不成熟,尤其是胎龄小于32周的早产儿。3,5胃肠道运动受胃动素的影响,十二指肠和空肠粘膜的肠嗜铬细胞产生的一种22氨基酸的肽。6胃排空尤其依赖于胃窦和十二指肠运动活动之间的协调。由于胃肠系统的机械和激素控制不成熟,早产儿通常难以耐受口服饲料。3饲料耐受需要食物的尾肠道协调运输。传播食物的3期迁移性运动复合体(MMC)集群与血浆胃动素水平增加2至4倍有关。7但早产儿胃动素、MMC与饲料耐受性之间的关系尚不清楚。尽管妊娠期小于32周的早产儿8的MMCs数量在妊娠20周时减少,但胃动素的肠道分布与成人相似。禁食早产儿的胃动素水平也与足月儿相似,足月儿很少表现出早产儿特有的进食不耐受程度。5还应注意的是,胎儿肠道在妊娠25周时结构成熟,能够消化和吸收乳汁,运动活动发展得更慢,可能会限制对肠内喂养的耐受性。9 FI通常需要延长肠外营养,这会导致医院感染、肝功能障碍和延长住院时间。10,11尽管FI是一个常见问题,但文献中几乎没有关于早产对喂养不耐受的影响的信息。关于早产儿喂养不耐受的患病率,也缺乏信息,而早产儿越频繁地出现喂养不耐受,尚待确定。
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引用次数: 6
Parent opinion in medical care for congenital diaphragmatic hernia 家长对先天性膈疝医疗护理的意见
Pub Date : 2018-08-22 DOI: 10.21767/2471-805x-c3-010
D. Ireland
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引用次数: 0
Recurring E. coli meningitis in an infant 婴儿复发性大肠杆菌脑膜炎
Pub Date : 2018-08-20 DOI: 10.15406/JPNC.2018.08.00337
S. Villar, F. T. Piedra, Robert Cilveti Portillo, J. Coromina, R. G. Puig
Despite advances in diagnostic approach, antibiotic therapy and intensive care, bacterial meningitis is still associated with a significant mortality and a high number of complications and neurological sequelae. Although the vast majority are single episodes, approximately 1-4.8% of all cases of acute bacterial meningitis are recurrent, which is defined as the reappearance of signs and symptoms of meningitis by the same microorganism or a new episode by a different microorganism that occurs at least 3 weeks after the sterilization of the cerebrospinal fluid (CSF). Different factors predisposing to recurrent meningitis have been identified, such as deficits in humoral and cellular immunity and congenital or acquired structural defects that establish an anatomical communication between the subarachnoid space and the skin or the middle ear and the paranasal cavities. The clinical history and the findings of the physical examination and the microbiological data can help guide the origin of meningitis. A prompt identification of immunological deficiencies or anatomical defects is important in preventing new episodes and potential sequelae.
尽管在诊断方法、抗生素治疗和重症监护方面取得了进展,但细菌性脑膜炎仍与显著的死亡率、大量并发症和神经后遗症有关。尽管绝大多数是单次发作,但在所有急性细菌性脑膜炎病例中,约有1-4.8%是复发性的,这被定义为在脑脊液(CSF)灭菌后至少3周,由同一微生物再次出现脑膜炎的体征和症状,或由不同微生物再次出现新的发作。已经确定了易患复发性脑膜炎的不同因素,如体液和细胞免疫缺陷以及先天性或后天性结构缺陷,这些缺陷在蛛网膜下腔与皮肤或中耳与鼻腔之间建立了解剖联系。临床病史、体检结果和微生物学数据有助于指导脑膜炎的起源。及时发现免疫缺陷或解剖缺陷对于预防新的发作和潜在的后遗症很重要。
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引用次数: 0
Recurrent anemia in an infant with pneumonia: be vigilant for uncommon presentation of cystic fibrosis 肺炎婴儿的复发性贫血:警惕罕见的囊性纤维化
Pub Date : 2018-08-16 DOI: 10.15406/JPNC.2018.08.00336
M. Ramzan, Mohammed Lukman, S. Sharma, S. Katewa
Cystic fibrosis (CF) is an autosomal recessive multisystem disorder characterized by the formation of thick, sticky mucus that can hamper our various organs.1 CF is caused by the alteration of a gene located on the long arm of chromosome7 that encodes a protein, the cystic fibrosis trans membrane conductance regulator (CFTR), which functions as a chloride channel on the apical membrane of epithelial cells.2 The disorder’s common symptomatology is due to progressive damage to the respiratory system, digestive system, exocrine pancreatic insufficiency (diarrhea and failure to thrive), though their severity varies among affected individual.3 Those infant who are not having classical clinical manifestations suggestive of CF or uncommon presentation, neonatal screening or early suspicion is helpful in detection of disease which allows prompt treatment of CF related complications, improving survival and conceptualizing the treatment strategies.4 Meconium ileus may be the first manifestation of CF in the neonatal period, occurring in approximately 20% of patients with pancreatic insufficiency.5 Though iron deficiency anemia may be present due to malabsorption in children,6 transfusion dependent severe anemia in early infancy associated with CF is not mentioned in literature. Our objective of the present publication is to report the case of an infant who had uncommon presentation of CF with recurrent anemia and pneumonia without full blown evolution of the disease despite the clinical suspicion of CF.
囊性纤维化(CF)是一种常染色体隐性遗传的多系统疾病,其特征是形成粘稠的粘液,会阻碍我们的各个器官。1 CF是由位于染色体长臂7的一个基因改变引起的,该基因编码一种蛋白质,即囊性纤维化跨膜电导调节因子(CFTR),该疾病的常见症状是由于呼吸系统、消化系统的进行性损伤、胰腺外分泌功能不全(腹泻和发育不良),尽管其严重程度因受影响个体而异。3那些没有提示CF的经典临床表现或不常见表现的婴儿,新生儿筛查或早期怀疑有助于检测疾病,从而及时治疗CF相关并发症,提高生存率并制定治疗策略。4胎粪性肠梗阻可能是CF在新生儿期的第一种表现,约20%的胰腺功能不全患者会出现这种情况。5尽管儿童吸收不良可能导致缺铁性贫血,6文献中未提及婴儿早期与CF相关的输血依赖性严重贫血。我们本出版物的目的是报告一例婴儿,尽管临床上怀疑患有CF,但他患有罕见的CF,并伴有复发性贫血和肺炎,但疾病没有完全发展。
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引用次数: 0
Recurrent respiratory tract infections in children; beyond medical causes! 儿童反复呼吸道感染;超越医学原因!
Pub Date : 2018-08-13 DOI: 10.15406/JPNC.2018.08.00335
Kashish Khanna, Shilpa Sharma, D. Gupta
required ventilator support. A paediatric surgery referral was sent in view of history of repeated admissions for severe recurrent RTI every 3-4 weeks since birth. A flexible endoscopic examination revealed a fistulous opening in the posterior wall of the trachea raising the suspicion of an H type trachea-oesophageal (TEF) fistula. On deeper probing, the mother gave a history of choking during feeds. After proper stabilization rigid bronchoscope under general anaesthesia revealed a fistula in the posterior wall of trachea which was first cannulated, and then ligated and repaired from the right cervical route (Figure 1). The child was discharged after 10 days and was followedup for the next 10 months. He had no other episode of RTI which required admission.
所需的呼吸机支架。鉴于出生后每3-4周因严重复发性RTI而反复入院的历史,我们发送了儿科手术转诊。一项灵活的内窥镜检查显示,气管后壁有一个瘘管开口,这使人怀疑是H型气管食道瘘。在更深入的调查中,这位母亲给出了喂食时窒息的病史。在全身麻醉下进行适当的稳定后,硬支气管镜检查发现气管后壁有瘘,首先插管,然后结扎并从右侧颈路修复(图1)。孩子在10天后出院,并随访了接下来的10个月。他没有其他需要入院的RTI发作。
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引用次数: 0
Renal involvement in epidermolysis bullosa patients: a case series study 大疱性表皮松解症患者肾脏受累:一个病例系列研究
Pub Date : 2018-07-25 DOI: 10.15406/jpnc.2018.08.00334
F. Cavagnaro, M. J. Yubero, I. Fuentes, F. Palisson
specifically looking at renal dysfunction (urinary nitrogen/plasmatic creatinine, serum amyloid A and electrolytes in plasma), hematuria (urinalysis) and proteinuria (micro albuminuria). When a urological cause is discarded, a renal biopsy is highly advice to obtain a specific diagnosis, and eventually its, treatment and prognosis. The biopsy is afterwards sent for histopathology analysis, which includes light microscopy, immune fluorescence and electron microscopy.
特别关注肾功能障碍(尿氮/血浆肌酐、血清淀粉样蛋白A和血浆电解质)、血尿(尿液分析)和蛋白尿(微量蛋白尿)。当放弃泌尿系统病因时,强烈建议进行肾活检,以获得特定的诊断,并最终进行治疗和预后。活检后进行组织病理学分析,包括光学显微镜、免疫荧光和电子显微镜。
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引用次数: 1
Dental concerns of children with lip cleft and palate - a review 唇腭裂儿童的牙科问题综述
Pub Date : 2018-07-19 DOI: 10.15406/JPNC.2018.08.00333
S. Nirmala, Degala Saikrishna
These are the most severe of congenital anomalies which affect the mouth and related structures. There are one of the most common congenital defects and occur about once in 1000 births. 1 There is a family history in only about one third of cases. Clefts of the palate only are more common in girls while clefts of the lip, with or without palatal involvement, are more common in boys. It is interesting to know that the left side is more often than the right.2 The exact cause of clefting is unknown in most cases. For most cleft conditions, no single factor can be identified as the cause. However, it is important to distinguish between isolated clefts (in which the patient has no other related health problems) and clefts associated with other birth disorders or syndromes.3,4 In the absence of the family history the occurrence of a congenital abnormality may be due to the action of mutation or some chance occurrence during the pregnancy. The lips and palate develop during the 5 -8 weeks of intrauterine life and any factor disrupting the heir formation must exert its influence during this relatively short period. In this respect it is difficult to offer positive views on factors related to maternal health during the pregnancy. it is accepted that German measles and x-ray examination during early pregnancy may produce congenital abnormalities and are to be avoided. The teratogenic action of certain drugs, particularly thalidomide is well known. Infarct the expectant mother is well advised to avoid all necessary drugs taking during the early stages of pregnancy.5-7 (Table 1-3) A useful classification of clefts is that of Kernahan and stark which is bases on an embryological considerations of the tissues involved there are essentially three main groups.7,8
这些是影响口腔和相关结构的最严重的先天性畸形。这是最常见的先天性缺陷之一,大约每1000个新生儿中就有一个出现。1只有大约三分之一的病例有家族史。腭裂只在女孩中更常见,而唇腭裂,无论是否涉及腭,在男孩中更常见。有趣的是,左侧比右侧更常见。2在大多数情况下,劈开的确切原因尚不清楚。对于大多数裂隙情况,没有单一因素可以确定为原因。然而,重要的是要区分孤立的腭裂(患者没有其他相关的健康问题)和与其他出生障碍或综合征相关的腭裂。3,4在没有家族史的情况下,先天性异常的发生可能是由于突变的作用或妊娠期间的一些偶然发生。唇和腭在子宫内生命的5-8周内发育,任何干扰继承人形成的因素都必须在这段相对较短的时间内发挥作用。在这方面,很难对妊娠期间与产妇健康有关的因素提出积极的看法。人们普遍认为,德国麻疹和早孕期的x光检查可能会产生先天性异常,应避免。某些药物,特别是沙利度胺的致畸作用是众所周知的。梗塞建议孕妇在怀孕早期避免服用所有必要的药物。5-7(表1-3)一种有用的裂隙分类是Kernahan和stark,这是基于对所涉及组织的胚胎学考虑,主要有三类。7,8
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引用次数: 5
期刊
Journal of pediatrics & neonatal care
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