Importance of screening the peripheral smear

A 5-year-old boy presented with history of failure to thrive from infancy. There was a history of one sibling death due to similar problems and history of severe abortions in the mother. Routine examination of peripheral smear revealed more than 50% acanthocytes. Based on this tests were streamlined to doing lipid profile and Lipo protein electrophoresis which revealed hypolipidemia and absent Β hypo protein band. Jejuna) mucosal biopsy confirmed the diagnosis of A Beta Lipo proteinemia which revealed lipid laden enterocytes. This case illustrates the importance of simple tests like peripheral smear examination in streamlining further tests in the diagnosis of major diseases.     

外周血涂片检查对婴儿型庞贝病筛查和诊断的应用价值

目的探讨外周血涂片检查在婴儿型庞贝病(IOPD)筛查和诊断中的价值。方法回顾分析2013—2017年根据酶学和基因检测确诊的29例IOPD患儿的外周血涂片检查结果,并以同期诊断的12例非IOPD的肥厚型心肌病患儿的血涂片结果作为对照。结果 IOPD组血涂片中可见到大量含空泡的淋巴细胞,含空泡淋巴细胞中位百分比为36%(19%～67%);而对照组中几乎观察不到含空泡的淋巴细胞,含空泡淋巴细胞中位百分比为0%(0%～6.7%)。IOPD组血涂片中可见到大量过碘酸雪夫氏(PAS)染色阳性的淋巴细胞,PAS染色阳性淋巴细胞的中位百分比为36%(19%～66%);而对照组患儿中几乎观察不到PAS染色阳性的淋巴细胞,PAS染色阳性淋巴细胞的中位百分比为0%(0%～6.0%)。两组之间含空泡淋巴细胞以及PAS染色阳性淋巴细胞的百分率差异有统计学意义(P0. 01),且在散点图上的分布无重叠。结论外周血涂片检查快速、简单、易行,通过观察和计算含空泡和PAS染色阳性的淋巴细胞百分率有助于IOPD的筛查和早期诊断。     

Red Cell Distribution Width in the Diagnosis of Iron Deficiency Anemia

Objective: 1. To compare peripheral smear (PS) and Red cell distribution width (RDW) in diagnosis of Iron deficiency anemia (IDA) in various grades. 2. To study the changes in RDW and PS after therapy.Methods : Children in the age group of six months to five years with microcytic (MCV < 80fl) anemia (Hemoglobin < 11g%) were evaluated. Those who had received blood transfusion and /or were already on iron therapy were excluded. Evaluation included clinical examination, complete blood count (CBC), RDW estimation microscopic examination of peripheral smear, measurement of serum iron and transferrin saturation. Children with IDA were treated with oral iron for 8 weeks and PS, CBC including RDW were repeated.Result: Of the 100 children evaluated, 89 had IDA. 48% had mild, 42% had moderate and 10% had severe anemia. Transferrin saturation correlated with severity of anemia. Peripheral smear showed microcytosis and hypochromia in all cases with severe anemia, 61.5% and 22.5% of those with moderate and mild anemia respectively. RDW was suggestive of iron deficiency in 100%, 82.05% and 100% of patient with mild, moderate and severe anemia respectively.Conclusion : In the diagnosis of mild and moderate iron deficiency anemia, RDW had a higher sensitivity than PS. Red cell morphology, Hb, PCV and RDW showed significant improvement after iron-therapy     

初乳口腔涂抹/滴注预防早产儿坏死性小肠结肠炎meta分析

目的评价初乳口腔涂抹或滴注预防早产儿坏死性小肠结肠炎的有效性和安全性。方法计算机系统检索PubMed、Ovid-Embase、Web of Science、Cochrane Library、中国知网、万方数据库、维普数据库,收集早产儿初乳口腔涂抹/滴注的随机对照试验(RCT),检索时间从建库至2019年1月。2名研究员独立筛选文献,提取资料并对文献进行质量评价;采用RevMan5.3软件进行meta分析。结果共纳入7篇随机对照试验(研究对象368例)。Meta分析结果显示,与对照组相比,初乳口腔涂抹/滴注早产儿坏死性小肠结肠炎的发生率(OR=0.85,95%CI:0.40～1.79,P=0.67)、住院时间(WMD=-7.09,95%CI:-16. 84～2. 67,P=0. 15)、病死率(OR=0. 64,95%CI:0. 20～2. 06,P=0. 46)的差异均无统计学意义,而达到全肠道喂养时间(WMD=-1.90,95%CI:-2.15～-1.65,P0.01)、抗生素使用天数(WMD=-2.57,95%CI:-4.13～-1.01,P0. 01)的差异有统计学意义。结论初乳口腔涂抹/滴注不能降低早产儿坏死性小肠结肠炎的发生率和病死率,不能缩短早产儿住院天数,但能缩短住院早产儿达到全肠道喂养时间与抗生素使用天数。     

Exchange transfusion in newborns via a peripheral artery and vein

Exchange transfusion using a peripheral artery and vein was carried out 18 times in 17 newborn infants. The arteries used to withdraw blood were the radial [13], the ulnar [3] and the posterior tibial [2]. Infusion of blood was carried out simultaneously through a peripheral vein. There was no mortality or morbidity directly associated with the procedure. We recommend this technique for exchange transfusion in general, and especially in the very ill newborn.     

Noninvasive peripheral perfusion index as a possible tool for screening for critical left heart obstruction

Aim: Peripheral perfusion index (PPI) has been suggested as a possible method to detect illness causing circulatory embarrassment. We aimed to establish the normal range of this index in healthy newborns, and compare it with newborns with duct‐dependent systemic circulation. Design: We conducted a case–control study. Setting: Our study population comprised 10 000 prospectively recruited newborns from Västra Götaland, Sweden. Patients: A total of 10 000 normal newborns and 9 infants with duct‐dependent systemic circulation (left heart obstructive disease [LHOD] group) participated in the study. Methods: We conducted single pre‐ and postductal measurements of PPI with a new generation pulse oximeter (Masimo Radical SET) before discharge from hospital. Results: PPI values between 1 and 120 h of age show an asymmetrical, non‐normal distribution with median PPI value of 1.70 and interquartile range of 1.18–2.50. The 5th percentile = 0.70 and 95th percentile = 4.50. All infants in the LHOD group had either pre‐ or postductal PPI below the interquartile range, and 5 of 9 (56%) were below the 5th percentile cut‐off of 0.70 (p < 0.0001, Fisher's exact test). A PPI value <0.70 gave an odds ratio for LHOD of 23.75 (95% CI 6.36–88.74). Conclusion: PPI values lower than 0.70 may indicate illness and a value <0.50 (1st percentile) indicates definite underperfusion. PPI values might be a useful additional tool for early detection of LHOD.     

The etiology of peripheral lymphadenopathy in children

This prospective study evaluated 382 pediatric patients with peripheral lymphadenopathy (LA) presenting at the Pediatric Oncology and Hematology Departments of Social Security Children's Hospital and Gazi University Medical Faculty Hospital. The ages of the patients ranged between 2 months and 16 years (median 7 years); 72% of the patients were male. Of the 382 patients, 138 had localized LA (a single anatomic area involved), 171 had limited LA (two or three areas involved), and 73 had generalized LA ( four or more anatomic areas involved). The specific etiology (either benign or malign) was defined in 79% of patients with generalized LA. However, in patients with localized LA and limited LA, specific etiology could be identified only in 43 and 53% of patients, respectively. Based on this study, BCG-LA and pyogenic infections are more frequently manifested by localized LA; LA of unknown origin, Hodgkin's disease, tuberculosis, nasopharyngeal carcinoma, and toxoplasmosis are frequently manifested by localized or limited LA; and cytomegalovirus infection (CMV), infectious mononucleous, rubella, acute leukemia, non-Hodgkin's lymphoma are frequently manifested by limited or generalized LA. Out of 382 patients, 196 patients had a maximum lymph node diameter of less than 2 cm. A benign etiology was shown in 159/196 of these patients. In 37/196 of these patients LA was due to a malignancy, and these cases almost invariably had some apparent additional diagnostic clinical and laboratory findings. Based on this observation a maximum lymph node size of 2 cm was considered an appropriate limit to distinguish malignant disease from benign causes except when there is other evidence of an underlying malignant disease. However, lymphadenopathies located at supraclavicular region (27 patients) either localized or as part of generalized LA had a specific benign or malignant disease in etiology (malignancy in 20, tuberculosis in 3, CMV in 2, sarcoidosis in 1, and lipoma in 1) even though they were less than 2 cm in diameter.     

不同菌型空肠弯曲菌免疫诱发大鼠变态反应性周围神经病的对比研究

目的　明确空肠弯曲菌（CJ）两种不同血清型（Pen19和Pen43）与格林-巴利综合征（GBS）的关系。方法　分别用CJ-Pen19和CJ-Pen43菌体灭活粗抗原加完全福氏佐剂并充分乳化后,经皮下多点注射反复致敏60只Wistar大鼠,对两种菌株空肠弯曲菌(Pen19和Pen43)进行分组对比研究。结果　（1）Pen19和Pen43免疫后,两组大鼠血清IgG类抗CJ抗体滴度均增高,并于3～4周后稳定于高峰水平;（2）Pen19组坐骨神经病变率（60.0%）及原纤维病变率（17.7%）均比Pen43组（分别为10.0%和0.5%）和生理盐水组（分别为5.0%和0.5%）明显增高（P＜0.001）,后两组间比较差异无显著意义（P＞0.05）;（3）Pen19组原纤维病变类型（轴索变性∶髓鞘脱失）比较,初期以轴索变性为主,比值为3.4%∶0.7%,后期则以髓鞘脱失占优势,比值为5.3%∶24.1%;（4）Pen19组大鼠血清IgG类特异性抗CJ抗体水平与神经轴索变性率呈正相关(r=0.801),而与脱髓鞘率关系不大(r=0.353),IgM的水平与轴索变性及髓鞘脱失的发生均无关（r分别为0.253和0.281）。结论　（1）CJ对周围神经免疫性损伤能力与其血清类型密切相关;（2）CJ-Pen19所诱发的实验性周围神经病变病理类型初期以轴索变性为主,后期髓鞘脱失占优势;（3）特异性IgG类抗CJ抗体在轴索变性的发生中可能起重要作用。     

Percutaneous US-guided biopsies of peripheral pulmonary lesions in children

Background: A wide variety of diseases in children can present with peripheral lung lesions. Minimally invasive percutaneous techniques are preferred diagnostic tools when thoracoscopic resection is not indicated. Significant improvements in US resolution have increased the range of its applications for many diagnostic and therapeutic purposes. Objective: To determine the adequacy and safety of US-guided biopsy of peripheral pulmonary lesions in children. Materials and methods: A retrospective review was performed of the clinical, imaging and pathology records of 33 children (13 females and 20 males) in whom 38 US-guided percutaneous lung lesion biopsies had been performed between January 1996 and March 2004. Their mean age was 8.3 years (range 1–19 years, median 6.6 years). All procedures were done under general anesthesia and controlled respiration. Two techniques were used: a single-needle technique and a coaxial-needle technique. In each case, the data recorded included age, sex, lesion’s location and size, number of cores, pathology results (adequate, inadequate and indeterminate), and complications. In order to categorize the sample, the lesions were divided into four groups based on the size of the pleural surface: group 1 1–5 mm, group 2 6–10 mm, group 3 11–20 mm, and group 4 21 mm or more. Results: The mean pleural surface size of the lesions was 12 mm (range 2.3–24 mm). The coaxial-needle technique was used for 13 biopsies and the single-needle technique for 25 biopsies. Of the 38 biopsies, 32 were considered adequate (technical success 84%), 4 were truly inadequate, and 2 were indeterminate at the time of the biopsy, requiring surgical biopsy for confirmation. Minor complications occurred following 44% of the procedures, including: pain (n=5), small pneumothorax (n=4), pulmonary hematoma (n=4), atelectasis (n=4), small hemothorax (n=3), respiratory distress (n=1) and hemoptysis (n=1). No major complications occurred. No significant correlation was found between the size of the pleural surface and technical success (P=0.106) or the incidence of complications (P=0.23). Minor complications occurred following 6 out of 13 procedures using the coaxial-needle technique (16% of total) and following 11 out of 25 procedures using the single-needle technique (28% of total), with no statistically significant difference (P=0.1081). Conclusion: This small retrospective study suggests that US-guided lung biopsies are a safe and adequate method to sample peripheral pulmonary lesions in children, with a high rate of technical success and low morbidity, even for lesions with a small pleural surface (<5 mm).     

Bacterial infection of peripheral artery cannulae in newborn infants

ABSTRACT. The incidence of bacterial colonization of peripheral artery cannulae in 58 sick newborn infants was documented using a semi-quantitative culture technique. Of the 86 cannulae inserted, a positive "high-density" semi-quantitative culture not related to distant bacteraemia and indicative of local infection was found in nine cannulae (10%) and "low-density" colonization was found in one cannula (1%). One of the 58 infants died from cannual-related septicaemia and meningitis due to Klebsiella pneumoniae. Bacterial colonization was significantly more common in cannulae which had been present for more than seven days compared with cannulae present for seven days or less (P<0.05).     

Calcification of cartilages,brachytelephalangy and peripheral pulmonary stenosis. Confirmation of the Keutel syndrome

A 13-year-old slightly mentally retarded female is reported with diffuse calcification of the cartilage, brachytelephalangy, mixed hearing loss and peripheral pulmonary stenosis. The present observation confirms the existence of the Keutel syndrome as a distinct syndrome, with probable autosomal recessive inheritance.     

170例小儿急性白血病首诊时周围血象分析

目的 探讨首诊时外周血象对白血病诊断、治疗时的指导意义。方法 回顾10余年我院就诊的170例白血病患儿首诊时外周血象的变化。用法国产Hemacell Plus全自动血液细胞分析仪在严格质量控制下于首次抽骨髓前,由专人采取指尖血对170例急性白血病(AL)患儿首诊时进行周围血象中的白细胞、血小板计数及血红蛋白测定及幼稚细胞检查,并对结果进行了统计分析。结果 发现大部分患儿血小板计数中重度减少(BPC≤50×109／L 120例,占70.59％)、白细胞数多为减低或接近正常(WBC≤10×109／L 91例占53.53％)、多为中重度贫血(Hb≤90 g／L 148例占87.06％)。170例中有54例外周血片中可检出幼稚细胞,占31.76％。结论 小儿急性AL时血小板不同程度减低(占87％),呈中、重度贫血(占87.06％)。WBC多减低或接近正常(53.53％)。血片中检出幼稚细胞率(31.76％)。认为AL时外周血细胞数的变化可能与白血病细胞的类型、数量及其形成的张力对髓血屏障的累积程度以及骨髓基质细胞的功能等因素有关。     

Diagnostic approach to neonatal hypotonia: retrospective study on 144 neonates

The objectives of our study were to determine the actual frequency of the different disorders causing neonatal hypotonia and to assess the reliability of the first physical examination as well as the contribution of the main standard diagnostic tests. One hundred and forty-four infants diagnosed with neonatal hypotonia between January 1st 1999 and June 30th 2005 in our tertiary care facility were retrospectively included in the study. Perinatal history, clinical type of hypotonia, results of standard diagnostic tests, final diagnosis and outcome were abstracted from the original charts. A final diagnosis was reached in 120 cases. Central (cerebral) causes represented 82% of the elucidated cases, mostly hypoxic and hemorrhagic lesions of the brain (34%), chromosomal aberrations and syndromic disorders (26%) and brain malformations (12%). Peripheral (neuromuscular) causes were mainly represented by spinal muscular atrophy (6%) and myotonic dystrophy (4%). Positive predictive value of the initial clinical examination was higher in central type hypotonia. Neuroimaging, karyotype analysis and DNA-based tests were the most helpful diagnostic tools. These recent clinical data can be used to improve our strategy in investigating neonatal hypotonia and a diagnostic algorithm is proposed based on our findings.     

单纯性肾病综合征患儿外周血单个核细胞病毒分子表达的研究

目的　探讨外周血单个核细胞 (PBMC)呼吸道病毒感染与糖皮质激素敏感型单纯性肾病综合征 (SSNS)的发生和反复的关系。方法　采用免疫组化、原位杂交、酶联免疫吸附和病毒分离技术 ,对青岛大学医学院附属医院儿科和滨州医学院附属医院 2 0 0 0年 1月至 2 0 0 2年 12月收治的 36例初发组、15例反复组和 2 1例缓解组SSNS患儿检测了PBMC病毒分子表达和血清中病毒特异性IgM抗体水平。结果　初发组与反复组比较 ,PBMC中呼吸道合胞病毒抗原和肠道病毒基因表达的阳性率差异均无显著性 ;而初发组与缓解组和对照组比较及反复组与缓解组和对照组比较 ,PBMC中两种病毒感染的阳性率差异均有显著性 (P <0 0 5 ,P <0 0 1)。初发组与其他各组比较 ,不同病毒的特异性IgM抗体阳性率明显增高 ( P <0 0 5 ) ;而反复组与缓解组和对照组比较 ,多种病毒的特异性IgM抗体的阳性率差异均无显著性。 结论　初发和反复SSNS患儿的PBMC均易被病毒感染 ,糖皮质激素的应用并不影响细胞内病毒抗原和基因的表达 ,但可使病毒特异性IgM抗体的产生明显减少 ;PBMC内病毒抗原或基因表达阳性可做为SSNS有反复倾向的重要分子标志之一。     

Duration of peripheral intravenous catheter patency in children

ObjectiveThis study aimed to compare the impact of saline lock to running a slow continuous infusion to-keep-vein-open (TKVO) on the total time a peripheral intravenous (PIV) catheter remained patent.MethodA retrospective chart review of all children admitted to the paediatric ward of a regional hospital in Saskatchewan December 1, 2013 through February 28, 2014. Characteristics of patients with PIV catheters were abstracted from the health records, including patient size, catheter size and site, and total time each PIV catheter spent (i) infusing therapeutic fluids or medications, (ii) running a TKVO infusion, or (iii) saline locked. The duration of catheter patency was compared with the proportion of time that TKVO infusions were run, as well as patient gender and age.ResultsDuring 375 admissions, there were 189 PIVs which met inclusion criteria. The proportion of nontherapeutic time a PIV catheter spent TKVO compared to saline locked did not affect the total time the PIV catheter was patent (P=0.33). Gender had no influence, but older age, a nonmodifiable factor, was associated with increased time a PIV catheter remained patent (P=0.028).DiscussionPeripheral intravenous (PIV) catheter insertion can be a painful and traumatic procedure. On the paediatric ward of a regional hospital, TKVO infusions were not superior to saline lock for prolonging PIV catheter patency.