A new classification for pericarditis associated with meningococcal infection

Acute meningococcal pericarditis is a rare clinical disorder. Our review of the literature disclosed that current classifications are confusing since they fail to differentiate between two distinct criteria: time and causality. We suggest a new classification of the various states of meningococcal pericarditis on the basis of the pathophysiological process: disseminated meningococcal disease with pericarditis (purulent, culture-positive, associated with meningococcal bacteraemia); isolated meningococcal pericarditis (purulent, culture-positive but without signs of meningeal or other clinical systemic involvement); and reactive meningococcal pericarditis (immunological, late-onset, culture-negative, resembling post-viral pericarditis). It is essential that clinicians recognize the various states of the disease, since they differ in natural history, treatment and prognosis. Conclusion From personal experience and a literature review it emerges that meningococcal pericarditis should be classified as: (1) Pericarditis as local mani festation of disseminated meningococcal disease; (2) isolated minengococcal pericarditis; (3) reactive (immunopathic) meningococcal pericarditis. Received: 15 May 1996 / Accepted: 9 January 1997     

A new classification of acute protein-energy malnutrition

Current schemes to classify protein-energy malnutrition (PEM) in hospitalized children below 5 years of age in developing countries are of limited usefulness, because age is often not known and wasted children are insufficiently discriminated. In this study, a newly proposed scheme, based on the presence of wasting (weight-for-height < or = -2 z scores) and oedema, was compared with clinical and the original Wellcome criteria to classify PEM in 538 under-fives hospitalized for PEM in a rural hospital in Nchelenge, Zambia. Wasting in the absence of oedema was clinically false-positively diagnosed in 34.0 per cent, whereas the Wellcome classification gave rise to 28.6 per cent false-positive and 39.9 per cent false-negative diagnosis. In the presence of oedema, clinical diagnosis of wasting was 44.7 per cent false-positive and 41.4 per cent false-negative, while the Wellcome scheme had a false-negative rate of 54.3 per cent. We conclude that the Wellcome classification did not add to the clinical diagnostic accuracy of PEM as opposed to the proposed scheme, which merits further study. In an Appendix the use and calculation of z scores of weight-for-height is further explained.     

Glucose: A fuel for thought!

It is a privilege and an honour to deliver this, the Turner Gibson Oration for 1989. I chose the title of my lecture because glucose, as the essential substrate for brain function, is indeed the fuel for our thoughts. Although important at all ages, it has a particular significanice for the young child, since a normal supply of glucose is essential to protect brain development and hence the potential for thought in the child's brain. Thus, glucose should have a high priority in our own thinking as paediatricians. With this in mind, the purpose of my lecture is to fuel your own thoughts on your management of children who have disordered glucose homeostasis by examining controversy, challenging current dogma and providing new information.     

一种新的儿童先天性胫骨假关节的X线分型

目的 探讨一种新的儿童先天性胫骨假关节的X线分型,为临床治疗和判断预后提供依据.方法 回顾性分析本单位2004年至2014年期间收治的171例先天性胫骨假关节患儿的X线片,男125例(73％),女46例(27％),手术时平均年龄42.5个月.胫骨X线表现包括以下四种情况:Ⅰ型,胫骨前弓,在畸形的顶点处骨皮质增厚、但骨髓腔正常;Ⅱ型,胫骨前弓,在畸形的顶点处髓腔狭窄、骨皮质增厚和骨小梁的缺失;Ⅲ型,胫骨囊性病变,可以发生在胫骨的任何部位;Ⅳ型,胫骨假关节.腓骨X线表现包括以下四种情况:a:腓骨发育正常;b:腓骨发育不良(腓骨直径小于健侧腓骨直径);c:腓骨囊性变,多数发生在腓骨下1/3;d:腓骨假关节.根据患儿就诊时胫骨和腓骨的X线表现,提出一种新的X线分型方法.无论患儿是否曾经历手术治疗、是否有内外固定.结果 171例儿童先天性胫骨假关节按新的X线分型方法,不同类型的病例数如下:Ⅰa型9例,Ⅰb型3例,Ⅰc型3例,Ⅰd型4例.Ⅱa型9例,Ⅱb型15例,Ⅱc型3例,Ⅱd型6例.Ⅲa型6例,Ⅲb型12例,Ⅲc型8例,Ⅲd型17例.Ⅳa型13例,Ⅳb型10例,Ⅳc型4例,Ⅳd型49例.结论 结合儿童先天性胫骨假关节的胫骨和腓骨的X线表现、提出的这种新的X线分型方法,能够囊括目前本单位所有的病例.有可能为先天性胫骨假关节的治疗、术后并发症的防治、预后判断提供重要的指导作用,但仍有待进一步研究.     

Hemangiomas: new insights and classification

Infantile hemangiomas are the most common tumors of infancy, but less common vascular tumors also can affect young infants and children. In most cases, the diagnosis of IH can be made clinically, but imaging studies and even biopsy may be required in less-than-typical cases. With a careful history and physical examination focusing on the timing, location, and type of hemangioma, as well as extracutaneous signs and symptoms, the general pediatrician will know when to be concerned about a high risk vascular tumor and proceed with referral or further evaluations.     

Congenital iris ectropion and a new classification for anterior segment dysgenesis

Congenital iris ectropion has recently been added to the spectrum of neural-crest-derived anterior segment dysgenesis syndromes. Major features include a nonprogressive ectropion of the iris pigment epithelium, a glassy smooth cryptless iris surface, a high iris insertion, dysgenesis of the drainage angle, glaucoma, and in many cases, ipsilateral ptosis. Anterior segment dysgenesis syndromes have been subdivided into disorders of neural crest cell migration, proliferation, or differentiation. The congenital iris ectropion syndrome does not clearly fit into this classification. A new classification based on a theory of developmental arrest is presented which is more consistent with current knowledge of embryologic development and with recent clinical and histopathologic findings. The new classification links the congenital iris ectropion syndrome with the Axenfeld-Rieger spectrum but separates it from classic congenital glaucoma and the irido-corneal endothelial (ICE) syndromes. In addition, a histopathologically-supported etiologic theory for congenital iris ectropion is presented that supports the new classification.     

A mobile Meckel!

A technetium-99m pertechnetate Meckel scan is the standard diagnostic test to diagnose Meckel diverticulum. Although a negative scan does not exclude Meckel diverticulum, it should be kept in mind that the diagnosis can be missed on the basis of a single negative scan. Another important point is the typical position of this anatomical abnormality in the scan. We report a peculiar anatomical variation of Meckel diverticulum as seen in the technetium-99m pertechnetate scan. Recognizing this variant of Meckel diverticulum is important to avoid missing such a potentially life-threatening congenital anomaly.     

A new hierarchical classification of causes of infant deaths in England and Wales

In 1986 The Office of Population Censuses and Surveys (OPCS) introduced new certificates for stillbirths and neonatal deaths. This allowed certifiers more flexibility in the completion of the certificate, and the number and ordering of the causes given. Tabulations have been published of the fetal and maternal causes of death mentioned on the certificates for every year from 1986 to 1991 in annual reference volumes. It has not been possible either to derive a single cause group for each death, however, or to compare the information available on neonatal deaths with that on postneonatal deaths, which are still derived from the standard death certificate. The aim of the work described here was to adapt previous classifications to derive a single cause grouping for stillbirths and infant deaths which would provide the maximum information about preventability and yet meet the national and international responsibilities of OPCS. The methods used and the tests carried out on the validity and consistency of the chosen classification are described.     

Lymphangioma colli--a new classification contributing to prognosis.

The aim of the study was to introduce a classification of lymphangioma colli, which allows us to predict the expected morbidity and prognosis with surgical treatment. We defined 4 types, dependent on the volume of the lymphangioma as assessed by prenatal ultrasound or clinically: type I included tumors which had no or only a minimal effect on the contour of the neck; type II lymphangiomas were smaller than a line drawn at the lateral border of the head; type III tumors exceeded this line; in type IV the lymphangioma extended beyond the midline of the body. We report on the pre- and postoperative findings in 36 cases operated on between 1988 and 2000, mean follow-up was 4.2 years. 25 % of the cases were classified as type I, 36 % as type II, 22 % as type III, and 17 % as type IV. Cesarean section was carried out for 79 % of type III and IV lymphangiomas following prenatal diagnosis (71 %) and for 14 % of type I and II. MRT was indicated in 38 % of type II compared to 86 % in type III and IV lymphangiomas. Endoscopy was necessary in 25 % of type III and in 66 % of type IV lymphangiomas. In 2 cases (type I and IV), imaging revealed an intrathoracic portion. In 64 % of type I and II cases, resection was complete compared to 21 % in infants with more extended tumors. In all type IV cases, at least one further operation was required, likewise in 25 % of type III lymphangiomas. Postoperative effusion and infection occurred significantly more often in group IV than in groups I to III. 5 nerval lesions remained in children with operated type IV lymphangioma and 2 in type III cases. A visible lack of facial symmetry was evident at the follow-up examination in 83 % of type IV cases and moderate asymmetry was found in 19 % of all type I and II cases. Tracheostomy and gastrostomy were required only in type IV. The introduced classification of lymphangioma colli enables predictive statements to be made concerning the morbidity and prognosis with surgical therapy. The prognosis in type I is excellent; in type II, a cosmetically significant facial asymmetry remains in only 15 %. In type III lymphangiomas postoperative complications occurred in 38 % of cases, the necessity for reoperation with definitive lesions occurred in only 25 %. Morbidity gets significantly higher and the prognosis is worse if the lymphangioma exceeds the midline of the body.     

Heterogeneity in Klippel-Feil syndrome: a new classification

Background. Klippel-Feil syndrome (KFS) is characterised by congenital vertebral fusion of the cervical spine and a wide spectrum of associated anomalies. KFS has often been considered a sporadic syndrome. However, since the publication of the original KFS classification early this century, a number of KFS families have indicated heterogeneity complicated by a broad range of variable expression. Objective. The two major objectives of this study were (1) to identify differences and similarities in the postnatal appearance, morphology, position and inheritance of vertebral fusions within and between KFS families and (2) to establish a new KFS classification focussed on KFS aetiology. Materials and methods. Vertebral fusions were assessed via spinal radiography. Chromosomal karyotypes were performed using routine cytogenetics. Results. The medical histories of three KFS families are presented. The postnatal time, position and appearance of vertebral fusions, associated anomalies and mode of inheritance were different for the three KFS families. Four classes of KFS are described in a comprehensive classification table that allays much of the uncertainty arising from KFS heterogeneity and variable expression. Conclusion. We have described four different KFS classes (KF1–4) within a comprehensive classification that addresses KFS genetic heterogeneity. The position of vertebral fusions in the cervical spine and their incidence within affected families are delineating features of KFS. Received: 10 October 1997 Accepted: 20 April 1998