Congenital aganglionic megacolon

Twenty-one pull-through procedures for congenital aganglionic megacolon (Hirschsprung's disease) have been performed at the Los Angeles Children's Hospital since the adoption of the etiological concept of a distal aganglionic segment in 1949. In 14 cases the Swenson procedure as modified by Hiatt was employed, with perineal excision of the colon segment. There were four postoperative deaths and three symptomatic recurrences in this group. Three patients were treated by transabdominal resection of colon and rectum with subsequent pull-through reconstruction (Swenson). Anterior resection (State) was carried out in two cases. Three children with recurrence of symptoms following primary operation were subjected to a secondary pull-through procedure with an eventual successful outcome. The major portion of the postoperative mortality (29 per cent) in this group occurred in infants less than six months of age in whom anastomotic disruption or proximal segment infarction occurred after operation.A study of 31 cases of congenital aganglionic megacolon in very young infants drew attention to the difficulty of establishing a diagnosis in this age group even at exploratory laparotomy. Among these infants the mortality rate was excessive, regardless of the form of therapy employed. Colostomy appeared to be the indicated surgical procedure if a conservative regimen failed to control intractable colonic obstruction during the first year of life.     

Long-term results of frontal lobe suspension in children with congenital dystrophic ptosis

Long-term results achieved by our own operative technique in children with congenital dystrophic ptosis, with frontal muscle lobe shaping with or without shaping of corrugator muscle lobe attached to the tarsal plate, are presented. Data on 146 patients with congenital dystrophic ptosis operated on during the 1984-1998 period at Zagreb University Hospital Center were retrospectively analyzed. Postoperative success was defined as a situation with eyes open in which 1) upper eyelid covers the cornea at 12 o'clock position by 1-2 mm; 2) there is a good contour of the eyelid margin; 3) there is no lagophthalmos; and 4) there is symmetry with the other eye. Immediate re-operation due to undercorrection was required in 26 of 146 (18%) patients. Upon re-operation, 133 (91%) patients met the criteria for successful outcome at 6 months, 124 (85%) at one year, and 121 (83%) at 5 years. Correction of congenital dystrophic ptosis using a shaped frontal/corrugator lobe is an efficient and safe procedure ensuring long-lasting success.     

Clinical Problems: Results of Closure of Loop Transverse Colostomies

The postoperative course of 139 patients who underwent closure of a loop transverse colostomy at St. Mark''s Hospital between 1961 and 1970 after distal resection and anastomosis of the large intestine for neoplastic or diverticular disease has been reviewed in detail. There were no deaths and the incidence of breakdown at the site of the colostomy closure was 2·9%. The procedure can be straightforward and safe.     

Causal connection of non-specific low back pain and disc degeneration in children with transitional vertebra and/or Spina bifida occulta: role of magnetic resonance--prospective study

The problem of low back pain (LBP) in children is very common and many specialists are dealing with it in everyday practice. The cause for low back pain often is not found and classified under the diagnosis of non specific low back pain. The objective of this prospective study is to determine wether children with non specific low back pain and existence of anomalies in LS spine (transitional vertebra- TV and/or Spina bifida occulta SBO) also have the degeneration of the intervertebral disc (DD) L4-L5 and/or L5-S1. This prospective study included 69 patients from 8 to 16 years of age (X 12.81) of whom 40 were male (57.97%), and 29 female (42.03%). They all were examinated in University of Zagreb, "Sestre milosrdnice" University Hospital Center, Zagreb Children's Hospital, Department of Orthopaedic, Zagreb, Croatia. The reason of their visit was non specific low back pain. Pain was measured by visual analog scale (VAS) and mean score was three, duration of pain was between two and four weeks. Also, pain was sporadic, during daytime and not connected with level of physical activity. They all have undergone an algorithm of radiological examinations. Standard AP and LL radiographs (RTG) were made, as well as magnetic resonance (MR) of LS spine and sacrum in sagittal and transversal plane in T1 and T2 weighted sequence. The anomalies of L5 and S1 were found in 65 patients: transitional vertebra classified according to Castellvi et al. and SBO. In MRI in T2 weighted sequence DD was found in 61 patients which was classified modified from Pearce. Data analysis and comparison showed that 56patients with TV and/or SBO have changes on vertebral dynamic segment L5-S1 (VDS) and that means DD. In 13 patients only DD or spinal anomaly (TV and/or SBO) were found. Correlation between anomalies and DD in those patients was established by McNemar analysis and has shown significant difference (p=0.581) in favour of the patients with anomaly and DD. This has established that all of 56 patients with spinal anomaly could have DD as known cause of LBP.     

Congenital heart disease in sibships ascertained by two affected siblings

Summary Families containing at least 2 children affected by cardiologically diagnosed congential heart disease were selected from the files of the Children's Memorial Hospital, Chicago, Willis J. Potts Children's Heart Center. From the data of 54 such families a recurrence rate of CHD for additional children of between p (K=0, r_(min)=2)=0.0554±0.0256 and p (K=1, r_(min)=2)=0.0801±0.0349 was calculated. Incompleteness of families or voluntary termination of reproduction after the second affected child was born could be excluded as effective bias-inducing factors. In the great majority of cases, sibs displayed identical or closely related malformations. No paternal or maternal age effect was found. Consanguinity was not noticeably increased, but the material was unsuitable for the study of this question. A recurrence rate which was definitely higher in families with at least two affected children than in families selected by a single proband, is taken as further evidence for the importance of genetic factors in congenital heart disease. Any single-gene hypothesis can be fitted to the actual data only if far-reaching additional assumptions are made. A multifactorial genetic system explains the observations more easily. This work has been carried out in part during the author's stay at the Genetic Clinic of the Children's Memorial Hospital, Dept. of Pediatrics, Northwestern University Medical School (Prof. D. Y.-Y. Hsia, M.D.). Data have been collected in collaboration with Dr. M. H. Paul, director of the Dept. of Cardiology of the Children's Memorial Hospital, Willis J. Potts Children's Heart Center. The author was supported in part by a grant from the US Public Health Service, National Institutes of Health and a Fulbright Travel Grant.     

Reossification of the orbital wall following ventral translocation of the fronto-orbital bar and cranial vault remodeling.

The purposes of this study were to determine the extent of ossification of the orbit following ventral translocation of the fronto-orbital bar and to find out whether age at the time of the procedure and presence of a concomitant syndrome adversely affect ossification. A retrospective review of 27 patients with craniosynostosis was conducted at the St. Louis Children's Hospital and the Children's Hospital of Oklahoma. Patients with preoperative, perioperative, and postoperative three-dimensional computed tomography scans were included. Eighty-eight percent of the lateral orbital wall defects and 92 percent of the defects within the roof of the orbit ossified completely in the postoperative period. When syndromic patients were compared with nonsyndromic patients (based on clinical findings only), three of the 19 syndromic defects and three of the 30 nonsyndromic defects demonstrated incomplete ossification in the lateral orbital wall (p > 0.05). Similarly, two of the 19 syndromic defects and two of the 30 nonsyndromic defects demonstrated incomplete ossification within the roof of the orbit (p > 0.05). With respect to age at the time of the procedure, four of the 37 defects and two of the 12 defects demonstrated incomplete ossification in the lateral orbital wall for age at the time of the procedure less than 12 months and greater than 12 months, respectively (p > 0.05). Similarly, two of the 37 defects and two of the 12 defects had incomplete ossification within the roof of the orbit for age at the time of the procedure less than 12 months versus more than 12 months, respectively (p > 0.05). Ossification of the orbital wall and roof is complete in the majority of cases within 1 year after the procedure, and neither age at the time of the procedure nor presence of a concomitant syndrome adversely affects ossification of the orbit after ventral translocation of the fronto-orbital bandeau.     

A novel neuropeptide,pituitary adenylate cyclase-activating polypeptide (PACAP), in human intestine: evidence for reduced content in Hirschsprung's disease

Summary A novel neuropeptide, pituitary adenylate cyclase-activating polypeptide (PACAP), exhibits sequence homology with vasoactive intestinal polypeptide (VIP) and occurs in the mammalian brain, lung and gut. The distribution of PACAP in ganglionic and aganglionic portions of the large intestine of patients with Hirschsprung's disease was examined by immunohistochemistry and radioimmunoassay. PACAP-immunoreactive nerve fibers were distributed in all layers of the ganglionic and aganglionic segments of the intestine, although they were less numerous in the latter, and PACAP-immunoreactive nerve cell bodies were seen in the ganglionic portion of the intestine. The concentration of immunoreactive PACAP was lower in the aganglionic than in the ganglionic segment of the intestinal wall. PACAP and VIP were found to coexist in both ganglionic and aganglionic segments of the intestine. Apparently, PACAP participates in the regulation of gut motility. The scarcer PACAP innervation of the aganglionic segment may contribute to the defect in intestinal relaxation seen in patients with Hirschsprung's disease.     

Phacoemulsification and silicone oil removal through the planned posterior capsulorhexis

The purpose of the study was to present operative technique and results of a passive hydrodynamic expression of silicone oil through planned posterior capsulorhexis during cataract surgery in patients after pars plana vitrectomy. The retrospective analysis was done on 57 eyes with cataract after a previous pars plana vitrectomy, operated on between 2001 and 2004 at the Clinical hospital "Sestre milosrdnice" Zagreb. Preoperative and postoperative best corrected visual acuity (BCVA), preoperative and postoperative intraocular pressure (IOP), and postoperative complications were reviewed. Visual acuity improved or stabilized in all patients with an attached retina. Retinal detachment occurred in 11 eyes. Transient vitreous hemorrhage, that resolved within 1 week of surgery without treatment, was observed in 4 eyes. Asymptomatic intraocular lens (IOL) decentration occurred in 2 eyes. Our findings suggest that silicone oil removal and cataract surgery can be performed as a single procedure in selected patients in the absence of macular pucker and retinal reproliferation, and in a presence of a stable retina.     

先天性心脏病患儿术后急性肾损伤的影响因素及尿NGAL、KIM-1的诊断价值分析

摘要 目的：探讨影响先天性心脏病患儿术后急性肾损伤（AKI）的影响因素及尿中性粒细胞明胶酶相关脂质运载蛋白（NGAL）、肾损伤分子1（KIM-1）的诊断价值。方法：选择2018年1月至2019年12月我院心胸外科收治的60例先天性心脏病术后并发AKI患儿（AKI组）和同期收治的172例先天性心脏病术后未发生AKI患儿（NAKI组）作为研究对象。收集患儿临床基线资料,检测尿NGAL、KIM-1水平,采用Logistic回归分析先天性心脏病患儿术后发生AKI的影响因素,受试者工作特征曲线（ROC）分析尿NGAL、KIM-1诊断先天性心脏病患儿术后发生AKI的价值。结果：AKI组年龄、体重低于NAKI组（P＜0.05）,手术时间、心肺转流（CPB）时间、主动脉阻断（ACT）时间、机械通气时间、重症监护室（ICU）住院时间长于NAKI组（P＜0.05）,术后平均动脉压（MAP）、尿素氮（BUN）、血肌酐（Scr）、NGAL、KIM-1高于NAKI组（P＜0.05）。Logistic回归分析结果显示低龄、低体重、CPB时间长、高NGAL、KIM-1水平是先天性心脏病患儿术后发生AKI的危险因素（P＜0.05）。ROC分析显示尿NGAL、KIM-1诊断先天性心脏病患儿术后发生AKI的灵敏度分别为81.67%,83.33%,特异度分别为84.30%,87.79%。结论：低龄、低体重、CPB时间长、高NGAL、KIM-1水平是先天性心脏病患儿术后发生AKI的危险因素,尿NGAL、KIM-1诊断先天性心脏病术后AKI具有较高价值。     

Neuron and glia generating progenitors of the mammalian enteric nervous system isolated from foetal and postnatal gut cultures

Cultures of dissociated foetal and postnatal mouse gut gave rise to neurosphere-like bodies, which contained large numbers of mature neurons and glial cells. In addition to differentiated cells, neurosphere-like bodies included proliferating progenitors which, when cultured at clonal densities, gave rise to colonies containing many of the neuronal subtypes and glial cells present in the mammalian enteric nervous system. These progenitors were also capable of colonising wild-type and aganglionic gut in organ culture and had the potential to generate differentiated progeny that localised within the intrinsic ganglionic plexus. Similar progenitors were also derived from the normoganglionic small intestine of mice with colonic aganglionosis. Our findings establish the feasibility of expanding and isolating early progenitors of the enteric nervous system based on their ability to form distinct neurogenic and gliogenic structures in culture. Furthermore, these experiments provide the rationale for the development of novel approaches to the treatment of congenital megacolon (Hirschsprung's disease) based on the colonisation of the aganglionic gut with progenitors derived from normoganglionic bowel segments.     

Toxoplasma gondii antibody titers in sera of children admitted to the Seoul National University Children's Hospital

A total of 542 children under 10 years of age, admitted to the Seoul National University Children's Hospital, was examined for antibody titers of Toxoplasma gondii using indirect latex agglutination (ILA) test. Among them, 7.7% showed positive titers higher than 1:32, without significant difference between males (7.3%) and females (8.5%). The seropositive rate increased with age although the statistical significance was negligible (0.05 < P < 0.1). By residential areas, the prevalence appeared higher among children from southern provinces (Kyongsang-do and Cholla do) than those from other areas, but the statistical significance was also very low (0.05 < P < 0.1). When the seropositive cases were analyzed by coincidental diseases, the prevalence was significantly higher in patients with congenital diseases than in patients with non-congenital diseases (P < 0.05). The results showed that the seropositive rate of toxoplasmosis in children examined was not high compared with other endemic countries. Some correlations are suggested between toxoplasmosis and congenital anomalies in Korea.     

快通道麻醉在小儿先天性心脏病手术中的应用及对镇静、镇痛效果的影响

目的:探讨快通道麻醉在小儿先天性心脏病手术中的应用及对镇静、镇痛效果的影响。方法:选择2016年6月至2018年5月在桂林医学院附属医院进行手术治疗的小儿先天性心脏病患儿82例,按照随机数字表法分为对照组(41例)和观察组(41例),对照组进行常规麻醉,观察组则采用快通道麻醉。对比两组患儿麻醉药物用量、手术时间、阻断时间、体外循环时间、术后拔管时间、住院时间、住院费用及并发症发生情况,观察两组患儿术后12h镇静、镇痛效果。结果:观察组患儿芬太尼和罗库溴铵用量明显低于对照组(P0.05)。两组患儿的术中各指标比较无统计学差异(P0.05),而与对照组相比,观察组患儿术后住院时间、拔管时间均缩短,且住院费用减少(P0.05)。观察组患儿术后12h的镇静及镇痛效果均优于对照组(P0.05)。观察组患儿术后并发症发生率为17.07%(7/41),低于对照组的39.02%(16/41)(P0.05)。结论:快通道麻醉可减少小儿先天性心脏病手术的芬太尼和罗库溴铵用量,不仅缩短术后拔管时间和住院时间,降低住院费用,而且可改善镇静及镇痛效果,安全有效。     

Hirshsprung's disease; the clinical differentiation and treatment of children with Hirschsprung's disease and pseudo-Hirschsprung's disease

Hirschsprung's disease is marked by constipation from the time of birth, with the development, if uncorrected, of a protuberant abdomen and flared costal margins. The rectal ampulla is empty and the abdomen is filled with fecal masses. Pain is not prominent. Flatus is passed in large amounts. Encopresis does not occur. Barium enema shows the characteristic narrowed distal rectal segment and biopsy of the rectum shows absence of the ganglion cells of the myenteric plexus. Treatment is operative resection of the distal narrow segment and a primary anastomosis.Hirschsprung's disease may be mimicked in children with:1. Psychogenic constipation-pseudo-Hirschsprung's disease. Unlike Hirschsprung's disease, symptoms do not appear at birth, encopresis is common, and the barium enema shows no narrow distal segment.2. Mental retardation and cerebral defect.3. Corrected imperforate anus-on the basis of stenosis, imperfect innervation or poor habit training.4. Cretinism-with severe constipation and intestinal dilatation perhaps the presenting symptoms. Treatment of these four groups of children with severe constipation not due to Hirschsprung's disease is:For Group 1, open discussion with parent and child. Assumption by the physician of full control of the details of treatment, and relegation of parent to the role of the physician's agent in following the prescribed regimen. For Group 2, an enema regimen. Whereas fairly rapid restoration (and then persistence) of normal bowel habit can be expected in Group 1, the basic defects in Group 2 may require indefinite continuation of treatment. For Group 3, regular enema regimen, in the less severe cases-one identical with that used in Group 1, and dilatation of strictures or anoplasty. In Group 4, thyroid hormone therapy relieves the constipation of hypothyroidism and causes reversion of radiographic changes in the colon and rectum.     

Self-esteem in children and adolescents differently treated for locomotory trauma

Self-esteem involves the evaluative and affective dimensions of self-concept. It could be influenced by stress situations such as diseases or injuries, especially in the period of puberty and adolescence. The aim of this study was to establish the influence of isolated long tubular bone limbs' fractures in children and adolescents and type of its treatment (conservative or active surgical treatment-various techniques) on self-esteem of patients, as well as to establish relationships between self-esteem, depression, anxiety and perception of the social support in the mentioned patients. This prospective clinical trial comprehends 135 patients, 94 male and 41 female, aged 10 to 18, treated for the mentioned fractures in the period from October 2003 until March 2005 in Departments for Pediatric Surgery of three hospitals: the Clinical Hospital Center in Rijeka (88.8% patients), the Clinical Children's Hospital in Zagreb (9.7%), both in Croatia, and 1.5% of the patients in the Clinical Hospital in Mostar (Bosnia and Herzegovina). 53.3% of the patients were treated conservatively, 29.6% of them underwent the elastic stable intramedullary nailing (ESIN), while the remaining 17.1% of the patients were treated with other surgical techniques (AO-plates or Kirschner-wire ostheosyntheses). The basic methods of work were self-reported questionnaires: Rosenberg Self-esteem Scale (RSS), to establish the degree of self-esteem; Children Depression Inventory (CDI), to establish existence and degree of depression; Spielberg State Trait Anxiety Inventory (STAI)-form STAI2, to establish general anxiety; and Test of Perception of Social Support (TPSS). RSS, CDI and STAI2 were administered twice to the patients: at baseline and after 6 months of the trauma, whereas TPSS once, after 1 month of the trauma. Our results point at an decreased self-esteem followed by increased depression and increased general anxiety indicators in all patients within 1 week of experienced trauma, especially in those who underwent the ESIN method, whereas after 6 months of the experienced trauma, self-esteem significantly increased and depression and general anxiety indicators were greatly reduced in all patients. The type of treatment of fractures has no influence on the perception of the social support. Our results suggest that the ESIN method (regardless of its good surgical results and advantages in relation to many other surgical techniques) reduces self-esteem more than conservatively treatment of fractures and AO-plates and K-wire ostheosyntheses. At the same time self-esteem has been recuperated faster in patients treated with ESIN method. Thus, there is no difference in the influence of the type of the treatment of fractures on self-esteem, but in the dynamics of its recuperation in patients according to type of treatment of fractures. Anyway, from a psychological point of view, any type of surgical treatment could be additional stressor, so it should be practiced with criticism and according to the strict surgical indications.     

X-ray diagnosis of different forms of Hirschsprung's disease in children

The purpose of this study was to examine and compare X-ray signs and roentgenometric parameters in different forms of childhood Hirschsprung's disease for their more accurate diagnosis. A hundred and thirty-eight children with different forms of Hirschsprung's disease were followed up. Among them, the children with a supershort segment of the disease amounted to 55%. All the children underwent a comprehensive examination including barium irrigography according to the procedure described by M.D. Levin, followed by an estimation of roentgenometric parameters. The long forms of Hirschsprung's disease had characteristic X-ray symptoms and their appropriate roentgenometric parameters. The supershort segment of the disease had not a complete complex of characteristic X-ray symptoms and its roentgenometric parameters were in direct opposition to the similar parameters in the long form of this disease.     

Smooth muscle from aganglionic bowel in Hirschsprung's disease impairs neuronal development in vitro

Hirschsprung's disease results from the congenital absence of enteric neurons in human distal colon. The reason for aganglionosis is unknown but may reflect an unfavourable microenvironment for neuronal development. We asked if smooth muscle cells from the aganglionic region could affect neuronal development in vitro. Neurons from neonatal mouse superior cervical ganglia were added to cultures of smooth muscle obtained from normal or aganglionic regions of five patients with Hirschsprung's disease. Although neurons initially showed more rapid attachment to aganglionic smooth muscle, this was equal by 60 min and thereafter. Progressive increase in the diameter of the nerve cell body was characteristic of normal maturation in vitro. This was consistently inhibited by 15–22% in neurons grown on aganglionic muscle compared with normal controls over the 6-day test period (P<0.05). This phenomenon was preserved when the smooth muscle cells were lysed by brief exposure to distilled water before initiation of coculture (16–18% inhibition; P<0.05). These data imply that smooth muscle of the aganglionic colon is less favourable for neuronal development than the normally innervated region and suggest a membrane-linked factor. Clearly, this persists in postnatal life and in vitro and may reflect an abnormality of cellular interaction causing Hirschsprung's disease.     

Microbiological analysis of a mummy from the archeological museum in Zagreb

In this paper we report the results of the microbiological analysis of the samples taken from the mummy from the collection of the Archaeological museum in Zagreb, Croatia. Samples were taken from specific places such as oral, orbital, abdominal cavity and bandages surrounding the mummy, and analyzed in Department of Microbiology and Hospital Infections in University Hospital "Dubrava" in Zagreb and in National Reference Laboratory for systemic mycoses of Croatian National Institute of Public Health in Zagreb. The analysis indicated that all of the found organisms were non-primary pathogenic and are not harmful for healthy humans. Isolated microorganisms mainly belonged to the group of saprophytic fungi as listed: Monilia spp., Penicillium spp., Alternaria spp., Aspergillus fumigatus, Aspergillus nidulans, Rhizopus spp. and Chrysosporium spp. and to the genus of saprophytic bacteria, Bacillus spp.     

Paradigms and Politics: Shaping Health Care Access for Sickle Cell Patients Through the Discursive Regimes of Biomedicine

The emergence of two different sickle cell disease and disease/treatment paradigms in two clinics, Children's Hospital West (CHW) and Children's Hospital East (CHE), demonstrates how physicians can influence institutional regimes of truth to improve patient access. Physicians at both clinics, far from simply acquiescing to dominant biomedical paradigms, recognize that their paradigms are in part rhetorical strategies designed to subvert problematic staff biases and perceptions, and to encourage a particular "self-efficacy" ethic in the patients. This paper positions physicians as struggling within the discursive regimes of biomedicine to create an institutional space where the disease and the sickle cell patient matter, and where patients comply with the performative rules of that space. This paper explores how physicians, patients, and institutions collaborate in the construction of sickle cell disease in such a way that biomedicine becomes a plural, as opposed to a singular and oppressive, discursive regime.     

Change in the Distribution of Acetylcholinesterase Molecular Forms in Hirschsprung's Disease

Abstract: Density gradient ultracentrifugation shows that two molecular forms of acetylcholinesterase (4S and 10S) can be distinguished in the bowels of both normal subjects and Hirschsprung's disease patients. In this disease, besides the very large elevation of acetylcholinesterase activity, the relative distribution of the heavy and light forms was also changed. In the affected bowel the 10S/4S ratio was 2.5 times higher than the normal value. It is assumed that the accumulation of the 10S form might be a response of the intestine to this pathological state. It is also suggested that the increase in the heavy form is closely connected with the nerve fibre proliferation in the aganglionic megacolon.     

A family study in transposition of the great vessels and in tricuspid atresia

Summary A group of 186 propositi with transposition of the great vessels (TGV) and 33 propositi with tricuspid atresia (TA) were studied. Data were obtained from hospital records and complemented by questionnaires and in part by personal interview and clinical examination. The main results were among sibs of propositi with TGV an increased incidence of TGV (0.0135±0.0054) and of congenital heart disease in general (0.0495±0.0103) (K=0, r_min=1). Among 92 sibs of propositi with TA only one with patent ductus arteriosus was found. The sex ratio of the propositi with TGV was 1.95. There was also noted a highly abnormal sex ratio among sibs of parents of patients with TGV in part confirming the findings of Knox.A male excess was found in paternal and maternal sibships and was also present in sibships of propositi. Data about seasonal distribution were inconclusive as were data about consanguinity of parents. No parental age or birth order effect was found in either group.This work has been carried out in part during the author's stay at the Genetic Clinic of the Children's Memorial Hospital, Dept. of Pediatrics, Northwestern University Medical School (Prof. D. Y.-Y. Hsia, M.D.). Data have been collected in collaboration with Dr. M. H. Paul, director of the Dept. of Cardiology of the Children's Memorial Hospital, Willis J. Potts Children's Heart Center. The author was supported in part by a grant from the US Public Health Service, National Institutes of Health and a Fulbright Travel Grant.