Megacystis-microcolon-intestinal hypoperistalsis syndrome.
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Abnormalities, Multiple↗
Biomedical subjects
P Puri
Publications and source records attributed to P Puri.
Mesothelioma of tunica vaginalis testis in a child.
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Child↗
Newly diagnosed contralateral reflux after successful unilateral endoscopic correction: is it due to the pop-off mechanism?
PURPOSE: Our aim was to analyze the incidence of newly diagnosed contralateral vesicoureteral reflux after successful endoscopic correction of unilateral reflux by subureteral Teflon (polytetrafluoroethylene) injection and to explain the possible mechanisms responsible for new contralateral reflux. MATERIALS AND METHODS: A total of 1,195 children underwent successful endoscopic correction of high grade primary and duplex grades III to V vesicoureteral reflux by subureteral polytetrafluoroethylene injection. Unilateral and bilateral reflux was noted in 495 and 700 children, respectively. The 495 cases of unilateral reflux were retrospectively analyzed for new contralateral reflux. Voiding cystourethrography was performed in all cases 3 months and 1 year after injection. RESULTS: New contralateral reflux was diagnosed in 37 children (7%), including reflux in a single system in 33 and into a lower pole of a completely duplicated system in 4. There was no correlation of grade of ipsilateral reflux with the subsequent development of new contralateral reflux. No abnormality of the contralateral ureteral orifice was identified at initial cystoscopy, except for laterally placed ureteral orifices in 2 patients. CONCLUSIONS: The low incidence of new contralateral vesicoureteral reflux diagnosed after subureteral polytetrafluoroethylene injection versus open ureteral reimplantation may be due to noninterference with the contralateral trigone in endoscopic correction. The risk of new contralateral reflux did not increase with reflux severity (grades IV and V), suggesting that the pop-off mechanism is unlikely to be the cause of contralateral reflux.
Adolescent↗
The vanishing testis: anatomical and histological findings.
OBJECTIVES: To review anatomical and histological findings in 105 vanishing testes. METHODS: Records of 2,509 boys with 3,064 cryptorchid testes treated at our hospital between 1969 and 1995 were reviewed. RESULTS: 691 (23%) testes were clinically impalpable. Exploration in 691 impalpable testes revealed absent testis in 144 (21%). In 39 (27%) of the 144 absent testes, there was complete agenesis of testis along with the epididymis and vas deferens whereas 105 (73%) were associated with blind-ending cord structures-the vanishing testis. The site of blind-ending cord structures in 105 vanishing testes was intra-abdominal in 22 (21%), inguinal canal in 62 (59%), superficial inguinal ring in 19 (18%) and scrotum in 2 (2%). Histological information was available in 47 vanishing testes and revealed vas, epididymis, or both in 32 (68%), fibrous/vascular tissue in 11 (23%) and testicular cords in 4 (9%). Dystrophic calcification and/or haemosiderin were present in 7 (15%). CONCLUSIONS: Our data show that the incidence of vanishing testis in boys with non-palpable testes is over twice the incidence of testicular agenesis. The most common site of blind-ending cord structures is distal to the internal inguinal ring. The finding of viable testicular tissue at the end of the attenuated cord structures in 4 of our patients, and also reported in other series, suggests that inguinal exploration should be carried out in all patients who on laparoscopy are found to have cord structures entering the internal ring.
Child, Preschool↗
Increased levels of circulating endothelial leukocyte adhesion molecule-1 (ELAM-1) in children with reflux nephropathy.
OBJECTIVES: The aim of this study was to determine circulating levels of ELAM-1 in sera collected from patients with vesicoureteral reflux, and to investigate the relationship between circulating concentrations of ELAM-1 and reflux nephropathy. METHODS: The serum levels of ELAM-1 were measured in 117 patients aged between 3 months and 13 years, with grade III to grade V vesicoureteral reflux, and 22 controls, by ELISA technique. Renal scarring was demonstrated in 46 of the 117 children on radionuclide scans. RESULTS: The mean serum ELAM-1 levels were 54.23 +/- 20.11 ng/ml in controls and 77.79 +/- 34.50 ng/ml in children with vesicoureteral reflux (p < 0.05). The mean serum ELAM-1 levels in children with vesicoureteral reflux and renal scarring (104.48 +/- 34.53 ng/ml) were significantly higher than in children with VUR without renal scarring (60.51 +/- 20.85 ng/ml; p < 0.001). CONCLUSION: These findings suggest that circulating ELAM-1 levels reflect increased tissue damage in children with vesicoureteral reflux, probably as a result of progressive localised inflammatory responses occurring in these patients.
Adolescent↗
Historical aspects of congenital diaphragmatic hernia.
The first description diaphragmatic hernia appeared in 1575. In 1848, Bochdalek described congenital diaphragmatic hernia (CDH) occurring through a posterolateral defect. Successful surgical treatment of CDH in an infant was first performed in 1902, whereas the first neonate operated within 24 hours of life was reported in 1946. However, early surgery did not improve survival rates and the mortality was in the region of 50%. One reason for this was that more neonates underwent surgery who previously would have died without the repair of the CDH. Pulmonary hypoplasia and pulmonary hypertension were early recognised as important reasons for the high mortality rate. In recent years, an enormous effort has been made by research groups all over the world to describe the pathogenesis and pathophysiology of CDH, and apply these findings to clinical practice. Attempts have been made to define prognostic factors. Extracorporeal membrane oxygenation (ECMO) has produced encouraging results. Fetal surgical therapy remains an option in selected cases despite huge technical and ethical problems. Recently, several new therapeutic methods have been suggested, such as high frequency oscillatory ventilation, partial liquid ventilation, nitric oxide inhalation, surfactant therapy, and fetal tracheal ligation. However, more experience is required before the value of these approaches is clear. Despite these efforts, the mortality remains unacceptably high. The challenge for the future is to continue development of therapeutic approaches in order to improve survival of neonates with CDH.
Female↗
Is a histologically normal appendix following emergency appendicectomy alway normal?
BACKGROUND: Appendixes removed from patients with suspected appendicitis often appear normal on histological examination. We examined appendix specimens for expression of abnormal amounts of cytokines, an indicator of an inflammatory response. METHODS: Tumour necrosis factor alpha (TNFalpha) and interleukin-2 (IL-2) expression was measured by in-situ hybridisation in ten specimens from patients with acute appendicitis, 12 normal appendix specimens removed from patients undergoing elective abdominal surgery, and 31 appendix specimens from patients with a clinical diagnosis of appendicitis but an appendix histologically classified as normal. Cytokine-specific RNA antisense probes were prepared by in-vitro transcription and digoxigenin (DIG) labelled. In-situ hybridisation was done on 5 micrometer paraffin sections. Tissue sections hybridised by sense probes acted as negative control for each cytokine. Following hybridisation, the probes were detected by alkaline phosphatase labelled anti-DIG monoclonal antibody and visualised by nitroblue tetrazolium staining. FINDINGS: All histologically proven acute appendicitis specimens demonstrated intense cellular TNFalpha mRNA expression in germinal centres and moderate levels of expression throughout the mucosa. IL-2 mRNA was strongly expressed in the lamina propria and only moderately expressed in germinal centres. Normal appendixes all showed almost complete absence of TNFalpha and IL-2 mRNA expression. Seven of the 31 histologically classified normal appendix specimens from patients with a clinical diagnosis of appendicitis demonstrated TNFalpha and IL-2 mRNA expression similar to acute appendicitis specimens in germinal centres, submucosa, and lamina propria layers. INTERPRETATION: TNFalpha and IL-2 mRNA expression is a sensitive marker of inflammation in appendicitis. A substantial proportion of histologically normal appendixes showed clear evidence of an inflammatory response in the form of increased cytokine expression.
Appendectomy↗
Abnormal internal anal sphincter innervation in patients with Hirschsprung's disease and allied disorders.
The morphology of the intrinsic innervation of internal anal sphincter (IAS) in Hirschsprung's disease (HSCR) and allied disorders has not been clearly defined. At the time of IAS myectomy, specimens of the IAS were taken from four patients with HSCR, five patients with intestinal neuronal dysplasia (IND), five patients with IAS achalasia, and two patients with hypoganglionosis. Specimens also were taken from five normal controls. The specimens were examined using neural cell adhesion molecule (NCAM) immunohistochemistry, NADPH-diaphorase histochemistry, and acetylcholinesterase (AChE) histochemistry. The number of AChE-positive nerve fibers was markedly increased in the IAS of patients with HSCR, IND, and IAS achalasia compared with controls. NCAM and NADPH-diaphorase activity was absent or markedly reduced in the IAS of patients with HSCR, IND, and IAS achalasia. The IAS of patients with hypoganglionosis show markedly reduced NCAM and NADPH-diaphorase activity and occasional AChE-positive nerve fibers. These findings show that patients with HSCR, IND, hypoganglionosis, or IAS achalasia have abnormal innervation of the IAS and this may contribute to disturbances in gut motility in these conditions.
Acetylcholinesterase↗
NADPH-diaphorase histochemistry: a reliable test for the intraoperative diagnosis of Hirschsprung's disease.
Seromuscular biopsies were obtained from eight cases, at various levels, during a Swanson pull-through operation. The specimens were examined by using NADPH-diaphorase histochemistry. In the ganglionic segment of bowel, there were a large number of NADPH-diaphorase-positive nerve fibers in the circular and longitudinal muscles, and the myenteric ganglia stained strongly. In the transitional segment of bowel, there were a few NADPH-diaphorase-positive nerve fibers in the circular and longitudinal muscles, and the myenteric ganglion cells were smaller. In the aganglionic segment of bowel, there was a lack of expression of NADPH-diaphorase in the circular and longitudinal muscles. Hypertrophied nerve trunks in the myenteric plexus were stained. NADPH-diaphorase histochemistry is a simple, reliable, and rapid method for the intraoperative evaluation of the aganglionic segment of bowel. This technique allows intraoperative demonstration of innervation patterns of smooth muscle in normal and HD bowel, and provides accurate identification of ganglion cells in the ganglionic segment.
Biomarkers↗
Defective neutrophil actin polymerisation and chemotaxis in stressed newborns.
Abnormalities of polymorphonuclear leukocyte (PMN) function contribute to high rates of postoperative infection in the newborn and to the vulnerability of newborns to overwhelming bacterial and fungal sepsis. The authors investigated (1) the effects of major surgery and sepsis on PMN chemotaxis in the newborn and (2) the role of cytoskeletal rearrangements in regulating chemotaxis. The subjects studied included newborns with sepsis (n = 16), newborns who underwent major surgery (n = 7), healthy full-term newborns (n = 21), and healthy adult volunteers (n = 28). Peak actin polymerisation was diminished in all newborns (relative to the adults) after stimulation with formyl methionyl leucyl phenylalanine (FMLP) (10 nmol/L), and with zymosan activated serum (ZAS) (10%). Major surgery and sepsis in newborns caused no further reduction in actin polymerisation. Changes in PMN shape after stimulation with FMLP were reduced in the newborns. PMN chemotaxis was significantly lower in healthy newborns than in adults (17 +/- 4 microns v 24 +/- 5 microns; P < .0001) and was even lower in septic newborns (11 +/- 4 microns; P < .005). Surgery and anaesthesia did not alter chemotaxis.
Actins↗
Is intestinal neuronal dysplasia a disorder of the neuromuscular junction?
Intestinal neuronal dysplasia (IND) is a malformation of the enteric plexus and clinically resembles Hirschsprung's disease. The pathogenesis of IND is not known. There is no information available regarding the nature of the neuromuscular junction in this disease. The authors examined immunohistochemically full-thickness biopsy specimens from 14 patients (aged 3 weeks to 26 months) with IND and 10 age-matched controls, using monoclonal antibodies to growth associated protein-43 (GAP-43), synaptophysin, and neural-cell adhesion molecule (NCAM) as neuromuscular junction markers. In the normal bowel, GAP-43, synaptophysin, and NCAM immunoreactivity was abundant in the submucous and myenteric plexuses as well as in the muscularis mucosae and circular and longitudinal muscle layers. However, of the biopsy specimens from patients with IND, five had absence of GAP-43, synaptophysin, and NCAM immunoreactivity in muscularis muscosae and the circular and longitudinal muscle layers; five had no immunoreactivity in the longitudinal muscle; and four had no immunoreactivity in the muscularis mucosae. The submucous and myenteric plexuses of all patients with IND displayed strong immunoreactivity for GAP-43, synaptophysin, and NCAM. The findings demonstrate that patients with IND have defective innervation of the neuromuscular junction of the affected bowel.
Biomarkers↗
Novel mutations of the endothelin-B receptor gene in isolated patients with Hirschsprung's disease.
Hirschsprung's disease (HSCR) is characterized by the absence of autonomic ganglion cells in the terminal bowel and is a relatively common cause of intestinal obstruction in the newborn. The incidence of HSCR is estimated to be 1 in 5000 live births. Recently, the endothelin-B receptor (EDNRB) gene has been shown as a susceptibility gene for HSCR by the production of aganglionic colon in mice with a null mutation of this gene and by demonstrating a missense mutation in a large inbred kindred with a high incidence of HSCR (Mennonite pedigree). However, no further mutations have been demonstrated in other clinical cases. We analysed alterations of the EDNRB gene in 41 isolated patients of HSCR. Two novel mutations were detected: a G to A transition at nucleotide 824 and an insertion of T at nucleotide 878. Both mutations resulted in stop codons, predicted to produce a truncated and non-functional endothelin-B receptor. These observations indicate that dysfunction or loss of function of endothelin-B receptor may be involved in the aetiology of some isolated patients with HSCR.
Codon↗
Intestinal neuronal dysplasia in twins.
Intestinal neuronal dysplasia (IND) has been described proximal to the aganglionic segment in Hirschsprung's disease and less frequently as an isolated condition. Familial occurrence of IND is rare. We report a case of monozygotic twins affected with IND. One-year-old male monozygotic twins presented with a history of chronic constipation and abdominal distension since birth. At presentation, both twins had fecal impaction with palpable fecal-filled loops of bowel. Barium enema in both patients showed gross fecal loading of the sigmoid colon and rectum. Delayed films taken at 48 h showed marked barium retention. Suction rectal biopsy specimens examined by acetylcholinesterase (AChE) histochemistry in both patients showed moderately increased AChE-positive nerve fibers in the lamina propria, muscularis mucosae, and around submucosal blood vessels, as well as hyperplasia of submucous plexus with formation of giant ganglia. Both children underwent extended internal sphincter myectomy. Six months later, both patients had normal bowel function. The occurrence of IND in twins suggests that genetic factors are involved in the etiology of this disease.
Acetylcholinesterase↗
Endoscopic correction of vesicoureteral reflux secondary to posterior urethral valves.
PURPOSE: We review our experience with children with high grade vesicoureteral reflux secondary to posterior urethral valves treated endoscopically with subureteral polytetrafluoroethylene (Teflon) injection. MATERIALS AND METHODS: Between 1985 and 1994, 54 infants and children were diagnosed with posterior urethral valves of whom 38 (70%) had vesicoureteral reflux. In 9 patients reflux resolved after valve ablation. Seven patients underwent nephroureterectomy for nonfunctioning kidneys and in 2 reflux was treated with continuous chemoprophylaxis. The remaining 20 patients underwent an antireflux procedure, including reimplantation of the ureters in 5 and subureteral polytetrafluoroethylene injection in 15. Of the 15 patients who underwent injection 6 had unilateral and 9 had bilateral vesicoureteral reflux. According to the international classification reflux was grade IV in 6 ureters and grade V in 18. Patient age at injection ranged from 8 months to 6 years (mean 2.2 years). RESULTS: Reflux was eliminated in 17 ureters after a single subureteral polytetrafluoroethylene injection. Five ureters required 2 injections and 2 ureters required 3 to correct reflux. Followup of the 15 patients ranges from 1 to 10 years. Followup voiding cystography in the 15 patients (24 ureters) showed no evidence of recurrence of reflux. No clinically untoward effects were noted in these children from the use of polytetrafluoroethylene as an injectable material. CONCLUSIONS: Subureteral polytetrafluoroethylene injection is a simple and effective endoscopic alternative to open surgery for the management of high grade vesicoureteral reflux secondary to posterior urethral valves.
Child↗
Splenic and intestinal lymphocyte proliferation response in mice fed milk or yogurt and challenged with Salmonella typhimurium.
Two groups of 4-5 week old DBA/2J Nii mice were put on either a yogurt-based (n = 33) or a milk-based (n = 32) diet for a period of 4 weeks. At the end of the feeding trial one sub group of mice each from the two dietary groups was sacrificed for assessment of immune response. The remaining mice were challenged intragastrically with 2 x 10(10) live Salmonella typhimurium organisms and continued on their respective diets for 8 days after which they were also sacrificed. The immune response was measured by tritiated thymidine uptake by splenic or intestinal lymphocytes in response to the mitogens concanavalin A (Con A), Phytohaemaggutinin (PHA), and Lipopolysaccharide from Escherichia coli (LPS). Serum Immunoglobulin A levels were also estimated. Feed efficiency, measured as weight gain per unit energy intake, was significantly higher for the yogurt diet than for the milk diet. The mitogenic response of splenic and intestinal lymphocytes in the two groups of unchallenged mice was not different. In the Salmonella-challenged mice the stimulation index (SI) of splenic lymphocytes from yogurt-fed mice (mean +/- SD) was significantly higher (P = 0.001) in response to Con A (24.71 +/- 3.40) than that of milk-fed mice (15.85 +/- 2.09). Further, in these mice the SI of intestinal lymphocytes from yogurt-fed mice was higher than that of milk-fed mice in response to Con A (7.35 +/- 0.61 vs 5.65 +/- 0.78, P = 0.016) and LPS (9.04 +/- 0.93 vs 6.15 +/- 1.32, P = 0.016). Serum IgA levels in Salmonella-challenged mice were significantly higher 8 days after the challenge in the yogurt-fed group than in the milk-fed group (P < 0.001). The experiments indicate an improvement in local gastrointestinal as well as systemic immunity on a yogurt diet as compared to a milk diet.
Animals↗
Orbital myocysticercosis presenting as subconjunctival abscess.
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Abscess↗
Induction of interleukin-1 receptor antagonist (IL-1ra) following surgery is associated with major trauma.
Injury and trauma are major inducers of the acute-phase response. Among the major cytokine mediators of this response is interleukin-6, which is considered to be an early indicator of tissue damage following trauma. We have previously reported, in a group of children undergoing a single abdominal surgical procedure, the early induction of interleukin-1 receptor antagonist following the commencement of surgery. In the present study, we investigated the production of cytokines IL-1ra, IL-1 beta, and IL-6 in patients undergoing a range of surgical procedures to examine whether IL-1ra release is a general phenomenon or is restricted to certain categories of surgery. Peripheral blood mononuclear cells and polymorphonuclear leukocytes from patients were studied as a possible source of induced IL-1ra. IL-1ra and IL-6 were induced in 44 and 53 of the 73 patients, respectively. Induction of these cytokines was associated with major operative procedures of the abdomen and thorax and in hip replacement. Levels of these two cytokines varied widely within the different surgical categories. IL-1ra reached maximum levels before IL-6 in 18 patients and at the same time in 20 patients. IL-1 beta levels were induced in only 6 patients. Endotoxin levels were not detected in association with induction of IL-1ra. IL-1ra was not upregulated in peripheral blood mononuclear cells or polymorphonuclear leukocytes obtained from patients following surgery suggesting that these cells are not the source of plasma IL-1ra induced following trauma. These results provide new insights into the regulation of IL-1ra in vivo in humans. They show that IL-1ra can be induced as an early-response cytokine following major trauma in the absence of an infectious etiology.
Adolescent↗
Overexpression of intercellular adhesion molecule-1 (ICAM-1) and MHC class II antigen on hypertrophic nerve trunks suggests an immunopathologic response in Hirschsprung's disease.
The pathogenesis of Hirschsprung's disease (HD) is not fully understood. The authors studied expression of intercellular adhesion molecule-1 (ICAM-1) and major histocompatibility complex (MHC) class II antigen in the resected bowel specimens of 18 patients with HD who had no evidence of enterocolitis and in eight age- and site-matched controls, using indirect immunohistochemistry. There was strong expression of ICAM-1 and MHC class II antigen on hypertrophic nerve trunks, in both the submucous and myenteric plexuses of the aganglionic colon. The transition zone showed strong expression of ICAM-1 and MHC class II antigen on small ganglia in the myenteric and submucous plexuses. However, no staining of ganglia or nerve fibers was found in the submucous and myenteric plexuses of the colon from controls or in the ganglionic colon from patients with HD. The expression of both antigens on hypertrophic nerve trunks suggests the presence of an immunologic response in the pathogenesis of HD.
Adolescent↗