Nutritional and pharmacological strategy in children with short bowel syndrome.

Short bowel syndrome in neonates is a severe and life-threatening disease after a major loss of small bowel with or without large bowel. Intestinal adaptation, by which the organism tries to restore digestive and absorptive capacities, is entirely dependent on stimulation of the active enterocytes by enteral nutrition. This review summarizes recent knowledge about the pathophysiologic consequences after the loss of different intestinal parts and outlines the options for enteral nutrition and pharmacological therapies to support the adaptation process.

Expanding intestinal segment using osmotic hydrogel: An in vivo study.

Intestinal circumferential expansion is essential for bowel lengthening in patients with Short Bowel Syndrome. We hypothesized use of an endoluminal osmotic hydrogel expander (EOHE) as a novel approach for intestinal expansion. An EOHE was introduced into an isolated intestinal segment of New Zealand rabbits, with a similar segment created as a control.

Heparan sulfate phage display antibodies recognise epitopes defined by a combination of sugar sequence and cation binding.

Phage display antibodies are widely used to follow heparan sulfate (HS) expression in tissues and cells. We demonstrate by ELISA, that cations alter phage display antibody binding profiles to HS and this is mediated by changes in polysaccharide conformation, demonstrated by circular dichroism spectroscopy. Native HS structures, expressed on the cell surfaces of neuroblastoma and fibroblast cells, also exhibited altered antibody binding profiles following exposure to low mM concentrations of these cations.

MYCN-dependent expression of sulfatase-2 regulates neuroblastoma cell survival.

Heparan sulfate proteoglycans (HSPG) play a critical role in the interaction of tumor cells and their microenvironment. HSPG activity is dictated by sulfation patterns controlled by sulfotransferases, which add sulfate groups, and sulfatases (Sulf), which remove 6-O-sulfates. Here, we report altered expression of these enzymes in human neuroblastoma cells with higher levels of Sulf-2 expression, a specific feature of MYCN-amplified cells (MYCN-A cells) that represent a particularly aggressive subclass.

Enhancing safety of laparoscopic vascular control for neonatal sacrococcygeal teratoma.

Life-threatening bleeding is a hazard of major tumor excision in children. However, fatalities from inadvertent arterial ligation should not be overlooked. Sacrococcygeal teratoma is the commonest neonatal tumor.

Elective suprarenal and infrarenal cavectomy for excision of giant retroperitoneal teratoma in infancy.

Retroperitoneal teratomas are rare, often massive tumors usually presenting in infancy; being mostly mature lesions, their treatment is surgical but may represent a formidable challenge. Major vessel displacement may not be well demonstrated on imaging: vascular injuries are well-recognized surgical complications with urgent repair, ligation, or even segmental excision of major vessels being required. However, the literature provides few suggestions to avoid these problems.

Laparoscopic excision of a retroperitoneal lymphatic malformation in a newborn.

Abdominal lymphatic malformations may be challenging to eradicate. Retroperitoneal lesions may more difficult to resect than mesenteric ones; however, the latter may predispose to intestinal volvulus, leading to calls for their prompt excision. Such lesions identified perinatally may pose particular challenges: in one case, respiratory failure caused by abdominal distension required emergency drainage followed by later laparoscopic excision; laparoscopy has also been used promptly to diagnose and resect neonatal mesenteric lymphatic malformations with their inherent volvulus risk.

Excision of extensive metastatic dysgerminoma to control refractory hypercalcaemia in a child at high risk for tumour-lysis syndrome.

Hypercalcaemia is a rare life-threatening complication of paediatric cancer that is commoner in haematological than solid malignancies and associated rarely with acute renal failure. Often refractory to medical therapy, control of hypercalcaemia in children with solid tumours may necessitate excision of localised tumours or urgent chemotherapy for metastatic ones. We present a child with refractory hypercalcaemia, bulky chemosensitive metastatic tumours and acute renal failure in whom chemotherapy posed high-risk of tumour lysis syndrome (TLS).

Excision of ganglioneuroma from skull base to aortic arch.

High retropharyngeal neuroblastic tumors in children have been excised and debulked transorally or cervically, often with a covering tracheostomy. Although we and others have approached high thoracic lesions thoracoscopically, the trapdoor incision (or modification thereof) is generally reserved for cervicothoracic tumors with significant vessel encasement around the thoracic inlet. We report a case of symptomatic ganglioneuroma extending from the nasopharynx, at the level of the skull base, down to the aortic arch: macroscopic clearance was achieved via an extended trapdoor incision and without recourse to tracheostomy, transoral surgery, or transfusion.

Association of transforming growth factor-beta1 gene polymorphism with familial vesicoureteral reflux.

Purpose: Familial clustering of vesicoureteral reflux suggests that genetic factors have an important role in the pathogenesis of vesicoureteral reflux. Transforming growth factor-beta1 is a multifunctional peptide that controls proliferation and differentiation in many cell types. Recently an association between the transforming growth factor-beta1 -509 and +869 gene polymorphism, and renal parenchymal scarring was reported.

Association of transforming growth factor-beta1 gene polymorphism with reflux nephropathy.

Purpose: Reflux nephropathy (RN) is recognized as a major cause of end stage renal failure in children and young adults. Transforming growth factor-beta1 (TGF-beta1) is a potent proinflammatory and fibrogenetic cytokine known to have a key role in the regulation of renal tissue fibrosis. We investigate genotype frequencies for polymorphisms of the TGF-beta1 gene at position -509, codon 10 and 25, and examine circulating levels of TGF-beta1 in patients with reflux nephropathy.

Expression of vasoactive mediators during mechanical ventilation in nitrofen-induced diaphragmatic hernia in rats.

The high mortality in patients with congenital diaphragmatic hernia (CDH) has been attributed to pulmonary hypoplasia and persistent pulmonary hypertension (PPH). Endothelin-1 (ET-1), nitric oxide (NO), and calcitonin gene-related peptide (CGRP) have been reported to be important vasoactive mediators in the perinatal pulmonary circulation. The exact mechanism by which these vasoactive mediators interact to regulate the perinatal pulmonary vascular tone in CDH with PPH is not fully understood.

The significance of serum erythropoietin levels in assessing the severity of renal damage in children with reflux nephropathy.

Purpose: Erythropoietin (EPO) is the principal factor regulating red blood cell production in humans. It has been shown that EPO gradually decreases with the progression of diabetic nephropathy and may be used as a marker of severity of disease. In vitro studies have shown that interleukin-10 (IL-10) acts synergistically with EPO to increase stimulation of erythroid differentiation and proliferation.

Tumor necrosis factor-alpha gene polymorphism in reflux nephropathy.

Purpose: Interstitial scarring contributes to the progression of renal failure in reflux nephropathy. The proinflammatory cytokine tumor necrosis factor-alpha (TNF-alpha) has been implicated in the disease susceptibility and pathogenesis of several inflammatory diseases promoting interstitial infiltration of inflammatory cells. We evaluate the frequency of TNF-alpha gene polymorphism in patients with reflux nephropathy.

The role of nitric oxide in bladder urothelial injury after bladder outlet obstruction.

Objective: To investigate the role of nitric oxide (NO) in the pathogenesis of injury to the bladder mucosa after bladder outlet obstruction (BOO).

Materials And Methods: The response of bladder mucosa to BOO consists of thickening with fibrous connective tissue; NO is a multipurpose messenger important in blood vessel relaxation, neuronal communication and inflammatory activities of macrophages, and is synthesized by NO synthase (NOS), with three distinct isoforms; inducible (iNOS), endothelial (eNOS) and neuronal (nNOS). Fifteen male guinea pigs had a silk ligature placed around the bladder neck to induce BOO; controls included five sham-operated animals.

Interstitial cells of Cajal in the human normal urinary bladder and in the bladder of patients with megacystis-microcolon intestinal hypoperistalsis syndrome.

Objective: To investigate the distribution of c-kit-positive interstitial cells of Cajal (ICCs) in normal bladder and bladders from patients with megacystis-microcolon-intestinal peristalsis syndrome (MMIHS, a rare congenital and generally fatal cause of functional intestinal obstruction in the newborn), the most characteristic feature of which is abdominal distension caused by a distended unobstructed urinary bladder.

Patients And Methods: Full-thickness bladder specimens were obtained from four infants with MMIHS and four controls, and processed as paraffin-wax and frozen sections. Sections were assessed using single immunohistochemistry with monoclonal and polyclonal anti-c-kit antibodies.

Decreased expression of voltage-gated K+ channels in pulmonary artery smooth muscles cells in nitrofen-induced congenital diaphragmatic hernia in rats.

The newborn with congenital diaphragmatic hernia (CDH) is at high risk of developing persistent pulmonary hypertension (PPH). Recently, smooth muscle K(+) channels have been implicated in hypoxic pulmonary vasoconstriction in adults. We hypothesized that the hyperreactivity of the newborn pulmonary vasculature in CDH might reflect a relatively low level of smooth muscle K(+) channel activity because of hypoxemia, which could give rise to excessive smooth muscle cell depolarisation and lead to failure of the pulmonary vasculature to adapt to extrauterine life.

Increased expression of EGFR and TGF-alpha in segmental renal dysplasia in duplex kidney.

Renal dysplasia (RD) is a disorganised development of renal parenchyma that results in a deficit of functional renal tissue. It is known that the epidermal growth factor (EGF) and the transforming growth factor-alpha (TGF-alpha) enhance renal cell proliferation, migration and differentiation during kidney development through binding to the same EGF receptor (EGFR). The aim of the study was to analyse the expression of TGF-alpha and EGFR in the dysplastic kidney.

Genetic polymorphisms of angiotensin system genes in congenital diaphragmatic hernia associated with persistent pulmonary hypertension.

Background/purpose: The renin-angiotensin system plays an important role in pulmonary artery remodelling. Several polymorphisms of genes encoding for components of the renin angiotensin system such as the angiotensin converting enzyme (ACE), the angiotensinogen (AGT) gene, and the angiotensin II type 1 receptor (ATIR) have been associated with the development of pulmonary hypertension. The aim of this study was to investigate the ACE I/D genotype, the M235 T polymorphism of the AGT gene and the A1166 C polymorphism of AT1R in the lungs of congenital diaphragmatic hernia (CDH) complicated by persistent pulmonary hypertension (PPH) in the newborn.

Upregulated expression of EGF and TGF-alpha in the proximal respiratory epithelium in the human hypoplastic lung in congenital diaphragmatic hernia.

Newborn infants with congenital diaphragmatic hernia (CDH) still have a high mortality rate. Epidermal growth factor (EGF) and transforming growth factor-alpha (TGF-alpha) are peptide growth factors involved in the fetal lung growth and development. The EGF and TGF-alpha have been reported to promote pulmonary branching activity and alveolar type-II pneumocyte proliferation.

Increased expression of mast cells in reflux nephropathy.

End-stage renal disease is characterized by interstitial infiltrate of inflammatory cells in association with tubular atrophy and interstitial fibrosis. Mast cells (MCs) secrete a large number of fibrogenic factors and have been implicated in chronic inflammatory processes with fibrous tissue deposition. The aim of this study was to investigate the distribution of MCs in kidneys with reflux nephropathy (RN) and to determine the relationship between MCs and the interstitial fibrotic process in RN.

Altered expression of interstitial cells of Cajal in congenital ureteropelvic junction obstruction.

Purpose: Peristaltic contractions in the upper urinary tract serve to move urine from the kidney through the ureter to the bladder. Ureteropelvic junction (UPJ) obstruction is the most common cause of congenital hydronephrosis in children. To our knowledge the pathophysiology of UPJ obstruction is unknown.

Cyclooxygenase-2 up-regulation in reflux nephropathy.

Purpose: Reflux nephropathy (RN) is a major cause of end stage renal failure in children and hypertension is a frequent complication. Cyclooxygenase-2 (COX-2) is an enzyme responsible for the prostaglandin synthesis. It has been shown that COX-2 up-regulates renin production leading to renovascular hypertension.

Distribution of heme oxygenase-2 in nerves and interstitial cells of Cajal in the normal pylorus and in infantile hypertrophic pyloric stenosis.

Context: Interstitial cells of Cajal (ICCs) are pacemaker cells, which are of fundamental importance in regulating gastrointestinal motility. Recent evidence suggests that carbon monoxide is a neurotransmitter involved in neurotransmission between ICC and smooth muscle cells. Heme oxygenase-2 (HO-2) is the major physiological mechanism for the generation of carbon monoxide in the enteric nervous system.

Distribution of interstitial cells of Cajal in the internal anal sphincter of patients with internal anal sphincter achalasia and Hirschsprung disease.

Context: Interstitial cells of Cajal (ICCs) are pacemaker cells in the smooth muscles of the gut. The internal anal sphincter (IAS) is the most caudal part of gastrointestinal tract. It has the important function of maintaining fecal continence.