Duct In Infants Research Articles

Cosmetic Results in Transumbilical Resection of Patent Vitellointestinal Duct in Infants.

Cosmetic Results in Transumbilical Resection of Patent Vitellointestinal Duct in Infants.

Differentiation Between Umbilical Granuloma and Patent Vitellointestinal Ducts in Infants and its Associated Complications- 8 Years Retrospective Study: Novel Approach

Umbilical granuloma is a very common cause of umbilical discharge. It is managed by chemical cauterization, Bipolar electric cauterization and sometimes by simple thread ligation. However, it must be differentiated from omphalo-mesenteric remnants otherwise it can lead to disastrous complications like prolapsed of ileum and subsequent gangrene of bowel. Ultrasonography is very important tool for differentiation between umbilical granuloma and ramnants of viltello-intestinal ducts or urachus. Umbilical granuloma is a very common cause of umbilical discharge. It is managed by chemical cauterization or simple thread ligation. However, it can be a differential diagnosis of patent vitello intestinal duct and this should be ruled out before managing patients.

Human amniotic fluid stem cells attenuate cholangiocyte apoptosis in a bile duct injury model of liver ductal organoids

PurposeBiliary atresia (BA) is a fibro-obliterative cholangiopathy that involves both extrahepatic and intrahepatic bile ducts in infants. Cholangiocyte apoptosis has an influence on the fibrogenesis process of bile ducts and the progression of liver fibrosis in BA. Human amniotic fluid stem cells (hAFSCs) are multipotent cells that have ability to inhibit cell apoptosis. We aimed to investigate whether hAFSCs have the potential to attenuate cholangiocyte apoptosis and injury induced fibrogenic response in our ex vivo bile duct injury model of liver ductal organoids. MethodsThe anti-apoptotic effect of hAFSCs was tested in the acetaminophen-induced injury model of neonatal mouse liver ductal organoids (AUP #42681) by using direct and indirect co-culture systems. Cell apoptosis and proliferation were evaluated by immunofluorescent staining. Expression of fibrogenic cytokines was analyzed by RT-qPCR. Data were compared using one-way ANOVA with post hoc test. ResultsIn our injury model, liver ductal organoids that were treated with hAFSCs in both direct and indirect co-culture systems had a significantly smaller number of apoptotic cholangiocytes and decreased expression of fibrogenic cytokines, transforming growth factor beta-1 (TGF-β1) and platelet-derived growth factor-BB (PDGF-BB). Moreover, hAFSCs increased cholangiocyte proliferation in injured organoids. ConclusionhAFSCs have the ability to protect the organoids from injury by decreasing cholangiocyte apoptosis and promoting cholangiocyte proliferation. This protective ability of hAFSCs leads to inhibition of the fibrogenic response in the injured organoids. hAFSCs have high therapeutic potential to attenuate liver fibrogenesis in cholangiopathic diseases such as BA.

Innate Immunity and Pathogenesis of Biliary Atresia.

Biliary atresia (BA) is a devastating fibro-inflammatory disease characterized by the obstruction of extrahepatic and intrahepatic bile ducts in infants that can have fatal consequences, when not treated in a timely manner. It is the most common indication of pediatric liver transplantation worldwide and the development of new therapies, to alleviate the need of surgical intervention, has been hindered due to its complexity and lack of understanding of the disease pathogenesis. For that reason, significant efforts have been made toward the development of experimental models and strategies to understand the etiology and disease mechanisms and to identify novel therapeutic targets. The only characterized model of BA, using a Rhesus Rotavirus Type A infection of newborn BALB/c mice, has enabled the identification of key cellular and molecular targets involved in epithelial injury and duct obstruction. However, the establishment of an unleashed chronic inflammation followed by a progressive pathological wound healing process remains poorly understood. Like T cells, macrophages can adopt different functional programs [pro-inflammatory (M1) and resolutive (M2) macrophages] and influence the surrounding cytokine environment and the cell response to injury. In this review, we provide an overview of the immunopathogenesis of BA, discuss the implication of innate immunity in the disease pathogenesis and highlight their suitability as therapeutic targets.

Transcatheter closure of elongated and pulmonary hypertensive patent arterial duct in infants using Amplatzer vascular plug II.

To evaluate the feasibility, efficacy, and safety of Amplatzer vascular plug II in large and elongated ducts in infants. Patent arterial duct device closure is technically challenging in infants with large and elongated ducts because Amplatzer duct occluder and Amplatzer duct occluder II have high chances of causing aortic coarctation and left pulmonary artery stenosis, respectively. The Amplatzer vascular plug II being soft with no retention discs on either sides helps in mitigating these problems. This is a prospective, observational study involving infants with clinical, echocardiographic and angiographic evidence of large left to right shunt. All the children underwent duct closure using Amplatzer vascular plug II. Eighteen infants qualified for the study. Mean age and weight were 8.63 ± 3.84 months and 6.3 ± 1.7 kg, respectively. The angiographic mean duct diameter at the pulmonary artery end was 4.66 ± 0.92 mm, and the mean duct length was 9.4 ± 2.48 mm. The size of Amplatzer vascular plug II used varied from 6 mm to 10 mm. Technical success was achieved in 16/18 cases. One patient had device embolisation, and in the other, the device was found to be unstable. The ratio of Amplatzer vascular plug II size to the duct diameter was 1.65 ± 0.27, while the ratio of ductal length to device length was 1.48 ± 0.46 in those with successful outcome. Amplatzer vascular plug II is a safe and effective option in appropriately selected infants with elongated ducts. Diameter and length of Amplatzer vascular plug II vis-a-vis those of the ductus are important determinants of the successful outcome.

Transcatheter Closure of Arterial Duct in Infants < 6 kg: Amplatzer Duct Occluder Type I vs Amplatzer Duct Occluder II Additional Sizes

Transcatheter closure of arterial duct (AD) remains challenging in low-weight patients and using Amplatzer Duct Occluder devices is still considered off-label in infants < 6kg. This study aimed to report a large, single-center experience of percutaneous AD closure in infants < 6kg as well as to compare the most frequently used devices, Amplatzer Duct Occluder type I (ADO I) and Amplatzer Duct Occluder type II Additional Sizes devices (ADO II-AS) (St. Jude Medical Corp, St. Paul, MN, USA). From March 2000 to March 2017, among the 762 patients submitted to percutaneous closure of AD at our Institution, 33 were infants < 6kg (age 4.8 ± 2.1months; weight 5.0 ± 0.9kg). Fourteen patients (45%) underwent ADO I (Group I) and 19 patients ADO II-AS (Group II) device implantation. AD diameter was 2.6 ± 0.8 (range 1.5-4.0) mm resulting in QP/QS of 2.6 ± 0.0.9 (range 1.1-4.5). Successful device deployment was achieved in all patients without procedural morbidity or mortality. Procedural and fluoroscopy times were not significantly different between the groups. However, total X-ray absorbed dose was significantly higher in Group I (121 ± 69 vs 29 ± 16mGy/cm2, p < 0.01). Immediate, 24h, and mid-term (46 ± 37 months, median 39) complete occlusion were recorded in 72.7, 90.9, and 97% of patients, respectively without significant difference between the groups. In conclusion, transcatheter closure of AD with Amplatzer Duct Occluder devices is feasible, safe, and effective also in infants less than 6kg, without significant difference between the most commonly used devices, namely, ADO I and ADO II-AS.

Immunolocalization of surfactant protein D in the liver from infants with cholestatic liver disease

PurposeSurfactant protein D (SP-D) is one of specific surfactant proteins constituting pulmonary surfactant. Recent studies have revealed that SP-D is detected in various non-pulmonary tissues and is involved in the host defense and immunomodulation. However, the relationship between SP-D and liver diseases has not yet been investigated. The aim of this study was to detect the immunolocalization of SP-D in the livers of infants with cholestatic liver disease. MethodsThe expression of immunoreactive SP-D was assessed in infants with cholestasis, including biliary atresia (BA, n=7), neonatal hepatitis (NH, n=2), and paucity of the intrahepatic bile duct (PIBD, n=4). Immunoreactive SP-D was also assessed in six infants who died of non-liver disease as controls. Tissue samples were obtained at liver biopsy, or by post-mortem sampling. The tissue sections were incubated with anti-SP-D polyclonal antibodies and were counterstained with hematoxylin. ResultsIn the normal livers, SP-D was detected in the intrahepatic bile ducts, but was not detected in hepatocytes. In contrast, intense SP-D staining was noted in the hepatocytes from infants with BA, NH, and PIBD. Although SP-D was detected in the intrahepatic bile ducts in the infants with NH, negative or weak staining was seen in the intrahepatic bile ducts in infants with BA. ConclusionOur data showed that SP-D is present in the bile ducts of the normal infant liver, and it was found to accumulate in the hepatocytes of cholestatic livers. These results suggest that SP-D is produced in hepatocytes and is secreted into the bile ducts.

Bile duct to portal space ratio and ductal plate remnants in liver disease of infants aged less than 1 year

To validate the bile duct to portal space ratio as an independent factor useful for the prognosis of neonatal liver disease. We assessed the maturation of the intrahepatic bile duct system (IBDS) in 87 consecutive infants aged less than 1 year undergoing non-subcapsular, adequate (at least six portal tracts), liver needle biopsies because of hepatomegaly and/or cholestasis. The maturation of the IBDS was evaluated by immunohistochemistry with an antibody directed to cytokeratin 7 (CK7), a biliary-type intermediate filament of the cytoskeleton, and a schema showing the IBDS remodelling. We used five categories to fit the different patterns of the IBDS remodelling using the ratio between the number of bile ducts and the number of portal tracts (BD/PT) and the presence of abnormal reaction patterns (marked intra-acinar pseudorosettes and/or periportal ductular proliferation): (A) abnormal reaction patterns with any BD/PT; (B) BD/PT = 0; (C) 0.1 < or = BD/PT < 0.5; (D) 0.5 < or = BD/PT < 0.9; and (E) BD/PT > 0.9 (B-E categories: no abnormal reaction patterns). Further, we evaluated cholestasis, portal fibrosis (PF), portal inflammation (PI), giant cell transformation (GCT), and extramedullary haematopoiesis (EMH). We identified A-E categories in 24, 14, 17, 8, and 24 biopsies, respectively. B and C categories were frequently observed in biliary atresia (BA), A category in neonatal hepatitis (NH), A-C categories in paucity of intrahepatic bile ducts (PIBD), and E category in 'other liver diseases' (OLD). Cholestasis, PI, GCT, and EMH were more frequent in A and C, while PF was variably seen in all categories. The lowest survival rate occurred in B (Kaplan-Meier estimator). (1) Biliary epithelial cell patterns recapitulate the primitive stages of the IBDS maturation; (2) abnormal reaction patterns occur mainly in NH, whilst BD/PT < 0.5 in BA; and (3) lack of intrahepatic bile ducts in infants aged less than 1 year is an adverse prognostic factor independent from aetiology of neonatal liver disease.

IS-012 Cystic dilatation of bile duct in infants : an entity closer to biliary atresia than choledochal cyst in older children?(International Session III,Better Life for Sick Children, Better Future for Pediatric Surgery,the 45th Annual Meeting of Japanese Society of Pediatric Surgeons)

IS-012 Cystic dilatation of bile duct in infants : an entity closer to biliary atresia than choledochal cyst in older children?(International Session III,Better Life for Sick Children, Better Future for Pediatric Surgery,the 45th Annual Meeting of Japanese Society of Pediatric Surgeons)

Initial clinical experience in transcatheter closure of large patent arterial ducts in infants using the modified and angled Amplatzler duct occluder

To establish the feasibility and efficacy of closing large patent arterial duct in infants, using the modified and angled variants of the Amplatzer duct occluder. Closure of large patent arterial ducts by inserting devices in sick and underweight infants, particularly those weighing around 5 kilograms, remains a challenge. Bigger devices require larger delivery sheaths and may cause obstruction either to the aorta or left pulmonary artery. Negotiating a large device is difficult or impossible, as the sheath gets kinked. Because of these problems, such underweight infants with large ducts who are failing to thrive, and in left ventricular failure with associated lesions, are typically referred for surgery, often leading to higher morbidity and mortality. We attempted to close such large patent arterial ducts using the new Amplatzer occluder, modified with single layer of polyester, and the angled occluder, with no polyester material, inserted through a specially braided kink-resistant sheath. Closure was achieved in 10 infants, with mean age of 8.2 months, mean weight of 5.5 kilograms, the lowest weighing 3.9 kilograms. The mean size of the patent ducts was 6.3 millimetres, with the largest measuring 8.6 millimetres. We implanted 6 modified and 4 angled occluders. In one patient, suffering from hydronephrosis, a 14/12 angled device embolized and was retrieved, but the patient died. In the remaining patients, all ducts were closed completely, with no obstruction to either the aorta or left pulmonary artery. On follow up, all showed excellent clinical improvement. Complete closure of very large patent arterial ducts is now possible, even in very sick and underweight infants, using the large but low profile custom-made angled or modified versions of the Amplatzer occluder.

Extrahepatic biliary atresia: A review of current management

Macroscopic examination of the extrahepatic bile ducts in infants with biliary atresia reveals fibrous occlusion of variable extent from an inflammatory process of unknown aetiology. Histological studies have shown that bile duct remnants at the porta hepatis frequently contain small epithelium-lined channels which communicate with intrahepatic ducts and through which effective bile drainage may be established by the operation of portoenterostomy. The 4-year survival rate in untreated cases is 2 per cent, but surgical treatment can improve the outlook and recent reports suggest that a 5-year survival rate of over 35 per cent can be achieved with portoenterostomies performed before 10 weeks of age. Complications after surgery include progressive liver disease, ascending bacterial cholangitis and portal hypertension.

Spontaneous perforation of the bile duct in infants

Spontaneous perforation of the bile duct is a rare but well documented condition of infants. Six infants are described, five presenting within 2 months of birth without apparent antecedent factors and one associated with an 'acquired' type I biliary atresia at 8 months. All infants underwent definitive surgery, which included decompression of the biliary tree with cholecystenterostomy or T tube drainage. There was no morbidity or mortality associated with such surgical intervention.

Amplatzer Angled Duct Occluder for Closure of Patent Ductus Arteriosus Larger than the Aorta in an Infant

Transcatheter closure of patent ductus arteriosus (PDA) by Amplatzer duct occluder is the treatment of choice. However, closure of very large ducts in infants with low weight is a challenge for the interventionalist because a large device may obstruct the aorta or left pulmonary artery. Difficulty is also encountered in advancing the device around the curve of the right ventricular outflow tract toward the pulmonary artery; this curve is tight, more or less at a right angle in infants, leading to kinking of the sheath, which increases fluoroscopic time. This is the first reported case of a very large PDA (8.7 mm), larger than the aorta (8.2 mm), successfully closed by an Amplatzer angled duct occluder in an infant weighing 5 kg.

Echocardiographic follow-up of percutaneous closure of the arterial duct in infants

SummarySince February 1990, we have attempted nonsurgical occlusion of persistent patency of the arterial duct using the Rashkind double-disk occluding device, of either 12 or 17 mm diameter. Results are presented from 31 patients (23 female and eight male, ages 15 months to 16 years). M-Mode, cross-sectional, Doppler and color Doppler echocardiographic studies were made prior to closure, in the following 24 hours, and six months later. Left ventricular diastolic dimension and shortening fraction, systolic intervals, and left atrium/aortic ratio were evaluated by M-Mode echo. We examined carefully the location of the device relative to the left pulmonary artery and descending aorta, also checking for disturbed flows and residual shunts. These studies showed a clear tendency for the left ventricular diameter to decrease, and revealed a significant normalization of the left atrium/aortic ratio. Successful closure of the duct was achieved in 27 cases. Follow-up studies six months later showed residual shunting in four cases. In one patient, a second device was implanted with an excellent result. At the immediate follow-up, turbulences were noted in the area of the device (nine cases) and in the left pulmonary artery (seven cases). Distortion of the anatomic orientation of the left pulmonary artery was observed in three cases in which we discovered 20 mm Hg systolic gradients. In our opinion, echocardiography is the best technique with which to follow-up these patients. The high sensitivity of color Doppler echocardiography revealed minimal distortion of the left pulmonary artery, probably generated by regional anatomic adjustment to the insertion of the device.

Balloon dilatation of the arterial duct in congenital heart disease

The systemic circulation of newborn infants with congenital left-heart obstruction is supplied from the right ventricle via a patent arterial duct between the pulmonary artery and descending aorta. The duct closes during the first few days of life, but infusion of prostaglandin E2 can prevent closure in some cases. We report four newborn infants (aged 3-8 days) with intractable heart failure due to severe obstruction of the left heart in the presence of a closing arterial duct. Infusion of prostaglandin E2 did not improve their clinical condition. Cardiac catheterisaton and balloon dilatation of their arterial ducts resulted in a dramatic improvement in the babies' clinical condition; during subsequent surgical repair of the infants' hearts, the arterial ducts were found to be widely patent. Balloon dilatation gives immediate and sustained wide patency of the arterial duct in infants who do not respond adequately to prostaglandin E2.

Ultrasonographic Examination of Intrapancreatic Part of the Bile Duct in Infants with Pancreaticobiliary Maljunction (PBM)

Ultrasonographic Examination of Intrapancreatic Part of the Bile Duct in Infants with Pancreaticobiliary Maljunction (PBM)

A Simple Aid to Successful Probing of Congenitally Obstructed Nasolacrimal Ducts

For successful probing of congenitally obstructed nasolacrimal ducts in infants, the probe must reach the inferior nasal meatus. Previous methods to determine the position of the probe in the nose require intranasal examination method using two probes of equal length. The first probe is placed inside the lacrimal outflow system and the second probe is placed against the nose. The second probe can be used to measure the distance between the point of the first probe and the anterior narial margin. This method may be particularly valuable in those cases in which the probe point is less than 5 mm below the anterior narial margin.

Manifestations of nonsuppurative cholangitis in chronic hepatobiliary diseases: morphologic spectrum, clinical correlations and terminology.

The features of nonsuppurative cholangitis were studied in liver biopsy specimens from 185 patients with chronic active hepatitis (CAH), 280 patients with primary biliary cirrhosis (PBC), and 55 patients with primary sclerosing cholangitis (PSC). Specimens from patients with other liver diseases in which the presence of nonsuppurative cholangitis had been recorded were also studied. We identified four types of nonsuppurative cholangitis: granulomatous cholangitis, lymphoid cholangitis, fibrous cholangitis, and pleomorphic cholangitis. Granulomatous cholangitis almost always seemed to be destructive; the other types were either destructive or nondestructive. Granulomatous cholangitis was, for all practical purposes, diagnostic of PBC and the obliterative form of fibrous cholangitis was similarly diagnostic for the hepatic manifestations of PSC in adults and paucity of intrahepatic bile ducts in infants. All other types of cholangitis were found in CAH, PBC, PSC, and other liver diseases. Thus, the term "nonsuppurative cholangitis" describes a spectrum of morphologic lesions that differ in incidence, morphogenesis, usefulness for liver biopsy diagnosis, and, probably, pathogenesis.

Anomalous Arrangement of Pancreatico-biliary Duct in Congenital Dilatation of Biliary Duct in Infants and Children

Anomalous Arrangement of Pancreatico-biliary Duct in Congenital Dilatation of Biliary Duct in Infants and Children

Abnormalities associated with orificial insufficiency of prostatic ducts in infants and children.

48 boys examined with urethrocystography on all together 129 occasions and shown to have orificial insufficiency of prostatic ducts were studied for associated for associated abnormalities. In 44 cases the insufficiency could be attributed to urinary infection complicating urethral obstruction or neurogenic dysfunction of the urinary bladder or to any one of these three abnormalities alone. The age at which the insufficiency occurred, the time it persisted, and the extent to which it involved the prostate were also studied.