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Article: A Congenital Portosystemic Shunt in a Child With Heterotaxy, Situs Inversus, Polysplenia, and Interrupted Inferior Vena Cava With Azygous Continuation.

Carvajal, Victoria / Reddy, Saigopala / Gopalareddy, Vani / Bean, Adrienne / Wallis, Gonzalo

2023 Volume 10, Issue 12, Page(s) e01201

Abstract: Congenital portosystemic shunts are rare vascular malformations in which portal venous blood from the intestines and spleen bypasses the liver and diverts directly into the systemic circulation through abnormal vessels. We report a case of a 4-year-old ... ...

Abstract	Congenital portosystemic shunts are rare vascular malformations in which portal venous blood from the intestines and spleen bypasses the liver and diverts directly into the systemic circulation through abnormal vessels. We report a case of a 4-year-old girl with heterotaxy syndrome, polysplenia, and situs inversus presenting with persistent hypoxemia who was found to have pulmonary arteriovenous malformations (PAVMs) and hypoxemia secondary to a congenital portosystemic shunt. Management of this patient's PAVMs involved endovascular occlusion of the portosystemic shunt with subsequent resolution of hypoxemia. PAVMs secondary to extrahepatic portosystemic shunt should be explored as a cause of progressive cyanosis in children with heterotaxy, polysplenia, and interrupted inferior vena cava with azygous continuation.
Language	English
Publishing date	2023-12-04
Publishing country	United States
Document type	Case Reports
ZDB-ID	2814825-3
ISSN	2326-3253
ISSN	2326-3253
DOI	10.14309/crj.0000000000001201
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article: Successful Salvage of an Extracardiac Fontan in the Setting of Purulent Mediastinitis using Antibiotic-Impregnated Beads.

Coghill, Matthew T / Wallis, Gonzalo A / Kirshbom, Paul M / Maxey, Thomas S

Journal of pediatric intensive care

2018 Volume 7, Issue 3, Page(s) 163–165

Abstract: Post-cardiotomy mediastinitis is an especially serious complication after the implantation of prosthetic vascular grafts. Standard of care is irrigation, debridement, and removal of all prosthetic material present in the surgical field. The use of ... ...

Abstract	Post-cardiotomy mediastinitis is an especially serious complication after the implantation of prosthetic vascular grafts. Standard of care is irrigation, debridement, and removal of all prosthetic material present in the surgical field. The use of antibiotic impregnated beads at the site of infection has been reported in the salvage of vascular grafts in the adult population. We present the case of a 3-year-old child with hypoplastic left heart syndrome who developed mediastinitis following the Fontan operation. In a nontraditional approach, the Fontan conduit, which was surrounded by gross purulence, was successfully salvaged with the adjunctive use of vancomycin-impregnated beads.
Language	English
Publishing date	2018-02-06
Publishing country	Germany
Document type	Case Reports
ISSN	2146-4618
ISSN	2146-4618
DOI	10.1055/s-0038-1624562
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Article ; Online: Identification of patient variables that are associated with ventricular end-diastolic pressure before the bidirectional Glenn operation.

Schwartz, Matthew C / Karunanandaa, Aravinth / Anderson, William E / Herlong, J Rene / Paolillo, Joseph / Wallis, Gonzalo / Kirshbom, Paul / Maxey, Thomas S

Cardiology in the young

2021 Volume 31, Issue 10, Page(s) 1644–1650

Abstract: Introduction: Systemic ventricular end-diastolic pressure is important in patients with single ventricle heart disease. Predictors of an elevated systemic ventricular end-diastolic pressure prior to bidirectional Glenn operation have been incompletely ... ...

Abstract	Introduction: Systemic ventricular end-diastolic pressure is important in patients with single ventricle heart disease. Predictors of an elevated systemic ventricular end-diastolic pressure prior to bidirectional Glenn operation have been incompletely identified. Methods: All patients who underwent bidirectional Glenn operation operation at our centre between January 2007 and March 2017 were retrospectively identified and patient variables were extracted. For patients who had undergone Fontan operation at the time of this study, post-Fontan patient variables were also extracted. Results: One-hundred patients were included with a median age at pre-bidirectional Glenn operation catheterisation of 4.5 months. In total, 71 (71%) patients had a systemic right ventricle. At the pre-bidirectional Glenn operation catheterisation, the mean systemic ventricular end-diastolic pressure was higher amongst those with systemic right ventricle compared to left ventricle (9.1 mmHg ± 2.1 versus 7.7 ± 2.7 mmHg, p < 0.01). On univariate analysis, pre-bidirectional Glenn operation systemic ventricular end-diastolic pressure was positively associated with the presence of a systemic right ventricle (p < 0.01), history of recoarctation (p = 0.03), history of Norwood operation (p = 0.04), and ventricular systolic pressure (p < 0.01). On multivariate analysis, systemic ventricular end-diastolic pressure was positively associated with the presence of a systemic right ventricle (p < 0.01) and ventricular systolic pressure (p < 0.01). Amongst those who had undergone Fontan operation at the time of study (n = 49), those with a higher pre-bidirectional Glenn operation systemic ventricular end-diastolic pressure were more likely to have experienced death, transplantation, or listed for transplantation (p = 0.02) and more likely to have had heart failure symptoms (p = 0.04) at a mean time from Fontan of 5.2 years ± 1.3. Conclusions: In patients undergoing bidirectional Glenn operation operation, the volume-loaded, pre-bidirectional Glenn operation state may expose diastolic dysfunction that has prognostic value.
MeSH term(s)	Blood Pressure ; Fontan Procedure ; Heart Ventricles/diagnostic imaging ; Heart Ventricles/surgery ; Humans ; Infant ; Norwood Procedures ; Retrospective Studies ; Treatment Outcome ; Ventricular Pressure
Language	English
Publishing date	2021-03-09
Publishing country	England
Document type	Journal Article
ZDB-ID	1078466-4
ISSN	1467-1107 ; 1047-9511
ISSN (online)	1467-1107
ISSN	1047-9511
DOI	10.1017/S1047951121000810
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Article ; Online: Obesity and dyslipidemia predict cardiac allograft vasculopathy and graft loss in children and adolescents post-heart transplant: A PHTS multi-institutional analysis.

Bogle, Carmel / Cantor, Ryan / Koehl, Devin / Lochridge, Jillien / Kirklin, James K / Barnes, Aliessa / Wallis, Gonzalo / Amdani, Shahnawaz / Ameduri, Rebecca / Pahl, Elfriede / Simpson, Kathleen E / Blume, Elizabeth D

Pediatric transplantation

2022 Volume 26, Issue 5, Page(s) e14244

Abstract: Background: Obesity and dyslipidemia afflict children of all ages. We explored the prevalence of obesity and dyslipidemia in pediatric heart transplant (HT) recipients and its effects on cardiac allograft vasculopathy (CAV) and survival.: Methods: ... ...

Abstract	Background: Obesity and dyslipidemia afflict children of all ages. We explored the prevalence of obesity and dyslipidemia in pediatric heart transplant (HT) recipients and its effects on cardiac allograft vasculopathy (CAV) and survival. Methods: This study included primary HT recipients (≤18 years) transplanted between 01/1996 and 12/2018 included in the Pediatric Heart Transplant Society database. Obesity was categorized according to WHO/CDC guidelines and dyslipidemia according to the National Cholesterol Education Program. Kaplan-Meier analyses for CAV and graft loss stratified for BMI and lipid panels were generated and risk factors identified using multivariate analyses. Results: Among 6291 HT patients (median age [range] at HT = 4.3 [0.6-12.8] years; 45% Female; 68% White), 56% had a normal BMI at HT. Obese patients at HT had an increased risk for graft loss (HR 1.19, 95% CI 1.01-1.4, p = .04). Poor total cholesterol (TC), LDL-C, and TG were associated with the risk of both CAV (HR 1.79, p < .0001; HR 1.65, p = .0015; HR 1.53, p < .0001, respectively) and graft loss (HR 1.58, p = .0008; HR 1.22, p = .04; HR 1.43, p = .0007, respectively). Conclusions: Pediatric patients who are obese at the time of HT and dyslipidemic at 1 year post-HT are at an increased risk for CAV and graft loss. Preventative interventions may reduce morbidity and mortality among this cohort.
MeSH term(s)	Adolescent ; Allografts ; Child ; Child, Preschool ; Dyslipidemias/complications ; Dyslipidemias/epidemiology ; Female ; Graft Rejection/complications ; Graft Rejection/epidemiology ; Heart Diseases/etiology ; Heart Transplantation/adverse effects ; Humans ; Male ; Obesity/complications ; Retrospective Studies ; Risk Factors
Language	English
Publishing date	2022-02-05
Publishing country	Denmark
Document type	Journal Article ; Multicenter Study
ZDB-ID	1390284-2
ISSN	1399-3046 ; 1397-3142
ISSN (online)	1399-3046
ISSN	1397-3142
DOI	10.1111/petr.14244
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Article: Successful Salvage of an Extracardiac Fontan in the Setting of Purulent Mediastinitis using Antibiotic-Impregnated Beads

Coghill, Matthew T. / Wallis, Gonzalo A. / Kirshbom, Paul M. / Maxey, Thomas S.

Journal of Pediatric Intensive Care

2018 Volume 07, Issue 03, Page(s) 163–165

Abstract	Post-cardiotomy mediastinitis is an especially serious complication after the implantation of prosthetic vascular grafts. Standard of care is irrigation, debridement, and removal of all prosthetic material present in the surgical field. The use of antibiotic impregnated beads at the site of infection has been reported in the salvage of vascular grafts in the adult population. We present the case of a 3-year-old child with hypoplastic left heart syndrome who developed mediastinitis following the Fontan operation. In a nontraditional approach, the Fontan conduit, which was surrounded by gross purulence, was successfully salvaged with the adjunctive use of vancomycin-impregnated beads.
Keywords	mediastinitis ; Fontan ; salvage ; antibiotic beads
Language	English
Publishing date	2018-02-06
Publisher	Georg Thieme Verlag KG
Publishing place	Stuttgart ; New York
Document type	Article
ISSN	2146-4626 ; 2146-4618
ISSN (online)	2146-4626
ISSN	2146-4618
DOI	10.1055/s-0038-1624562
Database	Thieme publisher's database

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Article ; Online: Coarctation of the aorta in late adolescence.

Hall, David J / Wallis, Gonzalo A / Co-Vu, Jennifer G / Lossius, Michele N

The Journal of pediatrics

2013 Volume 162, Issue 3, Page(s) 646–646.e1

MeSH term(s)	Adolescent ; Angiography ; Aortic Coarctation/diagnosis ; Aortic Coarctation/diagnostic imaging ; Echocardiography ; Humans ; Male ; Tomography, X-Ray Computed
Language	English
Publishing date	2013-03
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	3102-1
ISSN	1097-6833 ; 0022-3476
ISSN (online)	1097-6833
ISSN	0022-3476
DOI	10.1016/j.jpeds.2012.09.022
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Article ; Online: Congenitally corrected transposition.

Wallis, Gonzalo A / Debich-Spicer, Diane / Anderson, Robert H

Orphanet journal of rare diseases

2011 Volume 6, Page(s) 22

Abstract: Congenitally corrected transposition is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculo-arterial connections, usually accompanied by other cardiovascular malformations. Incidence has been ... ...

Abstract	Congenitally corrected transposition is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculo-arterial connections, usually accompanied by other cardiovascular malformations. Incidence has been reported to be around 1/33,000 live births, accounting for approximately 0.05% of congenital heart malformations. Associated malformations may include interventricular communications, obstructions of the outlet from the morphologically left ventricle, and anomalies of the tricuspid valve. The clinical picture and age of onset depend on the associated malformations, with bradycardia, a single loud second heart sound and a heart murmur being the most common manifestations. In the rare cases where there are no associated malformations, congenitally corrected transposition can lead to progressive atrioventricular valvar regurgitation and failure of the systemic ventricle. The diagnosis can also be made late in life when the patient presents with complete heart block or cardiac failure. The etiology of congenitally corrected transposition is currently unknown, and with an increase in incidence among families with previous cases of congenitally corrected transposition reported. Diagnosis can be made by fetal echocardiography, but is more commonly made postnatally with a combination of clinical signs and echocardiography. The anatomical delineation can be further assessed by magnetic resonance imaging and catheterization. The differential diagnosis is centred on the assessing if the patient is presenting with isolated malformations, or as part of a spectrum. Surgical management consists of repair of the associated malformations, or redirection of the systemic and pulmonary venous return associated with an arterial switch procedure, the so-called double switch approach. Prognosis is defined by the associated malformations, and on the timing and approach to palliative surgical care.
MeSH term(s)	Congenitally Corrected Transposition of the Great Arteries ; Electrocardiography ; Humans ; Incidence ; Myocardium/pathology ; Transposition of Great Vessels/diagnosis ; Transposition of Great Vessels/epidemiology ; Transposition of Great Vessels/genetics ; Transposition of Great Vessels/pathology
Language	English
Publishing date	2011-05-14
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	2225857-7
ISSN	1750-1172 ; 1750-1172
ISSN (online)	1750-1172
ISSN	1750-1172
DOI	10.1186/1750-1172-6-22
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Article ; Online: Rapid resolution of colitis related to immunosuppressive medication after infliximab administration.

Coghill, Matthew / Howell, Mary / Wallis, Gonzalo / Dranove, Jason

Pediatric transplantation

2020 Volume 24, Issue 7, Page(s) e13754

Abstract: Immunosuppression is necessary after solid organ transplantation. The non-infectious side effects associated with many of these agents are not well understood. We report a case of colitis, most resembling inflammatory bowel disease, that persisted ... ...

Abstract	Immunosuppression is necessary after solid organ transplantation. The non-infectious side effects associated with many of these agents are not well understood. We report a case of colitis, most resembling inflammatory bowel disease, that persisted despite withdrawal of tacrolimus and mycophenolate mofetil and transition to alternative agents. The patient was treated for clostridium difficile without improvement. Endoscopic biopsies demonstrated non-specific inflammation without evidence of active infection. An extensive immunologic and oncologic workup was negative. Ultimately, we trialed the administration of infliximab, a monoclonal antibody that inhibits TNF-alpha receptors that is commonly used in the treatment of inflammatory bowel disease. With infliximab treatment, the patient experienced rapid resolution of his disease and has remained in remission.
MeSH term(s)	Antibiotics, Antineoplastic/adverse effects ; Antirheumatic Agents/administration & dosage ; Biopsy ; Child, Preschool ; Colitis/chemically induced ; Colitis/diagnosis ; Colitis/drug therapy ; Colonoscopy ; Drug Therapy, Combination/adverse effects ; Graft Rejection/prevention & control ; Heart Transplantation ; Humans ; Immunosuppressive Agents/adverse effects ; Infliximab/administration & dosage ; Male ; Mycophenolic Acid/adverse effects ; Tacrolimus/adverse effects
Chemical Substances	Antibiotics, Antineoplastic ; Antirheumatic Agents ; Immunosuppressive Agents ; Infliximab (B72HH48FLU) ; Mycophenolic Acid (HU9DX48N0T) ; Tacrolimus (WM0HAQ4WNM)
Language	English
Publishing date	2020-06-30
Publishing country	Denmark
Document type	Case Reports
ZDB-ID	1390284-2
ISSN	1399-3046 ; 1397-3142
ISSN (online)	1399-3046
ISSN	1397-3142
DOI	10.1111/petr.13754
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Zs.A 4947: Show issues

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Article ; Online: Cardiac allograft vasculopathy and graft failure in pediatric heart transplant recipients after rejection with severe hemodynamic compromise.

Kleinmahon, Jake A / Gralla, Jane / Kirk, Richard / Auerbach, Scott R / Henderson, Heather T / Wallis, Gonzalo A / Ramakrishnan, Karthik / Singh, Rakesh K / Caldwell, Randall L / Savage, Andrew J / Everitt, Melanie D

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation

2018 Volume 38, Issue 3, Page(s) 277–284

Abstract: Background: Rejection with severe hemodynamic compromise (RSHC) carries a mortality risk approaching 50%. We aimed to identify current risk factors for RSHC and predictors of graft failure after RSHC.: Methods: Data from 3,259 heart transplant (HT) ... ...

Abstract	Background: Rejection with severe hemodynamic compromise (RSHC) carries a mortality risk approaching 50%. We aimed to identify current risk factors for RSHC and predictors of graft failure after RSHC. Methods: Data from 3,259 heart transplant (HT) recipients between January 2005 and December 2015 in the Pediatric Heart Transplant Study (PHTS) were analyzed. Predictors for RSHC and outcome after RSHC were sought. Time to RSHC was analyzed using the Cox proportional hazards regression model. Cardiac allograft vasculopathy (CAV) after HT and CAV after RSHC were analyzed as time-dependent covariates. Timing of RSHC was analyzed as occurring before and after 4 years after RSHC. Results: There were 309 patients (9.5%) with ≥ 1 RSHC episodes. In 143 patients with RSHC, the first episode was within 1 year after HT. Independent risk factors for RSHC were age 1 to 5 years at HT (hazard ratio [HR], 1.51; 95% confidence interval [CI], 1.04-2.18), age > 10 years at HT (HR, 1.83; 95% CI, 1.29-2.60), black race (HR, 1.64; 95% CI, 1.25-2.15), prior cardiac surgery (HR, 1.55; 95% CI, 1.03-2.31), ventricular assist device support at HT (HR, 1.65; 95% CI, 1.18-2.29), maintenance steroids (HR, 1.39; 95% CI, 1.06-1.82), and recipient on inotropes, pressors, or thyroid hormones (HR, 1.45; 95% CI, 1.09-1.94). Graft survival at 5 years after RSHC was 45.7%. RSHC was a greater risk factor for earlier CAV (HR, 7.78; 95% CI, 5.82-10.40) than other rejection types (HR, 2.31; 95% CI, 1.79-3.00). Patients with late RSHC, after 1 year after RSHC had increased risk of graft loss 4 years after RSHC (HR, 7.12; 95% CI, 2.18-23.22). The 5-year graft survival after RSHC was 50.5% for early RSHC and 39.0% for late RSHC. Conclusions: Mortality after RSHC is high in the current treatment era. Many patient risk factors for RSHC cannot be modified, including age, race, prior cardiac surgery, and ventricular assist device support. After RSHC, CAV is the only predictor of graft failure. Patients who have late RSHC fare worse than those who have RSHC within the first year after HT.
MeSH term(s)	Child ; Child, Preschool ; Coronary Artery Disease/complications ; Coronary Artery Disease/epidemiology ; Coronary Artery Disease/physiopathology ; Coronary Vessels/pathology ; Female ; Graft Rejection/complications ; Graft Rejection/epidemiology ; Graft Rejection/physiopathology ; Heart Transplantation ; Hemodynamics ; Humans ; Hyperplasia/complications ; Hyperplasia/epidemiology ; Hyperplasia/physiopathology ; Infant ; Male ; Postoperative Complications/epidemiology ; Postoperative Complications/physiopathology ; Prognosis ; Prospective Studies ; Risk Factors ; Severity of Illness Index
Language	English
Publishing date	2018-12-21
Publishing country	United States
Document type	Journal Article ; Multicenter Study
ZDB-ID	1062522-7
ISSN	1557-3117 ; 1053-2498
ISSN (online)	1557-3117
ISSN	1053-2498
DOI	10.1016/j.healun.2018.12.011
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Article ; Online: Congenitally corrected transposition

Debich-Spicer Diane / Wallis Gonzalo A / Anderson Robert H

Orphanet Journal of Rare Diseases, Vol 6, Iss 1, p

2011 Volume 22

Abstract: Abstract Congenitally corrected transposition is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculo-arterial connections, usually accompanied by other cardiovascular malformations. Incidence has ... ...

Abstract	Abstract Congenitally corrected transposition is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculo-arterial connections, usually accompanied by other cardiovascular malformations. Incidence has been reported to be around 1/33,000 live births, accounting for approximately 0.05% of congenital heart malformations. Associated malformations may include interventricular communications, obstructions of the outlet from the morphologically left ventricle, and anomalies of the tricuspid valve. The clinical picture and age of onset depend on the associated malformations, with bradycardia, a single loud second heart sound and a heart murmur being the most common manifestations. In the rare cases where there are no associated malformations, congenitally corrected transposition can lead to progressive atrioventricular valvar regurgitation and failure of the systemic ventricle. The diagnosis can also be made late in life when the patient presents with complete heart block or cardiac failure. The etiology of congenitally corrected transposition is currently unknown, and with an increase in incidence among families with previous cases of congenitally corrected transposition reported. Diagnosis can be made by fetal echocardiography, but is more commonly made postnatally with a combination of clinical signs and echocardiography. The anatomical delineation can be further assessed by magnetic resonance imaging and catheterization. The differential diagnosis is centred on the assessing if the patient is presenting with isolated malformations, or as part of a spectrum. Surgical management consists of repair of the associated malformations, or redirection of the systemic and pulmonary venous return associated with an arterial switch procedure, the so-called double switch approach. Prognosis is defined by the associated malformations, and on the timing and approach to palliative surgical care.
Keywords	Medicine ; R
Subject code	610
Language	English
Publishing date	2011-05-01T00:00:00Z
Publisher	BMC
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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