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Article: Successful treatment of ulcerated hemangioma with diversion colostomy in a neonate with LUMBAR syndrome.

Shah, Ananya / Tollefson, Megha / Ahn, Edward S / Gibreel, Waleed / Polites, Stephanie

2024 Volume 2024, Issue 3, Page(s) rjae114

Abstract: We present the case of a 3-week-old girl with LUMBAR syndrome, a rare condition involving segmental infantile hemangiomas (IH) in the lumbosacral region, myelopathy, and other congenital anomalies. The patient developed severe ulceration of a left ... ...

Abstract	We present the case of a 3-week-old girl with LUMBAR syndrome, a rare condition involving segmental infantile hemangiomas (IH) in the lumbosacral region, myelopathy, and other congenital anomalies. The patient developed severe ulceration of a left buttock IH. Treatment included broad-spectrum antibiotics, debridement, and a laparoscopic temporary colostomy. Propranolol therapy, wound care, and fecal diversion led to successful healing of the ulceration. The report highlights the challenges of ulcerated IH in the perineal area due to exposure to urine and stool. The comprehensive approach resulted in positive outcomes, including the successful surgical treatment of spinal dysraphism, successful colostomy reversal, and developmental progress. This case contributes insight into the surgical management of severe perianal ulcerated IH associated with LUMBAR syndrome, highlighting the importance of a tailored surgical approach with a multidisciplinary framework.
Language	English
Publishing date	2024-03-07
Publishing country	England
Document type	Case Reports
ZDB-ID	2580919-2
ISSN	2042-8812
ISSN	2042-8812
DOI	10.1093/jscr/rjae114
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Article ; Online: Single incision endoscopic strip craniectomy for sagittal craniosynostosis.

Ahn, Edward S / Bhandarkar, Archis R

Neurosurgical focus: Video

2021 Volume 4, Issue 2, Page(s) V10

Abstract: The authors describe an endoscopic strip craniectomy through a single incision for the treatment of sagittal craniosynostosis in a young infant. The endoscopic strip craniectomy was first introduced with the use of two incisions on either end of the ... ...

Abstract	The authors describe an endoscopic strip craniectomy through a single incision for the treatment of sagittal craniosynostosis in a young infant. The endoscopic strip craniectomy was first introduced with the use of two incisions on either end of the fused suture. This single-incision technique offers several advantages. There is a cosmetic advantage and a reduced risk of wound complications. This technique also allows for early control of emissary veins and an inside-out identification of the lambdoid sutures. Endoscopic visualization is optimized to reduce the risk of blood loss, especially because circulating blood volume is very limited in these young infants. The video can be found here: https://vimeo.com/514366415.
Language	English
Publishing date	2021-04-01
Publishing country	United States
Document type	Journal Article
ISSN	2643-5217
ISSN (online)	2643-5217
DOI	10.3171/2021.1.FOCVID20120
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Article: Managing the positive periosteal margin after Mohs excision of scalp dermatofibrosarcoma protuberans in children with underlying calvarial bone thinning and resorption: The value of multidisciplinary treatment.

Zheng, Eugene / Cherukuri, Sai / Arpey, Christopher J / Ahn, Edward S / Mardini, Samir / Gibreel, Waleed

JAAD case reports

2023 Volume 39, Page(s) 26–29

Language	English
Publishing date	2023-07-13
Publishing country	United States
Document type	Case Reports
ZDB-ID	2834220-3
ISSN	2352-5126
ISSN	2352-5126
DOI	10.1016/j.jdcr.2023.06.044
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Article ; Online: Above-The-Hairline Suboccipital Transverse Approach for the Treatment of Chiari I Malformation.

Naylor, Ryan M / Rotter, Juliana / Proctor, Mark R / Bite, Uldis / Ahn, Edward S

Operative neurosurgery (Hagerstown, Md.)

2023 Volume 24, Issue 5, Page(s) 542–547

Abstract: Background: Chiari I malformation is a common pediatric neurosurgical disorder with an established treatment paradigm. Posterior fossa decompression and duraplasty (PFDD) is associated with symptom improvement but it carries postoperative risk, ... ...

Abstract	Background: Chiari I malformation is a common pediatric neurosurgical disorder with an established treatment paradigm. Posterior fossa decompression and duraplasty (PFDD) is associated with symptom improvement but it carries postoperative risk, particularly cerebrospinal fluid (CSF) leak and wound complications. In addition, the cosmetic outcomes of PFDD have been overlooked in the literature. Objective: To describe a novel approach for PFDD in which the transverse surgical incision is completely hidden above the hairline and to report early outcomes in a prospective patient cohort. Methods: Clinical and cosmetic outcomes were recorded for 15 consecutive pediatric patients who underwent PFDD for Chiari I malformation via the above-the-hairline transverse suboccipital approach. Results: The median clinical follow-up time was 6 months (range 1-12 months), and the majority of patients experienced significant improvement of their preoperative symptoms. Three patients (20%) experienced complications associated with surgery, which included injury to the greater occipital nerve, CSF hypotension and subfascial pseudomeningocele, and superficial wound dehiscence that resolved spontaneously with oral antibiotics. Zero patients (0%) returned to the operating room for persistent CSF leak, deep wound infection, or revision decompression. An excellent cosmetic outcome was achieved in 12 patients (80%). No patient had a poor cosmetic outcome. Conclusion: The above-the-hairline transverse suboccipital approach for PFDD in patients with Chiari I malformation offers favorable cosmetic outcomes and fascial closure while permitting adequate decompression.
MeSH term(s)	Humans ; Child ; Decompression, Surgical ; Prospective Studies ; Dura Mater/surgery ; Arnold-Chiari Malformation/diagnostic imaging ; Arnold-Chiari Malformation/surgery ; Postoperative Complications/surgery ; Cerebrospinal Fluid Leak/surgery ; Cerebrospinal Fluid Leak/complications
Language	English
Publishing date	2023-01-19
Publishing country	United States
Document type	Journal Article
ZDB-ID	2767575-0
ISSN	2332-4260 ; 2332-4252
ISSN (online)	2332-4260
ISSN	2332-4252
DOI	10.1227/ons.0000000000000619
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Article ; Online: Lumbosacral Nevus Simplex in a Newborn Girl with an Asymmetrical Y-Shaped Gluteal Cleft.

Leopold, Kaitlin N / Ahn, Edward S / Youssef, Molly J / Gregory, Seth W

Pediatrics in review

2021 Volume 42, Issue 10, Page(s) e41–e44

MeSH term(s)	Female ; Humans ; Infant, Newborn ; Nevus ; Skin Neoplasms
Language	English
Publishing date	2021-10-01
Publishing country	United States
Document type	Journal Article
ZDB-ID	774515-1
ISSN	1526-3347 ; 0191-9601
ISSN (online)	1526-3347
ISSN	0191-9601
DOI	10.1542/pir.2020-002162
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Article ; Online: Machine learning for enhanced prognostication: predicting 30-day outcomes following posterior fossa decompression surgery for Chiari malformation type I in a pediatric cohort.

El-Hajj, Victor Gabriel / Ghaith, Abdul Karim / Elmi-Terander, Adrian / Ahn, Edward S / Daniels, David J / Bydon, Mohamad

Journal of neurosurgery. Pediatrics

2024 , Page(s) 1–9

Abstract: Objective: Chiari malformation type I (CM-I) is a congenital disorder occurring in 0.1% of the population. In symptomatic cases, surgery with posterior fossa decompression (PFD) is the treatment of choice. Surgery is, however, associated with peri- and ... ...

Abstract	Objective: Chiari malformation type I (CM-I) is a congenital disorder occurring in 0.1% of the population. In symptomatic cases, surgery with posterior fossa decompression (PFD) is the treatment of choice. Surgery is, however, associated with peri- and postoperative complications that may require readmission or renewed surgical intervention. Given the associated financial costs and the impact on patients' well-being, there is a need for predictive tools that can assess the likelihood of such adverse events. The aim of this study was therefore to leverage machine learning algorithms to develop a predictive model for 30-day readmissions and reoperations after PFD in pediatric patients with CM-I. Methods: This was a retrospective study based on data from the National Surgical Quality Improvement Program-Pediatric database. Eligible patients were those undergoing PFD (Current Procedural Terminology code 61343) for CM-I between 2012 and 2021. Patients undergoing surgery for tumors or vascular lesions were excluded. Unplanned 30-day readmission and unplanned 30-day reoperation were the main study outcomes. Additional outcome data considered included the length of hospital stay, 30-day complications, discharge disposition, and 30-day mortality. Training and testing samples were randomly generated (80:20) to study the 30-day readmission and reoperation using logistic regression, decision tree, random forest (RF), K-nearest neighbors, and Gaussian naive Bayes algorithms. Results: A total of 7106 pediatric patients undergoing PFD were included. The median age was 9.2 years (IQR 4.7, 14.2 years). Most of the patients were female (56%). The 30-day readmission and reoperation rates were 7.5% and 3.4%, respectively. Headaches (32%) and wound-related complications (30%) were the most common reasons for 30-day readmission, while wound revisions and evacuation of fluid or blood (62%), followed by CSF diversion-related procedures (28%), were the most common reasons for 30-day reoperation. RF classifiers had the highest predictive accuracy for both 30-day readmissions (area under the curve [AUC] 0.960) and reoperations (AUC 0.990) compared with the other models. On feature importance analysis, sex, developmental delay, ethnicity, respiratory disease, premature birth, hydrocephalus, and congenital/genetic anomaly were some of the variables contributing the most to both RF models. Conclusions: Using a large-scale nationwide dataset, machine learning models for the prediction of both 30-day readmissions and reoperations were developed and achieved high accuracy. This highlights the utility of machine learning in risk stratification and surgical decision-making for pediatric CM-I.
Language	English
Publishing date	2024-04-12
Publishing country	United States
Document type	Journal Article
ZDB-ID	2403985-8
ISSN	1933-0715 ; 1933-0707
ISSN (online)	1933-0715
ISSN	1933-0707
DOI	10.3171/2024.2.PEDS23523
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Article ; Online: Deciphering the Kidney Matrisome: Identification and Quantification of Renal Extracellular Matrix Proteins in Healthy Mice.

Rende, Umut / Ahn, Seong Beom / Adhikari, Subash / Moh, Edward S X / Pollock, Carol A / Saad, Sonia / Guller, Anna

International journal of molecular sciences

2023 Volume 24, Issue 3

Abstract: Precise characterization of a tissue's extracellular matrix (ECM) protein composition (matrisome) is essential for biomedicine. However, ECM protein extraction that requires organ-specific optimization is still a major limiting factor in matrisome ... ...

Abstract	Precise characterization of a tissue's extracellular matrix (ECM) protein composition (matrisome) is essential for biomedicine. However, ECM protein extraction that requires organ-specific optimization is still a major limiting factor in matrisome studies. In particular, the matrisome of mouse kidneys is still understudied, despite mouse models being crucial for renal research. Here, we comprehensively characterized the matrisome of kidneys in healthy C57BL/6 mice using two ECM extraction methods in combination with liquid chromatography tandem mass spectrometry (LC-MS/MS), protein identification, and label-free quantification (LFQ) using MaxQuant. We identified 113 matrisome proteins, including 22 proteins that have not been previously listed in the Matrisome Database. Depending on the extraction approach, the core matrisome (structural proteins) comprised 45% or 73% of kidney ECM proteins, and was dominated by glycoproteins, followed by collagens and proteoglycans. Among matrisome-associated proteins, ECM regulators had the highest LFQ intensities, followed by ECM-affiliated proteins and secreted factors. The identified kidney ECM proteins were primarily involved in cellular, developmental and metabolic processes, as well as in molecular binding and regulation of catalytic and structural molecules' activity. We also performed in silico comparative analysis of the kidney matrisome composition in humans and mice based on publicly available data. These results contribute to the first reference database for the mouse renal matrisome.
MeSH term(s)	Humans ; Mice ; Animals ; Extracellular Matrix Proteins/metabolism ; Chromatography, Liquid ; Tandem Mass Spectrometry ; Mice, Inbred C57BL ; Extracellular Matrix/metabolism ; Kidney/metabolism
Chemical Substances	Extracellular Matrix Proteins
Language	English
Publishing date	2023-02-01
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2019364-6
ISSN	1422-0067 ; 1422-0067 ; 1661-6596
ISSN (online)	1422-0067
ISSN	1422-0067 ; 1661-6596
DOI	10.3390/ijms24032827
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Article ; Online: Image processing and machine learning for telehealth craniosynostosis screening in newborns.

Bookland, Markus J / Ahn, Edward S / Stoltz, Petronella / Martin, Jonathan E

Journal of neurosurgery. Pediatrics

2021 Volume 27, Issue 5, Page(s) 581–588

Abstract: Objective: The authors sought to evaluate the accuracy of a novel telehealth-compatible diagnostic software system for identifying craniosynostosis within a newborn (< 1 year old) population. Agreement with gold standard craniometric diagnostics was ... ...

Abstract	Objective: The authors sought to evaluate the accuracy of a novel telehealth-compatible diagnostic software system for identifying craniosynostosis within a newborn (< 1 year old) population. Agreement with gold standard craniometric diagnostics was also assessed. Methods: Cranial shape classification software accuracy was compared to that of blinded craniofacial specialists using a data set of open-source (n = 40) and retrospectively collected newborn orthogonal top-down cranial images, with or without additional facial views (n = 339), culled between April 1, 2008, and February 29, 2020. Based on image quality, midface visibility, and visibility of the cranial equator, 351 image sets were deemed acceptable. Accuracy, sensitivity, and specificity were calculated for the software versus specialist classification. Software agreement with optical craniometrics was assessed with intraclass correlation coefficients. Results: The cranial shape classification software had an accuracy of 93.3% (95% CI 86.8-98.8; p < 0.001), with a sensitivity of 92.0% and specificity of 94.3%. Intraclass correlation coefficients for measurements of the cephalic index and cranial vault asymmetry index compared to optical measurements were 0.95 (95% CI 0.84-0.98; p < 0.001) and 0.67 (95% CI 0.24-0.88; p = 0.003), respectively. Conclusions: These results support the use of image processing-based neonatal cranial deformity classification software for remote screening of nonsyndromic craniosynostosis in a newborn population and as a substitute for optical scanner- or CT-based craniometrics. This work has implications that suggest the potential for the development of software for a mobile platform that would allow for screening by telemedicine or in a primary care setting.
MeSH term(s)	Cephalometry/methods ; Craniosynostoses/diagnosis ; Female ; Humans ; Image Interpretation, Computer-Assisted/methods ; Infant, Newborn ; Machine Learning ; Male ; Mass Screening/methods ; Software ; Telemedicine/methods
Language	English
Publishing date	2021-03-19
Publishing country	United States
Document type	Journal Article
ZDB-ID	2403985-8
ISSN	1933-0715 ; 1933-0707
ISSN (online)	1933-0715
ISSN	1933-0707
DOI	10.3171/2020.9.PEDS20605
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Article ; Online: Incorporation of a biparietal narrowing metric to improve the ability of machine learning models to detect sagittal craniosynostosis with 2D photographs.

Anderson, Megan G / Jungbauer, Dana / Leclair, Nathan K / Ahn, Edward S / Stoltz, Petronella / Martin, Jonathan E / Hersh, David S / Bookland, Markus J

Neurosurgical focus

2023 Volume 54, Issue 6, Page(s) E9

Abstract: Objective: Sagittal craniosynostosis is the most common form of craniosynostosis and typically results in scaphocephaly, which is characterized by biparietal narrowing, compensatory frontal bossing, and an occipital prominence. The cephalic index (CI) ... ...

Abstract	Objective: Sagittal craniosynostosis is the most common form of craniosynostosis and typically results in scaphocephaly, which is characterized by biparietal narrowing, compensatory frontal bossing, and an occipital prominence. The cephalic index (CI) is a simple metric for quantifying the degree of cranial narrowing and is often used to diagnose sagittal craniosynostosis. However, patients with variant forms of sagittal craniosynostosis may present with a "normal" CI, depending on the part of the suture that is closed. As machine learning (ML) algorithms are developed to assist in the diagnosis of cranial deformities, metrics that reflect the other phenotypic features of sagittal craniosynostosis are needed. In this study the authors sought to describe the posterior arc angle (PAA), a measurement of biparietal narrowing that is obtained with 2D photographs, and elucidate the role of PAA as an adjuvant to the CI in characterizing scaphocephaly and the potential relevance of PAA in new ML model development. Methods: The authors retrospectively reviewed 1013 craniofacial patients treated during the period from 2006 to 2021. Orthogonal top-down photographs were used to calculate the CI and PAA. Distribution densities, receiver operating characteristic (ROC) curves, and chi-square analyses were used to describe the relative predictive utility of each method for sagittal craniosynostosis. Results: In total, 1001 patients underwent paired CI and PAA measurements and a clinical head shape diagnosis (sagittal craniosynostosis, n = 122; other cranial deformity, n = 565; normocephalic, n = 314). The area under the ROC curve (AUC) for the CI was 98.5% (95% confidence interval 97.8%-99.2%, p < 0.001), with an optimum specificity of 92.6% and sensitivity of 93.4%. The PAA had an AUC of 97.4% (95% confidence interval 96.0%-98.8%, p < 0.001) with an optimum specificity of 94.9% and sensitivity of 90.2%. In 6 of 122 (4.9%) cases of sagittal craniosynostosis, the PAA was abnormal while the CI was normal. This means that adding a PAA cutoff branch to a partition model increases the detection of sagittal craniosynostosis. Conclusions: Both CI and PAA are excellent discriminators for sagittal craniosynostosis. Using an accuracy-optimized partition model, the addition of the PAA to the CI increased model sensitivity compared to using the CI alone. Using a model that incorporates both CI and PAA could assist in the early identification and treatment of sagittal craniosynostosis via automated and semiautomated algorithms that utilize tree-based ML models.
MeSH term(s)	Humans ; Infant ; Retrospective Studies ; Craniosynostoses/diagnostic imaging ; Craniosynostoses/surgery ; Skull/surgery ; Neurosurgical Procedures ; Algorithms
Language	English
Publishing date	2023-06-07
Publishing country	United States
Document type	Journal Article
ZDB-ID	2026589-X
ISSN	1092-0684 ; 1092-0684
ISSN (online)	1092-0684
ISSN	1092-0684
DOI	10.3171/2023.3.FOCUS2349
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Article ; Online: Low occurrence of long-term subsequent fusion in pediatric patients following decompressive surgery for Chiari malformation: an institutional review.

Kerezoudis, Panagiotis / Everson, Megan C / Miller, Kai J / Daniels, David J / Ahn, Edward S

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery

2022 Volume 38, Issue 8, Page(s) 1455–1460

Abstract: Objective: Chiari I malformation is treated with suboccipital craniectomy with cervical laminectomy, a procedure which has been associated with the possibility of pre-existing or iatrogenic occipitocervical instability. The long-term risk of subsequent ... ...

Abstract	Objective: Chiari I malformation is treated with suboccipital craniectomy with cervical laminectomy, a procedure which has been associated with the possibility of pre-existing or iatrogenic occipitocervical instability. The long-term risk of subsequent spinal deformity and need for occipito cervical fusion after standard Chiari decompression in pediatric patients has not yet been characterized. Methods: We queried our institutional electronic database for patients aged 18 and under, with at least 5 years of follow-up, that underwent surgical decompression for Chiari I malformation. Occurrence of subsequent occipitocervical fusion at follow-up comprised the primary endpoint. Cases with myelomeningocele, Chiari II, or fusion at time of decompression were excluded. Results: A total of 30 patients (median age 5.5 years, 60% males) were analyzed. Age distribution was as follows: n = 3 for 0-1 years, n = 11 for 1-5 years, n = 4 for 5-10 years, and n = 12 for 10-18 years. Median tonsillar descent below the foramen magnum was 12.5 mm (interquartile range [IQR]: 10.8-19.5 mm). Syringomyelia was observed in 43%, retroflexion of the dens in 55%, basilar invagination in 6.7%, and medullary kinking in 27%. The median clivo-axial angle was 142° (132-150°). The majority of patients underwent C1 laminectomy (n = 24, 80%), followed by C1-C2 laminectomy (n = 4, 13%), while one patient had C1-upper C2 and C1-C3 laminectomy each, respectively. At a median follow-up of 6.3 years, there was only one patient (3.3% of overall cohort) that underwent subsequent occipitocervical fusion. The patient (4-year-old male) initially had a suboccipital craniectomy with C1 laminectomy and duraplasty and presented with recurrence of posterior headaches and neck pain 4 months after original surgery. We proceeded with occiput-C2 fusion with subsequent resolution of his symptoms. Conclusion: Current analysis shows that in the absence of clinical or imaging features suggestive of craniocervical instability, Chiari I decompressive surgery is associated with very low long-term risk of requiring occipitocervical fusion. This observance can be used to guide surgical treatment decisions, especially in young children with Chiari I malformations.
MeSH term(s)	Arnold-Chiari Malformation/complications ; Arnold-Chiari Malformation/diagnostic imaging ; Arnold-Chiari Malformation/surgery ; Child ; Child, Preschool ; Decompression, Surgical/adverse effects ; Decompression, Surgical/methods ; Female ; Foramen Magnum/surgery ; Humans ; Laminectomy/adverse effects ; Magnetic Resonance Imaging ; Male ; Syringomyelia/surgery ; Treatment Outcome
Language	English
Publishing date	2022-05-19
Publishing country	Germany
Document type	Case Reports ; Journal Article ; Review
ZDB-ID	605988-0
ISSN	1433-0350 ; 0302-2803 ; 0256-7040
ISSN (online)	1433-0350
ISSN	0302-2803 ; 0256-7040
DOI	10.1007/s00381-022-05550-3
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