Esophageal Web: A Previously Unrecognized Complication of Epidermolysis Bullosa

PEDIATRICS ◽  
1981 ◽  
Vol 67 (5) ◽  
pp. 678-682
Author(s):  
Craig Hillemeier ◽  
Robert Touloukian ◽  
Richard Mccallum ◽  
Joyce Gryboski
Keyword(s):  
Epidermolysis Bullosa ◽  
Esophageal Disease ◽  
Colonic Interposition ◽  
Epidermolysis Bullosa Dystrophica ◽  
Esophageal Web ◽  
Life Saving

The course of the esophageal disease in four patients with epidermolysis bullosa dystrophica recessive is examined. Three of four patients experienced web formation, a previously unrecognized finding, and the significance of this lesion in the evaluation and management of these patients is emphasized. One offour patients underwent a successful colonic interposition for severe esophageal disease and the role of this potentially life-saving procedure is discussed.

scholarly journals Role of regional anaesthesia in a patient with epidermolysis bullosa dystrophica for emergency surgery

2021 ◽  
Vol 65 (12) ◽  
pp. 906
Author(s):  
Thirumurugan Arikrishnan ◽  
Deepak Chakravarthy ◽  
Vijayanthi Vijayan ◽  
Gnanasekaran Srinivasan
Keyword(s):  
Epidermolysis Bullosa ◽  
Emergency Surgery ◽  
Regional Anaesthesia ◽  
Epidermolysis Bullosa Dystrophica

Vascular access for chronic hemodialysis in a patient with epidermolysis bullosa dystrophica Hallopeau-Siemens

2012 ◽  
Vol 17 (1) ◽  
pp. 126-129 ◽  
Author(s):  
Sanja Sakan ◽  
Nikolina Basic-Jukic ◽  
Boris Tomasevic ◽  
Petar Kes ◽  
Daniela Bandic Pavlovic ◽  
...  
Keyword(s):  
Vascular Access ◽  
Epidermolysis Bullosa ◽  
Chronic Hemodialysis ◽  
Epidermolysis Bullosa Dystrophica

The Role of Histones and Heparin in Sepsis: A Review

2021 ◽  
pp. 088506662199232
Author(s):  
Xiaojuan Zhang ◽  
Xin Li
Keyword(s):  
Septic Shock ◽  
Multiple Organ ◽  
Clinical Settings ◽  
The Past ◽  
Life Saving ◽  
Extracellular Histones ◽  
Sepsis And Septic Shock ◽  
Development And Management ◽  
Made In

Septic shock with multiple organ failure is a devastating situation in clinical settings. Through the past decades, much progress has been made in the management of sepsis and its underlying pathogenesis, but a highly effective therapeutic has not been developed. Recently, macromolecules such as histones have been targeted in the treatment of sepsis. Histones primarily function as chromosomal organizers to pack DNA and regulate its transcription through epigenetic mechanisms. However, a growing body of research has shown that histone family members can also exert cellular toxicity once they relocate from the nucleus into the extracellular space. Heparin, a commonly used anti-coagulant, has been shown to possess life-saving capabilities for septic patients, but the potential interplay between heparin and extracellular histones has not been investigated. In this review, we summarize the pathogenic roles of extracellular histones and the therapeutic roles of heparin in the development and management of sepsis and septic shock.

scholarly journals The Role of Liver Transplantation in the Management of Paediatric Liver Tumours

2007 ◽  
Vol 89 (1) ◽  
pp. 12-21 ◽  
Author(s):  
Mark D Stringer
Keyword(s):  
Liver Transplantation ◽  
Hepatocellular Cancer ◽  
Good Prognosis ◽  
Liver Tumours ◽  
Primary Hepatic Malignancies ◽  
Life Saving ◽  
Time Of Presentation ◽  
Benign Liver ◽  
Hepatic Malignancies

In recent years, considerable progress has been made in the treatment of children with hepatoblastoma largely due to effective pre-operative chemotherapy. Total hepatectomy and liver transplantation has emerged as an effective treatment for the small proportion of children with unresectable hepatoblastoma limited to the liver. A 5-year survival of 70% can be achieved in such cases. In contrast, the results of liver transplantation in children with hepatocellular cancer remain poor because these tumours are usually advanced with evidence of major vascular invasion and/or extrahepatic spread at the time of presentation. An exception is those children in whom the hepatocellular carcinoma is detected during surveillance of chronic liver disease – they typically have smaller tumours and frequently have a good prognosis after liver transplantation. The role of liver transplantation in children with other primary hepatic malignancies remains uncertain because experience is very limited. Liver transplantation is rarely needed in the management of children with benign liver tumours but, if other treatments have failed, it can be a life-saving intervention.

Role of the General Pediatrician in Neonatology: Relevance of Recent Advances

1981 ◽  
Vol 3 (3) ◽  
pp. 71-76
Author(s):  
Ann R. Stark
Keyword(s):  
Newborn Infant ◽  
Transport Mechanism ◽  
Major Influence ◽  
Recent Advances ◽  
General Pediatrician ◽  
Life Saving

The general pediatrician plays important roles even as neonatology becomes more specialized: first in the evaluation of the newborn infant and in the organization of resources for care; and second as a major influence in the education of the parents. Awareness of significant advances will allow appropriate counseling of parents and obstetricians. Inasmuch as perinatal centers can provide care for mothers and babies that other hospitals cannot, pediatricians must ensure that the regionalization network and transport mechanism are established so that life-saving advances are available for their patients. Then, the pediatrician and the obstetrician, as members of a team, can provide the best possible care to mother and infant.

Wundtherapie mit kaltem Plasma bei Epidermolysis bullosa dystrophica

Der Hautarzt ◽  
2021 ◽  
Author(s):  
Alexander Thiem ◽  
Cristina Has ◽  
Anja Diem ◽  
Alfred Klausegger ◽  
Henning Hamm ◽  
...  
Keyword(s):  
Epidermolysis Bullosa ◽  
Epidermolysis Bullosa Dystrophica
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