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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A Turkish family with four children is described, two boys suffering from Cockayne's syndrome and a girl from recessive dystrophic epidermolysis bullosa (Hallopeau-Siemens). The outstanding features in the brothers are a pronounced delay in growth which began in both children at the age of about 1.5 years, progeria-like facial features, and a high degree of light sensitivity noticed very soon after birth. The daughter died of septicemia at the age of 4 years. Both diseases are rare and probably the consequence of extremely high consanguinity.
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PMID:[Cockayne syndrome and epidermolysis bullosa dystrophica (Hallopeau-Siemens). Simultaneous occurrence in a family]. 717 21

We report on a newborn boy with junctional epidermolysis bullosa and pyloric atresia. Blisters were found on his skin at birth, especially in places exposed to pressure, and appeared later on his mucous membranes. Epidermolysis bullosa was confirmed by electron microscopy. Radiography revealed pyloric atresia, and a gastroduodenostomy was carried out at 7 days of age. A connective tissue septum was found between his ventricle and duodenum. The skin changes were mild, and the clinical course was determined by his protein-losing enteropathy. He died at 66 days of age from pseudomonas sepsis.
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PMID:Protein-losing enteropathy in a child with junctional epidermolysis bullosa and pyloric atresia. 774 38

A case of junctional epidermolysis bullosa was reported. A four-month-old boy was presented with generalised blisters after minor trauma since birth. There was no family history of blistering diseases or consanguinity. Skin examination revealed blisters primarily on the upper and lower extremities. Erosions were also noted on the face, abdomen and buttocks. The lesions healed without milia or scarring formation. Anonychia of all fingers and toe nails were noted. Skin biopsy from the blisters showed subepidermal bulla with a few inflammatory cells. Electron microscopic examination revealed cleavage plane at the lamina lucida and the absence of hemidesmosomes. These findings supported a diagnosis of junctional epidermolysis bullosa. The patient was treated with topical and systemic antibiotics. However, the blisters gradually increased. He died of uncontrolled sepsis and diarrhea.
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PMID:Epidermolysis bullosa letalis (Herlitz disease): a case report. 779 34

Vesiculopustular diseases of neonates and infants can be divided into infectious and noninfectious categories. Recent clinical and scientific literature has focused mainly on the infectious diseases and on epidermolysis bullosa. This review covers the following disorders: neonatal sepsis, staphylococcal scaled skin syndrome, neonatal herpes simplex, neonatal varicella, congenital syphilis, scabies, mastocytosis, incontinentia pigmenti, eosinophilic pustular folliculitis, and epidermolysis bullosa.
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PMID:Vesiculopustular diseases of neonates and infants. 795 66

We describe three neonates who had large eroded areas of skin on their extremities. The clinical course and ultrastructural findings were consistent with a diagnosis of epidermolysis bullosa herpetiformis (Dowling-Meara type). In each case blisters developed around eroded areas after birth and enlarged centrifugally in a herpetiform fashion. One patient died of sepsis at 8 days of age. In the two survivors blister formation subsided gradually within 1 year. Ultrastructural studies confirmed intraepidermal blister formation associated with spheric aggregates of tonofilaments in the lower epidermis. Spheric aggregates were also found in clinically uninvolved skin.
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PMID:Three neonatal cases of epidermolysis bullosa herpetiformis (Dowling-Meara type) with severe erosive skin lesions. 849 81

We report a patient with severe epidermolysis bullosa acquisita (EBA) whose disease was refractory to conventional treatments. New bullae continued to develop over greater than 50% of his body surface area despite therapy. His course was complicated by hyperglycaemia, sepsis, hypoxia caused by pulmonary Aspergillus infection and an idiopathic cardiomyopathy. His EBA resolved after treatment with extracorporeal photochemotherapy (ECP). Hence, ECP may be effective in the treatment of severe EBA which has failed to respond to standard treatment regimens.
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PMID:Remission of severe epidermolysis bullosa acquisita induced by extracorporeal photochemotherapy. 854 7

A full-term infant with junctional epidermolysis bullosa (JEB) is described. The distribution and morphologic characteristics of generalized blistering in areas of pressure in conjunction with perioral and perinasal granulation tissue suggested the diagnosis of generalized gravis (Herlitz) JEB. The family history was consistent with autosomal recessive inheritance. Electron microscopy demonstrated a subepidermal cleft arising in the lamina lucida with hemidesmosomal hypoplasia, findings consistent with gravis JEB. Immunofluorescent antigenic mapping localized laminin and type IV collagen exclusively to the blister base and weak reactivity of bullous pemphigold antigen to both the roof and the base. Type VII collagen (LH 7:2 epitope) was detected solely at the base of the cleavage plane, and abnormal staining of laminin 5 (kalinin, GB3, nicein) and 19-DEJ-1 antigen was observed. The patient died of sepsis at age 3 months. DNA extracted from cultured keratinocytes for molecular genetic analysis demonstrated a mutation with the LAMB3 gene encoding the beta 3 chain of laminin 5. We present the clinical and laboratory findings and briefly review recent advances in the diagnosis and management of JEB.
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PMID:Generalized gravis junctional epidermolysis bullosa: case report, laboratory evaluation, and review of recent advances. 879 Feb 63

Congenital epidermolysis bullosa is an inherited disorder characterized by an anomaly of joint structures between epidermis and dermis. This anomaly involves different malpighian epithelium and particularly in the digestive chorioepithelial junction. Digestive and oropharyngeal manifestations include bullae, erosions and ulcerations that heal by fibrosis, leading to retraction and stenosis. We report two cases of esophageal stenosis, the first one in a young patient with a congenital epidermolysis bullosa of Hallopeau-Siemens and the second one, in a young woman with a Pasini variant. In the first case, the esophageal stenosis was treated by careful dilation. In the second case, the endoscopy induced hemorragic bullae and septicemia, which were treated with antibiotics and total parenteral nutrition.
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PMID:[Digestive involvement in dystrophic bullous epidermolysis. Presentation of 2 cases and review of the literature]. 911 72

The anesthetic management used in a 4-yr-old boy with dystrophic epidermolysis bullosa (DEB) submitted to long-lasting urological surgery is reported. Medical treatment of cutaneous bullae in various stages of healing was undertaken preoperatively. Other preoperative therapies were performed to treat sepsis and to improve poor nutritional state. Different measures were used during monitoring and inhalation induction and maintenance of anesthesia in order to prevent frictional trauma to the skin and oropharyngeal mucosa, leading to blistering. Anesthesia and surgery proceeded uneventfully, while surgical complications and sepsis occurred postoperatively. However the patient was discharged from intensive care unit to surgical ward on 35 postoperative days without evidence of oropharyngeal bullae and with a good improvement of his cutaneous condition. The conclusions are drawn that, even if the patient with DEB represents a challenge to the anesthesiologist, a long-lasting surgical intervention can be performed successfully. The main aspect in the anesthetic management of these patients is the detailed knowledge of all problems that anesthesiologist must face and solve to preserve skin and mucosa integrity, avoiding the risk of severe complications.
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PMID:[Anesthetics management in patients with epidermolysis bullosa]. 1052 36

Epidermolysis bullosa is a group of hereditary blistering disorders for which there is no definitive therapy. Wound care is an important component of management. Regular dressing changes are required to protect blistered and eroded skin, and to prevent secondary infection and sepsis. These dressing changes can be very painful for patients with extensive erosions. We report our experience of pain management in an 11-year-old boy with severe junctional epidermolysis bullosa. Amitryptiline and cognitive behavioral techniques were effective in relieving chronic pain and discomfort. Oral midazolam 0.33 mg/kg administered 20 minutes prior to baths and dressing changes substantially improved his tolerance of wound care.
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PMID:Pain management of junctional epidermolysis bullosa in an 11-year-Old boy. 1063 47


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