Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018133 (graft-versus-host disease)
 18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Stevens-Johnson syndrome (SJS) is a mucocutaneous disorder induced by an immune-complex-mediated hypersensitivity reaction. Nearly half of cases are caused by a reaction to drugs or appear during viral infections and malignancies. A very few cases are caused by a bacterial infection (Streptococcus) or Mycoplasma pneumoniae. Graft versus host disease is another well-established cause, independent of drugs. No specific etiology has been identified in up to half of cases. We report a 54-year-old man with SJS induced by carbamazepine. Reported patient had prodromal symptoms like fever, headache and polyarthralgia, which preceded mucocutaneous lesions by 3 days. Physical examination on admission, revealed asthenic male with a temperature of 37.2 degrees C and generalized dermatitis with positive Nikolsky sign, large erosions of the palms and soles, onychomadesis, numerous oral and vermilion border of the lips erosions. The patient was administered systemic steroidotherapy and carbamazepine dose was gradually decreased and finally replaced with valproic acid and valproate sodium. During the hospitalization, temperature normalized and the skin lesions disappeared after 3 weeks of treatment.
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PMID:Stevens-Johnson syndrome induced by carbamazepine. 1766 56

This study report clinical course, etiology, management, and long-term outcome of children who developed toxic epidermal necrolysis-like reaction (TEN-LR) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). We retrospectively collected children with TEN-LR occurring after allo-HSCT performed in 2 pediatric bone marrow units between 2005 and 2014. We identified 6 cases of TEN-LR of 322 patients (1.8%). Possible triggers of TEN included antibiotics, antiepileptics, antimycotics, and Mycoplasma infection. In 3 patients TEN-LR occurred concurrently with severe multiorgan acute graft versus host disease. The management of TEN included administration of high doses of intravenous immunoglobulins and steroids (n=6), anti-tumor necrosis factor (n=3), and plasmapheresis (n=3) and whenever possible, discontinuation of the potentially causative drugs. Four patients (66%) reached a complete clinical response of TEN-LR after a median of 11.2 days. Two children (34%) are presently alive, 1 with long-term ocular sequelae. TEN-LR is a potentially lethal complication that may occur after HSCT also in pediatric patients. In our experience, TEN-LR and acute graft versus host disease probably coexisted and an overlap between the 2 forms is suggested. The multidisciplinary approaches involving specialized nurses, hematologists, dermatologists, burn surgeons, and infectious disease specialists is crucial to treat these patients.
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PMID:Toxic Epidermal Necrolysis-like Reaction After Hematopoietic Stem Cell Transplantation in Children. 2826 83

Bronchiolitis obliterans (BO) is a rare, chronic form of obstructive lung disease, often initiated with injury of the bronchiolar epithelium followed by an inflammatory response and progressive fibrosis of small airways resulting in nonuniform luminal obliteration or narrowing. The term BO comprises a group of diseases with different underlying etiologies, courses, and characteristics. Among the better recognized inciting stimuli leading to BO are airway pathogens such as adenovirus and mycoplasma, which, in a small percentage of infected children, will result in progressive fixed airflow obstruction, an entity referred to as postinfectious bronchiolitis obliterans (PIBO). The present knowledge on BO in general is reasonably well developed, in part because of the relatively high incidence in patients who have undergone lung transplantation or bone marrow transplant recipients who have had graft-versus-host disease in the posttransplant period. The cellular and molecular pathways involved in PIBO, while assumed to be similar, have not been adequately elucidated. Since 2016, an international consortium of experts with an interest in PIBO assembles on a regular basis in Geisenheim, Germany, to discuss key areas in PIBO which include diagnostic workup, treatment strategies, and research fields.
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PMID:Postinfectious Bronchiolitis Obliterans in Children: Diagnostic Workup and Therapeutic Options: A Workshop Report. 3207 69