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. 2018 Sep 20;(139):58392. doi: 10.3791/58392
Data Type Example #1 Example #2 (Cameron and McClain 1986)
Document and Annotation Identification
Internal ID CCR005 CCR2000
Annotation Date Mar 2 2018 Mar 1 2018
Case Report Identification
Title A case of endocarditis. Ocular histopathology of acrodermatitis enteropathica.
Authors Grant AB;Chang CD Cameron JD;McClain CJ
Year 2017 1986
Journal World Journal of Medicine and Case Reports British Journal of Ophthalmology
Institution Department of Medicine, Division of Cardiology, First General Hospital, Boston, Massachusetts, USA Department of Ophthalmology, University of Minnesota Medical School, Minneapolis, Minnesota 55455
Corresponding Author Grant AB Cameron JD
PMID 25555555 3756122
DOI 10.1011/wjmcr.2017.11.001 NA
Link https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9555555/ https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1040795/
Language English English
Medical Content
Key Words brucellosis; endocarditis; mitral valve NA
Demography 37-year-old male male child
Geographic Locations Florida; Rio de Janeiro, Brazil NA
Life Style smoker; drinks alcohol occasionally NA
Family History third of five children of consanguineous parents; younger brother has chronic eczema NA
Social History construction worker NA
Medical/Surgical History history of fatigue 8 pound 9 ounce (3884 g) product of an uncomplicated, full term pregnancy; in good health until age 1 month when he developed a blistering skin rash on his cheeks; rash spread to involve the skin around the eyes, nose, and mouth; skin lesions were also noted on the abdomen and extremities; diarrhoea and failure to thrive; skin biopsy at that time showed parakeratosis typical of acrodermatitis enteropathica; treated over the next six years with intermittent courses of broad spectrum antibiotics, breast milk, and diodoquin; partially responded; developed total alopecia, intermittent acrodermatitis, and intermittent diarrhoea with suboptimal weight gain; spasticity attributed to central nervous system involvement by the ae had developed by 8 months of age; several episodes of cardiopulmonary arrest at 11 months; lack of co-ordination of his vocal cords; tracheostomy; by age 18 months the child developed searching nystagmus associated with bilateral optic atrophy and slight attenuation of retinal vessels as well as signs of psychomotor retardation; bilateral keratoconjunctivitis; skin rash; second skin biopsy performed at age 3 again showed parakeratosis typical for ae; severe skin rash and diarrhoea; bilateral gross anterior corneal opacities were seen which had completely resolved by the time he was reexamined at age five; frequent infections including otitis media, urinary tract infections, and skin infections
Disease System cardiovascular; infectious digestive; skin; eye; rare
Signs and Symptoms palpitations and dyspnea in the previous week; presented with lethargy, headache, and chills severe blepharoconjunctivitis and bilateral anterior corneal vascularisation; severe skin rash and diarrhoea; gram-negative bacterial sepsis; skin lesions typical of acrodermatitis enteropathica, absence of thymic tissue, marked degeneration of the optic nerves, chiasm, and optic tracts and extensive cerebellar degeneration
Comorbidity hypertension; hyperlipidemia NA
Diagnostic Techniques and Procedures Physical examination; electrocardiography; blood cultures ocular examination; necropsy
Diagnosis Brucella endocarditis acrodermatitis enteropathica
Laboratory Values increase in c-reactive protein (9 mg/dl); alkaline phosphatase (250 u/l) NA
Pathology Brucella melitensis was cultured from blood samples right and left eyes were similar in appearance; corneal epithelium was reduced in thickness to one to three cell layers of flattened squamous epithelial cells over the entire surface of the cornea; all polarity of the epithelium was lost. bowman's membrane could be identified only in the periphery of the right cornea. no bowman's membrane could be identified in the left cornea. neither degenerative nor inflammatory pannus could be identified in either eye; extensive atrophy of the circular and oblique muscles of the ciliary body; some posterior migration of lens capsular epithelium and early cortical degenerative changes; extensive degeneration of the retinal pigment epithelium throughout the posterior pole; retina was attached and showed mild autolytic changes throughout; some preservation of rod and cone outer segments in the posterior pole, however, these structures were completely lost anterior to the equator; extensive loss of the ganglion cell and nerve fibre layers of both eyes; nearly complete atrophy of the disc and adjacent optic nerve
Pharmacological Therapy gentamycin 240 mg/iv/daily NA
Inverventional Therapy prosthetic valve replacement NA
Patient Outcome Assessment recovery was uneventful; discharged home died in 1971 (age 7)
Diagnostic Imaging/Videotape Recording 2;1;0;1 7;0;0;0
Relationship to Other Case Reports 5555555 23430849
Relationship with Clinial Trial NCT05555123 NA
Crosslink with Database MedlinePlus Health Information: https://medlineplus.gov/ency/article/000597.htm HighWire - PDF: http://bjo.bmj.com/cgi/pmidlookup?view=long&pmid=3756122; Europe PubMed Central: http://europepmc.org/abstract/MED/3756122; Genetic Alliance: http://www.diseaseinfosearch.org/result/143
Acknowledgements
Funding Source National Institutes of Health/National Heart, Lung, and Blood Institute The Minnesota Lions Club; Research to Prevent Blindness; Veterans Administration; Office of Alcohol and Other Drug Abuse Programming of the State of Minnesota
Award Number R01HL123123 (to AG) NA
Disclosures/Conflict of Interest Dr. Grant is a paid spokesperson for DrugCo. NA
References 4 27