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: To evaluate the clinical characteristics, including spectral domain optical coherence tomography (SD-OCT) findings, of pediatric-onset Behçet's disease (PBD) patients. : Medical records of 23 PBD (15 males and 8 females) and 24 (15 males and 9 females) healthy subjects were evaluated retrospectively. The main outcomes were compared between PBD patients, with and without ocular involvement, and healthy subjects. : The mean age at onset was 12.00 ± 2.10 years. Mean follow-up period was 25.17 ± 15.36 months (range 6-48). Retinal vasculitis was the most common ocular finding (7 patients). Most of the complications of systemic treatment were associated with long term corticosteroid therapy. There was no significant difference between the mean retinal thickness of the PBD patients and healthy controls (p > 0.05). The mean choroidal thickness was significantly increased in all measured segments of PBD patients with ocular involvement (p < 0.01). : Choroidal thickness of PBD patients with ocular involvement was significantly thicker compared to the PBD patients without ocular involvement and to healthy control subjects.
This article was published in the following journal.
Name: Ocular immunology and inflammation
Celiac disease is an immune-mediated disorder characterized by a variable clinical manifestations, specific antibodies, HLA-DQ2/DQ8 haplotypes, and enteropathy.
To evaluate the impact of macrophage activation syndrome (MAS) on clinical features in patients with Kikuchi-Fujimoto disease (KFD) and to compare the features of MAS in KFD with those of adult-onset ...
Pulmonary arterial hypertension (PAH) is a rare disease with high mortality despite recent therapeutic advances. Clinical management of children with PAH is particularly challenging due to increased c...
The aim of this study was to compare optical coherence tomography (OCT) findings in pediatric patients with sickle cell disease (SCD) and healthy individuals and to investigate associations between th...
The rate of pediatric inflammatory bowel disease (IBD) has been increasing over the last decade and this increase has occurred most rapidly in the youngest children diagnosed
The primary objective of the study is to identify demographic and non-spirometric clinical features predictive of the asthma-COPD overlap population. The study aims to explore and identify...
The purpose of this study is to define clinical and demographic features within the population suffering from cervical deformity to assist health professionals to make important treatment ...
This study indented to evaluate the use of Optical laryngoscope in pediatric
This study is directed to evaluate the role of Optical Coherence Tomography Angiography (OCT-A) in the evaluation of the perifoveal vascular network in type 1 diabetic patients, and to inv...
This study evaluates the retinal and vascular features in patients with macular degeneration under the effects of Bevacizumab intravitreal injections using optical coherence tomography and...
An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal signs, and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration. (From Brain Pathol 1998 Jul;8(3):499-513; Brain Pathol 1995 Jan;5(1):61-75)
Rare autosomal recessive disorder of the urea cycle which leads to the accumulation of argininosuccinic acid in body fluids and severe HYPERAMMONEMIA. Clinical features of the neonatal onset of the disorder include poor feeding, vomiting, lethargy, seizures, tachypnea, coma, and death. Later onset results in milder set of clinical features including vomiting, failure to thrive, irritability, behavioral problems, or psychomotor retardation. Mutations in the ARGININOSUCCINATE LYASE gene cause the disorder.
A rare, slowly progressive disorder of myelin formation. Subtypes are referred to as classic, congenital, transitional, and adult forms of this disease. The classic form is X-chromosome linked, has its onset in infancy and is associated with a mutation of the proteolipid protein gene. Clinical manifestations include TREMOR, spasmus nutans, roving eye movements, ATAXIA, spasticity, and NYSTAGMUS, CONGENITAL. Death occurs by the third decade of life. The congenital form has similar characteristics but presents early in infancy and features rapid disease progression. Transitional and adult subtypes have a later onset and less severe symptomatology. Pathologic features include patchy areas of demyelination with preservation of perivascular islands (trigoid appearance). (From Menkes, Textbook of Child Neurology, 5th ed, p190)
An early onset form of phytanic acid storage disease with clinical and biochemical signs different from those of REFSUM DISEASE. Features include MENTAL RETARDATION; SENSORINEURAL HEARING LOSS; OSTEOPOROSIS; and severe liver damage. It can be caused by mutation in a number of genes encoding proteins involving in the biogenesis or assembly of PEROXISOMES.
A sporadic neurodegenerative disease with onset in middle-age characterized clinically by Parkinsonian features (e.g., MUSCLE RIGIDITY; HYPOKINESIA; stooped posture) and HYPOTENSION. This condition is considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Pathologic features include a prominent loss of neurons in the zona compacta of the SUBSTANTIA NIGRA and PUTAMEN. (From Adams et al., Principles of Neurology, 6th ed, p1075-6)
Pediatrics is the general medicine of childhood. Because of the developmental processes (psychological and physical) of childhood, the involvement of parents, and the social management of conditions at home and at school, pediatrics is a specialty. With ...