Items tagged “cases”
5,504 results found
Article
IgA vasculitis
IgA vasculitis (formerly known as Henoch-Schönlein purpura (HSP)) is a type of non-thrombocytopenic immune-mediated small vessel acute leukocytoclastic vasculitis.
Epidemiology
IgA vasculitis tends to occur in the pediatric population (peak incidence 3-10 years) 3. In predominantly White popul...
Article
Werner syndrome
Werner syndrome is a progeroid rare autosomal recessive condition attributed to chromosome 8. It should not to be confused with similarly sounding Wermer syndrome.
Pathology
Werner syndrome is characterized by
premature senescence
cataracts
short stature
scleroderma-like skin changes such ...
Article
Slipped upper femoral epiphysis - grading
Grading of SUFE (slipped upper femoral epiphysis) can be made on both AP and true lateral projections.
On an AP radiograph a line along the superior margin of the femoral neck (line of Klein) should intersect the lateral corner of the epiphysis.
As the epiphysis slips, the metaphysis can be d...
Article
Restrictive cardiomyopathy
Restrictive cardiomyopathy is the least common subtype of cardiomyopathy and is characterized by a marked decrease in ventricular compliance.
Clinical presentation
Patients can present with symptoms and signs of left ventricular failure and/or right ventricular failure 9.
Pathology
It is p...
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Hepatic attenuation on CT
Hepatic attenuation on CT, reflected by Hounsfield values, depends on a combination of factors including the presence or absence, as well as the phase, of IV contrast administration.
Allowing for all these factors, the mean unenhanced attenuation value is around 55 HU 4.
Pathology
Several int...
Article
Hepatic peliosis
Hepatic peliosis is a rare benign vascular condition characterized by dilatation of sinusoidal blood-filled spaces within the liver. There may be involvement of other organs, most commonly the spleen and bone marrow. It can be seen in a variety of settings and is important as appearances may mim...
Article
Pseudocalculus sign (common bile duct)
The pseudocalculus sign is a term coined to describe a mimic of a distally impacted common bile duct (CBD) stone on ERCP, MRCP and the various forms of cholangiography, including T-tube, CT, intraoperative, and percutaneous 1.
It results from the forceful contraction of the choledochal sphincte...
Article
Autosplenectomy
Autosplenectomy denotes spontaneous infarction of the spleen with resulting hyposplenism.
Epidemiology
Autosplenectomy is most frequently encountered in patients with homozygous sickle cell disease, although it has also been reported in pneumococcal septicemia 1, and systemic lupus erythematos...
Article
Pulmonary artery atresia
Pulmonary artery atresia (or sometimes known as pulmonary atresia) is a congenital cardiovascular anomaly in which there is complete disruption between the right ventricular outflow tract (RVOT) and the pulmonary trunk.
Epidemiology
The estimated incidence is 1 in 10,000 births.
Pathology
Th...
Article
Line of Klein
The line of Klein describes an arbitrary line drawn along the superior edge of the femoral neck, which is useful in detecting early slipped upper femoral epiphysis in adolescents.
The line should normally intersect the lateral aspect of the superior femoral epiphysis. Failure of intersection ca...
Article
Drash syndrome
Drash syndrome, also known as the Denys-Drash syndrome, is associated with an abnormal WT1 gene (Wilms tumor gene) and consists of:
Wilms tumor
male pseudohermaphroditism
progressive glomerulonephritis
Article
Phakomatoses
The phakomatoses (also known as neurocutaneous syndromes) are a heterogeneous group of disorders most characterized by the involvement of structures that arise from the embryonic ectoderm (thus central nervous system, skin, and eyes), although other systems may also be involved.
Pathology
As a...
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Harrison sulcus
The Harrison sulcus or Harrison groove refers to a groove at the lower end of the rib cage seen in young children/infants with abnormally weak bones (e.g. rickets) or chronic respiratory disease (e.g. severe asthma). The lower chest is drawn in with flaring of the rib margin. The exact cause is ...
Article
Fitz-Hugh-Curtis syndrome
Fitz-Hugh-Curtis syndrome (FHCS), or perihepatitis, is the inflammation of the liver capsule and overlying peritoneum associated with adhesion formation, without the involvement of the hepatic parenchyma. It is a chronic complication of pelvic inflammatory disease (PID).
Epidemiology
The preva...
Article
COACH syndrome
The acronym COACH describes a syndrome associated with:
C: cerebellar vermis defect (Joubert syndrome)
O: oligophrenia
A: ataxia
C: coloboma
H: hepatic fibrosis
Epidemiology
COACH syndrome is considered extremely rare.
Associations
The association with infantile polycystic kidney diseas...
Article
Meckel-Gruber syndrome
Meckel-Gruber syndrome (MGS) is classically characterized by the triad of:
renal cystic dysplasia: multiple renal cysts (present in most cases)
occipital encephalocele / holoprosencephaly (~70%)
postaxial polydactyly: usually hexadactyly (~65%)
Additional hepatic developmental de...
Article
VATER
VATER (equivalent to VACTERL) is the non-random constellation of a number of congenital anomalies.
V: vertebral anomalies
A: anal atresia
TE: tracheo-oesophageal fistulas
R: radial ray hypoplasia, polydactyly, and renal agenesis
Epidemiology
The prevalence of at least 3/5 anomalies occur...
Article
Feingold syndrome
Feingold syndrome is characterized by the combination of:
microcephaly
digital abnormalities
alimentary tract atresias especially esophageal atresia
Article
Currarino syndrome
The Currarino syndrome is a complex condition variably comprised of characteristic congenital anomalies of the sacrum, anorectum and presacral soft tissues. It is also known as the Currarino triad or ASP triad, however, not all three features are always present 6.
anorectal malformation or ...
Article
Tumors of muscular origin
There are a number of tumors of muscular origin, which overall are relatively uncommon, representing ~2% of benign soft tissue tumors and ~10% of malignant soft tissue tumors 1.
Pathology
The tumors can be divided according to the type of muscle fiber:
Skeletal muscle origin
benign
rhabdom...