HAGEMAN FACTOR
factor XII
HAILEY-HAILEY DISEASE
benign familial chronic pemphigus
HALDANE EFFECT
deoxygenated hemoglobin having a greater affinity for CO2 than oxygenated hemoglobin
HALLERMANN-STREIFF SYNDROME
autosomal recessive syndrome with bird-like facies with hypoplastic mandible and beaked nose, proportionate dwarfism, hypotrichosis, microphthalmia, and congenital cataract
HALLERVORDEN-SPATZ DISEASE
autosomal recessive juvenile-onset generalized neuroaxonal dystrophy characterized by progressive movement alterations such as dystonia, tardive dyskinesia, rigidity, choreoathetosis, pyramidal signs, and mental retardation
HAM'S TEST
for diagnosing paroxysmal nocturnal hemoglobinuria, uses increased sensitivity of PNH-affected RBCs to lysis by complement; introduced in late 1930's
HAMMAN'S SIGN
mediastinal crunch is a series of precordial crackles synchronous with the heart beat, not with respiration. Best heard in the left lateral position, it is due to mediastinal emphysema (pseudomediastinum), seen with Boerhaave's syndrome
HAMMAN-RICH SYNDROME
acute interstitial pneumonia, a rare fulminant form of lung injury that presents acutely (days to weeks from onset of symptoms), most commonly occurs in previously healthy individual, characterized by a temporally uniform lesion which reflects an episode of acute lung injury at a single point in time (different from usual interstitial pneumonia where lesions are different age); path shows diffuse alveolar damage (a nonspecific reaction pattern)
HAMPTON'S HUMP
in pulmonary embolism/infarction, a wedge-shaped infiltrate that abuts the pleura; often associated with a small pleura effusion that is usually exudative and may be hemorrhagic; not specific
HAMPTON'S LINE
radiolucent collar of granulation tissue across the base of an ulcer
HAND-SCHάLLER-CHRISTIAN TRIAD
in multifocal Langerhans' cell histiocytosis, triad of calvarial bone defects, diabetes insipidus, and exophthalmos
HANGMAN'S FRACTURE
fracture of pars interarticularis of C2, hyperextension injury
HANNINGTON-KIFF SIGN
ipsilateral loss of the adductor reflex with preservation of patellar reflex in strangulated obturator hernia
HANSEN'S DISEASE
leprosy
HANSEN'S STAIN
special stain used to detect eosinophiluria on the urine sediment
HARDY-WEINBERG EQUILIBRIUM
p^2 + 2pq + q^2 for describing the frequency of these genotypes; sexual reproduction doesn't cause a constant reduction in genetic variation in each generation but remains constant
HARRISON'S GROOVE
during active rickets, the protuberant rachitic abdomen pushes the plastic lower ribs outward on a fulcrum formed by the costal attachments of the diaphragm; the line of bending forms a groove or sulcus in the rib cage
HARTMAN'S PROCEDURE
proximal colostomy followed by stapled-off colon or rectum that is left in the peritoneal cavity
HARTNUP DISEASE
an autosomal recessive disorder in which there is a reduction of small-intestinal and renal transport of certain neutral amino acids, including (Trp, a precursor of niacin), causing elevated amino acid excretion in urine and feces
HASHIMOTO'S THYROIDITIS
first described in 1920, goitrous chronic autoimmune thyroiditis; in areas with sufficient iodine, elevated TSH is often viewed as evidence of chronic autoimmune thyroiditis as well as antithyroid antibodies; antithyroglobulin antibodies in 60% of patients and antithryoid microsomal antibodies in 95%
HATCHCOCK'S SIGN
upward pressure applied to the angle of the mandible (ramus) produces tenderness with mumps but no tenderness with adenitis
HAWTHORNE EFFECT
the effect (usually positive or beneficial) of being under study, upon the persons being studied; their knowledge of the study often influences behavior [city in Illinois; site of the Western Electric plant]
HEBERDEN'S NODES
characteristic in women, but not in men, represent prominent osteophytes at the DIP joints in OA (enlargements of tubercles at the articular extremities of the distal phalanges
HEERFORDT-WALDENSTRΦM SYNDROME
sarcoidosis associated with fever, parotid enlargement, anterior uveitis, and facial nerve palsy
HEGAR'S SIGN
softening of the uterus at the junction between the cervix and the fundus during the first trimester of pregnancy
HEINEKE-MUKULICZ PYLOROPLASTY
longitudinal incision through all layers of the pylorus, sewed closed in a transverse direction to make the pylorus nonfunctional (used after truncal vagotom)
HEINZ BODIES
seen in unstable hemoglobin and oxidant stress; precipitates of denatured hemoglobin on red blood cells; only visible when blood is supravital stained (crystal violet); not seen on routine blood smears
HEISTER, SPIRAL VALVES OF
found in the neck of the gallbladder, where tiny folds of mucosal epithelium coalesce to form these valves, may assist in retaining bile between meals
HEMOGLOBIN BART'S
four gamma globin chains, seen in hydrops fetalis (alpha thalassemia), very high oxygen affinity
HEMOGLOBIN GOWER
embryonic, primitive hemoglobin. Gower 1, zeta2 episilon2,; Gower 2, alpha2 epsilon2
HEMOGLOBIN LEPORE
no beta chain; delta chain by delta-beta hybrid
HEMOGLOBIN PORTLAND
zeta2gamma2, primitive embryonic hemoglobin
HENOCH-SCHΦNLEIN PURPURA
systemic hypersensitivity disease of unknown cause characterized by purpuric rash, colicky abdominal pain (presumably due to focal hemorrhages into the GI tract), polyarthralgia, and acute glomerulonephritis; may result from deposition of circulating immune complexes within stuff; hypersensitivity purpura, etiology group A streptococci
HERING, CANALS OF
at the fringes of the portal tract, from the joining of bile canaliculi
HERING'S NERVE
carotid sinus nerve, CN IX, carries information to the vasomotor center in the brainstem
HERING-BREUER REFLEX
stretch receptor (in smooth muscle of airways) reflex, responsible for apnea, i.e. decreased breathing frequency, as a result of lung inflation
HERLITZ SYNDROME
epidermolysis bullosa lethalis; mutation that prevents the folding of laminin 5 Gillis Herlitz, Swedish pediatrician, born 1902
HERMANSKY-PUDLAK SYNDROME
characterized by oculocutaneous albinism, a platelet storage pool deficiency, and ceroid lipofuscinosis from defects of multiple cytoplasmic organelles: melanosomes, platelet dense granules and lysosomes.
HERRING BODIES
neurosecretory granules consisting of either vasopressin or oxytocin in neurohypophysis
HESS TEST
see Rumpel-Leede sign. Noticed phenomenon while treating children with scurvy.
HESSELBACH'S TRIANGLE
where direct inguinal hernias occur, bound by the inguinal ligament, inferior epigastric a., and rectus abdominus muscle
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HILL'S SIGN
in aortic regurgitation, refers to popliteal cuff systolic pressure exceeding brachial cuff pressure by more than 60 mm Hg
HIPPOCRATIC FINGERS
clubbing
HIPPOCRATICA, FACIES
the mask of death following peritonitis, i.e. as a result of vomiting and depressed circulation, the face becomes pinched and anxious, the cheeks hollow, and the eyes dim and beringed with dark circles
HIRANO BODIES
in Alzheimer's, intracytoplasmic proximal dendritic eosinophilic inclusions consisting of actin
HIRSCHPRUNG'S DISEASE
megacolon; congenital disorder characterized by colonic dilatation proximal to an aganglionic, contracted distal colon and rectum; caused by gestational failure of neural crest cells to migrate to distal colon; an AD form has been reported with mutations of the RET gene, and an AR form with mutation of the endothelin-B-receptor gene
HODGKIN'S DISEASE
lymphoma characterized by arising in a single node or chain of nodes, spreading in anatomically contiguous nodes, Reed-Sternberg cells, three subtypes (nodular sclerosis (most common, more common in women), mixed cellularity, lymphocyte predominance (lymphocyte depletion rarer))
HOFFMAN'S SIGN
involuntary flexion of the digits when tapping or striking over the plamar digital aspects of the 2nd, 3rd, and 4th digits in upper motor neuron defect
HOLLENHORST PLAQUES
cholesterol emboli visible as small bright flecks lodged in arterial bifurcations in retina
HOLME'S HEART
single ventricle with normally-related great arteries
HOLMES-ADIE SYNDROME
Adie's pupil, frequently affects young women, benign familial disorder that may be associated with depressed DTRs (especially in legs), segmental anhidrosis, orthostatic hypotension, or cardiovascular autonomic instability, may be caused by degeneration of ciliary ganglion, followed by aberrant reinnervation of the pupilloconstrictor muscles
HOLT-ORAM SYNDROME
AD syndrome, dysplasia of the upper limbs and atrial septal defect, reduction anomalies of the upper limbs, heart hand syndrome from mutation in TBX5
HOMAN'S SIGN
deep thrombi in the larger outflow veins, causing edema of the foot and ankle and producing pain and tenderness on compression of the calf muscles (by either squeezing the calf muscles or forced dorsiflexion of the foot)
HOMER-WRIGHT PSEUDOROSETTES
in neuroblastoma, tumor cells arranged about a central space filled with fibrillar extensions of the cells
HONEYMOONER'S PALSY
radial nerve palsy from compression of nerve between middle third of the humerus between the brachoradialis and forearm extensor muscles
HOOVER'S SIGN
a modification in the movement of the costal margins during respiration, caused by a flattening of the diaphragm; suggestive of empyema or other intrathoracic condition causing a change in the contour of the diaphragm
HOOVER'S SIGN
in hysterical weaknes, patient does not push down into your hand when you ask patient to lift weak extremity
HORNER'S SYNDROME
enophthalmos, ptosis, miosis, and anhidrosis, unilateral; small (miotic) pupil associated with mild ptosis (of the upper lid, not as pronounced as with oculomotor lesions) and sometimes loss of sweating (anhidrosis); if present since infancy, the ipsilateral iris is lighter and blue (heterochromia iridis); topical 4% cocaine will dilate normal pupil but not a desympathectomized pupil
HOUNDSFIELD UNIT
scale named after inventor of CT, water 0 H, -1024 for air to 3000-4000 H for bone, relative scale
HOWELL-JOLLY BODY
in asplenia or malfunctional spleen, nuclear remnants on red blood cells as small, round, darkly-stained nuclear fragments; no special stains necessary; may be seen in 30-50% of adults but not in children with untreated celiac sprue
HOWSHIP'S LACUNAE
resorption pits on bone formed by osteoclasts
HOWSHIP-ROMBERG SIGN
pain along the inner aspect of the thigh; seen with an obturator hernia due to nerve compression
HUNTER'S SYNDROME
mucopolysaccharidosis (II), iduronate sulfatase deficiency, X-linked, affecting degradation of dermatan sulfate and heparan sulfate, resulting in physical deformity and mental retardation, no corneal clouding
HUNTINGTON'S DISEASE
autosomal dominant from expanded CAG trinucleotide repeat 4p16.3 associated with chorea initially as restlessness, psychiatric symptoms, and dementia; Westphal variant, atypical variant where there is progressive rigidity and akinesia with little or no chorea
HURLER'S SYNDROME
AR mucopolysaccharidosis (I H) that is caused by deficiency of alpha-L-iduronidase, with consequent accumulations of the mucopolysaccharides heparan sulfate and dermatan sulfate in the heart, brain, liver, and other organs; progressive deterioration, hepatosplenomegaly, dwarfism, gargoyle-like facies, stubby fingers, corneal clouding, progressive mental retardation, and death by age 10
HURST'S DISEASE
acute hemorrhagic leukoencephalitis
HάRTHLE CELLS
in Hashimoto's thyroiditis, deeply stained colloid or clusters of these oncocytes having an abundant, brightly eosinophilic granular cytoplasm, thought to represent a degenerated state of the follicular epithelium
HUTCHINSON FRECKLE
lentigo maligna, a nonfamilial precursor to lentigo maligna melanoma
HUTCHINSON PUPIL
blown third pupil in uncal herniation
HUTCHINSON'S SIGN
in herpes zoster ophthalmicus, vesicular rash at nasal tip indicating involvement of the external nasal branch of CN V associated with increased incidence of ocular zoster
HUTCHINSON'S SIGN
pigment in the paronychial area suggesting melanoma
HUTCHINSON'S TEETH
smaller and more widely spaced than normal and are notched on their biting surfaces; sign of congenital syphilis
HUTCHINSON'S TRIAD
congenital syphilis manifesting late at around 2 years old with triad of interstitial keratitis, notched incisors, and eighth nerve deafness
HUTCHINSON-GUILFORD SYNDROME
aka progeria a condition in which the normal development of the first year is followed by gross retardation of growth, with a senile appearance characterized by dry wrinkled skin, total alopecia, and bird-like facies; genetics unclear
Note: Eponyms Database Author: Andrew J. Yee, M.D. (website: eponyms.net)
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