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61 Cards in this Set

  • Front
  • Back

Pancreatic Mass

1. SPEN


2. Congenital Cyst


3. Lymphangioma


4. Pseudo cyst


5. necrotic Lymphnode

Renal, Hepatic, and Pancreatic Cyst

VHL

VHL

Renal, pancreatic, hepatic Cysts


Inc RCC


Hemangioblastoma

Denys-Drash

Triad


1. Medullary Glomerulosclerosis


2. Wilm's


3. Ambiguous genitalia

WAGR

Wilms, Aniridia, Genitourinary Anomaly, retardation

Frasier Syndrome

Chronic Renal Failure, XY gonadal dysgenesis, increased gonadalblastoma

What to screen for in esophageal atresia?

VACTERL

What study needs to be done when a pt is found to have meconium ileus?

Sweat Test to look for CF

Omphalocele

Midline


Liver and bowel


Covered


Has malrotation


Elevated AFP

Gastroschisis

Off midline


Bowel only


Not covered


Elevated AFP

Clinical syndrome with henangioendothelioma

CHF

Multi cystic dysplastic kidney

Unilateral, Multiple cysts, non functioning renal tissue. Bilateral is not compatible with life.

Types of nephroblastomatosis

Intralobar and perilobar

What syndromes are associated with nephroblastomatosis?

Beck width-Weideman, WAGR, sporadic aniridia

Cloacal Exstrophy (OEIS)

Widened pubis, omphalocele, Exstrophy of the bladder, spinal defects

What syndrome is associated with RCC in kids?

Tuberous Sclerosis

Wilm's Tumor is associated with what syndromes (5)

Nephroblastomatosis, WAGR, Beckwidth Weidman,

Disease

Caffey's - infantile cortical hyperostosis

What is the defect in osteopetrosis?

Osteoclasts - impaired absorption

Erlenmeyer flask

Fibrous dysplasia, anemia

Vertebra Plana

Langerhan's Histiocytosis

Alagille syndrome assc with which liver tumors?

Hepatoblastoma and HCC

Osteomyelitis occurs in what part of bone in non neonates?

Metaphysis- rich blood supply

Osteomyelitis occurs where in neonates?

Metaphysis and epiphysis because of transphyseal vessels.

McCune Abright Syndrome (3 things)

1. Polyostotic Fibrous Dysplasia


2. Precocious puberty


3. Hyper pigmentation

Where is the third tumor in hereditary retinoblastoma?

Pineal gland

Disease

Caffey's - infantile cortical hyperostosis

Defect in muscle eye brain disease.

O-mannosyl glycan synthesis.

Joubert syndrome

- molar tooth brain stem


- absent vermis


- ataxia and MR


- renal issues

Birt Hogg Dube

multiple lung cysts and secondary spontaneous pneumothoraces


multiple bilateral renal tumors (particularly chromophobe renal cell cancer and oncocytoma)


cutaneous manifestations (angiofibromas, perifollicular fibromas, acrochordons, fibrofolliculomas, etc.)

Parotitis and thymic cysts

HIV

Rosai Dorfman Disease

Benign reactive lymphadenopathy with dilated sinuses

What heart defect is associated with Alagille syndrome?

TOF

Kikuchi syndrome

Histiocytic necrotizing LAD

Kawasaki Disease

Mucocutaneous lymph node syndrome

Choanal Atresia associated with what acronym disease

CHARGE (Coloboma, Heart, Choanal atresia, Redardation of growth, Genital, Ear anomalies)

High output cardiac failure

Hemangioendothelioma


Infantile Hemangioma

Infantile hemangioma is associated with what?

Hypothyroidism

Schwachman Diamond Syndrome

Exocrine pancreatic insufficiency


Bone marrow dysfunction


Skeletal abnormalities

Kasabach Meritt Syndrome

Hemangioma Thrombocytopenia - mass traps blood causing coagulation

Lesion in Kasabach Meritt

Henangioendothelioma

Beckwith Weidemann Syndrome

Exophalos


Macroglossia


Gigantism


Hemihypertrophy


Wilms tumor


Hepatoblastoma


Nephroblastomatosis

Front (Term)

Morquio

What is Alagille syndrome?

AD inherited


Few or absent bile ducts


TOF


Butterfly vertebrae

Loeys Dietz Syndrome

Dilated aortic root.

Scurvy

Scurvy line- dense metaphyseal band with lucency along the diaphyseal side

Stipples Epiphyses

Chondroplasia Punctata


Conradi-Hunermann Syndrome

Where does the periosteal resorption of bone occur in hyperparathyroidism?

Radial side of middle phalanges


Distal tufts (acroosteolysis)

Double layer patella

Multiple epiphyseal dysplasia

Front (Term)

Morquio

The duct of (blank) joins with the CBD and inserts into the (blank) papilla.

Wirsung


Major papilla

In pancreatic divisum, which papilla is superior?

Minor is superior - connects to Dorsal duct (Santorini)


Major is inferior

Cystic mass in infant

Cystic nephroma


Partially cystic nephroblastoma

Meyer Rokatansky Kauster Hauser Syndrome

Right kidney agenesis


Uterine agenesis


Partial Vagina hypoplasia


Middle ear and hearing loss

Middle Ear and Hearing loss associated with what other anomaly

Meyer Rokatansky KH

McCune Albright

Polyostotic Fibrous dysplasia


Precocious Puberty


Cafe au lait spots

What syndrome has GLUT1 reactivity?

Infantile Hemangioma

What syndrome has congenital hypothyroidism?

Infantile Hemangioma

Treatment for Infantile Hemangioma?

Propranolol

Hepatic tumor with elevated afp?

Hepatoblastoma

Cystic hepatic mass on CT and MR but solid on US?

UES Undifferentiated Embyroneal Carcinoma