SECONDARY GN (GENERAL)

Question 1

What is caused by circulating antibodies directed against an antigenic site on type IV collagen in the GBM?

Answer 1

ANTI-GBM DISEASE

Question 2

What is is a systemic vasculitic syndrome with involvement of the skin, gastrointestinal tract, and joints in association with a characteristic glomerulonephritis?

Answer 2

HSP

Question 3

What is a is a rare giant cell arteritis of unknown pathogenesis characterized by inflammation and stenosis of medium-sized and large arteries, with a predilection for the aortic arch and its branches?

Answer 3

TAKAYASU ARTERITIS

Question 4

What is a systemic vasculitis with giant cell involvement characterized by segmental transmural inflammation of medium-sized and large elastic arteries by a mixed infiltrate of lymphocytes, monocytes, polymorphonuclear leukocytes?

Answer 4

TEMPORAL ARTERITIS

Question 5

What is a systemic necrotizing vasculitis primarily affecting medium-sized, muscular arteries, often at branch points, producing lesions of varying ages with focal aneurysm formation?

Answer 5

PAN

Question 6

What is now considered an autoimmune “conformeropathy” involving perturbation of the quaternary structure of the α345 NC1 hexamer of type 4 collagen?

Answer 6

GOODPASTURE SYNDROME

Question 7

What disease is characterized by a chronic inflammatory cell infiltration of the exocrine salivary and lacrimal glands and is associated with the “sicca complex” of xerostomia and xerophthalmia?

Answer 7

SJOGREN SYNDROME

Question 8

What disease is characterized by the extracellular deposition of characteristic fibrils in various organs?

Answer 8

AMYLOIDOSIS

Question 9

What disease should be suspected in all patients with circulating serum monoclonal M proteins?

Answer 9

AMYLOIDOSIS

Question 10

What are the 3 types of Fibrillary renal disease?

Answer 10

1. FIBRILLARY
2. IMMUNOTACTOID
3. FIBRONECTIN GLOMERULOPATHY

Question 11

What is a systemic disease caused by the overproduction and extracellular deposition of a fragment of monoclonal immunoglobulins?

Answer 11

MIDD

Question 12

What is the disease condition wherein patients have an abnormal circulating monoclonal IgM protein in association with a B-cell lymphoproliferative hematologic disorder?

Answer 12

WALDENSTROM MACROGLOBINEMIA

Question 13

What is an inherited (X-linked) disorder of collagen α3α4α5(IV) with almost inevitable progression to ESKD, and is frequently associated with hearing loss and ocular abnormalities?

Answer 13

ALPORT SYNDROME

Question 14

What is the other name of Nail-Patella Syndrome?

Answer 14

HEREDITARY OSTEO-ONYCHODYSPLASIA

Question 15

What is an autosomal dominant condition affecting tissues of both ectodermal and mesodermal origin, manifested as symmetrical nail, skeletal, ocular, and renal anomalies?

Answer 15

Nail–patella syndrome (NPS)

Question 16

What disease that results from an X-linked inborn error of glycosphingolipid metabolism involving a lysosomal enzyme, α-galactosidase A (also known as ceramide trihexosidase), and caused by mutations of the GLA gene.

Answer 16

FABRY DISEASE

Question 17

What is the defect in Fabry Disease?

Question 18

What is the mutation in Fabry Disease?

Answer 18

GLA GENE

Question 19

What is a disorder that is associated with insulin resistance in which there is loss of fat, which may be localized to the upper part of the body or more diffuse?

Answer 19

LIPODYSTROPHY

Question 20

What is a familial disorder characterized by proteinuria, anemia, hyperlipidemia, and corneal opacity?

Answer 20

LCAT

Question 21

What disease is a characterized by dysbetalipoproteinemia and lipid deposition in the kidney, leading to glomerulosclerosis and renal failure?

Answer 21

Lipoprotein glomerulopathy (LPG)

Question 22

Refers to the pattern of sclerosing glomerulopathy in HIV-infected patients

Answer 22

HIVAN