Renal

Question 1

Autosomal dominant polycystic
Hypertension in ADPKD
DD from ARPKD

Answer 1

ADPK
●Gross or microscopic hematuria/
● Abdominal, flank, or back pain
●Proteinuria/Hypertension
●Infection of the cyst/
●Rarely, renal insufficiency during childhood
Hypertension has been attributed to a reduced renal blood flow due a (Compression) by cysts and a subsequent activation of the renin angiotensin system
DD; The ultrasonographic appearance may not distinguish autosomal recessive from autosomal dominant disease.In this setting, a careful family history and analysis of the parents are helpful. Affected parents with ADPKD under the age of 25 to 30 years may not yet have cysts detectable on ultrasonography and establishing the diagnosis may require evaluation of the grandparents In addition, extrarenal (hepatic, pancreatic) cysts and cerebral Anurysm also favor the presence of autosomal dominant disease, while portal fibrosis or signs of portal hypertension, cholangitis, or biliary dysgenesis favor the diagnosis of autosomal recessive disease.
Ttt; Treatment is focused on managing pain and complications including hypertension and urinary infections. Children with significant nephromegaly may wish to avoid contact sports due to increased risk of cyst rupture with minor trauma.
We also advise affected children to avoid nonsteroidal anti-inflammatory drugs due to potential for increased risk of cyst hemorrhage. In addition, patients should have a large daily fluid intake and avoid caffeine intake to suppress antidiuretic hormone secretion and prevent stone formation.

Other promising therapies, which are being tested in clinical trials, include vasopressin receptor antagonists, mammalian target of rapamycin (mTOR) signaling pathway inhibitors, and rigorous blood pressure control with maximal inhibition of the renin-angiotensin-aldosterone system

Question 2

Vasculitis classification

Answer 2

The 2013 CHCC classification scheme is as follows:
●Large-vessel vasculitis
•Giant cell (temporal) arteritis
•Takayasu arteritis
●Medium-vessel vasculitis
•Polyarteritis nodosa
•Kawasaki disease
●Small-vessel vasculitis
•Antineutrophil cytoplasmic antibody-associated vasculitis
-Microscopic polyangiitis
-Granulomatosis with polyangiitis – Previously Wegener’s granulomatosis
-Eosinophilic granulomatosis with polyangiitis – Previously Churg-Strauss syndrome
•Immune complex small-vessel vasculitis
-Antiglomerular basement membrane disease
-Cryoglobulinemic vasculitis
-IgA vasculitis (Henoch-Schönlein purpura)
-Hypocomplementemic urticarial vasculitis
●Variable-vessel vasculitis
●Behçet’s syndrome
●Cogan’s syndrome
●Single-organ vasculitis
●Vasculitis associated with a systemic disease
●Vasculitis associated with probable etiology