Hereditary Angioneurotic Edema

Etiology
D/t deficiency of C1 esterase inhibitor

Pathogenesis
Increased production of bradykinin and c2 kinins due to enhanced activity of activated C1 and enhanced expression of activated kallikrein and factor 12

Laboratory features
. ↓C1 esterase inhibitor (Type 1)
. Dysfunctional/non functional C1 esterase inhibitors (Type2)
. Normal C1 levels with reduced C4 and C2 levels
. ↑ levels of bradykinin and C2 kinins

Clinical features
. Positive family history
. Recurrent episode of edema ( characteristic provoked by emotional stress or trauma)
. Laryngeal edema
. Edema affects intestinal mucosa (colic)
. Affects skin and extremities

JVP, details, abnormalities in JVP

Haemophilia A and Von Willebrand ds , features, lab investigation, differences

Haemophilia A is an X linked disorder that affects only BOYS AND MEN.

Von Willebrand disease is an autosomal inherited condition and may afferct both BOYS and GIRLS.

Both disorders cause bleeding in joints along with prolongation APTT and normal PT.

Antiphospholipid syndrome (APS) definition, causes, lab findings

Antiphospholipid antibody syndrome is associated with thrombotic disorders and not with bleeding disorders.
           It is primarily a disorder of coagulation resulting in a hypercoagulation state.

STERILE PYURIA CAUSES

STERILE PYURIA
Definition:- presence of more than 5 WBC per HPF without any bacteria on routine urinalysis is termed as sterile pyuria.

CAUSES
1. Infectious
2.Non-infectious

INFECTIOUS CAUSES
1. Tuberculosis of urinary tract
2. Chlamydia trachomatis
3. Mycoplasma
4. Ureplasma
5. Fungi
6. Inadequate treated UTI
( compiled from Harrison 17th, spiral manual of nephrology 7th, spiral manual of clinical infectious disease 5th, oxford textbook of nephrology 3rd)

NONINFECTIOUS CAUSES
1. Interstitial nephritis
2. Renal papillary necrosis
3. Urate nephropathy
4. Nephrocalcinosis
5. PCKD
6. Calculi
7. Prostatitis
8. Vasicouretheral reflux
9. SLE

Esophagial dysphagia causes

Non progressive (episodic) dysphagia only for SOLIDS is characteristic of LOWER ESOPHAGEAL RINGS (Schatzki's ring)

Progressive dysphagia for SOLIDS in PEPTIC STRICTURE and Ca ESOPHAGUS.

Progressive dysphagia for both SOLIDS and LIQUID in ACHLASIA