28. Acute nephritic syndrome. Acute renal injury. Flashcards

Question

what are the clinical manifestations of good pasture syndrome

Answer 1

lungs antedate the kidney hemoptysis cough dyspnea nephritic syndrome symptoms

Answer 2

serum antibody -ELISA assay for circulating anti-GBM antibodies renal biopsy immunofluorescence prescence of anti GBM antibodies- IgG and complement (C3) along the basement membrane. Predominantly, subclass IgG-1 specifically showing a strong linear ribbon-like appearance x ray/ ct - alveolar haemorrhages A chest radiograph shows patchy parenchymal opacifications, which are usually bilateral and bibasilar. The apices and costophrenic angles are generally spared

Answer 3

6-30 years

Answer 4

may be critically ill on presentation - Urgent hemodialysis often is needed for standard indications, and intubation for respiratory failure Upon diagnosis, patients should be started on prednisone, cyclophosphamide, and daily plasmapheresis daily plasmapheresis is performed until anti-glomerular basement membrane antibodies are undetectable 1.5-times plasma volume on a daily basis for at least 5 days, followed by a change to alternate day for an additional 6-7 treatments. Replacement is usually with fresh-frozen plasma or 5% albumin with steroid 6 months and 3 moths cyclophosphamide continuing months until full remission is achieved Rituximab f therapy is initiated early: full recovery of renal function without treatmnet rapid progressive

Answer 5

Acute kidney injury (AKI) (previously called acute renal failure) is characterized by a reversible increase in the blood concentration of creatinine and nitrogenous waste products and by the inability of the kidney to regulate fluid and electrolyte homeostasis appropriately

Answer 6

Pre-renal injury Decreased true intravascular volume = In children, gastroenteritis is the most common cause of hypovolemia and can result in pre renal AKI adrenal diseases, central or nephrogenic diabetes insipidus, i burns, and in disease states associated with third space losses, such as sepsis, nephrotic syndrome, Congenital and acquired heart diseases ========= Intrarenal Acute tubular necrosis (vasomotor nephropathy) Rapidly progressive glomerulonephritis HEMOLYTIC UREMIC SYNDROME - MOST COMMON CAUSE OF AKI in children secondary to medications (eg, aminoglycosides, NSAIDs) Acute interstitial nephritis (AIN) as a result of a reaction to a drug or due to idiopathic AIN. Children with Medications commonly associated with AIN include methicillin and other penicillin analogs, cimetidine, sulfonamides Renal artery thrombosis Renal vein thrombosis contrast nephropathy ========= Postrenal congenital malformation in the kidney and collecting system Bilateral ureteral obstruction posterior urethral valves, bilateral ureteropelvic junction obstruction or bilateral obstructive ureteroceles ========= intrinsic in neonates cortical necrosis and renal vein thrombosis, occur more commonly in neonates maternal drugs in utero that interfere with nephrogenesis such as angiotensin-converting enzyme inhibitors, angiotensin receptor blockers and nonsteroidal anti-inflammatory drugs

Answer 7

urine osmolality, urine sodium concentration, the fractional excretion of sodium, and the renal failure index, have all been proposed to be used to help differentiate pre-renal injury from hypoxic/ischemic AKI Renal tubules are working appropriately in pre-renal injury and are able to conserve salt and water appropriately, whereas, in vasomotor nephropathy, the tubules have progressed to irreversible injury and are unable to conserve salt appropriately pre-renal injury the tubules respond to decreased renal perfusion by appropriately conserving sodium and water such that the urine osmolality is greater than 350 mosmol/l, (400-500 - older children) urine sodium is less than 20-30mEq/l, (10-20) and the fractional excretion of sodium is less than 2.5 percent (1percent) Serum BUN:creatinine ratio > 20:1 Fractional excretion of sodium Intrinsic BUN:creatinine ratio < 15:1. urine sediments Hyaline casts ====== intrinsic urine osmolality is less than 350 mosmol/l, the urine sodium is greater than 30–40 mEq/l, and the fractional excretion of sodium is greater than 2.0%. ``` Muddy brown, epithelial, or granular casts (ATN) RBC casts (glomerulonephritis) Fatty casts (nephrotic syndrome) ``` ===== post renal urine sediment absent urine osmolality - <350

Answer 8

rash, fever, arthralgias, eosinophilia, and pyuria with or without eosinophiluria.

Answer 9

withdrawal of the drug implicated in causing the AIN. In addition, corticosteroids may aid in the resolution of the renal failure

Answer 10

thrombotic microangiopathy in which microthrombi, consisting primarily of platelets, form and occlude the arterioles and capillaries. These occlusions result in the simultaneous occurrence of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI).

Answer 11

mainly children under 5 years of age

Answer 12

bacterial toxins, most commonly the Shiga-like toxin (verotoxin ) of enterohemorrhagic Escherichia coli (E. coli) transmitted via contaminated fod shiga toxin - shigella dysenteriae strep pneumonia infection

Answer 13

diarrhoea illness commences for the past 5-10 days bloody stools dehydration thrombocytopenia - petechia , purpura mucosal bleeding ``` micorangiopathic hemolytic anemia fatigue dyspnea pallor jaundice ``` irritability seizures occur ``` impaired renal function hematurea , protein urea oliguria anuria volume overload ``` 2) Atypical HUS: • have a similar microangiopathic syndrome, only that they have a preceding or concomittend pneumonia

Answer 14

diarrhea and presence of toxin-producing e.coli have resolved by the time HUS is diagnosed peripheral blood smear reveals microangiopathic hemolysis schistocytes ``` haematology reduced hemoglobin reduced haptoglobin increased indirect bilirubin increased reticulocytes Increased ldh ``` coagulation profile deceased platelets normal or slightly elevated pt and aptt increased FDP and D- dimer results serum chemistry - increased BUN and creation urinanalysis - hematurea and proteinurea

Answer 15

Volume substitution → Early hydration during the diarrheal phase may lessen the severity of renal insufficiency isotonic hydration • control of hypertension (not with diuretics or RAAS drugs!) → CCB such as amlodipine Dialysis: ◦ 50%of patients require a period of dialysis ◦ Peritoneal dialysis is widely used for pediatric patients Red blood cell transfusions Transfuse platelets only if the patient has active bleeding ( should be avoided because they may add to the thrombotic microangiopathy) • Antibiotics and antidiarrheal agents may increase the risk of developing HUS (—> lysis of bacteria and more release of toxins) anti motility drugs not given • Most children (>95%) with the classical HUS survive the acute phase and recover normal renal function ====== atypical hus plasma therapy +immunosuppresive therapy eculizmab renal +liver transplant

Answer 16

CNS Seizures Paresis Stroke GI tract Hemorrhagic colitis Bowel necrosis, perforation, stricture Peritonitis Heart: ischemia and fluid overload Pancreas: transient or permanent diabetes mellitus Liver: hepatomegaly, transaminase elevations

Answer 17

Markedly elevated serum CPK level ``` Hyperkalemia, metabolic acidosis, hyperphosphatemia, hypocalcemia, hyperuricemia, and/or dyslipidemia (especially hypertriglyceridemia) may be seen. Low Hb (anemia) due to decreased EPO ``` Urine test findings Renal tubular epithelial cells or granular, muddy brown, or pigmented casts Urine dipstick is positive for blood but negative for RBCs in rhabdomyolysis

Answer 18

Postrenal: Remove outflow obstructions with Foley catheter insertion, an indwelling bladder catheter, nephrostomy, or stenting.

Answer 19

occurs in less than 10 years! preceding infection Up to 90% of cases preceded by viral or bacterial infection 1–3 weeks prior Most commonly an upper respiratory tract infection caused by group A Streptococcus [4] GI infections also possible

Answer 20

Skin: (∼ 100% of cases) Symmetrically distributed, raised, erythematous macules or urticarial lesions that coalesce into palpable purpura Most common sites: the lower extremities, buttocks, ======= Joints: arthritis/arthralgia, most common in the ankles and knees ====== Gastrointestinal tract Colicky abdominal pain (may be severe enough to mimic an acute abdomen) Can cause intussusception Bloody stools or melena Nausea/vomiting ``` ===== Kidneys IgAV nephritic syndrome or worst nephrotic syndrome ====== scrotal swelling, pain, and tenderness ``` ====== central and peripheral nervous system headaches, seizures,

Answer 21

Complete blood cell count with differential ↑ Platelet count Thrombocytopenia would indicate a condition other than IgAV. Mild normochromic anemia may appear as a result of GI bleeding increased wbc ===== IgA in serum high low complements Strep infection - streptolysin O Serum chemistry - BUN and creatinine high ==== skin biopsy - leukocytoclastic vasculitis with igA and C3 immune complex deposition in small vessels = hallmark ======== nephritic urineanalysis renal biopsy lm - mesangial proliferation IF -mesangial iga deposits c3 and fibrin

Answer 22

bad prognosis signs are - hypertension proteinurea fre than 1g/24 hours rapidly progressive crescentic glomerulonephritis IgAV are self-limiting only require supportive care (e.g., pain management) Systemic glucocorticoids for severe abdominal pain not relieved by NSAIDs IV fluids to maintain hydration ===== renal patients with isolated hematurea / persistent proteinurea >1mg/day or no porteinurea / normal GFR / no signs of progressive disease / normal serum creatinin general interventions to slow progression ACE and ARB ===== nephrotic range porteinurea despite ACE and ARB renal biopsy with more severe histological changes but no chronic changes rising creatinin reduced GFR therapy with prednisolone such as minimal change disease and the following therapy for nephrotic syndrome ======= progressive active disease heamturea with increasing porteinurea and serum creating despite ace and arb and glucorticoids two regimens used IV methylprednisolone 0.5-1g/kg /day for three consecutive days at the beginning months one , three and five with alternating daily oral prednisone 0.5mg/kg for six months avid pulse therapy 2mg/kg prednisone every other day for two months with rapid taper to dose of 0.5mg/kg every other day for additional four months still persistent prednisone 1mg/kg for two three months with tapering down to maintenance of 10mg/day and cyclophosphamide 1.5mg/kg/day orally for three months ===== crescentic glomerulonephritis IV pulse glucocorticoids + cyclophosphamide iv methylprednisolone 3 days followed by oral prednisolone for to to three months then slow taper to maintenance with IV cyclophosphamide monthly for three months atleast can use azathioprine micophenolate

Answer 23

extrarenal clinical signs found only in HSPN 15 and 30 years for a diagnosis of IgAN, whereas HSPN is mainly seen in childhood Nephritic and/or nephrotic syndromes are more often seen at presentation in HSPN. HSPN has been described in association with hypersensitivity