Nephrolithiasis

Question 1

dietary factors that lead to nephrolithiasis

Answer 1

low fluid intake, calcium, K
high oxalate, protein, Na, sugar drinks

hipercalciuria= droga =tiazidicos-diminuir proteina, sodio, acucar e ingesta de calcio adequada

hiperoxaluira-diminuir a ingestao de oxalato, aumentar a ingesta de calcio,evitar vitamina c- piridoxina

hiperuricosuria= evitar purinas= inibidor da xantila oxilase

hipocitraturia= citrato de k, diminuir a ingesta de proteina, aumentar ingesta de frutas e vegetais

Question 2

Inhibitors of stone formation

Answer 2

magnesium

, citrate

, pyrophosphate→retards the crystal growth of CaP and CaOx crystals by binding to the surface of basic CaP crystals

THprotein

Question 3

acts as both promoter and inhibitor of stone formation THProtein

Answer 3

THProtein

Tamm-Horsfall protein and uromodulin are two names for the same gene.

Casts only form in the distal tubule & collecting duct, not in the proximal tubule. What is its function?

1. Tamm-Horsfall protein acts as a constitutive inhibitor of calcium-based stone formation. Mice deficient for Tamm-Horsfall protein show an increased tendency towards nephrolithiasis.
2. Tamm-Horsfall protein acts to prevent urinary tract infection. There is some data that certain strains of E. coli may be bound by Tamm-Horsfall protein; once cleaved this could represent a means of eliminating the organism from the urinary tract.
3. Mutations in Tamm-Horsfall protein cause the autosomal dominant disorder medullary cystic kidney disease type 2 (MCDK2) as well as the disorder familial juvenile hyperuricemic nephropathy (FJHN) . This is a pediatric-onset disease characterized by hyperuricemia, gout, and progressive renal failure. Interestingly it appears that the pathophysiologic mechanism here is that mutations in this gene lead to defects in protein folding and intracellular deposition of mutant Tamm-Horsfall protein.

Question 4

metabolic processes that induce ca stone formation

Answer 4

Increase urine ca >250-300 mg/day;

Urine Uric acid > 800 mg/day

, urine oxalate > 45 mg/d

, hipocitraturia < 320 mg/day;

alterations in urine ph

Question 5

most common metabolic abnormality in ca stone formation

Answer 5

absorptive hypercalciuria

most common cause of resoprtive hypercalciuria primary hyperparathyroidism

uso de tiazidicos se hipercalciuria >200

Question 6

clinical conditions with hypocitraturia

Answer 6

overproduction acidosis,

underexcretion acidosis

K deficiency

excess dietary protein

Question 7

inflammatory bowel disease, jejunoileal bypass, bariatric surgery for morbid obesity

Answer 7

enteric hyperoxaluria

Question 8

predispose to caphos lithiasis

Answer 8

alkaline urine (pH > 6.7)

Question 9

most impt in uric acid stones

Answer 9

low urine pH

Uric Acid crystals

Precipitate in acid urine • Pleomorphic,rhombic plates or rosettes. • Seen under polarised light • Hyperuricemia • Diabetes mellitus • Metabolic syndrome • Acute uric acid nephropathy • Chronic uratenephropathy • Familial Juvenile Hyperuricemic Nephropathy (Medullary Cystic Kidney disease

Alkalinization of urine—urine pH is most important risk factor for uric acid stones – Potassium citrate or bicarbonate – Patient follow urine pH (aim for ~6.5 or higher

Question 10

patients with gouty arthritis and kidney stones, UA > 10 md/d, urine ua > 1000 mg/day

Answer 10

hyperuricosuria

Question 11

Increases saturation of all stone forming elements

Answer 11

low urine volume

Question 12

Pathophysio of uric acid stones

Answer 12

Low urine pH, volume
Hyperuricosuria

Question 13

genetic disease caused by inactivating mutations of the subunits of a dibasic aminoacid transporter in then proximal tubule

Answer 13

cystinuria

family hx of cystinuria (> 400 mg/day vs 30 mg), staghorn calculi, hexagonal crystals on urinalysis

Question 14

most prevalent component of kidney stones

Answer 14

calcium oxalate

Question 15

most likely cause of nephrolithiasis in a patient with distal RTA

Answer 15

hypocitraturia

Question 16

depressions near the papillary tips, yellow crystalline deposits in the ducts of bellini, some randall plaques

Answer 16

Calcium phosphate

Question 17

1st kidney stone work up

Answer 17

medical hx, stone analysis, urine analysis

Question 18

Recurrent kidney stone

Answer 18

full metabolic evaluation - serum panel, PTH, VitD if hyperCa, 24ag urine >= 2 samples Na Ca Oxalate Uric acid citrate

calcio, citrato, oxalato, sodio, creatinina,acido urico

Complete Metabolic Evaluation for Nephrolithiasis

Blood

Serum calcium (if elevated, suggests primary hyperparathyroidism or other cause of hypercalcemia)Serum electrolytes (low total CO

2

raises the possibility of distal RTA)

Urine

Urinalysis—urine pH >7 with phosphate crystals in the urine sediment suggests calcium phosphate or struvite stones (struvite stones have a typical coffin-lid appearance); hexagonal cystine crystals arediagnostic of cystinuria; other crystals (calcium, uric acid, indinavir, etc.) may suggest etiology of stones24-hour urine collection(s) for urine stone risk diagnostic profile. This test is now standardized in mostclinical laboratories, with most using a reference laboratory that provides a graphical display of results(seeFig. 20

Question 19

indicative of minimal fluid intake

Answer 19

urine volume less than 2.5L

Question 20

pH with increase risk of uric acid precipitation, idiopathic uric acid stone, intestinal disease and diarrhea and intestinal bypass surgery

Answer 20

less than 5.5

Question 21

Increase risk of caphos precipitation, dRTa, primary hyperparathyroidism, alkali, ca tx

Answer 21

pH > 6.7

Question 22

pH that indicates urinary tract infection fom urease producing bacteria

Answer 22

> 7-7.5

Question 23

Normal UCrea

Answer 23

F: 15-20 mk
M: 20-25 mk

Question 24

reflects dietary Na and K intake

Answer 24

24H urine Na and K

O valor obtido em mEq/24h deve ser dividido por por 17 (Já que 1 g de NaCl corresponde a 17 mEq de sódio, dividimos o resultado por 17 para obter a quantidade em gramas de NaCl consumido no dia).

3- O KDIGO (Kidney Disease Improving Global Outcomes) de Hipertensão e Doença renal crônica, recomenda a ingestão < 2g de Sódio ou 5 gramas de NaCl por dia.

Question 25

Major cause of hypercalciuria

Answer 25

High Na intake

Question 26

Normal Urine Na K Ca

Answer 26

Na 100 K 40-60 Ca \< 250-300 mg

Question 27

Low Mg increases risk of

Answer 27

calcium stones | (UMg \<30-120 mg)

Question 28

Higher Uphos \> 1100 mg

Answer 28

calcium phosphate formation

Question 29

hyperuricosuria, Uuric acid \> 600-800

Answer 29

CaOx stones Uph \> 5.5 Uric acid stones UpH \<5.5

Question 30

marker of dietary acid intake

Answer 30

sulfate

Question 31

high ammonium/sulfate ratio

Answer 31

GI alkali loss

Question 32

Normal Urine NH4

Answer 32

30-40 meq

Question 33

Petsistent high Uca on a restricted diet

Answer 33

intestinal hyperabsorption of Ca

Question 34

Elevated fasting Ca/Crea (0.11 mg/100 ml or \<2.7 umil/100 mg), high serum calcium, elevated pth

Answer 34

primary hyperparathyroidism

Question 35

Elevated Ca/Crea, normal serum Ca, normal or suppressed PTH

Answer 35

resoprtive hypercalciuria

Question 36

elevated Ca/Cr, normal Ca, elevated pth

Answer 36

Renal hypercalciuria

Question 37

Elevated Ca/Cr (0.2 mg/mg or 0.56 mmol/mmol) 4h Ca/Crea after 1 g oral load

Answer 37

absorptive hypercalciuria

Question 38

dumbless shaped crystals

Answer 38

calcium oxalate monohydrate

Question 39

Envelope shaped crystals

Answer 39

calcium oxalate dihydrate

Question 40

Flat shaped or wedge shaped prisms, often in rosettes

Answer 40

Calcium phosphate

Question 41

Gold standard for kidney stone diagnosis

Answer 41

noncontrast ct un enhanced ct scan ct stonogram

Question 42

time when surgical intervention is indicated

Answer 42

4 weeks

Question 43

average days of stone passage

Answer 43

40 days

Question 44

medical expulsive therapy

Answer 44

tansulosina, doxasozina indicacoes-\> calculo maior que 0,5 e menor q 1,0 em ureter distal sem infeccao, sem insuf renal, sem dor calculo menor q 1cm expulsoiu? nova imagem 1 mes apos

Question 45

target fluid intake

Answer 45

Urine volume \> 2.5L cystinuria \> 4L water intake

Question 46

Dietary mgt

Answer 46

Na \< 100 meq/day animal protein 50-60 g/day Ca 1000-1200 mg avoid \> 1g Vit C Fruits/vegetables

Question 47

Drug of choice hypercalciuria in caOx and Caphos stone formers

Answer 47

Thiazides 25 bid

Question 48

Tx hypocitraturia in caox and caphos, uric acid stones, cystine stones

Answer 48

20-80 meqs 3-4 doses uric acid: pH goal \> 6, cysteine pH \> 7,

Question 49

inhibits caox crystal formation and growth binding with oxalate

Answer 49

Magnesium

Question 50

Treatment Ca stone formers with hyperuricosuria

Answer 50

Allopurinol 100-300 mg/d

Question 51

Struvite stones when other interventions have failed

Answer 51

acetohydroxamic acid 15 mkd

Question 52

first line therapy for uric acid stone formers

Answer 52

Urine alkalinization

Question 53

tx of choice struvite stones, impacted stone; largest stone free rate and less recurrenfe rate

Answer 53

percutaneous nephrolithotomy

Question 54

hexagonal stones- tx

Answer 54

Cystine, tiopronin penicillamine thiola captopril treatment • Reduce [cystine] \< 250mg/L • Increase fluid intake? – Urine output \> 5 L/d • Tiopronin (“Thiola”) – Typically need at least 900 mg/d (300 mg TID) • Continue alkalinization= bic ou citrato de k= alvo ph 7,7 7,5 RESTRICAO DE SODIO E PROTEINA ANIMAL

Question 55

coffin lid like

Answer 55

struvite

Question 56

uric acid stones are

Answer 56

pleomorphic nao da pra ver na radiografia

Question 57

calculo de estruvita

Answer 57

são chamados de “cálculos de infecção” e são compostos por uma mistura de magnésio, amônia, fosfato e carbonato de cálcio. Alguns tipos de bactérias formam amônia, elevam o pH, deixando a urina mais alcalina e promovendo a formação de estruvita. bacterias produtoras de urease= proteus, klebsiella, cirurgia

Question 58

hiperoxaluria primaria autosomal recessive condition in childhood with CaOx stones and nephrocalcinosis, frequent stone recurrence

Answer 58

disturbio do metabolismo hepatico do oxalato demais funcoes hepatocelulares=normais achados= osseos, coracao, tecidos e sangue nefrolitiase, nefropatia por oxalato de calcio (nefrocalcinose) e drc o q agrava= hipovolemia, inibidor do sraa, uso de diuretico, aumento da ingesta de OXALATO, use de antiinflamatorios alguns antibioticos tipo1 = tx de figado e rim tipo2 =tx renal tipo3 =nao requer tto autosomal recessive condition in childhood with CaOx stones and nephrocalcinosis, frequent stone recurrence

Question 59

UOxalate \> 100 mg/day

Answer 59

primary hyperoxaluria

Question 60

hiperoxaluria secundaria

Answer 60

aumento da abs intestinal de oxalato= nao absorve a gorduta , normal o oxalato se liga ao calcio e sai pelas fezes, quando tem muita gordura = se liga ao calcio e deixa o oxalato livre para ser absorvido cirurgia bariatrica y de roux by pass jejunoileal doença inflamtoria intestinal aumento da ingestao de oxalato excesso de vitamina c fibrose cistica /insuf pancreatica/

Question 61

hiperoxaluria

Answer 61

\>0,5 enterica= calcio 1 a 2g , depende da excrecao de oxalato + agnesio 200-400mg/dia \>1,0mmol /dia = primaria= piridoxima 5-20mg/kg/dia **novas drogas oxalobacter** lumasiram /nedosiram individuals likely to form oxalate stones due to hyperoxaluria have a relative deficiency of the gut bacterium Oxalobacter formigenes, part of the normal GI flora which naturally digest oxalate.

Question 62

hiperoxaluria

Answer 62

aumentar ingesta hidrica= \>2-3L/dia dieta com pouco oxalato dieta pobre em gorduras calcio normal , suplemento decalcio colestiramina= absorve sair biliares e o oxalato oxalobacter formigens

Question 63

calculos de indinavir

Answer 63

nao da pra ver no exame de imagem

Question 64

composicao dos calculos frequencia

Answer 64

Calcium oxalate 60 Calcium oxalate and phosphate 10 Calcium phosphate 10 Magnessium, ammonium phosphate(struvite)5-10 Uric acid5-10 Cystine1Others1 Crystalline composition of renal calculi & their frequency

Question 65

análise fisica do calculo

Answer 65

difracao do rx espectrocopia de infravermelho microscopia eletronica

Question 66

Urease producing bacteria are associated with the formation of?

Answer 66

Magnesium ammonium phosphate stone (Struvite). ## Footnote .Struvite (magnesium ammonium phosphate) stones are typically largestones associated with urease producing bacteria and an **alkaline urine4** . Ureabreakdown produces excess ammonium and hydroxyl ions, a rise in urinary pH,and a decrease in phosphate solubility thus encouraging the precipitation of insoluble magnesium ammonium phosphate Common bacteria producing urease Proteus Haemophilus Pseudomonas Klebsiella Yersinia Staphylococcus epidermidis Citrobacter, Serratia and UreaplasmaurealyciumNote: E.coli does not produce urease

Question 67

oxalato de calcio

Answer 67

Calcium oxalate dihydrate – enveloped shaped crystals Calcium oxalate monohydrate – dumbbell, spindle shaped crystals The mainstain of treatment of calcium oxalate stones is to increase fluid intake to reach the goal of at least 2 L of urine output per day. A reduction in calcium excretion by a low sodium diet (80-100 meg/day) may also be suggested. Low sodium excretion will enhance proximal sodium and calcium absorption, thereby decreasing urinary calcium excretion and stone formation. Drug therapy can also be added depending on the cause. If there is hypercalciuria, thiazide diuretics may be helpful. Potassium citrate can be used in cases of hypocitraturia and type 1 renal tubular acidosis. For hyperuricosuria, treatment of choice would be allopurinol or potassium citrate. oxalato de cálcio aumentar ingesta hidrica diminuir ingesta de sal tiazidico citrato se hipocitraturia

Question 68

Crystals

Answer 68

Presence & type of crystals is determined by urine concentration, constituents, inhibitors, pH etc4 Important drugs/toxins: – **Acyclovir (needle shaped)** **– Sulphonamide antibiotics (variable, needle shaped/rosettes) –** **Ethylene glycol (calcium phosphate)** **– High dose Vit C (calcium oxalate)** **– Methotrexate (rarely seen, variable) –** **Indinavir (needle shaped)** **– Phosphate laxatives (calcium phosphate**

Question 69

estruvita

Answer 69

Triple phosphate crystals- estruvita • Magnesium ammonium phosphate • Struvite • ‘Coffinlid’shapedcrystals • Seeninalkalineurine • Associatedwithinfectionwithurea-splitting organisms

Question 70

types of Stones and Cause

Answer 70

Calcium oxalate—low urine volume, low citrate, high calcium, high oxalate * Calcium phosphate—alkaline urine, high calcium, low urine volume, low citrate * Uric acid—persistently acid urine * Cystine—autosomal recessive disorder * Struvite—urease producing bacteria

Question 71

exames

Answer 71

* Hypercalciuria – Males: \> 300 mg/d – Females: \> 250 mg/d – Either: \> 4 mg/kg/d * Hyperoxaluria: \> 45 mg/d • Hyperuricosuria – Males: \> 800 mg/d – Females: \> 750 mg/d

Question 72

epidemiologia nefrolitiase

Answer 72

Kidney stones (nephrolithiasis) will occur in 12% of men and 5% of women during their lifetime. 1 em cada dez pessoas apresentara The major types of stones are calcium (70%-80%, oxalate more common thanphosphate), uric acid (5%-10%), cystine (\<1%), and magnesium ammonium phosphate (struvite) (10%).Combined calcium/urate stones are also often seen A patient with a calcium-containing stone episode has anoverall 50% chance of recurrence within 10 years, which is higher for men than for women. Some patients willhave many recurrent stones, leading to substantial morbidity, medical costs, and even mortality

Question 73

avaliacao do pac

Answer 73

historia clinica, ocupacao, antecedentes de litiase, antecedente familiar, sindrome metabolica, uso de medicacoes, dieta, ingesta de sal e agua infeccoes urinarias

Question 74

exames na uergencia

Answer 74

hemograma= ureia, creat, na , k, calcio, acido urico, pcr, urina rotina , urocultura

Question 75

analgesia

Answer 75

dipirona paracetamol , aines opioides

Question 76

abordagem urologica

Answer 76

urgencia= sepse, crise algica, recorrencia da dor, questoes de trabalho, dificuldade de manejo pos alta,solicitacao do pac, calculos ureterai \>1cm eletiva= calculos ureterais\>15mm

Question 77

baixa evidencia clinica

Answer 77

escopolamina para manejo da dor bloqueador alfa para calculo renal ou calculo menor 1 5 mm bcc corticoide

Question 78

dent disease

Answer 78

is a rare, X-linked inherited disease of pediatric nephrology. It can be filed under the category of “renal tubular dysfunction” and is also known as “X-linked Recessive Nephrolithiasis.” Clinical characteristics of Dent Disease include recurrent renal stones, nephrocalcinosis, hypercalciuria, low molecular weight proteinuria, and a gradual progression to ESRD, usually within childhood. The disease is caused by mutations in the gene CLCN5, a chloride channel present in proximal tubular cells which appears to be necessary for the processing of endosomes. It is not clear why these individuals are so predisposed to nephrolithiasis and hypercalciuria. There is no good therapy for Dent Disease, though a logical case could be made for treating with oral citrate supplementation and thiazide diuretics in an attempt to mitigate hypercalciuria and delay the nephrocalcinosis-induced renal failure as much as possible.

Question 79

dent disease

Answer 79

is a rare, X-linked inherited disease of pediatric nephrology. It can be filed under the category of “renal tubular dysfunction” and is also known as “X-linked Recessive Nephrolithiasis.” Clinical characteristics of Dent Disease include recurrent renal stones, nephrocalcinosis, hypercalciuria, low molecular weight proteinuria, and a gradual progression to ESRD, usually within childhood. The disease is caused by mutations in the gene CLCN5, a chloride channel present in proximal tubular cells which appears to be necessary for the processing of endosomes. It is not clear why these individuals are so predisposed to nephrolithiasis and hypercalciuria. There is no good therapy for Dent Disease, though a logical case could be made for treating with oral citrate supplementation and thiazide diuretics in an attempt to mitigate hypercalciuria and delay the nephrocalcinosis-induced renal failure as much as possible.

Question 80

drpad

Answer 80

Patients with ADPKD are predisposed to nephrolithiasis. The postulated reasons for this increased prevalence of stone disease have ranged from metabolic abnormalities (previous reports have shown an increased tendency for hyperoxaluria, hypercalciuria, hypocitraturia, hyperuricosuria, and distal RTA) to anatomic abnormalities (large, obstructing cysts and dilated tubular lumens may create a situation of tubular stasis, which predisposes to stone formation).

Question 81

rim em ferradura= fusao dos polos inferiores

Answer 81

litiase em 60% risco de neop renal e infeccao has renovascular

Question 82

avaliacao ambulatorial por exames

Answer 82

solicitacao de exames : hemograma, gaso, funcao renal, sodio, k, calcio, fosforo, PTH, glicemia, acido urico, lipidograma e magnesio. solicitacao de exames de urina= urina pH , densidade / urocultura com TSA ca u \> 250-300 oxalato\>40 citrato\<340 acido urico \>750-800 pesquisa qualitativa de cistina na urina positiva