Nephro/Uro/GI Flashcards

Question

What are the categories of renal stones?

Answer 1

Calcium oxalate/phosphate Struvite (infectious stones) Uric acid Most treatment: high fluid intake, pain control

Answer 2

- Biopsy diagnosis - Primary in children - Infection, autoimmune

Answer 3

Barter Syndrome Like giving someone a loop diuretic Can be associated with growth and MR

Answer 4

Gitelman syndrome Like giving too much thiazide

Answer 5

1. Advanced maternal age 2. Maternal Obesity 3. Low parity 4. Preterm birth 5. Low birth weight

Answer 6

Reduced risk of malignancy

Answer 7

1. Urinalysis & Micropscopy 2. Lytes, BUN, Creatinine, Ca, PO,MG C3/C4 ASOT antiDNA B throat swab CBC, smear, coags, coombs HBV/HCV/HIV IgA

Answer 8

1. Fluid overload 2. Hyperkalemia 3. HyperPO with hypoCa 4. ACidosis 5. Uremia

Answer 9

\>40 mg/m2/hr

Answer 10

24 hour urine collection Second best: protein to creatinine ratio

Answer 11

Glomerular: large losses of large proteins (albumin, IGG) TUbular: small losses of small proteins (alpha 1, amino acids)

Answer 12

- Thin, tall, adolescents - Less than 1 gram/day - "Postural test": protein free urine during the night, first am increase protein - Benign, disppears with age

Answer 13

TRANSIENT under certain conditions: exercise, stress, fever, acute illness, pressors

Answer 14

24 urine collection Postural protein test if \>10 years Serum RFTs Serum albumin and cholesterol C3/C4 ASOT Anti DNAse HCV.HBV.HIV

Answer 15

- Edema - Hypoalbuminemia - Albuminuria - Hypercholesterolemia

Answer 16

Minimal change nephrotic syndrome

Answer 17

Males, 2-6, caucasian or asian

Answer 18

1. Age \<1 year 2. Age\>13years 3. Black race 4. Renal failure 5. Macrohematuria 6. No response within 4 weeks of steriods

Answer 19

- Proteinuria - Decrease in small protein (albumin, OgG, protein C/s) - Increase in serum cholesterol Increase in large proteins (fibrinogen, iGM)

Answer 20

FENA: \<1%: underfilled

Answer 21

Infection (SBP) Thrombosis Hypercholesterolemia

Answer 22

Focal segmental glomerulosclerosis -More serious, steriod resistant ESRD if untreated Steriods

Answer 23

Na-CL-HCO3

Answer 24

Acidosis, hypoK, HypoNa, hypoP, gluosuria

Answer 25

6 weeks GA

Answer 26

8 weeks in response to testosterone

Answer 27

5 alpha reductase Critical for normal male : DHT

Answer 28

1. 46 XX CAH (masculinized female) 2. 45 X/46 XY Mixed gonadal dysgenesis

Answer 29

When pregnancy confirmed, \<9 weeks

Answer 30

-Assess for VUR and PUV

Answer 31

functional information Differential scans always =100%

Answer 32

1% of pregnancies Transient in 44-88% Not all HN represents significant obstruction

Answer 33

RBUS Urgent: bilateral severe CH, solitary kidney Elective: within 30 days

Answer 34

1. UPJ obstruction 2. high grade VUR UVJ ostruction PUV

Answer 35

SFU III and IV Will need VCUG and diuretic renal scan (4-6 weeks)

Answer 36

Suspect when: male, hydronephrosis, distended thick walled bladder, keyhole sign, oligohydramnios Urgent postnatal RBUS Confirmatory VCUG Surgery

Answer 37

1. Febrile UTI work up: if AbN RBUS, \>1 febrile UTI 2. Work up of SFU III or IV CH 3. Work up of late presentation HUN 4. Follow of VUR if will alter management 5. Management of neurogenic baldder 6. Suspicion of PUV 7. Recurrent cysitis in a prepubertal male (r/o PUV)

Answer 38

1. Neuroblastoma 2. Wilms tumor

Answer 39

Increased fluid intake Avoidance of excess salt intake Normal calcium intake

Answer 40

2 weeks to 2 months Male x 4 female Risk factors: FHx+, first born, maternal feeding

Answer 41

Metabolic alkalosis due to loss of electrolytes in vomit (H, CL) Kidney will try to correct by excreting Na and HCO When volume contraction occurs, kidney reabsorbs NA and dumps H and K (aldosterone), paradoxical aciuria

Answer 42

IV bolus of NS until u/o Maintane fluids with D50.45NS or NS with 20-40 KCL Aim for Cl\>95, HCO\<28, K\>3.5

Answer 43

Ileo-colic

Answer 44

1-4/1000 Age 3M-3Y M 3x F

Answer 45

1. Peritonitis 2. Persistent hypotension 3. Free air/pneumoperitoneum

Answer 46

Angiocath Abdominal compartment syndrome

Answer 47

2% of pop 2 xM to F 2-6% symptomatic 2 years 2 feet from the ileocecal valve 2 inches long 2 types: gastric or pancreatic

Answer 48

Painless, episodic LGI with drop in HGB

Answer 49

Intubate on first breath NG to decompress MV\< HFO, NO, ECMO Stable over 24-48 hr then repair

Answer 50

Failure to pass meconium within 24 hours VOmiting, abdo distension

Answer 51

Suction rectal biopsy

Answer 52

PREMATURITY

Answer 53

Risk of infertility

Answer 54

Down syndrome Turner syndrome WIlliam syndrome Type 1 DM IgA deficiency Other autoimmune FIrst degree relative (Not russell silver)

Answer 55

Autommune enteropathy with antibodies against gluten?

Answer 56

Villious atrophy on intenstinal biopsy

Answer 57

WHEAT RYE BARLEY

Answer 58

Dermatitis herpetiformis Dental enamel hypoplasia Osteopenia Short stature Delayed puberty Iron deficiency anemia Hepatitis

Answer 59

Crohns Lymphoma Yersinia TB CGD Severe eosinophilic gastroenteropathy

Answer 60

Hypercalcemia HypoK Hypothyroidism Celiac disease Lead and mercury poisoning UC Medications CP NTD Lactose intolerance

Answer 61

IgE: earlier onset (\<30 days), FTT, immediate symptoms, FH of atropy No IgE: later onset (1-4 months), less FTT, delay in symptoms

Answer 62

C diff Parasite Consider campylobacter Support: cholera, yersinia Don't: ecoli, shigella, salmonella

Answer 63

1. Gilberts

Answer 64

7% of the population AD Bili \>40, \<100 50% of infants with persistant unconjugated bili

Answer 65

Biliary atresia

Answer 66

1. EtOH 2. Myopathies 3. Renal syndromes 4. Hemolysis 5. Intestinal inflammation

Answer 67

2, 7, 9, 10

Answer 68

Factor VIII

Answer 69

Hep B vaccine within 12 hours Hep B Ig at the same time, max 7 days given in the first 24 hours

Answer 70

T- toxic, tropical, metabolic I- idiopathic G- genetic A- autoimmune R- recurrent acute O- obstructive

Answer 71

1. Tubular proteinuria 2. Lytes wasting 3. Glycosuria

Answer 72

AR lysosomal storage d/o Accumulation of cysteine dimers in lysosomes Loss of HCO3, PO4, glucose, AA, Na, K Normal AG metabolic acidosis

Answer 73

Post infectious GN MPGN Lupus nephritis

Answer 74

1. Abdo muscle deficienct 2. Severe urinary tract abN 3. B/L cryptorchidism

Answer 75

1: Acidosis 2. Electrolyte AbN: HyperK, HypoNa, HyperPO4 3. Ingestions (methanol, ethylene glycol, ASA, Li) 4. Fluid overload 5. Uremia

Answer 76

AR, sBDS mutation 1. Bone marrow failure 2. Panreatic dysfunction (second most common to CF) 3. Skeletal AbN (short stature, osteopenia, dystrophies) 4. INfections 5. Myelodysplasia/AML

Answer 77

Biopsy Supported by CBC, IGE, albumin

Answer 78

Acid suppression Steriod, PO pulicort for strutures Dietary elimination (targeted: milk, wheat, soy, eggs, nuts, peanuts, fish, seafood)

Answer 79

Bile salts and Vitamin B12

Answer 80

LIVER DISEASE

Answer 81

Serumimmunoassay for alpha 1 antitrypsin Serum electrophoresis Genotype by PCR

Answer 82

Systemic vasculitis HLA B51 Aphthous stomatitis, erthma nodosum and arthritis

Answer 83

IgA TTG Gliadin Endomysial Ab Albumin, CBC, ESR\< CRP

Answer 84

IBD CMPA NOT CELIAC

Answer 85

Direct visualization of the worms in the perianal regoin 2-3 hours after the child goes to sleep Transparent tape to see eggs in the morning

Answer 86

Intractable singultus

Answer 87

AP: esophagus en face Lat: trachea en face

Answer 88

Worried abut pancreatic insufficiency

Answer 89

2% glucose, 20 mEqK/l, 90 mEq Na/L, 80 mEq CL/l, 30 mEq HCO3/l Add 3/4 tsp salt, 1 tsp bakingsoda, 1 cup orange jioce and 8 tsp sugar to 1 L water

Answer 90

Paradoxical bradycardia with fever in Salmonella (typhoid fever)

Answer 91

Cow milk Eggs Peanuts

Answer 92

Paroxysmal dystonic posturing with opisthotonus and unusual twisting of the head and neck in association with GERD

Answer 93

kwashiorkor: edematous malnutrition lowserum oncotic pressure Marasmus: severe nonedematous malnutrition mixed deficiency of protein and calories.

Answer 94

most spontaneously close before 2 y \>1.5 cm at 2Y, ?closure

Answer 95

As soon as possible : incarceration

Answer 96

1. Colicky pain 2. Vomiting 3. Passage of bloody stool

Answer 97

Ileocolic intussusception

Answer 98

Recognizable jaundice in the third week of life Inreasingly dark urine and acholic stools Spleen enlargement

Answer 99

3-6 weeks Progressive non-bilious projectile vomiting Dehydration with hypoCL, hypoK metabolic alkalosis

Answer 100

UNa x Pcreat/ PNa x Ucreat \<1%- Prerenal \>1% Renal

Answer 101

EPO= eliminated blood transfusions Vitamin D= renal osteodystrophy GH= growth acceleration ACE/ARB= HTN rx

Answer 102

POST infectious Lupus Membranoprolierative C3 glomerulopathy

Answer 103

Chorinc hyperclroemic metabolic acidosis INability to acidify the ruine Normal AG Growth failure, polyuria, polydipsia

Answer 104

Cystinosis

Answer 105

Cystinosis: photophobia, Fanconi syndrome

Answer 106

Useful to rule out mechanical obstruction: malrotation, achalasia

Answer 107

4-6 weeks of same dose

Answer 108

1. EBV 2. CMV 3. Hepatitis A & B 4. Mycoplasma 5. Mumps

Answer 109

Valproic acid L-aspariginase Azathioprine Mercaptopurine Think chemotherapy and anti-epileptics

Answer 110

1. Hypercalcemia 2. Hypertriglyceridemia

Answer 111

Need 2/3 1. Acute RUQ abdominal pain 2. Elevated amylase or lipase at least 3x normal 3. Imaging findings consistent with pancreatitis

Answer 112

Ranitidine or prevacid

Answer 113

1. NPO --\> can start feeds after 24-48 hrs if improved - enteral feeds are preferred to TPN if child is clinically tolerating and not septic 2. Fluids fluids fluids --\> give bolus and 1.5 maintenance 3. Analgesia --\> morphine 4. Stop any possible aggravating factors 5. Anti-emetics

Answer 114

1. Pseudocyst 2. Multi-organ system failure

Answer 115

1. Cholesterol stones: increased secretion of cholesterol into bile (see in hyperlipidemia, obesity, pregnancy, females) 2. Black pigment stones: increased conjugated bilirubin into bile (hemolytic disease, pancreatic insuffiency, TPN) 3. Brown pigment stones: from bacterial and parasitic infections

Answer 116

1. Infectious: - viral: Hep A/B/C/D/E, adenovirus, coxsackie, enterovirus, EBV, HSV, HIV, VZV, paramyxovirus (RSV, mumps, measles) - non-viral: abscess, amebiasis, bacterial sepsis, histoplasmosis, leptospirosis, TB 2. Autoimmune: autoimmune hepatitis, sclerosing cholangitis, SLE, JIA 3. Metabolic: alpha-1 antitrypsin deficiency, tyrosinemia, Wilson's 4. Toxic: drug-induced (anti-seizure, chemo, acetaminophen) 5. Anatomic: choledochal cyst, biliary atresia 6. Hemodynamic: shock, congestive heart failure 7. Non-alcoholic fatty liver disease: idiopathic, Reye syndrome

Answer 117

1. Cytopathic injury - rise in serum ALT and AST: magnitude of rise does NOT correlate with extent of hepatocellular necrosis and has little prognostic value - slowly improve over several weeks but lag behind serum bilirubin level (normalizes first) - rapidly falling ALT and AST in conjunction with increased bilirubin and INR can mean massive hepatic injury (injured or dead cells don't produce enzymes) 2. Cholestasis - elevated serum conjugated bilirubin from abnormal bile flow at the canalicular and cellular level due to hepatocyte damage and inflammation - can also have increased ALP and GGT 3. Altered synthetic function - this should be MAIN FOCUS of monitoring - indication for prompt referral to transplant center if abnormal - abnormal protein synthesis: decreased coags, decreased albumin - metabolic disturbances: hypoglycemia, hyperammonemia, lactic acidosis - hepatic encephalopathy: altered LOC with hyperreflexia

Answer 118

Neck contortions (arching, turning of head) secondary to GERD

Answer 119

Good for studying the anatomy of the upper GI tract (esophageal strictures, hiatal hernia, gastric outlet or intestinal obstruction) but has poor sensitivity and specificity in the diagnosis of GERD since it has limited duration and the inability to differentiate between physiologic GER from GERD

Answer 120

1. Assessing efficacy of acid suppression during treatment 2. Evaluating apneic episodes in conjunction with a CXR 3. Evaluating atypical GERD presentations such as chronic cough, stridor and asthma

Answer 121

1. Conservative therapy and lifestyle modification -infants: thickening of feeds or use of commercially prethickened formulas (ie. add 1 tbsp of rice cereal per oz of formula), short trial of hypoallergenic diet can be used to exclude milk allergy, positioning measures -older children: avoid reflux inducing foods such as tomatoes, chocolate, mint, juice, carbonated and caffeinated drinks, alcohol; weight reduction, stop smoke exposure 2. Pharmacotherapy -mild-moderate reflux esophagitis: histamine-2 receptor antagonists -severe reflux esophagitis: PPIs (more potent than H2 blockers but more expensive) -2nd line: prokinetic agents (metoclopramide, erythromycin) = increase LES pressure or improve gastric emptying

Answer 122

Chronic use (\>3 mo) linked with tardive dyskinesia

Answer 123

Malrotation with volvulus - UGI series with small bowel follow through and STAT gen surg consult - obstruction: distal to the ampulla of vater (halfway along the duodenum, formed by the pancreatic duct and common bile duct)

Answer 124

Stool fecal leukocytes

Answer 125

Inflammatory diarrhea: organisms and cytotoxins invade mucosa, killing mucosal cells = bleeding - think EECYSTS 1. E coli (0157:H7) 2. E. histolytica 3. Campylobacter/C. diff 4. Yersinia 5. Shigella 6. Salmonella Typhi 7. Strongyloides

Answer 126

Salmonella bacteremia can quickly become invasive and cause sepsis, meningitis, especially in infants

Answer 127

Azithromycin -if it is NOT severe, do NOT treat as this may worsen chance of HUS (endotoxin release)

Answer 128

Ceftriaxone

Answer 129

Bacillus cereus -management: supportive care

Answer 130

Vibrio cholerae -supportive care OR doxycycline for severe illness

Answer 131

Entamoeba histolytica -mebendazole

Answer 132

1. Narrow diameter stools (stools squeezing past an obstruction or Hirschsprung's where not enough strength is generated to push the entire stool mass through) 2. Abdominal distention 3. Lack of encopresis (almost never see encopresis in Hirschsprung's)

Answer 133

In order of prevalence: 1. Functional abdominal pain 2. Lactose intolerance 3. Constipation

Answer 134

Upper = above ligament of Treitz Lower = below ligament of Treitz (suspensory ligament attaching duodenum to connective tissue)

Answer 135

1. Swallowed blood (epistaxis, dental work, etc.) 2. esophagitis 3. gastritis 4. peptic ulcer disease 5. mallory-weiss tear 6. esophageal varices 7. vascular malformations

Answer 136

1. CMPA 2. NEC 3. Meckel diverticulum 4. Vasculitis (HSP) 5. Polyp 6. Intestinal or colonic ulcer 7. Colitis (infectious vs. IBD) 8. Vascular malformation 9. Anal fissure

Answer 137

1. CMPA 2. Anal/rectal fissure

Answer 138

GAS! - clinical features: perianal erythema, pain, pruritis - diagnosis: rapid strep test or culture - workup: CBC, CRP, ESR, gonorrhea/chlamydia swabs, tape test for pinworms - treatment: 10 d course penicillin

Answer 139

Abdominal U/S

Answer 140

HIDA scan (radionuclide scan) - in biliary atresia: hepatic uptake of radionuclide is normal but there is no excretion into the intestines - in neonatal hepatitis: hepatic uptake of radionuclide is normal but excretion into the intestines is normal

Answer 141

Gilbert disease = benign disorder of mildly impaired bilirubin conjugation found in 5-10% of population

Answer 142

Results from direct obstruction of bile flow from the liver into the biliary system, then into the intestine (can be from obstruction inside the liver or outside the liver) = aka cholestasis Intrahepatic: 1. Alagille syndrome (congenital abnormalities of the intrahepatic biliary tract) 2. TPN 3. Wilson disease 4. Viral hepatitis 5. Autoimmune hepatitis 6. Metabolic disorders (ie. Dubin-Johnson syndrome) 7. Drug-induced cholestasis 8. Alpha-1 antitrypsin deficiency 9. Neonatal hepatitis Extrahepatic: 1. Biliary atresia 2. Choledochal cyst 3. Abdominal mass 4. Obstructive gallstone

Answer 143

Results from increased RBC turnover or impaired conjugation 1. Breastmilk jaundice 2. Breastfeeding jaundice (neonatal physiologic jaundice) 3. Gilbert disease 4. Hemolytic anemia 5. Crigler-Najjar syndrome 6. Hypothyroidism 7. Cystic fibrosis 8. Viral hepatitis

Answer 144

Abdominal U/S

Answer 145

Loss of LES relaxation due to high resting LES pressure and absent or non-peristaltic esophageal contractions - gold standard for diagnosis: esophageal manometry) - UGI = "bird's beak" = tapering of esophagus

Answer 146

1. Esophageal strictures 2. Barrett's esophagus 3. Adenocarcinoma

Answer 147

1. Bloodwork: peripheral eosinophilia, elevated IgE 2. Endoscopy = gold standard = biopsy showing \>15-20 eosinophils/hpf

Answer 148

Infectious esophagitis -common etiologies: candida, HSV, CMV

Answer 149

Pill esophagitis: irritation/erosion of the esophageal wall due to a pill temporarily lodging in the esophagus 1. Antibiotics - specifically tetracycline, doxycycline, cilndamycin 2. Anti-inflammatories - aspirin 3. Bisphosphanates - treatment: stop the offending drug if possible; if not, switch to liquid formulation - prevention: always take pills standing upright, with at least 8 oz of water

Answer 150

1. Acute onset of severe epigastric/retrosternal pain and dysphagia 2. Looks unwell with fever, tachycardia, abdominal pain - diagnose by obtaining C spine and CXR = will see free retrosternal air and pneumomediatstinum - if xrays are normal but you have a high suspicion, get an esophagram with water-soluble contrast - treatment: NPO, gastric drainage, broad spectrum IV abx for small esophageal tears in stable patients. If unstable or large esophageal teams, need emergent surgical repair

Answer 151

Pharmacological: IV octreotide surgical: endoscopic sclerotherapy, band ligation or thrombin injection

Answer 152

Primary PUD: usually caused by H. pylori, duodenal location Secondary PUD: gastric in location, usually caused by: 1. hypergastrinemia 2. Zolllinger-Ellison syndrome: autonomous gastrin secretion by gastrinoma, associated with MEN-I (recurrent, multiple, atypically located ulcers and treated with PPI and octreotide to inhibit tumor growth) \*\*\*gastric ulcers = pain worsens with meals, especially with spicy foods \*\*\*duodenal ulcers = pain improves with meals

Answer 153

1. Caffeine/alcohol/tobacco use 2. G tubes 3. NSAIDs 4. Burn injuries 5. Systemic illnesses: sepsis 6. Steroids -gold standard for diagnosis: endoscopy with biopsy -treatment: 1. Avoid triggering substances 2. PPIs 3. Treatment of H pylori with clarithromycin +amoxil (abx x 2 wks) + PPI (x 1 mo) (OR amox + flagyl + PPI OR clarithro + flagyl + PPI) 4. Surgery for severe and/or refractory cases

Answer 154

Developmental abnormality in which there is incomplete rotation of the midgut and thus, the cecum is in the RUQ instead of the RLQ and the mesentary is fixed on a narrow base -this places the small bowel at increased risk for twisting on the pedicle of mesentary

Answer 155

Early infancy = more than 90% of cases present in first year of life BUT can present at any age -definitive treatment: surgery (Ladd procedure)

Answer 156

1. 2:1 M:F ratio 2. 2% of population 3. 2 feet above ileocecal valve 4. 2 year of age most common presentation 5. 2 inches in size 6. 2 types of tissue: gastric and pancreatic

Answer 157

Meckel diverticulum: remnant of embryonic yolk sac that results from failure of the omphalomesenteric/vitteline duct to involute. - clinical features: 1. PAINLESS rectal bleeding (gastric mucosa secrets acid leading to ulceration and bleeding of intestinal mucosa) 2. Can also present with intussusception or volvulus (acts as lead point) - diagnostic test: Meckel scan (technietium-99 radionuclide scan) - treatment: surgical excision

Answer 158

Meckel diverticulum

Answer 159

Admit for observation x 24-48 hrs = recurrence can occur - reduction rate with air enema: 80-95% - recurrence rate after reduction: 10%

Answer 160

Duodenal hematoma = swelling causes intestinal obstruction - diagnosis: UGI series ("coiled spring appearance") or CT abdo - treatment: NPO, NG decompression, TPN until obstructive symptoms resolve (usually 7-10 d)

Answer 161

SMA syndrome = compression of the duodenum by the SMA against the aorta - 2 main risk factors: 1. rapid weight loss from malnutrition or catabolic states = loss of mesenteric fat pad which collapses the duodenum in between the SMA and the aorta 2. full body cast (extra-abdominal compression) - clinical features: 1. Severe epigastric pain 2. Vomiting

Answer 162

SMA syndrome - treatment: 1. Lateral or prone positioning to shift duodenum away from obstructing structures and resme oral intake 2. If that doesn't work, may need NJ tube or TPN

Answer 163

Gross pathology: - CD: cobble stoning, strictures, fistulas, fissures - UC: diffuse continuous inflammation Histology: - CD: transmural involvement - UC: mucosal and submucosal inflammation, crypt abscesses

Answer 164

1. Spondyloarthropathy 2. Pyoderma gangrenosum (painless, necrotic tissue) 3. Erythema nodosum (PAINFUL) 4. Aphthous ulcers 5. Uveitis 6. Primary sclerosing cholangitis

Answer 165

1. Pyoderma gangrenosum 2. Primary sclerosing cholangitis 3. Thrombosis

Answer 166

All 3 are more common in UC

Answer 167

1. Steroids 2. Mesalamine 3. Sulfasalazine 4. azathioprine 5. Methotrexate 6. Infliximab 7. Surgical resection

Answer 168

1. Autoimmune thyroiditis 2. Type 1 DM 3. Down syndrome 4. Turner syndrome 5. IgA deficiency 6. William's syndrome

Answer 169

Intestinal manifestations: 1. FTT/wt loss 2. Diarrhea (with occult blood loss) 3. Irritability 4. Vomiting 5. Anorexia or excessive appetite 6. Foul-smelling, bulky stools 7. Abdominal pain 8. Rectal prolapse Signs: 1. Poor growth for both height and weight 2. Wasted muscles 3. Abdominal distention 4. Edema 5. Digital clubbing Biopsy findings: 1. Villous atrophy 2. Crypt hyperplasia 3. Intraepithelial lymphocytosis Non GI manifestations: 1. Dermatitis herptiformis 2. Dental enamel hypoplasia of permanent teeth 3. Iron deficiency anemia resistant to oral Fe

Answer 170

Initial testing: elevated tissue transglutaminase (can be falsely negative in IgA deficiency so ALWAYS order an IgA with tissue transglutaminase) -definitive diagnosis: small intestinal biopsy showing villous atrophy

Answer 171

IDIOT. Acquired lactase deficiency is VERY rare in children

Answer 172

VACTERL - Vertebral anomalies - Anal atresia - Cardiac defects - TracheoEsophageal fistula - Renal abnormalities - Limb abnormaliies

Answer 173

Perf rate in infants: 50% Perf rate in general population: 10% -due to delayed diagnosis/atypical presentation

Answer 174

Refers to the fact that patients with appendicitis will usually not even want to eat their favourite food due to nausea/anorexia. Thus, if they're hungry, it's probably NOT appendicitis

Answer 175

Rovsing's sign: press in the LLQ, if the patient feels pain in RRQ, this is positive Psoas sign = seen with retrocecal appendicitis -so remember that the psoas muscle is posterior SO, get the patient to roll onto their left side and EXTEND the right hip. If this causes the patient pain, this is a positive psoas sign Obturator sign: obturator muscle causes internal rotation of the hip SO flex the patient's hip and knee and internally rotate. If painful, this is a positive obturator sign \*\*\*\*These tests have high specificity but LOW sensitivity

Answer 176

IV antibiotics = amp/gent/flagyl until symptomatically improved +/- percutaneous drainage of abscess if present

Answer 177

Viral hepatitis

Answer 178

Type I autoimmune hepatitis: usually in young women aged 15-25 yrs, associated with other autoimmune conditions -type II autoimmune hepatitis: occurs in young children

Answer 179

1. Hypergammaglobulinemia 2. Positive ANA and anti-smooth-muscle antibodies (70% positive on presentation); some patients have anti-liver-kidney antibodies 3. Elevated transaminases - definitive diagnosis: liver biopsy - treatment: steroids +/- azathioprine, may need liver transplant if ESLD

Answer 180

Reye syndrome! Acute encephalopathy and fatty degeneration of the liver - associated with aspirin use in children with viral infections including influenza and varicella - think encephalopathy, liver failure, and FATTY LIVER on biopsy

Answer 181

Non-alcoholic steatohepatitis - fatty infiltration of the liver - 2 risk factors: 1. obesity 2. type 2 DM - treatment: weight loss through diet and exercise

Answer 182

AR condition = deficient fumarylacetoacetate hydrolase = affects liver, kidney, peripheral nervous system -clinical features (begin in infancy): 1. FTT 2. Jaundice 3. HSM with increased risk of hepatocellular carcinoma -labwork: 1. Elevated tyrosine 2. Elevated AFP 3. Elevated urine succinylacetone -treatment: dietary restriction of tyrosine, phenylalanine, methionine, medical management with tyrosine degradation inhibitor, supportive care for liver failure

Answer 183

1. Treament of underlying disease 2. Medications to decrease portal pressure (beta blockers, vasopressin) 3. Surgical portosystemic shunt +/- liver transplant 4. Treat complications (ie. esophageal varices)

Answer 184

1. E. coli 2. Klebsiella - empiric treatment: IV ceftriaxone

Answer 185

Need 3/3: 1. Biochemical evidence of acute liver injury 2. No evidence of chronic liver disease 3. Hepatic-based coagulopathy defined as: a. In presence of hepatic encephalopathy: PT \> 15 sec or INR \> 1.5 not corrected by vitamin K b. In absence of hepatic encephalopathy: PT \> 20 sec or INR \> 2 -50% of cases are idiopathic

Answer 186

1. Neurologic: avoid sedatives, limit protein intake (to decrease ammonia), neurovitals and monitor for increased ICP due to cerebral edema 2. Respiratory: intubation to prevent aspiration due to impaired LOC 3. GI: antacids, glucose control 4. Renal: avoid hypovolemia (risks hepatorenal syndrome) 5. Hematology: give vitamin K, FFP, platelets, etc 6. Liver transplant (mortality is 70% without transplant)

Answer 187

Biliary atresia: atresia of any portion of the extrahepatic biliary system = 50% of liver transplants are because of biliary atresia! - clinical presentation: conjugated hyperbilirubinemia in an otherwise healthy, growing infant in the first few months of life - diagnosis: elevated liver enzymes, conjugated hyperbilirubinemia, HIDA scan showing lack of excretion of tracer from liver into the intestinal tract - definitive diagnosis: liver biopsy showing bile duct proliferation and widened portal area - treatment: Kasai procedure, eventual liver transplant

Answer 188

Charcot's triad: 1. Fever 2. RUQ pain 3. Jaundice Reynold's pentad: 1. Fever 2. RUQ pain 3. Jaundice 4. Hypotension 5. Altered mental status \*\*\*\*See both in ascending cholangitis

Answer 189

1. Ocular abnormalities: posterior embryotoxin 2. Vertebral anomalies: butterfly vertebrae 3. Intrahepatic bile duct paucity 4. Triangular face 5. Cardiac defects: peripheral pulmonic stenosis, pulmonary valve stenosis, etc. 6. Renal abnormalities

Answer 190

5 types overall - Type I = most common = diltation of the common bile duct - affects females 4x more than males - type 5 = Caroli disease = intrahepatic bile duct cysts

Answer 191

Pathognomonic for CF! Need to do sweat chloride

Answer 192

Bacterial infection of obstructed common bile duct usually due to stricture of stones - see Charcot's triad or Reynold's pentad - treatment: after diagnosis via ultrasound/ERCP/percutaneous transhepatic cholangiography, do NPO, antibiotics, stone removal or dilation via ERCP or PTC

Answer 193

Inflammation and fibrosis of hepatobiliary system (occurs in

Answer 194

Similarities: 1. Both are AD 2. Both have average onset in 2nd decade 3. Both present with rectal bleeding, abdo pain, bowel obstruction 4. Both have 100% risk of colon cancer Difference: 1. FAP has adenomas in large intestine only while Gardner syndrome has adenomas in both small and large intestine

Answer 195

Protein-hydrolyzed formula with MCT in order to ensure absorption (ie. alimentum with medium chain triglycerides) -start with TPN, then small enteral feeds, then slowly increase volume in order to increase pancreatic-biliary flow

Answer 196

1. Increased IgE 2. Increased eosinophils 3. Increased platelets 4. Decreased albumin

Answer 197

CMPA - clinical features: edema, hypoalbuminemia, iron deficiency anemia, vomiting, bloody stools, irritability - Treatment: quick response to removal of cow's milk protein (remember that 50% react also to soy protein) - need hydrolyzed protein formula (nutramigen, progestamil, alimentum) OR if this fails, then use amino-acid based formula (neocate) - most grow out of CMPA and tolerate cow's milk by 2 yr

Answer 198

Stool reducing substances

Answer 199

Can last up to 10 weeks - peaks at 5-15 days - confirm with trial of pumping

Answer 200

Asians: 40% Blacks: 85% -testing is not required if symptoms improve with removal of dairy -hydrogen breath test if you need to confirm

Answer 201

1. Portal vein thrombosis (most common) 2. Portal vein agenesis/stenosis 3. Splenic vein thrombosis

Answer 202

Autsomal dominant disease causing hamartomas 1. Hyperpigmented lesions on lips and oral mucosa (other mucosal areas can be involved too) 2. Hamartomas in small and large bowel (also elsewhere in GI) Complications: 1. hamartomas can serve as lead point for intussusception 2. GI malignancy: yearly colonoscopy needed 3. Breast/testicular malignancy

Answer 203

1. Hydrogen breath test 2. Biopsy

Answer 204

1. Abdominal compartment syndrome 2. NEC

Answer 205

Endoscopy for biopsies and H pylori cultures -supportive test: Hydrogen breath test

Answer 206

1. Peripheral eosinophilia 2. Elevated IgE - appearance on endoscopy: FURROWED esophagus

Answer 207

1. Dietary restriction of major food allergens (usually milk, soy, wheat, egg, peanuts and treenuts, seafood) 2. Topical and systemic corticosteroids

Answer 208

AXR: dilated bowel loops at level of obstructon (usually at terminal ileum) - see bubbly, granular pattern at the level of obstruction - Contrast enema: microcolon from disuse

Answer 209

EE usually does NOT respond to acid blockade therapy

Answer 210

Toxic megacolon

Answer 211

Absence of ganglion cells in the submucosal and myenteric plexus = inadequate relaxation of the bowel wall with bowel wall hypertonicity

Answer 212

Hirshsprung/s disease -M:F = 4:1 for short segment disease

Answer 213

Hirschsprung's: 1. Abdominal distension 2. Empty rectum on DRE with possible explosive passage of stool 3. Possible poor weight gain - on history: 1. Onset of constipation at birth (for functional constipation, usually after 2 yr of age) 2. Encopresis VERY rare

Answer 214

1. Anorectal manometry: initial diagnostic test = will see failure of internal anal sphincter relaxation in response to dilating a balloon in the rectum 2. Barium enema: delayed evacuation \> 24 hrs 3. Rectal biopsy = GOLD STANDARD = will see no ganglion cells, increased acetylcholinesterase staining

Answer 215

1. Recurrent abdmoinal pain 2. Cramping 3. Bloating 4. Weight loss 5. Intermittent diarrhea

Answer 216

1. Mandibular central incisors: 5-7 mo 2. Maxillary central incisors: 6-8 mo 3. Lateral incisors (mandibular first, then maxillary): 7-11 mo 4. First molars: 10-16 mo 5. Cuspids: 16-20 mo 6. 2nd molars: 20-30 mo

Answer 217

Extensive maxillary decay (especially frontal) and posterior maxillary and manibular delay but NORMAL mandibular frontal teeth -weaning from bottle: when they turn 1 yo

Answer 218

- @ 30 minutes: 90% chance - @ \> 2 hrs: t need to replace since it may damage the underlying permanent tooth if shoved back in

Answer 219

Stool culture on sorbitol-MacConkey medium (won't grow on regular stool cultures)

Answer 220

Vitamin C deficiency (ie. scurvy)

Answer 221

1. Abscesses. 2. Perforation 3. Toxic megacolon (less risk than UC) 4. GI bleed 5. Bowel obstruction 6. Infection

Answer 222

1. Perforation 2. Hemodynamically unstable 3. Signs of ischemia

Answer 223

1. Pathologic lead point 2. Ileo-ileo intussusception (usually doesn't respond to air enema, usually requires surgery)

Answer 224

1. Disease \> 10 years 2. Onset before age 15 yo 3. Pancolitis 4. PSC

Answer 225

1. Sepsis 2. Primary sclerosing cholangitis 3. Fulminant colitis 4. Toxic megacolon/perforation 5. Adenocarcinoma

Answer 226

1. Crohn's disease 2. Yersinia infection 3. TB 4. Lymphoma 5. CGD

Answer 227

1. Hypercalcemia 2. Hypokalemia 3. Hypothyroidism 4. Celiac disease 5. Hirschsprung's 6. Ulcerative colitis

Answer 228

1. More than 1 week of symptoms for 2 or more months 2. Abdominal discomfort or pain associated with at least 2 of the following: a. Improved with defecation b. onset associated with change in frequency of stool c. onset associated with change in form of stool 3. No organic cause

Answer 229

1. ETOH 2. Myopathy 3. Renal syndromes 4. Hemolyasis 5. Adenovirus infection \*\*\*\*Abnormally low ALP: think zinc deficiency

Answer 230

1. Giardia 2. HIV 3. Intestinal TB 4. Primary immunodeficiency 5. Crohn's disease 6. Celiac's 7. Malnutrition

Answer 231

1. Prematurity 2. Low birth weight 3. Sepsis 4. Prolonged duration of TPN 5. NEC

Answer 232

Problems with filtration: glomeruli = act as sieves to filter serum, creating ultrafiltrate Problems with secretion and reabsorption: tubules = reabsorb and secrete solutes, concentrate the urine and create and respond to endocrine signals -GFR = glomerular filtration rate = amount of ultrafiltrate produced by all of the glomeruli (ml/min). Varies with age and height -Creatinine clearance = estimate for GFR since creatinine is not significantly secreted or absorbed in the renal tubules

Answer 233

\> 2+ protein = need to do further testing -can have false positives Further testing: 1. Protein-Cr ratio: only requires one sample but not as accurate as 24-h protein collection 2. 24 hr urine for protein = GOLD STANDARD for severity of protein loss but difficult to obtain (Microalbumin/Cr = good for tracking progression of disease but is not used for initial diagnosis)

Answer 234

- Proteinuria = \> 0.2 urine spot protein/Cr ratio or \>4 mg/m2/h on 24 hr urine collection - nephrotic range proteinuria = \> 2.0 urine spot protein/Cr ratio or \> 40 mg/m2/h on 24 hour urine collection (10x more than proteinuria) = this is NEVER benign and always warrants further evaluation

Answer 235

Benign causes: 1. Acute illness 2. Fever 3. Pregnancy 4. Trauma 5. Exercise 6. Orthostatic proteinuria Pathologic causes: - Glomerular: 1. Nephrotic syndrome 2. Glomerulonephritis 3. Reflux nephropathy 4. Long-standing infections - Tubular: 5. Fanconi syndrome 6. Metal poisoning 7. Ischemic injury (ie. ATN) - Increased protein burden (overwhelms tubular reabsorption) 8. Rhabdomyolysis 9. Neoplasms

Answer 236

Orthostatic proteinuria -occurs in up to 60% of children with persistent proteinuria Clinical features: - usually asymptomatic and condition is discovered on routine urinalysis - patients excrete normal or minimally increased amounts of protein in the supine position; however, in the upright position, urinary protein excretion increases up to 10 fold (1 g/24 h) once patient is up and moving. - Hematuria, hypertension, hypoalbuminemia, edema and renal dysfunction MUST BE ABSENT to make this diagnosis - diagnosis: Collect first morning urine sample (must be first voided urine immediately upon rising in the morning) = absence of proteinuria (dipstick negative or trace for protein and urine Pr:Cr

Answer 237

Initial labs: UA with microscopy, lytes, BUN, Cr, albumin, serum protein, urine protein, urine Cr to calculate Ur Pr:Cr ratio. Consider CRP, ESR, complement - always order renal U/S to rule out structural abnormality that might lead to obstruction of renal vessels - renal biopsy may or may not be indicated depending on suspected diagnosis

Answer 238

\>5 RBCs/HPF on urine microscopy - gross hematuria = visible redness of urine with \> 5 RBCs/HPF on microscopy - microscopic hematuria = normally colored urine with \> 5 RBCs/HPF on microscopy x 2 occasions or more

Answer 239

Pre-renal: (not true hematuria) 1. Hemolysis = hemoglobinuria = positive heme in urine but NO RBCs 2. Rhabdomyolysis = myoglobinuria = positive myoglobin in urine but NO RBCs \*\*\*other causes of red urine but not hematuria: rifampin, chloroquine, beets, food coloring, etc. 3. Coagulopathy Renal: 1. IgA nephropathy 2. Postinfectious glomerular nephritis 3. Alport syndrome (hereditary nephritis) 4. Thin glomerular basement membrane nephropathy 5. Membranous nephropathy 6. Focal segmental glomerulosclerosis Multisystem disease: 7. SLE nephritis 8. HSP nephritis 9. Wegener granulomatosis 10. HUS Tubulointerstitial disease: 11. Pyelonephritis 12. Interstitial nephritis 13. ATN Vascular: 14. Arterial or venous thrombosis Malignancy: 15. Wilms, rhabdomyosarcoma, angiomyolipoma = look for abdo mass and order renal U/S Post-renal: 16. Trauma (look at urethra, genital lesions) 17. Cystitis 18. Renal calculus

Answer 240

Look at the RBC shape! - If normally shaped, think post-renal causes - if abnormally shaped RBCs, hemoglobin, RBC casts, or proteinuria, think renal parenchymal causes

Answer 241

Glomerulus! - will usually see brown, cola or tea colored urine - will see proteinuria, RBC casts and deformed urinary RBCs (acanthocytes)

Answer 242

False negatives: presence of formalin or high urinary concentrations of vitamin C False positives: alkaline urine, contamination with hydrogen peroxide used to clean the perineum before obtaining a specimen

Answer 243

1. Prolonged seizures 2. viral myositis 3. Crush injury 4. Severe electrolyte abnormalities (hyperNa, hypoPO4) 5. hypotension 6. extreme exercise 7. DIC 8. toxins 9. metabolic disorders

Answer 244

Convoluted or collecting tubules

Answer 245

Bacterial urinary tract infection

Answer 246

1. Repeat the UA and BP in one month 2. If positive, initiate further work up at that time

Answer 247

Obstruction due to outflow anomalies

Answer 248

Prerenal: 1. Dehydration leading to hypoperfusion 2. Cardiogenic shock 3. Sepsis 4. Hemorrhage Renal: 1. Glomerular disease: acute glomerulonephritis (postinfectious, SLE, HSP, membranoproliferative, anti-glomerular basement membrane) 2. Tubular disease: ATN, AIN, HUS, tumor lysis syndrome 3. Vascular: vasculitis, renal vein thrombosis Postrenal: 1. acute urinary obstruction = posterior urethral valves, ureterocele, tumor, urolithiasis, neurogenic bladder

Answer 249

1. Decreased urine output 2. Fluid overload (weight gain, peripheral edema, pulmonary edema) 3. Hypertension (headaches, papilledema) 4. Electrolyte abnormalities: hyperkalemia, acidosis 5. Elevated BUN and Cr = signs of uremia (pericardial rub)

Answer 250

RIFLE = risk, injury, failure, loss, end-stage - Risk: estimated creatinine clearance (CCI) decreased by 25%, U/O 4 wk - End stage: persistent failure \> 3 mo

Answer 251

FENa helps you differentiate between prerenal cause of ARF from renal cause of ARF FENa calculation: 100 x (Urinary sodium x plasma creatinine) / (plasma sodium x urinary creatinine) -think "U PE, PE U!!" Gotta pee first to get a "peeewww, it stinks!" -OR just remember that U pee sodium = urine sodium is on top (numerator) and then you can figure out the rest! -Prerenal cause: patients whose urine showed an ELEVATED specific gravity (ie. concentrated), elevated urine osmolality (UOsm \> 500 mOsm/kg), low urine sodium (UNa 40 mEq/L) and FENa \> 2% = kidney is not able to concentrate urine and thus losing lots of Na and fluids

Answer 252

AIN = immune-mediated inflammatory response affecting renal interstitium and tubules (spares glomeruli and vasculature) -clinical features: acute renal failure, fever, flank pain, rash, arthralgias from hypersensitivity reaction and inflammation

Answer 253

1. Antibiotics (penicillin analogs, cephalosporins, sulfonamides, rifampin) 2. NSAIDs 3. Diuretics (thiazides and furosemide) - lab findings: urinary sediment (RBCs, EOSINOPHILS, WBC casts), proteinuria, FENa \> 1%, electrolyte abnormalities - treatment: usually high fluid volume to dilute and wash through the offending agent

Answer 254

1. Anemia (decreased Epo production) 2. Growth failure 3. Fluid overload 4. Bone deformities (vit D deficiency) 5. Electrolyte abnormalities 6. Hypertension

Answer 255

Minimal change disease - always assume that young children with nephrotic syndrome have minimal change disease and treat them with steroids - only get a renal biopsy if they do not respond to steroids - steroids 2 mg/kg/day x 4-6 weeks, then taper - most patients with minimal change disease will respond within the first month of treatment with normalization of urinary protein excretion

Answer 256

Peritoneal dialysis: - slow, small volume fluid shifts - done at home every night during sleep - need initial minor surgery to place peritoneal catheter - infection risk: peritonitis - slow toxin removal - better for growth than HD - children feel better with PD since there are no acute fluid shifts and less cytokine release as a result HD: - faster large volume fluid shifts - rapid toxin removal - needs to be done 3x/wk at medical centre - need initial minor surgery to place graft - infection risk: graft infection/bacteremia

Answer 257

hink PALE 1. Proteinuria \> 40 mg/m2/hr, Pr:Cr ratio \> 2 2. hypoAlbuminemia 3. hyperLipidemia (since liver is working overtime to try to replace lost proteins ) 4. Edema \*\*\*may also have hypercoagulability from loss of protein C & S (thus increasing risk of clots) and high risk of infections from loss of complement and antibodies in urine

Answer 258

1. Minimal change disease 2. Membranous nephropathy 3. Focal segmental glomerular sclerosis 4. Diabetic nephropathy

Answer 259

Podocyte effacement, vacuolation, appearance of microvilli on electron microscopy - unclear etiology - called minimal change disease because changes to kidneys are very subtle

Answer 260

Membranous nephropathy = rare but seen in older children, autoimmune or infectious cause in about 33% - gold standard for diagnosis and only way to diagnosis = renal biopsy = serum IgG complex deposition leading to glomerular basement membrane damage - on pathology: see basement membrane spikes, granular C3 and IgG deposits - treatment: immunosuppression and steroids but may not change course of illness - prognosis: 1/3 remission spontaneously, 1/3 ongoing proteinuria, 1/3 ESRD

Answer 261

Nephrotic syndrome vs. Nephritic syndrome

Answer 262

1. Children of any age who do NOT respond to initial course of steroids or have persistent relapses despite steroids 2. Children \> 8 years old with nephrotic syndrome (minimal change disease less likely in older children so gotta rule out FSGS, membranous nephropathy) 3. Presence of hypertension, renal insufficiency, RBC casts = should NOT be seeing this in minimal change disease and so biopsy

Answer 263

Usually see FSGS in patients with HIV, hep B, IV drug users, sickle cell disease - pathophysiology: sclerosis and scarring of glomeruli - diagnosis: renal biopsy - treatment: long-term immunosuppression and plasmaphresis - prognosis: poor (high rate of ESRD in kids)

Answer 264

Basement membrane damage from glucose end products -thick basement membrane, Kimmesteil-wilson nodules

Answer 265

PHAROH 1. Proteinuria (but less than nephrotic range) 2. Hematuria 3. Azotemia (elevated BUN) 4. RBC casts 5. Oliguria 6. Hypertension

Answer 266

PIGS Always Pick the Most Wealthy Hogs 1. Postinfectious glomerulonephritis 2. IgA nephropathy 3. Alport syndrome 4. Membranoproliferative GN 5. Thin basement membrane 6. Goodpasture/anti-GBM 7. Wegener's 8. HUS 9. SLE 10. Pauci-immune microscopic polyangitis

Answer 267

1. Postinfectious glomerulonephritis (C3 will normalize 1-8 wks after presentation) 2. Membranoproliferative GN 3. SLE

Answer 268

- IgA nephropathy = hematuria starts within 1-2 days after a viral URTI. Duration of hematuria = short (5 days) - Postinfectious GN = hematuria starts after 7-21 days after a viral URTI or impetigo skin infection (has a latency period). Duration of hematuria = long (4-6 wks) - labwork: IgA = NORMAL C3 levels whereas in postinfectious GN, have low C3 levels

Answer 269

Renal biopsy = see IgA deposits in glomerulus with MESANGIAL inflammation/proliferation - more common in boys - no benefit in ordering serum IgA - treatment: supportive (treat hypertension and proteinuria with ACE-I, may benefit from steroids) - prognosis: 20-40% with ESRD but MOST have benign courses. Need long term follow up

Answer 270

A cause of glomerulonephritis = presence of persistent microscopic hematuria and isolated thinning of the glomerular basement membrane - type 4 collagen mutation - familial (sporadic or AD trait) - treatment: none needed

Answer 271

Type 4 collagen mutation (major component of basement membranes) - inheritance: 85% are X-linked, rest are autosomal recessive - renal biopsy: mesangial proliferation, glomerular sclerosis, FOAM CELLS (lipid containing interstitial cells)

Answer 272

1. Asymptomatic microscopic hematuria - some may have recurrent gross hematuria 1-2 d after URTI 2. Proteinuria in boys more commonly than in girls 3. Bilateral sensorineural hearing loss 4. Ocular abnormalities (anterior lenticonus = extrusion of the lens into the anterior chamber is pathognomonic, corneal erosions) Investigations: 1. Urinalysis of patient and all 1st degree relatives 2. Ophtho exam 3. Hearing test 4. Skin or Renal biopsy Treatment: No treatment available although ACE-I can slow progression to ESRD Prognosis: all progress to ESRD in adulthood and eventually require dialysis or renal transplant. - 15% of affected boys have ESRD before age 15. - Boys have worse prognosis than girls

Answer 273

Alport syndrome! -anterior lenticonus is pathognomonic

Answer 274

Anti-GBM glomerulonephritis = autoimmune condition where IgG attacks type 4 collagen in glomerular basement membrane - if pulmonary involvement = Goodpasture disease - renal biopsy: see anti-GBM antibiodies - treatment: plasmapheresis and steroids

Answer 275

1. Microangiopathic hemolytic anemia - need to see acute onset anemia with microangiopathic changes on peripheral blood smear (schistocytes, burr cells, helmet cells) 2. Renal failure - need to see hematuria, proteinuria or elevated Cr 3. Thrombocytopenia - may not see initially early in the illness Criteria for CONFIRMED diagnosis: 1. Lab criteria: need both ANEMIA and RENAL INJURY (thrombocytopenia is variably seen and thus not necessary for diagnosis) 2. Acute illness that began 3 weeks after onset of an episode of acute or bloody diarrhea \*\*\*\*Probable diagnosis = no clear history of acute or bloody diarrhea in preceding 3 weeks but meets lab criteria

Answer 276

1. Infection: verotoxin producing E coli, shiga toxin-producing shigella, neuraminidase-producing Strep pneumo, HIV 2. Genetic 3. Diseases causing microvascular injury: SLE, HELLP, antiphospholipid antibody syndrome 4. Medication induced: cyclosporine, tacrolimus, etc.

Answer 277

1. Undercooked meat 2. Unpasteurized milk or apple cider 3. Petting farms 4. Swimming in contaminated ponds/lakes/pools

Answer 278

Based on clinical diagnosis!!! - rarely need renal biopsy since the risk of biopsy is significant during active phase of disease - pathogenesis: toxins cause direct endothelial cell damage in the glomerulus which then activates platelets and localized thrombosis = then the RBCs being filtered through the glomerulus get sheared and you get anemia

Answer 279

- E coli HUS = usually have diarrheal prodrome - strep pneumo = usually have pneumonia and empyema when they develop HUS - Strep pneumo has much worse prognosis (mortality 20% compared to

Answer 280

Hyperkalemia due to renal failure AND hemolysis spilling K into the serum and not being able to pee it out

Answer 281

Majority of patients with HUS have some CNS involvement = most are mild (irritability, lethargy, nonspecific encephalopathic features) -some have seizures and strokes (due to microvascular CNS thrombosis)

Answer 282

Conditions causing acute kidney failure with hemolytic anemia and thrombocytopenia: 1. SLE 2. Malignant hypertension 3. Bilateral renal vein thrombosis

Answer 283

NO! Antibiotics can result in increased toxin release worsening the HUS so don't use it! -if pneumonia associated HUS, then yes use antibiotics

Answer 284

Nephrotic syndrome is considered a hypercoagulable condition! 1. DVT with pulmonary embolism 2. Renal vein thrombosis 3. Sinus venous thrombosis (Neuro recommends CT head in any patient with nephrotic syndrome presenting with headache)

Answer 285

Hypertensive encephalopathy! -take BP right away

Answer 286

\*\*\*Overall, related to high blood pressure 1. Hypertensive encephalopathy 2. Heart failure from hypervolemia or hypertension

Answer 287

Usually full recovery = acute phase resolves within 6-8 wks with normalization of proteinuria, hypertension but persistent microscopic hematuria can persist x1-2 years after initial presentation

Answer 288

1. Acute renal failure 2. Nephrotic syndrome 3. Absence of evidence of strep infection 4. Normal complement levels

Answer 289

1. Increasing ASOT titres 2. Positive streptozyme or anti-DNase B 3. Decreased C3 level - treatment: supportive

Answer 290

1. Hypertension (due to renin production by the damaged kidney) 2. Recurrent UTIs 3. Abdominal mass (secondary to obstruction) - should have normal renal function since their one kidney works well enough for both! - indication for nephrectomy: recurrent UTIs or severe HTN not managable by medical therapy

Answer 291

Either stillborn or die from respiratory failure -renal agenesis = cannot produce urine in utero = oligohydramnios = pulmonary hypoplasia

Answer 292

Presents either prenatally with oligohydramnios or early in infancy 1. Abdominal mass from cysts = SMALL CYSTS 2. Hypertension 3. Potter facies 4. Polyuria 5. Polydipsia 6. MAY see cysts in lung, pancreas, liver 7. HEPATIC FIBROSIS = can have signs of portal hypertension - see big echogenic kidneys with cysts on ultrasound - prognosis: fast progression to ESRD in 100% of patients - treatment: dialysis, growth hormone, bone growth support, eventual transplant

Answer 293

1. Protein loss = malnutrition 2. Poor bone growth secondary to deficient Vit D production 3. May be secondary to steroid therapy 4. Possibly anemia (not great evidence supporting this)

Answer 294

Presents in adulthood and has slower, variable course than ARPKD 1. Abdominal mass from renal cysts (big cysts) 2. Family history 3. UTIs 4. Chronic renal failure - associated findings: hepatic cysts, cerebral aneurysms (all patients with ADPKD need head imaging to rule out aneurysms) - ultrasound: can be normaly initially - prognosis: slow progression to ESRD in middle age or later, some people don't progress to ESRD - traetment: BP management with ACE-I, occasional need for transplant

Answer 295

Autosomal recessive disease caused by a lysosomal transporter mutation = leads to intracellular cystine accumulation and subsequent Fanconi syndrome from proximal tubule damage - cystine accumulates in kidneys, thyroid, cornea, pancreas - clinical manifestations: 1. chronic renal failure (growth failure, rickets) 2. ocular abnormalities (corneal opacities, visual impairment) 3. Hypothyroidism 4. Diabetes \*\*\*\*THERE ARE NO STONES WITH CYSTINOSIS (only with cystinuria)

Answer 296

1. Urinalysis: elevated pH, bicarb, glucose and amino acids due to Fanconi syndrome 2. Slit lamp exam = accumulation of cystine crystals in the cornea 3. Serum cystine levels are normal, urine cystine levels mildly elevated 4. Confirmation by cystine in leukocytes - treatment: 1. Oral cysteamine (enzyme that cleaves cystine) 2. Electrolyte and vit D replacement 3. Growth hormone 4. Renal transplant

Answer 297

1. Uric acid 2. Myoglobin 3. Hemoglobin

Answer 298

Idiopathic or familial hypercalciuria -most common type of renal calculi = calcium stones

Answer 299

1. Proteus UTI 2. Foreign body

Answer 300

1. UA: may see hematuria 2. AXR first to see if you can visualize the stone, then US or CT if xray negative (calcium stones are easily seen on xray and they are the most common type) 3. BUN/Cr to evaluate renal function 4. Serum calcium to look for hypercalcemia 5. 24 hr urine collection for calcium, lytes, pH 6. Straining the urine to obtain stones for analysis

Answer 301

1. 1.5-2x IV hydration 2. Analgesia 3. Strain urine for stones for analysis and to document stone passage 4. Surgical removal or lithotripsy if large stone \> 7 mm 5. Treat underlying etiology - normocalcemic hypercalciuria: thiazide diuretics to increase tubular calcium reabsorption, mild decrease in dietary calcium, low Na, high K diet - hypercalcemic hypercalciuria = reduce serum calcium based on etiology

Answer 302

Increased anion gap: MUDPILES - methanol - uremia - DKA, other causes of ketosis - paraldehyde - isoniazid, iron, inborn errors of metabolism - lactic acidosis - ethylene glycol - salicylate Normal anion gap: - Diarrhea - renal tubular acidosis

Answer 303

1. Distal RTA (type I) = hypokalemia, hypercalciuria, nephrolithiasis and nephrocalcinosis, urine pH \> 5.5 (cannot acidify their urine!!) - can be congenital or acquired (medications, acquired renal disease) - think HIGH things = high calcium in urine, high urine pH 2. Proximal RTA (type II) = rarely present in isolation, usually part of Fanconi syndrome - get hypoPO4, glycosuria, aminoaciduria, urine pH

Answer 304

Proximal RTA! (secondary to hypophosphatemia)

Answer 305

Increased urinary output: 1. DM 2. DI 3. Sickle cell disease 4. Excessive water intake Increased bladder irritability: 5. UTI 6. Constipation Structural problems: 7. Ectopic ureter 8. Epispadias (females) 9. Thickened bladder wall (males) Abnormal sphincter control: 10. Spinal cord abnormalities 11. Neurogenic bladder 12. Sphincter weakness

Answer 306

Night time incontinence after 6 yo with no preceding period of dryness

Answer 307

Lack of consistent daytime continence by age 4 -usual causes: stress incontinence, constipation

Answer 308

- Hypertension: BP \> 95th% on 3 separate occasions - hypertensive urgency: BP \>99th% with headache or vomiting but no evidence of end organ damage - hypertensive emergency: BP \> 99th% with end organ effects (encephalopathy, seizure and/or renal dysfunction)

Answer 309

1. At rest x 3-5 mins 2. Right arm at heart level resting on solid surface 3. Width of cuff bladder = 40% of mid-humoral circumference 4. Length of cuff bladder = 80% of mid-humoral circumference 5. Inflate to 20 mm Hg above point at which radial pulse is gone

Answer 310

Older children = more likely to be primary htn, especially obese or family history -younger children = more likely to have secondary htn due to an underlying etiology

Answer 311

remember that for hypertensive emergency, you NEED to give IV meds

Answer 312

Start with trial of exercise, calorie reduction and sodium restriction before treating with medications

Answer 313

Remember that medications are used to treat refractory primary HTN and secondary HTN 1. ACE-Inhibitors 2. Beta blockers 3. Calcium channel blockers 4. Diuretics 5. Clonidine

Answer 314

1. Abdominal pain 2. Abdominal mass 3. Growth failure 4. UTI 5. Oliguria Causes: 1. Hydronephrosis 2. Ureteropelvic junction obstruction 3. Ureterocele 4. Posterior urethral valves 5. Duplicated collecting system

Answer 315

Multicystic dysplastic kidney disease

Answer 316

Grades 4-5 VUR on VCUG

Answer 317

Downward curvature of erect penis due to scarring of tissue on ventral surface of penis = can be associated with hypospadias -treatment: lysis of ventral adhesions for minor repairs, tissue graft for severe cases

Answer 318

Hypospadias = urethral meatus is on ventral surface of penis

Answer 319

Occurs only in males = absence of abdominal musculature 1. Complete lack of abdominal wall muscles (thus belly looks wrinkly) 2. Abdominal testes (cryptorchidism) 3. GU abnormalities with dilated urinary collecting system (unobstructed) - 30% progress to end stage renal disease and end up needing renal transplant 4. Clubfeet (from oligohydramnios if kidneys are not making urine) 5. Malrotation Treatment: 1. Orchidopexy by 1 year 2. Most likely ureteral implantation due to valve incompetence and reflux leading to UTIs 3. Abdominoplasty later in life

Answer 320

Incarceration and ischemia of the penile glans after insult or flipped retraction of the prepuce

Answer 321

Blue dot sign = testicular appendiceal torsion! - blue dot = engorged hypoxic appendage - treatment: need to get an ultrasound to rule out testicular torsion. If normal, then treat testicular appendiceal torsion with supportive care only since there are no complications from this condition. Give NSAIDs! Pain usually resolves within 2 weeks

Answer 322

Adolescent males: STI Children: E coli -useful screening test = urinalysis to look for pyruria, bacteriuria

Answer 323

Usually none since most are asymptomatic and resolve by adolescence when estrogen levels increase - can use daily estrogen cream x 1 wk followed by petroleum jelly daily x 1 mo to prevent recurrence for symptomatic or persistent adhesions - surgical lysis option also

Answer 324

Disease of proximal tubule of kidney = failure to reabsorb glucose, amino acids, uric acid, phosphate and bicarb! -clinical features: 1. Polyuria, polydipsia and dehydration (from glycosuria) 2. Growth failure -causes: Inherited = 1. CYSTINOSIS (most common cause) 2. Wilson's disease 3. Galactosemia Acquired 4. Medications: tetracyclines, valproic acid, etc. - complications: 1. Type II RTA (Proximal) 2. Rickets due to hypophosphatemia

Answer 325

1. Grade I = reflux of urine into an undilated ureter 2. Grade II = reflux into ureter and collecting system without dilatation 3. Grade III = dilatation of the ureter and collecting system without blunting of the calyces 4. Grade 4 = blunting of the calyces 5. Grade 5 = Even more dilatation and tortuosity of the ureter

Answer 326

C! Undescended testis are highly mobile and increase risk of testicular torsion! -orchidopexy improves fertility but the patient may still have reduced sperm counts

Answer 327

Dilatation of the pampiniform venous plexus due to valvular incompetence of the spermatic vein

Answer 328

1. Recurrent gross hematuria 2. Persistent microscopic hematuria 3. Dysuria or abdominal pain - test to confirm diagnosis: urine calcium:creatinine ratio (\>0.2 is diagnostic)

Answer 329

Cause: autosomal recessive condition with defect in thick ascending limb of loop of henle -usually present with persistent vomiting, polyuria/polydipsia 1. Hypokalemia 2. Hypercalciuria 3. Metabolic Alkalosis 4. Hyperaldosteronism 5. Hyperreninemia

Answer 330

Proximal tubule: reabsorption of 1. 66% of NaCl 2. 99% Glucose 3. 99% Amino acids 4. 99% Potassium 5. 99% Phosphate 6. 75% of bicarbonate - production of 1,25(OH)2-cholecalciferol (calcitriol) Loop of Henle: reabsorption of 1. 25% of NaCl Distal tubule (distal convoluted tubule and collecting ducts): reabsorption of 1. DCT (impermeable to water) = The rest of the NaCl via Na-K exchange (this is where aldosterone acts to reabsorb Na in exchange for K going into urine) 2. Collecting duct = responds to ADH to reabsorb water and also secretes H+

Answer 331

Non-heritable 1. Contralateral hydronephrosis 2. VUR in contralateral kidney 3. Late hypertension 4. Wilms tumor - monitoring: ultrasound, renal function tests and BP annually. Only obtain VCUG if contralateral hydronephrosis is found - prognosis: in 50% of patients, cysts regress by age 7 but this does NOT change the risk of developing a malignancy

Answer 332

1. Hypocalcemia 2. Hyperglycemia

Answer 333

HSP -IgA deposited into the kidney

Answer 334

1. Urinalysis x 6 months 2. BP x 6 months

Answer 335

1. Fluid restriction if child is hyponatremic 2. If dehydrated, then give albumin and lasix

Answer 336

1. Hypernatremia 2. Hypokalemia 3. Metabolic alkalosis - remember that Na+ is coupled with HCO3- and kicks out K+

Answer 337

1. Aortic root abnormalities 2. Mitral valve prolapse 3. Cysts on liver, ovaries, spleen 4. Berry aneurysms in brain 5. Increased risk of renal cell carcinoma

Answer 338

1. Von Hippel Lindau 2. Tuberous Sclerosis

Answer 339

No more than 0.5 meq increase per hour (no more than 12 mEQ/day) -1 mmol Na increases serum Na by 1 mEQ

Answer 340

- Hypovolemic hyponatremia: 1. vomiting 2. diarrhea 3. burns 4. 3rd spacing Hypervolemic hyponatremia: 1. Heart failure 2. Liver failure 3. Sepsis 4. Nephrotic syndrome Euvolemic hyponatremia: 1. SIADH 2. Water intoxication 3. Hyperosmolar (high serum glucose)

Answer 341

Any pelvicaliectasis \> 5 mm requires postnatal ultrasound prior to discharge to rule out obstructive uropathy

Answer 342

Trimethoprim or Septra

Answer 343

If UTI free \> 1 yr, can stop prophylaxis antibiotics

Answer 344

First morning urine analysis x 2 to rule out orthostatic proteinuria -if this is ruled out, THEN pursue Pr:Cr ratio or 24 hr urine collection

Answer 345

1. Testicular cancer 2. Varicocele 3. Spermatocele 4. Hydrocele 5. Nonincarcerated inguinal hernia

Answer 346

1. PCKD = inherited disorder; MCKD is not 2. PCKD = bilateral; MCKD = unilateral (bilateral is not compatible with life)

Answer 347

DMSA! - better visualizes renal parenchymal abnormalities - can also diagnose pyelonephritis

Answer 348

1. Posterior urethral valves 2. Vesicourethral reflux

Answer 349

1. Development of nephrotic syndrome 2. Gross hematuria in childhood 3. Prominent glomerular basement membrane thickening on renal biopsy

Answer 350

Nothing! -single umbilical artery only suggests renal anomaly if associated with other abN

Answer 351

1. External ear anomalies 2. Imperforate anus 3. Scoliosis 4. Single umbilical artery associated with other abnormalities on exam

Answer 352

Fluid restriction = 1 L/m2/day

Answer 353

1. Septations in the cyst 2. Calcifications in the cyst 3. Solid component in the cyst

Answer 354

Age 2-5 yo. After that, new scars are super rare. SO, if you see scarring from vesicourethral reflux, it must've happened when they were really young. Don't need to repeat VCUG once kids are older and are UTI free even if they have residual VUR.

Answer 355

1. Hyperhydration (2x maintenance) 2. Diuresis with mannitol perfusion

Answer 356

1. Lasix and albumin infusion for severe edema 2. Volume restriction 3. Steroids if typical picture for minimal change disease and no indications for renal biopsy 4. May need antihypertensives 5. Restriction of salt intake

Answer 357

1. Complete history and physical looking at blood pressure, optic discs, skin, abdomen, genitalia 2. Confirmation of true hematuria by urine R&M 3. Urine culture 4. Urine calcium, protein, creatinine 5. CBC, BUN, Cr, Calcium level, total protein and albumin 6. Streptozyme or ASOT titres, C3, C4 7. Renal U/S

Answer 358

Renal osteodystrophy = related to phosphate retention from low GFR and diminished 1,25-dihydroxyvitamin D production in the kidney. Also get secondary parahyperthyroidism due to decreased calcium (from too much phosphate retention and thus loss of calcium into the urine) 1. Restrict phosphate in the diet 2. Oral phosphate binders 3. Supplemental calcium

Answer 359

Essential primary hypertension

Answer 360

Vertebral anomalies Anal atresia Cardiac defects TEF Renal dysplasia Limb defects

Answer 361

Renal cause = most commonly ureteropelvic junction obstruction (according to baby nelsons) OR multicystic dysplastic kidney disease (according to mama nelsons)

Answer 362

1. Hematuria 2. Concentrated urine 3. Alkalotic urine 4. Use of antiseptic agents

Answer 363

* Alagille syndrome (arteriohepatic dysplasia). Also called syndromic bile duct paucity * constellation of conjugated hyperbilirubinemia and cholestasis; typical triangular facies * butterfly vertebrae; and eye findings of posterior embryotoxon and Axenfeld anomaly or iris processes * pulmonic stenosis; peripheral pulmonic stenosis

Answer 364

Most umbilical hernias smaller than 0.5 cm spontaneously close before a patient is 2 years old.

Answer 365

Intussusception

Answer 366

colicky pain, vomiting, and passage of bloody stool Most common: Ileocolic intussusception

Answer 367

* Underweight: \< 5th percentile * Healthy weight: 5th to 85th percentile * Overweight: 85th to 95th percentile, * Obese: 95th to 98th percentile * Severely (morbidly) obese: ≥ 99th percentile

Answer 368

IIa is the most common

Answer 369

*_Kwashiorkor_* is edematous malnutrition as a result of low serum oncotic pressure. The low serum proteins result from a disproportionately low protein intake compared with the overall caloric intake. These children appear replete or fat, but they have dependent edema, hyperkeratosis, and atrophic hair and skin. They generally have severe anorexia, diarrhea, and frequent infections, and they may have cardiac failure. *_Marasmus_* is severe nonedematous malnutrition caused by a mixed deficiency of both protein and calories. Serum protein and albumin levels are usually normal, but there is a marked decrease in muscle mass and adipose tissue. Signs are similar to those noted in hypothyroid children, with cold intolerance, listlessness, thin sparse hair, dry skin with decreased turgor, and hypotonia. Diarrhea, anorexia, vomiting, and recurrent infections may be noted.

Answer 370

the serum sodium concentration should not be allowed to decrease faster than 0.5 mEq/L per hour and ideally not more than 10 to 12 mEq/L/24 hr.

Answer 371

* Use of erythropoietin, a hormone normally produced in the kidney, has largely eliminated the need for blood transfusions in children with CKD. * Use of 1,25-dihydroxycholecalciferol, an active form of vitamin D normally produced in the kidney, has dramatically prevented or treated osteodystrophy. * Growth hormone (GH) is not produced by the kidney. However, recombinant GH administration results in growth acceleration in growth-retarded children with CKD. * Inhibition of the renin-angiotensin-aldosterone system with ACE inhibitors or angiotensin receptor blockers (ARBs) has helped control hypertension and also prevented progression of glomerular fibrosis.

Answer 372

IgA nephropathy (IgAN) The simultaneous occurrence of upper respiratory symptoms and gross hematuria make poststreptococcal glomerulonephritis less likely.

Answer 373

Postinfectious glomerulonephritis, including poststreptococcal (ASPGN); staphylococcus in subacute bacterial endocarditis * Lupus nephritis * Membranoproliferative glomerulonephritis * C3 glomerulopathy * aHUS

Answer 374

Symptoms and signs begin about 7 to 14 days after pharyngitis and as long as 6 weeks after a pyoderma

Answer 375

Recurrent gross hematuria and persistently low C3 are extremely rare in ASPGN. C3 glomerulopathy

Answer 376

Hypercalciuria

Answer 377

Prehypertension: BP readings between the 90th and 95th percentile or \> 120/80 mm Hg in adolescents. Hypertension: systolic BP and/or diastolic BP ≥ 95th percentile for age, sex, and height (on 3 repeated measurements on different [nonconsecutive] days). Stage 1 hypertension: BP ≥ the 95th percentile and less than the 99th percentile + 5 mm Hg. Stage 2 hypertension: BP \> 99th percentile + 5 mm Hg.

Answer 378

Indications are severe edema with incapacitating anasarca, cellulitis, skin breakdown, or respiratory embarrassment from pleural effusions

Answer 379

* Hypercoagulable state, which may result in sagittal sinus, cavernous sinuses, and renal veins thrombosis * Peritonitis caused by S. pneumoniae or E. coli

Answer 380

Modified Schwartz The modified formula is [0.413 × (height in cm/serum creatinine in mg/dL)]

Answer 381

Cystine crystals

Answer 382

Chronic hyperchloremic metabolic acidosis with an inability to acidify the urine and a normal serum anion gap

Answer 383

Type 1 (distal) RTA:Inability of the distal tubule to secrete hydrogen; in the presence of significant acidosis, urine is not maximally acidified (pH \< 5.5) Type 2 (proximal) RTA:Decreased ability of the proximal tubule to reabsorb filtered HCO3 at normal plasma HCO3 concentrations Type 4 RTA: Acquired or inherited tubular insensitivity to aldosterone or to an absence of aldosterone

Answer 384

Macroscope hematuria Severe HTN Persistent Renal insufficiency Low serum C3 Persistent proteinuria beyond 4 weeks of steriods

Answer 385

Urine protein/Cr ratio \<0.2 normal \>3 nephrotic

Answer 386

MONSTER M: MEds Obesity Neonatal Hx Symptoms/Signs Trends in the family Endocrine Renal

Answer 387

AR, sBDS Bone marrow failure Panceatice dysfunction Sketletal abN INfections

Answer 388

b. hypocalcemia, hyperglycemia

Answer 389

Hypercalciuria - 24 hour urine calcium \> 4mg/kg/day or urine calcium to creatinine \> 0.2 for hypercalciuria

Answer 390

- Labetolol (contraindicated in asthma patients) - Hydralazine (can lead to reflex tachycardia) - Nicardipine (can lead to reflex tachycardia) - Sodium nitroprusside

Answer 391

d. Zinc deficiency

Answer 392

- Nephrotic syndrome (most common cause for hypoalbuminemia is renal) - Liver disease (impaired production) - Malnutrition - Protein losing enteropathy (CMP allergy, Celiac disease, lymphangiectasia) - Inflammatory bowel disease - Congestive heart failure - Acute and chronic inflammatory conditions - Burns

Answer 393

- Disease \> 10 years - Onset before age 15 - Pancolitis - PSC

Answer 394

- Eat small meals, avoid eating before bed, avoid fatty, spicy, chocolate, caffeine, nicotine, peppermint, tomatoes - Stay upright 30 minutes after meal - Efficacy of sleep positioning in older children unclear, some evidence for left side position and head elevation - Weight loss in obese children - Eliminate smoke exposure (smoke reduces LES pressure)

Answer 395

- Recurrent aspiration - Refractory or Barrett esophagitis - Reflux associated apnea - GERD associated FTT not responsive to medical therapy

Answer 396

1. Alport's 2. Thin GBM disease 3. HUS FSGS

Nephro/Uro/GI Flashcards

(445 cards)