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Board Review nephrology

Board Review nephrology. Karen Estrella-Ramadan. Question 1.

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Board Review nephrology

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  1. Board Review nephrology Karen Estrella-Ramadan

  2. Question 1 • You are seeing a 10-year-old girl who has SLE for a WCC. You diagnosed SLE when she was 8y/o, based on an initial presentation of idiopathic thrombocytopenic purpura and a positive ANA test and the subsequent development of arthritis and a positive anti-double-stranded DNA test. She is currently doing well in school and has no concerns about her memory or problem-solving skills. The only finding of note on her physical examination is a slight erythematous rash in a malar distribution. • Of the following, the MOST useful screening test for other organ involvement in this girl is:

  3. Of the following, the MOST useful screening test for other organ involvement in this girl is: • Coombs test • erythrocyte sedimentation rate • magnetic resonance imaging of the brain • Urinalysis • Venereal Disease Research Laboratory (VDRL) testing 10 Countdown

  4. SLE patients have high mortality from Renal and CNS disease: • Renal: do a U/A to screen , initially may be subclinical • Renal disease occurs in 75% to 80% of children who have SLE, usually within 2 years of diagnosis. Renal manifestations are not typically apparent at diagnosis; most patients present with fever, rash, arthritis, and mucositis • Typical screening besides U/A, include: urine protein-to-creatinine ratio, and serum creatinine and albumin. • Renal manifestations can range from asymptomatic hematuria and proteinuria to overt nephritis (gross hematuria, azotemia, and hypertension), nephrotic syndrome, or hypertension. • Patients in whom proteinuria (>1+ on a dipstick or urine protein-to-creatinine ratio >0.2 [mg/mg]) is discovered need Renal referral for assessment and possible renal biopsy. • CNS: MRI if pt develops: seizures, psychosis, headaches, difficulty with concentration, or memory problems. • Other organs: • Hem: hemolytic anemia pallor, do Coombs + • ESR: non specific ; VDRL: false positive

  5. Question 2 • An 8-year-old boy presents with cola-colored urine without blood clots. He was well until 2 days ago, when he developed a sore throat with upper respiratory tract infection symptoms. He denies any dysuria, frequency, urgency, flank pain, or trauma. On physical examination, his temperature is 37.8°C, heart rate is 84 beats/min, respiratory rate is 18 breaths/min, and blood pressure is 118/78 mm Hg. He has no costovertebral tenderness, abdominal tenderness, or edema. • Labs show: • Urine: Specific gravity, 1.025, pH, 6.0, 3+ blood, 3+ protein, 1+ leukocyte esterase, Nitrite, negative • Microscopy shows more than 100 RBC/hpf and 5-10 WBC-hpf • BUN 24 mg/dL (8.6 mmol/L) • Creatinine, 0.9 mg/dL (79.6 mcmol/L) • C3: 140 mg/dL (normal, 80 to 200 mg/dL) • C4: 30 mg/dL (normal, 16 to 40 mg/dL) • ANA: negative

  6. Of the following, the most likely diagnosis is: • acute pyelonephritis • immunoglobulin A glomerulonephritis • postinfectious glomerulonephritis • Urolithiasis • viral cystitis 10 Countdown

  7. Dx: acute glomerulonephritiscola-colored urine, hematuria/proteinuria, and mild azotemia • Things to differentiate: • Urine color: • bright red: Lower tract bleeding viral hemorrhagic cystitis or stones often accompanied by clots. • Cola-colored urine without clots glomerularhematuria. • Yellow but on UA dipstick + RBC, do a UA and likely + for myoglobin, • Time: • Very recent URI: of but not specific for immunoglobulin A (IgA) glomerulonephritis. • 7-21 days later: PSGN

  8. Remember • Piuria: > 10WBC/hpf • Hematuria: >3 RBC/hpf in2 centrifugated samples of fresh urine • Proteinuria: >3.5 gr/day

  9. Evaluation of a child with suspected AGN • Follow up Blood pressure and creatinine levels • If HTN, or rising creatinine levels  renal bx • Follow serologic markers: C3 and C4, ANA and antiDNAds • The combination of a normocomplementemic GN and a synpharyngitic pattern =IgA nephropathy • Can also present with asymptomatic hematuria or hematuria/proteinuria. Patients who have gross hematuria have a better prognosis. • Treatment usually consists of steroids, ACE • Alternative diagnostic include: • Alport :renal, sensorineural hearing loss, ocular defects • Membranoproliferativeglomerulonephritis: low C3 + low C4 • PIAGN: strep infection, low C3, normal C4 • Focal glomerulonephritis (HIV): normal complement

  10. Question 3 • A 17-year-old sexually active girl presents for a follow-up evaluation after her third episode of a urinary tract infection. She is currently asymptomatic. The results of renal ultrasonography and voiding cystourethrography are negative. She asks you how to prevent further episodes.

  11. Of the following, you are MOST likely to advise her to • drink cranberry juice frequently • increase her daily water intake • make sure to void after intercourse • self-medicate with antibiotics for 3 days when symptomatic • use single-dose postcoital antibiotic prophylaxis 10 Countdown

  12. She has…acute cystitis • > common in girls, > 3 UTI in a yr • Best preventive measure ( even after 5y of use): • symptoms with sexual intercoursepostcoital antibiotic prophylaxis (taken within 2 hours of intercourse). • nitrofurantoin 50 mg, TMP-SMX] 40/200 mg, or cephalexin 500 mg • Those who fail, UTI not associated with a modifiable cause or at risk of complicated UTI give: continuous • TMP-SMX (40/200 mg daily at bedtime or 3 times per week) • nitrofurantoin (50 to 100 mg daily at bedtime or 3 times per week), • norfloxacin (200 mg daily at bedtime or 3 times per week) • and cephalexin (250 mg daily). • Stop meds after 6-12 months: trial, if fails, restart abx • Self-medication when symptomatic with a 3-day regimen is another option • Women with one to two UTI per year). • IF UTI after sexually active: no imaging • ~Behavior changes: “proper wiping,” avoid tight pants, avoid urge-initiated voiding, +postcoital voiding, increased fluid intake, and the daily consumption of cranberry juice

  13. Question 4 • You are examining a newborn who has wrinkling of the abdominal wall skin. His mother recalls her obstetrician mentioning that her “fluid was low.” The infant was born at 37 weeks’ gestation, and his birthweight was appropriate for gestational age.

  14. Of the following, the MOST likely additional findings expected in this infant are cryptorchidism and • bilateral hydronephrosis • Hypospadias • Nephrocalcinosis • polycystic kidney disease • unilateral renal agenesis 10 Countdown

  15. BL hydroureteralnephrosis + absence of abdominal wall musculature +undescended testicle= prune-belly syndrome • PBS  obstructive uropathy • Associated with renal dysplasia BUT NOT: renal agenesis, nephrocalcinosis, cystic renal disease, hypospadias. • almost exclusively in males • 30% of affected patients eventually develop end-stage renal disease

  16. PRUNE-BELLY SYNDROME CRIPTORCHIDISM ABSCENSE OF ABDOMINAL WALL MUSCLE-WRINKLY

  17. Question 5 • A 6y/o girl presents for a WCC that was scheduled as a follow-up appointment after she had an elevated blood pressure at an urgent care facility during an evaluation for abdominal pain. Her abdominal pain has resolved. Her mother recalls the blood pressure in the urgent care center as 135/90 mm Hg. The girl has had 2 UTI with fever in the past, and her father had hypertension diagnosed at age 45 years. • On physical examination, the girl’s temperature is 37.3°C, heart rate is 90 beats/min, respiratory rate is 20 breaths/min, and blood pressure is 146/86 mm Hg. A repeat blood pressure reading is 142/88 mm Hg. The four limb blood pressures are: 142/88 mm Hg in the right arm, 144/84 mm Hg in the left arm, 156/100 mm Hg in the right leg, and 160/96 mm Hg in the left leg. You find no cardiac murmurs, abdominal bruits, or edema. Femoral pulses are 2+ and symmetrical bilaterally. • Renal ultrasonography shows the left kidney to be 8.5 cm with normal corticomedullary differentiation and the right kidney to be 5.5 cm with increased echogenicity.

  18. Of the following, the MOST likely cause for this patient’s elevated blood pressure is • coarctation of the aorta • essential hypertension • renal artery stenosis • renal hypoplasia/dysplasia • renal scarring from prior pyelonephritis 10 Countdown

  19. HTN, + hx of UTIs and US findings (increased ecogenicity) renal scaring from pyelonephritis • (+)VUR: HTN in 20% of pts • Up to 8 yrs to develop • (+)HTN: 30-40% had scarring Hypertension can occur in up to 20% of patients who have (+)VUR • Patients usually respond well to ACEIs • caution in female patients of childbearing age due to risks of teratogenicity. • If BL stenosis or on monorenal pt—DON’T USE ACEIs will worsen renal failure due to inhibition contraction of efferent arteriole that will maintain glomerular filtration

  20. Why not? • Coartation of the aorta: femoral pulses are present and blood pressures are higher in the legs than in the arms • Essential HTN: is a diagnosis of exclusion mild hypertension in adolescent patients who are overweight and have a positive family history of hypertension. • Renal artery stenosisis rare, associated with abdominal bruits on examination and a small kidney on ultrasonography. • Renal hypoplasia/dysplasia can be associated with an isolated small kidney, BUT hypertension is uncommon in this setting.

  21. Question 6 • You are called to attend the delivery of a newborn male who had been monitored carefully prenatally for moderate oligohydramnios. Prenatal ultrasonography suggested bilateral cystic dysplastic kidneys, with no other congenital anomalies identified.

  22. Of the following, the MOST likely immediate life-threatening problem in this infant is • bladder outlet obstruction • cardiac arrhythmias • liver failure • renal failure • respiratory failure 10 Countdown

  23. The most immediate possibility of respiratory failure from pulmonary hypoplasia. • due to decreased amniotic fluid volume, which may lead to reduced fetal respiratory excursion which impedes pulmonary development. • “Potter sequence” (oligohydramnios with pulmonary hypoplasia, clubfeet, and flattened facies)

  24. Question 7 • A 1-year-old girl presents for a health supervision visit. Her father underwent kidney transplantation for end-stage renal disease due to autosomal dominant polycystic kidney disease. The parents request assessment of the child's kidney function. Her serum creatinine measurement is normal for age.

  25. Of the following, the MOST correct statement about serum creatinine measurements in the pediatric patient is that • serum creatinine value of 0.6 mg/dL (53.1 mcmol/L) is considered normal for a 1-year-old child • healthy preterm infants (<36 weeks' gestation) typically have lower serum creatinine values than term infants • males and females have similar serum creatinine values up to 17 years of age • newborns in the first few days after birth have lower serum creatinine concentrations than those seen in 2-month-old infants • serum creatinine values are related more closely to patient height than to patient age in children 10 Countdown

  26. Kidney function is assessed best by measurement of the glomerular filtration rate (GFR • The serum creatinine is the most frequently used agent to assess GFR clinically. • Skeletal muscle metabolism, high values during growth • The GFR in the pediatric patient increases throughout the first postnatal year before reaching adult values by approximately 18 months of age. • Newborn: high values-represent maternal value, declines after 10 days • Preterms have higher values • 1y/o: <0.4 mg/dl

  27. Question 8 • A parent requests referral to a urologist for her 7-year-old son because of his bedwetting problem. He has no urinary incontinence during the day and is otherwise healthy. Physical examination reveals normal growth parameters and normal genitalia with a circumcised phallus. Dipstick urinalysis results are normal, with a specific gravity of 1.025.

  28. Of the following, the MOST appropriate next step is • a complete blood count • psychological evaluation • reassurance and a follow-up visit in 6 months • referral to a urologist • renal/bladder ultrasonography 10 Countdown

  29. Nocturnal enuresis • Involuntary passage of urine during sleep in children >5 y/o and occurs in approximately 15% of children at age 5 and 1% of teens at age 15. There is a male predominance and often a positive family history. • Child with NO daytime wetting, NO dysuria and normal UA: renal very unlikely • reassurance and a follow-up visit in 6 months • Child with BOTH daytime and nighttime wetting, older than 6y/o, enuresis + encopresis, or urinary symptoms further eval • assess genitourinary anatomy, exclude trauma from sexual abuse, detect lumbosacral spine anomalies, and exclude bladder dilatation or abdominal mass. • Older children and children for whom nocturnal enuresis is related to social dysfunction, family stress, or poor self-esteem may benefit from therapy to target those effects. • The use of a bedwetting alarm has the highest rate of success in young children.

  30. Question 9 • You are examining an asymptomatic adolescent male for his annual health supervision visit. Genital examination reveals genitalia at Sexual Maturity Rating 5 and a nontender mass in his left scrotum that extends from the inguinal canal to the upper pole of the testis The mass decreases in size when he lies down.

  31. Of the following, the MOST likely diagnosis is • direct inguinal hernia • Hydrocele • Spermatocele • testicular tumor • varicocele 10 Countdown

  32. Varicocele • Develops slowly • usually asymptomatic • >15-25y/o • > left side (drain to renal vein) • vary in size, can extend from the testis to the inguinal canal, and feel like a "bag of worms" • INCREASE in size with standing or valsalva • REDUCE when lying down • If a varicocele develops suddenly, does not reduce in the supine position, or is on the right side, a cause for obstruction of the spermatic vein should be sought. Pampiniform plexus

  33. Why not? • Inguinal hernias: painless, intermittent groin masses that appear with straining and usually reduce spontaneously. • They may increase in size and become obstructed, resulting in a painful scrotal mass. • Hydrocele: fluid within the tunica vaginalis that surrounds the testis rather than being distinct from it. • Transilluminates • Spermatoceles are cystic lesions within the spermatic cord that are above and distinct from the testis and transilluminate. • Testicular tumors are painless solid masses within the testicle that do not transilluminate.

  34. Question 10 • The attending neonatologist notifies you that she is caring for a newborn boy in your practice who has multiple congenital anomalies. The baby was transferred from the newborn nursery to the intensive care unit due to cyanosis. Echocardiography reveals findings consistent with tetralogy of Fallot. The infant has a cleft palate. Chest radiographs show multiple thoracic hemivertebrae. Renal ultrasonography documents unilateral renal agenesis. On physical examination, the infant has hypoplastic thumbs. The baby is normally grown and vigorous.

  35. Of the following, the MOST likely diagnosis for this infant is • CHARGE syndrome • Fanconi anemia • trisomy 18 • 22q11 deletion syndrome • VACTERL association 10 Countdown

  36. CHARGE syndrome • C: coloboma • H: heart • A: atresia choanae • R: retardation of growth and/or development • G: genital anomalies • E: ear anomalies

  37. Fanconi anemia • AR • thumb hypoplasia, short stature, microcephaly, abnormal ears, epicanthal folds, site of ureteralreimplantation, congenital dislocated hips • bone marrow failure • more common among persons of Ashkenazi Jewish heritage

  38. VACTERL • V: vertebral anomalies • A: anal atresia • TE: tracheoesophageal fistula • R: renal anomalies • C: cardiac defects • L: (radial)limb anomalies

  39. The combination of tetralogy of Fallot, hypoplastic thumbs, cleft palate, and a renal abnormality 22q11 deletion syndrome Recent studies have shown that tetralogy of Fallot with pulmonary atresia belongs to a spectrum of conotruncal cardiac malformations that are often associated with monosomy 22q11. The clinical presentation of monosomy 22q11 includes patients with conotruncal anomaly face syndrome, velo-cardio-facial syndrome, and DiGeorge syndrome. More recently these syndromes have been incorporated as a group under the acronym catch 22 (cardiac defect, abnormal face, thymichypoplasia, cleft palate, hypocalcaemia, and microdeletion 22q11)

  40. Question 11 • You are evaluating a newborn in whom intrauterine renal US results are abnormal. The child appears very edematous, but requires resuscitation with fluids for initial respiratory distress and hypotension. One day later, the child appears more edematous. • He has an excellent urine output. Measurements of his serum electrolytes reveal: • Na: 127mEq/L • K: 4.6mEq/L • Cl: 92mEq/L • HCO3: 27 mEq/L • BUN: 10mg/dL • Creatinine: 0.7 mg/dl • Albumin: 0.9g/dl • UA: no blood, but (4+) protein with a random urine protein to creatinine ratio of 43.5.

  41. Of the following, the MOST likely cause of this child’s clinical status is: • Acute glomerulonephritis • Congenital nephrotic syndrome • Diffuse mesangial sclerosis • Focal segmental glomerulosclerosis • Membranous nephropathy 10 Countdown

  42. Congenital nephrotic syndrome • Cystic formation sec to mutation in: nephrin (BM) leakage • Massive proteinuria decrease oncotic pressure edema • By 2 months: kidneys enlarged with loss of coticomedullary differentiation • By 12 mo: decrease in size with hyperechogenicity fibrosis/ glomerular sclerosis failure

  43. Rarely in newborn period: • Acute glomerulonephritis • Membranous nephropathy • Focal segmental glomerulosclerosis

  44. Question 12 • A 3 mo old boy is admitted to the hospital for evaluation of FFT. His bwt was at %50 and length at %75. Currently, his wt is below the %5 and length is at %25, His VS and PE are otherwise normal. • He appears well hydrated. • Measurement of serum electrolytes reveals; Na:139, K: 4.7, Cl: 114, bicarbonate : 12, BUN: 8, creatinine: 0.3. • A consulting nephrologist recommends measurement of urine pH (which is 7.5) and urine ammonium (which is 12000 mcm/L—normal is > 60000)

  45. Of the following the MOST likely cause of child’s acidosis is? • Inborn error of metabolism • Lactic acidosis • Type I (distal renal tubular) acidosis • Type II (proximal renal tubular) acidosis • Type IV (renal tubular acidosis) 10 Countdown

  46. Step 1 • Calculate anion gap help you narrow your differential • HIGH: • severe CRF • Ketoacidosis: diabetic, alcoholic, starvation • Lactic acidosis • Poisonings: salycilates, methanol, ethylene glycol • NORMAL: • Renal tubular acidosis • Diarrhea • Carbonic anhydrase inhibitors • Hyperalimentation with TPN

  47. RTA • 3 types • Type 1: distal • Type 2: proximal • Type 4: distal • NO TYPE 3

  48. Type 2-proximal • Inability to reabsorb HCO3 Very very alkaline urine • If distal is normal, will compensate a little by excreting H+ and the urine is ~6 • K: is low or normal • CAUSES • Fanconisd • Acetazolamide, 6 mercaptopurine • Heavy metals: lead, copper, mercury, cadmium • Disorders in protein, CHO, AA • Multiple myeloma

  49. Type2

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