THG21-001
An 8-year-old girl child was evaluated for abdominal pain. Her ultrasound KUB (kidney, ureter, and bladder) showed an abnormality for which the following investigation was done.
a. Identify the investigation.
b. What are the three different substances used for this study?
c. What is t½, intrarenal transit time (IRT), and its significance?
d. Interpret the investigation given in image below.
e. What is the next line of management?
Answer
| a. | Diuretic renogram/dynamic renal scintigraphy |
| b. | Diethylenetriamine pentaacetate (DTPA), ethylene dicysteine (EC), and mercaptoacetyltriglycine (MAG3) |
| c. | T½: time taken for 50% of the trace to get cleared, IRT: time taken for trace to appear in medial pelvis |
| d. | Significance: T½ >20 minutes and IRT >4 minutes is suggestive of pelvic ureteric junction obstruction (PUJO) |
| e. | Refer to pediatric surgeon for left pyeloplasty |
THG21-002
A 1-year-old male infant was evaluated for urinary tract infection (UTI). The following investigation was performed.
a. What is the investigation?
b. What is your diagnosis?
c. What are the three indications to perform the test in children with UTI as per current Indian Society of Pediatric Nephrology (ISPN) guidelines?
d. When should this test be performed after an episode of UTI?
e. Name the best alternative test for the investigation given in image here.
Answer
| a. | Micturating cystourethrogram (MCU) |
| b. | Right grade IV VUR |
| c. | (i) Recurrent UTI, (ii) abnormal ultrasound, (iii) non- Escherichia coli UTI in children below 2 years of age |
| d. | Any time after completion of UTI treatment |
| e. | DRCG: Direct radionuclide cystography |
THG21-003
A 2-year-old male infant was treated for pyelonephritis. During further evaluation, the following investigation was performed.
a. What is the investigation given in the image?
b. What is the substance used for this investigation?
c. What is your diagnosis?
d. When is this investigation performed after UTI according to current ISPN guideline?
e. What are the two indications for this investigation?
Answer
| a. | Static renal scintigraphy |
| b. | DMSA |
| c. | Right kidney is small with photopenic areas in both poles suggestive of scarring |
| d. | 4–6 months after an UTI episode |
| e. | (i) High grade (grade III/IV) VUR and (ii) Recurrent UTI |
THG21-004
Q21.4
a. What is the investigation given in the above image?
b. What is the diagnosis
c. Mention one indication for the above investigation.
d. Name two advantages of this investigation.
e. Name two disadvantages of the above investigation.
Answer
| a. | DRCG: Direct radionuclide cystography |
| b. | Right grade III VUR |
| c. | Alternative for MCU during follow-up of VUR. Children to check for resolution of reflux |
| d. | Less radiation, urinary catheterization not required |
| e. | Grading and anatomical details are inadequate. |
THG21-005
A 1-year-old male infant was evaluated for UTI and was found to have grade IV vesicoureteral reflux (VUR) on the right side. His dimercaptosuccinic acid (DMSA) scan showed of 42% function in the right kidney with scar in the upper pole. Child is on regular follow-up and has no breakthrough UTI.
a. Mention two indications for uroprophylaxis.
b. Name one drug for uroprophylaxis that can be used in this child and mention the dose.
c. When can you stop uroprophylaxis?
d. Is surgery indicated in this child?
e. Should we repeat the micturating cystourethrogram (MCU) in this child?
Answer
| a. | (i) Recurrent febrile UTI and bowel bladder dysfunction (BBD); (ii) Infant with recurrent febrile UTI and lower grade VUR; (iii) High-grade VUR |
| b. | Cotrimoxazole 2 mg/kg and nitrofurantoin 1–2 mg/kg |
| c. | Child older than 2 years if they satisfy all the three criteria: |
| i. | Toilet trained |
| ii. | Absence of BBD |
| iii. | No febrile UTI in preceding 1 year |
| d. | Surgery is not recommended in children as there is no break through UTI. |
| e. | According to the current ISPN guideline, repeat MCU is not routinely indicated. To check for resolution of reflux, it needs to be performed 4–8 years after initial diagnosis if deemed necessary by a treating physician. |
THG21-006
A 11-year-old female child was brought with second episode of UTI. She had a history of lower urinary tract symptoms, such as dysuria, urgency, and frequency. She does not urinate in the school and on further probing the history; she was constipated and passes hard stools once in 2–3 days. Her ultrasound showed the right kidney size of 10.1 cm and left kidney size of 10.8 cm, bilateral pelvicalyceal dilatation with anteroposterior diameter (APD) 6 mm, and postvoid residue (PVR) of 43 mL.
a. What is your diagnosis?
b. Mention the imaging to be done in these children.
c. How to calculate bladder capacity and what is the capacity in this child?
d. How do you initially manage this child?
e. What drug will you use if the initial management fails?
Answer
| a. | Recurrent UTI with BBD |
| b. | Ultrasound, MCU, and DMSA |
| c. | (Age in year + 1) × 30 mL = (11 + 1) × 30 = 360 mL |
| d. | She needs to be initiated on uroprophylaxis for recurrent UTI and initiate treatment for BBD. |
| Management of BBD: |
| • | Diet with increased fiber-rich food and water 6–8 cups per day |
| • | Laxative for constipation |
| • | Timed and double voiding |
| e. | Tamsulosin, Doxazosin, and biofeedback therapy |
THG21-007
Answer the following:
a. Mention two conditions for false-positive and negative in urine dipstick for albuminuria.
b. Define normal, subnephrotic and nephrotic range proteinuria according to spot urine protein creatinine ratio.
c. Define orthostatic proteinuria.
d. What precautions should a parent take while collecting a 24-hour urine sample.
e. Name two conditions in which transient proteinuria are seen.
Answer
| a. | False positive | False negative |
|---|---|---|
| Hematuria | Proteinuria other than albumin | |
| High specific gravity | Low specific gravity | |
| pH >8.0 | pH <4.5 |
| b. | SpotUPCR | Raang |
|---|---|---|
| <0.2 | Normal | |
| <0.2 to <2.0 | Subnephrotic range proteinuria | |
| >2.0 | Nephrotic range proteinuria |
| c. | Orthostatic proteinuria, also known as postural proteinuria, is a condition where an abnormally large amount of protein is excreted in the urine when the patient is in an upright position and normal protein excretion in the supine position. |
| d. | (i) Collection duration 24-hour without missing even a single void of urine; (ii) Empty bladder at start of collection; (iii) Empty bladder at the finish and add to the collected sample |
| e. | Fever, exercise, dehydration, seizures, and congestive heart failure (CHF). |
THG21-008
Match the following in Table A and B
| Table A | Table B (urine crystal) |
|---|---|
| Acyclovir | Diamond-shaped crystal |
| Cystinuria | Dumbbell-shaped crystal |
| Tumor lysis syndrome | Triple phosphates |
| Primary hyperoxaluria | Needle-shaped crystal |
| Recurrent UTI | Hexagonal-shaped crystal |
Answer
| Acyclovir | Needle-shaped crystal |
| Cystinuria | Hexagonal-shaped crystal |
| Tumor lysis syndrome | Diamond-shaped crystal |
| Primary hyperoxaluria | Dumbbell-shaped crystal |
| Recurrent UTI | Triple phosphates |
THG21-009
A 7-year-old male child presented with acute onset of gross painless cola colored urine, edema, and oliguria. He had skin lesions over the legs a week prior to the illness. On examination, his blood pressure (BP) was 120/90 mm Hg. He also had facial puffiness, ascites, and pedal edema.
a. What is the probable diagnosis?
b. What are the complications of the above condition?
c. When will you do renal biopsy in this child?
d. What is the best single antibody titer to document cutaneous streptococcal infection?
e. List any two other causes of hypocomplementemia.
f. What is the prognosis of this condition?
Answer
| a. | The probable diagnosis is postinfectious glomerulonephritis |
| b. | Hypertension (60%)/hypertensive encephalopathy (10%)/uremia/congestive cardiac heart failure/dyselectrolytemia (hyperkalemia and metabolic acidosis)/hyperphosphatemia, and hypocalcemia. |
c. • Nephritic nephrotic presentation
| • | Rapidly progressing renal failure |
| • | Normal complement levels at onset |
| • | Low C3 levels persisting for >2 months |
| • | Systemic features suggestive of secondary glomerulonephritis |
| d. | Anti-deoxyribonuclease B level |
| e. | Postinfectious glomerulonephritis, systemic lupus erythematosus, c3 glomerulopathy/membranoproliferative glomerulonephritis |
| f. | It carries very good prognosis (95% recover from the illness) |
THG21-010
A 2-year-old boy was brought to outpatient department (OPD) with history of progressive abdominal distension for the past few months noticed by his mother while bathing him. No other significant history present except for oligohydramnios during antenatal period and neonatal intensive care unit (NICU) stay for respiratory distress. His physical examination revealed height and weight between 3rd to 25th centile, blood pressure in the 95th centile, hepatomegaly, and bilateral ballotable kidneys. Ultrasonography (USG) of abdomen revealed bright echogenic kidneys and liver.
a. What is your diagnosis?
b. What is the gene involved in this condition?
c. What is Caroli’s disease?
d. What liver complication will you expect in this child?
e. List three cystic kidney diseases.
f. List three extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD).
g. What is the most common cause of an abdominal mass in a newborn?
h. Name two associated physical findings to suspect renal disease in a child.
Answer
| a. | Autosomal recessive polycystic kidney disease (ARPKD) |
| b. | The polycystic kidney and hepatic disease 1 (PKHD1) gene |
| c. | Cystic dilatation of the intrahepatic bile ducts (Caroli’s disease) |
| d. | Portal hypertension with hepatosplenomegaly, gastroesophageal varices, ascending cholangitis, reversal of portal vein flow, and thrombocytopenia |
| e. | Polycystic kidney diseases (ARPKD and ADPKD), multicystic dysplastic kidney, nephronophthisis, simple renal cyst, glomerulocystic kidney disease, tuberous sclerosis, and von Hippel–Lindau disease |
| f. | Cysts in other organs, such as liver, pancreas, spleen, and ovaries/intracranial aneurysms/mitral valve prolapse/aortic and coronary artery aneurysms, aortic valve insufficiency, and hernia/bronchiectasis, intestinal diverticula |
| g. | Multicystic dysplastic kidney |
h. i. Single umbilical artery
| ii. | Abnormality of another organ system (congenital heart disease) |
| iii. | External ear anomalies |
| iv. | Imperforate anus |
| v. | Vertebral anomalies (scoliosis) are associated with renal anomalies. |
| vi. | Infants with these physical findings should undergo renal ultra- sound. |
THG21-011
A 3-year-old female child presented with faltering of growth, retarded motor milestones, and difficulty in getting up from sitting posture with bony deformities. Child weighed 7 kg, height was 78 cm, and had features of clinical rickets. Her laboratory values were:
a. What is your diagnosis? What is the classical metabolic abnormality?
b. How will you calculate urine anion gap? What is the significance?
c. What are the other common metabolic defects that cause nephro- calcinosis?
d. What is Fanconi syndrome?
e. Write two causes of hyperkalemic renal tubular acidosis (RTA).
f. What is the line of management for the above child in the scenario?
g. What is Lowe syndrome?
h. Write two metabolic causes of Fanconi syndrome.
Answer
| a. | Distal RTA/normal anion gap; hyperchloremic hypokalemic metabolic acidosis |
| b. | Urine anion gap: (Urine Na+ + Urine K+) – Urine Cl–; A positive gap suggests a deficiency of ammonia genesis and the possibility of distal RTA |
| c. | Hypercalciuria, hypocitraturia, furosemide intoxication, and vitamin D intoxication |
| d. | Global proximal tubular dysfunction causes Fanconi syndrome. It is characterized by low molecular weight proteinuria, glycosuria, phosphaturia, and aminoaciduria. |
| e. | Obstructive uropathy (posterior urethral valve, ureteropelvic junction obstruction); Acute pyelonephritis/hypoaldosteronism, pseudohypo- aldosteronism |
| f. | Alkali therapy (bicarbonate) 2–4 mEq/kg/day |
| g. | Oculocerebrorenal syndrome characterized by congenital cataracts, mental retardation, and Fanconi syndrome |
| h. | Galactosemia/hereditary fructose intolerance/tyrosinemia/Wilson disease |
THG21-012
A 5-day-old newborn boy with history of oligohydramnios in the antenatal period underwent MCU.
a. Describe the MCU?
b. What antenatal intervention would have helped in this child?
c. What is the cause of respiratory distress in newborn with this condition?
d. What is the surgical management of this condition?
e. What is the triad of Prune belly syndrome?
f. What are the favorable prognostic factors of this condition?
g. What are the unfavorable prognostic factors of this condition?
h. What is VURD syndrome?
i. What is Hinman syndrome?
Answer
| a. | MCU shows narrow anterior urethral stream, dilated posterior urethra, and possible trabeculated bladder with diverticulae. |
| b. | Vesicoamniotic shunt (bladder decompression) |
| c. | Pulmonary hypoplasia |
| d. | Cystoscopic transurethral ablation of valve leaflets |
| e. | Deficient abdominal musculature, undescended testes, and urinary tract abnormalities |
| f. | Normal prenatal ultrasonogram between 18 and 24 weeks of gestation |
| • | Serum creatinine level <0.8–1.0 mg/dL after valve fulguration |
| • | Preserved corticomedullary differentiation on USG |
| g. | Oligohydramnios in antenatal scans |
| • | Identification of hydronephrosis before 24 weeks of gestation |
| • | Serum creatinine >1 mg/dL after valve fulguration |
| • | Cortical cysts in both kidneys |
| • | Persistence of diurnal incontinence beyond 5 years of age |
| h. | VURD syndrome—valves, unilateral reflux and dysplasia |
| i. | Failure of the external sphincter to relax during voiding in children without any neurologic abnormalities |
THG21-013
A 4-year-old boy presented with periorbital puffiness, abdominal distension, leg swelling, and reduced urine output of 1 week duration. Examination was significant for anasarca and urine dipstick showed 4+ proteinuria.
a. What is your diagnosis?
b. What are the drugs indicated in case of steroid resistance?
c. Which organism is most common to cause peritonitis in this child?
d. What are the indications for renal biopsy in this child?
e. What are the types of proteinuria?
f. What are the causes of transient proteinuria?
Answer
| a. | The diagnosis is nephrotic syndrome. |
| b. | Calcineurin inhibitors (tacrolimus and cyclosporine)/anti-CD20 monoclonal antibody (rituximab) |
| c. | Streptococcal pneumonia (pneumococcus) |
| d. | Age of onset <1 year and >10 years/AKI not attributed to hypovolemia/persistent microscopic or gross hematuria/persistent microscopic or gross hematuria/systemic features, such as fever, rash, arthralgia, low C3/initial or late steroid-resistant nephrotic syndrome/prior to starting calcineurin inhibitors, or reduced kidney function during their use |
| e. | Glomerular, tubular, orthostatic, and overflow proteinuria |
| f. | Fever, exercise, dehydration, cold exposure, seizures, stress, and CCF |
THG21-014
A 2-year-old boy presented with failure to thrive. He had genu valgum and short stature. His investigations revealed elevated parathyroid hormone and phosphorus levels.
a. What is your diagnosis?
b. Write the X-ray findings of rickets.
c. When will you suspect nonnutritional rickets?
d. What is burosumab? In which condition is it used?
e. What is the treatment of vitamin D dependent rickets?
Answer
| a. | Rickets due to CKD or mineral bone disease (clue: hyperphosphatemia) |
| b. | Loss of provisional zone of calcification; Long bones reveal widening and irregularity of all the physes; Fraying and broadening of the metaphyses; Bones are demineralized |
| c. | Presentation before 6 months or after 2 years of age; failure to respond to vitamin D therapy; obvious clues in history, clinical examination, and initial investigations suggestive of renal/hepatic and oncogenic rickets |
| d. | Burosumab is a human anti-FGF23 monoclonal antibody, used in hypophosphatemic rickets. |
| e. | Active vitamin D or calcitriol or 1,25-dihydroxyvitamin D |
THG21-015
A 6-year-old girl child presented with dysuria, hematuria, urgency, and colicky abdominal pain for 2 days. Ultrasound abdomen revealed a stone at the mid ureter. Her urine microscopy showed the below image.
a. Describe the finding.
b. What urinary investigations are done to confirm the diagnosis?
c. What is the chemical reaction used in dipstick to find hematuria in a urine sample?
d. Define microscopic hematuria.
e. Name two types of hereditary nephritis with hematuria.
Answer
| a. | Coffin-lid appearance—triple phosphate crystals |
| b. | 24 hours urine for crystalluria evaluation—calcium, oxalate, uric acid, cysteine, and citrate |
| c. | Peroxidase chemical reaction: In the clinical setting, qualitative estimates are provided by a urinary dipstick that uses a very sensitive peroxidase chemical reaction between hemoglobin (or myoglobin) and a colorimetric chemical indicator impregnated on the dipstick. The dipstick uses the peroxidase-like activity of hemoglobin (in the red cells or free) to convert a benzidine dye into a chromogen. The amount of color change in the dye is proportional to the amounts of red cells present on the dipstick pad. |
| d. | Presence of at least 5 RBCs per mL of urine in a centrifuged urine sample |
| e. | Alport syndrome and thin basement membrane disease |
THG21-016
Parents brought a 9-year-old boy with primary enuresis. As a pediatrician how do you counsel the parent about the diagnosis and management.
Answer
Make eye contact, greet the parents, check the language they are comfortable speaking, and their understanding of the child’s problem
Bedwetting diagnosis overview: Discrete episodes of urinary incontinence during sleep in children ≥5 years of age. Sixteen percent of children are incontinent at 5 years of age and 1–2% at ≥15 years of age.
General measures and motivational therapy:
| ■ | Restrict fluid before bedtime |
| ■ | Empty bladder before going to bed |
| ■ | Encourage the child to make bedtime resolution |
| ■ | Keep a bladder diary to mark dry and wet nights |
| ■ | Reward for dry nights |
| ■ | Avoid punishment/criticism |
| ■ | Battery operated alarm therapy |
| ■ | Medication—desmopressin/oxybutynin |
| ■ | Check their understanding/queries/greet |
THG21-017
A 12-year-old girl came with history of fever for a month, weight loss, anorexia, arthralgia, arthritis, and rash over the malar area of face. She presented with hematuria and mild renal insufficiency. Diagnosis of systemic lupus erythematosus (SLE) nephritis was entertained.
a. International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification of lupus nephritis (LN).
b. Renal involvement in SLE, American College of Rheumatology (ACR) criteria and immunological criteria (mention any two).
c. What is the serological marker for drug-induced lupus?
d. Mention three drugs which can cause the same?
Answer
| a. | Class I: Minimal mesangial LN |
| Class II: Mesangial proliferative LN | |
| Class III: Focal LN (<50% of glomeruli) | |
| Class IV: Diffuse LN (≥50% of glomeruli) | |
| Class V: Membranous LN | |
| Class VI: Advanced sclerotic LN (90% of glomeruli globally sclerosed without residual activity) | |
| b. | Renal involvement: |
| i. | Proteinuria: >500 mg/24 hour urine |
| ii. | Active urine sediment (>5 RBC, >5WBC, and cellular casts) |
| iii. | Class II-V in renal biopsy |
Immunological criteria:
| • | Low C3 and C4 |
| • | Positive antiphospholipid antibodies (ALPAs) (anti-cardiolipin antibodies, positive anti-β2GP1 antibodies, and lupus anticoagulant) |
| • | Anti-dsDNA antibodies or anti-Smith (Sm) antibodies |
| c. | Antihistone antibodies |
| d. | Hydralazine, penicillamine, isoniazid, methyldopa, chlorpromazine, phenytoin ethosuximide, quinidine, antithyroid drugs, sulfonamide, and rifampicin |
THG21-018
A 1.5-year-old male child, firstborn of third-degree consanguineous marriage, had a birth weight of 2.4 kg (present weight 6.5 kg) has failure to thrive, polyuria, polydipsia, and history of recurrent infections treated by local practitioner. Laboratory test showed Hb— 8.5 g/dL, blood urea nitrogen (BUN)—24, serum creatinine—0.4, serum Na—112, serum K—2.8, serum Cl—94, and serum HCO3—28, serum Mg—1.8. His blood pressure is normal. USG KUB is normal.
a. What is your probable diagnosis?
b. What is the channel involved in it?
c. What are the additional investigations you will do?
d. Mention appropriate management for the condition.
e. What is the long-term renal complication?
Answer
| a. | Classical Bartter syndrome—type 3 |
| b. | ClC-Kb channel |
| c. | Hearing assessment and urine calcium creatinine ratio |
| d. | Potassium supplements preferable potassium chloride, indomethacin 1–4 mg/kg/day (in case dyselectrolytemia not correctable by potassium supplements) |
| e. | Rarely patients develop CKD |
THG21-019
A 13-year-old girl referred for evaluation of dilated cardiomyopathy with features of congestive cardiac failure (CCF). Her BP is 180/120 mm Hg in right upper limb. Physical examination findings were significant with weak carotids, brachial and radial pulses, and bilateral carotid bruits. Renal USG with Doppler was suggestive of bilateral renal artery stenosis. Serum creatinine—0.35 mg/dL, urinalysis—normal, erythrocyte sedimentation rate (ESR)—80 mm/hour, and C-reactive protein (CRP)—27 mg/L.
a. How will you choose the appropriate cuff size for BP measurement?
b. What is the stage of hypertension in this child?
c. Classify hypertension in children between 1 to 12 years.
d. What is the likely diagnosis in this child?
e. Which group of antihypertensives should be avoided in bilateral renal artery stenosis?
Answer
| a. | Appropriate cuff size—the width of the cuff bladder should be 40% of the arm circumference midway between olecranon and acromion and its length 80–100% of the arm circumference. |
| b. | Stage 2 (stage 2 HTN >140/90 mm Hg) |
| c. | AAP definitions of BP categories and stages |
| Children aged 1–12 years | | |
| | | |
| • | Normal BP: <90th percentile | |
| • | Elevated BP: ≥90th percentile to <95th percentile or 120/80 mm Hg to <95th percentile (whichever is lower) | |
| • | Stage 1 HTN: 95th percentile to <95th percentile + 12 mm Hg or 130/80–139/89 mm Hg (whichever is lower) | |
| • | Stage 2 HTN: >95th percentile + 12 mm Hg or ≥140/90 mm Hg (whichever is lower) | |
| | | |
| d. | Takayasu arteritis |
| e. | Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) |
THG21-020
A 15-day-old girl baby born by normal delivery to second-degree consanguineous parents, borderline preterm (35 weeks), birth weight of 2.5 kg, antenatal period—uneventful, admitted for edema and decreased urine output since birth. No history suggestive of renal disease in family. General examination revealed sacral and vulval edema, vitals stable, massive ascites, anterior fontanel (AF)—wide open; posterior fontanel (PF)—open; and widened cranial sutures. No syndromic features were present. TORCH [toxoplasmosis, rubella cytomegalovirus (CMV), herpes simplex, and HIV] screening was negative, and USG abdomen revealed normal-sized kidney, increased echotexture, and free fluid abdomen.
a. What is the most probable diagnosis and how do you define it?
b. What are the common genes involved in this condition?
c. Mention at least two secondary causes of this condition.
d. What is the method of choice for precise diagnosis of the above condition?
e. What is the management of this condition?
Answer
| a. | Most probable diagnosis is congenital nephrotic syndrome which is defined as onset of nephrotic syndrome in <3 months of age. |
| b. | Common genes involved in central nervous system (CNS): |
| i. | Mutation in nephrin gene (NPHS1) |
| ii. | Podocin gene (NPHS2) |
| iii. | Wilms’ tumor factor 1 gene (WT1) |
| iv. | Laminin B2 gene (LAMB2) |
| v. | Phospholipase C epsilonv1 (PLCE1) gene mutation (NPHS3) |
| c. | Congenital syphilis, toxoplasmosis, malaria, CMV, rubella, hepatitis B, HIV, and maternal SLE |
| d. | Genetic analysis: As it helps in assessing the management and prognosis. |
| e. | Management of CNS: |
| • | Conservative management: Hypercaloric diet (130 kcal/kg/day) and proteins 4 g/kg/day along with other supplements, such as thyroxine, fat-soluble vitamins, IV albumin with diuretics, and ACE inhibitors to reduce proteinuria and medical nephrectomy. |
| • | Surgical management: Surgical nephrectomy and renal trans- plantation. |
THG21-021
A 3-year-old child had acute kidney injury (AKI), gross hematuria, and documented to have hypertension following bloody diarrhea.
a. What is the most probable diagnosis and its triad?
b. Classify the causes of this condition.
c. Where can you have a positive Coombs test in this condition?
d. What is the specific treatment for the typical and atypical varieties of this condition?
Answer
| a. | Diagnosis: Hemolytic uremic syndrome |
| Criteria: Microangiopathic hemolytic anemia [Hb <10 g/dL, hematocrit (HCT) <30%, schistocytes >2%, either elevated lactate dehydrogenase (LDH) >450 IU/L or undetectable haptoglobin), thrombocytopenia <150,000), and AKI (increase in serum creatinine 50% from baseline). | |
| b. | Classify the causes: |
| i. | Infection induced: Verotoxin-producing E. coli, Shiga toxin-producing Shigella dysenteriae type 1, Streptococcus pneumoniae, HIV |
| ii. | Mutations in CFH, CFI, CFB, autoantibodies to factor H |
| iii. | Cobalamine deficiency |
| iv. | Medication induced: Quinine and calcineurin inhibitor |
| v. | Secondary hemolytic uremic syndrome (HUS): SLE and APLA syndrome |
| c. | Pneumococcal-induced HUS |
| d. | Atypical HUS—plasma exchange, eculizumab, and hemodialysis if required; Typical HUS—supportive therapy (fluid management) |
THG21-022
A 6-year-old child who is a diagnosed case of focal segmental glomerulosclerosis FSGS, progressed to chronic kidney disease (CKD), now on intermittent hemodialysis for past 2 years. His height was 100 cm and weight was 20 kg, BP of 140/90 mm Hg. He had bony deformities. Investigations showed a serum creatinine of 6.8 mg/dL, serum K of 6.0 mEq/L, serum HCO3 of 8.0 mEq/L with serum Ca of 7.0 mg/dL and a serum PO4 of 8.2 mg/dL. His parathyroid hormone (PTH) was 321 pg/mL.
a. What is the stage of CKD and bone X-ray diagnosis?
b. Mention Kidney Disease Improving Global Outcomes (KDIGO) staging of CKD.
c. What are the steps in management?
d. What is the pathogenesis of this bony condition?
Answer
| a. | CKD stage 5 (G5) with renal osteodystrophy |
| b. | KDIGO staging of CKD: |
| • | G1: Normal GFR ≥90 mL/min/1.73 m2 |
| • | G2: GFR between 60 and 89 mL/min |
| • | G3a: GFR between 45 and 59 mL/min |
| • | G3b: GFR between 30 and 44 mL/min |
| • | G4: GFR between 15 and 29 mL/min |
| • | G5: GFR of <15 mL/min |
| c. | Calcium supplements, phosphate binders, active vitamin D3, inactive vitamin D3 as per need, bicarbonate supplementation, and renal replacement therapy |
| d. | Secondary hyperparathyroidism, metabolic acidosis inhibiting 1α-hydroxylase, reduced levels of 1α-hydroxylase, metabolic acidosis leaching bone to maintain blood buffers, growth hormone deficiency, overall malnutrition, defective diet, and uremic toxins |
THG21-023
An 8-year-old boy weighing 24 kg with the height of 130 cm is admitted following a road traffic accident with crush injury of the left upper limb. His renal function test shows a BUN of 168 mg/dL and serum creatinine of 8.3 mg/dL and needs hemodialysis.
a. Calculate the estimated glomerular filtration rate (eGFR) for this child by modified Schwartz formula.
b. What is the KDIGO classification of AKI?
c. What is the stage of AKI in this child?
d. Mention at least four indications for kidney replacement therapy (KRT) in AKI.
e. List two urinary indices which are useful to differentiate intrinsic renal from prerenal AKI
Answer
| a. |  |
 |
| 0 | KDIGO: AKI classification and criteria | ||
| b. | Stage | Serum creatinine | Urine output |
| 1 | 1.5- to 1.9-fold within 7 days or >0.3 mg/dL within 48 h | <0.5 mL/kg/h for 6-12 hours | |
| 2 | 2.0- to 2.9-fold | <0.5 mL/kg/h for >12 h | |
| 3 | >3 times increase from baseline or serum creatinine >4 mg/dL or initiation of RRT OR eGFR <35 mL/min/1.73 m2 | <0.3 mL/kg/h for >24 h or anuria >12 h |
| c. | Stage 3 (KDIGO: AKI classification) |
| d. | Indications of KRT in AKI: |
| i. | Volume overload with evidence of hypertension/pulmonary edema refractory to diuretic therapy |
| ii. | Persistent hyperkalemia |
| iii. | Severe metabolic acidosis unresponsive to medical management |
| iv. | Uremia (encephalopathy, pericarditis) |
| v. | Calcium: phosphorus imbalance |
| e. | 0 | Prerenal AKI | Renal AKI |
|---|---|---|---|
| Urine analysis | Hyaline casts | Abnormal | |
| Specific gravity | >1.020 | <1.010 | |
| U Osm | >500 | <300 | |
| UNa | <20 | >40 | |
| FeNa | <1% | >2% | |
| FeUr | <0.35 | >0.35 |
THG21-024
A 2.5-month-old child with a weight of 3.8 kg and coexisting transposition of great arteries (TGA) with a baseline urea and creatinine of 35/0.4 mg/dL, underwent cardiopulmonary bypass for 2 hours. Creatinine postoperative 0.8 mg/dL. Urine output is 0.3 mL/kg/hour. Child is on inotropic support (adrenaline, dopamine, and noradrenaline). Initially child was given IV Lasix 0.1 mg/kg/hour. But urine output failed to improve. Below are mentioned laboratory parameters (creatinine and urea on increasing trend) and child was started on peritoneal dialysis (PD).
a. Explain the principles of PD?
b. How to investigate a child with suspected peritonitis?
c. What is the glucose concentration of acute PD?
d. What are the absolute contraindications for PD (mention any four)?
Answer
| a. | Principles of PD: |
| • | Diffusion |
| • | Convection/ultrafiltration |
| • | Absorption |
| b. | (i) Dialysis effluent >100 white cell count/hpf, with >50% polymorphonuclear cells and (ii) positive dialysis effluent culture |
| c. | 1.7% glucose concentration |
| d. | Absolute contraindications for PD: |
| i. | Omphalocele |
| ii. | Gastroschisis |
| iii. | Bladder exstrophy |
| iv. | Diaphragmatic hernia |
| v. | Obliterated peritoneal cavity and peritoneal membrane failure |
THG21-025
A 4-year-old male child has undergone MCU.
a. Describe the findings in this MCU?
b. What is Hinman syndrome?
c. What step done on daily basis with sterile precautions will prevent or retard the progression of CKD in this child?
d. Is there any need for chemoprophylaxis?
Answer
| a. | Christmas tree appearance/fir tree appearance seen in neurogenic bladder |
| b. | Hinman syndrome: A form of nonneurogenic neurogenic bladder, characterized by functional bladder obstruction in absence of anatomical or neurological deficits |
| c. | Clean intermittent catheterization |
| d. | On routine basis chemoprophylaxis is not required. |
THG21-026
A 13-day-old term baby girl with antenatally diagnosed renal anomaly was now brought for follow-up. She had stable vitals. Other systems are normal. Evaluation showed serum urea/creatinine—20/0.4, serum Na—138, serum K—4.8, serum HCO3—22, and urinary protein-creatinine ratio (UPCR)—0.5.
a. What is the possible diagnosis in this antenatally detected cystic kidney disease which involves only one kidney?
b. Mention any three complications of the above mentioned condition.
c. What is the common association in contralateral kidney?
d. Mention at least two syndromes associated with cystic kidney disease.
Answer
| a. | Multicystic dysplastic kidney |
| b. | Complications of MCDK: |
| i. | VUR |
| ii. | UTI |
| iii. | Hypertension |
| c. | Wilms tumor; Chance of VUR in contralateral kidney (up to 30%) |
| d. | Tuberous sclerosis, Bardet–Biedl, Beckwith–Wiedemann, Meckel–Gruber syndrome, Joubert syndrome, and Zellweger syndrome |
THG21-027
An 8-year-old male child presented with complaints of lower abdomen pain for 5 days. On evaluation, urine routine showed urine albumin—nil, RBCs—10–15/hpf, pus cells—1–2/hpf, and spot calcium/creatinine—0.8.
a. What is age-specific definition of hypercalciuria in spot urine sample?
b. What is the normal 24 hours urine calcium value?
c. What is the drug of choice for hypercalciuria?
d. What is the type of RTA associated with nephrocalcinosis?
Answer
| a. | Age-specific spot urine Ca/Cr ratio: |
| • | <6 months <0.8 |
| • | 6 months to 2 years <0.5 |
| • | >2 years <0.2 |
| b. | <4 mg/kg/day in a 24-hour urine sample |
| c. | Hydrochlorothiazide (to increase the distal tubular reabsorption of calcium) along with alkali therapy may be needed. |
| d. | Distal RTA |
THG21-028
Q21.28
a. What procedure does the figure depict and define the same?
b. Mention at least two indications of this procedure.
c. What is white-coat hypertension?
d. What percentage of total BP readings measured by this device above the threshold is suggestive of hypertension?
Answer
| a. | Ambulatory blood pressure monitoring (ABPM) is a diagnostic test to determine the presence of hypertension over a span of 24 hours during normal daily activities. |
| b. | Indications of ABPM: |
| i. | Stage 1 hypertension over three clinical visits |
| ii. | High-risk children (CKD, coarctation of aorta/diabetes mellitus, obesity, and obstructive sleep apnea) |
| iii. | Masked hypertension and white-coat hypertension |
| iv. | Adjusting drug doses in CKD |
| c. | White-coat hypertension is defined as BP >95th percentile in the office or clinical setting but <95th percentile outside of the office setting |
| d. | >25% |
THG21-029
A 6-year-old female child presented with complaints of fever, joint pain in both legs, diffuse abdominal pain, and rashes noted in both lower limbs since 5 days. She also complaints of passing cola colored urine for past 2 days.
a. What is your diagnosis?
b. What are the diagnostic criteria?
c. What is the finding expected in skin biopsy?
d. What are the expected renal manifestations in this condition (mention any four)?
Answer
| a. | Henoch–Schönlein purpura with nephritis |
| b. | Palpable purpura with any one of the following: |
| i. | Arthritis or arthralgia |
| ii. | Diffuse abdominal pain |
| iii. | Renal involvement (hematuria and/or proteinuria) |
| iv. | Leukocytoclastic vasculitis with predominant IgA deposit or proliferative glomerulonephritis with predominant IgA deposits |
| c. | Skin biopsy suggestive of leukocytoclastic vasculitis with predominant IgA deposits |
| d. | Gross/microscopic hematuria: |
| i. | Subnephrotic range proteinuria |
| ii. | Nephrotic syndrome |
| iii. | Nephritic syndrome |
| iv. | Decreased eGFR |
| v. | Nephrotic nephritic presentation |
THG21-030
An 8-year-old male child presented with pain abdomen. On taking a detailed history, the parents revealed that the mother was told to have RBCs in urine and his maternal uncle aged 48 years has deafness and is on dialysis. On examination, his BP is 120/90 mmHg. On evaluation, his urine routine showed albumin 2+, RBC—15–20/hpf.
a. What is the most likely diagnosis in this child?
b. What other tests would you do in this child to support the diagnosis?
c. How will you confirm the diagnosis?
d. What is the likely mode of inheritance in this child?
e. What is the characteristic electron microscopy findings in renal biopsy?
Answer
| a. | Alport syndrome |
| b. | Audiological evaluation to look for high frequency sensorineural hearing loss, ophthalmological findings—anterior lenticonus and macular flecks |
| c. | Genetic analysis |
| d. | X-linked recessive pattern |
| e. | Basketweave appearance (thickening and thinning of glomerular basement membrane) |