Pediatric Nephrology

1. A 8-year-old female presents to the emergency department with a seizure. On initial evaluation, the patient appears to be post-ictal, but is otherwise okay. Initial vital signs are significant for blood pressure of 180/110. She has never had a seizure before. On further questioning, the patient s parents report that she had several episodes of coca-cola colored urine a few days ago, and was seen by her doctor and given an antibiotic for presumed urinary tract infection. Over the last few days prior to her presentation, she has been complaining of headaches and ankle swelling. Otherwise the patient has been fine. She has never been hospitalized, takes no medications, and she has no known allergies. She is in 3rd grade, and plays soccer in a local league. She is very seldom ill, and with the exception of a sore throat 2 weeks ago, she has had no other recent illnesses. CT of the head is normal. What is the most likely diagnosis?

Alport's syndrome

Post-infectious glomerulonephritis

Goodpasture's syndrome

IgA nephropathy

Focal segmental glomerulonephritis

The most likely diagnosis in this case is post-infectious glomerulonephritis. The patient's history of recent episodes of coca-cola colored urine, headaches, and ankle swelling, along with a recent urinary tract infection and normal CT scan, are consistent with the presentation of post-infectious glomerulonephritis. This condition is characterized by inflammation of the glomeruli in the kidneys following an infection, often caused by streptococcal bacteria. It can present with symptoms such as hematuria, hypertension, and edema.

Explanation

The most likely diagnosis in this case is post-infectious glomerulonephritis. The patient's history of recent episodes of coca-cola colored urine, headaches, and ankle swelling, along with a recent urinary tract infection and normal CT scan, are consistent with the presentation of post-infectious glomerulonephritis. This condition is characterized by inflammation of the glomeruli in the kidneys following an infection, often caused by streptococcal bacteria. It can present with symptoms such as hematuria, hypertension, and edema.

2. What would be the most likely urinalysis and laboratory findings in the patient in the previous question?

Red blood cell casts, 2+ protein, pyruria, and RBCs too numerous to count

Numerous maltese crosses

4+ protein, 1+ ketones, 0-5 RBC/HPF, 0-5 WBC/HPF

5 RBC/HPF and WBC casts

Greater than 50 RBC/HPF, 20 WBC/HPF and many bacteria

The most likely urinalysis and laboratory findings in the patient would include red blood cell casts, 2+ protein, pyuria, and RBCs too numerous to count. This combination of findings suggests a significant amount of blood in the urine, as indicated by the presence of red blood cell casts and numerous RBCs. The presence of protein and pyuria also indicates inflammation or infection in the urinary tract. The high protein level (2+) suggests kidney damage or dysfunction. Overall, these findings are consistent with a severe renal condition.

Explanation

The most likely urinalysis and laboratory findings in the patient would include red blood cell casts, 2+ protein, pyuria, and RBCs too numerous to count. This combination of findings suggests a significant amount of blood in the urine, as indicated by the presence of red blood cell casts and numerous RBCs. The presence of protein and pyuria also indicates inflammation or infection in the urinary tract. The high protein level (2+) suggests kidney damage or dysfunction. Overall, these findings are consistent with a severe renal condition.

3. A mother brings her six-month-old son to see you in the emergency department in January. The child had an uneventful prenatal course, and growth and development have been normal to this point. The child is primarily breast fed, but his mother did begin to introduce solids at 4 months of age. The child now presents with emesis and diarrhea of two days duration. She has noted no blood in his stools. In addition, the patient has had several episodes of emesis, and does not seem to have tolerated any oral intake. You inquire about urine output, and the patient s mother reports one wet diaper yesterday and none today. She denies any fevers. The patient does attend day care, and according to his mother, there have been several other children there with a stomach flu. On physical examination, heart rate is 165, and the remainder of the vital signs are within normal limits. Weight is 6.3 kg. The patient weighed 7 kg at his pediatrician s office earlier this week. The patient is crying but consolable with the examination. He appears ill and listless. Eyes appear sunken and lips are dry. The patient is not producing tears with his crying. Anterior fontanel is sunken. The patient s lungs are clear, and auscultation of the heart reveals no murmurs. Capillary refill is 3-4 seconds, but skin turgor appears normal. Examination of the rectal area reveals redness and excoriation. Given the patient s history, you suspect gastroenteritis. You give the pts mother a bottle of oral rehydration solution and instruct her to give the child a teaspoon every 5 minutes. While explaining the instructions to his mother, the child drinks several ounces from his bottle and promptly vomits on you. Of the following, the next best step in this patient s management is:

Allow the mother to try infant formula instead of oral rehydration solution before considering intravenous (IV) fluid replacement

Give the patient 50 mg/kg of ceftriaxone (Rocephin) IM x 1 after drawing blood cultures

Send stool for routine stool cultures, O&P, fecal leukocytes, and rotavirus

Place a peripheral IV and give 140 ml of 1/2 normal saline (saline 0.45 %) as a bolus

Place a peripheral IV and give 140 ml of normal saline (saline 0.9 %) as a bolus

The next best step in this patient's management is to place a peripheral IV and give 140 ml of normal saline (saline 0.9%) as a bolus. This patient is presenting with signs of dehydration, including decreased urine output, sunken fontanel, dry lips, and poor skin turgor. These findings indicate a need for immediate fluid resuscitation. The recommended initial fluid bolus for pediatric patients is 20 ml/kg, and in this case, the patient's weight is 6.3 kg, so 140 ml of normal saline is appropriate. The other options, such as trying infant formula or giving antibiotics, are not indicated at this time. Stool cultures and other investigations may be considered later if the patient's condition does not improve.

Explanation

The next best step in this patient's management is to place a peripheral IV and give 140 ml of normal saline (saline 0.9%) as a bolus. This patient is presenting with signs of dehydration, including decreased urine output, sunken fontanel, dry lips, and poor skin turgor. These findings indicate a need for immediate fluid resuscitation. The recommended initial fluid bolus for pediatric patients is 20 ml/kg, and in this case, the patient's weight is 6.3 kg, so 140 ml of normal saline is appropriate. The other options, such as trying infant formula or giving antibiotics, are not indicated at this time. Stool cultures and other investigations may be considered later if the patient's condition does not improve.

4. A previously healthy 4-month-old male infant presents to the emergency room with decreased PO intake, increased fussiness, and fever. His parents state that he has not been taking feeds well for the past two days and has a decreased number of wet diapers daily. He has had no vomiting. Fever at home was 101.4 rectally this morning. He has become increasingly more irritable and seems to cry each time he urinates. The parents have not noticed any blood in the urine or on the diaper. Physical examination reveals heart rate of 155, BP 90/50, sunken anterior fontanel and sticky oral mucous membranes. Blood cultures and a catheterized urine specimen are obtained for culture and urinalysis. Urinalysis reveals presence of leukocyte esterase and nitrites. Microscopic urine evaluation shows greater than 50 WBC/HPF. You diagnose urinary track infection (UTI) with dehydration and admit the patient for intravenous fluid and antibiotic therapy. After initiation of therapy, the patient s clinical picture rapidly improves, and urine culture shows greater than 100,000 CFU of Escherichia coli. After initiating appropriate antibiotic therapy, the MOST appropriate next step in the the management of this patient is:

IVP (intravenous pyelogram) to evaluate for urinary tract obstruction along with initiation of IVantibiotic therapy

Reassure the patient's parents that this is unlikely to occur again; observe without furthersurveillance testing

Reassure the patient's parents and observe with repeat urine cultures monthly as survelliancefor a repeat UTI

Renal ultrasound with initiation of IV antibiotic therapy; prophylactic antibiotics until a VCUG(voiding cystourethrogram) is obtained

Urology referral for cystoscopy to evaluate for urinary tract obstruction along with initiation of IVantibiotic therapy

The correct answer is renal ultrasound with initiation of IV antibiotic therapy; prophylactic antibiotics until a VCUG (voiding cystourethrogram) is obtained. This is the most appropriate next step in the management of this patient because the presence of leukocyte esterase, nitrites, and greater than 50 WBC/HPF in the urine indicates a urinary tract infection (UTI). The patient's symptoms, such as decreased PO intake, increased fussiness, and fever, along with physical examination findings of sunken anterior fontanel and sticky oral mucous membranes, suggest dehydration. The rapid improvement in the patient's clinical picture after initiation of antibiotic therapy confirms the diagnosis of UTI. Renal ultrasound is necessary to evaluate for urinary tract obstruction, and a VCUG is needed to further assess the urinary system. Prophylactic antibiotics should be given until the VCUG is obtained to prevent recurrent UTIs.

Explanation

The correct answer is renal ultrasound with initiation of IV antibiotic therapy; prophylactic antibiotics until a VCUG (voiding cystourethrogram) is obtained. This is the most appropriate next step in the management of this patient because the presence of leukocyte esterase, nitrites, and greater than 50 WBC/HPF in the urine indicates a urinary tract infection (UTI). The patient's symptoms, such as decreased PO intake, increased fussiness, and fever, along with physical examination findings of sunken anterior fontanel and sticky oral mucous membranes, suggest dehydration. The rapid improvement in the patient's clinical picture after initiation of antibiotic therapy confirms the diagnosis of UTI. Renal ultrasound is necessary to evaluate for urinary tract obstruction, and a VCUG is needed to further assess the urinary system. Prophylactic antibiotics should be given until the VCUG is obtained to prevent recurrent UTIs.

5. After consultation with a pediatric nephrologist, what would be the most appropriate combination of medications for the treatment of the patient in the previous question?

ACE inhibitor, furosemide and prednisone

Prednisone, ACE inhibitor, albumin

Prednisone, furosemide, albumin

Furosemide, spironolactone, digoxin

Furosemide, ACE inhibitor, IV immunoglobulin

The most appropriate combination of medications for the treatment of the patient in the previous question would be prednisone, furosemide, and albumin. Prednisone is a corticosteroid that helps reduce inflammation and suppress the immune system. Furosemide is a diuretic that helps remove excess fluid from the body. Albumin is a protein that helps maintain blood volume and prevent fluid from leaking out of blood vessels. This combination of medications would help manage the patient's condition and address the underlying issues causing their symptoms.

Explanation

The most appropriate combination of medications for the treatment of the patient in the previous question would be prednisone, furosemide, and albumin. Prednisone is a corticosteroid that helps reduce inflammation and suppress the immune system. Furosemide is a diuretic that helps remove excess fluid from the body. Albumin is a protein that helps maintain blood volume and prevent fluid from leaking out of blood vessels. This combination of medications would help manage the patient's condition and address the underlying issues causing their symptoms.

6. A 3-year-old boy is seen in the emergency room with edema and shortness of breath. He was previously healthy, but his mother has noted worsening edema over the past 2 weeks and over the past 2 days he appears increasingly dyspneic. Physical examination reveals mild tachypnea, periorbital edema, decreased lung sounds in the bilateral bases, and pitting edema of bilateral lower extremities. CXR reveals bilateral pleural effusions. BMP and CBC are normal. Urinalysis reveals 3+ protein. Serum albumin is markedly decreased with a value of 2 g/dl. A 24-hour urine collection reveals 3 grams of protein. Light microscopy of a kidney biopsy would most likely reveal:

Diffuse crescent formation

Diffuse thickening of the glomerular basement membrane

Mesangial cell proliferation

Normal findings

The given clinical presentation suggests nephrotic syndrome, which is characterized by edema, proteinuria, hypoalbuminemia, and hyperlipidemia. In this case, the decreased serum albumin and significant proteinuria indicate glomerular dysfunction. However, the normal findings in the BMP, CBC, and kidney biopsy suggest that the underlying cause is likely minimal change disease (MCD), the most common cause of nephrotic syndrome in children. MCD is characterized by normal findings on light microscopy, while electron microscopy may reveal effacement of podocyte foot processes. Therefore, the correct answer is "Normal findings."

Explanation

The given clinical presentation suggests nephrotic syndrome, which is characterized by edema, proteinuria, hypoalbuminemia, and hyperlipidemia. In this case, the decreased serum albumin and significant proteinuria indicate glomerular dysfunction. However, the normal findings in the BMP, CBC, and kidney biopsy suggest that the underlying cause is likely minimal change disease (MCD), the most common cause of nephrotic syndrome in children. MCD is characterized by normal findings on light microscopy, while electron microscopy may reveal effacement of podocyte foot processes. Therefore, the correct answer is "Normal findings."

7. A 15-year-old boy is seen in the ER for evaluation after fall from a tree. He fell a short distance and had no loss of consciousness. His parents brought him in for evaluation of a small laceration to his knee. During physical examination, his blood pressure is noted to be 148/90 using a large adult blood pressure cuff that completely encircles his right upper arm. His weight is 110 kilograms. His laceration is easily repaired and his physical examination is otherwise normal. He has no other physical complaints or concerns. You speak with the patient and his parents about his blood pressure. They relate that his blood pressure has been high on other occasions at the pediatrician's office. In fact, on the advice of the pediatrician, they have been checking his blood pressure at home and have recorded several readings in 150/90 range. Additionally, they report that since the last visit to the pediatrician two months ago, the boy's weight has increased by 2 kilograms. His height is currently at the 75 %ile for age. Calculation of the patient's body mass index (BMI) reveals a value of 32. Of the following, which of the following is the BEST initial course of action for this patient?

Admit the patient for blood pressure control and angiography to evaluate for possible renalartery stenosis

Caloric restricion and exercise program

Pharmacologic therapy for hypertension

Pharmacologic therapy for weight reduction

The patient is a 15-year-old boy who has a high blood pressure reading, elevated weight, and a high BMI. These findings suggest that the patient is overweight or obese, which is a common cause of hypertension in adolescents. The best initial course of action in this case would be to recommend a caloric restriction and exercise program to help the patient lose weight and improve his blood pressure. This approach is recommended as the first-line treatment for hypertension in overweight or obese individuals, and pharmacologic therapy is typically reserved for cases where lifestyle modifications are ineffective.

Explanation

The patient is a 15-year-old boy who has a high blood pressure reading, elevated weight, and a high BMI. These findings suggest that the patient is overweight or obese, which is a common cause of hypertension in adolescents. The best initial course of action in this case would be to recommend a caloric restriction and exercise program to help the patient lose weight and improve his blood pressure. This approach is recommended as the first-line treatment for hypertension in overweight or obese individuals, and pharmacologic therapy is typically reserved for cases where lifestyle modifications are ineffective.

8. A previously healthy 5-year-old child is brought urgently to the ED for evaluation. He has had bloody diarrhea for the past day. He has become increasingly listless over the past several hours and his parents are very concerned. He has no known medical problems. The patient has not traveled anywhere recently and owns no pets. Several days ago, his family ate out at a fish restaurant. The patient had a hamburger because he does not like fish. No one else in the family is ill. Physical examination reveals a very ill-appearing child. He is at least moderately dehydrated. Laboratory analysis is significant for hemoglobin of 7.2 g/dl, platelets of 30,000, BUN 80 and creatinine 2.3. Serum potassium level is 5.6. Peripheral blood smear is shown below.

Based on the MOST LIKELY diagnosis, WHICH of the following therapies would be contraindicated at this time?

Dialysis, if and when indicated, to maintain proper fluid and electrolyte balance

Parenteral (IV) antibiotic therapy upon hospital admission

Supportive care, including hydration and ongoing evaluation of fluid status

Telemetry monitoring of the patient's cardiac rhythm

Transfusion of packed red blood cells (PRBCs) for symptomatic anemia

The patient's presentation, with bloody diarrhea, listlessness, and laboratory findings of anemia, thrombocytopenia, and renal dysfunction, is consistent with hemolytic uremic syndrome (HUS). HUS is most commonly caused by infection with Shiga toxin-producing Escherichia coli (STEC), often acquired through contaminated food. Antibiotic therapy is contraindicated in HUS caused by STEC infection because it can increase the release of Shiga toxin and worsen the disease. Supportive care, including hydration, monitoring of fluid status, and transfusion of packed red blood cells for symptomatic anemia, is important in managing HUS. Dialysis may be indicated if there is severe renal dysfunction. Telemetry monitoring is not specifically contraindicated in this situation.

Explanation

The patient's presentation, with bloody diarrhea, listlessness, and laboratory findings of anemia, thrombocytopenia, and renal dysfunction, is consistent with hemolytic uremic syndrome (HUS). HUS is most commonly caused by infection with Shiga toxin-producing Escherichia coli (STEC), often acquired through contaminated food. Antibiotic therapy is contraindicated in HUS caused by STEC infection because it can increase the release of Shiga toxin and worsen the disease. Supportive care, including hydration, monitoring of fluid status, and transfusion of packed red blood cells for symptomatic anemia, is important in managing HUS. Dialysis may be indicated if there is severe renal dysfunction. Telemetry monitoring is not specifically contraindicated in this situation.