Nephrology

The parents of a healthy 13-year-old girl recently moved to your town and bring her to you for a physical examination prior to summer camp. They have no complaints and the girl denies any problems. Her last menses was normal 2 weeks prior. The camp requires a urine screen. To your surprise, the clean-catch urine screen is found to have significant hematuria. Red cell casts are noted on centrifuged urine. You discuss your findings with the parents, and they respond that “everyone on dad’s side of the family has blood in their urine and they are all doing well.” The family history is negative for deafness and for renal failure. Mi-croscopy of renal tissue from this patient or her father will likely reveal:
A. Thinning of the basement membrane.
B. Immune complex deposition in the mesangium.
C. Endothelial cell swelling and fibrin in the subendothelial space.
D. Large numbers of crescentic glomeruli.
E. Renal cell carcinoma.

A. This history is consistent with benign familial hematuria, an autosomal dominant condition that causes either persistent or intermittent hematuria without progression to chronic renal fail¬ure. Biopsy reveals a thin basement membrane; in some cases, however, the biopsy is normal. Immune complex deposition with IgA in the mesangium is seen in Henoch Schonlein pur¬pura and IgA nephropathy; endothelial cell swelling with fibrin deposition is seen in hemolytic-uremic syndrome, and crescen¬tic glomeruli are seen in rapidly progressive glomerulonephritis.

You are not surprised to see again one of your most challenging patients, a 16-year-old adolescent girl who has been in the of¬fice several times a week over the last 2 months complaining of cough, occasional hemoptysis, malaise, and intermittent low-grade fever. Thus far you have identified a microcytic hypochromic anemia for which she has been taking iron (without response) and migratory patchy infiltrates on chest radiograph that seem unaffected by antibiotic treatment. She has no tuberculosis exposure risks and her tuberculin skin test was negative. Today she also complains of facial edema and tea-colored urine. You suddenly realize her symptoms can be grouped as:
A. Alport syndrome.
B. Hemolytic-uremic syndrome.
C. Nephrotic syndrome.
D. Goodpasture syndrome.
E. Denys-Drash syndrome.

D. Good pasture syndrome is the clinical diagnosis when pa¬tients exhibit nephritis and pulmonary hemorrhage. It can be secondary to several conditions, including systemic lupus erythematosus and Henoch-Schonlein purpura. Alport syndrome is a genetic defect in collagen synthesis that leads to abnormal basement membrane formation; patients will develop hematuria, proteinuria, and renal failure. Denys-Drash syndrome is a group of findings composed of Wilms tumor, gonadal dysgenesis, and nephropathy.

A previously healthy 2-year-old girl had 3 days of bloody diar¬rhea last week that spontaneously resolved. She is now in your office because her mother thinks she looks pale. On physical examination you see that she is afebrile, her heart rate is 150 beats per minute, her systolic blood pressure is 150 mmHg and her diastolic is 80 mmHg. She is pale and irritable, has pitting edema of the lower extremities, and has scattered petechiae. Af¬ter appropriate laboratory studies, initial management of her condition should include:
A. Intravenous antibiotics and platelet transfusion
B. Careful management of fluid and electrolyte balance
C. Intravenous steroids and aggressive fluid resuscitation
D. Contrast upper gastrointestinal series with small bowel delay films
E. Intubation and mechanical ventilation

B. HUS is typically seen after an episode of bloody diarrhea, and presents with anemia, thrombocytopenia, and nephropathy The child in question is hypertensive and has edema, so the amounts of fluids may be counterproductive. Steroids are not typically helpful in HUS. The thrombocytopenia in HUS is consumptive; thus, unless the patient is actively bleeding platelets transfusion is not helpful. Most of the care for patients with ¬HUS is supportive, concentrating on fluids and electrolytes regulation. Early dialysis may be needed as well. Hypertensive patients should have appropriate control of their blood pressure.

An 8-month-old girl presents to the emergency department with fever and vomiting. Her serum white blood cell (WBC) count is elevated, and urinalysis has 100 WBC per high-power field; the urine is positive for nitrates and leukocyte esterase. After urine culture results confirm the diagnosis of urinary tract infection, renal sonography and VCUG reveal that the infant has mild hydronephrosis and grade I vesicoureteral reflux on the right side. Which of the following statements is true?
A. She will require surgical reimplantation of her right ureter.
B. After completion of her present antibiotic course, antimicrobial prophylactic therapy is warranted.
C. VCUG should be performed on a monthly basis.
D. Subsequent urine specimen must be obtained only by suprapubic aspiration.
E. Renal arteriography is indicated.

B. Infants and children with VUR receive prophylactic antimi¬crobial therapy and close monitoring for infection, with urinal¬ysis and urine culture at 3- to 4-month intervals. VUR is graded from I to V based on the degree of reflux. Higher-grade reflux is less likely to resolve spontaneously and is more likely to re¬sult in renal damage

A 16-year-old boy comes to your office complaining of inter-mittent cola-colored urine of several years, usually when he has a “cold.” He is otherwise well and has no other complaints. When the dark-colored urine is present, he does not have dys¬uria. None of his family members has similar complaints, nor is the family history positive for renal disease. On physical ex¬amination he is normotensive and has no obvious abnormalities. The most likely cause of this teen’s intermittent hematuria is:
A. Acute poststreptococcal glomerulonephritis.
B. Henoch-Schonlein purpura nephritis.
C. IgA nephropathy.
D. Recurrent kidney stones.
E. Rapidly progressive glomerulonephritis (RPGN).

C. Recurrent painless gross hematuria, frequently associated with an upper respiratory tract infection, is typical of IgA nephropathy. These patients may develop chronic renal disease over decades. If proteinuria, hypertension, or impaired renal function were found, a biopsy would be necessary.

A 3-month-old boy presents to the emergency department with fever without a source. As part of his evaluation for suspected sepsis a urine specimen is collected. Based on initial results of laboratory testing, urinary tract infection is suspected. Which of the following statements is true?
A. If the urine culture reveals urinary tract infection, renal sonography and a voiding cystourethrogram should be performed.
B. Only if this infant has a second urinary tract infection, should a voiding cystourethrogram be performed.
C. Antimicrobial therapy should be initiated after urine culture and sensitivity are obtained
D. A renal biopsy should be performed
E. Do urine culture by mid stream clean-catch and bag urine.

A. If this infant has a urinary tract infection, evaluation of the urinary tract anatomy and function is necessary. The preferred methods of urine collection include bladder catheterization and suprapubic bladder aspiration. Antimicrobial therapy should be started empirically while awaiting urine culture and sensitivity results

An 8-year-old boy presents with bedwetting three to four times per week for “as long as he can remember.” He has a strong stream of urine, is continent of urine during the day, and has not had frequent urinary tract infections. His physical examination is normal. Which of the following is the most appropriate next course of action?
A. Urinalysis and urodynamic studies
B. Reassurance; he has secondary nocturnal enuresis
C. Use of enuresis alarm
D. Desmopressin acetate may be administered every 6 hours to control enuresis
E. Behavior modification that includes punishment for each wet night and a reward for each dry night

C. Nocturnal enuresis occurs in 15% of 5-year-old children and has a natural resolution rate of 15% per year. Males are more frequently affected, and family history of nocturnal enuresis is common. Initial evaluation includes thorough history of pattern of wetting, prior urinary tract infections, developmental, social, and emotional history. Physical examination includes palpation for kidneys, neurologic examination, and examination of the back looking for sacral dimple or hairy nevus. The enuresis alarm has a success rate of 70% to 90% and requires parental support and involvement. Pharmacological interventions in¬clude nighttime doses of oral imipramine or desmopressin ac¬etate, administered orally or intranasally. Following use of desmopressin acetate, fluid intake is restricted to avoid water in¬toxication. Behavior modification does not include punishment.

A 17-year-old boy is brought to the emergency department by his parents with the complaint of coughing up blood. He is stabilized, and his hemoglobin and hematocrit levels are determined to be in the safe range. During his hospitalization, he is noted to have persistently elevated blood pressures, and his urinalysis is remarkable for hematuria and proteinuria. You are suspicious the patient has which of the following?
a. Hemolytic-uremic syndrome
b. Goodpasture syndrome
c. Nephrotic syndrome
d. Poststreptococcal glomerulonephritis
e. Renal vein thrombosis

The answer is b. The patient in the question has a classic description of Goodpasture syndrome, a rare disease in children. The pulmonary hemorrhage can be life-threatening and the renal function progressive. Diagnosis is suggested by kidney biopsy and finding antibodies to the glomerular basement membrane

A 1-year-old child presents with failure to thrive, frequent large voids of dilute urine, excessive thirst, and three episodes of dehydration not asso¬ciated with vomiting or diarrhea. Over the years, other family members reportedly have had similar histories. Which of the following is the most likely diagnosis?
a. Water intoxication
b. Diabetes mellitus
c. Diabetes insipidus
d. Child abuse
e. Nephrotic syndrome

The answer is c. Nephrogenic diabetes insipidus is a hereditary congenital disorder in which the urine is hypotonic and pro¬duced in large volumes because the kidneys fail to respond to antidiuretic hormone. Males are primarily affected, through an X-linked recessive inheritance causing inactivating mutation of the vasopressin V2 receptor; autosomal dominant and recessive forms are also known. The disorder is felt to result from pri¬mary unresponsiveness of the distal tubule and collecting duct to vaso-pressin. Although the condition is present at birth, the diagnosis is often not made until several months later, when excessive thirst, frequent void¬ing of large volumes of dilute urine, dehydration, and failure to thrive become obvious. Maintenance of adequate fluid intake and diet and use of diuretic drugs are the bases of therapy for this incurable disease.
Water intoxication would not present with episodes of dehydration; diabetes mellitus rarely presents with a protracted course in such a young child (it is usually a more acute illness and often with vomiting). Child abuse would be unlikely, especially with a family history as noted. Nephrotic syndrome would be expected to present with other signs such as edema and proteinuria.

An 18-year-old adolescent develops anorexia, polydipsia and polyuria, vomiting, and unexplained fevers. Laboratory evaluation demon¬strates glucosuria, but normal blood sugar concentration, abnormally high urine pH in the presence of moderate serum hyperchloremic metabolic acidosis, and mild albuminuria with normal serum protein and albumin. Which of the following ingested agents, is most likely to result in these symptoms?
a. Penicillamine
b. Lithium
c. Outdated tetracycline
d. Nonsteroidal anti-inflammatory drugs (NSAIDs)
e. Sulfonamide antibiotics

The answer is c. The nonspecific findings of anorexia, polydipsia and polyuria, vomiting, and unexplained fevers, along with the more specific laboratory abnormalities of glucosuria but normal blood sugar, abnormally high urine pH in the face of mild or moderate serum hyperchloremic metabolic acidosis, and mild albuminuria in the presence of normal serum protein and albumin, suggest Fanconi syndrome (proximal tubular dysfunction). A number of agents can cause FS, including gentamicin (or other aminoglycosides), outdated tetracycline, cidofovir, valproic acid, streptozocin, 6-mercaptopurine, azathioprine, cisplatin, ifosfamide, heavy metals (e.g., lead, mercury, cadmium, uranium, platinum), paraquat, maleic acid, and glue sniffing (toluene). The other agents listed are also potentially nephrotoxic. The mechanisms of actions of these agents is through acute tubular necrosis, alteration of renal blood flow, intratubular obstruction, or allergic reactions within the kidney itself. Many of these toxic effects are reduced or eliminated with removal of the offending agent. Nephrotic syndrome is associated with penicillamine; lithium can cause renal tubular acidosis and nephrogenic diabetes insipidus; NSAIDs can cause nephrotic syndrome or interstitial nephritis; and sulfonamide agents can be associated with vasculitis and nephritis (although newer agents tend to have reduced incidence of this).

A 14-year-old girl is noted over a several-year period to have pro-gressive renal deficiency. She now has an 80% reduction of glomerular filtration rate and elevated levels of blood urea nitrogen (BUN) and creatinine. Which of the following is she also likely to demonstrate?
a. Polycythemia
b. Rickets
c. Frequent episodes of hypoglycemia
d. Early onset of puberty
e. Metabolic alkalosis

The answer is b. A wide variety of physiologic abnormalities result from chronic renal failure. Renal osteodystrophy encompasses osteitis fibrosa cystica, caused by secondary hyperparathyroidism, and/or rickets, or osteomalacia. caused by deficiency of 1,25(OH)2D3. Growth retarda¬tion, rather than precocious puberty, occurs in chronic renal failure due to malnutrition (poor appetite, low somatomedin C level), renal osteodystro¬phy excessive urinary loss of water and sodium, metabolic acidosis, and anemia. Normocytic, normochromic anemia is seen in advanced chronic renal failure, mainly as a result of a relative deficiency of erythropoietin; other causes include shortened RBC life span, GI blood loss caused by abnormal platelet function, iatrogenic blood loss during hemodialysis and from frequent blood sampling, folic acid deficiency, dialysis-related alu¬minum toxicity, and poor appetite Hyperglycemia results from peripheral resistance to endogenous insulin, and possibly from elevated glucagon are growth hormone levels.

A 4-year-old boy, whose past medical history is positive for 3 urinary tract infections, presents with a blood pressure of 135/90 mmHg. He is likely to exhibit which of the following symptoms or signs?
a. Multiple cranial nerve palsy
b. Headache
c. Hyporeflexia
d. Increased urine output
e. Right ventricular hypertrophy

The answer is b. Blood pressures in children must be interpreted in relation to the child’s age and height. Normograms are available. Routine testing is performed on children age 3 and beyond and for subgroups of children at younger ages, including children who were premature, had frequent uri¬nary tract infection, had umbilical artery catheters, and so on. Hyperten¬sion is usually asymptomatic, but with marked hypertension which is demonstrated by this 4-year-old, children can develop headache, dizzi¬ness, visual disturbances, irritability, and nocturnal wakening. Hyperten¬sive encephalopathy can be preceded or accompanied by vomiting, hyperreflexia, ataxia, and focal or generalized seizures. Facial nerve palsy can be the sole manifestation of severe hypertension. Isolated left ventricular hypertrophy, decreased urine output, and abdominal bruits are possi¬ble. When marked fundal changes are present or when there are signs of vascular compromise, emergency treatment of the accompanying hyper¬tension is warranted. Such hypertensive persons require immediate hospitalization for diagnosis and therapy.

A 6-week-old child is being evaluated for a fever of unknown etiol-ogy. As part of the laboratory evaluation, a urine specimen was obtained that grew Escherichia coli with a colony count of 2000/mL. These findings would be definite evidence of a urinary tract infection if which of the fol¬lowing is true about the sampled urine?
a. It has a specific gravity of 1.008
b. It is from a bag attached to the perineum of an uncircumcised boy
c. It is from an ileal-loop bag
d. It is from a suprapubic tap
e. It is the first morning sample

The answer is d. The mechanism by which urine is collected is paramount in determining the interpretation of cultures. No bacteria should grow in a properly obtained urine sample from a suprapubic tap unless infection is present. For children who undergo bladder catheterization, colony counts of 103 to 104 are considered significant. Ileal-loop bags are usually contam¬inated and are not useful. Bag specimens in smaller children (obtained by placing an adhesive, sealed, sterile collection system onto the perineum) can be helpful if the culture is negative. Contamination is common, espe-cially in girls and in uncircumcised boys; positive cultures in this latter group of children, especially if they contain more than one organism, are suspect. In properly obtained midstream clean-catch urines, colony counts greater than 105 in the asymptomatic child and colony counts of 104 in the symptomatic child may be significant. Clean-catch urines in an uncircumcised male who cannot retract the foreskin, “bagged” specimens in girls, and “bagged” specimens in uncircumcised boys are suspect.

A 4-year-old boy and his family have recently visited a local amuse-ment park. Several of the members of the family developed “gastroenteri¬tis” with fever and diarrhea, but the 4-year-olds stool was slightly different, as it contained blood. His mother reports that in the past 24 hours he developed pallor and lethargy; she relates that his face looks swollen and that he has been urinating very little. Laboratory evaluation reveals low hematocrit and platelet count and positive blood and protein in the urine. Which of the following diagnoses is most likely to explain these symp¬toms?
a. Henoch-Schonlein purpura
b. IgA nephropathy
c. Intussusception
d. Meckel diverticulum
e. Hemolytic-uremic syndrome

The answer is e. Hemolytic-uremic syndrome is most common in children younger than 4 years of age and is characterized by an acute microangiopathic hemolytic anemia, thrombocytopenia from increased platelet utilization, and renal insufficiency from vascular endothelial injury and local fibrin deposition. Ischemic changes result in renal cortical necro¬sis and damage to other organs such as colon, liver, heart, brain, and adrenal. Laboratory findings associated with hemolytic-uremic syndrome include low hemoglobin level, decreased platelet count, hypoalbuminemia, and evidence of hemolysis on peripheral smear (burr cells, helmet cells, schistocytes Urinalysis reveals hematuria and proteinuria. A marked reduction of renal function leads to oliguria and rising levels of blood urea nitrogen (BUN) and creatinine. Gastrointestinal bleeding and obstruction, ascites. and central nervous system findings such as somnolence, convul¬sions, and coma can occur. In the past decade, infection by the verotoxin- producing Escherichia coli 0157:H7 has been implicated as a cause of hemolytic-uremic syndrome. This organism is epizootic in cattle. Out¬breaks associated with undercooked contaminated hamburgers have been reported in several states. Roast beef, cow’s milk, and fresh apple cider have been implicated as well. The Coombs test is not positive in this type of hemolytic anemia.

A 6-year-old girl is brought to the emergency room because her urine is red. She has been healthy her whole life, and has recently returned from an outing with her grandmother to a local amusement park. Her urine dip for heme is positive, suggesting which of the following is a possibility?
a. Ingestion of blackberries
b. Ingestion of beets
c. Phenolphthalein catharsis
d. Presence of myoglobin
e. Ingestion of Kool-Aid

The answer is d. A number of pH-dependent substances can impart a red color to urine. Use of phenolphthalein, a cathartic agent can cause red urine; ingestion of blackberries, red Kool-Aid or beets also can lead to red coloration (“beeturia”). None of these choices, however, would cause the heme test to be positive. Myoglobin, on the other hand, is the only choice listed that can test positive for heme. “Hematuria” should be confirmed by dipstick testing as well as by microscopic examination of uri¬nary sediment.

A 12-year-old boy comes to the emergency department at midnight with a complaint of severe scrotal pain since 7 p.m. There is no history of trauma. Which of the following is the most appropriate first step in man¬agement?
a. Order a surgical consult immediately
b. Order a radioisotope scan as an emergency
c. Order a urinalysis and Gram stain for bacteria
d. Arrange for an ultrasound examination
e. Order a Doppler examination

The answer is a. The majority of all cases of acute scrotal pain and swelling, in boys are cause by testicular torsion.
If surgical exploration occurs without delay the testes can be saved in 90% of the time. Too often, delay caused by ordering imaging and laboratory tests-, such as those outlined in the question which results in an unsalvageable gonad.

The mother of a 2-year-old girl reports that her daughter complains of burning when she urinates and that she has foul-smelling discharge from her vagina. She has found some slight staining on the front of her under-wear, but she denies the child has fever, nausea, vomiting, or other consti¬tutional signs. The child does not attend day care, and she has demonstrated no change in behavior. The physical examination is normal with an intact hymen, but the child’s vulva is reddened and with a malodorous disharge. Her urinalysis and culture are normal. Which of the following is the most likely explanation for this child’s condition?
a. Sexual abuse
b. Pinworms in the vagina
c. non specefic vulvovaginitis
d. Pediculosis pubis
e. Giardiasis

The answer is c. Symptoms listed are those of vulvovaginitis, with nonspecific vulvovaginitis accounting for 70% of all pediatric vulvovaginitis cases. The discharge in nonspecific vulvovaginitis is usually brown or green and with a fetid odor. The burning with urination occurs because of con¬tact between raw skin and urine. Further history in this case might reveal use of tight-fitting clothing (including rubber pants prolonged bubble baths with contamination of the vagina with soap products, use of per¬fumed lotions in the vaginal urea, or improper toilet habits (wiping of fecal material toward rather than away from vagina). Attention to these causative conditions usually results in resolution of the symptoms. The finding of a normal hymen put it away from sexual abuse. Pinworms can infest the vagina, but symptoms usually include significant itching of the rectum and vagina. Pediculosis pubis requires pubic hair and thus is usually not seen before adolescence. Giardiasis can result in vaginal discharge, but associated symptoms usually include diarrhea and malabsorption syndrome as well. In ‘a sexually active adolescent (or in a sexually abused younger child), a variety of infectious agents, such as Candida, Chlamydia trachomatis, Trichomonas vaginalis, Gardnerella vaginalis, and Neisseria gonorrhea, would be higher on the differential.

A 7-year-old boy has crampy abdominal pain and a rash mainly on the back of his legs and buttocks as well as on the extensor surfaces of his forearms. Laboratory analysis reveals proteinuria and microhematuria. Which of the following is the most likely diagnosis?
a. Systemic lupus erythematosus
b. Anaphylactoid purpura
c. Poststreptococcal glomerulonephritis
d. Takayasu arteritis
e. Dermatomyositis

The answer is b. The rash of anaphylactoid purpura most often involves extensor surfaces of the extremities buttocks and lower limbs; the face, soles, palms, and ,trunk are less often affected. Other significant symptoms include edema. arthralgia or arthritis, colicky abdominal pain with GI bleeding, acute scrotal pain, and renal abnormalities ranging from microscopic hematuria to renal failure. Both lupus erythematosus and dermatomyositis are often accompanied by typical facial rashes (butterfly and heliotrope, respectively). People who have Takayasu arteritis usually do not present with a rash. The scarlatiniform rash characteristic of streptococcal infections generally does not coincide with the development of poststreptococcal nephritis.

A 2-year-old patient arrives late to your office with his father and a sign-language translator. They are very apologetic, but the father communi-cates that he had car trouble at his dialysis center and thus was late picking up the child from day care. The father is concerned about his child’s having intermittent red, bloody-looking urine. A gross inspection of the child’s urine in your office looks normal, but the dipstick demonstrates 3+ blood. Which of the following is the most likely cause of this child’s hematuria?
a. Alport syndrome
b. Berger’s nephropathy (IgA nephropathy)
c. Idiopathic hypercalcuria
d. Membranous glomerulopathy
e. Goodpasture syndrome

The answer is a. The most common type of hereditary nephritis is Alport syndrome. Clinically, patients present with asymptomatic microscopic hematuria, but gross hematuria is also possible, especially after an upper respiratory infection. Hearing loss, eventually leading to deafness, is associated with Alport syndrome in 30 to 75% of cases. End-stage renal disease, as hinted at by the question from this father, is common by the second or third decades of life. This syndrome is mostly commonly an X-linked dominant disorder, which explains the more severe course in males. Other findings include ocular abnormalities (30 to 40%) and, rarely, leiomyomatosis of the esophagus or respiratory tree.
Berger’s nephropathy (IgA nephropathy) results from IgA deposits within the mesangial glomerulus in the absence of systemic disease; it can be associated with microscopic or gross hematuria, but is not associated with a heredity condition resulting in deafness and end-stage renal disease. Idiopathic hypercalciuria, which may be autosomal domi¬nant, causes recurrent gross hematuria, persistent microscopic hematuria, and complaints of dysuria or abdominal pain without stone formation. Membranous glomerulopathy is an unusual cause of hematuria in children occurring in the second decade of life when seen; it is the most common cause of nephritic syndrome in adults. It usually presents with signs and symptoms of nephrotic syndrome. Goodpasture syndrome results in pul¬monary hemorrhages in addition to the signs and symptoms of glomerulonephritis; it is a rare disease in children.

The parents of a 7-year-old boy complain that they have been unable to potty-train their son. Although he is dry during the day, he rarely goes an entire night without wetting the bed. A thorough physical examination and a screening urinalysis are normal. Which of the following is the most appropriate statement to the family?
a. The condition is three times more common in girls than boys
b. Most patients with this condition have a psychiatric illness as the
cause
c. Spontaneous cure rates are high regardless of therapy
d. Family history of this condition is uncommon
e. Short courses of desmopressin acetate (DDAVP) lead to permanent cure in 25% of cases

The answer is c. Nocturnal enuresis is involuntary voiding at night at an age when control of micturition is expected. It is common in children after the age of 5 years (approximately 20%), resolving in approximately 15% of these cases per year thereafter. A careful history and physical exam¬ination will usually identify any potential organic causes. In most cases, no etiology is found. A family history is common. Minimal laboratory testing beyond screening urinalysis is indicated. The condition is more common in boys than in girls. Therapy is aimed at reassurance to the parents that the condition is self-limited, avoidance of punitive measures, aid considera¬tion of purchasing a bed-wetting alarm. Spontaneous cure rates are high regardless of therapy. Short courses of DDAVP lead to control in 60 to 75% of cases while the patient takes the medication, but frequently the episodes return when the medication is stopped.

A 21-year-old woman presents to the emergency room in active labor. She has had no prenatal care, but her last menstrual period was approximately 9 months prior. Her membranes are artificially ruptured, yielding no amniotic fluid. She delivers an 1800-g (4 Ib) term infant who develops significant respiratory distress immediately at birth. The first chest radiograph on this infant demonstrates hypoplastic lungs. After this infant is stabilized, which of the following is the most appropriate next step for this infant?
a. Cardiac catheterization
b. Renal ultrasound
c. MRI of the brain
d. Liver and spleen scan
e. Upper Gl

The answer is b. Oligohydramnios can cause a number of serious prob-lems in the infant, including constraint deformities (such as clubfoot) and pulmonary hypoplasia. These infants have usually experienced intrauterine growth retardation and frequently have an associated serious renal abnormality. Ultrasound of the kidneys is important to rule out renal involvement as a cause of the oligohydramnios. None of the other testing would be expected to identify an etiology for the oligohydramnios and pul¬monary hypoplasia, although this child’s ultimate clinical course might necessitate additional testing.

A 6 year old girl with global developmental delay presents with fever and gross haematuria,
with visible pink urine and clots. Full term normal delivery with no neonatal complications.
Diagnosed with developmental delay at 9 months, and receives special schooling.
Immunizations up to date. There is no family or social history of note.
On examination she has a temperature of 38.9°C, respiratory rate of 18/min and heart rate of
90/min. She has suprapubic tenderness. Urine dipstix testing shows 4+ haematuria, 3+
proteinuria and 3+ nitrites.
What is the most likely diagnosis?

a) Benign familial haematuria
b) Glomerulonephritis
c) Haemorrhagic cystitis
d) Henoch-Schonlein Disease
e) Urinary tract infection

Answer is c
The history of fever, gross haematuria with clots and suprapubic tenderness in a child
with global delay suggests a delayed presentation of UTI resulting in haemorrhagic
cystitis. This should be treated aggressively with antibiotics. A renal US should be
performed to look for structural abnormalities and to confirm resolution.

Which of the following is a known risk factor for the development of chronic rejection of kidney
transplantation:-
a) Age
b) Anti-smooth muscle antibodies
c) Presence of anti-HLA antibodies
d) Smoking
e) Toxoplasma infection

Answer is c
All other options are not valid