What is the most common complication of juvenile rheumatoid arthritis?
Correct Answer A:
Juvenile rheumatoid arthritis (JRA) is a rheumatic disease that begins at or before age 16. Arthritis, fever, rash, adenopathy, splenomegaly, and iridocyclitis are typical of some forms.
Patients with JRA can have joint stiffness, swelling, effusion, pain, and tenderness. JRA may interfere with growth and development. Micrognathia (receded chin) due to early closure of mandibular epiphyses may occur. Iridocyclitis may develop, which may cause conjunctival injection, pain, and photophobia but can be asymptomatic; scarring and glaucoma with band keratopathy can result.
Diagnosis is clinical. Treatment involves NSAIDs and often disease-modifying antirheumatic drugs.
Which of the following is not required for a diagnosis of juvenile rheumatoid arthritis?
Correct Answer C:
Juvenile rheumatoid arthritis (JRA) should be suspected in children with symptoms of arthritis, signs of iridocyclitis, generalized adenopathy, splenomegaly, or unexplained fever lasting more than a few days, or rash. Diagnosis is primarily clinical. Patients suspected of having JRA should be tested for rheumatoid factor (RF), antinuclear antibodies (ANA), and ESR as these tests may be helpful in diagnosing JRA and distinguishing its subtypes. In Still's disease, RF and ANA are absent. In pauciarticular-onset JRA, ANA are present in up to 75%. In polyarticular-onset JRA, RF usually is negative, but in some patients, mostly adolescent girls, it can be positive.
Which one of the following is a manifestation of pauci-articular juvenile rheumatoid arthritis?
Pauci-articular juvenile rheumatoid arthritis (JRA) is more common than polyarticular JRA and systemic JRA. Pauci-articular JRA is further subdivided into two subtypes. Type I is most common in toddlers, especially girls, and can be associated with chronic anterior uveitis, which is often asymptomatic yet potentially destructive. The incidence is higher in children with positive antinuclear antibodies; therefore, regular ophthalmologic surveillance is necessary. Type II is more common in boys and has a later onset. Eye problems are less common.
The presentation of systemic JRA includes prolonged fever, rash, pericarditis or pleuritis, lymphadenopathy, hepatosplenomegaly, anemia, leukocytosis, thrombocytosis, and, of course, arthritis. Glomerulonephritis is uncommon in systemic JRA. Erythema nodosum is not a feature of JRA.
A 2-year-old boy has developed grade 4 vesicoureteral reflux.
What is the most appropriate management?
Correct Answer B:
Vesicoureteral reflux (VUR) is the backup of urine from bladder into the ureter during urination. VUR may result in urine reflux into the renal pelvis, causing hydronephrosis and kidney damage. In children, this condition is usually caused by an abnormality that is present at birth (congenital) and is often diagnosed during prenatal ultrasound.
Reflux is graded according to its severity:
Since many children will outgrow their reflux, they can be followed carefully, with their reflux monitored at intervals by tests such as VCUG, renal ultrasound, or nuclear voiding cystogram. All patients with grades III to V VUR regardless of age receive antibiotic prophylaxis (choice B). Although no large randomized trials have been conducted establishing the efficacy of prophylactic antibiotics in prevention of either urinary tract infection (UTI) or renal scarring, several decades of clinical practice have demonstrated that antibiotic prophylaxis is usually well-tolerated, and clinicians are reluctant not to offer some treatment. The typical dose is one fourth of the therapeutic dose.
grade IV are not preferred as they are not considered protective enough against recurrent infection.Prophylaxis should be started once a child has completed treatment of the initial UTI and continues at least until imaging reveals vesicoureteral reflux. If no vesicoureteral is seen, prophylaxis is discontinued. If vesicoureteral reflux is present, prophylactic antibiotics are continued until (1) vesicoureteral reflux resolves, (2) vesicoureteral reflux is surgically corrected, or (3) the child grows old enough that prophylaxis is deemed no longer necessary.
→ At this time surgery (choice D) is not indicated in this child. Accepted indications for surgical treatment include the following:
→ Pyridium (choice E) is often used to alleviate the pain, irritation, discomfort, or urgency caused by urinary tract infections, surgery, or injury to the urinary tract. While children with VUR are at increased risks of UTI, pyridium is not given simply for confirmed VUR without an infection and the symptoms that warrant its use.
A 10-month-old child presents for the 5th time with symptoms of pain when he urinates, fever, and abdominal tenderness.
What is the most likely underlying cause of his recurrent UTIs?
Vesicoureteral reflux (choice B) is retrograde passage of urine from the bladder back into the ureter and renal pelvis. This may damage the upper urinary tract by bacterial infection and occasionally by increased hydrostatic pressure. Bacteria in the lower urinary tract can easily be transmitted by reflux to the upper tract, leading to recurrent parenchymal infection with potential scarring and renal dysfunction. Chronically elevated emptying pressures (> 40 cm H2O) and increased bladder volume and pressure often cause progressive kidney damage, even without infection or reflux.
Symptoms and signs are typically those of UTI; these may include fever, abdominal or flank pain, dysuria or flank pain with voiding, frequency, and urgency. Pyuria, hematuria, proteinuria, and bacteriuria may be present on urinalysis. Tests for reflux include filling and voiding cystourethrogram (which is best to diagnose bladder outlet obstruction) and radioisotope cystogram. Renal ultrasonography evaluates for size, hydronephrosis, and scarring. Vesicoureteral reflux is usually mild to moderate. Mild cases often resolve spontaneously over months to several years while daily antibacterial prophylaxis is maintained.
→ Ureteropelvic junction obstruction (choice A) is the most common cause of neonatal and antenatal hydronephrosis. Prior to the use of prenatal ultrasonography, most patients with this condition presented with pain, hematuria, urosepsis, failure to thrive, or a palpable mass. It is found in approximately 50% of patients diagnosed with antenatal hydronephrosis.
→ Potter's Syndrome (bilateral renal agenesis) (choice C) - would have been diagnosed in his antepartum or neonatal period.
→ Cryptorchidism (choice D) is the most common genital problem encountered in pediatrics. It presents with an empty and hypoplastic or poorly rugated scrotum or hemiscrotum.
→ Nephroblastoma (Wilms tumor) (choice E) presents as a large, solid tumor (abdominal mass) of renal origin. Average age of incidence is 3 years.
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