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Symptoms » Darkened urine » Book Sections
 

Dark Urine - Case 20-2: 15-Year-Old Boy

I. History of Present Illness

A 15-year-old boy was well until 12 days before admission. At that time, he complained of a sore throat that resolved spontaneously. Three days later, he noted swelling and tenderness of the right knee that also resolved on its own. There was no associated trauma or redness. One week before admission, he developed “iced tea-colored urine.” He did not complain of fever, nausea, vomiting, dysuria, urinary frequency, or urgency. He did have one episode of watery diarrhea. The dark urine continued, and he complained of decreased energy, malaise, and anorexia. He had lost 10 pounds over the course of the last few weeks. Three days before admission, he had developed a nonproductive cough. In his primary physician 's office, he had orthostatic hypotension (blood pressure 104/70 mm Hg seated, and 80/40 mm Hg standing). He was hospitalized for further evaluation.

II. Past Medical History

The patient had never been hospitalized or had surgery. He had been treated several times for sinusitis, most recently 3 years earlier. He did not take any medicines and had no allergies. There was no known family history, because the patient is adopted.

III. Physical Examination

T, 36.8°C; RR, 20/min; HR, 82 bpm; BP, 104/54 mm Hg
Weight, 47.5 kg (5th percentile)
On examination, the patient was thin and in no distress. His mucous membranes were moist, his oropharynx clear, and he had no nasal discharge. He had a normal cardiac examination. His breath sounds were slightly coarse and decreased at the bases bilaterally, but he had no wheezes or crackles. He had no hepatomegaly or splenomegaly. His right knee was slightly swollen without erythema or tenderness, and it had full range of motion. He had no significant lymphadenopathy and a normal neurologic examination.

IV. Diagnostic Studies

A complete blood count revealed 8,200 WBCs/mm3 (3% band forms, 55% segmented neutrophils, 28% lymphocytes, 2% eosinophils, and 15% basophils). His hemoglobin was 7.5 g/dL, and his platelet count was 289,000 cells/mm 3. A reticulocyte count was 3%, and a blood smear demonstrated hypochromia. Serum electrolytes were normal, with the exception of an elevated creatinine concentration of 1.5 mg/dL. Liver enzymes were normal. A chest radiograph was significant for bilateral alveolar infiltrates in a butterfly pattern. Blood, urine, and stool samples were sent for culture. A renal ultrasound study showed increased echogenicity of both kidneys.

V. Hospital Course

On the first day of admission, the patient developed hemoptysis and became anuric. The diagnosis was suggested by the clinical presentation and confirmed by renal biopsy.
Discussion: Case 20-2

I. Differential Diagnosis

Hemoglobin or myoglobin in the urine causes tea-colored urine. This patient had many additional symptoms to help guide a differential. He complained of a prodrome consisting of a sore throat that lasted 3 days. Classically, poststreptococcal acute glomerulonephritis manifests 10 days after such a prodrome; malaise and anorexia may also be associated with this disease. Symptoms of benign recurrent hematuria and IgA nephropathy may also be brought on by respiratory tract infections. This patient, however, had not had dark urine before. He had no urinary urgency or frequency to indicate hemorrhagic cystitis. Coagulopathies typically cause gum bleeding or spontaneous joint bleeding rather than hematuria and hemoptysis. Teenagers are more likely to have vasculitis than younger children are, and this patient 's weight loss speaks to a chronic process. He did not have any other symptoms of lupus, but his past medical history of sinusitis was put together with his new symptoms of tea-colored urine and hemoptysis to derive a diagnosis of Wegener granulomatosis.

II. Diagnosis

The renal biopsy demonstrated severe crescentic glomerulonephritis. Immunofluorescence staining showed pauciimmune glomeruli. The anti-neutrophil cytoplasmic antibody (ANCA) was positive at a dilution of 1:320 with a cytoplasmic distribution (cANCA), and a diagnosis of Wegener granulomatosis was made. The patient received plasmapheresis and was treated with intravenous methylprednisone. He was then given a dose of cyclophosphamide and changed to oral prednisone. He was transferred to a hospital closer to his home to continue receiving plasmapheresis every other day.

III. Incidence and Epidemiology

Wegener granulomatosis is a rare vasculitis, and its cause remains unknown. As a result, much of the epidemiology is not well understood, especially in children. The annual incidence of Wegener granulomatosis has been estimated to be approximately 1 in 2 million persons. In the 1990s, corticosteroids and cyclophosphamide became the standard of care, and the 5-year survival rate exceeded 60%. Fifty-six patients with Wegener granulomatosis in Norway were evaluated retrospectively, looking for predictors of survival and organ damage. The youngest patient was 10 years old, but the majority of the patients were adults. Estimates for survival at 1, 5, and 10 years were calculated to be 93%, 79%, and 75%, respectively. In general, the study found that impaired renal function placed patient at higher risk for ultimate end-stage renal disease and death. However, younger patients had a higher rate of recovery of renal function. As a result, it is difficult to predict outcome in children with Wegener granulomatosis. Younger patients were also found to have more nasal and sinus symptomatology and less kidney impairment. Many patients test positive for cANCA, antibodies that are thought to play a role in pathogenesis, perhaps by stimulating neutrophil activity.

IV. Clinical Presentation

Clinical symptoms result from widespread granulomas of arteries and veins. The primary manifestations of Wegener granulomatosis are symptoms of the upper respiratory tract, lungs, and kidneys. Bloody sinusitis, rhinorrhea, and ulceration of the nasal mucosa are common symptoms of respiratory tract involvement and are frequently the first manifestations of the disease. Pulmonary complaints include cough, hemoptysis, and pleuritis. Tea-colored urine or hematuria indicates kidney involvement. Patients also experience generalized symptoms of malaise, anorexia, and weight loss, all of which were reported by this patient. Peripheral symptoms include arthritis, arthralgia, skin ulceration, serous otitis media, conjunctivitis, exophthalmos, corneal ulceration, and hepatitis. None of these symptoms is specific or can be considered pathognomonic. A clustering of these symptoms, especially those affecting the primary organ systems (sinuses, lungs, and kidneys) should prompt evaluation for this disease entity.

V. Diagnostic Approach

Pathology. Biopsies of affected organs provide the strongest basis for making this diagnosis. Affected skin, mucosa, or lung tissue demonstrates granulomas and a necrotizing vasculitis characterized by leukocytic, lymphocytic, and giant cell infiltration. Kidney pathology includes a crescentic glomerulonephritis without immunoglobin deposits (pauciimmune).
Chest radiography or computed tomographic scan of the chest.  Nodular lesions or alveolar infiltrates characterize these studies.
Immunology. Most patients have serum that tests positive for cANCA. Combined with progressive glomerulonephritis, a positive cANCA result has a 98% positive predictive value.
Inflammatory markers. Active disease is marked by an elevated erythrocyte sedimentation rate, usually greater than 100 mm/hour.

VI. Treatment

Immunotherapy has dramatically improved the survival and morbidity associated with this disease. Corticosteroids are the mainstay of therapy. Azathioprine and cyclophosphamide have been used successfully in steroid-resistant disease. Cyclophosphamide can be given as an intravenous pulse every other week, or as daily oral therapy. A study of 56 patients in Norway found that intravenous pulse therapy was ultimately associated with a smaller total dose of cyclophosphamide and fewer days on corticosteroids. Methotrexate has been used with some success as well. Plasmapheresis can be used in acute, life-threatening circumstances.

VII. References

 1. Koldingses W, Nossent H. Predictors of survival and organ damage in Wegener's granulomatosis. Rheumatology 2002;41:572–581.
2. Eddy AA. Glomerular disorders. In: Rudolph CD, Rudolph AM, Hostetter MK, et al., eds. Rudolph's pediatrics, 21st ed. New York: McGraw-Hill, 2003:1677–1699.
3. Tunnessen WW Jr. Signs and symptoms in pediatrics, 3rd ed. Philadelphia: Lippincott Williams & Wilkins, 1999.
4. Yalcindag A, Sundel R. Vasculitis in childhood. Curr Opin Rheumatol 2001;13:422–427.

Book Source Details

  • Book Title: Pediatric Complaints and Diagnostic Dilemmas
  • Author(s): Samir S Shah MD; Stephen Ludwig MD
  • Year of Publication: 2003
  • Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2003 Lippincott Williams & Wilkins.

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Copyright Details: Pediatric Complaints and Diagnostic Dilemmas, Copyright © 2008 Williams & Wilkins.

More About Causes of Darkened urine




More About This Book:
Title: Pediatric Complaints and Diagnostic Dilemmas
Authors: Samir S Shah MD; Stephen Ludwig MD
Publisher: Lippincott Williams & Wilkins
Copyright: 2003
ISBN: 0-7817-4188-2

 » Next page: Dark Urine - Case 20-3: 10-Year-Old Boy (Pediatric Complaints and Diagnostic Dilemmas)

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