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
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