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Year : 2010 | Volume
: 21
| Issue : 5 | Page : 935-939 |
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Goodpasture's syndrome in children |
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Banani Poddar1, Sanjay Singhal1, Afzal Azim1, Sanjeev Gulati2, Arvind Baronia1
1 Department of Critical Care Medicine, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India 2 Department of Nephrology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
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Date of Web Publication | 31-Aug-2010 |
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Abstract | | |
Goodpasture's syndrome is an autoimmune disease that has rarely been described in children, and only 21 cases have been reported till now. Here is a case of a 9-year-old boy who was diagnosed to have Goodpasture's syndrome.
How to cite this article: Poddar B, Singhal S, Azim A, Gulati S, Baronia A. Goodpasture's syndrome in children. Saudi J Kidney Dis Transpl 2010;21:935-9 |
How to cite this URL: Poddar B, Singhal S, Azim A, Gulati S, Baronia A. Goodpasture's syndrome in children. Saudi J Kidney Dis Transpl [serial online] 2010 [cited 2021 Mar 4];21:935-9. Available from: https://www.sjkdt.org/text.asp?2010/21/5/935/68896 |
Introduction | |  |
The term pulmonary-renal syndrome has frequently been used to describe the clinical manifestations of a great number of diseases in which pulmonary hemorrhage and glomerulonephritis coexist. [1],[2],[3],[4] Though rare in childhood, pulmonary renal syndrome presents a medical emergency. Goodpasture's syndrome (GPS) as a cause of pulmonary-renal syndrome is rare in childhood. [5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22]
Case Report | |  |
A 9-year-old boy was admitted to his local hospital with a two weeks history of low-grade fever and vomiting. There was no past or family history of renal disease. Five days later, he developed cola-colored urine. The urinalysis revealed protein 2(+), blood 3(+) and numerous dysmorphic red blood cells per high power field. Renal function deteriorated progressively with oligoanuria and marked uremia over the next few days for which peritoneal dialysis was started. As he was anemic, he received two units of whole blood transfusion. For further evaluation, he was referred to the department of Nephrology in our hospital, where peritoneal dialysis was continued. On admission, examination revealed a pale patient with bilateral pedal edema and blood pressure of 180/100 mmHg. His hemoglobin was 7.2 g/dL, total leukocyte count 16800/mm 3 and platelets 497,000/mm 3 with normal coagulation tests. Serum biochemistry revealed blood urea nitrogen (BUN) 83 mg/dL, creatinine 8.8 gm/dL and normal liver function tests. He was seronegative for HIV and Hepatitis B and C. Chest radiograph and electrocardiogram were normal. An ultrasound of abdomen revealed enlarged kidneys with increased cortical echogenicity with loss of corticomedullary differentiation. While planning for a renal biopsy, the patient developed respiratory distress associated with an episode of hemoptysis. Recent chest radiograph revealed bilateral interstitial alveolar infiltrates [Figure 1]. Hemodialysis was initiated, but respiratory distress continued. Because of increasing respiratory distress, the patient was transferred to the ICU where he was intubated and put on mechanical ventilation. The enzyme linked immunosorbent assay (ELISA) for perinuclear antineutrophil cytoplasm antibodies (p-ANCA) and anti-myeloperoxidase (antiMPO) was positive. ELISA for anti-GBM (glomerular basement membrane) antibodies was strongly positive (titre 1514.4 U/mL). Assays for ANCA revealed negative indirect immunofluorescence for c-ANCA (cytoplasmic) and p-ANCA. Complements levels were elevated (C3-139 and C4-32.3 mg/dL). Pulse methylprednisolone followed by oral prednisolone, cyclophosphamide, and plasmapheresis on a daily basis were started. Intermittent hemodialysis was continued and 21 sessions of plasmapheresis were completed. The blood pressure was controlled with 5 antihypertensive drugs (ramipril, nifedipine, propranolol, prazosin and clonidine). The weekly measured titer of anti-GBM antibody levels started to decrease and was strongly associated with clinical improvement. The pulmonary hemorrhage resolved and the patient was gradually weaned off the ventilator, but his renal function did not improve and he continued to require intermittent dialysis. After weaning from venti lation, ambulatory peritoneal dialysis was started but could not be sustained due to peritonitis not responding to local and systemic antibiotics. Eventually, he was discharged home on intermittent hemodialysis, anti-hypertensive drugs and immunosuppressants, but he succumbed to his illness around two months after discharge. | Figure 1 :Chest radiograph showing bilateral interstitial alveolar infiltrates (right > left) suggestive of pulmonary hemorrhage.
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Discussion | |  |
Pulmonary renal syndrome can be categorized into specific and nonspecific. Nonspecific pulmonary renal syndrome [2] refers either to pulmonary edema, thromboemblism, or infection complicating the course of glomerular disease, or to glomerular diseases following a pulmonary disease, mostly an infection. Though encountered in both childhood and in adulthood, nonspecific pulmonary renal syndrome is more frequent in adults.
Specific pulmonary renal syndromes [2] are of three major categories:
- Goodpasture's syndrome with circulating antibodies against GBM or linear immunofluorescent staining of IgG in the basement membranes of pulmonary or renal tissue;
- Immune-complex-related glomerulonephritis, including the types associated with systemic lupus erythematosus, Henoch-Schonlein purpura, mixed connective-tissue disease, and cryoglobulinemia;
- glomerulonephritis associated with antineutrophil cytoplasmic antibodies (ANCA), without immune complexes or anti-GBM antibody, including Wegener's granulomatosis, microscopic polyarteritis, the Churg-Strauss syndrome, and classic polyarteritis nodosa.
In childhood, the most frequent causes of pulmonary renal syndrome are systemic lupus erythematosus, ANCA-associated systemic vasculitis, Henoch-Scholein purpura and hemolyticuremic syndrome. [3] Goodpasture's syndrome as a cause of pulmonary-renal syndrome in childhood is extremely rare with only 21 cases reported until now in the medical literature. [4],[5],[6],[7],l[8],[9],[10],[11],[1],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22] The previously reported cases of Goodpasture's syndrome are detailed in [Table 1]. Among the 22 pediatric patients with Goodpasture's syndrome, including ours, there are six males in comparison to 14 females with male to female ratio of 0.43, and the majority of the cases (15 out of 20) are between 6-13 years of age. In contrast to this, in adults, it is more common in males (male to female ratio = 4:1) with peak incidence between 20-30 years of age. [23] Anti-GBM titers were positive in all the patients in which it was done (16/16); out of them, only one patient showed an indeterminate result. Anti-MPO titer was positive in only four patients along with the anti-GBM titers. Our patient also had both antiMPO and anti-GBM. Renal biopsy was diagnostic in 12 out of 15 (80%) of the patients; biopsy could be obtained after death in 3 patients. Pulmonary biopsy was not obtained from any patient during life. In most of the patients (10/14, 71.4%), the treatment strategy consisted of steroid and immunosuppressants along with plasmapheresis. In 4 patients, the diagnosis could be made only after death. Only a third of the patients (5/15) showed improvement in renal function, of which one became dialysis-dependent later. In patients with non improved renal function, renal transplant was performed in 2/11 (18.2%) of the patients, whereas the majority (9/11, 81.8%) remained on intermittent dialysis. Six out of 18 (33.3%) patients died in comparison to mortality of 11-21% in adults. [24]
The diagnosis of Goodpasture's syndrome can be confirmed by the presence of circulating anti-GBM antibodies and/or deposition of antibodies on the glomerular basement membrane demonstrated by immunofluorescent (IF) staining of the renal biopsy specimen. Pulmonary biopsy is rarely indicated because renal tissue is more easily attainable with less morbidity. [24]Management should be aggressive and started immediately upon suspicion. Therapy is based on corticosteroids; immunosuppressive agents such as cyclophasphamide in addition to plasmapheresis. [25],[26] Renal transplantation can be performed in patients requiring chronic hemodialysis after disappearance of the circulating antiGBM antibodies. Advanced renal failure at presentation and/or presence of crescents affecting more than 50% of glomeruli is a serious prognostic sign. [24]
In conclusion, pulmonary-renal syndrome is a rare event in childhood. Although rare, GPS should be considered in the differential diagnosis. Pulmonary involvement is related to acute presentation, whereas renal involvement is related to long-term morbidity. Early diagnosis is crucial for a favorable outcome of this syndrome.
References | |  |
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Correspondence Address: Banani Poddar Department of Critical Care Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow 226014 India
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PMID: 20814136 
[Figure 1]
[Table 1] |
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