Mohammad Ali Khan ( Department of Paediatrics, Hayat Shaheed Teaching Hospital, Peshawar. )
Amin Jan Gandapur ( Department of Paediatrics, Hayat Shaheed Teaching Hospital, Peshawar. )
Abdul Hameed ( Department of Paediatrics, Hayat Shaheed Teaching Hospital, Peshawar. )
Ashfaq Ahmad ( Department of Paediatrics, Hayat Shaheed Teaching Hospital, Peshawar. )
Nephrotic syndrome is a rate presentation of Hodgkin’s disease. Majority of these cases have minimal change disease and some cases present as membranous glomenilopathy. This report presents the case of a child with nephrotic syndthme associated with Hodgkins disease.
Nephrotic syndrome (NS) is a common disease in children 2-7 years of age. Majority of cases are idiopathic with minimal change in pathology. Infrequent causes of NS in children include focal segmental glomeruloscierosis, membranous ncphropathy and therapy with pencillamine, gold or mercury compounds. Rarely NS has also been described in association with extra renal neoplasms like lymphoma and carcinoma1,2. A case of NS in association with Hodgkin’s disease is described below.
A 6 years old Afghan refugee boy presented with history of well circumscribed, painless swelling on the left side of the neck noticed by the parents. Five days later, the child started to develop generalized swelling of the whole body without any other systemic complaint. A local doctor gave him some treatment with no improvement. The swelling of the body gradually increased and spread to the external genitalia. On day 14, the patient also developed high grade fever thus necessitating hospitalization in our unit. The past history has been unremarkable except for an attack of poliomyelitis at the age of 2 years resulting in flaccid paralysis of the lower limbs. There was no history of drug use oriminunization inthe recent past. Clinical examination revealed a developmentally normal child, with gross pitting oedema of the whole body (anasarca). His weight was 20 kg with abdominal girth of 67 cm. There were no features suggestive of protein calorie malnutrition. He was pale looking and had a temperature of 38°C, pulse rate of 120 min and supine blood pressure of 110/70 mmHg. He had flaccid paralysis of both the lower limbs with a reflexia. He had multiple soft rubbery, discrete, mobile and non-tenderlymph nodes on the left side of the neck. The patient also had ascites, which made abdominal visceral palpation difficult. There were no other positive findings on general and systemic clinical examination.
Investigations: Blood picture showed haemoglobin of 10.8 Gm/dl and ESR 60 mm 1st hour. Total leukocyte count was 16700 mm3 with 47% polymorphs and 50% lymphocytes. Blood urea, sugar and senim electrolyte were normal. Urine examination showed albuminurea 4+ and pus cells of 5-10 cells per high power feed. Urine culture was negative. Urinary protein excretion was 3.5 grams per 24 hours. Serum protein was 4.3 gmm% and cholesterol 240 mg%. Mauntoux test was negative. Chest radiograph and ECG were normal. Smears of peripheral blood and bone marrow aspirate were normal. Abdominal ultrasonography showed ascites, normal kidneys and no enlargement of para aortic lymph nodes. Lymph node biopsy from cervical glands confirmed the diagnosis of Hodgkin’s disease, mixed cellular type. Diagnosis of nephrotic syndrome (NS) with Hodgkin’s disease was made based on clinical and laboratory evidence. In aetiology, idiopathic NS as well as nephrotic syndrome secondary to tuberculosis or malignancy was considered.
NS is a rare complication of Hodgkin’s disease. Minimal change NS was found only in 0.4% patients in two combined series of cases of Hodgkin’s disease with a total of 1700 patients4,6,7. In 90% cases, NS is idiopathic. Approximately 10% cases of idiopathic NS are associated with different neoplastic disorders including solid tumours, carcinomas, lymphoma. Amongst lymphoma, nephrotic syndrome is ten times more common in patients withHodgkin’s disease. NS is associated more frequently with mixed cellular type Hodgkin’s disease1,3,4. In Hodgkin’s disease, minimal change disease makes the pathological basis of NS in majority of cases4,5. Membranous glomerulopathy is associated with only 10% of cases of NS with Hodgkin’s disease. In contrast, NS in patients with carcinoma has membranous glomerulonephritis as underlying pathology in 80-90% of cases4’5. In most patients with Hodgkin’s disease and nepbrotic syndrome, the two diseases may develop simultaneously or one after the other. Nephrotic syndrome regresses with the treatment and returns with the relapse of Hodgkin’s disease4,8-10.
1. Behnnan, R. E., Kliegman, R. M.and Nelson, W. B. et al.Nelson textbook of pediatrics, In fourteenth ed., London, WB Saunders Company, 1992.
2. Behrman, R. E., Kliegman, R. M. and Nelson W. E. Essential ofpediatrics. In ed. London, WB Saunders company, 1990, pp. 569-71.
3. Andreoli, T. E., Carpenter, C. C. J., Plum, F. eta!. Cecil essential of medicine, In 2nd ed., London, WB Saunders company, 1990, p. 227.
4. Scully, R. E., Mark, E. J. and McNeely, B. U. Caserecords oftheMassachusetts General Hospital, N. Engi. J. Med., 1983,308,15:888.
5. Eagen, J. W. and Lewis, E. J. Glomerulopathies of neoplasia, Kidney Int., l977;1 1:297-303.
6. Plager, J. and Stutzman, L. Acute nephrotic syndrome as a manifestation of active.Hodgkin’s disease: Report of four cases and review of the literature. Am. J. Med., 1971.;50:56-66.
7. Kramer, P., Sizoo, W. and Twiss, E. E. Nephrotic syndrome in Hodgkin’s disease: Report of 5 cases and review of literature. Neth. J. Med., 1981 ;24: 114-119.
8. Alper, C. E. and Cotran, S.. S. Neoplasia and glomerular injury, Kidney Int., 1986;30:465.
9. Dabbs, D. J., Striker, L., Mignon, F. et al. Glomerular lesions in lymphoma and leukaemia. Am. J, Med., 1986;80:63-65.
10. Moorthy,A. W.,Zimmerman, S. W. and Burkholder, P.M. Nephrotic syndrome in Hodgkin’s disease: Evidence for pathogenesis alternativeto immune complex depostion. Am. J. Med., 1976;61 :471-77.