Journal of Clinical and Diagnostic Research. 2018 Jul, Vol-12(7): QD08-QD09 8 8 DOI: 10.7860/JCDR/2018/36044.11836 Case Report Obstetrics and Gynaecology Section Childhood Onset Nephrotic Syndrome- A Journey Through Two Pregnancies CASE REPORT First Pregnancy A 25-year-old female presented in 2012 with 16 weeks amenorrhoea, and recent onset of rapidly progressive, gross oedema. She was a known case of steroid sensitive NS, from the age of three years. At the age of 10, she was diagnosed to have minimal change disease based on renal biopsy (the patient’s histopathology report was available to the authors). She has had many episodes of relapse that would respond to treatment with prednisolone. Married for about one year, she did not have any problems with conception. Her last relapse was about one year before her pregnancy. Her renal functions were normal (serum creatinine 0.6 mg/dL), and she had hypoalbuminemia with massive proteinuria (serum total protein 62 gm/L, albumin 30 gm/L, urine protein 3.6 gm/day). Other haematological and biochemical tests, including thyroid function tests, were normal. She was diagnosed to have a relapse of her NS during the second trimester of pregnancy. Treatment was initiated in consultation with Departments of Internal Medicine and Nephrology, using prednisolone 1 mg/kg body weight, along with salt and fluid restriction. The patient had a gradual reduction of oedema and proteinuria, and the dose of prednisolone was gradually reduced by about 0.25 mg/kg body weight every 2-4 weeks with close medical and obstetric monitoring. At 30 weeks, she developed gestational hypertension, while on 10 mg/day of prednisolone that was managed with oral labetalol, starting with 50 mg twice daily (100 mg of tablet labetalol, half tablet twice daily). By 35 weeks, her requirement for prednisolone was stable at 10 mg per day, and labetalol requirement was 200 mg twice daily. She underwent emergency caesarean delivery at 35 weeks of gestation, due to severe pre-eclampsia and intrauterine growth restriction. Birth weight of the baby was 1800 grams. Her blood pressure was very high perioperatively and required continued usage of parenteral antihypertensive agents for 24 hours in the postoperative period. The baby had neonatal hypoglycaemia and needed neonatal intensive care for six days. She went into remission of NS postnatally, and steroids, as well as antihypertensives, were tapered and stopped about four weeks after delivery. Second Pregnancy Though she was advised to avoid conception for at least two years, she conceived during lactational amenorrhea. She had no significant oedema or proteinuria during this pregnancy and was not started on prednisolone this time. She was under close fortnightly follow- up. She showed trace proteinuria; however, renal functions and serum protein levels were within normal ranges. In consultation with internal medicine and nephrology services, she received a total fluid intake of less than 1.2 L/day and salt <5 gm/day. She developed gestational hypertension at 24 weeks of gestation and started on labetalol 50 mg twice daily (100 mg of tablet labetalol, half tablet twice daily). She was also found to be hypothyroid and started on levothyroxine 50 μg once daily. She neither had a relapse of NS during second pregnancy nor did she develop pre-eclampsia. However, there was foetal growth restriction during this pregnancy too, and elective caesarean delivery with bilateral tubal ligation was done at 38 weeks. The birth weight was 2100 grams. There were no perioperative complications. She did not need antihypertensives in the postnatal period after the second delivery, and there were no neonatal problems. DISCUSSION Preconceptional counselling is of utmost importance in females with kidney disease, as there could be worsening during pregnancy, especially if the disease is active. In women with advanced renal insufficiency, and if the woman is on cytotoxic alkylating agents, fertility may be a concern. At least six months of remission is considered optimal before conception [1]. This patient had consulted the nephrologist and was counselled to plan the first pregnancy as she was in remission for more than a year and her renal function tests were normal. Pregnancy is a state of altered haemodynamic and hormonal physiology [2]. These changes have significant implications for renal function too. Patients with pre-existing renal diseases pose a unique challenge. Management of such patients requires a multidisciplinary approach and close supervision [3]. Kidney disease has the propensity to affect fertility as well as pregnancy outcome [4]. Two related aspects here are the effect of gestation on kidney and effect of kidney disease on pregnancy. SHASHIKALA K BHAT 1 , SHASHIKIRAN UMAKANTH 2 , KRUPA SHAH 3 , AP ASHWINI 4 , RAVINDRA PRABHU 5 Keywords: Hypertension, Pregnancy, Proteinuria, Renal disease, Steroids ABSTRACT Nephrotic Syndrome (NS), a unique renal disease complex associated with heavy proteinuria (protein excretion >3.5 gm/day), hypoalbuminemia (<30 gm/L) and peripheral oedema. It is a rare entity in pregnancy, with an incidence of about 0.012-0.025%. There have been very few reports of pregnancy with co-existing NS. We hereby report a case of steroid sensitive childhood onset NS, due to minimal change disease and her course through two pregnancies. While she had a relapse of NS during her first pregnancy, she sailed through her second pregnancy without relapse. Women with NS can have a good maternal and perinatal outcome with multidisciplinary approach. Pregnancy does not always worsen the existing NS by causing a relapse or disease progression.