Miki Murakoshi1,2, Koichi Kamei3, Masao Ogura1, Mai Sato1, Taishi Nada1, Ryutaro Suzuki1, Chikako Kamae1,4, Kentaro Nishi1, Toru Kanamori1,2, China Nagano5, Kandai Nozu5, Koichi Nakanishi6, Kazumoto Iijima5,7. 1. Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, Japan. 2. Department of Pediatrics, Tokyo Medical and Dental University, Tokyo, Japan. 3. Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, Japan. kamei-k@ncchd.go.jp. 4. Department of Pediatrics, Self Defense Forces Central Hospital, Tokyo, Japan. 5. Department of Pediatrics, Kobe University Graduate School of Medicine, Hyogo, Japan. 6. Department of Child Health and Welfare (Pediatrics), Graduate School of Medicine, University of the Ryukyus, Okinawa, Japan. 7. Hyogo Prefectural Kobe Children's Hospital, Hyogo, Japan.
Abstract
BACKGROUND: The management of congenital nephrotic syndrome of the Finnish type (CNF) is challenging. It is difficult to withdraw intravenous albumin infusions, resulting in long-term hospitalization. In addition, fatal hypotension after bilateral nephrectomy has been reported. In our center, we have performed unilateral nephrectomy during early infancy. METHODS: Infants diagnosed with CNF between 2011 and 2020 in our institution were enrolled. We examined the clinical course before and after unilateral nephrectomy and evaluated the effectiveness of this strategy. RESULTS: Seven patients (all showing NPHS1 mutations) were enrolled. All required daily intravenous albumin infusion via central venous catheter (CVC). Unilateral nephrectomy was performed at a median of 76 days of age (59-208 days). Surgical complications did not occur in any of patients. The mean albumin dose was decreased after unilateral nephrectomy (2.0 vs 0.4 g/kg/day; p = 0.02). Intravenous albumin infusion could be withdrawn at a median of 17 days, the CVC removed at a median of 21 days, and they discharged at a median of 82 days after unilateral nephrectomy. Although bacterial infections were noted seven times before unilateral nephrectomy, only one episode occurred after surgery. Four patients initiated peritoneal dialysis at two to three years of age and all of them underwent kidney transplantation thereafter. CONCLUSIONS: Unilateral nephrectomy during early infancy may be an effective treatment allowing for withdrawal from albumin infusion, prevention of complications, withdrawal from CVCs and shortening hospital stay for patients with CNF.
BACKGROUND: The management of congenital nephrotic syndrome of the Finnish type (CNF) is challenging. It is difficult to withdraw intravenous albumin infusions, resulting in long-term hospitalization. In addition, fatal hypotension after bilateral nephrectomy has been reported. In our center, we have performed unilateral nephrectomy during early infancy. METHODS: Infants diagnosed with CNF between 2011 and 2020 in our institution were enrolled. We examined the clinical course before and after unilateral nephrectomy and evaluated the effectiveness of this strategy. RESULTS: Seven patients (all showing NPHS1 mutations) were enrolled. All required daily intravenous albumin infusion via central venous catheter (CVC). Unilateral nephrectomy was performed at a median of 76 days of age (59-208 days). Surgical complications did not occur in any of patients. The mean albumin dose was decreased after unilateral nephrectomy (2.0 vs 0.4 g/kg/day; p = 0.02). Intravenous albumin infusion could be withdrawn at a median of 17 days, the CVC removed at a median of 21 days, and they discharged at a median of 82 days after unilateral nephrectomy. Although bacterial infections were noted seven times before unilateral nephrectomy, only one episode occurred after surgery. Four patients initiated peritoneal dialysis at two to three years of age and all of them underwent kidney transplantation thereafter. CONCLUSIONS: Unilateral nephrectomy during early infancy may be an effective treatment allowing for withdrawal from albumin infusion, prevention of complications, withdrawal from CVCs and shortening hospital stay for patients with CNF.
Keywords:
Bilateral nephrectomy; Central venous catheter (CVC); Congenital nephrotic syndrome of the Finnish type (CNF); Intravenous albumin infusion; NPHS1; Unilateral nephrectomy
Authors: Alexandru R Constantinescu; Tej K Mattoo; William E Smoyer; Larry A Greenbaum; Jianli Niu; Noel Howard; Melissa Muff-Luett; Elizabeth B Benoit; Avram Traum; Ali A Annaim; Scott E Wenderfer; Emilee Plautz; Michelle N Rheault; Robert L Myette; Katherine E Twombley; Yu Kamigaki; Belkis Wandique-Rapalo; Mohammad Kallash; Tetyana L Vasylyeva Journal: Front Pediatr Date: 2022-09-14 Impact factor: 3.569