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child nephrotic syndrome

Md Habibur Rahman, Md Abdul Qader, Syed Saimul Haque, Md Abdullah Al Mamun, Golam Muin Uddin
Posterior reversible encephalopathy syndrome (PRES) is a rare clinical and radiological phenomenon is encountered in children compared to adults. In our center, a 5-yearold boy with steroid-dependent nephrotic syndrome (SDNS) presented with headache and blurring of vision during relapse after a long course of immunosuppressive therapy. Evaluation by computed tomography scan of the brain showed that the child had hypodense areas throughout the occipital region of the brain. All signs of PRES, except papilledema, resolved after seven days of supportive treatment evidenced by subsequent radiological evaluation...
September 2016: Saudi Journal of Kidney Diseases and Transplantation
Ryszard Grenda, Wioletta Jarmużek, Joanna Latoszyńska, Sylwester Prokurat, Jacek Rubik
SIOD is rare disorder related to SMARCAL1 or SMARCAL2 gene mutation, including (among other comorbidities) T-cell immunodeficiency, nephrotic syndrome, and renal failure. Up to 22% of primary patients may develop various autoimmune disorders. We report the case of 11-year-old male with SIOD, who presented ITP at 2 years after renal transplantation with decrease in platelet count (from normal) to 56 000/μL and then (gradually) to 2000/μL. There was no effect of iv. methylprednisolone/dexamethasone. As the presence of antibodies against GPIIb/IIIa, GPIb, and GPIaIIa platelet glycoproteins was confirmed, patient was given 50 g of IVIG and then was put on plasmapheresis; however, both showed poor direct effect...
September 26, 2016: Pediatric Transplantation
Syed Sajid Hussain Shah, Farkhanda Hafeez
OBJECTIVE: To compare the efficacy of tacrolimus versus cyclosporine (Calcineurin Inhibitors) in the management of childhood steroid-resistant nephritic syndrome (SRNS). STUDY DESIGN: Quasi-experimental study. PLACE AND DURATION OF STUDY: Department of Paediatric Nephrology at The Children's Hospital and Institute of Child Health, Lahore, from August 2014 to September 2015. METHODOLOGY: Patients of either gender aged 1 - 12 years, with the diagnosis of mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal-change disease (MCD) were included...
July 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Cengiz Zeybek, Gokalp Basbozkurt, Salih Hamcan, Ayhan Ozcan, Davut Gul, Faysal Gok
Galloway-Mowat syndrome (GMS) is an autosomal recessive disorder with a poor prognosis that was first defined as a triad of central nervous system involvement, hiatal hernia, and nephrotic syndrome. However, this syndrome is now known to have a heterogeneous clinical presentation. The nephrotic syndrome is steroid resistant and is responsible for the outcome. The combination of collapsing glomerulopathy and GMS is very rare. A 26-month-old boy presented with steroid-resistant nephrotic syndrome associated with neurologic findings, including microcephaly, psychomotor retardation, and nystagmus...
2016: Case Reports in Nephrology
Daiki Kondo, Atsuko Noguchi, Hiroaki Tamura, Satoko Tsuchida, Ikuko Takahashi, Hiroki Kubota, Tamami Yano, Chikako Oyama, Yukio Sawaishi, Shinichi Moriwaki, Tsutomu Takahashi
Nucleotide excision repair (NER) is an essential biological pathway protecting against ultraviolet light-induced DNA damage. Deficient NER causes a group of rare genetic disorders including two autosomal recessive diseases, xeroderma pigmentosum (XP) and Cockayne syndrome (CS). In addition to the cutaneous photosensitivity shared in XP and CS, CS is featured by growth failure, neurological deterioration, microcephaly, and deep sunken eyes. XP/CS complex is an extremely rare type of NER disorder with a distinct phenotype that is characterized by the skin and eye pathology of XP and the somatic and neurological abnormalities of CS...
2016: Tohoku Journal of Experimental Medicine
Kyoung Hee Han, Ji Youn Park, Seung-Kee Min, Il-Soo Ha, Hae Il Cheong, Hee Gyung Kang
Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%-5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and femoral arteries, and is associated with the use of steroids and diuretics. Popliteal artery thrombosis in children has been described in cases of traumatic dissection, osteochondroma, Mycoplasma pneumoniae infection, and fibromuscular dysplasia...
May 2016: Korean Journal of Pediatrics
C Nayyar, N Gulati, B L Sherwal
Dematiaceous fungi are the etiological agents of phaeohyphomycosis and are now increasingly being recognized for causing disease in humans. A high level of suspicion and routine fungal cultures are required to identify these cases. There is no consensus regarding their management. Here, an unusual presentation of phaeohyphomycosis (secondary to Fonsecaea pedrosoi) presenting as a disseminated infection in a case of nephrotic syndrome is described.
May 2016: Indian Journal of Nephrology
Song-Yi Kim, Ahram Han, Chanjoong Choi, Sang-Il Min, Hyo-Cheol Kim, Jongwon Ha, Seung-Kee Min
Acute arterial thromboembolism (ATE) is rare in childhood, but this medical emergency requires immediate treatment. Described herein are separate instances of lower extremity ATE in 2 children, both of whom were successfully managed through image-guided thromboembolectomy (IGT). One patient, a 34-month-old female child with nephrotic syndrome, developed bilateral iliac and popliteal thromboembolic arterial occlusions after high-dose steroid therapy. Another 9-year-old girl suffered an embolism of left popliteal artery due to infectious endocarditis...
July 2016: Annals of Vascular Surgery
S Agrawal, M A Chanley, D Westbrook, X Nie, T Kitao, A J Guess, R Benndorf, G Hidalgo, W E Smoyer
Glucocorticoids are the primary therapy for nephrotic syndrome (NS), but have serious side effects and are ineffective in ~20-50% of patients. Thiazolidinediones have recently been suggested to be renoprotective, and to modulate podocyte glucocorticoid-mediated nuclear receptor signaling. We hypothesized that thiazolidinediones could enhance glucocorticoid efficacy in NS. We found that puromycin aminonucleoside-induced proteinuria in rats was significantly reduced by both high-dose glucocorticoids (79%) and pioglitazone (61%), but not low-dose glucocorticoids (25%)...
2016: Scientific Reports
Liru Qiu, Jianhua Zhou
BACKGROUND: LAMB2 mutations cause Pierson syndrome (OMIM 609049), an autosomal recessive genetic disease typically characterized by congenital nephrotic syndrome (CNS) and early onset renal failure, as well as bilateral microcoria. NPHP1 mutations cause familial juvenile nephronophthisis type 1 (NPHP1, OMIM 256100), another autosomal recessive renal disease that usually occurs years after birth. Both Pierson syndrome and nephronophthisis cause end-stage renal disease and rare kidney diseases in children...
2016: BMC Pediatrics
Karlota Borges, Jovanka Vasilevska-Ristovska, Neesha Hussain-Shamsy, Viral Patel, Tonny Banh, Diane Hebert, Rachel J Pearl, Seetha Radhakrishnan, Tino D Piscione, Christoph P B Licht, Valerie Langlois, Leo Levin, Lisa Strug, Rulan S Parekh
BACKGROUND: Studies in the USA report differences in opinion among parents of different ethnic groups toward genetic testing for their child; however, there are no studies that address this issue in the diverse ethnic and immigrant population in Canada. OBJECTIVE: This study aims to determine whether ethnicity and immigration status influences parental interest in clinical genetic testing for a potentially progressive kidney disease. DESIGN: This is a cross-sectional study...
2016: Canadian Journal of Kidney Health and Disease
Keisuke Sugimoto, Tomoki Miyazawa, Takuji Enya, Kouhei Miyazaki, Mitsuru Okada, Tsukasa Takemura
BACKGROUND: Cyclosporine A (CsA) is used globally as an immunosuppressant for the treatment of immune-mediated nephrotic syndrome (NS). However, its long-term use causes nephrotoxicity characterized by tubulointerstitial injury and glomerulosclerosis. The present study aimed to investigate the associations between histomorphological findings and immunohistological expression of Cathepsin L (CatL) and CD2-associated protein (CD2AP) in patients with NS mediated with CsA. METHODS: A total of 18 patients with child-onset NS were divided into two groups after treatment with CsA for 2 years (group A; n = 10) and more than 4 years (group B; n = 8), respectively...
March 14, 2016: Clinical and Experimental Nephrology
Songül Yılmaz, Z Birsin Özçakar, Aysel Taktak, Saba Kiremitçi, Arzu Ensari, Handan Dinçaslan, Fatoş Yalçınkaya
BACKGROUND: Targeting the vascular endothelial growth factor (VEGF) signaling pathway has become an important approach to current cancer therapy. Anti-VEGF therapy-related renal adverse effects may present as hypertension, non-nephrotic proteinuria, and rarely as nephrotic syndrome (NS) and acute kidney injury. CASE-DIAGNOSIS/TREATMENT: In this report, we present a 15-year-old boy who had developed nephrotic syndrome and thrombotic microangiopathy 26 months after administration of anti-VEGF therapy...
June 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Yoichi Iwafuchi, Tetsuo Morioka, Takashi Morita, Kanako Watanabe, Yuko Oyama, Ichiei Narita
Nephrotic syndrome without hematuria due to infection-related glomerulonephritis is uncommon. The present report describes a case of nephrotic syndrome due to infection-related glomerulonephritis without hematuria and hypertension in an older child. A 14-year-old boy was referred to our hospital because of a 5-day history of fever, nausea, weight gain and recent leg edema without hypertension. Laboratory data showed nephrotic-range proteinuria, hypoalbuminemia, mild hypocomplementemia and acute renal injury without hematuria...
January 2016: Case Reports in Nephrology and Dialysis
C I Esezobor, T A Ladapo, F E Lesi
BACKGROUND: The clinical course of steroid-sensitive nephrotic syndrome (SSNS) among Nigerian children has rarely been reported; this makes prognostication difficult. OBJECTIVES: The objective was to determine the frequency of relapses including frequent relapses (FR) and steroid-dependence (SD) in a cohort of Nigerian children with SSNS. A secondary objective was to identify clinical and demographic factors associated with relapse in these children. METHODS: Medical records of children with SSNS in a Tertiary Hospital in Nigeria were reviewed...
March 2016: Nigerian Journal of Clinical Practice
Osama Y Safdar, Sherif M El-Desoky, Detlef Bockenhauer, Neil Sebire, Jameela A Kari
No abstract text is available yet for this article.
January 28, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
H Barman, R Dass, S G Duwarah
Inducing remission in nephrotic children on anti-tubercular therapy is difficult due to the increased metabolism of prednisolone induced by rifampicin. We report a child with nephrotic syndrome treated successfully with an increased dose of steroids without discontinuing anti-tubercular therapy.
January 2016: Saudi Journal of Kidney Diseases and Transplantation
Ali Derakhshan, Dorna Derakhshan, Mohammad Hossein Fallahzadeh, Mitra Basiratnia
Recurrence of original disease is a common threat in the field of transplantation. Recurrence of nephrotic syndrome is not common in children with congenital nephrotic syndrome (CNS). We report a case of a female child with CNS who presented with nephrotic state at first month of age and became dialysis dependent at 17 months of age. After seven months of continuous ambulatory peritoneal dialysis, she received a kidney from a deceased donor. Eight months after transplantation, she presented with a full-blown feature of nephrotic syndrome...
January 2016: Saudi Journal of Kidney Diseases and Transplantation
Motoshi Hattori, Masayuki Iwano, Mayumi Sako, Masataka Honda, Hirokazu Okada, Yuko Akioka, Akira Ashida, Yukihiko Kawasaki, Hideyasu Kiyomoto, Yoshio Terada, Daishi Hirano, Mikiya Fujieda, Shouichi Fujimoto, Takao Masaki, Shoichi Maruyama, Seiich Mastuo
BACKGROUND: Transition of adolescent and young adult (AYA) patients with childhood-onset chronic kidney diseases (C-CKD) from pediatric to adult renal services has received increasing attention. However, information on transition of Japanese patients with C-CKD is limited. METHODS: The Transition Medicine Working Group, in collaboration with the Japanese Society for Nephrology, the Japanese Society for Pediatric Nephrology and the Japanese Society of Pediatric Urology, conducted a retrospective cross-sectional study in 2014 on issues concerning the transition of Japanese patients with C-CKD...
January 19, 2016: Clinical and Experimental Nephrology
Shogo Kobayashi, Yukihiko Kawasaki, Hideki Sano, Kazuhiro Mochizuki, Mitsuaki Hosoya, Atsushi Kikuta
BACKGROUND: Hematopoietic stem cell transplantation (HSCT)-related nephrotic syndrome (NS) is a rare event and has been described as a clinical form of chronic graft-versus-host disease (GVHD). Although immunological mechanisms are thought to play important roles in NS after HSCT, the exact mechanisms have not been clarified. CASE-DIAGNOSIS/TREATMENT: We report a 4-year-old boy with acute lymphoblastic leukemia (ALL) who developed NS during the tapering of immunosuppressants 5 months after an allogeneic HSCT (allo-HSCT)...
April 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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