Goldston Syndrome,
2011
Aga Khan University
Goldston Syndrome, Zainab Hussain, Imrana Masroor, Qurrat-Ul-Ain Haider, Tariq Alam
Department of Radiology
Goldston syndrome is a rare entity describing the association of polycystic kidneys and Dandy Walker malformation with or without hepatic fibrosis. A 28 years old pregnant woman came to radiology department for the first antenatal scan. Ultrasound showed a single fetus corresponding to 27 week of gestation. The fetal brain revealed deficient vermis with a posterior fossa cyst communicating with the 4th ventricle suggestive of Dandy-Walker malformation (DWM). The kidneys were enlarged, and echogenic containing small cysts with associated amniotic fluid index of 5 cm suggestive of Oligohydramnios. A diagnosis of Goldston syndrome was suggested. These findings were confirmed on …
Rho Activation Of Mdia Formins Is Modulated By An Interaction With Inverted Formin 2 (Inf2),
2011
Shanghai Jiaotong University
Rho Activation Of Mdia Formins Is Modulated By An Interaction With Inverted Formin 2 (Inf2), Hua Sun, Johannes S. Schlondorff, Elizabeth J. Brown, Henry N. Higgs, Martin R. Pollak
Dartmouth Scholarship
Inverted formin 2 (INF2) encodes a member of the diaphanous subfamily of formin proteins. Mutations in INF2 cause human kidney disease characterized by focal and segmental glomerulosclerosis. Disease-causing mutations occur only in the diaphanous inhibitory domain (DID), suggesting specific roles for this domain in the pathogenesis of disease. In a yeast two-hybrid screen, we identified the diaphanous autoregulatory domains (DADs) of the mammalian diaphanous-related formins (mDias) mDia1, mDia2, and mDia 3 as INF2_DID-interacting partners. The mDias are Rho family effectors that regulate actin dynamics. We confirmed in vitro INF2_DID/mDia_DAD binding by biochemical assays, confirmed the in vivo interaction of these …
Serum 25-Hydroxyvitamin D Level And Acute-Phase Reaction Following Initial Intravenous Bisphosphonate.,
2011
Children's Mercy Hospital
Serum 25-Hydroxyvitamin D Level And Acute-Phase Reaction Following Initial Intravenous Bisphosphonate., Tarak Srivastava, Hongying Dai, Connie J. Haney, Uri S. Alon
Manuscripts, Articles, Book Chapters and Other Papers
No abstract provided.
Is Iron Sucrose (Venofer) A Safe Treatment For People With Chronic Kidney Disease?,
2011
Philadelphia College of Osteopathic Medicine
Is Iron Sucrose (Venofer) A Safe Treatment For People With Chronic Kidney Disease?, Kait Reid
PCOM Physician Assistant Studies Student Scholarship
OBJECTIVE: The objective of this study is to determine if Iron Sucrose (Venofer) is a safe treatment for people with Chronic Kidney Disease.
Ten Year Experience Of Pediatric Kidney Biopsies From A Single Center In Pakistan,
2010
Aga Khan University
Ten Year Experience Of Pediatric Kidney Biopsies From A Single Center In Pakistan, A Absar, M Diamond, Y Sonia, R Arshalooz, A Safia, K Waqar, P Shahid
Section of Nephrology
There are many established registries of kidney biopsies around the world. In addition, there are several reports available in literature from many countries on pediatric kidney biopsy. This study was done to determine the indications and pathological patterns of kidney biopsies of children referred to our hospital, and compare our data with the data available from other countries. This is a cross-sectional study of pediatric kidney biopsies over a 10-year period, from January 1997 to December 2006. All biopsies were done in Aga Khan University Hospital, Karachi, Pakistan. Age range was from 1 to 14 years. Data were analyzed for …
Contrast-Induced Nephropathy In Patients Undergoing Percutaneous Coronary Intervention,
2010
Aga Khan University
Contrast-Induced Nephropathy In Patients Undergoing Percutaneous Coronary Intervention, Sajid H. Dhakam, Saqib A Gowani, Asif Jafferani, Sajid H Dhakam
Department of Medicine
Contrast Induced Nephropathy (CIN) is a feared complication of numerous radiological procedures that expose Patients to contrast media. The most notorious of these procedures is percutaneous coronary intervention (PCI). Not only is this a leading cause of morbidity and mortality, but it also adds to increased costs in high risk Patients undergoing PCI. It is thought to result from direct cytotoxicity and hemodynamic challenge to renal tissue. CIN is defined as an increase in serum creatinine by either ≥0.5mg/dL or by ≥25% from baseline within the first 2-3 days after contrast administration, after other causes of renal impairment have been …
Hypertension In Children And Adolescents: Epidemiology And Natural History.,
2010
Pediatrics and Medicine, Thomas Jefferson University
Hypertension In Children And Adolescents: Epidemiology And Natural History., Bonita Falkner
Department of Medicine Faculty Papers
Primary hypertension is detectable in children and adolescents and, as in adults, is associated with a positive family history of hypertension, obesity, and life-style factors. Owing to the well-established childhood obesity epidemic, the population prevalence of high blood pressure (BP) in the young is increasing. Hypertension in childhood is commonly associated with other cardiovascular risk factors as well as obesity. Although death and cardiovascular disability do not occur in hypertensive children, intermediate markers of target organ damage, such as left ventricular hypertrophy, thickening of the carotid vessel wall, retinal vascular changes, and even subtle cognitive changes, are detectable in children …
Prostaglandin E(2) Is Crucial In The Response Of Podocytes To Fluid Flow Shear Stress.,
2010
Children's Mercy Hospital
Prostaglandin E(2) Is Crucial In The Response Of Podocytes To Fluid Flow Shear Stress., Tarak Srivastava, Ellen T. Mccarthy, Ram Sharma, Patricia A. Cudmore, Mukut Sharma, Mark L. Johnson, Lynda F. Bonewald
Manuscripts, Articles, Book Chapters and Other Papers
Podocytes play a key role in maintaining and modulating the filtration barrier of the glomerulus. Because of their location, podocytes are exposed to mechanical strain in the form of fluid flow shear stress (FFSS). Several human diseases are characterized by glomerular hyperfiltration, such as diabetes mellitus and hypertension. The response of podocytes to FFSS at physiological or pathological levels is not known. We exposed cultured podocytes to FFSS, and studied changes in actin cytoskeleton, prostaglandin E(2) (PGE(2)) production and expression of cyclooxygenase-1 and-2 (COX-1, COX-2). FFSS caused a reduction in transversal F-actin stress filaments and the appearance of cortical actin …
Range For Normal Body Temperature In Hemodialysis Patients And Its Comparison With That Of Healthy Individuals,
2010
Aga Khan University
Range For Normal Body Temperature In Hemodialysis Patients And Its Comparison With That Of Healthy Individuals, Rabia Hasan, Mehreen Adhi, Syed Faisal Mahmood, Fatima Noman, Safia Awan, Fazal Akhtar, Anwar Naqvi, Adeeb Ul Hassan Rizvi
Section of Nephrology
Background/Aims: Patients with chronic kidney disease undergoing hemodialysis have an altered homeostasis leading to altered body temperatures. We aimed to determine the range for normal body temperature in hemodialysis Patients and compared it to healthy individuals. Also, we determined how much axillary temperatures differed from oral temperatures in both groups and whether axillary temperature is affected by the presence of an arteriovenous fistula (AVF) in hemodialysis Patients. Methods: Oral and axillary (left & right) temperatures were recorded using an ordinary mercury-in-glass thermometer in 400 subjects (200 hemodialysis Patients, 200 healthy individuals) at the Sindh Institute of Urology and Transplantation from …
Ewing's Sarcoma/Pnet Of Kidney In 13-Year-Old Girl,
2010
Aga Khan University
Ewing's Sarcoma/Pnet Of Kidney In 13-Year-Old Girl, Quratulain Badar, Nasir Ali, Nadeem Abbasi, Shamvil Ashraf, Farrok Karsan, Raheel Hashmi
Department of Radiology
Ewing's sarcoma is the second most common primary tumour of bone in childhood. Less frequently it occurs in soft tissues. Ewing's sarcoma/primitive neuroectodermal tumour (ES/PNET) is an extra ordinarily rare primary tumour in the kidney. Only very few cases of primary renal Ewing's sarcoma have been reported in the literature to date. We present a case of primary right renal Ewing's sarcoma in a 13-year-old girl who was diagnosed as a case of stage IV ES/PNET of kidney with metastases to lung and liver. Right nephrectomy was done followed by adjuvant radiotherapy and chemotherapy with complete response to local and …
Hereditary 1,25-Dihydroxyvitamin D-Resistant Rickets With Alopecia Resulting From A Novel Missense Mutation In The Dna-Binding Domain Of The Vitamin D Receptor.,
2010
Children's Mercy Hospital
Hereditary 1,25-Dihydroxyvitamin D-Resistant Rickets With Alopecia Resulting From A Novel Missense Mutation In The Dna-Binding Domain Of The Vitamin D Receptor., Peter J. Malloy, Jining Wang, Tarak Srivastava, David Feldman
Manuscripts, Articles, Book Chapters and Other Papers
The rare genetic recessive disease, hereditary vitamin D resistant rickets (HVDRR), is caused by mutations in the vitamin D receptor (VDR) that result in resistance to the active hormone 1,25-dihydroxyvitamin D(3) (1,25(OH)(2)D(3) or calcitriol). In this study, we examined the VDR from a young boy with clinical features of HVDRR including severe rickets, hypocalcemia, hypophosphatemia and partial alopecia. The pattern of alopecia was very unusual with areas of total baldness, adjacent to normal hair and regions of scant hair. The child failed to improve on oral calcium and vitamin D therapy but his abnormal chemistries and his bone X-rays normalized …
