Nephrocalcinosis in Premature Infants: Variability in Ultrasound Detection

Total Page:16

File Type:pdf, Size:1020Kb

Nephrocalcinosis in Premature Infants: Variability in Ultrasound Detection Original Article nnnnnnnnnnnnnn Nephrocalcinosis in Premature Infants: Variability in Ultrasound Detection Thomas Campfield, MD Francis J. Bednarek, MD CONCLUSION: Mariann Pappagallo, MD There is significant variability among radiologists in the ultrasound identification of nephrocalcinosis in premature infants; a 7.5-MHz ul- Frederick Hampf, MD trasound transducer is associated with less variability in recognizing this John Ziewacz, MD lesion. Jacqueline Wellman, MD Gary Rockwell, MD Gregory Braden, MD Renal calcification is an uncommon problem in children, but recent Patrecia Flynn-Valone, RD reports have called attention to the development of nephrocalcinosis Michael Neylan in premature infants. These calcium oxalate deposits may be identi- Antonio Pangan, MD fied on plain films of the abdomen but are more readily detected by ultrasound.1,2 Estimates of the incidence of nephrocalcinosis in pre- 3 OBJECTIVE: mature infants have varied considerably. Jacinto et al. have reported 4 To measure variability among radiologists in the ultrasound diagnosis of a 65% incidence of nephrocalcinosis, whereas Short and Cooke re- nephrocalcinosis in premature infants. ported a 27% incidence in very low birth weight infants. Differences in image quality obtained using different ultrasound transducers or METHODOLOGY: observer variability in image interpretation may have contributed to In this prospective multicenter study, renal ultrasounds were performed this variation in incidence. No data are available regarding these two on 54 very low birth weight infants using a 5.0- and 7.5-MHz transducer, potential sources of variability. It is also possible that improvements and these ultrasounds were read independently by three radiologists. k in neonatal care, leading to a decrease in the severity of chronic lung coefficients were calculated to assess variability in identification of disease and less exposure to diuretic therapy, may have contributed to nephrocalcinosis among the radiologists. a change in the incidence of this lesion. This study was designed to examine the hypothesis that renal RESULTS: ultrasounds performed using a 7.5-MHz transducer are associated The k coefficient (6 confidence intervals) using a 5.0-MHz transducer with less inter-observer variability compared with a 5.0-MHz trans- was 0.143 (0.108, 0.178); using the 7.5-MHz transducer, the k coefficient ducer in the identification of neonatal nephrocalcinosis. was 0.268 (0.243, 0.293). All three radiologists agreed in their identifica- tion of nephrocalcinosis on 3 of 54 ultrasounds using a 5.0-MHz trans- METHODS ducer; a total of 6 of 54 ultrasounds obtained using a 7.5-MHz transducer Patients were read as positive by all three radiologists. This prospective study took place between August 1, 1995 and July 31, 1996 at three neonatal intensive care units. All infants with a birth weight ,1500 gm were considered eligible for the study. Infants were not included in the study for the following reasons: failure to obtain Departments of Pediatrics (T. C., G. R.), Radiology (F. H.), and Medicine (G. B.), Baystate Medical Center, Springfield, MA; Departments of Pediatrics (T. C., G. R.), Radiology (F. H.), informed consent from parents, transfer of the infant to another facil- and Medicine (G. B.), Tufts University School of Medicine, Boston, MA; Division of Neonatol- ity before images could be obtained, or the appearance of hydrone- ogy (F. J. B.), Memorial Health Care, University of Massachusetts Medical Center, Worcester MA; Department of Pediatrics and Obstetrics and Gynecology (F. J. B.), University of Massachu- phrosis or other congenital renal abnormalities on the ultrasound setts School of Medicine (A. P.), Worcester MA; Departments of Pediatrics (M. P.) and Radiology images. In addition, some infants could not be included in the study (J. Z.), University of Connecticut Health Center, Farmington, CT; Diagnostic Radiology (J. W.), Memorial Health Care, Worcester, MA; Department of Nutrition, University of Massachusetts, because one of the ultrasound transducers was broken or unavailable Amherst, MA; and Ross Products Division, Abbott Laboratories, Columbus, OH. when the images were obtained. Renal ultrasounds were performed at This work was supported by Ross Products Division of Abbott Laboratories. 7 to 8 weeks of age using both a 5.0-MHz sector transducer and a Address correspondence and reprint requests to Thomas Campfield, MD, Baystate Medical 7.5-MHz linear array transducer. The ultrasound equipment used in Center, Newborn Medicine, W2810, Springfield MA 01199. this study included a Toshiba SSH 140 A (Tokyo, Japan) and an ATL Journal of Perinatology (1999) 19(7) 498–500 © 1999 Stockton Press. All rights reserved. 0743–8346/99 $12 498 http://www.stockton-press.co.uk Ultrasound Detection of Nephrocalcinosis Campfield et al. 400 Apogee (Bothell, WA). Three longitudinal and three transverse views of each kidney were obtained. Ultrasound images were obtained at the bedside by radiologists and ultrasound technicians, but their bedside impressions of these images were not included in this study. Patient confidentiality was maintained by covering the patient’s name on the ultrasound hard copy image. Image Interpretation Hard copy images of each renal ultrasound were interpreted indepen- dently by three radiologists. Of the three radiologists involved in the study, two had training in ultrasonography; one radiologist had train- ing in pediatric radiology as well as ultrasonography. These radiolo- gists were not aware of the infant’s clinical condition, and they were unaware of other radiologists’ interpretation of these images. The only images shown to these radiologists were those described above; if other views were obtained because of a suspected abnormality, these Figure 1. k coefficients (6 confidence intervals) based on the presence or ab- other images were not included in the study. These criteria were estab- sence of nephrocalcinosis on ultrasounds on 54 premature infants, obtained with 5.0- and 7.5-MHz transducers, read independently by three radiologists. lished as part of the study to minimize bias in interpretation. Images were interpreted as normal or positive for nephrocalcinosis based on the presence of echogenic foci with acoustic shadowing. Positive read- ings were classified as “focal” or “diffuse” nephrocalcinosis. For the obtained using a 5.0-MHz transducer was 0.143 (0.108, 0.178), k purposes of this study, diffuse nephrocalcinosis was defined as whereas a 7.5-MHz transducer resulted in a value of 0.268 (0.243, nephrocalcinosis involving .50% of both kidneys. Nephrocalcinosis 0.293). Thus, ultrasounds obtained using a 7.5-MHz transducer re- involving ,50% of one or both kidneys was classified as focal sulted in significantly less variability in interpretation compared with nephrocalcinosis. a 5.0-MHz transducer (Figure 1). The percentage of ultrasounds interpreted as positive by individ- Data Analysis ual radiologists using a 5.0-MHz transducer was 28%, 6%, and 35% Interobserver variability in the diagnosis of nephrocalcinosis was for radiologists 1, 2, and 3, respectively. The percentage of ultrasounds k k measured using the coefficient. The coefficient measures agree- interpreted as positive using a 7.5-MHz transducer was 35%, 11%, and ment among observers above that caused by chance alone. In general, 39% for radiologists 1, 2, and 3, respectively (Figure 2). These propor- k values between 0.00 and 0.20 indicate minimal agreement; at the tions were significantly different for both transducers by x-squared k other extreme, values between 0.80 and 1.00 indicate nearly perfect analysis. Because radiologists 1 and 3 were similar with regard to k agreement. values were calculated for both the 5.0-MHz and 7.5- their percentage of positive readings, their image interpretations were MHz transducers, and confidence intervals were used to determine compared using the k statistic. This comparison gave a value of 0.433 whether one transducer gave results significantly less variable than with the 5.0-MHz transducer and a k value of 0.400 with the 7.5-MHz the other. transducer, reflecting only moderate agreement between these two To determine whether the variability observed in ultrasound observers. In other words, although the percentages of positive read- interpretation could be attributed to “over-reading” or “under-read- ings were similar, different images were interpreted as positive fre- ing” by an individual radiologist, the proportion of positive interpre- quently enough to show only moderate agreement using the k tations for each transducer by each radiologist was determined. These statistic. x proportions were compared using the -squared analysis. The proportion of ultrasounds interpreted as positive by all three To determine whether either transducer was associated with more radiologists using the 5.0-MHz transducer was 3 of 54 ultrasounds frequent identification of nephrocalcinosis, the proportion of ultra- (5.6%), whereas 6 of 54 ultrasounds (11.1%) were interpreted as posi- sounds read as positive for nephrocalcinosis by all three radiologists tive by all three radiologists using a 7.5-MHz transducer. Although was determined for each transducer, and these proportions were then there was a trend toward more uniformly positive image interpreta- compared using Fisher’s exact test. tion with the 7.5-MHz transducer, this difference did not achieve sig- nificance (Fisher’s exact test). The three ultrasounds read as abnor- RESULTS mal with the 5.0-MHz transducer were also uniformly read as A total of 54 patients were studied. The mean birth weight of these 54 abnormal by all three radiologists with the 7.5-MHz transducer. infants was 976 6 28 gm (mean 6 SEM) and the mean gestational There were a total of 38 positive readings among all three radiol- age was 27.1 6 0.3 weeks; ultrasounds were performed at 49.7 6 1.8 ogists using the 5.0-MHz transducer; only 2 of these 38 (5.3%) were days of age. interpreted as showing diffuse rather than focal nephrocalcinosis. The k statistic (6 confidence intervals) for renal ultrasounds Similarly, of the 46 positive image interpretations with the 7.5-MHz Journal of Perinatology (1999) 19(7) 498–500 499 Campfield et al.
Recommended publications
  • An Unusually Dry Story
    555050 Case Report An unusually dry story Srinivas Rajagopala, Gurukiran Danigeti, Dharanipragada Subrahmanyan We present a middle-aged woman with a prior history of central nervous system (CNS) Access this article online demyelinating disorder who presented with an acute onset quadriparesis and respiratory Website: www.ijccm.org failure. The evaluation revealed distal renal tubular acidosis with hypokalemia and DOI: 10.4103/0972-5229.164808 medullary nephrocalcinosis. Weakness persisted despite potassium correction, and ongoing Quick Response Code: Abstract evaluation confi rmed recurrent CNS and long-segment spinal cord demyelination with anti-aquaporin-4 antibodies. There was no history of dry eyes or dry mouth. Anti-Sjogren’s syndrome A antigen antibodies were elevated, and there was reduced salivary fl ow on scintigraphy. Coexistent antiphospholipid antibody syndrome with inferior vena cava thrombosis was also found on evaluation. The index patient highlights several rare manifestations of primary Sjogren’s syndrome (pSS) as the presenting features and highlights the differential diagnosis of the clinical syndromes in which pSS should be considered in the Intensive Care Unit. Keywords: Acute demyelinating encephalomyelitis, distal renal tubular acidosis, hypokalemic paralysis, nephrocalcinosis, neuromyelitis optica, Sjogren’s syndrome Introduction was diagnosed with postviral encephalomyelitis 3 years ago and treated with steroids with complete resolution of Primary Sjogren’s syndrome (pSS) is a relatively symptoms and signs. She had normal menstrual cycles common autoimmune disease affecting 2–3% of the adult and had one spontaneous second trimester abortion population. It is characterized by lymphocyte infi ltration 8 years ago. She had one living child and had undergone and destruction of exocrine glands.
    [Show full text]
  • Diagnostic Imaging and Risk Factors Downloaded from by EERP/BIBLIOTECA CENTRAL User on 26 August 2019
    ISSN 2472-1972 Nephrocalcinosis and Nephrolithiasis in X-Linked Hypophosphatemic Rickets: Diagnostic Imaging and Risk Factors Downloaded from https://academic.oup.com/jes/article-abstract/3/5/1053/5418933 by EERP/BIBLIOTECA CENTRAL user on 26 August 2019 Guido de Paula Colares Neto,1,2 Fernando Ide Yamauchi,3 Ronaldo Hueb Baroni,3 Marco de Andrade Bianchi,4 Andrea Cavalanti Gomes,4 Maria Cristina Chammas,4 and Regina Matsunaga Martin1,2 1Department of Internal Medicine, Division of Endocrinology, Osteometabolic Disorders Unit, Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-900 S~ao Paulo, SP, Brazil; 2Department of Internal Medicine, Division of Endocrinology, Laborat´orio de Hormoniosˆ e Gen´etica Molecular (LIM/42), Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-900 S~ao Paulo, SP, Brazil; 3Department of Radiology and Oncology, Division of Radiology, Computed Tomography Unit, Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-001 S~ao Paulo, SP, Brazil; and 4Department of Radiology and Oncology, Division of Radiology, Ultrasound Unit, Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-001 S~ao Paulo, SP, Brazil ORCiD numbers: 0000-0003-3355-0386 (G. P. Colares Neto); 0000-0002-4633-3711 (F. I. Yamauchi); 0000-0001-7041-3079 (M. C. Chammas). Context: Nephrocalcinosis (NC) and nephrolithiasis (NL) are described in hypophosphatemic rickets, but data regarding their prevalence rates and the presence of metabolic risk factors in X-linked hypophosphatemic rickets (XLH) are scarce.
    [Show full text]
  • Radiological Imaging of the Kidney
    Medical Radiology / Diagnostic Imaging Radiological Imaging of the Kidney von Emilio Quaia 1st Edition. Springer 2010 Verlag C.H. Beck im Internet: www.beck.de ISBN 978 3 540 87596 3 schnell und portofrei erhältlich bei beck-shop.de DIE FACHBUCHHANDLUNG Contents Part I Embryology and Anatomy 1 Embryology of the Kidney .................................... 3 Marina Zweyer 2 Normal Radiological Anatomy and Anatomical Variants of the Kidney . 17 Emilio Quaia, Paola Martingano, Marco Cavallaro, and Roberta Zappetti 3 Normal Radiological Anatomy of the Retroperitoneum ............ 79 Emilio Quaia Part II Imaging and Interventional Modalities 4 Ultrasound of the Kidney . 87 Emilio Quaia 5 Computed Tomography. 129 5.1 General Concepts........................................ 129 Emilio Quaia, Paola Martingano, and Marco Cavallaro 5.2 Multidetector CT Urography and CT Angiography . 160 Roberto Pozzi Mucelli, Giulia Zamboni, Livia Bernardin, and Alberto Contro 6 Magnetic Resonance Imaging of the Kidney . 179 Maria Assunta Cova, Marco Cavallaro, Paola Martingano, and Maja Ukmar 7 Renal Angiography and Vascular Interventional Radiology . 197 Fabio Pozzi-Mucelli and Andrea Pellegrin 8 Nuclear Medicine . 229 Egesta Lopci and Stefano Fanti xi xii Contents 9 The Role of Kidney Biopsy in the Diagnosis of Renal Disease and Renal Masses............................. 257 Michele Carraro and Fulvio Stacul 10 Nonvascular Interventional Radiology Procedures ................ 271 Raul N. Uppot Part III Non-Tumoral Pathology 11 Congenital and Development Disorders of the Kidney ............. 291 Veronica Donoghue 12 Renal Cystic Disease ......................................... 311 Kyongtae T. Bae, Alessandro Furlan, and Fadi M. El-Merhi 13 Renal Parenchymal and Inflammatory Diseases . 339 Emilio Quaia 14 Obstructive Uropathy, Pyonephrosis, and Reflux Nephropathy in Adults . 357 Emilio Quaia, Paola Martingano, and Marco Cavallaro 15 Nephrocalcinosis and Nephrolithiasis ..........................
    [Show full text]
  • Urogenital Radiology ESUR 2012 Gratefully Acknowledges the Support of the Following Sponsors
    British Society of Urogenital Radiology ESUR 2012 gratefully acknowledges the support of the following sponsors Main Sponsors Other Sponsors Our thanks also to www.esur2012.org __________________________________________________________________________________ ESUR – BSUR 2012 19th European Symposium on Urogenital Radiology and 7th BSUR Annual Scientific Meeting Congress Chairman: Sami Moussa (UK) Scientific Programme Committee President ESUR: Gertraud Heinz‐Peer (AT) Chairman: Sami Moussa (UK) Chairman BSUR: Phil Cook (UK) Boris Brkljacic (HR) SAR Honorary Lecture: Stuart Silverman (US) Michel Claudon (FR) Phil Cook (UK) MAIN TOPICS: Imaging and Management of Stone Nigel Cowan (UK) Disease Lorenzo Derchi (IT) Vikram Dogra (US) ACCREDITATION Nicolas Grenier (FR) CPD accreditation has been awarded by the Royal Gertraud Heinz‐Peer (AT) College of Radiologists as follows: Vibeke Løgager (DK Thursday 13 September (Members’ Day): 3 Sameh Morcos (UK) Friday 14 September: 7 Parvi Ramchandani (US) Saturday 15 September: 7 Michael Riccabona (AT) Sunday 16 September: 4 John Spencer (UK) Harriet Thoeny (CH) A total of 15 European CME credits (ECMEC) have Ahmet Turgut (TR) been awarded by the European Accreditation Council for Continuing Medical Education Local Committee (EACCME). Sami Moussa Julian Keanie VENUE: John Brush Surgeons’ Hall, Royal College of Surgeons of Sameh Morcos Edinburgh, Nicolson Street, Edinburgh EH8 9DW Edinburgh, UK LOCAL CONGRESS ORGANISER Intelligent Events Limited www.intel‐events.co.uk Page | 1 www.esur2012.org __________________________________________________________________________________
    [Show full text]
  • RENAL IMPAIRMENT in SARCOIDOSIS with SPECIAL REFERENCE to NEPHROCALCINOSIS by K
    Postgrad Med J: first published as 10.1136/pgmj.31.360.516 on 1 October 1955. Downloaded from 5I6 ARENAL IMPAIRMENT IN SARCOIDOSIS WITH SPECIAL REFERENCE TO NEPHROCALCINOSIS By K. M. CITRON, M.D.(Lond.), M.R.C.P. Senior Medical Registrar, Brompton Hospital Introduction Case Record* Although impaired renal function is uncommon Miss I.E., aged 23, had in 1948 commenced in sarcoidosis the recognition of this complication work in a factory where she was engaged in coating is of importance since it may cause death and be- the inside of fluorescent tubes with a mixture con- cause prompt treatment may result in recovery of taining beryllium phosphor. This exposure to renal function. beryllium lasted one year. A chest radiograph at The kidney is a common site for sarcoid lesions. the end of this time was stated to be normal. Thus, in a combined series of 45 autopsies the However, ten months later, in April 1953, she kidneys showed macroscopic or microscopic in- attended the Brompton Hospital complaining of volvement in 20 per cent. (Ricker and Clark, I949; dry cough and dyspnoea on exertion. Physical Longcope and Freiman, I952). In view of the examination at this time showed no abnormalityby copyright. known tendency of sarcoid lesions to infiltrate but the chest radiograph showed miliary mottling organs extensively and impair function, it was in both lungs and enlarged hilar shadows (Fig. i). assumed by earlier authors that renal impairment The Mantoux reaction was negative i :ioo O.T. was due to massive invasion of the kidneys (Kline- and the E.S.R.
    [Show full text]
  • Nephrocalcinosis and Nephrolithiasis in X-Linked Hypophosphatemic Rickets: Diagnostic Imaging and Risk Factors
    ISSN 2472-1972 Nephrocalcinosis and Nephrolithiasis in X-Linked Hypophosphatemic Rickets: Diagnostic Imaging and Risk Factors Guido de Paula Colares Neto,1,2 Fernando Ide Yamauchi,3 Ronaldo Hueb Baroni,3 Marco de Andrade Bianchi,4 Andrea Cavalanti Gomes,4 Maria Cristina Chammas,4 and Regina Matsunaga Martin1,2 1Department of Internal Medicine, Division of Endocrinology, Osteometabolic Disorders Unit, Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-900 S~ao Paulo, SP, Brazil; 2Department of Internal Medicine, Division of Endocrinology, Laborat´orio de Hormoniosˆ e Gen´etica Molecular (LIM/42), Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-900 S~ao Paulo, SP, Brazil; 3Department of Radiology and Oncology, Division of Radiology, Computed Tomography Unit, Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-001 S~ao Paulo, SP, Brazil; and 4Department of Radiology and Oncology, Division of Radiology, Ultrasound Unit, Hospital das Cl´ınicas da Faculdade de Medicina da Universidade de S~ao Paulo, 05403-001 S~ao Paulo, SP, Brazil ORCiD numbers: 0000-0003-3355-0386 (G. P. Colares Neto); 0000-0002-4633-3711 (F. I. Yamauchi); 0000-0001-7041-3079 (M. C. Chammas). Context: Nephrocalcinosis (NC) and nephrolithiasis (NL) are described in hypophosphatemic rickets, but data regarding their prevalence rates and the presence of metabolic risk factors in X-linked hypophosphatemic rickets (XLH) are scarce. Objective: To determine the prevalence rates of NC and NL and their risk factors in patients with XLH with confirmed PHEX mutations.
    [Show full text]
  • Risk Factors and Outcome of Nephrocalcinosis in Very Low Birth Weight Infants
    대 한 주 산 회 지 제26권 제1호, 2015 � Original Article � Korean J Perinatol Vol.26, No.1, Mar., 2015 http://dx.doi.org/10.14734/kjp.2015.26.1.35 Risk Factors and Outcome of Nephrocalcinosis in Very Low Birth Weight Infants Ho Sung Kim, M.D., Kumi Jeong, M.D., Young Youn Choi, M.D., Ph.D., Eun Song Song, M.D., Ph.D. Department of Pediatrics, Chonnam National University Hospital, Gwangju, Korea Purpose: The aim of this study was to determine the incidence, risk factors, and long-term outcome of nephrocalcinosis in very low birth weight (VLBW) infants. Methods: A retrospective chart review was performed in VLBW infants between 2006 and 2012 in the neonatal intensive care unit. Results: The incidence of nephrocalcinosis in VLBW infants was 10.2%. By univariate analysis, oligohydramnios and use of antenatal steroids were more frequent in the nephrocalcinosis group. In the nephrocalcinosis group, the gestational age and birth weight were lower and there were more number of female infants. Also, the initial blood pH, the lowest systolic blood pressure, and urine output on the first day of life were lower and bronchopulmonary dysplasia, sepsis, and urinary tract infection were more prevalent in the nephrocalcinosis group. The use of dexamethasone or ibuprofen and the lowest levels of phosphorus, protein and albumin were significantly lower in the nephrocalcinosis group. By binary logistic regression analysis, the use of antenatal steroids, female sex, 5-minute Apgar score, duration of oxygen therapy and total parenteral nutrition, and the lowest albumin level were found to be significant risk factors for nephrocalcinosis.
    [Show full text]
  • Understanding the Pathophysiology of Nephrocalcinosis
    Chapter 1 Understanding the Pathophysiology of Nephrocalcinosis Giovanna Priante, Monica Ceol, Liliana Terrin, Lisa Gianesello, Federica Quaggio, Dorella Del Prete and Franca Anglani Additional information is available at the end of the chapter http://dx.doi.org/10.5772/intechopen.69895 Abstract Many in vitro and in vivo studies on the mechanisms underlying calcium nephrolithiasis have provided evidence of a frequently associated condition, i.e., a microscopic renal crys‐ tal deposition that can occur within the tubular lumen (intratubular nephrocalcinosis) or in the interstitium (interstitial nephrocalcinosis). Medullary nephrocalcinosis is the typi‐ cal pattern seen in 98% of cases of human nephrocalcinosis, with calcification clustering around each renal pyramid. It is common in patients with metabolic conditions that pre‐ dispose them to renal calcium stones. Cortical nephrocalcinosis is rare and usually results from severe destructive disease of the cortex. It has been described in chronic glomerulone‐ phritis, but often in association with another factor, such as an increased calcium ingestion, acute cortical necrosis, chronic pyelonephritis or trauma. The most accredited hypothesis to explain the onset of interstitial nephrocalcinosis is purely physicochemical, relating to spontaneous Ca2PO4 crystallization in the interstitium due to oversaturation of Ca2PO4salts in this milieu. The theory that nephrocalcinosis is a process driven by osteogenic cells was first proposed by our group. We review nephrocalcinosis in terms of its definition, genetic associations, and putative mechanisms, pointing out how much evidence in the literature suggests that it may have some features in common with, and pathogenic links to vascular calcification. Keywords: nephrocalcinosis, genetics, Randall’s plaque, calcium crystals, vascular calcification, osteogenic transdifferentiation © 2017 The Author(s).
    [Show full text]
  • UNILATERAL MEDULLARY SPONGE KIDNEY FIG. 1 Multiple Right
    Case Report doi: 10.22374/jeleu.v1i1.12 UNILATERAL MEDULLARY SPONGE KIDNEY Rodrigo Neira S, Diego Barrera C, Gastón Astroza E Facultad de Medicina, Pontifi cia Universidad Católica de Chile, Santiago, Chile. Corresponding Author Gastón Astroza E, MD: gaeulufi @gmail.com Submitted: May 15, 2018. Accepted: July 5, 2018. Published: August 21, 2018. ABSTRACT A 40-year-old female with an allergy to medium contrast, presented with bilateral loin pain and renal colic type radiation. Image study with non-contrast computerized tomography and magnetic resonance imaging revealed multiple nephrolithiasis and a complex cyst at lower pole of the right kidney and no findings in the left one. The patient was treated with laser lithotripsy by flexible ureterorenoscopy revealing multiple lithiasis within cystic dilatations of the urothelium. The complex cyst was submitted to partial nephrec- tomy. Finally, biopsy revealed renal parenchyma with polycystic changes in the external corticomedullary area. All findings were compatible with unilateral medullary sponge kidney diagnosis. CLINICAL CASE A 40-year-old female patient presented with a his- tory of insulin resistance (treated with metformin), allergy to contrast medium, and maternal urolithia- sis. The patient consulted for bilateral loin pain and right renal colic pain. Non-contrast computerized tomography (NCCT) (Figures 1 and 2) demonstrated multiple right nephrolithiasis and a 3-cm hypodense right lower pole lesion with some calcifications. This was diagnosed as a complex cyst. Related to this cyst, a magnetic resonance imaging (MRI) of the abdo- men was requested which showed a 3.5 cm Bosniak 3 right renal cyst. Resolution of the lithiasis was decided by means of flexible ureterorenoscopy (fURS) with laser lithotripsy.
    [Show full text]
  • Guidelines on Urolithiasis
    GUIDELINES ON UROLITHIASIS (Update March 2011) C. Türk (chairman), T. Knoll (vice-chairman), A. Petrik, K. Sarica, C. Seitz, M. Straub Epidemiology Between 120 and 140 per 1000,000 will develop urinary stones each year with a male/female ratio of 3:1. A number of known factors of influence to the development of stones are discussed in more detail in the extended version of the Urolithiasis guidelines. Classification of stones Correct classification of stones is important since it will impact treatment decisions and outcome. Urinary stones can be classified according to the follow- ing aspects: stone size, stone location, X-ray characteristics of stone, aetiology of stone formation, stone composition (mineralogy), and risk group for recurrent stone formation (Tables 1-3). Urolithiasis 289 Table 1: X-ray characteristics Radiopaque Poor radiopaque Radiolucent Calcium oxalate Magnesium Uric acid dihydrate ammonium phosphate Calcium oxalate Apatite Ammonium urate monohydrate Calcium Cystine Xanthine phosphates 2,8-dihydroxy- adenine ‘Drug-stones’ Table 2: Stones classified according to their aetiology Non- Infection Genetic Drug stones infection stones stones stones Calcium Magnesium- Cystine Indinavir oxalates ammonium- (see extended phosphate document) Calcium Apatite Xanthine phosphates Uric acid Ammonium 2,8-dihydro- urate xyadenine 290 Urolithiasis Table 3: Stones classified by their composition Chemical composition Mineral Calcium oxalate monohydrate whewellite Calcium-oxalate-dihydrate wheddelite Uric acid dihydrate uricite Ammonium urate Magnesium ammonium phosphate struvite Carbonate apatite (phosphate) dahllite Calcium hydrogenphosphate brushite Cystine Xanthine 2,8-dihydroxyadenine ‘Drug stones’ unknown composition. Risk groups for stone formation The risk status of a stone former is of particular interest as it defines both probability of recurrence or (re)growth of stones and is imperative for pharmacological treatment (Table 4, Figure 1).
    [Show full text]
  • Fourteen Monogenic Genes Account for 15% of Nephrolithiasis/Nephrocalcinosis
    BRIEF COMMUNICATION www.jasn.org Fourteen Monogenic Genes Account for 15% of Nephrolithiasis/Nephrocalcinosis † †‡ ‡ Jan Halbritter,* Michelle Baum,* Ann Marie Hynes, Sarah J. Rice, David T. Thwaites, Zoran S. Gucev,§ Brittany Fisher,* Leslie Spaneas,* Jonathan D. Porath,* Daniela A. Braun,* | † Ari J. Wassner, Caleb P. Nelson,¶ Velibor Tasic,§ John A. Sayer, and Friedhelm Hildebrandt*** *Division of Nephrology, Department of Medicine, |Division of Endocrinology, Department of Medicine, and ¶Department of Urology, Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts; †Institute of Genetic Medicine, International Centre for Life and ‡Epithelial Research Group, Institute for Cell and Molecular Biosciences, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom; §Medical Faculty Skopje, University Children’s Hospital, Skopje, Macedonia; and **Howard Hughes Medical Institute, Chevy Chase, Maryland ABSTRACT Nephrolithiasis is a prevalent condition with a high morbidity. Although dozens of the overall population of stone formers. monogenic causes have been identified, the fraction of single-gene disease has not been Furthermore, absence of a positive family well studied. To determine the percentage of cases that can be molecularly explained by history, which will be the rule in recessive mutations in 1 of 30 known kidney stone genes, we conducted a high-throughput genes and may be frequent in dominant mutation analysis in a cohort of consecutively recruited patients from typical kidney stone genes with incomplete penetrance, often clinics. The cohort comprised 272 genetically unresolved individuals (106 children and leads to the false assumption that a mono- 166 adults) from 268 families with nephrolithiasis (n=256) or isolated nephrocalcinosis genic cause is unlikely.
    [Show full text]
  • Aetiology, Diagnosis and Clinical Characteristics of Nephrocalcinosis
    Review Article Clinician’s corner Images in Medicine Experimental Research Case Report Miscellaneous Letter to Editor DOI: 10.7860/JCDR/2018/32270.11317 Original Article Postgraduate Education Aetiology, Diagnosis and Clinical Case Series Characteristics of Nephrocalcinosis Paediatrics Section Short Communication LIDVANA SPAHIU1, ARBNORE BATALLI KËPUSKA2, VLORA ISMAILI JAHA3, BESART MEROVCI4, HAKI JASHARI5 ABSTRACT children. Clinical manifestations were failure to thrive in 8 (32%), Introduction: Nephrocalcinosis (NC) is the increased deposition polyuria and polydipsia in 5 (20%), haematuria and renal colic in of calcium in the renal parenchyma due to different aetiologies. 2 (8%), urinary tract infection in 7 (28%) and in 3 (12%) cases, NC was found accidentally during routine checkup. Chronic Aim: The present study aimed to investigate the aetiology of NC renal insufficiency developed in two patients with dRTA and in and its effect on renal function and growth in children. one with hyperoxaluria. The degree of NC worsened in 3 (23%) Materials and Methods: In the present study, 25 children patients, remained the same in 7 (54%) and improved in 3 (23%) diagnosed with NC between 1999 and 2016 were retrospectively patients. However, the effect of nephrocalcinosis grade change analysed. Relevant demographic, clinical and laboratory data on renal function and growth was not significant. were extracted from patients’ records. Conclusion: It is not the degree of NC rather the underlying Results: The median (range) age at diagnosis was 9 (3-84) months. cause and the age at diagnosis that affects the growth and renal The leading cause of NC was distal Renal Tubular Acidosis (dRTA) function of children.
    [Show full text]