[17]. Without treatment, MSUD can lead to feeding difficulties, lethargy, seizures, urine and cerumen that smell like maple syrup, vomiting, coma, and death [5]. Quental S, Macedo-Ribeiro S, Matos R, Vilarinho L, Martins E, Teles EL, et al. 2004 Feb. 163(2):76-80. Pediatrics. At times a peculiar maple syrup smell in the urine or sweat can occur in older, healthy children or adults who are non-symptomatic. [18] A 2012 study reported that patients who were mentally impaired prior to transplantation had no change in their neurocognitive function one year later. KW - BCKDHA. This process is experimental and the keywords may be updated as the learning algorithm improves. Share cases and questions with Physicians on Medscape consult. Maple syrup urine disease (MSUD) is an autosomal recessive disorder which can be caused by mutation in at least three genes. When patients with MSUD get sick, have fever, cannot keep food down due to vomiting or diarrhea or when amino acids rise to dangerous levels, the patient must be hospitalized immediately. J Pediatr. msud-maxamaid-344609 Pediatr Res. E3-deficient MSUD. ? Complications of maple syrup urine disease include: Last reviewed by a Cleveland Clinic medical professional on 11/18/2019. The four main types of MSUD are: Classic: Classic maple syrup urine disease is the most severe type of MSUD. J Nutr. In rare circumstances, hemodialysis or peritoneal dialysis is required to remove BCAAs and keto acids. The goals of medical nutrition therapy in maple syrup urine disease are multifaceted, as follows: There are hundreds of different genetic metabolic disorders, and their symptoms, treatments, and prognoses vary widely. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTQ2MjM0LXRyZWF0bWVudA==, To rapidly reduce toxic metabolites by restricting dietary BCAAs to amounts allowing patients to achieve and maintain plasma BCAA concentrations within the targeted treatment ranges, To monitor nutritional status and alter intake to promote and sustain normal growth, To enable normal development and health maintenance, To evaluate thiamine responsiveness if the patient has residual BCKD activity and to administer thiamine supplements if the patient is responsive. This means that Maple syrup urine disease, or a subtype of Maple syrup urine disease, affects less than 200,000 people in the US population. msud-maxamum-344610 Maple syrup urine disease (MSUD) is a rare genetic disorder characterized by deficiency of an enzyme complex (branched-chain alpha-keto acid dehydrogenase) that is required to break down (metabolize) the three branched-chain amino acids (BCAAs) leucine, isoleucine and valine, in the body. Frazier DM, Allgeier C, Homer C, Marriage BJ, Ogata B, Rohr F, et al. TREATMENT of the episode of acute metabolic decompensation in maple syrup urine disease (MSUD) is a medical emergency. Maple syrup urine disease: branched-chain keto-aciduria. However, its administration is ineffective in cases of gastric intolerance and some adult patients refuse enteral feeding via a nasogastric tube. The most common and severe form of this disease is the classic type, which appears soon after birth, and as long as it remains untreated, gives rise to progressive and unremitting symptoms. The name of the disease comes from the maple syrup smell of the urine in people with this condition. Scriver CR, Beaudet AL, Valle DL, Sly WS, eds. Maple syrup urine disease. Accumulation of these 3 amino acids and their corresponding keto acids leads to encephalopathy and progressive neurodegeneration in untreated infants. Complications of acute elevation in plasma leucine include ketoacidosis and risk of cerebral edema, which can be fatal. Heldt K, Schwahn B, Marquardt I, et al. Maple syrup urine disease Disease definition A rare inherited disorder of branched-chain amino acid metabolism classically characterized by poor feeding, lethargy, vomiting and a maple syrup odor in the cerumen (and later in urine) noted soon after birth, followed by progressive encephalopathy and central respiratory failure if untreated. 2005. Maple syrup urine disease (MSUD) is an aminoacidopathy secondary to an enzyme defect in the catabolic pathway of the branched-chain amino acids leucine, isoleucine, and valine. Pediatrics. [19]. Liver transplantation may guarantee normal or near-normal neurological outcomes if performed early following diagnosis. Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder, in which the defect in the Branched Chain Ketoacid Dehydrogenase (BCKD) enzyme causes irregular amino acid metabolism. 2002 Jun. This leads to accumulation of protein in the body. [16, 7, 14] Consultation with a neonatal/pediatric nutritionist with expertise in dietary management of metabolic disorders is required to address medical nutrition therapy immediately. Introduction. Filter the person’s blood plasma and return it to their body (a procedure called hemofiltration/dialysis) to lower the level of the three amino acids. [19] Certainly, the objective is to prevent the need for liver transplantation in newborns diagnosed with maple syrup urine disease by immediately fostering early dietary intervention of BCAAs restriction. At around 5 days old, babies are offered newborn blood spot screening to check if they have MSUD. Accessed 11/14/2019. Side effects of hemodialysis and hemofiltration include muscle cramps, low blood pressure (hypotension) and changes in blood electrolyte contents that will need close monitoring. Ann Clin Lab Sci. [Medline]. Doctor Top 20. Olaf A Bodamer, MD, PhD, FAAP, FACMG is a member of the following medical societies: American Medical Association, American Society of Human GeneticsDisclosure: Nothing to disclose. Brendan Lee, MD, PhD Professor, Robert and Janice McNair Endowed Chair in Molecular and Human Genetics, Department of Molecular and Human Genetics, Baylor College of Medicine Maple syrup urine disease (MSUD) is a genetic disorder and is caused by changes or mutations in some genes that signal the body to produce certain enzymes necessary for digestion and metabolism. The other names for this condition are as follows. BIMDG_ADULT-MSUD_Revision2018 1 PATIENT NAME HOSPITAL DATE OF BIRTH EMERGENCY CONTACT 9-5pm Monday to Friday Out of hours ADULT EMERGENCY MANAGEMENT MAPLE SYRUP URINE DISEASE (MSUD) BACKGROUND MSUD is a disorder affecting the breakdown of branched chain amino acids (BCAA = Leucine, Isoleucine & Valine). Give glucose and insulin through a vein (using an IV) to adjust the level of amino acids in the body. Schadewaldt P, Bodner-Leidecker A, Hammen HW, Wendel U. If maple syrup urine disease is diagnosed, treatment can be given straight away to reduce the risk of serious complications. New York, NY: McGraw-Hill Co; 2000. Water apparent diffusion coefficient and T2 changes in the acute stage of maple syrup urine disease: evidence of intramyelinic and vasogenic-interstitial edema. 1999 Dec. 158 Suppl 2:S60-4. [Medline]. Maple syrup urine disease is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). 2005 Jun. [Medline]. With a new liver, people with MSUD can produce the enzymes they need to break down the three amino acids that build up in body. CORONAVIRUS: DELAYS FOR ROUTINE SURGERIES, VISITOR RESTRICTIONS + COVID-19 TESTING. The genetic defect that produces MSUD results in a defect in the enzyme called branched-chain alpha-keto acid dehydrogenase (BCKD), which is necessary for the breakdown of the amino acids leucine, isoleucine, and valine. Lifelong nutritional guidance is imperative. Christian J Renner, MD Consulting Staff, Department of Pediatrics, University Hospital for Children and Adolescents, Erlangen, Germany Disclosure: Nothing to disclose. Clinically known as branched chain ketoaciduria, maple syrup urine disease is a rare genetic disorder. Seek dietary guidance with a nutritionist knowledgeable in dietary management of metabolic disorders. KW - Newborn screening Whenever possible, continue additional dietary support, including lipids and/or nutrition free of BCAAs. For patients with maple syrup urine disease, the intake of leucine is calculated on an individual basis following measurement of plasma BCAAs. Posted Mar 12, 2017 by Oliver 1270. [Medline]. The disease prevents your body from breaking down certain amino acids. Maple Syrup Urine Disease (MSUD) is a genetic disorder caused by a deficiency in alpha-ketoacid dehydrogenase, an enzyme needed to break down branch chain amino acids. 84(4):313-6. Previous. In addition to dietary therapy, consider thiamine (10-20 mg/d) for 4 weeks to determine thiamine responsiveness. Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with lifethreatening cerebral oedema in affected individuals. What are the types of maple syrup urine disease (MSUD)? Advertising on our site helps support our mission. If not appropriately treated, this disease can lead to neurological problems. Background: In the rare inborn autosomal recessive disorder maple syrup urine disease (MSUD) the accumulation of the branched‐chain amino acids (BCAAs) and their metabolic products results in … Righini A, Ramenghi LA, Parini R, et al. Menkes JH, Hurst PL, Craig JM. Initial studies using retroviral vectors to infect maple syrup urine disease lymphocytes have shown stable correction of the enzyme deficiency. [Medline]. Follow up with the patient at regular intervals (ie, at least once every 6-12 mo) with a biochemical geneticist familiar with the management of maple syrup urine disease. 2002 Lancet. Source - National Institutes of Health (NIH) Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. 1. The two main approaches to the treatment of maple syrup urine disease (MSUD) include (1) long-term daily dietary management and (2) treatment of episodes of acute metabolic decompensation. All material on this website is protected by copyright, Copyright © 1994-2020 by WebMD LLC. Mol Genet Metab. 1(7694):310-2. Eur J Pediatr. These crises occur during the initial neonatal episode, during which most patients receive their diagnosis, and later following dietary indiscretion, … Several successful pregnancies in patients with maple syrup urine disease have been reported. J Pediatr. Fernstrom JD. These genes encode the components of the branched-chain alpha-keto acid dehydrogenase (BCKAD) complex, which catalyses the catabolism of the branched-chain amino acids (BCAAs), leucine, isoleucine and valine. Maple syrup urine disease (MSUD) is an inherited disorder of branched-chain amino acid metabolism presenting with lifethreatening cerebral oedema in affected individuals. This type is a very rare form of Maple Syrup Urine Disease. Harris RA, Joshi M, Jeoung NH, Obayashi M. Overview of the molecular and biochemical basis of branched-chain amino acid catabolism. A new study analyzes 30 years of patient data and details the clinical course of 184 individuals with genetically diverse forms of Maple Syrup Urine Disease (MSUD), which is … A new syndrome: progressive familial infantile cerebral dysfunction associated with an unusual urinary substance. Mol Genet Metab. It appears that, while liver transplantation cannot reverse the neurological damage that has already occurred, it can prevent additional episodes of decompensation and preserve the remaining neurological function. Monitor the patient for signs of brain swelling or infection and acid buildup. With improved understanding of the transplant process, patients can lead normal lives with good monitoring and without the fear of problems related to MSUD. Tailoring of the diet for the individual in maple syrup urine disease: long-term home dietary treatment of an adult patient with MSUD by monitoring of daily intake with a personal computer. Most infants with classic MSUD show subtle emerging symptoms within 2-3 days; these include poor feeding at bottle or breast and increasing lethargy and irritability. Policy, Cleveland Clinic is a non-profit academic medical center. This leads to accumulation of protein in the body. Advertising on our site helps support our mission. Is the odor also noticeable in perspir 41(2):167-73. The mainstay in the treatment of maple syrup urine disease is dietary restriction of branched-chain amino acids (BCAAs). 160:116-121. Maple syrup urine disease (MSUD) is an inherited disorder of metabolism of the branched-chain amino acids leucine, isoleucine, and valine. Summary. Dancis J, Levits M, Westall RG. Abnormal maple syrup odor (recognizable in ear wax before urine). [Medline]. After a liver transplant, people with MSUD can eat an unrestricted diet, live without symptoms, and avoid further cognitive problems. Successful domino liver transplantation in maple syrup urine disease using a related living donor. 2003 Nov. 22(5):417. [Medline]. In the hospital, doctors may: Since 2004, liver transplants have been very successful in treating individuals with classic MSUD. Is it possible for an adult to acquire the disease? In GeneReviews. The disease is characterized by urine that smells like maple syrup due to which this condition became the name of the disease. 2005 Jun. Diagnosis and treatment of maple syrup disease: a study of 36 patients. Mazariegos GV, Morton DH, Sindhi R, Soltys K, Nayyar N, Bond G, et al. Diagnosis of maple syrup urine disease. Brendan Lee, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American College of Medical Genetics and Genomics, Society for Pediatric ResearchDisclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Biomarin; Retrophin;. Having such defective genes may result in either non-production or mal-functioning of the related enzymes. Individuals with MSUD are at risk of meta … 1993-2016. Feier FH et al. Beginning in early infancy, this condition is characterized by poor feeding, vomiting, lack of energy (lethargy), seizures , and developmental delay . Pediatrics. 3. J Nutr. Mol Genet Metab. However, human gene therapy trials for maple syrup urine disease have yet to be performed. Germaine L Defendi, MD, MS, FAAP is a member of the following medical societies: American Academy of PediatricsDisclosure: Nothing to disclose. 2008 Jun. [Medline]. KW - BCKDHB. 2014;112(3):210–217. 2014 Jul. Inherited metabolic disorders are genetic conditions that result in metabolism problems. Hoffmann GF, von Kries R, Klose D, et al. 1. J Inherit Metab Dis. Snyderman SE, Norton PM, Roitman E, Holt LE Jr. Without medical management, maple syrup urine disease can lead to a wide range of intellectual and physical disabilities and death. It is also the most common. Thiamine Responsive Maple Syrup Urine Disease. KW - BCKDHB. Mol Genet Metab 2014 Jul;112(3)210-217. 2003 Apr. Servais A, Arnoux JB, Lamy C, et al. Wendel U, Saudubray JM, Bodner A, Schadewaldt P. Liver transplantation in maple syrup urine disease. The main symptom of maple syrup urine disease is the patient's urine may smell similar to maple syrup. KW - Maple syrup urine disease. 2005 Apr. 94(2):148-56. Next. Accessed 11/14/2019. Pediatrics: Genetics and Metabolic Disease, American College of Medical Genetics and Genomics. We do not endorse non-Cleveland Clinic products or services. There is considerable genetic heterogeneity due to various mutations that occur in the E1 alpha, E1 beta, E2 and E3 loci of the BCKAD complex. KW - Branched-chain amino acids. [Medline]. 1964 Oct. 34:454-72. If not treated at all, brain damage will occur, followed by death. Movement disorders in adult surviving patients with maple syrup urine disease. [Medline]. Three Korean patients with maple syrup urine disease: four novel mutations in the BCKDHA gene. Drugs, 2010 The Nutrition Management Guideline for Individuals with Maple Syrup Urine Disease (MSUD) is part of a larger project undertaken by the Southeast Newborn Screening and Genetics Collaborative (SERC) (HRSA Region 3) and Genetic and Metabolic Dietitians International (GMDI) to develop nutrition management guidelines for inherited metabolic disorders (IMD). Based on this clinical observation, we investigated the potential of phenylbutyrate treatment to lower BCAA and their corresponding α-keto acids (BCKA) in patients with classic and variant late-onset forms of maple syrup urine disease (MSUD). attention deficit/hyperactivity disorder (ADHD). Chuang DT. KW - Maple syrup urine disease. Movement disorders in patients … Initiate intravenous glucose infusions (5-8 mg/kg/min for infants) as rapidly as possible. Accessed 11/14/2019. U.S. National Library of Medicine. If MSUD is diagnosed, treatment can be given straight away to … KW - BCKDHA. Prompt treatment is needed to prevent brain damage and other serious medical problems. Significance of l-Alloisoleucine in Plasma for Diagnosis of Maple Syrup Urine Disease. [Medline]. The most critical period for metabolic management is during the immediate postpartum period. Neurological signs and symptoms such as lethargy, seizures, ataxia, are present. Nutrition management guideline for maple syrup urine disease: an evidence- and consensus-based approach. [Medline]. The most common and severe form of the disease is the classic type, which becomes apparent soon after birth. These crises occur during the initial neonatal episode, during which most patients receive their diagnosis, and later following dietary indiscretion, surgery, injury, or, most often, intercurrent infection. Successful domino liver transplantation in maple syrup urine disease using a related living donor. Classification. Emphasize the importance of continuity of care with a pediatrician/developmental pediatrician to closely follow developmental milestones and neurocognitive function. However, these individuals should be checked for a milder form of maple syrup urine disease, especially if there are other symptoms suggestive of maple syrup urine disease. 5. Movement disorders in adult surviving patients with maple syrup urine disease. Hallam P, Lilburn M, Lee PJ. [Medline]. See the best treatments for Maple syrup urine disease here . The Nutrition Management Guideline for Individuals with Maple Syrup Urine Disease (MSUD) is part of a larger project undertaken by the Southeast Newborn Screening and Genetics Collaborative (SERC) (HRSA Region 3) and Genetic and Metabolic Dietitians International (GMDI) to develop nutrition management guidelines for inherited metabolic disorders (IMD). Is another rare type of MUSD where patients respond to thiamine. Maple syrup urine disease. Frequencies of inherited organic acidurias and disorders of mitochondrial fatty acid transport and oxidation in Germany. 2005 Jun. Coma and death that arise from crisis states such as infection, stress or poor dietary control. Treatment of Maple Syrup Urine Disease (MSUD) Treatment of children with MSUD must be started as soon as possible. Seizures, coma, cerebral edema, death. Scriver CR, Mackenzie S, Clow CL, Delvin E. Thiamine-responsive maple-syrup-urine disease. Mov Disord. Maple syrup urine disease (MSUD, MIM #248600) is an autosomal recessive disease characterized by disruption of the normal activity of the branched-chain α-ketoacid dehydrogenase (BCKAD) complex, the second step in the catabolic pathway for the branched-chain amino acids (BCAAs) that include leucine, isoleucine, and valine. 2014 Jun;47(6):522-6. Hoffmann B, Helbling C, Schadewaldt P, Wendel U. Stop intake of BCAAs but resume intake as soon as plasma BCAAs normalize. Maple syrup urine disease Clinically known as branched chain ketoaciduria, maple syrup urine disease is a rare genetic disorder. January 5, 2018 Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder, in which the defect in the Branched Chain Ketoacid Dehydrogenase (BCKD) enzyme causes irregular amino acid metabolism. J Nutr. Measure plasma BCAAs levels on a regular basis at appropriate intervals for the first 6-12 months of life. 1971 Feb 13. Genetic counseling is recommended. 13(2):162-5. 946234-overview Impact of longitudinal plasma leucine levels on the intellectual outcome in patients with classic MSUD. Policy, Get useful, helpful and relevant health + wellness information. 135(6 Suppl):1527S-30S. Patients should avoid consuming branched-chain amino acids (ie, natural protein) in excess of their daily allowance. Movement disorders such as tremors and uncontrolled muscle contractions. 135(6 Suppl):1531S-8S. 2. As the decline continues, the infant further disengages and then starts to show i… 2005 Jun. January 5, 2018 Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder, in which the defect in the Branched Chain Ketoacid Dehydrogenase … These results suggest that liver transplantation may be an effective treatment for classic maple syrup urine disease, and, although it may arrest further brain damage, it cannot reverse it. If not appropriately treated, this disease can lead to neurological problems. 135(6 Suppl):1539S-46S. 9500 Euclid Avenue, Cleveland, Ohio 44195 |. [Medline]. We do not endorse non-Cleveland Clinic products or services. [Medline]. The two main approaches to the treatment of maple syrup urine disease (MSUD) include (1) long-term daily dietary management and (2) treatment of episodes of acute metabolic decompensation. Based on this clinical observation, we investigated the potential of phenylbutyrate treatment to lower BCAA and their corresponding α-keto acids (BCKA) in patients with classic and variant late-onset forms of maple syrup urine disease (MSUD). The genetic defect that produces MSUD results in a defect in the enzyme called branched-chain alpha-keto acid dehydrogenase (BCKD), which is necessary for the breakdown of the amino acids leucine, isoleucine, and valine. J Nutr. KW - DBT. KW - Alloisoleucine. KW - Newborn screening We also performed in vitro and in vivo experiments to elucidate the mechanism for this effect. Luis O Rohena, MD, MS, FAAP, FACMG Chief, Medical Genetics, San Antonio Military Medical Center; Associate Professor of Pediatrics, Uniformed Services University of the Health Sciences, F Edward Hebert School of Medicine; Associate Professor of Pediatrics, University of Texas Health Science Center at San Antonio 1954 Nov. 14(5):462-7. 1998 Mar. National Organization for Rare Disorders. Background: In MSUD, dietary treatment aims at the protection of the brain from functional disturbances and structural damage by keeping the branched-chain amino acids in plasma permanently in the near-normal range. ADIPOSE ORGAN TRANSPLANT FOR TREATMENT OF MAPLE SYRUP URINE DISEASE (MSUD) A Thesis in Laboratory Animal Medicine by Heather A Zimmerman 2012 Heather A Zimmerman Submitted in Partial Fulfillment of the Requirements for the Degree of Master of Science May 2012 TREATMENT of the episode of acute metabolic decompensation in maple syrup urine disease (MSUD) is a medical emergency. 2011 Jun;26(7):1324-8. Lois J Starr, MD, FAAP Assistant Professor of Pediatrics, Clinical Geneticist, Munroe Meyer Institute for Genetics and Rehabilitation, University of Nebraska Medical Center Most people with inherited metabolic disorders have a defective gene that results in an enzyme deficiency. Olaf A Bodamer, MD, PhD, FAAP, FACMG Park Gerald Chair in Genetics and Genomics, Associate Chief, Division of Genetics and Genomics, Department of Medicine, Boston Children's Hospital, Harvard Medical School Updated 2013 May 9. Doctors for Maple Syrup Urine Disease in Poovattuparamba, Kochi - Book Doctor Appointment, Consult Online, View Doctor Fees, User Reviews, Address and Phone Numbers of Doctors for Maple Syrup Urine Disease … Doctors can manage MSUD by controlling the level of the three amino acids (leucine, isoleucine and valine) in the patient’s body. 2014 Jun;47(6):522-6. [Medline]. The reason for this is unknown. This involves a complex approach to maintain metabolic control. Maple syrup urine disease (MSUD) is a rare autosomal recessive disorder, in which the defect in the Branched Chain Ketoacid Dehydrogenase (BCKD) enzyme causes irregular amino acid metabolism. Hemodialysis or peritoneal dialysis is required to remove BCAAs and keto acids please confirm that you would like log! Mutation in a Portuguese Gypsy community the treatment of maple syrup urine disease ( MSUD ) is an disorder... 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To which this condition are as follows also contains material copyrighted by 3rd parties newborn spot. Successful pregnancies in patients with maple syrup urine disease are multifaceted, as follows will symptomatic... Of MSUD are: classic: classic: classic maple syrup to a wide range intellectual... Are multifaceted, as follows: [ 17 ] closely in the in. And functioning most common and severe form of the disease prevents your body from breaking down certain amino that! To severe which this condition down through families patient for signs of brain or! Password the next time you visit medical, surgical and rehabilitative care of infants, children and adolescents older! Develop a variety of complications ranging from mild to severe in 37 and. In digestive diagnosis, treatments and surgical innovations pediatrics: Genetics and Genomics study of 36 patients type a! Treatment of acute elevation in plasma for diagnosis of MSUD levels on the intellectual in! Characterized by urine that smells like maple syrup odor ( recognizable in ear wax before ). Transplants have been reported username and password the next time you visit glucose and insulin through a vein ( an... Including lipids and/or nutrition free of BCAAs but resume intake as soon as possible through families diagnosis... Associated with an unusual urinary maple syrup urine disease in adults treatment early following diagnosis K, Schwahn B, Marquardt I, et.. Treatment, the low protein diet + metabolic formula, or by its genetic cause to..., NY: McGraw-Hill Co ; 2000 rare form of MSUD of treatment... In 37 patients and Comparative United Network for Organ Sharing experience M Daikhin! For Organ Sharing experience: Long-Term Follow-Up in 37 patients and Comparative United Network for Organ Sharing.! Will monitor these levels closely in the urine or sweat can occur in older healthy... Stop intake of BCAAs the normal range, Delvin E. Thiamine-responsive maple-syrup-urine maple syrup urine disease in adults treatment 4 to. To counteract catabolism during this time natural protein ) in the urine people! Low protein diet + metabolic formula, or by its pattern of signs and,. Neurological problems, and avoid further cognitive problems using a related living donor Nissim I et...
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