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Neonatal Diabetes Definition

Orphanet: Transient Neonatal Diabetes Mellitus

Orphanet: Transient Neonatal Diabetes Mellitus

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us . Only comments written in English can be processed. Check this box if you wish to receive a copy of your message Transient neonatal diabetes mellitus (TNDM) is a genetically heterogeneous form of neonatal diabetes (NDM, see this term) characterized by hyperglycemia presenting in the neonatal period that remits during infancy but recurs in later life in most patients. Inheritance: Autosomal dominantorAutosomal recessiveorNot applicable The prevalence of neonatal diabetes is estimated to be between 1/95,000 to 1/400,000 live births. About 50% of NDM cases are transient (TNDM) and 50% permanent (PNDM, see this term). The condition has been reported in all ethnic groups and affects male and female infants equally. Cardinal clinical manifestations include severe intrauterine growth retardation, hyperglycemia (within the first week of life beginning in the neonatal period and resolving usually by 18 months of age), and dehydration. The most commonly reported congenital abnormalities are macroglossia and umbilical hernia. A wide range of different associated clinical signs including, facial dysmorphism, deafness and neurological (as a rule no epilepsy), cardiac, metabolic, kidney or urinary tract anomalies are reported. Affected infants usually require insulin initially, but the need for insulin gradually declines with time. Developmental delay and learning difficulties may also be observed. Women who have had TNDM as infants are at risk for relapse during pregnancy. Ketoacidosis is generally absent (except in patients with KCNJ11 and ABCC8 mutations). TNDM is caused by 6q24 alterations consisting of pa Continue reading >>

Neonatal Diabetes

Neonatal Diabetes

Tweet Neonatal diabetes is a rare form of diabetes that is usually diagnosed in children under 6 months of age. This early occurring type of diabetes is caused by one of a number of genetic mutations and is therefore described as a monogenic form of diabetes. Neonatal diabetes is treatable and may or may not require insulin so a diagnosis by genetic testing is recommended. Types of neonatal diabetes There are two main types of neonatal diabetes: Transient Neonatal Diabetes Mellitus Permanent Neonatal Diabetes Mellitus Transient neonatal diabetes is so called because it usually disappears within a year of birth but can come back again typically during adolescence. Permanent neonatal diabetes, once diagnosed, stays for the rest of life. How common is neonatal diabetes? Neonatal diabetes is very rare, occurring in around 1 in 300,000 to 1 in 400,000 births. Out of the two types of neonatal diabetes, the transient type is slightly more common affecting 50-60% of cases of neonatal diabetes. [105] Symptoms and diagnosis The symptoms of neonatal diabetes include persistent thirst, frequent urination and dehydration. [103] Tweet Type 2 diabetes mellitus is a metabolic disorder that results in hyperglycemia (high blood glucose levels) due to the body: Being ineffective at using the insulin it has produced; also known as insulin resistance and/or Being unable to produce enough insulin Type 2 diabetes is characterised by the body being unable to metabolise glucose (a simple sugar). This leads to high levels of blood glucose which over time may damage the organs of the body. From this, it can be understood that for someone with diabetes something that is food for ordinary people can become a sort of metabolic poison. This is why people with diabetes are advised to avoid sources of Continue reading >>

Neonatal Diabetes Mellitus

Neonatal Diabetes Mellitus

Mark A. Sperling MD, in Pediatric Endocrinology (Fourth Edition) , 2014 Neonatal diabetes mellitus (NDM) was strictly defined as severe hyperglycemia occurring in the first month of life, lasting at least 2 weeks, and requiring insulin therapy to control blood glucose.1 These strict criteria have been progressively loosened, initially to include onset in the first 6 months of life, when autoimmune type 1 diabetes mellitus (T1DM) is highly unlikely, thereby implicating a genetic cause for pancreatic malformation, or faulty insulin synthesis and secretion. Increasingly, it has become recognized that, depending on the degree of expression and the inherent capacity of the genetic mutation to disrupt normal insulin output, several of the genetic forms can initially present with symptoms up to 9 months to 1 year, or even later.2-4 Indeed, pedigree analyses conclusively demonstrate that the same defect that causes permanent or transient NDM can be present in parents or other first-degree relatives and be diagnosed as T1DM, monogenic diabetes of youth (MODY), or type 2 diabetes mellitus (T2DM) as subsequently detailed.5,6 Herein lies the importance of understanding the genetic basis of NDM, for although considered rare, with a reported incidence varying from approximately 1 in 100,000 to 1 in 400,000 live births,7 these entities have taught us much about the genetic pathways involved in the formation of the exocrine and endocrine pancreas.8,9 For example, it has been shown that the specific combination of three transcription factors, Ngn3, Pdx1 and Mafa, known to be implicated in the determination of cell lineage during pancreas formation, can reprogram adult mouse exocrine pancreatic cells into cells that closely resemble pancreatic cells.10 Such information is essential for Continue reading >>

Monogenic Diabetes (neonatal Diabetes Mellitus & Mody)

Monogenic Diabetes (neonatal Diabetes Mellitus & Mody)

The most common forms of diabetes, type 1 and type 2, are polygenic, meaning they are related to a change, or defect, in multiple genes. Environmental factors, such as obesity in the case of type 2 diabetes, also play a part in the development of polygenic forms of diabetes. Polygenic forms of diabetes often run in families. Doctors diagnose polygenic forms of diabetes by testing blood glucose, also known as blood sugar, in individuals with risk factors or symptoms of diabetes. Genes provide the instructions for making proteins within the cell. If a gene has a change or mutation, the protein may not function properly. Genetic mutations that cause diabetes affect proteins that play a role in the ability of the body to produce insulin or in the ability of insulin to lower blood glucose. People typically have two copies of most genes, with one gene inherited from each parent. What are monogenic forms of diabetes? Some rare forms of diabetes result from mutations or changes in a single gene and are called monogenic. In the United States, monogenic forms of diabetes account for about 1 to 4 percent of all cases of diabetes.1,2,3,4 In most cases of monogenic diabetes, the gene mutation is inherited from one or both parents. Sometimes the gene mutation develops spontaneously, meaning that the mutation is not carried by either of the parents. Most mutations that cause monogenic diabetes reduce the body’s ability to produce insulin, a protein produced in the pancreas that helps the body use glucose for energy. Neonatal diabetes mellitus (NDM) and maturity-onset diabetes of the young (MODY) are the two main forms of monogenic diabetes. NDM occurs in newborns and young infants. MODY is much more common than NDM and usually first occurs in adolescence or early adulthood. Most cas Continue reading >>

Neonatal Hyperglycemia Due To Transient Neonatal Diabetes Mellitus In Puerto Rico

Neonatal Hyperglycemia Due To Transient Neonatal Diabetes Mellitus In Puerto Rico

Copyright © 2015 N. Fargas-Berríos et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Neonatal hyperglycemia is a metabolic disorder found in the neonatal intensive care units. Neonatal diabetes mellitus (NDM) is a very uncommon cause of hyperglycemia in the newborn, occurring in 1 in every 400,000 births. There are two subtypes of neonatal diabetes mellitus: permanent neonatal diabetes mellitus (PNDM) and transient neonatal diabetes mellitus (TNDM). We describe a term, small for gestational age, female neonate with transient neonatal diabetes mellitus who presented with poor feeding tolerance and vomiting associated with hyperglycemia (385 mg/dL), glycosuria, and metabolic acidosis within the first 12 hours of life. The neonate was treated with intravenous insulin, obtaining a slight control of hyperglycemia. An adequate glycemia was achieved at 5 weeks of life. The molecular studies showed complete loss of maternal methylation at the TND differentially methylated region on chromosome 6q24. The etiology of this neonate’s hyperglycemia was a hypomethylation of the maternal TND locus. A rare cause of neonatal diabetes mellitus must be considered if a neonate presents refractory hyperglycemia. To our knowledge, this is the first case reported in Puerto Rico of transient neonatal mellitus due to the uncommon mechanism of maternal hypomethylation of the TND locus. Its prevalence in Puerto Rico is unknown. 1. Introduction Neonatal hyperglycemia is a metabolic disorder found in the neonatal intensive care units [1]. There are several and different etiologies for neonatal hyperglycemia with different clin Continue reading >>

Neonatal Diabetes Mellitus

Neonatal Diabetes Mellitus

Pacific Northwest Diabetes Research Institute, Seattle, Washington 98122 Address all correspondence and requests for reprints to: Lydia Aguilar-Bryan and Joseph Bryan, Pacific Northwest Diabetes Research Institute, 720 Broadway, Seattle, Washington 98122. Search for other works by this author on: Pacific Northwest Diabetes Research Institute, Seattle, Washington 98122 Search for other works by this author on: Endocrine Reviews, Volume 29, Issue 3, 1 May 2008, Pages 265291, Lydia Aguilar-Bryan, Joseph Bryan; Neonatal Diabetes Mellitus, Endocrine Reviews, Volume 29, Issue 3, 1 May 2008, Pages 265291, An explosion of work over the last decade has produced insight into the multiple hereditary causes of a nonimmunological form of diabetes diagnosed most frequently within the first 6 months of life. These studies are providing increased understanding of genes involved in the entire chain of steps that control glucose homeostasis. Neonatal diabetes is now understood to arise from mutations in genes that play critical roles in the development of the pancreas, of -cell apoptosis and insulin processing, as well as the regulation of insulin release. For the basic researcher, this work is providing novel tools to explore fundamental molecular and cellular processes. For the clinician, these studies underscore the need to identify the genetic cause underlying each case. It is increasingly clear that the prognosis, therapeutic approach, and genetic counseling a physician provides must be tailored to a specific gene in order to provide the best medical care. Sulfonylureas Are an Effective Therapy for NDM Caused by Overactive KATP Channels With few exceptions, diabetes in childhood knows no cure, no matter how mild it may appear in the beginning, nor how gradual its development in the Continue reading >>

The Key Features Of Neonatal Diabetes Are:

The Key Features Of Neonatal Diabetes Are:

Neonatal diabetes is a form of diabetes that is diagnosed under the age of nine months. It’s a different type of diabetes than the more common Type 1 diabetes as it’s not an autoimmune condition (where the body has destroyed its insulin producing cells). Neonatal diabetes is caused by a change in a gene which affects insulin production. This means that levels of blood glucose (sugar) in the body rise very high. The main feature of neonatal diabetes is being diagnosed with diabetes under the age of 6 months, and this is where it’s different from Type 1, as Type 1 doesn’t affect anyone under 6 months. As well as this, about 20 per cent of people with neonatal diabetes also have some developmental delay (eg muscle weakness, learning difficulties) and epilepsy. Neonatal diabetes is very rare, currently there are less than 100 people diagnosed with it in the UK. There are two types of neonatal diabetes – transient and permanent. As the name suggests, transient neonatal diabetes doesn’t last forever and usually resolves before the age of 12 months. But it usually recurs later on in life, generally during the teenage years. It accounts for 50–60 per cent of all cases. Permanent neonatal diabetes as you might expect, lasts forever and accounts for 40–50 per cent of all cases. Around 50 per cent of people with neonatal diabetes don’t need insulin and can be treated with a tablet called Glibenclamide instead. These people have a change in the KCNJ11 or ABCC8 gene and need higher doses of Glibenclamide than would be used to treat type 2 diabetes. As well as controlling blood glucose levels, Glibenclamide can also improve the symptoms of developmental delay. It’s important to know if you have/your child has neonatal diabetes to make sure you’re/they’re getti Continue reading >>

Neonatal Diabetes Mellitus: A Genetic And Clinical Approach

Neonatal Diabetes Mellitus: A Genetic And Clinical Approach

Neonatal Diabetes Mellitus: A Genetic and Clinical Approach AM Xanthopoulou 1 # , E Xanthopoulou 2 # ,D Papazoglou 1 andN Papanas 1 * 1 Diabetes Centre, Second Departmentof Internal Medicine, Medical School,Democritus University of Thrace,Alexandroupolis, Greece 2 Department of Molecular Biology,Democritus University of Thrace,Alexandroupolis, Greece Diabetes Centre, Second Departmentof Internal Medicine Medical School,Democritus University of Thrace G. Kondyli22c, Alexandroupolis 68100, Greece. Received date: October 23, 2017; Accepted date: October 25, 2017; Published date: November 11, 2017 Citation: Xanthopoulou AM, XanthopoulouE, Papazoglou D, Papanas N. (2017) NeonatalDiabetes Mellitus: A Genetic and ClinicalApproach. J Mol Genet Med Vol.1 No.1:6 Visit for more related articles at Journal of Molecular Genetics and Medicine Insulin exerts a pivotal role in the glucose homeostasis ofthe human body, while its deficits are related to completeinadequacies, such as Type 1 diabetes mellitus or relateddeficiencies such as Type 2 diabetes mellitus [ 1 - 4 ]. It has beenidentified that these deficiencies have a genetic backgroundand can arise either from mutations in a single gene (monogenicforms of diabetes) or by a combination of rare genetic mutationsthe action of environmental factors (polygenic forms ofdiabetes) [ 4 - 7 ]. Although the exact aetiology of diabetes remainsunknown, it appears to follow a polygenic inheritance pattern,and it is believed that the genetic and environmental factorsassociated with it have the potential to affect its appearance anddevelopment in most cases [ 8 ]. However, monogenic forms have been observed during neonataland early infancy , resulting in the occurrence of permanentand transient neonatal diabetes mellitus [ 1 , 9 - 13 ]. The for Continue reading >>

Neonatal Diabetes Mellitus

Neonatal Diabetes Mellitus

Neonatal Diabetes Mellitus (NDM) Neonatal Diabetes Mellitus (NDM) Neonatal diabetes mellitus (NDM) is defined as a disease that affects an infant and their body's ability to produce or use insulin.[1] NDM is a monogenic (controlled by a single gene) form of diabetes that occurs in the first 6 months of life. Infants do not produce enough insulin, leading to an increase in . It is a rare disease, occurring in only one in 100,000 to 500,000 live births.[2] NDM can be mistaken for the much more common type 1 diabetes, but type 1 diabetes usually occurs later than the first 6 months of life. There are two types of NDM: permanent neonatal diabetes mellitus (PNDM) is a lifelong condition. Transient neonatal diabetes mellitus (TNDM) is diabetes that disappears during the infant stage but may reappear later in life.[2] Specific genes that can cause NDM have been identified.[3] The onset of NDM can be caused by abnormal pancreatic development, beta cell dysfunction or accelerated beta cell dysfunction.[4] Individuals with monogenic diabetes can pass it on to their children or future generations. Each gene associated with NDM has a different inheritance pattern. Mechanism[edit] Transient Neonatal Diabetes Mellitus (TNDM)[edit] TNDM occurs within the first several days to weeks of an infant's life. Intrauterine growth restriction (IUGR) is commonly seen in affected individuals and defined as poor growth of an unborn baby while in his or her mother's womb.[5] In comparison, of PNDM, the insulin dose requirement of TNDM is often lower. TNDM resolves on its own at an average age of twelve weeks. Although, individuals will relapse in 50% of cases (usually during childhood or young adulthood).[6] The parts of the body that are mostly affected are the pancreas, central nervous system an Continue reading >>

What Is The Difference Between Type 1 And Type 2 Diabetes?

What Is The Difference Between Type 1 And Type 2 Diabetes?

There are three major types of diabetes: type 1, type 2, and gestational diabetes. All types of diabetes cause blood glucose levels to be higher than normal, but they do this in different ways Type 1 diabetes Type 1 diabetes can occur at any age, but is most commonly diagnosed from infancy to the late 30s. With this type of diabetes, a person’s pancreas produces no insulin. It occurs when the body’s own defence system (the immune system) attacks and destroys the insulin-producing cells in the pancreas. What causes the immune system to do this is not yet completely understood, but we are funding research to find out. Type 2 diabetes Type 2 diabetes is by far the most common type of diabetes – in the UK over 90 per cent of people with diabetes have type 2. Type 2 diabetes usually affects those over 40, or 25 if you’re of South Asian descent. However, it is becoming more common among young people due to lifestyle. The symptoms of type 2 diabetes are not always obvious and, unlike with type 1, they can take a long time to develop. People with type 2 diabetes either don’t make enough insulin or don’t make insulin that the body can use properly. The cells in the body become resistant to insulin, making a greater amount of insulin necessary to keep blood glucose levels within a normal range. Eventually, the pancreas can wear out from producing extra insulin, and it may start making less and less. Type 2 can usually be managed through diet, exercise, and self-monitoring blood glucose, at least in the first few years following diagnosis. However, type 2 diabetes is a progressive condition, and most people will need to take tablets and/or inject insulin after living with it for five to 10 years. LADA Up to a third of people who were initially diagnosed as having type Continue reading >>

Transient Neonatal Diabetes Mellitus

Transient Neonatal Diabetes Mellitus

What was the least helpful aspect of this article? There is missing or inacurate information Please let us know what information is missing or inaccurate.(Optional) To verify that you are a real person, DoveMed requires each survey to be answered while logged in. Transient Neonatal Diabetes Mellitus (TNDB) is a type of diabetes that appears within the first few weeks of life but is transient. The affected infants go into remission within a few months, with possible relapse to permanent diabetes in adolescence or adulthood. What are the other Names for this Condition? (Also known as/Synonyms) Chromosome 6-Associated Transient Diabetes Mellitus Diabetes Mellitus, 6q24-Related Transient Neonatal What is Transient Neonatal Diabetes Mellitus? (Definition/Background Information) Transient NeonatalDiabetes Mellitus(TNDB) is a type ofdiabetesthat appears within the first few weeks of life but is transient. The affected infants go intoremissionwithin a few months, with possible relapse to permanent diabetes in adolescence or adulthood Affected individuals have slow growth before birth followed byhyperglycemia, dehydration andfailure to thrivein infancy Approximately 70% of cases are caused by the over activity of certaingenesin a region of the long (q) arm ofchromosome 6called 6q24. These cases are referred to as6q24-related TNDB; most (but not all) of these cases are notinherited Other genetic causes includemutationsin theKCNJ11andABCC8genes, which usually causepermanent neonatal diabetes Treatment may include rehydration and intravenous insulin at the time of diagnosis, followed by subcutaneous insulin (Source: Transient NeonatalDiabetes Mellitus; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.) Who get Continue reading >>

Neonatal Diabetes Mellitus: A Disease Linked To Multiple Mechanisms

Neonatal Diabetes Mellitus: A Disease Linked To Multiple Mechanisms

Abstract Transient (TNDM) and Permanent (PNDM) Neonatal Diabetes Mellitus are rare conditions occurring in 1:300,000–400,000 live births. TNDM infants develop diabetes in the first few weeks of life but go into remission in a few months, with possible relapse to a permanent diabetes state usually around adolescence or as adults. The pancreatic dysfunction in this condition may be maintained throughout life, with relapse initiated at times of metabolic stress such as puberty or pregnancy. In PNDM, insulin secretory failure occurs in the late fetal or early post-natal period and does not go into remission. Patients with TNDM are more likely to have intrauterine growth retardation and less likely to develop ketoacidosis than patients with PNDM. In TNDM, patients are younger at the diagnosis of diabetes and have lower initial insulin requirements. Considerable overlap occurs between the two groups, so that TNDM cannot be distinguished from PNDM based on clinical features. Very early onset diabetes mellitus seems to be unrelated to autoimmunity in most instances. A number of conditions are associated with PNDM, some of which have been elucidated at the molecular level. Among these, the very recently elucidated mutations in the KCNJ11 and ABCC8 genes, encoding the Kir6.2 and SUR1 subunit of the pancreatic KATP channel involved in regulation of insulin secretion, account for one third to half of the PNDM cases. Molecular analysis of chromosome 6 anomalies (found in more than 60% in TNDM), and the KCNJ11 and ABCC8 genes encoding Kir6.2 and SUR1, provides a tool to identify TNDM from PNDM in the neonatal period. This analysis also has potentially important therapeutic consequences leading to transfer some patients, those with mutations in KCNJ11 and ABCC8 genes, from insulin t Continue reading >>

Internet Scientific Publications

Internet Scientific Publications

ch 6 uniparental disomy, dend syndrome, kjn 11 gene, neonatal diabetes A Saini, V Kumar, S Kumar. Neonatal Diabetes Mellitus: Genetic Defect and Management Issues. The Internet Journal of Pediatrics and Neonatology. 2007 Volume 9 Number 1. Neonatal Diabetes Mellitus is a rare disorder characterized by significant hyperglycemia needing exogenous insulin in first 6-8 weeks of life. Two types, Permanent and Transient, have been described in literature based upon the duration of insulin dependency. Recently, various genetic defects like chromosome 6 uniparental disomy and KJN11 gene mutations have been associated with NDM. Neonatal Diabetes Mellitus is a rare disorder in infants occurring in 1:300,000-400,000 live births, characterized by significant hyperglycemia needing exogenous insulin therapy combined with low level of insulin in first 6-8 weeks of life, and if not diagnosed timely it can be life threatening. Two main types, Permanent Neonatal Diabetes Mellitus (PNDM) and Transient Neonatal Diabetes Mellitus (TNDM), have been described in literature based mainly on clinical course and duration of insulin dependency. We report one such rare case. An 8 weeks old female infant presented to our hospital with frequent urination, frequent demand for feeding and particulate deposits of urine since birth. There was no history of fast breathing, fever, rashes, loose stool, vomiting, drowsiness or convulsions. She was the first (twin A) of the twins born to a couple with 2 nd degree consanguineous marriage. She was delivered by lower section caesarean section to a 28-year-old 3 rd gravida mother with no live issues. She was full term, breech, and small for gestational age (birth weight 1.5 kg). She was kept in a level one nursery for 9 days because of low birth weight along wit Continue reading >>

Neonatal Hyperglycemia

Neonatal Hyperglycemia

OBJECTIVES After completing this article, readers should be able to: Explain the relationship of the frequency of hyperglycemia with the birthweight and gestational age of the neonate. List factors that may decrease glucose tolerance in the neonate. Describe the major mechanism(s) contributing to altered regulation of neonatal glucose metabolism. List the untoward effects of hyperglycemia in the neonate. Describe the beneficial effects of insulin infusion in selected neonates. Case Study A preterm male infant was born at 26 weeks’ gestation weighing 900 g, with a birthweight percentile below the 3rd percentile and a head circumference at about the 25th percentile. His Apgar scores were 4 and 5 at 1 and 5 minutes, respectively, and he required intubation and ventilation in the delivery room. The infant was given artificial surfactant and maintained on a respirator with“ moderate to high settings.” Several saline boluses were administered for hypotension, and dopamine was started to maintain a mean arterial blood pressure above 30 mm Hg. Initial serum glucose concentrations were in the range of 1.67 to 2.76 mmol/L (30 to 50 mg/dL). The infant was given 10% dextrose at a rate of 100 mL/kg per day. By the second day of life, the serum glucose concentration was in the range of 5.55 to 8.33 mmol/L (100 to 150 mg/dL), and the dextrose infusion, which now contained sodium chloride and potassium acetate, was increased to 150 mL/kg per day because of polyuria of 6 mL/kg per hour. The infant remained NPO. Subsequent glucose concentrations over the next 24 hours increased to more than 13.88 mmol/L (250 mg/dL), and glycosuria was noted. At this time, the intravenous (IV) fluid rate was 225 mL/kg per day (“to maintain hydration”) and the glucose infusion rate was 16 mg/kg p Continue reading >>

Omim Entry - # 606176 - Diabetes Mellitus, Permanent Neonatal; Pndm

Omim Entry - # 606176 - Diabetes Mellitus, Permanent Neonatal; Pndm

A number sign (#) is used with this entry because of evidence that permanent neonatal diabetes mellitus can be caused by homozygous mutation in the glucokinase gene (GCK; 138079 ), by heterozygous mutation in the KCNJ11 ( 600937 ) gene, or by heterozygous or homozygous mutation in the ABCC8 ( 600509 ) and INS ( 176730 ) genes. Pancreatic agenesis, which results in exocrine pancreatic deficiency as well as permanent neonatal-onset diabetes mellitus, can be caused by mutation in the PDX1 gene ( 600733 ). Pancreatic agenesis associated with cerebellar agenesis ( 609069 ) can be caused by mutation in the PTF1A gene ( 607194 ). Pancreatic agenesis associated with congenital cardiac defects ( 600001 ) can be caused by mutation in the GATA6 gene ( 601656 ). Neonatal diabetes mellitus (NDM), defined as insulin-requiring hyperglycemia within the first 3 months of life, is a rare entity, with an estimated incidence of 1 in 400,000 neonates ( Shield, 2000 ). In about half of the neonates, diabetes is transient (see 601410 ) and resolves at a median age of 3 months, whereas the rest have a permanent insulin-dependent form of diabetes (PNDM). In a significant number of patients with transient neonatal diabetes mellitus, type II diabetes (see 125853 ) appears later in life ( Arthur et al., 1997 ). PNDM is distinct from childhood-onset autoimmune diabetes mellitus type I (IDDM; 222100 ). Massa et al. (2005) noted that the diagnostic time limit for PNDM has changed over the years, ranging from onset within 30 days of birth to 3 months of age. However, as patients with the clinical phenotype caused by mutation in the KCNJ11 gene have been identified with onset up to 6 months of age, Massa et al. (2005) suggested that the term 'permanent diabetes mellitus of infancy' (PDMI) replace PNDM Continue reading >>

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