{"id":6769,"date":"2020-07-16T01:09:47","date_gmt":"2020-07-15T20:09:47","guid":{"rendered":"https:\/\/drmohans.com\/?p=6769"},"modified":"2024-11-27T10:02:09","modified_gmt":"2024-11-27T10:02:09","slug":"what-is-neonatal-diabetes-mellitus-ndm","status":"publish","type":"post","link":"https:\/\/drmohans.com\/backend\/what-is-neonatal-diabetes-mellitus-ndm\/","title":{"rendered":"What is Neonatal Diabetes Mellitus (NDM)?"},"content":{"rendered":"

Neonatal Diabetes Mellitus<\/strong><\/h3>\n

We all have heard of diabetes occurring in children. The usual type of diabetes which occurs in children and adolescence is, what is called as \u2018Type 1 diabetes\u2019, previously called as \u2018Insulin dependent diabetes mellitus\u2019 or \u2018IDDM\u2019. Children with type 1 diabetes have an acute onset of the condition and rapidly become insulin dependent. Their pancreatic insulin reserve goes down to near zero and there afterwards they need lifelong insulin injections to maintain life and good health. However, there is another form of diabetes which occurs in infants and neonates. This is called as \u2018Neonatal Diabetes Mellitus\u2019 or \u2018NDM\u2019 and it typically occurs before the first 6 months of age, although occasionally it can occurupto 1 year of age. Onset of NDM beyond 1 year of age is very unusual, and in all such cases, it is most likely to be \u2018Type 1 diabetes<\/a>\u2019.<\/p>\n

Why should we know about Neonatal Diabetes and how does it differ from type 1 diabetes?<\/u><\/strong><\/p>\n

Neonatal Diabetes<\/a> is classified under \u2018Monogenic\u2019 forms of diabetes which means it is due to a mutation of agene. Mutations in several genes can be involved in causing\u00a0 neonatal diabetes. There are 2 main forms<\/p>\n

1) Transient neonatal diabetes (TNDM) and
\n2) Permanent neonatal diabetes (PNDM).<\/p>\n

Transient neonatal diabetes (TNDM) : <\/strong>TNDMsets in before six months of age but usually before the first birthday the child recovers and the diabetes seems to go away. In many cases, however, during adolescence or early childhood, the diabetes may come back. But until then, there is no evidence of the diabetes. TNDM is due to specific mutations in certain genes and knowledge about which mutation the child is suffering from, helps to decide the prognosis as well as to accurately classify the child. In our experience at the Madras Diabetes Research Foundation (MDRF)<\/a>, we have seen only a very\u00a0 few\u00a0 cases of TNDM and most of the cases referred to us have had the Permanent Neonatal Diabetes .<\/p>\n

Permanent Neonatal Diabetes (PNDM)<\/strong>: In PNDM the diabetes again sets usually before 6 months of age, but unlike TNDM, the diabetes continues even beyond the first birthday and is usually lifelong.<\/p>\n

Why should one know about neonatal forms of diabetes ?<\/u><\/strong><\/p>\n

As mentioned above, neonatal diabetes is due to mutations in specific genes. The common genes which are affected in neonatal diabetes are the KCNJ11<\/em> gene, the ABCC8<\/em> gene, the INS<\/em> gene, theGATA <\/em>gene, etc.,\u00a0 The vast majority of neonatal diabetes have mutations either in the KCNJ11<\/em> gene or in the ABCC8<\/em> genes. Children having mutations in these genes can respond to an oral sulphonylurea(SU) tablet and insulin injections may not be necessary at all.\u00a0 In our experience of the several hundred cases of neonatal diabetes referred to us, the majority have either the KCNJ11<\/em> mutation or the ABCC8<\/em> mutation. It is very gratifying that these children who were on insulin prior to being referred to us and before an accurate genetic diagnosis being made at our centre, were put on insulin\u00a0 by their physicians\u00a0 but after the accurate diagnosis of neonatal diabetes was made, the insulin injections were stopped and they were switched over to oral sulphonylurea agents. We have had such patients referred to us from all parts of India.<\/p>\n

To the children and their parents, it is nothing short of a miracle when a child who was diagnosed to have diabetes was told to take insulin injections several times a day throughout life, can now go off\u00a0 insulin injections. Indeed, the oral sulphonylrea agents respond even better than insulin.<\/p>\n

The other very gratifying aspect of diagnosing neonatal diabetes\u00a0 is that very often these children have other associated abnormalities like developmental delay and delayed milestones, neurological deficits and even epileptic fits because the same mutations are seen in other organs like the nervous system and once\u00a0 the child is started on the sulphonylurea agent, the neurological symptoms may also remit.<\/p>\n

In 2012, we reported on our first series of cases of neonatal diabetes (1). Thereafter, we have seen several hundred cases of neonatal diabetes at\u00a0 our centre, who were referred to us from various parts of the country.<\/p>\n

Figure 1:<\/strong><\/p>\n

\"Neonatal
Figure 1<\/strong> shows the current numbers of neonatal diabetes cases seen at our centre.<\/figcaption><\/figure>\n

Table 1 : CASE STUDY OF A CHILD WITH NEONATAL DIABETES <\/u><\/strong><\/p>\n

    \n
  • 28 day old child with high blood sugar was diagnosed as Type 1Diabetes by her physician and administered multiple daily dose of insulin.<\/li>\n
  • Came to our centre for a second opinion. Genetic testing done. Identified a mutation in KCNJ11<\/em> gene, p. Gly334 Val, c.1001G>T. This causes Permanent Neonatal Diabetes.<\/li>\n
  • Insulin was stopped and the child was shifted to Sulphonylurea tablets.<\/li>\n
  • Now she is completely off insulin and only on tablets.<\/li>\n
  • This is an example of successful transition to SU therapy from insulin in PNDM patient after finding a genetic defect .<\/li>\n<\/ul>\n

    Figure 2<\/strong><\/p>\n

    \"Figure
    Figure 2 shows the mutation in this child causing Neonatal Diabetes<\/figcaption><\/figure>\n

    Figure 3<\/strong><\/p>\n

    \"Figure
    Figure 3 shows some write ups in the press after the child was taken off insulin and switched to tablets.<\/figcaption><\/figure>\n

    If you know of any child whose diabetes was diagnosed before 6 months of age or even I year, do get in touch with us so that we may be able to help the child and the family by doing a genetic test.\u00a0 We would be happy to carry out this genetic test free of cost on humanitarian grounds. This also applies to all patients with diabetes diagnosed before 1 year of age, \u00a0irrespective of their current age. For more information, kindly visit our website http:\/\/www.monogenicdiabetes.in<\/a> & <\/strong>www.mdrf.in<\/a> which will give you details about how to send the blood samples for\u00a0 neonatal diabetes genetic testing.<\/p>\n