Neonatal diabetes with hyperchylomicronemia |
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Institution: | (1) Present address: Department ofPediatric Endocrinology, Ankara University Medical School, Ankara, Turkey;(2) Sokullu Mehmet Pasa Cad.Salkim S?güt Sok. Cinarel Apt. 37/10, Ankara, Turkey |
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Abstract: | Neonatal diabetes mellitus (NDM) is defined as hyperglycemia occurring in the first few weeks of life. It can be either transient
(TNDM) or permanent (PNDM). A 25 days old newborn was brought to the hospital with restlessness, respiratory depression and
cyanosis. He was born at term with a birth weight of 2000 g. There was no consanguinity between his parents. His physical
examination findings were as follows: Weight and height were under 3th percentile, he was hypoactive and dehydrated. Serum glucose level was 800 mgJdl; C-peptide was 0.41 ngJml. Upon investigation
for dyslipidemia in association with his neonatal diabetes, hyperchylomicronemia was found both in the patient and his father.
Pancreatitis, anemia and cholestasis were also observed. Insulin treatment was started for his diabetes together with a special
diet for dyslipidemia. At the end of 28 months of follow-up, dyslipidemia has resolved but the need for insulin therapy was
still existing. However, TNDM was considered in differential diagnosis because he was small for gestational age(SGA) at birth
and his symptoms had started at the 25th day of the neonatal period. Delayed recovery from insulin dependency brought out the possibility of PNDM. Furthermore, neonatal
diabetes combined with hypechylomicronemia is a rare clinical picture. Reported cases of NDM with different clinical evaluation
will help to better understanding of this disorder. |
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Keywords: | Neonatal diabetes Hyperchylomicronemia |
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