Remission of Severe Neonatal Diabetes With Very Early Sulfonylurea Treatment
نویسندگان
چکیده
This 6-year-old male (Case-1-male) was previously reported at birth (3). Mother and sister (Case-1-female) both had NDM and were given insulin for 24 and 6 years, respectively, then treated with high-dose glyburide; mother still requires some insulin. Case-1-male had NDM at 3 days and was given glyburide 0.2 mg/kg/day, weaned to 0.05 mg/kg/day, and maintained normal hemoglobin A1c for 5 years (Fig. 1A) continuing glyburide 0.075 mg thrice daily (mixing a crushed 5 mg tablet with 10 cc water, giving 0.15 cc for each dose, 0.012mg/kg/day). All had a heterozygous mutation in KCNJ11 (R201H).
منابع مشابه
Acute Sulfonylurea Therapy at Disease Onset Can Cause Permanent Remission of KATP-Induced Diabetes
OBJECTIVE Neonatal diabetes mellitus (NDM) can be caused by gain-of-function ATP-sensitive K(+) (K(ATP)) channel mutations. This realization has led to sulfonylurea therapy replacing insulin injections in many patients. In a murine model of K(ATP)-dependent NDM, hyperglycemia and consequent loss of β-cells are both avoided by chronic sulfonylurea treatment. Interestingly, K(ATP) mutations may u...
متن کاملGenetic Susceptibility to Transient and Permanent Neonatal Diabetes Mellitus
Neonatal diabetes mellitus (NDM) is a rare kind of diabetes characterized by hyperglycemia and low levels of insulin. Clinically, it is categorized into two main types: transient NDM (TNDM) and permanent NDM (PNDM). These types are diagnosed based on duration of insulin dependence early in the disease. In TNDM, diabetes begins in the first few weeks of life with remission in a few months. Howev...
متن کاملTransient neonatal diabetes mellitus caused by a de novo ABCC8 gene mutation
Transient neonatal diabetes mellitus (TNDM) is a rare form of diabetes mellitus that presents within the first 6 months of life with remission in infancy or early childhood. TNDM is mainly caused by anomalies in the imprinted region on chromosome 6q24; however, recently, mutations in the ABCC8 gene, which encodes sulfonylurea receptor 1 (SUR1), have also been implicated in TNDM. Herein, we pres...
متن کاملNeonatal Presentation of Unremitting Inflammatory Bowel Disease
Very-early-onset inflammatory bowel disease (VEO-IBD) has a distinct phenotype and should be considered a specific entity. VEO-IBD presents with very severe clinical pictures and is frequently known by an indeterminate colitis whose clinical remission is unmanageable. This study examines the case of a neonate with VEO-IBD, not responding to medical and surgical treatment. A 7-day-old Iranian fe...
متن کاملNeonatal diabetes mellitus: a disease linked to multiple mechanisms
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 l...
متن کامل