Insulin, Insulin Everywhere: A Rare Case Report of Rabson-Mendenhall Syndrome.

Rabson-Mendenhall syndrome (RMS) is a rare autosomal recessive disorder characterized by severe insulin resistance, a condition in which the body's tissues and organs do not respond appropriately to the hormone insulin. Insulin resistance impairs blood sugar regulation and ultimately leads to diabetes mellitus. A 19-year-old male presented with joint pain, blurring of vision, and generalized weakness. Investigations revealed hyperglycemia (random blood sugar (RBS) > 625 mg/dL, glycosylated hemoglobin (HbA1c) 18%), as well as sugars, protein, and ketone bodies in urine routine examination. An ultrasound of the abdomen was normal. Cardiac status was normal. Based on the clinical features, particularly the head to toe examination, skin changes, and the onset of type 2 diabetes mellitus, RMS syndrome was considered. The joint pain was alleviated with intravenous tramadol. Actrapid®, a fast-acting insulin, was given to control sugar levels, along with metformin. Vitamin B12 and pregabalin were also supplemented. A dermatological cream containing ammonium chloride, calcium lactate, glycerin, potassium chloride, sodium dihydrogen phosphate, and urea was given for skincare. It is an extremely rare disease with a frequency of fewer than one million people worldwide. Most patients survive only up to 15 years of age, although some can live into their third decade of life. Hepatic gluconeogenesis and fatty acid oxidation are affected, leading to ketoacidosis. The progression is much faster in RMS. In RMS, the genetic defect affects the insulin receptor (INSR) gene transcription with non-sense mutations and causes splicing defects. This results in premature chain termination and eventually to lower amounts of the insulin receptor messenger ribonucleic acid (mRNA). Ultimately, the number and density of insulin receptors in the plasma membrane are smaller, making the cells resistant to insulin. Herein, we report the case of a 19-year-old patient with RMS who was treated in our hospital, leading to a successful improvement in symptoms and discharge of the patient.

Two degree of freedom internal model control-PID design for LFC of power systems via logarithmic approximations.

Load frequency controller has been designed for reduced order model of single area and two-area reheat hydro-thermal power system through internal model control - proportional integral derivative (IMC-PID) control techniques. The controller design method is based on two degree of freedom (2DOF) internal model control which combines with model order reduction technique. Here, in spite of taking full order system model a reduced order model has been considered for 2DOF-IMC-PID design and the designed controller is directly applied to full order system model. The Logarithmic based model order reduction technique is proposed to reduce the single and two-area high order power systems for the application of controller design.The proposed IMC-PID design of reduced order model achieves good dynamic response and robustness against load disturbance with the original high order system.

Physiological, anatomical and transcriptional alterations in a rice mutant leading to enhanced water stress tolerance.

Water stress is one of the most severe constraints to crop productivity. Plants display a variety of physiological and biochemical responses both at the cellular and whole organism level upon sensing water stress. Leaf rolling, stomatal closure, deeper root penetration, higher relative water content (RWC) and better osmotic adjustment are some of the mechanisms that plants employ to overcome water stress. In the current study, we report a mutant, enhanced water stress tolerant1 (ewst1) with enhanced water stress tolerance, identified from the ethyl methanesulfonate-induced mutant population of rice variety Nagina22 by field screening followed by withdrawal of irrigation in pots and hydroponics (PEG 6000). Though ewst1 was morphologically similar to the wild type (WT) for 35 of the 38 morphological descriptors (except chalky endosperm/expression of white core, decorticated grain colour and grain weight), it showed enhanced germination in polyethylene glycol-infused medium. It exhibited increase in maximum root length without any significant changes in its root weight, root volume and total root number on crown when compared with the WT under stress in PVC tube experiment. It also showed better performance for various physiological parameters such as RWC, cell membrane stability and chlorophyll concentration upon water stress in a pot experiment. Root anatomy and stomatal microscopic studies revealed changes in the number of xylem and phloem cells, size of central meta-xylem and number of closed stomata in ewst1. Comparative genome-wide transcriptome analysis identified genes related to exocytosis, secondary metabolites, tryptophan biosynthesis, protein phosphorylation and other signalling pathways to be playing a role in enhanced response to water stress in ewst1. The possible involvement of a candidate gene with respect to the observed morpho-physiological and transcriptional changes and its role in stress tolerance are discussed. The mutant identified and characterized in this study will be useful for further dissection of water stress tolerance in rice.