Diabetes Case Reports

Therapeutic Strategies in Wolfram Syndrome and Diagnostic Problems in Diabetes

Wenkins Ruben^* Department of Diabetes and Metabolic Diseases, University of Bilkent, Ankara, Turkey
^*Correspondence: Wenkins Ruben, Department of Diabetes and Metabolic Diseases, University of Bilkent, Ankara, Turkey, Email:

Received: 19-Aug-2022, Manuscript No. DCRS-22-18227; Editor assigned: 23-Aug-2022, Pre QC No. DCRS-22-18227 (PQ); Reviewed: 12-Sep-2022, QC No. DCRS-22-18227; Revised: 20-Sep-2022, Manuscript No. DCRS-22-18227 (R); Published: 28-Sep-2022, DOI: 10.35841/2572-5629.22.7.131

Description

Several bodily systems are impacted by the illness known as Wolfram syndrome. Wolfram syndrome is characterized by high blood sugar levels brought by a lack of the hormone insulin (a disease known as diabetes mellitus) and gradual vision loss brought on by neurons that transmit information from the eyes to the brain degenerating (a condition called optic atrophy). In addition to urinary tract issues, neurological or mental diseases, sensor neural deafness, hearing loss brought on by changes in the inner ear, and pituitary gland malfunction that leads in excessive urine output are all common in people with Wolfram syndrome. The first sign of Wolfram syndrome, which is often identified around age 6, is typically diabetes mellitus. Insulin replacement therapy is necessary for almost everyone with Wolfram syndrome who develops diabetes mellitus. The following sign frequently appears after optic atrophy, generally around age 11. Loss of colour vision and side (peripheral) vision are the initial symptoms of optic atrophy. People with optic atrophy typically become blind within 8 years after the initial signs of optic atrophy, as the visual issues worsen with time.

Causes and effects

Wolfram syndrome can have a wide range of symptoms and rates of development. Diabetes mellitus, optic atrophy, diabetes insipidus, and hearing loss-the four main symptoms of Wolfram syndrome can appear at various ages and progress at various rates. A WFS1-related disorder would be used to describe the patient's condition if some of these symptoms never manifest. Diabetes insipidus can occur in certain Wolfram syndrome patients (42%). Insulin or diabetes has nothing to do with this. Only the signs of increased thirst and urination are similar to those of diabetes.

Before the age of 16 (87% of those with wolfram syndrome), they begin to develop insulin-dependent diabetes. The digestive system typically converts the starches and sugars (carbohydrates) in our diet into glucose, which circulates in the blood as one energy source for bodily operations. Muscle and fat cells can take up glucose to a hormone called insulin that is produced by the pancreas.

In diabetes mellitus, the pancreas produces insufficient amounts of insulin, which prevents cells from properly absorbing glucose, causing the blood sugar to become too high. Patients with Wolfram-gene-associated diabetes mellitus require daily insulin injections to maintain blood sugar control. Frequent urination, excessive thirst, an increased hunger, weight loss, and blurred vision are all possible signs of diabetes.

• In diabetes insipidus, the posterior pituitary gland produces insufficient amounts of the hormone vasopressin, preventing the body from concentrating urine. As a result, having extreme thirst, frequent urination, and highly diluted urine.

• In optic atrophy it refers to the optic nerve's gradual wasting away, which results in colour blindness and progressive eyesight loss. At some point, optic atrophy will affect everyone with Wolfram Syndrome.

• In Thiamine the megaloblastic anemia, sensorineural hearing loss, and diabetes mellitus are all characteristics of the autosomal recessive condition known as Responsive Megaloblastic Anaemia Syndrome (RMAS).

Conclusion

In order to successfully treat WS, a rare and deadly condition that affects various organs and systems, a multidisciplinary approach is necessary. Through the avoidance and treatment of complications, an early diagnosis reduces morbidity and mortality. WS is a monogenic disorder, making it a better model for studying the processes of stress and how this situation results in cell death than other common diseases where a number of variables interact to cause the disease's symptoms. The identification of novel clinical characteristics not previously described, including pituitary dysfunction, specific neurological manifestations, and heart abnormalities, may lower the morbidity and mortality of WFS.