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KIMMELSTEIL WILSON SYNDROME TREATMENT in Nepal

KIMMELSTEIL WILSON SYNDROME, also known as diabetic kidney disease, is the chronic loss of kidney function occurring in those with diabetes mellitus. Diabetic nephropathy is the leading causes of chronic kidney disease (CKD) and end-stage renal disease (ESRD) globally.

The triad of:

  • protein leaking into the urine (proteinuria or albuminuria),
  • rising blood pressure with hypertension and
  • then falling renal function is common to many forms of CKD. 

Protein loss in the urine due to damage of the glomeruli may become massive, and cause a low serum albumin with resulting generalized body swelling (edema) so called nephrotic syndrome.

SIGNS AND SYMPTOMS

  • The onset of symptoms is 5 to 10 years after the disease begins.
  • A usual first symptom is frequent urination at night: nocturia.
  • Other symptoms include tiredness, headaches, a general feeling of illness, nausea, vomiting, frequent daytime urination, lack of appetite, itchy skin, and leg swelling.
  • The clinical presentation of diabetic nephropathy (DN) is characterized by
  • proteinuria (protein in the urine),
  • hypertension and
  • progressive loss of kidney function.
  • The process may be initially indolent, making regular screening for diabetic nephropathy in patients with diabetes mellitus of great importance

RISK FACTORS

  • Not all patients with diabetes go on to develop diabetic nephropathy. The main risk factors that increase the likelihood of developing diabetic nephropathy are:
  • Poor control of blood glucose
  • Uncontrolled high blood pressure
  • Type 1 diabetes mellitus, with onset before age 20
  • Past or current cigarette use
  • A family history of diabetic nephropathy- certain genes have been identified that are associated with DN. ( However, no direct correlation has been established yet.[19] One of these genes is APOL1, which has been found to be associated with nephropathy in African American individuals.
  • Certain racial groups (African Americans, Mexican Americans, and Pima Indians are at higher risk).

DIAGNOSIS

Diagnosis is based on the measurement of abnormal levels of urinary albumin in a diabetic coupled with exclusion of other causes of albuminuria. Albumin measurements are defined as follows:

  • Normal albuminuria: urinary albumin excretion <30 mg/24h;
  • Microalbuminuria: urinary albumin excretion in the range of 30–299 mg/24h;
  • Macroalbuminuria: urinary albumin excretion ≥300 mg/24h

Urinary albumin excretion can also be measured by urinary albumin/creatinine ratio in a spot urine sample, which is as accurate but more convenient than a 24-hour urine collection.