Diabetes renalis

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Classification according to ICD-10
E74.8 Other specified disorders of carbohydrate metabolism
R81 Glucosuria
ICD-10 online (WHO version 2019)

As diabetes, renal dysfunction of the kidney is known, by a permanent excretion of glucose in the urine ( glucosuria ) with normal glucose tolerance and non-elevated blood glucose level is marked. Synonyms names are renal glucosuria and, less commonly, diabetes innocens , diabetes innocuus , kidney diabetes , Nierenharnruhr and normoglycemic glucosuria .

The symptoms of diabetes renalis were first described in 1895. The Swiss pediatrician Guido Fanconi coined the term familial renal glucosuria for this form of carbohydrate metabolism disorder . In addition to congenital diabetes renalis, there are also acquired and temporary forms. Since this disorder is usually almost symptom-free and therefore remains symptom-free for the patient and there are no known late effects, therapy is usually not necessary.

distribution

There are no reliable data on the prevalence and incidence of primary and secondary diabetes. In an older publication, Horst Bibergeil gives an estimate of 0.3 percent for the frequency of the familial form; according to this information, the age group from 18 to 49 years is primarily affected. However, due to the mostly symptom-free course, this may also be due to a late diagnosis as part of a routine examination. Men are more often affected than women .

Cause and development of the disease

Primary forms

The cause of primary or familial diabetes renalis, i.e. the form that does not arise as a result of other diseases, is an inherited disorder of the reabsorption of glucose in the kidneys. The blood glucose , which initially reaches the primary urine via the glomeruli when urine is formed in the kidneys , is normally fully actively reabsorbed from there and fed back into the bloodstream. The reabsorption takes place in the proximal tubular cells . A disruption of this reabsorption of glucose in the kidneys can either be inherited as an autosomal incomplete recessive trait (familial form of diabetes renalis), or it is due to acquired changes in kidney function. There are two possibilities as a physiological basis, on the one hand a restricted function of the glucose transporter in the kidney with a reduced reabsorption for glucose (reduced maximum glucose transport capacity) and on the other hand a reduction of the kidney threshold for glucose with an increased release of glucose into the urine ( normal maximum glucose transport capacity)

According to Reubi , a distinction can be made between type A, the simultaneous presence of both disorders, and type B, the sole presence of a reduced renal threshold with a normal reabsorption rate. However, this distinction is questioned by some authors. Type 0 is a form that has only been described so far and is characterized by a complete lack of reabsorption. A mutation in the gene for the sodium-glucose cotransporter SGLT2 has been identified in the kidneys for the autosomal incompletely recessive form of diabetes renalis . Further studies have shown that the extent of glucosuria probably depends on the type and location of the mutation and whether the mutation is homozygous or heterozygous .

Secondary forms

In secondary diabetes renalis, i.e. the acquired form, there is usually a lowering of the renal threshold . A distinction must be made, on the one hand, between temporarily existing conditions and, on the other hand, permanently increased glucose excretion as a side effect of other diseases.

A temporary form of diabetes renalis can occur, for example, during pregnancy and is then also referred to as pregnancy glucosuria. The cause here is a pregnancy-related increase in adrenal hormones . Temporary renal glucosuria has also been described as a possible side effect of tetanus disease and kidney transplants . Transient glucosuria in newborns is also common.

One possible cause of permanent secondary diabetes is acute or chronic inflammation of the kidney. Other diseases or conditions in which glucosuria can appear temporarily or permanently as a symptom are, for example, acromegaly , Cushing's disease , hyperthyroidism , pheochromocytoma , tumors of the alpha cells of the islets of Langerhans , myocardial infarction , pancreatitis , pneumonia , high blood pressure , liver cirrhosis , Wilson's disease , Diseases or injuries of the central nervous system , Fanconi syndrome , various nephropathies , poisoning , prolonged fasting conditions and acute or chronic oxygen deficiency . In these cases, the specific differentiation from primary diabetes renalis is made by means of a corresponding differential diagnosis .

Clinical manifestations

The diagnosis of diabetes renalis is often an accompanying finding of other examinations, since in the majority of cases there are no clinically meaningful symptoms when this disorder is present alone. The occurrence of increased urine excretion ( polyuria ) and an increased feeling of thirst and hunger ( polydipsia and polyphagia ) is possible. A diabetic renal can, usually during exercise, due to the increased glucose excretion occasionally also cause reduced blood sugar levels and thus temporarily symptoms of mild hypoglycemia ( hypoglycemia trigger). About ten to twelve percent of those affected occasionally suffer from shortness of breath ( dyspnoea ), weakness, nervousness, tiredness or tightness in the chest ( stenocardia ).

Investigation methods

The most important diagnostic feature of diabetes renalis is the evidence of permanent excretion of glucose in the urine . A characteristic of diabetes renalis is the constancy of glucosuria, largely independent of food intake, physical stress or the administration of insulin or oral antidiabetic drugs . For differential diagnosis (differentiation) against a diabetes mellitus in which a glucosuria may also occur as a symptom, is an oral glucose tolerance test , the normal precipitates in the presence of a renal diabetes. A family history can also help in making the diagnosis. To distinguish it from other disorders of kidney function, it is possible to determine the excretion rate of other substances that are reabsorbed in the proximal tubules.

The ICD-10 code for diabetes renalis is E74.8, together with essential pentosuria , oxalosis and oxaluria as "other specified disorders of carbohydrate metabolism". The code for glucosuria of unknown cause is R81.

Treatment and prospect of recovery

In the isolated presence of diabetes renalis, specific therapy is usually not necessary due to the lack of clinically relevant effects. A causal treatment is just as unavailable as a symptomatic treatment that focuses on the key symptom of increased urine output. Therapeutic measures are therefore limited to the secondary symptoms such as the occasional occurrence of hypoglycaemia or shortness of breath in situations in which these reach a level that requires treatment.

No long-term consequences are known for diabetes renalis, so that a mild ( benign ) course without progressive deterioration ( progression ) is generally assumed. The level of glucosuria can even decrease in the course of life, as it can lead to an increase in the kidney threshold, which also occurs in healthy people. In the presence of mild diabetes renalis without the occurrence of further symptoms such as polyuria, polydipsia, polyphagia and hypoglycaemia, its relevance as a disease is partly called into question for these reasons.

A transition from diabetes renalis to diabetes mellitus is documented several times in the literature in the form of case descriptions. However, it is not certain whether the presence of diabetes renalis is generally associated with an increased risk of later developing diabetes mellitus. A related long-term follow-up study did not provide any corresponding indications.

literature

Web links

Individual evidence

  1. ↑ a b c Dietrich Michaelis: Differential diagnosis of diabetes mellitus. Section 7.3.1: Diabetes renalis (familial glucosuria). (Pp. 193–194) and Section 7.3.2: Concomitant glucosurias in endocrine and organic diseases. (Pp. 194-195). In: Horst Bibergeil (Ed.): Diabetes mellitus. A reference work for diabetological practice. 3rd, expanded, redesigned edition. VEB Gustav Fischer Verlag, Jena 1989, ISBN 3-334-00087-7 .
  2. ↑ Sabine Scholl-Bürgi, René Santer, Jochen HH Ehrich: Long-term outcome of renal glucosuria type 0: the original patient and his natural history. In: Nephrology Dialysis Transplantation , Volume 19, No. 9, 2004, ISSN  0931-0509 , pp. 2394-2396, doi: 10.1093 / ndt / gfh366 .
  3. ↑ Lambert P. van den Heuvel, Karin Assink, M. Willemsen, Leo Monnens: Autosomal recessive renal glucosuria attributable to a mutation in the sodium glucose cotransporter (SGLT2). In: Human Genetics , Vol. 111, No. 6, 2002, ISSN  0340-6717 , pp. 544-547, doi: 10.1007 / s00439-002-0820-5 .
  4. ↑ Willy Weiya Chen, Linda Sese, Prayuth Tantakasen, Vincent Tricomi: Pregnancy associated with renal glucosuria. In: Obstetrics & Gynecology , Vol. 47, No. 1, 1976, ISSN  0029-7844 , pp. 37-40, online .
  5. ↑ Gholam-Reza Rezaian, Parviz Khajehdehi, Shohreh Beheshti: Transient Renal Glucosuria in Patients with Tetanus. In: Nephron , Volume 80, No. 3, 1998, ISSN  0028-2766 , pp. 292-295, doi: 10.1159 / 000045189 .
  6. ↑ Antonio Secchi, Aoumeur Hadj-Aissa, Nicole Pozet, Jean Louis Touraine, Guido Pozza, Jean M. Dubernard, Jules Traeger: Renal glucose transport after kidney transplantation. In: European Journal of Clinical Investigation , Volume 14, No. 2, 1984, ISSN  0014-2972 , pp. 142-145, doi: 10.1111 / j.1365-2362.1984.tb02103.x .
  7. ^ Ten-year follow-up report on Birmingham Diabetes Survey of 1961. Report by the Birmingham Diabetes Survey Working Party. In: British Medical Journal . Vol. 2, No. 6026, 1976, pp. 35-37, JSTOR 20410465 .