Normal blood sugar levels chart for adults, This simple chart shows target blood sugar levels for before and after meals, after fasting, before exercise, and at bedtime, as well as an a1c target.. Diabetes blood sugar levels chart: what is a normal blood, Keep in mind that the blood glucose level before a meal for a non diabetic person and a person with prediabetes may be very similar. Blood sugar – wikipedia, the free encyclopedia, The blood sugar concentration or blood glucose level is the amount of glucose (sugar) present in the blood of a human or animal. When your “normal” blood sugar isn’t normal (part 1), In the next two articles we’re going to discuss the concept of “normal” blood sugar. Blood glucose levels : testing and normal range, A blood glucose test measures the amount of a type of sugar, called glucose, in your blood. Home « blood sugar basics, Make your next conversation with your diabetes healthcare team count by asking these quick questions about blood sugar!. Alkaptonuria is a rare disorder of metabolism caused by deficiency of homogentisic acid oxidase enzyme and characterized by triad of homogentisic aciduria (dark urine), relentlessly progressive arthritis and ochronosis.
A 49 year male, was admitted in medical ward for evaluation of progressive low backache of 15 years duration.
Biochemical examination of the urine with ferric chloride and silver nitrate were positive.
The disease is the result of deficiency of homogentisic acid (HGA) oxidase leading to accumulation of HGA in various tissues which later polymerizes to benzoquinones forming melanin-like polymers.6 There are several theories as to how alkaptonuria results in ochronosis and the associated connective tissue changes.
In the neutral, relatively anaerobic environment of the body, HGA polyphenol oxidase, an enzyme found in skin and connective tissue cells,is required to catalyze the reaction. The hallmark of the disorder is a triad of degenerative arthritis, ochronotic pigmentation and urine that turns black on alkalanization. Benedicts test: gives dark supernatant, initial greenish brown precipitate followed by a yellow precipitate of cuprous oxide on standing. Detecting and measuring the amount of homogentisic acid in the urine confirms the diagnosis of this disease. Recent diagnostic innovations have advanced the characterization of alkaptonuria as a disease entity.
While it is true that the diversity of presentation in ochronosis has seemingly increased, it should also be noted that advances in technology have promoted the detection of many manifestations that would have remained unseen in early studies. Diffusion- weighted and gradient echo magnetic resonance findings of hemichorea-hemiballismus associated with diabetic hyperglycemia: A hyperviscosity syndrome? Putaminal petechial haemorrhage as the cause of non-ketotic hyperglycaemic chorea: A neuropathological case correlated with MRI findings. We have documented a case with typical features of alkaptonuria along with intramedullary calcification which has not been reported in the literature before.
The three major features of alkaptonuria are the presence of homogentisic acid in the urine, ochronosis (bluish-black pigmentation in connective tissue) and arthritis of the spine and large joints. There was history suggestive of gradually worsening arthritis of bilateral knee, hip, shoulder and elbow joints for last 12 years which used to increase in winter and decrease in summer initially. CT of lower end of left femur shows intra medullary calcification and soft tissue calcification (Fig. It has the distinction of being the first disease in which a Mendelian recessive inheritance pattern was proposed and described under the heading of a€?inborn errors of metabolisma€™. The HGA may act as a chemical irritant, causing inflammation and more rapid degeneration of the joints.


6 One of the first symptoms of alkaptonuria is darkening of the urine upon standing which is of normal color when fresh. This is done by enzymatic spectrophotometry, gas liquid chromatography or high pressure liquid chromatography.
So active surveillance for cardiac, renal, and prostate complications after the fourth decade of life should be done.2 There are anecdotal reports that a diet low in protein especially in amino acids, phenylalanine and tyrosine can help delay joint problems. This case of alkaptonuria is unique for its presentation with intramedullary calcification. While these manifestations have long been recognized, new imaging techniques allow the diagnosis of other connective tissue manifestations of alkaptonuria that often constitute the seminal presentation of ochronosis.1 In this report, we describe a 49 year old male who presented with typical features of alkaptonuria along with intramedullary calcification which has not been reported in the literature before. It is also possible that HGA physically binds to connective tissue and alters the structure and interactions of the macromolecules.1 Other evidence suggests that it is not HGA itself but a byproduct of its oxidation that causes degeneration. CT and echocardiographic studies can reveal coronary-artery calcifications and cardiac-valve defects, respectively. Vitamin C, an anti-oxidant given in the dose of 500 mg twice a day to reduce connective tissue damage has been tried. The literature of clinical features along with management of this rare condition is reviewed in this article. As HGA accumulates both inter and intracellularly, it is oxidized to benzoquinoneacetate (BQA).This oxidation occurs spontaneously in urine, where the environment is alkaline and aerobic.
It is also pH-dependent and hence in some patients it is never seen if their urine has an acidic pH. It partially ameliorates the condition by impeding the polymerization of HGA.6 Ochronotic arthropathy is treated with physiotherapy, analgesia, rest, and prosthetic joint replacement when necessary.
Patient had noticed black discolouration at places soiled with his urine passed few hours back since childhood.
A review of cases of alkaptonuria throughout the world noted that approximately 600 cases had been described through 1962.
Dark urine stains on the diaper are sometimes the first telltale sign of the disease in infants.
Darkening of the urine is the only feature suggestive of alkaptonuria in the pediatric age group in most patients. There was HC with occasional ballismic component and mild hypotonia in left upper and lower limbs [Video 1]. On further review patient gave history of noticing change in colour of urine to black when left standing for few hours. Apart from the above phenomenon; the patient is usually asymptomatic until the third or fourth decade. However, the average life span of the patient is unchanged and they die of causes comparable with the general population.
Nitisinone a triketone herbicide has been shown to significantly lower the urinary excretion of HGA in both murine models and humans. As was told to patient by his mother there was history of darkening of clothing moistened with urine to black colour, when left unwashed for many hours. There is bluish black or grayish blue pigmentation of the outer ocular tissues like the sclera and cornea and conjunctiva. Nitisinone is approved by the Food and Drug Administration for the treatment of tyrosinemia type I.


Side effects of nitisinone are elevated plasma tyrosine levels and resulting corneal irritation. He was the product of nonconsanguineous marriage and was the first of three children in birth order. Long term studies are required regarding the dose, safety profile and outcome parameter of nitisinone therapy in alkaptonuria. Blood counts, liver, and renal functions, chest radiograph, ultrasonography of abdomen, blood and urine cultures, and ECG were normal. Effects are most noticeable in areas where the body is exposed to the sun and where sweat glands are located. On examination patient was detected to have grade II hypertension (JNC VII) with grade 3 hypertensive retinopathy.
The ochronotic changes are best appreciated in regions in which the overlying skin is of minimal thickness.
One of the first sites to be involved is the ear cartilage, which becomes thickened with bluish black pigmentation. Examination of the spine revealed tenderness in the dorsolumbar region and the movements were restricted in all directions. Nonketotic hyperglycemia remains an underecognized cause of HC-HB probably due to lack of awareness of this entity as well as reversibility in euglycemic state. He had a painful and restricted range of movements of both knee joints, both hip joints right more than left, bilateral shoulder and elbow joints. 1), ochronotic pigmentation of the skin over dorsum of hands, nose, cheeks, forehead, eyelids, ear lobes and nail beds. Hyperglycemia-induced regional cerebral blood flow impairment in the absence of ketoacidosis leads to attenuation of Kreb's cycle in brain and increases gamma-aminobutyric acid depletion via the succinic semialdehyde pathway. Systemic examination of cardiovascular system, respiratory system, abdomen and central nervous system was normal.
Complete haemogram, renal function tests, liver function tests, uric acid and calcium profile were within normal limits.
Older women are predisposed due to postmenopausal estrogen deficiency causing dopamine hypersensitivity. Awareness of HC-HB with HHS is important - it may be initial presentation of diabetes mellitus.
In diabetics presenting with movement disorders, this reversible entity should be considered. Pigment deposits also can lead to the formation of atherosclerotic plaques containing cholesterol and fat. Pigment deposits form stones in the prostate, kidneys and urethra probably because of the extremely high levels of urinary HGA excretion.



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