ALKAPTONURIA SYNDROME-A REVIEW

Alkaptonuria, also called endogenous ochronosis, and also called as Black Urine Disease, is a rare metabolic autosomal recessive disorder. It occurs by complete inhibition of homogentisic acid oxidase enzyme having its deposition in various tissues. Alkaptonuria is caused due to deficiency of homogentisic acid oxidase involved in the metabolism of tyrosine. Dark discolouration of urine, ochronosis at cartilage and connective tissues, arthritis at the third of fourth decade of life, renal stone disease, spontaneous tendon rupture etc. May be seen in alkaptonuria. Disease severity varies among individual patients, even between siblings, and increase with age because of homogentisic acid accumulation. Usually, life span is not shortened in AKU, but the quality of life is severely effected. Several studies have suggested that Nitisinone may be effective in the treatment of alkaptonuria. Characteristically, the excess HGA means sufferers pass dark urine, which upon standing turns black. This is a feature present from birth. Over time patients develop other manifestations of AKU, due to the deposition of HGA in collagenous tissues, namely ochronosis and ochronotic osteoarthropathy. Although this condition does not reduce life expectancy, it significantly affects the quality of life. The natural history of this condition is becoming better understood, despite gaps in knowledge. Clinical assessment of the condition has also improved along with the development of potentially disease-modifying therapy. Furthermore, recent developments in AKU research have to lead to new understanding of the disease, and further study of the AKU arthropathy has the potential to influence therapy in the management of osteoarthritis

<b>Interaction of 2-(2’-hydroxy)phenylbenzothiazoline with some metal ions: determination of its dissociation constant in aquo-organic media</b>

Potentiometric titrations were carried out with 2-(2'-hydroxy)phenyl benzothiazoline (HPBT) in various 50%v/v aquo-organic media (dioxan-water, acetone-water, methanol-water, and ethanol-water) and in different percentages of dioxan. The results indicated that the ligand is a monoprotic acid with pKa corresponding to the hydroxyl group. The pKa value of HPBT determined in various aquo-organic media followed the regular order of the reciprocal of dielectric constant. The solid metal complexes employing HPBT were synthesized and characterized. Spectral studies of these metal complexes [M(II) = Co, Ni, Cu and Zn] complexes indicated ring cleavage and formation of bis benzylidene disulphide (BBDS) during complex formation

Cytology of suture granuloma in a recurrent thyroid nodule

Foreign body giant cell (FBGC) reaction leading to suture granu-loma is known to occur in response to suture material used during surgery. It occurs most commonly in the gastrointestinal tract (GIT). It is rare to find a suture granuloma in the thyroid. After ex-tensive search of the literature, it was found that such a lesion has been reported so far on guided aspirates, but not on fine needle aspiration cytology (FNAC) alone. The cytomorphology on FNAC is important to clinch the diagnosis and avoid unnecessary surgery as it can mimic a malignant thyroid nodule, multinodular goiter or a metastatic lymph node. This is the first case report of such a lesion to date. We report a 54 year old male with a recurrent firm thyroid nodule on the right side of the neck, for whom FNAC was done. It was reported as a suture granuloma on cytology. Hence the case was managed conservatively since malignancy was ruled out