Wilms' tumor presenting as Cushing's syndrome.
We report a 2-year-old boy presenting with Cushing's syndrome caused by a Wilms' tumor. This is the fifth such case reported in the English literature.
Biomedical subjects
Publications and source records attributed to M S Seshadri.
We report a 2-year-old boy presenting with Cushing's syndrome caused by a Wilms' tumor. This is the fifth such case reported in the English literature.
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BACKGROUND: Following successful renal transplantation, blood erythropoietin (Epo) levels peak in two phases during the first 2-3 months, and blood haemoglobin/haematocrit (HB/Hct) levels are restored to normal in a period of 2-6 months. However, some transplant recipients continue to remain anaemic in spite of normal graft function and in the absence of recognizable causes. The role of endogenous Epo production in the causation of anaemia in such patients is poorly understood and has been investigated in this study. METHODS: Twenty-three post-renal transplant recipients with stable normal renal function were studied. Eleven of these patients had normal HB/Hct levels (group 1) and served as control for the rest 12 patients with anaemia (group 2). Patients included in group 2 had no readily recognizable cause for their anaemia. Other laboratory and clinical findings were similar in both groups. Patients with erythrocytosis were excluded. Serum Epo levels were measured in all patients. Five patients in group 2 were treated with recombinant human erythropoietin (rHuEpo) and their erythropoietic response was assessed. rHuEpo was discontinued when the target Hb/Hct levels (lowest normal range) were achieved and the patients were followed up for a further period of 9-12 months. RESULTS: Five patients in group 1 had normal expected serum Epo levels whereas the other six patients had inappropriately high serum Epo levels with respect to their Hb/Hct status suggestive of relative ¿EPO resistance'. Serum Epo levels in all patients except two in group 2 were low indicative of 'Epo deficiency'. The two exceptional patients in group 2 had higher serum Epo levels in the presence of anaemia suggestive of relative ¿Epo resistance'. All five patients treated with rHUEpo responded adequately by achieving normal Hb/Hct levels. Three of them were originally ¿Epo deficient' and they reached target Hb/Hct levels in a mean period of 4 weeks, requiring a mean cumulative rHuEpo dose of 428.3 units/kg. The other two patients with higher initial serum Epo levels, and considered to be ¿Epo resistant' required an average of 11 weeks of treatment and a mean cumulative rHuEpo dose of 1582.5 units/kg, indicating an increased Epo demand. On cessation of therapy the Hb/Hct levels fell in all five patients to pretreatment values in 6 months. CONCLUSIONS: There are important variations in the endogenous Epo production in renal transplant patients with normal renal function, the cause of which is not clear. Epo deficiency and relative Epo resistance play a causative role for anaemia in some post-renal transplant recipients with stable normal renal function. They respond adequately to rHuEpo administration.
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Four cases of the rare disorder, inherited degenerative chondropathy have been previously reported (Kurien et al., 1989). A five-year follow-up of these patients and two additional cases are presented in this report. The progress of this disease appears to be arrested after regular dapsone therapy and there was no other organ involvement noted during the follow-up period.
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OBJECTIVES: To find out the common causes of amenorrhea and oligomenorrhea in south Indian women and to draw up a protocol for evaluation of women with these problems. METHODS: A retrospective study of 426 women with amenorrhea and oligomenorrhea was carried out. RESULTS: Forty-one patients had primary amenorrhea, 132 had secondary amenorrhea and 289 women presented with oligomenorrhea. Hypergonadotropic amenorrhea and congenital absence of the uterus and vagina accounted for 60% of primary amenorrhea. Chronic anovulatory disorders and premature ovarian failure were found in 72% of women with secondary amenorrhea but weight loss related amenorrhea was uncommon. CONCLUSIONS: On the basis of the observations made, a protocol for evaluation and management of women with amenorrhea and oligomenorrhea was evolved.
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This study was conducted to examine systematically the endocrine function in 10 patients diagnosed to have renal amyloidosis. Basal and dynamic endocrine tests (GnRh, TRH, and ACTH stimulation tests as well as modified glucose tolerance test) were performed in all patients. Eight patients had advanced renal disease (creatinine clearance < 10 mL/min). Hypofunction of the thyroid gland was present in 5 out of those 8 patients, and thyroid biopsy confirmed amyloid deposition. Of those 5 patients, pituitary and adrenal defects were present in 2. In conclusion, endocrine abnormalities were evident at a later stage of renal amyloidosis. Hypothyroidism was present in all patients who manifested endocrine defects and should be considered as an early sign of endocrine involvement with the disease.
A retrospective analysis of 55 cases of osteomalacia shows that poor calcium intake and poor sunlight exposure are the most common causes for osteomalacia. However, in patients with normal nutritional history, other disorders such as renal tubular acidosis and tumour induced osteomalacia should be looked for. A careful drug history, particularly anticonvulsant use is essential. In 4 patients there was an association between anti-tuberculous drug use and osteomalacia. Further prospective studies are needed to determine the relationship between Rifamicin use and osteomalacia.
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Seventy one cases of hyperproloactinemia (HPRL) in women were seen over five years. Of these, twenty five had pituitary tumors, thirty three had functional HPRL, five had drug induced HPRL and four had hypothyroidism. Surgery was undertaken in fifteen women, rest were treated with bromocriptine. There were thirteen pregnancies in patients with tumor after treatment. Seven of thirty three patients with functional HPRL conceived. No patient had tumor expansion or recurrence during pregnancy. Bromocriptine was required for most patients.
Exogenous contamination poses a major problem to accurate determination of trace metals in biological samples. Analysis of these elements in biological material entails adoption of special precautionary measures. In this study we have assessed the various procedural steps of sampling and analysis of zinc in order to identify and minimize extraneous contamination in the laboratory. In addition we have established reference limits (2 SD) for zinc in human plasma, erythrocytes and hair.
Primary hyperaldosteronism was detected in 1% of patients evaluated for secondary hypertension in a referral hospital in Southern India. The presence of hypokalemia with inappropriate kaliuresis (24 hr. urine K > 20mEq) was an important diagnostic clue. High resolution CT scans were found to be useful for localising the tumour. Preoperatively nifedipine and spironolactone were employed to correct hypertension and hypokalemia. The same drugs were also found to be excellent for long-term control of hypertension and hypokalemia in patients who had bilateral adrenal hyperplasia and aldosteronism.