Osteosarcoma: an immunophenotypic study for characterization and behavior.

Background: Osteosarcoma is the most common primary bone tumor occurring in second and third decades of life with a second peak later. Biopsy (needle or incision) is necessary for diagnosis along with imaging modalities (X-ray, CT scan etc) and serology. Due to diagnostic dilemma in certain cases and for prognosis of patients, immunohistochemistry is increasingly used. Aims: To assess the pathologic features and determinants of osteosarcoma in patients of the Indian subcontinent that would put an insight into its appearance and behavior. Methods and Material: Forty cases of biopsy proven osteosarcoma were selected over a period of three years. Histopathology was done for tumor typing, along with serology (pre and post-operative serum alkaline phosphatase). In all cases TNM staging and immunohistochemistry for antibodies to Osteonectin (ON) (diagnosis), S100 (differentiation), Ki 67 and Her2 (prognosis) was done. Results: Serum alkaline phosphatase was high in 37 (92%) cases initially and remained high in metastatic and recurrent lesions. Osteonectin was positive in 38 (95%) cases, S100 in 31 (77%), Ki 67 showed overlapping labeling indices between 4.8-18.8% and Her2 showed more positivity in higher stage tumors. Conclusions: Biopsy (along with imaging) is mandatory to diagnose osteosarcoma. Osteonectin is a good immunohistochemical marker to differentiate osteosarcoma from its mimics. For prognostication, serum alkaline phosphatase, post chemotherapy tumor necrosis (more than 90%), lack of Her2 expression are good parameters. S100 and Ki67 were found to have limited role in diagnosis and prognosis of osteosarcoma.

Urinary citrate excretion in idiopathic nephrolithiasis.

OBJECTIVE: To determine urinary citrate excretion in children with nephrolithiasis and normal controls. DESIGN: Prospective. SETTING: Tertiary care center in New Delhi. METHODS: This study was done on 50 children, below the age of 12 years, with idiopathic urinary calculi and 150 age and weight matched controls. The children were divided into 3 groups: Group 1 (1-4 years), Group 2 (5-8 years) and Group 3 (9-12 years). Urinary citrate was estimated in a 24-hour urine sample using colorimetric method. The stones removed from these children were also analysed. RESULTS: There was a preponderance of urinary stones in males; the highest incidence being in Group 1. Excretion of citrate in 24-hour urine sample was significantly lower in patients compared to controls, for males in all age groups and for females in Group 3. However, there was no statistically significant difference in the urinary citrate value between males and females in a given age group for either controls or patients. The urinary citrate excretion increased with age in patients and controls, but the levels in patients were lower. Depending upon the constituents, four types of stones were identified, calcium phosphate, calcium oxalate, uric acid and magnesium ammonium phosphate. Nine stones had at least more than one major constituent. Hypocitraturia was detected in 43 percent cases. The incidence was 76 percent for calcium phosphate, 87 percent for calcium oxalate, 40 percent for uric acid stones and 50 percent for magnesium ammonium phosphate. CONCLUSION: This study shows that low urinary citrate is associated with urinary stones in children, especially in endemic areas, in the absence of obvious etiological factors. Urinary citrate excretion should be determined in all children with nephrolithiasis.

Spinocerebellar ataxia--type 6.

Fifty six years lady presented with pure cerebellar ataxia with positive family history from paternal side presented to our clinic. DNA screening found to be SCA6. This is the first case report of SCA6 from India.

Cell mediated immune status in malignancy--pretherapy and post-therapy assessment.

Twenty-eight cases of malignancies of different kinds were studied to assess T-cell activity and population before and after institution of therapy. Fifteen cases were diagnosed as non-metastasising squamous cell carcinoma of larynx, pharynx, laryngopharynx, hypopharynx and tonsils. Seven cases were non-metastasising infiltrating duct carcinoma of breast and 6 cases were non-Hodgkin's lymphoma (NHL). It was observed that 3 out of 15 cases (20%) of squamous cell carcinoma cases were Mantoux test (MT) negative with a T-cell population of less than 40%, 2 out of 7 cases (28.6%) of infiltrating duct carcinoma of breast were MT negative with a T-cell population of less than 40% and 3 out of 6 cases (50%) of NHL were MT negative with a T-cell population of less than 40%. The normal controls, consisting of apparently normal healthy adults, had a T-cell population of more than 40% and were all MT positive. The patients who showed a negative skin test and a T-cell population less than 40% were further subjected to assessment of T-cell population and activity after appropriate therapy, and clinical cure of the disease. It was observed that 2 out of 3 cases (66.66%) of squamous cell carcinomas, 2 out of 2 cases (100%) of adenocarcinomas and one out of 3 cases (33.33%) of NHL showed positive conversion with a T-cell population of more than 40%.

Profile of electrocardiographic changes in Duchenne muscular dystrophy.

Cardiac changes often culminating in cardiac failure are at times a dramatic cause of death in patients of Duchenne muscular dystrophy. These changes are probably invariable in such cases though they may escape detection in early stages by clinical examination or radiological investigation. Electrocardiography serves as a sensitive, non-invasive and inexpensive tool to detect these changes. Fifty-four cases of Duchenne muscular dystrophy were studied clinically and were confirmed by biochemical and electroneuromyographic studies. They were then subjected to electrocardiographic studies. A number of electrocardiographic changes were observed, like tachycardia in 77.77%, deep Q in leads I, aVL, V6 in 53.70%, prolonged VAT in 37.03%, deep Q in leads II, III, aVF in 29.62% and prolonged Q-Tc interval in 25.92% cases. Some of these were distinctive enough to warrant attention for cases of Duchenne muscular dystrophy.

Effect of lead exposure on plasma phenylbutazone levels in rats.

The effect of lead exposure on phenylbutazone kinetics was studied in rats. The biological half-life (t1/2) of phenylbutazone was determined from the plasma level versus time curve in 3 groups of rats given (i) 10 mg/kg lead orally for 8 weeks (ii) 100 mg/kg single oral dose and (iii) no lead, after oral administration of 100 mg/kg phenylbutazone to all rats. The t1/2 of the drug was found to be 33% lower on chronic lead exposure and 46% higher on acute exposure than in unexposed control rats. This variation in the t1/2 values of the two different groups of rats indicates that probably phenylbutazone metabolism varies with the period of lead exposure.

Neurocysticercosis.

Sixty-two cases of neurocysticercosis (NCC) were examined over a period of five years. Convulsive seizure was the commonest presentation (57%). The other modes of presentation included features of raised intracranial pressure (19%) meningoencephalitis (9%), "Stroke" like onset (4%) and progressive dementia (6%). Clinical signs were scanty. Six patients had papilloedema, five had hemiparesis while three had isolated cranial nerve palsies. Soft tissue calcification and mucocutaneous nodules were infrequent and was found in 13 (21%) and 5 cases (8%) respectively. Clinical suspicion supported by CT scan and Immunobiologic tests using ELISA were the mainstay in diagnosis. Praziquantel and Albendazole were found effective in the treatment of neurocysticercosis, but because of serious side effects encountered in some cases, the drugs should be used cautiously in selected cases only.

Blood lead levels of children with pica and surma use.

Blood lead levels of 253 Delhi children were estimated by dithizone method. In 82 (controls) children with no symptoms mean blood lead level was 9.6 micrograms/dl (+/- SD 6.8: median 10 micrograms); only 6 had high levels between 30-33 micrograms/dl. In 88 children with pica, the mean blood lead level was 23.0 micrograms/dl (+/- SD 13.82; median 17 micrograms) which was significantly higher than the control; 26 had high levels between 30-92 micrograms/dl. Sixteen children with pica and surma-use and 46 children suspected of lead poisoning showed lead level patterns like the pica group. However, 21 surma-using children without pica resembled the control group. Children with pica were significantly more anemic than the controls and showed higher prevalence of abdominal-neurological symptoms. Because, in India, blood lead cannot be estimated in most of the hospitals, it is suggested that children with severe pica, anemia, abdominal-neurological symptoms and exposure to surma or lead, be suspected of lead poisoning, kept in lead-free environment with corrected nutrition, and be given a short cautious therapeutic trial with oral penicillamine.

DDT levels in human body fat & milk samples from Delhi.

A study of 45 human fat samples from autopsy and surgery specimens from Delhi showed a mean total DDT level of 6.37 micrograms/g (SE +/- 0.36; range 3.34-9.12 micrograms/g). While these values compare well with those reported from India and abroad, they are much lower than those obtained from other areas of Delhi, confirming wide variation in the quantity of DDT sprayed in different pockets of this city. Twenty one human milk samples from Delhi showed a mean total DDT level of 0.144 ppm (micrograms/ml). Thus, many infants in Delhi appear to be consuming 3 to 5 times the permissible 5 micrograms/kg/day quantity of DDT. This study too indicates the need of continuous monitoring of DDT and other insecticides commonly used in India.

Some aspects of pharmacological profile of sodium curcuminate.

Rapid i.v. injection of sodium curcuminate (NaC) produced transient hypotension and bradycardia in anaesthetized dogs and cats which were not blocked by bilateral vagotomy, atropine, mepyramine or propranolol. In open-chest anaesthetized cats, decrease in blood pressure and heart rate was accompanied by simultaneous transient reductions in left ventricular systolic pressure, maximal rate of rise of left ventricular pressure and a concomitant increase in left ventricular end-diastolic pressure. It was concluded that the transient hypotensive effect of NaC is due to its myocardial depressant action. NaC exhibited negative inotropic and chronotropic effect on isolated perfused rabbit heart, an antispasmodic effect on smooth muscle of dog s intestine in vivo and of vas deferens of guinea-pig in vitro but no effect on the rectus abdominis muscle of frog or its response to cholinergic stimulation.