Secondary thrombocytosis

To estimate the incidence and causes of secondary thrombocytosis in children, a 12 month study of all patients attending a children's hospital and discovered to have a platelet count over two times the upper normal limit (> 800 x 10(9)/l) was undertaken. Data so obtained were analysed both separately and together with those from two previous studies to gain as broad a perspective as possible. Of 7916 children who had platelet counts during the study period, 36 (0.5%) produced a value > 800 x 10(9)/l; there were 19 boys and 17 girls. There was a preponderance of young infants (median age 13 months). Twenty seven of the 36 had some sort of associated infection, bacterial in 18 and viral in nine. The other nine were either recovering from anti-neoplastic chemotherapy (n = 6), were post-operative (n = 2), or simply iron deficient (n = 1). Combining these patients with those described in previous studies allowed a review of 139 unselected children with very high platelet counts. Fifty three (38%) had infections, 29 (20%) had traumatic or surgical tissue damage, 16 (11%) had malignant disease undergoing chemotherapy or surgery, and 13 (9%) had connective tissue or autoimmune disorders. Secondary thrombocytosis is not rare and is most frequently seen in very young infants after infection. It can arise in a wide variety of other circumstances including rebound from myelosuppression, iron lack, or as part of an acute phase response. It is clinically unimportant in terms of morbidity and requires no treatment other than that for the primary condition.     

Congenital thiopurine methyltransferase deficiency and 6-mercaptopurine toxicity during treatment for acute lymphoblastic leukaemia

Two children with acute lymphoblastic leukaemia (ALL) taking daily 6-mercaptopurine as part of a national UK therapeutic trial repeatedly developed profound myelosuppression on 25% of the standard protocol dose. Both were found to have undetectable intracellular activity of thiopurine methyltransferase (TPMT), an enzyme controlling one of the major alternative catabolic pathways of 6-mercaptopurine, and both produced higher concentrations of cytotoxic drug metabolites at 10-25% of the protocol dose than other patients taking 100%. It is supposed that these patients represent the 0.33% of the normal population constitutionally lacking TPMT. It is important to recognise such individuals both to avoid fatal bone marrow failure through inadvertent overdosage, and to be reassured that an adequate drug effect can be achieved at around 10% of the standard dose.     

Pedunculated pleomorphic adenoma of the tongue base manifesting with dysponea. A case report

Pleomorphic adenomas of the minor salivary glands are rare. The most common site is in the palate. We have come across a case of pedunculated pleomorphic adenoma of the base of the tongue which came to us only when the tumour had caused respiratory distress.     

Multiple myeloma: evaluation by CT

Although patients who have multiple myeloma usually have straightforward clinical symptoms and corroborative radiographs, in some instances, these patients will present atypically, with symptoms suggesting active disease but radiographs that are normal or nonspecific. We reviewed the records of 32 patients who had documented multiple myeloma and had undergone CT examinations, assessing the value of those examinations. Although CT is not indicated in all patients who have multiple myeloma, it is especially useful in patients who have bone pain and normal or nonspecific radiographs. CT provided confirmatory information in all cases in which lesions were seen on radiographs. CT also frequently demonstrated a greater extent of disease than could be appreciated on the radiographs.     

Tarsal navicular stress fractures: radiographic evaluation

Tarsal navicular stress fractures are a potential source of disabling foot pain in physically active individuals. The diagnosis of tarsal navicular stress fracture requires a high index of clinical and radiographic suspicion because the fracture is only rarely evident on routine radiographs or standard tomograms. The radiographic diagnosis of a tarsal navicular stress fracture may require anatomic anteroposterior tomograms or a radionuclide bone scan with plantar views. Radiographic examinations of 23 fractures in 21 patients are evaluated.     

Hematopoietic stem cell transplantation for mucopolysaccharidoses and leukodystrophies

The only effective treatment for selected metabolic diseases is a successful allogeneic hematopoietic stem cell transplantation (HCT). Best results with HCT are obtained when performed early in the course of the disease. Hence, timely identification and referral are critical. Also, early identification of affected patients during the newborn period via screening may be invaluable, particularly for the infantile onset rapidly progressive forms of diseases. Rapid availability of a donor graft is often crucial for these patients. Preliminary experience suggests that results after umbilical cord blood (UCB) transplant may be comparable to those after marrow transplants. UCB grafts have certain benefits of rapid availability and even reduced risk of GvHD. Hence, UCB transplant represents an alternative to marrow HCT. Related haploidentical HCT, possibly with very high doses of CD34+ cells, may also represent an option. However, expertise has been developed in very few transplant centers and no large reports are available of its use for patients with inherited metabolic diseases.     

Three-legged pair of pants anastomosis: a rare technique for renal transplantation

Because of the shortage of donors, kidneys previously judged to be unsuitable are now accepted for transplantation. Successful engraftment of kidneys with multiple renal arteries is one of the challenges and requires meticulous vascular surgical technique. In one of our case of renal allograft with three renal arteries, we created a single lumen by suturing three arteries together, like a three-legged pair of pants. The end of the single lumen was anastomesed to the side of external iliac artery. There was brisk diuresis on table after transplantation. Postoperatively, the patient had an uneventful course and normal graft function.     

Nutritional status of obese hypercholesterolemic adults

The study was conducted to collect information regarding food and nutrient intake of hypercholesterolemic adults (male n = 30, female n = 20). The Body Mass Index (BMI) of the respondents indicated that 70% of them had a BMI more than 26. The food intake of the respondents was recorded for three consecutive days using 24 hour dietary recall and questionnaire method. The intake of cereals, sugar and oil was significantly higher than the Recommended Dietary Intake (RDI) prescribed for Indians, whereas intake of pulses was at par with RDI. The consumption of green leafy and other vegetable and roots and tubers was significantly lower among both male and female respondents. The intake of protein was at par with recommended dietary allowance (RDA), but fat and energy intake were significantly higher in both male and female respondents. The subjects were given diet counselling and this received a good response from the respondents.