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138 Archives ofDisease in Childhood: short reports

intestinal absorption of fructose. Arch Dl)s Child 1984;59: fructose related gastrointestinal symptoms we 735-8. prefer to take a careful dietary history, particu- 2 Wales JKH, Primhak RA, Rattenbury J, Taylor CJ. Isolated

fructose malabsorption. Arch Dis Child 1990;65:227-9. Arch Dis Child: first published as 10.1136/adc.68.1.138 on 1 January 1993. Downloaded from larly of fructose-containing foods and to per- 3 Perman JA. Breath analysis. In: Walker WA, Durie PR, form a two week trial of appropriate dietary Hamilton JR, Walker-Smith JA, Watkins JB, eds. Pediatric gastrointestinal disease. Philadelphia: B C Decker Inc, 1991: measures, rather than performing fructose 1354-62. breath hydrogen tests. 4 Fujisawa T, Riby J, Kretchmer N. Intestinal absorption of fructose in the rat. 1991;101:360-7. 5 Barnes G, McKellar W, Lawrance S. Detection of fructose Supported by a grant from Nutricia Nederland BV. malabsorption by breath hydrogen test in a child with diarrhea. JPediatr 1983;103:575-7. 6 Kneepkens CMF, Douwes AC, Jakobs C. Apple juice, fructose, and chronic nonspecific diarrhoea. Eur J Pediatr 1 Kneepkens CMF, Vonk RJ, Fernandes J. Incomplete 1989;148:571-3.

Transient S deficiency with deep venous during Salmonella typhimurium infection

Mehmet Ceyhan, Guler Kanra, Birgul Benderlioglu, Gulten Seqmeer, Gonul HiqSonmez, 5erafettin Kirazli

Abstract erythematous macular rash on the lower A patient with deep and extremities, hepatomegaly, and pitting oedema low activity during the course of in both pretibial regions and the dorsa of the Salmonella typhimurium infection is pre- feet. sented. Although protein S deficiency has Initial laboratory findings were: haemoglobin been reported in patients with disseminated concentration 100 g/l, packed cell volume 0-32, intravascular , it was not present white cell count 6x l09/l, count in this patient and his protein S activity was 480x 109/1, prothrombin time 12 seconds, normal after the findings of infection and deep partial thromboplastin time 42 seconds, venous thrombosis disappeared. fibrinogen concentration 2-9 g/l, fibrinogen degradation products 10-40 ,tg/ml, and the Ham test was normal. Stool and blood cultures (Arch Dis Child 1993;68:138-9) revealed S typhimurium. Venography of the lower extremities showed deep venous throm- http://adc.bmj.com/ Congenital protein S deficiency is inherited as bosis and computed tomography showed occlu- an autosomal dominant trait and it may cause sion of inferior vena cava and both iliac and recurrent thrombotic disease with or without a femoral veins. Abdominal ultrasonography precipitating condition.' Acquired decreases in showed hepatosplenomegaly and occlusion of protein S have been reported in pregnancy and the inferior vena cava. during oral anticontraceptive hormone treatment The patient was given anticoagulants with 50 and in patients with disseminated intravascular U/kg/hour until changed to 225 mg/day on October 1, 2021 by guest. Protected copyright. coagulation (DIC) and .2 Although dipyridamole, and 1 g/day salicylate on day 7. patients with sepsis frequently suffer throm- For salmonella infection ciprofloxacin was embolic complications,3 protein S deficiency or started and by day 3 his temperature was inactivity in these patients is often not recognised normal and on day 10 oedema of the lower if they do not have DIC. extremities disappeared. Here, we describe a patient who presented On day 20, antibacterial treatment was dis- with deep venous thrombosis together with low continued and the patient was discharged on protein S activity during the course ofSalmonella antiplatelet drugs. Sixty days later abdominal typhimurium infection. He did not have any ultrasonography and computed tomography clinical and laboratory findings of DIC and his showed no thrombosis and the antiplatelet Hacettepe University protein S activity returned to normal after the treatment was stopped. , findings ofinfection and deep venous thrombosis Ankara, Turkey, disappeared. Department of Methods Infectious Diseases Routine coagulation tests (prothrombin time, Mehmet Ceyhan concen- Guler Kanra Case report partial thromboplastin time, fibrinogen Birgul Benderlioglu A 13 year old boy was admitted with complaints tration) were performed in fresh plasma and Gulten Seqmeer of fever, swelling of the ankles and knees, a fibrinogen degradation products were measured Department of rash on the trunk and extremities, pain in the in serum using standard methods. Venous Paediatric Haematology lumbar region, and progressive oedema. There blood was drawn by direct venepuncture into Gonul Hiqsonmez or tubes containing 1 part 0-13 $erafettin Kirazli was no history of thromboembolic Vacutainer glass abnormalities in the patient nor in either mol trisodium citrate for 9 parts of blood, and Correspondence to: Dr Ceyhan. maternal or paternal family members. Physical centrifuged at 3000 rpm for 10 minutes. Plasma Accepted 2 September 1992 examination revealed fever (39 7°C), an was then separated and stored in 0 5 ml aliquots Archives ofDisease in Childhood: short reports 139

Protein S activity of the patient and his family members event. However, it is not possible to diagnose or Family member Age Protein S exclude hereditary protein S deficiency only on

(years) activity (%) the basis of the clinical features as they are not Arch Dis Child: first published as 10.1136/adc.68.1.138 on 1 January 1993. Downloaded from Father 48 109 pathognomonic. Patients with heterozygous Mother 45 96 deficiency of protein S usually show their first Sister 1 21 84 Sister 2 18 84 thrombotic event in early adulthood' and this Brother 15 100 might have been the first thrombotic attack of Patient: 13 during infection 37 hereditary protein S deficiency for our patient. After infection 91 Thus, the clinical findings had to be confirmed by laboratory studies. Laboratory findings con- *Normal value: 65-140%. sistent with the diagnosis of hereditary protein S deficiency demonstrate reduced plasma protein S and/or activity in the patient and in at -40°C until use. All tests were made within his/her family members.4 Because protein S five days of blood collection. activity was normal in the patient after recovery Antithrombin III antigen concentration was from salmonella infection and in the family evaluated according to a immunochemical members, he must have had an acquired rather turbidimetric method in fresh plasma using than a hereditary protein S deficiency. antiserum supplied by Behringwerke. Acquired decrease in protein S activity has activity was determined in stored plasma by the been reported in some diseases. Our patient had snake venom activated partial thromboplastin S typhimurium infection when he developed a time assay and protein S activity by the factor venous thrombosis and low protein S activity. Va activated partial thromboplastin time assay Because septicaemia is a well recognised cause (in both cases using commerically available of disseminated intravascular thrombosis, reagents from Diagnostica Stago). Hesselvik et al studied protein S concentrations Standards and test samples were run in in the patients with severe infection and septic duplicate in both protein C and protein S shock and found no deficiency.3 Madden et al assays. The results of protein C and protein S reported two patients with fulminans, a activities were expressed in percent of normal complication ofDIC.5 Both cases had a decreased plasma activity (activity in 1 ml of pooled protein S concentraton and one of them had plasma= 100% activity). oedema of the leg as in our patient, and, in contrast to those cases who had purpuric lesions, high prothrombin time, partial Results thromboplastin time, and concentrations of During hospitalisation of the patient when S fibrinogen degradation products, our patient typhimurium infection was present, the anti- had no evidence of DIC. thrombin III antigen concentration was 40-2 Although chronic coumarin treatment has mg/100 ml (normal range 0-22-0-41 mg/100 ml) been effective in patients with familial protein C and protein C activity was 71% of normal and protein S deficiencies and recurrent throm- http://adc.bmj.com/ (normal range 70-140%). Protein S activity was bosis,6 our patient did not need chronic anti- very low at 37% of normal (normal range coagulant treatment. 65-140%). Sixty days after discharge, protein C activity remained within normal limits (84%) and protein S activity returned to normal (91%). 1 Comp PC, Esmon CT. Recurrent venous thromboembolism in To exclude familial occurrence of protein S patients with a partial deficiency of protein S. N Engl J Med 1984;311:1525-8. deficiency, protein S activity was studied in the 2 D'Angelo A, Vigano-D'Angelo S, Esmon CT, Comp PC. on October 1, 2021 by guest. Protected copyright. patient's family. It was normal in all family Acquired deficiencies of protein S activity during oral anti- coagulation, in liver disease, and in disseminated intra- members (table). vascular coagulation. J Clin Invest 1988;81:1445-54. 3 Hesselvik JF, Malm J, Dahlback B, Blomback M. Protein C, protein S and C4b-binding protein in severe infection and septic shock. Thromb Haemostas 1991 ;65:126-9. Discussion 4 Engesser L, Broekmans AW, Briet E, Brommer EJP, Bertina The findings in our patient are not consistent RM. Hereditary protein S deficiency: clinical manifesta- tions. Ann Intern Med 1987;106:677-82. with those reported for hereditary protein S 5 Madden RM, Gill JC, Marlar RA. Protein C and protein S deficiency in the literature4; he had no previous levels in two patients with acquired . Br J7Haematol 1990;75:112-7. history of thromboembolism and none of the 6 Schafer Al. The hypercoagulable states. Ann Intern Med family members had evidence of a thrombotic 1985;102:814-28.