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Hypotonia and folate deficiency in children
EDITORIAL CORRESPONDENCE
Editorial correspondence or letters to the Editor relative to articles published in THE JOURNAL or to topics of current interest are subject to critical review and to current editorial policy in respect to publication in part or in full.
Hypotonia and folate deficiency in children To the Editor: I shall briefly report two cases in relation to the interesting paper by Shapira et al. 1
CASE REPORT An 8-year-old boy and a 12-year-old girl have been under our care since 1975. They underwent neurologic, gastrointestinal, metabolic, and laboratory assessments according to our previously reported method of investigation?- ~ Folate determinations were carried out, using both radioisotope and microbiologic assays. Both patients were referred to our outpatient clinic because of clumsiness in walking, difficulty in running, and inability to participate in any sport. Clinical examination of each subject revealed anorexia, loss of weight, easy fatigability, cramps during the night, deep tendon hyperreflexia, and generalized hypotonia involving mainly the truncal and lower limb muscles. The boy had black marks all over, due to frequent falls; because of his difficulties in running and playing with his classmates, he was transferred to a class for girls. Both patients had had an irritable colon syndrome for 3 and 5 years, respectively, and were ingesting a folate-deficient diet. They had low-serum folate concentrations (less than 2.4 ng/ml) in the absence of anemia. However, the boy had a borderline low RBC folate (160 ng/ml). He became normal after three months of folate therapy (5 mg daily by mouth); he subsequently received one tablet of placebo (same size, color, and shape as folic acid provided by I.C.N., Canada) for five months, and relapsed. Since then, both patients have been in good health with a maintenance dose of 5 mg folic acid orally each week or every two weeks. COMMENT Our experiences with these patients confirm the observation reported by Shapira et al ~ as well as our previous observations in adults?- 3 Such cases of occult folate deficiency could be much more frequent than are supposed, folate deficiency being the most common vitamin deficiency in North America and elsewhere?, ~ M. L Botez, M.D. Clinical Research Institute of Montreal and Hbtel-Dieu Hospital Montreal, P.Q. Canada
774
TheJournalofPEDIATRlCS
REFERENCES 1. Shapira y, Zvi AB, and Statter M: Folic acid deficiency: a reversible cause of infantile hypotonia, J PEDiAXR 93:984, 1978. 2. Botez MI, Cadotte M, Beaulieu R, Pichette LP, and Pison c: Neurologic disorders responsive to folic acid therapy, CMA Journal, 115:217, 1976. 3. Botez MI, Botez T, Lrveill6 J, Bielmann P, and Cadotte M: Neuropsychological correlates of folic acid deficiency, in Botez MI, and Reynolds EH, editors: Folic acid in neurology, psychiatry, and internal medicine, 'New York, 1979, Raven Press, pp 435-461. 4. Nutrition Canada. The Quebec Survey Report. A report from Nutrition Canada by the Bureau of Nutritional Sciences, Ottawa, 1975, Department of National Health and Welfare, pp 119-120. 5. Rosenberg I, and Dyer J: The prevalence and causes of folic acid deficiency in the United States, in Botez MI, and Reynolds EH, editors: Folic acid in neurology, psychiatry, and internal medicine. New York, 1979, Raven Press, pp 19-22.
Hemolytic uremic syndrome following pneumococcal infection To the Editor: In the October, 1979, issue of TI~E JOURNAL,Moorthy and Makker I reported a case of hemolytic uremic syndrome (HUS) associated with pneumococcal sepsis. Pneumococcal infections followed by the hemolytic uremic syndrome have been previous ly reported by Klein et al~ and Poschmann et al? These reports support one of the proposed pathogenetic mechanisms of HUS, following infections by neuraminidaseproducing organisms such as th~ pneumococcus? Neuraminidase is known to act directly on the membrane surfaces of red blood cells, platelets, and renal endothelium by removing sialic acid and exposing the Thomsen-Friedenreich antigen. When this antigen is exposed, it may react with a naturally occurring antibody present in normal human sera. This antibody, thought to be an immunoglobulin of the IgM class, 5 may then recruit
Editorial correspondence or letters to the Editor relative to articles published in THE JOURNAL or to topics of current interest are subject to critical review and to current editorial policy in respect to publication in part or in full.
Hypotonia and folate deficiency in children To the Editor: I shall briefly report two cases in relation to the interesting paper by Shapira et al. 1
CASE REPORT An 8-year-old boy and a 12-year-old girl have been under our care since 1975. They underwent neurologic, gastrointestinal, metabolic, and laboratory assessments according to our previously reported method of investigation?- ~ Folate determinations were carried out, using both radioisotope and microbiologic assays. Both patients were referred to our outpatient clinic because of clumsiness in walking, difficulty in running, and inability to participate in any sport. Clinical examination of each subject revealed anorexia, loss of weight, easy fatigability, cramps during the night, deep tendon hyperreflexia, and generalized hypotonia involving mainly the truncal and lower limb muscles. The boy had black marks all over, due to frequent falls; because of his difficulties in running and playing with his classmates, he was transferred to a class for girls. Both patients had had an irritable colon syndrome for 3 and 5 years, respectively, and were ingesting a folate-deficient diet. They had low-serum folate concentrations (less than 2.4 ng/ml) in the absence of anemia. However, the boy had a borderline low RBC folate (160 ng/ml). He became normal after three months of folate therapy (5 mg daily by mouth); he subsequently received one tablet of placebo (same size, color, and shape as folic acid provided by I.C.N., Canada) for five months, and relapsed. Since then, both patients have been in good health with a maintenance dose of 5 mg folic acid orally each week or every two weeks. COMMENT Our experiences with these patients confirm the observation reported by Shapira et al ~ as well as our previous observations in adults?- 3 Such cases of occult folate deficiency could be much more frequent than are supposed, folate deficiency being the most common vitamin deficiency in North America and elsewhere?, ~ M. L Botez, M.D. Clinical Research Institute of Montreal and Hbtel-Dieu Hospital Montreal, P.Q. Canada
774
TheJournalofPEDIATRlCS
REFERENCES 1. Shapira y, Zvi AB, and Statter M: Folic acid deficiency: a reversible cause of infantile hypotonia, J PEDiAXR 93:984, 1978. 2. Botez MI, Cadotte M, Beaulieu R, Pichette LP, and Pison c: Neurologic disorders responsive to folic acid therapy, CMA Journal, 115:217, 1976. 3. Botez MI, Botez T, Lrveill6 J, Bielmann P, and Cadotte M: Neuropsychological correlates of folic acid deficiency, in Botez MI, and Reynolds EH, editors: Folic acid in neurology, psychiatry, and internal medicine, 'New York, 1979, Raven Press, pp 435-461. 4. Nutrition Canada. The Quebec Survey Report. A report from Nutrition Canada by the Bureau of Nutritional Sciences, Ottawa, 1975, Department of National Health and Welfare, pp 119-120. 5. Rosenberg I, and Dyer J: The prevalence and causes of folic acid deficiency in the United States, in Botez MI, and Reynolds EH, editors: Folic acid in neurology, psychiatry, and internal medicine. New York, 1979, Raven Press, pp 19-22.
Hemolytic uremic syndrome following pneumococcal infection To the Editor: In the October, 1979, issue of TI~E JOURNAL,Moorthy and Makker I reported a case of hemolytic uremic syndrome (HUS) associated with pneumococcal sepsis. Pneumococcal infections followed by the hemolytic uremic syndrome have been previous ly reported by Klein et al~ and Poschmann et al? These reports support one of the proposed pathogenetic mechanisms of HUS, following infections by neuraminidaseproducing organisms such as th~ pneumococcus? Neuraminidase is known to act directly on the membrane surfaces of red blood cells, platelets, and renal endothelium by removing sialic acid and exposing the Thomsen-Friedenreich antigen. When this antigen is exposed, it may react with a naturally occurring antibody present in normal human sera. This antibody, thought to be an immunoglobulin of the IgM class, 5 may then recruit