Volume 88 Number 6
4.
5.
6.
7.
Letters to the Editor
logic responses to Hemophilus influenzae meningitis, J PEDIATR 80:209, 1972. Smith DH, Peter G, Ingram DL, Harding AL, and Anderson P: Responses of children immunized with the capsular polysaccharide of Hemophilus influenzae, Pediatrics 52:637, 1973. O'Reilly RJ, Anderson P, Ingram DL,. Peter G, and Smith DH: Circulating polyribophosphate in Hemophilus influenzae, type b meningitis: Correlation with clinical course and antibody response, J Clin Invest 56:1012, 1975. Norden CW, and Michaels R: Immunologic response in patients with epiglottitis caused by Haemophilus influenzae type b, J Infect Dis 128:777, 1973. Schneerson R, Rodfigues LP, Parke JC, and Robbins JB: Immunity to disease caused by Hemophilus influenzae type b. II. Specificity and some biologic characteristics of "natural," infection-acquired, and immunization-induced antibodies to the capsular polysaccharide of Hemophilus influenzae type b, J Immunol 107:1081, 1971.
Re#y To the Editor: We would agree with the following observations made by Dr. Granoff with regard to patients who contract H. influenzae type b epiglottitis. (1) They may have had prior exposure to H. influenzae type b. (2) They usually have very high and rapid anticapsular antibody responses. (3) They are older than other patients who contract H. influenzae type b infections. Dr. Granoff concludes that these facts support either the hypothesis that there is a genetic difference between epiglottitis patients and other patients who contract H. influenzae type b infections, or the hypothesis that prior exposure to the organism contributes to the pathogenesis of epiglottitis. Moreover, both hypotheses could be true. However, both hypotheses only explain why certain patients may be more susceptihle to severe 11. influenzae type b infections. Neither hypothesis explains why H. influenzae type b should preferentially attack the epiglottis. Carmi Z Margolis, M.D. Senior Lecturer in Pediatrics and Medical Education University Center for Health Sciences P.O. Box 653 Beersheva 84 120 Israel
Thyroid studies in anorexia nervosa To the Editor: Drs. Kim and Michael I describe a patient with decreased serum complement in association with anorexia nervosa. Among other symptoms and signs are included leg cramps, easy fatigability, carotenemia, bradycardia, hypothermia, and low blood pressure. The patient's serum thyroxine was low. I would be
10 6 9
interested to know if an estimation of thyroxine binding globulin was done, and if the T~ determination was repeated after the patient recovered. Philip G. Danufsky, M.D. Linn Central Clinic 35-37 Rotschild A re. Haifa, Israel REFERENCE
1. Kim Y, and Michael AF: Hypocomplementemia in anorexia nervosa, J PEDIATR 87:582, 1975.
Re#y To the Editor: Thyroid function studies repeated four months after the initiation of high caloric diet showed a T~ of 2.6/zg/dl (normal range 3.0-7.0/~g/dl) and flee T4 of 1.1 ng/dl (normal range 1.02.3 ng/dl). These studies were not repeated after the patient fully recovered. In a study of 29 patients with anorexia nervosa by Silverman, 1 hypercarotenemia, bradycardia, hypothermia, and low blood pressure were found in the majority of the patients. In addition, low serum concentration of T4 was found in 27% of the patients. Dr. Youngki Kim Dr. Alfred F. Michael Department of Pediatrics School of Medicine University of Minnesota Minneapolis, Minn. 55455
REFERENCE
1. Silverman JA: Anorexia nervosa. Clinical observations in a successful treatment plan, J PEDIATR 84:68, 1974.
Mucocutaneous lymph node syndrome in California To the Editor: We read with great interest the description of the three cases of mucocutaneous lymph node syndrome (MCLS) in Tennessee, 1 since we recently recognized two cases ( a 21-month-old Caucasian male and a 3Vz-year-old Mexican American male) at the Childrens Hospital of Los Angeles (CHLA). These two patients had the typical clinical presentation consisting of fever, adenitis, congested bulbar conjunctiva, oropharyngeal involvement, diarrhea, urethritis, polymorphous erythematous rash on the trunk, and induration of the hands and feet which progressed to desquamation around the fingers and toes. In addition, th~ older child had a prominent polyarticular arthritis, not found in the Tennessee cases? The arthritis involved the small joints of the
1070
Letters to the Editor
hand with fusiform swelling, and bilateral swelling of the knees and elbows. This form of arthritis has been documented in patients from Japan 2 and Hawaii? Laboratory investigation was similar to that of previous cases; leukocytosis with a left shift in the differential white count, elevated sedimentation rate, sterile pyuria, mononuclear cerebrospinal fluid pleocytosis in the second patient, and negative serologic, bacteriologic, and immunologic evaluation. We also noted thrombocytosis in the second patient, a feature noted in the patients from Japan 2 and Hawaii2 Cardiac examination and electrocardiographic findings have remained normal to date in both patients. Recent articles3-5 suggest that infantile polyarteritis nodosa (IPN) may in fact be the fatal form of MCLS. A comparative review of the pathologic findings in 19 infants with IPN from the continental United States, and of fatal cases of MCLS from Hawaii and Japan was recently done at our institution.6 The pathologic findings were indistinguishable in the two diseases. In addition, histories of the IPN patients included clinical features of MCLS, with recovery from the acute disease followed by sudden death several weeks later. These data suggest that IPN may represent the severe and fatal form of MCLS in the United States. The case fatality rate for MCLS in Japan is approximately 2%. Of the 19 examples of IPN reviewed, nine were from CHLA, over the years 1942 to 1974. If these deaths represent the 2% mortality of MCLS, at least 30 patients per year with MCLS over the past 32 years in Los Angeles have gone unrecognized. The true incidence is probably even greater, since the high frequency of sudden death in MCLS may direct a good number of such cases to coroner's services. Increased awareness of the presence of MCLS in the United States, with identification of an entity which has previously gone unrecognized, hopefully will permit screening of patients at risk for the serious and fatal complications of MCLS.
Ricki Robinson, M.D. Chief Pediatric Resident Childrens Hospital of Los Angeles Robert Adler, M.D. Chief Pediatric Resident Childrens Hospital of Los Angeles 4650 Sunset Blvd. Los Angeles, Calif. 90027 REFERENCES
1. Brown JS, Billmeier GJ, Cox F, Ibrahim M, Stepp WP, and Gibson R: Mucocutaneous lymph node syndrome in the continental United States, J PEDIATR88:81, 1976. 2. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, and Yanagawa H: A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan, Pediatrics 54:271, 1974. 3. Melish ME, Hicks RM, and Larson E: Mucocutaneous lymph node syndrome (MCLS) in the United States, Pediatr Res 8:427, 1974 (abstr). 4. Fetterman GH, and Hashida Y: Mucocutaneous lyriaph node syndrome (MLNS): A disease widespread in Japan which demands our attention, Pediatrics 54:268, 1974. 5. Jones ME, Nihill MR, Barrett FF, Singer DB, and Rosefiberg HS: Infantile periarteritis nodosaf The expanded
The Journal of Pediatrics June 1976
syndrome of Kawasaki-Fetterman (abstract), Second Joint Meeting Pediatric Pathology Club and Paediatric Pathology Society, Toronto, 25-27 September, 1975. Landing BH, and Larson EJ: Apparent identity of infantile periarteritis nodosa with coronary artery involvement (IPN) and fatal mucocutaneous lymph node syndrome (MCLS): Comparison of 20 patients from North America to patients from Hawaii and Japan (abstract), Second Joint Meeting Pediatric Pathology Club and Paediatric Pathology Society, Toronto, 25-27 September, 1975.
Clinical staging of Reye syndrome To the Editor: This letter is a plea for the development and acceptance of uniform criteria for classifying the different stages of severity of central nervous system involvement in Reye syndrome. Recent publications1-~ illustrate a lack of uniform criteria, making it difficult to evaluate the results of different methods of treatment. It is doubtful that properly controlled studies of different therapeutic modalities can be instituted in view of the reported successes observed in a variety of regimens. Some progress can be made, however, if uniform criteria are used in classifying the different levels of consciousness and of brainstem function. The lack of uniform criteria is illustrated in the report of Bobo and associates 4 and Schubert and associates?, 5 They observed 100% survival among 16 patients with Reye syndrome in 1973-74 classified in Stages III and IV according to their grading system. Some o f the latter patients in their Stage IV appear to belong in Stage III of Huttenlocher8 and of DeVivo and associates?. 2 DeVivo's group observed an overall survival rate of 100% among 12 patients in their Stage III. On the other hand, survival rates declined markedly among patients who progressed to Stage IV coma in DeVivo and associates' series. None of the patients in Schubert and associates' series 3, 5 reached Stage V coma (essentially comparable to Stage IV coma of DeVivo and associates.a. 2 The staging of coma described by McNealy and Plum 7 and modified by DeVivo and associates 1, ~and Huttentocher6 appears to be pathophysiologically the most accurate method of staging depth of coma. This approach describes brainstem dysfunction occurring in a rostral-caudal fashion and delineates the level of function of the comatose patient. Decerebrate posturing in a comatose patient with dilated and sluggishly reactive or fixed pupils who exhibits central neurogenic hyperventilation should appropriately be classified in Stage IlI coma. Stage IV coma should include deeply comatose patients unresponsive to stimuli, who are areflexic with absent brainstem reflexes (oculocephalic, ciliospinal, and oculovestibular reflexes) and who exhibit no spontaneous respirations. Accordingly, many patients classified as Stage V by Bobo and associates4 and Schubert and associates3. ~ actually belong in Stage III of Huttenlocher~ and DeVivo and associates.'. 2 This difference in staging may be extremely important in evaluating results of therapeutic
4.
5.
6.
7.
Letters to the Editor
logic responses to Hemophilus influenzae meningitis, J PEDIATR 80:209, 1972. Smith DH, Peter G, Ingram DL, Harding AL, and Anderson P: Responses of children immunized with the capsular polysaccharide of Hemophilus influenzae, Pediatrics 52:637, 1973. O'Reilly RJ, Anderson P, Ingram DL,. Peter G, and Smith DH: Circulating polyribophosphate in Hemophilus influenzae, type b meningitis: Correlation with clinical course and antibody response, J Clin Invest 56:1012, 1975. Norden CW, and Michaels R: Immunologic response in patients with epiglottitis caused by Haemophilus influenzae type b, J Infect Dis 128:777, 1973. Schneerson R, Rodfigues LP, Parke JC, and Robbins JB: Immunity to disease caused by Hemophilus influenzae type b. II. Specificity and some biologic characteristics of "natural," infection-acquired, and immunization-induced antibodies to the capsular polysaccharide of Hemophilus influenzae type b, J Immunol 107:1081, 1971.
Re#y To the Editor: We would agree with the following observations made by Dr. Granoff with regard to patients who contract H. influenzae type b epiglottitis. (1) They may have had prior exposure to H. influenzae type b. (2) They usually have very high and rapid anticapsular antibody responses. (3) They are older than other patients who contract H. influenzae type b infections. Dr. Granoff concludes that these facts support either the hypothesis that there is a genetic difference between epiglottitis patients and other patients who contract H. influenzae type b infections, or the hypothesis that prior exposure to the organism contributes to the pathogenesis of epiglottitis. Moreover, both hypotheses could be true. However, both hypotheses only explain why certain patients may be more susceptihle to severe 11. influenzae type b infections. Neither hypothesis explains why H. influenzae type b should preferentially attack the epiglottis. Carmi Z Margolis, M.D. Senior Lecturer in Pediatrics and Medical Education University Center for Health Sciences P.O. Box 653 Beersheva 84 120 Israel
Thyroid studies in anorexia nervosa To the Editor: Drs. Kim and Michael I describe a patient with decreased serum complement in association with anorexia nervosa. Among other symptoms and signs are included leg cramps, easy fatigability, carotenemia, bradycardia, hypothermia, and low blood pressure. The patient's serum thyroxine was low. I would be
10 6 9
interested to know if an estimation of thyroxine binding globulin was done, and if the T~ determination was repeated after the patient recovered. Philip G. Danufsky, M.D. Linn Central Clinic 35-37 Rotschild A re. Haifa, Israel REFERENCE
1. Kim Y, and Michael AF: Hypocomplementemia in anorexia nervosa, J PEDIATR 87:582, 1975.
Re#y To the Editor: Thyroid function studies repeated four months after the initiation of high caloric diet showed a T~ of 2.6/zg/dl (normal range 3.0-7.0/~g/dl) and flee T4 of 1.1 ng/dl (normal range 1.02.3 ng/dl). These studies were not repeated after the patient fully recovered. In a study of 29 patients with anorexia nervosa by Silverman, 1 hypercarotenemia, bradycardia, hypothermia, and low blood pressure were found in the majority of the patients. In addition, low serum concentration of T4 was found in 27% of the patients. Dr. Youngki Kim Dr. Alfred F. Michael Department of Pediatrics School of Medicine University of Minnesota Minneapolis, Minn. 55455
REFERENCE
1. Silverman JA: Anorexia nervosa. Clinical observations in a successful treatment plan, J PEDIATR 84:68, 1974.
Mucocutaneous lymph node syndrome in California To the Editor: We read with great interest the description of the three cases of mucocutaneous lymph node syndrome (MCLS) in Tennessee, 1 since we recently recognized two cases ( a 21-month-old Caucasian male and a 3Vz-year-old Mexican American male) at the Childrens Hospital of Los Angeles (CHLA). These two patients had the typical clinical presentation consisting of fever, adenitis, congested bulbar conjunctiva, oropharyngeal involvement, diarrhea, urethritis, polymorphous erythematous rash on the trunk, and induration of the hands and feet which progressed to desquamation around the fingers and toes. In addition, th~ older child had a prominent polyarticular arthritis, not found in the Tennessee cases? The arthritis involved the small joints of the
1070
Letters to the Editor
hand with fusiform swelling, and bilateral swelling of the knees and elbows. This form of arthritis has been documented in patients from Japan 2 and Hawaii? Laboratory investigation was similar to that of previous cases; leukocytosis with a left shift in the differential white count, elevated sedimentation rate, sterile pyuria, mononuclear cerebrospinal fluid pleocytosis in the second patient, and negative serologic, bacteriologic, and immunologic evaluation. We also noted thrombocytosis in the second patient, a feature noted in the patients from Japan 2 and Hawaii2 Cardiac examination and electrocardiographic findings have remained normal to date in both patients. Recent articles3-5 suggest that infantile polyarteritis nodosa (IPN) may in fact be the fatal form of MCLS. A comparative review of the pathologic findings in 19 infants with IPN from the continental United States, and of fatal cases of MCLS from Hawaii and Japan was recently done at our institution.6 The pathologic findings were indistinguishable in the two diseases. In addition, histories of the IPN patients included clinical features of MCLS, with recovery from the acute disease followed by sudden death several weeks later. These data suggest that IPN may represent the severe and fatal form of MCLS in the United States. The case fatality rate for MCLS in Japan is approximately 2%. Of the 19 examples of IPN reviewed, nine were from CHLA, over the years 1942 to 1974. If these deaths represent the 2% mortality of MCLS, at least 30 patients per year with MCLS over the past 32 years in Los Angeles have gone unrecognized. The true incidence is probably even greater, since the high frequency of sudden death in MCLS may direct a good number of such cases to coroner's services. Increased awareness of the presence of MCLS in the United States, with identification of an entity which has previously gone unrecognized, hopefully will permit screening of patients at risk for the serious and fatal complications of MCLS.
Ricki Robinson, M.D. Chief Pediatric Resident Childrens Hospital of Los Angeles Robert Adler, M.D. Chief Pediatric Resident Childrens Hospital of Los Angeles 4650 Sunset Blvd. Los Angeles, Calif. 90027 REFERENCES
1. Brown JS, Billmeier GJ, Cox F, Ibrahim M, Stepp WP, and Gibson R: Mucocutaneous lymph node syndrome in the continental United States, J PEDIATR88:81, 1976. 2. Kawasaki T, Kosaki F, Okawa S, Shigematsu I, and Yanagawa H: A new infantile acute febrile mucocutaneous lymph node syndrome (MLNS) prevailing in Japan, Pediatrics 54:271, 1974. 3. Melish ME, Hicks RM, and Larson E: Mucocutaneous lymph node syndrome (MCLS) in the United States, Pediatr Res 8:427, 1974 (abstr). 4. Fetterman GH, and Hashida Y: Mucocutaneous lyriaph node syndrome (MLNS): A disease widespread in Japan which demands our attention, Pediatrics 54:268, 1974. 5. Jones ME, Nihill MR, Barrett FF, Singer DB, and Rosefiberg HS: Infantile periarteritis nodosaf The expanded
The Journal of Pediatrics June 1976
syndrome of Kawasaki-Fetterman (abstract), Second Joint Meeting Pediatric Pathology Club and Paediatric Pathology Society, Toronto, 25-27 September, 1975. Landing BH, and Larson EJ: Apparent identity of infantile periarteritis nodosa with coronary artery involvement (IPN) and fatal mucocutaneous lymph node syndrome (MCLS): Comparison of 20 patients from North America to patients from Hawaii and Japan (abstract), Second Joint Meeting Pediatric Pathology Club and Paediatric Pathology Society, Toronto, 25-27 September, 1975.
Clinical staging of Reye syndrome To the Editor: This letter is a plea for the development and acceptance of uniform criteria for classifying the different stages of severity of central nervous system involvement in Reye syndrome. Recent publications1-~ illustrate a lack of uniform criteria, making it difficult to evaluate the results of different methods of treatment. It is doubtful that properly controlled studies of different therapeutic modalities can be instituted in view of the reported successes observed in a variety of regimens. Some progress can be made, however, if uniform criteria are used in classifying the different levels of consciousness and of brainstem function. The lack of uniform criteria is illustrated in the report of Bobo and associates 4 and Schubert and associates?, 5 They observed 100% survival among 16 patients with Reye syndrome in 1973-74 classified in Stages III and IV according to their grading system. Some o f the latter patients in their Stage IV appear to belong in Stage III of Huttenlocher8 and of DeVivo and associates?. 2 DeVivo's group observed an overall survival rate of 100% among 12 patients in their Stage III. On the other hand, survival rates declined markedly among patients who progressed to Stage IV coma in DeVivo and associates' series. None of the patients in Schubert and associates' series 3, 5 reached Stage V coma (essentially comparable to Stage IV coma of DeVivo and associates.a. 2 The staging of coma described by McNealy and Plum 7 and modified by DeVivo and associates 1, ~and Huttentocher6 appears to be pathophysiologically the most accurate method of staging depth of coma. This approach describes brainstem dysfunction occurring in a rostral-caudal fashion and delineates the level of function of the comatose patient. Decerebrate posturing in a comatose patient with dilated and sluggishly reactive or fixed pupils who exhibits central neurogenic hyperventilation should appropriately be classified in Stage IlI coma. Stage IV coma should include deeply comatose patients unresponsive to stimuli, who are areflexic with absent brainstem reflexes (oculocephalic, ciliospinal, and oculovestibular reflexes) and who exhibit no spontaneous respirations. Accordingly, many patients classified as Stage V by Bobo and associates4 and Schubert and associates3. ~ actually belong in Stage III of Huttenlocher~ and DeVivo and associates.'. 2 This difference in staging may be extremely important in evaluating results of therapeutic
