Volume 86 Number 4
Letters to the Editor
7.
Steele T, Gyorgy A, and Edwards K: Renal function in analgesic nephropathy, Br Med J 2:213, 1969. 8. Kincaid-Smith P: Pathogenesis of the renal lesion associated with the abuse of analgesics, Lancet 1:859, i967. 9. Goodman L, and Gilman A: The pharmacologic basis of therapeutics, ed 4, New York, 1970, The MacMillan Company, p 329-334.
639
centration of blood ammonia decreased to 42l /~g/dl. After a 24-hour fast, feeding was begun with a diet in which 63% of calories were derived from carbohydrates, 33% from lipids, and only 1% from amino acids. The amino acids were essential amino acids (L forms) and cysteine, arginine, and glutamic acid (Table I). DISCUSSION
Survival of infant with argininosuccinic aciduria to 3 months of age To the Editor: Among the inherited disorders of the urea cycle, argininosuccinic aciduria is the most frequent. The variety presenting in the neonatal period is less common than the one which becomes symptomatic at a later date. So far as could be ascertained, patients diagnosed in the neonatal period have died in the first few weeks of life. We have recently initiated treatment of an affected infant at 5 days of age; she survived until the age of 3 months. CASE REPORT
Although the infant was asymptomatic, she failed to thrive and at 3 months of age she weighed 2,650 gm and was 52 cm in length. The head circumference was 34 cm. The infant developed a normochromic anemia and hypoproteinemia. Blood concentrations of ammonia were within normal limits; the concentrations of argininosuccinic acid in serum decreased to 7 mg/dl while the dietary intake of amino acids was 0.66 gm/kg/ day, and increased to 30 mg/dl when the intake of amino acids was advanced to 1.26 gm/kg/day. An episode of hematemesis occurred at 2 3/4 months, and the infant died at the age of 3 months. The administration of a low-protein diet with the addition of arginine, cysteine, and glutarnic acid may have contributed to the survival of the infant to 3 months of age. The clinical course was complicated by failure to thrive. Although concentrations of blood ammonia became normal, concentrations of serum argininosuccinic acid varied with dietary intake of amino acids.
J. P. Farriaux C. Piemert G. Fontaine Cl&ique Pediatrique Cite Hospitaliere Lille, France
S., a female infant, was the firstborn of young, healthy, unrelated parents. At the age of 4 days she became stuporous and refused feedings. Initial laboratory data included: blood ammonial 960 p,g/dl argininosuccinic acid in serum, 46 mg/dl; in urine, CSF, and stool, 30.9 mg/mg, creatinine, 41 mg/dl and 51 rag/100 gin, respectively. The diagnosis of argininosuccinic aciduria was c o n f i r m e d by d e m o n s t r a t i o n of deficient argininosuccincase in liver, kidney, and brain. The initial therapeutic measure was an exchange transfusion, following which the clinical state was improved; the con-
T a b l e I. A m o u n t of a m i n o acids in t h e diet o f p r o b a n d
Age Amino Acids (mg)
12days
] l month ]2 88
Histidine Isoleucine Leucine Lysine Metbionine Phenylalanine Threonine Tryptophanne Valine Glutamate Arginine Cystine Tyrosine Total mg mg/kg/day
68 238 300 206 90 180 174 44 210 250 100 30
122.0 244.0 406.0 309.0 75.0 256.0 181.0 56.0 256.0 312.5 150.0 77.5
1,890 0.66
2,445.0 0.90
152.5 305.0 507.5 386.0 94.0 320.0 200.0 70.0 350.0 391.0 250.0 97.5 50.0 3,172.0 1.26
Posttraumatic purulent meningitis due to Bordetella bronchiseptica To the Editor: Although Bordetella bronchiseptica has been isolated from patients with symptoms of whooping cough, 1, 2 its pathogenecity in h u m a n beings is not well defined. We report a case of posttraumatic purulent meningitis due to B. bronchiseptica. CASE REPORT
A 9-year-old white boy was admitted to a local hospital after being kicked on his face by a horse. An open reduction for the fractured orbital bones was performed on the sixth day of hospitalization. He developed fever and neck stiffness on the tenth day. Examination of the cerebral spinal fluid (CSF) revealed a white blood cell (WBC) c o u n t of 2,034/mm 3 with 94% polymorphonuclear leukocytes and no red blood cells. The CSF
From the Department of Pediatrics and Clinical Pathology, Universityof Kansas Medical Center, College of Health Sciences and Hospital.
640
Letters to the Editor
glucose was 55 mg/dl; protein was 40 mg/dl. He was given ampiciUin and oxaciUin intravenously for the suspected meningitis. Two days later he was transferred to the University of Kansas Medical Center. The child was acutely ill and febri!e. He had a severe headache and neck stiffness. The right side Of the face and the periorbital tissue of the right eye were red, warm, tender, and swollen. A lumbar puncture revealed a pressure of 120 m m H20. T h e CSF W B C c o u n t was 5 , 5 6 0 / m m 3 w i t h 87% polymorphonuclears. The CSF protein was 75 mg/dl; glucose was 20 mg/dl with a concurrent blood glucose of 112 mgldl. He was given chloramphenicol, 100 mg/kg/day, and methicillin, 200 mg/kg/day, intravenously. A significant clinical improvement was noted after 48 hours of therapy. Cisternography failed to detect leakage of cerebrospinal fluid. On the ninth hospital day chloramphenicol and methicillin were discontinued and ampicillin, 400 mg/kg/day, intravenously, was given for an additional two weeks. The CSF findings returned to normal at the end of 3 weeks of hospitalization. Follow-up examination 3 months later showed no neurologic deficit. Two spinal fluid cultures, one obtained at the referring hospital and the other at our own, grew B. bronchiseptica. The nasal cavity cultures of household animals including a cat, a dog, two gerbils, and a horse were performed later; all were negative for B. bronchiseptica. The source of this organism remains obscure. DISCUSSION
B. bronchiseptica is a common inhabitant of the respiratory tract and middle ear of a variety of wild, domestic, and laboratory animals. B. bronchiseptica generally is not pathogenic for animals, however, atrophic rhinitis, pneumonia, and even death have been reported. 35
The Journal of Pediatrics April 1975
The clinical and spinal fluid findings of our patient were characteristic of a purulent meningitis, and there is little doubt that B. bronchiseptica was responsible for the disease. B. bronchiseptica generally is not conside~'ed pathogenic for man, but as illustrated by our patient it may produce significant infection in the anatomically compromised host.
K. C. Chang, M.D. R. M. Zakheim, M.D. C. T. Cho, M.D., Ph.D. J. C. Montgomery, B.S. Department of Pediatrics University of Kansas Medical Center 39th & Rainbow Blvd. Kansas City, Kan. 66103 REFERENCES 1. Brown JH: Bacillus bronchisepticus infection in a child with symptoms of pertussis, Bull Johns Hopkins Hosp 38:147, 1926. 2. Eldering G, Holwerda L, Davis A, and Baker J: Bordetella pertussis serotypes in the United States, Appl Microbiol 18:618, 1969. 3. Winsser J: A study of Bordetella bronchiseptica, Proc Anim Care Panel 10:87, 1960. 4. Switzer WP, Mare CJ, and Hubbard ED: Incidence of Bordetella bronchiseptica in wild life and man in Iowa, Am J Vet Res 27:1134, 1966. 5. Graves IL: Bordetella bronchiseptica isolated from a fatal case of bronchopneumonia in an African green monkey, Lab Anita Sci 18:405, 1968.
Letters to the Editor
7.
Steele T, Gyorgy A, and Edwards K: Renal function in analgesic nephropathy, Br Med J 2:213, 1969. 8. Kincaid-Smith P: Pathogenesis of the renal lesion associated with the abuse of analgesics, Lancet 1:859, i967. 9. Goodman L, and Gilman A: The pharmacologic basis of therapeutics, ed 4, New York, 1970, The MacMillan Company, p 329-334.
639
centration of blood ammonia decreased to 42l /~g/dl. After a 24-hour fast, feeding was begun with a diet in which 63% of calories were derived from carbohydrates, 33% from lipids, and only 1% from amino acids. The amino acids were essential amino acids (L forms) and cysteine, arginine, and glutamic acid (Table I). DISCUSSION
Survival of infant with argininosuccinic aciduria to 3 months of age To the Editor: Among the inherited disorders of the urea cycle, argininosuccinic aciduria is the most frequent. The variety presenting in the neonatal period is less common than the one which becomes symptomatic at a later date. So far as could be ascertained, patients diagnosed in the neonatal period have died in the first few weeks of life. We have recently initiated treatment of an affected infant at 5 days of age; she survived until the age of 3 months. CASE REPORT
Although the infant was asymptomatic, she failed to thrive and at 3 months of age she weighed 2,650 gm and was 52 cm in length. The head circumference was 34 cm. The infant developed a normochromic anemia and hypoproteinemia. Blood concentrations of ammonia were within normal limits; the concentrations of argininosuccinic acid in serum decreased to 7 mg/dl while the dietary intake of amino acids was 0.66 gm/kg/ day, and increased to 30 mg/dl when the intake of amino acids was advanced to 1.26 gm/kg/day. An episode of hematemesis occurred at 2 3/4 months, and the infant died at the age of 3 months. The administration of a low-protein diet with the addition of arginine, cysteine, and glutarnic acid may have contributed to the survival of the infant to 3 months of age. The clinical course was complicated by failure to thrive. Although concentrations of blood ammonia became normal, concentrations of serum argininosuccinic acid varied with dietary intake of amino acids.
J. P. Farriaux C. Piemert G. Fontaine Cl&ique Pediatrique Cite Hospitaliere Lille, France
S., a female infant, was the firstborn of young, healthy, unrelated parents. At the age of 4 days she became stuporous and refused feedings. Initial laboratory data included: blood ammonial 960 p,g/dl argininosuccinic acid in serum, 46 mg/dl; in urine, CSF, and stool, 30.9 mg/mg, creatinine, 41 mg/dl and 51 rag/100 gin, respectively. The diagnosis of argininosuccinic aciduria was c o n f i r m e d by d e m o n s t r a t i o n of deficient argininosuccincase in liver, kidney, and brain. The initial therapeutic measure was an exchange transfusion, following which the clinical state was improved; the con-
T a b l e I. A m o u n t of a m i n o acids in t h e diet o f p r o b a n d
Age Amino Acids (mg)
12days
] l month ]2 88
Histidine Isoleucine Leucine Lysine Metbionine Phenylalanine Threonine Tryptophanne Valine Glutamate Arginine Cystine Tyrosine Total mg mg/kg/day
68 238 300 206 90 180 174 44 210 250 100 30
122.0 244.0 406.0 309.0 75.0 256.0 181.0 56.0 256.0 312.5 150.0 77.5
1,890 0.66
2,445.0 0.90
152.5 305.0 507.5 386.0 94.0 320.0 200.0 70.0 350.0 391.0 250.0 97.5 50.0 3,172.0 1.26
Posttraumatic purulent meningitis due to Bordetella bronchiseptica To the Editor: Although Bordetella bronchiseptica has been isolated from patients with symptoms of whooping cough, 1, 2 its pathogenecity in h u m a n beings is not well defined. We report a case of posttraumatic purulent meningitis due to B. bronchiseptica. CASE REPORT
A 9-year-old white boy was admitted to a local hospital after being kicked on his face by a horse. An open reduction for the fractured orbital bones was performed on the sixth day of hospitalization. He developed fever and neck stiffness on the tenth day. Examination of the cerebral spinal fluid (CSF) revealed a white blood cell (WBC) c o u n t of 2,034/mm 3 with 94% polymorphonuclear leukocytes and no red blood cells. The CSF
From the Department of Pediatrics and Clinical Pathology, Universityof Kansas Medical Center, College of Health Sciences and Hospital.
640
Letters to the Editor
glucose was 55 mg/dl; protein was 40 mg/dl. He was given ampiciUin and oxaciUin intravenously for the suspected meningitis. Two days later he was transferred to the University of Kansas Medical Center. The child was acutely ill and febri!e. He had a severe headache and neck stiffness. The right side Of the face and the periorbital tissue of the right eye were red, warm, tender, and swollen. A lumbar puncture revealed a pressure of 120 m m H20. T h e CSF W B C c o u n t was 5 , 5 6 0 / m m 3 w i t h 87% polymorphonuclears. The CSF protein was 75 mg/dl; glucose was 20 mg/dl with a concurrent blood glucose of 112 mgldl. He was given chloramphenicol, 100 mg/kg/day, and methicillin, 200 mg/kg/day, intravenously. A significant clinical improvement was noted after 48 hours of therapy. Cisternography failed to detect leakage of cerebrospinal fluid. On the ninth hospital day chloramphenicol and methicillin were discontinued and ampicillin, 400 mg/kg/day, intravenously, was given for an additional two weeks. The CSF findings returned to normal at the end of 3 weeks of hospitalization. Follow-up examination 3 months later showed no neurologic deficit. Two spinal fluid cultures, one obtained at the referring hospital and the other at our own, grew B. bronchiseptica. The nasal cavity cultures of household animals including a cat, a dog, two gerbils, and a horse were performed later; all were negative for B. bronchiseptica. The source of this organism remains obscure. DISCUSSION
B. bronchiseptica is a common inhabitant of the respiratory tract and middle ear of a variety of wild, domestic, and laboratory animals. B. bronchiseptica generally is not pathogenic for animals, however, atrophic rhinitis, pneumonia, and even death have been reported. 35
The Journal of Pediatrics April 1975
The clinical and spinal fluid findings of our patient were characteristic of a purulent meningitis, and there is little doubt that B. bronchiseptica was responsible for the disease. B. bronchiseptica generally is not conside~'ed pathogenic for man, but as illustrated by our patient it may produce significant infection in the anatomically compromised host.
K. C. Chang, M.D. R. M. Zakheim, M.D. C. T. Cho, M.D., Ph.D. J. C. Montgomery, B.S. Department of Pediatrics University of Kansas Medical Center 39th & Rainbow Blvd. Kansas City, Kan. 66103 REFERENCES 1. Brown JH: Bacillus bronchisepticus infection in a child with symptoms of pertussis, Bull Johns Hopkins Hosp 38:147, 1926. 2. Eldering G, Holwerda L, Davis A, and Baker J: Bordetella pertussis serotypes in the United States, Appl Microbiol 18:618, 1969. 3. Winsser J: A study of Bordetella bronchiseptica, Proc Anim Care Panel 10:87, 1960. 4. Switzer WP, Mare CJ, and Hubbard ED: Incidence of Bordetella bronchiseptica in wild life and man in Iowa, Am J Vet Res 27:1134, 1966. 5. Graves IL: Bordetella bronchiseptica isolated from a fatal case of bronchopneumonia in an African green monkey, Lab Anita Sci 18:405, 1968.
