LETTERS
TO T H E E D I T O R
Clinical notes
"Clinical Notes" represent clinical and/or laboratory experiences which can be presented in 200 to 400 words, 3 or 4 references, and, if contributory, one illustration or short table. "Clinical Notes" are subject to the same critical peer review and editing as papers published in other sections of the JOURNAL.
Impetigo neonatorum congenita due to group B beta-hemolytic streptococcus infection To the Editor: Group B beta hemolytic streptococcus (group B, streptococcus) has been reported with increasing frequency as an etiologic agent in perinatal infections. Septicemia, meningitis, and pneumonitis of the newborn infant, puerperal sepsis, abortions, and stillbirths have been attributed to this organism. 1 We report here an unusual manifestation of group B streptococcal infection in a term infant.
on cord blood was nonreactive. Unfortunately, the placenta was not available for histopathologic and culture studies and no blood cultures were obtained from the infant. Culture of the mother's lochia was negative for group B streptococci. Bacitracin ointment was applied to the lesions twice a day for three days with little effect. At 64 hours of age, reports of the skin cultures became available, and procaine penicillin G, 300,000 units, Was administered daily for ten days. Rapid healing of all ulcers was noted by the third day of penicillin therapy and the infant was discharged at nine days of age. Complete healing of all ulcers with no scar formation had occurred at two weeks of age.
CASE REPORT A male infant weighing 3,500 gm was born at 40 weeks' gestation to a 25-year-old, gravida 1, para 0 woman. The membranes had ruptured spontaneously 22 hours before delivery; vaginal delivery was spontaneous and uncomplicated. At birth widespread superficial ulcerations were noted on the scalp, face, neck, trunk, and limbs. The ulcers were 0.5 to 1.5 cm in diameter, mostly discrete, but some (especially on the face) confluent. Lesions on the face and scalp were covered with a yellow crust, whereas lesions on the trunk, gluteal region, and limbs had a red, shiny weeping base (Fig. 1). Some lesions had an erythematous halo and were elevated above the general skin surface. There were no vesicles or bullae, and the intervening skin appeared normal. The epidermis could not be denuded by friction. A few cervical, posterior auricular, and axillary lymph nodes were enlarged. Constitutional symptoms were absent. There was no family history of epidermolysis bullosa. A tentative diagnosis of impetigo due to Staphylococcus pyogenes was entertained. Numerous gram-positive cocci and a few inflammatory cells were present in scraping of a crust. Cultures from the lesions at two distant sites produced a heavy growth of penicillin-sensitive group B beta hemolytic streptococci. The white blood cell count was 11,000/cm 3, with a normal differential. Concentrations of serum immunoglobulins were: (mg/dl) IgG, 1,150; IgA, 0; and IgM, 45 (increased; normal 12-16 mg/dl). VDRL
From the Departments of Pediatrics and Obstetrics, Grace Maternity Hospital and Dalhousie University.
982
The Journal of P E D I A T R I C S Vol. 86, No. 6, pp. 982-992
Fig. 1. A, Crusted lesions on face. B, Lesions on neck and trunk.
Volume 86 Number 6
DISCUSSION This is an unusual case both because the impetigo had developed in utero and because of the nature of the causative organism. So far, bullous impetigo due to Staphylococcus pyogenes is the only form described in the newborn infant. 2 Unlike this common type, the morphology of the lesions in the infant described was characterized by ulcers with yellow crusts and resembled the common form of impetigo in older children caused by group A beta hemolytic streptococcal infection. The author is indebted to Drs. A. J. Wort, Joan Crosby, and P. A. Cole for their cooperation in the study and follow-up of this infant. Teertharaj K. Belgaumkar, M.D. Grace Maternity Hospital University A re. Halifax, N. S. Canada B3H 1 W3
REFERENCES 1. Hood M, Vanney A, and Darneron G: Beta hemolytic streptococcus G r o u p B associated with problems of perinatal period, Am J Obstet Gynecol 82:809, 1961. 2. Rook A, Wilkinson DG, and Ebling F, editors: Text book of d e r m a t o l o g y , Oxford, 1972, Blackwell Scientific Publications, pp 482-485.
A metabolic myopathy associated with chronic lactic acidemia, growth failure, and nerve deafness To the Editor: Recently Hackett and associates t described a metabolic myopathy associated with chronic lactic acidernia, growth failure, and nerve deafness in two sisters. Biochemical assessment revealed an increased value of alanine, pyruvate, and lactic acid in the blood. Two possible defects were suggested by the authors: The first is in the conversion of pyruvate to glucose (gluconeogenesis). The second is a possible defect in the oxidative metabolism of pyruvate, which would be in agreement with the ultrastructural abnormality of the ~itochondria. We observed in our clinic a girl who, in our opin~ion, has the same hereditary metabolic defect. The clinical data are summarized in Table I in the manner presented by Hackett and associates. The serum alanine level was in the high normal range (570 p.mol/l). The excretion of alanine in urine, however, was increased (6.117/zrnol/gm creatinine). The serum pyruvate level in the resting basal state was slightly elevated, 0.91-1.09 mg/dl (normal 0.3-0.9 mg/dl), and the serum lactate level was clearly increased, 24.8-56.2 mg/dl (normal ( 2 0 rng/dl). The lactatepyruvate ratio was elevated. There was no metabolic acidosis. The mother, the sister of the mother, and the grandmother
Letters to the Editor
983
T a b l e I. S u m m a r y of clinical d a t a
Clinical findings in common with those of the patients of Hackett and associateJ Asyrnptomatic until the age of 8 Insidious onset of diffuse muscle weakness Generalized growth failure Normal mental and intellectual development Neurosensory hearing loss: in our patient it was detected at 10 years of age and was progressive Mitochondrial myopathy in skeletal muscle Electromyographic abnormalities compatible with a myopathy Normal basal metabolic rate Chronically elevated serum lactate and pyruvate values Liver function studies within normal limits Additional findings --Serum creatinin-phosphokinase was increased; on occasion the values were normal, in the cases of Hackett and associates the serum creatininphosphokinase value was normal - - I n the first year of clinical disease, our patient complained of paresthesias --Unexplained bilateral loss of vision (4/10); opthalmoscopically the optic discs seemed pale and there was irregular pigment stippling in the macula region --Electroretinography was normal
had a neurosensory hearing loss which became evident during adulthood. They had no symptoms of myopathy. A son of the sister of the mother had the same mitochondrial myopathy as our patient. Our patient had a normal fasting lucose tolerance test (after 20 hours of fasting, the blood glucose was 69 mg/dl). There was a decreased activity of pyruvate dehydrogenase (tested with pyruvate-l-14C) in leukocytes, 0.84 nrnol/hr/106 leukocytes (normal 2.56 +_ 0.15, n = 15). Metabolism of pyruvate-2-14C was also defective, 0.60 nmol/hr/106 leukocytes (normal 1.74 +__ 0.10, n = 15).2 Studies on isolated rnitochondria, performed by van Dam, revealed a deficiency of cytochrorne c oxidase in muscle tissue) L. Monnens F. Gabre~ls J. Willems Departments of Pediatrics, Neurology, and Biochemistry University of Nijmegen The Netherlands
REFERENCES 1.
2.
3.
Hackett TN Jr, Bray PF, Ziter FA, Nyhan WL, and Creer KM: A metabolic myopathy associated with chronic lactic acidernia, growth failure and n e r v e deafness, J. PED~ATa83:426, 1973. Blass JP, Avigan J, and Uhlendorf BW" A defect in pyruvate decarboxylase in a child with an intermittent movement disorder, J Clin Invest 49:423, 1970. Van Dam K: Unpublished data.
TO T H E E D I T O R
Clinical notes
"Clinical Notes" represent clinical and/or laboratory experiences which can be presented in 200 to 400 words, 3 or 4 references, and, if contributory, one illustration or short table. "Clinical Notes" are subject to the same critical peer review and editing as papers published in other sections of the JOURNAL.
Impetigo neonatorum congenita due to group B beta-hemolytic streptococcus infection To the Editor: Group B beta hemolytic streptococcus (group B, streptococcus) has been reported with increasing frequency as an etiologic agent in perinatal infections. Septicemia, meningitis, and pneumonitis of the newborn infant, puerperal sepsis, abortions, and stillbirths have been attributed to this organism. 1 We report here an unusual manifestation of group B streptococcal infection in a term infant.
on cord blood was nonreactive. Unfortunately, the placenta was not available for histopathologic and culture studies and no blood cultures were obtained from the infant. Culture of the mother's lochia was negative for group B streptococci. Bacitracin ointment was applied to the lesions twice a day for three days with little effect. At 64 hours of age, reports of the skin cultures became available, and procaine penicillin G, 300,000 units, Was administered daily for ten days. Rapid healing of all ulcers was noted by the third day of penicillin therapy and the infant was discharged at nine days of age. Complete healing of all ulcers with no scar formation had occurred at two weeks of age.
CASE REPORT A male infant weighing 3,500 gm was born at 40 weeks' gestation to a 25-year-old, gravida 1, para 0 woman. The membranes had ruptured spontaneously 22 hours before delivery; vaginal delivery was spontaneous and uncomplicated. At birth widespread superficial ulcerations were noted on the scalp, face, neck, trunk, and limbs. The ulcers were 0.5 to 1.5 cm in diameter, mostly discrete, but some (especially on the face) confluent. Lesions on the face and scalp were covered with a yellow crust, whereas lesions on the trunk, gluteal region, and limbs had a red, shiny weeping base (Fig. 1). Some lesions had an erythematous halo and were elevated above the general skin surface. There were no vesicles or bullae, and the intervening skin appeared normal. The epidermis could not be denuded by friction. A few cervical, posterior auricular, and axillary lymph nodes were enlarged. Constitutional symptoms were absent. There was no family history of epidermolysis bullosa. A tentative diagnosis of impetigo due to Staphylococcus pyogenes was entertained. Numerous gram-positive cocci and a few inflammatory cells were present in scraping of a crust. Cultures from the lesions at two distant sites produced a heavy growth of penicillin-sensitive group B beta hemolytic streptococci. The white blood cell count was 11,000/cm 3, with a normal differential. Concentrations of serum immunoglobulins were: (mg/dl) IgG, 1,150; IgA, 0; and IgM, 45 (increased; normal 12-16 mg/dl). VDRL
From the Departments of Pediatrics and Obstetrics, Grace Maternity Hospital and Dalhousie University.
982
The Journal of P E D I A T R I C S Vol. 86, No. 6, pp. 982-992
Fig. 1. A, Crusted lesions on face. B, Lesions on neck and trunk.
Volume 86 Number 6
DISCUSSION This is an unusual case both because the impetigo had developed in utero and because of the nature of the causative organism. So far, bullous impetigo due to Staphylococcus pyogenes is the only form described in the newborn infant. 2 Unlike this common type, the morphology of the lesions in the infant described was characterized by ulcers with yellow crusts and resembled the common form of impetigo in older children caused by group A beta hemolytic streptococcal infection. The author is indebted to Drs. A. J. Wort, Joan Crosby, and P. A. Cole for their cooperation in the study and follow-up of this infant. Teertharaj K. Belgaumkar, M.D. Grace Maternity Hospital University A re. Halifax, N. S. Canada B3H 1 W3
REFERENCES 1. Hood M, Vanney A, and Darneron G: Beta hemolytic streptococcus G r o u p B associated with problems of perinatal period, Am J Obstet Gynecol 82:809, 1961. 2. Rook A, Wilkinson DG, and Ebling F, editors: Text book of d e r m a t o l o g y , Oxford, 1972, Blackwell Scientific Publications, pp 482-485.
A metabolic myopathy associated with chronic lactic acidemia, growth failure, and nerve deafness To the Editor: Recently Hackett and associates t described a metabolic myopathy associated with chronic lactic acidernia, growth failure, and nerve deafness in two sisters. Biochemical assessment revealed an increased value of alanine, pyruvate, and lactic acid in the blood. Two possible defects were suggested by the authors: The first is in the conversion of pyruvate to glucose (gluconeogenesis). The second is a possible defect in the oxidative metabolism of pyruvate, which would be in agreement with the ultrastructural abnormality of the ~itochondria. We observed in our clinic a girl who, in our opin~ion, has the same hereditary metabolic defect. The clinical data are summarized in Table I in the manner presented by Hackett and associates. The serum alanine level was in the high normal range (570 p.mol/l). The excretion of alanine in urine, however, was increased (6.117/zrnol/gm creatinine). The serum pyruvate level in the resting basal state was slightly elevated, 0.91-1.09 mg/dl (normal 0.3-0.9 mg/dl), and the serum lactate level was clearly increased, 24.8-56.2 mg/dl (normal ( 2 0 rng/dl). The lactatepyruvate ratio was elevated. There was no metabolic acidosis. The mother, the sister of the mother, and the grandmother
Letters to the Editor
983
T a b l e I. S u m m a r y of clinical d a t a
Clinical findings in common with those of the patients of Hackett and associateJ Asyrnptomatic until the age of 8 Insidious onset of diffuse muscle weakness Generalized growth failure Normal mental and intellectual development Neurosensory hearing loss: in our patient it was detected at 10 years of age and was progressive Mitochondrial myopathy in skeletal muscle Electromyographic abnormalities compatible with a myopathy Normal basal metabolic rate Chronically elevated serum lactate and pyruvate values Liver function studies within normal limits Additional findings --Serum creatinin-phosphokinase was increased; on occasion the values were normal, in the cases of Hackett and associates the serum creatininphosphokinase value was normal - - I n the first year of clinical disease, our patient complained of paresthesias --Unexplained bilateral loss of vision (4/10); opthalmoscopically the optic discs seemed pale and there was irregular pigment stippling in the macula region --Electroretinography was normal
had a neurosensory hearing loss which became evident during adulthood. They had no symptoms of myopathy. A son of the sister of the mother had the same mitochondrial myopathy as our patient. Our patient had a normal fasting lucose tolerance test (after 20 hours of fasting, the blood glucose was 69 mg/dl). There was a decreased activity of pyruvate dehydrogenase (tested with pyruvate-l-14C) in leukocytes, 0.84 nrnol/hr/106 leukocytes (normal 2.56 +_ 0.15, n = 15). Metabolism of pyruvate-2-14C was also defective, 0.60 nmol/hr/106 leukocytes (normal 1.74 +__ 0.10, n = 15).2 Studies on isolated rnitochondria, performed by van Dam, revealed a deficiency of cytochrorne c oxidase in muscle tissue) L. Monnens F. Gabre~ls J. Willems Departments of Pediatrics, Neurology, and Biochemistry University of Nijmegen The Netherlands
REFERENCES 1.
2.
3.
Hackett TN Jr, Bray PF, Ziter FA, Nyhan WL, and Creer KM: A metabolic myopathy associated with chronic lactic acidernia, growth failure and n e r v e deafness, J. PED~ATa83:426, 1973. Blass JP, Avigan J, and Uhlendorf BW" A defect in pyruvate decarboxylase in a child with an intermittent movement disorder, J Clin Invest 49:423, 1970. Van Dam K: Unpublished data.
