Diagnosis, treatment, and follow-up of neonatal mepivacaine intoxication secondary to paracervical and pudendal blocks during labor.

Seven infants developed mepivacaine intoxication secondary to accidental injection during paracervical or pudental blocks or both. All presented with unexplained neonatal depression at birth, tonic seizures (often with apnea) within six hours, and characteristic neurologic findings. Twenty-four-hour urinary excretion produced 12.7 to 37.4 mg, exchange transfusions less than 1.02 to 3.5 mg, and gastric drainage or lavage or both 0.63 to 1.26 mg of mepivacaine. Thus promotion of urinary excretion is the treatment of choice. All six survivors are seizure free and neurologically and developmentally normal at one to 4 1/2 years. With early diagnosis and prevention of severe perinatal hypoxia, the prognosis from intoxication alone is very good.     

Hydrocephalus and mental retardation in craniosynostosis

We prospectively studied craniosynostosis, regardless of neurologic status, by cranial computed tomography or psychometric testing in 56 children. None of the 27 children with simple craniosynostosis (single or multiple suture involvement) had evidence of hydrocephalus on CT scan. Of the 24 patients with simple craniosynostosis who underwent psychometric testing, 17 were of average intelligence; six were in the low average range. The single mentally retarded child had a history of severe perinatal asphyxia. Hydrocephalus occurred more frequently (five of 23 cases) in children with complex craniosynostosis syndromes, including Pfeiffer syndrome, Crouzon syndrome, and kleeblattsch?del deformity. More striking than hydrocephalus, however, was the finding of dysmorphic ventricular dilation in eight patients, including the three children with Apert syndrome and four with Crouzon syndrome. Nineteen of the 25 children with complex craniosynostosis syndromes receiving psychometric testing were of normal intelligence. Four children with borderline normal intelligence had either hydrocephalus or ventricular dilation. The two children with mental retardation were sisters with Crouzon syndrome whose family included other retarded individuals. This study indicates that the incidence of hydrocephalus and mental retardation in craniosynostosis is lower than reported previously.     

Pseudohermaphroditis, with testes and a 46, XX karyotype.

A 14 4/12-year-old white girl, evaluated for progressive virilization and clitormegaly, was found to have the unusual combination of a 46, XX karyotype, well-developed Mullerian structures, and dysgenetic testes with Leydig cell hyperplasia. Although there have been previous case reports of 46, XX males, in all of these patients development of the Mullerian ducts had been suppressed. When contemporary classifications of human disorders of sexual differentation were reviewed, no report of a similar patient was found. We speculate that the genotype and phenotype in our patient correspond to the genetic intersexuality of the hornless goat, thereby raising the possibility that the human autosome may play a role in the control of sexual development.     

Prevalence of group B streptococci in parturient mothers and their infants

Eighty pregnant women and their newborn infants were studied for the presence of group B Beta hemolytic streptococci (G.B.S.). G.B.S. were recovered from endocervical cultures in 5 (6.5%) women and 2 (2.5%) had positive throat cultures. Six (7.5%) infants had umbilical and external ear cultures positive for G.B.S. Four infants presented with clinical signs of respiratory distress. All infected infants were treated with penicillin and gentamycin. One case developed fulminant sepsis and died in spite of early diagnosis and treatment.This study shows a high incidence of G.B.S. infection and sepsis with a high mortality rate.     

Absorption, dosage, and effect on mineral homeostasis of 25-hydroxycholecalciferol in premature infants: comparison with 400 and 800 IU vitamin D2 supplementation

Because the efficiency of vitamin D absorption or hepatic uptake and 25-hydroxylation appears decreased in very premature infants, the routine use of 25-hydroxycholecalciferol (25-OHD3) supplementation has been suggested. Absorption studies of a 3 micrograms/kg orally administered dose of 25-OHD3 showed peak serum 25-hydroxyvitamin D2 and -vitamin D3 (25-OHD) concentrations at 4 to 8 hours similar in timing but of lesser magnitude to those seen in adults. Administration of 1 microgram/kg birth weight/day of 25-OHD3 corrected moderately low, but not very low serum (25-OHD) concentrations, and 2 micrograms/kg BW/day resulted in rapid and sustained increase in serum 25-OHD. Administration of 800 IU ergocalciferol (D2) also produced significantly higher serum 25-OHD concentrations than those in infants given 400 IU vitamin D2, but increases in serum 25-OHD were more gradual than in infants given 25-OHD3. In treatment trials with infants weighing less than 1500 gm, those given 800 IU D2, compared with those given 400 IU D2, had higher serum calcium concentrations and less frequent moderate or severe hypomineralization. Infants given 2 micrograms/kg BW 25-OHD3 had a significant increase in serum phosphorus values, but a decrease in serum calcium and magnesium concentrations, and parathyroid hormone also was suppressed to low normal values. The frequency of moderate to severe hypomineralization remained the same as in infants given 400 IU D2. In a subgroup of infants, serum 1,25-dihydroxyvitamin D was elevated over adult values, both in infants given 25-OHD3 (68.5 +/- 8.4 pg/ml) and in infants given vitamin D2 (60 +/- 6.7 pg/ml). Serum vitamin D concentrations were undetectable in four of six infants receiving 25-OHD3, but were elevated (5 to 31 ng/ml) in four infants receiving vitamin D2. Although 800 to 1000 IU D2 can be recommended as routine vitamin D supplementation in very premature infants fed standard formula, the use of 25-OHD3 requires further study.     

Recent Chemotherapy for Child Pneunomia and Empyema

Recent progress in chemotherapy has contributed to the remarkable decrease in mortality from pneumonia in infancy and childhood; however, wide use of antimicrobials has resulted in incapability of etiologic diagnosis. Appropriate chemotherapy for pneumonia should be initiated based on the relative frequency of causative organisms related to age groups, and their antimicrobial susceptibility. Therapeutic development should be reevaluated by the pathogens isolated prior to antimicrobial administration. S. aureus is the most important causative agent in fatal cases of pneumonia. Judging from our studies on offending bacteria in bronchopulmonary infections, H. influenzae, S. pneumoniae and S. aureus are the major pathogens in pneumonia. Therefore, the main initial antimicrobials in uncompromised infants and children should be ampicillin (plus penicillinase-resistant penicillin) covering these organisms. Methicillin-resistant S. aureus, ampicillin-resistant H influenzae and penicillin-resistant S. penumoniae are of great concern. Chemotherapy for these strains is discussed. Treatment with macrolides is mainly recommended for M. pneumoniae. Chemotherapeutic results of treatment of C. trachomatis will be elucidated in a few years.     

Familial granulomatous arthritis, iritis, and rash

Eleven family members over four generations have had granulomatous disease of the skin, eyes, and joints. Ten have had arthritis; two had skin, eye, and joint involvement; one had skin and joint disease, and one had iritis only. The disease is transmitted as an autosomal dominant trait and is not associated with HLA-B27. The disease resembles sarcoidosis in some ways but not in others, and is probably a new syndrome. The major long-term problems are iritis and joint contractures.