Hypomagnesemia following correction of metabolic acidosis: a case of hungry bones

Severe symptomatic hypomagnesemia (0.15 mmol/L [0.3 mEq/L]) and hypocalcemia (1.47 mmol/L [5.9 mg/dL]) occurred in a 4-week-old infant coincidental with correction of a severe renal tubular acidosis with alkali therapy. The patient had no evidence of gastrointestinal abnormality and magnesium (Mg) intake was adequate for age and weight. Extreme renal conservation of Mg was observed, supporting the presence of Mg depletion. We suggest that Mg depletion in this infant occurred due to acidosis-induced bone demineralization and that symptomatic hypomagnesemia was precipitated by rapid remineralization accompanying correction of systemic acidosis. This patient represents a novel case of hungry bone syndrome (HBS). Since HBS has not been described previously in patients with acidosis undergoing therapy, several other factors may have contributed to this patient's severe hypomagnesemia, namely, prematurity, twin status, severity of acidosis, rapidity of correction of acidosis, catch-up growth and calcium supplementation. Clinicians should be vigilant for HBS in infants with severe acidosis undergoing alkali therapy.     

Occupational lead exposure and hearing loss

Studies of adults, children, and laboratory animals suggest an association between lead exposure and hearing loss. A causal relationship might direct mandated medical surveillance of lead-exposed workers to include audiometric testing. A cross-sectional, computerized dataset was obtained from a private occupational health screening company to examine the relationship between blood lead level and hearing loss. Audiometry and blood lead results were available for 183 workers. A statistically significant correlation was found between blood lead level and an elevated hearing threshold at 400 Hz (P = 0.03); no other frequencies showed such a correlation. This finding suggests either an interaction between nose exposure and lead, interaction of other exposure factors (such as cigarette smoking), or that factors other than biomechanical ones render the organ of Corti more susceptible at 4000 Hz. Further evaluation of these questions should be undertaken. Computerized databases created for worker surveillance may be a source for data useful for examining other causal connections in occupational settings.     

The use of quantitative bacteriologic assessment of bone

A new sub-class of binding protein-dependent transporter with specificity for a broad range of polar amino acids has been identified by sequence comparison, in Rhizobium leguminosarum, Rhodobacter capsulatus, Escherichia coli and Pseudomonas fluorescens. Southern blotting and PCR analysis has shown that transporters from this new sub-class are widely distributed in Gram-negative bacteria, including, in addition to the above, Citrobacter freundii, Erwinia carotovorum and Rhizobium meliloti. ABC transporters of polar amino acids can be divided into two groups: those with narrow solute specificity and the newly identified sub-class with broad solute specificity. The binding and inner membrane proteins from transporters with a broad solute specificity are larger by approximately 30% than those with a narrow solute specificity. Multiple alignment of the inner membrane proteins from all sequenced polar amino acid transporters indicates there is an N-terminal conserved region that may be involved in solute specificity. A conserved arginine or lysine at residue 30 of this region is changed to glutamate in arginine transporters. Residue 53 also has a strong correlation with the charge on the transported solute, with basic amino acid transporters replacing an aliphatic amino acid at this position with a negatively charged amino acid. The general amino acid permease from R. leguminosarum, which will transport aliphatic as well as basic and acidic amino acids, juxtaposes two prolines at residues 52 and 53 of the N-terminal conserved region.     

Transrectal electrostimulation therapy for neuropathic bowel dysfunction in children with myelomeningocele

PURPOSE: We attempted to evaluate the efficacy of transrectal bowel stimulation for neurogenic bowel dysfunction in children with myelodysplasia. MATERIALS AND METHODS: Daily sessions of transrectal electrostimulation were performed on an outpatient basis for 2 to 3 weeks on children with myelodysplasia and stool incontinence. If benefits were noted, 5 to 10 additional daily sessions were performed. Complete success was defined as improvement in all parameters of interest, including decrease in the frequency of daily bowel movements, increased sensation, increased ability to hold stool and a significant subjective change in bowel habits. Moderate success implied improvement in 1 to 3 parameters and treatment failure was defined as lack of improvement in any parameter. RESULTS: A total of 55 children 2 to 14 years old (mean age 6.7) completed a mean of 18 daily sessions per patient of bowel electrostimulation. Followup ranged from 1 to 6 years. Diapers are no longer required due to defecation problems in 14 children older than 3 years. Complete success was achieved in 20 cases (36.3%) and moderate success in an additional 30 (54.5%, overall success rate 90.8%). Specifically, 89% of the patients reported elimination of stooling accidents, 82% reported increased sensation and 71% were able to hold the bowel movement. Overall 68% of the patients noticed significantly improved bowel function. Complete/moderate success of transrectal electro-stimulation was statistically significant for all 4 parameters (p < 0.05), and complete success was significant for increased sensation, ability to hold and episodes of accidents. Therapy failed in 5 children (9%). There were no untoward effects. CONCLUSIONS: Transrectal electrostimulation is a well tolerated and minimally invasive modality that provides sustainable improvement in stool continence in children with myelomeningocele and neuropathic bowel dysfunction.     

Diagnostic and prognostic relevance of malignancy-associated changes in cervical smears

OBJECTIVE: For approximately 15 years, malignancy-associated changes (MACs) have been consistently found by means of high-resolution cytometry in different tissues, especially in visually normal appearing cervical cells. Their biologic nature is not yet fully understood. The aim of this investigation was to assess the expression of MACs in cervical smears and to evaluate their prognostic relevance. STUDY DESIGN: This study was performed on normal intermediate cells obtained from 53 cytologically positive and 78 cytologically negative cervical smears. From a second sample, 31 cases showing negative cytology were selected for a prospective longitudinal study. Densitometric and texture features were generated, and MACs were described on the basis of multivariate discriminant analysis. RESULTS: Discrimination between positive and negative cases was possible, with a correct classification rate of approximately 80%. After a mean period of 29.5 months, we noted no statistically significant increase in the incidence of cervical intraepithelial neoplasia in the group of healthy but MAC-positive women as compared to those who were MAC negative. CONCLUSION: MACs were constantly expressed in the epithelium of the cervix. Although their prognostic relevance remains unclear, MACs play an important role in the effort to automate cervical cytology.     

Measurement of neonatal responses to painful stimuli: a research review

The measurement of neonatal responses to painful stimuli remains a significant clinical problem. Although numerous measures have been evaluated, instruments that are valid, reliable, and clinically feasible are not yet available. The purpose of this paper is to critique the studies that have been done using biochemical, physiological, and behavioral measures to evaluate neonatal responses to painful stimuli. Specific issues regarding measurement in premature and critically ill neonates are emphasized. The intent of this review and critique of the literature is to stimulate additional research into the assessment of neonatal pain.     

Scabies associated with a bullous pemphigoid-like eruption

Intramuscular phenol neurolysis is a well-known procedure used to decrease spasticity and improve function in patients who have failed to respond to more conservative forms of intervention. Traditionally, this approach has been limited to spasticity reduction in limb muscles, and its use in managing spasticity of the facial muscles has not been described in the literature. This case report describes a new and previously unreported application of intramuscular neurolysis for managing severe unrelenting facial muscle spasticity in a postanoxic encephalopathic patient. Prior to the procedure, hypertonicity in the orbicularis oris muscle was so profound that it limited speech and affected cosmetic, hygienic, and nutritional status. After intramuscular phenol neurolysis of the orbicularis oris muscle, the patient's level of functioning improved.     

Reduced dosage of genes encoding ribosomal protein S18 suppresses a mitochondrial initiation codon mutation in Saccharomyces cerevisiae

A yeast mitochondrial translation initiation codon mutation affecting the gene for cytochrome oxidase subunit III (COX3) was partially suppressed by a spontaneous nuclear mutation. The suppressor mutation also caused cold-sensitive fermentative growth on glucose medium. Suppression and cold sensitivity resulted from inactivation of the gene product of RPS18A, one of two unlinked genes that code the essential cytoplasmic small subunit ribosomal protein termed S18 in yeast. The two S18 genes differ only by 21 silent substitutions in their exons; both are interrupted by a single intron after the 15th codon. Yeast S18 is homologous to the human S11 (70% identical) and the Escherichia coli S17 (35% identical) ribosomal proteins. This highly conserved family of ribosomal proteins has been implicated in maintenance of translational accuracy and is essential for assembly of the small ribosomal subunit. Characterization of the original rps18a-1 missense mutant and rps18a delta and rps18b delta null mutants revealed that levels of suppression, cold sensitivity and paromomycin sensitivity all varied directly with a limitation of small ribosomal subunits. The rps18a-1 mutant was most affected, followed by rps18a delta then rps18b delta. Mitochondrial mutations that decreased COX3 expression without altering the initiation codon were not suppressed. This allele specificity implicates mitochondrial translation in the mechanism of suppression. We could not detect an epitope-tagged variant of S18 in mitochondria. Thus, it appears that suppression of the mitochondrial translation initiation defect is caused indirectly by reduced levels of cytoplasmic small ribosomal subunits, leading to changes in either cytoplasmic translational accuracy or the relative levels of cytoplasmic translation products.