Dizziness and the autonomic dysfunction syndrome

Dizziness resulting from inner ear or CNS disease has been well described. However, there is a large number of patients for whom dizziness does not seem to be related to either the ears or the CNS. We postulate an abnormality in the autonomic nervous system of such patients and have used standard tilt testing and Valsalva methods to assess the abnormality. Based on the results of these tests, we can suggest therapeutic options.     

N-terminal brain natriuretic peptide in scleroderma-associated pulmonary arterial hypertension: reply

We read with interest the comments of Mathai and Hassoun, andagree that caution is required in using an NTproBNP level of     

A comparison of three methods of repairing the hard palate.

OBJECTIVE: To compare growth, speech, and nasal symmetry outcomes of three methods of hard palate repair. PATIENTS: Consecutive available records of children born with unilateral bony complete cleft lip and palate over the period 1972 to 1992. INTERVENTIONS: Identical management of lip, nose, alveolus, and soft palate. Hard palate repair by Cuthbert Veau (CV) from 1972 to 1981, von Langenbeck (vL) from 1982 to 1989, or medial Langenbeck (ML) from 1989 to 1991. OUTCOME MEASURES: For growth: GOSLON yardstick or 5-year model index. For speech: articulation test. Nasal anemometry. For nasal symmetry: Coghlan computer-based assessment. All these measures were developed during the period of data collection but not for this project. RESULTS: There was a strong trend toward more favorable anteroposterior maxillary growth with the change from CV to vL to ML techniques. This fell short of statistical significance because of the small sample size. There was a significant reduction in cleft-related articulation faults (p =.01) considered to be related to improved arch form. In the absence of improved rates of velopharyngeal insufficiency or nasal symmetry, increased surgical experience was discounted as a significant contribution to improved growth and articulation outcomes. CONCLUSIONS: Reduced periosteal undermining and residual exposed palatal shelf from CV to vL to ML improved incisor relationships and articulation.     

Impaired coagulation in accidental hypothermia of the elderly

Six elderly patients with accidental hypothermia were prospectively evaluated for impaired coagulation. All patients had abnormal coagulation profiles. Three patients with severe coagulopathies and serious underlying conditions died while hypothermic. Despite investigation, no cause for disordered coagulation was found in four patients. We conclude that hypothermia per se contributes to disordered coagulation in the elderly.     

A locus for Bowen-Conradi syndrome maps to chromosome region 12p13.3

Bowen-Conradi syndrome (BCS) is a lethal autosomal recessive disorder with an estimated incidence of 1 in 355 live births in the Hutterite population. A few cases have been reported in other populations. Here, we report the results of a genome-wide scan and fine mapping of the BCS locus in Hutterite families. By linkage and haplotype analysis the BCS locus was mapped to a 3.5 cM segment (1.9 Mbp) in chromosome region 12p13.3 bounded by F8VWF and D12S397. When genealogical relationships among the families were taken into account in the linkage analysis, the evidence for linkage was stronger and the number of potentially linked regions was reduced to one. Under the assumption that all the Hutterite patients were identical by descent for a disease-causing mutation, haplotype analysis was used to infer likely historical recombinants and thereby narrow the candidate region to a chromosomal segment shared in common by all the affected children. This study also demonstrates that BCS and cerebro-oculo-facial-skeletal syndrome (COFS) are genetically distinct.     

Functional and morphologic studies of the adrenal cortex and kidney in ovine toxaemia of pregnancy

Functional and morphologic studies of the adrenal cortex and kidney have been carried out in pregnant sheep with spontaneous or dietary restriction-induced ovine toxaemia. It was found that proteinuria was an inconstant feature and no animal showed glomerular lesions analogous to those found in human preeclampsia; thus ovine toxaemia cannot be regarded as a precise experimental model for human toxaemia of pregnancy. The elevation of blood cortisol levels and the morphologic appearance of the adrenal zona fasciculata found in such animals suggest an adrenal response comparable to that caused by adrenocorticotrophic hormone. In addition, animals with severe disease showed evidence of stimulation of the renin-angiotensin-aldosterone system as reflected by elevated blood renin and aldosterone concentrations and raised renal juxtaglomerular indices. Ultrastructural changes in the adrenal zona glomerulosa and renal juxtaglomerular myoepithelioid cells in toxaemic animals resembled those described in non-pregnant sodium-depleted sheep. The finding of juxtaglomerular peripolar cell mitoses and granule exocytosis, the latter only being previously observed in sodium depleted sheep, together with the ultrastructural changes in the adrenal zona glomerulosa and juxtaglomerular myoepithelioid cells, suggest that sodium depletion may play a role in this disease.     

ACTH-elicited sodium appetite in sheep

Intramuscular injections of long-acting synthetic ACTH (45 U twice daily for 5 days) caused a large increase in the intake of 0.5 M NaCl in sheep. Mean Na intake of the sheep on the last 3 days of treatment approximated 50% of their total extracellular fluid Na. The mineral appetite was specific for NaCl. Intakes of 0.5 M KCl or 0.25 M CaCl2 were not significantly altered. The enhanced appetite for Na induced by ACTH appeared to precede any increase in urinary Na excretion. ACTH treatment was ineffective in adrenalectomized sheep. However, an infusion into adrenalectomized sheep of a combination of adrenal steroid hormones (including aldosterone, deoxycorticosterone, 11-deoxycortisol, cortisol, and corticosterone) that contrived blood levels similar to those, obtained with ACTH treatment in normal sheep did induce Na appetite. Thus, ACTH induces a specific, adrenal-steroid hormone-dependent Na appetite in sheep.