Management and outcome of the dislocated hip hemiarthroplasty.

AIMS: This study describes and compares the operative management and outcomes in a consecutive case series of patients with dislocated hemiarthroplasties of the hip, and compares outcomes with those of patients not sustaining a dislocation. PATIENTS AND METHODS: Of 3326 consecutive patients treated with hemiarthroplasty for fractured neck of femur, 46 (1.4%) sustained dislocations. Of the 46 dislocations, there were 37 female patients (80.4%) and nine male patients (19.6%) with a mean age of 83.8 years (66 to 100). Operative intervention for each, and subsequent dislocations, were recorded. The following outcome measures were recorded: dislocation; mortality up to one-year post-injury; additional surgery; residential status; mobility; and pain score at one year. RESULTS: Of 43 dislocations, 30 (70%) occurred within one month and 42 (98%) occurred within three months of hip fracture surgery. Seven (16%) of these patients were treated with a single closed reduction and sustained no further dislocations. Four (9%) were treated with open reduction and experienced no further dislocations. Three (7%) hips were left dislocated and the remaining 32 (74%) patients required additional surgery of further closed reduction, revision, or excision arthroplasty. The one-year mortality rates for patients treated with two or fewer reductions (open or closed), successful revision arthroplasty, and excision arthroplasty were 3/14 (21%), 1/7 (14%), and 8/14 (57%) respectively. The only statistically significant difference in mortality was the difference between patients who did not sustain a dislocation and those who did and were treated by excision arthroplasty (p = 0.03). Patients treated by excision arthroplasty had the greatest reduction in mobility scores and highest pain scores. The excision arthroplasty group also included the greatest proportion of patients not able to mobilize and the smallest proportion of patients remaining in their own home. CONCLUSION: Most dislocations of hemiarthroplasties of the hip occur within one month of surgery. Closed reduction is generally unsuccessful. For those patients with unsuccessful closed reduction, revision arthroplasty should be considered when possible, as this results in a better functional outcome with a lower mortality than excision arthroplasty.

Definitive use of external fixation for pelvic ring injuries (open book/APC2) in pregnancy.

Pelvic fractures in pregnancy are rare, resulting in a paucity of evidence-based management. We describe a case of open book pelvic injury in a 32-year-old woman in her third trimester of pregnancy. She was successfully managed with a supra-acetabular external fixator, which allowed the safe delivery of a healthy baby boy at 34 weeks, via caesarean section. The external fixator was removed postpartum, when the pelvis was deemed stable, and mother and baby both continue to do well. This is the only case in the literature that demonstrates the successful use of external fixation for pelvic injuries in pregnancy.

Adverse medical complications: an under-reported contributory cause of death in New York City.

OBJECTIVES: The current death certification system in the USA fails to accurately track deaths due to adverse medical events. The aim of this study was to demonstrate the under-reporting of deaths due to adverse medical events due to limitations in the current death certification/reporting system, and the benefits of using the term 'therapeutic complication' as the manner of death. STUDY DESIGN: Retrospective review and comparison of death certificates and vital statistical coding. METHODS: The manner of death is certified as a therapeutic complication when death is caused by predictable complications of appropriate therapy, and would not have occurred but for the medical intervention. Based on medical examiner records, complications that caused or contributed to deaths over a five-year period were examined retrospectively. These fatalities were compared with deaths coded as medical and surgical complications by the New York City Bureau of Vital Statistics. RESULTS: The Medical Examiner's Office certified 2471 deaths as therapeutic complications and 312 deaths as accidents occurring in healthcare facilities. In contrast, the New York City Bureau of Vital Statistics reported 188 deaths due to complications of medical and surgical care. CONCLUSIONS: Use of the term 'therapeutic complication' as the manner of death identified nearly 14 times more deaths than were reported by the New York City Bureau of Vital Statistics. If these therapeutic complications and medical accidents were considered as a 'disease', they would rank as the 10th leading cause of death in New York City, surpassing homicides and suicides in some years. Nationwide policy shifts that use the term 'therapeutic complication' would improve the capture and reporting of these deaths, thus allowing better identification of fatal adverse medical events in order to focus on and assess preventative strategies.

Fatal descent from height in New York City.

All deaths due to descent from height that underwent autopsy at the Manhattan Office of Chief Medical Examiner of New York City over a two year period (1997-1999) were reviewed. The scene and autopsy findings, psychiatric history, and toxicology results were examined. There were 120 deaths: 77 suicides, 36 accidents, 5 undetermined, and 2 homicides. Psychiatric illness was reported in 86% of suicides. The toxicological detection of psychiatric medications supports the high percentage of psychiatric disease in the suicide group. In the accidental group, the detection of ethanol and illicit drugs was higher (36%) than expected from the case investigation and similar to the suicide group (29%). Accidental falls by women made up fewer than 3% of all manners. Due to the variation in the extent of injuries, it is unwise to attempt to conclude how high a person descended based on the autopsy findings. Descents into water commonly have minimal findings on external examination with marked internal injuries. The methods of investigation and criteria for death certification using the study results are discussed.

Mutations in the Na-Cl cotransporter reduce blood pressure in humans.

The relationship between salt homeostasis and blood pressure has remained difficult to establish from epidemiological studies of the general population. Recently, mendelian forms of hypertension have demonstrated that mutations that increase renal salt balance lead to higher blood pressure, suggesting that mutations that decrease the net salt balance might have the converse effect. Gitelman's syndrome, caused by loss of function mutations in the Na-Cl cotransporter of the distal convoluted tubule (NCCT), features inherited hypokalemic alkalosis with so-called "normal" blood pressure. We hypothesized that the mild salt wasting of Gitelman's syndrome results in reduced blood pressure and protection from hypertension. We have formally addressed this question through the study of 199 members of a large Amish kindred with Gitelman's syndrome. Through genetic testing, family members were identified as inheriting 0 (n=60), 1 (n=113), or 2 (n=26) mutations in NCCT, permitting an unbiased assessment of the clinical consequences of inheriting these mutations by comparison of the phenotypes of relatives with contrasting genotypes. The results demonstrate high penetrance of hypokalemic alkalosis, hypomagnesemia, and hypocalciuria in patients inheriting 2 mutant NCCT alleles. In addition, the NCCT genotype was a significant predictor of blood pressure, with homozygous mutant family members having significantly lower age- and gender-adjusted systolic and diastolic blood pressures than those of their wild-type relatives. Moreover, both homozygote and heterozygote subjects had significantly higher 24-hour urinary Na(+) than did wild-type subjects, reflecting a self-selected higher salt intake. Finally, heterozygous children, but not adults, had significantly lower blood pressures than those of the wild-type relatives. These findings provide formal demonstration that inherited mutations that impair renal salt handling lower blood pressure in humans.

Ketamine in non-hospital and hospital deaths in New York City.

We reviewed all ketamine-positive deaths (87) examined at the New York City Office of Chief Medical Examiner over a two-year period (1997 to 1999). There were 15 non-hospital deaths with 12 due to acute multidrug intoxications, one due to sarcoidosis, and two due to physical injury (blunt and thermal). In no instance was a fatal intoxication caused exclusively by ketamine. Opiates (10/15), followed by amphetamines (7/15) and cocaine (6/15), were the most frequent co-intoxicants. Ethanol was found in only one death. The race of all decedents was white and the majority were men (11/15) between the ages of 18 and 30 years. The remaining 72 instances of positive ketamine findings were hospital deaths following surgical procedures or burns.

Radiological risk in perspective: risks and benefits for comforters and carers.

This paper discusses the meaning and origins of the concept of 'comforters and carers' and cites some examples. The application of the Ionising Radiations Regulations 1985 to this group of individuals is described, and the requirements of the European Council directives on protection against ionising radiations are outlined. The paper looks forward to the way these individuals are likely to be treated under the forthcoming revised Ionising Radiations Regulations and describes, with examples, HSE's approach to 'comforters and carers'.

Predictive value of preoperative tests in discriminating bilateral adrenal hyperplasia from an aldosterone-producing adrenal adenoma.

In primary hyperaldosteronism, discriminating bilateral adrenal hyperplasia (BAH) from an aldosterone-producing adenoma (APA) is important because adrenalectomy, which is usually curative in APA, is seldom effective in BAH. We analyzed the results from our most recent 7-yr series to evaluate the predictive value of preoperative noninvasive tests compared with adrenal vein sampling (AVS). Forty-eight patients with hypertensive hyperaldosteronism underwent bedside testing, computed tomography (CT) imaging, and AVS. Those in whom the results of AVS indicated APA underwent adrenalectomy. Twelve (30%) and 14 (34%) of 41 patients with APA had paradoxical falls with ambulation in plasma aldosterone concentration (PAC) and 18-hydroxycorticosterone (18-OH-B), respectively. Twenty-nine (70%) and 26 (65%) APA patients had a rise in PAC and 18-OH-B, respectively, as did all 8 BAH patients. Significant identifiers of BAH were supine PAC values less than 15 ng/dL (P: = 0.04), an increase greater than 60% (P: = 0.02) in PAC with ambulation, and supine 18-OH-B values less than 60 ng/dL (P: = 0.04). CT imaging alone was not predictive for BAH or APA. In our population, patients with a positive bedside test result (e.g. a fall in PAC and/or 18-OH-B) and a unilateral adrenal nodule on CT (10 of 41 patients) could have proceeded directly to adrenalectomy for APA. However, a positive bedside test result with a negative CT or a negative bedside test result regardless of CT findings required AVS to confirm the diagnosis and site of disease.

Hyperplasia of the juxtaglomerular complex with hyperaldosteronism and hypokalemic alkalosis. A new syndrome. 1962.

A new syndrome, characterized by hypertrophy and hyperplasia of the juxtaglomerular apparatus of the kidneys, aldosteronism resulting from adrenal cortical hyperplasia, and persistently normal blood pressure is described in two patients. Overproduction of aldosterone could not be prevented by sodium loading or by administration of albumin intravenously; it was associated with hypokalemic alkalosis and Pitressin-resistant impairment of urinary concentrating ability. In both subjects, increased amounts of circulating angiotensin were demonstrated; infusion of angiotensin II produced rises of blood pressure in both subjects considerably less than the rises induced by comparable doses in normal subjects. The sequence of events, (1) primary resistance to the pressor action of angiotensin, (2) compensatory overproduction of renin and thus of angiotensin, and (3) stimulation of adrenal cortex by angiotensin is consistent with all the information available about the syndrome.

Early presentation of metastatic medullary carcinoma in multiple endocrine neoplasia, type IIA: implications for therapy.

A girl 5 years 11 months of age, belonging to an extensive kindred with multiple endocrine neoplasia, type IIA (MEN IIA), was found to have multifocal medullary thyroid carcinoma with metastasis in one paraglandular lymph node after positive findings on a calcium-pentagastrin stimulation test. Her sister, 3 years 8 months of age, also had an elevated calcitonin level, and thyroidectomy revealed C-cell hyperplasia and a focus of medullary thyroid carcinoma. These two cases underscore the need for prophylactic thyroidectomies in MEN IIA patients as young as 5 years of age and strict yearly provocative screening beginning at age 1 year.

Liddle's syndrome: heritable human hypertension caused by mutations in the beta subunit of the epithelial sodium channel.

Liddle's syndrome (pseudoaldosteronism) is an autosomal dominant form of human hypertension characterized by a constellation of findings suggesting constitutive activation of the amiloride-sensitive distal renal epithelial sodium channel. We demonstrate complete linkage of the gene encoding the beta subunit of the epithelial sodium channel to Liddle's syndrome in Liddle's original kindred. Analysis of this gene reveals a premature stop codon that truncates the cytoplasmic carboxyl terminus of the encoded protein in affected subjects. Analysis of subjects with Liddle's syndrome from four additional kindreds demonstrates either premature termination or frameshift mutations in this same carboxy-terminal domain in all four. These findings demonstrate that Liddle's syndrome is caused by mutations in the beta subunit of the epithelial sodium channel and have implications for the regulation of this epithelial ion channel as well as blood pressure homeostasis.

Erythrocyte and mononuclear blood cell magnesium concentrations are normal in hypomagnesemic patients with chronic renal magnesium wasting.

OBJECTIVE: The purpose of this study was to determine the plasma, erythrocyte, and mononuclear blood cell (MBC) magnesium concentrations in patients with chronic, severe hypomagnesemia due to a chronic magnesium-wasting tubulopathy. METHODOLOGY: Six patients with Bartter's syndrome and five patients with magnesium-wasting tubulopathy were compared with normal subjects. We determined magnesium in plasma, erythrocytes, and MBCs. RESULTS: Patients with chronic magnesium-wasting tubulopathy had a significantly lower plasma magnesium concentration than controls, but erythrocyte magnesium concentration and MBC magnesium concentration and content did not differ significantly between patients and controls. CONCLUSION: Two disorders with chronic magnesium-wasting tubulopathies are associated with a low plasma magnesium concentration but normal erythrocyte and MBC magnesium.

Urinary excretion rate of endothelin-1 in patients with essential hypertension and salt sensitivity.

To assess the possible role of ET-1 in the pathogenesis of hypertension and salt sensitivity levels of immunoreactive endothelin-1 (irET-1) were measured in plasma and urine of 17 patients with essential hypertension and in 19 normotensive control subjects. Effects of alterations in dietary sodium content on urinary irET-1 levels also were assessed. Plasma levels of irET-1 did not differ between the hypertensives and normotensive groups (1.1 +/- 0.3 and 1.3 +/- 0.1 pg/ml). Urine samples of both groups contained high concentrations of irET-1. However, the mean daily urinary excretion of irET-1 in the hypertensives was less than one-third that in controls (29 +/- 3 vs. 109 +/- 21 ng/day, respectively, P < 0.01). Changing dietary sodium content in the hypertensives had no effect on mean irET-1 excretion. However, on either low, intermediate, or high salt diet, "salt sensitive" hypertensives had lower levels of the peptide than "salt resistant" patients (23 +/- 3 vs. 36 +/- 5 ng/day, respectively, P < 0.05). The data demonstrate a marked reduction in irET-1 excretion in patients with essential hypertension, despite normal plasma levels of the peptide. Since ET-1 has diuretic and natriuretic properties, the decreased renal excretion of ET-1 may be of relevance to the pathophysiology of hypertension and salt sensitivity.

Role of muscarinic receptors in renal response to acetylcholine.

Renal arterial infusion of acetylcholine (ACh) (40 micrograms/min) in control dogs produced an ipsilateral increase in renal plasma flow (RPF) and in sodium excretion (UNaV) without a change in glomerular filtration rate (GFR). The increase in RPF and UNaV was maintained during the infusion of ACh. In indomethacin (Indo)-treated dogs (5 mg/kg) ACh produced a transient rise in RPF and UNaV, followed by a progressive decline in RPF and UNaV. The profound renal vasoconstriction was accompanied by a decline in GFR. To determine the role of the muscarinic receptor in the renal vasodilation and in vasoconstriction produced by ACh in Indo-treated dogs, atropine at 6, 60, and 600 micrograms/min was infused into the renal artery before and during the infusion of ACh. In Indo-treated dogs, all dosages of atropine prevented renal vasoconstriction by ACh. Renal arterial infusion of atropine at 600 micrograms/min completely inhibited the renal vasodilation produced by ACh. Atropine infused at 60 micrograms/min partially inhibited, whereas 6 micrograms/min atropine failed to inhibit, the renal vasodilation produced by ACh. Our data suggest that the renal vasodilator and vasoconstrictor effects of ACh in Indo-treated dogs are mediated by two separate types of muscarinic receptors.

Role of prostacyclin in the renal response to acetylcholine.

Renal arterial infusion of acetylcholine (ACh; 40 micrograms/min) in control dogs produced a sustained rise in urinary sodium excretion (UNa V) and in renal plasma flow (RPF). When prostaglandin (PG) synthesis was inhibited with indomethacin (5 mg/kg), ACh produced only a transient rise in UNa V and RPF followed by a progressive decline in UNa V and RPF. Renal arterial infusion of PGI2 (0.2 microgram/min) restored the response to ACh to normal in indomethacin-treated dogs. The RPF was 84 +/- 9 ml/min during control and increased to 132 +/- 19 ml/min with the infusion of PGI2 (p < 0.01). RPF increased further to 188 +/- 18 ml/min at 20-min infusion of ACh and was maintained at 189 +/- 14 ml/min at 100-min infusion of ACh (p < 0.01). Our data suggest that the initial rise in RPF by ACh is independent of the PG system, whereas maintenance of the rise in RPF by ACh requires an intact synthesis of PGs, presumably PGI2 synthesized in the vascular tissues.

Hereditary hypertension caused by chimaeric gene duplications and ectopic expression of aldosterone synthase.

Patients with glucocorticoid-remediable aldosteronism (GRA) from 12 kindreds possess chimaeric gene duplications arising from unequal crossing-over, fusing regulatory sequences of steroid 11 beta-hydroxylase to coding sequences of aldosterone synthase. These chimaeric genes are specific for GRA and explain the biochemistry, physiology and genetics of this form of hypertension. Sites of crossing over range from intron 2 to intron 4. Most mutations have arisen independently from either sister or non-sister chromatid exchange between these genes, which are only 45 kilobases apart. The possibility of a susceptibility allele for GRA of Irish origin is suggested. These findings indicate the utility of a direct genetic test for this disorder.