Risk factors for mortality-morbidity after emergency-urgent colorectal surgery.

BACKGROUND: The aim of this study was to assess the risk factors associated with mortality and morbidity following emergency or urgent colorectal surgery. MATERIALS AND METHODS: All data regarding the 462 patients who underwent emergency colonic resection in our institution between November 2002 and December 2007 were prospectively entered into a computerized database. RESULTS: The median age of patients was 73 (range 17-98) years. The most common indications for surgery were: 171 adenocarcinomas (37%), 129 complicated diverticulitis (28%), and 35 colonic ischemia (7.5%). Overall mortality and morbidity rates were 14% and 36%, respectively. In multivariate analysis, the only parameter significantly associated with postoperative mortality was blood loss >500 cm(3) (odds ratio (OR) = 3.33, 95% confidence interval (CI) 1.63-6.82, p = 0.001). There were three parameters which correlated with postoperative morbidity: ASA score > or =3 (OR = 2.9, 95% CI 1.9-4.5, p < 0.001), colonic ischemia (OR = 3.4, 95% CI 1.4-7.7, p = 0.006), and stoma creation (OR = 2.2, 95% CI 1.4-3.4, p = 0.0003). CONCLUSIONS: The main risk factors for postoperative morbidity and mortality following emergency colorectal surgery are related to: (1) patients' ASA score, (2) colonic ischemia, and (3) perioperative bleeding. These variables should be considered in the elaboration of future scoring systems to predict outcome of emergency colorectal surgery.

Long-term analysis of primary nonfunction in liver transplant recipients.

UNLABELLED: Long-term allograft and patient survival following liver transplantation continues to improve with the development of new surgical techniques and immunosuppressive agents. Complications such as primary nonfunction (PNF) have not been well characterized in terms of long-term allograft and patient survival. The aim of this study was to determine the incidence of PNF in liver transplant recipients and patient and graft survival, in addition to identifying temporal trends in these parameters. METHOD: Data were obtained from the United Network for Organ Sharing/Organ Procurement and Transplant Network for all adults (>18 years old) who received a deceased donor liver transplant between January 1990 and December 2004. RESULTS: Of the 58,576 liver transplant recipients, 2061 had PNF, an overall incidence of 3.5%. There was a 30% annual increase in the incidence of PNF between 1990 and 2000; the incidence of PNF peaked at 7%, and then decreased by 20% annually thereafter. No differences in donor and perioperative variables were identified to account for this variation. One-, 3-, and 5-year patient and graft survival for patients with PNF who underwent retransplant were significantly lower than those with primary liver transplant. In conclusion, there has been decreased incidence of PNF among liver transplant recipients in the last decade.

Serum cobalamin deficiency is uncommon in multiple sclerosis.

OBJECTIVES: To determine the frequency of serum cobalamin (Cbl) deficiency and to clarify the biologic importance of low screening Cbl levels in patients with multiple sclerosis (MS) and idiopathic myelopathy (MYL). BACKGROUND: A significant association between Cbl metabolism and MS has been postulated based on the observations that patients with MS have lower serum Cbl levels, higher unsaturated Cbl binding capacities, and a higher prevalence of macrocytosis than do normal controls. Whether such observations have biologic importance as documented by abnormal accumulation of metabolites that would result from Cbl deficiency has not yet been determined. METHODS: Serum Cbl and folate levels were determined in 208 consecutively evaluated patients seen in an outpatient MS clinic setting during a 7-month period. Necessary blood samples were obtained for 165 of these patients. One hundred twenty-five patients had clinically definite MS, 31 had clinically probable MS, and nine had MYL. Serum methylmalonic acid (MMA) and homocysteine (HCY) concentrations, which rise in biologically severe Cbl deficiency, were subsequently determined in all patients whose Cbl levels were lower than 301 pg/mL. RESULTS: A Cbl level lower than 301 was found in 32 of 156 patients with either clinically definite MS or clinically probable MS but in none of the patients with MYL. Elevated MMA or HCY levels were found in seven of 32 patients with either clinically definite MS or clinically probable MS, six of whom had an elevated HCY level and one of whom had elevated HCY and MMA levels. CONCLUSIONS: Despite the observation that 32 (19.4%) of 165 of these patients with MS and MYL had screening Cbl levels less than 301 pg/mL, only seven (4.2%) of 165 had elevated MMA or HCY levels. The frequency of biologically severe Cbl deficiency in these patients with MS and MYL was very low.

A phase II study of i.v. methylprednisolone in secondary-progressive multiple sclerosis.

OBJECTIVE: To compare the tolerability and efficacy of two doses of i.v. methylprednisolone in patients with secondary-progressive MS. METHODS: I.v. methylprednisolone administered in high or low dose every other month for up to 2 years to 108 patients with secondary-progressive MS. RESULTS: No significant difference in efficacy with the primary outcome, a comparison of the proportions of patients in each treatment group who experienced sustained progression of disability. A relative treatment effect was detected with the high-dose regimen as measured by the preplanned secondary analysis, a comparison of time to onset of sustained progression of disability. Drug-related adverse events were observed more frequently in high-dose recipients but serious drug-related adverse events were uncommon, and cessation of study drug was only required in one patient. CONCLUSION: The results of the secondary analysis of this study suggest that a phase III trial of corticosteroids for secondary-progressive MS is warranted.

Delayed gastric emptying affects outcome of Nissen fundoplication in neurologically impaired children.

BACKGROUND: Nissen fundoplication (NF) has a relatively high failure rate in neurologically impaired children with gastroesophageal reflux (GER). In 1990 we began to use routine technetium 99m sulfur colloid emptying scans and pyloroplasty with NF for delayed gastric emptying (DGE) in our neurologically impaired patients. The aim of this study was to determine the influence of DGE and pyloroplasty on the outcome of NF in neurologically impaired children. METHODS: One hundred neurologically impaired children underwent NF by a single surgeon between August 1986 and July 1995. Beginning in January 1990 emptying scans were routinely obtained, and patients with DGE underwent pyloroplasty with NF. Outcome analysis was performed for recurrence/wrap failure and other parameters. Mean follow-up was 5.8 years, with a minimum of 18 months. RESULTS: DGE was found in 35 (65%) of the 54 children who had emptying scans. All 11 children with normal scans had successful NF without recurrent reflux (100%). Forty (93%) of 43 children who underwent pyloroplasty and NF had successful outcomes. Thirty-eight children underwent NF without evaluation of gastric emptying with success in 30 of them (78.9%). Overall success improved from 34 (83%) of 41 in the first half of the study, when 3 (7%) of 41 children underwent emptying scans, to 55 (93%) of 59 in the second half, when 51 (86%) of 59 of the children underwent emptying scans. CONCLUSIONS: DGE is common in neurologically impaired children with GER. NF in children with normal gastric emptying has a high probability of success. Pyloroplasty improves the outcome of NF in children with DGE. Neurologically impaired children should be evaluated for DGE before operation for GER.

Quantitative short-term study of anal sphincter function after chemoradiation for rectal cancer.

HYPOTHESIS: Pelvic irradiation adversely affects anal sphincter function after proctectomy with coloanal anastomosis for low rectal and middle rectal (<10 cm from the anal verge) tumors. DESIGN: Case-control study. SETTING: Private, tertiary care referral center. PATIENTS: Patients treated for low rectal adenocarcinoma between January 1, 1994, and October 31, 1999. INTERVENTIONS: Anal manometric data were prospectively collected at the time of initial diagnosis and before ileostomy closure. MAIN OUTCOME MEASURES: Mean and maximum resting pressures (RPs) and squeeze pressures, threshold volume for sensation, and maximal tolerable volume. RESULTS: Twenty-three patients in the surgery group and 19 in the chemoradiotherapy group were considered for analysis; 15 patients had preoperative radiotherapy and 4 had postoperative radiotherapy. At the time of ileostomy closure, RPs were significantly lower in the chemoradiotherapy group than in the surgery group (32.7 +/- 17 vs 45.3 +/- 18 mm Hg; P =.03). Squeeze pressures were not significantly different between the surgery and chemoradiotherapy groups (108.7 +/- 56.7 vs 102.0 +/- 52.6 mm Hg; P =.69). The ratios of postresection to preresection RPs were also significantly lower in the chemoradiotherapy group (0.49 +/- 0.29) than in the surgery group (0.76 +/- 0.22) (P =.005). Eight to 12 weeks after proctectomy with coloanal anastomosis, a 24% decrease in RP was noted in the surgery group. The addition of adjuvant pelvic irradiation decreased RP by another 27%. CONCLUSION: Adequate shielding of the anal sphincter should be performed for low rectal cancers whenever a sphincter-preserving procedure is considered.

CD8+ T lymphocytes and cytomegalovirus retinitis in patients with the acquired immunodeficiency syndrome.

PURPOSE: We compared the levels of CD8+ and CD4+ cells in human immunodeficiency virus (HIV)-seropositive patients who had normal eye examinations, microvasculopathy, or ocular infections other than cytomegalovirus retinitis to those of patients with cytomegalovirus retinitis, to determine whether lymphocyte counts other than CD4+ are predictive of cytomegalovirus retinitis. METHODS: The records of HIV-positive patients who had a lymphocyte subset analysis within three months of a complete eye examination were reviewed for age, gender, mode of HIV transmission, stage of disease, ocular findings, and absolute lymphocyte counts. Data for patients without cytomegalovirus retinitis were compared with those for patients with cytomegalovirus retinitis. RESULTS: Ninety-three HIV-positive patients had a lymphocyte subset analysis within three months of a complete eye examination; 76 patients had no cytomegalovirus retinitis and 17 had cytomegalovirus retinitis. Patients without cytomegalovirus retinitis and those with cytomegalovirus retinitis had the following median cell counts: CD4+, 76.0 and 15.0 cells/microliters; CD8+, 634.5 and 280.0 cells/microliters, respectively. Patients with cytomegalovirus retinitis had significantly lower CD4+ and CD8+ cell counts than those without cytomegalovirus retinitis (P < .001). CD4+ and CD8+ cells are significantly correlated with each other, and the correlation is much higher in patients with cytomegalovirus retinitis (r = .80, P < .001) than in patients without cytomegalovirus retinitis (r = .57, P < .001). Stepwise logistic regression analysis showed that CD8+ cell counts were also predictive of cytomegalovirus retinitis. CONCLUSION: Patients with low CD4+ cell counts are known to be at high risk for cytomegalovirus retinitis. We showed that patients with low CD8+ cell counts are also at high risk for cytomegalovirus retinitis.

Characterization and comparison of wear debris from failed total hip implants of different types.

Particles of wear debris have been associated with loosening of implants and with osteolysis, but few studies have examined the relationship between characteristics of the implant and clinical variables and the concentration of particles isolated from periarticular tissues. We isolated and quantified particles of wear debris from orthopaedic implants in 123 tissue samples that had been obtained adjacent to a failed total hip prosthesis from eighty-eight patients. The concentration of these particles in the tissue and the size of the particles were then analyzed in relation to patient and implant-related variables. The number of particles ranged from 8.5 x 10(2) to 5.7 x 10(11) per gram of tissue (dry weight). More particles were found adjacent to failed titanium-alloy stems that had a cobalt-chromium-alloy modular head and failed titanium-alloy-backed cups than were found adjacent to all-cobalt-chromium-alloy prostheses. In addition, fewer particles were found adjacent to implants with a twenty-eight-millimeter femoral head than were found adjacent to implants with other femoral head sizes. Univariate analysis also showed correlations between a high concentration of particles and fixation without cement, an implant that had been in situ for a long duration, a young patient age, and an initial clinical diagnosis of avascular necrosis. Biopsy specimens from the proximal femoral membranes had higher concentrations than those from the joint capsules or the acetabular membranes. Although only five specimens were obtained directly from osteolytic lesions, the concentration of particles in those specimens was higher than that in biopsy specimens from other sites. Although many univariate correlations were identified, stepwise correlation regression analysis showed that the composition of the implant and the size of the modular femoral head were most strongly related to the concentration of debris in tissue.

P53 and Ki-67 as outcome predictors for advanced squamous cell cancers of the head and neck treated with chemoradiotherapy.

HYPOTHESIS: P53 and Ki-67 status will predict response to treatment, organ preservation, and survival in patients with advanced squamous cell cancers of the head and neck treated with chemoradiotherapy (CRT). STUDY DESIGN: Retrospective analysis of p53 and Ki-67 status from the CRT arm of a randomized, controlled trial (n = 50) and from patients receiving the same treatment but not enrolled in the trial (n = 55). METHODS: P53 and Ki-67 status were established from archived tissue samples using immunohistochemical (IHC) staining. Tumors were positive for p53 (p53+) when more than 2% of cells stained for p53 and were positive for Ki-67 (Ki-67+) when any cell stained for Ki-67. End points were tumor response, tumor recurrence, survival status, and organ preservation at last follow-up, and time to events. Predictive models were calculated for each outcome. RESULTS: Neither marker predicted tumor response to treatment. P53+ status was associated with tumor recurrence (P =.003) and locoregional recurrence (P =.003). Adjusting for time to event, p53+ status was significantly related to a lower recurrence-free survival (P =.004), lower disease-specific survival (P =.04), lower overall survival with primary site preservation (P =.03), and lower disease-specific survival with primary site preservation (P =.003). Multivariate analysis revealed that p53+ status was significantly related to a lower recurrence-free survival (P =.01, risk ratio [RR] = 3.65) and lower disease-specific survival with organ preservation (P =.02, RR = 3.41). Ki-67+ status was not related to any variables. However, multivariate analysis revealed that Ki-67+ was significantly related to a lower overall survival (P =.05, RR = 2.03). The combination of both markers negative (p53-/Ki-67-) was associated with a lower incidence of tumor recurrence (P =.02), lower locoregional recurrence (P =.01), and fewer second primary lesions (P =.04). Adjusting for time to event, p53-/Ki-67- status was significantly related to a higher recurrence-free survival (P =.02), higher disease-specific survival with primary site preservation (P =.02), and higher overall survival with primary site preservation (P =.02). Multivariate analysis revealed that p53-/Ki-67- status was significantly related to a higher overall survival with site preservation (P =.01, RR = 2.78). CONCLUSIONS: P53 and Ki-67 status appear to be related to the various survival end points considered in this study. However, this relation does not seem to be sufficient to warrant treatment modifications. Closer follow-up may be justified in both p53+ and Ki67+ patients to detect recurrence or a second primary at an earlier stage, possibly improving survival.

Contraction patterns of intrinsic laryngeal muscles induced by orderly recruitment in the canine.

The specific performance of intrinsic laryngeal muscles has been difficult to evaluate, especially in living subjects. To reproduce natural contractions, we artificially induced orderly recruitment of their innervating axons according to the size principle. In 5 dogs, both recurrent laryngeal nerves (RLNs) were stimulated with 50 through 10 Hz, 300 to 1000 microA currents while 600 Hz, 1000 to 0 microA decreasing blocking currents were administered. Surface electromyography electrodes placed on the thyroarytenoid, posterior cricoarytenoid, and lateral cricoarytenoid muscles were used to determine the amplitudes (in mA) of compound muscle action potentials. There was a highly statistically significant difference (P<.004) between the thyroarytenoideus which had the fastest rate of recruitment (8.38%), and posterior cricoarytenoideus, which had the slowest (4.81%). There was an intermediate recruitment rate (6.72%) of the lateral cricoarytenoideus, a divergence attributed to a more equal distribution in fast and slow types of myofibers and a smaller sample. We submit that RLN axons can be recruited in an orderly manner according to their sizes and that the rates are unique to the muscle classes they innervate. The parameters defining these contraction patterns may offer key information for laryngeal pacing.

Aggressive concurrent chemoradiotherapy for squamous cell head and neck cancer: an 8-year single-institution experience.

BACKGROUND: Since 1989, 105 patients with squamous head and neck cancer have been treated with combined chemoradiotherapy. OBJECTIVE: To examine the effectiveness of using combined chemoradiotherapy on patients with squamous head and neck cancer. DESIGN: Eight-year (1989-1997) single-institution evaluation of 105 patients. METHODS: Treatment consisted of fluorouracil, 1000 mg/m2 per day, and cisplatin, 20 mg/m2 per day, both given as continuous infusions during 4 days beginning on day 1 and 22 of a concurrent radiotherapy course. Radiation was given in single daily fractions of 1.8 to 2 Gy, to a total dose of 66 to 72 Gy. Salvage surgery was performed for any residual or recurrent locoregional disease. Planned neck dissection was recommended for all patients with N2+ neck disease, irrespective of clinical response. RESULTS: The 105-patient cohort consisted of 79 men and 26 women. The primary site was identified in the oral cavity in 6, oropharynx in 46, larynx in 30, and hypopharynx in 20 patients. Two patients had multiple primaries and 1 patient had an unknown primary. There were 4 patients with stage II, 24 with stage III, and 77 with stage IV disease. Grade 3 and 4 chemoradiotherapy toxic effects included mucositis in 88% of patients, cutaneous reaction in 50%, neutropenia in 49%, thrombocytopenia in 12%, and nausea in 5%. There were no deaths secondary to treatment. The mean weight loss was 12% of initial body weight. To date, primary site persistence or recurrence has occurred in only 14 patients (13%). With a mean follow-up of 39 months, 66 patients (63%) are alive and free of disease. The Kaplan-Meier 4-year projected overall survival is 60% with a disease-specific survival of 74%, a distant metastasis-free survival of 84%, and an overall survival with primary site preserved of 54%. CONCLUSIONS: This chemoradiotherapy regimen, although toxic, is tolerable with appropriate supportive intervention. Locoregional and distant control are likely. Primary site conservation is possible in most patients. Chemoradiotherapy appears to have an emerging role in the primary management of head and neck cancer.

Converted laparoscopic colorectal surgery.

BACKGROUND: Conversion rates following laparoscopic colorectal surgery vary widely between studies, and the outcome of converted patients remains controversial. METHODS: A comprehensive search of the English-language literature was updated until May 1999. RESULTS: Twenty-eight studies on 3232 patients were considered for analysis. The overall conversion rate was 15.38%. Seventy nine percent of the studies did not include a definition for conversion; in these studies, the conversion rate was significantly lower than in the series where a specific definition was considered (13.7% vs 18.9%, chi-square test, p < 0.001). Converted patients had a prolonged hospital stay (11.38 vs 7.41 days) and operative time (209 vs 189 min) in comparison with laparoscopically completed patients (95% confidence interval (CI), 1.70-4.00 and 35.90-37.10, respectively). The factors associated with an increased rate for conversion were left colectomy (Odds Ratio [OR] = 1.061), anterior resection of the rectum (OR = 1.088), diverticulitis (OR = 1.302), and cancer (OR = 2.944) (for each parameter, Wald chi-square value, p < 0.001). CONCLUSIONS: In nonrandomized studies, the rate of laparoscopically completed colorectal resections is close to 85%. Because converted patients have a distinct outcome, a clear definition of conversion is required to compare the results of randomized trials. Such trials should also consider a 20% rate of conversion when estimating the sample size for the desired power level. It is likely that converted patients will have a significant impact on the results of future clinical research in laparoscopic colorectal surgery.

Analysis of catecholamine and vasoactive peptide release in intracranial arterial venous malformations.

Craniotomy for resection of cerebral arterial venous malformation has been associated with postoperative hypertension, which necessitates administration of large doses of antihypertensive medications to control blood pressure. Controlling blood pressure is essential because hypertensive episodes can lead to postoperative cerebral hemorrhage with increases in morbidity and mortality. We measured vasoactive peptide and catecholamine release in 13 patients who underwent resection of an arterial venous malformation and in a control group of 6 patients who presented for clipping of unruptured cerebral aneurysms. Plasma renin activity, angiotensin I and II, vasopressin, aldosterone, epinephrine, and norepinephrine levels were measured intraoperatively and for 36 h postoperatively. Analysis of variance was used to assess sample and group effects. A significant interaction between sample and groups was found for norepinephrine (p < 0.001) and renin (p = 0.002). Our data suggest that elevated plasma renin and norepinephrine levels are in part responsible for postoperative hypertension in patients undergoing resection of arterial venous malformations. Blocking the release of these hormones may help control blood pressure after surgery for removal of arterial venous malformations.

Specialty versus generalist care of children with appendicitis: an outcome comparison.

BACKGROUND/PURPOSE: Some Health Maintenance Organizations (HMO) limit access of their members to specialists to lower costs. The purpose of this study is to determine whether this policy affects the outcome of children with appendicitis. METHODS: At a large academic medical center, children 17 years or younger with appendicitis were treated either by an HMO Adult General Surgical Service (group A) or a Pediatric Surgical Service (group B). Board certified pediatric surgeons were not available on the HMO surgical service. Anesthesia, surgical residents, nursing, and ancillary support services were identical in both groups. Study parameters included imaging tests performed, operation type, complications, readmissions, and length of stay. Results were analyzed using chi(2) and Fischer's Exact tests. RESULTS: One-hundred seventy-five consecutive children underwent appendectomy, 96 in group A and 79 in group B. In patients with simple acute appendicitis, there was no significant difference between group A and group B for complications, readmissions, second operation, or length of stay. In patients with gangrenous or perforated appendicitis there was a significant difference between group A and group B for type of operation (laparoscopic appendectomy, group A, 4 of 27 v. group B, 0 of 34; P =.04); complications (group A, 9 of 27 v. group B, 3 of 34; P =.025); readmissions (group A, 6 of 27 v. group B, 0 of 34; P =.001); second operation (group A, 6 of 27 v. group B, 2 of 34; P =.001); and mean total length of stay in days (group A, 8.6 of 27 v. group B, 5.4 of 34; P =.05). CONCLUSIONS: Children with significantly perforated appendicitis have lower complication rates and shorter lengths of hospital stay when treated by pediatric surgeons as compared with HMO adult general surgeons.

Decreased c-fos expression in experimental neonatal hydrocephalus: evidence for reduced neuronal activation.

Although neonatal hydrocephalus often results in residual neurological impairments, little is known about the cellular mechanisms responsible for these deficits. The immediate early gene, fos (c-fos), functions as a "third messenger" to regulate protein synthesis and is a good marker for neuronal activation. To identify functional changes in neurons at the cellular level, the authors quantified fos RNA expression and localized fos protein in the H-Tx rat model of congenital hydrocephalus. Tissue samples from sensorimotor and auditory regions were obtained from hydrocephalic rats and age-matched, normal litter mates at 1, 6, 12, and 21 days of age (four-six animals in each group) and processed for immunohistochemical analysis of fos and Northern blot analysis of RNA. At 12 days of age, hydrocephalic animals exhibited significant decreases in the ratio of fos immunoreactive cells to Nissl-stained neurons from both cortical regions, but no statistical differences were noted in fos expression. At 21 days of age, both the ratio of fos immunoreactive cells to Nissl-stained neurons and fos expression decreased significantly. The number of fos-positive neurons decreased in all cortical layers but was most prominent in layers V through VI. This decrease did not appear to be caused by neuronal death because examination of Nissl-stained sections revealed many viable neurons within the areas where fos immunoreactivity was absent. These results suggest that progressive neonatal hydrocephalus reduces the capacity for neuronal activation in the cerebral cortex, primarily in those neurons that provide corticofugal projections, and that this impairment may begin during relatively early stages of ventriculomegaly.

Comparison of stone size and response to analgesic treatment in predicting outcome of patients with renal colic.

The aim of this study was to compare the prognostic value of stone size and response to analgesic treatment in patients with renal colic. We reviewed the charts of patients treated for renal colic in our Emergency Department. The eligibility criteria were a radiological examination demonstrating direct or indirect signs of ureteral obstruction and/or a stone. The primary endpoint was the requirement for surgical treatment. The parameters considered as prognostic factors were pain relief with ketorolac (K) or ketorolac plus opiate treatment (KO), and stone size (>or= or <6 mm). Ninety-five patients were considered for analysis. Of these, 49 (52%) had a stone demonstrated radiologically. Four out of 27 patients (15%) in the KO group and six out of 68 patients (8.8%) in the K group required a surgical procedure to relieve the obstruction (NS). Four out of five patients (80%) with a stone >or=6 mm required a surgical procedure, compared with one out of 44 (2.2%) who had a stone smaller than 6 mm (P<0.001). In conclusion, stone size is a better prognostic factor than the response to analgesic treatment in predicting the clinical outcome of patients with renal colic. A stone >or=6 mm in patients with renal colic should alert the emergency physician that urological complications requiring surgical intervention may occur and that urological management may be warranted.

Influence of cholesterol screening and nutritional counseling in reducing cholesterol levels in children. The American Heart Association.

The purpose of this study was to determine whether cholesterol screening and nutritional counseling can reduce cholesterol concentrations in populations of otherwise unrecognized hypercholesterolemic children. A large pediatric practice in Parma Heights, Ohio, has conducted cholesterol surveillance of children over 2 years of age since 1986. The importance of cholesterol and other recognized risk factors for the progression of atherosclerosis is discussed with all families, and the American Heart Association's Step-One diet is recommended. The present study examines data from a cohort of 894 children (473 boys, 421 girls) who had cholesterol concentrations above 185 mg/dL (4.79 mmol/L) (the 90th percentile) at baseline and, after counseling, had a repeat measurement an average of 2.2 years later. Their mean ages were 7 years at the first testing and 9.2 years at the second. Children who had cholesterol concentrations above 200 mg/dL (5.18 mmol/L) (the 95th percentile) had lipoprotein profiles done, and if their LDL cholesterol exceeded 130 mg/dL (3.37 mmol/L) (the 95th percentile), they were referred to a nutritionist, and family members were advised to have their blood lipids analyzed. Mean cholesterol concentration for all 894 children over this time period decreased by 9.4% (19.5 mg/dL [0.51 mmol/L]; 95% CI = 17.5 mg/dL [0.45 mmol/L] to 21.5 mg/dL [0.56 mmol/L]; P < 0.001). A similar decrease of 8.6% (16.6 mg/dL [0.43 mmol/L); 95% CI = 14.0 mg/dL [0.36 mmol/L] to 19.3 mg/dL [0.50 mmol/L); P < 0.001) was observed for the 463 children who had initial cholesterol concentrations between 185 and 200 mg/dL (4.79 and 5.18 mmol/L) and who therefore received a less intense intervention. Cholesterol concentrations in groups of otherwise unidentified hypercholesterolemic children can be significantly reduced as a result of cholesterol screening and nutritional counseling in a pediatric practice setting.

Renal manifestations of tuberous sclerosis complex.

Patients with tuberous sclerosis complex (TSC) are at increased risk of renal disease, predominantly angiomyolipomas and renal cysts. We retrospectively reviewed clinical data of 71 patients diagnosed with TSC. Progression of renal lesions was noted. TSC patients with renal lesions were compared with TSC patients without renal disease. Fifteen of 38 patients had renal abnormalities by imaging at presentation. Six of 9 with initially normal kidneys subsequently developed new lesions. Although not of statistical significance, there was a trend toward increased retinal hamartomas, cardiac rhabdomyomas, and skin lesions in those patients who also had renal abnormalities. Renal disease should be considered and sought in all patients with TSC, both at initial presentation and subsequently, since renal disease is a very significant cause of morbidity and mortality.

Effects of heredity, age, weight, puberty, activity, and calcium intake on bone mineral density in children.

Osteoporosis is a disease that plagues older individuals, particularly women. At the usual age of diagnosis, limited therapy is available. By further delineating the factors that influence bone mineral acquisition before peak bone density is achieved, individuals at risk may be identified at an earlier age when therapies may be more effective. This was a study of 16 family units, 16 mothers, eight fathers, and their 28 children between the ages of 5 and 20 years. The evaluation consisted of a focused history, Tanner staging of adolescents, anthropometric data (height, weight), and spinal bone mineral density (BMD) by DEXA (dual-energy x-ray absorptiometry). Bone mineral density in the children was compared with multiple environmental factors. Bone mineral density Z-scores were then compared between children and their parents. Variables found to be positively correlated with children's BMDs were: age (r = 0.94, P < 0.001), Tanner stage (r = 0.90, P < 0.001), weight (r = 0.88, P < 0.001), height (r = 0.81, P < 0.001), and body mass index (r = 0.77, P < 0.001). No association was found between calcium intake and BMD, owing possibly to increased calcium intake among children with a family history of osteoporosis. Activity was not significantly associated with BMD. Significant correlations were noted between the children's BMD and that of their father's (r = 0.83, P = 0.01), premenopausal mother's (r = 0.58, P = 0.03), and midparental (the mean value of both parents' BMDs) (r = 0.86, P = 0.01). These data suggest that children who have parents affected by low BMD may be at risk for low BMD themselves.