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Biomedical subjects

S R Rose

Publications and source records attributed to S R Rose.

At least 37 records · Page 2Linked to original sources
PubMed1996

Jacobsen syndrome: report of a patient with severe eye anomalies, growth hormone deficiency, and hypothyroidism associated with deletion 11 (q23q25) and review of 52 cases.

We have evaluated a patient with Jacobsen syndrome. The patient presented with growth retardation, hypotonia, trigonocephaly, telecanthus, downward slanting palpebral fissures, bilateral inferior colobomas (of the iris, choroid, and retina), hydrocephalus, central nervous system (CNS) abnormalities, and an endocardial cushion defect, features commonly seen in Jacobsen syndrome. Endocrine evaluation showed growth hormone deficiency and central hypothyroidism. Chromosome analysis showed a 46,XX,del(11)(q23q25) de novo karyotype. Cytogenetically, the deletion appeared to include most of bands 11q23 and q24 and a portion of q25. Using chromosome specific paint probe, a combination of chromosome 11 centromere, telomere, and region specific cosmid probes from 11q14.1-14.3, 11q23.3, and 11q24.1, we have localised the deletion breakpoint to q24.1. Phenotype-karyotype correlation of patients with Jacobsen syndrome and specific deletions of chromosome 11q has enabled us to suggest that the critical region for this syndrome lies in close proximity to cytogenetic band 11q24. Although growth retardation is a consistent finding in 11q deletion syndrome, the presence of hypothalamic-pituitary hormone deficiency has not been reported previously.

Abnormalities, Multiple↗
PubMed1996

Overnight growth hormone concentrations are usually normal in pubertal children with idiopathic short stature--a Clinical Research Center study.

To estimate the incidence of low growth hormone (GH) concentration in children and adolescents with idiopathic short stature, overnight GH levels were measured in 167 subjects. The results were compared with data from 132 normal children of similar pubertal stage, bone age, or body mass index. The majority of short children had normal overnight GH concentrations in a distribution not significantly different from that observed in normal children. However, in 6% of children grouped by pubertal stage and in 13% of children grouped by bone age, overnight GH levels were below the 95% confidence limits of normal. The overnight GH levels were above normal in 6%. The observed frequencies of both low and high GH levels were significantly greater than expected (P < 0.001). However, when body mass index was included in the analysis, only 5% of the children had low GH measures, and only 4% had high GH measures (both not significant). This frequency of low overnight GH levels in short children is considerably less than that reported by others. Thus, these data do not support the hypothesis that a deficiency of spontaneous GH secretion is a common cause of short stature. We conclude that standard GH stimulation tests, despite their limitations, remain the best definitive test of GH secretion. Subsequent overnight GH studies may be useful, however, in selected clinical settings such as previous cranial irradiation or other central nervous system disorder.

Adolescent↗
PubMed1996

Insulin-like growth factor I (IGF-I) and IGF-binding protein-3 concentrations compared to stimulated and night growth hormone in the evaluation of short children--a clinical research center study.

To evaluate the relative usefulness of insulin-like growth factor I (IGF-I) and IGF-binding protein-3 (IGFBP-3) in screening for GH status, GH stimulation (arginine-insulin/L-DOPA) tests and overnight GH studies (every 20 min sampling) were performed in 104 healthy short children (32 girls), aged 3-16 yr (height, -1.8 or more SD). IGFBP-3 had no advantage over IGF-I in screening sensitivity or specificity. IGF-I correlated with mean nighttime GH. Both IGF-I and IGFBP-3 correlated with peak stimulated GH. To identify more than 90% of children with GH deficiency (GHD) and borderline GHD, the mean values for age for IGF-I and IGFBP-3 were required as the cut-off criterion. However, at this criterion, 70% or more of idiopathic short stature (ISS) children would have to undergo testing to identify 90% of GHD or borderline GHD. More stringent criteria (-1.0, -1.64, and -2.0 SD) were more specific, but lost sensitivity. A practical application is suggested. Screening use of IGF-I with criterion of -1.0 SD would identify a subgroup that includes 88% of GHD, 71% of borderline GHD, and 46% of ISS. Both IGF-I and IGF-BP-3 higher than -1.0 SD would accurately identify 68% of ISS as not needing GH testing. Evaluation of growth velocity would identify the remaining children requiring definitive testing. Thus, combined screening for GHD using both IGF-I and IGFBP-3 has no better sensitivity than either test alone. However, such combined screening will improve the specificity and thus decrease the number of normal but short children who might otherwise undergo unnecessary testing.

Adolescent↗
PubMed1995

Tracheal rupture following blunt chest trauma presenting as endotracheal tube obstruction.

In this report, we describe a patient in whom a tracheal tear followed blunt thoracic trauma. The diagnosis was made late resulting in problems with ventilation, endotracheal tube obstruction and cardiac arrest. Difficulties with early recognition of tracheobronchial injuries may be caused by non-specific findings as well as the lack of exposure of physicians to patients with these injuries. The signs and symptoms of tracheobronchial injuries are described, as well as their differential diagnoses. A review of airway management has been made as it requires combined anaesthetic and surgical expertise. Injuries of the trachea may have severe, life-threatening consequences and early diagnosis and management reduce morbidity and mortality.

Airway Obstruction↗
PubMed1995

Isolated central hypothyroidism in short stature.

Mild hypothyroidism can be difficult to diagnose, particularly when it is caused by deficiency of TSH or TSH-releasing hormone (TRH). Such central hypothyroidism occurs most often in association with growth hormone deficiency (GHD). Isolated central hypothyroidism has been considered rare. The TSH surge test is the most sensitive test currently available for confirming the diagnosis. Results are abnormal in many cases where response to TRH is normal. Short children referred between 1986 and 1994 for evaluation (n = 235, 74 girls; height 2 or more SD below mean for age; no history of radiation therapy) were selected for study. TSH samples were obtained hourly for study of TSH surge (1400-1800 h and 2200-0400 h). Growth hormone (GH) samples were obtained every 20 min (2000-0800 h). GH stimulation tests included arginine, insulin, and L-dopa. Thirty-nine children were found to have GHD (peak GH < or = 7 micrograms/L) and 15 had mild primary hypothyroidism (TSH 5-15 mU/L). Of the remaining 181 children with apparent idiopathic short stature (ISS), 30 had a blunted TSH surge. All 30 had free thyroxine (FT4) in the lowest third of the normal range, consistent with values seen in documented central hypothyroidism. They were 13% of the total group, 16% of the ISS children, and 32% of the ISS children with FT4 in the lowest third of normal. (In comparison, diagnosis of GHD was made in 16% of the total group.) In conclusion, use of a direct FT4 assay to screen short children for possible subtle hypothyroidism (along with other appropriate testing) identifies a subgroup with a one in three possibility of an easily treated cause--isolated central hypothyroidism.

Analysis of Variance↗
PubMed1995

Thyroid function in non-growth hormone-deficient short children during a placebo-controlled double blind trial of recombinant growth hormone therapy.

GH treatment in GH-deficient children has been reported to cause decreases in serum T4 and TSH and an increase in serum T3. We sought to determine whether GH treatment alters thyroid function in non-GH-deficient short children. Twenty children (18 boys) were followed for 12 months while receiving either GH (Humatrope, Eli Lilly; 0.074 mg/kg, sc, 3 times/week; n = 9) or placebo (n = 11). Total T4, free T4, T3, and TSH were measured every 6 months and in 12 children also at 1, 2, 3, and 9 months. A TRH test and measurement of nocturnal TSH surge were performed at baseline and after 6 months of treatment in 19 subjects. There were no significant differences at baseline in the clinical features between the placebo and GH groups. Total T4, free T4, T3, and TSH levels did not significantly differ between the placebo and GH groups at baseline and at 1, 2, 3, 6, 9, and 12 months. There were no significant differences between the two groups in TSH response to TRH or nocturnal TSH surge. Although an early transient effect of GH treatment could not be excluded, we conclude that GH treatment for 12 months does not produce sustained alterations in thyroid function in non-GH-deficient children.

Body Height↗
PubMed1995

Effects of early cysteamine therapy on thyroid function and growth in nephropathic cystinosis.

Primary hypothyroidism is a known complication of nephropathic cystinosis, a lysosomal storage disorder characterized by renal failure as well as deterioration of other organs. The drug cysteamine depletes lysosomes of cystine and helps preserve renal function and enhance growth in cystinosis patients. To determine whether cysteamine also prevents hypothyroidism, we retrospectively divided 101 patients into group A (n = 28; well treated), group B (n = 26; partially treated), and group C (n = 47; poorly treated). Lifetable analysis indicated a significantly higher probability of remaining free of L-T4 replacement in group A vs. group B (P = 0.09) or group C (P = 0.004). Cysteamine therapy also improved mean height z-scores (-2.17 in group A, -3.04 in group B, and -4.07 in group C) and reduced the bone age deficit (i.e. chronological age minus bone age) by 1.5 yr for every 10 yr of previous cysteamine therapy. We conclude that in addition to its other salutary effects, oral cysteamine therapy helps prevent hypothyroidism and enhances growth in patients with nephropathic cystinosis.

Adolescent↗
PubMed1994

Thyroid function and attention-deficit hyperactivity disorder.

OBJECTIVE: To examine thyroid indices in a community referred sample of boys with attention-deficit hyperactivity disorder (ADHD) for evidence of generalized resistance to thyroid hormone (GRTH). METHOD: TSH, T3, and T4 values were gathered prospectively in 53 physician, school, and/or parent referred ADHD subjects, and in 41 age and gender-matched normal controls. RESULTS: None were in the range suggestive of global or pituitary thyroid hormone resistance. CONCLUSIONS: GRTH is rare, and thyroid function should not be measured routinely in nonfamilial ADHD.

Attention Deficit Disorder with Hyperactivity↗
PubMed1994

Suppressed spontaneous and stimulated growth hormone secretion in patients with Cushing's disease before and after surgical cure.

Growth retardation to complete growth arrest is the hallmark of Cushing's syndrome in children. The major mechanism for this has been considered the glucocorticoid-induced resistance of target tissues to insulin-like growth factor-I (IGF-I) and other growth factors. The purpose of this study was to examine the GH secretory dynamics of patients with Cushing's disease before and up to 12 months after their cure by transsphenoidal adenomectomy. In 14 patients, blood sampling every 20 min over 24 h for determination of plasma GH was performed before and 10-11 days and 3, 6, and 12 months after therapy. These patients also underwent arginine infusion and L-dopa stimulation tests and had measurements of morning baseline GH-binding protein (GHBP), IGF-I, and IGF-binding protein-3 (IGFBP-3) plasma concentrations. Fourteen sex- and pubertal stage-matched normal volunteers were used as controls. Before therapy, the patient group had an increased body mass index (31.5 +/- 5 kg/m2) and markedly decreased plasma mean 24-h GH concentration, mean peak height, and peak area values, with pulse frequency (mean number of peaks) similar to that in the controls. GH values after arginine and L-dopa stimulation were also subnormal in many of these patients, with 2 of 8 and 8 of 10 failing to show GH responses greater than 7 ng/mL in the respective test. In spite of these findings, plasma concentrations of IGF-I, IGFBP-3, and GHBP were within the normal range in these patients. Surprisingly, a pattern of GH suppression similar to that observed in patients with active disease was also seen in patients who were studied 10-11 days and 3, 6, and 12 months after their cure, when their body mass indexes were progressively normalizing, being relatively stable at 10 days, 26.9 +/- 3.8 kg/m2 at 3 months, and 24.8 +/- 3.3 kg/m2 at 12 months. In these patients, plasma IGF-I and GHBP remained normal, whereas IGFBP-3 decreased significantly, albeit within the normal range. The growth rate of 4 patients who were Tanner stage III or below and had not completed their growth at the time of the study increased the year after surgical cure. These findings suggest that patients with Cushing's disease have marked GH suppression during their illness, which, however, does not appear to be a major contributor to the growth suppression observed in this condition. GH hyposecretion continues for at least a year during convalescence, in spite of significant increases in the growth rate in all growing patients.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1994

Emergency department response to hazardous materials incidents.

Emergency physicians must stay alert to situations that involve toxic chemical exposures. An appropriate response to victims of chemical contamination is important for the victim's outcome and the safety of hospital personnel. Successful management of such a situation requires managing information, resources, and patients with complex medical conditions. Chemical contamination may affect one or hundreds of victims. Community planning is essential for an effective response to toxic chemical accidents (Haz-Mat).

Decontamination↗
PubMed1993

Effect of deslorelin-induced pubertal delay on the growth of adolescents with short stature and normally timed puberty: preliminary results.

LH-releasing hormone agonist (LHRHa) treatment slows bone maturation and improves adult height in children with LHRH-dependent precocious puberty. To determine whether pubertal delay induced by LHRHa can enhance final height in patients with short stature and a normally timed puberty, we enrolled 43 short children (28 girls and 15 boys) in a double blind, placebo-controlled trial. Patients were assigned randomly to receive either placebo or LHRHa (deslorelin), administered sc at a dose of 4 micrograms/kg.day for a period of 4 yr. This report describes the preliminary results in 16 children who have completed 4 yr of treatment (9 patients in the deslorelin group and 7 patients in the placebo group). Predicted adult height increased significantly in the deslorelin-treated patients, by 7.6 cm compared to the pretreatment baseline and by 10.3 cm compared to that in the placebo-treated patients (P < 0.005). Four of the 16 patients received concurrent GH treatment (3 among the deslorelin-treated patients and 1 among the placebo-treated patients). Omitting these patients from the analysis did not materially affect the results: predicted adult height in the deslorelin-treated patients increased by 7.2 cm compared to the pretreatment baseline and by 10.9 cm compared to that in the placebo-treated patients (P < 0.005). We conclude that pubertal delay induced by deslorelin significantly increases predicted adult height in adolescents with short stature and a normally timed puberty. Whether deslorelin treatment will increase the final height of these patients cannot be determined until they have stopped growing.

Adolescent↗
PubMed1992

Myalgia and elevated creatine kinase activity associated with subcutaneous injections of diluent.

A 16-year-old boy with short stature and mild primary hypothyroidism received subcutaneous injections of either recombinant human growth hormone or placebo in diluent that contained glycerol and m-cresol as a preservative. While he was receiving the study drug, myalgia developed and serum creatine kinase values were elevated. Both resolved when injections were stopped and recurred when injections of diluent alone were given. The myalgia and elevated creatine kinase activity were apparently caused by a component of the diluent.

Adolescent↗
PubMed1992

Nocturnal thyrotropin surge in growth hormone-deficient children.

Because some patients with growth hormone (GH) deficiency are found to be hypothyroid after initiation of treatment with GH, we assessed the predictive value of the nocturnal thyrotropin surge (a sensitive test for central hypothyroidism) in 56 untreated GH-deficient children and adolescents. Eighteen patients had a subnormal thyrotropin surge (mean 18% (range -30% to 46%)), significantly less than that of 96 normal control subjects (mean 124%; 95% confidence limits, 47% to 300%; p less than 0.01); 13 of the 18 had a subnormal total thyroxine (T4) level or a subnormal free T4 level, or both. These 18 patients were given thyroid hormone replacement therapy; GH deficiency was confirmed during treatment with thyroxine. Of the remaining 38 patients, who had no initial evidence of dysfunction of the hypothalamic-pituitary-thyroid axis, 23 were re-examined while they were receiving GH treatment. Hypothyroidism developed in none of those 23 children during GH therapy. The nocturnal thyrotropin surge test and determination of iodothyronine levels were repeated in 14 of these euthyroid patients. There was no significant change in mean thyrotropin surge (129% (range +49% to +300) vs 125% (range +51% to +222%)), mean serum level of total T4 (111 +/- 4 vs 103 +/- 3 nmol/L), mean serum level of free T4 (19 +/- 0.7 vs 18 +/- 0.8 pmol/L), mean serum level of triiodothyronine (2.5 +/- 0.1 vs 2.5 +/- 0.1 nmol/L), or mean serum level of thyrotropin (2.9 +/- 0.3 vs 2.9 +/- 0.5 mU/L (mean +/- SEM)). We conclude that GH treatment does not appreciably alter thyroid function in GH-deficient patients who have no evidence of thyroid axis dysfunction before GH treatment.

Adolescent↗
PubMed1992

Combining insulin-like growth factor-I and mean spontaneous nighttime growth hormone levels for the diagnosis of growth hormone deficiency.

There is no gold standard for the diagnosis of GH deficiency. Recent data show that spontaneous GH levels may lack sensitivity, and that GH stimulation tests lack specificity as currently performed. Serum insulin-like growth factor-I (IGF-I) measurements lack both sensitivity and specificity. Some of these problems may be explained by nutritional effects. In children, overnutrition decreases GH and increases IGF-I, while undernutrition decreases IGF-I and increases GH. To overcome these difficulties and improve diagnostic accuracy, we combined mean spontaneous nighttime GH levels with IGF-I levels in a statistically based bivariate model. On a two-dimensional plot of mean spontaneous nighttime GH level (in SD units) vs. IGF-I level (in SD units), we defined a new variable, S (sum) score, where S = (1/square root of 2) x (nighttime mean GH SD+IGF-I SD). While IGF-I (SD) and the mean spontaneous nighttime GH (SD) showed a significant correlation with body mass index, the S score was independent of body mass. We, therefore, used the S score to define a new test for GH deficiency. A child failed this bivariate test if his S score was less than -2 SD. We applied this model to 47 normal children and 48 short or slowly growing children (all prepubertal). We measured spontaneous nighttime GH levels and IGF-I levels in all children. In addition, the short children underwent 3 GH stimulation tests. Forty-six of the 47 normal children passed the bivariate test for GH sufficiency. Twenty-three of the 48 short or slowly growing children failed the bivariate test, whereas only 11 children had an abnormally low mean spontaneous nighttime GH measurement alone. Sixteen of 23 children who were GH deficient by the bivariate test were also GH deficient by the stimulation tests. In summary, the bivariate test for GH deficiency appears 1) to be independent of body mass, unlike either IGF-I or GH individually; 2) to identify more children than the mean spontaneous nighttime GH level alone; and 3) to be highly specific in the normal population, unlike stimulation tests.

Body Mass Index↗
PubMed1991

Poisoning with equine phenylbutazone in a racetrack worker.

Phenylbutazone is a potent nonsteroidal, anti-inflammatory drug often used by veterinarians to treat racetrack animals. Its use in human beings is limited because of significant adverse effects and the availability of newer, safer drugs. We report the case of a 24-year-old man who ingested 17 g of equine phenylbutazone over a 24-hour period to treat the pain of a toothache. He developed grand mal seizures, coma, hypotension, respiratory and renal failure, and hepatic injury. Serum phenylbutazone concentration obtained approximately eight hours after presentation was 900 micrograms/mL. The patient recovered during six weeks of intensive supportive care and repeated hemodialysis.

Acute Kidney Injury↗
PubMed1991

Simulated acetaminophen overdose: pharmacokinetics and effectiveness of activated charcoal.

STUDY OBJECTIVE: To determine the absorption rate of a supratherapeutic dose of acetaminophen elixir and compare the effect of activated charcoal (AC) given at different time intervals on preventing acetaminophen absorption. DESIGN: Randomized, nonblinded, crossover controlled study. SETTING: A certified regional poison control center. PARTICIPANTS: Ten healthy, adult male volunteers from 21 to 39 years old. INTERVENTIONS: Each subject received 5 g acetaminophen (elixir) on four occasions: a control phase plus 30 g of AC administered 15, 30, or 120 minutes after acetaminophen. Serum acetaminophen levels were obtained during the control phase only, and 24-hour urine collections were obtained during all four phases. MEASUREMENTS AND MAIN RESULTS: The highest serum acetaminophen levels were measured 1.4 +/- 0.52 hours after ingestion, and absorption was 97% complete by a mean of 2.05 hours. The administration of AC at 15, 30, and 120 minutes after acetaminophen reduced urinary recovery of acetaminophen and metabolites by 48%, 44%, and 33%, respectively. CONCLUSION: AC significantly reduces urinary recovery but not absorption of acetaminophen when administered two hours after acetaminophen elixir.

Acetaminophen↗
PubMed1991

Low growth hormone levels are related to increased body mass index and do not reflect impaired growth in luteinizing hormone-releasing hormone agonist-treated children with precocious puberty.

To test the hypothesis that GH deficiency might explain the low growth velocity of some LHRH agonist (LHRHa)-treated children with central precocious puberty, we measured stimulated (n = 81) and spontaneous (n = 32) GH levels during or after LHRHa treatment. GH stimulation tests in the children who were receiving LHRHa treatment were performed after 2 days of ethinyl estradiol administration. Thirty-one of 81 children (38%) who underwent GH stimulation tests had subnormal responses (less than or equal to 7 micrograms/L) to all tests administered (at least 2 stimuli), including 22 of 67 (33%) who had precocious puberty that was idiopathic or associated with hypothalamic hamartoma. Eleven of 32 children (34%) who underwent measurement of the mean nighttime spontaneous GH level had levels below the normal range for prepubertal children (less than 1.2 microgram/L). Despite the high incidence of subnormal GH levels, there appeared to be no relationship between the GH levels of these children and their growth characteristics. The height, growth velocity, bone maturation rate, predicted height, and insulin-like growth factor-I levels were not different between the children with low GH levels and the children with normal GH levels. Conversely, the GH levels were not different between the children with subnormal growth rates and the children with normal growth rates. Thus, variation in the growth rates of these LHRHa-treated children with central precocious puberty could not be explained by variation in the stimulated or spontaneous secretion of GH. In attempting to understand the high incidence of low GH levels in children with precocious puberty, we examined the relationship between GH level and body mass index (BMI). Both the stimulated (r = -0.33; P less than 0.002) and the spontaneous (r = -0.61; P less than 0.0002) GH levels were inversely related to BMI. Moreover, the children with precocious puberty as a group had significantly elevated BMI [1.2 +/- 0.1 (+/- SE) SD units] compared to normal children of the same age (P less than 0.0001). Thus, increased body mass may explain the high incidence of subnormal GH levels in these patients, and normative GH levels adjusted for body mass are needed before it can be concluded that the apparently subnormal GH levels in LHRHa-treated children with precocious puberty are in fact low.

Adolescent↗