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Biomedical subjects

L Lazar

Publications and source records attributed to L Lazar.

At least 37 records · Page 2Linked to original sources
PubMed2000

Delayed growth and puberty in patients with Gaucher disease type 1: natural history and effect of splenectomy and/or enzyme replacement therapy.

BACKGROUND: Growth retardation in childhood was only recently recognized as a prominent feature of Gaucher disease type 1, but there are few data on both the pubertal development and the final outcome of growth and sexual maturation. OBJECTIVE: To investigate the natural pattern of growth and puberty in patients with Gaucher disease type 1 and the effect of splenectomy and enzyme replacement therapy. METHODS: We retrospectively analyzed growth and puberty in 57 patients with Gaucher disease type 1; 52 were followed since childhood and/or prepuberty and 42 have reached sexual maturity and final height. In the analysis we considered severity of disease, time of splenectomy, and start of enzyme replacement therapy. RESULTS: Deceleration of growth at age 3-5 years was observed in 30 of 57 patients followed since early childhood while untreated: height-SDS decreased from -0.34 +/- 0.42 at age 0-3 years to -1.93 +/- 0.95 (P < 0.01) at age 7-10 years and was more pronounced with severe disease. A high prevalence (59.6%) of delayed puberty, which was more frequent with severe disease, was observed in 47 patients followed before and throughout puberty. No primary endocrine pathology was found. All patients, untreated as well as treated, with growth and pubertal delay had a spontaneous catch-up, achieved full sexual maturation, and most (83.3%) reached a final height within the range of parental height-standard deviation score. Splenectomy (partial and/or total) performed in 20 patients while still growing had a beneficial effect on growth, which was temporary in some and did not affect puberty. ERT improved growth in 11 patients who started therapy before puberty, as evidenced by a progressive increase in the height-SDS, and seemed to normalize the onset of puberty. CONCLUSIONS: Growth retardation in childhood and delay of puberty are characteristic of Gaucher disease type 1 and are more frequent with severe disease. There is a spontaneous catch-up later in life and most patients reach a final height within their genetic growth potential. Enzyme replacement therapy apparently normalizes growth and possibly also the onset of puberty.

Adolescent↗
PubMed1999

A conservative treatment for ingrown toenails in children.

Ingrowing nails are not uncommon in children, and even so receive little attention in the pediatric surgery literature. The purpose of the present study was to demonstrate our modification of the conservative treatment of ingrowing nails. Our technique of local treatment combined with patient education for better foot and toe care was successfully used and well tolerated by 19 out of 20 young patients with ingrown toenails.

Adolescent↗
PubMed1999

Subtotal gastrectomy in a teenager with gastroparesis.

Disorders of gastric emptying are rare in healthy infants and children. Delayed gastric emptying is encountered in adults after operations on the stomach, such as vagotomy and partial gastrectomy, and is extremely rare in young patients. The authors report on a 15-year-old patient with gastroparesis after three attempts to repair a congenital diaphragmatic hernia. Medical therapeutic trials consisting of all combinations of diet regimes with various gastrokinetic drugs failed to alleviate the intractable vomiting. All the patient's symptoms resolved after subtotal gastrectomy with gastroduodenostomy (Billroth I).

Adolescent↗
PubMed1998

[Bowel-lengthening in a newborn with short bowel syndrome].

Advances in parenteral nutrition and supportive therapy have led to improvement in survival of babies with short-bowel syndrome. Those whose intestinal mass is very unlikely to be adequate should have surgical therapy as soon as possible, before they develop the complications of long-term parenteral nutrition or significant enteritis. We present a newborn with short-bowel syndrome due to prenatal midgut volvulus. At operation the remaining viable jejunum, 15 cm long, was anastomosed to the cecum. All feeding attempts failed, and the infant suffered from malabsorption. Calories and proteins had to be supplied by intravenous total parenteral nutrition. At 3 months of age there was significant widening of the remaining bowel and Bianchi's bowel-lengthening procedure was performed. The postoperative course was uneventful and there was gradual improvement in intestinal absorptive capacity. The patient was weaned from parenteral nutrition at 3 years of age. Now, 2 years later, she eats a normal diet.

Anastomosis, Surgical↗
PubMed1998

Diagnosis of deep venous thrombosis by use of soluble necrosis factor receptor.

The purpose of this clinical study was to find a relationship between soluble tumor necrosis factor (TNF) receptor, deep venous thrombosis (DVT), and pulmonary emboli (PE). Clinical diagnosis of deep venous thrombosis and pulmonary emboli is a major dilemma for the clinician. Most existing tests are technician dependent, are expensive, or require sophisticated equipment. Advances in molecular biology will permit investigation of the value of TNF receptor as a test for screening, diagnosis, and evolution of DVT and PE. Forty patients with diagnosis of DVT, documented by real-time ultrasound imaging, were the subject of this clinical study. Five cc of plasma from each patient was studied by enzyme-linked immunosorbent assay (Elisa) for measurement of soluble TNF receptor. Significantly increased concentration of plasma-soluble TNF receptor was present in patients with DVT as opposed to normal individuals. There was a close relationship between plasma concentration of soluble TNF receptor and evaluation of the disease. In this preliminary clinical study, it appears that plasma measurement of soluble TNF receptor could be used as a clinical test for diagnosis of DVT and PE.

Adult↗
PubMed1998

MR findings in growth hormone deficiency: correlation with severity of hypopituitarism.

BACKGROUND AND PURPOSE: Growth hormone deficiency may present as an isolated deficit (IGHD) or in association with multiple deficiencies (MPHD). Previous studies have not compared the MR imaging findings with the severity of hypopituitarism. Our purpose was to determine whether MR imaging can distinguish between IGHD and MPHD. METHODS: Forty-four patients with growth hormone deficiency who were examined by MR imaging were included in this retrospective study. On the basis of the endocrinologic findings, 21 were determined to have IGHD and 23 to have MPHD. The presence, size, location, and morphologic characteristics of the stalk, the neurohypophysis, and the adenohypophysis were recorded in each case. Findings in the two groups were compared. Statistical significance was determined by t-test. RESULTS: The stalk was normal in one patient with IGHD and in none of those with MPHD; it was truncated or thin in 19 patients with IGHD (90%) and in only one with MPHD (4%); it was absent in 22 patients with MPHD (96%) and in only one patient with IGHD (5%). These differences between the two groups were highly significant. In 81% of the IGHD patients and in 91% of the MPHD patients the location of the neurohypophysis was ectopic. This difference between the two groups was not significant. Among IGHD patients, the adenohypophysis was of normal size in 13 patients (62%), small in six (29%), and absent in two (9%); the corresponding findings in MPHD patients were seven (30%), six (26%), and 10 (44%). CONCLUSION: The majority of IGHD patients had a truncated or thin stalk and a normal or small adenohypophysis. An absent stalk and adenohypophysis are characteristic of MPHD. MR imaging can contribute to the prediction of the pattern and severity of hypopituitarism in patients with growth hormone deficiency.

Adolescent↗
PubMed1997

Incidence of colorectal adenocarcinoma by anatomic subsite. An epidemiologic study of time trends and racial differences in the Detroit, Michigan area.

BACKGROUND: Colorectal adenocarcinoma may represent more than one disease process. Numerous epidemiologic studies suggest that rates of occurrence of colorectal adenocarcinoma at particular anatomic subsites (e.g., right colon, left colon, and rectum) may be associated with distinctive geographic, demographic, and risk factor profiles. This study explored time trends over a 22-year period of the incidence of adenocarcinoma of the colon and rectum at various subsites among patients of different race, gender, and stage of disease. METHODS: Data on the incidence of colorectal adenocarcinoma were obtained from a population-based cancer registry in the Detroit, Michigan area funded by the National Cancer Institute. Age-adjusted incidence rates were analyzed by year of diagnosis. Relative survival rates were also obtained for different race and gender categories, along with disease stage at diagnosis. RESULTS: A major rise was revealed in the incidence of adenocarcinoma in the right colon among African American men and women between the mid-1970s and the early 1980s. The rise was greatest among African American men and accounts for increases in late stage disease among them. Corresponding decreases in survival among African American men were noted. CONCLUSIONS: These findings indicated widely differing disease patterns based on anatomic subsite and patient demography and also indicated a need for targeted efforts at early detection of adenocarcinoma of the right colon among African Americans.

Adenocarcinoma↗
PubMed1997

Brain concussion produces transient hypokalemia in children.

Hyperglycemia and hypokalemia caused by catecholamine discharge have been reported to occur in patients after severe head trauma. The aim of this prospective study was to evaluate whether a similar neuroendocrine and metabolic response is found in children after minor head trauma such as brain concussion (Glasgow Coma Scale (GCS) > or = 13). One hundred fifty patients aged 2 to 14 years (average, 6 years) were divided into three groups (n = 50 in each group). Group 1 included patients admitted to the emergency department for brain concussion (Glasgow Coma Scale (GCS) > or = 13); group 2 included patients admitted for fractures of long bones without head injury; and group 3 were control patients electively admitted for hernia repair. All patients had complete physical and neurological examinations. Complete blood count and blood chemistry were obtained on admission. All blood tests were repeated at 6, 12, and 24 hours in patients belonging to group 1. An electrocardiogram was obtained in selected patients and catecholamine levels were measured in some patients. Statistical analysis was performed using analysis of variance (ANOVA). Serum potassium and sodium levels in patients with brain concussion (group 1) were 3.6 +/- 0.6 and 136 +/- 3 mEq/L, respectively and were significantly lower (P < 0.01) than those in patients belonging to group 2, 4 +/- 0.4 and 138 +/- 3, respectively, and the controls (group 3), 4.2 +/- 0.5 and 140 +/- 2, respectively. Serum glucose level was 124 +/- 34 and 118 +/- 32 mg% in groups 1 and 2 and was significantly higher than that of the controls (group 3), 90 +/- 23 mg%. There was no correlation between serum electrolytes and GCS. No electrocardiogram changes or elevation of serum catecholamines were found. Hypokalemia resolved spontaneously within 24 hours. All patients recovered without neurological sequalae. Transient hypokalemia frequently occurs in children even with minor head trauma. This hypokalemia resolves spontaneously, without treatment and within 24 hours.

Adolescent↗
PubMed1997

Pernicious anemia and adenocarcinoma of the stomach in an adolescent: clinical presentation and histopathology.

Primary gastric-carcinoma accounts for only 0.05% of pediatric gastrointestinal malignancies. Although elderly patients who have pernicious anemia are at greater risk of the development of atrophic gastritis and gastric carcinoma, pernicious anemia caused by vitamin B12 deficiency is extremely rare in otherwise healthy adolescents. The authors present a 14.5-year-old boy who had dimorphic anemia caused by vitamin B12 and iron deficiencies in whom atrophic gastritis and gastric carcinoma developed. To the best of our knowledge, this association has not been previously reported in children or adolescents.

Adenocarcinoma↗
PubMed1997

Final height of girls with central precocious puberty, untreated versus treated with cyproterone acetate or GnRH analogue. A comparative study with re-evaluation of predictions by the Bayley-Pinneau method.

This study was designed to determine the benefit of therapy on final height (FHt) in girls with central precocious puberty (CPP). A total of 102 patients were evaluated--28 untreated, 26 treated with cyproterone acetate (CyA), and 48 treated with GnRH analogue (GnRHA)-and their achieved FHt was compared to the respective target height (THt). Of the untreated girls, half (14/28) had a slow course of puberty and reached THt +/- 0.5 SD (FHt 160.2 +/- 7.1, THt 159.5 +/- 6.6 cm); the other half (14/28) had an accelerated course of puberty with a FHt well below THt (FHt 150.8 +/- 4.3, THt, 159.2 +/- 5.9 cm) and in most cases (14/28) below the height-SDS of both parents. The treated girls (both regimens) reached THt above (CyA group: FHt 157.8 +/- 5.1, THt 156.8 +/- 5.1 cm; GnRHA group: 159.6 +/- 6.3, THt 157.7 +/- 5.7 cm). We conclude that without treatment the FHt of girls with CPP may be significantly compromised and that therapy is more beneficial if started before bone age exceeds 12 years. Our data also showed that for final height predictions in CPP the Bayley and Pinneau tables for average children should be used, regardless of the advanced bone age of the patients.

Administration, Oral↗
PubMed1997

MR findings in hereditary isolated growth hormone deficiency.

PURPOSE: To describe the MR characteristics by which patients with hereditary isolated growth hormone deficiency (GHD) can be distinguished from patients with other types of GHD. METHODS: A total of 51 patients with GHD were examined prospectively with MR imaging. On the basis of familial occurrence of GHD and genetic analysis, 10 patients met the criteria for hereditary deficiency. In each case, the height of the pituitary gland, the presence and location of the posterior neurohypophysis, and the completeness of the stalk were recorded. The findings in the hereditary group were compared with those in the rest of the patients. RESULTS: In all 10 patients with hereditary GHD, the adenohypophysis, the neurohypophysis, and the stalk were normal. Of the other 41 patients, the height of the gland was normal in three (7%), the neurohypophysis was abnormal in all, and the stalk was truncated in all but two patients (95%). CONCLUSIONS: The subgroup of patients with hereditary GHD exhibited an anatomically normal pituitary-hypothalamic region. This is in contrast to the majority of patients with idiopathic GHD. MR imaging can contribute to the classification of patients with GHD.

Adolescent↗
PubMed1996

Can ultrasonography replace computed tomography in the initial assessment of children with blunt abdominal trauma?

The evaluation of injured children with suspected blunt abdominal trauma (BAT) is clinically challenging. Computed tomography (CT) requires that patients be sedated, stable, and transportable, and even so, it is considered the diagnostic modality of choice for children with BAT. The authors questioned whether abdominal ultrasonography (US) performed during the initial assessment of the injured child is accurate enough to replace CT in the detection of intraabdominal injury. One hundred twenty-four children with BAT aged 2 to 14 years; (average, 8.3) were admitted to the authors' institution during 1992 and 1993. Some had associated injuries (head, 60; chest, 25; extremities, 15; pelvis, 5). The indications for US were pelvic, abdominal, or lower chest trauma, tenderness, or guarding; altered consciousness; microhematuria; and/or low hemoglobin/hematocrit values. Three patients underwent abdominal CT at the time of admission. For 121 children, an emergency US examination was performed using a 3.5-MHz transducer and a portable machine. The examination evaluated the kidneys, liver, and spleen for parenchymal injuries, and the subhepatic, subphrenic, and paracolic spaces and the pelvis for evidence of free peritoneal fluid. The presence of fluid and/or parenchymal injury was interpreted as a positive US result. Twenty-eight patients had positive US findings. Ten of these had a subsequent positive CT result, eight had a normal CT result, and 10 had a negative second US result. Eleven patients (with a total of 17 visceral injuries) were treated conservatively. One patient underwent emergency surgery for liver and caval injuries. Four patients required blood transfusions. Ninety-three of the 121(78%) had a negative US result. For one of these patients, a subsequent CT scan showed a minor subcapsular splenic hematoma, which resolved spontaneously. The authors conclude that US is sensitive in detecting free peritoneal fluid or visceral injuries and is an effective screening modality. It has replaced abdominal CT in 76% of our patients with suspected BAT. In view of the reliability, simplicity, low cost, and bedside availability of US, the authors suggest that this modality be used in the initial assessment and diagnosis of children with suspected intraabdominal injury from blunt trauma.

Abdominal Injuries↗
PubMed1995

Interrupted air column in the large bowel on plain abdominal film: a new radiological sign of intussusception.

Intussusception is a frequent cause of small bowel obstruction in infants and toddlers. Early confirmation and treatment are paramount. We have recognized a fairly consistent radiological finding in plain abdominal x-rays that had not been described previously. The study compares the x-ray findings of patients with proven intussusception with those of patients with colicky abdominal pain related to other causes. In association with the relevant symptoms, this "interrupted air column" in the large bowel should alert one to the possibility of intussusception.

Colic↗
PubMed1995

Early polycystic ovary-like syndrome in girls with central precocious puberty and exaggerated adrenal response.

Exaggerated adrenal response (ExAR), i.e. hypersecretion of both 17-hydroxypregnenolone (170HPreg) and 17-hydroxyprogesterone(17OHP) in response to adrenocorticotropic hormone (ACTH) stimulation, is frequently found in women with polycystic ovary (PCO) syndrome who had precocious adrenarche. In an earlier study we found an abnormal adrenal response in girls with idiopathic true central precocious puberty (CPP) at early stages of puberty. On follow-up it was noted that a significant number of girls with CPP develop PCO-like syndrome at a relatively young age. The aim of the present study was to determine if there is an association between ExAR and early PCO in girls with a history of CPP. Included were 49 girls with a history of CPP, 34 of whom were treated with gonadotropin-releasing hormone (GnRH) analog. All 49 were evaluated at full maturity, at ages 12.5-14 years, 0.5-4 years after menarche or resumption of menses. Of the 49 girls, 20 had at least 3/4 clinical signs of PCO (irregular menses, hirsutism, acne and obesity) and were defined as PCO-like+, whereas 29 did not fulfil the criteria and were considered PCO-like-. Girls with a definite enzyme deficiency were excluded from the study. All participants underwent a combined iv ACTH-GnRH test at early follicular phase. The PCO-like+ girls all revealed ExAR, i.e. an elevated stimulated 17OHPreg of 63.4 +/- 9.6 nmol/l (normal 28.6 +/- 9.2 nmol/l) and a normal stimulated 17OHPreg/17OHP ratio of 7.1 +/- 1.8 (normal 6.2 +/- 2.7), whereas all the PCO-like- had a normal adrenal response (30.0 +/- 8.7 and 5.3 +/- 2.0 nmol/l, respectively).(ABSTRACT TRUNCATED AT 250 WORDS)

17-alpha-Hydroxypregnenolone↗
PubMed1995

High prevalence of abnormal adrenal response in girls with central precocious puberty at early pubertal stages.

Abnormal adrenal response is often observed in girls with precocious adrenarche (1). We studied the adrenal response in 112 girls with idiopathic true central precocious puberty (CPP) at early stages of puberty compared to that in 21 girls with normal puberty (controls). The aims of this study were to determine the prevalence of abnormal adrenal response at early stages of puberty, the possible correlation of abnormal adrenal response with pubertal signs at onset of puberty and with plasma androgen levels, and a possible association with the activity of the hypothalamic-pituitary-gonadal (HPG) axis. All participants underwent a combined i.v. adrenocorticotropic hormone (ACTH)-gonadotropin-releasing hormone (GnRH) test at Tanner stage 2-3: 62 of the CPP girls before and 50 during treatment with GnRH analog. The stimulated levels of 17-hydroxypregnenolone (17OHPreg) and the stimulated 17OHPreg/17-hydroxyprogesterone ratio were analyzed and compared to previously reported norms. The result revealed three patterns of adrenal response: normal (17OHPreg < or = 24 nmol/l and 17OHPreg/17OHP ratio < or = 7) in 50/112 (44.6%) CPP patients and 17/21 (80.9%) controls; exaggerated (17OHPreg > 24 nmol/l, 17OHPreg/17OHP ratio < or = 7) in 50/112 (44.6%) CPP patients and 3/21 (14.3%) controls; and non-classical 3 beta-hydroxysteroid dehydrogenase deficiency (17OHPreg > 24 nmol/l and 17OHPreg/17OHP ratio > 7) in 12/112 (10.8%) CPP patients and 1/21 (4.8%) controls. The clinical features at onset of puberty were comparable in all girls with the CPP in spite of the different adrenal response patterns.(ABSTRACT TRUNCATED AT 250 WORDS)

17-alpha-Hydroxypregnenolone↗
PubMed1994

GH and GHBP activity and not IGF-1 and its receptor activity express growth velocity reduction during treatment of central precocious puberty by a superactive GNRH analogue.

We studied six patients with central precocious puberty (CPP) (five girls and one boy, aged 9-14.4 years) before and after 5 and 12 months of treatment with GnRH-A (D-TRP-6-LHRH-depo, DECAPEPTYL Ferring, Sweden), and 14 age-matched prepubertal children serving as controls. GnRH suppressed gonadal sex hormone secretion and arrested the gonadarche development. Growth velocity decreased from 9.8 +/- 1.6 (mean +/- SD) to 4.6 +/- 1.0 cm/year after 1 year of treatment. Basal serum IGF-1 levels of the CPP patients were 35.4 +/- 2.8 nmol/l, which was significantly higher than that of the controls (18.8 +/- 1.9, P = 0.00007). After GnRH-A there was a slight but significant increase in serum IGF-1 levels from 35.4 +/- 2.8 (mean +/- SE) to 40.3 +/- 2.1 nmol/l after 5 months (mean difference of 4.9 +/- 1.7, P = 0.02), reversing to 35.6 +/- 2.8 nmol/l after 12 months. The number of high affinity IGF-1 binding sites on erythrocytes (RBC) in the controls was 3.3 +/- 0.3 sites/cell, similar to that found in the patients before treatment. Following therapy, the number of the binding sites decreased from 4.0 +/- 0.8 (mean +/- SE) to 2.5 +/- 0.6 sites/cell after 5 months (mean difference of -1.4 +/- 0.5, P = 0.01), to 2.7 +/- 0.7 sites/cell after 12 months. Basal levels of plasma GH in the CPP patients were 7.1 +/- 1.3 ng/ml, significantly higher than those of the controls (1.2 +/- 0.2, P = 0.00001). Treatment decreased plasma GH in patients from 7.1 +/- 1.3 to 1.3 +/- 0.3 ng/ml after 5 months (mean difference of -5.8 +/- 1.3, P = 0.003) and to 2.7 +/- 1.5 ng/ml after 12 months. The GH binding protein (GHBP) activity in the controls was 76.2 +/- 6.2% relative specific binding, similar to that of the patients before therapy. The mean basal GHBP activity of the patients increased from 75.5 +/- 7.3 to 101 +/- 6.4% (mean difference of 25.5 +/- 9.7, P = 0.01) after 5 months and to 90.1 +/- 5.6% after 12 months of treatment. In conclusion, plasma GH and its receptor activity are a better indicator of the GnRH-A effect on growth than IGF-1 and its receptor activity.

Adolescent↗