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Zvi Laron - One of the best experts on this subject based on the ideXlab platform.
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Laron Syndrome – A historical perspective
Reviews in Endocrine and Metabolic Disorders, 2020Co-Authors: Zvi Laron, Haim WernerAbstract:Laron Syndrome (LS) [OMIm#262500], or primary GH insensitivity, was first described in 1966 in consanguineous Jewish families from Yemen. LS is characterized by a typical phenotype that includes dwarfism, obesity and hypogenitalism. The disease is caused by deletions or mutations of the GH-receptor gene, causing high serum GH and low IGF-I serum levels. We studied 75 patients from childhood to adult age. After early hypoglycemia due to the progressive obesity, patients tend to develop glucose intolerance and diabetes. The treatment is by recombinant IGF-I, which improves the height and restores some of the metabolic parameters. An unexpected finding was that patients homozygous for GH-R defects are protected from malignancy lifelong, not so heterozygotes or double heterozygote subjects. We estimate that there are at least 500 patients worldwide, unfortunately only few treated.
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Laron Syndrome - A historical perspective.
Reviews in endocrine & metabolic disorders, 2020Co-Authors: Zvi Laron, Haim WernerAbstract:Laron Syndrome (LS) [OMIm#262500], or primary GH insensitivity, was first described in 1966 in consanguineous Jewish families from Yemen. LS is characterized by a typical phenotype that includes dwarfism, obesity and hypogenitalism. The disease is caused by deletions or mutations of the GH-receptor gene, causing high serum GH and low IGF-I serum levels. We studied 75 patients from childhood to adult age. After early hypoglycemia due to the progressive obesity, patients tend to develop glucose intolerance and diabetes. The treatment is by recombinant IGF-I, which improves the height and restores some of the metabolic parameters. An unexpected finding was that patients homozygous for GH-R defects are protected from malignancy lifelong, not so heterozygotes or double heterozygote subjects. We estimate that there are at least 500 patients worldwide, unfortunately only few treated.
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IGF-I deficiency, longevity and cancer protection of patients with Laron Syndrome.
Mutation research. Reviews in mutation research, 2016Co-Authors: Zvi Laron, Rivka Kauli, Lena Lapkina, Haim WernerAbstract:Laron Syndrome (LS) is a unique model of congenital IGF-I deficiency. It is characterized by dwarfism and obesity, and is caused by deletion or mutations of the growth hormone receptor (GH-R) gene. It is hypothesized that LS is an old disease originating in Indonesia and that the mutated gene spread to South Asia, the Middle East, the Mediterranean region and South America.
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LESSONS FROM 50 YEARS OF STUDY OF Laron Syndrome
Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists, 2015Co-Authors: Zvi LaronAbstract:ABSTRACT Objective: To describe the characteristics of untreated and recombinant insulin-like growth factor 1 (IGF-1)- treated patients with the Laron Syndrome (LS) as seen in our clinic over a period of over 50 years. In 1966, we reported a new disease, characterized by dwarfism (-4 to -10 height standard deviation score) typical facial features, small head circumference, obesity, and small genitalia. They resembled congenital growth hormone (GH) deficiency but had high levels of serum human GH and low IGF-1. Since then, our cohort grew to 69 patients, consisting of Jews of oriental origin, Muslins, and Christians originating from the Middle East or Mediterranean area. Many belong to consanguineous families. Methods: Molecular genetic investigations revealed that these patients had deletions or mutations in the GH receptor gene, but only individuals homozygous for this defect express the disease, coined “Laron Syndrome” (LS; Online Mendelian Inheritance in Man# 262500). Results: During childhood, LS patients grow slowly, have a retarded bone age and sexual development, but reach full sexual development. The treatment of LS is recombinant IGF-1, which stimulates the linear growth but increases the degree of obesity. Adult-age patients with congenital IGF-1 deficiency are protected from cancer but can develop insulin resistance, glucose intolerance, diabetes, and cardiovascular disease. Due to pathologic changes in the brain related to the type of molecular defect in the GH receptor, they vary in their intellectual capacity. A number of LS patients marry, and with help of pregestational genetic diagnosis, have healthy children. Conclusion: LS is a unique disease model presenting a dissociation between GH and IGF-1 activity. Abbreviations: GH = growth hormone hGH = human growth hormone IGF-1 = insulin-like growth factor 1 LS = Laron Syndrome rIGF-1 = recombinant IGF-1 SDS = standard deviation score
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CLINICAL STUDY Pituitary size in patients with Laron Syndrome (primary GH insensitivity)
2015Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:Objective: The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron Syndrome, leads to an increase in the size of the pituitary gland. Methods: Eleven patients (six females, five males) with Laron Syndrome underwent magnetic reso-nance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36–68 years and two children, a 4-year-old boy and a 9-year-old girl. The latter patient had been treated with IGF-I (150–180 mg/kg per day) since the age of 3 years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and com-pared with reference values for age and sex. Results: The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary–hypo-thalamic region were detected. Conclusion: Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary–hypothalamic region in Laron Syndrome is normal. European Journal of Endocrinology 148 339–34
Liora Kornreich - One of the best experts on this subject based on the ideXlab platform.
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CLINICAL STUDY Pituitary size in patients with Laron Syndrome (primary GH insensitivity)
2015Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:Objective: The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron Syndrome, leads to an increase in the size of the pituitary gland. Methods: Eleven patients (six females, five males) with Laron Syndrome underwent magnetic reso-nance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36–68 years and two children, a 4-year-old boy and a 9-year-old girl. The latter patient had been treated with IGF-I (150–180 mg/kg per day) since the age of 3 years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and com-pared with reference values for age and sex. Results: The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary–hypo-thalamic region were detected. Conclusion: Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary–hypothalamic region in Laron Syndrome is normal. European Journal of Endocrinology 148 339–34
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Laron Syndrome Abnormalities: Spinal Stenosis, Os Odontoideum, Degenerative Changes of the Atlanto-odontoid Joint, and
2015Co-Authors: Small Oropharynx, Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:hormone resistance. We investigated abnormalities in the upper airways and cervical spine in patients with Laron Syndrome. METHODS: We prospectively examined 11 patients (one child aged 9 years and 10 adults aged 36–68 years), 10 of whom underwent MR imaging of the spine or head; nine, radiography of the cervical spine; and four, CT of C1-C2. The width of the spinal canal was evaluated visually and quantitatively and compared with reference values. The smallest diameter of the orophar-ynx and the thickness of the palate were measured and compared with reference values. Nine age-matched female patients referred for MR imaging for unrelated reasons served as control subjects. RESULTS: Cervical spinal stenosis was present in seven of the adult patients, within a confidence interval of 95%. Anomaly of the dens compatible with os odontoideum was present in three patients, causing focal myelomalacia in two. The atlanto-odontoid joint showed osteoarthritic changes in six of the adult patients. The mediolateral diameter of the oropharynx was significantly smaller in the patients with Laron Syndrome than in the control subjects (P <.005). There was no difference in the thickness of the soft palate. CONCLUSION: Patients with Laron Syndrome develop significant narrowing of the cervica
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CLINICAL STUDY Craniofacial and brain abnormalities in Laron Syndrome (primary growth hormone insensitivity)
2013Co-Authors: Liora Kornreich, Gadi Horev, Boaz Karmazyn, M Schwarz, Z. LaronAbstract:Objective: To investigate abnormalities in the craniofacial structures and in the brain in patients with Laron Syndrome. Design: Eleven patients with classical Laron Syndrome, nine untreated adults aged 36–68 years and two children aged 4 and 9 years (the latter treated by IGF-I), were studied. Methods: Magnetic resonance images of the brain were obtained in all the patients. One patient also underwent computed tomography. The maximal diameter of the maxillary and frontal sinuses was measured and compared with reference values, the size of the sphenoid sinus was evaluated in relation to the sella, and the mastoids were evaluated qualitatively (small or normal). The brain was evaluated for congenital anomalies and parenchymal lesions. Results: In the adult untreated patients, the paranasal sinuses and mastoids were small; in six patients, the bone marrow in the base of the skull was not mature. The diploe of the calvaria was thin. On computed tomography in one adult patient, the sutures were still open. A minimal or mild degree of diffuse brain parenchymal loss was seen in ten patients. One patient demonstrated a lacunar infarct and another periventricular high signals on T2-weighted images. Two patients had cerebellar atrophy. Conclusions: The present study has demonstrated the important role IGF-I plays in the development of the brain and bony structures of the cranium. European Journal of Endocrinology 146 499–50
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Imaging Findings in Laron Syndrome
Laron Syndrome - From Man to Mouse, 2010Co-Authors: Liora Kornreich, Zvi LaronAbstract:The imaging findings by the various modalities (X-rays, CT, and MR) in our cohort of Laron Syndrome patients, during 50 years of follow-up, are described. The face, the orbits and the base of skull are small with underdeveloped paranasal sinuses and mastoids. The skull is relatively large for the small body. Frequently occurring is the anomaly of the dens of the os odontoideum type. Reduced dimensions of the larynx and oropharynx result in propensity for sleep apnea.The brain presents no specific pathological findings, and no abnormalities of the pituitary gland are detected. In the spine and appendicular skeleton every depicted bone is proportionally small. In the cervical spine this results in spinal stenosis. In the lumbar spine, the spinal canal is narrow as well. There is a tendency for early osteoarthritic changes. No signs of osteopenia are detected. There is marked retardation of bone age.
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Hand size and growth in untreated and IGF-I treated patients with Laron Syndrome.
Journal of pediatric endocrinology & metabolism : JPEM, 2009Co-Authors: Osnat Konen, Pearl Lilos, Liora Kornreich, Aviva Silbergeld, Zvi LaronAbstract:UNLABELLED We have previously reported on the linear growth, growth of the head circumference and foot length in untreated and IGF-I treated patients with Laron Syndrome (LS) (primary GH insensitivity). AIM To assess the size and growth of the hands in patients with LS from early childhood to adult age. PATIENTS Ten IGF-I treated children with LS (4 M, 6 F) and 24 untreated patients (10 M, 14 F) were studied. METHODS Measurements of palm length were made on available standardized hand X-rays from infancy to adult age. The measurements were compared to normal references and SD values were calculated for each measurement. The growth of the hand was compared to the concomitant height of the body. RESULTS Hand SDS in untreated patients with LS decreased with age, from a mean of -2.8 +/- 0.7 (age 1-3 years) to -7.3 +/- 0.8 (age 13-15 years) and to -9.0 +/- 3.9 (age 40-50 years). During 9 years of IGF-I treatment the hand size deficit SDS did not improve in contradistinction to the height SDS which decreased from -6.2 +/- 1.2 to -3.9 +/- 0.5. CONCLUSION Congenital IGF-I deficiency, as in Laron Syndrome, profoundly affects the size and growth of the hand as part of its growth retardation characteristics, resulting in acromicria.
Michael Schwarz - One of the best experts on this subject based on the ideXlab platform.
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CLINICAL STUDY Pituitary size in patients with Laron Syndrome (primary GH insensitivity)
2015Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:Objective: The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron Syndrome, leads to an increase in the size of the pituitary gland. Methods: Eleven patients (six females, five males) with Laron Syndrome underwent magnetic reso-nance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36–68 years and two children, a 4-year-old boy and a 9-year-old girl. The latter patient had been treated with IGF-I (150–180 mg/kg per day) since the age of 3 years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and com-pared with reference values for age and sex. Results: The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary–hypo-thalamic region were detected. Conclusion: Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary–hypothalamic region in Laron Syndrome is normal. European Journal of Endocrinology 148 339–34
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Laron Syndrome Abnormalities: Spinal Stenosis, Os Odontoideum, Degenerative Changes of the Atlanto-odontoid Joint, and
2015Co-Authors: Small Oropharynx, Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:hormone resistance. We investigated abnormalities in the upper airways and cervical spine in patients with Laron Syndrome. METHODS: We prospectively examined 11 patients (one child aged 9 years and 10 adults aged 36–68 years), 10 of whom underwent MR imaging of the spine or head; nine, radiography of the cervical spine; and four, CT of C1-C2. The width of the spinal canal was evaluated visually and quantitatively and compared with reference values. The smallest diameter of the orophar-ynx and the thickness of the palate were measured and compared with reference values. Nine age-matched female patients referred for MR imaging for unrelated reasons served as control subjects. RESULTS: Cervical spinal stenosis was present in seven of the adult patients, within a confidence interval of 95%. Anomaly of the dens compatible with os odontoideum was present in three patients, causing focal myelomalacia in two. The atlanto-odontoid joint showed osteoarthritic changes in six of the adult patients. The mediolateral diameter of the oropharynx was significantly smaller in the patients with Laron Syndrome than in the control subjects (P <.005). There was no difference in the thickness of the soft palate. CONCLUSION: Patients with Laron Syndrome develop significant narrowing of the cervica
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abnormalities of the axial and proximal appendicular skeleton in adults with Laron Syndrome growth hormone insensitivity
Skeletal Radiology, 2008Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Osnat Konen, Y Siegel, Israel Hershkovitz, Z. LaronAbstract:Objective To investigate abnormalities in the skeleton (with the exclusion of the skull, cervical spine, hands and feet) in patients with Laron Syndrome, who have an inborn growth hormone resistance and congenital insulin-like growth factor-1 (IGF-1) deficiency.
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Pituitary size in patients with Laron Syndrome (primary GH insensitivity).
European journal of endocrinology, 2003Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:Objective: The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron Syndrome, leads to an increase in the size of the pituitary gland. Methods: Eleven patients (six females, five males) with Laron Syndrome underwent magnetic resonance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36 ‐ 68 years and two children, a 4-year-old boy and a 9-year-old girl. The latter patient had been treated with IGF-I (150 ‐ 180 mg/kg per day) since the age of 3 years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and compared with reference values for age and sex. Results: The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary ‐ hypothalamic region were detected. Conclusion: Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary ‐ hypothalamic region in Laron Syndrome is normal.
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Craniofacial and brain abnormalities in Laron Syndrome (primary growth hormone insensitivity).
European journal of endocrinology, 2002Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:Objective: To investigate abnormalities in the craniofacial structures and in the brain in patients with Laron Syndrome. Design: Eleven patients with classical Laron Syndrome, nine untreated adults aged 36 ‐ 68 years and two children aged 4 and 9 years (the latter treated by IGF-I), were studied. Methods: Magnetic resonance images of the brain were obtained in all the patients. One patient also underwent computed tomography. The maximal diameter of the maxillary and frontal sinuses was measured and compared with reference values, the size of the sphenoid sinus was evaluated in relation to the sella, and the mastoids were evaluated qualitatively (small or normal). The brain was evaluated for congenital anomalies and parenchymal lesions. Results: In the adult untreated patients, the paranasal sinuses and mastoids were small; in six patients, the bone marrow in the base of the skull was not mature. The diploe of the calvaria was thin. On computed tomography in one adult patient, the sutures were still open. A minimal or mild degree of diffuse brain parenchymal loss was seen in ten patients. One patient demonstrated a lacunar infarct and another periventricular high signals on T2-weighted images. Two patients had cerebellar atrophy. Conclusions: The present study has demonstrated the important role IGF-I plays in the development of the brain and bony structures of the cranium. European Journal of Endocrinology 146 499‐503
Gadi Horev - One of the best experts on this subject based on the ideXlab platform.
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CLINICAL STUDY Pituitary size in patients with Laron Syndrome (primary GH insensitivity)
2015Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:Objective: The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron Syndrome, leads to an increase in the size of the pituitary gland. Methods: Eleven patients (six females, five males) with Laron Syndrome underwent magnetic reso-nance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36–68 years and two children, a 4-year-old boy and a 9-year-old girl. The latter patient had been treated with IGF-I (150–180 mg/kg per day) since the age of 3 years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and com-pared with reference values for age and sex. Results: The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary–hypo-thalamic region were detected. Conclusion: Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary–hypothalamic region in Laron Syndrome is normal. European Journal of Endocrinology 148 339–34
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Laron Syndrome Abnormalities: Spinal Stenosis, Os Odontoideum, Degenerative Changes of the Atlanto-odontoid Joint, and
2015Co-Authors: Small Oropharynx, Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:hormone resistance. We investigated abnormalities in the upper airways and cervical spine in patients with Laron Syndrome. METHODS: We prospectively examined 11 patients (one child aged 9 years and 10 adults aged 36–68 years), 10 of whom underwent MR imaging of the spine or head; nine, radiography of the cervical spine; and four, CT of C1-C2. The width of the spinal canal was evaluated visually and quantitatively and compared with reference values. The smallest diameter of the orophar-ynx and the thickness of the palate were measured and compared with reference values. Nine age-matched female patients referred for MR imaging for unrelated reasons served as control subjects. RESULTS: Cervical spinal stenosis was present in seven of the adult patients, within a confidence interval of 95%. Anomaly of the dens compatible with os odontoideum was present in three patients, causing focal myelomalacia in two. The atlanto-odontoid joint showed osteoarthritic changes in six of the adult patients. The mediolateral diameter of the oropharynx was significantly smaller in the patients with Laron Syndrome than in the control subjects (P <.005). There was no difference in the thickness of the soft palate. CONCLUSION: Patients with Laron Syndrome develop significant narrowing of the cervica
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CLINICAL STUDY Craniofacial and brain abnormalities in Laron Syndrome (primary growth hormone insensitivity)
2013Co-Authors: Liora Kornreich, Gadi Horev, Boaz Karmazyn, M Schwarz, Z. LaronAbstract:Objective: To investigate abnormalities in the craniofacial structures and in the brain in patients with Laron Syndrome. Design: Eleven patients with classical Laron Syndrome, nine untreated adults aged 36–68 years and two children aged 4 and 9 years (the latter treated by IGF-I), were studied. Methods: Magnetic resonance images of the brain were obtained in all the patients. One patient also underwent computed tomography. The maximal diameter of the maxillary and frontal sinuses was measured and compared with reference values, the size of the sphenoid sinus was evaluated in relation to the sella, and the mastoids were evaluated qualitatively (small or normal). The brain was evaluated for congenital anomalies and parenchymal lesions. Results: In the adult untreated patients, the paranasal sinuses and mastoids were small; in six patients, the bone marrow in the base of the skull was not mature. The diploe of the calvaria was thin. On computed tomography in one adult patient, the sutures were still open. A minimal or mild degree of diffuse brain parenchymal loss was seen in ten patients. One patient demonstrated a lacunar infarct and another periventricular high signals on T2-weighted images. Two patients had cerebellar atrophy. Conclusions: The present study has demonstrated the important role IGF-I plays in the development of the brain and bony structures of the cranium. European Journal of Endocrinology 146 499–50
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abnormalities of the axial and proximal appendicular skeleton in adults with Laron Syndrome growth hormone insensitivity
Skeletal Radiology, 2008Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Osnat Konen, Y Siegel, Israel Hershkovitz, Z. LaronAbstract:Objective To investigate abnormalities in the skeleton (with the exclusion of the skull, cervical spine, hands and feet) in patients with Laron Syndrome, who have an inborn growth hormone resistance and congenital insulin-like growth factor-1 (IGF-1) deficiency.
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Pituitary size in patients with Laron Syndrome (primary GH insensitivity).
European journal of endocrinology, 2003Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:Objective: The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron Syndrome, leads to an increase in the size of the pituitary gland. Methods: Eleven patients (six females, five males) with Laron Syndrome underwent magnetic resonance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36 ‐ 68 years and two children, a 4-year-old boy and a 9-year-old girl. The latter patient had been treated with IGF-I (150 ‐ 180 mg/kg per day) since the age of 3 years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and compared with reference values for age and sex. Results: The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary ‐ hypothalamic region were detected. Conclusion: Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary ‐ hypothalamic region in Laron Syndrome is normal.
Boaz Karmazyn - One of the best experts on this subject based on the ideXlab platform.
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CLINICAL STUDY Pituitary size in patients with Laron Syndrome (primary GH insensitivity)
2015Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:Objective: The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron Syndrome, leads to an increase in the size of the pituitary gland. Methods: Eleven patients (six females, five males) with Laron Syndrome underwent magnetic reso-nance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36–68 years and two children, a 4-year-old boy and a 9-year-old girl. The latter patient had been treated with IGF-I (150–180 mg/kg per day) since the age of 3 years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and com-pared with reference values for age and sex. Results: The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary–hypo-thalamic region were detected. Conclusion: Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary–hypothalamic region in Laron Syndrome is normal. European Journal of Endocrinology 148 339–34
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Laron Syndrome Abnormalities: Spinal Stenosis, Os Odontoideum, Degenerative Changes of the Atlanto-odontoid Joint, and
2015Co-Authors: Small Oropharynx, Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:hormone resistance. We investigated abnormalities in the upper airways and cervical spine in patients with Laron Syndrome. METHODS: We prospectively examined 11 patients (one child aged 9 years and 10 adults aged 36–68 years), 10 of whom underwent MR imaging of the spine or head; nine, radiography of the cervical spine; and four, CT of C1-C2. The width of the spinal canal was evaluated visually and quantitatively and compared with reference values. The smallest diameter of the orophar-ynx and the thickness of the palate were measured and compared with reference values. Nine age-matched female patients referred for MR imaging for unrelated reasons served as control subjects. RESULTS: Cervical spinal stenosis was present in seven of the adult patients, within a confidence interval of 95%. Anomaly of the dens compatible with os odontoideum was present in three patients, causing focal myelomalacia in two. The atlanto-odontoid joint showed osteoarthritic changes in six of the adult patients. The mediolateral diameter of the oropharynx was significantly smaller in the patients with Laron Syndrome than in the control subjects (P <.005). There was no difference in the thickness of the soft palate. CONCLUSION: Patients with Laron Syndrome develop significant narrowing of the cervica
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CLINICAL STUDY Craniofacial and brain abnormalities in Laron Syndrome (primary growth hormone insensitivity)
2013Co-Authors: Liora Kornreich, Gadi Horev, Boaz Karmazyn, M Schwarz, Z. LaronAbstract:Objective: To investigate abnormalities in the craniofacial structures and in the brain in patients with Laron Syndrome. Design: Eleven patients with classical Laron Syndrome, nine untreated adults aged 36–68 years and two children aged 4 and 9 years (the latter treated by IGF-I), were studied. Methods: Magnetic resonance images of the brain were obtained in all the patients. One patient also underwent computed tomography. The maximal diameter of the maxillary and frontal sinuses was measured and compared with reference values, the size of the sphenoid sinus was evaluated in relation to the sella, and the mastoids were evaluated qualitatively (small or normal). The brain was evaluated for congenital anomalies and parenchymal lesions. Results: In the adult untreated patients, the paranasal sinuses and mastoids were small; in six patients, the bone marrow in the base of the skull was not mature. The diploe of the calvaria was thin. On computed tomography in one adult patient, the sutures were still open. A minimal or mild degree of diffuse brain parenchymal loss was seen in ten patients. One patient demonstrated a lacunar infarct and another periventricular high signals on T2-weighted images. Two patients had cerebellar atrophy. Conclusions: The present study has demonstrated the important role IGF-I plays in the development of the brain and bony structures of the cranium. European Journal of Endocrinology 146 499–50
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Pituitary size in patients with Laron Syndrome (primary GH insensitivity).
European journal of endocrinology, 2003Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:Objective: The purpose of the present study was to investigate whether lifelong secretion of high levels of GH, characteristic of Laron Syndrome, leads to an increase in the size of the pituitary gland. Methods: Eleven patients (six females, five males) with Laron Syndrome underwent magnetic resonance imaging of the pituitary region with a system operating at 0.5 T. There were nine adults aged 36 ‐ 68 years and two children, a 4-year-old boy and a 9-year-old girl. The latter patient had been treated with IGF-I (150 ‐ 180 mg/kg per day) since the age of 3 years; all the other patients were untreated. The height of the adenohypophysis was measured on the sagittal images and compared with reference values for age and sex. Results: The height of the adenohypophysis was within the normal range for age and gender in all patients, except for one male, who had a small gland. No congenital anomalies of the pituitary ‐ hypothalamic region were detected. Conclusion: Despite the lifelong high levels of GH, no pituitary hypertrophy was detected. The anatomy of the pituitary ‐ hypothalamic region in Laron Syndrome is normal.
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Craniofacial and brain abnormalities in Laron Syndrome (primary growth hormone insensitivity).
European journal of endocrinology, 2002Co-Authors: Liora Kornreich, Gadi Horev, Michael Schwarz, Boaz Karmazyn, Zvi LaronAbstract:Objective: To investigate abnormalities in the craniofacial structures and in the brain in patients with Laron Syndrome. Design: Eleven patients with classical Laron Syndrome, nine untreated adults aged 36 ‐ 68 years and two children aged 4 and 9 years (the latter treated by IGF-I), were studied. Methods: Magnetic resonance images of the brain were obtained in all the patients. One patient also underwent computed tomography. The maximal diameter of the maxillary and frontal sinuses was measured and compared with reference values, the size of the sphenoid sinus was evaluated in relation to the sella, and the mastoids were evaluated qualitatively (small or normal). The brain was evaluated for congenital anomalies and parenchymal lesions. Results: In the adult untreated patients, the paranasal sinuses and mastoids were small; in six patients, the bone marrow in the base of the skull was not mature. The diploe of the calvaria was thin. On computed tomography in one adult patient, the sutures were still open. A minimal or mild degree of diffuse brain parenchymal loss was seen in ten patients. One patient demonstrated a lacunar infarct and another periventricular high signals on T2-weighted images. Two patients had cerebellar atrophy. Conclusions: The present study has demonstrated the important role IGF-I plays in the development of the brain and bony structures of the cranium. European Journal of Endocrinology 146 499‐503
