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Printable Handouts
Navigable Slide Index
- Introduction
- 1958: stature of three siblings
- LS appearance
- High hGH levels
- A new syndrome involving hGH
- Two possibilities
- Growth hormone resistance
- Liver biopsy in two LS patients
- Studying the GH-R gene in LS
- GH-receptor mRNA & protein
- Exon deletions
- Molecular analysis: exon 6
- GH-R gene defects
- LS patients
- Definition of LS (OMIM 262500)
- LS: The Israeli Cohort (1)
- Genetic aspects
- Gestation & delivery
- Features of LS
- Blue sclerae & tooth decay
- Audiogram examination
- Height deficit
- Upper / lower segment ratio
- Sexual maturation, puberty & reproduction
- Delayed puberty
- Sexual development
- LS: The Israeli Cohort (2)
- No difficulties with reproduction
- Nutritional state
- Serum cholesterol & fatty liver
- Obesity & insulin resistance
- Serum adiponectin levels are elevated
- Skeletal system
- Neuro-psychological development: CNS
- Spinal stenosis
- DEXA
- Blood chemistry
- LS & eye structure
- Cardio-pulmonary system
- LS: The Israeli Cohort (3)
- Intellectual capacity (IQ)
- LS: The Israeli Cohort (4)
- LS: The Israeli Cohort (5)
- Treatment
- Intravenous IGF-I administration
- Acute IGF-I effects
- IGF-I & acceleration of linear growth
- IGF-I treatment & head circumference
- IGF-I treatment & erythropoiesis
- Adverse effects during IGF-I administration
- LS: The Israeli Cohort (6)
- Results: A
- Results: B
- Results: C
- Immortalized lymphocytes
- Gene chip analysis
- Validation data
- Summary
- The LS clock
- LS & ancient humans
- Hypothesis of the origin of LS
- Italy, 1454
- Our team & patients
- Acknowledgements
- The Laron mouse
- Thank you!
Topics Covered
- Laron syndrome (LS) appearance and symptomology
- Laron syndrome and hGH levels
- Dwarfism
- Laron syndrome and GH insensitivity
- Laron syndrome and GH-R
- Laron syndrome and IGF-I
- IGF-I treatment
- Laron Syndrome: The Israeli Cohort
- Genetics of Laron syndrome
- Features of Laron syndrome
- Sexual maturation, puberty & reproduction
Links
Categories:
Therapeutic Areas:
Talk Citation
Laron, Z. (2023, April 30). Laron syndrome (OMIM#262500): the Israeli cohort 60 years’ experience [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved November 14, 2024, from https://doi.org/10.69645/WHBW4271.Export Citation (RIS)
Publication History
Financial Disclosures
- Prof. Zvi Laron has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.
A selection of talks on Cardiovascular & Metabolic
Transcript
Please wait while the transcript is being prepared...
0:00
In this lecture, I will tell
you about the discovery,
the etiology, and
the critical aspects
of what is called
Laron Syndrome (LS).
My experience is about 60 years,
as I will show you.
0:22
In 1958, at a time when
no assay from human growth
hormone was available,
three siblings, aged three,
one and a newborn baby,
were referred to me.
Two of those are shown
on the left panel.
They belong to a consanguineous
Jewish family of
the Yemenite origin,
seen in the right panel.
Five older siblings
were of normal stature.
0:54
Their appearance was dwarfism,
obesity, acromicria,
hypogenitalism, and
severe hypoglycemia,
which resembled children with
pituitary isolated growth
hormone deficiency.
When comparing the gender, one
who is LS on the left
side, with a same aged
boy with isolated growth
hormone deficiency,
we see their great resemblance.
However, they are smaller
than the patient with
growth hormone deficiency,
denoting already the lack of
efficiency of this hormone.
1:37
In the early 1960s, when
radioimmunoassays for growth
hormone became available,
we found to our
great surprise that
the circulating hGH was very
high reaching acromegalic
levels, as seen in the left,
up to 140 ng/ml.
On the right, we see
the night secretion of
growth hormone with
very high peaks in
the LS patients as compared to
a normal control of the same
age, in the lower side.
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