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עמוד בית
Mon, 26.02.24

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August 2019
Tamar Laron-Kenet MD, Aviva Silbergeld MSc, Pearl Lilos BSc and Zvi Laron MD PhD (hc)
March 2019
Efrat Ben-Nun Yaari BSc, Rivka Kauli MD, Pearl Lilos MA and Zvi Laron MD PhD

Background: Treatment of patients with childhood growth hormone deficiency is usually terminated at the end of puberty. Follow-up into adult age is rare, even more so in patients with congenital isolated growth hormone deficiency (cIGHD).

Objectives: To assess the clinical and social characteristics of adults with cIGHD who received growth hormone (hGH) treatment in childhood.

Methods: Thirty-nine patients (23 men, 16 women) diagnosed in our clinic with cIGHD at 7 ± 4.2 years, and treated with hGH during childhood for 2–18 years, were followed into adulthood (mean age 30.7 ± 13.3 years). Ascertained detailed data were found for 32 patients.

Results: Mean ± SD height for males was 160.2 ± 10.6 cm and for females 146.4 ± 5.4 cm. All patients achieved full sexual development and 14 were married. After cessation of GH treatment and with advanced age all exhibited a progressive increase in adiposity to the degree of obesity. Twelve patients suffered from hyperlipidemia, 4 developed diabetes mellitus, and 5 have cardiovascular diseases. One patient died in an accident. None developed cancer. Of the 39 patients, 22 have an education level of high school or higher, and 2 are in special institutions. Most are employed in manual labor.

Conclusions: Patients with congenital IGHD who do not receive early and regular replacement treatment are prone to lag in achieving normal height and suffer from educational and vocational handicaps.

May 2017
Alon Farfel MD, Rona Rabinowicz MD, Gadi Abebe-Campino MD, Estela Derazne MsC, Tami Laron-Kenet MD and Zvi Laron MD
January 2017
Haim Werner PhD, Lena Lapkina-Gendler PhD and Zvi Laron MD
February 2012
A. Farfel, E. Derazne, D. Tzur, N. Linder and Z. Laron

Background: Measurements of adolescents who at birth were large (long and/or heavy) for gestational age are scant.

Objectives: To determine the correlation between birth length and weight in female and male neonates born long and/or overweight for gestational age, with their height and weight at age 17.

Methods: We reviewed the records of the Rabin Medical Center for birth data of 96 full-term neonates born long and overweight for gestational age (FT-lo,ow), 33 full-term neonates born long but with normal weight for gestational age (FT-lo,nw), 148 full-term neonates born overweight but with normal length for gestational age (FT-nl,ow), and 401 full-term neonates born with normal birth length and weight (FT- nl,nw).

Results: Neonates of both genders born long and overweight at birth (FT-lo,ow) were taller and heavier at age 17 years than those born FT-nl,nw: females: 167.8 ± 5.1 cm and 64.6 ± 10.3 kg vs. 162.6 ± 5.5 cm and 59.3 ± 11.1 kg (P < 0.001 for height and P = 0.026 for weight) and males: 182.4 ± 8.1 cm and 80.6 ± 20.4 kg vs. 174.5 ± 6.2 cm and 67.4 ± 12.3 kg (P < 0.001). The correlations between birth length and height at age 17 for both genders were statistically significant (P < 0.001), as were those between birth weight and the weight and body mass index (BMI) at age 17 for both genders (P < 0.001). There was no correlation between birth length and weight or BMI at age 17.

Conclusions: Full-term neonates of both genders born large for gestational age become tall adolescents and weigh more at age 17 than children with a normal birth length and weight.

July 2008
Z. Laron

The question of who discovered insulin is controversial. One of the scientists working on pancreas extracts was Nicolae Paulescu, the so-called Forgotten Man. In addition to his scientific research he was also active in politics. He was the father of the virulent antisemitic fascist movement “Garda de Fer” in Romania; he raved against the “Jewish Peril,” claimed in his writings that the Jews are a genetically degenerate people trying to cheat and poison the Rumanian people by alcoholism, and more. His name came up in 2003 when Romanian diabetologists initiated a move to honor him. But voices rose in protest, claiming that persons who incite hatred, support persecution and genocide and distort science as their political tools cannot be accepted or rewarded. The protesters won.

June 2005
Z. Laron, H. Lewy, I. Wilderman, A. Casu, J. Willis, M.J. Redondo, I. Libman, N. White and M. Craig
 Background: Type 1 childhood-onset diabetes mellitus has a multifactorial origin involving an interplay between genetic and environmental factors. We have previously shown that many children who subsequently develop T1DM[1] have a different seasonality of birth than the total live births of the same population, supporting the hypothesis that perinatal viral infection during the yearly epidemics are a major trigger for the autoimmune process of T1DM.

Objectives: To compare the seasonality of children with T1DM in different populations around the world for which data were available.

Methods: We analyzed large cohorts of T1DM patients with a clinical disease onset before age 14 or 18 years.

Results: We found a seasonality pattern only in ethnically homogenous populations (such as Ashkenazi Jews, Israeli Arabs, individuals in Sardinia and Canterbury, New Zealand, and Afro-Americans) but not in heterogeneous populations (such as in Sydney, Pittsburgh and Denver).

Conclusions: Our findings attempt to explain the controversial data in the literature by showing that ethnically heterogeneous populations with a mixture of patients with various genetic backgrounds and environmental exposures mask the different seasonality pattern of month of birth that many children with diabetes present when compared to the general population.


 





[1] T1DM = type 1 childhood-onset diabetes mellitus


October 2004
O. Shevah, M. Rubinstein and Z. Laron

Background: Laron Syndrome, first described in Israel, is a form of dwarfism similar to isolated growth hormone deficiency caused by molecular defects in the GH[1] receptor gene.

Objective: To characterize the molecular defects of the GH-R[2] in Laron syndrome patients followed in our clinic.

Methods: Of the 63 patients in the cohort, we investigated 31 patients and 32 relatives belonging to several ethnic origins. Molecular analysis of the GH-R gene was performed using the single strand conformation polymorphism and DNA sequencing techniques.

Results: Eleven molecular defects including a novel mutation were found. Twenty-two patients carried mutations in the extracellular domain, one in the transmembrane domain, and 3 siblings with typical Laron syndrome presented a normal GH-R. Of interest are, on one hand, different mutations within the same ethnic groups: W-15X and 5, 6 exon deletion in Jewish-Iraqis, and E180 splice and 5, 6 exon deletion in Jewish-Moroccans; and on the other hand, identical findings in patients from distinct regions: the 785-1 G to T mutation in an Israeli-Druze and a Peruvian patient. A polymorphism in exon 6, Gly168Gly, was found in 15 probands. One typical Laron patient from Greece was heterozygous for R43X in exon 4 and heterozygous for Gly168Gly. In addition, a novel mutation in exon 5: substitution of T to G replacing tyrosine 86 for aspartic acid (Y86D) is described.

Conclusions: This study demonstrates: a) an increased focal incidence of Laron syndrome in different ethnic groups from our area with a high incidence of consanguinity; and b) a relationship between molecular defects of the GH-R, ethnic group and geographic area.






[1] GH = growth hormone

[2] GH-R = growth hormone receptor


February 2000
Rivka Kauli MD, Rina Zaizov MD, Liora Lazar MD, Athalia Pertzelan MD, Zvi Laron MD, Avinoam Galatzer MA, Moshe Phillip MD, Yitzhak Yaniv MD and Ian Joseph Cohen MB ChB

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.

____________________________________

 

ERT = enzyme replacement therapy

SDS = standard deviation score

September 1999
Ben Zion Garty, MD, Itzhak Levy, MD, and Zvi Laron, MD.
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