Multicentre observational study on multisystem inflammatory syndrome related to COVID-19 in Argentina.

Background: The impact of the pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) in low- and middle-income countries remains poorly understood. Our aim was to understand the characteristics and outcomes of PIMS-TS in Argentina.

Methods: This observational, prospective, and retrospective multicenter study enrolled patients younger than 18 years-old manifesting PIMS-TS, Kawasaki disease (KD) or Kawasaki shock syndrome (KSS) between March 2020 and May 2021.

Growth hormone receptor deficiency (Laron syndrome) in black African siblings.

Non-Caucasians with growth hormone receptor (GHR) deficiency/Laron syndrome among the approximately 180 recognised cases are rare, and include a Japanese and 3 African Americans. Black African siblings, a brother and a sister seen initially at 11 years 9 months and 5 years 6 months of age respectively were -7,4 and -8,0 on the standard deviation score for height. They had characteristic features and biochemical findings including prominent forehead; depressed nasal bridge; central adiposity; high-pitched voices; micropenis; high GH levels and low levels of insulin-like growth factor (IGF)-I, IGF-II, insulin-like growth factor-binding protein 3 (IGFBP-3), and GH-binding protein (the solubilised extracellular domain of the GH cell surface receptor).

Effects of insulin-like growth factor I treatment on the molecular distribution of insulin-like growth factors among different binding proteins.

The molecular distribution of insulin-like growth factor I (IGF-I) and IGF-II among the IGF binding proteins (IGFBPs) was studied before and during IGF-I therapy in Ecuadorean adults with growth hormone receptor deficiency (GHRD). Of the total circulating IGF-I and IGF-II, 70% was carried by the 150 kDa complex in normal subjects, while in patients with GHRD, 50% of serum IGF-I, but only 30-35% of serum IGF-II, was measured within the 150 kDa IGFBP-3 region. Administration of IGF-I altered the concentration of IGF-I and IGF-II, although the percentage of total IGF measured within each IGFBP region was not affected, as the increase in IGF-I and the decrease in IGF-II were proportional.

The composition and distribution of insulin-like growth factors (IGFs) and IGF-binding proteins (IGFBPs) in the serum of growth hormone receptor-deficient patients: effects of IGF-I therapy on IGFBP-3.

We have previously reported that adult GH receptor-deficient (GHRD) patients treated subcutaneously with recombinant human insulin-like growth factor (IGF)-I have increased serum IGF-I levels and decreased IGF-II levels, whereas IGF-binding protein-3 (IGFBP-3) levels were unchanged. To further investigate the effects of IGF-I administration upon the IGF-IGFBP axis in GHRD, we have examined: 1) the molecular distribution of IGF-I and IGF-II among the IGFBPs; 2) the composition and distribution of the IGFBPs, in particular IGFBP-3; and 3) the acid labile subunit (ALS). Serum samples from adult GHRD patients who were treated sc with recombinant human IGF-I (40 micrograms/kg, sc, twice a day) or from normal Ecuadorian adults were incubated with [125I]IGF-II and subjected to neutral size-exclusion chromatography.

Pharmacokinetics of recombinant human insulin-like growth factor I given subcutaneously to healthy volunteers and to patients with growth hormone receptor deficiency.

The pharmacokinetics of recombinant human insulin-like growth factor I (rhIGF-I) were studied in healthy volunteers and in patients with growth hormone receptor deficiency (GHRD; Laron syndrome). Following single subcutaneous injections of rhIGF-I, 40 and 80 micrograms/kg, to healthy volunteers, the peptide was absorbed slowly, with a maximum concentration reached after about 7 hours. Following daily multiple subcutaneous injections of rhIGF-I, 40 micrograms/kg, trough concentrations of IGF-I were increased by 277 +/- 50 micrograms/l (mean +/- SD) from baseline.

Hormonal and metabolic effects and pharmacokinetics of recombinant insulin-like growth factor-I in growth hormone receptor deficiency/Laron syndrome.

Profound growth failure despite elevated GH levels in GH receptor deficiency (GHRD) results from reduced insulin-like growth factor-I (IGF-I) synthesis. Recent reports of improved growth velocity in children with GHRD during IGF-I therapy indicate growth-promoting potential in humans. We evaluated the pharmacokinetics and metabolic/hormonal effects of recombinant human IGF-I (40 micrograms/kg every 12 h) given sc for 7 days to six adults with GHRD.

Serum profiles of insulin-like growth factors and their binding proteins in adults with growth hormone receptor deficiency treated with insulin-like growth factor I.

Six adult patients with growth hormone receptor deficiency (GHRD) (2 men, 4 women) with an identical defect in the growth hormone receptor (GHR) gene, were treated with recombinant human insulin-like growth factor I (IGF-I), 40 micrograms/kg s.c. twice daily, for 7 days.

Growth hormone receptor deficiency (Laron syndrome): clinical and genetic characteristics.

Approximately 60 cases of GHRD (Laron syndrome) were reported before 1990 and half of these were from Israel. We have described 47 additional patients from an inbred population of South Ecuador and have emphasized certain clinical features including: markedly advanced osseous maturation for height age; normal body proportions in childhood but child-like proportions in adults; much greater deviation of stature than head size, giving an appearance of large cranium and small facies; underweight in childhood despite the appearance of obesity and true obesity in adulthood; blue scleras; and limited elbow extension. The Ecuadorean patients differed markedly and most importantly from the other large concentration, in Israel, by being of normal or superior intelligence, suggesting a unique linkage in the Ecuadorean population.

Severe hemolytic uremic syndrome associated with rhabdomyolysis and insulin-dependent diabetes mellitus.

Hemolytic uremic syndrome has been associated with insulin-dependent diabetes mellitus eight times in the past, while the hemolytic uremic syndrome and rhabdomyolysis association has been described once. The occurrence of both conditions in a patient with the hemolytic uremic syndrome has not been reported. In this report, we described a 28-month-old girl who presented with severe hemolytic uremic syndrome muscle weakness and elevated muscle enzymes.