Successful Intravenous Immunoglobulin Treatment in Pediatric Severe DRESS Syndrome.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening delayed drug-induced hypersensitivity reaction. The most frequently reported drugs causing DRESS are aromatic antiepileptic agents. Prompt withdrawal of the offending drug and administering systemic corticosteroids is the most widely accepted and used treatment. The treatment of severe DRESS not responsive to systemic corticosteroids is uncertain. OBJECTIVE: The objective of this study was to describe a case series of pediatric patients with DRESS who were treated successfully with intravenous immunoglobulins (IVIGs). METHODS: A retrospective review of all children hospitalized in a tertiary care children's hospital with severe DRESS syndrome who received IVIG in addition to offending drug withdrawal and systemic corticosteroids during 1999-2017 is performed. RESULTS: Seven severe DRESS patients (4 males, age: 9.5 ± 5.7 years) are described. The offending drugs were antiepileptics in all but one case. Clinical findings included fever, rash, lymphadenopathy, dyspnea, anasarca, and hepatic involvement. After IVIG treatment (total dosage: 1-2 g/kg), fever resolved within a median time of 1 (range, 0-5) day, rash disappeared after 6.3 ± 1.6 days, and liver enzymes substantially improved after 3.8 ± 1.6 days. Patients were discharged 6.1 ± 2.7 days after IVIG commencement. There was no mortality. CONCLUSION: The addition of IVIG in DRESS syndrome resistant to regular drug withdrawal and systemic corticosteroid therapy may hasten disease recovery.

"Real-world" pediatric endocrine practice; how much is it influenced by physician's gender and region of practice. Results of an international survey.

OBJECTIVE: To determine whether hormonal treatments for frequent clinical cases (short stature, delayed and precocious puberty) are prescribed strictly according to clinical guidelines or based on personal tendencies, and whether the decisions correlate with physician's personal demographics (age, sex, and place of practice). METHODS: Cross-sectional survey, with made-up clinical cases, distributed to pediatric endocrinologists using 2 web-based professional forums, Israeli and an international. The questionnaire included 8 clinical cases and 5 demographic questions regarding the physician. Differences in practice between Israeli and international endocrinologists were assessed, and correlation between the physician's gender and their decisions regarding treatment. RESULTS: One hundred fifty-five physicians responded, 28% Israeli and 72% international. In girls with early puberty, 60% of international and 26% of Israeli physicians chose not to treat with a gonadotropin-releasing hormone agonist. In girls with short stature, 79% of Israeli and 34% of international physicians offered growth hormone treatment. In girls with early puberty, both male and female physicians responded similarly in the international group, but in the Israeli group 47% of male and 15% of female doctors would not treat. In girls with constitutional growth delay, 67% of Israeli male doctors would not treat with growth hormone compared to 30% of Israeli female physicians. CONCLUSIONS: Our study demonstrated significant practice differences between Israeli and international pediatric endocrinologists. Within the Israeli group, significant practice differences were seen between male and female physicians. Given that Israeli physicians follow the same clinical guidelines it is clear that a large "grey zone" of clinical cases exist and much of the decisions on treatment are personal and influenced by personal beliefs or gender.

Growth, development, puberty and adult height before and during treatment in children with congenital isolated growth hormone deficiency.

OBJECTIVE: To describe the growth, development and puberty in children with congenital IGHD before and during hGH treatment. SUBJECTS: Patients with cIGHD treated by hGH between the years 1958-1992. SETTING: All patients were diagnosed, treated and followed in our clinic. PARTICIPANTS: Data were found in 37/41 patients (21 m, 16 f). 34 had hGH-1A deletions, 7 GHRH-R mutations. Patients, referred after age 25, were excluded. RESULTS: The birth length of 10/37 neonates was 48.29±2.26 (44-50) cm. Birth weight of 28/37 neonates was 3380±370 g (m), 3230±409 g (f). Neuromotor milestones were variable. Age at referral was 5.7±4.2 y (m) and 5.6±3.8 y (f). Initiation of hGH treatment (35µg/kg/d) was 7.5±4.8, (0.8-15.08) y (m) and 6.8±4.36 (0.8-16.5) y (f). Height SDS increased from -4.3 to -1.8 (m) and from -4.5 to -2.6 (f). Head circumference increased from -2.6 to -1.3 (m) and from -2.7 to -2.3 (f). BMI increased from 15.8 to 20.6 (m) and from 15.5 to 20.4 (f). There was a negative correlation between age of hGH initiation and change in height SDS (r=-0.66; ρ<0.01), same for bone age (r=-0.69; ρ<0.01). Upper/lower body ratio decreased from 2.5±2.1 (m±SD) to 1.08±0.1 (ρ<0.0005). Puberty was delayed in boys, less so in girls. Mean age of 1st ejaculation (14 m) was 17.6±2.2 y and of menarche (14 f. was 13.7±1.2 y. In both genders there was a positive correlation between age at start of hGH and age at onset of puberty (r=0.57; ρ<0.01). All reached full sexual development but the penile and testicular sizes were below normal. There was a positive correlation between length of hGH treatment and final testicular volume (r=0.597, ρ=0.05) and a negative correlation between the age at initiation of hGH treatment and final testicular volume(r=-0.523, ρ=0.018). All were obese and hGH treatment increased the adiposity progressively (r=0.418, ρ=0.013). CONCLUSION: Early diagnosis and treatment of cIGHD enables normal or near normal growth, development and puberty.