Caustic Ingestion in Children: 1 Year Experience in 3 Italian Referral Centers.

OBJECTIVES: Despite the efforts to reduce the exposure to corrosive household products, caustic ingestion in children is currently a significant medical problem. The aims of the present study were to evaluate the clinical consequences of caustic ingestion and to identify prognostic factors that could concur in driving both diagnostic and therapeutic management. METHODS: All consecutive children referred for ingestion of a caustic substance from June 2017 to June 2018 were enrolled. Medical records, laboratory and endoscopic findings were reviewed and analyzed. RESULTS: We enrolled 44 children with caustic ingestion. Alkaline agents were ingested by 26 of 44 (59.1%) patients, whereas acid agents were ingested by 18 of 44 patients (40.9%). Alkaline rather than acid agents were associated with a worse endoscopic score (r: 0.45) and a higher probability of early esophageal stricture occurrence (r: 0.38). The specific risk of the presence of severe esophageal lesions rose progressively with increasing number of symptoms whereas no esophageal injury was found in asymptomatic patients. CONCLUSIONS: Our data suggest that endoscopic evaluation is mandatory in symptomatic patients to direct therapeutic management, but it could be avoided in asymptomatic patients after accidental ingestion, particularly if the ingestion is only suspected and patients have no oropharyngeal burns.

Magnetic resonance enterography, small-intestine contrast US, and capsule endoscopy to evaluate the small bowel in pediatric Crohn's disease: a prospective, blinded, comparison study.

BACKGROUND: Small-bowel (SB) disease is a severe clinical entity among the phenotypes of Crohn's disease (CD). OBJECTIVE: To assess sensitivity, specificity, and accuracy of magnetic resonance enterography (MRE), small-intestine contrast US (SICUS), and capsule endoscopy (CE) in the diagnosis of pediatric SB-CD. DESIGN: Prospective, blinded, comparison study. SETTING: Tertiary center for pediatric inflammatory bowel disease. PATIENTS: Children with known or suspected CD. Diagnosis of SB obstruction at SICUS or MRE excluded patients from the study. INTERVENTION: Patients underwent ileocolonoscopy, MRE, SICUS, and CE over a 7-day period. For the imaging evaluation, SB was divided into 3 segments: jejunum, proximal and mid ileum, and terminal ileum. MAIN OUTCOME MEASUREMENTS: The performance of each method was compared to a consensus reference standard for upper SB and to ileocolonoscopy for the terminal ileum. RESULTS: Twenty-five patients completed the study. In the jejunum, the sensitivity of SICUS and CE was 92%, which was not significantly higher than MRE (75%); the specificity of CE (61%) was significantly lower than that of MRE (P = .04). In the proximal and mid ileum, MRE and CE did not have significantly higher sensitivity (100%) than SICUS (80%), but CE was less specific (P > .05). At the terminal ileum, SICUS and MRE were slightly more sensitive than CE (94% vs. 81%); however, the latter was more specific. LIMITATIONS: Use of the consensus reference standard for upper SB. Small number of patients. CONCLUSION: SICUS, MRE, and CE are all effective options for imaging SB. An integrated use of different tools should be suggested to achieve a complete assessment of the SB in children with suspected or confirmed CD.

Assessment of intrafamilial clinical variability of poikiloderma with neutropenia by a 10-year follow-up of three affected siblings.

Clericuzio-type poikiloderma with neutropenia is a well-defined nosological entity, but despite a remarkable number of clinical reports, no long term follow-up data has been presented to date regarding patients with this rare condition. Here we describe the results of clinical follow-up of three siblings, one male (Patient 1) and two females (Patients 2 and 3), subsequent to their first clinical and then molecular diagnosis of Clericuzio-type poikiloderma with neutropenia syndrome due to mutation of USB1gene. Patient 1 always expressed the most severe phenotype, while patients 2 and 3 showed an intermediate and mild phenotype, respectively, as observed since their first clinical evaluation. None of the patients developed skin cancer and/or myelodysplastic disorders considering the peripheral haematological findings. Lens opacity, never reported before, was found in two of the three patients. The long term follow-up observations confirm the stability over time of the pronounced intra-familial heterogeneity of clinical manifestations observed prior to and upon molecular diagnosis. We conclude that prolonged follow-up is an adjunct tool to monitor intra-familial variability of PN clinical spectrum which may favour surveillance of more serious complications of the disease among siblings, when a patient-specific clinical expressivity is present.

Prediction of Transient or Permanent Congenital Hypothyroidism from Initial Thyroid Stimulating Hormone Levels.

OBJECTIVE: To identify factors that discriminate between transient and permanent congenital hypothyroidism. METHODS: Retrospective evaluation of 58 children with congenital hypothyroidism and eutopic thyroid gland. Gender, gestational age, birth weight, TSH and serum thyroxine levels at diagnosis and L-thyroxine dose at 12 and 24 months of age were analyzed. RESULTS: Median (IQR) initial TSH levels were 73.3 (276.5) µIU/mL in permanent hypothyroidism and 24.24 (52.7) µU/mL in transient hypothyroidism (P =0.0132). The optimum cut-off value of initial TSH to predict transient hypothyroidism was 90 µIU/mL. Mean (SD) L-thyroxine doses at 24 months of age were 2.64 (0.98) µg/kg/day in permanent hypothyroidism and 1.91 (0.65) µg/kg/day in transient hypothyroidism. Requirement of L-thyroxine dose at 24 months of ≤0.94 µg/kg/day had the highest sensitivity (100%) to predict transient hypothyroidism. CONCLUSIONS: L-thyroxine doses at 24 months can predict transient hypothyroidism in patients with eutopic thyroid gland earlier than at 36 months.