Residual Lesions After Pharmacological and Dye-Laser Treatment of Infantile Hemangiomas: Critical Review of 432 Cases.

BACKGROUND AND OBJECTIVES: Infantile hemangiomas (IHs) are the most common benign tumors in infanthood. Although they are often self-limiting, management of IHs is still controversial because residual lesions may persist in some cases. The aim of this study is to report our experience with patients affected with IH and investigate the frequency of residual lesions in treated versus untreated patients. STUDY DESIGN/MATERIALS AND METHODS: This retrospective observational study enrolled patients with IHs evaluated over the past 10 years. Patients were managed with systemic or local pharmacotherapy, laser therapy, a combination of them, or with observation only. RESULTS: A total of 432 patients were included: 71% received one or more therapies for IHs; 75.2% of untreated patients had at least one residual lesion compared with 41.4% of treated patients (P < 0.001). Patients treated with laser therapy or topical timolol had the lowest rate of residual lesions. CONCLUSIONS: This rather large case series suggests that IHs management with pharmacotherapy and especially laser therapy is associated with a lower number of residual lesions than observation only. Although propranolol can be very useful to avoid life-threatening complications and severe tissue impairment, laser therapy and topical timolol are potential effective treatments to decrease the incidence of residual lesions, mostly associated with superficial IHs. Lasers Surg. Med. © 2019 Wiley Periodicals, Inc.

Pain management in children has significantly improved in the Italian emergency departments.

AIM: Pain in children is often poorly assessed and treated in Italian emergency departments (EDs) as found in a survey conducted among the centres of the "Pain in Pediatric Emergency Room (PIPER)" Study Group in 2010. Our aim was to evaluate the changes in pain management in Italian EDs in the last years. METHOD: A structured questionnaire about pain assessment, protocols, use of local anaesthetics before venipuncture, opioids and adjuvants was mailed to 46 Italian EDs between November 2017 and April 2018. RESULTS: Pain was always assessed at triage in 34 centres (73.9%). Algometric scales were used in over 90% of EDs. Triage protocols were available in 37 centres (80.4%). Local anaesthetics before venipuncture were always used in six centres (13.0%). Protocols included opioids and adjuvants in 73.0% and 47.2%, respectively. Triage pain assessment was always done in 91.0% of the centres joining the PIPER Study Group up to 2015 and 56.5% in ones that joined the Group after 2015 (P = .017). Local anaesthetics before venipuncture were given in 39.1% of the centres joining until up to 2015 and 13.0% in ones that joined the Group after 2015 (P = .003). CONCLUSION: Paediatric pain management has significantly improved in Italian EDs in the last 8 years. The centres joining the PIPER Study Group for longer time have shown better results for the indicators considered in the survey.

Systematic review finds that fever phobia is a worldwide issue among caregivers and healthcare providers.

AIM: Fever phobia describes exaggerated concerns about the consequences of childhood fever and broader awareness is needed in everyday clinical practice. We investigated the factors associated with fever phobia in caregivers and healthcare providers and the geographical distribution of the issue. METHODS: The National Library of Medicine, Excerpta Medica and Google Scholar databases were searched. RESULTS: We retrieved 76 papers, published in English from 1985 to 2018, which covered wide areas of Asia, Europe, America, Africa and Australia. The occurrence of fever phobia was confirmed in 65 papers covering 26 521 caregivers. A number of factors were significantly associated with fever phobia, including low educational or socioeconomic levels, a history of febrile seizures in the child and young maternal age. Fever phobia was also more common in Bedouins and in people from Latin America, Southern Italy and Turkey. There were also 15 papers that addressed fever phobia among 4566 healthcare providers. All the reports suggested that a fear of fever and a tendency to over treat was common among physicians and nurses. CONCLUSION: Fever phobia was a common world phenomena that affected caregivers and healthcare providers.

Bioimpedance Spectroscopy Imprecisely Assesses Lean Body Mass in Pediatric Dialysis Patients.

OBJECTIVES: Alterations in body compositions are strongly associated with poor outcomes in end-stage renal disease patients. Hence, assessment of lean body mass is crucial for clinically monitoring these patients. The use of multifrequency bioimpedance spectroscopy measurements has also been advocated, but their usefulness in children is questioned. We investigated whether their application is appropriate for lean body mass measurement in pediatric patients receiving chronic dialysis. METHODS: Lean body mass estimates as assessed by multifrequency bioimpedance spectroscopy and by deuterium dilution were obtained for 15 patients (mean age 10.9 ±â€Š3.6 years). RESULTS: Lean body mass (mean ±â€Šstandard deviation) determined by bioimpedance was 24.2 ±â€Š10.7 and 24.4 ±â€Š10.3 kg by deuterium technique. Bland-Altman analysis showed a mean (±standard deviation) difference between the 2 methods of -0.25 ±â€Š2.30 kg with 95% limits of agreement of -4.80 to 4.25 kg. In a multiple linear regression model, the hydration status was associated with measurement bias after adjusting for age, sex, weight, and body surface area. CONCLUSIONS: Our results show a high level of agreement between measurements by bioimpedance and deuterium technique, but the limits of agreement were wide. These findings do not support the use of bioimpedance to individually assess lean body mass in pediatric dialysis patients with and without overhydration.

D-lactic acidosis in humans: systematic literature review.

BACKGROUND: D-lactic acidosis is an uncommon and challenging form of metabolic acidosis that may develop in short bowel syndrome. It has been documented exclusively in case reports and small case series. METHODS: We performed a review of the literature in the National Library of Medicine and Excerpta Medica databases. RESULTS: We identified 84 original reports published between 1977 and 2017. D-lactic acidosis was observed in 98 individuals ranging in age from 7 months to 86 years with short bowel syndrome. The clinical presentation included Kussmaul breathing, confusion, slurred speech, and gait disturbances. Furthermore, among 99 consecutive patients with short bowel syndrome, 21 reported having episodes with symptoms consistent with D-lactic acidosis. In addition, D-lactic acid might also contribute to acidosis in diabetes mellitus. Finally, abnormally high D-lactic acid was documented after administration or ingestion of large amounts of propylene glycol, as paraneoplastic phenomenon and perhaps also in a so far poorly characterized inherited inborn error of metabolism. CONCLUSIONS: In humans with short bowel syndrome (or carbohydrate malabsorption), D-lactic acidosis is likely rather common and under-recognized. This condition should be included in the differential diagnosis of unexplained high-gap metabolic acidosis where the anion causing the acidosis is not known. Furthermore, diabetic acidosis might be caused by accumulation of both ketone bodies and D-lactic acid. Finally, there are endogenous sources of D-lactic acid in subjects with propylene glycol intoxication.

Clinical Practice: Nausea and vomiting in acute gastroenteritis: physiopathology and management.

Currently recommended management of acute gastroenteritis is supportive. Although the affected children habitually have vomiting, recommendations do not focus on the correction of this symptom. In this condition, elevated ketone bodies and stimuli initiated by gut mucosa damage produced by the enteral pathogen likely underlay nausea and vomiting. As compared to 0.9% saline, intravenous administration of a dextrose-containing bolus of 0.9% saline is associated with a greater reduction of circulating ketones and a shorter duration of nausea and vomiting. Nonetheless, this treatment strategy is not followed by a lower rate of hospitalization. CONCLUSION: Well-designed investigations suggest that antagonists of the type 3 serotonin receptor, most frequently oral ondansetron, reduce the rate of vomiting, improve the tolerance of oral rehydration, and reduce the need for intravenous rehydration. What is Known: • Although children with gastroenteritis habitually have vomiting, current recommendations do not focus on the correction of this symptom. What is New: • Recently acquired evidence supports the prescription of ondansetron, an antagonist of the type 3 serotonin receptor, to increase the success rate of oral rehydration therapy.

Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review.

Approximately 500 cases of idiopathic systemic capillary leak syndrome (Clarkson syndrome) have been reported worldwide. This life-threatening condition is characterized by episodes of increase in vascular permeability with loss of fluid into the interstitium and presents with acute onset of edema, signs of tissue hypoperfusion, hemoconcentration, and low blood protein level. It has been diagnosed mainly in middle-aged adults with a monoclonal gammopathy. We performed a systematic review of the literature on Clarkson syndrome in subjects ≤ 18 years of age. We identified 24 reports, published since 1989, providing data on 32 otherwise healthy subjects, who experienced 67 well-documented episodes of Clarkson syndrome. The condition affected more frequently girls (21, 66%) than boys, presented throughout childhood, and was preceded by a mostly viral illness in 75% of cases. A monoclonal gammopathy was never reported. Uncompensated circulatory shock, muscle compartment syndrome, acute kidney injury, pulmonary edema, and either pleural or pericardial effusion were, in decreasing order of frequency, the most common complications. Four patients died.Conclusion: Clarkson syndrome develops not only in adulthood but also in childhood. In this age group, this condition is not linked to a monoclonal gammopathy. What is Known: • Clarkson syndrome is a rare condition that has been diagnosed mainly in middle-aged adults and is mostly linked to a monoclonal gammopathy. What is New: • In subjects ≤ 18 years of age, Clarkson syndrome is not linked to a monoclonal gammopathy.

Bioimpedance and Fluid Status in Children and Adolescents Treated With Dialysis.

BACKGROUND: Assessment of hydration status in patients with chronic kidney failure treated by dialysis is crucial for clinical management decisions. Dilution techniques are considered the gold standard for measurement of body fluid volumes, but they are unfit for day-to-day care. Multifrequency bioimpedance has been shown to be of help in clinical practice in adults and its use in children and adolescents has been advocated. We investigated whether application of multifrequency bioimpedance is appropriate for total-body water (TBW) and extracellular water (ECW) measurement in children and adolescents on dialysis therapy. STUDY DESIGN: A study of diagnostic test accuracy. SETTING & PARTICIPANTS: 16 young dialysis patients (before a hemodialysis session or after peritoneal dialysis treatment) from the Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, and the Emma Children's Hospital-Academic Medical Center, Amsterdam, the Netherlands. INDEX TEST: TBW and ECW volumes assessed by multifrequency bioimpedance. REFERENCE TESTS: TBW and ECW volumes measured by deuterium and bromide dilution, respectively. RESULTS: Mean TBW volumes determined by multifrequency bioimpedance and deuterium dilution were 19.2±8.7 (SD) and 19.3±8.3L, respectively; Bland-Altman analysis showed a mean bias between the 2 methods of -0.09 (95% limits of agreement, -2.1 to 1.9) L. Mean ECW volumes were 8.9±4.0 and 8.3±3.3L measured by multifrequency bioimpedance and bromide dilution, respectively; mean bias between the 2 ECW measurements was +0.6 (95% limits of agreement, -2.3 to 3.5). LIMITATIONS: Participants ingested the deuterated water at home without direct supervision by investigators, small number of patients, repeated measurements in individual patients were not performed. CONCLUSIONS: Multifrequency bioimpedance measurements were unbiased but imprecise in comparison to dilution techniques. We conclude that multifrequency bioimpedance measurements cannot precisely estimate TBW and ECW in children receiving dialysis.

Acute pain management: acetaminophen and ibuprofen are often under-dosed.

Most children with pain are managed by either acetaminophen or ibuprofen. However, no study has so far investigated if children are prescribed adequate doses of acetaminophen or ibuprofen in emergency department. Aim of this retrospective study was to investigate the prevalence of under-dosage of these drugs in children presenting with pain in emergency department. Children initially prescribed with acetaminophen or ibuprofen for pain management were included. The χ 2 automatic interaction detection method was used considering the percentage variation from the minimum of the appropriate dose as dependent variable while prescribed drug, age, gender, body weight, type of hospital (pediatric or general), and availability of internal guidelines on pediatric pain management in the emergency department as independent variables. Data on 1471 children managed for pain were available. Under-dosage was prescribed in 893 subjects (61%), of whom 577 were prescribed acetaminophen and 316 ibuprofen. The use of acetaminophen suppositories, body weight <12 kg or >40 kg, and the use of oral ibuprofen identified clusters of children associated with under-dosage prescription. CONCLUSION: Prescription of acetaminophen and ibuprofen was frequently under-dosed. The use of suppositories, lower and higher body weight, and the use of ibuprofen were associated with under-dosage. Under-dosing may reflect prescription of anti-pyretic doses. CLINICAL TRIAL REGISTRATION: Agenzia Italiana del Farmaco-Observational Study Register (RSO). Registration code: PIERRE/1 What is Known: • Pain is frequent in children presented to emergency department. • International recommendations on pain management are often not implemented. What is New: • Acetaminophen and ibuprofen were frequently underdosed in children prescribed for pain in the Italian emergency departments. • Under-dosage may be related to the habit of using acetaminophen and ibuprofen in the recommended range for fever treatment.

Accuracy of Prediction Formulae for the Assessment of Resting Energy Expenditure in Hospitalized Children.

BACKGROUND AND AIM: The resting energy expenditure (REE) of ill children is commonly estimated from prediction formulae developed in healthy children. The aim of the present study was to evaluate the accuracy of commonly employed REE prediction formulae versus indirect calorimetry in hospitalized children. METHODS: We performed a cross-sectional study of 236 infants, children, and adolescents consecutively admitted to the Intermediate Care, Nephrology, Intensive Care, Emergency, and Cystic Fibrosis Units of the De Marchi Pediatric Hospital (Milan, Italy) between September 2013 and March 2015. REE was measured by indirect calorimetry and estimated using the World Health Organization (WHO), Harris-Benedict, Schofield, and Oxford formulae. RESULTS: The mean (standard deviation) difference between the estimated and measured REE was: -1 (234) kcal/day for the WHO formula; 82 (286) kcal/day for the Harris-Benedict formula; 2 (215) kcal/day for the Schofield-weight formula; -2 (214) kcal/day for the Schofield-weight and height formula; and -5 (221) kcal/day for the Oxford formula. Even though the WHO, Schofield, and Oxford formulae gave accurate estimates of REE at the population level (small mean bias), all the formulae were not accurate enough to be employed at the individual level (large SD of bias). CONCLUSIONS: The WHO, Harris-Benedict, Schofield, and Oxford formulae should not be used to estimate REE in hospitalized children.

Micro- and macroscopic hematuria caused by renal vein entrapment: systematic review of the literature.

BACKGROUND: Hematuria secondary to renal vein entrapment is mentioned only passing in textbooks and reviews. METHODS: We performed a search of the National Library of Medicine database for peer-reviewed publications using the terms "renal vein" or "nutcracker" and "hematuria". RESULTS: We identified 187 published reports/studies that covered 736 patients, of whom 288 had microscopic hematuria and 448 had macroscopic hematuria. The patient cohort comprised 159 patients aged ≤17 years. Abdominal pain was absent in approximately 65% of all patients, and a clinically relevant left-sided varicocele was observed in 29% of the male patients. A normal pre-aortic left renal vein and an anomalous anatomy were noted in 680 and 56 patients, respectively. The body mass index (BMI) was lower in patients with renal vein entrapment than in the controls, with a regression of hematuria correlating with an increase in BMI. A surgical procedure was attempted in 34% of the patients, of which the most common were endovascular stenting and transposition of the renal vein distally into the vena cava. CONCLUSIONS: In cases of unexplained hematuria with or without abdominal pain, clinicians should consider the diagnosis of renal vein congestion, especially in males with varicocele. Ultrasonic Doppler flow scanning is the recommended initial diagnostic modality in these patients. Expectation management is advised in the great majority of cases.

Na(+), K(+), Cl(-), acid-base or H2O homeostasis in children with urinary tract infections: a narrative review.

Guidelines on the diagnosis and management of urinary tract infections in childhood do not address the issue of abnormalities in Na(+), K(+), Cl(-) and acid-base balance. We have conducted a narrative review of the literature with the aim to describe the underlying mechanisms of these abnormalities and to suggest therapeutic maneuvers. Abnormalities in Na(+), K(+), Cl(-) and acid-base balance are common in newborns and infants and uncommon in children of more than 3 years of age. Such abnormalities may result from factitious laboratory results, from signs and symptoms (such as excessive sweating, poor fluid intake, vomiting and passage of loose stools) of the infection itself, from a renal dysfunction, from improper parenteral fluid management or from the prescribed antimicrobials. In addition, two transient renal tubular dysfunctions may occur in infants with infectious renal parenchymal involvement: a reduced capacity to concentrate urine and pseudohypoaldosteronism secondary to renal tubular unresponsiveness to aldosterone that presents with hyponatremia, hyperkalemia and acidosis. In addition to antimicrobials, volume resuscitation with an isotonic solution is required in these children. In secondary pseudohypoaldosteronism, isotonic solutions (such as 0.9 % saline or lactated Ringer) correct not only the volume depletion but also the hyperkalemia and acidosis. In conclusion, our review suggests that in infants with infectious renal parenchymal involvement, non-renal and renal causes concur to cause fluid volume depletion and abnormalities in electrolyte and acid-base balance, most frequently hyponatremia.

Using a high-flow nasal cannula provided superior results to low-flow oxygen delivery in moderate to severe bronchiolitis.

AIM: An observational study was carried out on infants with moderate to severe bronchiolitis to compare the clinical outcomes following treatment with a high-flow nasal cannula (HFNC) or standard low-flow oxygen. METHODS: We enrolled subjects below 12 months of age who were affected by their first bronchiolitis episode. Non-formal randomisation, based on HFNC availability, was used to assign subjects to either the HFNC or standard oxygen groups. Respiratory rate, respiratory effort and the ability to feed were compared between the two groups at enrolment and at regular time points. The oxygen requirements and the length of hospital stay were also analysed. RESULTS: Overall, 36 of the 40 enrolled infants completed the study: 18 treated with HFNC (mean age 3.2 months, range 1.2-5.4 months) and 18 with low-flow oxygen delivery (mean age 3.6 months, range 1.3-5.0 months). Improvements in the respiratory rate, respiratory effort and ability to feed were significantly faster in the HFNC group than the low-flow oxygen group. The HNFC group needed oxygen supplementation for two days less than the other group and hospital stays were three days shorter. CONCLUSION: HFNC provided superior clinical outcomes for infants under 12 months with moderate-to-severe bronchiolitis compared to low-flow oxygen.

Treatment of Severe Hypervolemic Hyponatremia in a Child With Pneumonia.

A 21-month-old boy came to our attention because of pneumonia. His weight increased before presentation, and his blood test results showed hyponatremia (116 mEq/L), low plasma osmolarity (241 mOsm/L), and high urine osmolarity (435 mOsm/L). He was treated with 0.9% sodium chloride solution and intravenous furosemide, and sodium levels rose up to 135 mEq/L in 36 hours. No standard treatment is available for severe hyponatremia in children. The use of vaptans in pediatric patients is described in literature, but it lacks evidence about safety and effectiveness. We suggest that furosemide administration plus salt replacement is effective in restoring normal values of plasma sodium concentration in severe euvolemic and hypervolemic hyponatremia.

Identification of Human Adenovirus in Respiratory Samples with Luminex Respiratory Virus Panel Fast V2 Assay and Real-Time Polymerase Chain Reaction.

In order to compare the last version of the Respiratory Virus Panel (RVP) Fast assay for human Adenovirus (hAdv) detection with a specific real-time polymerase chain reaction (qPCR), which is considered the gold standard for hAdv detection, nasopharyngeal samples collected from 309 children (age range, four months to eight years) with respiratory tract infection were tested using the RVP Fast v2 assay (Luminex Molecular Diagnostics, Inc., Toronto, ON, Canada) and a specific TaqMan qPCR to identify hAdv DNA. The RVP Fast v2 assay detected 30/61 (49.2%) hAdv infections that had been identified by real-time qPCR, demonstrating a significantly lower detection rate (p < 0.001). The sensitivity of the RVP Fast v2 assay in comparison to qPCR was lower in younger children (42.9% vs. 57.7%; Cohen's kappa coefficient, 0.53); in samples with co-infections (40.0% vs. 56.7%; Cohen's kappa coefficient, 0.52); and in samples with hAdv type C (45.9% vs. 57.1%; Cohen's kappa coefficient, 0.60). Samples with lower viral loads were associated with a significantly lower sensitivity of the RVP Fast v2 assay (35.1% vs. 68.2%, p = 0.01; Cohen's kappa coefficients, 0.49). The RVP Fast v2 assay has important limitations for the detection of hAdv and cannot be used to evaluate whether hAdvs are the main etiologic agent responsible for an outbreak or when epidemiological studies are performed.

Electrolyte abnormalities in cystic fibrosis: systematic review of the literature.

BACKGROUND: Cystic fibrosis per se can sometimes lead to hyponatremia, hypokalemia, hypochloremia or hyperbicarbonatemia. This tendency was first documented 60 years ago and has subsequently been confirmed in single case reports or small case series, most of which were retrospective. However, this issue has not been addressed analytically. We have therefore systematically reviewed and analyzed the available literature on this subject. METHODS: This was a systematic review of the literature. RESULTS: The reports included in this review cover 172 subacute and 90 chronic cases of electrolyte imbalances in patients with cystic fibrosis. The male:female ratio was 1.57. Electrolyte abnormalities were mostly associated with clinically inapparent fluid volume depletion, mainly affected patients aged ≤2.5 years, frequently tended to recur and often were found before the diagnosis of cystic fibrosis was established. Subacute presentation often included an history of heat exposure, vomiting, excessive sweating and pulmonary infection. History of chronic presentation, in contrast, was often inconspicuous. The tendency to hypochloremia, hypokalemia and metabolic alkalosis was similar between subacute and chronic patients, with hyponatremia being more pronounced (P < 0.02) in subacute compared to chronic presentations. Subacute cases were treated parenterally; chronic ones were usually managed with oral salt supplementation. Retention of urea and creatinine was documented in 38 % of subacute cases. CONCLUSIONS: The findings of our review suggest that physicians should be aware that electrolyte abnormalities can occur both as a presenting and a recurring feature of cystic fibrosis.

Severe and isolated headache associated with hypertension as unique clinical presentation of posterior reversible encephalopathy syndrome.

BACKGROUND: Posterior reversible encephalopathy syndrome is a potentially reversible clinicoradiologic syndrome characterized by headache, mental confusion, visual disturbances and seizures associated with posterior cerebral lesions on radiological imaging. Prompt treatment of this condition is mandatory to avoid severe irreversible complications. CASE PRESENTATION: We report a 9-year-old boy with arterial hypertension and headache as unique clinical presentation of posterior reversible encephalopathy syndrome. CONCLUSIONS: Severe and isolated headache associated with arterial hypertension can be the unique clinical presentation of posterior reversible encephalopathy syndrome. This syndrome must be considered even in absence of all typical symptoms to prevent the progression of a potentially life threatening condition.

Clinical dehydration and glomerular filtration rate in acute paediatric gastroenteritis.

AIM: To evaluate changes in glomerular filtration rate in acute gastroenteritis. METHODS: The correlation between two clinical diagnostic scales and glomerular filtration rate has been investigated in 113 children with acute gastroenteritis in a paediatric emergency setting. RESULTS: A significant reduction of GFR was found in 10% children less than, and 5% children higher than, 2 years of age with acute gastroenteritis. CONCLUSION: The differences observed as for risk of renal hypoperfusion suggests to consider the age of children as an important determinant to consider the dehydration status in acute gastroenteritis.

Episodic idiopathic systemic capillary leak syndrome in a girl.

Episodic idiopathic systemic capillary leak syndrome is a rare disorder that presents with attacks of circulatory shock due to plasma leakage into the extravascular space. Reported here is the case of a girl who had recurrent circulatory shock. The diagnosis of episodic idiopathic systemic capillary leak syndrome was made following the fourth episode. The course was favorable following acute i.v. cristalloids and methylprednisolone.