Tuberkuloza je nalezljiva bolezen, ki jo povzročajo bacili tuberkuloze (bakterije vrste Mycobacterium tuberculosis). Bolezen najpogosteje prizadene pljuča, pri tretjini bolnikov tudi druge organe ...(sklepe, možgane, jetra, bezgavke). Vir okužbe so odrasli bolniki ali občasno mladostniki, ki kašljajo in v okolico izločajo bacile. Majhni otroci običajno niso kužni. Okužba ne pomeni vedno tudi aktivne bolezni. Poznamo več oblik tuberkuloze. Pri majhnih otrocih je pogosto nema, medtem ko imajo mladostniki in odrasli običajno tipične klinične znake, kot so dolgotrajno povišana telesna temperatura, kašelj, inapetenca ter nočno potenje in hujšanje. Diagnozo postavimo na osnovi anamneze, klinične slike, izvidov imunoloških testov in radioloških preiskav ter mikrobioloških izvidov odvzetih kužnin. Zgodnje odkritje in zdravljenje tuberkuloze pri mladostnikih sta izjemno pomembni, saj so bolniki pogosto kužni. Tuberkuloza je nalezljiva bolezen, ki jo moramo obvezno prijaviti. V prispevku predstavljamo klinični primer mladostnika s sumom na postprimarno tuberkulozo.
Factors associated with COVID-19 presentation in children with asthma are poorly defined. Our study aimed to assess the clinical course of COVID-19 in children with asthma, with particular attention ...to possible risk factors for severe disease and long-term sequelae in this group of patients. We assessed the occurrence of SARS-CoV-2 infection in children with asthma six months before their regular outpatient visit to the asthma clinic. Characteristics of patients presenting with signs of SARS-CoV-2 upper (URTI) or lower respiratory tract infection (LRTI) were compared. We focused on factors previously associated with COVID-19 severity. Twenty-seven percent of patients (57/210) reported exposure to SARS-CoV-2 infection. In the symptomatic group, 36% (15/42) reported symptoms of LRTI and 64% (27/42) of URTI. Poorer asthma control was observed in patients with LRTI compared to URTI (80% vs. 7%, p < 0.001). In addition, children with poorer asthma control had a higher risk of presenting with SARS-CoV-2 LRTI in a multiple logistic regression analysis. COVID-19 disease course was not associated with regular ICS use and asthma severity. However, patients on regular ICS had better asthma control (p = 0.026). We found no PFT deterioration post-COVID-19 in either group of patients. Our results suggest good asthma control and treatment adherence prior to infection are associated with better COVID-19 outcomes in children with asthma.
Izhodišča: Cistična fibroza (CF) je najpogostejša kronična avtosomno recesivno dedna bolezen belcev. Kaže se s prizadetostjo številnih organov, zato se bolniki spremljajo v centrih CF s ...specializiranim multidisciplinarnim timom strokovnjakov. Eden takih je center CF Pediatrične klinike Ljubljana, kjer se vodijo vsi otroci in mladostniki s CF v Sloveniji. Od ustanovitve centra dalje vodimo zbirko podatkov o bolnikih, ki nam je v pomoč pri analizi razmer in pri primerjavi kakovosti obravnave naših bolnikov z drugimi evropskimi centri CF.
Metode: Izvedli smo retrospektivno raziskavo z namenom pregledati in oceniti kazalce bolezni otrok in mladostnikov s CF, vodenih na Pediatrični kliniki Ljubljana v letu 2020. Pri 78 vključenih bolnikih smo analizirali podatke o demografskih značilnostih, genetskih mutacijah, pljučni funkciji, prehranjenosti, kroničnih okužbah in zdravljenju s CFTR modulatornimi zdravili (angl. cystic fibrosis transmembrane conductance regulator, CFTR – regulator transmembranske prevodnosti pri cistični fibrozi). Za primerjavo z drugimi evropskimi centri smo uporabili podatke iz letnega poročila Evropskega registra CF za leto 2020.
Rezultati: Leta 2020 smo v našem centru obravnavali 78 bolnikov, izmed katerih je bilo 56 % (44/78) bolnikov moškega spola. Povprečna starost bolnikov je znašala 13,2 leta (SD 6,4 let), z razponom od 1,2 leta do 25,5 leta. Mutacija F508del je bila najpogosteje zastopana, saj je bilo kar 88,5 % nosilcev vsaj ene, pri 64,1 % obolelih pa je bila mutacija prisotna na obeh alelih. Povprečni forsirani izdihani volumen v 1 sekundi (FEV1) naših bolnikov je bil nad evropskim povprečjem, prevalenca kronične okužbe s povzročiteljem Pseudomonas aeruginosa pa nižja od evropskega povprečja. V letu 2020 smo 21 bolnikom uvedli CFTR modulatorno zdravilo, od tega 5 bolnikom lumakaftor/ivakaftor in 16 bolnikom eleksakaftor/tezakaftor/ivakaftor.
Zaključek: Kazalci kakovosti obravnave otrok in mladostnikov s CF v Sloveniji dosegajo in pogosto presegajo evropsko povprečje. Med prvimi državami v Evropi smo pričeli uvajati CFTR modulatorna zdravila, ki obetajo pomembno izboljšanje kliničnega stanja in kakovosti življenja bolnikov s CF.
Tuberculosis is an infectious disease caused by tuberculosis bacilli (bacteria from the Mycobacterium tuberculosis species). The disease most commonly affects the lungs, but in a third of patients, ...other organs are also affected (joints, brain, liver, lymph nodes). The source of infection for a child is an adult patient or occasionally an adolescent, who coughs and excretes bacilli. Young children are usually not contagious. The infection does not always mean active disease. Several types of tuberculosis are recognised. Tuberculosis is often silent in young children, but adolescents and adults usually have typical clinical signs such as prolonged fever, cough, malaise, loss of appetite, night sweats and weight loss. The diagnosis is based on the history, clinical signs and examination, tuberculin skin testing (and interferon-gamma release assays, if available), chest x-ray and microbiological confirmation. Early detection and treatment of tuberculosis in adolescents are very important to prevent transmission to other persons. Tuberculosis is an infectious disease that must be reported. In this article, we present the case of an adolescent with suspected post-primary tuberculosis.
The integration of pediatric palliative care (PPC) should become a standard of care for all children with life-limiting and life-threatening illnesses. There are many barriers and misperceptions in ...pediatrics which hinder the early implementation of PPC. The aim of the study was to design starting points for the establishment of accessible PPC with early involvement of patients in a tertiary-level children’s hospital. An intervention, presentation, and discussion on PPC were offered by the hospital PPC team to all employees in the hospital. A total of 237 participants (physicians 30.4%, nurses 49.4%, psychologists 8.4%, and others) completed a questionnaire before and after the intervention. The personnel’s knowledge, self-assessment of their ability to perform PPC, attitude to participate in PPC, and their awareness and understanding of the need for PPC were evaluated. The results were analyzed using Pandas and SciPy libraries in Python. The knowledge, awareness, and attitude of the physicians, nurses, and other professionals improved significantly after the intervention. However, the self-assessment of their ability to perform PPC did not increase. Previous experience with the death of a patient has proven to be a stimulus for self-initiative in acquiring knowledge in PPC and was linked with a better attitude and higher awareness of the need for PPC.
Conclusions
: More education and practical work tailored to the different professional profiles are needed, with adjustments for specific subspecialist areas, especially where patients could be included in early PPC. Although additional studies are needed, we identified the main directions for the further implementation of PPC in clinical practice in our setting.
What is Known:
•
Every child and adolescent living with a life-limiting or life-threatening condition should receive pediatric palliative care (PPC) to alleviate suffering and enhance their quality of life. There exists a plethora of recognized barriers to the effective implementation of palliative care, specifically PPC. These barriers are often connected to the emotional burden of requesting PPC. Early identification and inclusion of patients is important for improving PPC in hospital settings. Finding strategies to overcome the barriers is crucial for improving the well-being and improving the quality of life of the patients and their families.
•
Early identification is only possible with a high level of knowledge and understanding of PPC among healthcare professionals. In a hospital setting where there are interdisciplinary palliative care teams, the inclusion is still only possible if all staff are capable of recognizing patients in need of PPC and are willing to start the process. Since most healthcare education systems only recently included PC into the healthcare curriculum, most of the professionals currently working in hospitals are only educated to the extent of self-initiative.
What is New:
•
To bridge the existing gap in knowledge, the hospital PC team organized an intervention, presentation, and discussion on PPC, which was offered to all employees in the hospital who are in contact with patients. The personnel’s knowledge, self-assessment of their ability to perform PPC, attitude to participate in PPC, and their awareness and understanding of the need for PPC were evaluated. These four categories have not been tested together before. The knowledge, awareness, and attitude of the physicians, nurses, and other professionals improved significantly after the intervention. All the profiles that work together in a team were evaluated simultaneously for the first time.
•
The self-assessment of their ability to perform PPC did not increase—in fact, it decreased. This was unexpected, as existing literature establishes a link between education and quality of PC. Previous experience with the death of a patient has proven to be a stimulus for self-initiative in acquiring knowledge in PPC and was linked with a better attitude and higher awareness of the need for PPC. We re-established the importance of education and practical work tailored to the different professional profiles, with adjustments for specific subspecialist areas, especially where patients could be included in early PPC. Although additional studies are needed, we identified the main directions for the further implementation of PPC in clinical practice in our setting.
We evaluated the safety and efficacy of day-and-night fully closed-loop insulin therapy using faster (Faster-CL) compared with standard insulin aspart (Standard-CL) in young adults with type 1 ...diabetes.
In a double-blind, randomized, crossover trial, 20 participants with type 1 diabetes on insulin pump therapy (11 females, aged 21.3 ± 2.3 years, HbA
7.5 ± 0.5% 58.5 ± 5.5 mmol/mol) underwent two 27-h inpatient periods with unannounced afternoon moderate-vigorous exercise and unannounced/uncovered meals. We compared Faster-CL and Standard-CL in random order. During both interventions, the fuzzy-logic control algorithm DreaMed GlucoSitter was used. Glucose sensor data were analyzed by intention-to-treat principle with the difference (between Faster-CL and Standard-CL) in proportion of time in range 70-180 mg/dL (TIR) over 27 h as the primary end point.
The proportion of TIR was similar for both arms: 53.3% (83% overnight) in Faster-CL and 57.9% (88% overnight) in Standard-CL (
= 0.170). The proportion of time in hypoglycemia <70 mg/dL was 0.0% for both groups. Baseline-adjusted interstitial prandial glucose increments 1 h after meals were greater in Faster-CL compared with Standard-CL (
= 0.017). The gaps between measured plasma insulin and estimated insulin-on-board levels at the beginning, at the end, and 2 h after the exercise were smaller in the Standard-CL group (
= 0.029,
= 0.003, and
= 0.004, respectively). No severe adverse events occurred.
Fully closed-loop insulin delivery using either faster or standard insulin aspart was safe and efficient in achieving near-normal glucose concentrations outside postprandial periods. The closed-loop algorithm was better adjusted to the standard insulin aspart.
Aims/hypothesis
Hypoglycaemia during and after exercise remains a challenge. The present study evaluated the safety and efficacy of closed-loop insulin delivery during unannounced (to the closed-loop ...algorithm) afternoon physical activity and during the following night in young people with type 1 diabetes.
Methods
A randomised, two-arm, open-label, in-hospital, crossover clinical trial was performed at a single site in Slovenia. The order was randomly determined using an automated web-based programme with randomly permuted blocks of four. Allocation assignment was not masked. Children and adolescents with type 1 diabetes who were experienced insulin pump users were eligible for the trial. During four separate in-hospital visits, the participants performed two unannounced exercise protocols: moderate intensity (55% of
V
⋅
O
2
max
) and moderate intensity with integrated high-intensity sprints (55/80% of
V
⋅
O
2
max
), using the same study device either for closed-loop or open-loop insulin delivery. We investigated glycaemic control during the exercise period and the following night. The closed-loop insulin delivery was applied from 15:00 h on the day of the exercise to 13:00 h on the following day.
Results
Between 20 January and 16 June 2016, 20 eligible participants (9 female, mean age 14.2 ± 2.0 years, HbA
1c
7.7 ± 0.6% 60.0 ± 6.6 mmol/mol) were included in the trial and performed all trial-mandated activities. The median proportion of time spent in hypoglycaemia below 3.3 mmol/l was 0.00% for both treatment modalities (
p
= 0.7910). Use of the closed-loop insulin delivery system increased the proportion of time spent within the target glucose range of 3.9–10 mmol/l when compared with open-loop delivery: 84.1% (interquartile range 70.0–85.5) vs 68.7% (59.0–77.7), respectively (
p
= 0.0057), over the entire study period. This was achieved with significantly less insulin delivered via the closed-loop (
p
= 0.0123).
Conclusions/interpretation
Closed-loop insulin delivery was safe both during and after unannounced exercise protocols in the in-hospital environment, maintaining glucose values mostly within the target range without an increased risk of hypoglycaemia.
Trial registration
Clinicaltrials.gov
NCT02657083
Funding
University Medical Centre Ljubljana, Slovenian National Research Agency, and ISPAD Research Fellowship
Background: Young children and rarely adolescents with cystic fibrosis can develop hyponatremic hypochloremic dehydration with metabolic alkalosis. The purpose of this article was to review the ...incidence of this metabolic disorder in our CF patients.Methods: We investigated the medical records of all children diagnosed with cystic fibrosis who are under follow-up in the CF center at the University Childrenʹs Hospital Ljubljana, and were hospitalised or treated on an outpatient basis due to hyponatremic, hypochloremic dehydration in the period from 2007–2012. Data analysis included clinical and laboratory findings.Results: A total of 4 children (7.2 %) from Ljubljana CF center (55 patients under the age of 19 years) were enrolled in the study. We observed 5 episodes of hyponatremic hypochloremic dehydration in 4 patients (one boy had two episodes). All were homozigous for ΔF 508 mutation. Two had episodes in summer and two in autumn, so that no season prevalence of its occurence was found. Median age at admission to the hospital due to hyponatremic hypochloremic dehydration was 7 months (range 4–34). One boy had a hypovolemic shock at the time of admission to the hospital.Conclusions: The results of our study show that dehydration with hypoelectrolytaemia is a rare complication in children with CF in Slovenia, but due to the severity of clinical signs it is an important disorder. Vomiting and fatigue are the warning signs that should alert parents and physicians to consider the possibility of this complication which can be prevented by proper hydration and salt replacement. If left untreated, it can cause seizures, arrhythmias and even death.
Background: Young children and rarely adolescents with cystic fibrosis can develop hyponatremic hypochloremic dehydration with metabolic alkalosis. The purpose of this article was to review the ...incidence of this metabolic disorder in our CF patients.Methods: We investigated the medical records of all children diagnosed with cystic fibrosis who are under follow-up in the CF center at the University Childrenʹs Hospital Ljubljana, and were hospitalised or treated on an outpatient basis due to hyponatremic, hypochloremic dehydration in the period from 2007–2012. Data analysis included clinical and laboratory findings.Results: A total of 4 children (7.2 %) from Ljubljana CF center (55 patients under the age of 19 years) were enrolled in the study. We observed 5 episodes of hyponatremic hypochloremic dehydration in 4 patients (one boy had two episodes). All were homozigous for ΔF 508 mutation. Two had episodes in summer and two in autumn, so that no season prevalence of its occurence was found. Median age at admission to the hospital due to hyponatremic hypochloremic dehydration was 7 months (range 4–34). One boy had a hypovolemic shock at the time of admission to the hospital.Conclusions: The results of our study show that dehydration with hypoelectrolytaemia is a rare complication in children with CF in Slovenia, but due to the severity of clinical signs it is an important disorder. Vomiting and fatigue are the warning signs that should alert parents and physicians to consider the possibility of this complication which can be prevented by proper hydration and salt replacement. If left untreated, it can cause seizures, arrhythmias and even death.