Turkish archives of pediatrics最新文献

Objective: The objective of this study is to evaluate the prognosis of idiopathic premature ventricular beats (PVBs) in children.

Materials and methods: We retrospectively evaluated 73 children (<18 years old) with idiopathic PVBs and structurally normal hearts. All patients were evaluated by 24-hour Holter electrocardiography (ECG) and echocardiography at the first admission and followed with a mean of 27 ± 7.6 months after diagnosis. Baseline Holter ECG and echocardiographic findings were compared to the last visit.

Results: The mean age of the patients was 11.1 ± 4.8 years, and half were symptomatic at initial examination. Baseline Holter showed complex beats in 35 cases, non-sustained ventricular tachycardia in 7 cases, and frequent PVBs in 19 cases. Complete recovery (CR) of PVBs was observed in 37 cases (50.7%) at a median of 15 (minimum: 5, maximum: 33) months after diagnosis. There were no significant differences in CR rates between patient groups with left bundle branch block (LBBB) vs. right bundle branch block (RBBB) morphology of PVBs, simple vs. complex PVBs, and daytime vs. nighttime dominance of PVBs (P > .05 for all parameters). The CR rate of PVBs was different among patients with infrequent, moderate, and frequent PVBs (62.8%, 36.4%, and 31.6%, respectively). Premature ventricular beats disappeared more often during follow-up in patients with infrequent PVBs (P = .045). However, the absolute decrease and disappearance rates of PVBs were similar across all groups (72.1%, 81.8%, and 89.5%, respectively; P = .319). The resolution rates of PVBs were not statistically different between the patient group who received pharmacological treatment and the group who followed up without treatment (P = .070). No myocardial dysfunction was observed in any patient during follow-up. No cases experienced major cardiac events.

Conclusion: Idiopathic PVBs usually regress in childhood regardless of frequency and complexity or receiving antiarrhythmic medication. The risk of ventricular dysfunction is low during childhood; however, they require careful evaluation and follow-up.

Swelling, effusion, tenderness, and pain seen in the joints of juvenile idiopathic arthritis (JIA). This disease may cause limitation in joint movements, muscle weakness, atrophy, balance, and gait disorders. Physical fitness is accepted as an important determinant of health in both childhood and adolescence. The aim was to evaluate the physical fitness of children/adolescents with JIA and compare it with healthy peers. Seventy children/adolescents were included (35 JIA and 35 healthy). The Childhood Health Assessment Questionnaire (CHAQ) and the Brockport physical fitness test battery were used for evaluation. The Brockport physical fitness test battery consists of dominant handgrip strength, curl-up, push-up, trunk lift, shoulder stretch, sit and reach tests, skinfold thickness (calf/triceps/subscapular) measurements, and PACER 20 m test. A significant difference was found in all sub-parameters of CHAQ (P < .05) and dominant hand grip strength (P = .037), curl-up test (P < .001), trunk lift test (P = .018), shoulder stretch (P < .001) and PACER 20 m test (P < .001) tests in favor of the healthy group. Children/adolescents with JIA demonstrated lower performance compared to their healthy peers in muscular and cardiovascular capacity tests (curl-up test, PACER 20 m test, trunk lift test, dominant hand grip strength test, and shoulder stretch test). Their functional abilities are more impaired, and they experience higher levels of pain and lower levels of general well-being compared to healthy peers.

Coarctation of the aorta (CoA) accounts for 3.5% of all congenital heart diseases in children. The clinical manifestations range from heart failure to asymptomatic hypertension. Treatment options include surgical repair, balloon angioplasty, and stenting. We aimed to investigate the long-term results of surgery and balloon angioplasty to identify the possible risk factors for recoarctation and predictors associated with early success in treatment modalities. The data of 138 children who underwent examinations at a tertiary center between 2015 and 2020 with the diagnosis of CoA were evaluated. The basic demographic characteristics, clinical and echocardiographic findings, results, and treatment methods of the patients were evaluated retrospectively. The mean follow-up period was 75.1 months (range of 1-223). As initial treatment, 75 patients (60.5%) underwent balloon angioplasty, 44 (35.5%) underwent surgery, and 5 (4%) underwent stenting. The early success rate of balloon angioplasty and surgery was 72.5% and 79.5%, respectively. Recoarctation occurred in 47.6% of patients following the first therapy. The median reintervention-free survival time was 138 months for all patients and was significantly higher in the surgery group (P = .025). The recoarctation rate was slightly lower in the surgery group than in those who underwent balloon angioplasty, but it was not statistically significant. None of the clinical and echocardiographic findings were found to be associated with recoarctation or early success. The rate of recoarctation is still high in long-term follow-up after aortic coarctation treatment, and clinical and echocardiographic findings are insufficient to predict the chance of early success and the risk of recoarctation.

Mixed gonadal dysgenesis (MGD) is an uncommon chromosomal Disorder of Sexual Development (DSD). There is insufficient information regarding clinical findings and growth patterns. This study aimed to provide more information about mixed gonadal dysgenesis, which has not yet been sufficiently defined. Data from 10 patients diagnosed with mixed gonadal dysgenesis were retrospectively reviewed. Clinical presentations, complaints at admission, imaging, genetic results, and treatments received by the patients were examined. Gonadal status and the gender of the patients were reared and evaluated by a multidisciplinary council decision. If received, growth hormone treatment doses and height gains were examined. The patients' ages at admission range from 6 months to 17.5 years. The median height SDS of the patients was -0.75 (2.73), the mean body weight SDS was -0.49 (±1.46), and the mean body mass index (BMI) SDS was 0.26 (±0.97). The complaints at admission varied, including ambiguous genitalia, short stature, and absence of menstruation. Some patients are completely in the female phenotype, while some are inadequately virilized male phenotype. External Masculinization Score (EMS) ranges from 1 to 6.5. The decision to raise 6 patients as female and 4 patients as male was made by a multidisciplinary council. Growth hormone treatment was administered to patients raised as female and diagnosed with short stature. The height SDS gain in treated patients was 0.42 (±0.49). Due to its rarity and varied clinical presentation, our knowledge about mixed gonadal dysgenesis is limited. Therefore, early diagnosis and individualized treatment plans are crucial for this patient group.

Biliary atresia (BA) is an obstructive cholangiopathy that involves the intrahepatic and extrahepatic bile ducts. Ultrasound (US) can aid in evaluation of the biliary system and be efficiently used in daily practice. However, most studies on US for diagnosing BA have been conducted in developed countries. Therefore, we have aimed to evaluate the diagnostic accuracy of US in BA in infants with cholestasis from a developing country. This retrospective study used data collected from our hospital medical records. The US findings were compared with the gold standard intraoperative or cholangiography findings. Thirty-five BA patients (19 males and 16 females) and 36 controls (20 males and 16 females) were included in the study. Most of the patients (85.7%) were ≤ 6 months old. The absence of a gallbladder demonstrated 71.42% sensitivity (Sn), 91.67% specificity (Sp), 89.29% positive predictive value (PPV), 76.74% negative predictive value (NPV), 8.57 positive likelihood ratio (LR+), and 0.31 negative likelihood ratio (LR-) for diagnosing BA. The triangular cord sign demonstrated 14.28% Sn, 100% Sp, 100% PPV, 76.74% NPV, ∞ LR+, and 0.86 LR- for diagnosing BA. The combination of gallbladder absence and a positive triangular cord sign demonstrated 82.85% Sn, 91.67% Sp, 90.63% PPV, 84.61% NPV, 9.95 LR+, and 0.19 LR- for diagnosing BA. The diagnostic accuracy of US in BA is high, indicating that it can be the imaging tool of choice in infants with cholestasis. Ultrasound is safe and can be easily used in daily practice without the risk of radiation exposure.

We aimed to investigate the relationship between sociodemographic and clinical characteristics, as well as the utilization of diabetes technologies, with diabetes management in individuals with type 1 diabetes (T1D). Our study included 134 cases diagnosed with T1D who were followed for at least 1 year with T1D. Of the cases, 67.2% were using insulin pens as their insulin regimen, while 37.8% were using insulin pumps. The rate of continuous glucose monitoring (CGM) usage was 29.9%. The rate of CGM usage was 5% in families with low income levels. Glycosylated hemoglobin A1c(HbA1c) level of children with working mothers was found to be higher compared to those with non-working mothers (median 9.2% vs. 8%; P = .009). Cases with 3 or more siblings had higher HbA1c levels compared to those with 2 or fewer siblings (median 8.7% vs. 8.1%; P = .044).The median HbA1c was 8.7% in cases using insulin pens and checking fingerstick blood glucose (SMBG); 8.3% in cases using insulin pumps and SMBG; 7.6% in cases using insulin pens with CGM, and 7.5% in cases using insulin pumps with CGM (P = .003). The utilization of insulin pumps with CGM in T1D cases exhibited lower HbA1clevels. Similarly, even the usage of insulin pens with CGM demonstrated improved diabetes management. Maternal employment and having a higher number of siblings may negatively affect diabetes management due to increased caregiver burden. We believe that personalized healthcare delivery tailored to the individual needs of T1D patients based on family and clinical characteristics could have positive effects on diabetes management.