Esophagitis, treatment outcomes, and long-term follow-up in children with esophageal atresia.

OBJECTIVES: Survival rates in children born with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) have improved; however, morbidity associated with the disease remains high. This study aimed to assess the prevalence of gastroesophageal reflux disease (GERD), eosinophilic esophagitis (EoE), fungal esophagitis, esophageal strictures, and long-term outcomes in children with EA/TEF. METHODS: We conducted a retrospective chart review on patients with EA/TEF who were seen at Children's Wisconsin from January 2003 to January 2023. Patients born with EA/TEF were included if they underwent at least one endoscopy after 1 year of age. GERD was diagnosed based on abnormal findings on endoscopy, pH-metry, and/or history of fundoplication. EoE and fungal esophagitis were diagnosed based on abnormal endoscopy. Esophageal stricture diagnosis was based on findings on endoscopy and/or esophagram, and clinical symptoms necessitating esophageal dilation. RESULTS: Eighty-five patients (64.7% males, mean age 7.5 years) were included, the majority had type C EA/TEF (90.6%). GERD was diagnosed in 61.1% (n = 52), 49.4% (n = 42) by macro and/or microscopic endoscopic findings, 22.3% (n = 19) by abnormal pH-metry, and 21.1% (n = 18) by the need for fundoplication for refractory reflux and/or esophageal stricture. Risk of GERD increased with lower gestational age (p = 0.0030), lower birth weight (p = 0.023), and long-gap EA (p = 0.034). In children diagnosed with GERD, only 13.4% of patients (n = 7/52) were able to be weaned off proton pump inhibitor (PPI) without disease recurrence. However, overall, at the completion of the study, 44.7% (n = 38) of patients were successfully weaned off PPI without evidence of GERD. EoE was diagnosed in 20% of the patients (n = 17). All patients diagnosed with EoE required escalation of therapy from PPI alone to swallowed corticosteroids in 52.9% (n = 9), dupilumab in 23.5% (n = 4), elemental formula in 17.6% (n = 3), and elemental formula and swallowed steroids in 5.8% (n = 1). Fungal esophagitis was diagnosed in 15.3% of patients (n = 13). An esophageal stricture requiring dilation was diagnosed in 77.6% (n = 66) of patients at a mean age of 28.5 months, with over 60% diagnosed by 24 months of age. CONCLUSIONS: Children born with EA/TEF continue to be at high risk of developing GERD, EoE, fungal esophagitis, and esophageal stenosis. Diagnostic and therapeutic endoscopy remains a high-yield test to identify and treat these comorbidities.

Comparison of predictive accuracy of knee height equations in children.

Knee height can be a proxy for height when standing height cannot be reliably measured. We compared two commonly used equations (Chumlea and Rumapea) that estimate standing height from knee height. We prospectively enrolled 210 children without scoliosis or kyphosis aged 7-12 years (mean age: 10.2 years, 47.6% males) and measured their knee heights and standing heights. A two-tailed T-test was used to compare predicted heights from each of the equations to actual standing height. Chumlea equation was found to be unreliable (p = 0.0376) while Rumapea equation was found to be reliable in estimating standing height (p = 0.878). Additionally, Rumapea equation was also found to be more accurate than Chumlea equation when results were segregated based on gender and race. In conclusion, the Rumapea equation yields more accurate estimates of standing heights than the Chumlea equation in US children aged 7-12 years.

Understanding Challenges Women Face in Flood-Affected Areas to Access Sexual and Reproductive Health Services: A Rapid Assessment from a Disaster-Torn Pakistan.

Introduction: According to the Global Climate Risk Index, Pakistan is ranked as the fifth-most vulnerable country to climate change. Most recently, during June-August 2022, heavy torrential rains coupled with riverine, urban, and flash flooding led to an unprecedented disaster in Pakistan. Around thirty-three million people were affected by the floods. More than 2 million houses were damaged, leaving approximately 8 million displaced and approximately 600,000 people in relief camps. Among those, 8.2 million women and 16 million children are the worst affected, with many requiring urgent medical and reproductive healthcare. To plan an efficient healthcare program and a climate-resilient health system, it is crucial to understand the issues that the affected people face during floods. Methodology. This rapid assessment included the population in the most severely affected districts across the four provinces of Pakistan. A mixed methods approach using qualitative and quantitative techniques was utilized. A total of 52 qualitative, in-depth interviews were conducted with community-level healthcare providers, national and provincial government departments, and development partners involved in relief activities. Using a structured questionnaire, the quantitative cross-sectional survey was conducted with a final sample of 422 women, married and unmarried (15-49 years old), residing in the relief camps in the flood-affected areas. The outcome variable of the survey was the access to sexual and reproductive health services faced by the women in the flood-affected districts. Data collection took place four months postfloods during Nov-Dec 2022, while the data analysis was conducted between Dec 2022 and Jan 2023. The quantitative data was analyzed using SPSS (Statistical Package for the Social Sciences) version 20, and qualitative data was analyzed using NVivo 12. Ethical consent was sought from all the participants. Ethical approval was also sought from the ethics committee of the Health Services Academy, Government of Pakistan. Results: The findings indicated that, overall, all the provinces were unprepared for a calamity of such a large magnitude. Access to services and health data reporting from the flood-affected areas was challenging mainly due to a shortage of trained health workforce because of the displacement of a large volume of the health workforce. Overall, equipment, medicines, supplies, and food were scarce. Women residing in the camps were markedly affected, and 84% (375) were not satisfied with the flood relief services provided to them. The floods impacted their monthly income as 30% (132) of respondents started depending on charity postfloods. Almost 77% (344) reported limited access to sexual and reproductive health services and had yet to receive sanitary, hygiene, and delivery kits, while 69% (107 out of 154) of girls stopped schooling postfloods. Almost 77% (112) of the married women reported having a child less than one year of age. Yet, only 30% (44 out of 144 currently married women) were using any form of family planning method-damage to the health facilities affected access to overall maternal care services. Conclusion: The findings concluded that there was no planning for sexual and reproductive health services in the flood-affected areas. Several barriers were identified. The government and development partners needed to prepare to cater to women's needs during the floods. The findings highlight the need for collaborative efforts between the government, civil society, and development partners to address the challenges faced in disaster management and strengthen disaster management capacity.

Efficacy and Safety of Daratumumab, Pomalidomide, and Dexamethasone (DPd) Compared to Daratumumab, Bortezomib, and Dexamethasone (DVd) in Daratumumab-Naïve Relapsed Multiple Myeloma.

Daratumumab-based combinations with pomalidomide/dexamethasone (DPd), or bortezomib/dexamethasone (DVd), have shown activity in relapsed/refractory multiple myeloma (RRMM) patients. However, no direct comparisons of safety or efficacy of the two regimens have been published to date. We conducted a retrospective study to compare the safety and efficacy of DPd and DVd in daratumumab-naïve RRMM patients. We included 140 daratumumab-naïve patients who had received DPd or DVd for RRMM. Overall, the DPd group had a greater number of patients who had high-risk disease characteristics. Although response was deeper in the DPd group, the median progression-free survival (PFS) and overall survival (OS) were similar between the two groups. The DPd group exhibited a higher incidence of hematologic toxicities, whereas the DVd group had a higher incidence of peripheral neuropathy. The study results showed that while DPd may provide a deeper response, there was no significant difference in PFS or OS compared to DVd. For the high proportion of difficult-to-treat patients, duration of treatment may have contributed to these results, indicating that patient and disease characteristics should be considered when selecting salvage treatments.

A Cross-Sectional Study of Pediatric Feeding Disorder in Children with Cystic Fibrosis.

BACKGROUND: The exact prevalence of feeding problems in children with cystic fibrosis (CF) is unknown. Pediatric feeding disorder (PFD) encompasses poor oral intake with associated medical, nutrition, psychosocial, or feeding skill dysfunction. We hypothesized that PFD is common in CF and aimed to categorize feeding dysfunction across various domains in children with CF. METHODS: An observational cross-sectional study was conducted in children with CF. Data collected included anthropometrics, nutrition data (including need for tube feeding/enteral nutrition [EN] or high-energy beverages, dietary diversity), feeding skills (Pediatric version of the Eating Assessment tool [pEAT]), and psychosocial function (About Your Child's Eating questionnaire [AYCE] in children 2-17 years of age/Behavioral Pediatric Feeding Assessment Scale [BPFAS] in children 12-23 months of age). PFD was defined as poor oral intake with: (a) pEAT score > 5; and/or (b) AYCE or BPFAS score > 2 standard deviation of normative controls; and/or (c) nutrition dysfunction (body mass index/weight-for-length z score < -1 and/or preference of oral high energy beverages or dependence on EN and/or decreased dietary diversity). RESULTS: Of 103 children in the study, 62 (60.1%) had PFD, 7 children (6.8%) were malnourished, 10 needed EN (9.7%), and 30 (29.1%) needed oral high-energy beverages. Dietary diversity was decreased in 42 children (41.5%), 1 child had feeding skill dysfunction, and 11 (10.8%) met criteria for psychosocial dysfunction. CONCLUSION: Almost 2/3rd of children with CF have PFD and many have poor dietary diversity. A significant percentage of children rely on EN and oral supplements, but psychosocial dysfunction is less prevalent.

Hypereosinophilic Syndrome with Pulmonary Involvement in Ulcerative Colitis.

Reactive eosinophilia is associated with inflammatory bowel disease and is more common in patients with ulcerative colitis (UC) compared with Crohn's disease. The prevalence rate of peripheral blood eosinophilia in patients with inflammatory bowel disease has been described to be as high as 30%-40% of patients in a pediatric study. The coexistence of hypereosinophilic syndrome (HES) and UC is uncommon. We present a 15-year-old boy with UC associated with HES who presented with chest pain and shortness of breath. Laboratory evaluation showed marked eosinophilia. Alternative causes of eosinophilia including eosinophilic leukemia, infections, or drug-induced eosinophilic pneumonia were ruled out. The patient was ultimately diagnosed with HES responsive to mepolizumab.

Trichobezoar: A Rare Cause of Protein-losing Enteropathy.

Protein-losing enteropathy (PLE) is caused by protein loss through the gastrointestinal tract which results in hypoalbuminemia. The most common causes of PLE in children include cow milk protein allergy, celiac disease, inflammatory bowel disease, hypertrophic gastritis, intestinal lymphangiectasia, and right-sided heart dysfunction. We present a case of a 12-year-old male with bilateral lower extremity edema, hypoalbuminemia, elevated stool alpha-1-antitrypsin, and microcytic anemia. He was found to have a trichobezoar in the stomach extending to the jejunum, an unusual cause of PLE. The patient underwent an open laparotomy and gastrostomy to remove the bezoar. Follow-up confirmed resolution of hypoalbuminemia.

Color doppler ultrasound for diagnosis of testicular carcinoma: A comparison with gold standard histopathology.

Introduction: Testicular carcinoma is the most common cancer among males aged 15-34 years. The known risk factors for testicular cancer include undescended testis (cryptorchidism), testicular dysfunction, perinatal factors and prior history of cancer in one testis. We aimed to determine the diagnostic accuracy of color doppler ultrasound in diagnosis of testicular carcinoma using histopathology as GOLD STANDARD. Method: ology: A cross sectional study was conducted from July 2015 to Feb 2016 at the Department of Radiology, Jinnah Post Graduate Medical Center, Karachi. 311 subjects were selected through inpatient/outpatient or emergency department. Patients were evaluated for testicular carcinoma by color doppler ultrasound on Toshiba nemio. Finding of color doppler ultrasound was compared with histopathology. True positive, true negative, false positive, false negative as per operational definition was determined. Results: Mean age of the patients of the study was 41.76 ± 8.11 (30-50) and mean and SD of Duration of symptoms was 5.5 ± 3.5 (4-15) months. Of 175(56.27%) subjects diagnosed as testicular carcinoma on CDUS, only 160(48.55%) were subsequently found to have testicular carcinoma. sensitivity of CDUS in diagnosing scrotal diseases was 88.8% while specificity was 78.1%. Conclusion: We conclude that CDUS is an excellent, safe, and reliable method for evaluating patients with testicular carcinoma. It helps to improve patient's management, especially by preventing unnecessary surgical exploration. It is also convenient and easy to perform. But it has its own limitations, and requires adequate expertise and experience. Its results are also equipment dependent.

A retrospective observational study of biomarker levels and severity assessment in pediatric community-acquired pneumonia.

METHODS: A retrospective chart review was conducted on children (aged 60 days to 18 years) diagnosed with CAP, and admitted to a regional, tertiary hospital (Charleston, WV, USA) for 3 years (2015-2018). Patients were stratified into 2 severity cohorts, mild (no ICU care), and moderate/severe (required ICU care). Biomarker values were then compared between the severity cohorts and area under the curve (AUC), and cut-off values and performance characteristics were calculated. RESULTS: A total of 108 patients met inclusion criteria with 46% having moderate/severe CAP. Elevated levels of CRP (51.7 mg/L in mild vs. 104.8 mg/L in moderate/severe, P = .003, PCT (0.29 ng/ml in mild vs. 4.02 ng/mL in moderate/severe, P = .001) and band counts (8% in mild vs. 15% moderate/severe, P = .009) were associated with increased pneumonia severity. In predicting moderate/severe CAP, PCT had the highest AUC of 0.77 (P = .001) followed by bands AUC of 0.69 (P = .009) and CRP AUC of 0.67 (P = .003). Cut-off for PCT of 0.55 ng/mL had a sensitivity of 83% and a specificity of 65%. Cut-off level of 53.1 mg/L for CRP had a sensitivity of 79% and specificity of 52%. Cut off level of 12.5% bands had a sensitivity of 61% and specificity of 71%. In a multivariable model controlled for patient demographics and other biomarker levels, only PCT levels significantly predicted moderate/severe CAP (adjusted odds ratio: 1.40 [95% CI, 1.14-1.73], P = .002). CONCLUSION: Biomarkers, in particular PCT, obtained early in hospitalization may perform as possible predictors for CAP severity in children and be beneficial in guiding CAP management. However, biomarkers in pneumonia should not drive severity assessment or patient management independent of clinical presentation.

Childhood Obesity as a Predictor of Coronary Artery Disease in Adults: A Literature Review.

Obesity in children is becoming a worldwide epidemic that requires immediate attention. Despite all the efforts directed towards controlling this issue, its prevalence is increasing overtime both in developed and developing countries. With an increasing prevalence in the younger age groups, it is emerging as a public health crisis. A rise in body mass index (BMI) results in an increased risk of developing a variety of metabolic and cardiovascular diseases, particularly coronary artery disease (CAD). The early onset of the disease affects the peak productivity years in young individuals leading to disability at a later age. It makes it essential that we understand the contributory factors towards the development of obesity as a risk factor for CAD and develop strategies that hinder the progression towards adverse outcomes. There is an urgent need to screen these children at a younger age and educate them to change their lifestyle to decrease the BMI within the normal range to promote cardiovascular health. It requires a multidisciplinary approach involving dietary, physical, and behavioral-centered strategies. Failure to control this epidemic timely may cause widespread consequences for the quality of life and longevity.

Effects of Diet, Nutrition, and Exercise in Children With Autism and Autism Spectrum Disorder: A Literature Review.

Diet and nutrition play an important and essential role in everyone's life. It helps build a healthy body and a strong mind. We know that food rich in nutrients can remove toxins from the body, make an excellent immune system, curb hunger, and prevent obesity. Obesity is one of the most concerning, alarming, and fastest-growing pandemics. It affects not only adults but also adolescents and children. The disease's early-onset calls for prompt attention to control the physical, psychological, financial, and social burden it creates. Children with autism and autism spectrum disorders (ASDs) are commonly affected by eating disorders. Their preference for energy-dense food with low nutrition can alter their metabolism, leading to the accumulation of oxidative radicals, causing them to deteriorate mentally and physically. Although dieting and losing weight are now commonly seen in the general population, it has become hard to bring awareness to children with special needs about diet, nutrition, and obesity. Despite efforts, parents of such children usually cannot help control the eating because tantrums and behavioral problems are common. It is now imperative for doctors and parents to work alongside nutritionists and dieticians to help these children eat healthy to be fit and improve the quality of life.

Celiac Disease in a 16-Month-Old Child Presenting as Motor Regression.

Neurodevelopmental symptoms were previously believed to be a complication of celiac disease (CD) and rarely seen as presenting symptoms. One case has been reported so far where motor regression was the presenting symptom. We present a 16-month-old girl with postprandial vomiting and regression of motor skills. Examination revealed abdominal distension, hypotonia, and decreased motor movements in lower extremities. Celiac serology showed elevated tissue transglutaminase (tTG) immunoglobulin A (IgA) levels. Esophagogastroduodenoscopy with biopsies confirmed CD. Gluten-free diet led to the improvement of neurological and gastrointestinal complaints. We recommend keeping CD as one of the differentials in children with neurodevelopmental symptoms.

Systemic Lupus Erythematous Presenting as Catatonia and its Response to Electroconvulie Therapy.

Neuropsychiatric systemic lupus erythematous (SLE) encompasses various psychiatric and neurological manifestations that develop in SLE patients, secondary to involvement of central nervous system. Neuropsychiatric SLE, presenting as catatonia is very uncommon, and treatment of this condition is not well defined. Previously the role of benzodiazepines, immunosuppression, plasma exchange, and electroconvulsive therapy (ECT) has been described in its management. Here we describe a case of neuropsychiatric lupus presenting as catatonia that did not respond to benzodiazepines or immunosuppression. The symptoms of catatonia showed improvement with ECT. Furthermore, we have discussed the pathology of the disorder and the role of ECT in the treatment of cases of catatonia associated with SLE, who do not respond to benzodiazepines.