Congenital Skin Rashes in an IVF Baby Progressed to Multisystem Langerhans Cell Histiocytosis with Lung and Bone Involvement: A Case Report and Literature Review.

Langerhans cell histiocytosis is an uncommon proliferative disorder that may influence many organs; so, the clinical presentations vary. Here we describe an 85-day-old female who was born with In vitro fertilization after 10 years of infertility. She referred to us due to severe pulmonary insufficiency and congenital progressive maculopapular rash with desquamation. There were significant cystic changes in chest imaging studies. Further evaluation demonstrated lytic lesions in cranial, femoral, and humorous bones. The skin biopsy verified the diagnosis of LCH. A combination of Vinblastine, VP16, and Dexamethasone regimen was applied for the patient. In the course of the disease, she encountered multiple bilateral pneumothoraxes but didn't respond to tube thoracostomy and chemotherapy management. The patient died due to respiratory failure raised from complications of lung involvement as a multisystem LCH, 29 days later. Pediatricians should pay much more attention to the cutaneous lesions in the neonatal period especially if there is any risk factor for presenting LCH such as IVF. The lesions should be monitored closely owing to a high correlation between skin lesions and MS LCH.

Combination of Serologic Tests and Sputum Culture Results as the Best Diagnostic Method of Pseudomonas aeruginosa Infection in Cystic Fibrosis Patients.

Background: One of the most common reasons for mortality in patients with cystic fibrosis (CF) is lung infections, among which Pseudomonas aeruginosa (Pa) infection has the largest share. Diagnosis of Pa can be assessed by various methods such as sputum culture results and IgG antibody level via measuring the specific anti-Pa antibodies. This study aimed to select the best predictive technique in the diagnosis of Pa in CF patients through spirometry, sputum culture, and serum IgG antibody levels. Materials and Methods: In this cross-sectional study, blood and sputum or pharyngeal samples were taken from 68 patients with cystic fibrosis. Because spirometry was not possible in all patients, 34 patients could do the spirometry. The samples were studied concerning Pa infection. The data including variables such as age, sex, and spirometry results were obtained. Then, in the serologic method, 3 serum-specific antibody levels were determined by enzyme-linked immune sorbent assay (ELISA). Results: The average age of children was 7.4 ± 5.6 (ranging from 0.5 to 23) years. Generally, the percentage of Pa infection increased in CF patients with higher ages. A statistically direct significant relationship was observed between the concentration of serum IgG antibodies in patients with CF and Pa-positive sputum culture results (p<0.05). Conclusion: Serum IgG antibodies against specific Pa antigens could be a diagnostic method against Pa infection, especially in patients who cannot expectorate. However, because of the positive and negative predictive value of both serum IgG antibody levels and the results of the sputum culture, we suggested that utilizing the combination of these methods could be beneficial in earlier diagnosis of Pa.

A Rare Case of Pulmonary Adenocarcinoma in an 8-Year-Old Patient with Persistent Respiratory Manifestation: A Case Report Study.

Pulmonary adenocarcinoma is an extremely infrequent cancer in children. This cancer usually presents with unspecific manifestations that lead to delays in diagnosis. The treatment protocol for adenocarcinoma in children remains challenging due to its rarity. We presented an 8 years old with a chief complaint of a non-purulent cough, dyspnea, hemoptysis, and weight loss. Decreased lung sounds and wheezing in the left lung were heard during auscultation. The radiographic evaluation showed a mediastinal mass in the left middle upper. A biopsy was performed, and adenocarcinoma was reported. Based on being at stage 1, a lobectomy was the proper treatment for her. Although adenocarcinoma is rare in pediatrics, we suggested that physicians consider taking chest X-rays in patients with persistent respiratory manifestations, especially those with critical symptoms. Early detection leads to diagnosing patients in lower stages, which results in a good prognosis and better treatment outcomes.

A case series of pediatric COVID-19 with complicated symptoms in Iran.

People in different age groups are susceptible to SARS-CoV-2 infection as a newly emerging virus. However, the clinical course, symptoms and disease outcome vary from case to case. Although COVID-19 is usually milder in children than adults, some studies reported nonspecific symptoms. Here, we report eight pediatric cases of COVID-19 admitted in the Taleghani Children Hospital in Gorgan city, north of Iran, with complicated symptoms. The current case series poses several challenges to the pediatricians regarding the pediatric cases of COVID-19. As most literature relating to adults are not always transferable to children, clinicians should be warned about such presentations among children with COVID-19.

A Case Report of ROHHAD Syndrome in an 8-year-old Iranian Boy.

INTRODUCTION: Rapid-onset obesity concurrently with hypoventilation, hypothalamic, autonomic dysregulation (ROHHAD) is an uncommon disease that presents with multiorgan disorders during early childhood, with fewer than 100 cases reported around the world. We aim to present a case of ROHHAD syndrome admitting with rare neurologic symptoms. We also present our treatment regimen. CASE PRESENTATION: An 8-year-old boy was admitted to our department with ataxia and gait disturbance that led us to the final diagnosis after a thorough investigation. He had multiple admissions and was treated for other diagnoses. His first symptoms started from age 5 with obstructive apnea. He underwent an adenectomy surgery at that time, but the symptoms continued. A year after the surgery, he was admitted again due to his somnolence but was diagnosed only with hypothyroidism and anemia. At the age of 7 years and 8 months, he was admitted to our department with ataxia and abnormal gait from the past year with instability and numerous falls. He also had shown hyperphagia that had been resulted in 10 kilograms of weight gain in six months. He was experiencing gradual behavioral symptoms, including episodes of self and hetero aggression and impulsivity. His other symptoms included fatigue, somnolence, gastrointestinal dysmotility, hyperhidrosis, central hypothyroidism, polyuria, precocious puberty, and rapid obesity. His laboratory investigation revealed hyperprolactinemia. CONCLUSIONS: Our case indicates that ROHHAD is a complex disease with divergent signs and symptoms that needs to be kept in mind for diagnosis and should be treated with a high level of collaboration of various medical specialties. Since late diagnosis of this syndrome leads to a magnificent increase in morbidity and mortality rates, it is vital to pay extreme attention to this syndrome. The diagnosis should be considered even more in children over two years old with rapid-onset obesity, which is accompanied by other symptoms. Here, our patient's complaint was ataxia that revealed the underlying cause after investigation.

The role of IL-6 for predicting neonatal sepsis: a systematic review and meta-analysis.

OBJECTIVE: Neonatal sepsis (NS) is a common and life-threatening disorder in infants. Previous studies showed that interleukin-6 (IL-6) may be a valid non-invasive and rapid method for diagnosis of NS. We conducted this review to assess the validity of IL-6 for predicting NS. METHODS: This was a systematic review with meta-analysis. Embase, Medline and Web of Science databases were searched between January 1990 and December 2009. The search terms used were "cytokine", "neonate", "sepsis" and "interleukin-6". We used standard methods recommended for meta analyses of diagnostic test evaluations. The analysis was based on a summary ROC (SROC) curve. Meta-regression analysis was used to assess the effects of some confounding factors on the results of meta-analysis. Potential presence of publication bias was tested using funnel plots and the Egger test. FINDINGS: Meta-analysis was performed on 13 publications including 353 infants with sepsis and 691 control infants. The pooled sensitivity and specificity of IL-6 was 0.79 and 0.84, respectively. The maximum joint sensitivity and specificity (i.e., the Q value) in SROC curve was 0.82 and the area under curve (AUC) was 0.89 (95% CI: 0.84-0.94). Meta-regression analysis showed that the diagnostic accuracy of IL-6 was not affected by confounding variables. The evaluation of publication bias showed that the Egger test was not significant (P=0.07). CONCLUSION: IL-6 seems to be a valid marker for predicting NS. It may be considered for early diagnosis of sepsis in neonatal care units.