A GIRL WITH ADENOVIRUS TYPE 2 INDUCED INTERSTITIAL PNEUMONITIS ACUTELY EXACERBATED WITH PRIMARY HUMAN HERPESVIRUS 7 INFECTION.

We encountered a previously healthy 3-year-old girl with interstitial pneumonitis that initially developed due to human adenovirus type 2 infection and exacerbated by primary human herpesvirus 7 infection. A comprehensive serum biomarker analysis showed patterns that differed by viral infection, suggesting that respiratory and lymphotropic viral infections might have different pathophysiology in interstitial pneumonitis.

Practical guide for the diagnosis and management of primary ciliary dyskinesia.

OBJECTIVE: Primary ciliary dyskinesia (PCD) is a relatively rare genetic disorder that affects approximately 1 in 20,000 people. Approximately 50 genes are currently known to cause PCD. In light of differences in causative genes and the medical system in Japan compared with other countries, a practical guide was needed for the diagnosis and management of Japanese PCD patients. METHODS: An ad hoc academic committee was organized under the Japanese Rhinologic Society to produce a practical guide, with participation by committee members from several academic societies in Japan. The practical guide including diagnostic criteria for PCD was approved by the Japanese Rhinologic Society, Japanese Society of Otolaryngology-Head and Neck Surgery, Japanese Respiratory Society, and Japanese Society of Pediatric Pulmonology. RESULTS: The diagnostic criteria for PCD consist of six clinical features, six laboratory findings, differential diagnosis, and genetic testing. The diagnosis of PCD is categorized as definite, probable, or possible PCD based on a combination of the four items above. Diagnosis of definite PCD requires exclusion of cystic fibrosis and primary immunodeficiency, at least one of the six clinical features, and a positive result for at least one of the following: (1) Class 1 defect on electron microscopy of cilia, (2) pathogenic or likely pathogenic variants in a PCD-related gene, or (3) impairment of ciliary motility that can be repaired by correcting the causative gene variants in iPS cells established from the patient's peripheral blood cells. CONCLUSION: This practical guide provides clinicians with useful information for the diagnosis and management of PCD in Japan.

Invasive pneumococcal disease caused by non-vaccine Streptococcus pneumoniae serotype 24B in an immunocompetent child.

Invasive pneumococcal disease typically occurs in immunocompromised patients, although some vaccine strains of Streptococcus pneumoniae have been reported to cause invasive pneumococcal disease in immunocompetent vaccine recipients. In this study, we presented a case of a 16-month-old immunocompetent patient with lung abscess and empyema caused by nonvaccine S. pneumoniae serotype 24B. A consolidation occupying the right upper lobe in the chest computed tomography results, as observed at presentation, changed to thick-walled cavitary lesions at the end of a month of intravenous antibiotics, and antibiotics were continued for a total of two months. To the best of our knowledge this is the first report that focuses on the risk of invasive pneumococcal disease caused by S. pneumoniae serotype 24B in an immunocompetent child.

Comparison of Postoperative Respiratory Function Between Patients After Thoracoscopic and Open Lobectomy.

Purpose: This study aimed to compare respiratory functions of patients after thoracoscopic lobectomy (TS) with those after thoracotomy (TR). Methods: This retrospective study was conducted in two centers, one of which adapted TS as a standard procedure in 2009 and the other performs it via TR. Data on patients who underwent lobectomy for congenital lung disease between 2009 and 2021 and underwent pulmonary function test (spirometry) were collected. Results: Ten patients underwent TS and 36 underwent TR. Distribution based on sex, prenatal diagnosis, pathological diagnosis, and resected lobe were similar between the two groups. The median [interquartile range] age at procedure in the TR group was significantly smaller than that in the TS group (13 [11-18] months versus 38 [13-79] months, P = .03). The procedure duration in the TR group was significantly shorter than that in the TS group (230 [171-264] minutes versus 264 [226-420] minutes, P = .02). Pulmonary function test was conducted at the age of eight in both groups, but the interval between the procedure, and the test was significantly shorter in the TS group (TR: 7 [5-8] years versus TS: 5 [2-7] years, P = .03). The ratio of forced vital capacity compared to predicted one (TR: 86.6 [76.6-95.3] versus TS: 88.7 [86.8-89.1], P = .58) and the ratio of forced expiratory volume in 1 second against that predicted (TR: 84.0 [80.5-88.7] versus TS: 88.7 [86.8-89.1], P = .08) were not significantly different between the two groups. Conclusions: Although TR was performed earlier than TS, respiratory function was similar between the two groups.

Successful rituximab treatment for severe rapidly progressive interstitial lung disease with anti-MDA5 antibody-positive juvenile dermatomyositis: a case report and literature review.

BACKGROUND: Rapidly progressive (RP) interstitial lung disease (ILD) is a life-threatening complication of juvenile dermatomyositis (JDM); however, it is generally refractory to treatment; to the best of our knowledge, no evidence-based treatment has been established for RP-ILD yet. We present the case of a 2-year-old girl with RP-ILD who showed resistance to treatment with methylprednisolone, cyclosporine A, cyclophosphamide, immunoglobulin, and plasma exchange (PE) and was finally treated with extracorporeal membrane oxygenation. We further present a literature review of 18 cases of JDM with RP-ILD. CASE PRESENTATION: A 2-year-old girl presented with malar rash, mild muscle weakness, and weight loss for a few months before admission. She had a history of dry cough and dyspnea for a few days, followed by rapid respiratory failure. The patient was diagnosed with JDM with RP-ILD through physical examination (malar rashes and Gottron's sign) and based on the finding of myositis on femoral magnetic resonance imaging, elevated levels of serum muscle enzymes, positive anti-melanoma differentiation-association gene 5 (MDA5) antibody (> 7,500 index), elevated level of Krebs von den Lungen-6 glycoprotein (KL-6; 3,420 U/mL), and extensive ground-glass opacities with consolidation in the bilateral lungs on chest high-resolution computed tomography. She received combination therapy, including methylprednisolone pulse therapy, followed by oral prednisolone and intravenous cyclosporine A, cyclophosphamide, and immunoglobulin. On day 11 of hospitalization, she was placed on ventilation support and PE was initiated. However, her respiratory condition continued to deteriorate and veno-venous extracorporeal membrane oxygenation was started on day 24 of hospitalization. Rituximab was administered on day 28. After 2 weeks of rituximab therapy initiation, her respiratory condition showed gradual improvements. Eventually, on day 52 of hospitalization, the patient could be weaned off extracorporeal membrane oxygenation. Finally, she was discharged with minimal ventilation support and no neurological complications 11 months after admission. CONCLUSIONS: Our literature review suggest that JDM with RP-ILD has a high mortality rate. In JDM, rituximab may be a promising treatment option for RP-ILD. In the future, the efficacy of rituximab in the early phases of ILD should be investigated.

Post-esophageal atresia repair double acquired tracheoesophageal fistulas treated successfully by gastric transposition: a case report.

BACKGROUND: Postoperative recurrence of tracheoesophageal fistula (TEF) is a frequent complication in the repair of esophageal atresia (EA). Based on the recent etiologic classification, a TEF that develops in a different new pathway from the original one is categorized as an acquired TEF. The TEFs that fall into this category have been reported to be refractory and their mechanisms have not been fully understood. Here, we report the complicated case of an acquired TEF derived from mediastinitis after the original TEF repair developed an anastomotic stricture. The TEF contained double fistulas, both towards the right lobe bronchi, and was repaired by gastric transposition through a retrosternal route. CASE PRESENTATION: The patient was diagnosed with Gross C esophageal atresia after birth and underwent tracheoesophageal fistula banding during the neonatal period. He experienced an intractable anastomotic stenosis after surgery which was treated with repeated balloon dilation therapy. By the age of 11 months, he developed a mediastinal abscess that improved with conservative treatment. At 18 months old, a fistula from the esophagus to the right superior lobe bronchus was identified. The patient underwent a right upper lobectomy to resect the fistula. However, at 21 months old, another fistula to the right lower lobe was revealed. An esophageal banding was done to relieve the respiratory symptoms. This was followed by esophagectomy and gastric transposition through the retrosternal route at 26 months old. The patient started rehabilitation and oral intake gradually after surgery. By 3 years after gastric transposition, he could already take blended food orally with the support of small amounts of enteral feeding. CONCLUSION: Cases of TEF derived from severe inflammation have the potential to form a complicated network and lead to recurrence. Surgeons should consider the possibility of multiple tiny fistulas in cases of severe acquired TEF. These may be repaired successfully by gastric transposition through the retrosternal route.

Round pneumonia due to Chlamydia pneumoniae in a child.

Round pneumonia is a disease commonly recognized in the pediatric age group, especially under the age of 8 years. Streptococcus pneumoniae is the most common agent causing this characteristic type of pneumonia. We herein report a case of a 6-year-old boy with only mild cough and low-grade fever who was then diagnosed with round pneumonia due to Chlamydia pneumoniae. He had multiple round opacities in the right lower lung field, and the symptoms subsided soon after oral administration of antibiotics.

A long-term survival case of tracheal agenesis: management for tracheoesophageal fistula and esophageal reconstruction.

Tracheal agenesis is a very rare disorder which leads to severe respiratory disorders immediately after birth. Reports are very limited on long-term survival cases. We report here a long-term survival case with Floyd's type I tracheal agenesis. During the neonatal stage, the patient underwent abdominal esophageal banding to substitute esophagus for trachea and transection at the cervical esophagus with esophagostomy. Subsequently, airway management was difficult due to a fragile tracheoesophageal fistula, but the fistula was conservatively treated and stabilized with the patient's growth. This patient is a very rare case in whom oral feeding was achieved after esophageal reconstruction using a gastric tube. For this case, we describe mainly (1) the management method of the tracheoesophageal fistula and (2) esophageal reconstruction without thoracotomy.

Upper airway obstruction in neonates and infants with CHARGE syndrome.

Upper airway obstruction can be life-threatening in neonates and infants with CHARGE syndrome, many of whom undergo intratracheal intubation early in life. Although some of these patients are successfully extubated, others require tracheotomy. Deciding whether to complete tracheotomy is challenging since there are no clear criteria upon which to base this decision. We assessed 10 infants with CHARGE syndrome, 5 of whom required tracheotomy. Fiberoptic laryngoscopy showed that all of these patients shared certain features: anteroposterior flattening of the larynx; short vocal cords; anteriorly positioned, tall and hypertrophic arytenoids obscuring the glottis; uncoordinated movement of the vocal cords, epiglottis and arytenoids; salivary pooling. In addition, we observed only in those requiring tracheotomy an obstructive supraglottis that prevented visualization of the vocal cords throughout respiration. Salivary retention was much more severe in this group. These findings might be helpful for predicting the need for an early tracheotomy in situations where the vocal cords are not visible throughout the entire respiratory cycle. Given the high prevalence of malformed larynx and abnormal cranial nerve function, which are not alleviated by supraglottoplasty, we suggest that a thorough investigation of upper airway obstructive entities other than laryngomalacia be performed before embarking on supraglottoplasty in patients with CHARGE syndrome.

X-linked severe combined immunodeficiency (X-SCID) with high blood levels of immunoglobulins and Aspergillus pneumonia successfully treated with micafangin followed by unrelated cord blood stem cell transplantation.

In this report, we describe a patient with X-linked severe combined immunodeficiency (X-SCID) who had high serum IgG, IgA, and IgM levels. The boy did well until 6 months of age, when he developed interstitial pneumonia caused by Aspergillus species, with a white cell count of 12,840/microL and only 10% lymphocytes; IgG, 991 mg/dL; IgA, 65 mg/dL; IgM, 472 mg/dL. Cell markers showed only 6.3% CD3, 2.1% CD4, 0.7% CD8, but 92% CD19 and 0.1% CD16+CD56+ (NK cells). A mutation was detected within exon 2 (C196 A-->C), leading to the substitution of proline for glutamine, which has not been reported previously. The boy was successfully treated with the new antifungal drug, micafangin (MCFG), at 5 mg/kg/day for 89 days. After resolution of the pneumonia, the patient underwent successful hematopoietic stem cell transplantation with completely matched unrelated female cord blood. The CD34 stem cell dose was 3.4 x 10(4) cells/kg. In conclusion, MCFG can be a first line agent for Aspergillus pneumonia in immunocompromised hosts.

Congenital bronchial atresia in infants and children.

BACKGROUND/PURPOSE: Congenital bronchial atresia (CBA) usually presents incidentally in asymptomatic young male adults but is rarely diagnosed in children. The aim of this study was to clarify the clinical characteristics of CBA in childhood and to describe the spectrum of this condition. METHODS: The clinical features in 29 patients with CBA, aged from 1 day to 13 years (median, 4 years), were reviewed retrospectively. Diagnosis was confirmed by pathological findings of a blind-ending bronchus associated with distal mucous-filled bronchocele surrounded by hyperinflated lung parenchyma. RESULTS: All but 1 patient were symptomatic. The most frequent symptom was productive cough and fever owing to recurrent pneumonia found in 26 children. Two infants suffered from respiratory distress. Chest x-ray showed various findings of infiltrative pneumonia, emphysema, and a large cyst. Computed tomography, bronchography, and bronchoscopy were useful modalities for demonstrating bronchocele associated with hyperinflated lung or proximal blind-ending bronchus even in infected cases. The right lower lobe was predominantly affected in 12 cases, followed by left or right upper lobe in 7 cases. Lobectomy or segmentectomy resulted in remarkable clinical improvement. CONCLUSIONS: Congenital bronchial atresia presents differently in children than in young adults. Modern imaging techniques and careful pathological analyses lead to an accurate diagnosis of bronchial atresia, which may be misdiagnosed as intralobar sequestration or pulmonary bronchial cysts. Bronchial atresia is a distinct pathological entity that accounts for recurrent pneumonia or respiratory distress in childhood, requiring surgical treatment.