Accessory cardiac bronchus with associated lung parenchyma: rare congenital tracheobronchial anomaly.

Accessory cardiac bronchus (ACB) has been described mainly as case reports finding (frequency 0.08%-0.39%). Even though 50% of all ACBs have a blind extremity, imaging studies have demonstrated that some develop into a series of bronchioles with cystic degeneration or a ventilated lobule demarcated by an anomalous fissure and extremely rare with an abnormal pulmonary artery. In this case, ACB was demonstrated on several imaging methods arising from the intermediate bronchus's medial wall with correspondent blood vessels and fissure. Although an ACB is not a pathological entity and most patients with ACB are asymptomatic, it can become symptomatic due to recurrent infection, empyema, hemoptysis, and malignant transformation.  In conclusion, both pulmonologists and radiologists should recognize normal bronchial anatomy and developmental bronchial anomalies, as these may be important to establish a correct diagnosis.

Case Report: Advanced Skeletal Muscle Imaging in S-Adenosylhomocysteine Hydrolase Deficiency and Further Insight Into Muscle Pathology.

Introduction: S-Adenosylhomocysteine hydrolase deficiency (SAHHD) is a rare inherited multisystemic disease with muscle involvement as one of the most prominent and poorly understood features. To get better insight into muscle involvement, skeletal muscles were analyzed by magnetic resonance imaging (MRI) and MR spectroscopy (MRS) in three brothers with SAHHD in the different age group. Method: The study was based on analysis of MRI and MRS of skeletal muscles of the lower and the proximal muscle groups of the upper extremities in three SAHHD patients. Results: Three siblings presented in early infancy with similar signs and symptoms, including motor developmental delay. All manifested myopathy, more pronounced in the lower extremities and the proximal skeletal muscle groups, and permanently elevated creatine kinase. At the time of MRI and MRS study, the brothers were at the age of 13, 11, and 8 years, respectively. MRI revealed lipid infiltration, and the MRS curve showed an elevated muscle lipid fraction (higher peak of lipid), which increased with age, and was more prominent in the proximal skeletal muscles of the lower extremities. These results were consistent with muscle biopsy findings in two of them, while the third patient had no specific pathological changes in the examined muscle tissue. Conclusions: These findings demonstrate that an accessible and non-invasive method of MRI and MRS is useful for an insight into the extent of muscle involvement, monitoring disease progression, and response to treatment in SAHHD.

Risk factors for nontuberculous mycobacterial pulmonary disease (NTM-PD) in Croatia.

BACKGROUND: The incidence, geographical distribution and clinical relevance of different nontuberculous mycobacteria (NTM) in Croatia are well described. There are few data on the risk factors for developing NTM pulmonary disease (NTM-PD) in this setting. METHODS: We conducted a retrospective cohort study on all Croatian residents with NTM isolated from respiratory samples in the period from 2006 to 2015 with follow-up to 2018. The American Thoracic Society/Infectious Diseases Society of America (ATS/IDSA) guidelines were used to establish NTM-PD diagnosis. Clinical, radiological and treatment data were collected from hospital records. RESULTS: Risk analysis calculations were made on the 439 isolation episodes that were classified as definitive NTM-PD (n = 137) or no disease (n = 302). Female gender, presence of bronchiectasis, low BMI and long-term systemic corticosteroid treatment were independent risk factors associated with NTM-PD. Hemoptysis and malaise were presenting symptoms independently associated with NTM-PD. Chronic obstructive pulmonary disease (COPD) and low/moderate dose inhaled corticosteroid (ICS) treatment were not associated with NTM-PD. High dose ICS treatment was a significant risk factor for developing NTM-PD (aOR = 4.73, CI 1.69-13.23 p = 0.003). CONCLUSION: The NTM-PD patients in Croatia are similar to those in other published cohorts in terms of their characteristics and risk factors. The significant dose-dependent association between ICS use and NTM-PD adds to the body of evidence suggesting that high dose ICS use is associated with NTM-PD.

Nodular distribution pattern on chest computed tomography (CT) in patients diagnosed with nontuberculous mycobacteria (NTM) infections.

BACKGROUND: This study evaluated the prevalence of spreading pathways in nontuberculous mycobacteria (NTM) pulmonary infections according to nodular distribution patterns seen on chest computed tomography (CT). METHODS: This study included 63 patients diagnosed with NTM lung infections who underwent CT at our institution. A retrospective analysis of CT images focused on the presence and distribution of nodules, presence of intrathoracic lymphadenopathy and the predominant side of infection in the lungs. The findings were classified into five groups; centrilobular (bronchogenic spread), perilymphatic (lymphangitic spread), random (hematogenous spread), combined pattern and no nodules present. The groups were then compared according to other CT findings. RESULTS: Among 51 (81%) patients identified with a nodular pattern on chest CT, 25 (39.8%) presented with centrilobular, 7 (11.1%) with perilymphatic, 6 (9.5%) with random and 13 (20.6%) with combined nodular patterns but located in different areas of the lungs. The right side of the lungs was predominant in 38 cases (60.3%). Intrathoracic lymphadenopathy was evident in 20 patients (31.7%). Significant differences in distributions of nodular patterns were seen in patients infected with Mycoplasma avium complex (MAC) associated with centrilobular pattern (p = 0.0019) and M. fortuitum associated with random pattern (p = 0.0004). Some of the findings were related to perilymphatic nodules between other isolated species of NTM (p = 0.0379). CONCLUSION: The results of this study showed a high proportion of perilymphatic nodules and right-sided predominance in the upper lobe, which, combined with intrathoracic lymphadenopathy is highly suggestive of the lymphangitic spread of lung NTM infections.