Sensory and Motor Median Nerve Neuropathy Due to a Deep Giant Hand Lipoma: A Case Report.

Lipomas are the most common soft-tissue tumors in the human body, but their location in the hand is rare. Symptomatic hand lipomas, due to nerve compression, are even rarer. We present a case of median nerve neuropathy as a result of a giant palm lipoma, located on the thenar and hypothenar areas of the hand. The patient had typical symptoms of carpal tunnel syndrome, along with compromised thumb motion. Intraoperatively, the recurrent motor branch of the median nerve was sitting on the lipoma under a great tension. This particular location of the motor branch of the median nerve in relation to the lipoma makes this case unique. The tumor was excised protecting the neurovascular structures, and a few weeks later the patient regained full thumb motion, grip strength, and resolution of dysesthesia.

Ventilation efficiency to exercise in patients with cystic fibrosis.

INTRODUCTION: Exercise ventilation efficiency index in cardiopulmonary exercise testing (CPET) is elevated in patients with heart failure providing useful information on disease progression and prognosis. Few data, however, exist for ventilation efficiency index among cystic fibrosis (CF) patients. AIMS: To assess ventilation efficiency index (ΔVE/ΔVCO2 or V'E/V'CO2 slope) and intercept of ventilation (VE-intercept) in CF patients with mild, moderate, and severe cystic fibrosis (CF) lung disease. To assess possible correlations with ventilation inhomogeneity and structural damages as seen on high resolution computed tomography (HRCT). METHODS: CF patients with mild (FEV1 > 80%, n = 47), moderate (60% < FEV1 < 80%, n = 21), and severe (FEV1 < 60%, n = 9) lung disease, mean age 14.9 years participated. Peak oxygen uptake (VO2 peak), pulmonary ventilation at peak exercise (VE), respiratory equivalent ratios for oxygen and carbon dioxide at peak exercise (VE/VO2 , VE/VCO2 ), end-tidal CO2 (PetCO2 ), and ΔVE/ΔVCO2 , ΔVE/ΔVO2 in a maximal CPET along with spirometry and multiple breath washout indices were examined. HRCT scans were performed and scored using Bhalla score. RESULTS: Mean ΔVE/ΔVCO2 showed no significant differences among the three groups (P = .503). Mean VEint discriminated significantly among the different groups (p 2 < 0.001). Ventilation efficiency index did not correlate either with LCI or Bhalla score. However, VE together with ΔVE/ΔVCO2 slope could predict Bhalla score (r 2 = 0.869, P = .006). CONCLUSION: No significant differences were found regarding ΔVE/ΔVCO2 slope levels between the three groups. Ventilation intercept (VEint ) was elevated significantly as disease progresses reflecting increased dead space ventilation. CF patients retain their ventilation efficiency to exercise even as lung function deteriorates by adopting a higher respiratory rate along with increased dead space ventilation.

Two cases of myositis ossificans in children, after prolonged immobilization.

Myossitis ossificans (MO) is a benign disorder characterized by heterotopic bone formation in skeletal muscle. It is divided in three types, fibrodysplasia ossificans progressive (FOP), myositis ossificans circumscripta or traumatica (MOT) and myositis ossificans without a history of trauma (non traumatic or pseudomalignant MO). Myositis ossificans is extremely rare in children younger than 10 years. We present the clinical and radiological findings of two 5-year-old children with pseudomalignant MO due to prolonged immobilization. Plain x-ray films and CT scan with their characteristic findings of mature bone in the periphery of the lesion with smooth contour and well separated from the bone, enabled us to diagnose the lesion. To the best of our knowledge, no such cases have been reported in the literature.

Chest wall lipoblastoma in a 3 year-old boy.

BACKGROUND: Lipoblastoma is a rare, benign, fatty tissue tumor that occurs in infancy and early childhood. The most common tumor locations are the extremities and the torso. The location of this tumor in the chest wall and an intrathoracic extension is uncommon. CASE REPORT: We present a case of a 3-year-old boy with anterior chest wall lipoblastoma with an intrathoracic extension. Computed tomography was suggestive of lipoblastoma. The mass was completely excised through a right posterolateral thoracotomy. The histologic examination of the lesion confirmed the diagnosis of lipoblastoma. CONCLUSION: Although extremely rare, chest wall lipoblastoma should be included in the differential diagnosis of thoracic mass in childhood.

Does Pseudomonas aeruginosa Colonization Affect Exercise Capacity in CF?

INTRODUCTION: Cardio-Pulmonary Exercise Testing (CPET) has been recognized as a valuable method in assessing disease burden and exercise capacity among CF patients. AIM: To evaluate whether Pseudomonas aeruginosa colonization status affects Exercise Capacity, LCI and High-Resolution Computed Tomography (HRCT) indices among patients with CF; to check if Pseudomonas colonization can predict exercise intolerance. SUBJECTS: Seventy-eight (78) children and adults with CF (31 males) mean (range) age 17.08 (6.75; 24.25) performed spirometry, Multiple Breath Washout (MBW) and CPET along with HRCT on the same day during their admission or follow up visit. RESULTS: 78 CF patients (mean FEV1: 83.3% mean LCI: 10.9 and mean VO2 peak: 79.1%) were evaluated: 33 were chronically colonized with Pseudomonas aeruginosa, 24 were intermittently colonized whereas 21 were Pseudomonas free. Statistically significant differences were observed among the three groups in: peak oxygen uptake % predicted (VO2 peak% (p < 0.001), LCI (p < 0.001), as well as FEV1% (p < 0.001) and FVC% (p < 0.001). Pseudomonas colonization could predict VO2 peak% (p < 0.001, r 2: -0.395). CONCLUSION: Exercise capacity as reflected by peak oxygen uptake is reduced in Pseudomonas colonized patients and reflects lung structural damages as shown on HRCT. Pseudomonas colonization could predict exercise limitation among CF patients.

A Case Report of Pulmonary Exacerbation after Initiation of Lumacaftor/Ivacaftor Therapy in a CF Female with Complicated Lung Disease.

Novel targeted treatments for Cystic Fibrosis give rise to new hope for an ever-growing number of CF patients with various mutations. However, very little evidence and guidelines exist to steer clinical decisions regarding patients whose illness takes an unexpected course. In such cases, the benefits and risks of discontinuing these treatments must be carefully and individually weighed, since their long-term effects remain mainly uncharted territory. In this report we document the case of a homozygous F508del CF patient with severe lung disease who presented with a pulmonary exacerbation shortly after the beginning of treatment with lumacaftor/ivacaftor and the complicated initial phase of therapy, which was followed by significant improvements.

Primary aldosteronism in patients with adrenal incidentaloma: Is screening appropriate for everyone?

Primary aldosteronism (PA) is a common form of secondary hypertension. Several guidelines recommend that patients with adrenal incidentaloma have a high probability of suffering from PA. We conducted a prospective study of 269 consecutive adults with adrenal incidentaloma to investigate the prevalence and clinical characteristics of PA. In total, 9 participants were detected with PA, suggesting a prevalence of 3.35% among the study population. PA participants had a higher blood pressure level by 14/20.8 mm Hg and a lower serum potassium level by 0.8 mmol/L (P < .05). Importantly, all patients with PA presented with concurrent indications (hypertension with or without hypokalemia) for screening of the disease, but they have not undergone relative screening by the referring physician, thus casting doubts about the appropriate implementation of current guidelines in real-life practice. Intense efforts are needed to familiarize physicians with recommendations for PA to minimize undiagnosed cases and the detrimental sequelae of this endocrine form of hypertension.

Exercise responses are related to structural lung damage in CF pulmonary disease.

INTRODUCTION: Early detection of lung disease is a primary objective in monitoring patients with Cystic Fibrosis (CF); High-Resolution-Computed-Tomography (HRCT) assesses structural damage. Spirometry and cardiopulmonary exercise testing are used for functional evaluation of CF lung disease. AIM: To evaluate the deterioration of exercise testing parameters over a 2-year period compared to the change of spirometry and HRCT parameters among CF patients. METHODS: Twenty-eight CF patients were evaluated with HRCT, spirometry, and exercise testing; 15 had two assessments with an interval of 2 years. Correlation analyses between Bhalla score parameters and functional measures were performed. RESULTS: Twenty-eight patients with CF (mean age 14.9 years, mean forced expiratory volume in 1 sec [FEV1 ] 83.2%) were evaluated. FEV1 was not found to change significantly in the 2-year period (P = 0.612). Both mean Bhalla score and mean peak oxygen consumption (VO2 peak %) deteriorated significantly (P = 0.014 and P = 0.026, respectively). VO2 peak and respiratory equivalents for O2 and CO2 at peak exercise were found to be significant predictors of Bhalla score (r = -0.477, P = 0.010; r = 0.461, P = 0.018; r = 0.402; P = 0.042, respectively). Anaerobic threshold was associated with changes in Bhalla score over the following 2 years. CONCLUSIONS: Exercise testing is more sensitive than spirometry to detect structural changes in CF lungs. Pediatr Pulmonol. 2016; 51:914-920. © 2016 Wiley Periodicals, Inc.