Respiratory failure in a patient with hypophosphatemic rickets: can an endobronchial stent make the difference?

Abnormalities associated with phosphate metabolism can lead to thoracic deformities that result in respiratory failure, which is conventionally managed by means of supplemental oxygenation, positive airway pressure and physiotherapy. However, when these measures fail, the clinician faces a dilemma, since many patients cannot tolerate a major surgical procedure. A minimally invasive technique, insertion of an endobronchial stent, might offer a solution.

Risk factors for intensive care admission in patients with COVID-19 pneumonia: A retrospective study.

BACKGROUND: Coronavirus disease 2019 (COVID-19)-associated mortality is predominantly due to respiratory failure. However, risk factors and predictive models for disease progression in patients with COVID-19 are not consistent across the globe. In this study, we aimed to assess the risk factors associated with intensive care (ICU) admission and mortality in patients with COVID-19 pneumonia. METHODS: Information was retrieved from the database of all patients admitted with COVID-19 pneumonia between March 2020 and July 2020 at a tertiary care center in Saudi Arabia. The patients' demographic, clinical, laboratory and radiological characteristics were analyzed. RESULTS: Of 1054 patients admitted with PCR proven COVID-19, 254 patients (24%) with radiological evidence of pneumonia were enrolled. The median age was 55, with 25.6% above 65 years and 55.1% males. The comorbidities included hypertension (45%), diabetes (43%), dyslipidemia (24%), solid organ and bone marrow transplantation (14.5%), malignancy (13.4%), ischemic heart disease (10.6%) and chronic kidney disease (9.4%). The mortality rate was 4.7%, and 22.8% were admitted to the ICU. The risk factors for ICU admission were> 65 years of age (RR: 1.74, CI 95%, 1.10-2.74, p = 0.017), diabetes melitus (RR: 1.66, CI 95% 1.06-2.62, p = 0.028), heart failure (RR: 2.51, CI 95%, 1.28-4.93, p = 0.007), respiratory rate> 25 (RR: 2.75, CI 95%, 1.66-4.55, p < 0.001), upper lobe involvement (RR: 1.68, CI 95%, 1.02-2.77, p = 0.043), and C-reactive protein (CRP)> 140 (RR: 1.89, CI 95%, 1.14-3.13, p = 0.013). The risk factors for mortality were> 65 years of age (RR: 5.82, CI 95%, 1.81-18.68, p = 0.003), upper lobe involvement on chest radiography (RR:4.40, CI 95%, 1.22-15.86, p = 0.016), diffuse chest computed tomography changes (RR: 7.36, CI 95%, 2.31-23.46, p < 0.011), ischemic heart disease (RR: 4.20, CI 95%, 1.36-13.04, p = 0.028), chronic kidney disease (RR: 6.85, CI 95%, 2.35-19.90, p < 0.003), cerebrovascular disease (RR:13.61, CI 95%, 5.01-36.96 p < 0.001), respiratory rate> 25 (RR: 3.94, CI 95%, 1.32-11.78 p = 0.023), oxygen saturation< 90% on admission (RR: 12.19, CI 95%, 3.71-40.01, p < 0.001), thrombocytopenia (RR:4.16, CI 95%, 1.37-12.64, p = 0.013), and elevated troponin (RR: 6.20, CI 95%, 1.73-22.24, p = 0.003). CONCLUSIONS: In this study, nearly a quarter of the patients with COVID-19 pneumonia required intensive care. We identified several risk factors associated with ICU admission and mortality that may be useful for predicting, triaging, and managing COVID-19 pneumonia patients. However, these findings need to be validated prospectively.

Successful therapeutic plasma exchange for a patient with sickle cell disease and fat embolism syndrome after a failure of a response to red cell exchange transfusion.

BACKGROUND: Fat embolism syndrome (FES) is a rare complication, which was reported mostly with milder forms of heterozygous sickle cell disease (SCD). It may present in a catastrophic way with multi-organ failure, particularly involving the pulmonary and neurological systems. Diagnosis is often missed or delayed; and the standard recommended treatment is red cell exchange (RCE) transfusion, which has sub-optimal results, such as debilitating long-term neurological complications. Recently, few reports suggested that the addition of Therapeutic Plasma Exchange (TPE) might further improve the outcome. CASE DESCRIPTION: A 23-year-old woman with homozygote SCD was admitted with bony pains and vaso-occlusive crises. However, her course evolved to respiratory failure requiring mechanical ventilation, decreased level of consciousness, skin rash, severe anemia and thrombocytopenia and a picture consistent with thrombotic microangiopathy. MRI of the brain showed scattered multi-focal ischemic foci and cytotoxic edema. The patient received RCE on the third day after admission without improvement. On the seventh day, TPE was instituted (2 L/day of fresh frozen plasma for 5 days), following which she regained her consciousness and showed an improvement in her laboratory abnormalities. On follow up, she had gradual full neurological recovery and resolution of the MRI findings within a few months. CONCLUSION: FES remains a diagnostic and therapeutic challenge, with significant morbidity and mortality. Success in the management of this reported case with the addition of TPE to RCE supports the notion that TPE may be a potentially helpful modality that deserves further research.

Fatal rapidly progressive interstitial lung disease in a patient with amyopathic dermatomyositis.

Interstitial lung disease (ILD) is a well-established common manifestation of idiopathic inflammatory myopathies. Yet, till now, the pathogenetic mechanisms are still poorly understood, classification is evolving and prognosis is variable. A refractory and rapidly progressive ILD (RPILD) that is associated with dermatomyositis (DM) with minimal muscle weakness and normal creatine kinase (termed clinically amyopathic DM) is increasingly being recognized, with more incidence in Asians. However, we are not aware of reports of the Arab region. Herein, we present a 38-year-old male with this condition that ended with a fatal outcome despite aggressive therapy, with a review of recent literature.

Nigella sativa for the treatment of COVID-19: An open-label randomized controlled clinical trial.

BACKGROUND: Effective treatment for Coronavirus Disease-2019 (COVID-19) is under intensive research. Nigella sativa oil (NSO) is a herbal medicine with antiviral and immunomodulatory activities, and has been recommended for the treatment of COVID-19. This study aimed to evaluate the efficacy of NSO treatment in patients with COVID-19. METHODS: All adult patients with mild COVID-19 symptoms presented to King Abdulaziz University Hospital, Jeddah, Saudi Arabia, were recruited for an open label randomized clinical trial (RCT). They were randomly divided into control or treatment groups, with the latter receiving 500 mg NSO (MARNYS® Cuminmar) twice daily for 10 days. Symptoms were daily monitored via telecommunication. The primary outcome focused on the percentage of patients who recovered (symptom-free for 3 days) within 14-days. The trial was registered at clinicaltrials.gov (NCT04401202). RESULTS: A total of 173 patients were enrolled for RCT. The average age was 36(±11) years, and 53 % of patients were males. The control and NSO groups included 87 and 86 patients respectively. The percentage of recovered patients in NSO group (54[62 %]) was significantly higher than that in the control group (31[36 %]; p = 0.001). The mean duration to recovery was also shorter for patients receiving NSO (10.7 ± 3.2 days) compared with the control group (12.3 ± 2.8 days); p = 0.001. CONCLUSIONS: NSO supplementation was associated with faster recovery of symptoms than usual care alone for patients with mild COVID-19 infection. These potential therapeutic benefits require further exploration with placebo-controlled, double-blinded studies.

Nocardiosis at an Organ Transplant Center in Saudi Arabia: 15 years' experience.

BACKGROUND: Nocardiosis is a rare infection that affects immunocompromised patients on immunosuppressive medications used for transplantation and cancer therapy. Such therapies are becoming more widely available in the Middle East region. Yet, reports on nocardiosis are scarce. MATERIALS AND METHODS: This was a retrospective analysis of patients who were diagnosed with nocardiosis from 2004 to 2018 at a transplantation and cancer center. Nocardiosis were defined per the European Organization for Research and Treatment of Cancer criteria. RESULTS: During the study period, 35 patients with nocardiosis (male: 68.5%) were identified. The most common underlying associated condition was transplantation 11 (31.4%), followed by malignancy 7 (20%), connective tissue disease and sarcoidosis 7 (20%), chronic lung disease 5 (14%), miscellaneous conditions 4 (11%), and one patient with human immunodeficiency virus. Nocardia was disseminated in 8 patients (22.9%) and isolated in 27 (77.1%); the latter included 13 patients (37.1%) with bronchial form, 11 (31.4%) with isolated visceral form, and 3 (8.6%) with cutaneous form. Pulmonary involvement occurred in 90% of the cases with cough, fever, and dyspnea being the most common symptoms. The main strain isolate was Nocardia asteroides, and the cure rate was 90%. Mortality related to nocardiosis occurred in 3 transplant patients (8.6%). CONCLUSION: Wider use of immunosuppressive therapy warrants vigilance to nocardiosis, which can present in a myriad of clinical forms. In our series, mortality was confined to the transplantation group, probably because of the relatively heavy immunosuppression. Nonetheless, prognosis is favorable if the infection is recognized and treated early.

Relapsing paradoxical reaction in miliary tuberculosis: a case report and literature review.

Paradoxical reaction (PR) after initiating anti-tuberculous therapy (ATT) is a well-recognized immune phenomenon. Less recognized, however, is a pulmonary reaction that is associated with miliary tuberculosis (TB), which can be a source of diagnostic confusion and progress to respiratory failure and acute respiratory distress syndrome (ARDS). We report an elderly patient who developed PR associated with respiratory failure following ATT for miliary TB, with radiological and pathological documentation. He responded to corticosteroids, but relapsed twice when the dose was reduced. It is imperative to be familiar with this form of PR to avoid diagnostic pitfalls and initiate appropriate therapy.

Nigella sativa supplementation to treat symptomatic mild COVID-19: A structured summary of a protocol for a randomised, controlled, clinical trial.

OBJECTIVES: To investigate the potential efficacy of Nigella sativa (NS) oil supplementation on the outcomes of patients with mild Coronavirus Disease 2019 (COVID-19). TRIAL DESIGN: Prospective, two-arm, parallel-group, randomised (1:1 allocation ratio), open-label, controlled, exploratory phase II clinical trial of oral NS oil in patients with mild COVID-19. PARTICIPANTS: Inclusion Criteria: - Patients with mild COVID19 (defined as upper respiratory tract infection symptoms in the absence of clinical or radiological signs of pneumonia). - Adult (18 - 65 years old). - Written informed consent by the patient (or legally authorized representative) prior to initiation of any study procedures. - All patients should understand and agree to comply with planned study procedures. - Polymerase chain reaction (PCR)-confirmed infection with Severe Acute Respiratory Syndrome Coronavirus-2 (SARS-CoV-2) from throat swab. EXCLUSION CRITERIA: - Patients with pneumonia or severe illness requiring admission to intensive care unit. - Severe chronic kidney disease (i.e. estimated glomerular filtration rate [eGFR] < 30 mL / min ) or end stage renal disease requiring dialysis - Severe chronic liver disease (Alanine transaminase [AlT] or Aspartate transaminase [AST] > 5 times the upper limit of normal). - Pregnancy or breast feeding. - Anticipated transfer within 72 hours to another hospital that is not a study site. - Allergy to the study medication The trial is currently conducted on patients recruited from King Abdulaziz University Hospital, Jeddah, Saudi Arabia. INTERVENTION AND COMPARATOR: Intervention group: Nigella sativa oil (MARNYS® Cuminmar) 500 mg softgel capsules, one capsule orally twice daily for 10 days plus standard of care treatment (antipyretic, antitussive). Comparator group: standard of care treatment. MAIN OUTCOMES: Proportion of patients who clinically recovered (defined as 3 days of no symptoms) within 14 days after randomisation. RANDOMISATION: Patients will be randomly assigned to treatment or control groups in a 1:1 ratio using a computer-generated randomization scheme (Random permuted blocks of 10) developed using the web-based program: http://www.randomization.com . BLINDING (MASKING): No blinding. NUMBERS TO BE RANDOMISED (SAMPLE SIZE): Up to 200 eligible patients will be randomly assigned to either treatment or control groups. TRIAL STATUS: Protocol version 1, as of July 14, 2020. Recruitment was started on May 21, 2020. The intended completion date is December 31, 2020. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04401202 . Date of trial registration: May 26, 2020. FULL PROTOCOL: The full protocol is attached as an additional file, accessible from the Trials website (Additional file 1). In the interest of expediting dissemination of this material, the familiar formatting has been eliminated; this letter serves as a summary of the key elements of the full protocol.

Organizing pneumonia secondary to cetuximab in a patient with colorectal cancer.

Organizing pneumonia (OP) may be idiopathic or secondary to a variety of causes including drugs. OP and other forms of pulmonary toxicity secondary to cetuximab, however, have been described rarely. It is paramount to recognize and differentiate OP from other common conditions that cancer patients are prone to such as infection and pulmonary embolism. A 69-year-old man with colorectal cancer received ten cycles of palliative chemotherapy [FOLFIRI (5-Fluorouracil, Leucovorin, Irinotecan) and cetuximab] with clinical and radiological response. He developed dyspnea following cycle 4, then 6 weeks later presented with cough, fever, tachypnea, hypoxia, bilateral crackles and diffuse pulmonary shadows. He was started on antibiotics but his condition deteriorated further. Cultures, including blood and bronchioalveolar lavage, grew no pathogens and molecular analysis and cytology for bacteria viruses were negative. Trans-bronchial biopsy was consistent with organizing pneumonia. Treatment with corticosteroids resulted in dramatic clinical and radiological resolution with normalization of gas exchange and pulmonary function. Corticosteroids were stopped and he was restarted on FOLFIRI and remained well with no relapse over a year of follow up. Although pulmonary toxicity secondary to cetuximab is uncommon, it is important to recognize, as it may be associated with poor prognosis. To the best of our knowledge, this is the first report of OP attributed to cetuximab with histopathological evidence.

An atypical pulmonary fibrosis is associated with co-inheritance of mutations in the calcium binding protein genes S100A3 and S100A13.

BACKGROUND: Pulmonary fibrosis is one of the leading indications for lung transplantation. The disease, which is of unknown aetiology, can be progressive, resulting in distortion of the extracellular matrix (ECM), inflammation, fibrosis and eventual death. METHODS: 13 patients born to consanguineous parents from two unrelated families presenting with interstitial lung disease were clinically investigated. Nine patients developed respiratory failure and subsequently died. Molecular genetic investigations were performed on patients' whole blood or archived tissues, and cell biological investigations were performed on patient-derived fibroblasts. RESULTS: The combination of a unique pattern of early-onset lung fibrosis (at 12-15 years old) with distinctive radiological findings, including 1) traction bronchiectasis, 2) intralobular septal thickening, 3) shrinkage of the secondary pulmonary lobules mainly around the bronchovascular bundles and 4) early type 2 respiratory failure (elevated blood carbon dioxide levels), represents a novel clinical subtype of familial pulmonary fibrosis. Molecular genetic investigation of families revealed a hypomorphic variant in S100A3 and a novel truncating mutation in S100A13, both segregating with the disease in an autosomal recessive manner. Family members that were either heterozygous carriers or wild-type normal for both variants were unaffected. Analysis of patient-derived fibroblasts demonstrated significantly reduced S100A3 and S100A13 expression. Further analysis demonstrated aberrant intracellular calcium homeostasis, mitochondrial dysregulation and differential expression of ECM components. CONCLUSION: Our data demonstrate that digenic inheritance of mutations in S100A3 and S100A13 underlie the pathophysiology of pulmonary fibrosis associated with a significant reduction of both proteins, which suggests a calcium-dependent therapeutic approach for management of the disease.

Anti-IgE therapy for asthma: an audit at atertiary care centre in Saudi Arabia.

BACKGROUND: Although anti-IgE therapy has been shown to offer numerous benefits, we suspect it is underutilized locally. To date, there are no studies on any aspect of its use in the Arab region. There is also no information on whether physicians follow current guidelines nor on patient response to this form of therapy. OBJECTIVE: Assess the use of omalizumab for patients with difficult asthma at a tertiary care center. DESIGN: Retrospective, descriptive. SETTING: Tertiary care hospital. PATIENTS AND METHODS: Information was collected from medical records and interviews of all patients who received a minimum of 6 months of omalizumab, including data on practices of the prescribing physician (pulmonary versus allergy), indications, dose, subjective response, number of emergency room visits and hospitalizations, changes in asthma medications, adverse effects, and the setting for delivery of therapy. MAIN OUTCOME MEASURES: Extent to which current guidelines for prescribing omalizumab were followed. Patient subjective and objective responses to treatment as reflected by changes in the use of medications and lung function before and after therapy. SAMPLE SIZE: 50 patients. RESULTS: Of the 50 consecutive patients, 35 were female and the mean (SD) age was 46.3 (9.2) years. Only 28 patients (56 %) met all the criteria for the prescription of omalizumab as per current guidelines; 18 (64%) by pulmonary and 10 (36%) by allergy physicians (P less than .05). Pulmonary physicians performed more tests for conditions complicating or simulating asthma (P less than .05). The mean (SD) duration of treatment by omalizumab of 35 (22) months was longer in patients managed by allergists (42 [24] months) than pulmonary physicians (30 [21] months) (P greater than .05). Both physician groups prescribed a lower initial dose than recommended (P less than .05 recommended vs. prescribed). Patients reported a significant improvement in symptoms, reduction in the use of broncho-dilators and oral steroids and in the use of healthcare services (from 16.28 [7.9] to 2.08 [1.78], P less than .0001) mean values from sum of hospitalizations/year, ER visits/year, exacerbations/year, but not in other medications or pulmonary function tests (P greater than .05). CONCLUSION: Despite several benefits, notably a reduction in utilization of health services and asthma medication, anti-IgE therapy is probably underutilized locally. Pulmonary physicians are more likely to follow the guidelines than allergy physicians. This study suggests that there is room for improvement in the prescription practices, particularly in dosing and the setting for delivery. Further multicenter prospective studies are required to identify gaps in the current practices and improve asthma management. LIMITATIONS: Too few patients met inclusion criteria, lack of control group, and use of a subjective assessment for patient symptoms as opposed to validated questionnaires. CONFLICT OF INTEREST: None.

The Saudi Thoracic Society guidelines for diagnosis and management of noncystic fibrosis bronchiectasis.

This is the first guideline developed by the Saudi Thoracic Society for the diagnosis and management of noncystic fibrosis bronchiectasis. Local experts including pulmonologists, infectious disease specialists, thoracic surgeons, respiratory therapists, and others from adult and pediatric departments provided the best practice evidence recommendations based on the available international and local literature. The main objective of this guideline is to utilize the current published evidence to develop recommendations about management of bronchiectasis suitable to our local health-care system and available resources. We aim to provide clinicians with tools to standardize the diagnosis and management of bronchiectasis. This guideline targets primary care physicians, family medicine practitioners, practicing internists and respiratory physicians, and all other health-care providers involved in the care of the patients with bronchiectasis.

Tracheobronchial anomalies in a patient with Schimke immuno-osseous dysplasia (SIOD).

Schimke Immuno-osseous Dysplasia (SIOD) is a rare genetic disorder with multiple systemic manifestations. Pulmonary manifestations have been described but not well characterized. They are believed to be secondary to decreased elasticity, and include emphysema, pulmonary hypertension and bronchiectasis. We describe a 24-year-old female patient with SIOD with tracheobronchial anomalies not reported before, including tracheomalacia and a tracheal bronchus with Epstein-Barr virus (EBV) related leiomyoma causing endobronchial obstruction. Such anomalies, in addition to the difficult upper airway associated with SIOD present specific challenges during management. This case reports documents tracheobronchial abnormalities that have not been described before in SIOD.

Ribavirin and interferon-α2b as primary and preventive treatment for Middle East respiratory syndrome coronavirus: a preliminary report of two cases.

Middle East respiratory syndrome coronavirus (MERS-CoV) is a newly recognized transmissible viral infection with high virulence and case fatality rates for which there is no currently defined primary treatment or prophylaxis. Saudi Arabia has the largest reported number of cases so far. Like severe acute respiratory syndrome (SARS), MERS is caused by a coronavirus. Combination therapy with interferon-α2b and ribavirin has been used successfully as primary treatment and prophylaxis in SARS. Because of similarities between the two coronaviruses, treatment with ribavarin and interferon-α2b has been suggested as a potential therapy for MERS-CoV. Studies in animal models of MERS-CoV have shown the combination of ribavirin and interferon-α2b to be effective both as primary treatment and prophylaxis. In this report, we describe for the first time use of this combination as a primary treatment for a patient with MERS-CoV infection and as prophylaxis for his spouse and discuss its possible role.

Gastroesophageal reflux disease and its association with bronchiolitis obliterans syndrome in allogeneic hematopoietic stem cell transplant recipients.

OBJECTIVES: Bronchiolitis obliterans syndrome is a significant postallogeneic hematopoietic stem cell transplant problem. Recent data in lung transplant patients suggest an association with gastroesophageal reflux disease and bronchiolitis obliterans syndrome. We studied posthematopoietic stem cell transplant patients with bronchiolitis obliterans syndrome for gastroesophageal reflux disease and its response to a proton pump inhibitor. MATERIALS AND METHODS: Seven postallogeneic hematopoietic stem cell transplant patients with bronchiolitis obliterans syndrome were studied. Gastroesophageal reflux disease was assessed by 24-hour pH monitoring with a Bravo catheter-free radio pH capsule. Patients with positive gastroesophageal reflux disease were started on omeprazole. Pretreatment and posttreatment pulmonary function tests were done at 3-month intervals. RESULTS: Of 7 patients, 5 had positive results for gastroesophageal reflux disease (71%). Omeprazole had a disease-stabilizing effect on the patients' pulmonary function tests. CONCLUSIONS: Our study shows a significant association between bronchiolitis obliterans syndrome and gastroesophageal reflux disease in postallogeneic hematopoietic stem cell transplant patients. Use of omeprazole may have a disease-stabilizing effect in short-term follow-up.

Eosinophilic pneumonia: experience at two tertiary care referral hospitals in Saudi Arabia.

BACKGROUND AND OBJECTIVES: Eosinophilic lung diseases are a diverse group of disorders characterized by pulmonary opacities associated with tissue or peripheral eosinophilia. DESIGN AND SETTING: A retrospective study conducted at two tertiary care hospitals from January 1999 to December 2009. METHODS: All cases with the diagnosis of pulmonary eosinophilia were reviewed over a period of 10 years. Data on demographic, clinical, and radiologic characteristics were collected. RESULTS: Thirty-five patients with a mean age of 33.9 (16.2) years, of which 20 (57.1%) were male and meeting the criteria of eosinophilic lung disease were identified. Cough and dyspnea were the most frequent symptoms at presentation in 29 (82.9%) and 27 (77.1%) patients, respectively. Reticulonodular and airspace patterns were the most common radiographic findings in 17 (48.6%) and 15 (42.9%) patients, respectively. Peripheral eosinophilia was present in 33 (94.3%) patients. Twenty-four patients (68.6%) were labeled as having idiopathic pulmonary infiltrate with eosinophilia. Complete remission was achieved in 13 (54.2%) of 24 patients, while 10 (41.7%) patients relapsed within a few months of discontinuation of therapy. Specific therapy for a specific disease was administered in 8 patients: 2 patients for pulmonary tuberculosis, 2 for Churg-Strauss syndrome, 1 for lymphoma, 1 for schistosomiasis, 1 for acute eosinophilic pneumonia, and 1 for Wegener granuloma; 3 patients were treated as allergic bronchopulmonary aspergillosis. CONCLUSIONS: Pulmonary eosinophilia remains rare but challenging, and it can have the same diverse clinical and radiographic presentations seen with other common pulmonary conditions. Clinicians should be alert to these syndromes and must think of them in any lung disease differential diagnoses.