All participants to receive study intervention.

Retinitis Pigmentosa

After completing the pretreatment visits, all patients will enter into the treatment phase where patients will receive 1800 mg of NAC effervescent tablets twice a day. The patients will be followed up every 3 months for 2 years. Therefore, there will be a total of 9 treatment visits (baseline, months 3, 6, 9, 12, 15, 18, 21 and 24).

NAC effervescent tablets

Wilmer Eye Institute at Johns Hopkins University

FIGHT-RP 1 Extension Study

A Phase 1 Open-Label Extension Study to Assess the Long-Term Safety and Tolerability of N-Acetylcysteine (NAC) in Patients With Retinitis Pigmentosa

Blood pressure in mmHg will be used in assessing tolerability of N-Acetylcysteine.

Participants will be assessed for the occurrence of any drug-related symptoms which includes nausea, stomach upset, diarrhea, heartburn, constipation, and vomiting. Based on the number and severity of the symptoms, the physician would conclude whether the patient is tolerating the medication or not.

Medication diary provided to participants will be reviewed to see if participants are taking the medication within the designated time frame.

Medication diary provided to participants will be reviewed to see if participants are taking the medication twice a day

Scoring is determined by the number of letters gained or lost per month using Early Treatment Diabetic Retinopathy Study (ETDRS) Letter Score and visual acuity score together with an overall score range of 0 to 20/20 where 0 is the worst vision and 20/20 is the best.

Microperimetry (MAIA) is used to test whether there is an increase or decrease in sensitivity (dB) in the retina after initiation of N-Acetylcysteine

This will be assessed by spectral domain optical coherence tomography (SD-OCT) after initiation of N-Acetylcysteine.

This will be calculated from the proportions of reduced glutathione and oxidized glutathione in the aqueous.

Johns Hopkins University

This group will receive all standard of care therapies as per the pediatric ED Asthma Severity Protocol. To summarize, all moderate and severe acute asthma exacerbations receive albuterol and atrovent continuous aerosols, oral or intravenous steroids and intravenous magnesium sulfate. At the discretion of the treating physician, the patient may also receive subcutaneous terbutaline or epinephrine

This group will receive all standard of care therapies as per the pediatric ED Asthma Severity Protocol. All nebulized treatments will be given via the NIPPV/BiPAP machine (EnVe Ventilator). Optimal settings for each participant should be achieved within 20 minutes of initiation of NiPPV. Pediatric asthma scores will continue to be recorded at least every hour or with every physician suggested NIPPV ventilator setting change for an 8 hour period. Furthermore, the NIPPV machine in conjunction with NM3 volumetric end tidal CO2 monitoring will record ventilatory data in this NIPPV/BiPAP group and will be compared to other study groups. The subjects will remain in the study for minimum of 4 hrs NIPPV therapy and total of 8 hours.

Bronchial Asthma

Status Asthmaticus

If at any point during the treatment it is deemed medically necessary by the treating PED attending that a subject from the control arm (Group 1) requires an escalation of therapy, they may be moved from the group to the NIPPV/BiPAP group (Group 2) or be removed from the study (i.e. if intubated).

Standard of Care therapies

If at any point during the treatment it is deemed medically necessary by the treating PED attending that a subject from a NIPPV/BiPAP group (Group 2) is not tolerating NIPPV they may be removed from the study. Patients may not tolerate NIPPV due to agitation, vomiting or need for escalation of care.

NiPPV Therapy

It may be up to the treating physicians discretion to treat patient with other medications that are currently used to treat severe asthma exacerbations, such as Epinephrine and Terbutaline

Routine Respiratory Care

Arkansas Children's Hospital

NiPPV in the Treatment of Acute Asthma Exacerbations

A Randomized Controlled Trial of Non-Invasive Positive Airway Pressure in the Pediatric Emergency Department for the Treatment of Acute Asthma Exacerbations

The Research coordinator or Respiratory Therapist will measure a PAS every hour for a total of 8 hours with the goal of lowering the PAS

The goal of the treatment strategy is to lower the PAS to less than 4 so that the patient may be transitioned from the NiPPV/Bipap to standard oxygen therapy for the treatment of an exacerbation of their asthma

The goal of the therapy is to be able to reduce the amount of inspiratory pressure the patient receives as their disease process improves. We hope to measure this with the end-tidal CO2 device. When the exhaled CO2 trends down we hope to reduce the inspiratory pressure the patient is receiving via NiPPV/BiPAP

By the Respiratory therapist and physician examining the flow volume loops from the patient data, we hope to capture the time that the patients lungs have seen a reduction in bronchospasms

By adding NiPPV/Bipap to the patient to increase their inspiratory pressure while receiving their respiratory medications, we hope to be able to get the medications to the lower airways faster to help patient recover faster from asthma exacerbation which will hopefully reduce patient's length of stay in the hospital

We hope to show improvements in Cardiac output measurements from the start of therapy until after treatment has been discontinued. We hope to show in increase in cardiac output due to the decrease in air trapping that is caused when a patient is undergoing an asthma exacerbation.

Arkansas Children's Hospital Research Institute

Subjects will receive two 5mg tablets of Montelukast/day.

Subjects will receive one 5mg tablet of montelukast and 1 placebo tablet per day.

Subjects will receive two placebo tablets per day.

Eosinophilic Esophagitis

Those in Montelukast 10mg/day group will receive two 5mg tablets of Montelukast.

Montelukast

Those in the placebo group will receive 2 placebo tablets per day. Those in the Montelukast 5mg/day will receive 1 placebo tablet per day.

placebo

Subject will receive one 5mg tablet of Montelukast and one placebo tablet per day.

5 mg Montelukast

Children's Mercy Hospitals and Clinics

A Trial of Montelukast for Maintenance Therapy of Eosinophilic Esophagitis in Children

Eosinophils/hpf in the esophagus will be measured after 12 weeks of therapy.

pulmonary fibrosis/oksentaminen

FIGHT-RP 1 Extension Study

NiPPV in the Treatment of Acute Asthma Exacerbations

A Trial of Montelukast for Maintenance Therapy of Eosinophilic Esophagitis in Children

Pomalidomide for Cough in Patients With Idiopathic Pulmonary Fibrosis

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