Acute hypercapnic respiratory failure in patients with obesity hypoventilation syndrome during the COVID-19 pandemic: Four case reports.

Obesity hypoventilation syndrome (OHS) can cause acute hypercapnic respiratory failure (AHRF). The onset of AHRF in four patients with OHS during the coronavirus disease 2019 (COVID-19) pandemic is reported in this study. Two men (23 and 45 years old) and two women (both 77 years old) presented to our hospital with AHRF. In the two elderly women, a prolonged supine position due to falls seemed to be the cause of AHRF. Treatment was started with bilevel positive airway pressure for all patients. While one patient died, the condition of the other three improved; they were discharged with continuous positive airway pressure. AHRF due to OHS was rarely reported in the rural region of Japan. It is suggested that increased rates of obesity due to lifestyle changes during the COVID-19 pandemic may be responsible for an increase in the prevalence of OHS-associated AHRF.

Remote monitoring and virtual follow-up ventilation clinics in the age of Covid-19

Background: To review the efficacy of long-term non-invasive ventilation (LTV) with virtual follow-up, using assistive technologies and remote monitoring. Objective(s): Compliance greater than 4 hours, correction of hypercapnia and improvements in sleep and quality of life (QoL) were the objectives in improving patient outcome. Method(s): We conducted a retrospective cohort study including patients established on LTV over an 18 month period from the start of the pandemic. Initiation of LTV was established as an inpatient (n= 17;35%), outpatient (n= 28;57%) or from the patient's home (n= 4;8%) and follow-up was by telephone, video consultation or from the patient's own home. LTV was considered successful if compliance achieved was > 4 hours using remote monitoring, corrected hypercapnia (tCO2 < 6.5Kpa) and patient-reported improvements in sleep and QoL. Result(s): Forty-nine patients were included. LTV was initiated for: neuromuscular disease (n=19;39%), obesity hypoventilation or overlap (n=12;24%), chronic obstructive pulmonary disease (n=11;22%) and chest wall deformity (n=6;14%). Compliance > 4 hours was achieved in 69% with effective correction of hypercapnia (tCO2 < 6.5Kpa) in 67% and reported improved sleep and QoL at 63%. Overall success in all 3 objectives was 61%. Patient preference for future follow-up was 45% requesting face-to-face consultations and 33% to continue remotely. A proportion of the most vulnerable (14%) would prefer to be seen at home. Conclusion(s): Successful LTV can be achieved with remote compliance data and transcutaneous carbon dioxide monitoring to support virtual clinics. Patient preference for face-to-face consultations remains important in overall satisfaction.

Nocturnal hypoventilation and respiratory muscle/cough weakness in COVID-19 ICU survivors

Background: The impact of severe COVID-19 in nocturnal hypoventilation and respiratory muscles/cough strength is unknown. Aim(s): Characterize respiratory function in post-ICU COVID-19 patients and correlate these findings with COVID-19 associated outcomes. Method(s): Retrospective study with 55 post-ICU COVID-19 patients admitted to a rehabilitation center (RC). Clinical data were collected and patients performed arterial blood gas analysis, nocturnal oxy-capnography, maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP) and peak cough flow (PCF) at admission. Result(s): Mean age was 64.4+/-10.2 years, 39 (70.9%) were male. Co-morbidities were present in 50 (90.9%): 39 (70.9%) hypertension;20 (36.4%) diabetes;3 (5.5%) OSA;1 (1.8%) COPD. None had neuromuscular disorders. Median of ICU length was 25.5 (min 5;max 190) days. Fifty-three (96.4%) needed mechanical and invasive ventilation, 6 (10.9%) ECMO and 22 (40%) tracheostomy (all decannulated before admission to our RC). Eighteen (32.7%) had criteria of nocturnal hypoventilation, 17 (30.9%) inspiratory, 16 (29.1%) expiratory and 17 (30.9%) cough weakness. Sepsis correlated with increased length of ICU stay (p=0.007) and with lower PCF values (p=0.048). Neurologic disfunction was associated with lower minimum SpO2 and more time of SpO2<88% (p=0.032;p=0.035). Renal disfunction was associated with higher values of mean and maximum TcCO2 (p=0.003;p=0.011). Patients with previously diagnosed OSA had higher values of MIP and PCF (p=0.006;p<0.001) at evaluation. Conclusion(s): In COVID-19 ICU survivors, nocturnal hypoventilation, inspiratory/expiratory muscle and cough weakness are common and may have an impact in rehabilitation outcomes.

Oxygen home therapy after discharge of hospitalized patients with COVID-19

Introduction: Patients hospitalized with COVID-19 may have many healthcare needs after hospital discharge. Providing transitional care in pandemic circumstances, must adapt to specific needs and limitations. Aim(s): Define the profile of patients with COVID-19 who needed oxygen home therapy after discharge. Method(s): Retrospective study including patients infected with COVID-19 ;confirmed either by RT-PCR or Rapid Antigen Test and hospitalized in COVID-19 unit from September 2020 to June2021. Patients who deceded and those who were transferred to ICU without any data about evolution, were excluded. We defined 2 groups: G0: Patients discharged without oxygen home therapy G1 : Patients who required oxygen home therapy Results: A total of 528 patients were included: G0: 491 patients (93%), G1: 37 patients (7%). The mean age was significantly different between both groups (G0: 61,4 ;G1:68,0 p=0,003). There was no significant correlation regarding the extent of the lesions on the chest CT scan (G0:45%, G1:47% p=0.698), however maximum oxygen needs were higher in G1 (G0:7L, G1: 11L;p=0.001). Smoking, COPD, and hypercapnia were significantly related to prolonged oxygen need (G0: 30%, G1: 50%;p=0,026);(G03,7%, G1:24,3%;p=0,000) and (G0:34, G1:43;p=0.003) respectively. Conclusion(s): Chronic hypoventilation seems to be a predictive factor of prolonged oxygen needs. Oxygen home therapy can be a transitional solution to shorten hospital stay.

Unusual case of Obesity and Hypoventilation in Childhood

Objectives Childhood obesity can be monogenic or polygenic in etiology and is associated with significant morbidities. Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation, and neural crest tumor (ROHHHAD[NET]) syndrome, is a rare autonomic and respiratory pediatric disorder presenting with rapid weight gain in early childhood, hypothalamic-pituitary dysfunction, central hypoventilation, and an association with neural crest tumors. Methods A 6-year-old Asian girl with abnormal weight gain since the age of 3 years, presented to the pediatrician's office due to pulse oximeter readings in the 60s at home. Parents were monitoring saturations at home as a way of screening for COVID-19 infection. The pediatrician confirmed hypoxemia and transferred the patient to the Children's Medical Center emergency department on oxygen via EMS. She had occasional snoring and nighttime cough, but no history of respiratory distress, or signs of infection. There was no hyperphagia, neonatal hypoglycemia, or developmental or behavioral concerns. On examination her body weight was 30 kg (+1.56 SD) and height was 113 cm (-1.46 SD) with a body mass index (BMI) of 23.4 kg/m2 (+2.33 SD). No acanthosis nigricans, cushingoid features, or respiratory distress were noted on examination. In the intensive care unit, she was diagnosed with central hypoventilation requiring mechanical ventilation. Her laboratory work-up revealed central hypothyroidism (low Free T4 of 0.64 ng/dl, TSH 1.553 microIU/L). Other anterior pituitary hormones were normal (adrenocorticotropic hormone, 16.3 pg/mL;cortisol, 10.7 mug/dL;prolactin, 9.95 ng/ml;Insulin-like growth factor-1, 83 ng/mL;and IGF binding protein 3, 3.02 mg/L). Genetic investigations revealed no known mutations in the PHOX2B gene, making a diagnosis of central hypoventilation syndrome unlikely. Results Rapid onset weight gain around 3 years of age, central hypoventilation, and anterior pituitary hormone deficiency in our patient with negative PHOX2B is consistent with a clinical diagnosis of ROHHHAD[NET]. Our patient was started on levothyroxine;received tracheostomy for mechanical ventilation;and gastrostomy for pharyngeal dysphagia. She is doing well, goes to school, and is tolerating trials off the ventilator during the day. Conclusions ROHHAD is an important differential to consider for any child with rapid and early obesity and hypoventilation as early diagnosis is critical in improving the clinical management and the prognosis.

ATYPICAL PRESENTATION OF BIOT'S BREATHING IN A PATIENT WITH ARNOLD CHIARI MALFORMATION II WITH COMPLEX SLEEP APNEA/SLEEP HYPOVENTILATION/HYPOXEMIA NOT ON OPIOID AND WITH NO HISTORY OF MENINGITIS

Introduction: Sleep disordered breathing is very common in Arnold Chiari malformation but Biots's breathing has not been reported in these cases. Report of Cases: 3-year-old with history of Arnold Chiari II malformation with hydrocephalus (s/p VP shunt and surgical decompression), spina bifida myelomeningocele (s/p in utero repair), subglottic stenosis with prior tracheostomy and eventual decannulation presented for evaluation of sleep disordered breathing. Presenting symptoms included witnessed apneas, cyanosis, daytime sleepiness and frequent awakenings. Physical examination was largely unremarkable. Initial PSG demonstrated complex sleep apnea, with an AHI 20.6, REM AHI 57.8 per hour of sleep, ETCO2 peak of 69 cm H20 and an O2 saturation nadir of 34%, with sleep related hypoventilation/hypoxemia and Biot's breathing in the absence of opioid use and CNS infection. During titration study, Biot's breathing, complex apnea and sleep related hypoventilation/ hypoxemia responded well to BiPAP ST of IPAP 18 cm H20 and EPAP 14 cm H20 BUR of 12 and oxygen of 1 L/min. She later developed intolerance to BiPAP due to high pressures and was decreased to BIPAP 13/11 cm H2O. Later the patient discontinued the use of BIPAP due to intolerance and was switched to night time O2 at 3-4 L/min. Per the parents, the patient has been maintaining her oxygen saturation in the absence of BIPAP therapy with oxygen use. Due to COVID, patient was unable to follow up but will be scheduled for a repeat PSG in the near future. She followed with Neurosurgery for Arnold Chiari II and they recommended no surgical intervention at this time due to functional VP shunt. Conclusion: This is an atypical presentation of Biot's breathing in the absence of CNS infections and opioid use in a patient with Arnold Chiari malformation II. Patient has complex sleep apnea, initially well controlled with BiPAP ST, but developed BiPAP intolerance. She is on oxygen with good control of hypoxemia in the absence of BiPAP therapy.

Case Report: COVID-19-Associated ROHHAD-Like Syndrome.

It is known that the SARS-CoV-2 virus may cause neurologic damage. Rapid-onset obesity, hypoventilation, hypothalamus dysfunction, and autonomic dysregulation (ROHHAD) syndrome is a disease of unknown etiology with a progressive course and unclear outcomes. The etiology of ROHHAD syndrome includes genetic, epigenetic, paraneoplastic, and immune-mediated theories, but to our knowledge, viral-associated cases of the disease have not been described yet. Here we present the case of a 4-year-old girl who developed a ROHHAD syndrome-like phenotype after a COVID-19 infection and the results of 5 months of therapy. She had COVID-19 pneumonia, followed by electrolyte disturbances (hypernatremia and hyperchloremia), hypocorticism and hypothyroidism, central hypoventilation-requiring prolonged assisted lung ventilation-bulimia, and progressive obesity with hypertriglyceridemia, dyslipidemia, hyperuricemia, and hyperinsulinemia. The repeated MRI of the brain and hypothalamic-pituitary region with contrast enhancement showed mild post-hypoxic changes. Prader-Willi/Angelman syndrome as well as PHOX2B-associated variants was ruled out. Treatment with non-steroidal anti-inflammatory drugs and monthly courses of intravenous immunoglobulin led to a dramatic improvement. Herein the first description of ROHHAD-like syndrome is timely associated with a previous COVID-19 infection with possible primarily viral or immune-mediated hypothalamic involvement.

Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) Syndrome in an 8-Year-Old Girl with Ganglioneuroma and SARS-COV-2 infection: A Case Report and Literature Review

Background: ROHHAD syndrome (rapid-onset obesity, hypoventilation, hypothalamic dysfunction, and autonomic dysregulation) is an extremely rare disease in children. This article describes the clinical features, laboratory findings, imaging results, and treatment of a case of ROHHAD syndrome with ganglioneuroma. Case Report: An 8-year-old girl in Covid-19 pandemic was admitted to our emergency department due to dyspnea, cyanosis, and respiratory distress. She had a history of close contact with his father (a case of covid 19) prior to hospital admission. Spiral chest CT scan of the patient revealed bilateral ground-glass opacities and also a right paravertebral mass. She had a history of rapid onset morbid obesity that had started 2 years ago. Furthermore, she had sleep disturbances and hypoventilation, electrolyte imbalance, polyuria and enuresis, irritability, and impulsivity. She was diagnosed as a case of ROHHAD syndrome based on her clinical manifestations. She was also diagnosed as a case of SARS-COV-2 infection. The paravertebral mass was resected which pathology was consistent with ganglioneuroma. Conclusion: Because of the high prevalence of morbidity and mortality in ROHHAD syndrome and accompanying neural crest tumors, early diagnosis of this syndrome should be kept in mind. It is essential to pay attention to the cases with rapid onset obesity associated with hypoventilation to prevent severe complications. © 2021, Iranian Pediatric Hematology and Oncology Society. All rights reserved.

Hipercapnia refractaria en paciente con síndrome de obesidad-hipoventilación maligno y COVID-19. Reporte de caso y propuesta de manejo

RESUMEN El síndrome de obesidad-hipoventilación asociado a la COVID-19, conlleva rápidamente a la insuficiencia respiratoria aguda severa con la necesidad de ventilación mecánica invasiva, convirtiéndose en un reto terapéutico hacia el personal del cuidado intensivo debido a la ausencia de estrategias ventilatorias. A continuación, se presenta el caso de un paciente masculino de 51 años con antecedentes de síndrome de Pickwick que presentó neumonía grave por SARS-CoV-2, el cual progresa tempranamente a un síndrome de dificultad respiratoria aguda grave requiriendo soporte mecánico ventilatorio invasivo con presión positiva y la necesidad de soporte vasoactivo, cursando además con un síndrome de obesidad-hipoventilación de fenotipo maligno. Con base a lo anterior se realiza una propuesta de manejo clínico institucional basado en la literatura científica actual del síndrome de obesidad-hipoventilación y neumonía grave secundario a SARS-CoV-2. A pesar de la alta mortalidad relacionada con la COVID-19 y la dificultad presentada durante la ventilación mecánica invasiva, el desenlace final del paciente fue favorable. The obesity-hypoventilation syndrome associated with COVID-19 rapidly leads to severe acute respiratory failure with the need for invasive mechanical ventilation, becoming a therapeutic challenge for intensive care personnel due to the absence of ventilatory strategies. The following is the case of a 51-year-old male patient with a history of Pickwick syndrome who presented with severe SARS-CoV-2 pneumonia, which early progresses to severe acute respiratory distress syndrome requiring invasive mechanical ventilatory support with pressure. positive and the need for vasoactive support, also presenting a malignant phenotype obesity-hypoventilation syndrome. Based on the above, a clinical management proposal based on the current scientific literature of obesity-hypoventilation syndrome and severe pneumonia secondary to SARS-CoV-2 is made. Despite the high mortality related to COVID-19 and the difficulty presented during invasive mechanical ventilation, the patient's evolution was favorable.

Obesity Hypoventilation Syndrome-Related Challenges in Acute Respiratory Failure.

Obesity hypoventilation syndrome (OHS) is a condition commonly found in severely obese patients in which they fail to breathe deeply or rapidly enough to offset the body's need for oxygen consumption and carbon dioxide release. This report presents a case of a 49-year-old super-super-morbid obese female with a body mass index (BMI) of 90 kilogram per meter squared (kg/m²), chronic obstructive pulmonary disease (COPD), and end-stage cor pulmonale, who was brought to the emergency department for altered mental status and requiring emergent airway due to respiratory failure secondary to OHS. The continued increase in rates of obesity worldwide, especially in those with BMI ≥ 50 kg/m², may lead to an increase in the incidence of OHS. With comorbidities secondary to obesity and associated complexity, this medically challenging case emphasizes the need for refined management strategies in caring for OHS in super-super-morbidly obese patients.

Contemporary Concise Review 2020: Sleep.

Obstructive sleep apnoea (OSA) now affects one-seventh of the world's population. Treatment of even mild OSA can improve daytime sleepiness and quality of life. Recent modifications to uvulopalatopharyngoplasty may make it a more widely applicable treatment option in selected patients with OSA. Diet and exercise have effects on sleep apnoea severity independent of weight loss. Insomnia has become increasingly common during the coronavirus disease 2019 (COVID-19) pandemic.

A novel perspective approach to explore pros and cons of face mask in prevention the spread of SARS-CoV-2 and other pathogens.

Corona virus disease 2019 (COVID-19) outbreak has become a severe community health threat across the world. Covid-19 is a major illness, presently there is no as such any medicine and vaccine those can claim for complete treatment. It is spreading particularly in a feeble immune people and casualties are expanding abruptly and put the health system under strain. Among the strategic measures face mask is one of the most used measures to prevent spread of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Wearing a face mask possibly create a false sense of security lead to decline others measures. Face mask could be risk for the people of under lying medical conditions, old age group, outdoor exercise, acute and chronic respiratory disorders and feeble innate immune. Restrictive airflow due to face mask is the main cause of retention of CO2 called hypercapnia that can lead to respiratory failure with symptoms of tachycardia, flushed skin, dizziness, papilledema, seizure and depression. According to latest updates face shield and social distancing could be better substitute of face mask.