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Pulmonary Interstitial Glycogenosis
Infantile Cellular Interstitial Pneumonitis

Presentation

Pulmonary interstitial glycogenosis (PIG) is an enigmatic lung disorder of unknown etiology that presents with neonatal respiratory distress. [ncbi.nlm.nih.gov]

treatment and outcome of eleven children with PIG were collected systematically.Results10/11 children presented with respiratory distress immediatly after birth and 8/11 needed invasive ventilation. [docwirenews.com]

All seven infants presented with tachypnea, hypoxemia, and diffuse interstitial infiltrates with overinflated lungs on chest radiographs in the first month of life. [semanticscholar.org]

Respiratoric

  • Respiratory Distress

    The infant was well initially and then developed respiratory distress at 18 days of life. Radiographs at first day of life were normal, but CT and radiographic findings at 18 days of life showed severe interstitial lung disease, mimicking BPD. [ncbi.nlm.nih.gov]

Neurologic

  • Meningism

    B4481Allergic bronchopulmonary aspergillosis B909Sequelae of respiratory and unspecified tuberculosis D860Sarcoidosis of lung D861Sarcoidosis of lymph nodes D862Sarcoidosis of lung with sarcoidosis of lymph nodes D863Sarcoidosis of skin D8681Sarcoid meningitis [cms.gov]

Workup

She has been evaluated at duke university with a genetic workup. ...Read more See 1 more doctor answer [healthtap.com]

A proper and detailed history looking for the occupational exposures, and for signs of conditions listed above is the first and probably the most important part of the workup in patients with interstitial lung disease. [en.wikipedia.org]

X-Ray

  • Atelectasis

    There is also some gravity-dependent atelectasis ofposterior lung elds (asterisks).364 Onland et al.this entity recently. [docslide.com.br]

Treatment

Apparent treatment benefit of glucocorticosteroids needs to be evaluated systematically. [docwirenews.com]

This unique case demonstrates that pulmonary hypertension can be seen in the setting of PIG without associated cardiac anomalies, and persists despite treatment in an otherwise self-limited condition. [ncbi.nlm.nih.gov]

Surgery Children with severe chILD may need a lung transplant if other treatments haven't worked. Lung transplants are the only effective treatment for certain types of chILD. [childrens.com]

Treatments are aimed at preventing more lung scarring, managing symptoms, and helping you stay active and healthy. Treatment can’t fix lung scarring that has already occurred. [choc.org]

Options Treatment options depend on the diagnosis. [stanfordchildrens.org]

Prognosis

Most literature suggests that PIG is one of the few pediatric interstitial lung diseases that has a favorable prognosis with rare mortality in the absence of co-morbidities. [ncbi.nlm.nih.gov]

Despite its dramatic clinical presentation the diagnosis of PIG has a favorable prognosis with rare mortality in the absence of co-morbid conditions. [humpath.com]

Etiology

Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]

We present a case of pulmonary interstitial glycogenosis that developed in a nonintubated, 31-week gestation infant in whom infectious etiologies had been excluded. [ncbi.nlm.nih.gov]

The etiology of pulmonary interstitial glycogenosis remains unclear. [basicmedicalkey.com]

Pathophysiology

While the underlying pathophysiologic mechanism of PIG is unclear, we suggest that the mitogen-activated protein kinase signal transduction pathway members (PTPN11, KRAS, SOS1, RAF1, SHOC2, NRAS) involved in cellular growth factor signaling, which are [ncbi.nlm.nih.gov]

Prevention

الصفحة 158 - Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease. NHLBI/WHO Global Initiative for Chronic Obstructive Lung Disease (GOLD) Workshop summary. ‏ [books.google.com]

Is there a way to prevent chILD? There’s no way to prevent chILD but you can take steps to avoid diseases that may make it worse. Frequent hand-washing and keeping your child away from pollutants like cigarette smoke can improve her quality of life. [childrens.com]

The goal of treatment for people with ILD is to prevent more scarring and manage symptoms. [choc.org]

Treatment of the underlying condition is essential, but additional treatment may include optimization of nutrition, prophylactic immunizations, treatment of secondary infections, bronchodilator therapy, oxygen (both for comfort and prevention of hypoxia [fortherecordmag.com]

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