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Pulmonary Interstitial Glycogenosis

PIG


Presentation

  • Pulmonary interstitial glycogenosis (PIG) is an enigmatic lung disorder of unknown etiology that presents with neonatal respiratory distress.[ncbi.nlm.nih.gov]
  • We present the clinical presentation and pathological findings from a term infant with atypical neonatal lung disease. A full term Caucasian male presented at birth with signs of respiratory distress.[ncbi.nlm.nih.gov]
  • We present a case of PIG with pulmonary hypertension without underlying cardiac disease. This patient presented with respiratory distress and spontaneous pneumothorax at 6 months of age.[ncbi.nlm.nih.gov]
  • Because histologic examination is required to establish the diagnosis, we speculate that PIG, although rare, may be underrecognized in neonates presenting with PPHN in the setting of CHD.[ncbi.nlm.nih.gov]
  • Here, we present a case of pulmonary interstitial glycogenosis associated with trisomy 21, pulmonary arterial hypertension, and congenital heart disease in a 34 week premature infant.[ncbi.nlm.nih.gov]
Gangrene
  • Alveolar capillary dysplasia with vein misalignment J84.848 Other interstitial lung diseases of childhood J84.89 Other specified interstitial pulmonary diseases J84.9 Interstitial pulmonary disease, unspecified J85 Abscess of lung and mediastinum J85.0 Gangrene[icd10data.com]
Multiple Organ Dysfunction Syndrome
Hydrops Fetalis
  • We describe an infant prenatally diagnosed with hydrops fetalis ultimately found to have Noonan syndrome (NS).[ncbi.nlm.nih.gov]
Suggestibility
  • 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]
  • 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]
  • Although it was initially suggested to represent a developmental abnormality, more recent theories suggest a nonspecific reactive and/or reparative process of the growing infant lung, particularly in light of its wider recognition, numerous associated[basicmedicalkey.com]
  • Although some patients improved with age, 7 died of respiratory failure or complications of CHD, suggesting that PIG may be clinically significant when associated with other severe disorders.[ncbi.nlm.nih.gov]
  • The case reports by Lanfranchi et al. [1] and now Castillo et al. [2] in this issue of Pediatric Radiology highlight the clinical contexts in which PIG occurs and suggest associated imaging findings… CONTINUE READING From This Paper Figures, tables, and[semanticscholar.org]
Neonate-Onset
  • Clinical synopsis first month of life neonatal onset respiratory failure pulmonary hypertension tachypnea hypoxemia diffuse interstitial infiltrates overinflated lungs Pathology synopsis expansion of the interstitium by spindle-shaped cells containing[humpath.com]

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]
Ground Glass Appearance
  • A chest CT scan demonstrated a diffuse ground glass appearance and lung biopsy demonstrated increased alveolar septae cellularity with glycogen-containing cells, consistent with a diagnosis of PIG.[ncbi.nlm.nih.gov]

Treatment

  • 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]
  • Apparent treatment benefit of glucocorticosteroids needs to be evaluated systematically.[docwirenews.com]
  • Treatment depends on the type of exposure and the stage of the disease. It may involve medicines, oxygen therapy, or a lung transplant in severe cases.[icdlist.com]
  • Focus on the development, function, and treatment of a wide range of disease entities with the text’s clear, logical, organ-system approach.[books.google.com]
  • A preliminary study of long-term treatment with interferon gamma- Ib and low-dose prednisolone in patients with idiopathic pulmonary fibrosis. N Engl J Med 1999;341(17): 1264-1269. ‏[books.google.com]

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]
  • The fatal outcome for this patient contrasts with the reported favorable prognosis associated with isolated pulmonary interstitial glycogenosis.[ncbi.nlm.nih.gov]
  • Despite its dramatic clinical presentation, the diagnosis of PIG has a favorable prognosis with rare mortality in the absence of comorbid conditions.[ncbi.nlm.nih.gov]
  • Abstract Transposition of the great arteries with intact ventricular septum and persistent pulmonary hypertension (TGA-IVS PPHN) is a rare association with a poor prognosis.[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]
  • Pulmonary interstitial glycogenosis (PIG) is an enigmatic lung disorder of unknown etiology that presents with neonatal respiratory distress.[ncbi.nlm.nih.gov]
  • The etiology of pulmonary interstitial glycogenosis remains unclear.[basicmedicalkey.com]
  • Pathology by systems Respiratory system Lungs pulmonary interstitial glycogenosis Wednesday 16 September 2009 Definition : Pulmonary interstitial glycogenosis (PIG) is an enigmatic lung disorder of unknown etiology that presents with neonatal respiratory[humpath.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]
  • The underlying pulmonary pathophysiology of PIG has not been well characterized. Herein we report a term-gestation infant who presented with persistent tachypnea and hypoxia.[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]
  • What they found adds to... [ Read Full Story ] May 10, 2019 If your provider is ordering nebulizers and the drugs used in them for their patients here are things in the documentation that will help prevent a resubmission to Medicare and ease medical coding[coder.aapc.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]
  • 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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