Pulmonary hypoplasia and persistent pulmonary hypertension in the newborn with homozygous α-thalassemia: a case report and review of the literature

2003 ◽  
Vol 14 (6) ◽  
pp. 411-416 ◽  
Author(s):  
M. J. Bizzarro ◽  
J. A. Copel ◽  
H. A. Pearson ◽  
B. Pober ◽  
V. Bhandari
2020 ◽  
Vol 07 (02) ◽  
pp. 40-45
Author(s):  
Andreea Luciana Avasiloaiei ◽  
Ecaterina Iftime ◽  
Daniela Claudia Scripcaru ◽  
Andrei Petrariu ◽  
Gabriela Ildiko Zonda ◽  
...  

PEDIATRICS ◽  
1996 ◽  
Vol 98 (4) ◽  
pp. 706-713 ◽  
Author(s):  
Allan P. Goldman ◽  
Robert C. Tasker ◽  
Sheila G. Haworth ◽  
Paul E. Sigston ◽  
Duncan J. Macrae

Objective. To determine the clinical role of inhaled nitric oxide (iNO) in the treatment of persistent pulmonary hypertension of the newborn (PPHN). Study Design. Prospective open observational clinical study. Setting. A regional cardiac and pediatric intensive care unit. Methods. Twenty-five consecutive near-term neonates (>35 weeks gestation) with severe PPHN (oxygenation index [OI]> 25) were given a trial of iNO of 20 ppm for 20 minutes. Neonates who showed a greater than 20% improvement in Pao 2 as well as a decrease in the OI to below 40 were defined as responders and continued on this therapy. Results. Four patterns of response emerged to the iNO therapy: Pattern 1 neonates (n = 2) did not respond to the initial trial of iNO—one survived. Pattern 2 neonates (n = 9) responded to the initial trial of iNO, but failed to sustain this response over 36 hours, as defined by a rise in the OI to >40. Six survived, five with extracorporeal membrane oxygenation. Pattern 3 neonates (n = 11) responded to the initial trial of iNO, sustained this response, and were successfully weaned from iNO within 5 days—all survived to discharge. Pattern 4 neonates (n = 3) responded to the initial trial of iNO, but developed a sustained dependence on iNO for 3 to 6 weeks. All three died and lung histology revealed severe pulmonary hypoplasia and dysplasia. These neonates (pattern 4) not only required iNO for a longer period of time than did the sustained responders (pattern 3), but they required significantly higher doses of iNO during their first 5 days of iNO therapy. Conclusions. Early responses to iNO may not be sustained. Neonates with pulmonary hypoplasia and dysplasia may have a decreased sensitivity and differing time course of response to iNO when compared with patients who have PPHN in fully developed lungs.


Respiration ◽  
2010 ◽  
Vol 79 (4) ◽  
pp. 341-345 ◽  
Author(s):  
Rebecca L. Toonkel ◽  
Alain C. Borczuk ◽  
Gregory D. Pearson ◽  
Evelyn M. Horn ◽  
Byron M. Thomashow

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Nihat Demir ◽  
Erdal Peker ◽  
İbrahim Ece ◽  
Sultan Kaba ◽  
Kemal Ağengin ◽  
...  

Short-rib polydactyly syndrome is an autosomal recessively inherited lethal skeletal dysplasia. The syndrome is characterized by marked narrow fetal thorax, short extremities, micromelia, cleft palate/lip, polydactyly, cardiac and renal abnormalities, and genital malformations. In cases with pulmonary hypoplasia, persistent pulmonary hypertension of the newborn can develop. In this paper, we present a term newborn with persistent pulmonary hypertension of the newborn, which has developed secondary to short-rib polydactyly syndrome and was resistant to therapy with inhaled nitric oxide and oral sildenafil.


Author(s):  
Zahra Ansari Aval ◽  
Mohsen Mirhosseini ◽  
Sepideh Jafari Naeini

A young woman with systemic sclerosis, hypothyroidism and pulmonary hypertension was admitted to our center with massive pericardial effusion and left ventricular (LV) collapse. Despite undergoing successful pericardiocentesis, she passed away a month later. The best therapeutic approach in this situation remains to be determined


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