Papillon-Lefevre syndrome: a study of the long-term clinical course of recurrent pyogenic infections and the effects of etretinate treatment

1988 ◽  
Vol 119 (6) ◽  
pp. 731-736 ◽  
Author(s):  
R. BERGMAN ◽  
R. FRIEDMAN-BIRNBAUM
Heart ◽  
2001 ◽  
Vol 86 (1) ◽  
pp. 88-90
Author(s):  
D Boshoff ◽  
L Mertens ◽  
M Gewillig

A 14 year old girl presented with severe tricuspid regurgitation after she was diagnosed with “transient tricuspid regurgitation of the newborn”. In the neonatal period she had presented with severe tricuspid regurgitation without an obvious underlying anatomical cause. This spontaneously regressed during the first months of life. She was dismissed from follow up at the age of 5 years after complete normalisation of the clinical and echocardiographic examination. The subsequent evolution and management of the patient, as well as the possible pathogenesis responsible for the unusual clinical course, is discussed. This case stresses the importance of long term follow up of patients with transient tricuspid regurgitation.


2021 ◽  
pp. 135245852110196
Author(s):  
Rosa Cortese ◽  
Marco Battaglini ◽  
Francesca Parodi ◽  
Maria Laura Stromillo ◽  
Emilio Portaccio ◽  
...  

The mechanisms responsible for the favorable clinical course in multiple sclerosis (MS) remain unclear. In this longitudinal study, we assessed whether magnetic resonance imaging (MRI)-based changes in focal and diffuse brain damage are associated with a long-term favorable MS diseases course. We found that global brain and gray matter (GM) atrophy changes were milder in MS patients with long-standing disease (⩾30 years from onset) and favorable (no/minimal disability) clinical course than in sex-age-matched disable MS patients, independently of lesions accumulation. Data showed that different trajectories of volume changes, as reflected by mild GM atrophy, may characterize patients with long-term favorable evolution.


Author(s):  
Amrit K. Kamboj ◽  
Saam Dilmaghani ◽  
Beth A. Schueler ◽  
John M. Barlow ◽  
David A. Katzka
Keyword(s):  

2010 ◽  
Vol 84 (7) ◽  
pp. 3576-3585 ◽  
Author(s):  
Marit J. van Gils ◽  
Evelien M. Bunnik ◽  
Judith A. Burger ◽  
Yodit Jacob ◽  
Becky Schweighardt ◽  
...  

ABSTRACT A substantial proportion of human immunodeficiency virus type 1 (HIV-1)-infected individuals has cross-reactive neutralizing activity in serum, with a similar prevalence in progressors and long-term nonprogressors (LTNP). We studied whether disease progression in the face of cross-reactive neutralizing serum activity is due to fading neutralizing humoral immunity over time or to viral escape. In three LTNP and three progressors, high-titer cross-reactive HIV-1-specific neutralizing activity in serum against a multiclade pseudovirus panel was preserved during the entire clinical course of infection, even after AIDS diagnosis in progressors. However, while early HIV-1 variants from all six individuals could be neutralized by autologous serum, the autologous neutralizing activity declined during chronic infection. This could be attributed to viral escape and the apparent inability of the host to elicit neutralizing antibodies to the newly emerging viral escape variants. Escape from autologous neutralizing activity was not associated with a reduction in the viral replication rate in vitro. Escape from autologous serum with cross-reactive neutralizing activity coincided with an increase in the length of the variable loops and in the number of potential N-linked glycosylation sites in the viral envelope. Positive selection pressure was observed in the variable regions in envelope, suggesting that, at least in these individuals, these regions are targeted by humoral immunity with cross-reactive potential. Our results may imply that the ability of HIV-1 to rapidly escape cross-reactive autologous neutralizing antibody responses without the loss of viral fitness is the underlying explanation for the absent effect of potent cross-reactive neutralizing humoral immunity on the clinical course of infection.


Author(s):  
Yusuke Murakami ◽  
Yoshito Koyanagi ◽  
Masatoshi Fukushima ◽  
Marika Yoshimura ◽  
Kohta Fujiwara ◽  
...  

2021 ◽  
Author(s):  
Haruka Takahashi ◽  
Takashi Sano ◽  
Sayumi Kawamura ◽  
Keiko Sano ◽  
Ryoma Miyasaka ◽  
...  

2013 ◽  
Vol 5 (Suppl 2) ◽  
pp. A17.1-A17
Author(s):  
A Elhadi ◽  
GAC Mendes ◽  
K Almefty ◽  
C McDougall ◽  
P Nakaji ◽  
...  
Keyword(s):  

2016 ◽  
Vol 88 (4) ◽  
pp. 249 ◽  
Author(s):  
Kemal Sarica ◽  
Fatma Narter ◽  
Kubilay Sabuncu ◽  
Ahmet Akca ◽  
Utku Can ◽  
...  

Objective: To investigate the possible effects of dietary, patient and stone related factors on the clinical course of the stone disease as well as the body and renal growth status of the infants. Patients and Methods: A total of 50 children with an history of stone disease during infancy period were studied. Patient (anatomical abnormalities, urinary tract infection - UTI, associated morbidities), stone (obstruction, UTI and required interventions) and lastly dietary (duration of sole breast feeding, formula feeding) related factors which may affect the clinical course of the disease were all evaluated for their effects on the body and renal growth during long-term follow-up. Results: Mean age of the children was 2.40 ± 2.65 years. Our findings demonstrated that infants receiving longer period of breast feeding without formula addition seemed to have a higher rate of normal growth percentile values when compared with the other children. Again, higher frequency of UTI and stone attacks affected the growth status of the infants in a remarkable manner than the other cases. Our findings also demonstrated that thorough a close follow-up and appropriately taken measures; the possible growth retardation as well as renal growth problems could be avoided in children beginning to suffer from stone disease during infancy period. Conclusions: Duration of breast feeding, frequency of UTI, number of stone attacks and stone removal procedures are crucial factors for the clinical course of stone disease in infants that may affect the body as well as kidney growth during long-term follow-up.


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