IDIOPATHIC PULMONARY HEMOSIDEROSIS

PEDIATRICS ◽  
1957 ◽  
Vol 19 (6) ◽  
pp. 1101-1108
Author(s):  
Konrad H. Soergel
Keyword(s):  
Young Patients ◽  
Review Of The Literature ◽  
Mild Form ◽  
Pathogenetic Mechanism ◽  
Diagnostic Measures ◽  
Pulmonary Hemosiderosis ◽  
Number Of Patients ◽  
Antihistaminic Drugs ◽  
Idiopathic Pulmonary Hemosiderosis ◽  
Better Than

Two fatal cases of idiopathic pulmonary hemosiderosis are reported. A review of the literature shows the prognosis of this disease to be, at least in young patients, somewhat better than generally thought. A positive diagnosis in the living patient is possible with the help of certain diagnostic measures which are discussed. Increasing familiarity with the rather typical manifestations of this disorder may lead to the discovery of a larger number of patients who have a mild form of the disease. The value of splenectomy and therapy with adrenocorticotropin and cortisone is still questionable, but further trials are necessary, possibly together with the use of antihistaminic drugs. Intermittent increases in pressure in the pulmonary circulation, due to a defective vasomotor control, appears to be the most likely pathogenetic mechanism but more investigations are needed to arrive at any positive conclusion.

scholarly journals Idiopathic pulmonary hemosiderosis presenting in an adult: A case report and review of the literature

Lung India ◽  
2015 ◽  
Vol 32 (4) ◽  
pp. 395 ◽  
Author(s):  
AbhayP Vakil ◽  
SamirS Sarkar ◽  
KhalidM Sherani ◽  
HineshN Upadhyay ◽  
FarhaK Sherani
Keyword(s):  
Case Report ◽  
Review Of The Literature ◽  
Pulmonary Hemosiderosis ◽  
Idiopathic Pulmonary Hemosiderosis

Hämophilie-Zentrum Bonn 1980 – 2009

2011 ◽  
Vol 31 (S 01) ◽  
pp. S4-S10 ◽  
Author(s):  
I. Besmens ◽  
H.-H. Brackmann ◽  
J. Oldenburg
Keyword(s):  
Young Patients ◽  
Severe Form ◽  
Coagulation Disorder ◽  
Care Providers ◽  
Long Distance ◽  
Patient Age ◽  
Patient Âgé ◽  
Number Of Patients ◽  
Clotting Disorder ◽  
Regional Character

SummaryThe Bonn Haemophilia Care Center provides patient care on a superregional level. The centre’s large service area is, in part, due to the introduction of haemophilia home treatment and related to this the individualized prophylaxis in children and adults by Egli and Brack-mann in Bonn in the early 1970s, that represented a milestone in German haemophilia therapy. Epidemiologic patient data from the two selected time points, 1980 and 2009, are evaluated to illustrate the change in the composition of the patient clientele. In 1980 a total of 639 patients were treated at the Bonn Haemophilia Center. 529 patients exhibited a severe form and 110 a non-severe form of the respective clotting disorder. In 2009 the Bonn Haemophilia Center took care for a total of 837 patients. There were 445 patients who suffered from a severe form of the considered clotting disorder while 392 showed a non-severe course. The number of less severely affected patients has increased significantly in 2009. Patients in 1980 were predominantly suffering from a severe form and most had to travel more than 150 km from their homes to the treatment center. In 2009 the number of patients living a medium-long distance from the care provider has significantly increased while the number of patients living more than 150km from the center has decreased. Comparing 2009 to 1980 a growth of the center’s regional character becomes apparent, especially when patient age and severity of the coagulation disorder are taken into consideration. The regional character was more strongly pronounced with milder disease severity and lower patient age. Due to the existence of well established primary haemophilia care in CCCs in Germany, the trend for the recent years is that the proportion of young patients that choose haemophilia care providers closer to their homes is increasing.

scholarly journals Acute Pancreatitis as a Complication of Antiepileptic Treatment: Case Series and Review of the Literature

2021 ◽  
Vol 13 (1) ◽  
pp. 98-103
Author(s):  
Agnieszka Pawłowska-Kamieniak ◽  
Paulina Krawiec ◽  
Elżbieta Pac-Kożuchowska
Keyword(s):  
Valproic Acid ◽  
Acute Pancreatitis ◽  
Metabolic Disorders ◽  
Genetic Factors ◽  
Clinical Symptoms ◽  
Gallstone Disease ◽  
Young Patients ◽  
Case Series ◽  
Review Of The Literature ◽  
Acid Therapy

Acute pancreatitis (AP) appears to be rare disease in childhood. In children, it has a different aetiology and course, and requires different management than in adult patients. The diagnosis of AP is based on at least two of the three criteria, which include typical clinical symptoms, abnormalities in laboratory tests and/or imaging studies of the pancreas. There are many known causes leading to AP in children including infections, blunt abdominal trauma, genetic factors, gallstone disease, metabolic disorders, anatomical defects of the pancreas, systemic diseases, as well as drugs, including antiepileptic drugs, and especially preparations of valproic acid. In our study, we present four cases of young patients diagnosed with acute pancreatitis as a complication of valproic acid therapy and we present a review of the literature. We believe that the activity of pancreatic enzymes should be monitored in children treated with valproate preparations in the case of clinical symptoms suggesting AP.

scholarly journals Medical Adjuvants in the Treatment of Surgically Refractory Arteriovenous Malformations of the Head and Face: Case Report and Review of Literature

2021 ◽  
pp. 1-7
Author(s):  
Vaidya Govindarajan ◽  
Joshua D. Burks ◽  
Evan M. Luther ◽  
John W. Thompson ◽  
Robert M. Starke
Keyword(s):  
Arteriovenous Malformations ◽  
Mtor Inhibitors ◽  
Molecular Target ◽  
Review Of Literature ◽  
Review Of The Literature ◽  
Early Results ◽  
Number Of Patients ◽  
Initiation Of Therapy ◽  
The Brain ◽  
Radiographic Improvement

<b><i>Background:</i></b> Arteriovenous malformations (AVMs) of the brain and face present unique challenges for clinicians. Cerebral AVMs may induce hemorrhage or form aneurysms, while facial AVMs can cause significant disfigurement and pain. Moreover, facial AVMs often draw blood supply from arteries providing critical blood flow to other important structures of the head which may make them impossible to treat curatively. Medical adjuvants may be an important consideration in the management of these patients. <b><i>Summary:</i></b> We conducted a systematic review of the literature to identify other instances of molecular target of rapamycin (mTOR) inhibitors used as medical adjuvants for the treatment of cranial and facial AVMs. We also present 2 cases from our own institution where patients were treated with partial embolization, followed by adjuvant therapy with rapamycin. After screening a total of 75 articles, 7 were identified which described use of rapamycin in the treatment of inoperable cranial or facial AVM. In total, 21 cases were reviewed. The median treatment duration was 12 months (3–24.5 months), and the highest recorded dose was 3.5 mg/m<sup>2</sup>. 76.2% of patients demonstrated at least a partial response to rapamycin therapy. In 2 patients treated at our institution, symptomatic and radiographic improvement were noted 6 months after initiation of therapy. <b><i>Key Messages:</i></b> Early results have been encouraging in a small number of patients with inoperable AVM of the head and face treated with mTOR inhibitors. Further study of medical adjuvants such as rapamycin may be worthwhile.

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