scholarly journals PENDERITA DENGAN HEMOKROMATOSIS PRIMER

Author(s):  
Kadek Mulyantari ◽  
A.A.Wiradewi Lestari ◽  
A.A.N. Subawa ◽  
Tjokorda Gede Oka ◽  
Sudewa Djelantik

Primary Hemochromatosis is a hereditary disease that occurs predominantly in man. Among men, clinical signs and symptomsfrequently appears on 40 years until more than 60 years of age. Meanwhile, the signs and symptoms among women appear on 50 yearsof age or after menopause. It is a very rare case in children or young adult. Secondary hemochromatosis can be differentiated fromprimary hemochromatosis based on existence of other underlying disease and secondary hemochromatosis often occurs in patient withmultiple blood transfusions. The diagnosis of primary hemochromatosis is confirmed by chromosomal test and liver biopsy to confirmthe liver damage caused by excessive iron accumulation. The main treatment of primary hemochromatosis is phlebotomy. The purposeof this method is to remove overload iron in body. In this case, the patient was man, unmarried, 51 years old, Australian. Four yearsago, he had complained about arthropathies, chronic asthenia, depression, decreased of concentration and sexual desire. Laboratoryevaluation revealed Ferritin level 2126 ug/L and transferrin saturation always more than 99%. Liver function tests also increasedsignificantly. Some of his family’s members have the same disease as he has. He was diagnosed as primary hemochromatosis and hadperformed phlebotomy routinely. After phlebotomy has done, he recovered based on clinical and laboratorial findings.

2021 ◽  
Vol 6 (4) ◽  
pp. 269-274
Author(s):  
Kaushlendra Kumar Pandey ◽  
Wilma Delphine Silvia CR ◽  
Aparna Pandey ◽  
Asha Agarwal

Renal diseases of different origin and nature may produce essentially similar disturbances of renal functions and may have clinical similarities and hence there was a need to classify renal diseases more scientifically. The basic approach was to correlate clinical signs and symptoms with histological changes in the tissue, using both simple and special staining techniques so as to reach to a definitive diagnosis.The present study was conducted on renal biopsy referred to pathology department. Criteria for successful biopsy were as follows-Adequate biopsy sample size, correct processing of specimen, informed interpretation and issue of an accurate report. A total of 29 renal biopsies were examined. In minimal change disease, only in 4 patients the glomerulus was sclerosed. Membranous glomerulonephritis comprised of the maximum number of cases (9/30). Total of 3 cases of renal biopsies revealed amyloidosis. Focal amyloid deposits with deposits either near the hilum or perivascular areas were found in 33.3% of cases, while extensive amyloid deposits were found in 33.3% of the cases.It is necessary to determine both the type of renal disease and the cause of the primary disorder in order to make the diagnosis and various staining techniques play a very helpful role. The likelihood that the biopsy specimen accurately reflects the type and severity of the underlying disease is directly related to both the diffuseness of the disease process and the amount of tissue examined.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4249-4249 ◽  
Author(s):  
Anat Gafter-Gvili ◽  
Benaya Rozen-Zvi ◽  
Liat Vidal ◽  
Uzi Gafter ◽  
Johan F. Vansteenkiste ◽  
...  

Abstract Abstract 4249 Anemia is an almost universal complication in cancer patients and an important contributor to morbidity of malignancy. For chemotherapy induced anemia (CIA), currently available guidelines recommend the use of erythropoiesis stimulating agents (ESAs), but are less certain regarding intravenous (IV) iron administration. Methods: Systematic review and meta-analysis of randomized controlled trials comparing IV iron with no iron or oral iron or for the treatment of CIA. A comprehensive search of The Cochrane Library, MEDLINE, conference proceedings and references was conducted until 2010. Two reviewers appraised the quality of trials and extracted data. Outcomes assessed were: number of patients achieving a hematopoietic response (defined as a hemoglobin level increase by more than 2gr/dl or an increase >12gr/dl), number of patients requiring blood transfusions, ferritin level, transferrin saturation, all-cause mortality, adverse events. For dichotomous data, relative risks (RR) with 95% confidence intervals (CIs) were estimated and pooled, for continuous data weighted mean differences (WMDs) were calculated. Results: Ten randomized trials including 1637 patients and conducted between the years 2004 and 2009 were included. The intervention was IV iron dextran in two trials, ferric gluconate in three trials, iron sucrose in four and one trial assessed both ferric gluconate and iron sucrose. ESAs were administered in nine trials. Most trials excluded patients with true iron deficiency. Treatment with IV iron significantly increased the number of patients achieving a hematopoietic response (RR 1.31 [95% CI 1.15, 1.49], 8 trials, figure), and significantly reduced the number of patients who required blood transfusions (RR 0.77 [95% CI 0.64, 0.94], 9 trials). When analyzed according to type of ESA administered to both arms of the trials, there was a consistent significant increase in the number of patients with a hematopoietic response in the IV iron arm, for all ESA types: darbepoetin alpha (RR 1.18 [95% CI 1.11, 1.26], 5 trials), epoetin alpha (RR 1.85 [95% CI 1.25, 2.74], 2 trials), epoetin beta (RR 1.66 [95% CI 1.18, 2.34], 1 trial). The ferritin level at the end of the trial was significantly increased in the IV iron arm compared with the control arm (WMD 234.47 [95% CI 136.94, 332.01], 4 trials), as was transferrin saturation(WMD 9.98 [95% CI 9.21, 10.75] 3 trials). There was no difference in all-cause mortality at the end of follow-up between the IV iron arm and the control arm (RR 1.01 [95% CI 0.65, 1.56], 6 trials). There was no difference in the rate of serious adverse events requiring intervention (RR 1.11 [95%CI 0.93, 1.31], 7 trials), in the occurrence of thromboembolic events (RR 1.03 [95%CI 0.59, 1.80], 4 trials) or cardiovascular events (RR 0.87 [95%CI 0.45, 1.70] [0.40-3.39], 5 trials). Conclusions: Our systematic review demonstrates better hematopoietic response and less need for transfusion, with no difference in mortality or adverse events. Thus, our results support the use of IV iron for the treatment of cancer patients with anemia. Figure: Number of patients who achieved a hematopoietic response in the IV iron arm compared to control Figure:. Number of patients who achieved a hematopoietic response in the IV iron arm compared to control Disclosures: Shpilberg: Roche: Consultancy, Honoraria.


2009 ◽  
Vol 03 (01) ◽  
pp. 19
Author(s):  
Giuseppe Lippi ◽  
Gian Cesare Guidi ◽  
◽  

The diagnosis of septic disseminated intravascular coagulation (DIC) relies on clinical signs and symptoms, identification of the underlying disease and results of laboratory testing. Since no single test result alone can definitely establish or rule out the diagnosis, the laboratory diagnostics of septic DIC encompass a combination of tests for which simple diagnostic algorithms are now available. Global tests of haemostasis provide evidence of activation of blood coagulation and, ultimately, consumption of coagulation factors, but their diagnostic efficiency is as yet questionable. Fibrinolytic markers, namely D-dimer, reflect the extent of activation of both coagulation and fibrinolysis, so a normal value can be used in a ruling-out strategy. Decreased levels of the natural inhibitors are frequently observed in patients with septic DIC, but antithrombin and protein C measurements are not incorporated in any of the widely used diagnostic algorithms. Among the inflammatory biomarkers, procalcitonin is currently regarded as the gold standard to differentiate the type of infection and guide antibiotic therapy, but its clinical usefulness in identifying and predicting the outcome of patients with septic DIC is still circumstantial.


1992 ◽  
Vol 2 (1) ◽  
pp. 73-77 ◽  
Author(s):  
Louis Tsun-cheung Chow ◽  
Wing-hing Chow ◽  
Christopher Chun-hing Tse ◽  
Eric Hon-man Wong ◽  
Koon-sang Wong ◽  
...  

SummaryTwo patients are described with sudden death as the first presenting symptom of Kawasaki disease. The first patient died of cardiac tamponade as a result of rupture of a coronary arterial aneurysm at the age of three months and nineteen days. The second patient, one and half years old, developed acute myocardial infarction secondary to coronary arterial aneurysm and thrombosis. The true nature of their underlying disease was realized only after postmortem examination. Their clinical signs and symptoms of Kawasaki disease might be so mild as to escape recognition. A high index of suspicion should be exercised by the clinician in order not to miss this condition, especially in places where Kawasaki disease is uncommon.


2016 ◽  
Vol 7 (04) ◽  
pp. 554-558 ◽  
Author(s):  
Samira Yadegari ◽  
Askar Ghorbani ◽  
S Roohollah Miri ◽  
Mohammad Abdollahi ◽  
Mohsen Rostami

ABSTRACT Introduction: Despite increasing the use of magnetic resonance imaging (MRI), cerebral venous sinus thrombosis (CVST) has remained an under-diagnosed condition. In this study, characteristics and frequency of various risk factors of CVST patients in a tertiary referral hospital were closely assessed. Methods: Patients with an unequivocal diagnosis of CVST confirmed by MRI and magnetic resonance venography during 6 years of the study were included. All data from the onset of symptoms regarding clinical signs and symptoms, hospital admission, seasonal distribution, medical and drug history, thrombophilic profile, D-dimer, neuroimaging, cerebrospinal fluid findings, mortality, and outcome were collected and closely analyzed. Result: A total of 53 patients with female to male ratio of 3.07 and mean age of 33.7 years were included in the study. Headache and papilledema were the most frequent clinical features (44 and 36 patients, respectively). An underlying disease (diagnosed previously or after admission) was the most common identified risk factor for CVST in both females and males (21 patients). A total of 15 women used the oral contraceptive pill (OCP) where 12 of them had simultaneously other predisposing factors. Overall, 19 patients (36%) had more than one contributing factor. D-dimer had a sensitivity of 71.4% in CVST patients. The mortality of patients in this study was 3.7% (n = 2). Focal neurologic deficit and multicranial nerve palsy were associated with poor outcome which defined as death, recurrence, and massive intracranial hemorrhage due to anticoagulation (P = 0.050 and 0.004, respectively). Conclusion: Unlike most of the CVST studies in which OCP was the main factor; in this study, an underlying disease was the most identified cause. Considering the high probability of multiple risk factors in CVST that was shown by this study, appropriate work up should be noted to uncover them.


2019 ◽  
Vol 10 (02) ◽  
pp. 364-366
Author(s):  
Jaypalsinh Gohil ◽  
Gopikrishnan Rajasekar ◽  
Pankaj Shivhare ◽  
Prakash Nair ◽  
Mathew Abraham

ABSTRACTEpidermoid cysts are benign slow-growing tumors, clinical signs and symptoms at the time of presentation are related to the location and extent of the lesion. The motor fibers of the trigeminal nerve are fairly resistant to extraneous compression. Intracranial lesions in the vicinity of the trigeminal nerve usually present with sensory symptoms without affecting the motor component. We report a rare case of a middle cranial fossa epidermoid presenting with pure motor trigeminal neuropathy.


2016 ◽  
Vol 12 (3) ◽  
Author(s):  
Ann Tammelin

Swedish nursing homes are obliged to have a management system for systematic quality work including self-monitoring of which surveillance of infections is one part. The Department of Infection Control in Stockholm County Council has provided a simple system for infection surveillance to the nursing homes in Stockholm County since 2002. A form is filled in by registered nurses in the nursing homes at each episode of infection among the residents. A bacterial infection is defined by antibiotic prescribing and a viral infection by clinical signs and symptoms. Yearly reports of numbers of infections in each nursing home and calculated normalized figures for incidence, i.e. infections per 100 residents per year, as well as proportion of residents with urinary catheter are delivered to the medically responsible nurses in each municipality by the Department of Infection Control. Number of included residents has varied from 4,531 in 2005 to 8,157 in 2014 with a peak of 10,051 in 2009. The yearly incidences during 2005 - 2014 (cases per 100 residents) were: Urinary tract infection (UTI) 7.9-16.0, Pneumonia 3.7-5.3, Infection of chronic ulcer 3.4–6.8, Other infection in skin or soft tissue 1.4–2.9, Clostridium difficile-infection 0.2–0.7, Influenza 0–0.4 and Viral gastroenteritis 1.2–3.7. About 1 % of the residents have a suprapubic urinary catheter, 6–7 % have an indwelling urinary catheter. Knowledge about the incidence of UTI has contributed to the decrease of this infection both in residents with and without urinary catheter.


Sign in / Sign up

Export Citation Format

Share Document