SURGERY IN A HEMOPHILIAC CHILD WITH INTRACRANIAL HEMORRHAGE

PEDIATRICS ◽  
1961 ◽  
Vol 28 (5) ◽  
pp. 800-804
Author(s):  
John H. Fuerth ◽  
Paul Teng ◽  
Erwin Goldenberg

THE UNUSUAL bleeding tendency in hemophiliacs has been known since biblical times, and its hazards have been recognized in even such simple surgical procedures as circumcision.1 Perhaps the most dangerous complication of hemophilia is bleeding into the central nervous system.2 It therefore seems worthwhile to report the case of a 2-year-old hemophiliac who survived several intracranial hemorrhages, with two surgical interventions, but who 4 months later had a fourth and fatal hemorrhage. CASE REPORT History D. H. was a 2-year-old hemophiliac with numerous admissions to the Kaiser Foundation Hospital, Los Angeles, for bleeding episodes. He was born at another hospital, was circumcised shortly after birth and had excessive bleeding following this. His first admission was at the age of 1 year for bleeding following a tongue bite. At that time he had an abnormal result of a prothrombin consumption test, with 55% residual prothrombin in the serum. The prothrombin consumption was corrected by fresh normal plasma and barium sulfate adsorbed plasma, but not by serum or plasma from a known hemophiliac type A. The diagnosis of hemophilia type A was thus established. He had two brothers who were investigated and found to be normal. There was no abnormal bleeding tendency on his father's side, but two uncles of his mother and two first cousins were said to be "bleeders." No further details were known. Physical and Laboratory Findings The boy was admitted to the hospital on the evening of October 13, 1957, with a history of vomiting and progressive lethargy of 2 days' duration.

1987 ◽  
Author(s):  
A Ihara ◽  
Y Kobayashi ◽  
Y Aramitsu ◽  
Y Hara ◽  
K Fujimura ◽  
...  

A 17-year-old boy with a life long history of easy bruising, epistaxis, subcutaneous hematoma and prolonged bleeding time from minor injuries, was initially diagnosed as having von Willebrand's disese, when he presented epistaxis at 2 years of age. Since his initial diagnosis, he has been treated with cryoprecipitate on many occasions to correct his bleeding tendency. The laboratory findings in the patient and his family are summarized in the table. The patient's mother, father and one brother have not complained any bleeding tendency.Multimeric analysis of vWF using SDS agarose gels showed absence of large multimer in the patient's plasma, decreased large multimer in mother's and brother's plasma. When the patient's PRP was tested for aggregation and release of ATP by ADP, epinephrine and collagen, using lumi aggregometer, platelet aggregation was abnormal as shown by disaggregation and almost no ATP activity was detectable in the supernatant. ATP contents of patient's and father's platelet was about 50% of normal platelets. Washed platelets of the patient in normal plasma did not aggregate normally, but washed normal platelets in the patient's plasma aggregate normally. No inhibitor of vWF could be demonstrated in the patient's plasma. Clot retraction was normal. These findings suggest that our patient has inherited vWD from materal side and the platelet aggregation defect, probably a kind of storage pool disease, from the paternal side of the family.


2021 ◽  
Vol 49 (1) ◽  
Author(s):  
Kishor Pandey ◽  
Anup Bastola ◽  
Gong Haiyan ◽  
Uttam Raj Pyakurel ◽  
Basu Dev Pandey ◽  
...  

Abstract Background Cutaneous leishmaniasis (CL) is endemic in 70 countries worldwide. Nepal is considered non-endemic for CL and hence the control program is targeted to visceral leishmaniasis (VL) only. Here, we report the emergence of CL cases in different parts of Nepal. Methods We analyzed the CL and VL cases reported to Epidemiology and Diseases Control Division (EDCD), Ministry of Health and Population, Nepal through District Health Information System 2 (DHIS-2) and Early Warning and Reporting System (EWRS) during the past 4 years (2016–2019). Any laboratory-confirmed case was included in the study. Demographic and clinical details of each patient were transcribed into Excel sheets, verified with the case report forms and analyzed. Results VL has been reported in Nepal since 1980, but CL was reported very recently. From 2016 to 2019, 42 CL cases were reported from 26 different hospitals to EDCD which had been diagnosed on the basis of clinical presentation, and laboratory findings (demonstration of amastigotes in Giemsa-stained smears and rK39 test results). Majority of the patients (31.0%, 13/42) visited to the hospital within 1–6 months of onset of lesions. Facial region (38.1%, 16/42) was the common place where lesions were found ompared to other exposed parts of the body. CL was successfully treated with miltefosine for 28 days. The majority of CL patients did not have history of travel outside the endemic areas and there was no report of sandfly from these areas. Conclusion These evidences highlight that the Government of Nepal need to pay more efforts on CL and include it in differential diagnosis by clinicians, and plan for an active surveillance when the country is targeting leishmaniasis elimination by the year 2025 with the decreasing number of VL cases.


1975 ◽  
Author(s):  
U. Seligsohn ◽  
N. Reichert ◽  
B. Ramot

Glanzmann’s thrombasthenia has been observed in Israel relatively frequently especially among Iraqi Jews. This paper presents a countrywide survey. Twenty two patients (12 males and 10 females) fulfilled the minimal criteria for establishing the diagnosis of thrombasthenia which were: A lifelong history of a bleeding tendency, a normal platelet count, absent clot retraction and at least 2 of the following: Prolonged bleeding time, isolated platelets on a blood smear and markedly impaired ADP aggregation. The frequencies of bleeding manifestations were: Purpura 100%, monorrhagia 100%, following circumcision 100%, epistaxis and gum 86%, gastrointestinal 68%, hematuria 32% and hemarthrosis 9%. Platelet transfusions seemed beneficial in preventing excessive bleeding in 4 patients who underwent a Caeserian section, 2 circumcisions and extensive abdominal surgery, respectively.Genetic analysis was possible for 21 patients who belong to 12 unrelated kindreds: 11 Jewish (of whom 10 are Iraqi) and one Arab. Parental consanguinity was present in 9 of sibships. The corrected segregation ratio was 0.20, which is compatible with autosomal recessive inheritance. Twelve obligatory carriers were available for studies. In none of them was it possible to detect impaired platelet functions.


2010 ◽  
Vol 134 (10) ◽  
pp. 1560-1563
Author(s):  
Jack Moskowitz ◽  
Geoffrey Mendelsohn

Abstract Neurocysticercosis is a significant public health issue within the United States. Although cysticercosis was once thought to have been eradicated in the United States, the number of documented cases is rising and immigrants from endemic areas are at the highest risk for acquiring and developing this disease. The clinical presentation of neurocysticercosis is variable and vague neurologic symptoms or sudden unexplained death in individuals with risk factors may be the only available information warranting a consideration of neurocysticercosis. Radiologic and laboratory findings can help guide medical and surgical interventions, while histologic confirmation establishes a more definitive diagnosis. Encysted larvae can be found throughout the central nervous system and undergo progressive stages of decay. Degenerating vesicles elicit an inflammatory response, involving surrounding structures, and cause the major clinical symptomatology.


2011 ◽  
Vol 31 (S 01) ◽  
pp. S11-S13 ◽  
Author(s):  
J. Oppermann ◽  
A. Siegemund ◽  
R. Schobess ◽  
U. Scholz

SummaryThe von Willebrand-Jürgens syndrome (VWJS) type 1 is a common hereditary bleeding disorder with a bleeding tendency located especially in the mucous membranes. Women suffering from VWJS type 1 show menorrhagia and prolonged postoperative bleedings. During pregnancy the clinical presentation varies by the increase of the von Willebrand factors.In this article the laboratory findings and the clinical presentation of patients with VWJS during pregnancy was examined. The necessity of interventions during pregnancy and at the time of delivery was under consideration.


1978 ◽  
Vol 40 (02) ◽  
pp. 532-541 ◽  
Author(s):  
Anders Lagrelius ◽  
Nils-Olov Lunell ◽  
Margareta Blombäck

SummaryThe aim of the present study was to investigate the effect on blood coagulation and fibrinolysis of a natural oestrogen preparation, piperazine oestrone sulphate, prospectively in menopausal women. Scopolamine was given to the control group.The women were investigated before and during treatment with regard to factors VIII, VII, X, V, fibrinopeptide A, antithrombin III, plasminogen, rapid antiplasmin and α1-antitrypsin. There was no significant change towards hypercoagulability or decreased fibrinolysis in any group. In the oestrogen group, however, a tendency towards an increased level of plasminogen and a decreased level of antiplasmin was demonstrated. In the scopolamine group there was an unexpected fall in factors X and V and also in plasminogen and α1,-antitrypsin. A low level of some blood coagulation factors in some of the women before treatment is somewhat astonishing; none of them had any history of excessive bleeding.


1999 ◽  
Vol 22 (1) ◽  
pp. 126-128
Author(s):  
Catherine S. Ramirez

Throughout the twentieth century (and now the twenty-first), the specter of a Latina/o past, present, and future has haunted the myth of Los Angeles as a sunny, bucolic paradise. At the same time it has loomed behind narratives of the city as a dystopic, urban nightmare. In the 1940s Carey McWilliams pointed to the fabrication of a “Spanish fantasy heritage” that made Los Angeles the bygone home of fair señoritas, genteel caballeros and benevolent mission padres. Meanwhile, the dominant Angeleno press invented a “zoot” (read Mexican-American) crime wave. Unlike the aristocratic, European Californias/os of lore, the Mexican/American “gangsters” of the 1940s were described as racial mongrels. What's more, the newspapers explicitly identified them as the sons and daughters of immigrants-thus eliding any link they may have had to the Californias/os of the eighteenth and nineteenth centuries or to the history of Los Angeles in general.


2016 ◽  
Vol 19 (1) ◽  
pp. 023 ◽  
Author(s):  
Mehmet Yildirim ◽  
Recep Ustaalioglu ◽  
Murat Erkan ◽  
Bala Basak Oven Ustaalioglu ◽  
Hatice Demirbag ◽  
...  

<strong>Background:</strong> Patients with recurrent pericardial effusion and pericardial tamponade are usually treated in thoracic surgery clinics by VATS (video-assisted thoracoscopic surgery) or open pericardial window operation. The diagnostic importance of pathological evaluation of the pericardial fluid and tissue in the same patients has been reported in few studies. We reviewed pathological examination of the pericardial tissue and fluid specimens and the effect on the clinical treatment in our clinic, and compared the results with the literature. <br /><strong>Methods:</strong> We retrospectively analyzed 174 patients who underwent pericardial window operation due to pericardial tamponade or recurrent pericardial effusion. For all patients both the results of the pericardial fluid and pericardial biopsy specimen were evaluated. Clinicopathological factors were analyzed by using descriptive analysis. <br /><strong>Results:</strong> Median age was 61 (range, 20-94 years). The most common benign diagnosis was chronic inflammation (94 patients) by pericardial biopsy. History of malignancy was present in 28 patients (16.1%) and the most common disease was lung cancer (14 patients). A total of 24 patients (13.8%) could be diagnosed as having malignancy by pericardial fluid or pericardial biopsy examination. The malignancy was recognized for 12 patients who had a history of cancer; 9 of 12 with pericardial biopsy, 7 diagnosed by pericardial fluid. Twelve of 156 patients were recognized as having underlying malignancy by pericardial biopsy (n = 9) or fluid examination (n = 10), without known malignancy previously. <br /><strong>Conclusion:</strong> Recurrent pericardial effusion/pericardial tamponade are entities frequently diagnosed, and surgical interventions may be needed either for diagnosis and/or treatment, but specific etiology can rarely be obtained in spite of pathological examination of either pericardial tissue or fluid. For increasing the probability of a specific diagnosis both the pericardial fluid and the pericardial tissues have to be sent for pathologic examination.


2019 ◽  
Vol 8 (1) ◽  
pp. 5
Author(s):  
Dimitrios Panagopoulos

Background: Meningeal melanocytoma is a rare benign tumor, most frequently located in the posterior fossa and spinal canal. Our objective is to illustrate a case of this tumor that originated in the thoracolumbar area of the spine and had an uneventful clinical course after total resection. Case description: We present the case of a 59 years old woman who presented with a medical history of ongoing neurological deterioration due to spastic paresis of the lower extremities. MRI of the thoracolumbar region identified a melanocytic melanoma as the underlying cause. Conclusions: Melanocytic tumors of the central nervous system have a typical appearance on MRI scans, varying with the content and distribution of melanin. However, the differential diagnosis between malignant melanoma and melanocytoma still depends on pathological criteria. Spinal meningeal melanocytoma has a benign course, and it is amenable for gross total resection. The outcome is favorable following complete resection.


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