Case Of Waterhouse-Friderichsen Syndrome Research Articles

Three Autopsy Cases of Non-Meningococcal Waterhouse-Friderichsen Syndrome with Hypoplastic Spleen or Post-Splenectomy Status.

We report three cases of Waterhouse-Friderichsen syndrome (WFS) that were confirmed during forensic autopsies. Case 1 involved a man in his 50s post-splenectomy. Bacteriological examination revealed Streptococcus pneumoniae (S. pneumonia). The patient was considered to have died of asphyxiation after aspirating vomit. Case 2 involved a man in his 40s. Bacteriological examination again revealed S. pneumoniae. Histopathological examination showed hypoplasia of the spleen. This patient was considered to have died of multiple-organ failure due to sepsis, disseminated intravascular coagulation, and WFS. Case 3 involved a post-splenectomy woman in her 60s with a history of systemic lupus erythematosus. Bacteriological examination revealed Streptococcus oralis. This patient was considered to have died of multiple-organ failure due to sepsis, disseminated intravascular coagulation, and WFS. These three cases were included among forensic autopsies conducted in the last 5 years. WFS has been considered a rare disease, but may be more frequent than previously assumed. If a mildly ill patient displays a sudden change in status and dies within a short period of time, we consider it necessary to perform not only bacteriological examinations, but also histopathological examination of the spleen during autopsy.

A four months infant survival case of waterhouse-friderichsen syndrome

A four months infant survival case of waterhouse-friderichsen syndrome

CT画像所見からWaterhouse-Friderichsen症候群が疑われた乳児症例

発症後急速に悪化し，画像所見よりWaterhouse-Friderichisen症候群（Waterhouse-Friderichsen syndrome, WFS）が疑われた乳児症例を経験したので報告する。症例は6ヶ月，男児。意識障害にて救急搬送された。当初，経過と頭部CT所見から低酸素脳症と診断した。入室時より高カリウム血症，低血糖，代謝性アシドーシスを認め，ショックが遷延していたが，血行動態を安定させることができたため脳保護目的で低体温療法を開始した。入室8時間後より血行動態などが急激に悪化し，播種性血管内凝固症候群（disseminated intravascular coagulation, DIC）の所見を認めた。そこで感染症を強く疑い，低体温療法を中止し抗菌薬投与を開始した。感染源精査のため全身のCTを撮像したところ腹部CTにて両側の副腎出血を認め，WFSを疑いステロイド大量投与などの治療を行ったが，治療に反応することなく入室29時間後に死亡した。乳児の感染症へのアプローチ，低体温療法の適応などを考えさせられる症例であった。

Localization and distribution of fibrinolysis inhibition in the walls of human arteries and veins

A systematic study on the location and intensity of fibrinolysis inhibition in human arteries and veins by the use of a standardized fibrin slide sandwich technique yielded data for an overall comparison of fibrinolysis inhibition along and across the human vessel wall. Arteries generally showed marked plasmin inhibition in the intimal and medial layers, but little inhibition in the adventitia. The external layer of loose connective tissue in the veins generally showed no plasmin inhibition. Plasmin inhibition was usually present in the combined intimal/medial/adventitial layer of the veins, but inhibition in these layers varied greatly according to the location of the vein in the body. Veins situated in the lower parts of the body exhibited more plasmin inhibition than veins at the upper levels. Inhibition of plasmin was found to be related to the presence of smooth muscle cells. However, in endotoxin shock, hyaline membrane disease and a case of Waterhouse-Friderichsen syndrome, additional plasmin inhibition was observed in vascular areas of loose connective tissue free from smooth muscle cells. Selective inhibition of urokinase-induced fibrinolysis was observed only in the connective tissue around the vessels of the human umbilical cord.

Distribution and variation of fibrinolytic activity in the walls of human arteries and veins.

A systematic study on the location and intensity of the fibrinolytic activity in more than 500 samples of human arteries and veins from 50 routine necropsies and 35 blood vessel biopsies was performed. Data were obtained for an overall comparison of the fibrinolytic activity along and across the walls of human blood vessels by the use of a standardized fibrin slide technique. Arteries generally showed little or no fibrinolytic activity in the intima and media but strong activity in the adventitia. Veins showed a comparable strong fibrinolytic activity in the external layer of loose connective tissue. Fibrinolytic activity in the venous intima, media and adventitia was generally weaker but varied greatly according to position in the body. Veins situated in the lower parts of the body had less fibrinolytic activity than the veins at the upper levels. Fibrinolytic activity was found to be related to the endothelium of the vasa vasorum and/or of the main lumen of the vascular wall. Increased fibrinolytic activity was observed in arteries and veins in cases of sudden death, vasogenic shock, cerebral hemorrhage and cirrhosis. Decreased fibrinolytic activity was encountered in blood vessels in cases of endotoxin shock, hyaline membrane disease and a case of Waterhouse-Friderichsen syndrome.

A case of Waterhouse-Friderichsen syndrome with recovery following the use of cortisone, aqueous adrenal cortical extract and chemotherapy.

A case of Waterhouse-Friderichsen syndrome with recovery following the use of cortisone, aqueous adrenal cortical extract and chemotherapy.

Further observations concerning hypofunction of the adrenals during early life: “Salt and water” hormone deficiency

I N A recent publication, I a theory of physiologic hypofunction of the adrenal glands early life was presented. The possibility that latent hypoadrenalism of a temporary nature may present clinical manifestations during early infancy has been long entertained but has lacked a demonstration sufficiently convincing to be accepted. Only a few publications were found which describe the use of adrenal hormones during early infancy. 2-s In these reports the adrenal hormones were used empirically. With the exception of four cases of macrogenitosomia, ~11a patients with Addison's disease, I~ a few reports of adrenal tumors, and cases of WaterhouseFriderichsen syndrome, there have been, to my knowledge, no cases of adrenal insufficiency during infancy which have been definitely proved. The purpose of this paper is threefold: I. To re-emphasize the necessity for the pediatrician to acquaint himself with the life-sustaining functions of the adrenal glands; and to think of the possibility of temporary hypofunction of these glands when dealing w~th infants during early life who present symptoms of protracted gastrointestinal disturbances, who fail to gain weight, who have an unexplained tendency to dehydration, and who respond unsatisfactori]y to the accepted therapeutic measures. 2. To present cases which I think demonstrate adequate proof of the e• ence of a fractional and transitory disturbance in adrenal function. 3. To describe a syndrome which is characteristic of this deficiency. The patients described in the following cause reports present symptoms of vomiting, failure to gain weight, and periods of unexplained dehydration a<tcompanied by ~nges in the blood chemistry that are commonly associated with adrenal insuf ~ncy. Each patient has had one or more hospitalizations, which clearly rew ~ the need of adrenal hormone therapy. A brief summary precedes the _oaf d report of each case.

Meningococcémie A ÉVolution Suraiguë Avec Purpura et Hémorragie Surrénalienne:A Propos D’un Cas De Syndrome De Waterhouse-Friderichsen

SummaryDescription of a typical case of Waterhouse-Friderichsen syndrome with extensive skin purpura, circulatory collapse, meningical reaction and hypoglycemia. At autopsy, an almost complete hemorrhagical necrosis of the adrenals was found. Cultures of blood and spinal fluid showed meningococcus. The observed symptoms result from meningococcus septicemia and from acute adrenal insufficiency. The role of the adrenals in the pathogenesis of the syndrom, in spite of recent publications, is sure.