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#46
14.11.2005, 09:13
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Цитата:
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#47
14.11.2005, 11:49
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Была сделана сигмоидоскопия с биопсией несколькими днями позже для последующего уточнения диагноза. Биопсия: искажение архитектоники восходящего отдела(left colon),лимфоплазматическое воспаление ламина проприа, острый криптит и абсцессы крипт.Т.о. признаки хр. воспаления мукозы с активным воспалением более всего предполагают IBD (inflammatory bowel disease).
Колит пролечен преднизолоном и сульфосалазином. Выписан домой на преднизолоне, мезаламине (Asacol) и 6-ти месячный курс Warfarin. Повт. УЗИ через 3 мес. - нет признаков тромбоза. Последующее наблюдение - состояние хорошее,нет болей в животе и кровавых поносов. Окончательный диагноз: УЛЬЦЕРАТИВНЫЙ КОЛИТ С ВТОРИЧНОЙ ГИПЕРКОАГУЛЯЦИЕЙ И ТРОМБОЗОМ ПОРТАЛЬНЫХ И МЕЗЕНТЕРИАЛЬНЫХ ВЕН.
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#48
14.11.2005, 14:17
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М-да... Спасибо.
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#49
14.11.2005, 14:21
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Наличие внутрисосудистого тромбоза особых сомнений не вызывало с самого начала (фрагментированные эритроциты, тромбоцитопения...) Я выбрал почку в качестве места тромбоза, т. к. течение заболевания показаось мне достаточно характерным - понос, ухудшение, потенурия, улучшение. В то же время, конечно, IBD оставалась возможной, особенно после упоминания язвы во рту. Итересно было бы исключить и холангит.
В целом хороший случай, хотя чуть запутанное описание. Спасибо.
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#50
14.11.2005, 16:14
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"Не забывая о других возможностях (например, IBD и пр)"
Алон первый сказал IBD... А описание - слово в слово из Pediatrics за август 2005 года. Там еще есть "Дискуссия". Сейчас выложу. 
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#52
14.11.2005, 18:07
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Дикуссия. (Ох как много!)
У пациента необычная комбинация диарреи, которая стала кровавой, тромбоцитопении,значительное повышение печеночных энзимов и удлиненное протромбиновое время.В ДД входит: -ГУС -Острый инфекционный гепатит -Злокачественность (malignancy) -Хр. заболевание печени. Но ни один из этих диагнозов не объясняет полностью клиническую картину. IBD, конечно, может вызывать диаррею с кровью, но нужно было также найти объяснение для тромбоцитопении, выраженного повышения печеночных ферментов и абнормального протромбинового времени. Активная IBD привела к вторичной гиперкоагулопатии, которая, в свою очередь, привела к тромбозу, который и объясняет все находки в этом случае.
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#53
14.11.2005, 18:12
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Цитата:
Я не знаю, будет ли работать ссылка, я заходила на работе через "тохну Макаби"
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#56
14.11.2005, 21:50
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Цитата:
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#57
14.11.2005, 22:00
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Я бы тоже начала с ж...живота. Мы сегодня с коллегами обсуждали этот случай на работе, и все решили, что начинать надо было с УЗИ живота. Хотя, м.б , они все делали одновременно, просто изложение такое...
Алон, то, что это разные журналы, я знаю, но сообразила позже, когда попыталась посмотреть одну статью в Pediatrics и - упс!- не могу. Просят денег
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#58
17.11.2005, 16:01
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Full text(1)
CLINICAL CHALLENGES: 18 YEAR OLD MALE WITH BLOODY DIARRHEA
[GRAND ROUNDS] KIM, HELEN J. MD; NEWMAN, BEVERLEY MD; KELJO, DAVID J. MD, PHD Department of Pediatrics, Division of Gastroenterology, Department of Radiology, University of Pittsburgh School of Medicine and Children's Hospital of Pittsburgh. Reprint requests: David J. Keljo, MD, PhD, Gastroenterology Department, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh PA 15213. E-mail: [Ссылки доступны только зарегистрированным пользователям ]. Submitted for publication Aug 19, 2004; last revision received Mar 11, 2005; accepted Apr 5, 2005. CT: Computed tomography; IBD: Inflammatory bowel disease CASE PRESENTATION An 18-year-old boy presented to the emergency department with a 2-week history of diarrhea and fatigue. His diarrhea was watery and nonbloody, occurring several times a day. A diagnosis of viral gastroenteritis had been made by his primary care physician. Four days before admission, his diarrhea became more frequent, accompanied by nonbloody, nonbilious emesis, and worsening fatigue. His parents noted that he looked pale and had low-grade temperatures. He denied night-time stooling but admitted to having 1 episode of fecal incontinence. He denied abdominal pain, night sweats, weight loss, jaundice, rash, easy bruising, or bleeding. There was no recent travel or unusual exposures. Medical history was notable for psoriasis. Family history was negative for inflammatory bowel disease or celiac disease but was positive for a maternal grandfather with a “ruptured colon.” There was no family history of bleeding or clotting disorders. Physical examination revealed a pale, well-grown boy in no distress. He was fever free, with tachycardia to 119 beats/min and hypertension with a blood pressure of 148/72 mm Hg, and he had a normal respiratory rate. His weight plotted to the 90th percentile and height plotted to the 75th percentile for age. Head, ears, eyes, nose, and throat examination showed no scleral icterus but revealed an ulcer on the left tonsil. Cardiac examination revealed a I/VI systolic murmur along the left sternal border. His abdomen was not distended and not tender with no hepatomegaly; however, a spleen tip was palpable. A rectal examination revealed gross blood with no fissures or tags. On skin examination, there were no bruises, petechiae, or rashes. The patient was admitted to the hospital for further evaluation. While hospitalized his diarrhea became grossly bloody with mucus. Initial laboratory results included a white blood cell count of 22,300 cells/mm3 with a differential of 44% neutrophils, 36% bands, 9% lymphocytes, 7% monocytes, 1% eosinophils, 1% metamyelocytes, and 2 % myelocytes. Hemoglobin was 8.3 g/dL, hematocrit 25.8 %, with a low mean corpuscular volume of 63 and elevated red cell volume distribution width of 19.4%. Platelet count was 38,000/mm3. The reticulocyte count was 4.5 %. The peripheral smear revealed red blood cell fragments, ovalocytes, teardrop cells, and hypochromia. Blood urea nitrogen level was 13 mg/dL, and creatinine was 1.5 mg/dL. Liver function tests revealed elevated alanine aminotransferase of 1030 IU/L, aspartate aminotransferase of 746 IU/L, alkaline phosphatase of 133 IU/L, gamma-glutamyltransferase of 73 IU/L, total bilirubin of 0.4 mg/dL, and albumin of 2.9 g/dL. The prothrombin time was elevated at 19.8 seconds, with an activated partial thromboplastin time of 29.4 seconds, and an elevated international normalized ratio of 1.8. The lactate dehydrogenase was 1118 IU/L, and uric acid was 8.8 mg/dL. Erythrocyte sedimentation rate was 24 mm/h. Urinalysis revealed 2 red blood cells, trace ketones, small amount of protein, and 26 hyaline casts. Evaluation for infectious enteritis with stool cultures, ova, and parasites, and Clostridium difficile toxin proved negative. Blood cultures were negative. Tests for infectious hepatitis including a hepatitis panel and cytomegalovirus antigen were negative. Epstein-Barr virus titers were consistent with a remote prior infection. Serial complete blood count and liver function tests revealed that the low platelet count and elevated transaminases returned toward normal levels. Renal function remained normal throughout the admission. An opthalmologic examination was negative for Kayser-Fleischer rings, and serum ceruloplasmin and copper levels, as well as 24-hour urinary copper levels, were within normal limits. Abdominal ultrasonography with Doppler scanning revealed no flow in the left portal vein, but patent and normal flow direction in the right portal, main portal, and splenic veins. There was a mildly enlarged spleen, nonspecific gallbladder wall thickening, and a moderate amount of free fluid within the abdomen. The kidneys and liver were otherwise normal. Because there was evidence of possible thrombosis in the portal vein by ultrasound examination, initial therapy with vitamin K was discontinued, and fresh frozen plasma was not administered. A computed tomography (CT) angiogram was obtained to further evaluate for possible thrombosis in the portal system and to inspect the liver and colon. This imaging revealed extensive subocclusive thrombosis of the portal and mesenteric venous systems, patchy enhancement of the liver, and colonic changes suggestive of colitis (Figure). There was no suggestion of appendicitis, ruptured appendix or other source of abdominal sepsis. -------------------------------------------------------------------------------- Figure. Images from the portal venous phase of a contrast enhanced CT scan. A, Almost complete thrombosis of the intrahepatic portal venous branches (arrows). Liver has patchy heterogeneous appearance with peripheral geographic areas of low attenuation. B, Subocclusive thrombus of portal confluence and medial splenic vein (black arrows). Splenic flexure of colon has thickened, edematous walls (white arrows) with prominent mucosal enhancement. C, Enlarged superior mesenteric vein with subocclusive thrombus (black arrow). Walls of descending and transverse colon (white arrows) are thickened and edematous with prominent mucosal enhancement. -------------------------------------------------------------------------------- The patient was evaluated for known primary causes of hypercoagulable states. The evaluation was negative for factor V Leiden and prothrombin G20210A mutations. There was normal activated protein C resistance. Low antithrombin III, protein C activity, and protein S activity were believed to be caused by liver disease or the recent thrombosis itself. There was no evidence of an antiphospholipid antibody syndrome, with normal tissue thromboplastin inhibition indexes, negative hexagonal lipid neutralization test, normal anticardiolipin and antithyroid antibody levels, and normal dilute Russell viper venom time. The patient was heterozygous for the methylenetetrahydrofolate reductase thermolabile variant (C677T) but had normal homocysteine levels. Flow cytometry was negative for paroxysmal nocturnal hemoglobinuria as a cause of thrombosis. A colonoscopy was performed. At colonoscopy the colon was found to be diffusely inflamed and edematous with mucosal ulceration and friability, indicating a pancolitis. The terminal ileum was not inspected. Biopsy specimens revealed acute colitis with inflammatory pseudomembranes, organizing submucosal thrombi, and crypt abscess formation. No viral inclusions, organisms, or granulomas were appreciated. There were no chronic changes at this point. Concern was raised about a thrombotic/ischemic colitis. The patient was transferred to the intensive care unit for optimal management of anticoagulation in the face of active gastrointestinal bleeding. Heparin was used for anticoagulation. A sigmoidoscopy with biopsy was performed on a later date to further delineate the underlying diagnosis. These biopsy specimens of the left colon showed architectural distortion and lamina propria lymphoplasmacytic inflammation with gland lift-off, acute cryptitis, and crypt abscesses. These features of chronic mucosal injury with active inflammation were more suggestive of chronic inflammatory bowel disease. The colitis was treated with prednisone and sulfasalazine. The patient was eventually discharged on prednisone, mesalamine (Asacol), and a 6-month course of warfarin. Repeat abdominal ultrasound examination approximately 3 months after the initial ultrasound examination revealed patent portal vessels with no evidence of thrombus. On follow-up visits, the patient's ulcerative colitis was noted to be under good control, with resolution of abdominal pain and bloody diarrhea. The final diagnosis was ulcerative colitis with secondary hypercoagulable state and portal and mesenteric vein thrombosis.
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#59
17.11.2005, 16:02
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2
DISCUSSION
The patient presented with the unusual combination of diarrhea that became bloody, thrombocytopenia, markedly elevated liver enzymes, and prolonged prothrombin time. A number of diagnoses were considered and excluded by the treating team after testing. These included hemolytic uremic syndrome, acute infectious hepatitis, malignancy, and chronic liver disease. None of these diagnoses fully explained the constellation of findings. Inflammatory bowel disease certainly could have caused diarrhea that became bloody, but an additional explanation was needed for the thrombocytopenia, marked elevation of liver enzymes, and abnormal prothrombin time. Active inflammatory bowel disease resulting in a secondary hypercoagulable state leading to portal and mesenteric vein thrombosis explains all of the key findings of the case. It is difficult to make a clinical diagnosis of acute portal vein thrombosis. The common presenting symptoms are extremely vague—abdominal pain, anorexia, and vomiting.1 Extension of the thrombus into the mesenteric venous system may progress to intestinal ischemia and infarct,2,3 with marked abdominal pain and development of peritoneal signs. The marked elevation of liver enzymes seen in this patient is not a reported feature of acute portal vein thrombosis,4 except in the immediately post-liver-transplantation setting.5,6 In the case at hand with extensive clot formation, the degree of elevation may represent ischemic insult from diffuse clotting in small intrahepatic vessels as well. (The heterogeneous enhancement of the liver seen at CT scanning would be compatible with this.) Portal hypertension may develop if the thrombus persists, leading to related signs of splenomegaly, esophageal varices, and gastrointestinal bleeding.3 If the tests are done, the diagnosis is easily made by ultrasonography with Doppler scanning and computed tomography.3 Both anticoagulant therapy with heparin and thrombolytic therapy with urokinase have been successfully used to prevent progression to portal hypertension and intestinal infarct.2,3,7 Peritonitis and signs of intestinal infarction require excision of necrotic bowel.2 Deep vein thromboses are uncommon in childhood and always raise the question of a hypercoagulable state. Hypercoagulable states may occur as a result of inherited coagulation disorders or acquired coagulation risk factors.8,9 The most common inherited procoagulant disorder would be factor V resistance to the anticoagulant activity of activated protein C, caused by the Leiden mutation in factor V. Somewhat less common is the procoagulant G20210A mutation in the prothrombin gene. Deficiency of or resistance to the anticoagulant proteins antithrombin III, protein C, and protein S are recognized causes of hypercoagulability. Hyperhomocystinemia can predispose to thrombosis. An inherited cause of hyperhomocystinemia is deficiency of methylenetetrahydrofolate reductase. Paroxysmal nocturnal hemoglobinuria is an acquired genetic mutation in the multipotential hematopoietic stem cell, which predisposes to thrombosis.9,10 Antiphospholipid antibodies are well-recognized risk factors for thromboses. Secondary causes of hypercoagulation must also be kept in mind when evaluating any patient with thrombosis. These acquired risk factors include infection, dehydration, immobility, prior surgery, placement of a central venous catheter, and medications such as corticosteroids and oral contraceptives.8 Disease states that involve protein loss such as nephrotic syndrome 8,9,11 (or colitis—see below) are associated with an increased risk of thrombosis. Inflammatory diseases such as malignancies, systemic lupus erythematosis, and inflammatory bowel disease are also associated with a prothrombotic state.9 Deep vein thrombosis is a recognized complication of inflammatory bowel disease (IBD). The reported incidence of thrombotic complications in IBD to ranges from 1% to 6.7%.12 The prevalence in one postmortem study was 39%.12 Thrombosis with IBD tends to occur in younger patients 13,14 and is associated with a high mortality rate (8%).14 Nine percent of thromboembolic events in IBD are found in the portal venous system, and this is associated with a mortality rate as high as 50%.15 Cerebral vascular events, including cerebral venous thrombosis, carotid artery thromboembolism, and retinal branch artery occlusion, account for 10% of thrombotic events in IBD and are associated with high mortality and neurologic morbidity rates.15 Thrombi affecting the pulmonary, gonadal, cardiac, and peripheral vessels have also been described.15 Because of the increased risk of thrombosis, thromboprophylaxis with low-molecular-weight heparin of all hospitalized patients with IBD has been suggested, although it has not yet been studied.16 The pathogenesis of the thrombotic complications of IBD appears to be multifactorial. Some studies cite a high incidence of inherited procoagulant disorders in patients with IBD 17; however, no inherited disorder has been found more frequently in patients with IBD than in the general population.12,16 Inflammation results in elevated platelet counts and elevated levels of the procoagulant factors V, VIII, fibrinogen, and von Willebrand factor.16 It decreases levels of the clot-stabilizing factor XIII, which may counter the procoagulant effects of inflammation.16 Fecal losses and consumption in the microvasculature of anticoagulant factors protein C, protein S, and antithrombin III could increase clotting tendency.16 Folic acid and vitamin B12 deficiency can be seen in patients with IBD and can contribute to elevated homocysteine levels, which promote thrombosis through an unknown mechanism.16 Antiphospholipid antibodies are somewhat more common in patients with IBD.16 Other prothrombotic factors common in patients with IBD include dehydration, immobility, central venous catheters, steroid use, and prior surgery. Recent evidence suggests that although greater than 50% of children with portal vein thrombosis are deficient in 1 or more anticoagulant proteins, they do not appear to have a higher prevalence of hereditary prothrombotic disorders.18 Inflammation, dehydration, and decreased levels of protein S, protein C, and antithrombin III likely contributed to the thrombosis in this case. Although it seems hazardous to treat a patient bleeding from colitis with heparin, heparin has actually been used in therapeutic trials to treat ulcerative colitis. These trials were based on the theory that thromboses in the microvasculature and resultant ischemia contributed to the disease process.19 One prospective study with unfractionated heparin resulted in improvement of ulcerative colitis clinical activity, inflammatory laboratory values, and histologic changes.20 In one small randomized trial the efficacy of heparin in treating active colitis was similar to that of steroids, and the heparin did not exacerbate the bleeding.21 The apparently beneficial effect of heparin in ulcerative colitis has been hypothesized to be related to anticoagulant effect, enhanced mucosal repair by promotion of growth factor activity, and antiinflammatory effects.22
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#60
06.12.2006, 01:17
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Weezing
A 7-week-old girl of Middle Eastern descent is admitted to the hospital for a cough of 10 days’ duration that is worsening and has produced posttussive emesis for the last 7 days. An episode of perioral cyanosis lasting less than 1 minute was noted 5 minutes after a feeding. There has been no fever, rhinorrhea, emesis, or diarrhea. The baby was born at term without perinatal complications.
Three days into her illness, the child’s pediatrician had placed her on nebulized bronchodilator therapy for presumed viral bronchiolitis. Re-evaluation on the day of admission shows no improvement. A chest radiograph reveals marked cardiomegaly with clear lung fields. The physical examination reveals a well-nourished baby who looks alert but manifests slightly decreased activity. Her heart rate is 130 beats/min, respiratory rate is 35 breaths/min, blood pressure is 85/45 mm Hg, and pulse oximetry saturation is 100% in room air. The child’s length is at the 95th percentile, and her weight and head circumference are at the 75th percentile. Auscultation reveals scattered end-expiratory wheezes, more prominent at the bases, but no grunting, retractions, or nasal flaring. A soft grade I/VI systolic flow murmur that does not radiate is audible at the left sternal border. Both heart sounds are normal. The baby appears well perfused and has normal femoral pulses. The rest of her physical findings are normal. CBC, electrolyte levels, and liver function tests all yield normal results. Что будем делать дальше?
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Линейный вид
