Polymyxin B-immobilized fiber hemoperfusion with low priming volume in an elderly septic shock patient with marked endotoxemia

An 84-year-old woman with septic shock caused by pyelonephritis is described herein. She was admitted for severe back pain and high fever. Her white blood cell (WBC) count and C-reactive protein (CRP) and endotoxin levels were elevated at 38,000/microl, 40.0 mg/dl, and 8,400 pg/ml, respectively. Her blood pressure was 80/34 mm Hg. Urinalysis revealed occult blood with innumerable WBCs. Plain abdominal radiography showed calcium stones in both kidneys. Septic shock with endotoxemia was diagnosed, and the patient was treated with antibiotics, gamma-globulin, and dopamine. However, her plasma endotoxin level remained high for 3 days. We performed direct hemoperfusion twice using a polymyxin B-immobilized fiber (PMX-F) column with a low priming volume. After PMX-F treatment, the patient's temperature decreased to 36.8 degrees C; her WBC count and CRP level decreased to 9,200/microl and 3.8 mg/dl, respectively. Her plasma endotoxin level decreased to 840 pg/ml after the first treatment and to 188 pg/ml after the second treatment. The next day, her blood endotoxin level further decreased to 32 pg/ml. Her blood pressure increased to 92/60 mm Hg after the first treatment and to 118/76 mm Hg after the second treatment. The patient was discharged on day 26 after admission. Our experience in this case suggests that PMX-F treatment with a low priming volume may be beneficial in elderly patients with septic shock and marked endotoxemia.     

The various faces of autoimmune endocrinopathies: Non-tumoral hypergastrinemia in a patient with lymphocytic colitis and chronic autoimmune gastritis

Serum gastrin levels exceeding 1000 pg/ml (normal, < 100) usually raise the suspicion for a neuroendocrine tumor (NET) that secretes gastrin. Rarely, such elevated gastrin levels are seen in patients with pernicious anemia which most commonly is associated with autoimmune gastritis (AG). AG can occur concomitantly with other autoimmune disorders including lymphocytic colitis (LC). Gastrin stimulates enterochromaffin-like cells which increase histamine secretion. Histamine excess can cause diarrhea as can bacterial overgrowth or LC. We present a 57-year-old woman with diarrhea, sporadic epigastric pain, and bloating. She also had a history of interstitial cystitis and took pentosan polysulfate and cetirizine. She had no history of ulcers, renal impairment or carcinoid syndrome. Fasting serum gastrin was 1846 pg/ml. Esophagoduodenal gastroscopy and biopsies revealed chronic gastritis and a pH of 7 with low stomach acid. Serum gastrin and plasma chromogranin A were suggestive of a gastrinoma or NET. Pernicious anemia was unlikely. Imaging studies did not reveal any tumor. Random colonic biopsy was compatible with LC, possibly explaining her diarrhea, although we also considered excessive histamine from elevated gastrin, bacterial overgrowth, and pentosan polysulfate which can cause diarrhea and be misleading in this setting, pointing to the diagnosis of gastrinoma. At 4 year follow-up in 2012, fasting serum gastrin was 1097 pg/ml and the patient asymptomatic taking only cetirizine for nasal allergies. This case illustrates that diarrhea may be associated with very high serum gastrin levels in the setting of chronic gastritis, LC, and interstitial cystitis (pentosan use), without clear evidence for a gastrinoma or NET. If no history of ulcers or liver metastases is present in such cases, watchful observation rather than an extensive/invasive and costly search for a NET may be justified. Considering the various forms of polyglandular syndrome, this may represent a variant and we here provide an algorithm for working up such patients, while also reviewing literature on the intertwined relationship between the immune and endocrine systems.     

Effects of polymyxin B immobilized fiber on urinary N-acetyl-beta-glucosaminidase in patients with severe sepsis

Severe sepsis is known to cause multiple organ failure, including renal dysfunction. During sepsis, endotoxin targets the renal proximal tubular cells, the function of which can be evaluated on the basis of urinary N-acetyl-beta-glucosaminidase (NAG). We investigated whether urinary NAG activity is altered in patients with severe sepsis and whether treatment with polymyxin B immobilized fibers (PMX-F) affects this activity. Subjects of this study were 120 patients with severe sepsis and 60 healthy volunteers matched for age and gender. Patients were randomly assigned to one of two treatments: PMX-F treatment (n = 70) or conventional treatment (n = 50). The plasma endotoxin level was significantly reduced, from 34.6 +/- 10.2 to 6.8 +/- 2.4 pg/ml (p < 0.01) in patients treated with PMX-F, and the urinary NAG/creatinine ratio was reduced from 46.5 +/- 26.8 U/gm to 18.6 +/- 13.6 U/gm (p < 0.01). The plasma endotoxin level and urinary NAG/creatinine ratio were unchanged in patients who received conventional treatment. The increased urinary NAG/creatinine ratio in patients with severe sepsis may reflect proximal tubular dysfunction. PMX-F is effective in reducing proximal tubular dysfunction, in part owing to reduced plasma endotoxin levels.     

Hemoperfusion with polymyxin B immobilized fibers for urinary albumin excretion in septic patients with trauma

We investigated whether microalbuminuria/urinary creatinine ratio (MACR) is increased in septic patients with trauma and whether polymyxin B immobilized fiber (PMX-F) treatment decreases MACR. Twelve trauma patients without sepsis, 18 trauma patients with sepsis, and 10 healthy controls were included in this study. The 18 trauma patients with sepsis were randomly assigned to one of two groups, PMX-F treatment or conventional treatment. Urinary microalbumin and creatinine were measured before and after treatment. Plasma endotoxin levels were determined by endospecy test. Hemoperfusion with PMX-F was carried out twice, for 2 hours, at a flow rate of 100 ml/min. MACR increased in the 30 trauma patients (5.2+/-2.2 mg/mmol) in comparison to that in the healthy controls (1.0+/-0.6 mg/mmol, p < 0.01). In the 18 trauma patients with sepsis, MACR after sepsis (16.6+/-4.8 mg/mmol) was significantly greater than that before sepsis (5.5+/-2.3 mg/mmol, p < 0.01). There was a significant correlation between plasma endotoxin levels and MACR in septic trauma patients (p < 0.001). MACR was reduced from 17.0+/-5.0 mg/mmol to 4.2+/-1.5 mg/mmol (p < 0.01) with PMX-F, and plasma endotoxin levels were also reduced from 34.5+/-18.5 pg/ml to 10.8+/-6.6 pg/ml (p < 0.01). Neither MACR nor plasma endotoxin levels were affected by conventional treatment, however. In summary, trauma patients with sepsis appear to show increased MACR, and PMX-F therapy may be effective for attenuating the increase in MACR.     

Successful bosentan therapy in a case of pulmonary arterial hypertention developed during immunosuppressive therapy for lupus nephritis

We report a 43-year-old female who developed pulmonary arterial hypertension (PAH) during intensive immunosuppressive therapy for systematic lupus erythematosus (SLE). She was diagnosed as SLE at the age of 32 years based on serological and hematological abnormalities, oral ulcers, and facial erythema. She experienced frequent flare-ups of disseminated discoid lupus between the ages of 33 and 36 years and developed immune thrombocytopenia at the age of 39 years. In 2007 when she was 43 years old, she developed lupus nephritis (LN) with elevated serum anti-double stranded DNA antibodies and urine protein of less than 1 g/day. Combination therapy for the LN with 35 mg/day prednisolone and intravenous cyclophosphamide (IVCY) led to renal remission. After the seventh monthly session of IVCY, she developed dyspnea on exertion. PAH was diagnosed based on enlarged main pulmonary arteries on the chest x-ray, right ventricular outflow and a peak tricuspid regurgitant pressure gradient exceeding 45 mmHg on echocardiography, an elevated plasma brain natriuretic peptide (BNP) level of 260 pg/ml, the exclusion of pulmonary thromboembolism, and no lung fibrosis. The PAH was treated successfully with bosentan. At present the tricuspid regurgitation has disappeared, and the plasma BNP level has normalized.     

An autopsy case of HELLP syndrome with disseminated aspergillosis]

Disseminated aspergillosis is an uncommon and unpredictable complication in severely immunocompromised patients and poses a challenging problem in the management and care of seriously ill patients receiving intensive care therapy. We report an autopsied case of disseminated aspergillosis occurring ina 31-year-old female patient who was treated for HELLP (hemolysis, elevated liver enzymes, and low platelet count) syndrome. She initially presented with edema and proteinuria at a pregnancy check-up. At gestational age 33 weeks and 2 days, she had right lower abdominal pain, nausea, and jaundice. The next day she delivered a male neonate transvaginally, followed by excessive postpartum uterine bleeding. Although an emergency hysterectomy was performed, her hemorrhagic diathesis could not be controlled even after transcatheter embolization of the internal iliac arteries with subsequent ligation and repeated blood transfusions totalling to 31,070 ml. She eventually died of a cerebral hemorrhage 21 days after the parturition. Autopsy showed generalized jaundice and petechiae, as well as extensive hemorrhage observed in the abdominal wall, peritoneal cavity, and retroperitoneal and pelvic spaces. In addition,there were multifocal hemorrhages found in the left temporal, right frontal and posterior lobes of the cerebrum, and pons. Disseminated aspergillosis was found in the lungs, trachea, brain, esophagus, stomach, heart, and thyroid gland. These findings suggest that systemic postoperative complications, associated with massive blood transfusions and hepatic failure, mutually contribute to the overall deterioration of host defense mechanism, and may underlie the occurrence of devastating systemic fungal infection.     

Sjogren's syndrome presenting as remitting seronegative symmetric synovitis with pitting edema (RS3PE)

Remitting seronegative symmetric synovitis with pitting edema (RS3PE) syndrome is characterized by symmetrical and acute synovitis, pitting edema, the absence of rheumatoid factor, increased acute phase reactants, lack of bony erosions on radiography, and benign and short clinical course. Half of all patients with Sjogren's syndrome experience arthritis during the disease course. We here describe the first case of Sjogren's syndrome presenting as RS3PE. She had swelling in knees, ankles, and wrists. After then the swelling spread to her lower legs, feet, face, and both hands. She was admitted to another hospital and was suspected of lupus or rheumatoid arthritis. Three months later, she had dry mouth and had lower lip biopsy. She was admitted to this hospital due to development of swelling in face and lower legs for 3 days. On physical examination, she had pitting edema in both hands and feet dorsum. Laboratory test showed elevated erythrocyte sedimentation rate, positivity of rheumatoid factor, anti-nuclear antibody, and anti-Ro antibody. There was no erosion in the hands radiography. Schirmer's test and lip biopsy was compatible with Sjogren's syndrome. She was diagnosed RS3PE and Sjogren's syndrome. She was begun with prednisolone and her symptoms improved gradually.     

A case of the latex-induced anaphylaxis by contact with barium enema catheter]

The subject was a 34-year-old female with asthma and atopic dermatitis who had previously undergone a Cesarean section. In December 1995, the patient had an acute abdominal pain, so she underwent a barium enema examination. During the procedure, severe anaphylactic shock developed, and the examination was stopped. The patient responded well to appropriate emergency therapy, and her symptoms were resolved. However, the woman visited our outpatient clinic because the symptoms of her atopic dermatitis got worse. She indicated that she felt itchy when using rubber gloves. Also, at the age of thirty, she had urticaria and dyspnea after drinking a glass of fruit juice. Subsequently radioallergosorbent testing demonstrated the presence of specific IgEs against latex, banana, kiwi, grapefruit, and avocado. Skin prick tests were positive for banana, grapefruit, avocado, and latex extract at a dilution of 1:1000. A scratch test was positive to an extract of a balloon catheter which included 0.3 microg/g latex proteins by the LEAP method. In conclusion, the patient was diagnosed with an anaphylaxis to the latex contained in a balloon catheter used for the barium enema.     

Inflammatory bowel disease in children--clinical, endoscopic, radiologic and histopathologic investigation.

This paper reviews our five years' clinical experience (1987 to 1991) of 22 patients with inflammatory bowel disease (IBD). There were 12 patients with Crohn's disease and 10 patients with ulcerative colitis. The mean age at diagnosis was 8.7 years (2 to 14 years). Clinical impressions before referral were chronic diarrhea in 11, irritable bowel syndrome in 5, colon polyp in 4, lymphoma in 3, intestinal tuberculosis in 2, amoebic colitis in 2, ulcerative colitis in 2 children and other diseases. The mean interval from the onset of symptoms to the diagnosis of IBD was 18 months. Diagnosis of Crohn's disease was delayed for more than 13 months in 8 (67%), whereas that of ulcerative colitis was delayed for more than 13 months in 4 (40%). Diarrhea (50%), abdominal pain (36%) and rectal bleeding (36%) were the three most frequent presenting complaints of IBD. Moderately severe abdominal pain was a more common chief complaint in Crohn's disease (58%) than in ulcerative colitis (10%). Hematochezia (90% vs 17%) and moderately severe diarrhea (90% vs 75%) were more common gastrointestinal manifestations in ulcerative colitis than in Crohn's disease. The associated extraintestinal manifestations were oral ulcer in 7, arthralgia in 11 and arthritis in 4, skin lesions in 2, eye lesions in 2 and growth failure in 9 patients. Of 12 children with Crohn's disease, granuloma was found in 5, aphthous ulcerations in 8, cobble stone appearance in 8, skip area or asymmetric lesions in 6, transmural involvement in 7, and perianal fistula in 3. Among 10 children with ulcerative Colitis, there were crypt abscess in 8, granularity or friability in 10 and rectosigmoid ulcerations with purulent exudate in 8 children. The main sites of involvement in children with Crohn's disease were both the small and large bowels in 7 (58%), small bowel only in 2 (16%), and colon only in 3 (25%). Terminal ileum involvement was seen in 75% of Crohn's disease cases. The main sites of involvement in children with ulcerative colitis were total colon in 4 (40%), up to the splenic flexure in 2 (20%), rectosigmoid in 3 (30%) and rectum only in one (10%). Medical treatment including sulfasalazine, and systemic or topical steroid was administered initially in most patients. Seven of 12 patients with Crohn's disease and 2 of 10 patients with ulcerative colitis were operated on.(ABSTRACT TRUNCATED AT 400 WORDS)     

Treatment with polymyxin B-immobilized fiber reduces platelet activation in septic shock patients: Decrease in plasma levels of soluble P-selectin, platelet factor 4 and β-thromboglobulin

OBJECTIVE: To clarify whether plasma concentrations of soluble P-selectin, platelet factor-4 (PF-4) and beta-thromboglobulin (betaTG) are altered in patients with septic shock and whether polymyxin B-immobilized fiber (PMX-F) treatment affects these changes. SUBJECTS: Thirty patients with septic shock who were treated with PMX-F (group A), 20 such patients who received conventional therapies (group B) and 20 healthy control subjects(group C). METHODS: ELISA using commercial kits. Endotoxin elimination by direct hemoperfusion using PMX-F. RESULTS: Blood endotoxin levels decreased significantly from 49.4+/-8.8 pg/ml to 13.0+/-4.5 pg/ml after PMX-F treatment. The pretreatment plasma concentrations of soluble P-selectin, PF-4 and betaTG in patients in groups A and B were significantly higher than those in group C (p<0.001). Plasma concentrations of these factors decreased significantly in group A after PMX-F treatment (p<0.01); however, the concentrations in group B were not altered after conventional treatment. The survival rate of group A (60%) was higher than that of group B (30%). CONCLUSIONS: Our findings suggest that soluble P-selectin, PF-4 and betaTG may be associated with septic shock and that PMX-F is effective in reducing these markers in patients with septic shock.     

Oral cromolyn sodium in milk induced anaphylaxis

The medical literature contains numerous references to the use of oral cromolyn sodium in gastrointestinal disturbances (abdominal pain, diarrhea, ulcerative colitis) and dermatologic illnesses (urticaria, infantile eczema, purpura). But there are few, if any, reports of anaphylactic reactions to foods which have been successfully controlled using oral cromolyn sodium. This paper reports the results of treatment with oral cromolyn in a 7-year-old child who had acute, severe anaphylaxis after ingestion of milk or foods containing milk. After 3 months of oral cromolyn therapy, the patient was able to tolerate small amounts of milk and moderate amounts of foods containing milk.     

Polymyxin B-immobilized fiber hemoperfusion in a high school football player with septic shock caused by osteitis pubis

A 17-year-old male high school football player treated by polymyxin B-immobilized fiber (PMX-F) hemoperfusion for mild-moderate septic shock caused by osteitis pubis is described in this study. He was admitted for inguinal pain, gait disturbance, and high fever (40.6°C). His white blood cell (WBC) count and C-reactive protein (CRP), endotoxin, and procalcitonin (PCT) levels were significantly elevated. His blood pressure was 76/46 mm Hg. Magnetic resonance imaging showed bone and muscle injury at the pubic symphysis. Septic shock with high blood endotoxin and PCT concentrations was diagnosed, and the patient was treated with antibiotics, γ-globulin, and dopamine on the admission day. However, the septic shock did not improve. On day 3, we performed direct hemoperfusion twice using a PMX-F column. After the second PMX-F treatment, the patient's temperature decreased to 37.0°C, and his WBC count, CRP levels, blood endotoxin, and PCT levels decreased. The inguinal pain diminished, and the patient's blood pressure increased to 112/76 mm Hg. He was discharged on day 10 after admission. This case reflects association of PMX-F with decreased endotoxin, PCT, and CRP, suggesting the association of PMX-F with clinical improvement in mild-moderate sepsis in a young athlete.     

Effects of hemoperfusion on serum cardiac troponin T concentrations using polymyxin B-immobilized fibers in septic patients undergoing hemodialysis

We investigated whether serum cardiac troponin T levels are altered in septic patients undergoing hemodialysis and whether polyinyxin B-immobilized fiber (PMX-F) treatment affects these levels. Fourteen heinodialysis patients with sepsis, 14 hemodialysis patients without sepsis, and 12 age matched healthy controls were included in this study. Cardiac troponin T levels in hemodialysis patients with sepsis (0.56+/-0.28 microg/L) were higher than levels in hemodialysis patients without sepsis (0.16+/-0.06 microg/L, p < 0.01) and healthy control subjects (0.03+/-0.01 microg/L, p < 0.001). The 14 hemodialysis patients with sepsis were randomly assigned to one of two treatment approaches: PMX-F treatment (n = 7) or conventional treatment (n = 7). Plasma endotoxin levels were significantly reduced from 46.6+/-17.8 pg/mI to 8.2+/-2.4 pg/ml, p < 0.01, in patients treated with PMX-F, and serum cardiac troponin T levels were also reduced from 0.62+/-0.30 microg/L to 0.26 = 0.12 microg/L, p < 0.05. Cardiac troponin T levels were unchanged in patients under conventional treatment. These data suggest that cardiac troponin T is indeed elevated in septic patients undergoing hemodialysis and niay reflect subclinical myocardial cell damage. PMX-F is effective in reducing myocardial damage, in part, due to reducing plasma endotoxin levels.     

A case of intestinal Behcet's disease similar to Crohn's colitis

Behcet's disease is a multi-systemic vasculitis and characterized by systemic organ involvement. Although the gastrointestinal and systemic features of Behcet's disease and inflammatory bowel disease overlap to a considerable extent, they are generally viewed as two distinct diseases. A 39-yr-old female was diagnosed as having Behcet's disease. She was admitted to our hospital because of oral and genital ulcer, lower abdominal pain, and frequent diarrhea. Colonoscopy showed diffuse involvement of multiple longitudinal ulcers with inflammatory pseudopolyps with a cobblestone appearance and ano-rectal fistula was suspected. These findings are extremely rare in Behcet's disease. However, there were no granulomas, the hallmark of Crohn's colitis. Microscopically, perivasculitis and multiple lymph follicles compatible with Behcet's disease were seen. Although being rarely encountered, multiple longitudinal ulcers, cobblestone appearance, and ano-rectal fistula can develop in Behcet's disease, as in Crohn's colitis. Therefore, Behcet's disease and Crohn's disease may be closely related and part of a spectrum of disease.     

Gorlin syndrome with ulcerative colitis in a Japanese girl

We present the case of a 14-year-old Japanese girl who had both Gorlin syndrome and ulcerative colitis. She had complained of blood stools for 6 months and severe scoliosis from her infancy. Physical examination revealed multiple nevi, palmar and plantar pits, jaw cysts, and calcification of the falx cerebri, leading to the diagnosis of Gorlin syndrome. Total colonoscopy revealed an edematous and spotty bleeding mucosa extending from the anus to the transverse colon. Histological examination was also compatible with ulcerative colitis. Thus, we diagnosed her as having Gorlin syndrome with ulcerative colitis. Gene analysis revealed a mutation, 1247InsT, in the human patched gene (PTCH), resulting in the truncation of PTCH protein. Since Gorlin syndrome and ulcerative colitis are rare disorders in childhood, this association is interesting, suggesting a correlation between the hedgehog signaling and intestinal disorders.     

Hypersensitivity peumonitis in a patient with rheumatoid arthritis]

We report a case with rheumatoid arthritis and hypersensitivity pneumonitis. A 66-year-old female was admitted to our hospital because of fever, cough, and progressive dyspnea on October 10, 1997. She had a history of rheumatoid arthritis from 1987 and was treated with cyclophosphamide when she developed pulmonary symptoms in September 1997. On admission arthritis was subsided. Fine crackles on ausculation of lung, hypoxia, ground-glass appearance on chest X-ray were detected. The computed tomography of the chest disclosed diffuse interstitial shadow with patchy destruction of alveolar structures. Bronchoalveolar lavage demonstrated an increase in lymphocytes with predominance of suppressor-cytotoxic T cell subset (CD 8+). The histopathological examination of transbronchial lung biopsy showed interstitial inflammation with marked predominance of lymphocyte with intraalveolar exudate. Her condition got better and she discharged without definitive diagnosis and treatment for her respiratory symptoms. Eight hours after she went back home, she suddenly presented high fever and cough and gradually developed dyspnea. She was readmitted 5 days after the previous discharge. Although no specific precipitin antibody against various microorganisms was detected in her sera, the diagnosis of hypersensitivity pneumonitis was made. Thirty mg per day of prednisolone was resolved her symptoms promptly. There was no reported case with hypersensitivity pneumonitis and rheumatoid arthritis of other collagen diseases. The clinical course that arthritis and pulmonary symptoms appeared alternatively is of considerable interest to investigate for the pathogenesis of these two immune disorders.     

A case of common variable immunodeficiency with intractable diarrhea and the functional disorder of renal tubules]

We report a case of a 31-year-old woman with common variable immunodeficiency (CVID) complicated with intractable diarrhea and the functional disorder of renal tubules. The patient became hypogammaglobulinemic after she suffered from measles at 6 years of age. She also suffered from lupus-like syndrome at 7 years of age. The complete remission was obtained by glucocorticosteroid treatment. An intravenous immunoglobulin replacement therapy was introduced at 11 years of age, since then her general condition was stable for more than 20 years. When she was 29 years old, she suffered from generalized malaise, anorexia with body weight loss, and numbness of face. The intractable diarrhea as protein loosing syndrome, and the severe abnormality of electrolyte balance with metabolic acidosis as the functional disorder of renal tubules were found. Her condition was not improved by the electrolytes or alkali replacement therapy. She was admitted for further evaluation and treatment. The intractable diarrhea and the functional disorder of renal tubules were dramatically improved after absolute restriction of food intake under hyperalimentation. When she began to take food, the symptom and sign became worse again. The interstitial nephritis and nonspecific inflammation of intestine were found by the tissue biopsy. The most characteristic finding was the infiltration of lymphocytes (predominantly CD 8 + T lymphocytes) in both intestinal mucosa and renal interstitium. The introduction of glucocorticosteroids improved her general condition and biochemical findings. This CVID case is complicated with intractable diarrhea and the functional disorder of renal tubules which is associated with the infiltration of CD 8 + lymphocytes in intestine and kidney. We consider that such case is very rare and valuable to report.     

A case of systemic scleroderma complicating pulmonary hypertension]

The patient was a 7-year-old girl. At the age of 6, deposits of pigment had appeared on the skin of her face and limbs, the skin had become sclerosed, and she had developed dyspnea on exertion. Her previous physician had hospitalized her. She was diagnosed as systemic scleroderma that accompanied pulmonary hypertension by her symptoms and laboratory findings. She was referred to our hospital at 7 years of age, and she was hospitalized. At that time, the entire skin showed deposition of brown pigment, the skin of the limbs was sclerotic. And the face was mask-like, flexion of the joints of the fingers and knees was limited, and the fingertips were ulcerated. Raynaud's phenomenon was present. She was positive for antinuclear antibodies, and negative for other autoantibodies. Echocardiography revealed pulmonary hypertension. After admission, steroid pulse therapy and cyclophosphamide (CY) pulse therapy were initiated, and for aftercare, 15 mg/day of prednisolone (PSL) and mizolibin (MZB) were administered orally. After several months, the sclerosis of the skin improved and the restriction of limb flexion was almost eliminated. The pulmonary hypertension advanced temporarily (maximum: 70 mmHg), but after oral administration of a PGI2 preparation and low-flow supplemental oxygen therapy and the initiation of anticoagulant therapy, the systolic pressure of the pulmonary artery improved to 34 mmHg. The CY pulse therapy was terminated after two years, and internal use of PSL and MZB was continued. The patient's condition is now stable. This case was treated from an early stage with steroid pulse therapy and CY pulse therapy, accompanied with oral administration of a PGI2 preparation for the pulmonary hypertension. The dermal symptoms improved, and it was possible to maintain a state of remission.     

Infarction of the midgut associated with oral contraceptives. Report of two cases

2 cases of midgut infarction in patients taking oral contraceptives are reported. Case 1 was a 38-year-old married woman with 3 children. After 2 isolated bouts of severe abdominal pain and diarrhea, examination revealed only minimal epigastric and left loin tenderness. Blood counts were normal. Other tests were negative. She had been taking cyclical tablets of 2.5 mg norethynodrel and .1 mg mestranol (Con ovid-E) for 48 months and continued after 8 days in the hospital. 18 weeks later severe abdominal pain, vomiting, and diarrhea occurred with abdominal tenderness and rigidity. The white-cell count was 25,000 with 85-90% segmented forms. Other blood tests were normal. At operation the superior mesenteric artery was found to be occluded distal to the origin of the middle colic artery. The thrombus was removed and the circulation to the gut seemed adequate. Intravenous heparin was given. Reoperation at 12 and again at 36 hours revealed viable intestine. 8 days after hospital admission ileus symptoms occurred. Reoperation revealed gangrene of almost all of the small intestine and part of the large intestine. The patient died 3 days later. Autopsy showed thrombosis of the superior mesenteric artery which was apparently not associated with local atheroma. Minimal atheroma in the aorta and an infarct of the spleen were noted. Case 2 was a 45-year-old married woman with 2 children who complained of severe abdominal pain and vomiting of 8 hours duration. A similar attack 1 week earlier had subsided in 6 hours. She had been taking tablets of 5 mg ethinyl-esternol (lynestrenol) and .15 mg mestranol (Noracyclin) for 11 months. There was no fever. The white-cell count was 19,500 with 85% segmented forms. Other laboratory tests and X-ray were normal. A loud bruit was heard over the upper abdomen. Bowel sounds were hyperactive. A diagnosis of acute small-bowel obstruction was made. At operation a definite diagnosis could not be made. Symptoms became worse. Reoperation 10 days later revealed gangrenous small intestine and part of the large intestine. The gangrenous parts were removed. After a complicated convalescence the patient recovered, but has moderate steatorrhea. Histologic examination of the resected intestine showed no evidence of atheroma in the mesenteric vessels. Considering these 2 cases with premonitory warning symptoms and without evidence of an atheromatous cause but associated with oral contraceptive therapy the immediate discontinuance of such therapy in women who develop acute abdominal pain is irecommended.     

Severe Rejection of an HL-A Identical Sibling Renal Transplant

A twenty-year-old patient with end-stage pyelonephritis received an HL-A identical renal transplant from her brother. She remained well for 28 days and then suffered a severe rejection episode while on decreased immunosuppressive therapy because of low white blood counts, reducing renal function to a creatinine clearance of 32 ml/min. Two weeks later she suffered a second major rejection episode which was arrested at a clearance of 10 ml/min. For the last 10 months renal function has remained stable at this level.