Hepatic hydrothorax associated with vitamin a toxicity

We report the first case of an adult presenting with respiratory symptoms caused by hepatic hydrothorax secondary to vitamin A intoxication. The patient was a 52-year-old woman who presented to the hospital with progressive dyspnea. Evaluation demonstrated mild elevation of her liver function tests, ascites, and a right pleural effusion. The patient consumed a variety of vitamins, including vitamin A. Her estimated vitamin A intake was at least 162,300,000 international units (IU) during 18 years. She dramatically escalated her dose the year before admission for a total acute dose of 98,550,000 IU, with a daily intake of 270,000 IU. The recommended daily allowance is 4,000 IU. A transjugular liver biopsy revealed histopathologic changes consistent with vitamin A toxicity: hypertrophy and hyperplasia of hepatic stellate cells, focal pericellular fibrosis, mild perivenular fibrosis, and minimal, predominantly microvesicular steatosis. Despite the absence of cirrhosis, pressure readings demonstrated portal hypertension. During her hospitalization, the patient's symptoms and biochemical profile improved. As the large and generally unregulated United States dietary supplement industry continues to grow, it is increasingly likely that individuals will present with the signs and symptoms of vitamin excess rather than vitamin deficiency. Physicians need to remain alert to the varied presentations and toxic manifestations of excessive vitamin use.     

Ataxic gait following total gastrectomy for gastric cancer

A 58-year-old woman, who had undergone total gastrectomy for early gastric cancer 9 years previously, visited the outpatient clinic complaining of progressive difficulty in walking for 15 d. Laboratory examinations showed macrocytic anemia and a decreased serum vitamin B12 concentration and increased serum concentrations of folate, vitamin E and copper. Magnetic resonance imaging showed multifocal high signal intensities along the posterior column of the cervical and thoracic spinal cord. Treatment consisted of intramuscular injections of vitamin B12 for 7 d, which increased her serum level of vitamin B12 to normal. This was followed by weekly intramuscular injections of vitamin B12 for another 2 wk and oral administration of vitamin B12 three times per day. After comprehensive rehabilitation for 4 wk, she showed sufficient improvements in strength and ataxic gait, enabling her to return to her normal daily activities.     

A female with asymptomatic primary biliary cirrhosis associated with pernicious anemia

We experienced a female case with asymptomatic primary biliary cirrhosis that was associated with pernicious anemia after 16 years from the onset. She was 52 years old when she first visited a clinic in 1981 for liver dysfunction treatment. Antimitochondrial antibody was negative and antipyruvate dehydrogenase complex antibody was positive in a low titer in its immunoglobulin (Ig)M type. Histological examination of her liver revealed a presence of definite chronic non-suppurative destructive cholangitis with numerous epithelioid cell granuloma. She had been given 600 mg of the oral daily dose of ursodeoxycholic acid since 1992. Macrocytic anemia incidiously appeared in September 1999. An immunological examination detected negative antiparietal cell antibodies and positive anti-intrinsic factor antibodies. Her bone marrow smear showed numerous megaloblasts and serum vitamin B12 in her blood was low at 99 pg/mL. Severe reversed atrophic-type gastritis (type A gastritis) was demonstrated by the use of dye-endoscopy with Congo red. Her macrocytic anemia dramatically improved after intramuscular administration of vitamin B12. In conclusion, attention should be given to the association of pernicious anemia during the follow up of primary biliary cirrhosis.     

Drug adverse events and drop-out risk: a clinical case

We report a brief discussion on a clinical case of a female patient, 85 years old, affected by severe cognitive impairment and chronic obstructive pulmonary disease (COPD). The patient was not taking drugs at home (apart from promazine: 10 drops when necessary to control her behavioral diseases). A previous neuropsychological evaluation had shown a severe cognitive impairment MMSE=16/30; ADL=3/6; IADL=0/8) due to multiple brain ischemic areas (confirmed in 2003 by MRI neuroimaging). When the patient was admitted to our center she was able to perform some basic activities of daily living such as eating and walking and was not too confused. She was included in cognitive rehabilitation groups. Since she showed signs of Parkinsonism, a therapy based on omeprazol 20mg, acetylsalicylic acid, donepezil 10mg, pramipexol 0.18 mg, nimodipine 10 drops, levodopa+carbidopa 100/25mg was started. A few days later she became sleepy during daytime and, once, she lost her balance and fell. She was not self-sufficient any more. At first this was attributed to a lung infection that the patient had, but her state continue after the infection was completely cured with appropriate antibiotics therapy. At that point an adverse drug reaction was suspected and therapy with pramipexol 0.18 mg was interrupted. In a few days the patient regained her previous level of consciousness and self-sufficiency. We consider this a typical case of complex management in a patient with dementia and comorbidity in which adverse drug reactions can play an important role in lowering the level of cognitive functions. In this case the relationship with the family of the patient was made difficult by the attitude of the patient's daughter who decided, after the onset of the adverse drug reaction, to interrupt her mother's stay in our center even at risk of the worst consequences.     

Multiple cognitive functional assessments used to modify the disturbed behavior of an elderly woman with dementia from the viewpoint of psychoeducation for the patient and her family

We report a case of a patient with dementia for whom a psychoeducational intervention based on a cognitive functional assessment by multiple cognitive functional tests was useful. She was an 82-year-old woman with hypertension and hyperlipidemia. She often asked about the date, scorched pans several times, and bought large amounts of the same items over 2 years. Her family worried that she might have dementia and hospitalized her. Mixed type dementia was diagnosed from the results of a cognitive functional assessment and magnetic resonance imaging findings. A psychoeducational program was conducted based on the findings of retained and impaired cognitive abilities derived from the above assessment. Her daily life disturbances were modified by an external compensation method. For example, her son told her repeatedly when it was not necessary to make supper, and made use of a block calendar. To consider how to cope appropriately with daily life disturbances derived from the symptoms of dementia, it is important to understand retained and impaired cognitive abilities, by comparing multiple cognitive functional assessments with the type of disturbances, and to deepen understanding of patients' psychological profiles, from the viewpoint of psychoeducation for the patient and family.     

Hepatopulmonary syndrome can show spontaneous resolution: Possible mechanism of portopulmonary hypertension overlap?

Hepatopulmonary syndrome (HPS) (hypoxaemia due to intrapulmonary vasodilation and a right-to-left shunt associated with liver disease) resolves after liver transplantation. The authors describe a case of spontaneous resolution of HPS prior to liver transplantation. This patient was diagnosed with HPS associated with extra-hepatic biliary atresia when she was 10 years old. She exhibited digital clubbing, facial vascular dilation, cyanosis, and suffered from dyspnoea during exercise. The patient's PaO(2) at rest was 53.8 mm Hg in room air and a Technetium-99m macro-aggregated albumin lung perfusion scan demonstrated a right-to-left shunt. Although her symptoms and laboratory data supported a diagnosis of HPS, she nevertheless showed spontaneous resolution within 2 years. When she was 14 years old, pulmonary hypertension was evident upon examination of her echocardiogram. HPS may be improved or masked by an accidental overlap with pulmonary hypertension in the terminal stage of liver disease.     

A case of two successful deliveries by a woman with Kallmann syndrome and NIDDM

A 37 year-old female with Kallmann syndrome and NIDDM who had two successful deliveries is reported. She had experienced no menstruation until she had treatment with gestagen in her early twenties. She had withdrawal bleeding only once. At the age of 25, she consulted her family doctor, complaining of amenorrhea. Estrogen progesterone cyclic therapy caused withdrawal bleeding, and clomiphene citrate failed to induce apparent ovulation. In January 1978, 150 IU of hMG was administered daily for 9 days, and then 3000 IU of hCG daily for the following 2 days. This therapy induced pregnancy, which failed spontaneously on June 4th. A year later, in January 1979, 150 IU of hMG was again administered daily for 7 days followed by 6000 IU of hCG for 3 days. This therapy again induced pregnancy. On September 27th, 1979, she delivered a girl vaginally, weighing 3830 g. After this delivery, she experienced no menstruation. In June 1985, she consulted her family doctor again, and she was diagnosed as being pregnant. Since her fasting blood glucose was 145 mg/dl, she was admitted to Kosei Hospital to control her blood glucose. On October 15th, she delivered a girl weighing 2600 g. On June 13th, 1989, she was referred to Kosei Hospital by her family doctor to achieve an accurate control of her blood glucose. During this admission, she was diagnosed as having Kallmann syndrome because of congenital anosmia and hypogonadotropic hypogonadism without any abnormal morphological changes. Vitamin B1 infusion test was negative.(ABSTRACT TRUNCATED AT 250 WORDS)     

Three pediatric cases of erythroleukemia: review of the literature on prognostic factors

We encountered 3 patients with erythroleukemia who showed differing outcomes. The first patient was an 11-year-old girl who was treated with an ANLL 91 national protocol followed by bone marrow transplantation from an HLA-identical brother. She is still in complete remission after 6 years. The second patient was a 15-year-old girl. Treatment with low dose Ara-C was effective. She experienced a relapse once, but achieved her second remission with low dose-Ara-C plus vitamin D. Up to the present, she has maintained remission for 5 years. The third patient was a 1-month-old girl who initially presented with an increase of proerythroblasts with infiltration to the liver. Although her response to Ara-C and etoposide was favorable, she died of a generalized fungal infection in the leukopenic phase. Chromosomal analyses of bone marrow cells were normal for patients 1 and 2, but patient 3 had an abnormal complex karyotype. We think the prognosis for erythroleukemia in childhood is not necessarily poor in all cases. Appropriate treatment should be based on the patient's age, the proportion of proerythroblasts, and the presence of chromosomal abnormalities.     

A case of cerebral venous angioma with paresis of the left arm and face

A 75-year-old woman was admitted to our hospital because of sudden onset of paresis in her left arm and face. She had untreated hypertension and hyperlipidemia. When she came back home after playing with children in the park, she felt weakness in her left hand. On admission, physical examination revealed that her blood pressure was very high (200/102 mmHg). Only slight weakness in her left arm and left facial palsy were recognized neurologically. An electroencephalogram showed normal findings. Brain CT and MRI revealed a venous angioma near the right central sulcus. Gadolinium-DTPA enhanced MRI showed a group of small radiating veins (so called "the caput medusae sign") connected to the venous angioma. The remaining symptoms decreased with the normalization of blood pressure. It is suggested the intracranial motor tracts of the face and arm in the precentral gyrus are adjacent to the location of this venous angioma. The dilation of venous angioma due to high blood pressure was thought to cause the paresis of face and arm in this patient.     

Coexistence of Megaloblastic Anemia and Iron Deficiency Anemia in a Young Woman with Chronic Lymphocytic Thyroiditis

Pernicious anemia is a megaloblastic anemia caused by vitamin B12 deficiency, and is the end-stage of autoimmune gastritis that typically affects persons older than 60 years. It is the most common cause of vitamin B12 deficiency. Pernicious anemia can also be diagnosed concurrently with other autoimmune diseases. We report the occurrence of megaloblastic anemia in a 22-year-old woman with chronic autoimmune thyroiditis for 10.5 years. Recently, she presented with microcytic anemia, and iron deficiency anemia was diagnosed initially. After administration of ferrous sulfate, macrocytic anemia was revealed and vitamin B12 deficiency was detected. Pernicious anemia was highly suspected because of the endoscopic finding of atrophic gastritis, and high titer of antigastric parietal cell antibody, as well as elevated serum gastrin level. After intramuscular injections of hydroxycobalamine 100 microg daily for 10 days, and monthly later, her blood counts returned to normal.     

Cardioembolic Stroke Due to Cardiac Sarcoidosis Diagnosed by Pathological Evaluation of the Retrieved Thrombus

A 70-year-old woman undergoing glucocorticoid therapy for cardiac sarcoidosis was brought to our hospital with the sudden onset of right hemiplegia and aphasia. Brain magnetic resonance imaging showed a high diffusion-weighted imaging signal in the left frontotemporal lobe and disruption of blood flow in the M1 segment of the left middle cerebral artery. Hence, she underwent thrombolysis and mechanical thrombectomy, resulting in marked improvement in her neurological symptoms. A pathologic evaluation of the thrombus suggested its cardiogenicity, and the absence of any obvious abnormality other than a left ventricular aneurysm indicated stroke due to a cardioembolic etiology secondary to cardiac sarcoidosis.     

Acarbose-induced acute hepatitis. Report of two events in the same patient

A 57-year-old woman with non-insulin-dependent diabetes mellitus and inadequate glycemic control was prescribed acarbose (100 mg 3 times daily). Two months later she presented with acute hepatitis (ALT 2,300 IU/l). Other causes of liver damage were excluded. Three months after acarbose had been discontinued, all results of laboratory tests returned to normal values. Three years later the patient was given acarbose again. Acarbose (100 mg three times daily) had been added to glibenclamide (15 mg daily) 2 weeks before she presented with acute hepatitis (ALT 2,778 IU/l). Acarbose was stopped and the results of liver tests returned to normal within 2 months. Of the eight cases of acarbose-associated hepatotoxicity previously reported, five (as well as the two presented herein) were Spanish. The latency period, from the start of drug therapy to the onset of liver injury, was relatively long (> 2 months). We suggest that acarbose be included in the list of drugs which may induce acute hepatitis.     

Arrhythmogenic right ventricular cardiomyopathy

It is a case of 35 year-old woman with arrhythmogenic right ventricular cardiomyopathy, who was without any clinical symptoms of the disease, but she had a family history of sudden cardiac death of her twin sister. This was a reason for initiate a wide diagnostic process in our patient.     

Rate-related left bundle branch block with chest pain and normal coronary arteriograms treated by exercise training

A clinical observation of chest pain associated with the onset of rate-related left bundle branch block has been described in patients with normal coronary arteriograms. The authors used standard cardiac rehabilitation techniques for exercise training in a 47-year-old woman with these manifestations. Serial treadmill tests revealed that during the course of 3 months of exercise training, the heart rate at onset of LBBB gradually rose from 133 to 175 beats per minute, and she no longer developed symptoms during her routine daily activities or exercises. Exercise training was a successful nonpharmacologic strategy that delayed the onset of rate-related LBBB and chest pain in this patient and avoided the need for beta blocker therapy.     

Pacing for hypertrophic obstructive cardiomyopathy does it work?

Sixty-nine years old lady presented with sudden cardiac arrest, she was found to have hypertrophic obstructive cardiomyopathy, she refused septal myomectomy and had a dual chamber ICD implanted, she was put on right ventricular apical pacing with short AV interval, after pacing her max pressure gradient across left ventricular out flow tract (LVOT) dropped from 117 mmHg to 21 mmHg and her symptoms much improved over a follow up period of 1 year.     

ACTH and prolactin deficiency

A 35 year old woman suffering from ACTH and prolactin (Prl) deficiency is described. Her symptoms of adrenal insufficiency appeared gradually after her first pregnancy in 1970; however, she conceived twice more and delivered healthy babies in 1972 and 1974, which she could not breast feed due to lack of milk. During an episode of pneumonia in 1977 she suffered acute adrenal insufficiency, after which she began treatment with hydrocortisone. Her pituitary reserve for TSH, GH, LH and FSH was normal, but her ACTH and Prl levels were undectable and did not respond to acute iv challenges of corticotrophin-releasing factor (CRF) and TRH, respectively. Autoantibodies, including antilactotroph titres, were negative, except for a positive pituitary immunofluorescence to ACTH. There was also no ACTH stimulation to a prolonged infusion of CRF followed by an acute iv bolus. These results, together with the gradual onset of symptoms which worsened after each pregnancy, suggest a possible autoimmune aetiology of her pituitary ACTH and Prl deficiencies.     

Octreotide-sensitive ectopic ACTH production by islet cell carcinoma with multiple liver metastases

We report a 21-year-old woman with ectopic ACTH syndrome due to islet cell carcinoma with multiple liver metastases. On admission, she showed Cushingoid appearance (moon face, central obesity etc.) and had acute respiratory distress syndrome due to pneumocystis carinii pneumonia. Laboratory examination revealed marked elevations of plasma ACTH (735 pg/ml) and cortisol (145 microg/dl) with a profound hypokalemia (2.0 mEq/l). She was found to have multiple masses in the liver and a solid mass in the tail of pancreas by abdominal computerized tomography scanning. Treatment with octreotide successfully reduced elevated plasma ACTH and cortisol levels, and she received frequent transhepatic arterial embolization and chemotherapy. The primary pancreatic tumor was surgically removed, revealing islet cell carcinoma which contained high content of ACTH (100 microg/g wet weight) and abundantly expressed proopiomelanocortin and somatostatin receptor subtype-2 mRNAs as determined by Northern blot analysis. Postoperatively, she was free from symptoms for almost one year. However, progressive enlargement of multiple liver metastases refractory to chemotherapy led her to decide on total hepatectomy and liver transplantation from her father. After liver transplantation, she remained almost free from symptoms for almost one year. However, metastases developed to the mediastinal and paraaortic lymph nodes as detected by 111[In] pentetreotide scintigraphy. Eleven months after liver transplantation, she was again treated with octreotide and, 16 months after, with metyrapone, both of which were effective in reducing ACTH and cortisol levels, respectively, until she died of acute respiratory failure. This case of a young female patient with ectopic ACTH-producing islet cell carcinoma of the pancreas was quite unique in that she survived for 5 years despite the acute onset and rapid progression of the multiple liver metastases at least in part due to the long-lasting favorable response to octreotide and living-related liver transplantation.     

Campylobacter fetus subsp. fetus sepsis: a case report and review of the literatures in Japan

We report a 32-year-old female with eating disorder whose body weight was only 20 kg. She was admitted to the hospital with severe low nutrition, low proteinemia, liver dysfunction, hypokalemia and hypoglycemia. On the third hospital day, she had a high fever and Campylobacter fetus subsp. fetus (C. fetus) was isolated from the blood. After treatment with meropenem (1 g/day) intravenous drip injection, her condition improved. C. fetus sepsis is not common disease in Japan. A review of 37 cases of this disease in Japan revealed that the age range of adult patients was 20 to 60 years old. The male-to-female ratio was 4.6 to 1.0. Seventy-eight percent of the patients had underlying diseases which were composed of 11 patients with liver disease, 6 patients with blood dyscrasia and some with diabetes mellitus, heart disease, other malignant tumor and collagen disease. There was no case with eating disorder. All apparent sources of infection in Japan originate from eating raw food. Gastrointestinal symptoms were observed in only 16% of the patients. Recent recommendations for the treatment of C. fetus sepsis are to use gentamicin, imipenem and meropenem. Some strains of C. fetus have resistance to erythromycin, ciprofloxacin. The mortality of this infection is 14% in Japan.     

Acquired Selective Vitamin B12 Malabsorption

A 44-year-old woman underwent extirpation of a reticulum cell sarcoma in the right anxilla 10 years previously, followed by local roentgen treatment. Subsequently, the patient was irradiated because of enlarged lymph nodes in the left axilla and in the abdomen. Because of megalocytic anaemia and normal serum iron, the patient was given three injections of liver extract, whereupon the haemoglobin values rose to normal levels. An impaired absorption of vitamin B₁₂ was demonstrated which did not improve on intrinsic factor. Otherwise, no hitherto known causes of the reduced vitamin B₁₂ absorption could be found, in particular no other malabsorption symptoms were present. When normal intestinal juice was given concurrently, vitamin B₁₂ was absorbed normally, which indicates that the patient lacks a factor in her intestinal juice which is of importance for a normal absorption of vitamin B₁₂. The underlying mechanism of the biochemical defect in the vitamin B₁₂ absorption is difcussed.     

A case of constrictive bronchiolitis]

A 31-year-old woman was admitted to our hospital because of a dry cough and progressive exertional dyspnea after her second delivery. She had almost normal laboratory data except for an elevated value for antibody for nucleic acid, and her chest radiograph was normal. However, pulmonary function tests revealed a mixed pattern of dysfunction, and chest CT revealed a mosaic perfusion pattern. The VATS lung biopsy demonstrated complete occlusion of the membranous bronchiole. These results led to a diagnosis of constrictive bronchiolitis (CB). The patient and her family rejected the option of lung transplantation, and selected immunosuppressive therapy (steroid pulse therapy and cyclophosphamide pulse therapy). Her condition improved temporarily, but her respiratory condition worsened progressively, and finally she died one and a half years after the appearance of the symptoms. Although steroid pulse therapy and cyclophosphamide pulse therapy failed to cure the CB, they did retard its progression.