A case of middle ear tuberculosis; PCR of the otorrhea was useful for the diagnosis]

A 26-year-old female was admitted to our hospital with complaints of fever, cough, otorrhea and otalgia and progressive hearing loss of her left ear. Smears of her sputum were positive for acid-fast bacilli. Smears of her otorrhea were negative for acid-fast bacilli but PCR of her otorrhea was positive. Chest X-ray showed infiltrative shadows with the cavity. She was diagnosed as middle ear tuberculosis associated with pulmonary tuberculosis. After anti-tuberculous chemotherapy, fever, cough, otorrhea and pain of her left ear were improved, but her hearing level was not improved. In the case of middle ear tuberculosis, it is necessary to make an early diagnosis and treatment. This is the first reported case in Japan in which PCR of the otorrhea is positive.     

Lactate dehydrogenase (LDH)-linked immunoglobulin in a patient with Graves' disease treated with methimazole.

A 26-year-old woman who received methimazole treatment for Graves' disease is discussed. Two months following treatment, her serum GOT level rose to 45 K.U, her GPT to 60 K.U, and her lactate dehydrogenase (LDH) to 645 W.U; a hepatic disorder was then suspected. Later, the serum GOT and GPT concentrations decreased to a normal range, but her serum LDH continued to maintain a high level. An LDH isoenzyme analysis showed an abnormally broad LDH. The IgG that was linked to the LDH is suspected to have been the result of her underlying autoimmunity, the methimazole treatment, and the development of her hepatic disorder. Thus, this IgG was thought to be the autoantibody to LDH.     

Beneficial Effect of Aggressive Low‐Density Lipoprotein Apheresis in a Familial Hypercholesterolemic Patient with Severe Diabetic Scleredema

Abstract: We present a 59‐year‐old woman with severe diabetic scleredema (DS) associated with heterozygous familial hypercholesterolemia (FH). She had been treated with drugs to lower blood glucose, with insulin for diabetes mellitus (DM), and with low‐density lipoprotein (LDL) apheresis therapy monthly or every 2 weeks in addition to drugs to lower serum lipids for FH. However, her scleredema had not improved. After we had tried weekly LDL apheresis therapy for a period of 3 years to treat her hyperlipidemia, the levels of her serum lipids were reduced to normal ranges, and scleredema in her nape improved. We also demonstrated the histopathological improvement in dermis of her cervical skin. We conclude that weekly LDL apheresis therapy is effective for diabetic scleredema that is resistant to conventional treatments.     

Lessons Learned from Arti—Collaborating to Improve Care: the Past,Present, and Future of the Cancer and Aging Research Group

Dr Arti Hurria, who died tragically in November 2018, was a driving force in the growing field of geriatric oncology. She led the field through her commitment to her goals, collaboration with her colleagues, and the creation of the Cancer and Aging Research Group (CARG). As two of her closest colleagues, we are carrying her work forward with our colleagues through an infrastructure grant we led with Arti from the National Institute on Aging (R21/33; CARinG). This grant will provide a national structure for continuing to build the field through research, mentorship, and dissemination. We miss our friend dearly but are committed to carrying her legacy forward through CARG. J Am Geriatr Soc 67:879–883, 2019.     

The impact of change in exercise tolerance on activities of daily living and quality of life in COPD: a patient's perspective

Patient-centered outcomes are a critical goal in the management of COPD. This personalized patient account of living with COPD indicates the importance of health-related quality of life (HRQoL) when considering the profound impact of reduced exercise tolerance on activities of daily living. The recognition of breathlessness indicating damage to the lungs from smoking and exacerbations of dyspnea associated with activity avoidance sent this patient to her clinician for a diagnosis of COPD. The eventual acceptance of her condition led to her attendance in a pulmonary rehabilitation program that made a "tremendous difference" for both the exercise and the social support it offered, and it reinforced her determination to maintain her independence and mobility that seem to contribute to a positive attitude in dealing with this challenging disease.     

Recurrence of sarcoidosis: The follow-up of splenic involvement

We report the case of a 53-year-old woman who initially presented with an intermittent dry cough that had lasted for 6 months. An investigation into these symptoms with thoracic computed tomography demonstrated hyperdense shadows in her lungs, and ultrasonography showed the presence of multiple hypoechoic nodules on her spleen. A lung biopsy was then performed, which revealed noncaseating epithelioid cell granulomas. Magnetic resonance imagining of her spleen was performed, which showed low signal intensity. The patient was treated with glucocorticoids, and 3 months later the lesions in her lungs had disappeared completely. Five months later, the lesions in her spleen had disappeared also. However, after 13 months of low-dose prednisone, a miliary pattern was observed on the patient's chest x-ray, although no lesions were observed in her spleen. Laboratory tests demonstrated that her blood level of angiotensin-converting enzyme had increased to 96 IU/L. The dose of prednisone was then increased, and the lung images returned to normal after 2 months of therapy.     

Complex regional pain syndrome type I induced by pacemaker implantation,with a good response to steroids and neurotropin

An 84-year-old woman was referred to our hospital because of aches and pain in her left hand and foot. Three months before her symptoms occurred, a pacemaker had been implanted for the treatment of a 2:1 atrioventricular block with bradycardia. In an X-ray examination, prominently decreased bone density was noted in her left fingers and toes. She was diagnosed to have CRPS-I, which was considered to have been induced by the pacemaker implantation. After treatment with methylprednisolone and Neurotropin, her symptoms dramatically improved.     

Pulmonary cryptococcosis presenting as endobronchial lesions in a patient under corticosteroid treatment

We report a case of pulmonary cryptococcosis in an immunocompromised patient who had focal endobronchial lesions. When we first examined her, she complained of a productive cough that had lasted for two months. Seventeen months prior to this examination, she had been diagnosed with Sjogren syndrome and Sweet syndrome. Since that diagnosis, her condition had been maintained with low-dose prednisolone. We performed a chest CT scan, which revealed a mass, 3 cm in diameter, in the upper lobe of her left lung. A bronchoscopic examination revealed 3 white, elevated lesions in the upper lobe bronchus of her left lung. After 40 days of treatment with fluconazole, the shadow of her lung mass decreased in size to that of a scarred lesion and her white, elevated bronchial lesions disappeared.     

Warfarin and acetaminophen interaction: a summary of the evidence and biologic plausibility

Ms TS is a 66-year-old woman who receives warfarin for prevention of systemic embolization in the setting of hypertension, diabetes, and atrial fibrillation. She had a transient ischemic attack about 4 years ago when she was receiving aspirin. Her INR control was excellent; however, over the past few months it has become erratic, and her average dose required to maintain an INR of 2.0 to 3.0 appears to have decreased. She has had back pain over this same period and has been taking acetaminophen at doses at large as 650 mg four times daily, with her dose varying based on her symptoms. You recall a potential interaction and wonder if (1) her acetaminophen use is contributing to her loss of INR control, and (2) does this interaction place her at increased risk of warfarin-related complications?     

Cytogenetic studies in a patient with acute granulocytic leukemia of seven and one-half years duration

During a 7 1/2-yr period we monitored a chromosomally aberrant cell line in a woman with acute granulocytic leukemia (AGL) whose disease followed a rather unusual course. Her initial remission induced with 6- mercaptopurine (6-MP) and prednisone was maintained for 52 mo with biweekly doses of methotrexate (MTX) given orally. Because signs of liver dysfunction occurred, maintenance therapy was stopped. After 15 mo without chemotherapy, she suffered her first relapse (5 yr 7 mo after the initial diagnosis). A second remission, again induced with 6- MP and prednisone, was maintained for 1 yr, after which a second relapse occurred. Another remission lasting for only 4 mo was followed by a relapse of the leukemic process which led to her death. Cytogenetic studies of marrow cells and peripheral blood at the time of her initial diagnosis showed abnormal stem lines with characteristic chromosome markers. A small percentage of malignant cells bearing these markers persisted in her marrow during the years of her prolonged remission. At the time of her first relapse, 75% of her marrow cells had the marker karyotype, and at the time of her death (7 1/2 yr after the leukemia was diagnosed) all analyzable marrow metaphases had the characteristic chromosome changes.     

Difficulties in taking care of business: women addicts as mothers

This paper is devoted to a discussion of women addicts as mothers. Women who are addicted while pregnant begin their careers as mothers with extreme guilt and a sense of initial failure. Heroin becomes a mechanism for coping with the routine difficulties of childraising. Children can also act as a controlling force on their mother's addiction if she has the option to perform her mothering duties in an otherwise "normal" fashion. If the woman is being supported adequately and can be available for her children, it is possible to combine addiction and mothering. Often, however, the woman has to work outside the home (usually in criminal pursuits) and the general chaos of her life greatly impinges on her ability to fulfill her mothering duties. Children are occasionally mistreated, sometimes neglected physically, and often neglected psychologically by a mother who is frequently absent. Addicted mothers feel extreme guilt and remorse over this neglect, and often take stock of their situation when their roles as a mother is threatened; the children are being taken away physically or growing up and she is losing them to time. The woman addict most often wants "out" of the heroin life when her children and her role as mother--her last remaining option--are in jeopardy.     

Successful treatment with rituximab for autoimmune hemolytic anemia concomitant with proliferation of Epstein-Barr virus and monoclonal gammopathy in a post-nonmyeloablative stem cell transplant patient

A 30-year-old Japanese woman who underwent nonmyeloablative stem cell transplantation from her HLA-matched sister developed autoimmune hemolytic anemia (AIHA). There was proliferation of EBV-DNA in her peripheral blood and monoclonal gammopathy, both predictive factors of post-transplant lymphoproliferative disorder (PTLD). As conventional immunosuppressive therapy for AIHA could lead to overt PTLD, we decided to give her rituximab 375 mg/m² once weekly for a total of four doses. After this therapy, both her AIHA and monoclonal gammopathy were resolved and EBV-DNA became undetectable. Rituximab therapy deserves consideration for treatment of post-allogeneic stem cell transplant patients with AIHA, especially for patients who cannot be given immunosuppressive therapy.     

New evidence confirms risks associated with prehypertension and benefits of therapeutic lifestyle changes in management

TJ is a 57-year-old female high school biology teacher who presents for a routine checkup. She has a strong family history of heart disease and wants to ensure that she does everything possible to keep her risk low. TJ is postmenopausal and stopped hormone replacement therapy 3 years ago. Physical examination is unremarkable other than the fact that the patient is overweight, with a body mass index of 29.2 kg/m2, and her blood pressure (BP) is 132/84 mm Hg. Her fasting plasma glucose level is 103 mg/dL, high-density lipoprotein cholesterol level 38 mg/dL, and triglyceride level 165 mg/dL. Based on her BP and concurrent cardiometabolic syndrome, what do you tell her about the risks associated with her BP, and how do you approach her management?     

Hashimoto's thyroiditis with heterogeneous antithyrotropin receptor antibodies: unique epitopes may contribute to the regulation of thyroid function by the antibodies

Blocking-type TSH-binding inhibitor Igs (TBIIs) are known to cause hypothyroidism and an atrophic thyroid gland in patients with primary myxedema. They can block the activity of thyroid-stimulating antibodies (TSAbs) in Graves' patients as well as the activity of TSH. The majority of the epitopes for these blocking-type TBIIs have been, and are shown herein, to be present on the C-terminal region of the extracellular domain of the human TSH receptor (TSHR), whereas those for Graves' TSAbs are on the N-terminus. We report on a patient with Hashimoto's thyroiditis who suffered from mild hypothyroidism and a moderately sized goiter. Her serum had a potent blocking-type TBII and a weak TSAb in human and porcine TSHR systems. Using human TSHR/lutropin-CG receptor chimeras, we determined that the functional epitope of her blocking-type TBII was uniquely present on the N-terminal, rather than the C-terminal, region of the extracellular domain of the TSHR, unlike the case for blocking-type TBIIs in primary myxedema patients. The epitope of her TSAb was also unusual. Although the functional epitopes of most TSAbs are known to involve the N-terminal region of the receptor, her TSAb epitope did not seem to be present solely on the N- or C-terminus of the extracellular domain of the receptor. Blocking-type TBIIs from patients with primary myxedema blocked her TSAb activity as well as stimulation by TSH; her blocking-type TBII was able to only partially block her TSAb. In contrast, her blocking-type TBII almost completely blocked TSAbs from Graves' patients. Thus, we suggest that the unique epitopes of this patient's heterogeneous population of TSH receptor antibodies, at least in part, contribute to regulation of her thyroid function.     

Reversible dilated cardiomyopathy associated with glucagonoma

An association between dilated cardiomyopathy and glucagonoma has not previously been described. A case of a 54 year old woman with tachycardia and congestive heart failure is described. Initial evaluation included an echocardiogram, which showed dilated cardiomyopathy with an ejection fraction of 15%. Coronary angiography and endomyocardial biopsy did not identify a secondary cause of her cardiomyopathy. She subsequently developed necrolytic migratory erythema, and imaging of her pancreas identified a pancreatic mass with a major increase of her serum glucagon concentration. Tachycardia persisted despite treatment with beta blockers. After resection of her tumour, her heart rate normalised and subsequently her heart returned to normal size and function. Glucagon is used to treat overdoses of beta blockers and calcium channel blockers, increasing heart rate by increasing myocardial cyclic AMP concentrations. Although rare, in the appropriate clinical setting, glucagonoma should be considered in the differential diagnosis for tachycardia and dilated cardiomyopathy.     

Liver cirrhosis as a delayed complication of Stevens-Johnson syndrome

A 26-year-old woman was referred to our department due to fever and skin rash after having taken medication for a common cold. Physical examination revealed erythematous skin changes on her body associated with mucosal involvement in her eyes and oral cavity. Peripheral blood examination revealed leukopenia and thrombocytopenia. Liver function test showed hyperbilirubinemia. She was managed with high dose intravenous immunoglobulin (IVIG) at 1.0 gm/kg of body weight infused for 5 consecutive days. Although the patient's skin lesion improved dramatically with IVIG therapy, her hyperbilirubinemia aggravated progressively. Eighteen months after her presentation, liver cirrhosis was diagnosed by ultrasonography, laboratory and liver biopsy findings.     

Rhnull Red Cells and Pregnancy

Abstract. The haemoglobin, haematocrit and osmotic fragility red cell values in a South African white woman with Rh_null cells and the corresponding haematological syndrome were shown to vary only minimally during her third pregnancy. This occurred in spite of the precautionary donation by her of two units of her blood at 20 and at 27 weeks of pregnancy for storage in liquid nitrogen. Although there was fear to the contrary, the woman's infant was found at birth to be suffering only mildly from haemolytic disease of the newborn due to the anti-Rh29 antibodies present in her plasma.     

Unusual late presentation of X-linked chronic granulomatous disease in an adult female with a somatic mosaic for a novel mutation in CYBB

Most patients with chronic granulomatous disease (CGD) have mutations in the X-linked CYBB gene that encodes gp91phox, a component of the phagocyte NADPH oxidase. The resulting X-linked form of CGD is usually manifested in boys. Rarely, X-CGD is encountered in female carriers with extreme expression of the mutated gene. Here, we report on a woman with a novel mutation in CYBB (CCG[90-92]-->GGT), predicting Tyr30Arg31-->stop, Val in gp91phox, who presented with clinical symptoms at the age of 66. The mutation was present in heterozygous form in genomic DNA from her leukocytes but was fully expressed in mRNA from these cells, indicating that in her leukocytes the X chromosome carrying the nonmutated CYBB allele had been inactivated. Indeed, only 0.4% to 2% of her neutrophils showed NADPH oxidase activity. This extreme skewing of her X-chromosome inactivation was not found in her cheek mucosal cells and is thus not due to a general defect in gene methylation on one X chromosome. Moreover, the CYBB mutation was not present in the DNA from her cheek cells and was barely detectable in the DNA from her memory T lymphocytes. Thus, this patient shows a somatic mosaic for the CYBB mutation, which probably originated during her lifetime in her bone marrow.     

Denopamine responsive pulmonary hypertension in a patient with collagen disease

We report a 52-years-old female patient with collagen disease and pulmonary hypertension. Denopamine, beta-adrenergic agonist, decreased her high pulmonary arterial pressure and improved dyspnea on exercise after long term use. She had suffered from Raynaud's phenomenon, pulmonary fibrosis, shortening of lingual frenulum and positive ANA and RA test. Although her pulmonary fibrosis had been well controlled by azathioprine, dyspnea on exercise became worse, so she admitted to our hospital for further examination in Feb 1988. Right heart catheterization revealed her high pulmonary arterial pressure (mean 29 mmHg). Under right heart catheterization, denopamine markedly decreased her pulmonary arterial pressure and increased her cardiac output. After about 6 weeks' use of denopamine, her mean pulmonary arterial pressure decreased to 15 mmHg, PO2 increased from 43 to 62 mmHg and dyspnea improved. Denopamine has been regarded as a selective beta 1-adrenergic agonist. In this case, denopamine might have beta 2-agonist effect to dilate pulmonary vasculature, or have secondary effect to increase PO2 by the improvement of cardiac function. Denopamine might be useful for pulmonary hypertension with collagen diseases.     

Pure red cell aplasia with inhibitor to erythroid precursors in serum

A patient with pure red cell aplasia (PRCA), who had the inhibitor to erythroid precursors in serum, is described. A 72-year-old female was referred to Nagoya National Hospital because of progressing anemia in April 1988. On admission, her hemoglobin was 4.8 g/dl, reticulocyte 0.8%, and bone marrow specimen contained only 1.2% erythroblast. On these bases, she was diagnosed as pure red cell aplasia. After small amount of blood was transfused, her hemoglobin and erythroblast in bone marrow (EBM) increased to 7.8 g/dl and 39.1%, respectively, and she was discharged. However, after a month, her hemoglobin dropped to 4.6 g/dl, reticulocyte to 0.1%, and EBM to 0%. Soon after corticosteroid therapy (prednisolone, 40 mg, daily) was started, a marked elevation of reticulocyte count was observed, and then her hemoglobin increased to 11.0 g/dl, and EBM to 31.6%. The reason for a transient spontaneous remission at the onset of her disease was occurred is unclear. The number of BFU-E in her bone marrow was within normal range, but it was suppressed significantly (65%) after the addition of her serum and the complement purified from rabbit plasma. This finding suggest the presence of inhibitor to erythroid precursors in her serum.