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CEN Case Reports

Gurinder Kumar, Rajendran Nair, Bassem Soliman Hendawy, Omar Ahmed AlShkeili, Ahmed Abdulla Alabdouli, Adnan Mohamed Al Ali, Amal Mohamed Jasem AlTenaiji
Metabolic disorders, although rare, can involve multiple organ systems and have a varied presentation. Renal involvement has been reported in several metabolic disorders in the pediatric age group. We report a rare metabolic disorder, long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency, in a child who developed steroid-resistant nephrotic syndrome at the age of 5 years. Renal biopsy showed features of collapsing glomerulopathy. The child had progressive chronic kidney disease. Alternative immunosuppressants including tacrolimus failed to show any clinical improvement...
February 12, 2019: CEN Case Reports
Fumiaki Obata, Hideharu Abe, Taichi Murakami, Sayo Ueda, Taizo Inagaki, Masanori Minato, Hiroyuki Ono, Kenji Nishimura, Eriko Shibata, Masanori Tamaki, Fumi Kishi, Seiji Kishi, Kojiro Nagai, Toshio Doi
Thromboembolism is a major complication of nephrotic syndrome, with the renal vein being the most frequent site. However, the incidence of portal vein thrombosis (PVT) in patients with nephrotic syndrome is rare. We report a case of a relapsed steroid-dependent minimal change disease with incidental PVT. A 38-year-old man presented with anasarca. Elevated liver enzymes were discovered during routine blood testing within days after commencing treatment. Although drug-induced liver injuries are frequently observed with mild aminotransferase abnormality during therapy with steroid or immune-suppressive agents, imaging revealed a massive thrombus of the portal vein, which was treated by anticoagulant therapy with edoxaban...
February 5, 2019: CEN Case Reports
Shinya Kawamoto, Yuji Hidaka, Yu Kaneko, Hideo Misawa, Katsuhiro Nagahori, Atsunori Yoshino, Takamitsu Okamura, Shinichi Ban, Yoshihiko Ueda, Tetsuro Takeda
Light chain proximal tubulopathy is a rare manifestation of monoclonal gammopathy. A 73-year-old Japanese woman was noted to have urinary protein and hypertension on health examination and visited the regional clinic. She was noted to have IgG λ M protein and suspected of multiple myeloma. She was referred to us with massive proteinuria (7.5 g/g creatinine) and Bence Jones proteinuria without renal dysfunction. A renal biopsy revealed no glomerular abnormalities, but a tubular cast was observed partially in tubules without tubular atrophy or a crystalline structure...
February 5, 2019: CEN Case Reports
Sami Alobaidi, Ammar AlDabbagh, Amany Alamoudi, Murad Almowarey, Ahmed Akl
Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare condition. It is characterized by very high maternal mortality and morbidity. Most cases of P-aHUS (79%) manifest in the postpartum period; this is probably due to the complement's involvement in aHUS pathogenesis. Eculizumab is approved for aHUS treatment, but its use is limited due to cost, unknown duration of treatment, and vague dose intervals to keep patients in remission. In this case report, we present a 26-year-old female with P-aHUS with hybrid CFHR1/CFH gene...
February 4, 2019: CEN Case Reports
Fransico Galeano-Valle, F J Díaz-Crespo, R Melero-Martín, J E Apaza-Chávez, J Del-Toro-Cervera, P Demelo-Rodríguez
Crystal-storing histiocytosis (CSH) is a rare disorder characterized by the accumulation of nonneoplastic histiocytes containing intracytoplasmic crystallized immunoglobulins. In most cases, there is an associated underlying lymphoplasmacytic neoplasm expressing Ig kappa light chain. About 131 cases of CSH have been identified. There is a localized and a generalized form of CSH and it can involve several sites including bone marrow, lungs, lymph nodes, liver, spleen, gastrointestinal tract, and kidney. Generalized CSH is less frequent and involves multiple organs and tends to have a worst prognosis than localized CSH...
February 2, 2019: CEN Case Reports
Tamayo Kato, Yasuyuki Ushiogi, Hitoshi Yokoyama, Shigeo Hara, Akira Matsunaga, Eri Muso, Takao Saito
A 47-year-old Japanese man with mild proteinuria was treated with an ACE inhibitor and antiplatelet agent for 7 years. However, urinary protein levels increased and renal biopsy was performed. Eight out of 20 glomeruli showed global or segmental sclerosis with foamy changes or bubbles, but with a different appearance to typical foam cells or lipoprotein thrombi. "Spike" formation, as observed in membranous nephropathy (MN), was segmentally detected in methenamine silver-stained sections. In an immunofluorescence study, weak linear patterns for IgG and scanty deposits for C3 were observed in glomeruli, but were not specific for immunogenetic MN...
January 30, 2019: CEN Case Reports
Junki Morino, Keiji Hirai, Shohei Kaneko, Saori Minato, Katsunori Yanai, Yuko Mutsuyoshi, Hiroki Ishii, Momoko Matsuyama, Taisuke Kitano, Mitsutoshi Shindo, Akinori Aomatsu, Haruhisa Miyazawa, Kiyonori Ito, Yuichiro Ueda, Taro Hoshino, Susumu Ookawara, Kazuo Hara, Yoshiyuki Morishita
We herein report two cases of advanced stage rapidly progressive diabetic nephropathy that were effectively treated with combination therapy including renin-angiotensin-aldosterone system (RAS) blocker [angiotensin II receptor blocker (ARB)], glucagon-like peptide-1 (GLP-1) receptor agonist and sodium glucose transporter-2 (SGLT-2) inhibitor. A 30-year-old woman with advanced stage diabetic nephropathy [estimated glomerular filtration rate (eGFR): 20.7 mL/min/1.73 m2 ; proteinuria: 13.2 g/gCr], showing a rapidly progressive pattern (annual eGFR change: - 60...
January 20, 2019: CEN Case Reports
Xin Li, Chi Yuen Cheung
Anticoagulant-related nephropathy (ARN), a significant but frequently undiagnosed problem in patients receiving anticoagulation, is found to be associated with increased renal morbidity and all-cause mortality. While ARN is mainly associated with warfarin use, recent case reports suggest that it may also occur in patients taking direct oral anticoagulants (DOAC). We report a patient who had a history of alcoholic liver cirrhosis and paroxysmal atrial fibrillation, and received dabigatran 110 mg twice daily for 1 year...
January 18, 2019: CEN Case Reports
Nadeem Iqbal, Asfand Yar, Nadeem Iqbal, Rabiyya Khan, Abdul Ahad Ehsan Sheikh, Umair Syed Mahmud
A 58-year-old male patient, with end-stage renal disease secondary to hypertension, underwent living-related kidney transplant at our transplant unit. The transplant surgery went uneventful and brisk urine output was recorded. Four hours after the transplant, the output suddenly dropped despite normal central venous pressure. Doppler scan revealed an extensive peri-allograft hematoma and high renal arterial resistive indices (RI). The patient was taken to the operating room where capsulotomy of the subcapsular hematoma was done...
January 16, 2019: CEN Case Reports
Keiko Kai, Naoto Tominaga, Kenichiro Koitabashi, Daisuke Ichikawa, Yugo Shibagaki
Case 1: A 45-year-old man, admitted for symptomatic hyponatremia, was diagnosed with advanced small-cell lung cancer and severe hyponatremia due to the syndrome of inappropriate secretion of antidiuretic hormone. In addition to chemotherapy, the patient was treated with increased dietary salt intake (15 g/day), fluid restriction of 500 mL/day, and amino acid supplementation to maintain a urea load of 31 g/day. Due to the difficulty in changing his habit of drinking 2-3 L/day after discharge, tolvaptan was started...
January 14, 2019: CEN Case Reports
Masashi Morita, Yoshito Yamaguchi, Satoshi Masuyama, Jun Nakamura, Sachio Kajimoto, Ryota Haga, Yu Yamanouchi, Katsuyuki Nagatoya, Hideaki Miwa, Atsushi Yamauchi
A 47-year-old man presented with severe hypokalemic paralysis and respiratory failure. A large amount of potassium was administered along with providing intensive care, and his condition improved. Hypokalemia was attributed to increased urinary potassium excretion. A kidney biopsy was performed to make a definitive histological diagnosis. It revealed acute tubulointerstitial nephritis (TIN). After the diagnosis, prednisolone was administered, and the TIN gradually improved. From the clinical course and laboratory findings, the TIN was presumed to be an autoimmune disorder...
January 14, 2019: CEN Case Reports
Takahiro Shinzato, Taro Kubo, Toshihiro Shimizu, Koji Nanmoku, Takashi Yagisawa
Fibrosing cholestatic hepatitis (FCH) is a fatal disorder that presents as a progressive deterioration of liver function over a period of several weeks to several months. It is caused by the direct cytotoxic effect of the over-expression of viral antigens on hepatocytes in immunosuppressed patients. Our patient was a 59-year-old man with hepatitis C virus (HCV) infection of genotype 2a who had suffered from end-stage renal disease due to diabetic nephropathy and underwent kidney transplantation. His serum total bilirubin levels gradually increased to 20 mg/dl and liver atrophy progressed during several weeks after kidney transplantation, which was initially difficult to distinguish from drug-induced liver injury...
January 2, 2019: CEN Case Reports
Chikako Hirashima, Manabu Ogoyama, Miyuki Abe, Satoru Shiraishi, Taro Sugase, Toshiro Niki, Shigeki Matsubara, Akihide Ohkuchi
Measurement of the soluble fms-like tyrosine kinase 1 (sFlt-1)/placental growth factor (PlGF) ratio may be clinically useful to discriminate systemic lupus erythematosus (SLE) from preeclampsia. Here, we present a pregnant woman with new-onset SLE with hypertension, with the measurement of the sFlt-1/PlGF ratio during pregnancy. A 31-year-old Japanese nulliparous woman, who had been diagnosed with idiopathic thrombocytopenic purpura at 10 years, had a systolic blood pressure of 120 mmHg and was negative for proteinuria at 12+1 weeks...
December 18, 2018: CEN Case Reports
Ari Shemesh, Edward Margolin
Nonarteritic anterior ischemic optic neuropathy (NAION) is a well acknowledged rare complication of chronic hemodialysis in patients with end-stage renal disease (ESRD). We present a unique case of a patient on chronic hemodialysis who presented with an NAION in the right eye, then 3 months later developed an NAION in the left eye followed in 1 month by second NAION episode in the left eye. Every episode of NAION was accompanied by intradialytic hypotension (drop in systolic blood pressure of over 20 mmHg) where visual loss was noticed at the end of the dialysis session...
November 21, 2018: CEN Case Reports
Shunsuke Yamada, Hokuto Arase, Toshifumi Morishita, Akihiro Tsuchimoto, Kumiko Torisu, Takehiro Torisu, Kazuhiko Tsuruya, Toshiaki Nakano, Takanari Kitazono
A 57-year-old woman with pre-dialysis chronic kidney disease (CKD) was hospitalized because of fever and fatigue. On admission, increased inflammatory response and pyuria with bacteriuria were observed. Pyelonephritis was successfully treated with antibiotics, whereas her fatigue continued and she developed progressive hypercalcemia and hyponatremia; serum sodium level, 116 mEq/L and corrected serum calcium level, 13.4 mg/dL. Plasma concentrations of adrenocorticotropic hormone and cortisol and serum luteinizing hormone were under the detection level...
November 19, 2018: CEN Case Reports
Harini Bejjanki, Abhilash Koratala
No abstract text is available yet for this article.
November 12, 2018: CEN Case Reports
(no author information available yet)
No abstract text is available yet for this article.
November 2018: CEN Case Reports
Javier Naranjo Muñoz, Carlos Narváez, Florentino Villanego, María Auxiliadora Mazuecos, Manuel Ceballos
No abstract text is available yet for this article.
November 2018: CEN Case Reports
Rida Salman, Mikhael G Sebaaly, Karl Asmar, Mohammad Nasserdine, Sami Bannoura, Nabil J Khoury
Renal cell carcinoma (RCC) is a tumor that usually metastasizes to lung, liver, bone and brain, but rarely to skeletal muscles. We report a case of an elderly man with a history of bilateral metachronous RCC for which he underwent curative bilateral nephrectomies and renal transplantation, was in remission, and presented with a large solitary skeletal muscle metastasis from the initial RCC, 3 years later.
November 2018: CEN Case Reports
Bassel Lakkis, Alissar El Chediak, Jana G Hashash, Sahar H Koubar
Spinal muscular atrophy (SMA) is a genetic neuromuscular disease characterized by progressive muscle weakness and atrophy. We report a case of a 36-year-old man with SMA type 3 who presented to our emergency department with epigastric pain and vomiting. He was found to have severe ketoacidosis on laboratory evaluation. The patient's symptoms and ketoacidosis resolved after dextrose infusion and a relatively small amount of sodium bicarbonate infusion. Given the severity of the ketosis that seemed inconsistent with moderate starvation alone, we postulate that there must have been other contributing factors besides moderate starvation that might explain the severity of acidosis in this particular patient...
November 2018: CEN Case Reports
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