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A 41-year-old male patient was admitted to our hospital due to massive proteinuria and hematuria. His 24-hour urinary protein excretion and the number of urinary erythrocytes were 3.91 g/day and 50-99/high-power field, respectively. A renal biopsy showed a severe pathological pattern of immunoglobulin A nephropathy (IgAN) that involved marked endocapillary proliferation and segmental sclerosis (Oxford-MEST score: M0, E1, S1, T0). Because he had nephrotic-level proteinuria with severe pathological findings, which are tonsillectomy and corticosteroid pulse therapy-resistant characteristics, he received mizoribine for a long period as part of the combination therapy using corticosteroid, tonsillectomy, dipyridamole, warfarin and renin-angiotensin-aldosterone system blockers. Twelve months after the beginning of treatment, his urinary findings were normal. In this report, we describe the pathological findings and successful treatment course, and discuss the potential effects of long-term coadministration of mizoribine for adult IgAN treatment.

Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a rare and recently identified disease with a poor prognosis irrespective of the treatment. Recently, the possibility of recurrent or de novo PGNMID after kidney transplantation has been reported, which is associated with a better prognosis compared to PGNMID on native kidneys. Nevertheless, at present, due to the very few cases of recurrent PGNMID diagnosed, there is no proven effective treatment. Here, we report a case of recurrent PGNMID successfully treated with plasmapheresis, steroids and mycophenolate mofetil. Our report suggests that plasmapheresis might be a valid therapeutic option to treat recurrent PGNMID.

Kindler syndrome is a rare autosomal recessive genodermatosis characterized by trauma-induced acral blisters in infancy and childhood, photosensitivity, and progressive poikiloderma. Other clinical features include chronic erosive gingivitis, dysphagia, esophageal and urethral strictures, ectropion, and an increased risk of mucocutaneous squamous cell carcinoma. We describe a patient with Kindler syndrome associated with squamous cell carcinoma of the skin and larynx. He had squamous cell carcinoma on his left knee with simultaneous unresectable laryngeal carcinoma at the age of 43 years. The squamous cell carcinoma on his knee was excised and the laryngeal carcinoma was treated with radiation therapy. Although pathophysiology of Kindler syndrome and its frequency of association with cancer are still not fully elucidated, we speculate that long-term erosion and regeneration of mucosal and cutaneous surfaces may have induced squamous cell carcinoma on the patient’s knee and larynx.

Eosinophilic esophagitis (EoE) is a chronic esophageal disease increasingly recognized in adults for its gastrointestinal manifestations. This paper discusses a young woman with EoE who presented with persistent hiccups and intermittent dyspepsia. The patient was initially treated with trials of both H(2) blocker and proton pump inhibitor. However, her hiccups resolved only after treatment with topical fluticasone. A repeat upper endoscopy while on steroid treatment demonstrated both histologic remission of EoE and resolution of esophageal trachealization. Our patient's clinical course supports an association between hiccups and EoE, suggesting that EoE be considered in the differential diagnosis of patients with refractory hiccups.

Blastocystis hominis and Endolimax nana exist as two separate parasitic organisms; however co-infection with the two individual parasites has been well documented. Although often symptomatic in immunocompromised individuals, the pathogenicity of the organisms in immunocompetent subjects causing gastrointestinal symptoms has been debated, with studies revealing mixed results. Clinically, both B. hominis and E. nana infection may result in acute or chronic diarrhea, generalized abdominal pain, nausea, vomiting, flatulence and anorexia. We report the case of a 24-year-old immunocompetent male presenting with chronic diarrhea and abdominal pain secondary to B. hominis and E. nana treated with metronidazole, resulting in symptom resolution and eradication of the organisms. Our case illustrates that clinicians should be cognizant of both B. hominis and E. nana infection as a cause of chronic diarrhea in an immunocompetent host. Such awareness will aid in a timely diagnosis and possible parasitic eradication with resolution of gastrointestinal symptoms.

Ingestion of mushrooms from the genus Amanita can present detrimental consequences to the human body. The mushroom is frequently found in the coastal Pacific Northwest, Pennsylvania, New Jersey, and Ohio. Amanitin, one of the two distinct toxins isolated from the Amanita mushroom, is responsible for the majority of symptoms and signs seen with mushroom poisoning. Clinically, ingestion of these mushrooms can result in a wide range of clinical symptoms including nausea, vomiting, crampy abdominal pain, and diarrhea. There have been several case reports of patients who developed severe hepatic failure that required liver transplantation. Thus, it is important to recognize the symptoms early and treat the patients with the available agents including multidose activated charcoal, N-acetylcysteine, penicillin G, and Silybum. Through an extensive literature search, we found no published literature on amatoxin poisoning in the state of Texas. With new cases of amatoxin poisoning emerging in the state, it is important for healthcare providers and workers to have a better awareness and early recognition of the detrimental effects of Amanita species poisoning and to be educated to provide the proper care for this group of patients.

In the case reported here, after prolonged medical therapy resistance, severe proteinuria subsided following bilateral renal artery embolization (RAE). Thereafter, respiratory distress, anasarca edema, muscle mass, and serum albumin level improved after regular hemodialysis. Although RAE is reported to be a safe and effective therapeutic procedure, it is rarely used for severe proteinuria with prolonged medical therapy resistance. The limited use of bilateral RAE for nephrological purposes may be partly related to its tendency to destroy renal function, which results in anuria and subsequent regular dialysis. However, delayed RAE could cause the patient to reach a life-threatening cachexic state and could increase the risk of morbidity and mortality due to severe proteinuria-induced hypoalbuminemia. Our case and selected previous reports reveal important information for physicians and patients while discussing prognoses and considering the pros and cons of bilateral RAE.

Cryoglobulinaemia is a systemic inflammatory condition characterised by immune complex-mediated small-to-medium-sized vasculitis. It has a wide variety of presentations ranging from bruising, neuropathy, and hepatosplenomegaly to acute renal failure. Mixed cryoglobulinaemia is the most common type and is strongly associated with hepatitis C. Management approaches include use of cyclophosphamide, prednisolone, and plasmapheresis, with differing views on alternative treatments in resistant cases. Rituximab has emerged as an attractive option in resistant cases on account of its potent immunosuppressive effects on B cells. We describe a case of type 2 mixed cryoglobulinaemia in association with non-Hodgkin's lymphoma resistant to standard treatments which responded well to rituximab. This case is remarkable as mixed cryoglobulinaemia associated with non-Hodgkin's lymphoma presenting with nephritis is unusual, and, contrary to the high rate of recurrence in lymphoma-related cryoglobulinaemia, our patient has not shown any recurrence over 24 months. This highlights an alternative treatment modality which can be used in patients not responsive to existing managements for this condition with benefits of minimal side effects and no oncogenetic potential.

Cytomegalovirus (CMV)-associated gastric ulcers can be found not only in immunocompromised hosts but also in normal individuals. The accurate endoscopic diagnosis of CMV ulcers is not easy because of the absence of characteristic morphological features. We present a case of CMV-associated gastric ulcer in an immunocompetent patient. He was a 33-year-old male with epigastralgia. Upper gastrointestinal endoscopy showed multiple erupted papules and a large irregularly shaped shallow ulcer. We did not find intracellular inclusion bodies characteristic of CMV in hematoxylin-eosin-stained gastric biopsy specimens, while CMV antigens corresponding to intracellular inclusion bodies were confirmed using an immunoperoxidase method with the monoclonal CMV antibody. If CMV ulcers are suspected, it is important to examine for inclusion bodies using not only hematoxylin-eosin staining, but also CMV immunohistochemistry for a sensitive diagnosis.

Nephrotic syndrome (NS) associated with renal artery stenosis is not widely recognized or investigated as a cause of the NS. The mechanisms are incompletely understood, but have largely focused on hemodynamic factors resulting in hyperfiltration injury-induced focal and segmental glomerulosclerosis (FSGS) in the nonstenosed kidney with sparing of the stenotic kidney protected from hemodynamic stress. However, separation of hemodynamic from circulating factors (such as angiotensin II) as the cause of the nephrosis remains difficult. We report a patient presenting with NS who was incidentally discovered to have high-grade bilateral renal artery stenosis from fibromuscular dysplasia. Kidney biopsy revealed FSGS. Proteinuria in our patient did not initially respond to angiotensin-converting enzyme inhibition (ACEI) and correction of stenoses with angioplasties. There was prompt response to steroid treatment. A brief relapse several months later (without associated hypertension) responded to ACEI alone. This is the first reported case of an association between fibromuscular dysplasia and steroid-responsive nephrotic syndrome due to FSGS. This may shed insight into the nature of podocyte injury in patients with high angiotensin states and suggest a possible role for activated renin-angiotensin-aldosterone system (RAAS) triggering an immune-mediated injury, rather than hemodynamic insult. Furthermore the lack of initial response to angioplasty and ACEI suggests that RAAS-activated injury may in some cases require more aggressive immune modulatory therapy with steroids over and above angiotensin inhibition alone. This case also highlights the importance of being aware of possible occult renovascular disease contributing to idiopathic NS with FSGS even when hypertension is only modest.

An internal hernia may be either congenital or acquired. The reported incidence of such hernias is 1–2%. In rare cases, internal hernias are the cause of small bowel obstruction, with a reported incidence of 0.2–0.9%. Transmesocolic hernia of the ascending colon is especially rare. We report a case of transmesocolic hernia of the ascending colon with intestinal obstruction diagnosed preoperatively. A 91-year-old Japanese female was admitted to our hospital with abdominal distention and vomiting of 3 days duration. She had no past history of any abdominal surgery. Abdominal examination revealed distention and tenderness in the right iliac fossa. Abdominal computed tomography revealed ileus in the sac at the left side of the ascending colon and dilatation of the oral side of the intestine. We diagnosed a transmesocolic hernia of the ascending colon with intestinal obstruction and performed emergency surgery. At the time of operation, there was internal herniation of ileal loops through a defect in the ascending mesocolon, without any strangulation of the small bowel. The contents were reduced and the tear in the ascending mesocolon was closed. The postoperative course was uneventful and the patient was discharged 14 days after surgery. In conclusion, preoperative diagnosis of bowel obstruction caused by a congenital mesocolic hernia remains difficult despite the techniques currently available, so it is important to consider the possibility of a transmesocolic hernia when diagnosing a patient with ileus with no past history of abdominal surgery.

The major problem associated with the creation of an arteriovenous fistula (AVF), which is the optimal form of vascular access for hemodialysis, is the fact that fistulas do not necessarily mature into a usable vascular access, requiring subsequent revision and construction of another access. The caliber of the vessels used for fistula creation is a pivotal factor predicting the presumable maturation of the constructed fistula, and veins less than 2.5 mm in diameter have poor outcomes. This report describes the balloon-assisted creation and maturation of an autogenous radial-cephalic AVF in a patient with a small-caliber vein and a radial artery measuring approximately 2 and 1.5 mm in diameter, respectively. The clinical impact of percutaneous radiological intervention for expediting the maturation of an autogenous AVF with small-caliber vessels is also discussed.