Calciphylaxis: A condition mimicking necrotizing vasculitis

Summary A patient with end stage renal disease developed ischaemic skin necrosis and digital gangrene. He had diffuse arterial calcification associated with hyperparathyroidism secondary to renal failure. The patient received inappropriate cyclophosphamide therapy as he had been misdiagnosed as having an inflammatory vasculitis. This clinical picture, previously named calciphylaxis should come into the differential diagnosis of systemic vasculitis in a uraemic patient with hyperparathyroidism.     

Discarded Livers Find a New Life: Engineered Liver Grafts Using Hepatocytes Recovered From Marginal Livers

Treatment for end‐stage liver failure is restricted by the critical shortage of donor organs; about 4000 people die in the USA while waiting for a transplantable organ. This situation has been a major driving force behind the rise of tissue engineering to build artificial tissues/organs. Recent advancements in creating transplantable liver grafts using decellularized liver scaffolds bring the field closer to clinical translation. However, a source of readily available and highly functional adult hepatocytes in adequate numbers for regenerative liver therapies still remains unclear. Here, we describe a new method to utilize discarded livers to make transplantable new liver grafts. We show that marginal donor livers damaged due to warm ischemia could be treated with machine perfusion to yield 39 million viable hepatocytes per gram of liver, similar to fresh livers, and these cells could be used to repopulate decellularized liver matrix (DLM) scaffolds to make transplantable liver grafts. The hepatocytes from recovered livers sustained their characteristic epithelial morphology while they exhibited slightly lower protein synthesis functions both in plate cultures and in recellularized liver grafts. The dampened protein synthesis was attributed to residual endoplasmic reticulum stress found in recovered cells. The results here represent a unique approach to reengineer transplantable liver grafts solely from discarded organs.     

Hematopoietic stem cell transplantation from unrelated donors in children with DOCK8 deficiency

DIDS is a unique form of combined immune deficiency characterized by an unusual susceptibility to cutaneous viral infections, severe allergies with eosinophilia and elevated immunoglobulin E titers, autoimmunity, and cancer. HSCT is considered the standard of care for this deadly disease. We have retrospectively analyzed the outcome of allogeneic HSCT from unrelated donors in patients with DIDS. Data from four patients, with five transplants, are presented. All patients received transplants from unrelated donors' BM, except for one patient who received a cord blood transplant. The conditioning regimens were based on myeloablative protocols for BM derived transplants; a NM regimen was pursued for the patient who received a cord blood transplant, which resulted in graft rejection. Although recurrent pneumonia and skin infections resolved immediately after transplantation, all patients subsequently developed human herpesvirus infection, including cutaneous herpetic lesions, cytomegalovirus reactivation, and zona zoster, which could be attributed to the use of ATG. Despite the presence of serious morbidities prior to transplantation, all patients recovered successfully. DIDS can be successfully treated with allogeneic HSCT from unrelated donors following a myeloablative conditioning regimen, with a reasonable safety profile.     

Retrovesical leiomyosarcoma

A case of retrovesical leiomyosarcoma in a male patient is described. The preoperative radiological and laboratory studies were inconclusive in determining the primary origin of the tumor. Diagnosis of a malignant tumor with smooth muscle origin was suggested by needle biopsy. However, the definitive diagnosis and the primary site of origin could only be determined by surgical exploration and subsequent histopathologic examination after excision. No sign of recurrence or metastasis was present 12 months after complete surgical resection.     

Recurrent Merkel cell carcinoma of the gluteal region: A case report

Merkel cell carcinoma (MCC) is a rare malignant tumor of the skin. The development of MCC on non‐sun‐exposed skin is extremely rare, with few cases reported in the literature. The present authors aimed to highlight the characteristic features and treatment options of this tumor. The present authors present a 50‐year‐old man who developed MCC on the left gluteal region (non‐sun‐exposed skin). After surgery with clear margins, adjuvant radiotherapy was given. Three months after radiotherapy, lymphatic recurrence was observed and he was treated with chemotherapy. On follow‐up, systemic metastases were found and palliative treatment was planned.     

Studies on Photoinduced ATRP in the Presence of Photoinitiator

Photoinduced reverse and simultaneous reverse and normal initiation (SR&NI) atom transfer radical polymerizations (ATRPs) of methyl methacrylate are investigated. The type and concentration of photoinitiators directly influences the photoinduced reverse ATRP. Although the polymerization can be initiated in the absence of alkyl halide, loss of control over the polymerization process is observed. The photoinduced SR&NI ATRP proceed in a well‐controlled manner under UV light at room temperature, as is evident in kinetic and chain extension studies. Better agreement between the experimental and theoretical molecular weights with a narrow molecular weight distribution is observed with photoinduced SR&NI ATRP system.

     

Amyand’s hernias in childhood (a report on 21 patients): a single-centre experience

Purpose

Amyand’s hernia is a very rare type of hernia and is characterised by the presence of a vermiform appendix in the inguinal hernia sac. It may present as a tender inguinal or inguinoscrotal swelling, and is usually misdiagnosed as an irreducible or strangulated inguinal hernia. This is a presentation of our experiences with these unusual hernias.

Methods

This study conducted a retrospective analysis of 21 patients with Amyand’s hernias operated on at our institution between April 2007 and February 2011. The age and sex distributions of the patients, the types of hernias, and preoperative diagnostic tests were evaluated.

Results

Twenty patients were male (95.3 %) and one was female (4.7 %). The median age was 20.3 months (ranging from 2 months to 10 years). In nine patients, the conditions were diagnosed using ultrasonographic (USG) imaging preoperatively. Nine of the 21 patients underwent emergency operations. The 12 remaining patients were operated on after preparations were completed. The operative findings included 12 normal appendixes, five inflamed appendixes, one perforated appendix, and three hernias whose inner hernia sac surface was adhered to the appendix. Nine patients underwent inguinal hernia repair with appendectomy, and 12 patients had hernia repair without an appendectomy. Recurring hernias and appendicitis were not detected in any patients within the follow-up time.

Conclusions

Although a routine appendectomy is not required for a normal-looking appendix, in a case where the inflammatory status of the appendix adheres to the surface of the hernia sac, an appendectomy is required. We believe that USG examination in the diagnosis of Amyand’s hernias can provide additional contribution.     

An unusual case of recurrent endometrial cancer presented with isolated cervical lymph node metastasis

Endometrial cancer (EC) is the most common malignancy of the female genital tract. Lymph node involvement is one of the major prognostic factors. Therefore, pelvic and paraaortic lymph nodes dissection is a part of the surgical management of these patients. Isolated peripheral lymph node metastasis has not been previously reported as a finding of recurrence in EC. We report a 67-year-old woman with recurrent EC presented with an isolated cervical lymph node metastasis (ICLM). Following the combination chemotherapy of doxorubicin, cisplatin and cyclophosphamide, her cervical lymph node was completely regressed. To our knowledge, this is the first case of recurrent EC presented with ICLM. We suggest that for women with EC who had isolated peripheral lymphadenopathies, peripheral lymph node metastasis should be considered as the finding of recurrence in patient with EC.