case report

Pseudomyxoma peritonei (PMP) or Gelatinous Peritoneal Disease is a rare condition that refers to an anatomo-clinical entity characterized by ascites of variable abundance in the peritoneal cavity, viscous or mucinous, associated or not with neoplastic epithelial cells. It predominates in women. Diagnosis is guided by imaging and confirmed by histology. Prognosis is good in case of early management. We report the case of a male diagnosed with Pseudomyxoma peritonei revealed by isolated ascites.

Meigs’ syndrome is a rare condition characterized by the presence of a benign fibroma of the ovary, ascites and pleural effusion. Other benign cysts of the ovary (such as struma ovarii, mucinous cystadenoma, serous cystadenoma and teratomas), leiomyoma of the uterus, and secondary metastatic tumours to ovary if associated with hydro thorax and ascites are referred to as ‘Pseudo-Meigs” syndrome. It very uncommon and diagnosis is made difficult by symptoms that usually mimic disseminated malignancy or tuberculosis. The gold standard treatment is laparotomy and, by definition of the syndrome, after tumor removal, the symptoms resolves and the patients become asymptomatic. We presented an 18 years old girl with giant ovarian serous cystadenoma with associated pseudo-meigs syndrome, successfully managed in a low resources setting.

Puerperal acute uterine inversion is a rare obstetric condition observed as a serious complication during the third stage of labor. Reported as one of the causes of postpartum haemorrhage, it commonly requires quick diagnosis and surgical treatment in order to reduce morbidity and lethality. The authors describe a case of uterine inversion with hypovolemic shock after home birth, brought to Hospital Leonor Mendes de Barros. The purpose of this article is to describe a case of acute uterine inversion and its management and a review of aetiology, predictive and risk factors, diagnosis and treatment.

The lumbar epidural analgesia is commonly used for labour analgesia. The “loss of resistance to air” LORA technique is commonly used for recognition of epidural space. One of the rare complications of this technique is Pneumocephalus (PC). We want to present a case of Pneumocephalus which the mother developed during epidural analgesia in labour. The patient complained of severe headache immediately after attempt at epidural catheter insertion. The symptoms progressively worsened following delivery. A postnatal anaesthetic review was performed and an urgent CT scan of the brain was arranged that showed pneumocephalus. A conservative management pathway was followed with liberal analgesia, oxygen inhalation and keeping the patient mostly in supine position. Her symptoms regressed in severity over the next three days and subsided after one week. We believe that the amount of air used for LORA should be minimized; LORA should not be used after dural puncture and the use of normal saline would alleviate the risk.

Primary umbilical cutaneous endometriosis is a rare umbilical endometrioma that affects women who are within the reproductive age group. It may be associated with infertility and severe dysmenorrhea and can be difficult to diagnosed in an asymptomatic patient. We report a case of a 38-year-old nulliparous with seven years history of infertility and severe dysmenorrhea. Her hormonal profile assay and hysterosalpingogram results were normal while her husband semen analysis was also within normal range. She complained of monthly bleeding from a painful rubbery multilobate cutaneous nodule on the umbilicus of one year duration. She was diagnosed of cutaneous endometriosis. The diagnosis was confirmed histologically and she had surgical excision with good outcome.

Advanced technologies, such as electrochemical impedance spectroscopy (EIS), are a valuable tool which can enhance and simplify the industrial process monitoring if used correctly. State-of-the-art approaches for screening the cell growth of for example yeast during the brewing process still heavily rely on offline methods such as methylene blue or florescence dye-based staining, and/or the usage of flow cytometric measurements. These methods, while being accurate, are very time consuming and require heavy manual effort. Furthermore, the time span needed to obtain the counting result can lead to a time-delayed response signal and can impact the quality of the final product. In recent studies, applications of low-frequency EIS in the α-regime were used for the determination of cell counts and the metabolic state in Saccharomyces cerevisiae. This method has proven to be a reliable tool which has also shown high potential in industrial scale applications. The online biomass monitoring, as well as viable cell count, for feasibility study was performed in-house at Stiegl Brewery in Salzburg/Austria founded in 1492.

Age related macular degeneration is a severe disease of mainly elderly people and leads to central vision loss because of the degeneration of the retinal pigment epithelium [1]. Genetic and environmental factors are responsible for the accumulation of extracellular material and deposit formation near the retinal pigment epithelial (RPE) layer, which leads to loss of photoreceptors and induction of chronic inflammation. The deposits are composed of lipids and proteins including many complement proteins, indicating the involvement of the complement system in the degenerative process and chronic inflammation [2]. So far there is no treatment for the dry form of AMD, except nutritional supplementation with antioxidants and vitamins [3]. Combined with a prolonged lifetime expectation in developed countries, AMD is developing to a social and economic burden. Therefore, there is an urgent need for a treatment of AMD that can delay disease manifestation and progression for several years. 

We present a case of hypocomplementemic urticarial vasculitic syndrome (HUVS) who developed severe renal failure requiring ICU-level care. Our patient is a 66-year-old man who presented with abdominal pain, rash, confusion, oliguria, and shortness of breath. He was found to be in acute renal failure with leukocytosis and elevated lactate. Work-up for infectious, autoimmune, and hematologic malignant diseases was negative. The presence of chronic urticaria, abdominal pain, hypocomplementemia, and leukocytoclastic vasculitis on skin biopsy confirmed the diagnosis of HUVS. He required hemodialysis for renal failure as well as gastrostomy tube placement for nutritional support secondary to the development of mucosal ulcers, a rare finding in HUVS. He recovered with several months of high-dose steroids and hemodialysis. This case highlights the effectiveness of steroids for initial treatment of HUVS, and the relapsing and remitting nature of the disease. Providers should also be aware of the broad range of presenting symptoms such as mucosal lesions that may require nutritional support. Interestingly, unlike many previously reported cases of HUVS, our patient had not yet developed signs and symptoms of systemic lupus erythematosus, which often overlaps with HUVS.

Liver cyst infection in patients with autosomal dominant polycystic kidney disease (ADPKD) is a rare but life-threatening complication. Diagnosis and treatment remain challenging. We report the case of a 64-year-old kidney transplant patient presenting with fever and abdominal pain. The diagnosis of liver cyst infection was made by positron emission tomography scan (PET/CT). Moreover, the course of our patient illustrates the interest of subsequent PET/CT during follow-up as our patient failed on antibiotherapy and required liver cyst drainage.

Brucellosis is a zoonotic infection that is endemic in some Mediterranean countries, North Africa and the Middle East. Brucella is a rare cause of peritonitis in Peritoneal Dialysis (PD) population and in non-dialysis patients alike. We report here a challenging case of PD-related Brucella peritonitis in a 45-year-old Saudi male with late peritonitis that delivered some key learning throughout its course from presentation, diagnosis and treatment to catheter salvage attempts so as to circumvent PD failure. We provide an in-depth review of limited published literature on PD-related Brucella peritonitis (seven cases, and present case) and summarized the data on key clinical characteristics, management and PD technique outcome to benefit nephrologists when encountered with this rare presentation.

Prednisone-sensitive hypocomplementemia, renal insufficiency, and kidney biopsy demonstrating severe tubulointerstitial nephritis (TIN), storiform fibrosis, and tubulointerstitial immune deposits are typical of IgG4-related tubulointerstitial nephritis and hypocomplementemic interstitial nephritis. A diagnosis of hypocomplementemic interstitial nephritis requires clinical and pathologic exclusion of IgG4-related tubulointerstitial nephritis. We describe a patient with hypocomplementemic interstitial nephritis who did not develop diagnostic features of IgG4 related disease (RD) over 2-year follow-up. We conclude that hypocomplementemic interstitial nephritis could be on a biologic spectrum with IgG4-related disease, but not all cases will develop the abundance of IgG4-positive plasma cells, systemic manifestations, or elevated immunoglobulin levels characteristic of IgG4-RD.

Anti-glomerular basement membrane (anti-GBM) disease is an uncommon autoimmune disease that classically presents as an aggressive necrotizing and crescentic glomerulonephritis (CG), with or without pulmonary hemorrhage, and typically does not relapse. The pathologic hallmark is linear staining of GBM for Immunoglobulin G (IgG) which binds to the alpha-3 chain of type IV collagen. Recent reports have noted the occurrence of anti-GBM disease with atypical clinical presentations. We describe a case of a 22-year-old female presenting an anti-GBM disease with typical histological features with a singular clinical presentation with lower limb edema, elevated serum creatinine and nephrotic range proteinuria. Renal biopsy showed signs of chronicity and direct immunofluorescence microscopy demonstrated diffuse, intense linear positivity for IgG. All serologic tests were negative, including anti-GBM antibodies. Hence, a diagnosis of atypical anti-GBM disease was made. The patient was treated initially with mycophenolate mofetil and corticosteroids. After 3 months she began to aggravate renal function so mycophenolate of mofetil was discontinued and a low dose cyclophosphamide was initiated.

We report a case involving infection with Strongyloides stercoralis found in the sputum of a 66-year-old male patient who had a medical history of nephrotic syndrome and was treated with methylprednisolone and monthly intravenous cyclophosphamide therapy 3 months previously. This patient presented with stubborn pulmonary symptoms and signs, which was the mechanical destruction caused by larval migration. We found strongyloides in his sputum that provided diagnostic proof.

Background: Knowledge of pulmonary complications (PCs) in children after hematopoetic stem cell transplantation (allo-HSCT) is limited; most data are from adult studies. Case: We describe a 8 year old girl with high risk acute myeloid leukemia who developed graft versus host disease (GVHD) on Day 20, Cytomegalovirus (CMV) pneumonia on Day 50 and Cryptogenic organizing pneumonia (COP) on Day 170 after allo-HSCT. Discussion: Cryptogenic organizing pneumonia is a rare noninfectious PCs that can be idiopathic or have several risk factors as a secondary causes, such as viral respiratory infections, drugs, GVHD and allo-HSCT. Viral respiratory infections and alloimmune lung syndromes have been reported in a few patients who have undergone transplantation. Conclusion: Transplant physicians should be kept in mind for the development of alloimmune lung syndrome in the form of COP following CMV pneumonia in patients after allo- HSCT

An enquiry into the lack of attention awarded to serotonin antagonism in the treatment of arterial thrombosis revealed that the mode of action of serotonin and its platelet receptor antagonists was an action upon thrombus growth, and not, as with other anti-platelet drugs upon the initiation of thrombosis. This lack of effect could explain why this approach has been considered not to be effective. However under conditions of arterial stenosis in which there is platelet activation by increased shear stress, and during the growth phase of arterial thrombi, serotonin 5HT2A antagonism has been demonstrated to have great potentcy in dispersing thrombotic obstruction to blood flow. This mode of action, the lack of participation of serotonin in haemostasis, and the absence of serotonin in wounds accounts for the proven lack of effect of effect of pure specific 5HT2A antagonists (i.e., not those with other actions) on operative bleeding and skin bleeding times. This lack of effect on haemostasis solves the dosing problem encountered with other anti-thrombotic drugs, with which drug concentration cannot be controlled with single fixed doses, leading to the association between increased anti-thrombotic efficacy and increased bleeding complications. Thus 5HT2A antagonism appears to be the preferred approach, from the point of view of safety and lack of bleeding risk; this consideration applies particularly to thrombosis therapy in the context of traumatic accidents, surgical operations and invasive procedures such as angioplasty.

Background: Invasive fungal infections cause significant morbidity and mortality in patients with hematologic malignancies and in recipients of hematopoietic stem cell transplantation. Case: We report a patient with relapsed acute myeloid leukemia who developed disseminated Fusarium infection during the neutropenic period following the salvage cycle of chemotherapy given at King Fahad specialist Hospital in Dammam, Saudi Arabia. The invasive fungal infection was successfully managed with a combination of voriconazole and liposomal amphotericin-B. Discussion: Fusarium species can cause invasive infections that may become disseminated and life-threatening in patients with acute myeloid leukemia. Conclusion: Combined antifungal therapy and recovery of neutrophil count are essential to control invasive Fusarium infections

Background: Pure red cell aplasia is characterized by anemia, reticulocytopenia and diminished bone marrow erythroid precursors. It has multifactorial etiology and consequently several therapeutic interventions. Case: In August 2017, a young patient was diagnosed to have pure red cell aplasia. She was given immunosuppressive therapy for approximately two months but this treatment was stopped due to intolerance. Later on she developed herpes zoster infection that was treated with valacyclovir. Subsequently, it was noted that the patient became blood transfusion independent due to normalization of her hemoglobin and regeneration of the erythroid precursors in the bone marrow. Discussion: Varicella zoster virus behaves differently from other members of the herpes group of viruses such as cytomegalovirus and Epstein-Barr virus. Two retrospective studies, performed in patients with malignant hematological disorders and bone marrow failure, have shown that infection with the virus may cause stimulation of the three cell lines in the bone marrow and superior overall survival. Conclusion: The outcome of the patient presented confirms the findings of the two studies showing long-term beneficial effects of varicella zoster virus infections in immunocompromised individuals.

Introduction: Erdheim-Chester disease (ECD) is a rare and difficult-to-treat non-Langerhans cell histiocytosis characterized by the excessive production and accumulation of histiocytes. This study reports a case of ECD, emphasizing both its diagnosis, assessment and treatment of the pain associated with the disease. Case Report: Six years ago, a 39-year-old male patient presented with generalized pain of moderate intensity in the lower limbs that involved periods of greater intensity associated with ambulation. The diagnosis of histiocytosis associated with panhypopituitarism and adrenal insufficiency was proposed. For a specific diagnosis, a bone lesion biopsy was performed, revealing the presence of histiocytic proliferation that was CD1 negative, S100 protein positive, and CD68 negative. Therefore, the diagnosis of non-Langerhans histiocytosis known as ECD was confirmed. During the two years that followed, the patient presented with severe bone pain, particularly in the lower limbs and cranial vault, and the pain subsided to a certain extent with the use of tramadol and paracetamol. Because of the pain, the patient was unable to walk and became bedridden As the patient remained in severe pain, even after the administration of morphine, the opioid was changed from morphine (60mg/day) to oxycodone (30mg/day) for a convenient dosing schedule; furthermore, the oxycodone dosage was scheduled to increase to 40mg/day that same week. The patient experienced significant pain reduction, requiring rescue analgesia only once or twice a week. Conclusion: To the best of our knowledge, this is the first case report on the characterization and treatment of pain specific to ECD, and we highlight that the patient had a good response to treatment.

Celiac disease affects 1% of the world population; however it is under diagnosed in UAE. The disease has many clinical manifestations, ranging from severe malabsorption to minimally symptomatic or non-symptomatic presentation. Hypocalcaemia is a common finding in celiac disease and could be the only presentation of the disease; however hypercalcemia has been previously reported in patients with celiac disease either due to primary hyperparathyroidism or tertiary hyperparathyroidism due to prolonged hypocalcaemia. A normal calcium level on the other hand in patients with untreated celiac disease who also have primary hyperparathyroidism can be due to interplay of these two conditions and may delay the diagnosis of primary Hyperparathyroidism. We report the very first case from our practice in UAE with untreated celiac disease and normal calcium level at presentation, where a diagnosis of primary hyperparathyroidism was not entertained initially. Patient went on gluten free diet which then caused normalization of intestinal abnormalities and likely calcium absorption manifesting as hypercalcemia on subsequent labs. This led to further work up and finally the diagnosis of Primary hyperparathyroidism due to parathyroid adenoma.

Familial adenomatous polyposis is an autosomal dominant syndrome of variable penetration and constitutes the second frequent inherited syndrome enunciating the emergence of a colorectal carcinoma. The syndrome is accompanied by exemplification of defective adenomatous polyposis coli (APC) gene located upon chromosome 5q21 with a prototypic denomination of colonic adenomatous polyps usually exceeding a > 100. Incriminated individuals develop innumerable colonic and rectal polyps, particularly during early teenage years and are accompanied by an almost 100% possible emergence of colorectal carcinoma within 40 years in untreated subjects [1]. Prophylactic colectomy is advisable to substantially reduce possible occurrence of colorectal carcinoma. Familial adenomatous polyposis is concurrent with associated neoplasms such as gastric or duodenal cancer, hepatoblastoma or desmoid tumour along with a probable emergence of extra-colonic carcinomas [1,2].