Purkayastha K, Seth R, Amitabh S, Xess I, Kapil A and Sreenivas V
Background: Infections are major cause of morbidity and mortality in children receiving cancer chemotherapy particularly when they are neutropenic, mainly due to immune deficiency. Between 48-60% neutropenic patients with fever have an underlying infection which can often be life threatening. Before putting the child on empiric antimicrobial regimes for FN, it is essential to know the spectrum of locally prevalent pathogens and their susceptibility patterns. Often these children don’t manifest fever even in presence of infection and fever may be present in neutropenic patients receiving chemotherapy even in the absence of infection. Present diagnostic tools available for diagnoses in FN are often not so robust and do not differentiate between various classes of organisms causing these infections. Procedure: Blood culture is time consuming and negative blood culture does not exclude bacteremia, which leads to the empirical use of broad-spectrum antibiotic treatment in pediatric patients with neutropenia, even where signs of infection are absent. We propose to evaluate the role of PCT, as a sensitive marker to evaluate pediatric oncology patients presenting with FN. Results: Blood-culture was positive in 18.05% of the patients, with majority of patients having gram-negative bacterial infections. On comparison with the focus of infection, high PCT and CRP values were obtained in patients with pulmonary infection than in extra-pulmonary infections. In our study the sensitivity of PCT was high upto 73.3% at a cut-off of ≥0.25 ng/ml for ruling out bacteremia, when compared to blood culture and CRP in our patients. Conclusion: The PCT value is certainly helpful in guiding the physicians in clinical decisions and thus the better approach towards the management of pediatrics oncology patients with FN.
Elousrouti LT, Erreggad FZ, Ebang GA, Efared B, Sory I, Hammas N, Chbani L, Elfatemi H and Harmouch T
Introduction: Melanotic neurofibromas are rare tumours, derived from peripheral nerve sheath, whose originality consists in the presence of melanic pigment. The clinical and histological diagnosis is often difficult to make requiring the immunohistochemical exam to make the difference between melanotic neurofibroma and the other pigmented tumors. Melanotic neurofibroma has a good prognosis and the malignization is rare. Case Report: A 22 year-old man presented since childhood a left occipital tumor, it had a firm consistency and pigmented color. CT objectified isodense occipital mass, which enhanced heterogeneously after contrast product. Lack of bone loss beside. Complete surgical excision was performed. Histological and immunohistochemical analysis led to the diagnosis of melanotic neurofibroma. Discussion: Pigmented neurofibroma is a rare variant of neurofibroma showing melanin production and constitute less than 1% of all cases. It can occur on their own or be associated with neurofibromatosis. They must be distinguished from classical neurofibromas when pigmentation occurs in the latter. Melanotic neurofibromas usually appear in the second or third decade of life and rarely in childhood. The clinical diagnosis is difficult to establish requiring the histopathological examination to differentiate between the melanotic neurofibroma and other pigmented tumours. It can mimicking a giant naevus or a neurocristic cutaneous hamartoma. These are the two main differential diagnoses among children. Among adults, the main difficulty is to distinguish melanotic neurofibroma from pigmented dermatofibrosarcoma. The elective treatment is surgical, represented by the complete excision of the tumor. Conclusion: Pigmented neurofibroma is a unique subtype of neurofibroma which is rare and contains melanin producing cells. The histopathological features with unique pattern of melanogenesis, ultrastructural findings and immunohistochemistry will enable us to diagnose this entity and differentiate it from other pigmented tumours of skin.
Tahir Majeed, Aadil Bashir Rather and Ghulam Nabi Dhobi
We are reporting a case of PUO in a young male with no underlying co-morbities, which was managed as plasmodium malaria due to false positive card test initially but later found to be due to a gram negative bacteria Achromobacter denitrificans (A. denitrificans), which was sensitive to cephalosporin. The patient was treated with ceftriaxone and got cured. To our knowledge A. denitrificans bacteremia with sensitivity to cephalosporin’s in a immunocompetent person with no underlying illness, hospitalization or in person who has never underwent any medical procedure has never been described in medical literature before.
Kassab P, Ilias EJ, Castro OAP, Nara F, Thuler FR, Freitas Jr W, Mancini FC, da Silva PF, Jukemura J and Malheiros CAM
Duodenal Brünner gland hamartomas are usually asymptomatic and benign. They are relatively rare with around 300 cases described in the literature. In general the patients present mild symptoms, sometimes with silent bleeding leading the patient to anemia. In other cases they may present an acute episode of bleeding or obstruction1. In cases of bigger tumors, occupying almost the total duodenal lumen, the endoscopic treatment is difficult.
Chao-Yen Ho, Yi-Chia Lin, Ta-Nien Lu and Thomas IS Hwang
Renal artery pseudoaneurysm (RAP) is a rare complication after partial nephrectomy. Here we present a 63-yearold male who had an incidental finding of a right renal cancer, with stage T1bN0M0. The patient underwent a robotassisted laparoscopic partial nephrectomy and presented with a giant RAP since one month after the operation. He was successfully treated by an angiography with selective embolization.
Nam H Chan, Janis E Blair, Sydney A Westphal, Leila K Tehrani and Maria Teresa A Seville
Fluconazole, voriconazole, and itraconazole may potentiate the hypercalcemic effect of other medications, although to date, triazoles alone have not been linked to hypercalcemia. We describe a case of new-onset hypercalcemia in the setting of high-dose fluconazole that resolved after changing the antifungal regimen. We reviewed our institutional database to assess the frequency of moderate or severe hypercalcemia (serum calcium ≥12 mg/dL) among patients with coccidioidomycosis treated with a triazole. We identified 2,133 patients, seen from January 1, 2005, through December 20, 2012, with a diagnosis of coccidioidomycosis. Twenty-three patients (1%) had subsequent hypercalcemia; of these, 20 patients (87%) had moderate or severe hypercalcemia. Nine of the 20 patients (45%) with moderate or severe hypercalcemia were taking triazoles, and most had comorbid conditions that were risk factors for hypercalcemia (tertiary hyperparathyroidism, n=4; multiple myeloma, n=2; adrenal insufficiency, n=1). The effect of fluconazole on calcium metabolism possibly is small or not clinically significant until the dose is high. Fluconazole may also contribute to hypercalcemia in patients with underlying hyperparathyroidism because of the loss of calcium’s inhibitory effect on parathyroid hormone secretion. In summary, moderate or severe hypercalcemia is uncommon in coccidioidomycosis and rare in those treated with triazoles. High-dose fluconazole may be associated with symptomatic hypercalcemia, especially in patients with predisposing comorbid conditions.
Bolier EA, Sierink JC and Den Boer FC
Although a Meckel’s Diverticulum is the most common congenital malformation of the gastro-intestinal tract, it mostly is discovered by chance and remains asymptomatic. However, a Meckel’s Diverticulum can cause multiple complications with an incidence range of 4-16 per cent. In this case report, we present a patient at the emergency ward with symptoms of acute abdominal pain in the epigastric region. Laboratory findings showed elevated infection parameters and an abdominal computerized tomography scan revealed fat infiltration around a structure suspected to be a Meckel’s Diverticulum. At emergency laparotomy, we found a fully necrotic giant Meckel’s Diverticulum. As presented in our case, clinical decision making at the emergency ward remains challenging. Therefore, multiple factors should be considered and may lead to decision for an emergency laparotomy as a diagnostic and therapeutic tool.
Vincent Fontaine, Pierre-Luc Villeneuve, Etienne L Belzile and Mélissa Laflamme
Background: Bone graft is frequently used during orthopaedic procedures. Multiple complications are associated with bone graft harvest from the iliac crest. Structural bone graft harvest can lead to the development of a rare complication of a hernia through the iliac deficit. Methods: We report two cases of transiliac herniation following a structural bone graft harvest. The first case was a 71 years-old woman who initially underwent a foot fusion with a ipsilateral bone graft harvest. She underwent three surgical hernia repairs using a mesh sutured to the adjacent muscles to cover the defect. At the last follow-up, the patient presented with a third recurrence of the hernia. The second case is a 79 years-old female who initially underwent a total hip replacement associated with an ipsilateral bone graft harvest. Twenty-four years later, she presented with a transiliac hernia. The patient underwent surgery and in this case, the mesh was sutured to bone with a corkscrew anchor. At the last follow-up, the patient did not present any recurrence of the hernia. Conclusion: The use of a corkscrew anchor to fix the mesh rigidly through the iliac bone seems to offer a fast recovery with minimal complications compared to the standard technique where the mesh is loosely fix to the adjacent muscles. This hernia repair technique seems to offer better results and less recurrence. A study comparing the two techniques with more patients involved would help confirm our findings.
Kunihiro Nishimura, Shuho Tanaka, Yasuhiko Takahashi, Yasue Uchida, Tooru Tanigawa, Hiromi Ueda and Tetsuya Ogawa
Background: Amyloidosis is the general term for deposition of fibrous abnormal protein aggregates (amyloid) in organs or tissues that leads to dysfunction of the affected organs. Treatment and follow-up of localized amyloidosis are particularly difficult because its pathogenesis is still unclear. Localized amyloidosis of the head and neck is rare and that of the nose is rarer still. Here, we report a case of localized amyloidosis of the nasal cavity, which affected an area larger than those in previous reports. Case presentation: A 60-year-old woman presented with the chief complaints of headache, proptosis and nasal congestion. There was a lack of response to chemotherapy and endoscopic sinus surgery was performed. As of 18 months after surgery, subjective symptoms have been alleviated and are consistent with the objective findings. There have been no signs of recurrence. Conclusions: Cases of localized amyloidosis of the nasal cavity are rare, and standard treatment has not yet been established. In this case, proactive surgical intervention was successful for this condition.
Ramesh Gowda and Vishwajit Hegde
Transient neonatal non-ketotic hyperglycinaemia (NKH) is a rare metabolic disorder in neonates which is indistinguishable from its classic form during the neonatal period. To our knowledge, only a few cases (about 14) of transient neonatal hyperglycinaemia have been reported. We report a 2 day old neonate who presented with clinical (seizure) and biochemical (raised CSF/Plasma glycine ratio) features of neonatal NKH. EEG on day 2 of life showed burst suppression pattern; hiccups noted during this EEG did not show any cortical origin except for concurrent artefacts. Biochemical features normalised by 4 weeks of age; EEG was encephalopathic at 4 and 17 week (current age). Infant currently has truncal hypotonia and developmental delay. There is no case report describing concurrent hiccups during EEG and timing of encephalopathy appearance on EEG, which in our case is 3-4 week of age.
Sven Pannach, Stefan Brückner, Florian Ehehalt and Jan Beyer Westendorf
We present the case of a 63 year old male who developed recurrent massive gastric varicose vein bleedingepisodes years after necrotising pancreatitis. Endoscopy revealed extensive gastric but no esophageal varices. Ultrasonographic findings included an enlarged spleen, normal portal vein diameter and flow and a regular liver texture without signs of cirrhosis. CT scan demonstrated massive perigastric and splenic collateral veins due to chronic splenic vein occlusion. Left-sided portal hypertension was diagnosed and the patient treated with splenectomy. In an endoscopic 2-year follow-up, gastric varicose veins have completely regressed and the patient has remained free of recurrent GI bleeding complications.
Prashan Buddhika Illeperuma and Ananda Jayanaga
Thymoma is a rare mediastinal tumour that is often (40%) accompanied by different paraneoplastic syndromes and glomerulonephritis is one of the recognized entities. Minimal change disease is the most common paraneoplastic glomerulonephritis associated with thymoma, followed by membranous nephropathy, focal segmental glomerulosclerosis, rapidly proliferative glomerulonephritis and lupus nephritis. Literature review illustrates that most of the thymoma associated nephropathy cases were reported in association with more malignant thymic tumors like WHO grade B and C tumours. Herein we report a rare case of focal segmental glomerulosclerosis in a patient with grade A benign thymoma, a combination which has never been reported previously. She had poor response to corticosteroids and died due to neutropenic sepsis following azathioprine therapy.
Shakeel Rahman, Faisal Salim and Asit Khandwala
We presentented a case of septic arthritis of the hand and wrist in an 8 year old girl due to group A streptococcus pyogenes. Important differentials are discussed along with diagnostic strategies. An unusual feature of this case is the lack of involvement of the radiocarpal joint despite involvement of the mid carpal and carpometacarpal joints.
Tahiri Elousrouti L, Erragad FZ, Jayi S, Hammas N, Harmouch T, Chbani L and Elfatemi H
Primary fallopian tube cancer is the rarest cancer of female genital tract. In fact, it represents less than 1% of all the gynaecological and breast cancers. Adenocarcinoma is the most common histological type. This paper revels two cases of primary fallopian tube adenocarcinoma. They are diagnosed in pathology department. There are two patients to 53 years and 62 years respectively, with no significant personal or family history. They are consulted for chronic pelvic pain with pelvic mass. The clinical assessment revealed an abdomino-pelvic mass of hard consistency. We review the epidemiological, clinical, anatomo-pathological, therapeutic and prognosis specificities of this tumor. This cancer occurs in patients in their sixties, in a context of infertility, pauci-parity, chronic tubal infection or a genetic predisposition. The pelvic pain and the perception of a pelvic mass are the most frequent clinical manifestations. Paraclinical examinations (pelvic ultrasound, CT, MRI and serum markers) are not specific and diagnosis is often made peroperatively or postoperatively by anatomo-pathological examination. In fact, the tubal carcinoma is often confused with his ovarian counterpart which have several similarities. The prognosis is relatively dark, but still better than ovarian carcinoma. It mainly depends on the stage of disease and quality of surgical resection.
Edvard Ehler, Leona Formanova, Alena Melekova, Petra Mandysova and Martin Valis
Background: Miller Fisher syndrome is a well-known disorder that belongs to a group of conditions with a high level of antibodies against ganglioside GQ1b. It is characterized by acute onset of ataxia, areflexia, and ophthalmoplegia. In cases of acute-onset ophthalmoplegia without ataxia, the diagnosis is more difficult; therefore, it is frequently delayed. Case Report: We report one such case of rapid onset of internal ophthalmoplegia, followed by external ophthalmoplegia. The diagnostic process was challenging, but eventually, a high level of antibodies against GQ1b confirmed the diagnosis. Because the clinical and electrophysiological findings were limited only to the presence of anti-GQ1b antibodies, a combination with a generalized form of Guillain-Barre syndrome could be excluded. Conclusion: Consequently, the patient was not treated with immunotherapy. At 4 months, the patient had made a good recovery.
Hongtao Hu and Hua Shui
Systemic lupus erythematosus with retroperitoneal fibrosis, a rare disease with the mechanism obscure, is considered an autoimmune disorder at present. Computed tomography and Magnetic resonance imaging are the two tests of choice in diagnosis and follow-up. A combination of corticosteroids and immunosuppressive drugs is usually applied for therapy. Laparoscopic ureterolysis, or robotic ureterolysis, if available, is the technique of choice to relieve obstruction.
Chenbao Wang, Yanan Gu and Lei He
A right impacted mandibular third molar (IMTM) accidently displaced into the parapharyngeal space was dislodged successfully in only 5 minutes, with no sequel. Cone-beam computed tomography (CBCT) images were used to direct the removal. Five minutes is the shortest time having been reported. Considering that the event happens rarely, this report could add another case, and provide further reference to the dentists.
Diedert Luc De Paep, Vincent De Coninck, Nele Van De Winkel and Dirk Michielsen
Introduction: Both totally extraperitoneal herniorrhaphy (TEP) and spermatic vein embolization are minimal invasive techniques gaining in popularity in the treatment of inguinal hernia and varicocele respectively. A previous spermatic vein embolization makes a TEP more difficult because of fibrotic changes of the spermatic vein. This is to our knowledge the first case report describing these difficulties and changes after spermatic vein embolization. Case Presentation: We report the case of a 37 year old male who presented with an uncomplicated left sided inguinal hernia and ipsilateral varicocele. He first underwent a percutaneous left spermatic vein embolization for a grade 3 varicocele and later a TEP for the inguinal hernia. Reduction of the peritoneum and proper mesh placement were complicated due to fibrotic changes of the spermatic vein. Due to the dissection and resection of the embolized spermatic vein there was minor blood loss, increased operating room time and postoperative pain was more then average. Conclusions: In case of a concomitant inguinal hernia and ipsilateral varicocele requiring embolization we propose to treat these diseases concomitantly or first treat the inguinal hernia and embolising the varicocele after repair of the hernia.
Ciro Manzo
Polymyalgia rheumatica (PMR) is considered the commonest rheumatic inflammatory disease in the elderly. The relationship between PMR and cancer is still complex. The possibility that PMR can represent a paraneoplastic syndrome has been more and more descripted. A lack of response to corticosteroid therapy is considered the principal element for looking for a paraneoplastic syndrome. Gastric gastrin-secreting tumor represents an uncommon gastric tumor, especially in the elderly. We describe the clinical case of a patient in which PMR associated with a mild macrocytic anemia was the key for diagnosis of a gastric gastrin-secreting tumor. Endoscopic removal of neoplastic polyps and an octreotide long-lasting therapy caused the prompt and permanent disappearance of PMR manifestations. According to our knowledge, it’s the first time that this association is descripted.
Masayuki Shibata, Toru Matsui, Hirotoshi Ishiwatari, Hiroyuki Matsubayashi, Takahiro Tsushima, Keiko Sasaki and Hiroyuki Ono
A 66-year-old man was referred to our hospital due to carbohydrate antigen 19-9 (CA19-9) elevation. Further examination revealed no obvious malignancy, but abdominal enhanced computed tomography (CT) showed soft tissue density around the celiac axis and superior mesenteric artery (SMA). Although pancreatic cancer was considered as a possible diagnosis, there was no clear evidence within the pancreas itself. Furthermore the patient was observed without intervention. Another abdominal enhanced CT performed 10 months later showed a dilated pancreatic duct in the tail of the pancreas, along with a low density area measuring 10 mm in the body of the pancreas. Endoscopic ultrasound guided-fine needle aspiration (EUSFNA) led to a diagnosis of pancreatic cancer. In the present case, the tumor in the pancreas was inconspicuous, but it might be characterized by extensive extrapancreatic invasion.