Clin. Pract., Volume 7, Issue 4 (September 2017) – 12 articles

The Schnitzler syndrome (SCS) is a rare, late-onset acquired autoinflammatory syndrome often underdiagnosed. The diagnosis is based on the Lipsker and recently on validated Strasbourg diagnostic criteria (chronic urticarial rash, monoclonal gammopathy, intermittent fever, arthritis, arthralgia, bone involvement, hepatomegaly, splenomegaly, lymphadenopathy, dermal infiltration of neutrophils and laboratory markers of inflammation). Conventional therapies including anti-histamines, anti-inflammatory drugs, corticosteroids and immunosuppressive drugs that are usually ineffective. Recently the gold standard therapy of SCS are considered IL-1 blocking agents as anakinra, canakinumab, rilonacept that led to a significant control of clinical symptoms, even if a relapse could appear at suspension of the treatment. We report a case of a 63-year-old man with a recent diagnosis of SCS - after 6 years of symptoms of disease - refractory to several conventional immunosuppressive therapies and treated with anakinra, with sustained remission of clinic manifestations during treatment at 24 months of follow up. Full article

Hemispherectomy is an established neurosurgical procedure for unilateral refractory epilepsy . Even though the surgical approach has evolved greatly, prevention of catastrophic intraoperative bleeding is a challenge. It is important that surgeons know the abnormal blood vessel before surgery. Herein, we report our experience with two patients in whom computed tomographic angiography (CTA) was used in the preoperative evaluation for hemispherectomy. CTA allowed for precise anatomical delineation of the hemispheric vascular abnormalities. Preoperative CTA showed that the specific cerebral arteries and their branches ipsilateral to the lesion were slender. Elaborate preoperative planning for the surgical approach helped prevent catastrophic intraoperative bleeding. Favorable outcomes were achieved in both children. CTA appears to confer a considerable advantage in the preoperative vascular and anatomical delineation in patients scheduled for hemispherectomy. To our knowledge, this is the first report about the application of CTA for hemispherectomy preoperative planning. Full article

Manual lymph drainage has become the mainstay in the treatment of lymphedema for decades now. Five evolving variants have been described by Godoy & Godoy over the years: i) manual lymph drainage using rollers; ii) self-applied manual lymph drainage using rollers; iii) manual lymph drainage using the hands (manual lymphatic therapy); iv) mechanical lymphatic therapy using the RAGodoy® device; and v) lymphatic therapy using cervical stimulation in general lymphatic treatment. After breast cancer treatment using adapted technique with intermittent compression therapy. Lymphoscintigraphy, volumetry and bioimpedance were employed to analyze such treatment techniques applied to the upper and lower extremities. These treatment and evaluation topics are described in this brief report. Full article

Dermatomyositis with subcutaneous edema is a rare process with few reported cases. We report a 63-year-old with lung cancer who presented with an erythematous skin rash and was found to have biopsyproven dermatomyositis. Her course was complicated by generalized edema, myalgias, muscle weakness, dysphagia, and laryngeal edema. The edema was severe and caused respiratory distress requiring intubation. The patient underwent therapy with high-dose glucocorticoids and intravenous immunoglobulin but failed treatment. Altogether, she presented as an extreme case and rare variant of dermatomyositis, known as edematous dermatomyositis. Diagnostic and treatment guidelines do not account for this variant and literature pertaining to edematous dermatomyositis is sparse. Moreover, this disease was a paraneoplastic manifestation of her small cell lung cancer, which is rarely observed. There are no cases reporting edematous dermatomyositis as a paraneoplastic manifestation of small cell lung cancer, and we highlight the high rate of morbidity and mortality in such patients. Full article

Oral vegetable granuloma represents an inflammatory lesion of foreign body origin resulting from the implantation of vegetable matter. Controversy regarding its pathogenesis is reflected by the various terminologies used to describe the lesion. Its diverse clinical presentations are due to variations in the antigenic potential of the vegetable material and the host response. As the diagnosis is solely histopathological, it is critical to differentiate vegetable granuloma from other oral granulomatous lesions like tuberculosis, sarcoidosis and Wegner’s granulomatosis. Here, we report six cases with the varied clinicopathological presentation of hyaline ring granulomas in association with different pathological lesions. Full article

Osteoporosis characterized by low bone mass/osteopenia can be identified using radiomorphometric indices in routine panoramic radiographs. This study estimates the prevalence of osteopenia in 50-80 years age group, using panoramic mandibular index (PMI), mental index (MI) and mandibular cortical index (MCI). PMI, MI and MCI were applied on 36 panoramic radiographs; MI and MCI were compared with PMI. The prevalence of osteopenia was 11.1% with PMI and 44.4% with MCI. Using MI, the prevalence was 2.8% and 33.3% with 3mm and 4.77mm threshold respectively. The prevalence of osteopenia detected was highest using MCI (44.4%). Considering PMI as gold standard, MI with 4.77 mm threshold showed better agreement with PMI. Full article

Bacterial endocarditis is an infection of the endothelial surface of the heart, including heart valves and is potentially life threatening. Antibiotic prophylaxis is indicated prior to invasive procedures in patients with certain high-risk cardiac conditions and thorough history needs to be elicited prior to performing such procedures. Herein, we present a case of splenorenal infarction secondary to septic emboli in a 42-year-old patient with prosthetic aortic valve who underwent a dental procedure without endocarditis prophylaxis. Full article

In oto-rhino-laryngology, cases of submandibuillar sialolithiasis are common. Submandibular sialoadentis with sialolith may cause severe complications such as deep neck abscess and sepsis. We introduce a rare case of a cervical fistula with abscess caused by submandibular sialolith. The patient had diabetes. We performed drainage of the left submandibular gland that included a Wharton duct stone and abscess by an external skin incision approach. Submandibular sialoadentis due to sialolith would likely progress to neck abscess and the formation of a neck skin fistula; moreover, the condition can be worsen by the coexistence of diabetes. This neck abscess with skin fistula could have caused potentially fatal complications such a carotid artery rupture or sepsis. In such cases the infected source should be carefully removed as soon as possible. Full article

Mucoepidermoid carcinomas (MEC) of minor salivary gland origin are rare in children and adolescents. A 17-year young male, reported with a painless swelling in the left posterior area of the hard palate since 6 months. Several clinical, radiographic, and histopathological investigations were carried out. Incisional biopsy confirmed the diagnosis of lesion as low grade MEC of the palate following which a wide surgical excision with adjacent free margins was carried out. This case report highlights the need for considering malignant lesions in the differential diagnosis of palatal swellings even in younger age groups. Full article

Hemoglobin A1c (HbA1c) is affected by the red blood cell (RBC) lifespan. The association of HbA1c with RBC indices in pregnant women is not widely investigated. In this study, we investigated the association between HbA1c and Hb/RBCs count and RBCs indices; specifically, Hb/RBCs count and indices in non- diabetic pregnant women. Across-sectional study was carried- out at the antenatal care of Saad Abu Elela Hospital, Khartoum, Sudan. Obstetrics history was gathered using questionnaire and body mass index was calculated. Fasting blood sugar, at one hour, at two hours post prandial and HbA1c were investigated. Complete blood count parameters of RBCs count was performed, including, hematocrit, Hb, RBCs indices of MCV, MCH, and MCHC. One hundred twenty three women were enrolled. The mean age of the participants was 28±5.6 years and a mean body mass index was 27.65±6.8 k/m². There was significant positive correlation between HbA1c and Hb (r=0.174, P=0.037), Hct (r=0.174, P=0.037), and MCHC (r=0.180, P=0.031). A negative correlation between HbA1c and the platelet index PDW (r= –0.198, P=0.017) was documented. The rest of the other factors showed no correlation with HbA1c. In linear regression analysis, HbA1c correlated positively with Hb (P=0.044) and Hct (P=0.047). The present study shows a significant positive correlation between HbA1c value with Hb, Hct, and MCHC. No significant correlation between HbA1c and other RBCs parameters was observed. Full article

The bidirectional communication between the central nervous system and gut microbiota, referred to as the gut-brain-axis, has been of significant interest in recent years. Increasing evidence has associated gut microbiota to both gastrointestinal and extragastrointestinal diseases. Dysbiosis and inflammation of the gut have been linked to causing several mental illnesses including anxiety and depression, which are prevalent in society today. Probiotics have the ability to restore normal microbial balance, and therefore have a potential role in the treatment and prevention of anxiety and depression. This review aims to discuss the development of the gut microbiota, the linkage of dysbiosis to anxiety and depression, and possible applications of probiotics to reduce symptoms. Full article

Cryptococcal meningoencephalitis is a leading of morbidity and mortality in immunocompromised individuals worldwide. However, there are few documented cases in immunocompetent patients. We present a rare case of disseminated Cryptococcus with progression to meningoencephalitis in an immunocompetent patient, with a possible atypical presentation. Magnetic resonance imaging of the brain and electroencephalogram to rule out brain metastasis were negative. Lumbar puncture resulted positive for Cryptococcus neoformans antigen at titers of 1:2048 and a detailed history later revealed occupational exposure to bird dander by cleaning floors and cages. Diagnosis is challenging, with delays often resulting in increased morbidity and mortality. Cerebrospinal fluid and serum Cryptococcus antigen play a key role in both diagnosis and determining treatment efficacy. Furthermore, current treatment guidelines are used for immunocompromised individuals. Due to the significant side effects of these medications, further research is needed to determine the optimal treatment duration for immunocompetent patients to minimize the need for unnecessary therapy. Full article