Vitamin B12 deficiency-induced pseudothrombotic microangiopathy without macrocytosis presenting with acute renal failure: a case report

Vitamin B12 deficiency-induced thrombotic microangiopathy, known as pseudothrombotic microangiopathy, is a rare condition which resembles the clinical features of thrombotic thrombocytopenic purpura but requires ...

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Swept source optical coherence tomography and optical coherence tomography angiography in pediatric enhanced S-cone syndrome: a case report

Enhanced S-cone syndrome is an autosomal recessive retinal dystrophy related to a defect in a nuclear receptor gene (NR2E3) that leads to alteration in cells development from rod to S-cone. This retinal dystrophy...

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Penile cutaneous horn: still an enigma

Ajay Aggarwal<br />Sep 10, 2018; 2018:bcr-2018-225930-bcr-2018-225930<br />Images in...

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Haematometrocolpos due to transverse vaginal septum with distal atretic vagina

Description 

A 15-year-old girl presented with cyclical pelvic, lower abdominal pain and cramping beginning at age 13. She had not yet had onset of menarche and no history of sexual intercourse. On examination, she presented with lower abdominal distension, which felt like a firm mass extending up to the umbilicus and into the pelvic cavity. Abdominal ultrasound showed an ectopic right kidney in the paramedian position at the level of the umbilicus. Ultrasound also showed a large midline cystic structure located between the urinary bladder and rectum, containing low-level homogenous echoes suggestive of blood products. MRI confirmed the ultrasound findings as it showed the endometrial, endocervical and vaginal canal were distended by low-signal material (T2 and short-tau inversion recovery). Images appeared hyperintense on T1W1, suggestive of haematometrocolpos due to a complete transverse vaginal septum at the level of the pubic symphysis, distal portion of vagina was atretic (

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Autoimmune autonomic ganglionopathy associated with Sjögrens syndrome presenting with recurrent abdominal distension

A 65-year-old woman with Sjögren's syndrome presented with recurrent abdominal distension, constipation, weight loss, orthostatic dizziness, loss of sweating and incomplete emptying of the bladder. Gastrointestinal dilatation but no evidence of malignancy or obstruction was found on CT of the abdomen, oesophagogastroduodenoscopy or colonoscopy. Postvoiding residual urine volume was increased. Antiganglionic acetylcholine receptor antibody was positive. We diagnosed as autoimmune autonomic ganglionopathy. The patient responded to corticosteroid treatment. One year after treatment, she continued to have mild gastrointestinal symptoms, but overall condition was stable without further intervention.

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Spontaneous rupture of a pancreatic pseudocyst

Description 

This is a case of a 50-year-old woman with medical history significant for chronic pancreatitis secondary to alcohol abuse who initially presented with a 3-week history of progressively worsening epigastric pain. The patient described the pain as sharp, radiating to the back, worsened with intake of both solid foods and liquids and relieved with self-induced emesis. On admission, the patient was hypertensive, but other vitals were within normal limits. The abdomen was soft; however, there is a firm palpable mass extending from the epigastric to the right and left upper quadrants. Labs were remarkable for hypokalaemia with potassium (K) 3 meq/L (normal range 3.5–4.9 meq/L), mildly elevated lipase 132 U/L (7–60 U/L) and an elevated alkaline phosphatase of 164 U/L (41–108 U/L). CT of the abdomen with contrast (figures 1 and 2) showed a large cystic structure measuring 12.9x21.2 cm directly adjacent to the body of the pancreas and...

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Olmesartan-induced enteropathy

Olmesartan-induced enteropathy (OIE) typically presents with a constellation of signs and symptoms including chronic diarrhoea, weight loss and villous atrophy on biopsy. We describe a 68-year-old Caucasian woman with a history of hypothyroidism and hypertension who presented to our hospital with recurrent episodes of acute intermittent diarrhoea, nausea, vomiting, renal failure and 15 lbs weight loss. After an extensive workup, she was diagnosed with possible OIE. Cessation of the offending drug resulted in improvement of clinical symptoms and also hospital admissions for severe diarrhoea reinforcing the diagnosis of OIE. Among the adverse effects of drug therapy, diarrhoea is a relatively frequent adverse event accounting for about 7%. This report serves as an addition to existing literature and to increase the awareness of olmesartan-induced sprue-like enteropathy among the primary care physicians and gastroenterologists.

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Saccular internal carotid artery aneurysm masquerading as a pituitary macroadenoma

Description 

An 84-year-old woman presented to the emergency department with a 3-day history of progressive binocular diplopia on right-sided horizontal gaze, accompanied with gradual-onset right-sided periorbital headache, vomiting and diarrhoea. Medical history consisted of hypertension and chronic kidney disease, stage 2.

Clinical examination demonstrated right-sided VI cranial nerve palsy, normal pupillary light reaction, visual acuity and fields. No other neurological deficits elicited. No clinical features of hypercortisolism, growth hormone excess or adrenal insufficiency were displayed.

Initial blood tests revealed an acute-on-chronic kidney injury (AKI). Admission estimated glomerular filtration rate was 42 mL/min, baseline 74; admission creatinine 180 μmol/L, baseline 142. An urgent CT head, non-contrast in view of the AKI, was performed. Intravenous fluids were administered. CT revealed a 39x18x14 mm sellar mass, involving the pituitary fossa, extending into the left and right cavernous sinus, sphenoid sinus and posterior sella turcica.

On the next day, MRI of the pituitary (figure...

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Miliary tuberculosis and herpes pharyngitis after a trip to a developing country: dangers of biologics

The use of biologic drugs has expanded since its introduction in the late 1990s. With growing medical use and newer biologic drugs in development, opportunistic infections like Mycobacterium tuberculosis remain important adverse effects. It carries major public health concerns, yet evidence-based clinical guidelines for more routine interval screening in patients taking immunosuppressants and exposed to tuberculosis (TB) are lacking. We illustrate a case of an elderly Indian-born man living in the USA with psoriatic arthritis who was on adalimumab for 10 years. He presented with disseminated TB and herpes simplex virus type 1 (HSV-1) pharyngitis, a year after an innocuous trip to India. Our case draws attention to the adverse effects of biologic drugs and highlights the importance of regular rescreening for a high-risk population. As the use of biologic treatment increases, physicians must be vigilant in more frequent screening, monitoring and identifying related opportunistic infections, notably M. tuberculosis infections.

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Ace the case: a 14-year-old with lower extremity weakness and blurry vision

Description 

A 14-year-old girl presented with progressive upper and lower extremity weakness. Symptoms started 5 months prior with intermittent bilateral calf pain coincident with onset of lower extremity weakness. Initial evaluation revealed normal complete blood count, complelete metabolic panel, inflammatory markers, muscle enzymes and MRI brain and spine with/without contrast. Electromyography demonstrated an acute to subacute polyradiculitis affecting the upper and lower extremities.

She was admitted for expedited evaluation. Examination was significant for lower extremity weakness and absent reflexes. Lumbar puncture (LP) showed lymphocytic pleocytosis and markedly elevated protein. Thorough infectious, oncological and rheumatological evaluations were all unremarkable. Most notable was that CSF Neuromyelitis Optica IgG and paraneoplastic antibody panel were negative, and serum ACE was normal. She was started on intravenous immunoglobulin for empiric treatment of chronic inflammatory demyelinating polyneuropathy (CIDP).

While hospitalised, she developed left eye blurred vision. Ophthalmological examination demonstrated bilateral optic nerve oedema. Repeat MRI...

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Unusual and rare case of generalised lymphadenopathy: Kimuras disease

We report a case of Kimura's disease in a 65-year-old woman who presented with generalised itching, abdominal pain, facial puffiness, difficulty in swallowing and loss of appetite. She was found to have generalised lymphadenopathy and a fine-needle aspiration cytology initially done revealed 'reactive lymphadenitis' which was inconclusive. PET-CT done showed features suggestive of lymphoma. Hence, lymph node biopsy was done for confirmation and incidentally after immunohistochemistry staining it turned out to be Kimura's disease. High-dose steroid therapy was started and patient showed dramatic clinical and symptomatic improvement. Kimura's disease almost always presents as cervical lymphadenopathy and usually never causes compressive symptoms. Our patient presented with compressive symptoms and generalised lymphadenopathy which is a rarity.

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Third-degree burns associated with transcutaneous pacing

A 69-year-old severely obese diabetic woman developed nausea, vomiting and diarrhoea which caused multiple metabolic alterations leading to hypotension and bradycardia due to slow atrioventricular junctional rhythm. Transcutaneous pacing (TCP) was initiated and maintained until the underlying heart rate and blood pressure normalised. TCP gel pads were kept in place prophylactically after pacing was terminated. Gel pads remained attached to the anterior thorax and back for a total of 36 hours. During this time the patient developed third-degree burns at the side of gel pad attachment. With appropriate wound care and after a long hospitalisation, the patient was discharged in stable condition. This case demonstrates that prolonged use of TCP gel pads without frequent replacement may lead to third-degree burns. It also suggest that prophylactic use of TCP gel pads should be avoided.

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Dual ectopic thyroid gland

Description 

Ectopic thyroid tissue (ETT) occurs due to a disorder in the embryonic development of the thyroid gland with incomplete migration of the thyroid tissue from the median plate of the floor of the pharyngeal gut to its usual site. ETT is a rare entity, with an estimated prevalence of 1/100 000–300 000 in the general population, increasing to 1/4000–8000 in patients with thyroid dysfunction. The mechanisms responsible for thyroid morphogenesis have not yet been fully elucidated but certain transcription factors such as TITF1/NKX2-1, PAX8, HHEX or FOXE1 play a key role in this process. ETT mainly affects young women and is often asymptomatic.1 In 90% of the cases, ETT is located at the base of the tongue. The occurrence of two ectopic foci simultaneously is particularly rare.2 The authors present the case of a 30-year-old female patient under endocrinology's care due to autoimmune subclinical hypothyroidism. She...

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Giant right coronary artery aneurysm complicated by a fistula to the right atrium, mimicking a ruptured sinus of Valsalva aneurysm

Patients with a coronary aneurysm alone do not generally exhibit continuous murmurs; however, murmurs may be detected in the presence of a fistula. A 57-year-old woman with chest pain was referred to us with a suspected diagnosis of a ruptured sinus of Valsalva aneurysm owing to the presence of a continuous murmur that was detected on physical examination. However, CT revealed a giant right coronary artery aneurysm draining into the right atrium. Consequently, surgery was performed. This case highlights the importance of implementing multiple imaging modalities for adequate differential diagnoses of patients presenting with continuous murmurs.

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Beliefs and reality of e-cigarette smoking

In this global health case study, we describe a case of nicotine addiction due to chronic use of electronic cigarettes (e-cigarette) and discuss the beliefs on safety and awareness of their side effects. Many people believe that smoking an e-cigarette (vaping) does not have any side effects, especially among teenagers and young adults. Teenagers using an e-cigarette at a young age are twice as likely to try cigarettes later in their life because of nicotine dependency or other social factors. More recent studies have shown long-term systemic side effects of vaping regardless of traditional cigarette smoking history. This report was done for further assessing their risk and to clear out misconceptions of this large-scale condition.

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Advancement of the Harrington technique for reconstruction of pathological fractures of the acetabulum

A 59-year-old man presented with hip pain secondary to metastatic prostate carcinoma affecting multiple sites, including his left acetabulum. The patient's prognosis was good, he was active, independent, with a good quality of life, so was offered surgical reconstruction. The Harrington method involves passing metal rods from the ileum to the ischium and pubis to create a scaffold for hip replacement. This is the gold standard for reconstruction of acetabular metastatic defects. However, this method is prone to failure by medialisation of the construct in the long term. We present our technique of employing a novel modification to the Harrington reconstruction that offers additional support medially with a suprapectineal plate. This construct is strong and durable enough to facilitate immediate weight-bearing and prevent long-term medialisation.

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Troubleshooting a dialysis line: when blue runs red

We present the case of a critically ill woman whose dialysis line was noted to be circulating bright red blood. Located in the right internal jugular vein, the line had previously been working normally with the change occurring shortly after the patient was liberated from positive pressure mechanical ventilation. An arterial malposition was ruled out and subsequent investigations revealed the presence of a left-sided partial anomalous pulmonary venous connection (PAPVC) that had been previously undiagnosed. The identification of a left-sided PAPVC from blood gas measurements taken from a right internal jugular vein dialysis catheter in this case provides an informative opportunity to consider the intricate physiological relationship between the respiratory and cardiovascular systems in critically ill patients requiring invasive procedures and treatments.

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Ehrlichiosis masquerading as thrombotic thrombocytopenic purpura

Ehrlichiosis is a rare tickborne illness that can manifest from an asymptomatic, self-limiting disease to a severe presentation with encephalopathy and renal failure. Ehrlichiosis is diagnosed largely based on patient history with confirmatory tests including peripheral blood smear, serology and PCR. Empiric treatment is warranted in patients with suspected tick bites as a delay in treatment can result in multiorgan failure. We discuss a case of ehrlichiosis that presented with the classic pentad of thrombotic thrombocytopenic purpura (TTP). A history of a tick bite was elicited and intravenous doxycycline 100 mg two times a day was initiated. Tick panel results revealed a positive Ehrlichia chaffeensis IgG and IgM titres, consistent with human monocytic ehrlichiosis. Autoimmune workup and antibodies to Borreliaburgdorferi were negative, and ADAMTS13 activity assay results were inconsistent with TTP. The patient completed 14 days of intravenous doxycycline and had an uneventful recovery.

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Diabetic ketoalkalosis: misnomer or undiagnosed variant of diabetic ketoacidosis

Usually, hyperglycaemia crisis presents with acidotic pH, but ketoalkalosis is a rare and unheard entity presenting in diabetic ketoacidosis. We describe three unique cases where the patients present with hyperglycaemia >250 mg/dL, normal or alkalotic pH, and bicarbonate >20 meq/L, which does not meet criteria for diabetic ketoacidosis. However, once these patients were supplemented with intravenous fluids, diagnosis of diabetic ketoacidosis was evident in laboratory analysis. These case series provide a learning opportunity in diagnosing and management of this rare phenomenon.

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Pancake kidney: an incidental finding in a young man

Description 

An 18-year-old man presented to the surgery emergency department with signs and symptoms of acute intestinal obstruction. There was a previous history of exploratory laparotomy for intestinal obstruction 8 years ago. Abdominal examination revealed a healthy transverse scar in the right lumbar region extending to the umbilical region, abdominal distension and no tenderness or guarding. The patient was managed conservatively by nasogastric tube decompression and intravenous fluids support. A contrast-enhanced computed tomography (CECT) of the abdomen was done. The patient responded well to the conservative management, and the intestinal obstruction was relieved. The CECT abdomen reported an ectopic location of bilateral kidneys, lying in the lower abdomen. The kidneys were para-median in location and extending between L4 and S2 vertebrae. Upper and mid-pole of both kidneys were fused across the midline (figures 1 and 2). No evidence of any calculus or hydronephrosis was seen. Opacification of bilateral...

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First-line ibrutinib for Bing-Neel syndrome

The authors present a case of an elderly man with a history of Waldenstrom macroglobulinaemia in remission who presented with progressively worsening gait abnormalities and falls for several months. His examination was notable for bilateral lower extremity weakness and an unsteady gait. Brain and spinal MRI showed focal leptomeningeal enhancement in the brain and spinal column. Lumbar puncture was performed and cerebrospinal fluid flow cytometry demonstrated a monoclonal CD5/CD10-negative, CD20-positive B-cell lymphocyte population consistent with a diagnosis of Bing-Neel syndrome. He was started on ibrutinib, an oral Bruton's tyrosine kinase inhibitor, and had marked improvement in his weakness and gait. Repeat imaging 2 months after starting ibrutinib showed improvement in his leptomeningeal enhancement. During subsequent follow-up, he continued to tolerate ibrutinib and had a sustained clinical response.

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Case of pneumomediastinum due to alveolar rupture following endotracheal intubation

Description 

A 57-year-old woman presented to the outpatient department with complaints of shortness of breath and fever for the past 2 days. She had no known comorbidities. She was admitted in intensive care unit (ICU) in view of type 1 respiratory failure. Initial chest X-ray showed bilateral lower zone non-homogenous opacity (figure 1A). She was initially managed with antibiotics and non-invasive ventilation. However, due to worsening respiratory failure and metabolic acidosis, patient was intubated. Postintubation, patient was connected to a ventilator in volume control mode with fraction of inspired oxygen of 90%, Positive end expiratory pressure (PEEP) of 10, tidal volume of 350 mL, respiratory set rate of 20 and the ratio of duration of inspiration to the duration of expiration (I:E ratio) of 1:3.3. Chest X-ray repeated following intubation showed features suggestive of pneumomediastinum (figure 1B). The CT thorax done post intubation showed pneumoprecardium (

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Pigmentary retinopathy in Kearns-Sayre syndrome

Description 

An 11-year-old girl with moderate ptosis and chronic progressive ophthalmoplegia (figure 1) was referred for retinal evaluation in view of nyctalopia. Visual acuity on presentation was 20/40 OU. Slit-lamp examination revealed a normal anterior segment. There was no similar family history. Multicolour scanning laser photographs (figure 2A, B) showed retinal pigment granularity in OU that was better appreciated on autofluorescence imaging as speckled hypoautofluorescence and hyperautofluorescence (figure 2C, D). Horizontal spectral domain optical coherence tomography scans through fovea revealed hyper-reflective deposits above the retinal pigment epithelium (figure 2E, F). Systemic workup revealed the gross motor weakness of both upper and lower limbs, mild hearing loss and no cardiac conduction defects. The patient was referred to a paediatric neurologist for further evaluation. Cerebrospinal fluid tap was done which showed elevated lactate (3.10 mg/dL) and protein levels (96 mg/dL). ECG showed no conduction blocks. A muscle biopsy showed...

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Lipemia Retinalis, Macular Edema, and Vision Loss in a Diabetic Patient with a History of Type IV Hypertriglyceridemia and Pancreatitis

Background: Lipemia retinalis is a rare but known complication of elevated serum triglycerides. This case describes the clinical course of a diabetic patient who presented with lipemia retinalis and macular edema, which responded to systemic and local treatments. Case Report: A 40-year-old female with a history of type II diabetes mellitus, hypertriglyceridemia, and pancreatitis presented with decreased vision in the left eye. She had peripapillary and macular edema, intraretinal hemorrhages, and prominent exudates in the setting of lipemia retinalis due to type IV hypertriglyceridemia. She was treated with serial intravitreal bevacizumab injections for macular edema and systemic lipid lowering therapy, and her visual acuity improved back to baseline. Conclusions: In the setting of lipemia retinalis and hypertriglyceridemia, the current patient developed macular edema and vision loss. The macular edema was treated with intravitreal injections of bevacizumab, and the patient experienced a rapid recovery of visual acuity.
Case Rep Ophthalmol 2018;9:425–430

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