Beware spontaneous bilateral pneumothorax.

A previously healthy young man presented to A&E with severe dyspnoea. The cause of his distress was bilateral spontaneous pneumothoraces. Prompt insertion of chest drains relieved his distress. This rare clinical entity should be considered in an individual with severe respiratory distress. Diagnosis is difficult on clinical examination alone.     

Case report: severe respiratory alkalosis--unusual manifestation of viral hepatitis

A middle-aged man who had been in good general health presented with marked hyperventilation and severe respiratory alkalosis. Typical signs and symptoms of viral hepatitis subsequently developed. Investigation showed that he and 32 other patients with hepatitis had all eaten at the same restaurant prior to illness. Because of his distressing constitutional symptoms and markedly elevated hepatic enzyme levels, corticosteroid therapy was begun. The symptoms and hyperventilation improved dramatically. Although respiratory alkalosis has been observed during the course of other infectious diseases, to our knowledge it has not previously been reported in association with hepatitis A infection.     

Case of cannabinoid hyperemesis syndrome with long-term follow-up

Long-term cannabis use may be associated with attacks of severe nausea and vomiting, and a characteristic learned behavior of compulsive hot bathing, termed cannabinoid hyperemesis syndrome (CHS). Long-term follow-up and prognosis of CHS have not been reported previously. A 44-year-old Caucasian man with a long history of addiction to marijuana presented with chronic abdominal pain complicated by attacks of uncontrollable vomiting for 16 years. He had a compulsion to take scalding hot showers, as many as 15 times a day, to relieve his symptoms. All previous therapies had been ineffective. However, abstinence from marijuana led to rapid and complete resolution of all symptoms and his compulsive hot showering behavior. He has been followed for nine years, and is still doing well without recurrence of symptoms. Physicians should have a high index of suspicion for this under-recognized condition, as excellent long-term prognosis of CHS can be achieved when abstinence is maintained.     

Popliteal aneurysm as a source of phantom pain: a case report

A 42-year-old man with a remote history of right transtibial amputation but no history of phantom pain developed severe phantom pain 10 years after amputation. A literature review suggested that his presentation was contrary to the natural history of phantom pain, which is usually most prominent in the early stages after amputation. Diagnostic workup revealed a popliteal artery aneurysm, which was successfully treated with coil embolization. The patient had complete resolution of his phantom pain after treatment of the aneurysm, suggesting it was the source of the pain. As a result of successful diagnosis and treatment, the amputation level did not need to be revised and the patient was able to continue his previously high level of function.     

Hydrofluoric Acid‐Induced Burns and Life‐Threatening Systemic Poisoning—Favorable Outcome After Hemodialysis

Abstract

Background:?Skin contact with hydrofluoric acid (HF) may cause serious burns and life‐threatening systemic poisoning. The use of hemodialysis in fluoride intoxication after severe dermal exposure to HF has been recommended but not reported. Case report:?A 46‐year‐old previously healthy man had 7% of his body surface exposed to 71% HF. Despite prompt management, with subsequent normalization of the serum electrolytes, recurrent ventricular fibrillation occurred. On clinical suspicion of fluoride‐induced cardiotoxicity, acute hemodialysis was performed. The circulatory status stabilized and the patient fully recovered. High fluoride levels in the urine and serum were confirmed by the laboratory. Discussion:?There is no ultimate proof that the favorable outcome in this case was significantly attributable to the dialysis. However, most reported exposures of this magnitude have resulted in fatal poisoning. As our patient had normal serum electrolytes and no hypoxia or acidosis at the time of his arrhythmias, it was decided that all efforts should be focused on removing fluoride from his blood. The rationale for performing hemodialysis for this purpose is clear, even though such intervention is more obviously indicated in patients with renal failure. Conclusion:?Hemodialysis may be an effective and potentially lifesaving additional treatment for severe exposure to HF when standard management has proven insufficient.     

A case of congestive heart failure due to reversible dilated cardiomyopathy caused by hyperthyroidism

Thyrotoxicosis is an uncommon cause of low-output congestive heart failure. The case of a 41-year-old male who presented with severe symptomatic congestive heart failure, and was subsequently diagnosed with dilated cardiomyopathy secondary to hyperthyroidism, is presented. The cause of his hyperthyroidism was Graves disease. Despite an initial left ventricular systolic ejection fraction of 20% and evidence of global hypokinesis on echocardiography, treatment with antithyroid agents led to rapid improvement in his clinical status and normalization of his ejection fraction. The proposed mechanisms underlying the development of systolic dysfunction in thyrotoxicosis are discussed, and the literature on similar cases previously reported is reviewed.     

Spontaneous Bilateral Internal Carotid Artery Dissection and Migraine: A Potential Diagnostic Delay

A 36-year-old man with a history of migraine without aura, presented with recurrent bouts of severe headache, perception of flashing lights in both visual fields, and transient bilateral neurological deficits. In view of his history, migraine with aura was considered. Ancillary investigations showed bilateral extracranial internal carotid artery dissection. This case illustrates that when attacks of severe headache with scotomata and transient bilateral neurological deficits occur in a patient with a history of migraine, the diagnosis of carotid artery dissection should be considered, especially when the pattern of headache is different or when some clinical characteristics have not been experienced previously.     

Complicated secondary pneumonia after swine-origin influenza A virus infection in an immunocompetent patient

The pandemic of the swine-origin influenza A virus (S-OIV) in 2009 demonstrated severe viral pneumonia followed by acute respiratory distress syndrome (ARDS). Although ARDS would be caused by the influenza virus pneumonia itself, it has remained unclear whether other respiratory viral or bacterial infections coexist with S-OIV pneumonia. We report an immunocompetent patient with methicillin-resistant Staphylococcus aureus (MRSA) and Herpes simplex virus (HSV) pneumonia secondary to S-OIV infection. A 57-year-old man previously without major medical illness was admitted to our hospital with severe pneumonia accompanied by ARDS due to S-OIV. In his clinical course, anti-influenza treatment was not effective. Sputum culture revealed the presence of MRSA, and HSV was isolated in broncho-alveoler lavage (BAL) fluid. Administration of an antiviral agent (acyclovir), an antibacterial agent (linezolid), and a corticosteroid (methylprednisolone) successfully improved the pneumonia and ARDS. HSV pneumonia can scarcely be seen in healthy people. However recently it has been recognized as a ventilator-associated pneumonia. Although coexistence of Streptococcus pneumoniae and MRSA was reported in S-OIV pneumonia, secondary viral infection has not been reported. The present report is the first patient with HSV pneumonia secondary to S-OIV infection. We propose that a possibility of hidden HSV pneumonia should be taken into consideration in patients with prolonged severe pneumonia due to influenza infection.     

Chronic paroxysmal hemicrania-like headaches in a child: response to a headache diary

Chronic paroxysmal hemicrania and cluster headache are both characterized by recurrent, severe, unilateral headaches accompanied by symptoms and signs of autonomic dysfunction. They are differentiated by the frequency, duration, and medication responsiveness of the headaches. Both occur in childhood, although such reports are rare. A 6-year-old boy presented with chronic paroxysmal hemicrania-like headaches. Through the use of a headache diary, his headaches were found to follow stressful events and resolved shortly after the introduction of the diary. Precipitation of chronic paroxysmal hemicrania-like headaches by stress has not been previously reported. We recommend the use of a headache diary as both an aid to diagnosis and an initial nonpharmacological therapeutic intervention for children with such headaches.     

Severe orthostatic hypotension in a diabetic patient may not be due to diabetic autonomic neuropathy

This lesson describes an unusual case of a man who was recently diagnosed with type 1 diabetes and who presented with severe orthostatic hypotension. As his diabetes was recent in onset, well controlled, and he had no other signs of microvascular disease, other causes of orthostatic hypotension were sought. His serum and cerebrospinal fluid were strongly positive for Borrelia burgdorferi IgG, suggesting a diagnosis of Lyme neuroborreliosis. Autonomic instability in Lyme, while rare, has been previously reported.     

Preserving function in Duchenne dystrophy with long-term pulse prednisone therapy

We report a case of a patient with Duchenne muscular dystrophy who was treated with intermittent pulse prednisone for severe asthma from age 3 to 17 yr and had remarkable preservation of skeletal muscle function. He had a maternal uncle with Duchenne muscular dystrophy who had the identical familial deletion mutation and died at age 19 of respiratory failure. Compared with his untreated uncle, our patient remains partially ambulatory at age 20. This case provides interesting, albeit anecdotal, evidence of considerable clinical benefit from pulse prednisone used on a much longer term basis than has been previously studied and promotes the need for further investigation on this type of therapy in Duchenne muscular dystrophy.     

Sodium Losing Renal Disease: Two Cases and a Review of the Literature

Two patients with sodium-losing renal disease were studied indetail. Both presented with shock and hypotension which wasattributed at first to other causes, as was the recurrent hyponatraemiain one of them. In both patients the cause of the sodium losswas probably unrelieved urinary obstruction which has been reportedpreviously to cause water loss but not sodium loss. Both patientshad severe hyponatraemia when they were sodium depleted, whichhas previously been attributed to water retention from excessivesecretion of antidiuretic hormone. Plasma arginine vasopressinconcentrations were raised in one patient but not in the other.The cause of the water retention in the other patient is notknown. One of the patients, like others described in the literature,was only able to vary his sodium excretion within narrow limits.He became sodium depleted on a normal intake and oedematouswhen he was given saline intravenously. We suggest that theterm sodiumlosing renal disease should be replaced by the term‘fixed sodium’ excretion renal disease.     

First observation of inhibitor development against efmoroctocog alfa in France

In patients with severe haemophilia receiving clotting factor concentrates, the risk of immunisation against their usual treatment is still patent and feared. New haemophilia drug treatments with an extended half-life have become available over the past few years. The risk of inhibitor development to these new treatments is unclear. We report the case of a 51-year-old man with severe haemophilia A, who was previously treated with no history of inhibitor development. Soon after a switch in his treatment to efmoroctocog alfa he developed an inhibitor against this recombinant Fc fusion extended half-life FVIII (rFc-FVIII) product. The patient was on an on-demand treatment regimen and was treated for mucosal bleeding. The inhibitor was characterised as type I, with classical epitope mapping. The spontaneous evolution of this inhibitor was favourable, but an anamnestic response led to a switch in his treatment to emicizumab.     

Severe bronchiolitis probably caused by varicella-zoster virus

An unusual, severe pneumonia probably caused by varicella-zoster virus is reported in a 19-year-old previously healthy man. The diagnosis was based on high titer of varicella-zoster antibodies in serum, and demonstration of varicella-zoster antigen from lung biopsy specimen. The uncommon feature in the pathophysiological course of the disease was the selective hypercarbia that responded well to bronchodilator therapy with theophylline. Furthermore, the patient had no skin manifestations during his illness.     

Massive theophylline overdose with atypical metabolic abnormalities

We report a case of fatal theophylline overdose in a 16-year-old asthmatic boy who presented with seizures, respiratory arrest, and a theophylline concentration of 117 mg/L in serum. His hospital course was complicated by refractory hypotension and severe ischemic necrosis of skeletal muscle, bowel, and liver. The metabolic abnormalities observed early in his hospital course included severe hyperkalemia, hyperphosphatemia, hypermagnesemia, hypocalcemia, and profound metabolic acidosis. These metabolic abnormalities differ from those previously reported in cases of massive theophylline overdose. The metabolic abnormalities observed in this patient probably reflected his extensive ischemic tissue damage with release of intracellular ions and associated acidemia. Markedly increased catalytic activities of creatine kinase, aspartate aminotransferase, and alanine aminotransferase in serum were also noted.     

Severe lumbar spinal stenosis combined with Guillain-Barré syndrome: A case report

BACKGROUNDGuillain-Barré syndrome (GBS) is a rare disorder that typically presents with ascending weakness, pain, paraesthesias, and numbness, which mimic the findings in lumbar spinal stenosis. Here, we report a case of severe lumbar spinal stenosis combined with GBS.CASE SUMMARYA 70-year-old man with a history of lumbar spinal stenosis presented to our emergency department with severe lower back pain and lower extremity numbness. Magnetic resonance imaging confirmed the diagnosis of severe lumbar spinal stenosis. However, his symptoms did not improve postoperatively and he developed dysphagia and upper extremity numbness. An electromyogram was performed. Based on his symptoms, physical examination, and electromyogram, he was diagnosed with GBS. After 5 d of intravenous immunoglobulin (0.4 g/kg/d for 5 d) therapy, he gained 4/5 of strength in his upper and lower extremities and denied paraesthesias. He had regained 5/5 of strength in his extremities when he was discharged and had no symptoms during follow-up.CONCLUSIONGBS should be considered in the differential diagnosis of spinal disorder, even though magnetic resonance imaging shows severe lumbar spinal stenosis. This case highlights the importance of a careful diagnosis when a patient has a history of a disease and comes to the hospital with the same or similar symptoms.     

Aortoenteric fistula in a 21-year-old

We present the case of a 21-year-old male with recurrent, severe gastrointestinal hemorrhage, who eventually proved to have an aortoduodenal fistula. The fistula arose from an aortic repair done after a stab wound four years previously. The diagnosis of aortoenteric fistula was not initially suspected because of the patient's age, inability to provide details of his previous surgery, and false-negative endoscopic and radiologic studies. We believe that this is the youngest reported patient who developed an aortoenteric fistula after aortic surgery. The presence of an aortoenteric fistula should be suspected in any patient with a GI bleed and previous aortic surgery, regardless of the patient's age.     

A Lesson in Spirituality

Understanding spirituality during palliative care training is not easy. It slowly unravels itself when one starts caring for patients and meeting their caregivers. One such experience in the hospice has been described in this narrative. A person with advanced incurable cancer is initially in severe distress. Over time, he slowly comes to terms with the situation and eventually, a question from his illiterate wife—an insightful question about any last wish—brings out his desire to have certain religious rituals that were alien to his own religion. After his death, the family members concur with his last wish and also indulge in some religious rituals of their own choice. This story reaffirms that the essence of spirituality is the coexistence of harmony and humanity.     

Autoimmunity in transfusion babesiosis: A spectrum of clinical presentations

Transfusion‐acquired babesiosis can be an asymptomatic or self‐limited febrile hemolytic illness in a healthy host. A persistent, relapsing, and/or fulminant course with the development of life‐threatening complications may be seen in immunocompromised or splenectomized patients. As in malaria, erythrocyte parasitemia is often associated with nonimmune hemolysis, and can be treated with erythrocytapheresis. Just as warm autoantibodies have been reported in malaria infection, the development of autoantibody‐mediated immune hemolysis has been reported in babesiosis. We treated a previously healthy male with multiple injuries from a motor vehicle accident necessitating massive transfusion. Late in the hospitalization, his blood smear revealed Babesia microti, confirmed by PCR study and serology. This was eventually traced to a unit of blood from an asymptomatic blood donor that was transfused during his initial trauma care. Specific antibiotic therapy was begun, and severe hemolysis from a high parasite burden required red blood cell exchange which led to rapid abatement of the hemolysis. He had a positive DAT (IgG with a pan‐reactive eluate) but no serum autoantibody. This persisted for 10 days following cessation of hemolysis, and became negative while still on antibiotics while his parasite burden became undetectable. Reports of autoimmunity associated with community acquired babesiosis often have severe hemolysis from their autoantibodies, but our case shows that autoantibodies may also follow transfusion‐acquired babesiosis. The nature of the autoantigen is unknown. J. Clin. Apheresis, 2010. © 2010 Wiley‐Liss, Inc.     

The same IkappaBalpha mutation in two related individuals leads to completely different clinical syndromes

Both innate and adaptive immune responses are dependent on activation of nuclear factor kappaB (NF-kappaB), induced upon binding of pathogen-associated molecular patterns to Toll-like receptors (TLRs). In murine models, defects in NF-kappaB pathway are often lethal and viable knockout mice have severe immune defects. Similarly, defects in the human NF-kappaB pathway described to date lead to severe clinical disease. Here, we describe a patient with a hyper immunoglobulin M-like immunodeficiency syndrome and ectodermal dysplasia. Monocytes did not produce interleukin 12p40 upon stimulation with various TLR stimuli and nuclear translocation of NF-kappaB was impaired. T cell receptor-mediated proliferation was also impaired. A heterozygous mutation was found at serine 32 in IkappaBalpha. Interestingly, his father has the same mutation but displays complex mosaicism. He does not display features of ectodermal dysplasia and did not suffer from serious infections with the exception of a relapsing Salmonella typhimurium infection. His monocyte function was impaired, whereas T cell function was relatively normal. Consistent with this, his T cells almost exclusively displayed the wild-type allele, whereas both alleles were present in his monocytes. We propose that the T and B cell compartment of the mosaic father arose as a result of selection of wild-type cells and that this underlies the widely different clinical phenotype.