Influenza: diagnosis,management, and prophylaxis.

Influenza causes enormous morbidity, death, and economic loss. Annual vaccination is strongly recommended for groups at high risk. Amantadine is effective treatment for and prophylaxis against influenza A during epidemics. New developments include rapid laboratory diagnosis, live attenuated vaccines, and antiviral drugs.     

Terrestrial snakebites in the South East of the Arabian Peninsula: patient characteristics, clinical presentations, and management

Background

To describe the characteristics, clinical presentations, management and complications of snakebites in the border region between Al-Ain, United Arab Emirates (UAE) and Buraimi, Sultanate of Oman.

Methodology/Principal Findings

We carried out a retrospective review of medical records to study snakebite cases over four-year duration at three tertiary hospitals. Overall, 64 snakebite cases were studied with median hospitalization of 2 (interquartile range [IQR] 1–4) days. The majority of cases were male (87.5%), and most (61%) of the incidents occurred during summer months. The bite sites were predominantly (95%) to the feet and hands. Main clinical features included pain, local swelling, and coagulopathy, blistering and skin peeling. Overall, there were no deaths, but few major complications occurred; extensive skin peeling (n = 5, 8%), multi-organ failure (n = 1, 1.5%), and compartment syndrome (n = 1, 1.5%). Polyvalent anti snake venom (ASV), analgesia, tetanus toxoid, intravenous fluids, and antibiotics such as ampicillin, cloxacillin, and cephalosporins were commonly instituted as part of treatment protocols in the three hospitals.

Conclusion

The overwhelming majority of bites occurred during summer months, and envenomations were more common in, relatively, young male farmers, but with no serious clinical complications. Prevention and treatment strategies should include increasing public awareness, developing management guidelines, and manufacturing specific ASV for a wide spectrum of the local venomous snakes.     

Update on vesicoureteral reflux: pathogenesis, nephropathy, and management

Clinical reflux was first visualized over 100 years ago. In the 1950s and early 1960s, the assumption was that surgery to relieve bladder neck obstruction would have a positive effect on bladder function and reflux. By the early 1970s it was understood that the underlying structural problems leading to primary reflux were congenitally abnormal distal ureters and orifices. Researchers in the 1960s and 1970s demonstrated the connection between reflux and renal scarring. More recently, reflux nephropathy in the absence of urinary tract infections has been observed, leading researchers to investigate an association between bladder dysfunction and reflux with resulting nephropathy. The cornerstone of management of the child with vesicoureteral reflux is antibiotic prophylaxis; treatment regimens for various grades of reflux are reviewed. Indications for surgical treatment of reflux are also discussed. Controversies regarding vesicoureteral reflux, including duration of prophylactic treatment, remain to be resolved.     

Myasthenia gravis: pathophysiology,diagnosis, differential diagnosis and management

Myasthenia gravis is an autoimmune disorder caused by impaired synaptic transmission at the neuromuscular junction. The characteristic signs and symptoms of this disease manifest as increasing muscle fatigue. This article reviews myasthenia gravis with an emphasis on the pathophysiology, systemic and ocular manifestations, diagnostic testing, and management of this disease.     

Gastroesophageal reflux disease in baboons (Papio sp.): a new animal model

BACKGROUND: Gastroesophageal reflux disease (GERD) is increasingly prevalent in the human population. Current animal models require surgical or other manipulation to produce symptoms. An animal model that exhibits spontaneous GERD would provide the opportunity for much-needed research examining the susceptibility, diagnosis, and treatment of GERD. METHODS: Eight baboons (Papio hamadryas sp.) were diagnosed with GERD histopathologically using biopsies or postmortem tissues. RESULTS: The disease was characterized by a spectrum of symptoms comparable with that found in the human population. Some subjects had no gross signs of clinical disease, but were diagnosed by histopathological examination. Almost all subjects presented with at least one clinical sign of the disease. Regurgitation was the most common. CONCLUSIONS: The baboon may be a superior animal model for GERD research because it is a naturally occurring model and is anatomically and physiologically similar to humans.     

X-linked adrenoleukodystrophy (X-ALD): clinical presentation and guidelines for diagnosis, follow-up and management

ABSTRACT: X-linked adrenoleukodystrophy (X-ALD) is the most common peroxisomal disorder. The disease is caused by mutations in the ABCD1 gene that encodes the peroxisomal membrane protein ALDP which is involved in the transmembrane transport of very long-chain fatty acids (VLCFA; >C22). A defect in ALDP results in elevated levels of VLCFA in plasma and tissues. The clinical spectrum in males with X-ALD ranges from isolated adrenocortical insufficiency and slowly progressive myelopathy to devastating cerebral demyelination. The majority of heterozygous females will develop symptoms by the age of 60 years. In individual patients the disease course remains unpredictable. This review focuses on the diagnosis and management of patients with X-ALD and provides a guideline for clinicians that encounter patients with this highly complex disorder.     

Costochondritis: pathogenesis, diagnosis, and management considerations

The management of costochondritis of the chest wall is reviewed and four illustrative cases presented. Adequate debridement is the single most important factor to ensure eradication of this disease. Well-vascularized tissue coverage reduces ischemic factors, resists secondary infection by contamination, and promotes rapid healing. The pectoralis major, latissimus dorsi, and rectus abdominis muscle flaps are the primary choices for pedicle-flap coverage. Antibiotic coverage was not extended beyond 3 weeks in these cases, and no recurrences have been noted over a 1- to 3-year follow-up.     

X-linked adrenoleukodystrophy (X-ALD): clinical presentation and guidelines for diagnosis, follow-up and management

Background

Pompe disease (Glycogen storage disease type II, GSD II, acid alpha-glucosidase deficiency, acid maltase deficiency, OMIM # 232300) is an autosomal-recessive lysosomal storage disorder due to a deficiency of acid alpha-glucosidase (GAA, acid maltase, EC 3.2.1.20, Swiss-Prot P10253). Clinical manifestations are dominated by progressive weakness of skeletal muscle throughout the clinical spectrum. In addition, the classic infantile form is characterised by hypertrophic cardiomyopathy.

Methods

In a cross-sectional single-centre study we clinically assessed 3 patients with classic infantile Pompe disease and 39 patients with non-classic presentations, measured their acid alpha-glucosidase activities and analysed their GAA genes.

Results

Classic infantile patients had nearly absent residual enzyme activities and a typical clinical course with hypertrophic cardiomyopathy until the beginning of therapy. The disease manifestations in non-classic patients were heterogeneous. There was a broad variability in the decline of locomotive and respiratory function. The age of onset ranged from birth to late adulthood and correlated with enzyme activities. Molecular analysis revealed as many as 33 different mutations, 14 of which are novel. All classic infantile patients had two severe mutations. The most common mutation in the non-classic group was c.-32-13?T?>?G. It was associated with a milder course in this subgroup.

Conclusions

Disease manifestation strongly correlates with the nature of the GAA mutations, while the variable progression in non-classic Pompe disease is likely to be explained by yet unknown modifying factors. This study provides the first comprehensive dataset on the clinical course and the mutational spectrum of Pompe disease in Germany.     

Pyogenic vertebral osteomyelitis: diagnosis and management.

Vertebral osteomyelitis represents a diagnostic challenge to the clinician. Two cases of pyogenic vertebral osteomyelitis occurring weeks to months after a urinary tract infection with Escherichia coli are described. The rarity and subtle clinical presentation of this condition, the presence of pre-existing degenerative arthritic changes, and delayed appearance of radiologic signs of progression to destructive osteomyelitis contributed to a significant delay in diagnosis. Increased awareness of vertebral osteomyelitis as a clinical entity combined with information from radionuclide scanning may permit earlier detection of this condition.     

Frontonasal tumors: their diagnosis and management.

Fifty-three patients with frontonasal tumors were seen in a 17 -year period. Of these patients, 33 were females (62 percent) and 50 were caucasians (94 percent). The correct diagnosis was made preoperatively in 87 percent of the cases. Eighty-five percent of the patients were children, and most of the lesions (83 percent) were developmental anomalies rather than true neoplasms. The most common lesions were dermoids (17), hemangiomas (16), and encephaloceles (9), but 13 different types of lesions were encountered. The treatment of all is surgical excision, and a cooperative effort by the neurosurgeon and the plastic surgeon is required in some cases.     

Merkel cell tumor: diagnosis, prognosis, and management

The histologic features of the Merkel cell and the pathologic characteristics of the Merkel cell tumor are described. From a literature review, 68 cases are examined; 23 cases that have been followed up accurately are presented from the Mayo Clinic. A representative case is used to demonstrate the problems of diagnosis and suggested treatment. The latter should be wide local resection with removal of nodal metastases if present. When indicated, this should be followed up with radiation therapy. Chemotherapy is of little or no value. Survival rates are presented.