1/11. PFAPA syndrome (Periodic Fever, Aphthous stomatitis, pharyngitis, Adenitis).This paper aims to remind paediatric clinicians to suspect and confirm 'PFAPA' syndrome (Periodic Fever, Aphthous stomatitis, pharyngitis and cervical Adenitis syndrome). We report two cases of PFAPA syndrome: a 3-year-old healthy boy with atopic rhinitis and a boy aged 8 years 5 months who simultaneously had lymphocytic vasculitis syndrome treated with immunosuppressive drugs. Both met Marshall's criteria. The literature regarding PFAPA syndrome was complied using a medline search for articles published between 1963 and 1998 and we then reviewed the reference lists of the articles. The medline search revealed 28 cases with available clinical manifestations, management and prognosis. Our study describes two additional cases. We divided the cases into typical (28 cases) and atypical (two cases) PFAPA syndrome. In typical PFAPA, the age of onset was less than 5 years in most cases and the patients presented 4.9 /- 1.4 days of fever (100%), pharyngitis (89.3%), cervical adenitis (72.1%), stomatitis (71.4%), malaise (64.3%), headache (60.7%), abdominal pain (53.6%) and nausea/vomiting (17.9%). Afebrile intervals were 3.2 /- 2.4 months and increased with age. The time from initial onset to final episode was 3 years 7 months /- 3 years 6 months. The total number of episodes was 8.3 /- 2.5 (range 6-14). Effective treatment included steroids, tonsillectomy/adenoidectomy and cimetidine. The general outcome was good. In atypical PFAPF, the clinical manifestations were similar to those of typical PFAPA except that the age of onset was more than 5 years, and life-threatening intestinal perforation happened once in a patient with underlying Fanconi's anaemia. It was concluded that typical PFAPA syndrome is benign and can be diagnosed by detailed history-taking and from physical findings during repeated febrile episodes with tests to rule out other periodic fever syndromes. A review of the literatures since the first report in 1987 has shown that typical PFAPA syndrome is not associated with significant long-term sequelae and has a good response to steroids. One patient with atypical PFAPA, who received low-dose steroids for over 1 year, developed intestinal perforation after an increment of the 7-day steroid dose. If an underlying problem requires long-term immunosuppressive medication, it is wiser to choose cimetidine rather than increasing the steroid dosage to resolve atypical PFAPA.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/11. High fever. Experience in private practice.Experience with confirmed high fever, 40 C (104 F) or more, in a private practice during 14 years is presented. The records of 1,500 patients covering 8,000 patient years disclosed only 108 confirmed episodes of high fever. Eleven diagnostic categories included 149 diagnoses. Fourteen of 43 roentgenographic examinations yielded positive findings, including two cases of pneumonia not detected on physical examination. Two of six stool cultures yielded specific enteric pathogens. Convulsions occurred in 12 of the 108 episodes of high fever, and recurred only once in one child. There were no deaths in this series of children with high fevers. Only one diagnosis, pneumonia, was significantly more frequent in confirmed high fever than in unconfirmed high fever. Lastly, the ability of a group of mothers to read thermometers set at three different temperatures proved to be surprisingly good.- - - - - - - - - - ranking = 8.3101918985322keywords = physical examination, physical (Clic here for more details about this article) |
3/11. diagnosis, management and surgical treatment of non-tuberculous mycobacterial head and neck infection in children.The aim of this study was to present our experience with the clinical characteristics of non-tuberculous mycobacterial (NTM) head and neck lymph node infections, the use of modern diagnostic tools and the appropriate therapeutic measures. We have reviewed the cases of 14 Caucasian children with NTM head and neck lymphadenitis who were treated in our clinic in the last 5 years. Three of the patients were male and 11 were female. Their age ranged from 15 to 98 months (mean age 45.7 /- 21.76 months). Cervical lymph nodes were involved in all of our cases, while the submandibular region was found to be the area mostly affected. Overlying skin was involved in 7 cases. diagnosis was based on intradermal skin testing with specific antigens for atypical mycobacteria, histological examination and specimen culture. skin tests were positive for NTM in all of the patients with a predilection for mycobacterium avium complex. The diagnosis was confirmed by histological examination in 13 cases. Specimen culture was positive in 9 cases, most of them growing M. avium-intracellulare complex. Treatment included complete surgical excision of the affected lymph nodes and the overlying skin, as well as functional neck dissection when required. A second procedure was performed in 2 patients. Successful evaluation of NTM infections of the head and neck lymph nodes should include a detailed history, thorough physical examination and specific laboratory investigations. The treatment of choice is complete surgical excision of all affected tissue.- - - - - - - - - - ranking = 8.3101918985322keywords = physical examination, physical (Clic here for more details about this article) |
4/11. Update on treatment of Marshall's syndrome (PFAPA syndrome): report of five cases with review of the literature.Marshall's syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is a recently described pediatric periodic disease characterized by recurrent febrile episodes associated with head and neck symptoms. The origin of this syndrome is unknown, and it can last for several years. During healthy periods, patients grow normally. The differential diagnosis includes other diseases characterized by periodic fevers, such as familial mediterranean fever, familial Hibernian fever, hyperglobulinemia D syndrome, Behcet's disease, cyclic neutropenia, juvenile rheumatoid arthritis, and several infectious diseases. Many treatments have been used, with various results, including antibiotics, nonsteroidal anti-inflammatory drugs, acetylsalicylic acid, colchicine, antiviral medicines, steroids, cimetidine, and tonsillectomy. We describe 5 new patients affected by PFAPA syndrome who were observed at the Department of Pediatric Otorhinolaryngology, Spedali Civili, Brescia, italy, from November 2000 to August 2001. All children underwent physical examination, bacterial, fungal, and viral cultures, chest radiography, and several laboratory studies. The patients were treated by successful tonsillectomy, and after a mean follow-up of 10 months, no recurrence was observed. An analysis of the literature is also presented with particular emphasis on the differential diagnosis of this rare illness and the results of the different therapeutic options.- - - - - - - - - - ranking = 8.3101918985322keywords = physical examination, physical (Clic here for more details about this article) |
5/11. Remission of chronic inflammatory demyelinating polyneuropathy following adenotonsillectomy.patients with chronic inflammatory demyelinating polyneuropathy (CIDP) experience proximal- and distalextremity weakness, sensory loss, and often hyporeflexia or areflexia. CIDP is associated with a variety of concomitant medical illnesses, which often manifest weeks before the onset of muscle weakness and paresis. We describe what we believe is the first reported case of an association between CIDP and recurrent acute adenotonsillitis, which we observed in an 11-year-old girl. Following adenotonsillectomy, the patient's CIDP went into remission and her overall physical condition improved with physiotherapy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/11. PFAPA syndrome mimicking familial mediterranean fever: report of a Turkish child.The PFAPA (Periodic Fever, Aphthous stomatitis, pharyngitis, Adenitidis) syndrome is characterized by periodic fever, adenitis, pharyngitis, and aphthous stomatitis. Herein, we present a Turkish child with PFAPA syndrome mimicking familial mediterranean fever because of a rare presentation. A 9-year-old boy was admitted with recurrent fever, aphthous stomatitis, sore throat, headache, and general body pains, lasting 2 to 3 days since 3.5 years of age. He was completely symptom-free between the attacks. He was diagnosed as having familial mediterranean fever according to the clinical findings when he was 6 years of age and colchicum tablet was administrated. Despite colchicines therapy for 8 months, his attacks did not subside; therefore, the drug was discontinued. He had high fever, a painful cervical lymphadenopathy, aphthous stomatitis, and tonsillo-pharyngitis. The patient was then diagnosed as having PFAPA syndrome. He was given a single dose of prednisolone (0.35 mg/kg/dose). His complaints dramatically and completely disappeared 3 h after administration of the drug. During the 8th month of follow-up, a similar febrile attack lasting only 1 day was noted and it was controlled with a single dose of prednisolone (0.5 mg/kg/day). At this writing the patient is in the 12th month of follow-up, and there have been no symptoms after the second attack. In conclusion, our patient shows that PFAPA syndrome can be confused with familial mediterranean fever. We also would like to emphasize that the typical PFAPA syndrome can be easily diagnosed by detailed history-taking and physical findings.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/11. tularemia in adults and children: a changing presentation.tularemia, a febrile zoonosis with six clinical types, recently has been shown to occur at an increased incidence in children compared with previous reports. Ulceroglandular and glandular tularemia are still the most common types, but pneumonic tularemia has increased in incidence. However, with these changes, an overall decline in mortality has been observed. Children exhibit fever, pharyngitis, hepatosplenomegaly, and constitutional symptoms more often than affected adults. The complication of late lymph node suppuration is found in half of the tularemia patients with lymphadenopathy. A high index of clinical suspicion and a good medical history and physical examination confirmed by specific serologic studies are the critical factors in the successful diagnosis of tularemia in children.- - - - - - - - - - ranking = 8.3101918985322keywords = physical examination, physical (Clic here for more details about this article) |
8/11. femoral nerve palsy: compression by lymph glands in the inguinal region.femoral neuropathy caused by compression of the femoral nerve is not a common syndrome when compared with other compression neuropathies. In most cases reported, the compression was intraabdominal, either in the retroperitoneal region or in the course of the nerve through the psoas major muscle. In this case, the lesion was located extraabdominally. Enlarged and inflamed nodes in the inguinal region caused compression of the nerve resulting in femoral neuropathy. electromyography (emg) and nerve conduction studies revealed that the lesion was neurapraxia. As expected, the condition was reversible; the patient responded to treatment of the inflamed glands and to physical therapy. The use of emg was helpful for confirming the diagnosis and making the prognosis.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
9/11. Cyclic hematopoiesis: human cyclic neutropenia.Human cyclic neutropenia is a relatively rare disorder of unknown etiology. Study of patients and animals with the disorder has led to important information regarding the differentiation of blood cells and control mechanisms of hematopoietic regulation. It has a world-wide distribution, occurs in both sexes, and, in about one-fourth of the patients, a family history has been obtained. While usually benign, deaths from overwhelming infections occur. In addition to cycling of neutrophils, in the majority of cases the monocytes cycle and in about one-fifth of the cases eosinophils are elevated. In a small number of patients, cycling of platelets and reticulocytes occurs. Cycles of colony stimulating factor are present. Cycles of bone marrow cells are easily demonstrable. The recent transfer of human cyclic neutropenia following allogenic bone marrow grafting confirms the hypothesis that the disorder is of bone marrow origin. The following subjects are covered in this review article: A. Definition, history, and incidence; B. Etiology, geographic distribution, mode of transmission; C. Symptoms, physical signs, diagnosis, clinical course; D. Clinical laboratory studies; E. Experimental studies; F. prognosis; G. Treatment. It is felt that human cyclic neutropenia represents a heterogeneous group of disorders and that much remains to be learned about its cause(s).- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
10/11. Neuroretinitis, aseptic meningitis, and lymphadenitis associated with bartonella (Rochalimaea) henselae infection in immunocompetent patients and patients infected with human immunodeficiency virus type 1.bartonella (Rochalimaea) henselae causes a variety of diseases, including bacillary angiomatosis, peliosis hepatis, lymphadenitis, aseptic meningitis with bacteremia, and cat-scratch disease (CSD). Cases of B. henselae-related disease were collected from September 1991 through November 1993. patients with suspected CSD, unexplained fever and lymphadenitis, or suspected B. henselae infection who were seen in the Infectious Diseases Clinic at Wilford Hall Medical Center (Lackland air Force Base, TX) underwent physical and laboratory examinations. In addition to three previously described cases, 23 patients with R. henselae-related infection were identified. The patients included 19 immunocompetent individuals presenting with lymphadenitis (11), stellate neuroretinitis (5), Parinaud's oculoglandular syndrome with retinitis (1), chronic fatigue syndrome-like disease (1), and microbiologically proven adenitis without the presence of immunofluorescent antibodies to B. henselae (1) and four patients infected with human immunodeficiency virus type 1 presenting with isolated lymphadenitis (1), diffuse upper-extremity adenitis (1), neuroretinitis (1), and aseptic meningitis (1). A couple with neuroretinitis and their pet cat, a persistently fatigued patient, and a patient with Parinaud's oculoglandular syndrome were shown to have bacteremia. Tissue cultures were positive for B. henselae in three recent cases of adenitis. Twenty-two patients were exposed to cats. This series further demonstrates the similarities between B. henselae-related diseases and CSD and identifies several new syndromes due to B. henselae.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
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