The clinical patterns of myalgia in children with familial Mediterranean fever.

OBJECTIVES: To study the frequency and clinical patterns of myalgia in a defined group of children with familial Mediterranean fever (FMF). METHODS: A prospective 4-year (September 1995-September 1999) study of children with FMF seen in the pediatric FMF clinic of Jordan University teaching hospital. Diagnosis of FMF was made according to published criteria. Once the diagnosis of FMF and myalgia was made, details about myalgia were collected by interview with the child and his/her parents and entered into a special study form. RESULTS: Of 264 children with FMF seen over the study period, 65 (25%) developed myalgia. Three clinical patterns of myalgia were identified: the spontaneous pattern, the exercise-induced pattern, and the protracted febrile myalgia syndrome (PFMS), seen in 8%, 81%, and 11% of patients, respectively. The three patterns differed in the severity of pain, height of fever, and duration of the episode. In 33 children with the exercise-induced myalgia, in which response to colchicine could be reliably assessed, a favorable response was achieved in 97%. Three children with the PFMS had a dramatic response to corticosteroids. CONCLUSIONS: Myalgia in children with FMF is common and can follow three different clinical patterns.

Familial Mediterranean fever in children: the expanded clinical profile.

The clinical picture of familial Mediterranean fever (FMF) has been appreciably expanded in the last 10 years. Over 8 years, we studied the expanded clinical profile of FMF in 476 children. Of these, 81% had abdominal pain, 41% chest pain, 42% arthritis, 12% severe myalgia, 12% skin manifestations, 4% scrotal swelling, 3% recurrent episodic fever, and one child (0.2%) developed recurrent hyperbilirubinaemia. Two (0.4%) children developed renal complications which were reversed by colchicine; however of 19 probands, 36 family members suffered from chronic renal failure. Our study indicates a familial predisposition to nephropathy in certain families with FMF. This study is the first to report the expanded clinical profile of FMF in a large group of Arab children, giving an opportunity to compare the findings with those in children with FMF in other ethnic groups, and to help in the study of genotype-phenotype correlation.

Recurrent episodic fever. A presenting feature of familial Mediterranean fever.

OBJECTIVES: To study the natural course and outcome of recurrent episodic fever without serositis as a presenting feature of familial Mediterranean fever (FMF). PATIENTS: Of 309 children with FMF seen over a period of 5 years, 8 presented with recurrent episodes of fever without serositis, imposing a difficult diagnostic problem. RESULTS: The age at onset of fever ranged between 5 months and 8 years with a mean of 2.5 years. Five patients eventually developed serositis. The duration between onset of fever and onset of serositis ranged between 1.5-3 years with a mean of 2 years. Of the 3 patients who did not develop serositis, 2 had a family history of FMF. Therapeutic response to colchicine was dramatic in 7 children (one refused colchicine prophylaxis). CONCLUSION: Episodic fever alone without serositis is a presenting feature of FMF. In patients from Mediterranean ancestors and/or the presence of family history of FMF, a therapeutic diagnostic test with colchicine could be rewarding.

Visceral leishmaniasis from Bal'a, Palestine, caused by Leishmania donovani s.1. identified through polymerase chain reaction and restriction fragment length polymorphism analysis.

A 5-year old female from Bal'a, Tulkarm area, Palestine, was admitted with an 8 month history of fever, excessive night sweating, abdominal distension and enlargement, weight loss and sever anorexia. She was investigated elsewhere without reaching specific diagnosis. On admission, the history and symptoms were compatible with visceral leishmaniasis and bone marrow aspirate was positive for Leishmania amastigotes. The serum titer, using IFAT, was 1:640 for L. infantum and 1:320 for L. major promastigotes. When bone marrow material was also subjected to PCR followed by RFLP enzyme analysis, three fragments of the PCR product of the parasite present were obtained: two fragments of 260 bp and one fragment of 80 bp, identical with the pattern obtained with L. donovani. The patient received sodium stibogluconate, 200 mg IM for 30 days. Six months after treatment, the spleen was 2 cm below the costal margin, the liver was not palpable and she gained 1 kg. This case alerts general practitioners, pediatricians and health authorities to the presence of visceral leishmaniasis in Palestine and to the possibility of the disease being encountered in Jordan.

Progressive pseudorheumatoid dysplasia: report of a family and review.

Progressive pseudorheumatoid dysplasia is an inherited skeletal dysplasia with radiographic changes notably in the spine, similar to spondyloepiphyseal dysplasia tarda. There is also articular cartilage involvement which gives it some clinical resemblance to rheumatoid arthritis. We report here on six subjects from one inbred family from Jordan. Based on previously published reports and this one, we review the clinical and radiological features and discuss the genetics and differential diagnosis of the disorder. We suggest the addition of the word "spondyloepiphyseal" to the name adopted by the International Working Group on Constitutional Diseases of Bone, to become "progressive pseudorheumatoid spondyloepiphyseal dysplasia". We also speculate on candidate genes for this disorder.

Diagnosis and treatment of 106 cases of human brucellosis.

During the year 1987, 106 cases of human brucellosis were studied prospectively at the Jordan University Hospital. The disease was more often diagnosed among adults (73.6%) than children (26.4%). Serious clinical complications were observed in 5.7% patients. An initial Brucella antibody titre greater than or equal to 160 proved to be reliable in confirming suspected cases of acute and subacute brucellosis. Culture of blood was found to be more sensitive (44.4%) and significant (P less than 0.02) than bone marrow culture (27.7%) for detecting Brucella melitensis. All patients treated with rifampicin plus tetracycline or co-trimoxazole were considered to be clinically cured by disappearance of all major clinical features of brucellosis. By contrast, 2/10 patients treated with rifampicin alone, as well as 1/56 patients treated with tetracycline and streptomycin, clinically relapsed. It is evident from this study that the treatment with rifampicin alone is not as effective in brucellosis as it is when given with another appropriate drug.

The changing epidemiology of diphtheria in Jordan.

Outbreaks of diphtheria used to occur regularly in Jordan, the last such outbreak being in 1977-78. Since that time, a massive immunization programme targeted at pre-school-age children has been markedly successful. Hence, when an outbreak of diphtheria occurred in 1982-83, it was unexpected. Of the 35 patients who were treated at the Jordan University Hospital, two died and the remaining 33 recovered uneventfully. Contrary to our findings in previous diphtheria epidemics in Jordan, this outbreak largely involved adolescents and young adults.