Clinical presentation, genotype and microbiological data among cystic fibrosis children at King Hussein medical center

To describe the clinical presentations, genotype, microbiological data among children with cystic fibrosis treated at King Hussein Medical Center. A retrospective review of all the medical records of children [80] already diagnosed with Cystic fibrosis was conducted during the period 2002-2008 at King Hussein Medical Center. Clinical data collected included: age at presentation and diagnosis, clinical manifestation, delay in diagnosis, and family history. Laboratory tests that were done included: complete blood count, kidney and liver function tests, sweat chloride and genetic testing. PCR testing was performed for 36 mutations of cystic fibrosis. Those with borderline sweat chloride reading or atypical presentation were excluded. The study group consisted of 46 [57%] males and 34 [43%] females with classic cystic fibrosis. Age ranged from one month to 16 years of age with a median of eight months. Most of the patients presented between one and six months of age [35%] with the majority being in neonatal period [24%]. Only 13% presented after the age of two years. Most common presentation was recurrent wheezy chest [28%], while the least common was direct hyperbilirubinemia [2%]. A delay in diagnosis more than six months was seen in 18 patients [26%]. Twenty-one children [26%] had positive family history of Cystic Fibrosis, while 12 [15%] gave a history of male infertility in the family. Sputum cultures were positive in 24 patients; Pseudomonas Aureginosa was the most commonly found [24%]. Cystic Fibrosis mutations were found in 25 patients [31%]; Delta F508 mutation was the commonest 4%. Three patients died [4%]; one with respiratory failure and severe pulmonary hypertension, two with severe fatal sepsis. There are diverse clinical presentations and genotypic features among our study group. A complete analysis of the DNA mutation would be helpful in knowing the most prevalent mutations in our population

Cervical lymphadenopathy in children: a diagnostic approach

To determine the common causes of persistent cervical lymphadenopathy in children and to test a diagnostic approach. This study was conducted at King Hussein Medical Center/pediatric clinic over one year period to look for all children between ages of 6 months and 14 years with persistent lymph node enlargement. Persistent enlargement is defined as a lymph node > 1 cm in diameter, and > 2 weeks duration. A management algorithm was proposed and followed in the management. One hundred and thirty children presented to infectious diseases clinic with persistent lymph nodes enlargement. In 70 children [53%] the nodes regressed in 2 weeks time, in another 30 children [23%] they regressed in 4 weeks time. Fifteen children [12%] had tuberculous lymphadenitis; 10 children [8%] had lymph node abscess; 3 children [2%] had Epstein Barr virus infection and 2 children [1.5%] had Hodgkin's lymphoma on initial presentation. Reactive lymphadenitis is the commonest cause of cervical lymph node enlargement in children. Majority of lymph nodes regress in 4 weeks time. Persisting lymph nodes more than 4 weeks warrant histological examination. Tuberculosis is a common cause of cervical lymphadenopathy among Jordanian children

Bacille calmette-guerin lymphadenitis: a single center experience

To describe the clinical presentation, course and management of lymphadenitis post Bacille Calmette-Guerin vaccine. This descriptive study was conducted on 17 cases who were referred to the infectious disease clinic at King Hussein Medical Center with regional lymphadenitis post Bacille Calmette-Guerin vaccine. All cases were infants between two and 12 months of age. The results of lymph node aspirate and biopsy were shown as well. Different treatment protocols used were included. The study was conducted from September 2006 to September 2007. Seventeen patients, 9 males [52%] and 8 females [48%], were studied. Constitutional symptoms were found only in one infant. All infants received the same type of vaccine used by our Ministry of Health which is the Danish strain. Unilateral axillary lymph node enlargement was seen most commonly [47%]. Cervical lymph nodes were involved in four cases [24%] followed by supraclavicular lymphadenopathy in three cases [18%], and one case had cervical and supraclavicular lymphadenopathy. Sinus formation was seen in three [18%] cases. Five cases [30%] had fluctuating lymph nodes. Only one infant had disseminated infection. The majority [65%] of cases had their symptoms starting within the first two months post vaccine. Eight [47%] infants had conservative observational management. Anti-tuberculosis medications were used in five [30%]. Surgical approach was used in four [23%] cases. All infants had complete healing by the end of the study period. Since the introduction of the new vaccine more complications are seen. This stresses the importance of early recognition and implementation of appropriate treatment protocols. Complete healing of suppurative lymphadenitis post Bacille Calmette-Guerin vaccine can be achieved using different treatment protocols

Clinical profile of cystic fibrosis. Atypical presentation

To describe the unusual presentation among patients with confirmed cystic fibrosis. A retrospective review was carried out on all children [n=90] with the diagnosis of classical cystic fibrosis who attended the Respiratory Pediatric Clinic at King Hussein Medical Center, Amman, Jordan from January 2002 - December 2008. All children from one day old to 14 years of age were included. Files of those with unusual presentation were reviewed. Age at presentation and diagnosis, clinical presentation, and family history were collected. Relevant laboratory results, sweat chloride readings, and radiological features were also reviewed. Ninety children [males 51 [57%] and females 39 [43%]] with classic cystic fibrosis were included. The most common initial classical presenting manifestation was recurrent wheezy chest [24%]. The least common presentation was direct hyperbilirubinemia [3%]. Seven cases [8%] had unusual clinical presentations: early pulmonary hypertension, non-obstructive left hydronephrosis with metabolic alkalosis, single isolated episode of metabolic alkalosis, severe iron deficiency anemia with short stature, and the finding of ichthyotic skin lesions. Three of these patients had a positive family history of cystic fibrosis. Two patients with pulmonary hypertension died. The overall mortality rate was 4%. The wide variability of clinical presentations reflects the diversity of clinical picture of cystic fibrosis as a disease. Neonatal screening programs at a national level can decrease the burden of the disease