Hyposalivation in a 16‐year‐old girl: a case of salivary gland aplasia

Salivary gland aplasia is a rare condition with only a small number of cases reported worldwide. It is more commonly seen in males and can occur either in isolation or association with other defects or syndromes. It may or may not occur with a hereditary background. Scant literature exists detailing the status of sublingual salivary glands in patients with any form of major salivary gland aplasia. This case report describes the clinical and magnetic resonance imaging presentation of a 16‐year‐old girl with major salivary gland aplasia detailing the status of all six major salivary glands.     

Coronary artery disease in congenital single coronary artery in adults:A Dutch case series

AIM:To assess the current diagnostic and therapeutic management and the clinical implications of congenital single coronary artery(SCA) in adults.METHODS:We identified 15 patients with a SCA detected from four Dutch angiography centers in the period between 2010 and 2013.Symptomatic patients who underwent routine diagnostic coronary angiography(CAG) for suspected coronary artery disease and who incidentally were found to have isolated SCA were analyzed.RESULTS:Fifteen(7 females) with a mean age of 58.5 ± 13.78 years(range 43-86) had a SCA.ConventionalCAG demonstrated congenital isolated SCA originating as a single ostium from the right sinus of Valsalva in 6 patients and originating from the left in 9 patients.Minimal to moderate coronary atherosclerotic changes were found in 4,and severe stenotic lesions in another 4 patients.Seven patients were free of coronary atherosclerosis.Runs of non-sustained ventricular tachycardia were documented in 2 patients,one of whom demonstrated transmural ischemic changes on presentation.Myocardial perfusion scintigraphic evidence of transmural myocardial ischemia was found in 1 patient due to kinking and squeezing of the SCA with an interarterial course between the aorta and pulmonary artery.Multi-slice computed tomography(MSCT) was helpful to delineate the course of the anomalous artery relative to the aorta and pulmonary artery.Percutaneous coronary intervention was successfully performed in 3 patients.Eight patients were managed medically.Arterial bypass graft was performed in 4 patients with the squeezed SCA.CONCLUSION:SCA may be associated with transient transmural myocardial ischemia and aborted sudden death in the absence of coronary atherosclerosis.The availability and sophistication of MSCT facilitates the delineation of the course of a SCA.We present a Dutch case series and review of the literature.     

A retrospective intercenter comparison of two surgical protocols through the dental arch relationship of 5- to 6-year-old unilateral cleft patients

Clinical Oral Investigations - The objectives of this retrospective equivalence trial were to assess the dental arch relationship of 5- to 6-year-old patients with unilateral cleft lip and palate...     

Whipple's Disease in an Afro-Caribbean National

Whipple''s disease is a rare multi-organ infectious disease caused by Tropheryma whipplei. It is fatal without treatment. We report on a 40-year old Afro-Jamaican man who presented with a six-month history of weight loss and diarrhoea. Investigations revealed iron deficiency anaemia and hypoalbu-minaemia. Upper gastrointestinal endoscopy revealed white patchy lesions in the duodenum. The duodenal biopsy showed broadening and thickening of the villi by a dense infiltrate of foamy histiocytes within the lamina propria and focally extending into the attached submucosa. Periodic Acid-Schiff stains were positive. Electron microscopy was confirmatory and polymerase chain reaction testing conclusively identified the organisms as T whipplei. Antibiotic treatment resulted in resolution of symptoms. Although the diagnosis of Whipple''s disease is difficult, increased awareness should lead to an increase in reported cases with the improvements in diagnostic capabilities.     

Joint effusions: MR imaging

Magnetic resonance (MR) images of the hips and knees of three healthy volunteers were reviewed and compared with images obtained from the hips of three patients with effusions. Comparison was also made with MR images obtained from two swine knees following the injections of saline in one and blood in the other. The anatomy of the knees and hips was well defined. The hip effusions were all easily recognized. MR images of the swine knees clearly demonstrated the experimentally produced joint effusions. The hip effusions and the experimentally introduced blood and saline all had long T1 and T2 values allowing easy differentiation from articular cartilage. We were unable to distinguish fresh blood from saline in the knee joints.     

Development of IgG responses to mycobacterial antigens

Recent studies link mycobacterial and human heat shock protein antigens with autoimmune diseases. Little is known about the development of antibody responses to these antigens in children. IgG responses to mycobacterial antigens were studied in children living in the UK (an environment low in mycobacteria) who had not received BCG vaccination. Age curves of IgG response to sonicates from different species of mycobacteria were similar suggesting that the greater part of the developing IgG response is to the common antigens shared by all mycobacteria. The major part of the IgG response was to carbohydrate antigens: lipoarabinomannan is a mycobacterial cell wall carbohydrate and was confirmed as a major immunodominant antigen. Infants showed a marked early response to the mycobacterial 65 kilodalton (kDa) and 70 kDa heat shock proteins, but not to the human 65 kDa heat shock protein. The early IgG response to heat shock proteins may reflect cross reactivity to proteins released by a wide variety of bacteria (possibly from breakdown in the gut) or recognition of other immunodominant antigens with high levels of cross reactivity to self.     

Childhood Cutaneous Melanoma

Seventeen consecutive patients no older than 14 years with cutaneous melanoma were observed from 1975 to 1991 at the Istituto Nazionale Tumori, Milan; this series represented 0.8% of all cutaneous melanomas observed at the Institute during this period. Ten were males and seven females. Nine lesions arose on limbs, seven on the trunk, and one on the head and neck area. Reexamination of the clinical records suggests that childhood melanoma may present with unusual characteristics such as unusual appearance of the primary lesion and lack or scarcity of pigmentation. At first observation ten patients had stage I disease nine underwent surgery alone while in one surgery was performed in combination with regional lymph node dissection and chemotherapy. All the stage II patients underwent excision of the primary lesion and lymph node dissection. One of the 2 stage III patients received lymph node dissection only, and the other no treatment. Analysis of this series of 17 cases does not indicate that cutaneous melanoma in children is more aggressive than in adults. It is stressed that, although rare, the physician should be aware that cutaneous melanoma may occur in prepuberty.