Dyggve–Melchior–Clausen Syndrome.

Dyggve–Melchior–Clausen syndrome is a rare autosomal-recessive disorder, characterized by progressive spondylo-epi-metaphyseal dysplasia associated with mental retardation. The clinical and radiological findings resembles Morquio disease at the onset of condition, which may hinder its diagnosis. Two siblings with chatacteristic clinical (progressive postnatal dwarfism and mental retardation) and radiological features (irregular lace-like appearance of the iliac crests) are reported.

Celiac Disease Associated with Recurrent Guillain Barré Syndrome.

Celiac disease is associated with multiple extraintestinal presentations, including bone disease, endocrine disorders and neurological deficits. We report a 9 year old girl with celiac disease presenting with recurrent Guillain Barre syndrome (third episode). There was no other clinical manifestation except for refractory iron deficiency anemia. Molecular mimicry explaining the association between these two disorders, is far more interesting.

Hábitos alimentarios y experiencia de caries en adultos jóvenes en Rosario Argentina / Food habits and caries experience in young adult from Rosario Argentina

En Rosario la atención odontológica se brinda en ámbitos privados y públicos. Los servicios públicos carecen de programas para evitar la pérdida dentaria en adultos. Nuestro objetivo fue describir el perfil epidemiológico de experiencia de caries y hábitos dietéticos en tres sub-poblaciones de adultos jóvenes. Aplicando una anamnesis estandarizada se investigaron 517 pacientes, ambos sexos, 15-45 años, concurrentes a un servicio privado (19 por ciento) y dos públicos (81 por ciento), uno céntrico y otro marginal. Variables investigadas: consumo de lácteos y alimentos dulces, estado bucal y escolaridad. Se aplicaron pruebas estadísticas t de student, ANOVA y chi-cuadrado. Promedios hallados: cariados 1,7 ±2,0 en privado, 4,1±2,8 en públicos (p=0.0001); ausentes 5,4±5,2 en privado, 6,8±5,4 en públicos (p=0.04); obturados 4,4±4,2 en privado, 1,3±2,2 en públicos (p<0.0001). Nivel de escolaridad: superior en el privado (p<0.0001). Consumo diario de lácteos: mayoría en privados (p<0.001). En los servicios públicos los pacientes reemplazan la leche por el mate y relacionan su ingesta con la gravidez, lactancia o la dejan para los niños. Tipos de golosinas o dulces de riesgo consumidos: en el sector privado fueron panificados tipo facturas, tortas, galletitas y dulces, y en los públicos caramelos blandos, alfajores y dulce de leche. El consumo de alimentos dulces de menor riesgo fue de helados, pastillas, gaseosas y chocolates en los privados y de caramelos duros y chicles con y sin azúcar en los públicos. Si bien un alto número de personas los ingieren a cualquier hora, los pacientes privados fueron minoría, mientras que en su consumo sólo después de una comida éstos fueron mayoría.

Prevalence of dental caries and treatment needs in the school-going children of rural areas in Udaipur district.

Dental caries is among the most common dental problems affecting humans. This condition is a major health problem with high prevalence, globally involving the people of all regions and society. This study was conducted on 1587 government school children of Udaipur district in the age group of 5-14 years for recording the prevalence of dental caries and treatment needs. Dental caries was found in 46.75% children, and 76.87% children required some kind of dental treatment.

Prevalence of gingival diseases, malocclusion and fluorosis in school-going children of rural areas in Udaipur district.

High prevalence of dental diseases has been recorded in Rajasthan, however, not much work has been done to ascertain the prevalence of dental diseases in Udaipur district. This study was conducted among 1,587 government school children of Udaipur district in the age group of 5-14 years for recording the prevalence of gingival diseases, fluorosis and malocclusion. Gingivitis was found in 84.37% of children, malocclusion in 36.42% and fluorosis in 36.36%.

Pericardiectomy for constrictive pericarditis: is prolonged inotropic support a bad omen?

Sixty-seven patients who underwent pericardiectomy for constrictive pericarditis at JIPMER, Pondicherry between 1987 and 1998 were the subjects of the study. Pre-operatively 70% of cases were in the New York Heart Association classes III and IV categories with clinical signs suggestive of constriction ie, raised jugular venous pressure in 99%, pleural effusion in 77%, pedal oedema in 61% and ascites in 55% of the cases. Seventy-five per cent of the cases underwent pericardiectomy through a median sternotomy and the rest via left anterolateral thoracotomy. Low cardiac output was evidenced in 70% of cases postoperatively which was managed by early institution and prolonged use of inotropes. There was 9% mortality especially in the early part of the experience. Tuberculous pathology was confirmed histologically in 57% cases. Sixty-three per cent of cases are presenting in follow-up in New York Heart Association class I. Prolonged use of inotropes instituted early in postoperative period is recommended to prevent postoperative ventricular dysfunction with adrenaline being the preferred inotrope. It is concluded that postoperative New York Heart Association class and long term survival were not significantly influenced by pre-operative New York Heart Association class, operative approach or peri-operative low cardiac output syndrome requiring prolonged inotropic support.

Specificity of drug transport mediated by CaMDR1: a major facilitator of Candida albicans.

CaMDR1 encodes a major facilitator superfamily (MFS) protein in Candida albicans whose expression has been linked to azole resistance and which is frequently encountered in this human pathogenic yeast. In this report we have overexpressed CaMdr1p in Sf9 insect cells and demonstrated for the first time that it can mediate methotrexate (MTX) and fluconazole (FLC) transport. MTX appeared to be a better substrate for CaMdr1p among these two tested drugs. Due to severe toxicity of these drugs to insect cells, further characterization of CaMdr1p as a drug transporter could not be done with this system. Therefore, as an alternative, CaMdr1p and Cdr1p, which is an ABC protein (ATP binding cassette) also involved in azole resistance in C. albicans, were independently expressed in a common hypersensitive host JG436 of Saccharomyces cerevisiae. This allowed a better comparison between the functionality of the two export pumps. We observed that while both FLC and MTX are effluxed by CaMdr1p, MTX appeared to be a poor substrate for Cdr1p. JG436 cells expressing Cdr1p thus conferred resistance to other antifungal drugs but remained hypersensitive to MTX. Since MTX is preferentially transported by CaMdr1p, it can be used for studying the function of this MFS protein.

Study of head injury victims in fatal road traffic accidents in Delhi.

The study revealed 31% were the victims of Head injury injury in this part of Delhi in vehicular accidents. Although majority of the fatalities were on the spot quite a number of such victims survived for a varied period. A multipronged approach including the preventive and curative measures for this man-made calamity is the need of the hour. Amongst various preventive measure are wearing helmet be made compulsory for both the driver and pillion riders of two-wheelers, development of a safe traffic sense amongst the road users. Traffic Police should be honest, exemplary punishment for the reckless driver, decongestion of Delhi roads etc. The curative measures are facilities of CT scanners and neurosurgeons in all the major hospitals, provision of first-aid in the PCR (Police control room) vans, to institute immediate treatment to the victim without waiting for the medico-legal formalities, opening of more fully equipped Accident and trauma centres. It is high time the concerned authorities gave some serious thought towards this avoidable epidemic on Delhi roads.

In vivo localized proton magnetic resonance spectroscopy of intracranial tuberculomas.

In vivo proton magnetic resonance spectroscopy (MRS) was performed in ten patients with intracranial tuberculomas with the aim of finding biochemical finger prints which may help in the noninvasive diagnosis of the disease. Diagnosis of tuberculoma was confirmed in all these cases retrospectively. Ex vivo spectroscopy of formalin fixed samples of four granulomas and a normal brain (cerebellar) tissue, was performed to confirm the in vivo resonances. The in vivo study showed resonance at 1.3 ppm and 0.9 ppm assigned to methylene group (CH2)n and terminal methyl groups (-CH3) of fatty acids respectively which was subsequently confirmed by the ex vivo study. Large resonances of fatty acids in in vivo study appeared to be due to high lipid content of caseous material. It is concluded that in vivo proton spectroscopy may be helpful in differentiating tuberculomas from other intracranial mass lesions which have diagnostic difficulties on MR imaging.

Secondary involvement of femur in carcinoma of gall bladder.

A case with bony metastasis to right femur from a primary carcinoma of gall bladder is described. This is probably one of the most rare presentation of the disease without producing any signs or symptoms suggestive of a primary pathology in gall bladder.

Hyperkalemic periodic paralysis associated with thyrotoxicosis.

A 32 year old male presented with episodic pure motor weakness for 1 1/2 months. On evaluation he was found to be thyrotoxic. Hyperkalemic challenge test provoked similar weakness with raised serum potassium (6 meq/L). He responded to treatment with neomercazole. Till he became euthyroid, he responded to the addition of acetazolamide to his medication. He is symptom free on antithyroid drug alone over 8 months of follow up.

Transient achalasia of esophagus in tetanus.

A case with tetanus presenting with progressive dysphagia due to lower esophageal dysfunction is described. A barium swallow at the onset of symptoms showed esophageal dilatation with a smooth tapering at the lower end suggestive of achalasia cardia. The patient recovered from tetanus over the ensuing three weeks; repeat barium swallow at this time was normal, suggesting that esophageal dysfunction was a manifestation of tetanus.