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Biomedical subjects

A Kher

Publications and source records attributed to A Kher.

106 records · Page 6Linked to original sources

Asymmetric crying facies: the cardiofacial syndrome.

A 5 1/2 month old female child presented with cyanosis and recurrent respiratory infection. Asymmetric facies was noticed on crying. On investigation an ostium secundum atrial septal defect with right partial anomalous pulmonary venous connection and severe pulmonary hypertension was detected. The association of asymmetric crying facies with congenital cardiac anomalies, known as cardiofacial syndrome, is presented here to highlight the use of this clinical sign as a marker to congenital anomalies.

Cyanosis↗

Wildervanck syndrome (cervico-oculo-acoustic syndrome).

Wildervanck syndrome i.e. cervico (Klippel-Feil anomalad) -oculo (Duane-Stilling-Turk phenomenon with bilateral abducens palsy)-acoustic (deafness) is a rare syndrome. We report here 4 cases diagnosed as Wildervanck syndrome and analyse their findings. One patient had an an atrial septal defect. Such association of congenital heart disease with Wildervanck syndrome has not been reported previously.

Abnormalities, Multiple↗

A rare cause of steatohepatitis.

We present a rare case of steatohepatitis due to neutral lipid storage disorder in a 1.5-year-old male presenting with intermittent fever, hepatomegaly and dark-coloured urine. On examination, there was ichthyosis involving both the limbs. Liver biopsy showed steatohepatitis. The peripheral blood smear revealed fat vacuoles in the cytoplasm of leucocytes, characteristic of the Dorfman-Chanarin syndrome. Awareness of this condition helps in prompt diagnosis and avoids unnecessary further investigations.

Fatty Liver↗

[Treatment of deep venous thrombosis. Comparative study of a low molecular weight heparin fragment (Fragmin) by the subcutaneous route and standard heparin by the continuous intravenous route. A multicenter study].

This open, randomised multicenter trial compares the efficacy and safety of Fragmin administered subcutaneously twice daily with standard heparin administered by continuous infusion in the treatment of deep vein thrombosis (DVT). The initial dose of Fragmin is 100 U anti-Xa/kg/12 h and the further doses are adjusted according to the anti-Xa activity between 0.5 and 0.8 U/ml, 3 hours after the morning injection. The initial dose of standard heparin is 240 UI/kg/12 h. The dose adjustments are based on the daily results of APTT (1.5 - 3 times the control). Treatments efficacy are appreciated when comparing the venography performed before and after 10 days of treatment. The safety is evaluated on clinical parameters and iterative biological tests. Sixty-six patients have been included in this study. Efficacy of the two treatments is equivalent with a phlebographic improvement in respectively 79.3 p. 100 (Heparin Group) and 71.0 p. 100 (Fragmin Group) of the cases and an aggravation in 3.4 p. 100 and 6.4 p. 100 (NS) respectively. The frequency of dosage adjustments is lower and the stability of biological tests is better in the Fragmin group. In conclusion, the administration of Fragmin twice daily by subcutaneous route seems to be equivalent at least to standard heparin continuous infusion in the treatment of recent DVT. The better convenience and safety of Fragmin have to be verified on a larger panel of patients.

Adult↗

[Writing the clinical trial report].

The clinical study report is a key document, representing as it does one of the elements of the drug registration file. The authors propose here a general design for a controlled trial which offers the advantage of a methodical approach covering most clinical study situations.

Clinical Trials as Topic↗

[Arterial wall: a new pharmacological and therapeutic target?].

In recent years, endothelial dysfunction and arterial remodelling in various cardiovascular diseases have emerged as two key concepts, with numerous interrelationships. Both endothelial dysfunction and arterial remodelling occur in various pathologies including heart failure, atherosclerosis, restenosis after angioplasty, and pulmonary hypertension, and have modified the therapeutic approach by offering new pharmacological targets: specific receptors not only at the site of the vascular smooth muscle cells but also on the endothelial cells, growth factors that stimulate proliferation of smooth muscle, and receptors and enzymes of the extracellular matrix. Among the various substances under research, the present review will discuss angiotensin II receptor antagonists, endothelin receptor antagonists, nitrates-NO donors, potassium channel activators, and substances interfering with proteoglycans and other components of the extracellular matrix.

Animals↗

Massive haemoptysis controlled by bronchial artery embolisation: a case report.

Massive haemoptysis results in a high rate of mortality and morbidity. It is infrequently seen in children. Medical and surgical interventions are not very effective in controlling torrential bleeding. Therefore, newer modalities like bronchial artery embolisation are found to be promising in life threatening situations in both adults and children.

Bronchial Arteries↗

The Proteus syndrome.

A race case of Proteus syndrome is presented. The main features of this hamartomatous condition are partial gigantism of hands and feet, hemihypertrophy, subcutaneous masses, epidermal nevi and bony abnormalities. The condition is extremely rare. Though the child had severe cosmetic disability, motor intellectual and language development was found to be normal.

Aftercare↗

Pseudoachondroplastic dysplasia.

Pseudoachondroplasia is a heterogeneous inherited skeletal dysplasia in which dwarfism is a major feature. We report here a case of a 7 year old girl misdiagnosed as rickets, who presented with short stature, lordosis, genu varum and flexion deformities at both the elbows. Skeletal survey revealed epiphyseal and metaphyseal irregularities. A review of literature is also presented.

Achondroplasia↗

[Difficulties of assessing postoperative pulmonary embolisms. Risks of meta-analysis from clinical trials].

During the 1970's, several large clinical trials have shown low dose subcutaneous heparin to be effective in the prevention of post-operative lethal pulmonary embolism. These claims have been widely accepted mainly because the selected main criterion was effectively pulmonary embolism. To-date, this criterion appears to be poorly reliable clinically and ethically unacceptable due to lethal risk. In practically all recent trials with low molecular weight heparin fractions in this indication, the main criterion has been prevention of deep vein thrombosis and not of pulmonary embolism. This is why, among other reasons exposed in this study, it would be hazardous to perform any meta-analysis related to pulmonary embolism from trials which have not been designed for this purpose.

Drug Evaluation↗