Turner syndrome with pseudohypoparathyroidism: A case report / Journal of the ASEAN Federation of Endocrine Societies

@#<p style="text-align: justify;">The association of Pseudohypoparathyroidism (PHP) with Turner syndrome is very rare and only a single case has been reported so far. Both manifest with short stature and lack of secondary sexual characteristics along with other stigmata similar to each other, creating a diagnostic dilemma.<br />We describe a case of a 15-year-old Asian Indian female who presented with short stature and delayed puberty with overlapping phenotype of PHP and Turner syndrome. The diagnosis of Turner syndrome was made easily on the basis of typical history, clinical features and karyotype but the diagnosis of PHP was suspected only after radiological and biochemical investigations. The association of Turner syndrome with PHP can be easily missed due to similar phenotypes and subtle manifestations.</p>

Morquio’s Syndrome: A Rare Case.

Morquio’s syndrome is an autosomal recessive disorder due to deficiency of N-acetylgalactosamine-6 sulfate. Presented here is the case of a 4-year-old girl with complaints of deformity of bilateral lower limbs since 6 months. Examination revealed widely spaced teeth, corneal opacity, pectus carinatum, bilateral genu valgum and flat feet. X-rays of femur showed minimal metaphyseal widening, widened carpal bones and pointing metacarpals and beaking of the thoracic and lumbar vertebrae. She was diagnosed to be suffering from Morquio’s syndrome. Mucopolysaccharidoses (MPS) are a rare group of metabolic disorders due to deficiency of enzymes responsible for degradation of glycosaminoglycans. Treatment is mainly symptomatic.

Role of combining colour doppler and grey scale ultrasound in characterizing adnexal masses

To evaluate the adnexal masses with conventional gray scale and color Doppler flow imaging and to assess their diagnostic reliability to differentiate benign and malignant adnexal masses. We evaluated 30 patients with adnexal mass. Morphological characterization of the mass was done using Sassone score. Color Doppler parameters noted down in each patient and Caruso vascular score was also used. The results were compared with surgical/ pathological and/or follow up scans. Using sonomorphological score [Sassone] overall reliability of differentiating adnexal masses had sensitivity of 91.7% and specificity of 77.7%. Using Caruso score alone we had sensitivity of 83.3% and specificity of 88.9%. Using Sassone and Caruso score together we had sensitivity of 90.9% and specificity of 93.3%. In evaluation of adnexal masses combining both sonomorphological and color Doppler scores which gave higher specificity and positive predictive value [PPV] than using individual score alone

Ultrasound confirmation of laryngeal mask airway placement correlates with fiberoptic laryngoscope findings

Correct placement of a laryngeal mask airway [LMA] requires confirmation to appreciate the adequacy of laryngeal seal and pulmonary ventilation. The present study was designed to assess the feasibility of ultrasound use for confirmation of correct placement of LMA and its correlation with fiberoptic laryngoscopy as a confirmation tool for LMA position. 31 ASA I and II patients scheduled for same day surgery under general anesthesia underwent standard general anesthetic technique with AuraOnce[TM] or AuraFlex[TM] Disposable Laryngeal Mask Airways. The position of the LMA cuff was confirmed by transverse neck ultrasound [USG], and reconfirmed with intra-LMA fiberoptic laryngoscopy [FOL]. The ultrasound grade of LMA position strongly correlated positively with the fiberoptic grade of LMA position [r=0.92; p<0.0001]. This correlation was obtained immediately after LMA placement, as well as just before LMA removal. The Bland-Altman scatter plot showed insignificant differences between the two grading systems with small and good limits of agreement [-0.63 to +0.57]. Ultrasound examination can replace fiberoptic examination for confirmation of the correct placement of an LMA. Additionally, non-invasive ultrasound examination can further give insight into the cause of airway/ventilation events that may be interfering with the LMA placement and ventilation

Pediatric cancer pain management at a regional cancer center: implementation of who analgesic ladder

collect data on the prevalence of various types of cancer pain in a sample of children with cancer, and to implement the WHO Analgesic Ladder in the management of pain in pediatric cancer. Eighty four pediatric patients suffering of cancer pain were studied during the period 2001-2006. Patients were requested to rate their global intensity of pain on 0-100 mm visual analogue scale [VAS 0 = no pain 100 = maximum pain]. Pain management was performed in accordance with the WHO Analgesic Ladder for cancer pain. Patients were followed up weekly for three weeks. Of the 84 pediatric children with cancer, pain was nociceptive in 26 [31%], neuropathic in 12 [14.3%] and mixed in 46 [54.8%]. Almost 7 [8.3%] of patients were on WHO step 3 at baseline. Thereafter the WHO step 3 increased; first week visit 36 [43%] patients; second week visit 58[69%], and third week 69 [82.1%]. At baseline, 40 [47.6%] patients took NSAID only, 2 [2.4%] patients took adjuvant, while 38 [45.2%] patients took combination of NSAID and adjuvant treatment. There was statistically significant [p = 0.000] reduction in VAS as time progressed. Cancer pain in pediatric age group can be well managed in accordance with the WHO Analgesic Ladder. Aggressive symptoms and control of treatment of related side effect are also needed to ensure successful implementation and the WHO Analgesic Ladder

rate dependent bundle branch block -transition from left bundle branch block to intraoperative normal sinus rhythm

A chronic hypertensive patient with electrocardiogram [ECG] showing left bundle branch block [LBBB] was given general anesthesia for right modified radical mastectomy. Her ECG reverted to normal sinus rhythm intermittently during peri-operative period. This intermittent ratedependent LBBB is a rare entity. Though hypertension is one significant co-morbid condition, the risk evalution of LBBB during anesthesia only on an ECG finding, is not justifiable. Rather patient should be investigated further for any cardiac risk