Squamous cell carcinoma in long-standing untreated lichen sclerosus et atrophicus of the penis

Lichen sclerosus et atrophicus is an uncommon disease of skin and genital mucosa in both sexes. Involvement of the male genital mucosa is usually complicated by recurrent balanoposthitis, ulceration, phimosis, and meatal stenosis. The development of a squamous cell carcinoma is extremely rare in genital lichen sclerosus et atrophicus in males as compared with females. We report a 70-year-old male with a squamous cell carcinoma of the glans superimposed on long-standing lichen sclerosus et atrophicus of glans and prepuce. The patient in addition had a basal cell carcinoma on his face. Awareness of this rare complication of lichen sclerosus et atrophicus in males is emphasized.     

Necrotising fasciitis of the neck

Necrotising fasciitis is a fulminant soft tissue infection that causes necrosis of fascia and subcutaneous tissue while sparing skin and muscle initially. It is most commonly seen in adults involving the perineum, extremities and and minal wall. Immunncompromised patients are at an increased risk of developing this infection. These infections require early diagnosis, aggressive surgical debridaient and appropriate antibiotic therapy. Mortality rates have been reported to be as high as 52 and 73% in general surgery literature.(Freuschtag et al, 1985., Rouse et at 1982). Necrotising fasciitis of the head and neck is rare. The commonest cause is secondary to denial infections. We report a case of necrotising fasciitis of the neck secondary to a peritonsillar abscess in a previously healthy individual. The pathogenesis and treatment of this fulminant infection are also discussed.     

Interstitial fluid pressure in intracranial tumours in patients and in rodents.

Fluid transport parameters in intracranial tumours influence the delivery of therapeutic agents and the resolution of peritumoral oedema. The tumour and cortex interstitial fluid pressure (IFP) and the cerebrospinal fluid pressure (CSFP) were measured during the growth of brain and pial surface tumours [R3230AC mammary adenocarcinoma (R3230AC) and F98 glioma (F98)] in rats. Intratumoral and intracranial pressures were also measured in rodents and patients treated with dexamethasone, mannitol and furosemide (DMF), and hypocapnia. The results show that (1) for the R3230AC on the pial surface, IFP increased with tumour volume and CSFP increased exponentially for tumours occupying a brain volume of 5% or greater; (2) in F98 with volumes of approximately 10 mm3, IFP decreased from the tumour to the cortex, whereas for tumour volumes > 16 mm3 IFP equilibrates between F98 and the cortex; (3) DMF treatment reduced the IFP of intraparenchymal tumours significantly and induced a pressure gradient from the tumour to the cortex; and (4) in 11 patients with intracranial tumours, the mean IFP was 2.0 +/- 2.5 mmHg. In conclusion, the IFP gradient between intraparenchymal tumours and the cortex decreases with tumour growth, and treatment with DMF can increase the pressure difference between the tumour and surrounding brain. The results also suggest that antioedema therapy in patients with brain tumours is responsible in part for the low tumour IFP.     

Effects of pH and Dose on Nasal Absorption of Scopolamine Hydrobromide in Human Subjects

Purpose. The present study was conducted to evaluate theeffects of formulation pH and dose on nasal absorption of scopolaminehydrobromide, the single most effective drug available for the prevention ofnausea and vomiting induced by motion sickness. Methods. Human subjects received scopolamine nasally at adose of 0.2 mg/0.05 mL or 0.4 mg/0.10 mL, blood samples were collected atdifferent time points, and plasma scopolamine concentrations were determinedby LC-MS/MS. Results. Following administration of a 0.2 mg dose, theaverage C_max values were found to be 262 ± 118, 419± 161, and 488 ± 331 pg/mL for pH 4.0, 7.0, and 9.0formulations, respectively. At the 0.4 mg dose the average C_maxvalues were found to be 503 ± 199, 933 ± 449, and 1,308± 473 pg/mL for pH 4.0, 7.0, and 9.0 formulations, respectively. At a0.2 mg dose, the AUC values were found to be 23,208 ± 6,824, 29,145± 9,225, and 25,721 ± 5,294 pg.min/mL for formulation pH 4.0,7.0, and 9.0, respectively. At a 0.4 mg dose, the average AUC value wasfound to be high for pH 9.0 formulation (70,740 ± 29,381 pg.min/mL)as compared to those of pH 4.0 (59,573 ± 13,700 pg.min/mL) and pH 7.0(55,298 ± 17,305 pg.min/mL) formulations. Both the C_maxand AUC values were almost doubled with doubling the dose. On the otherhand, the average T_max values decreased linearly with a decreasein formulation pH at both doses. For example, at a 0.4 mg dose, the averageT_max values were 26.7 ± 5.8, 15.0 ± 10.0, and 8.8± 2.5 minutes at formulation pH 4.0, 7.0, and 9.0, respectively. Conclusions. Nasal absorption of scopolamine hydrobromidein human subjects increased substantially with increases in formulation pHand dose.     

Effect of pharyngeal stimulation on the motor function of the esophagus and its sphincters

OBJECTIVE/HYPOTHESIS: Sensory impulses from the pharynx induce contraction of the upper esophageal sphincter (UES), relaxation of the lower esophageal sphincter (LES), and inhibition of peristalsis. To determine 1) the magnitude of UES contractile response to threshold volume of fluid that induces LES relaxation and 2) the effect of rapid pharyngeal air stimulation on LES resting pressure and its concurrent influence on the UES and progression of esophageal peristalsis. METHODS: Eleven healthy volunteers (age, 31 +/- 2 y) were studied by concurrent UES, esophagealbody, and LES manometry. RESULTS: At a threshold volume of 0.3 +/- 0.05 mL, injections of water into the pharynx directed posteriorly, resulted in complete LES relaxation. Duration of these relaxations averaged 19 +/- 1 seconds. In 10 of 11 subjects, these relaxations were accompanied by a simultaneous increase in UES resting tone that averaged 142% +/- 27% above preinjection values. Pharyngeal stimulation by rapid air injection resulted in complete LES relaxation in 8 of the 11 subjects (threshold volume, 14 +/- 6 mL). Five of 8 developed a concurrent mild increase in resting UES pressure (17% +/- 6% above preinjection values) (P < .05). Pharyngeal water injection inhibited the progression of the peristaltic pressure wave at all tested sites and in all subjects, but pharyngeal air injection in only 2 of the 11 studied subjects. CONCLUSIONS: The inhibitory effect of pharyngeal water injection on LES resting pressure is accompanied by a substantial contractile effect on the UES. Although stimulation of the pharynx by rapid air injection may induce LES relaxation, its inhibitory effect on esophageal peristalsis and stimulatory effect on UES pressure are negligible compared with that of water injection.     

Maternal estimates of mental age in developmental assessment

Objective: To examine the accuracy and clinical utility of maternal estimates of mental age in young children referred for developmental assessment.Methods : Mothers of 100 children aged 16 to 60 months referred for developmental evaluation to psychology services of Department of Pediatrics of a tertiary care teaching hospital were asked to estimate the mental age of their child. Maternal estimates were converted to intelligence quotient (IQ) and were compared to results from developmental tests of cognitive and adaptive behavior functioning which were administered to all children.Results : Maternal estimate IQ was highly correlated with IQ calculated from Developmental Profile II (r=.83, p&lt;.001) and social quotient (SQ) calculated from Vineland Social Maturity Scale (r=.81, p&lt;.001). Maternal estimate IQ was 82% sensitive to cognitive delay and 81% specific in identifying children likely to have normal development. Twenty seven percent of the maternal estimates were within ±5 IQ points of actual IQ. Mothers were more likely to overestimate their child’s functioning. Maternal IQ (Mean=62.1, S.D. =25.8) was significantly higher (t=2.93, p&lt;.004) than the actual IQ (Mean=57.9, S.D.=21.9). Step-wise multiple regression analysis revealed that the child’s IQ and SQ explained 10% of the variance (F=6.40, p&lt;.001) in maternal accuracy. The lower the SQ and IQ of the child, more accurate the estimates.Conclusion : Maternal estimates of mental age provide an accurate measure of developmental functioning in young children and may be used as a screening technique to identify a subset of children who need more detailed evaluation.     

Dysmorphology diagnosis

A patient with multiple malformations poses a diagnostic dilemma to the pediatrician. There are thousands of malformation syndromes described and diagnosis of a syndrome appears a daunting task. An approach to diagnosis of a malformation syndrome is presented. Relevant details in the history and examination, important investigations, the process of differential diagnosis, and search engines used to aid in diagnosis of a malformation syndrome are described