Pharyngeal diverticula

A series of 73 hypopharyngeal (Zenker's) diverticula is reported and the choice of treatment and technique of excision are discussed. The problems of excision include operation on an infected site with limited access but few patients are unfit for the procedure. Preoperatively the sac should be packed and the oesophagus stented. The side of approach is unimportant as the condition is essentially midline. Cricopharyngeal myotomy appears to reduce the recurrence rate, possibly by improving coordination of pharyngeal contraction and upper oesophageal relaxation. The use of electrocoagulation is not recommended as 20% of patients require repeated general anaesthesia and the procedure fails in 13%. For all but the smallest pouches, excision is the treatment of choice.     

Sports-related pads

Knuckle pads, first described by Garrod in 1893, 1 are benign, asymptomatic, well- circumscribed, smooth, firm, skin colored papules, nodules, or plaques. They most commonly occur on the dorsal aspect of the proximal interphalangeal joint of the finger, 2 but also may occur on the dorsal aspects of the foot over joints. 3-5 Knuckle pads may be inherited or acquired. 1 While some authors suggest that trauma is not a significant factor, 6 acquired knuckle pads have been associated with repetitive friction and trauma, 2,7 and may resemble athlete's nodules (also referred to as collagenomas). 1 Histologically, knuckle pads are characterized by hyperkeratosis, hypergranulosis, proliferation of fibroblasts and capillaries, and thickened and irregular collagen bundles. 1 Few cases of knuckle pads involving the lower extremities have been reported. 7 Knuckle pads of the feet have been described in association with inherited syndromes, such as acrokeratoelastoidosis Costa, 3 a syndrome of knuckle pads, leukonychia and deafness, 4 and a syndrome of knuckle pads, leukonychia, deafness and keratosis palmoplantaris. 5 We report a case that illustrates an unusual presentation of acquired knuckle pads of the feet secondary to repetitive friction from athletic gear.     

Gastroschisis: Are prenatal ultrasonographic findings useful for assessing the prognosis?

Objective. The objective of this study was to assess various prenatal patterns in correlation with survival and the occurrence of complications of antenatally recognized gastroschisis (G).Materials and methods. We retrospectively studied 34 cases of G. Mortality and morbidity in the postnatal period were assessed and correlated with the prenatal presence or absence of: (1) bowel and/or stomach dilatation, (2) thickening and/or hyperechogenicity of the intestinal wall, (3) meconium peritonitis (in the abdomen) before 20 weeks of gestation, (4) asymmetrical bowel dilatation, and (5) associated malformations. Morbidity took into account the length of hospitalization and the number of surgical procedures.Results. The overall survival rate was 94 %. Neither bowel nor stomach dilatation was significantly correlated with mortality. However, evidence of intestinal dilatation greater than 17 mm had a positive predictive value of 67 % for atresia, with a negative predictive value of 86 %. Thickening and/or hyperechogenicity of the bowel wall were not significantly associated with mortality. Meconium peritonitis before 20 weeks and asymmetrical bowel dilatation were not statistically significant because of the small sample size. Twelve patients (35.3 %) had postnatal complications, with a mean hospital stay of 127 days. Outcome was not modified by the mode of delivery. Associated extradigestive anomalies were present in 20.6 % of cases. Chromosomal anomalies were not seen.Conclusion. The prognosis of prenatally detected G is excellent despite the frequency of small bowel atresia (67 %) in the group with postnatal complications. Meconium peritonitis before 20 weeks of gestation and/ or asymmetrical bowel dilatation also appear to be indicators of atresia (2/4, 50 %) or high morbidity (3/4,75%).     

Accelerated pubertal development in patients with shunted hydrocephalus

OBJECTIVE: To evaluate pubertal development and peripheral concentrations of gonadotrophins and sex hormones in children with shunted hydrocephalus compared with healthy controls. STUDY DESIGN: 114 patients (52 females, 62 males) and 73 healthy controls (35 females, 38 males) aged 5 to 20 years were analysed for stage of puberty, age at menarche, testicular volume, basal serum follicle stimulating hormone (FSH), luteinising hormone (LH), sex hormone binding globulin (SHBG), testosterone and oestradiol concentrations, and free androgen index. RESULTS: Male gonadal and male and female pubic hair development occurred significantly earlier in the patients than in the controls. The mean age at menarche was significantly lower in the female patients than in their controls (11.7 v 13.2 years; p < 0.001), and lower than it had been for their mothers (v 13.1 years; p < 0.001). Relative testicular volume was higher in the male patients than in their controls (1.2 standard deviation score (SDS) v 0.2 SDS; p < 0.001). The prepubertal patients had higher basal LH (0.13 U/l v 0.08 U/l; p < 0.001) and SHBG (132.3 nmol/l v 109.1 nmol/l; p < 0.01) than the controls. Both the prepubertal and pubertal females had significantly higher basal FSH than their controls (1.57 U/l v 1.03 U/l; p < 0.05, and 4.0 U/l v 2.9 U/l; p < 0.01, respectively). CONCLUSIONS: Hydrocephalic children experience accelerated pubertal maturation, reflected in a younger age at menarche in females and an increased testicular volume in males. This may be because of enhanced gonadotrophin secretion, possibly resulting from unphysiological variations in intracranial pressure.     

Serum profiles of insulin-like growth factors and their binding proteins in adults with growth hormone receptor deficiency treated with insulin like growth factor I

Six adult patients with growth hormone receptor deficiency (GHRD) (2 men, 4 women) with an identical defect in the growth hormone receptor (GHR) gene, were treated with recombinant human insulin-like growth factor I (IGF-I), 40 μgikg S.C. twice daily, for 7 days. Serum concentrations of IGF peptide and IGF binding protein-3 (IGFBP-3) were measured by specific radioimmunoassays; serum IGFBPs were also measured by Western ligand blotting. The size distribution of both IGF-I and IGF-II was measured in serum following size-exclusion fast-performance liquid chromatography. IGF-I treatment resulted in a normalization of serum IGF-I levels on days 1–7 of treatment and a decrease in serum IGF-II levels. The fall in IGF-II levels and the simultaneous rise in IGF-I levels, however, resulted in an unchanged total serum IGF level. The low IGFBP-3 values did not significantly change during treatment, whereas there was a slight increase in IGFBP-2 levels. Preliminary analysis of size-fractionated sera suggested an increase in IGF-I levels in the 40 and 150 kDa regions at the expense of IGF-II levels. The results suggest that despite the failure of IGF-I treatment to increase IGFBPs significantly, serum IGFBP concentrations were sufficient to maintain normal levels of IGF-I. 0 Laron syndrome, growth hormone receptor deficiency, insulin-like growth factors, insulin-like growth factor binding protein     

Phase I and pharmacologic study of CT-2584 HMS, a modulator of phosphatidic acid, in adult patients with solid tumours

CT-2584 HMS, 1-(11-dodecylamino-10-hydroxyundecyl)-3, 7-dimethylxanthine-hydrogen methanesulphonate, is a modulator of intracellular phosphatidic acid. We treated 30 patients as part of a Phase I and pharmacokinetic study to determine the maximum-tolerated dose of CT-2584 HMS, toxicity profiles, pharmacokinetic profile and antitumour effects at escalating dose levels. CT-2584 HMS was given as a continuous infusion for 6 hours for 5 consecutive days every 3 weeks. Plasma samples for pharmacokinetic studies were analysed using a validated high-performance liquid chromatographic assay. Mean C(max)and AUC values for each dose group were similar on days 1 and 5 and increases in plasma concentration (C(max)and AUC) appeared proportional to the dose. CT-2584 HMS had a mean elimination half-life of 7.3 hours. Values of V(d)and clearance were independent of dose and duration of treatment. Dose escalation was halted at 585 mg/m(2)because of malaise and lethargy, which was sometimes accompanied by nausea and headache. 26 patients were evaluable for response, one patient with pleural mesothelioma achieved a partial response to treatment confirmed by CT scanning. A dose level of 520 mg/m(2)daily x 5 days would be suitable for Phase II testing. Alternative schedules of CT-2584 HMS to overcome the limiting toxicity of malaise would be worthy of examination.