Interesting case no. 15. Double median neck cyst

The aim of this study was to assess which patients are referred from general practice, in order to target areas in which our service could be improved. An anonymous postal questionnaire was sent to 433 local general practitioners (GPs), 72.3% (313) of whom replied. The majority of GPs indicated that they would always send patients with a diagnosis of either gonorrhoea or syphilis to the department, while most would send less than 50% of patients with Chlamydia trachomatis infection. Viral infections (i.e. warts and herpes) were referred more frequently than chlamydia. Referral of trichomoniasis was uncommon, as was advice about contacts in all conditions except gonorrhoea and syphilis.     

Mutations in the carboxyl terminus of the agouti protein decrease agouti inhibition of ligand binding to the melanocortin receptors

Several mutations that cause ectopic expression of the agouti gene result in obesity, hyperinsulinemia, and yellow coat color. A candidate pathway for agouti induced obesity and hyperinsulinemia is through altered signaling by melanocortin receptors, as agouti normally regulates coat coloration through antagonism of melanocortin receptor 1. Furthermore, melanocortin peptides mediate functions including steroidogenesis, lipolysis, and thermoregulation. We report apparent inhibition dissociation constants for mouse and human agouti protein inhibition of ligand binding to the melanocortin receptors, to determine which of these receptors might be involved in agouti induced diabetes. The similarity in the apparent K(I) values for agouti inhibition of ligand binding to the brain melanocortin receptors 3 and 4 (mouse: K(I) app = 190 +/- 74 and 54 +/- 18 nM; human: K(I) app = 140 +/- 56 and 70 +/- 18 nM, respectively) suggests that the MC3-R is a potential candidate for a receptor mediating the effects of agouti protein overexpression. Agouti residues important for melanocortin receptor inhibition were identified through the analysis of deletion constructs and site-specific variants. Val83 is important for inhibition of binding to MC1-R (K(I) app for Val83Ala agouti increased 13-fold relative to wild-type protein). Arg85, Pro86, and Pro89 are important for selective inhibition of binding between MC1-R and MC3-R and MC4-R as their apparent K(I) values are essentially unchanged at MC1-R, while they have increased 6-10-fold relative to wild-type protein at MC3-R and MC4-R.     

Midline dorsal appendages

A case of a mature posterior cervical teratoma resembling two fingers and a case of a lumbosacral limb are presented. These are at least the seventh and eighth reported paraspinous or occipital teratomas with recognizable extremity development. Both anomalies had intradural extension and required exploration to decrease the likelihood of future spinal cord tethering. Preoperative evaluation, surgical technique, postoperative follow-up, and review of the literature are presented.     

Asphyxiated bladder syndrome in a pediatric intensive care unit

Sixteen neonates and infants with asphyxiated bladder syndrome (ABS) were prospectively studied over a two-year period. Their transient urinary bladder distention had developed as a sequel of asphyxial events, and presented as delayed micturition in the absence of obstructive lesion. The incidence was 1.9 percent in this Pediatric Intensive Care Unit. Five cases occurred in the neonatal period. All patients were younger than three years of age (mean: 9.1 months), and the incidence of ABS was 2.8 percent within this age group. For intermittent decompression of the bladder, all responded successfully to the suprapubic bladder expression method (Crede's maneuver). Most (81.3%, 13/16) ABS cases coexisted with intestinal dysfunction as either paralytic ileus (62.5%, 10/16) or as gas-less abdomen (18.8%, 3/16). There was no evidence of association with either systemic or urinary bacterial infection. Normal spontaneous voiding and bowel function were regained in all cases except one, who died early. Mean (SD) duration of bladder dysfunction was 66.9 (40.3) hours. Sonography showed normal kidneys and collecting system in all patients both during and after ABS episodes. Half of the patients (8/16) died ultimately. No urinary tract abnormality was found in post-mortem examinations in five cases.     

Phase I-II study of multiple daily fractions for palliation of advanced head and neck malignancies

PURPOSE: To assess palliation of advance head and neck malignancies with the use of rapid hyper fractionation studies similar to the RTOG 85-02. MATERIALS AND METHODS: 37 patients with 39 lesions were entered into the non-randomized Phase I-II protocol, between 1984 and 1991. Previously untreated malignancies were present in 24 lesions, primary recurrent diseases in six patients, metastasis to the head and neck in five patients and skin primaries in the remaining two cases. At presentation 15 of 37 patients (or 17 of 39 lesions) were in operable due to poor medical status, eight patients were considered technically in operable due to extent of disease, 10 patients had distant metastasis and four patients refused surgery. The protocol uses twice a day fraction (370 cGy per fraction) for 2 consecutive days totalling 1,480 cGy per course. Three courses were given at 3-week intervals for a final tumor dose of 4,440 cGy in twelve fraction over 8-9 weeks. RESULTS: Eleven of 39 lesions had complete response; 19 lesions had partial response; 4 lesions had no response; 3 lesions progressed under treatment. Response could not be assessed in two patients. The average survival after completion of therapy was 4.5 months ranging from 2 weeks to 31 months. Palliation was achieved in 33 of 39 lesions. The acute reactions were minimal and no late or long term complications were noted. CONCLUSION: The absence of significant complications with reasonable response in the high rate of palliation suggests that this rapid hyper fractionation palliation study should be studied for further evaluation.     

The organization of leg movements in preterm and full-term infants after term age

In a sample of 13 full-term and 10 preterm infants, the development of kicking movements was studied at 6, 12, and 18 weeks (corrected) age. In healthy full-term infants some characteristics are strikingly stable, such as the duration of the flexion and extension phase and the within-joint organization. These parameters did not differ in preterm compared to full-term infants. For other features, however, developmental changes and differences were observed. Full-term infants tended to decrease their kick frequencies slightly with age. In preterm infants much higher initial kick rates were found, followed by a steep decrease, which resulted in kick frequencies comparable to the full-term levels after the (corrected) age of 12 weeks. There is a tight coupling between the movements in the different joints of the leg in full-term newborns. Preterm infants, in contrast, initially show much lower cross-correlations between hip and ankle and between knee and ankle. This is particularly the case for those preterm infants who were born before 32 weeks gestation. Again, the differences resolved after the age of 12 weeks, which might be related to a transformation in neural functions reported previously around this age. The initial differences in the characteristics of kicking appeared to be more readily explainable by differences in neurological condition than by contrasts in leg volume or postural control.     

Coagulation and fibrinolytic responses of human peritoneal fluid and plasma to bacterial peritonitis

Significantly higher (P < 0.05) thrombin-antithrombin III complex levels were found in the abdominal exudate of patients with peritonitis (median 5500 ng/ml) than in that of controls (median 89 ng/ml). In patients, peritoneal fluid concentrations of tissue and urokinase-type plasminogen activator were increased by factors of 65 and 10 respectively (P < 0.05). The concentration of plasminogen activator inhibitor (PAI) 1 was increased by a factor of about 800 (median 395 versus 0.5 ng/ml, P < 0.05). Despite markedly raised concentrations of PAI, peritoneal fluid displayed fibrinolytic activity as demonstrated by significantly increased (P < 0.05) concentrations of plasmin-alpha 2-antiplasmin complex (median 10,952 versus 57 ng/ml) and fibrin degradation products (median 40,360 versus 126 ng/ml). There was no correlation between plasma and peritoneal fluid concentrations. Intraabdominal coagulation and fibrinolysis are stimulated in the abdominal cavity of patients with bacterial peritonitis.     

Experimental contact of bighorn sheep (Ovis canadensis) with horses and cattle, and comparison of neutrophil sensitivity to Pasteurella haemolytica cytotoxins

Peripheral blood neutrophils from horses, cattle, and Rocky Mountain bighorn sheep (Ovis canadensis canadensis) were evaluated for susceptibility to cytotoxin-dependent lysis of different biotypes and serotypes of Pasteurella haemolytica of domestic sheep, cattle, bighorn sheep, or mountain goat (Oreamnos americana) origin utilizing a cytotoxicity assay which measures the degree of bacteria cytotoxin-killing of neutrophils. All isolates of P. haemolytica (biotypes A and T) were noncytotoxic to horse neutrophils. Thirteen of 18 R haemolytica biotype A isolates were cytotoxic (> 50% neutrophil death in vitro) to bighorn sheep neutrophils, and four of 10 P. haemolytica biotype A isolates were cytotoxic to neutrophils of cattle; P. haemolytica biotype T (= Pasteurella trehelosi) isolates were noncytotoxic to neutrophils of bighorn sheep and cattle. When six bighorn sheep were pastured with three horses, only P. haemolytica biotype T isolates were recovered from the bighorn sheep throughout the study; Pasteurella spp. organisms were not isolated from the three horses. At initiation of a study where five bighorn sheep were pastured with three cattle, P. haemolytica biotype A, serotype 1, 2 was isolated from all three cattle, and only P. haemolytica biotype T isolates were recovered from the bighorn sheep. One bighorn sheep died in each of the horse and cattle copasturing experiments. Pasteurella haemolytica was not isolated from the bighorn sheep which died in the horse copasturing experiment. A noncytotoxic P. haemolytica biotype A, serotype 2 was isolated at necropsy from the bighorn which died in the cattle contact experiment. Based on these experiments, we believe bighorn sheep and horse association would not be detrimental to bighorns due to P. haemolytica induced pneumonia.