Abdominal cocoon in children: a report of four cases

Abdominal cocoon is a rare cause of intestinal obstruction. The authors report four cases (3 boys, 1 girl; age range, 6 to 8 years) that presented with features of intestinal obstruction. There was no history of previous surgery, peritonitis, or prolonged drug intake in any of these cases. One patient presented with acute intestinal obstruction and gangrene of bowel. The etiology, preoperative diagnosis, and management of this condition are discussed.     

Dog-bite induced sepsis: a report of four cases

Occasionally, a dog-bite is complicated by a systemic overwhelming infection. We report four consecutive patients who were admitted to our intensive care unit because of sepsis syndrome following dog-bites. The history of these patients did not reveal any immunocompromising conditions. Capnocytophaga canimorsus (C. canimorsus) was cultured from the blood culture of 2 patients. Our data illustrate that in patients with lack of immune-deficiency severe sepsis may develop.     

Tuberculosis of the sacroiliac joint

A 23-year-old woman presented with low back pain of several months' duration. A tuberculous infection of the left sacroiliac joint was diagnosed by closed-needle biopsy. The clinical presentation, radiological features and outcome of this patient are discussed.     

Case report: can sacroiliac joint dysfunction cause chronic Achilles tendinitis?

This case study discusses the possible relationship between chronic Achilles tendinitis and sacroiliac joint dysfunction. The patient presented is an active pole jumper, competing at both the national and international levels. He suffered from chronic Achilles tendinitis during the 1994-95 season, and conservative treatment applied locally was not successful. The athlete discarded the possibility of operative debridement of the tendon. Instead, an evaluation of the kinetic chain of the lower extremity and pelvic-lumbar area was performed, and the athlete was diagnosed with sacroiliac joint dysfunction and Achilles tendinitis. Evaluation findings, treatment program, and treatment outcome are also presented. The literature regarding sacroiliac joint mechanics and biomechanics of the foot-knee-hip and pelvic area is discussed and used to support the author's thesis that sacroiliac joint dysfunction, in this case a backward rotation of the right ilium, may have changed the kinematic chain of the lower extremity and caused a tendinitis in the Achilles tendon of the affected leg. Sacroiliac joint function and dysfunction, the reliability of sacroiliac joint mobility tests, and the validity of treatment programs are still considered controversial, and more research is needed to understand these mechanisms.     

Foreign bodies in the bladder: report of four cases

This report presents the author's experience with four cases of foreign bodies in the bladder during a urological practice that spanned three different geographical locations. Relevant clinical aspects of this condition are discussed as well as technical tips given by various authors for the safe retrieval of such objects.     

Spinal myoclonus: report of four cases

Four cases of spinal myoclonus are described, three males and one female. The mean age was 51 years (28-75 years). The mean time between the onset of the myelopathy and the myoclonic jerks was 4.3 months (1-8 months). The involuntary movements were determined by trauma, Devic's disease, tuberculous myelopathy and tumor. Three patients had spastic paraplegia with bilateral myoclonus more evident on the right side. The fourth patient had a flaccid paraplegia with symmetrical jerks. The data suggest that different processes (trauma, demyelinating, infection and tumor) affecting the spinal cord may cause the same type of involuntary movements.     

Joubert syndrome: a report of 5 cases

OBJECTIVE: To review clinical features, radiological findings and prognosis in Joubert syndrome. MATERIAL AND METHODS: We report 5 children (3 male and 2 female) with the diagnosis of Joubert syndrome by clinical and radiological findings. They were diagnosed in the first year of life, in the Hospital Infantil La Paz (Madrid, Spain), from 1971 to 1996. Three patients have already been published, and here, we report two new cases. RESULTS: Partial absence of the cerebellar vermis, hypotonia and developmental delay were seen in all patients. Other cardinal findings were episodic hyperpnoea (5/5) with periods of apnoea (2/5), abnormal eye movements (2/5) and strabismus (3/5), tongue protrusion (2/5), seizures (1/5), hemifacial spasms (1/5) and occipital meningocele (2/5). Clinical manifestations were first noticed soon after birth. Two patients died in the first 5 years of life, and the rest of the cases actually show severe mental retardation. CONCLUSIONS: Joubert syndrome is a rare and probably underdiagnosed syndrome with bad prognosis. This inherited condition is characterized by agenesis of the cerebellar vermis, mental retardation, hypotonia, episodic hyperpnoea and abnormal eye movements. Additional manifestations have been reported since the original cases were described.     

Megalourethra: a report of four cases and review of the literature

Megalourethra is a rare congenital anomaly characterized by severe dilatation of the penile urethra. Four cases of congenital megalourethra were seen at Sir Padampat Mother and Child Health Institute, Jaipur, during the last 10 years. Three cases of scaphoid megalourethra had no other associated congenital anomalies and were treat-ed successfully without any complications, while one patient with a fusiform megalourethra had severe associated congenital anomalies and died. These cases are reported with a review of the literature.     

Thorium dioxide granuloma of the neck: a report of four cases

Four patients with Thorotrast granuloma are reported. The first patient had a radical neck dissection performed; however, postoperative hemorrhage and fistula occurred with a six-month delay in healing. The granuloma in this patient had totally occluded his carotid system with a greatly enlarged vertebral artery. A second patient presented with spontaneous bleeding in the neck from a granuloma followed by encephalomalacia, hemiparesis and aphasia. The third and fourth patients presented as hoarseness with a hard mass in the neck. All four patients had Thorotrast in the liver and spleen. The latter three had laryngoscopies and incisional neck biopsies as their only surgical treatment. Neck malignancy from Thorotrast is very rare. Despite legal decisions suggesting removal and similar suggestions in the literature, the authors feel only small extravasations have had uncomplicated operations. Neck dissection is rarely indicated. Hoarseness can be improved by vocal cord injection. These patients, of course, must be followed periodically as any other tumor, checking the neck mass, liver, and carotid circulation.     

Long-term neurological sequelae of hemolytic-uremic syndrome: a preliminary report

Seven patients with hemolytic-uremic syndrome who had major neurological symptoms during the acute illness were neurologically and cognitively evaluated prospectively several years after recovery from the illness. Four patients showed evidence of subtle neurological sequelae, including posturing, clumsiness, poor fine-motor coordination, hyperactivity, and distractibility. Psychoeducational evaluation of all seven subjects revealed mean scores within the average range in cognitive abilities, academic achievement, single word receptive vocabulary, visual/motor planning, overall adaptive functioning, and hyperactivity. The lapse of time (minimum of 7 years) between the acute illness and the psychometric evaluation could have been responsible for our normal results.     

Cerebrospinal fluid levels of oxytocin in Prader-Willi syndrome: a preliminary report

BACKGROUND: Prader-Willi syndrome (PWS) is a genetic disorder characterized by mental retardation, appetite dysregulation, and a high risk for obsessive-compulsive disorder (OCD). Microscopic abnormalities of the hypothalamus have been described in PWS, and oxytocin has been implicated in both appetite regulation and OCD. METHODS: Oxytocin and arginine vasopressin (AVP) were measured in the cerebrospinal fluid of 5 subjects with PWS (2 male, 3 female) and in 6 normal control subjects (all female). RESULTS: CSF oxytocin was elevated in PWS (9.2 +/- 3.9 pmol/L) as compared to normal control subjects (5.1 +/- 0.9 pmol/L, p = 0.045), a finding that was more significant when excluding male subjects from analysis (p = 0.02). AVP was not significantly different between the groups as a whole. CONCLUSIONS: These data provide further evidence for hypothalamic and oxytocinergic dysfunction in PWS. The associations between oxytocin, appetite regulation, and obsessive compulsive symptomatology in PWS warrant further investigation.     

Olmsted syndrome: report of a new case

We report the case of a 20-year-old man, who was born with an intense erythema of the genital area, unresponsive to any treatment employed. When he was 9 months old, he presented with well-defined hyperkeratotic erythematous plaques around the mouth, eyes, nose, and perianal area, with similar plaques on the lateral aspect of the neck and axillae. At the same time the erythema of the genital area became hyperkeratotic. When he was 2 years old, he presented with a disabling palmoplantar keratoderma, initially focal, and later diffuse, also unresponsive to local or systemic treatments employed. The lesions have varied during the course of the disease without ever clearing completely. The axillary and inguinal plaques have shown spontaneous resolution on occasion. Six skin biopsies have been performed with no conclusive histological diagnosis of any of the typical disorders of keratinization. All treatments, topical and systemic, including etretinate and acitretin, have failed to improve the condition. We believe that this patient has Olmsted syndrome, a rare form of palmoplantar keratoderma with periorificial keratotic plaques.     

Acute renal failure after snakebite: a report of four cases

The effects of FR139317 ((R)2-[(R)-2-[(S)-2-[[1-(hexahydro-1 H-azepinyl)]-carbonyl]amino-4-methyl-pentanoyl]amino-3[3-(1-methyl -1 H-indolyl)]propionyl]amino-3-2(2-pyridyl)propionic acid), an endothelin ETA receptor antagonist, on renal hemodynamics and urine formation were examined using anesthetized deoxycorticosterone acetate (DOCA)-salt hypertensive rats, in which renal perfusion pressure was protected from FR139317-induced hypotension with an aortic clamp. An intravenous injection of FR139317 (10 mg/kg) to sham-operated normotensive control rats produced no significant changes in renal hemodynamic and excretory responses. In DOCA-salt hypertensive rats, FR139317 caused sustained renal vasodilation. Urine flow and urinary excretion of sodium were increased significantly following drug injection. We suggest that endothelin-1 and the endothelin ETA receptor play an important role in water and sodium retention, and in renal vasoconstriction in this model of hypertension.     

The Peutz-Jeghers syndrome: a report of 3 cases

Three cases of the Peutz-Jeghers syndrome are reported and the literature is reviewed. The difficulties in the management of these patients are described and the importance of radiology and endoscopy is emphasised.     

Effects of posterior teeth replacement on temporomandibular joint sounds: a preliminary report

Previous reports have shown the prevalence of jaw clicking to be significantly higher in a population with missing posterior teeth compared with an dentulous population. This study presents a 2- to 5-year assessment of the effects of posterior teeth replacement on the amplitude of jaw clicking in subjects who were asymptomatic in all respects except jaw clicking. Patients requiring removable partial dentures (RPDs) were clinically and anamnestically examined and temporomandibular joint (TMJ) sounds were recorded using a modified stethoscope attached to an adjustable headgear. Amplified signals were displayed on a strip chart recorder. The amplitude of TMJ sounds at preinsertion of RPDs were 27.5 +/- 17.7 dB at opening, and 11.1 +/- 12.7 dB at closing. Similar values after prosthesis insertion were 15.8 +/- 17.0 dB and 7.4 +/- 7.5 dB, respectively. Paired t test analyses showed significant differences in the amplitude of sound for opening and closing before inserting the prosthesis (p = 0.003) and for opening before and after treatment (p = 0.014). Following replacement of posterior teeth, the clicking amplitude decreased in 68% of recordings, remained unchanged in 11%, and increased in 21%.     

Colonic anastomosis-a new approach: preliminary report

A case of fetal death from abruptio placentae is reported in which the placenta was the seat of multiple chorangiomata. This association is believed not to have been fortuitous and a possible mechanism is suggested by which chorangioma may contribute to the pathogenesis of placental abruption. The incidence, morphological variation and clinical manifestations of placental chorangiomata are briefly discussed.