Primary hyperoxaluria type 3: from infancy to adulthood in a genetically unique cohort.

Background: Primary hyperoxaluria type 3 (PH3) is a rare autosomal recessive disorder caused by bi-allelic genetic variants in the 4 hydroxy-2 oxoglutarate aldolase (HOGA-1) gene. We report the natural history of PH3 in a 16-patient cohort, 15 from a unique genetically isolated population.

Methods: This retrospective single-center study followed PH3 patients between 2003 and 2023 with demographic, clinical, radiographic, genetic, and biochemical parameters.

Overcoming reduced hepatic and renal perfusion caused by positive-pressure pneumoperitoneum.

Hypothesis: Use of the intermittent sequential pneumatic compression (ISPC) device may improve splanchnic and renal perfusion caused by positive-pressure pneumoperitoneum (PPP) in patients undergoing laparoscopic cholecystectomy.

Design: Prospective controlled study.

Setting: University hospital.

Incidental detection of a transmigrated intrauterine device.

The intrauterine device (IUD) is among the most effective forms of birth control available, with important advantages over other methods of contraception. The most striking adverse event associated with IUD use is uterine perforation and migration of the device. Contrary to what one might assume, perforation is often silent and the wayward device is either detected after further sequellae or found incidentally by imaging.

Papillary cystic neoplasm of the pancreas in a teenage boy.

We present a case of a 13-year-old boy with a left-sided abdominal mass which proved to be a papillary cystic neoplasm of the pancreas. This low-grade malignant lesion of young patients is very rare, and exceedingly rare in males. The prognosis following resection of this tumor is good.

Rhabdomyosarcoma in a child with massive pleural effusion: cytological diagnosis from pleural fluid.

We report on a case of embryonal rhabdomyosarcoma in an 11-yr-old boy investigated for mild chest pain after trauma. Chest radiography showed a massive right pleural effusion. Cytological analysis of the pleural fluid demonstrated the presence of malignant small undifferentiated cells.

Closure of the ductus venosus in premature infants: findings on real-time gray-scale, color-flow Doppler, and duplex Doppler sonography.

Objective: Our objective was to use gray-scale, color-flow, and duplex Doppler sonography to study the anatomy, flow pattern, and time of closure of the ductus venosus in healthy premature infants.

Subjects And Methods: We prospectively examined the ductus venosus in 130 premature infants whom we divided into two groups: Group I comprised 27 neonates of gestational age 28-32 weeks, and group II comprised 103 neonates of gestational age 33-36 weeks. Neonates who had undergone umbilical vessel manipulation were excluded from the study.

Contracted gallbladder in hepatitis. Value of postprandial examination.

A completely contracted gallbladder is occasionally seen in patients with acute hepatitis. We have observed that postprandial examination in these patients shows the gallbladder filling with bile to a degree that permits an adequate diagnostic examination. This simple procedure may prevent having to recall the patient for follow-up examination.

Enteropathy-associated T-cell lymphoma: a case report with radiographic and computed tomography appearance.

We report a case of enteropathy-associated T-cell lymphoma (EATL) of the jejunum in a 56-year-old man. The patient suffered for several years from nonspecific abdominal complaints, with no clinical evidence of malabsorption. The patient underwent extensive imaging procedures including barium meal and computed tomography.

Posttraumatic intestinal stenosis: radiographic and sonographic appearance.

We report a case of posttraumatic intestinal stenosis (PIS), an uncommon sequela of blunt abdominal trauma, in which injury to the mesentery and bowel wall results in later focal ischemic stricture of that segment. We include CT images at the time of trauma, and barium meal and abdominal sonography obtained during the subsequent admission. Examination of the resected bowel loop showed transmural infarct and posttraumatic changes in the adjacent peritoneal fat.

A prospective study evaluating the usefulness of continuous supplemental oxygen in various endoscopic procedures.

The influence of oxygen supplementation on the prevalence of hypoxemia during endoscopic procedures was studied in 289 patients in a prospective clinical trial. The frequency of oxygen desaturation was found to decrease significantly in patients receiving oxygen via nasal prongs, compared to patients not receiving oxygen supplementation. The effect was demonstrated especially in patients who underwent an additional procedure during their endoscopic examinations.

Acute focal bacterial nephritis in childhood brucellosis.

Brucellosis is an infectious disease transmitted from various animal hosts to man. In children the most common route of infection is unpasteurized milk or milk products. Brucellosis affects the urinary tract uncommonly, most frequently causing orchioepididymitis.

Study of sympathetic innervation of cranial bones by axonal transport of horseradish peroxidase in the rat: preliminary findings.

This study performed by intraneuronal tracing directly demonstrates the presence of sympathetic postganglionic fibers in the cranial vault of the rat. Superior cervical sympathetic ganglia were injected with horseradish peroxidase (HRP), and after a 48-hour period, to permit anterograde axonal transport, the animals were sacrificed after in situ perfusion. An area of the calvaria that included portions of the frontal and parietal bones was fixed, decalcified and sectioned.

Closure of the ductus venosus in neonates: findings on real-time gray-scale, color-flow Doppler, and duplex Doppler sonography.

Objective: Our objective was to document the anatomy, flow pattern, and time of closure of the ductus venosus in healthy full-term neonates.

Subjects And Methods: We examined the ductus venosus in 73 neonates by using gray-scale sonography, color flow imaging, and duplex Doppler sonography. Each neonate was examined 1-2 days after birth, 6-7 days after birth, and then every 3-4 days until closure was confirmed or the neonate was 18 days old.

[Intrabiliary rupture of hepatic echinococcal cyst].

Echinococcal disease of the liver generally presents as single or multiple hepatic cysts. Rarely, these cysts may rupture into the biliary tree and cause cholangitis, biliary tract obstruction, or biliary colic. These complications are accompanied by high morbidity, and in the case of biliary tract obstruction, mortality rates as high as 50% have been reported.

Fibroblast growth factor: effects on osteogenesis and chondrogenesis in the chick embryo.

In vivo effects of fibroblast growth factor (FGF) on osteogenesis were evaluated in the chick embryo. Autoradiographic studies of 3H-proline labeling over bone matrix indicated that 24 h after treatment on day 11, FGF stimulated osteogenic cell proliferation, while inhibiting the production of bone matrix collagen. However, 4 days after multiple doses of FGF, the large pool of newly formed osteogenic and chondrogenic cells expressed their function with the increased formation of matrix.

The early effects of surgical sympathectomy on bone resorption in the rat incisor socket.

Earlier studies have shown that one week after sympathectomy in the rat, both bone apposition and resorption are significantly altered. Resorption in the incisor socket was now examined 1, 3 and 5 days after surgical sympathectomy. Morphometric analyses indicated that the total resorbing surface, active resorbing surface and number of osteoclasts per socket were increased one day after sympathectomy; the area and length of the bone surface of the socket were not significantly altered.

Effect of surgical sympathectomy on bone remodeling at rat incisor and molar root sockets.

Sympathectomy was carried out in 4-week-old Sprague-Dawley rats by unilateral surgical removal of the superior cervical ganglion. Sham-treated rats served as controls. All rats were injected with tetracycline hydrochloride at surgery as well as 36 hr prior to sacrifice.

Effect of beta-amino-propionitrile on the regenerating newt limb evaluated by uptake of 3H-proline and 3H-lysine.

Newt forelimbs were unilaterally amputated and Beta-amino propionitrile (BAPN) was added to the water in which the newts were maintained. Sixty-three days after amputation, animals were injected with either 3H-proline or 3H-lysine. Forelimbs distal to the elbow of both amputated and non-amputated sides were removed 72 hours later.

Autoradiography of [3H]-proline uptake by fibroblasts of the periodontal ligament and odontoblasts of the rat incisor after guanethidine-induced sympathectomy.

The uptake of [3H]-proline was monitored by quantitative autoradiography at 30, 45 and 90 days of age. Grain counts in sympathectomized rats did not vary significantly from control values at any time. Thus the sympathetic nervous system does not influence the metabolic activity of these cells, which agrees with morphologic evidence that the innervation of the rat incisor may not be autonomic.

Effect of guanethidine-induced sympathectomy on osteoblastic activity in the rat femur evaluated by 3H-proline autoradiography.

Osteoblastic activity in the rat femur was assessed following sympathectomy by injections of guanethidine sulfate from birth to 14 days of age. At ages 30, 45 and 90 days, osteogenesis was monitored by quantitative autoradiography using 3H-proline. Grain counts over periosteal osteoblasts of the femoral diaphysis showed a significant reduction in the uptake of 3H-proline in sympathectomized rats.

Histologic evaluation of the sympathectomy induced by guanethidine sulfate in neonatal rats.

Guanethidine sulfate was administered to rats from birth to 14 days of age. Cell counts were carried out in the superior cervical sympathetic ganglion at 30, 45, and 90 days of age. Massive permanent destruction of sympathetic ganglionic cells was demonstrated during the experimental period.

Autoradiographic assessment of 3H-proline uptake by osteoblasts following guanethidine-induced sympathectomy in the rat.

Sympathectomy was carried out in rats by injections of guanethidine-sulfate from birth to 14 days of age. At 45 days of age, the activity of osteoblastic cells was monitored by 3H-proline autoradiography. Effectiveness of sympathectomy was verified by light-microscopic examination of superior cervical and celiac ganglia.

Cultural differences in the perception of geometric illusions.

Data from 15 societies are presented showing substantial intersocietal differences of two types in susceptibility to geometric optical illusions. The pattern of response differences suggests the existence of different habits of perceptual inference which relate to cultural and ecological factors in the visual environment.