Arthritis in a patient with homozygous familial hypercholesterolemia

Familial hypercholesterolemia is a disorder of lipoprotein metabolism characterized by elevated cholesterol, low-density lipoprotein cholesterol, xanthomas and early onset atherosclerosis. Tendinitis and arthritis have been reported in patients with familial hypercholesterolemia. A report is presented of a 9-year-old girl with an acute arthritic attack who was diagnosed as homozygote familial hypercholesterolemia with hypercholesterolemic arthritis.     

Fructose-Sorbitol Malabsorption and Symptom Provocation in Irritable Bowel Syndrome: Relationship to Enteric Hypersensitivity and Dysmotility

Background: Fructose-sorbitol (F-S) mixtures can provoke symptoms in irritable bowel syndrome (IBS) patients, and a proportion of IBS patients also have enteric hypersensitivity to distension. We hypothesized, therefore, that sugar malabsorption and fermentation to produce hydrogen gas may provoke symptoms to a greater extent in IBS patients hypersensitive to distension than in those patients without such hypersensitivity. Our aims were therefore to compare, in IBS patients, symptoms and breath hydrogen responses after F-S, on the basis of jejunal sensitivity and jejunal motor function. Methods     

Reversible airway obstruction in rheumatic mitral valve disease

Lung function was studied in 24 patients with advanced mitral stenosis scheduled for mitral valve replacement (MVR), and revealed an obstructive ventilatory pattern [rewording of this sentence OK] . Forty per cent of the patients had a forced expiratory volume in 1 s (FEV₁)<60% of that predicted in the preoperative period. Twenty-five per cent of those operated upon showed a similar pattern up to 110 weeks postoperatively. A blind study of the effect of placebo and β₂ agonist (salbutamol) inhalation was performed preoperatively and 6 months postoperatively, to evaluate the reversibility of airflow obstruction in these patients, flow volume curve and body plethysmographic measurement of airway resistance (Rex) and intrathoracic gas volume (VTG). Patients in the pre and postoperative period showed a significant difference between the placebo and the β₂ agonist responses for FEV₁, FEV₁ as percentage of FVC (FEV₁% FVC), peak expiratory flow rate (PEFR), flow rate of 50% of expiratory vital capacity ([¨max₅₀), Rex and VTG (P<0.001). We conclude that salbutamol inhalation improves obstructive impairment in patients with MVR pre- and postoperatively.     

Tuberculous peritonitis in 11 children: Clinical features and diagnostic approach

BACKGROUND: Tuberculous peritonitis (TBP) is a rare manifestation of childhood tuberculosis characterized by long-lasting abdominal symptoms and exudate and lymphocytes in the ascitic fluid. The diagnosis of TBP is rarely established unless a high index of suspicion is maintained. METHODS: The diagnostic features of 11 cases who were hospitalized with TBP in the Pediatric Infectious Diseases Ward of Dicle University Hospital, Turkey, were evaluated retrospectively. RESULTS: Seven cases were male and the ages of all cases ranged between 1 and 11 years. The onset of symptoms was 1-12 months (mean +/- SD 3.1 +/- 2.7 months) prior to the admission time. Nine patients gave a history of familial tuberculosis. Three cases had Bacillus Calmette-Guérin (BCG) scars and the results of five tuberculin unit (TU) tests in cases without and with BCG were over 10 and 15 mm, respectively. The most common presenting clinical symptoms and signs at admission were abdominal distention and ascites (100%), fever (27%) and loss of weight (18%). One case had accompanying tuberculous meningitis and two cases had concomitant pulmonary tuberculosis. Only one of 11 samples of ascitic fluid yielded Mycobacterium tuberculosis by the polymerase chain reaction method and no other microbiologic evidence was obtained in culture specimens. Ultrasonographic and computed tomographic imagings revealed high-density ascites that contributed well to the diagnosis. The diagnosis in two patients was proven histopathologically via peritoneoscopy and laparoscopy. All cases were treated with isoniazide, rifampisin for 9 months and pyrazinamide for the first 2 months. CONCLUSIONS: Radiologic diagnostic techniques, positive skin tests and a history of exposure to tuberculosis may contribute to the diagnosis of TBP, helped by clinical symptoms and findings, particularly when invasive diagnostic methods via peritoneoscopy and laparoscopy are not available in developing countries.     

An alternative method for restoring opposition after median nerve injury: an anatomical feasibility study for the use of neurotisation

Opposition, one of the most important functions of the hand, is lost or impaired after median nerve injury. Complete recovery does not always occur after treatment, and various techniques of opponensplasty are used for restoring opposition. This study was performed in order to develop an alternative method for selective restoration of thenar muscle function. Ten arms from 5 cadavers were used. The median nerve with its thenar motor branch (Tb) and the anterior interosseous nerve with its motor branch to pronator quadratus (PQb) were prepared in the distal forearm. The mean widths and the number of myelinated fibres of these nerves were: PQb 1.3±0.10 mm, Tb 1.4±0.12 mm and PQb 912±88 mm, Tb 1020±93 mm. The minimum necessary distance from the distal flexor crease of the wrist for neurotisation of the Tb by the PQb was 60±5.41 mm. It was concluded that PQb-Tb neurotisation would be possible anatomically. The advantages are that motor function is reestablished with a motor nerve, the diameters and the number of myelinated fibres of both nerves are similar, the loss of function after denervation of the pronator quadratus is slight and opponensplasty still remains as a final option.     

Infant feeding and growth: A study on Turkish infants from birth to 6 months

BACKGROUND: To evaluate the impact of various feeding patterns on the physical growth and mental development of infants, particularly during the first 6 months of life, and to compare growth patterns of Turkish infants with those of infants living in various countries. METHODS: One hundred and seventy-two healthy newborn infants were included in the study and were divided into three feeding groups: (i) 62 infants were exclusively breast-fed (BF); (ii) 58 infants were mixed-fed (MF) with both breast milk and formula; and (iii) 52 infants were formula-fed (FF). Infants were assessed at birth and at 1, 2, 3, 4, 5 and 6 months of age. Anthropometry was repeated on each occasion. The weight and length of the infants was also recorded. Analysis of variance and modified t-test were used for statistical evaluation of the results. RESULTS: Values in the BF group were the closest to the tabular norms for weight. Infants in the FF group tended towards a lower weight during the first 3 months (P < 0.05). During the second 3 months, weight gain observed in the FF group was significantly higher than that of BF infants. In comparison with MF infants, a significant progressive weight gain was detected in BF infants (P < 0.05). The values obtained for length increments were consistent with those for weight (P < 0.05 for BF vs FF). No significant difference was found between the length increments detected for BF and MF infants from birth to 6 months. CONCLUSIONS: These results suggest that exclusive breast-feeding is the most appropriate feeding pattern for newborn infants in Turkey and is sufficient during the first 6 months, the most important fraction of life.     

Antithrombin III activity, von Willebrand factor antigen and platelet function in young diabetic patients treated with multiple insulin injections versus insulin pump treatment

In a prospective study with cross-over design 20 patients with insulin-dependent diabetes mellitus of more than 2 years duration were treated for 6 months with continuous subcutaneous insulin infusion (CSII) and multiple insulin injections (MII). Metabolic control, platelet aggregability, thromboxane B2 levels in serum and plasma as well as antithrombin III (ATIII) activity and von Willebrand factor antigen were evaluated. A good metabolic control was obtained by both intensified regimens. No difference could be demonstrated between either platelet function tests or serum level of von Willebrand factor antigen during treatment with CSII and MII. However, the plasma level of ATIII activity was significantly higher (P less than 0.01) during MII treatment as compared to CSII treatment. There was no correlation between ATIII activity and daily insulin requirement or serum fructosamine. In conclusion, long-term metabolic control with MII has a favourable effect on ATIII activity in plasma. This may be important for a delay in onset and progression of diabetic vascular complications.     

Stones in anomalous kidneys: Results of treatment by shock wave lithotripsy in 150 patients

OBJECTIVE: To determine the efficacy of shock wave lithotripsy (SWL) in anomalous kidneys. METHODS: From October 1990 to October 2002, 150 patients (93 men and 57 women) with anomalous urinary tracts, including 45 horseshoe kidneys, 57 duplex kidneys, 30 malrotated kidneys, 14 pelvic and four crossed ectopic kidneys were treated with SWL for urolithiasis at the Gazi University Faculty of Medicine. Shock wave lithotripsy was performed with Siemens Lithostar plus (Siemans, Erlanger, Germany) device and all procedures were carried under fluoroscopic control. Results: The mean shock wave number and intensity received by the patients was 3770 (range, 1380-4100) shocks and 18.4 (range, 16.1-19) kV per session, respectively. The minimum success rate was obtained in patients with lower calyceal (50%) followed by middle calyceal (60%) calculi. The stone-free rate decreased and the number of sessions per patient increased with increasing stone diameter (dm). In patients with a stone dm > 30 mm, only 34% could be stone-free, compared to a rate of 92% for calculi dm < 10 mm. The overall stone free rate at the third month was 68%. The best stone-free rates were obtained in patients with ureteral duplication (80.7%). The stone-free rates in horseshoe, malrotated, pelvic and crossed ectopic kidneys were found to be 66.7%, 56.7%, 57.2% and 25%, respectively. CONCLUSION: Shock wave lithotripsy might be an effective and minimally invasive treatment alternative in stone-bearing anomalous kidneys. The type of anomaly, stone burden and localization seem to be the main parameters effecting the treatment success.     

Papillary adenoma of the prostatic urethra: Report of two cases

Papillary adenoma of the prostatic urethra is a rare cause of hematuria, hemospermia and lower urinary tract obstruction, especially in young adults. We report two cases of papillary adenoma and their endoscopic views. One of the patients had a lesion located on the verumontanum and presented with hematuria, hemospermia and symptoms of lower urinary tract obstruction. The other patient had papillary lesions located in the paramontanal gutter and presented with persistent painless hematuria. The origin of both lesions was proven to be prostatic epithelial differentiation using immunohistochemical identification of prostate-specific antigen. Papillary adenoma is believed to be benign, therefore, both lesions were carefully excised and fulgurated transurethrally.