Congenital dislocation of patella with ipsilateral hip flexion-abduction deformity: a case report

Iliotibial band tightness and its abnormal insertion on patella is one of the contributing factors in congenital dislocation of patella. Although hip flexion-abduction deformity is caused by a tight iliotibial band, as frequently seen in postpolio residual paralysis, an association of hip flexion-abduction deformity with congenital dislocation of patella has never been reported previously. Such a case was presented to us, and surgical repositioning of the patella was carried out by means of release of the tight iliotibial band from the patella besides other tight lateral structures along with medial augmentation and distal realignment of the quadriceps mechanism. The hip deformity was corrected spontaneously after release of the tight iliotibial band from the knee without need for additional surgery at the hip.     

Reversible renal medullary hyperechogenicity in neonatal hypernatremic dehydration

Three exclusively breastfed term neonates were admitted with lethargy, poor feeding, and oligoanuria. All three babies were severely dehydrated and had a weight loss ranging from 18% to 40%. Serum sodium of more than 180 mEq/l and renal failure were observed in all three. Two had very high creatinine levels of 9.5 mg/dl and 6.7 mg/dl. Both these babies also had multiple seizures. One baby required mechanical ventilation. All three babies showed markedly hyperechoic renal medullary pyramids with speckled foci suggestive of crystal deposition that reversed completely on therapy. Urine showed abundant urate crystals in two and an elevated calcium/creatinine ratio of 1.6 in one. There was no evidence of distal renal tubular acidosis, Bartter syndrome, or high serum calcium. Supersaturation of the ions in a markedly hypertonic renal medulla may have led to crystallization, with resolubilization with hydration and restoration of good urine output. The hypernatremic dehydration was primarily due to lactation failure leading to inadequate fluid intake in the face of ongoing insensible losses. High breast milk sodium may have been a contributory factor in one patient.     

Is antegrade ureteroscopy better than retrograde ureteroscopy for impacted large upper ureteral calculi?

OBJECTIVE: This study was planned to compare the risks and advantages of antegrade and retrograde ureteroscopy for impacted large upper ureteral calculi. PATIENTS AND METHODS: From September 1996 to February 1998, ureteroscopy was offered to 43 patients. Of these, retrograde ureteroscopy was done in 20 patients, while antegrade ureteroscopy was performed in 23 patients. All these patients were followed up to evaluate the immediate and long-term success of the procedure. The incidence and nature of complications were also noted. RESULTS: During retrograde ureteroscopy, complete stone clearance was achieved in 11 patients (55%), while pushback of the whole or fragmented calculus was seen in the rest. These patients with residual calculi were later treated by extracorporeal shockwave lithotripsy (SWL). The stone-free rate at the end of 3 months was 85%. Three patients developed minor ureteroscopy-related complications. Complete stone clearance was achieved in all patients with antegrade ureteroscopy. No intraoperative or postoperative complications were encountered. CONCLUSION: In this series, antegrade ureteroscopy was found to be a safe and effective option for impacted upper ureteral calculi and assured better results than retrograde ureteroscopy.     

Contribution of spirituality to quality of life in patients with residual schizophrenia

There is increasing recognition that a person's spiritual or religious experiences contribute to quality of life (QOL). However, research exploring the relation between spirituality and QOL has mainly been in the context of chronic and life-threatening illnesses, and studies examining this important correlate of QOL in chronically mentally ill subjects are sparse. This study aimed to explore the relationship between spirituality and QOL, and to investigate if spirituality contributes to other domains of QOL (both physical and psycho-social) in subjects with residual schizophrenia. In a study with a cross-sectional design, 103 patients with residual schizophrenia were assessed with the Positive and Negative Syndrome scale, and their quality of life, spirituality and religiousness were assessed with the WHO Quality of Life-Spirituality, Religiousness and Personal Beliefs (WHOQOL-SRPB) scale. The SRPB domain and all its facets other than spiritual connection correlated significantly with all other domains of QOL and overall QOL. On regression analysis, the inner peace domain of spirituality explained 21.6 to 37.6% of variance of all QOL domains except the domain of level of independence. The spirituality domain explained 33.8% of the variance of the 'level of independence domain of QOL. Taken together, inner peace and spirituality facets explained 23 to 40% of the variance of the social relationships domain, the psychological domain and the level of independence domain of QOL. This study suggests that spirituality and religiosity have an important influence on overall QOL of patients with schizophrenia. Hence, besides pharmacological and non-pharmacological management for schizophrenia, clinicians should focus on this aspect and encourage their patients to follow their religious practices and spiritual beliefs.     

Prevalence and correlates of epileptic seizure in substance-abusing subjects

Life-time prevalence of epileptic seizures was assessed in 626 consecutive patients treated for substance abuse. Seizures were reported in 8.63% (9.2% in alcohol abusers, 12.5% in opioid abusers). A total of 64.8% of the seizures were associated with substance use. These occurred during withdrawal in the alcohol cohort and during intoxication with dextropropoxyphene and withdrawal from heroin or poppy husk in the opioid cohort. Results indicate that seizures may be more common in older patients with longer duration of dependence among those abusing alcohol.     

Prognostic and therapeutic value of the Hippo pathway,RABL6A,and p53-MDM2 axes in sarcomas

Additional prognostic and therapeutic biomarkers effective across different histological types of sarcoma are needed. Herein we evaluate expression of TAZ and YAP, the p53-MDM2 axis, and RABL6A, a novel oncoprotein with potential ties to both pathways, in sarcomas of different histological types. Immunohistochemical staining of a tissue microarray including 163 sarcomas and correlation with clinical data showed that elevated YAP and TAZ independently predict worse overall and progression-free survival, respectively. In the absence of p53 expression, combined TAZ and YAP expression adversely affect overall, progression free, and metastasis free survival more than TAZ or YAP activation alone. RABL6A independently predicted shorter time to metastasis and was positively correlated with p53, MDM2 and YAP expression, supporting a possible functional relationship between the biomarkers. Network analysis further showed that TAZ is positively correlated with MDM2 expression. The data implicate all five proteins as clinically relevant downstream players in the Hippo pathway. Finally, a novel inhibitor of MDM2 (MA242), effectively suppressed the survival of sarcoma cell lines from different histological types regardless of p53 status. These findings suggest both independent and cooperative roles for all five biomarkers across different histological types of sarcoma in predicting patient outcomes and potentially guiding future therapeutic approaches.