A Rare Case of Diffuse Neurofibroma of the Scalp With Destructive Lesions Involving the Base of the Skull in a Patient With Neurofibromatosis Type 1.

Diffuse neurofibroma is a benign tumor of peripheral nerves. Ten percent of neurofibromatosis type 1 (NF-1) patients can develop diffuse neurofibroma. Here, we report a case of diffuse neurofibroma involving the base of the skull in a 50-years-old patient with NF-1. The patient presented with diffuse involvement of the scalp with soft and mobile masses. Radiological investigations revealed skull bone lesions. Aggressive osteolytic lesions involving the base of the skull were present. Surgical excision with the repairing of the defects was suggested but the patient refused the treatment. The diagnosis of calvarial defects in diffuse neurofibroma is challenging. Early diagnosis can help in better management of the patients.

Comparison of Metformin and Repaglinide Monotherapy in the Treatment of New-Onset Type 2 Diabetes Mellitus.

Objectives We intend to investigate the feasibility of using repaglinide as initial therapy in patients with newly diagnosed type 2 diabetes mellitus naive to the oral anti-hyperglycemic agents by validating the effects of repaglinide on glycemic control (HbA1c) in comparison with metformin monotherapy. Methodology This parallel-controlled, randomized study was carried at the outpatient department of a tertiary care hospital. Two-hundred patients of both genders with newly diagnosed type 2 diabetes mellitus were included. After taking relevant history and physical examination, we drew venous blood samples of each patient and sent them to the institutional laboratory for analysis of fasting blood sugar (FBS) levels, HbA1c, and lipid profile. We divided the patients into two subgroups based on the lottery method. Group A was prescribed metformin, and group B was prescribed repaglinide, while the dosages were adjusted according to the blood sugar levels. All data were analyzed using SPSS Software 25.0 (SPSS Inc., Chicago, USA). We reported the data as means along with the standard error. Results All patients completed the study. There was a decline in fasting blood glucose levels after three months of therapy, both in the metformin (135 mg/dl ± 6 mg/dl versus 115 mg/dl ± 7 mg/dl, p < 0.01) and repaglinide groups (145 ± 6 mg/dl versus 122 ± 6 mg/dl, p < 0.01). Similarly, significant reductions in HbA1c were seen in both metformin (7.12 ± 0.15% versus 6.67 ± 0.06%, p < 0.01) and repaglinide treatment groups (7.83 ± 0.67% versus 6.81 ± 0.07%, p < 0.01). After three months of treatment, body mass index (BMI) was significantly decreased in the metformin group (26.87±1.1 kg/m2 versus 25.11 ± 0.44 kg/m2, p < 0.05). However, the patients in repaglinide group demonstrated a very slight decrease in BMI (27.11 ± 1.6 kg/m2 versus 26.47 ± 0.40 kg/m2). On follow-up, we found a significant decrease in triglyceride levels in both groups (p < 0.01 and p < 0.05. respectively). We also found that only the patients in metformin group showed some improvements in total cholesterol and low-density lipoprotein (LDL) levels (p < 0.05). Conclusion Our study concluded that both metformin and repaglinide have similar anti-hyperglycemic effects. Repaglinide can be prescribed as an alternative drug to metformin in patients with new-onset diabetes mellitus.

Hyponatremia and Its Correlation With Hepatic Encephalopathy and Severity of Liver Disease.

Background and objective Hepatic cirrhosis is one of the leading causes of morbidity and mortality worldwide. Patients with cirrhosis frequently develop complications such as ascites, variceal bleeding, and hepatic encephalopathy (HE). The clinical manifestations of HE range from the mildly altered level of sensorium to severely altered consciousness levels, difficulty in judgment, the day-night reversal of sleep, flapping tremor of hands, and irrelevant talking or speech. Patients with hyponatremia are at a higher risk of developing HE and electroencephalographic abnormalities. The severity of hyponatremia is directly related to the deterioration in terms of grades of HE. Our study sought to determine the frequency of hyponatremia in cirrhotic patients and its correlation with the frequency and severity of HE. Methodology This study was carried out at the inpatient department of medicine in a tertiary care hospital in Pakistan. A total of 260 patients of both genders with hepatic cirrhosis were approached. After taking relevant history and physical examination, the venous blood sample of each patient was drawn and sent to the institutional laboratory for estimation of serum electrolytes, liver function tests (LFTs), renal parameters (RPMs), prothrombin time (PT), activated partial thromboplastin time (aPTT), and international normalized ratio (INR). We classified the HE according to the West Haven classification system. Mild to moderate encephalopathy was classified under grades I-II, while severe encephalopathy was classified under grades III-IV. We documented the severity of liver disease according to the Child-Pugh score criteria. All data were analyzed by using SPSS Statistics version 25.0 (IBM, Armonk, NY). We reported the data as means along with the standard error. Results Overall, the serum sodium levels of the subjects ranged from 115 to 142 meq/L with a mean of 129.11 ±6.53 meq/L. In patients with hyponatremia, it ranged from 115 to 127 meq/L (mean 121.41 ±5.17 meq/L). Hyponatremia was present in 96 (36.9%) patients. Among these, 51 (53.12%) were male and 45 (46.8%) were female; 24 (9.2%) patients had mild hyponatremia, 56 (21.5%) had moderate, and 16 (6.2%) had severe hyponatremia. HE was present in 176 (67.7%) patients. HE grade I was present in 54 (20.8%), grade II in 62 (23.8%), grade III in 32 (12.3%), and grade IV in 28 (10.8%) patients. In 96 patients with hyponatremia, 84 were found to have HE (p-value: <0.001). Conclusion Based on our findings, cirrhotic patients with chronic hepatitis infections have a variable presence of low sodium levels. Sodium levels of <130 meq/L were associated with higher morbidity and mortality rate. Moreover, patients with lower levels of sodium had higher grades of HE.

Bilateral Persistent Hyperplastic Primary Vitreous: A Case Report and Review of the Literature.

Bilateral persistent hyperplastic primary vitreous (PHPV) is a rare ocular disorder. Its clinical manifestations include bilateral corneal haziness, microphthalmia, and cataract. It is the second most common cause of leukocoria after retinoblastoma. Most cases of PHPV are unilateral. The typical imaging features of PHPV comprise bilateral echogenic masses and a fibrous cord extending from the posterior surface of the lens to the optic disc. In this report, we present a case of bilateral PHPV in an infant who presented with bilateral corneal haziness and watery discharge. A detailed ocular examination and knowledge about its features on imaging can lead to a timely and accurate diagnosis of the condition.

Dyke-Davidoff-Masson Syndrome: A Case Report and Review of Literature.

Dyke-Davidoff-Masson syndrome (DDMS) is a rare neurological disorder that results from brain injury in intrauterine or early years of life. Prominent cortical sulci, dilated lateral ventricles, cerebral hemiatrophy, hyperpneumatization of the frontal sinus, and compensatory hypertrophy of the skull are the characteristic findings. We describe a male patient who presented with generalized tonic-clonic seizure and left-sided body weakness and neuroimaging findings of cerebral hemiatrophy, dilatation of right lateral ventricle, right frontal sinus hyperpneumatization, and asymmetric calvarial thickening. Knowledge of its features on imaging enables timely and accurate diagnosis, allowing appropriate management.

Routine ultrasonography surveillance of native kidneys for renal cell carcinoma in kidney transplant candidates.

Renal cell carcinoma (RCC) has a high incidence in the kidney transplant population and annual surveillance detects these tumors early in their natural history. Minimal guidelines exist regarding RCC surveillance in ESRD patients awaiting transplant. A retrospective review of our kidney transplant database examined the outcomes of annual ultrasonographic surveillance during initial kidney transplant evaluation and upon annual reassessment. Of 2642 patients listed for transplant, 145 patients were found to have masses during initial kidney transplant evaluation or annual imaging consistent with new complex cystic disease or RCC. A total of 71 patients had RCC identified, with 52 found on initial kidney transplant evaluation and 19 identified on annual surveillance. Male gender and African-American race were independently associated with RCC (P<.05). RCC was detected a median of 2.0 years after listing (two annual ultrasonography studies). Patients with complex cysts were more likely to undergo transplantation (48.7%) compared to patients with RCC (21.1%; P<.001). There was no significant difference in survival between RCC patients and those found to have complex cystic disease, suggesting incidental RCC can be diagnosed early in the natural history and at a curable stage through implementation of a biennial surveillance program.

Late presentation of adenovirus-induced hemorrhagic cystitis and ureteral obstruction in a kidney-pancreas transplant recipient.

We report a late presentation of adenovirus-induced renal allograft and bladder infection causing azotemia and hemorrhagic cystitis in a patient 5 years after simultaneous kidney-pancreas transplantation. Adenovirus has been increasingly recognized as a cause of morbidity and mortality in both solid organ and stem cell transplant recipients. We wish to emphasize the importance of early detection, as treatment options involve reduction of immunosuppression, followed by the addition of antiviral agents and supportive care.