گزارش شهريور

Image 1

BACKGROUND A 27-year-old man presents to the emergency department (ED) with a 3-day history of worsening epigastric pain and nonbilious vomiting. His bowel movements were normal until the day of presentation, when they turned black and sticky. He denies having any hematemesis, fever, chills, or any other associated symptoms, as well as any history of prior surgeries or medical problems. He has taken no medications recently except for Pepto-Bismol, which made the pain worse. He does not smoke tobacco but consumes 4 beers each day. He denies any illicit drug use.On physical examination, his oral temperature is 98.6°F (37.0°C). His pulse has a regular rhythm with a rate of 88 bpm. His blood pressure is 198/88 mm Hg. He is noted to be in mild distress secondary to his epigastric discomfort. The examination of his head and neck, including a check for icteric sclerae, is normal. His lungs are clear to auscultation with normal respiratory effort. A 1/6 soft systolic ejection murmur is detected. His S1 and S2 heart sounds are normal. His abdomen is soft but tender to deep palpation in the epigastric region. The rectal exam reveals normal tone and black, guaiac-negative stool. The peripheral arterial pulses in the lower extremities are palpable but diminished when compared to the pulses in the upper extremities.   The laboratory analysis, including a complete blood count and a basic metabolic panel, is normal; however, his serum amylase and lipase levels are elevated, at 240 U/L (normal range, 30-110 U/L) and 2118 U/L (normal range, 46-218 U/L), respectively. The patient is diagnosed with alcohol-induced pancreatitis and treated in the ED with bowel rest, intravenous fluids, antiemetics, and generous doses of intravenous opiate analgesics. The patient is to be admitted to the hospital for continued bowel rest and intravenous fluid therapy for complete resolution of his pancreatitis; however, despite having his pain eased, the patient is noted to have a persistently elevated systolic blood pressure in the 190-199 range and a diastolic blood pressure in the 90-109 range. When further queried for his past medical history and a thorough review of systems, the patient does not recall ever having had his blood pressure checked, although he does report that he frequently experiences cramping in his legs and sometimes feels as if his feet are “cold.a posteroanterior chest radiograph is obtained (Image 1) What is the most likely cause of this patient’s hypertension, and what further testing should be performed?diagnosis?

prenatal hydronephrosis

The widespread use of obstetrical ultrasonography has had a major effect on the diagnosis of hydronephrosis. In the past, patients with hydronephrosis were discovered after delivery because of signs of an abdominal mass or urinary tract infections. Dilation of the urinary tract frequently, but not necessarily, signifies obstruction. Conversely, a fetus can have obstructive uropathy in the absence of urinary tract dilation.
The most commonly used standard for evaluating neonatal hydronephrosis depends on measurement of the AP diameter of the fetal renal pelvis and of the kidney and assessment of the degree of caliectasis. Renal pelvis measurement less than 5mm is almost always clinically insignificant. Measurement of 5-9mm and pelvic to kidney ratio of less than 50% in the absence of caliectasis rarely progresses or requires postnatal management. Measurement greater than 10 mm and pelvic to renal ratio greater that 50% usually represents significant dilatation which often progresses and frequently requires postnatal management. Additionally, a grading system has been developed by Grignon et al (2) for the degree of hydronephrosis after the 20th gestational week:

Grade Size Renal Pelvis Calyceal Dilatation
I <10 mm Physiologic
II 10-15 mm Normal Calyces
III >15 mm Slight Dilatation
IV >15 mm Moderate Dilatation
V >15 mm Severe Dilatation
(w/ renal cortex atrophy)

Hydronephrosis can be caused by UPJ obstruction, obstruction of the distal ureter (UVJ), renal duplication anomalies, posterior urethral valves, vesico-ureteric reflux, and prune-belly syndrome. When fetal hydronephrosis is detected, it is important to evaluate the contralateral kidney, ureters, bladder, and amniotic fluid volume in an attempt to assess the degree and level of obstruction.

When fetal hydronephrosis is diagnosed, follow-up prenatal examinations are advocated and even necessary in many cases. Hydronephrosis detected on one prenatal examination, even if decreased or absent on subsequent examination, should be re-evaluated postnatally. It is recommended that initial postpartum ultrasound should not be performed until 4-5 days after delivery (unless newborn physical exam findings indicate a condition requiring immediate urologic surgery evaluation i.e posterior valves, prune belly syndrome). An exam performed sooner may be falsely negative or can underestimate the severity of hydronephrosis, because of a relative state of dehydration and decreased GFR immediately after delivery.

A possible management protocol is summarized as follows:

Fetal Hydronephrosis->Postnatal Ultrasound
Negative->Done
Positive->
Severe-> ABX Prophylaxis
& UrolConsult->
Mild/Mod-> ABX prophylaxis & VCUG->
Positive Reflux->Continue ABX
& follow w/ VCUG
Negative Reflux->D/C ABX->
Nuc Med MAG3 Renal Scan->
UPJ Obstruction->Urology Consult
No Obstruction->Follow w/ US

Achondroplasia

Achondroplasia is the most common type of short-limb (rhizomelic) dwarfism with the primary defect being abnormal endochondral bone formation. There is normal periosteal and oppositional bone growth. It is an autosomal dominant trait with at least 80% of cases being new random mutations. The condition has an incidence of 1:26,000 – 66,000 births.

Neonatal radiographs of the axial and appendicular skeleton demonstrate various classic findings. The base of the skull which is formed by endochondral ossification is shortened with a small foramen magnum. There is a concurrent frontal bossing. The interpedicular distances of the lumbar spine show progressive narrowing in the caudal direction. An exaggerated lumbar lordosis is present once walking begins. In addition, there are shortened pedicles and posterior vertebral body scalloping. The pelvis demonstrates flattened iliac wings with a characterisitc “champagne glass” appeareance along with a short sacrosciatic notch and decreased acetabular angle. The long bones have metaphysical flaring. The distal femoral physes may have an inverted-V configuration. The hands exhibit brachydactyly.