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This condition is the dilatation of the renal pelvis and calyces with accompanying destruction of the kidney parenchyma. Partial obstruction to the outflow of urine is usually the cause. This condition maybe congenital or acquired.

It may be unilateral or bilateral. Unilateral is encountered when the lesion is somewhere in the ureter, above the level of the urinary bladder. Bilateral hydronephrosis is seen when the obstruction is below the level of the urinary bladder like in stricture of the urethra, BPH and neuromuscular dysfunction of the internal sphincter. Bilateral hydronephrosis can also present when there is bilateral ureteric obstruction.

When there is no associated detectable cause it is called primary hydronephrosis. When the cause is detectable it is called secondary hydronephrosis; causes for which are as follows:

Since the ureter is most often the cause n more so the pelviuretric junction, the individual conditions can be split under the following:

v Unilateral hydronephrosis

· Extramural causes – pressure by loaded sigmoid colon, gravid uterus, uterine tumours, and ovarian tumours.

- Aberrant renal vessels

- Idiopathic retroperitoneal fibrosis

· Intramural causes- congenital stenosis or achalasia at the pelviureteric junction

- Uretrocele and congenital atretic ureteric orifice

- Inflammatory stricture

- Neoplasm’s of the ureter [mostly papilloma]

· Intraluminal causes – calculus of the ureter

- Congenital folds at the upper end of the ureter.

v Bilateral hydronephrosis

· In the urethra – pin-hole meatus

- Congenital valves

- BPH and carcinoma of the same

- Inflammatory stricture of the urethra

- Carcinoma of the cervix and uterus and rectum may involve the ureters to cause this.

· In the bladder- calculus

- Internal sphincter which may be unable to open due to neuromuscular dysfunction.

- Neoplasm’s of the bladder

What are the clinical features of hydronephrosis, how does it present?

1. - Unilateral- females usually affected more. In type 1- There is renal colic and haematuria. Type 2- Onset is insidious with dull aching pain and sense of weight on the affected side of the loin. These are usually experienced after excessive consumption of fluid and/or alcohol.

2. - Intermittent- here the patient complains of acute pain which is followed by the swelling in the loin. A few hours or even the following day there might be polyuria-sudden excessive voiding of urine; following which the pain is relieved and the swelling disappears. This is also known as “Dietl’s” crisis.

3. - Bilateral – in type 1- symptoms of causes like enlargement of prostate, or carcinoma.

In type 2- swelling in the loin on both sides is first complained of.

What investigations would one require to get done in order to diagnose the condition?

1. Straight X-ray.

2. Excretory urography (I.V.P)

3. Retrograde urography

4. Ultrasound

What are the available treatment options for this condition?

For the secondary variety of this condition: reverting or treating the primary cause generally is enough.

Indications for surgery:

- Increasing pain

- Increasing size of renal pelvis and calyces as seen in investigations

- When complicated by secondary infection.

When the hydronephrosis is extra renal the mode of surgery is mostly conservative [plastic operation]

When intrarenal- nephrectomy is preferred; but it must avoided as far as possible until it is absolutely required as sometimes the other kidney may get affected when the etiology is usually obscure. For mild to moderate cases careful follow up is the order of the day.

The post gives the general scheme of management of a patient coming into the emergency with status asthmaticus..the text gives only a general outline of the principles, the treatment modalities may be different in different parts of the world.the text is also meant to help medicos preparing for the board exam to write answers to the questions asked about the topic..

Introduction

Status Asthmatics(severe acute asthma)is the condition in which the attack of bronchial asthma is not relieved even on treatment with adrenaline and aminophylline.

Status Asthmaticus

1. All patients with asthma have the potential to develop STATUS ASTHMATICUS.
2. Severe at the onset or progresses rapidly despite routine therapy
3. May result in ventilatory failure and death

Precipitating factors

1. An acute respiratory infection
2. Abrupt omission of corticosteriods therapy
3. Drugs(NSAIDS) or inhaled allergens
4. Acute emotional stress

Clinical Presentation

Assessment of Severity
of Illness

STAGE 1 Can the patient be treated in OPD?

STAGE 2 Does the patient need admission to the hospital?

STAGE 3 Does the patient need admission to a critical care unit?

STAGE 4 Does the patient need intubation and mechanical ventilation?

Life Threatening Asthma

1. Silent chest
2. Cyanosis
3. Poor respiratory effort
4. Bradycardia or hypotension
5. Exhaustion, confusion or coma
6. PEF < 33% of best predicted
7. Normal or high paCO2 ( >36 mmHg)
8. Severe hypoxemia despite oxygen(< 60 mmHg)
9. Low pH

Bronchodilator Therapy for
Status Asthmaticus

1. Salbutamol and Ipatropium 0.5% by nebulization q15-20min,it is nebulised with oxygen in severe cases.
2. Epinephrine (1:1000) 0.3ml s.c. if needed.
3. Methylprednisolone 60-125mg iv q6h or prednisone 40mg orally q6h
4. Theophylline 5mg/kg i.v. over 30min loading dose followed by 0.4mg/kg/h

Indications for Mechanical Ventilation

1. Respiratory rate > 40 breaths/min
2. Climbing pulsus paradoxus
3. Falling pulsus paradoxus in the exhausted patient
4. Altered sensorium
5. Inability to speak
6. Patient’s subjective sense of exhaustion
7. Complicating barotrauma
8. Unresolving lactic acidosis
9. Diaphoresis in the recumbent position
10. Silent chest despite respiratory effort
11. Elevation of Pa_CO2 with progressive signs and symptoms

Management of the Patient on a Ventilator

1. The patient should be fully sedated and muscles relaxed
2. Small tidal volume (4-8ml/kg)
3. Fast inspiratory flow to reduce inspiratory time (Ti<1sec or flow 80-100L/min)
4. Ppeak < 55 cmH2O, Pplateau < 35cmH2O
5. Keep PaO2 60-100mmHg

Liberation from Mechanical Ventilation

1. It will require 24-48hs of aggressive bronchodilator and anti-inflammatory therapy until airway pressure fall.
2. As airway pressure fall and PaCO2 normalize , sedatives, muscle relaxants and bicarbonates should be withheld.
3. Change to spontaneous breathing mode
4. Assess respiratory muscle strength
5. Quick extubation à E-T tube itself may induce bronchospasm

REHYDRATION THERAPY

• Oral or parentral administration of 5% glucose – saline.It also makes the bronchial secretions less tenacious.
• Correction of acidosis by IV sodium bicarbonate

RECENT DIAGNOSIS

• Clinicopathological studies that 25% of cases have different diagnosis.
• Special proteins like tau,alpha synnuclein play a major role.
• Immunohistochemistry:LEWY BODIES –hallmark of parkinsonism

TAU PROTEIN DEPOSITS

• Hereditary factors : 13 different loci have been associated.
• Oxidative stress,impairment in mitochondrial complex 1 are also responsible.

SITE OF FIRST PATHOLOGICAL CHANGE.

• OLFACTORY NUCLEUSà
• VAGUS NERVE à
• LOWER BRAINSTEM NUCLEIà

• AMYGDALAà
• SUBSTANTIA NIAGRAà
• TEMPORAL LOBE

POSSIBLE GENETIC BASIS.

• 3 MAIN GENES ARE RESPONSIBLE

• GENE FOR SYNUCLEIN: shows amino acid substitution àaccumulation of this protein in LEWY BODIES àTissue destruction

• PARKIN GENE:Showing homozygous deletion.

• GENE FOR UBIQUITIN: missense mutations.

TREATMENT

• SURGERY
• STEM CELL CULTURE
• GENE THERAPY
• DRUGS

SURGERY

• PALLIDOTOMY by thermoregulation.
• THALOTOMY
• SUBTHALOTOMY
• DEEP BRAIN STIMULATION using a quadripolar electrode(100-180Hz)

STEM CELL TREATMENT

• First neurodegenerative disorder in which this technique was tried
• Aborted foetal cells were being transplanted
• Also porcine foetal cells were also being incorporated.

• DRAWBACKS: Immunoincomptability,ethical issues.

GENE THERAPY

• Cells like fibroblasts etc are modified.
• With the help of adeno associated vectors DOPAMINE inducing enzymes are incorporated into the modified cells.
• These are incorporated into the basal ganglia.

NEWER MEDICATIONS

• DOPAMINE AGONISTS:
• Not just as an adjuvent anymore.
• Neuroprotection of dopaminergic neurons as monotherapy.
• Egs; Bromocriptine,pergolide,mirapex,ropinerole

RULES

• Use only one at a time.
• Start at a lower dose.
• Evenly spaced 3 doses at a time.
• Slowly titrate the dose by doubling lowest dose no faster than every 1-2 weeks till beneficial effects/ intolerable side effects are seen.
• If dementia prexists Ropinerole

• COMT INHIBITORS
• Increases ½ life of L-Dopa,bioavailability.
• Egs;Tolacapone Entacapone.

Dopamine dysregulation syndrome.

1. Seen in a subgroup of people taking additional quantities of drug well beyound their dose.
2. This led to some behavioural changes &motor changes.