Osteochondritis is sometimes classified as-

1. Crushing type or Osteochondrosis
2. Osteochondritis dissecans
3. Tractions Osteochondritis or traction apophysistis

Common Osteochnodrites –

1. Perthes’ disease – Head of femur is affected
2. Panner’s disease- Capitulum affected
3. Kienbock’s disease- Lunate bone affected
4. Osgood-shlatter’s disease- Tibial tuberosity Osteochondritis
5. Sever’s disease- Calcaneal tuberosity Osteochondritis
6. Kohler’s disease- Navicular bone affected
7. Freiberg’s Disease- Metatarsal head affected
8. Scheurmann’s disease- Ring epiphyses of vertebra affected
9. Calve’s disease- Centralbody of nucleus of vertebral body affected.

Perthes’ Disease:-

Aka. Coxa Plana, Pseudocoxalgia

This is the Osteochondritis of the epiphysis of the femoral head. In the disease, the femoral head becomes partially or completely avascular and deformed as well.

Cause- not exactly known. ? recurrent ischemia of head of femur in susceptible age group. Usually precipitated by Synovitis

Age Group Susceptible- 5- 10 years                          Gender- Boys> Girls

Stages:

1. Stage of synovitis

2.Stage of Trabecular necrosis

3.Stage of healing

Presentation-

• Pain in the hip region, pain may radiate to knees.
• Hipstiffness and limping may be present
• On examination- not much findings, sometimes there is shortening of the affected limb, and the limb may be externally rotated and abducted.

X-Ray- Collapse and sclerosis od the epiphysis of the femoral head. Joint space increased- hip joint.

Bone scan- decreased uptake by femoral head.

Treatment-

Principle- preventing the Head from deforming in the softening phase, while keeping it in acetabulaum while revascularization takes place (Head Containment)

Methods- Plaster, special Braces, Splints, Operation- containment- osteotomy.

Further Reading:

AAOS

Patients Reading: patientcouk

March 21st every Year is Marked  as ” Down Syndrome Day” – 3rd month 21st

Karyotype-

1. Trisomy 21: 47 XX or XY +21 . Usually caused by an extra copy of Chromosome 21
2. Translocational type- 46 XX, der ( 14;21)(q10;q10),+21
3. Mosaic type- 46 XX/ 47 XX ,+21

Most common of the chromosomal disorders ( incidence 1 in 700 births).Risk increases with maternal age. Significant increase in risk with Maternal age above 40 years. 1:2000 in  under 25 years, 1: 300 at 35 years and 1:100 at 40 years. Co-relation with maternal age suggests that meiotic  non-disjunction of Chromosome 21 occurs in Ovum.

Male:Female ratio is 1.15:1 not much difference.

Pathogenesis

1. Meiotic non-dysjunction (95%)
2. Robertsonian translocation (4%)
3. Mosaicism due to miotic non-dysjunction during the embryogenesis. (1%) : Mosaics are people having 2 or more populations of cells within individuals.  They have variable features and milder.

Clinical features-

Severe mental retardation- most common cause of genetic mental retardation.  IQ is generally low to very low.

Facial  features :

• Mongoloid facies-flat face,low-bridged nose, epicanthal folds, mongoloid slant of eyes
• brachycephaly commonly , microcephaly, delayed closure of  fontanels, a patent metopic suture
• Absent frontal and sphenoid sinuses, and hypoplasia of the maxillary sinuses may be seen.
• low-set ears ,dysplastic ears-small ear with overfolded helix
• hypertelorism –wide set eyes
• high arched palate,open mouth with protruded and furrowed tongue ( macroglossia)

Click to view full size

Eyes-

• Brushfield spots-speckled appearance of the iris
• Cataract and squint sometimes
• Refractive error and nystagmus

Hands-

• Simian hand- short broad hands with short stubby fingers
• Simian Crease- Single palmar flexion crease.
• Clinodactyly -( Hypoplasia of the middle phalanx of 5^th finger producing incurving little finger)
• Dermatoglyphics-  more than 8 ulnar loops on fingers and distal axial triradii becomes obtuse.

Foot-

• Sandal gap- Increased gap between 1^st and 2^nd toe
• Sub-hallucial pad of fat
• Single deep longitudinal plantar crease

Cardiovascular System-Congenital heart defects- Endocardial cushion defect leads to formation of an atrioventricular canal ( a common connection between all 4 chambers), VSD, ASD, PDA

GI system – Duodenal atresia ( ‘ double bubble ‘ sign), Hirchsprung disease, tracheo-esophageal fistula, Imperforate anus.

Other System-

• Muscular hypotonia
• Broad short neck, Short height, Growth retardation
• Atlantoaxial subluxation with spinal cord compression <1 : A delay in recognizing atlantoaxial and atlanto-occipital instability may result in irreversible spinal-cord damage and add disability like visual and auditory loss.
• Infertility
• Personality- Cheerful, fond of Music.
• Premature Aging.
• Umbilcal hernia and diastasis rectii

Increase susceptibility to-

• Increased risk ( 15-20 X) of Acute Lymphoblastic Lymphoma- Trisomy 21, GATA 1 mutation and unknown genetic alternation.
• Alzheimer disease ( by age 40 virtually all will develop Alzheimer Disease)
• Coeliac disease may be associated .
• Increased susceptibility to infection (pneumonia, otitis media, sinusitis, pharyngitis, periodontal disease)
• Autoimmune hypothyroidism and Hashimoto disease
• Hyperuricemia, Diabetes Mellitus,

Prenatal Diagnosis-

1. In-utero:  Triple test using Maternal serum levels of alpha-fetoprotein, hCG, and unconjugated  estriol.
2. Ultrasonography- increased nuchal fold >4 mm, failure to visualize fetal nasal bone at age 11-13 weeks POG,femur and the humerus tend to be shortened.
3. Amniocentesis
4. Chorionic Villi sampling
5. Percutaneous umbilical blood sampling (PUBS)

Prognosis:

Down syndrome decreases prenatal viability and increases prenatal and postnatal morbidity as well. About 75%  with trisomy 21 die in fetal life.~ 85% of infants survive to age 1 year, and 50% can be expected to live longer than age 50 years. Down Syndrome is compatible to life . With good medical care , current median age at death is 47 years.

More Readings at:-

Down Syndrome: Prenatal Risk Assessment and Diagnosis
Prenatal Screening for Down Syndrome by Len Leshin, MD, FAA

Reference:

• Robbins and Cotran Pathological Basis of Disease
• Langman’s Medical embryology.
• USG measurement in down Syndrome
• Kundu Bedside Medicine
• Kaplan’s Lecture notes
• Medscape-Emedicine

Normally in each menstrual cycle 40-70 ml of bleeding occurs. Normally blood clots are also absent.
Menorrhagia is defined , if in cyclical bleeding at normal intervals , excessive bleeding of more than 80 ml occurs or cycle is of excessive duration or if both occurs. Menotaxis is a term used to define prolonged bleeding.

Medchrome Women's Health

Causes:
Organic:
Pelvic cause- Fibroid uterus ( Myoma), Adenomyosis, Pelvic endometriosis, Chronic tubo-ovarian mass, IUCD in utero, Tubercular endometritis, Retroverted gravid uterus, Granulosa cell tumor of Ovary.
Systemic- Liver failure, Congestive cardiac failure, Severe hypertension.
Endocrinal- Hypothyroid and Hyperthyroid states.
Blood dyscrasias: ITP, Leukemia, von-Willebrand’s disease.
Emotional upset- Functional component.

Commonest causes are:-
• DUB- Dysfunctional Uterine Bleeding
• Fibroid
• Adenomyosis
• Chronic tubo-ovarian mass

Treatment:
According to the Cause

Absolute:
1) Severe fluid overload with CCF
2) Severe hyperkalemia unresponsive to medical Rx or with ECG changes
3) Severe hyponeatrmia
4) Server metabloic acidosis requiring > 2 doses of NaHCO3 (2mEq/kg/dose)
5) Uraemia
6) Fluid restriction curtailling ample nutrition

Relative:
1) Anuria
2) High urea and creatinine values alone are not indications

METHOD
Materials:
1) Pertioneal dialysis tubing, trocar and cannula
2) Yellow intracath #14 gauge needle – 12 –24 inches long
3) Cut sown set with narrow blade
4) 2/0 silk on a straight needle
5) peritoneal dialysis fluid 1.5% dianeal (warm the fluid by placing in container of warm water
6) Lignocaine, Iodine, alcohol, mask and sterile gloves

Method: A
1) Consent form to be signed
2) Cross match 1 unit blood
3) Check PT, PTT platelets beforehand
4) Insert urethral catheter to empty bladder
5) Cleanse abdomen with Iodine and alcohol
6) Set up dialysis bags and attach to tubing (fluid should be tepid– not hot and not cold)
7) Determine, before dialysis starts, the volume to be run in and mark out on bag (See Figure 1).
The markings are not exactly in the same position on all bags, so this method is only an
approximation. If more accurate measurement is needed, a Biuretrol must be attached to the
dialysis bag
8 ) Fill tubing with fluid
9) If the abdomen is not tense with ascites, by infusing dialysis fluid until adequate tension is
present one reduces the risk of damaging internal structures eg aorta, inferior vena cava and
bowel. Proceed to step B
10) If the abdomen is already tense with ascites, proceed to step C

B

The procedure is as follows:
1) Remove guide wired from intracath
2) Set up tubing to dialysis fluid
3) Local anaesthetic to skin – superficial and deep at area of proposed intracath insertion
Direct the needle of the intracath downwards gently but firmly
When you feel the “pop” thread the tubing and withdraw the needle around it
Push tubing in as far as it will go – do not press deeply with the needle
4) Run in 30-50 cc/kg of dialysis fluid – sufficient to make abdomen tense
5) Withdraw intracath
6) Proceed to insertion of peritoneal dialysis catheter

C
Insertion of the peritoneal dialysis cannula:
1) Site of catheter – approximately 2 -3 cm inferior to the umbilicus and in the midline
2) Make a small incision at this point with the pointed end of the blade to include skin and subcutaneous
tissues – just big enough for catheter
Figure 1 – Dialysis bags and volume markings
85
3) Hold peritoneal dialysis catheter – with pointed trocar inside. Hand on top of catheter pushing
downwards firmly
4) When you feel the catheter enter the peritoneal cavity (a pop), remove the trocar (pointed internal
metal rod) and thread cannula (plastic part around the trocar) into peritoneal cavity quickly aiming the
cannula to the right or left iliac fossa (R preferably). Push in as far as it will go
5) Connect cannula (dialysis catheter ) to “elbow” and thus the remainder of tubing (already primed with
fluid)
6) Run out fluid from cavity into tubing – should run as a steady stream
7) Pour in expected exchange volume
a) 40-60cc/kg infants and small children
b) 30-40cc/kg older children
Limiting factors – abdominal discomfort, peritoneal leaks
If fluid runs in and out quickly after 3 rapid exchanges – without dwells – put purse string suture
around cannula – tie tightly, fasten securely at the level of the skin then tie 3-4 knots up the side of the
catheter with the same uncut suture

DIALYSIS
Cycles: Usually best clearance with 30 minute cycles :
• In over 5 minutes
• Dwell over 20 minutes
• Drain over 5 minutes
• Tepid fluid
Clearance: Urea > K > Cl > Na > Cr > PO4 > uric acid > HCO3 > Ca > Mg
Dianeal composition: Na 132 K 0 Ca 3.25 Mg 1.3 Cl 101.75 Lactate 35 mmol/l
Rate of water removal (assuming adequate drainage) depends on [glucose] in dialysis fluid
• 1.5% dianeal (1.5% glucose) usually adequate, but may increase to 2.5% or 4.25% if inadequat fluid
removal. If there is no pre-mixed 2.5 % or 4.25% dianeal, they can be prepared as follows
• to make 2.5% from 1.5% – add 40cc of D 50 W / 2 liters of fluid of 1.5% dianeal
• to make 4.25% from 1.5% – remove 110 cc fluid from 2 litre bag of 1.55 dianeal. Add 110 cc of
D50W to bag

ADDITIVES
1) KCl 6 mEq / 2 liters added when serum K <4.0mEq/l
- may be increased in increments of 2-4 mEq/2l bag depending on serum K
2) No antibiotics prophylatically
3) Heparin 1000 units /2 litres of dialysis fluid in all bags to minimize formation of fibrin strands

PERITONITIS
Initial broad spectrum cover for peritonitis.

Empiric therapies:
Intraperitoneal –
1) Cloxacillin 200mg/ 2litres + Gentamycin 10 mg /2 litres (regime used at UHWI with success despite
potential for inactivation when aminoglycosides mixed with penicillins) – Staph is commonest
pathogen for peritonitis here
2) Cefotaxime – 500mg/2 litres
Adjust when sensitivities available

Taken from “ Consensus guidelines for the treatment of peritonitis in pediatric patients receiving peritoneal dialysis” – Peritoneal Dialysis International Vol. 20: 610 – 624

PRECAUTIONS
• Daily peritoneal fluid: c/s, gram stain, cell count. > 100 WBC / ml and > 50% neutrophils suggests
peritonits. – start Rx as soon as sample taken for culture. Fluid is aspirated from the porthole (rubber
bung in tube in the dialysis line near patient entry) with a 25 gauge needle attached to a sterile syringe.
Clean the porthole with Iodine and wrap with Iodine soaked gauze for 10 minutes prior to inserting the
needle, in order to avoid iatrogenic peritonitis.*
• Nurse is asked to call if problems arise (see below)

PROBLEM SOLUTION

Change PD catheter if unable to get good drainage or inflow despite the above measures. Always flush the
tubing with about 20 –50 cc of fluid as catheter is being withdrawn to minimize possibility of omentum
coming up in the catheter as it is being removed. Discontinue dialysis when urine output has improved
sufficiently that the original indications for dialysis are unlikely to recur off dialysis (not just when the lab
results are normal on dialysis

Source- Manual of Nephrology for DM

References:
1) ISPD guidelines / recommendations. Consensus guidelines for the treatment of peritonitis in pediatric
patients receiving peritoneal dialysis. Warady, Schaefer et al. Peritoneal Dialysis International 2000.
20:610 – 624.
2) Paediatric Nephrology (1997) 1: 183 – 194 (for drug dose adjustments)
3) Pediatric Nephrology 15th Edition (1999) Barratt and Vernier Chapter 69 (1125 – 1126)